Special distraction osteogenesis before bone grafting for alveolar cleft defects to correct maxillary deformities in patients with bilateral cleft lips and palates: distraction osteogenesis performed separately for each bone segment.

PubMed

Mitsukawa, Nobuyuki; Saiga, Atsuomi; Morishita, Tadashi; Satoh, Kaneshige

2014-07-01

Patients with bilateral cleft lips and palates have premaxillary protrusion and characteristic jaw deformities involving three-dimensional malposition of the premaxilla and bilateral maxillary bone segments. This study examined patients with bilateral cleft lips and palates who had deviation and hypoplasia of the premaxillas and bilateral maxillary segments. Before bone grafting, the patients were treated with special distraction performed separately for each bone segment using a halo-type external device. This report describes this novel treatment method which produced good results. The subjects were five patients with severe jaw deformities due to bilateral cleft lip and palate. They were treated with maxillary Le Fort I osteotomy and subsequent distraction performed separately for each bone segment using a halo device. In three of five patients, premaxillary osteotomy was not performed, and osteotomy and distraction were performed only for the right and left lateral segments with severe hypoplasia. All patients achieved distraction close to the desired amount. The widths of the alveolar clefts were narrowed, and satisfactory occlusion and maxillary arch form were achieved. After the surgery, three of five patients underwent bone grafting for bilateral alveolar cleft defects and the bone graft survival was satisfactory. This method had many benefits, including narrowing of alveolar clefts, improvement of maxillary hypoplasia, and achievement of a good maxillary arch form. In addition, subsequent bone grafting for alveolar cleft defects was beneficial, dental prostheses were unnecessary, and frequency of surgery and surgical invasiveness were reduced. This method is a good surgical procedure that should be considered for patients with bilateral cleft lips and palates who have premaxillary protrusion and hypoplasia of the right and left lateral segments. Copyright © 2013 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

A 12-year anthropometric evaluation of the nose in bilateral cleft lip-cleft palate patients following nasoalveolar molding and cutting bilateral cleft lip and nose reconstruction.

PubMed

Garfinkle, Judah S; King, Timothy W; Grayson, Barry H; Brecht, Lawrence E; Cutting, Court B

2011-04-01

Patients with bilateral cleft lip-cleft palate have nasal deformities including reduced nasal tip projection, widened ala base, and a deficient or absent columella. The authors compare the nasal morphology of patients treated with presurgical nasoalveolar molding followed by primary lip/nasal reconstruction with age-matched noncleft controls. A longitudinal, retrospective review of 77 nonsyndromic patients with bilateral cleft lip-cleft palate was performed. Nasal tip protrusion, alar base width, alar width, columella length, and columella width were measured at five time points spanning 12.5 years. A one-sample t test was used for statistical comparison to an age-matched noncleft population published by Farkas. All five measurements demonstrated parallel, proportional growth in the treatment group relative to the noncleft group. The nasal tip protrusion, alar base width, alar width, columella length, and columella width were not statistically different from those of the noncleft, age-matched control group at age 12.5 years. The nasal tip protrusion also showed no difference in length at 7 and 12.5 years. The alar width and alar base width were significantly wider at the first four time points. This is the first study to describe nasal morphology following nasoalveolar molding and primary surgical repair in patients with bilateral cleft lip-cleft palate through the age of 12.5 years. In this investigation, the authors have shown that patients with bilateral cleft lip-cleft palate treated at their institution with nasoalveolar molding and primary nasal reconstruction, performed at the time of their lip repair, attained nearly normal nasal morphology through 12.5 years of age.

Velopharyngeal function of patients with cleft palate after primary palatoplasty: relevance of sex, age, and cleft type.

PubMed

Yang, Yunqiang; Li, Yang; Wu, Yeke; Gu, Yifei; Yin, Heng; Long, Hu; Shi, Bing; Zheng, Qian

2013-05-01

The aim of this study was to investigate the relevance of sex, age, and cleft type to velopharyngeal function after primary Sommerlad palatoplasty so as to improve velopharyngeal function after the procedure. Records of 503 patients with nonsyndromic cleft palate after primary Sommerlad palatoplasty were included in the retrospective study. Relevance between their velopharyngeal function and sex, age, and cleft type was analyzed. Statistical analysis was performed using SPSS 13.0 (SPSS Inc., Chicago, IL). There were no significant differences of velopharyngeal competence (VPC) rates between different sexes (P = 0.635). Specifically, VPC rates were significantly higher in younger-than-2-years groups than in older age groups (P < 0.05) and significantly lower in 6-years-or-older group (P < 0.05). No differences were found among 2- to 6-year-old groups (P > 0.05). The VPC rates were significantly lower in the bilateral complete cleft palate and the unilateral complete cleft palate than in the incomplete cleft palate before 2 years old (P < 0.05), whereas there were no significant differences totally (P = 0.875). Results showed that the disparity of the VPC rate among different cleft types would decrease with age. Moreover, results of multivariate logistic regression also indicated that operation age and cleft type are factors influencing velopharyngeal function. Primary palatoplasty should be completed before 2 years old, and the postoperative velopharygeal function will greatly decreases after 6 years old. The influence of cleft type on velopharyngeal function is limited to young patients. For those who have missed the best surgical timing, appropriate delay of operation age is reasonable, especially for patients with complete cleft palate. For patients 4 to 6 years old, the first choice is still simple palatoplasty no matter which cleft type they are classified into.

Facial profile esthetics in operated children with bilateral cleft lip and palate

PubMed Central

Lauris, Rita de Cássia Moura Carvalho; Capelozza, Leopoldino; Calil, Louise Resti; Lauris, José Roberto Pereira; Janson, Guilherme; Garib, Daniela Gamba

2017-01-01

ABSTRACT Objective: The aim of this study was to evaluate the facial profile esthetics of rehabilitated children with complete bilateral cleft lip and palate (BCLP), comparing the judgment of professionals related and not related to cleft rehabilitation and laypersons. Methods: Thirty children in the mixed dentition (24 male; 6 female) with a mean age of 7.8 years were evaluated using facial profile photographs by 25 examiners: 5 orthodontists and 5 plastic surgeons with experience in cleft care, 5 orthodontists and 5 plastic surgeons without experience in oral cleft rehabilitation and 5 graduated laymen. Their facial profiles were classified into esthetically unpleasant (grade 1 to 3), esthetically acceptable (grade 4 to 6), and esthetically pleasant (grade 7 to 9). Intraexaminer and interexaminer errors were evaluated using Spearman correlation coefficient and Kendall’s test, respectively. Inter-rater differences were analyzed using Friedman test and Student-Newman-Keuls test for multiple comparisons. Results: Orthodontists dealing with oral clefts rehabilitation considered the majority of the sample as esthetically pleasant. Plastic surgeons of the cleft team and laypersons classified most of the sample as esthetically acceptable. Most of the orthodontists and plastic surgeons not related to cleft care evaluated the facial profile as esthetically unpleasant. The structures associated to unpleasant profiles were the nose, the midface and the upper lip. Conclusions: The facial profile of children with BCLP was classified as esthetically acceptable by laypersons. Professionals related to cleft rehabilitation were more lenient and those not related to cleft care were stricter to facial esthetics than laypersons. PMID:28902248

Parental satisfaction in Ugandan children with cleft lip and palate following synchronous lip and palatal repair.

PubMed

Luyten, Anke; D'haeseleer, Evelien; Budolfsen, Dorte; Hodges, Andrew; Galiwango, George; Vermeersch, Hubert; Van Lierde, Kristiane

2013-01-01

The purpose of the present case control study was to assess parental satisfaction with speech and facial appearance in Ugandan children with complete unilateral or bilateral cleft lip and palate (CLP), who underwent a synchronous lip and palatal closure. The results are compared with an age- and gender-matched control group. The experimental group consisted of the parents or guardians of 44 Ugandan patients (21 males, 23 females) with complete unilateral or bilateral CLP (mean age: 3;1 years). The control group included the foster mothers of 44 orphan children matched by age and gender (mean age: 3;7 years). A survey based on the Cleft Evaluation Profile was used to assess the perceived satisfaction for individual features related to cleft care. Overall high levels of satisfaction were observed in the experimental group for all features (range: 56-100%). No significant differences could be established regarding age, gender, age of lip and palatal closure, cleft type or maternal vs. paternal judgments. In participants who were dissatisfied with the appearance of the lip, the time period between the cleft closure and the survey was significantly larger compared with satisfied participants. Furthermore, significantly lower levels of satisfaction were observed in the cleft group for speech and the appearance of the teeth and the nose compared with the control group. Satisfaction with speech and facial appearance in Ugandan children with cleft lip and/or palate is important since normal esthetics and speech predominantly determine the children's social acceptance in the Ugandan society. As a result of reading this manuscript, the reader will be able to explain the attitudes of parents toward the surgical repair of their children's cleft lip and palate. As a result of reading this manuscript, the reader will be able to identify differences in parental attitudes toward synchronous lip and palate repair. Copyright © 2013 Elsevier Inc. All rights reserved.

Assessing Angle's malocclusion among cleft lip and/or palate patients in Jammu.

PubMed

Gupta, Akshay; Gupta, Anur; Bhardwaj, Amit; Vikram, S; Gomathi, Ajeetha; Singh, Karanprakash

2016-04-01

The study was conducted to examine the patients with abnormalities of cleft lip and/or palate and its association with different types of malocclusion. This descriptive study was done among 168 patients with abnormalities of cleft lip and/or palate. Angle's classification of malocclusion was applied for assessment of occlusion as Class I, Class II, and Class III. The types of oral clefts classification such as cleft lip unilateral and cleft lip bilateral, cleft palate (CP), unilateral cleft lip with palate (UCLP) and bilateral cleft lip with palate (BCLP) was considered. Chi-square test was applied to analyze the data at P < 0.05. The study showed different categories of clefts patients as cleft lip (81), CP (31), and both cleft lip and palate (53). The occurrence of unilateral cleft lip (44) was maximum among the sample followed by UCLP (39), and bilateral cleft lip (31). Maximum subjects with Class II (10.7%) and Class III (4.9%) malocclusion were seen with unilateral cleft lip deformities. None of the patients with UCLP had Class III malocclusion. Cleft lip was the most commonly observed deformity and high frequency of Class II and III malocclusion was evident. Therefore, patients with such abnormalities should be screened timely.

Prevalence of dental anomalies in patients with cleft lip and palate.

PubMed

Eslami, Neda; Majidi, Mohammad Reza; Aliakbarian, Majid; Hasanzadeh, Nadia

2013-09-01

The aim of the present study was to investigate the prevalence of dental anomalies in a group of patients with cleft lip and palate (CL/P) in the northeast of Iran. Ninety-one patients referring to the Cleft Lip and Palate Clinic of Mashhad Dental School were enrolled and classified into right CL/P, left CL/P, and bilateral CL/P groups. Photographs, dental casts, and panoramic and periapical radiographs were retrieved, and dental anomalies were recorded. χ test was used to analyze the frequency of dental anomalies according to type of cleft and sex. Missing maxillary lateral incisors was the most frequent dental anomaly, which was slightly higher in the bilateral group (61.1%). There were significantly more cases of missing lateral incisors outside the cleft area in right CL/P (P = 0.015). Peg lateral incisors were observed in 33.3% of bilateral CL/P compared with 28% of right and 23.3% of left unilateral cases. The sample presented rotations of central incisors in the cleft area in 33.3% of bilateral clefts. In unilateral clefts, it occurred more frequently in the right side (48%). Sexual dimorphism appeared only for maxillary central incisor rotation in the cleft area, which showed significantly greater frequency in females (P = 0.025). Transposition of maxillary canine and first premolars was found in 5.5% of bilateral, 8% of right, and 3.3% of left unilateral clefts. The prevalence of dental anomalies in the studied sample seems to be higher than that reported in the normal population. More anomalies were observed at the cleft side. The frequency of most anomalies was not significantly different between the 2 sexes.

Unfavourable results in the repair of the cleft lip

PubMed Central

Narayanan, Puthucode V.; Adenwalla, Hirji Sorab

2013-01-01

Introduction: Unfavorable results in unilateral and bilateral cleft lip repair are often easy to spot but not always easy to prevent as to treat. We have tried to deal with the more common problems and explain possible causes and the best possible management options from our experience. Unilateral cleft lip repair: Unfavorable results immediately after repair involve Dehiscence and Scaring. Delayed blemishes include vermillion notching, a short lip, deficiency in the height of the lateral vermillion on the cleft side, white roll malalignment, oro-vestibular fistula, the cleft lip nose deformity, a narrow nostril and a “high-riding” nostril. We analyze the causes of these blemishes and outline our views regarding the treatment of these. Bilateral cleft lip: Immediate problems again include dehiscence as also loss of prolabium or premaxilla. Delayed unfavorable results are central vermillion deficiency, a lip that is too tight, bilateral cleft lip nose deformity, problems with the premaxilla and maxillary growth disturbances. Here again we discuss the causation of these problems and our preferred methods of treatment. Conclusion: We have detailed the significant unfavorable results after unilateral and bilateral cleft lip surgery. The methods of treatment advocated have been layer from our own experience. PMID:24501453

Modified Activation Technique for Nasal Stent of Nasoalveolar Molding Appliance for Columellar Lengthening in Bilateral Cleft Lip/Palate.

PubMed

Patil, Pravinkumar G; Nimbalkar-Patil, Smita P

2018-01-01

Bilateral cleft lip/cleft palate is associated with nasal deformities typified by a short columella. The presurgical nasoalveolar molding (NAM) therapy approach includes reduction of the size of the intraoral alveolar cleft as well as positioning of the surrounding deformed soft tissues and cartilages. In a bilateral cleft patient, NAM, along with columellar elongation, eliminates the need for columellar lengthening surgery. Thus the frequent surgical intervention to achieve the desired esthetic results can be avoided. This article proposes a modified activation technique of the nasal stent for a NAM appliance for columellar lengthening in bilateral cleft lip/palate patients. The design highlights relining of the columellar portion of the nasal stent and the wire-bending of the nasal stent to achieve desirable results within the limited span of plasticity of the nasal cartilages. With this technique the vertical taping of the premaxilla to the oral plate can be avoided. © 2016 by the American College of Prosthodontists.

Complete trisomy 9 with unusual phenotypic associations: Dandy-Walker malformation, cleft lip and cleft palate, cardiovascular abnormalities.

PubMed

Tonni, Gabriele; Lituania, Mario; Chitayat, David; Bonasoni, Maria Paola; Keating, Sarah; Thompson, Megan; Shannon, Patrick

2014-12-01

Trisomy 9 is a rare chromosomal abnormality usually associated with first-trimester miscarriage; few fetuses survive until the second trimester. We report two new cases of complete trisomy 9 that both present unusual phenotypic associations, and we analyze the genetic pathway involved in this chromosomal abnormality. The first fetus investigated showed Dandy-Walker malformation, cleft lip, and cleft palate) at the second trimester scan. Cardiovascular abnormalities were characterized by a right-sided, U-shaped aortic arch associated with a ventricular septal defect (VSD). Symmetrical intrauterine growth restriction and multicystic dysplastic kidney disease were associated findings. The second fetus showed a dysmorphic face, bilateral cleft lip, hypoplastic corpus callosum, and a Dandy-Walker malformation. Postmortem examination revealed cardiovascular abnormalities such as persistent left superior vena cava draining into the coronary sinus, membranous ventricular septal defect, overriding aorta, pulmonary valve with two cusps and three sinuses, and the origin of the left subclavian artery distal to the junction of ductus arteriosus and aortic arch. Complete trisomy 9 may result in a wide spectrum of congenital abnormalities, and the presented case series contributes further details on the phenotype of this rare aneuploidy. Copyright © 2014. Published by Elsevier B.V.

Premaxillay retraction in bilateral complete cleft lip and palate with custom made orthopaedic plate having anterior acrylic ring.

PubMed

Raffat, Arsalah; Ijaz, Abida

2009-06-01

To determine retraction and alignment of protruded and rotated premaxillary segment and to measure increase in columellar length by using modified orthopaedic plate with anterior acrylic ring in newborn babies with non syndromic bilateral cleft lip and palate. Pre surgical infant orthopaedic treatment of ten non syndromic bilateral cleft lip and palate new born babies was done. Modified orthopaedic plate with anterior acrylic ring was used. This was adjusted every week by adding increments of 1 mm acrylic along the inner surface contacting labial surface of pre maxillary segment and trimming the same amount along the anterior margin of the plate. Pre and post treatment dental cast and a 1:1 photocopy of dental cast was obtained to analyze the retraction, angulations and arch form. Data was compiled and analyzed in SPSS 10. A significant pre maxillary retraction, correction of rotation as well as increase of columellar length was achieved. The appliance proved most successful being used immediately after birth, making use of plasticity of the neonate cartilage due to maternal estrogen levels. Moreover the appliance maintained arch form facilitated function and rendered initial lip repair easy and tension free.

Effects of Nasoalveolar Molding Therapy on Alveolar and Palatal Cleft Deformities in Unilateral and Bilateral Cleft Lip and Palate.

PubMed

Isik Aslan, Belma; Gülşen, Ayşe; Findikçioğlu, Kemal; Uzuner, Deniz; Üçüncü, Neslihan

2018-03-01

The purpose of this study was to evaluate and compare the efficacy of nasoalveolar molding (NAM) therapy in the improvement of alveolar and palatal cleft deformity on unilateral (UCLP) versus bilateral (BCLP) cleft lip and palate. A total of 19 UCLP (14 boys and 5 girls) and 8 BCLP (7 boys and 1 girl) infants completed NAM therapy were included in this study. Standardized parameters of cleft width of alveol and palate were measured on photocopies taken from the pre- and post-treatment plaster casts. To assess the intragroup differences, paired-samples test in UCLP and Wilcoxon test in BCLP groups were used. Mann-Whitney U test was used to evaluate the differences between the groups. Significant decreases in alveolar and palatal cleft gaps were achieved in an average period of 3.3 ± 1.9 and 3.7 ± 1.6 months in UCLP and BCLP patients, respectively. The alveolar cleft decreased 7.85 ± 4.59 mm in UCLP and 4.25 ± 3.13 and 3.81 ± 3.50 mm in right and left alveolar cleft sides in BCLP, respectively. The palatal clefts were decreased 4.63 ± 2.44 mm in medial and 3.72 ± 2.62 mm in posterior parts in UCLP. The decrements in BCLP were 3.00 ± 2.75 mm in medial and 2.88 ± 2.75 mm in posterior palatal cleft distances. No significant differences were determined in the amount of alveolar or palatal cleft closure between UCLP and BCLP groups. The only decrease in arch width was 1.39 mm in medial part of palate in UCLP. The NAM device provides significant decreases in both alveolar and palatal cleft deformities in UCLP and BCLP infants, as compared with their birth status.

Branchial anomalies in children.

PubMed

Bajaj, Y; Ifeacho, S; Tweedie, D; Jephson, C G; Albert, D M; Cochrane, L A; Wyatt, M E; Jonas, N; Hartley, B E J

2011-08-01

Branchial cleft anomalies are the second most common head and neck congenital lesions seen in children. Amongst the branchial cleft malformations, second cleft lesions account for 95% of the branchial anomalies. This article analyzes all the cases of branchial cleft anomalies operated on at Great Ormond Street Hospital over the past 10 years. All children who underwent surgery for branchial cleft sinus or fistula from January 2000 to December 2010 were included in this study. In this series, we had 80 patients (38 female and 42 male). The age at the time of operation varied from 1 year to 14 years. Amongst this group, 15 patients had first branchial cleft anomaly, 62 had second branchial cleft anomaly and 3 had fourth branchial pouch anomaly. All the first cleft cases were operated on by a superficial parotidectomy approach with facial nerve identification. Complete excision was achieved in all these first cleft cases. In this series of first cleft anomalies, we had one complication (temporary marginal mandibular nerve weakness. In the 62 children with second branchial cleft anomalies, 50 were unilateral and 12 were bilateral. In the vast majority, the tract extended through the carotid bifurcation and extended up to pharyngeal constrictor muscles. Majority of these cases were operated on through an elliptical incision around the external opening. Complete excision was achieved in all second cleft cases except one who required a repeat excision. In this subgroup, we had two complications one patient developed a seroma and one had incomplete excision. The three patients with fourth pouch anomaly were treated with endoscopic assisted monopolar diathermy to the sinus opening with good outcome. Branchial anomalies are relatively common in children. There are three distinct types, first cleft, second cleft and fourth pouch anomaly. Correct diagnosis is essential to avoid inadequate surgery and multiple procedures. The surgical approach needs to be tailored to the type of anomaly of origin of the anomaly. Complete excision is essential for good outcomes. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

Ocular Manifestations of Oblique Facial Clefts

PubMed Central

Ortube, Maria Carolina; Dipple, Katrina; Setoguchi, Yoshio; Kawamoto, Henry K.; Demer, Joseph L.

2014-01-01

Introduction In the Tessier classification, craniofacial clefts are numbered from 0 to 14 and extend along constant axes through the eyebrows, eyelids, maxilla, nostrils, and the lips. We studied a patient with bilateral cleft 10 associated with ocular abnormalities. Method Clinical report with orbital and cranial computed tomography. Results After pregnancy complicated by oligohydramnios, digoxin, and lisinopril exposure, a boy was born with facial and ocular dysmorphism. Examination at age 26 months showed bilateral epibulbar dermoids, covering half the corneal surface, and unilateral morning glory anomaly of the optic nerve. Ductions of the right eye were normal, but the left eye had severely impaired ductions in all directions, left hypotropia, and esotropia. Under anesthesia, the left eye could not be rotated freely in any direction. Bilateral Tessier cleft number 10 was implicated by the presence of colobomata of the middle third of the upper eyelids and eyebrows. As the cleft continued into the hairline, there was marked anterior scalp alopecia. Computed x-ray tomography showed a left middle cranial fossa arachnoid cyst and calcification of the reflected tendon of the superior oblique muscle, trochlea, and underlying sclera, with downward and lateral globe displacement. Discussion Tessier 10 clefts are very rare and usually associated with encephalocele. Bilateral 10 clefts have not been reported previously. In this case, there was coexisting unilateral morning glory anomaly and arachnoid cyst of the left middle cranial fossa but no encephalocele. Conclusions Bilateral Tessier facial cleft 10 may be associated with alopecia, morning glory anomaly, epibulbar dermoids, arachnoid cyst, and restrictive strabismus. PMID:20856062

Rehabilitation of a One-day-Old Neonate with Cleft Lip and Palate using Palatal Obturator: A Case Report.

PubMed

Bansal, Rajesh; Kumar Pathak, Ashish; Bhatia, Baldev; Gupta, Sailesh; Kumar Gautam, Keshav

2012-05-01

Feeding a neonate with a complete cleft lip and palate is difficult pursuit due to communication between oral cavity and nasal cavity. A multidisciplinary approach is required to manage the complex problems involved in case of such neonates and their families. Present case is of a 1-day-old neonate having complete bilateral cleft lip and palate for which palatal obturator was constructed. A stepwise simple, easy and uncomplicated procedure for making accurate impressions, maxillary cast and fabrication of palatal obturator in infants with cleft lip and palate has been presented. The objective to present this case report is to emphasize the fact that how these palatal obturators /plates help in feeding, speech/language development, presurgical orthopedics and prevent other associated otorhinolaryngeal problems. How to cite this article: Bansal R, Pathak AK, Bhatia B, Gupta S, Gautam KK. Rehabilitation of a One-day Old Neonate with Cleft Lip and Palate using Palatal Obturator: A Case Report. Int J Clin Pediatr Dent 2012;5(2):145-147.

Siamese twins with craniofacial duplication and bilateral cleft lip/palate in a ceramic representation of the Chimú culture (Peru): a comparative analysis with a current case.

PubMed

Pachajoa, Harry; Hernandez-Amaris, Maria F; Porras-Hurtado, Gloria Liliana; Rodriguez, Carlos A

2014-06-01

Craniofacial duplication or diprosopus is a very rare malformation that is present in approximately 0.4% of conjoined twins. Here is presented a case of craniofacial duplication in association with bilateral cleft lip/palate in both heads found in a ceramic representation from the early Chimú culture from Peru. A comparative analysis is made with a current case of a 28-week-old fetus with similar characteristics. After reviewing the medical literature on conjoined twins, very few reports of facial cleft in both twins were found, with no reports at all of bilateral cleft lip/palate. This ceramic crock is considered one of the first representations suggestive of craniofacial duplication, and probably the first reporting it in association with facial cleft.

An Intraoral Appliance to Retract the Protrusive Premaxilla in Bilateral Cleft Lip Patients Presenting Late for Primary Lip Repair.

PubMed

Vura, Nandagopal; Gaddipati, Rajasekhar; Palla, Yudhistar; Kumar, Pranay

2018-04-01

The protruding and deviated premaxilla in bilateral complete cleft lip and palate (BCL/P) patients causes a perplexing problem in achieving a tension-free primary lip closure. An innovative noninvasive active intraoral Fixed Pre-Surgical Cleft Orthopedic (FPSCO) appliance is presented that has enabled to successfully reposition the premaxillary segment to a more favorable position prior to the surgical repair in 4 BCL/P patients, who reported late for treatment with a mean age of 18 ± 3 months. All the patients had at least 1 deciduous tooth erupted in 3 cleft segments, 1 premaxilla (PM), and 2 lateral maxillary (LM) segments. This innovative approach is a noninvasive method of getting the cleft segments closer prior to chelioplasty, where conventional presurgical nasoalveolar molding (PNAM) is not possible, which needs to be done early in life as it requires circulating maternal estrogen levels to achieve results preferably possible in patients younger than 3 to 6 months. Bonding to erupted teeth with glass ionomer cement was implemented to secure the appliance. Nickel-titanium (Ni-Ti) closed-coil springs were used that delivered continuous forces of 150 g/5 oz per side to retract the premaxillary segment. The mean active period of treatment time was 3.5 ± 1.6 months and the retention period was 1.2 ± 0.6 months, following which cheiloplasty was performed.

Surgeons’ Assessment of Presurgical Clefts and Predicted Surgical Outcome in Patients Treated with and without Nasoalveolar Molding

PubMed Central

Rubin, Marcie S.; Clouston, Sean; Ahmed, Mohammad M.; Lowe, Kristen; Shetye, Pradip R.; Broder, Hillary L.; Warren, Stephen M.; Grayson, Barry H.

2014-01-01

Obtaining an aesthetic and functional primary surgical repair in patients with complete cleft lip and palate (CLP) can be challenging due to tissue deficiencies and alveolar ridge displacement. This study aimed to describe surgeons’ assessments of presurgical deformity and predicted surgical outcomes in patients with complete unilateral and bilateral CLP (UCLP and BCLP, respectively) treated with and without nasoalveolar molding (NAM). Cleft surgeon members of the American Cleft Palate-Craniofacial Association completed online surveys to evaluate 20 presurgical photograph sets (frontal and basal views) of patients with UCLP (n=10) and BCLP (n=10) for severity of cleft deformity, quality of predicted surgical outcome, and likelihood of early surgical revision. Five patients in each group (UCLP and BCLP) received NAM and five did not receive NAM. Surgeons were masked to patient group. Twenty-four percent (176/731) of surgeons with valid email addresses responded to the survey. For patients with UCLP, surgeons reported that for NAM-prepared patients 53.3% had minimum severity clefts, 58.9% were anticipated to be among their best surgical outcomes, and 82.9% were unlikely to need revision surgery. For patients with BCLP, these percentages were 29.8%, 38.6%, and 59.9%, respectively. Comparing NAM to non-NAM prepared patients showed statistically significant differences (p < 0.001), favoring NAM-prepared patients. This study suggests that cleft surgeons assess NAM-prepared patients as more likely to have less severe clefts, to be among the best of their surgical outcomes, and to be less likely to need revision surgery when compared to patients not prepared with NAM. PMID:25534051

Assessment of presurgical clefts and predicted surgical outcome in patients treated with and without nasoalveolar molding.

PubMed

Rubin, Marcie S; Clouston, Sean; Ahmed, Mohammad M; M Lowe, Kristen; Shetye, Pradip R; Broder, Hillary L; Warren, Stephen M; Grayson, Barry H

2015-01-01

Obtaining an esthetic and functional primary surgical repair in patients with complete cleft lip and palate (CLP) can be challenging because of tissue deficiencies and alveolar ridge displacement. This study aimed to describe surgeons' assessments of presurgical deformity and predicted surgical outcomes in patients with complete unilateral and bilateral CLP (UCLP and BCLP, respectively) treated with and without nasoalveolar molding (NAM). Cleft surgeon members of the American Cleft Palate-Craniofacial Association completed online surveys to evaluate 20 presurgical photograph sets (frontal and basal views) of patients with UCLP (n = 10) and BCLP (n = 10) for severity of cleft deformity, quality of predicted surgical outcome, and likelihood of early surgical revision. Five patients in each group (UCLP and BCLP) received NAM, and 5 patients did not receive NAM. Surgeons were masked to patient group. Twenty-four percent (176/731) of surgeons with valid e-mail addresses responded to the survey. For patients with UCLP, surgeons reported that, for NAM-prepared patients, 53.3% had minimum severity clefts, 58.9% were anticipated to be among their best surgical outcomes, and 82.9% were unlikely to need revision surgery. For patients with BCLP, these percentages were 29.8%, 38.6%, and 59.9%, respectively. Comparing NAM-prepared with non-NAM-prepared patients showed statistically significant differences (P < 0.001), favoring NAM-prepared patients. This study suggests that cleft surgeons assess NAM-prepared patients as more likely to have less severe clefts, to be among the best of their surgical outcomes, and to be less likely to need revision surgery when compared with patients not prepared with NAM.

Cone Beam Computed Tomographic Evaluation of Mandibular Asymmetry in Patients with Cleft Lip and Palate.

PubMed

Paknahad, Maryam; Shahidi, Shoaleh; Bahrampour, Ehsan; Beladi, Amir Saied; Khojastepour, Leila

2018-01-01

Objective The purpose of the present study was to compare mandibular vertical asymmetry in patients with unilateral and bilateral cleft lip and palate and subjects with normal occlusion. Materials and Methods Cone beam computed tomography scans of three groups consisting of 20 patients with unilateral cleft lip and palate, 20 patients affected by bilateral cleft lip and palate, and a control group of 20 subjects with normal occlusion were analyzed for this study. Condylar, ramal, and condylar plus ramal asymmetry indices were measured for all subjects using the method of Habets et al. Kruskal-Wallis and Mann-Whitney tests were used to determine any significant differences between the groups for all indices at the 95% level of confidence. Results There were no significant differences regarding sex for all mandibular asymmetry indices in all three groups. All Asymmetry indices (condylar, ramal, and condylar plus ramal asymmetry) were significantly higher in the unilateral cleft group compared with the other two groups. Conclusion Cone beam computed tomography images showed that patients with cleft lip and palate suffered from mandibular asymmetry. Subjects with unilateral cleft lip and palate had a more asymmetric mandible compared with the bilateral cleft lip and palate and control groups. Therefore, the mandible appears to be the leading factor in facial asymmetry in subjects with unilateral cleft lip and palate.

An anatomical subunit-based outcome assessment scale for bilateral cleft lip and palate.

PubMed

Bonanthaya, K; Shetty, P N; Fudalej, P S; Rao, D D; Bitra, S; Pabari, M; Rachwalski, M

2017-08-01

As there is currently no internationally accepted outcome measurement tool available for complete bilateral cleft lip and palate (CBCLP), the goal of this prospective study was to develop a numerical evaluation scale that allows reliable scoring of this cleft deformity. Our cohort comprised 121 Indian subjects with CBCLP who underwent surgical repair (mean age at time of surgery 6.53 months) using a modified Millard technique. A panel of three professionals evaluated each subject's outcome of bilateral cleft lip repair 6 months postoperatively on two-dimensional (2D) full-face photographs in the frontal view and worm's eye view. A simple two-point rating system was applied to separately analyse a total of 12 components of lip, nose, and scar. The results and mean scores for the analysed anatomical areas were 2.2±1.01 (max=3) for nose, 5.4±1.54 (max=8) for lip, and 1.9±1.3 (max=3) for scar, with a total score 7.7±2.21 (max=12) indicating a good surgical outcome. The inter-examiner ICC for nose, lip, scar, and total score was calculated at 0.836, 0.889, 0.723, and 0.927 respectively and indicated a strong level of repeatability and reliability that was highly significant (P<0.001). In conclusion, we were able to develop and test a scoring system for measuring outcomes in CBCLP that warrants simplicity of use, reliability and reproducibility. Copyright © 2017 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Bilateral first branchial cleft anomaly with evidence of a genetic aetiology.

PubMed

Gonzalez-Perez, L M; Prats-Golczer, V E; Montes Carmona, J F; Heurtebise Saavedra, J M

2014-03-01

Anomalies of the first branchial cleft (FBC) are uncommon, and recognizing them can be difficult. Although present at birth, many cases do not become evident until later in childhood or adolescence, with an initial clinical presentation in adulthood being encountered only rarely. Typically, FBC anomalies present as a unilateral cyst, sinus, or fistula associated with the external auditory canal, or with swelling or an inflammatory opening in the peri-auricular/parotid area. They are commonly misdiagnosed and are often treated inadequately before being excised completely. A 40-year-old woman presented to the maxillofacial outpatient clinic with an episode of bilateral pre-auricular tumefaction, initially diagnosed as temporomandibular dysfunction syndrome. This was associated with bilateral pre-auricular pain that increased with mandibular movements. In relation to the patient's history, and given the bilateral presence of a pre-auricular pit, a diagnosis of FBC anomaly was made. Further investigation showed a related asymptomatic history in five other cases across four generations of the same family. The authors describe here the case, the diagnostic methodology, and the wide local excision technique used for removal of the branchial sinus. Copyright © 2013 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Nasal Airway Dysfunction in Children with Cleft Lip and Cleft Palate: Results of a Cross-Sectional Population-Based Study, with Anatomical and Surgical Considerations.

PubMed

Sobol, Danielle L; Allori, Alexander C; Carlson, Anna R; Pien, Irene J; Watkins, Stephanie E; Aylsworth, Arthur S; Meyer, Robert E; Pimenta, Luiz A; Strauss, Ronald P; Ramsey, Barry L; Raynor, Eileen; Marcus, Jeffrey R

2016-12-01

The aesthetic aspects of the cleft lip nasal deformity have been appreciated for over a century, but the functional implications have remained largely underappreciated or misunderstood. This study describes the frequency and severity of nasal obstructive symptoms among children with cleft lip and/or cleft palate, addressing the hypotheses that age, cleft type, and severity are associated with the development of nasal obstructive symptoms. Children with nonsyndromic cleft lip and/or cleft palate and a comparison group of unaffected children born from 1997 to 2003 were identified through the North Carolina Birth Defects Monitoring Program and birth certificates. Nasal airway obstruction was measured using the validated Nasal Obstruction Symptom Evaluation scale. The survey was completed by parental proxy for 176 children with cleft lip and/or cleft palate and 333 unaffected children. Nasal obstructive symptoms were more frequently reported in cleft lip with cleft palate compared with unaffected children (p < 0.0001); children who had isolated cleft lip with or without alveolus and isolated cleft palate were not statistically different from unaffected children. Patients with unilateral cleft lip with cleft palate were found to be more severely affected than bilateral cases. Nasal obstruction was observed in early childhood, although severity worsened in adolescence. This population-based study reports a high prevalence of nasal obstructive symptoms in children with cleft lip and/or cleft palate based on type and severity of the cleft. The authors encourage cleft teams to consider using this or similar screening methods to identify which children may benefit from functional rhinoplasty. Risk, I.

Dental Caries Experience in Texan Children with Cleft Lip and Palate.

PubMed

Sunderji, Sabrina; Acharya, Bhavini; Flaitz, Catherine; Chiquet, Brett

2017-09-15

The purpose of this study was to assess the caries experience in the primary dentition of children born with cleft lip and palate (CLP). A retrospective chart review was conducted on subjects between two and six years old recruited from a university-based pediatric dentistry residency clinic. The number of dental visits and professional fluoride applications, the plaque index and treatment modality, and the presence/location of caries, white spot lesions, and enamel hypoplastic lesions were compared between CLP patients and healthy age- and gender-matched controls. Descriptive statistics, Student's t test, Mann-Whitney U test, and regression analysis were completed. A total of 183 charts were reviewed. Compared to healthy children, CLP children had increases in number of dental visits (P<0.001), decayed-missing-filled surfaces (dmfs; P<0.001), decayed-missing-filled teeth (dmft; P<0.001), enamel hypoplastic lesions (P=0.003), treatment completed under general anesthesia (P<0.001), plaque score (P<0.001), and caries increment between baseline and most recent oral examination (P=0.003). Regression analysis revealed a positive association between age and dmft scores within the CLP group (P=0.018). The caries experience of unilateral and bilateral CLP cases was the same (P>0.05). Children with cleft lip and palate are at a greater risk of enamel hypoplasia and dental caries. No significant caries experience difference was found between unilateral or bilateral CLP cases.

Sonographic findings in a case of tetrasomy 9p associated with increased nuchal translucency and Dandy-Walker malformation.

PubMed

Nakamura-Pereira, Marcos; Cima, Luciana Carneiro do; Llerena, Juan Clinton; Guerra, Fernando Antonio Ramos; Peixoto-Filho, Fernando Maia

2009-10-01

We report a case of a 23-year-old pregnant woman, who underwent amniocentesis after ultrasound (US) examination in the first trimester which revealed a nuchal translucency thickness of 2.9 mm. Cytogenetic analysis revealed complete tetrasomy of the short arm of chromosome 9. Further US evaluation in the second trimester revealed Dandy-Walker malformation, ventriculomegaly, bilateral clubfoot, lip and palate clefts, arthrogryposis and hyperechoic kidneys with bilateral pelvic dilatation. At 30 weeks of gestation, a placental abruption was noted and a Cesarean section was performed. The infant died shortly after birth. A review of previous cases of tetrasomy 9p shows that the remarkable sonographic findings are ventriculomegaly, intrauterine growth restriction, genitourinary anomaly, Dandy-Walker malformation, cleft lip/palate and limb malformation, but the association of tetrasomy 9p and increased nuchal translucency had not been reported.

Factors Associated With Adherence to Nasoalveolar Molding (NAM) by Caregivers of Infants Born With Cleft Lip and Palate.

PubMed

Esmonde, Nick O; Garfinkle, Judah S; Chen, Yiyi; Lambert, William E; Kuang, Anna A

2018-02-01

Identify factors associated with adherence to nasoalveolar molding (NAM) therapy. Retrospective case-control study. Tertiary referral center. Infants with cleft lip, with or without cleft palate, referred for NAM. One hundred thirty-five patients met criteria. Adherence to NAM therapy, defined as continuous use of the appliance and attendance of NAM adjustment visits. Female sex (OR = 2.85, 95% CI 1.21-6.74), bilateral cleft (OR = 2.88, 95% CI 1.29-6.46), and travel distance (OR = 1.01, 95% CI 1.00-1.01) were independent predictors of nonadherence. Bilateral clefts (OR = 8.35, 95% CI 2.72-25.64) and public-payer insurance (OR = 3.67, 95% CI 1.13-11.91) for male patients were significantly associated with nonadherence, in our sex-stratified multivariate model. The majority of the families (58%) had public health insurance. Males comprised 77.0% of the cohort. NAM treatment adherence is impaired by bilateral clefts, female sex, increased travel distance, and public insurance. Further studies are warranted to investigate how these factors affect adherence, and to develop interventions to improve adherence in families at risk due to economic or psychosocial barriers.

[Clinical effect of functional repair of bilateral cleft lip in 66 patients].

PubMed

Li, Ming; Jiang, Hong-bing; Yuan, Hua; DU, Yi-fei; Wu, Yu-nong; Wan, Lin-zhong

2014-10-01

To explore the clinical effect of functional repair for bilateral cleft lip using modified Mulliken method. Sixty-six patients with bilateral cleft lip were selected and assigned to receive modified Mulliken method. During the operation, the prolabium was kept as narrow "tie" shape, the orbicularis oris was anatomically repositioned, and the orbicularis oris ring was re-built. Vermilion tubercle was reconstructed with the lateral red vermillion. The nasal deformity was preliminarily repaired. and the nasal columella was elongated at the same time. All the patients were followed-up for 0.5-2 years, there was no "trisection upper lip". The symmetry and natural shape of Cupid's bow were obtained in more than 80% patients. The width of philtrum was similar to normal children. Full vermilion of the lips, moderate-size vermilion tubercles and good dynamic and static shape were obtained without whistle deformities. Normal width of nasal base and nostril symmetry were gained. The columella was elongated. Satisfactory contour of the nasal tip was achieved. Modified Mulliken method could functionally repair bilateral cleft lip and effectively correct nasolabial deformities. It is worthy of wide clinical application.

One-Stage versus Two-Stage Repair of Asymmetric Bilateral Cleft Lip: A 20-Year Retrospective Study of Clinical Outcome.

PubMed

Chung, Kyung Hoon; Lo, Lun-Jou

2018-05-01

Both one- and two-stage approaches have been widely used for patients with asymmetric bilateral cleft lip. There are insufficient long-term outcome data for comparison of these two methods. The purpose of this retrospective study was to compare the clinical outcome over the past 20 years. The senior author's (L.J.L.) database was searched for patients with asymmetric bilateral cleft lip from 1995 to 2015. Qualified patients were divided into two groups: one-stage and two-stage. The postoperative photographs of patients were evaluated subjectively by surgical professionals and laypersons. Ratios of the nasolabial region were calculated for objective analysis. Finally, the revision procedures in the nasolabial area were reviewed. Statistical analyses were performed. A total of 95 consecutive patients were qualified for evaluation. Average follow-up was 13.1 years. A two-stage method was used in 35 percent of the patients, and a one-stage approach was used in 65 percent. All underwent primary nasal reconstruction. Among the satisfaction rating scores, the one-stage repair was rated significantly higher than two-stage reconstruction (p = 0.0001). Long-term outcomes of the two-stage patients and the unrepaired mini-microform deformities were unsatisfactory according to both professional and nonprofessional evaluators. The revision rate was higher in patients with a greater-side complete cleft lip and palate as compared with those without palatal involvement. The results suggested that one-stage repair provided better results with regard to achieving a more symmetric and smooth lip and nose after primary reconstruction. The revision rate was slightly higher in the two-stage patient group. Therapeutic, III.

The "Double" Tessier 7 Cleft: An Unusual Presentation of a Transverse Facial Cleft.

PubMed

Raveendran, Janani A; Chao, Jerry W; Rogers, Gary F; Boyajian, Michael J

2018-07-01

Congenital macrostomia, or Tessier number 7 cleft, is a rare craniofacial anomaly. We present a unique patient with bilateral macrostomia that consisted of a "double" transverse cleft on the left side and a single transverse cleft on the right side. A staged reconstructive approach was used to repair the "double" left-sided clefts. This staged technique produced a satisfactory aesthetic and functional outcome.

Maxillary growth after maxillary protraction: Appliance in conjunction with presurgical orthopedics, gingivoperiosteoplasty, and Furlow palatoplasty for complete bilateral cleft lip and palate patients with protruded premaxilla.

PubMed

Kobayashi, Shinji; Hirakawa, Takashi; Fukawa, Toshihiko; Maegawa, Jiro

2015-06-01

In bilateral cleft lip and palate (BCLP) with premaxillary protrusion, a good outcome with adequate maxillary development is difficult to achieve. The purpose of this article is to evaluate the maxillary growth after using presurgical orthopedics (PSO), gingivoperiosteoplasty (GPP), Furlow palatoplasty, and maxillary protraction appliance (MPA) for BCLP with premaxillary protrusion. Seven patients with complete BCLP with premaxillary protrusion were treated by PSO, cheiloplasty, GPP, and Furlow palatoplasty. MPA was used as part of the protocol for 6 months to 1 year for postoperative retardation of maxillary growth cases. Maxillary growth was evaluated by cephalometric analysis at 4 and 10 years of age, and bone formation at the alveolar cleft was evaluated by computed tomography (CT) imaging at 5 years of age. At 4 years of age, three of seven patients had apparent retardation of maxillary growth. The maxillary growth at 10 years of age was equivalent to the average value of normal Japanese after using MPA in three cases. At 5 years of age, only two of seven patients showed sufficient bone formation at the alveolar cleft to avoid alveolar bone grafting (ABG). Subsequently, ABG was performed in five patients. Although three of seven patients had apparent crossbite at 4 years of age, the maxillary growth of all patients at 10 years of age was approximately equivalent to the average value of normal Japanese after using MPA. A treatment protocol based on PSO, GPP, Furlow palatoplasty, and MPA may be an option, but long-term growth is unknown. Copyright © 2015 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

Comparative Evaluation of the Pharyngeal Airways and Related Soft Tissues of Unilateral and Bilateral Cleft Lip and Palate Patients With the Noncleft Individuals.

PubMed

Aras, Isil; Dogan, Servet

2017-01-01

  This study is a comparison of pharyngeal airways and associated soft tissues of unilateral and bilateral cleft lip and palate patients with the noncleft individuals.   Twenty-four unilateral cleft lip and palate patients (UCLP), 21 bilateral cleft lip and palate patients (BCLP), and 26 noncleft patients (NC) between ages 15 to 17 were included in the study. Eleven linear, 1 angular, and 1 proportional measurements were carried out on pretreatment lateral cephalometric head films of these individuals.   The nasopharyngeal depths were markedly reduced in BCLP when compared with the NC (P < .001) and UCLP (P < .01) with a significant difference (P < .01) also among UCLP and NC. Minimum space behind the tongue concerning BCLP and UCLP were significantly lower compared to the NC (P < .001 and P < .01, respectively). In pairwise comparison among the clefts, BCLP showed significant (P < .05) narrowing of this distance. Tongue heights and velar lengths were significantly (P < .001) lower in cleft groups compared to the controls. Similarly, the hyoid bone was positioned in a significantly (P < .01) anterior and inferior direction in cleft patients. Comparison of the mean ratio of velar length to nasopharyngeal depth of the three groups revealed significant (P < .001) inadequacies in cleft patients with significantly (P < .05) more severely reduced values in BCLP.   Pharyngeal airways and involved soft tissues of cleft patients show serious inadequacies compared to controls, with significant diversities among cleft types pertaining to some of the parameters investigated. It should be kept in mind that these variations can influence function in terms of respiration and phonation.

Palatoplasty: suturing the mucoperiosteal flaps to the hard palate through hole.

PubMed

Hwang, Kun; Lee, Ji Hun; Kim, Yu Jin; Le, Se Il

2009-05-01

We satisfactorily repaired a wide cleft palate using a method of V-Y pushback and anchoring the oral mucoperiosteal flap onto the bony ridge of the cleft. An 8-year-old Vietnamese girl had a wide incomplete bilateral posterior cleft palate associated with congenital cardiac malformations. The gap of the posterior cleft was 2.5 cm, which exceeded the total widths of the palatal shelves. We applied V-Y pushback and used a vomer flap to close the wide cleft palate. The posterior two thirds of the nasal mucosae from the cleft margins were sutured to the vomer flap. The nasal side of the anterior one third of the bony cleft was uncovered. The elevated bilateral mucoperiosteal flaps were brought together to the midline and sutured to the anterior triangular flap in a V-Y pushback fashion. Four holes were drilled 5 mm lateral to each bony cleft margin. The lateral sides of the mucoperiosteal flaps were fixed to the palate bone with 3-0 Vicryl through the hole. This method reduces the tension of the flap which might frequently cause oronasal fistula and also improve viability.

Gene p63: In ectrodactyly-ectodermal dysplasia clefting, ankyloblepharon-ectodermal dysplasia, Rapp-Hodgkin syndrome.

PubMed

van Straten, Cornelia; Butow, Kurt-W

2013-01-01

An analysis was made of three different syndromes associated with p63 gene mutations, known as ectrodactyly-ectodermal dysplasia-clefting syndrome (EEC), ankyloblepharon-ectodermal dysplasia clefting syndrome (AEC or Hay-Wells) and Rapp-Hodgkin syndrome (RHS). The postoperative complications associated with their cleft reconstructions were also evaluated. Extensive demographic information, in particular of the clinical appearances, associated malformations, and the types and complications of the reconstructive surgical procedures, were recorded of these syndromic cases occurring in a database of 3621 facial cleft deformity patients. The data was analyzed using the Microsoft Excel program. A total of 10 (0.28%) cases of p63 associated syndromes were recorded: EEC (6), RHS (3), and AEC (1). The following clinical cleft appearances were noted - EEC = 6: CLA 1 -right side unilateral (female); CLAP 4 - right side (1) + left side (1) unilateral (male + female); bilateral (2) (males); hPsP 1 (female) (divided in 3 Black, 2 White, 1 Indian); RHS = 3: CLAP 2 (White males); hPsP 1 (White female); AEC = 1: CLAP bilateral (White male). Other features of the syndromes were: skin, hand, foot, tooth, hair and nail involvement, and light sensitivity. Postoperative complications included: (i) stenosis of nasal opening, especially after reconstruction of the bilateral cleft lip and the columella lengthening (2 cases), (ii) premaxilla-prolabium fusion (2 cases), (iii) repeated occurrence of oro-nasal fistula in the hard palate (4 cases), and (iv) dysgnathial development of midfacial structures (3 cases). Three different p63 associated syndromes (EEC, AEC, and RHS) were diagnosed (0.27% of the total facial cleft deformities database). The majority of the cases presented with a bilateral CLAP in males only. A number of females and males had unilateral CLA. The hPsP-cleft was recorded in females only. The associated ectodermal component most probably had a profoundly negative influence on postoperatively wound healing, which was observed in particular at the nasal openings, the premaxilla sulcus and in the hard palate mucosa. The reconstruction of p63 associated syndromes is a greater challenge than the usual cleft reconstruction to the surgeon.

Prevalence of dental anomalies in a population of cleft lip and palate patients.

PubMed

Al Jamal, Ghaida A; Hazza'a, Abdalla M; Rawashdeh, Ma'amon A

2010-07-01

The aim of our study was to investigate radiographically the prevalence of dental anomalies in a group of Jordanian cleft lip and/or palate subjects. This is a retrospective review of panoramic radiographs of 78 subjects with cleft lip and/or palate that were evaluated from their file records and investigated for possible dental anomalies. Dental anomalies were found frequently in cleft lip and/or palate subjects. Missing teeth were found in 66.7% of the patients; the tooth most commonly missing was the maxillary lateral incisor. Supernumerary teeth were found in 16.7% of patients; 37% had microdontia; 70.5% had taurodontism; 30.8% had transposition and/or ectopic teeth; 19.2% had dilacerations; and 30.8% had hypoplastic teeth. There was no statistically significant difference in the above anomalies' prevalence between males and females. However, it was found that subjects with bilateral cleft lip and/or palate had significantly more microdontia (p = .005), dilaceration (p = .002), and hypoplastic teeth (p = .0001) than subjects with unilateral cleft lip and/or palate. The prevalence of dental anomalies in cleft lip and/or palate patients was higher than what had been reported in the normal Jordanian population. This emphasizes the relation of cleft lip and/or palate to all dental anomalies studied. Although our study represents a thorough and complete description of dental anomalies present in a sample of cleft lip and/or palate subjects, larger samples are required to effectively determine the relationship of each dental anomaly with cleft type.

Risk factors leading to mucoperiosteal flap necrosis after primary palatoplasty in patents with cleft palate.

PubMed

Rossell-Perry, Percy; Figallo-Hudtwalcker, Olga; Vargas-Chanduvi, Roberto; Calderon-Ayvar, Yvette; Romero-Narvaez, Carolina

2017-10-01

Few studies have been published reporting risk factors for flap necrosis after primary palatoplasty in patients with cleft palate. This complication is rare, and the event is a disaster for both the patient and the surgeon. This study was performed to explore the associations between different risk factors and the development of flap necrosis after primary palatoplasty in patients with cleft palate. This is a case-control study. A 20 years retrospective analysis (1994-2015) of patients with nonsyndromic cleft palate was identified from medical records and screening day registries). Demographical and risk factor data were collected using a patient´s report, including information about age at surgery, gender, cleft palate type, and degree of severity. Odds ratios and 95% confident intervals were derived from logistic regression analysis. All cases with diagnoses of flap necrosis after primary palatoplasty were included in the study (48 patients) and 156 controls were considered. In multivariate analysis, female sex, age (older than 15 years), cleft type (bilateral and incomplete), and severe cleft palate index were associated with significantly increased risk for flap necrosis. The findings suggest that female sex, older age, cleft type (bilateral and incomplete), and severe cleft palatal index may be associated with the development of flap necrosis after primary palatoplasty in patients with cleft palate.

Nasoalveolar molding improves appearance of children with bilateral cleft lip-cleft palate.

PubMed

Lee, Catherine T H; Garfinkle, Judah S; Warren, Stephen M; Brecht, Lawrence E; Cutting, Court B; Grayson, Barry H

2008-10-01

Bilateral cleft lip-cleft palate is associated with nasal deformities typified by a short columella. The authors compared nasal outcomes of cleft patients treated with banked fork flaps to those of patients who underwent nasoalveolar molding and primary retrograde nasal reconstruction. A retrospective review of 26 consecutive patients with bilateral cleft lip-cleft palate was performed. Group 1 patients (n = 13) had a cleft lip repair and nasal correction with banked fork flaps. Group 2 patients (n = 13) had nonsurgical columellar elongation with nasoalveolar molding followed by cleft lip closure and primary retrograde nasal correction. Group 3 patients (n = 13) were age-matched controls. Columellar length was measured at presentation and at 3 years of age. The number of nasal operations was recorded to 9 years. The Kruskal-Wallis and Tukey-Kramer tests were used for statistical analysis. Initial columellar length was 0.49 +/- 0.37 mm in group 1 and 0.42 +/- 0.62 mm in group 2. Post-nasoalveolar molding columellar length was 4.5 +/- 0.76 mm in group 2. By 3 years of age, columellar length was 3.03 +/- 1.47 mm in group 1, 5.98 +/- 1.09 mm in group 2, and 6.35 +/- 0.99 mm in group 3. Group 2 columellar length was significantly greater (p < 0.001) than that of group 1 and not statistically different from that of group 3 (p > 0.05). All group 1 patients (13 of 13) needed secondary nasal surgery. No nasoalveolar molding patients (zero of 13, group 2) required secondary nasal surgery. Nonsurgical columellar elongation with nasoalveolar molding followed by primary retrograde nasal reconstruction restored columellar length to normal by 3 years and significantly reduced the need for secondary nasal surgery.

Prevalence of non-syndromic orofacial clefts among Jews and Arabs, by type, site, gender and geography: a multi-center study in Israel.

PubMed

Shapira, Yehoshua; Haklai, Ziona; Blum, Itay; Shpack, Nir; Amitai, Yona

2014-12-01

Orofacial clefts are the most common craniofacial congenital malformations, with significant anatomic, ethnic, racial and gender differences. To investigate the prevalence, distribution and characteristic features of various types of non-syndromic clefts among Israeli Jews and Arabs. We conducted a retrospective multi-center survey in 13 major hospitals in Israel for the period 1993-2005. To obtain the true prevalence and detailed clinical characteristics, data on liveborn infants with non-syndromic clefts were obtained from the Ministry of Health's National Birth Defect Registry and completed by chart reviews in the 13 surveyed hospitals. Of 976,578 liveborn infants, 684 presented unilateral or bilateral clefts, with a prevalence of 7.00/10,000 live births; 479 were Jews and 205 were Arabs. The prevalence was higher among Arabs compared to Jews (11.12 and 6.22 per 10,000 live births in Arabs and Jews, respectively, P 0.00001). Males had higher cleft rates than females (7.69/10,000 and 6.17/10,000 live births, respectively, P = 0.05). Males had more cleft lips (P < 0.05) and cleft lips with cleft palate (P < 0.001). There was left-side predominance. Newborns of younger mothers (age < 20 years) and of older mothers (age ≥ 45 years) had higher cleft rates than those with mothers in the 20-44 year bracket (P < 0.009). Children born at or above the 5th birth order had a higher cleft rate (P < 0.001). The prevalence of non-syndromic clefts was 7.00/10,000 live births. The markedly higher rate in Arabs is related to the high rate of consanguinity. Both very young and old maternal age represents a higher risk of clefts in their offspring.

Bilaterally cleft lip, limb defects, and haematological manifestations: Roberts syndrome versus TAR syndrome.

PubMed

Urban, M; Opitz, C; Bommer, C; Enders, H; Tinschert, S; Witkowski, R

1998-09-23

We report on a 13-year-old patient followed since birth. He is the only offspring of young, non-consanguineous German parents. His mother has an isolated left cleft of lip and a cleft palate. At birth, our patient presented with bilaterally cleft lip/cleft palate, phocomelia of upper limbs with normal hands, and mild symmetrical deficiencies of the long bones of the lower limbs. Haematological evaluation demonstrated a leukaemoid reaction during a urinary tract infection as well as intermittent thrombocytopenia and episodes of marked eosinophilia during the first two years of life. Intellectual development has been normal. Comparison with two similar cases from the literature suggests a non-random phenotypic overlap of Roberts syndrome (MIM 268300) and TAR syndrome (MIM 274000). Such clinical constellations may be key observations to understand the genetic relationship of Roberts syndrome and TAR syndrome in future phenotype-genotype correlations.

Prevalence and Characteristics of Developmental Dental Anomalies in Iranian Orofacial Cleft Patients.

PubMed

Ajami, Shabnam; Pakshir, Hamidreza; Samady, Hedyeh

2017-09-01

Individuals with oral clefts exhibit considerably more dental anomalies than individuals without clefts. These problems could initially be among the symptoms of their disease and/or they may be the side effect of their treatments. Pushback palatoplasty could cause some interference during the development of teeth and result in tooth defects. The study was performed to assess the prevalence and characteristics of developmental dental anomalies in orofacial cleft patients who attended Shiraz Orthodontics Research Center-Cleft Lip and Palate Clinic. We managed to compare dental anomaly traits based on gender and cleft side. Eighty out of 121 cleft patients were included in this cross-sectional study. All the patients used pushback palatoplasty in their palate closure surgeries. Intraoral photographs, panoramic and intraoral radiographs, cone-beam computed tomography (CBCT) and dental and medical histories were examined and recorded by two observers. Data were analyzed using SPSS PC version 20.0. The differences in the side of cleft and dental anomalies were compared using the Mann-Whitney test. The mean age of patients was 14.27 years (SD=5.06). The most frequent cleft type was unilateral cleft lip and palate (50%) followed by bilateral cleft lip and palate (43.75%), cleft palate (2.5%) and cleft lip (1.25%). Male predominance (70%) was observed. 92.5 percent had at least one developmental dental anomaly. The most prevalent anomalies were hypodontia (71.25%) followed by microdontia (30%), root dilacerations (21.25%) and supernumerary teeth (15%). The most prevalent cleft types were unilateral and bilateral cleft lip and palate with male and left side predominance. Hypodontia, microdontia, dilacerations and supernumerary teeth were the most prevalent developmental dental anomalies among Iranian southwestern cleft patients. The surgical technique used to repair their cleft palate may have played a role in developmental dental defects.

3D-Printed Models of Cleft Lip and Palate for Surgical Training and Patient Education.

PubMed

Chou, Pang-Yun; Hallac, Rami R; Shih, Ellen; Trieu, Jenny; Penumatcha, Anjani; Das, Priyanka; Meyer, Clark A; Seaward, James R; Kane, Alex A

2018-03-01

Sculpted physical models and castings of the anatomy of cleft lip and palate are used for parent, patient, and trainee education of cleft lip and palate conditions. In this study, we designed a suite of digital 3-dimensional (3D) models of cleft lip and palate anatomy with additive manufacturing techniques for patient education. CT scans of subjects with isolated cleft palate, unilateral and bilateral cleft lip and palate, and a control were obtained. Soft tissue and bony structures were segmented and reconstructed into digital 3D models. The oral soft tissues overlying the cleft palate were manually molded with silicone putty and scanned using CT to create digital 3D models. These were then combined with the original model to integrate with segmentable soft tissues. Bone and soft tissues were 3D printed in different materials to mimic the rigidity/softness of the relevant anatomy. These models were presented to the parents/patients at our craniofacial clinic. Visual analog scale (VAS) surveys were obtained pertaining to the particular use of the models, to ascertain their value in parental education. A total of 30 parents of children with cleft conditions completed VAS evaluations. The models provided the parents with a better understanding of their child's condition with an overall evaluation score of 9.35 ± 0.5. We introduce a suite of 3D-printed models of cleft conditions that has a useful role in patient, parental, and allied health education with highly positive feedback.

Detection of orbicularis oris muscle defects in first degree relatives of cleft lip children using ultrasound.

PubMed

Mittal, M; Maheshwari, N; Ahlawat, K; Sharma, V; Sultan, A; Chopra, R

2012-01-01

The severity of cleft lip (CL) varies considerably from complete bilateral CL and palate at one end of the spectrum to a minimal CL at the other. In some cases of microform clefting, there may be no visible manifestation of the defect on the lip surface (i.e., the defect is occult) and no residual functional deficit. This study used high resolution ultrasonography to detect subclinical anomalies of orbicularis oris muscle (OOM) in first degree relatives of CL +- cleft palate children and compared it with controls. Thirty relatives of 25 children with non-syndromic CL or CL+ CP were identified for the study. Thirty subjects having negative family history of CL/P in three generations and absence of any minimal cleft features were taken as controls. Ultrasound scans of OOM of all the controls and relatives were taken. Statistical analysis was performed using standard χ2 tests with Yates correction. Defects were seen in 13.3% of relatives and no defects were seen in controls, this was not statistically significant. The data support the hypothesis that subclinical CL cases with subepithelial OOM defects do exist and Orbicularis oris discontinuities represent the mildest form of CL.

Dental Anomalies in Different Types of Cleft Lip and Palate: Is There Any Relation?

PubMed

Germec Cakan, Derya; Nur Yilmaz, Rahime Burcu; Bulut, Feyza Nur; Aksoy, Ayca

2018-02-26

The aims of this study were to evaluate the prevalence of dental anomalies in Turkish patients with different types of cleft lip and palate (CLP) and investigate the relationship between the type of cleft and the dental anomaly. Eighty-eight patients with cleft lip and/or palate (mean age: 14.1 ± 6.4 years) were enrolled in this retrospective study. Dental models, panoramic radiographs, and intraoral photographs of these patients were evaluated to detect any maxillary dental anomaly (number and size anomalies). Two hundred fifty unaffected subjects (mean age: 15.2 ± 7.2 years) composed the control group. Data were evaluated using the independent t test, χ, Fischer exact test, and the odds ratio. Dental anomaly frequency was significantly higher in the cleft group compared with the control group. Tooth agenesis was the most common dental anomaly, followed by microdontia and supernumerary tooth. Lateral incisor agenesis was seen in 69% of the unilateral CLP, in 78% of the bilateral CLP, and in 18% of the cleft palate patients. A significant association was revealed between the right unilateral CLP and the right lateral incisor agenesis (P = 0.0001), the left unilateral CLP and the left lateral incisor agenesis (P = 0.002), and the bilateral CLP and the bilateral lateral incisor agenesis (P = 0.0001). Dental anomalies are more frequently seen in patients with CLP compared with the general population. There is a relationship between the cleft type and the ipsilateral lateral incisor agenesis.

Use of early hard palate closure using a vomer flap in cleft lip and palate patients.

PubMed

Smarius, Bram J A; Breugem, Corstiaan C

2016-08-01

The aim of the present study was to determine the influence of the vomer flap during cleft palate closure. A retrospective review was performed of all consecutive unilateral/bilateral complete cleft lip and palate (Veau III en IV) children who were treated by a simultaneous lip and hard palate closure using a vomer flap. Data were collected for sex, date of birth, syndrome, adoption, cleft palate type, type of repair, date of cleft repair, cleft width, lateral incisions, fistula and location of fistula. Ninety-one children (M = 62, F 29) were operated. Mean age at time of lip closure and vomer flap was 5.8 months (range 2.9 months to 49.2 months, SD 7.1) and the mean age at palate closure was 13.6 months (range 6.3 months to 79.9 months, SD 10.8). The mean cleft width at first assessment was 13.0 mm (range 7-22 mm) compared to 8.8 mm (range 4-15 mm) at second assessment (mean difference 4.6 mm, 95% CI 3.93-5.35, p < 0.01). One patient developed a fistula (1.1%) and required secondary surgery for closure. The vomer flap leads to a substantial decrease in cleft width, subsequently leading to a low fistula incidence (1.1%). Copyright © 2016 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Cleft characteristics and treatment outcomes in hemifacial microsomia compared to non-syndromic cleft lip/palate.

PubMed

Dentino, K M; Valstar, A; Padwa, B L

2016-06-01

The goal of this study was to describe the clinical characteristics and treatment outcomes of patients with hemifacial microsomia (HFM) and cleft lip/palate (CL/P), and to compare them to a historic cohort of patients with non-syndromic CL/P treated at the same centre. A retrospective review of patients with HFM and CL/P was performed; the main outcome measures assessed were cleft type/side, surgical outcome, midfacial retrusion, and speech. Twenty-six patients (13 male, 13 female; mean age 22.7±14.9, range 1-52 years) with cleft lip with/without cleft palate (CL±P) were identified: three with cleft lip (12%), two with cleft lip and alveolus and an intact secondary palate (8%), and 21 with cleft lip and palate (CLP) (81%; 15 unilateral and six bilateral). Four patients (19%) had a palatal fistula after palatoplasty. Twelve of 22 patients aged >5 years (55%) had midfacial retrusion and two (9%) required a pharyngeal flap for velopharyngeal insufficiency (VPI). Fisher's exact test demonstrated a higher frequency of complete labial clefting (P=0.004), CLP (P=0.009), midfacial retrusion (P=0.0009), and postoperative palatal fistula (P=0.03) in HFM compared to non-syndromic CL±P. There was no difference in VPI prevalence. This study revealed that patients with HFM and CL±P have more severe forms of orofacial clefting than patients with non-syndromic CL±P. Patients with HFM and CL±P have more severe midfacial retrusion and a higher palatal fistula rate compared to patients with non-syndromic CL±P. Copyright © 2015 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Effects of cleft type, facemask anchorage method, and alveolar bone graft on maxillary protraction: a three-dimensional finite element analysis.

PubMed

Yang, Il-Hyung; Chang, Young-Il; Kim, Tae-Woo; Ahn, Sug-Joon; Lim, Won-Hee; Lee, Nam-Ki; Baek, Seung-Hak

2012-03-01

To investigate biomechanical effects of cleft type (unilateral/bilateral cleft lip and palate), facemask anchorage method (tooth-borne and miniplate anchorage), and alveolar bone graft on maxillary protraction. Three-dimensional finite element analysis with application of orthopedic force (30° downward and forward to the occlusal plane, 500 g per side). Computed tomography data from a 13.5-year-old girl with maxillary hypoplasia. Eight three-dimensional finite element models were fabricated according to cleft type, facemask anchorage method, and alveolar bone graft. Initial stress distribution and displacement after force application were analyzed. Unilateral cleft lip and palate showed an asymmetric pattern in stress distribution and displacement before alveolar bone graft and demonstrated a symmetric pattern after alveolar bone graft. However, bilateral cleft lip and palate showed symmetric patterns in stress distribution and displacement before and after alveolar bone graft. In both cleft types, the graft extended the stress distribution area laterally beyond the infraorbital foramen. For both unilateral and bilateral cleft lip and palate, a facemask with a tooth-borne anchorage showed a dentoalveolar effect with prominent stress distribution and displacement on the upper canine point. In contrast, a facemask with miniplate anchorage exhibited an orthopedic effect with more favorable stress distribution and displacement on the middle maxilla point. In addition, the facemask with a miniplate anchorage showed a larger stress distribution area and sutural stress values than did the facemask with a tooth-borne anchorage. The pterygopalatine and zygomatico-maxillary sutures showed the largest sutural stress values with a facemask with a miniplate anchorage and after alveolar bone grafting, respectively. In this three-dimensional finite element analysis, it would be more advantageous to perform maxillary protraction using a facemask with a miniplate anchorage than a facemask with a tooth-borne anchorage and after alveolar bone graft rather than before alveolar bone graft, regardless of cleft type.

Prevalence and Characteristics of Developmental Dental Anomalies in Iranian Orofacial Cleft Patients

PubMed Central

Ajami, Shabnam; Pakshir, Hamidreza; Samady, Hedyeh

2017-01-01

Statement of the Problem: Individuals with oral clefts exhibit considerably more dental anomalies than individuals without clefts. These problems could initially be among the symptoms of their disease and/or they may be the side effect of their treatments. Pushback palatoplasty could cause some interference during the development of teeth and result in tooth defects. Purpose: The study was performed to assess the prevalence and characteristics of developmental dental anomalies in orofacial cleft patients who attended Shiraz Orthodontics Research Center-Cleft Lip and Palate Clinic. We managed to compare dental anomaly traits based on gender and cleft side. Materials and Method: Eighty out of 121 cleft patients were included in this cross-sectional study. All the patients used pushback palatoplasty in their palate closure surgeries. Intraoral photographs, panoramic and intraoral radiographs, cone-beam computed tomography (CBCT) and dental and medical histories were examined and recorded by two observers. Data were analyzed using SPSS PC version 20.0. The differences in the side of cleft and dental anomalies were compared using the Mann-Whitney test. Results: The mean age of patients was 14.27 years (SD=5.06). The most frequent cleft type was unilateral cleft lip and palate (50%) followed by bilateral cleft lip and palate (43.75%), cleft palate (2.5%) and cleft lip (1.25%). Male predominance (70%) was observed. 92.5 percent had at least one developmental dental anomaly. The most prevalent anomalies were hypodontia (71.25%) followed by microdontia (30%), root dilacerations (21.25%) and supernumerary teeth (15%). Conclusion: The most prevalent cleft types were unilateral and bilateral cleft lip and palate with male and left side predominance. Hypodontia, microdontia, dilacerations and supernumerary teeth were the most prevalent developmental dental anomalies among Iranian southwestern cleft patients. The surgical technique used to repair their cleft palate may have played a role in developmental dental defects. PMID:29034274

Management of an infant with cleft lip and palate with phocomelia in dental practice.

PubMed

Muthu, M S

2000-12-01

Cleft lip and palate is a severe birth defect occurring approximately one in 800-1000 newborn infants. The incidence varies widely among races. Cleft lip and palate together account for approximately 50% of all cases whereas isolated cleft lip and isolated cleft palate occur in about 25% of cases. Many of these congenital anomalies appear to be genetically determined though the majority are of unknown causes or teratogenic influences. Presented here is a 3 day old infant with bilateral cleft lip and palate and phocomelia for whom a feeding obturator was made and delivered to facilitate feeding.

Bilateral congenital lacrimal fistulas in an adult as part of ectrodactyly-ectodermal dysplasia-clefting syndrome: A rare anomaly.

PubMed

Ghosh, Debangshu; Saha, Somnath; Basu, Sumit Kumar

2015-10-01

Ectrodactyly-ectodermal dysplasia and clefting syndrome or "Lobster claw" deformity is a rare congenital anomaly that affects tissues of ectodermal and mesodermal origin. Nasolacrimal duct (NLD) obstruction with or without atresia of lacrimal passage is a common finding of such a syndrome. The authors report here even a rarer presentation of the syndrome which manifested as bilateral NLD obstruction and lacrimal fistula along with cleft lip and palate, syndactyly affecting all four limbs, mild mental retardation, otitis media, and sinusitis. Lacrimal duct obstruction and fistula were managed successfully with endoscopic dacryocystorhinostomy (DCR) which is a good alternative to lacrimal probing or open DCR in such a case.

Effective retropulsion and centralization of the severely malpositioned premaxilla in patients with bilateral cleft lip and palate: a novel modified presurgical nasoalveolar molding device with retraction screw.

PubMed

Li, Wanshan; Liao, Lishu; Dai, Jingbo; Zhong, Yuxiang; Ren, Leixi; Liu, Yutao

2014-12-01

A novel, modified presurgical nasoalveolar molding (MPNAM) device with retraction screw was designed and used in patients with bilateral complete cleft lip and palate (BCCLP) to rapidly retract and centralize the protuberant and malpositioned premaxilla and correct the nasolabial and palatal deformities. The orthopedic effects and possible complications were evaluated. Nine patients with BCCLP who met the inclusion criteria were selected. After the maxillary model was obtained, the new MPNAM device with retraction screw was designed and worn until cheilorrhaphy. Changes in local deformities and complications were observed continuously, and the orthopedic effect was evaluated. All patients quickly adapted to the MPNAM appliance, and the treatment was finished after 5-8 return visits. The columella was significantly prolonged, the nasal tip was elevated, and the collapsed nasal dome was obviously improved. Simultaneously, the premaxilla was rapidly retracted and rotated, and gradually centralized; the clefts were gradually reduced and closed, and a nearly normal dental arch was formed. Although there were some complications, the orthopedic treatment was continued until cheiloplasty. The MPNAM device with retraction screw can simultaneously correct nasolabial and palatal deformities and also rapidly retract and centralize the premaxilla. Copyright © 2014 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

[Maxillary advancement osteotomy with sequelae cleft lip and palate: Dilemma between occlusion and aesthetic profile].

PubMed

Vigneron, A; Morand, B; Lafontaine, V; Lesne, V; Lesne, C; Bettega, G

2015-11-01

Maxillary hypoplasia is a common sequela of cleft lip and palate. Its surgical treatment consists in a maxillary advancement by distraction or by conventional orthognathic surgery but morphological results are unpredictable. Our goal in this study was to see if the esthetical results (on the lip and the nose) of maxillary advancement were correlated to the preservation of lateral incisor space of the cleft side. This retrospective study included 38 patients operated between 2002 and 2013. Unilateral clefts were studied independently from bilateral clefts. Profile aesthetics was evaluated independently and subjectively by two surgeons and scored on an 8-point scale. The result was classified as "good" if the score was superior or equal to 6. The score was correlated to the following parameters: amount of maxillary advancement, upper incisor axis, preservation of the missing lateral incisor space. In the "good result" group, the space of the lateral incisor was less often preserved. The nasolabial angle was more open and the upper central incisor axis more vertical. These results were more pronounced in bilateral clefts, but also found in unilateral clefts. Under reservation of the subjective evaluation and of the small number of patients, it seemed that lateral incisor space closure improved the profile of patients treated by maxillary advancement for cleft lip and palate sequelae. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

Cleft Lip and Palate Repair.

PubMed

Gatti, Gian Luca; Freda, Nicola; Giacomina, Alessandro; Montemagni, Marina; Sisti, Andrea

2017-11-01

Cleft lip and palate is the most frequent congenital craniofacial deformity. In this article, the authors describe their experience with cleft lip and palate repair. Data regarding patients presenting with primary diagnosis of cleft lip and/or palate, between 2009 and 2015, were reviewed. Details including demographics, type of cleft, presence of known risk factors, surgical details, and follow-up visits were collected. Documented complications were reported. Caregivers' satisfaction was assessed with a survey. The survey used to assess satisfaction with cleft-related features was based on the cleft evaluation profile (CEP). In addition, 4 assessors used visual analog scale (VAS) to assess the aesthetic satisfaction. Seven hundred fifty-two patients with primary diagnosis of cleft lip and/or palate underwent surgical correction at "S. Chiara" Hospital, 432 (57.45%) male and 320 (42.55%) female. The most common cleft types in our study were incomplete cleft palate (152 patients) and left unilateral complete cleft lip and palate (152 patients). Associated syndromes were found in 46 patients (6.12%). Cleft lip was repaired using a modified Tennison-Randall technique when the defect was unilateral, whereas a modified Mulliken technique was used for bilateral cleft lip. Cleft palate was repaired using the Bardach technique or Von Langenbeck technique at 5 to 6 months of age. Cleft lip and palate was repaired in several surgical steps. In total, complications were reported in 81 of 752 patients (14.16%). Average fathers' satisfaction score assessed using CEP was 4.5 (lip), 4.8 (nose), 4.7 (teeth), 4.8 (bite), 4.2 (breathing), 4.6 (profile). Average mothers' satisfaction score assessed using CEP was 4.3 (lip), 4.6 (nose), 4.4 (teeth), 4.5 (bite), 4.1 (breathing), 4.4 (profile). Average level of aesthetic satisfaction, assessed using VAS, was 8.7 (fathers), 8.1 (mothers), 7.9 (lay person), and 8.0 (senior cleft surgeon). The multidisciplinary management of children with oral cleft determinated good results, in terms of satisfaction and aesthetic appearance. From our experience, periosteoplasty and lip-adhesion are key surgical techniques.

A cross-sectional analysis of the prevalence of tooth agenesis and structural dental anomalies in association with cleft type in non-syndromic oral cleft patients.

PubMed

Konstantonis, Dimitrios; Alexandropoulos, Alexandros; Konstantoni, Nikoleta; Nassika, Maria

2017-12-01

The aim of this study was to investigate the prevalence of tooth agenesis, microdontia, and tooth malformation among non-syndromic oral cleft patients and their potential association with cleft type and gender. Intraoral records and radiographs of 154 patients (97 males and 57 females) were examined. The variables assessed were tooth agenesis, microdontia, dental malformations, and cleft types. The statistics included chi-square and Fisher's exact tests as well as logistic regression to assess any mutual effects of gender and cleft type on the dental variables. Tooth agenesis occurred in 50% of the sample and microdontia in 18%. Non-statistically significant odds ratios for the association of gender and cleft type with tooth agenesis were obtained. Tooth agenesis was substantially higher at the unilateral right CL + P and the bilateral CL + P in quadrant 1 and at the unilateral left CL + P and bilateral CL + P in quadrant 2. It was also higher, at the isolated cleft palate (CP) in quadrants 3 and 4. These results were attributed to teeth 22 (31.8%) and 12 (21.6%) in the maxilla and to teeth 35 (6.1%) and 45 (5.4%) in the mandible. In unilateral CL + P patients, the cleft quadrant that presented tooth agenesis was associated with the side of the cleft. Interdisciplinary treatment of the oral cleft patients should take into consideration the high prevalence of tooth agenesis and their association with the different cleft types. The most frequently affected teeth by cleft are by far the upper lateral incisors. Results indicate that tooth agenesis appears to be a genetically controlled anomaly related to the orofacial cleft development through various genetic links and not caused by the cleft disruptive process.

Spectral Analysis of Word-Initial Alveolar and Velar Plosives Produced by Iranian Children with Cleft Lip and Palate

ERIC Educational Resources Information Center

Eshghi, Marziye; Zajac, David J.; Bijankhan, Mahmood; Shirazi, Mohsen

2013-01-01

Spectral moment analysis (SMA) was used to describe voiceless alveolar and velar stop-plosive production in Persian-speaking children with repaired cleft lip and palate (CLP). Participants included 11 children with bilateral CLP who were undergoing maxillary expansion and 20 children without any type of orofacial clefts. Four of the children with…

Prevalence of abnormalities in dental structure, position, and eruption pattern in a population of unilateral and bilateral cleft lip and palate patients.

PubMed

Tortora, Chiara; Meazzini, Maria C; Garattini, Giovanna; Brusati, Roberto

2008-03-01

To evaluate the dental characteristics of patients subjected to a protocol that included early secondary gingivoalveoloplasty (ESGAP). Panoramic radiographs of 87 patients with unilateral cleft lip and palate (UCLP) and 29 with bilateral cleft lip and palate (BCLP) were evaluated. Missing and supernumerary teeth were also quantified on the cleft and noncleft side and in the maxilla and mandible. Crown and root malformations and tooth rotations were quantified. A subsample in permanent dentition was extrapolated to analyze canine eruption patterns. A total of 48.8% of the UCLP patients presented with missing permanent lateral incisors in the cleft area and 6.1% contralaterally. A total of 4.9% presented with missing second maxillary premolars on the cleft site and 1.2% contralaterally. A total of 7.3% presented with supernumerary lateral incisors, and 45% of the BCLP cleft sites presented with missing lateral incisors, while 25% of the cleft sites presented second maxillary premolars agenesis. Five percent of the cleft sites presented with supernumerary lateral incisors. Evaluation of the subsample in permanent dentition showed that 15.5% had a canine retention and 4.4% of the canines had to be surgically exposed. A significant association was observed between canine inclination and retention but not with absence of the lateral incisor. The frequency of dental anomalies in this sample was similar to other cleft populations. As surgical trauma has been suggested to damage forming teeth, the results of this study indicated that ESGAP has no detrimental influence on subsequent dental development.

Oblique lip-alveolar banding in patients with cleft lip and palate.

PubMed

Naidoo, S; Bütow, K-W

2015-04-01

We report an oblique lip-alveolar band, a rare banding of soft tissue that involves the lip and alveolus, which we have found in five patients with cleft lip and palate (0.2%), compared with an incidence of the Simonartz lip-lip band of 5.7%). To our knowledge this has not been reported previously. In two patients the bands affected the cleft lip and alveolus bilaterally, with or without the palatal cleft, and in three the bands were unilateral cleft lip and alveolus with or without the palatal cleft. Copyright © 2015 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Assessing the effectiveness of massage therapy for bilateral cleft lip reconstruction scars.

PubMed

McKay, Emilie

2014-06-01

Bilateral cleft lips occur when the bones that form the upper lip fail to fuse at birth. Surgical reconstruction creates scars, which may lead to the following impairments: adhesions, decreased oral range of motion, decreased strength of orbicularis oris muscle, and asymmetry of oral region leading to poor self-esteem. The purpose of this case study is to determine the effectiveness of massage therapy in its ability to improve these impairments. A five-week treatment plan consisting of fascial release, kneading, and intraoral techniques. Subjective information was assessed on two scales: restriction of scar and clients attitudes and acceptance of scar. Objective information was cataloged through photographs, a palpatory chart, and a self-created "Pen Test". Results included increase range of motion and strength, decrease restrictions (palpable and subjective), and increase of symmetry. Client's perceived confidence of scar and its appearance increased. The evidence suggests that massage therapy helped with the impairments associated with scars formed by bilateral cleft lip reconstruction.

A Comparative Study to Evaluate the Functional Effect of Unilateral Uvuloplasty after Primary Palatoplasty

PubMed Central

Olivencia-Flores, Claudia; Gavino-Gutierrez, Arquímedes M.; Caceres-Nano, Evelyn; Cotrina-Rabanal, Omar

2015-01-01

Background: The conventional method for uvular repair suturing the 2 hemi-uvulas of the palatal cleft together in the midline does not allow us to obtain a proper anatomical repair. In our hands, the midline straight closure frequently causes retraction of the uvular tissues with the consequent abnormal appearance of the uvula. We described before a method for uvular repair in patients with cleft palate. The technique consists in preserving one of the hemi-uvulas, which is moved to the midline to form the definitive uvula. The purpose of this study was to evaluate the functional effects of the unilateral uvuloplasty for uvular repair in a group of patients with bilateral cleft palate. Methods: This is a retrospective, single-blinded cohort study between 2 groups of 90 patients with bilateral cleft palate who were operated on using the conventional and unilateral uvuloplasty methods of uvular repair from 2000 to 2009. Data collection was accomplished by physical examination to evaluate the presence of postoperative fistulas and hypernasal speech determined at 6 months to 5 years after surgery. In addition, postoperative dimensions of the velopharynx were measured by a single-blind examiner using a computed tomography scan. Results: Our comparative study found statistically significant differences between the 2 groups in favor of the unilateral uvuloplasty group. Conclusions: We observed that the use of unilateral uvuloplasty for uvular reconstruction reduces the velopharyngeal space and the frequency of hypernasality in patients with bilateral cleft palate. PMID:26180716

Radiographic Study of the Prevalence and Distribution of Hypodontia Associated with Unilateral and Bilateral Clef Lip and Palate in a Hungarian Population

PubMed Central

Berniczei-Roykó, Ádám; Tappe, Jan-Hendrik; Krinner, Axel; Gredes, Tomasz; Végh, András; Gábor, Katona; Linkowska-Świdzińska, Kamila; Botzenhart, Ute Ulrike

2016-01-01

Background Cleft defects are one of the most frequent birth-deformities of the orofacial region and they are commonly associated with anomalies of the tooth structure, size, shape, formation, eruption, and tooth number. The aim of our study was to evaluate the prevalence, distribution, and potential association of combined hypodontia in cleft-affected patients with regard to all types of teeth in both jaws in the permanent dentition. Material/Methods This retrospective radiographic analysis included patients with various types of clefts treated orthodontically in the Department of Orofacial Orthopedics and Orthodontics at Heim Pàl Children’s Hospital, Budapest. There were 150 patients (84 males, 66 females) with non-syndromic unilateral (UCLP; n=120 patients) or bilateral (BCLP; n=30 patients) cleft formation (lip, alveolus and palate) who met the inclusion criteria. Statistical analysis was performed using the chi-square test and Fisher’s exact test (significance level p<0.05). Results Hypodontia was significantly more frequent in patients with cleft-sided lateral incisor (104 patients, 69%), with a total of 235 missing teeth, followed by the second premolars of the upper and lower jaw. A significant correlation of congenital missing teeth was observed in left-sided clefts between the upper and lower second premolar in the cleft area. Conclusions Hypodontia inside and outside the cleft area was frequently observed. This should affect the therapy plans, especially if the cleft-sided premolar is also absent. Further comprehensive research including numerous random samples is necessary for better estimating other possible associations. PMID:27767023

Modified SARME (Surgically Assisted Rapid Maxillary Expansion) in Conjunction with Orthodontic Treatment-A Case Report.

PubMed

Dahiya, Sagar; Chitra, Prasad; Rao, Sadam Srinivas; Bindra, Sukhvinder

2015-10-01

Transverse maxillary hypoplasia or maxillary constriction in conjunction with unilateral or bilateral posterior cross bites is a common finding in cleft palate patients. These situations are also commonly encountered in adults who have not had recourse to orthodontic treatment in childhood. In adults, after ossification of the mid palatal suture is complete, the accepted means of correcting transverse skeletal discrepancies is by Surgically Assisted Rapid Maxillary Expansion (SARME). The disadvantage of this technique in the Indian scenario is reduced patient acceptance and increased treatment costs. Le Fort-I down fracture and mid palatal suture sectioning requires hospitalization and increases morbidity. A case of a 21-year-old non-cleft male who presented with Class I malocclusion with transverse skeletal discrepancy and bilateral posterior cross bites is presented. A modified SAARME technique was performed without pterygomaxillary disjunction, as an outpatient procedure. The results obtained were satisfactory and the desired amount of transverse skeletal correction was achieved. The patient was discharged the same day. The technique can be used to successfully treat a large number of patients in India with maxillary skeletal transverse problems with increased predictability, reduced costs and morbidity and higher rates of acceptance.

The impact of a single surgical intervention for patients with a cleft lip living in rural Ethiopia.

PubMed

Fell, Matthew J; Hoyle, Tom; Abebe, Mekonen E; Kebede, Yegeremu; Medhin, Yohannes D; Hiwot, Fikre A; Cifeta, Taye H; Ali, Ibrahim M; McGurk, Mark

2014-09-01

Humanitarian organisations commonly provide reconstructive treatment for patients with cleft lip within developing countries, but follow-up is often non-existent, particularly for those living in rural areas. This study aimed to assess whether a single surgical intervention was sufficient to produce an observable change to the life of a patient with cleft lip living in rural Hararghe in eastern Ethiopia. 356 patients with isolated cleft lips, who received a single surgical treatment at least 6 months previously, were evaluated in 21 rural health centres. Patients and their families expressed unhappiness before treatment, mainly because the society reacted negatively towards the deformities, isolating the patients from community activities. Postoperatively, the percentage of school-aged children participating in education increased from 46% to 79%, some older patients were able to marry, but employment was unaffected. The prevalence of wound dehiscence in the lip repair was 3% and occurred more frequently in patients with a bilateral cleft lip compared to a unilateral cleft lip (p < 0.001, RR 49.25, 95% CI 6.7-1037.35). We demonstrated that follow-up can be achieved by charitable organisations treating rural patients and that the majority of patients report a positive impact following surgical intervention. We recommend that bilateral cleft lips have a more intense rural aftercare. Copyright © 2014 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

Influence of Propolis on Hygiene, Gingival Condition, and Oral Microflora in Patients with Cleft Lip and Palate Treated with Fixed Orthodontic Appliances

PubMed Central

Machorowska-Pieniążek, Agnieszka; Morawiec, Tadeusz; Tanasiewicz, Marta; Król, Wojciech

2013-01-01

The aim of this study was to evaluate the influence of 3% ethanol extract of propolis (EEP) on hygiene, gingival and microbiological status of oral cavity in patients with cleft lip and palate treated with fixed orthodontic appliances. The study included forty-one nonsyndromic complete unilateral of bilateral cleft lip and palate subjects with fixed appliance on at least 10 teeth. Twenty-one subjects were instructed to brush their teeth three times a day using toothpaste with propolis. Control group included twenty subjects who were asked to brush their teeth three times a day using a toothpaste without propolis. API, OPI, GI, and supragingival bacterial plaque were taken from each subject twice: baseline and after using the toothpaste for 35 days. The final examinations showed statistically significant decrease in OPI, GI, and the percentage of the Actinomyces spp. and Capnocytophaga spp. compared with baseline in propolis group subjects. The improvement in oral health in these patients confirms antibacterial, anti-inflammatory, and regenerative properties of propolis. PMID:23762106

Phonological Proficiency of Two Cleft Palate Toddlers with School-Age Follow-Up.

ERIC Educational Resources Information Center

Lynch, Joan I.; And Others

1983-01-01

A comprehensive analysis of the speech-sound production at ages two-three, five, and seven of two children with repaired bilateral cleft lip and palate. Results indicated individual differences in that one S's emerging phonological system was more characteristic of developmental delay while the other was more characteristic of structural…

Soft tissue molding technique in cleft lip and palate patient using laser surgery in combination with orthodontic appliance: A case report.

PubMed

Theerasopon, Pornpat; Wangsrimongkol, Tasanee; Sattayut, Sajee

2017-03-31

Although surgical treatment protocols for cleft lip and palate patients have been established, many patients still have some soft tissue defects after complete healing from surgical interventions. These are excess soft tissue, high attached fraena and firmed tethering scares. These soft tissue defects resulted shallowing of vestibule, restricted tooth movement, compromised periodontal health and trended to limit the maxillary growth. The aim of this case report was to present a method of correcting soft tissue defects after conventional surgery in cleft lip and palate patient by using combined laser surgery and orthodontic appliance. A bilateral cleft lip and palate patient with a clinical problem of shallow upper anterior vestibule after alveolar bone graft received a vestibular extension by using CO 2 laser with ablation and vaporization techniques at 4 W and continuous wave. A customized orthodontic appliance, called a buccal shield, was placed immediately after surgery and retained for 1 month to 3 months until complete soft tissue healing. The procedures were performed 2 episodes. Both interventions used the same CO 2 laser procedure. The first treatment resulted in partial re-attachment of soft tissue at surgical area. The second laser operation with the proper design of buccal shield providing passive contact with more extended flange resulting in a favorable outcome from 1 year follow up. Then the corrective orthodontic treatment could be continued effectively. The CO 2 laser surgery was a proper treatment for correcting soft tissue defects and the design of buccal shield was a key for success in molding surgical soft tissue.

A simple mechanism for measuring and adjusting distraction forces during maxillary advancement.

PubMed

Suzuki, Eduardo Yugo; Suzuki, Boonsiva

2009-10-01

Direct measurement of distraction forces on the craniofacial skeleton has never been reported. The present report describes the development of a method of assessing and adjusting traction forces applied through maxillary distraction osteogenesis. A simple mechanism to measure and adjust tension force during maxillary distraction osteogenesis was developed and connected bilaterally to the traction screws of a rigid external distraction device. Measurements were carried out before and after activation using a Shimpo (Nidec-Shimpo America Corporation, Itasca, IL) force gauge in 4 patients (2 with unilateral cleft lip and/or palate, 1 with bilateral cleft lip and palate, and 1 with noncleft) during the distraction process. Activation was performed twice a day at a rate of 1 mm/day. The average maximum force applied throughout the distraction period was 42.5 N (range 16.4 to 65.3 N), with increments, after activation, averaging 10.5 N (range 7.9 to 15.7 N). In patients with unilateral cleft lip and/or palate, distraction forces on the larger segment were 65.1% higher than on the lesser segment. A differential pattern of forces was also observed in the patients with asymmetric noncleft. However, the differential forces between lateral segments were not observed in the patient with bilateral cleft lip and palate. During the activation period, distraction forces progressively increased, whereas the amount of maxillary movement decreased. Pain and discomfort were reported with high forces. Through this mechanism, direct measurement and adjustment of distraction forces during maxillary advancement was possible. The unbalanced pattern of forces observed in patients with cleft suggests the necessity of individual adjustments for controlling pain and clinical symptoms. Accordingly, assessment of distraction forces during maxillary distraction osteogenesis is extremely helpful in understanding the biomechanics of the distraction process.

The effect of endoscopic olfactory cleft polyp removal on olfaction.

PubMed

Kuperan, Arjuna B; Lieberman, Seth M; Jourdy, Deya N; Al-Bar, Mohammad H; Goldstein, Bradley J; Casiano, Roy R

2015-01-01

The presence of olfactory cleft polyps in chronic rhinosinusitis with nasal polyposis is well documented, but the effect of endoscopic olfactory cleft polyp surgery on olfaction, versus observation, has not been well studied. This analysis assessed if microdebridement of olfactory cleft polyps yields significant objective smell improvements in those with anosmia or hyposmia. A randomized prospective single-blinded study was performed on patients undergoing bilateral endoscopic sinus surgery with profound bilateral nasal polyposis, excluding those younger than 18 years or without olfactory polyps. A preoperative University of Pennsylvania Smell Identification Test (UPSIT), visual analog scale (VAS), and sinonasal outcomes 20 score (SNOT-20), and a follow-up at 6 months was performed. Two cohorts were created, including one with cleft polyp removal (group A) and one with cleft polyps left in place (group B). There were 10 patients in group A and 7 in group B. By using the Wilcoxon signed rank test, the two groups were individually analyzed for changes in the preoperative UPSIT, VAS, and SNOT-20 versus the 6-month test results. In group A, the improvement in the UPSIT, VAS, and SNOT-20 were statistically significant at p < 0.05. For group B only the improvement in the VAS was statistically significant, at p < 0.05. There was a statistically significant difference in clinical smell improvement between group A and B at 6 months (p = 0.00512). Evidence exists that olfactory cleft polyp surgery improves olfactory function outcomes. Long-term data beyond 6 months is needed to further validate these early promising outcomes.

Slow versus rapid maxillary expansion in bilateral cleft lip and palate: a CBCT randomized clinical trial.

PubMed

de Almeida, Araci Malagodi; Ozawa, Terumi Okada; Alves, Arthur César de Medeiros; Janson, Guilherme; Lauris, José Roberto Pereira; Ioshida, Marilia Sayako Yatabe; Garib, Daniela Gamba

2017-06-01

The purpose of this "two-arm parallel" trial was to compare the orthopedic, dental, and alveolar bone plate changes of slow (SME) and rapid (RME) maxillary expansions in patients with complete bilateral cleft lip and palate (BCLP). Forty-six patients with BCLP and maxillary arch constriction in the late mixed dentition were randomly and equally allocated into two groups. Computer-generated randomization was used. Allocation was concealed with sequentially, numbered, sealed, opaque envelopes. The SME and RME groups comprised patients treated with quad-helix and Haas/Hyrax-type expanders, respectively. Cone-beam computed tomography (CBCT) exams were performed before expansion and 4 to 6 months post-expansion. Nasal cavity width, maxillary width, alveolar crest width, arch width, palatal cleft width, inclination of posterior teeth, alveolar crest level, and buccal and lingual bone plate thickness were assessed. Blinding was applicable for outcome assessment only. Interphase and intergroup comparisons were performed using paired t tests and t tests, respectively (p < 0.05). SME and RME similarly promoted significant increase in all the maxillary transverse dimensions at molar and premolar regions with a decreasing expanding effect from the dental arch to the nasal cavity. Palatal cleft width had a significant increase in both groups. Significant buccal inclination of posterior teeth was only observed for RME. Additionally, both expansion procedures promoted a slight reduction of the alveolar crest level and the buccal bone plate thickness. No difference was found between the orthopedic, dental, and alveolar bone plate changes of SME and RME in children with BCLP. Both appliances produced significant skeletal transverse gains with negligible periodontal bone changes. Treatment time for SME, however, was longer than the observed for RME. SME and RME can be similarly indicated to correct maxillary arch constriction in patients with BCLP in the mixed dentition.

Whole-genome sequencing in a pair of monozygotic twins with discordant cleft lip and palate subtypes.

PubMed

Takahashi, Masahiro; Hosomichi, Kazuyoshi; Yamaguchi, Tetsutaro; Nagahama, Ryo; Yoshida, Hiroshi; Maki, Koutaro; Marazita, Mary L; Weinberg, Seth M; Tajima, Atsushi

2018-06-06

Orofacial clefts (OFCs) are common and aetiologically complex birth defects. This study explored potential genetic differences in a pair of Japanese monozygotic (MZ) twins with different forms of OFC using whole-genome sequencing. One co-twin (MZ-1) presented with non-syndromic bilateral cleft lip and palate; the other co-twin (MZ-2) had non-syndromic bilateral cleft lip and unilateral left-sided cleft alveolus. Neither parent had an OFC. Craniofacial morphologic features and potential genetic differences were compared using standard cephalometry and whole-genome sequencing, respectively. Morphologically, MZ-1 had a smaller vertical mandibular height, compared to MZ-2. However, no discordant genetic differences were detected. Moreover, both twins and their parents harboured rare candidate gene variants (GRHL3; TPM1) considered to be associated with OFCs. The observed differences between MZ-1 and MZ-2 in craniofacial morphology assessed by cephalograms might be directly attributable to the effects of the OFC on growth and/or differences in surgical history, given the lack of any differences in genetic background. However, comparisons of discordant MZ twins should continue to identify novel candidates that might control OFC or that might partly explain the missing heritability for this common birth defect, in addition to understanding craniofacial growth and development. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

Induction of cleft palate in newborn pigs by maternal ingestion of poison hemlock (Conium maculatum).

PubMed

Panter, K E; Keeler, R F; Buck, W B

1985-06-01

Cleft palates were induced in newborn pigs of gilts fed Conium maculatum seed or plant during gestation days 30 through 45. Twelve of 23 newborn pigs born to 3 gilts given Utah-grown C maculatum seed and 9 of 12 newborn pigs born to a single gilt given the fresh Utah spring-growth C maculatum plant had cleft palates. The cleft palates ranged from a unilateral cleft, involving only 1 side of the palate, to a full bilateral cleft. Brachygnathia was also observed in some of these newborn pigs with cleft palate. Other malformations were not observed. Chemical analysis of seed and plant samples indicated that gamma-coniceine was the responsible teratogenic alkaloid. A daily dose of plant or seed that provided greater than or equal to 1.07 mg of gamma-coniceine/kg of body weight fed to gilts during the 30th through the 45th day of pregnancy resulted in teratogenic effects.

Maxillary Distraction Osteogenesis Using a Rigid External Distractor: Which Clinical Factors Are Related With Relapse?

PubMed

Kim, Jeenam; Uhm, Ki-Il; Shin, Donghyeok; Lee, Jina; Choi, Hyungon

2015-06-01

Maxillary distraction osteogenesis is a reliable treatment for cleft lip and palate with midfacial retrusion. The purpose of this study was to evaluate the results of long-term follow-up in patients with cleft lip and palate after maxillary distraction osteogenesis and to find clinical factors related to relapse. From February 2002 to June 2008, 21 patients with severe class III malocclusion were treated at our hospital. We performed distraction osteotomy with a rigid external distractor device. The distraction length was more than 15 mm in all patients. Preoperative and postoperative lateral cephalometric radiographs were used for analysis. The sella-nasion-subnasale, sella-nasion-supramentale, and point-A-point-B-nasion (sella-nasion-subnasale-sella-nasion-supramentale) angles were recorded. The timelines for follow-up were preoperatively, after distraction, after consolidation, at 3 years, and once fully grown (5- to 8-year follow-ups). A comparative analysis of clinical factors was performed for the relapsing and nonrelapsing groups. Of the 21 patients, 14 had relapsed. The mean age in the relapsing group was 9.1 years (7 boys and 7 girls) with 9 patients with unilateral cleft palate and 5 c bilateral cleft palate. The mean age in the nonrelapsing group was 11.7 years (4 boys and 3 girls) with 5 patients with unilateral cleft palate and 2 patients with bilateral cleft palate. Despite greater anterior overcorrection, relapse occurred owing to scar tissue retraction and mandibular compensatory hypertrophy. The results suggest that the younger the patient, the more likely relapse will occur.

The role of Nasoalveolar molding: A 3D Prospective analysis.

PubMed

Chou, Pang-Yun; Hallac, Rami R; Ajiwe, Tochi; Xie, Xian-Jin; Liao, Yu-Fang; Kane, Alex A; Park, Yong Jong

2017-08-29

Nasoalveolar molding (NAM) is commonly employed to reduce the alveolar segments into proper alignment and to improve nasal symmetry in patients with cleft lip and palate. This study examines the periodical progression of NAM treatment over time. 20 patients with complete unilateral cleft lip and palate were prospectively recruited. A 2 stage NAM treatment protocol was applied. Stage 1 involved adjustment of the alveolar segments (mean age 15.6 days), while Stage 2 added nasal stents and started average 43 days after stage 1. 3D images (n = 241) were obtained prior to NAM initiation and weekly until the end of treatment. The cleft lip area, bilateral nostril areas, and the nostril height and width were measured. Treatment was assessed in the Cleft (C) side and the Non-cleft (N). There was significant difference in the C/N ratio of the nostril area, width, and height at pre-treatment (0.9 ± 0.3, 4.1 ± 1.1, and 0.5 ± 0.2), at the end of stage 1 (1.1 ± 0.3, 2.2 ± 0.6, and 0.8 ± 0.2), and at the end of stage 2 treatment (1.8 ± 0.3, 1.8 ± 0.4, and 1.2 ± 0.1); p < 0.05. Comparative 3D analysis with dense sampling offers a precise methodology for showing effects of NAM treatment. The morphological changes achieved with NAM therapy occur in early treatment phase.

Microdeletion del(22)(q12.2) encompassing the facial development-associated gene, MN1 (meningioma 1) in a child with Pierre-Robin sequence (including cleft palate) and neurofibromatosis 2 (NF2): a case report and review of the literature.

PubMed

Davidson, Tom B; Sanchez-Lara, Pedro A; Randolph, Linda M; Krieger, Mark D; Wu, Shi-Qi; Panigrahy, Ashok; Shimada, Hiroyuki; Erdreich-Epstein, Anat

2012-03-22

Pierre-Robin sequence (PRS) is defined by micro- and/or retrognathia, glossoptosis and cleft soft palate, either caused by deformational defect or part of a malformation syndrome. Neurofibromatosis type 2 (NF2) is an autosomal dominant syndrome caused by mutations in the NF2 gene on chromosome 22q12.2. NF2 is characterized by bilateral vestibular schwannomas, spinal cord schwannomas, meningiomas and ependymomas, and juvenile cataracts. To date, NF2 and PRS have not been described together in the same patient. We report a female with PRS (micrognathia, cleft palate), microcephaly, ocular hypertelorism, mental retardation and bilateral hearing loss, who at age 15 was also diagnosed with severe NF2 (bilateral cerebellopontine schwannomas and multiple extramedullary/intradural spine tumors). This is the first published report of an individual with both diagnosed PRS and NF2. High resolution karyotype revealed 46, XX, del(22)(q12.1q12.3), FISH confirmed a deletion encompassing NF2, and chromosomal microarray identified a 3,693 kb deletion encompassing multiple genes including NF2 and MN1 (meningioma 1).Five additional patients with craniofacial dysmorphism and deletion in chromosome 22-adjacent-to or containing NF2 were identified in PubMed and the DECIPHER clinical chromosomal database. Their shared chromosomal deletion encompassed MN1, PITPNB and TTC28. MN1, initially cloned from a patient with meningioma, is an oncogene in murine hematopoiesis and participates as a fusion gene (TEL/MN1) in human myeloid leukemias. Interestingly, Mn1-haploinsufficient mice have abnormal skull development and secondary cleft palate. Additionally, Mn1 regulates maturation and function of calvarial osteoblasts and is an upstream regulator of Tbx22, a gene associated with murine and human cleft palate. This suggests that deletion of MN1 in the six patients we describe may be causally linked to their cleft palates and/or craniofacial abnormalities. Thus, our report describes a NF2-adjacent chromosome 22q12.2 deletion syndrome and is the first to report association of MN1 deletion with abnormal craniofacial development and/or cleft palate in humans.

Presurgical Nasoalveolar Molding for Correction of Cleft Lip Nasal Deformity: Experience From Northern India

PubMed Central

Mishra, Brijesh; Singh, Arun K.; Zaidi, Javed; Singh, G. K.; Agrawal, Rajiv; Kumar, Vijay

2010-01-01

Context: The cleft lip type nasal deformity presents one of the most complex surgical challenges. The long-term postoperative results are still not satisfactory despite an emphasis on primary nasal correction. This is attributed to tissue memory and healing. Nasoalveolar molding is used effectively to reshape the nasal cartilage and to mold the maxillary arch before cleft lip repair. Aims: This study was undertaken to evaluate the role of presurgical nasoalveolar molding in correction of cleft lip nasal deformity for patients with unilateral and bilateral clefts of the lip. Settings and Design: Twenty-three cases of clefts of lip and palate with nasal deformity were subjected to present study from May 2004 to May 2006. These cases were initially treated on outpatient basis, and they were admitted at the time of operation. All of these patients were children of less than 1 year of age, belonging to north Indian population. Material and Methods: Study consisted of patients of cleft lip and palate who were given presurgical nasoalveolar splints at early age. Lip repair was done after at least 2 months of molding. These patients along with control group (without presurgical nasoalveolar molding) were followed up for 1 year. Measurements were taken at different intervals in study over dental cast and on patients. Data obtained from comparison of 2 groups were analyzed using “MSTAT” analysis software (developed by Dr Russel Freed, Professor & Director, Crop & Soil Sciences Department, Michigan State University, East Lansing, Michigan). Results: In our study, we found that nostril height was more in patients of experimental group (P = .18), while nostril width and alar perimeter were not changed significantly. Children with nasoalveolar molding had significant lengthening of columella (P = .02). Patients of unilateral cleft lip had more reduction in alveolar gap (P = .08) than bilateral group (P = .15). Conclusions: Nasoalveolar molding can be a useful adjunct for treatment of cleft lip nasal deformity. It is a cost-effective technique that can reduce the number of future surgeries such as alveolar bone grafting and secondary rhinoplasties. PMID:20694165

Maxillary distraction in patients with cleft deformity using a rigid external distraction device: a pilot study on the distraction ratio of the maxilla to the device.

PubMed

Harada, Kiyoshi; Sato, Masaru; Omura, Ken

2004-01-01

We examined the ratio between actual maxillary distraction and the distraction of the rigid external distraction device (Rigid external distraction (RED) system) used for maxillary distraction in patients with a cleft deformity. Twelve patients were examined. The amount of maxillary advancement was measured on lateral cephalograms and divided by the activation amount on the RED system. The value obtained was represented as the distraction ratio of the maxilla to the system. The mean ratio in 10 patients with complete cleft lip, palate, and alveolus (complete cleft) was 0.24. However, the ratios in two patients with cleft lip and alveolus or soft cleft palate (incomplete cleft) were considerably higher than the mean ratios in patients with complete cleft. When the maxilla is distracted in patients with complete cleft using the RED system, the amount of activation on the system needs to be about four times the amount of planned maxillary distraction. However, the distraction ratio may be affected by the type of cleft.

Dental anomalies in children with cleft lip and palate in Western Australia.

PubMed

Nicholls, Wendy

2016-01-01

The purpose of this paper was to describe the prevalence and type of dental anomalies in the primary and permanent dentition in children with a cleft condition at Princess Margaret Hospital in Perth, Western Australia. The details of 162 current dental patients extracted from the main dental database through their year of birth for the period 1998-2001 were selected consecutively. Dental records and X-rays were examined by one examiner (WN) and verified by a second examiner (RB) to determine dental development. The mean age of the subjects was 10.8 years with equal numbers of males and females. Subjects were further divided into cleft type; unilateral cleft lip (UCL) and palate, bilateral cleft lip (BCL) and palate, UCL, BCL, and cleft palate. One hundred sixty-two subjects were grouped into 21 categories of anomaly or abnormality. Prevalence rates for the categories were calculated for the overall group and for gender and cleft type. Overall, 94% of patients were found to have at least one dental anomaly, with fifty-six (34%) patients having more than one anomaly or abnormality.

Presurgical nasoalveolar molding: A boon to facilitate the surgical repair in infants with cleft lip and palate

PubMed Central

Attiguppe, Prabhakar Ramasetty; Karuna, Y. M.; Yavagal, Chandrashekar; Naik, Saraswathi V.; Deepak, B. M.; Maganti, Rekhamani; Krishna, Chaithanya G.

2016-01-01

Cleft lip and palate (CLP) is the most common congenital craniofacial anomaly. Rehabilitation of CLP generally requires a team approach. Alveolar and nasal reconstruction for these patients is a challenge for the reconstructive surgeon. Various procedures have been attempted to reduce the cleft gap, so as to obtain esthetic results postsurgically. The presurgical nasoalveolar molding (PNAM) technique, developed by Grayson, is a new approach to presurgical infant orthopedics. PNAM reduces the severity of the initial cleft alveolar and nasal deformity. Thus, it enables the surgeon and the patient to enjoy the benefits associated with repair of a cleft deformity that is minimal in severity. This article presents a brief insight into PNAM with a case series of three different cases (one unilateral and two bilateral) which underwent PNAM treatment and gave an excellent surgical prognosis. PMID:27994432

Cone Beam Computed Tomography Analysis of Oropharyngeal Airway in Preadolescent Nonsyndromic Bilateral and Unilateral Cleft Lip and Palate Patients.

PubMed

Al-Fahdawi, Mahmood Abd; El-Kassaby, Marwa Abdelwahab; Farid, Mary Medhat; El-Fotouh, Mona Abou

2018-01-01

Objective The objective of this study was to assess the volume, area, and dimensions of the oropharyngeal airway (OPA) in a previously repaired nonsyndromic unilateral cleft lip and palate (UCLP) versus bilateral cleft lip and palate (BCLP) patients when compared with noncleft controls using cone beam computed tomography (CBCT). Design This was a retrospective case-control study. Setting The Cleft Care Center and outpatient clinic that are affiliated to our faculty were the settings for the study. Participants A total of 58 CBCT scans were selected of preadolescent individuals: 14 BCLP, 20 UCLP, and 24 age- and gender-matched noncleft controls. Variables Variables were volume, cross-sectional area (CSA), midsagittal area (MSA), and dimensions of OPA. Statistical analysis One-way analysis of variance and post hoc tests were used to compare variables. Statistical significance was set at P ≤ .05. Results UCLP showed significantly smaller superior oropharyngeal airway volume than both controls and BCLP ( P ≤ .05). BCLP showed significantly larger CSA at soft palate plane and significantly larger MSA than both UCLP and controls ( P < .05). Conclusions UCLP patients at the studied age and stage of previously repaired clefts have significantly less superior oropharyngeal airway volume than both controls and BCLP patients. This confirms that preadolescents with UCLP are at greater risk for superior oropharyngeal airway obstruction when compared with those BCLP and controls. Furthermore, BCLP patients showed significantly larger CSA at soft palate plane and MSA than both controls and UCLP patients. These variations in OPA characteristics of cleft patients can influence function in terms of respiration and vocalization.

Preliminary Analysis of the 3-Dimensional Morphology of the Upper Lip Configuration at the Completion of Facial Expressions in Healthy Japanese Young Adults and Patients With Cleft Lip.

PubMed

Matsumoto, Kouzou; Nozoe, Etsuro; Okawachi, Takako; Ishihata, Kiyohide; Nishinara, Kazuhide; Nakamura, Norifumi

2016-09-01

To develop criteria for the analysis of upper lip configuration of patients with cleft lip while they produce various facial expressions by comparing the 3-dimensional (3D) facial morphology of healthy Japanese adults and patients with cleft lip. Twenty healthy adult Japanese volunteers (10 men, 10 women, controls) without any observed facial abnormalities and 8 patients (4 men, 4 women) with unilateral cleft lip and palate who had undergone secondary lip and nose repair were recruited for this study. Facial expressions (resting, smiling, and blowing out a candle) were recorded with 2 Artec MHT 3D scanners, and images were superimposed by aligning the T-zone of the faces. The positions of 14 specific points were set on each face, and the positional changes of specific points and symmetry of the upper lip cross-section were analyzed. Furthermore, the configuration observed in healthy controls was compared with that in patients with cleft lip before and after surgery. The mean absolute values for T-zone overlap ranged from 0.04 to 0.15 mm. Positional changes of specific points in the controls showed that the nose and lip moved backward and laterally upward when smiling and the lips moved forward and downward medially when blowing out a candle; these movements were bilaterally symmetrical in men and women. In patients with cleft lip, the positional changes of the specific points were minor compared with those of the controls while smiling and blowing out a candle. The left-versus-right symmetry of the upper lip cross-section exceeded 1.0 mm in patients with cleft lip, which was markedly higher than that in the controls (0.17 to 0.91 mm). These left-versus-right differences during facial expressions were decreased after surgery. By comparing healthy individuals with patients with cleft lip, this study has laid the basis for determining control values for facial expressions. Copyright © 2016 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

Nasal airway and septal variation in unilateral and bilateral cleft lip and palate.

PubMed

Starbuck, John M; Friel, Michael T; Ghoneima, Ahmed; Flores, Roberto L; Tholpady, Sunil; Kula, Katherine

2014-10-01

Cleft lip and palate (CLP) affects the dentoalveolar and nasolabial facial regions. Internal and external nasal dysmorphology may persist in individuals born with CLP despite surgical interventions. 7-18 year old individuals born with unilateral and bilateral CLP (n = 50) were retrospectively assessed using cone beam computed tomography. Anterior, middle, and posterior nasal airway volumes were measured on each facial side. Septal deviation was measured at the anterior and posterior nasal spine, and the midpoint between these two locations. Data were evaluated using principal components analysis (PCA), multivariate analysis of variance (MANOVA), and post-hoc ANOVA tests. PCA results show partial separation in high dimensional space along PC1 (48.5% variance) based on age groups and partial separation along PC2 (29.8% variance) based on CLP type and septal deviation patterns. MANOVA results indicate that age (P = 0.007) and CLP type (P ≤ 0.001) significantly affect nasal airway volume and septal deviation. ANOVA results indicate that anterior nasal volume is significantly affected by age (P ≤ 0.001), whereas septal deviation patterns are significantly affected by CLP type (P ≤ 0.001). Age and CLP type affect nasal airway volume and septal deviation patterns. Nasal airway volumes tend to be reduced on the clefted sides of the face relative to non-clefted sides of the face. Nasal airway volumes tend to strongly increase with age, whereas septal deviation values tend to increase only slightly with age. These results suggest that functional nasal breathing may be impaired in individuals born with the unilateral and bilateral CLP deformity. © 2014 Wiley Periodicals, Inc.

Radiological differences between HIV-positive and HIV-negative children with cholesteatoma.

PubMed

McGuire, J K; Fagan, J J; Wojno, M; Manning, K; Harris, T

2018-07-01

HIV-positive children are possibly more prone to developing cholesteatoma. Chronic inflammation of the middle ear cleft may be more common in patients with HIV and this may predispose HIV-positive children to developing cholesteatoma. There are no studies that describe the radiological morphology of the middle ear cleft in HIV-positive compared to HIV-negative children with cholesteatoma. Compare the radiological differences of the middle ear cleft in HIV-positive and HIV-negative children with cholesteatoma. A retrospective, cross-sectional, observational analytical review of patients with cholesteatoma at our institute over a 6 year period. Forty patients were included in the study, 11 of whom had bilateral cholesteatoma and therefore 51 ears were eligible for our evaluation. HIV-positive patients had smaller (p=0.02) mastoid air cell systems (MACS). Forty percent of HIV-positive patients had sclerotic mastoids, whereas the rate was 3% in HIV-negative ears (p<0.02). Eighty-two percent of the HIV-positive patients had bilateral cholesteatoma compared to 7% of the control group (p<0.02). There was no difference between the 2 groups with regards to opacification of the middle ear cleft, bony erosion of middle ear structures, Eustachian tube obstruction or soft tissue occlusion of the post-nasal space. HIV-positive paediatric patients with cholesteatoma are more likely to have smaller, sclerotic mastoids compared to HIV-negative patients. They are significantly more likely to have bilateral cholesteatoma. This may have implications in terms of surveillance of HIV-positive children, as well as, an approach to management, recurrence and follow-up. HIV infection should be flagged as a risk factor for developing cholesteatoma. Copyright © 2018. Published by Elsevier B.V.

Comparative Study of Early Secondary Nasal Revisions and Costs in Patients With Clefts Treated With and Without Nasoalveolar Molding.

PubMed

Patel, Parit A; Rubin, Marcie S; Clouston, Sean; Lalezaradeh, Frank; Brecht, Lawrence E; Cutting, Court B; Shetye, Pradip R; Warren, Stephen M; Grayson, Barry H

2015-06-01

The present study aims to determine the risk of early secondary nasal revisions in patients with complete unilateral and bilateral cleft lip and palate (U/BCLP) treated with and without nasoalveolar molding (NAM) and examine the associated costs of care. A retrospective cohort study from 1990 to 1999 was performed comparing the risk of early secondary nasal revision surgery in patients with a CLP treated with NAM and surgery (cleft lip repair and primary surgical nasal reconstruction) versus surgery alone in a private practice and tertiary level clinic. The NAM treatment group consisted of 172 patients with UCLP and 71 patients with BCLP, whereas the non-NAM-prepared group consisted of 28 patients with UCLP and 5 with BCLP. The risk of secondary nasal revision for patients with UCLP was 3% in the NAM group and 21% in the non-NAM group. The risk of secondary nasal revision for patients with BCLP was 7% in the NAM group compared with 40% in the non-NAM group. Using multicenter averages, the non-NAM revision rates were calculated at 37.8% and 48.5% for U/BCLP, respectively. Applying these risks of revision, NAM treatment led to an estimated savings of between $491 and $4893 depending on the type of cleft. In conclusion, NAM can reduce the number of early secondary nasal revision surgeries and, therefore, reduce the overall cost of care.

Anatomic Severity, Midfacial Growth, and Speech Outcomes in Van der Woude/Popliteal Pterygium Syndromes Compared to Nonsyndromic Cleft Lip/Palate.

PubMed

Reardon, Jeffrey B; Brustowicz, Katherine A; Marrinan, Eileen M; Mulliken, John B; Padwa, Bonnie L

2015-11-01

To summarize the clinical characteristics and surgical and speech outcomes for patients with Van der Woude/popliteal pterygium syndromes (VWS/PPS) and to compare them with a historic cohort of patients with nonsyndromic cleft lip/cleft palate (CL/P). Retrospective chart review. Tertiary care center. All patients with VWS/PPS seen at Boston Children's Hospital from 1979 to 2012: 28 patients with VWS (n = 21)/PPS (n = 7) whose mean age was 17.3 ± 10.4 years, including 18 females (64%) and 10 males (36%); 18 patients (64%) had a family history of VWS/PPS. Cleft type, operative procedures, speech, and midfacial growth. Data were compared with historic cohorts of patients with nonsyndromic CL/P treated at one tertiary care center. There were 24 patients (86%) with CP±L, Veau types I (n = 4, 17%), II (n = 4, 17%), III (n = 5, 21%), and IV (n = 11, 46%). Nine patients (38%) had palatal fistula after palatoplasty. Fourteen of 23 (61%) patients with CL/P age 5 years or older had midfacial retrusion, and 10 (43%) required a pharyngeal flap for velopharyngeal insufficiency. Fisher's exact test demonstrated higher frequencies of Veau type IV CP±L (P = .0016), bilateral CL±P (P = .0001), and complete CL±P (P < .0001) in VWS/PPS compared with nonsyndromic patients. Incidences of midfacial retrusion (P = .0001), palatal fistula (P < .0001), and need for pharyngeal flap (P = .0014) were significantly greater in patients with VWS/PPS. Patients with VWS/PPS have more severe forms of labiopalatal clefting and higher incidences of midfacial retrusion, palatal fistula, and velopharyngeal insufficiency following primary repair as compared with nonsyndromic CL/P.

Analysis of the dentoalveolar effects of slow and rapid maxillary expansion in complete bilateral cleft lip and palate patients: a randomized clinical trial.

PubMed

de Medeiros Alves, Arthur César; Garib, Daniela Gamba; Janson, Guilherme; de Almeida, Araci Malagodi; Calil, Louise Resti

2016-09-01

The purpose of this study was to compare the dentoalveolar effects of slow (SME) and rapid (RME) maxillary expansions in patients with complete bilateral cleft lip and palate (BCLP). A sample of 50 patients with BCLP and maxillary arch constriction was randomly and equally allocated into two groups. Group SME comprised patients (mean age of 8.8 years) treated with quad-helix appliance. Group RME comprised individuals (mean age of 8.9 years) treated with Hyrax expander. Digital dental models obtained immediately pre-expansion (T1) and 6 months after the active expansion period (T2) were used for measuring maxillary dental arch widths, arch perimeter, arch length, palatal depth, buccolingual inclination of posterior teeth and differential amount of expansion accomplished at the canine and molar regions. Inter-phase and intergroup comparisons were performed using paired t tests and t tests, respectively (p < 0.05). SME and RME caused significant increase of arch widths and arch perimeter. Arch length and palatal depth decreased nonsignificantly with SME but significantly with RME. Buccal tooth inclination was significant only for maxillary deciduous canines in both groups. The quad-helix appliance showed a significant differential expansion between anterior and posterior regions. No differences were observed between SME and RME for all variables. Differences were not found between the dentoalveolar effects of SME and RME in patients with BCLP. SME demanded a greater therapy time compared to RME. Both expansion procedures can be similarly indicated to correct maxillary arch constriction in patients with BCLP in the mixed dentition.

Novel pathomorphologic classification of capsulo-articular lesions of the pubic symphysis in athletes to predict treatment and outcome.

PubMed

Hopp, Sascha; Ojodu, Ishaq; Jain, Atul; Fritz, Tobias; Pohlemann, Tim; Kelm, Jens

2018-05-01

Radiographic abnormalities of the symphysis as well as the formation of accessory clefts, indicating injury at the rectus-adductor aponeurosis, reportedly relate to longstanding groin pain in athletes. However, yet, no systematic classification for clinical and scientific purposes exists. We aimed to (1) create a radiographic classification based on symphysography; (2) test intra- and interobserver reliability; (3) characterise clinical significance of the morphologic patterns by evaluating success of injection therapy. We retrospectively reviewed symphysography, AP radiographs, and MRI of the pelvis from 70 consecutive competitive athletes, with chronic groin pain. Symphysographs were evaluated for intra- and interobserver variance using cohen's kappa statistics. Morphologic studies of the different contrast distribution patterns and their clinical and radiological correlation with symptom relief were investigated. All patients were followed up to evaluate immediate and long-term response to the initial therapeutic injection with steroid. Four reproducible symphysographic patterns were identified: type 0, no changes; type 1, symphyseal disk degeneration; types 2a with unilateral clefts, bilateral clefts (2b), suprapubic clefts (2c); and type 3, with expanded or multidirectional clefts. Analysis revealed excellent intra (0.94)-and interobserver (0.90) reliability. Our findings showed that 78.6% of our patients had significant short-term improvement enabling early resumption of physiotherapy, only in types 1 and 2 (p = 0.001), while type 0 and 3 did not respond. At follow-up, only 21.8% had permanent pain relief. Regarding the detection of pathologic clefts with symphysography, sensitivity (88%) and specifity (77%) were superior to that of MRI. A reproducible symphysography-based classification of distinct morphologic patterns is proposed. It serves as a predictive tool for response to injection therapy in a select group of pathologic lesions. Complete recovery after injection can only be expected in a lesser percentage, as this might indicate surgical treatment for long-term non-responders.

Cleft palate with/without cleft lip in French children: radiographic evaluation of prevalence, location and coexistence of dental anomalies inside and outside cleft region.

PubMed

Mangione, Francesca; Nguyen, Laure; Foumou, Nathalie; Bocquet, Emmanuelle; Dursun, Elisabeth

2018-03-01

Prevalence of dental anomalies in cleft patients is higher than that in general population. The objectives of this study were to assess the prevalence of dental anomalies and their coexistence in French children with cleft and, then, to investigate the relation between the dental anomalies and the cleft type. Seventy-four non-syndromic cleft patients (6-16 years old) from Lille Regional University and Mondor-Chenevier Hospitals (France) were included. Clefts were classified as right/left unilateral cleft lip and palate (UCLP), bilateral cleft lip and palate (BCLP) and cleft palate (CP). Dental anomalies were investigated on panoramic radiographs and categorized as agenesis, supernumerary teeth, incisor rotations, impacted canines and shape anomalies. Prevalence and gender distribution of dental anomalies, mean number of affected teeth per patient, agenesis occurrence and location, and coexistence of dental anomalies were analysed by cleft type. 96.0% of patients presented at least one dental anomaly (agenesis 83.8%, incisor rotations 25.7%, shape anomalies 21.6%, impacted canines 18.9%, supernumerary teeth 8.1%). BCLP patients had a higher number of affected teeth, and left UCLP patients had a higher one compared to right UCLP patients. Distribution of inside (45.3%) and outside (54.7%) cleft region agenesis was similar. Adjacent (31.8%) and not adjacent (33.3%) combined dental anomalies were often encountered. Dental anomalies were localized inside as well as outside cleft region and were often associated with each other. BCLP patients were more affected. Early radiographic evaluation allows a comprehensive diagnosis of inside and outside cleft region anomalies, required for the multidisciplinary dental treatment.

A Longitudinal Study of the Presence of Dental Anomalies in the Primary and Permanent Dentitions of Cleft Lip and/or Palate Patients.

PubMed

Suzuki, Akira; Nakano, Masayuki; Yoshizaki, Keigo; Yasunaga, Atsushi; Haruyama, Naoto; Takahashi, Ichiro

2017-05-01

  The aim is to survey primary and permanent dental anomalies: hypodontia, microdontia, a supernumerary tooth, and fused teeth in patients with cleft lip and/or palate.   Retrospective longitudinal study Subjects :  The subjects were selected from all 1724 patients with cleft lip and/or palate who were registered at the orthodontic clinic of Kyushu University Hospital, Fukuoka, Japan, from 1970 to 2009. Finally, 994 subjects were evaluated for primary dentition, 1352 for permanent dentition, and 871 for the longitudinal changes from primary to permanent dentition.   The prevalence of dental anomalies was compared for each tooth type, among various cleft types, between males and females, and between the alveolar cleft area and the noncleft area.   The prevalence of hypodontia was 16.2% for primary dentition and 52.7% for permanent dentition in the subjects with cleft lip and/or palate. Hypodontia increased with the severity of the cleft type. Multiple hypodontia was found more frequently in the subjects with bilateral cleft lip and palate and the subjects with unilateral cleft lip and palate. Microformed lateral incisors were found in 22.7% of permanent lateral incisors but not in primary dentition. Supernumerary teeth were found in 17.7% of the subjects with cleft lip and/or palate for primary maxillary dentition and in 5.7% for permanent maxillary dentition.   The prevalence of hypodontia was greater in permanent dentition than in primary dentition; although, it was not much different between males and females or between the right and left sides. The prevalence of dental anomalies was significantly different among four groups by cleft type: cleft lip, cleft lip and alveolus, cleft lip and palate, and cleft palate.

Effect of Carbon Dioxide Laser on Increasing Vestibular Depth in Cleft Lip and Palate Patients.

PubMed

Yassaei, Sogra; Aghili, Hossein; Azam, Alireza Navab; Moghadam, Mahjobeh Gholdani; Safari, Isa

2017-09-01

Shallow upper buccal sulcus deformity in cleft lip and palate patients is one of the common secondary deformities after primary cleft lip and palate repair; this deformity may prevent or complicate orthodontic and prosthodontic procedures causing aesthetic and functional problems. A number of methods are described to increase the anterior maxillary sulcus in these patients. This study assessed the use of a carbon dioxide laser (CO 2 ) to increase the sulcus depth. Fifteen patients with cleft lip and palate (eight unilateral and seven bilateral) were studied. The surgical procedure was performed using CO 2 laser. The vestibular depth and lip length were measured at three time points namely before surgery (T0), 1 week following surgery (T1), and 4 months following surgery (T2). After data collection, statistical analyses were done using PASW ® version 18 SPSS. The mean values of vestibular depth were 9.46 ± 1.92, 13.83 ± 1.88, and 13.23 ± 1.76 mm for T0, T1, and T2, respectively. The vestibular depth significantly increased after 4 months of follow-up (p = 0.001). The mean amount of vestibular depth gain was not significantly different in unilateral and bilateral cleft groups (p = 0.908). The mean value of upper lip length increased by a mean of 1.23 mm and was statistically significant (p = 0.001). Upper buccal sulcus reconstruction with CO 2 laser provides successful and stable results. CO 2 laser application is suggested as an alternative to conventional vestibuloplasty.

Dental anomalies in children with cleft lip and palate in Western Australia

PubMed Central

Nicholls, Wendy

2016-01-01

Objective: The purpose of this paper was to describe the prevalence and type of dental anomalies in the primary and permanent dentition in children with a cleft condition at Princess Margaret Hospital in Perth, Western Australia. Materials and Methods: The details of 162 current dental patients extracted from the main dental database through their year of birth for the period 1998–2001 were selected consecutively. Dental records and X-rays were examined by one examiner (WN) and verified by a second examiner (RB) to determine dental development. The mean age of the subjects was 10.8 years with equal numbers of males and females. Subjects were further divided into cleft type; unilateral cleft lip (UCL) and palate, bilateral cleft lip (BCL) and palate, UCL, BCL, and cleft palate. Results: One hundred sixty-two subjects were grouped into 21 categories of anomaly or abnormality. Prevalence rates for the categories were calculated for the overall group and for gender and cleft type. Conclusion: Overall, 94% of patients were found to have at least one dental anomaly, with fifty-six (34%) patients having more than one anomaly or abnormality. PMID:27095907

Language Parameters of 4- to 7-Year-Old Persian-Speaking Children with Cleft Lip and Palate.

PubMed

Ghayoumi Anaraki, Zahra; Faham, Maryam; Derakhshandeh, Fatemeh; Hashemi Hosseinabad, Hedieh; Haresabadi, Fatemeh

2016-01-01

There are several risk factors including hearing difficulties, lack of language stimulation, and parents' low level of expectation leading to language disorders in children with cleft palate. Therefore, formal language assessments of children with cleft palate are of great importance in order to prevent further disabilities. The purpose of the present study is to evaluate language parameters in 4- to 7-year old Persian-speaking children with cleft palate. 16 children with unilateral and bilateral cleft lip and palate aged between 4 and 7 years participated in the experiment. The Test of Language Development-Primary, third edition (TOLD-P3) was performed to evaluate the language parameters. The results were scored according to the test manual and compared to normative data published with the TOLD-P3. t test analysis showed a significant difference between language parameters in children with cleft lip and palate and the normative data (p < 0.0001). Furthermore, there was a positive correlation between different aspects of language performance. The findings emphasize that speech-language pathologists should also concentrate on early language assessment and treatment for children with cleft lip and palate. © 2016 S. Karger AG, Basel.

ATTITUDE OF CLEFT CARE SPECIALISTS IN AFRICA TOWARDS PRESURGICAL ORTHOPAEDICS.

PubMed

Adeyemi, A T; Bankole, O O

2012-12-01

To determine the attitude of cleft care specialists working in Africa towards pre-surgical orthopaedics. A cross sectional study. Pan-African conference on cleft lip and palate in Nigeria. Sixty cleft care specialists working in Africa. The general attitude towards pre-surgicalorthopaedics was positive. Majority of the specialists employ pre-surgical orthopaedics before surgical repair. Fifty four (91%) of the specialists use plaster strapping for cases of bilateral cleft, five (8.3%) use nasoalveolar molding and one (2%) use feeding plate. Half of the specialists (50%) felt that pre-surgical orthopaedics is to be managed by orthodontists alone. There were significant difference in attitude towards pre-surgical orthopaedics between providers (p < 0.05). Orthodontists had a strong positive attitude towards pre-surgical orthopaedics than other specialists. Younger specialists had a more positive attitude towards pre-surgical orthopaedics than older specialists. Cleft care specialists in Africa have a positive attitude towards pre-surgical orthopaedics. Majority of them use plaster strapping technique. Younger specialists have a more favorable attitude towards pre-surgical orthopaedics than older specialists.

Combining the Cutting and Mulliken methods for primary repair of the bilateral cleft lip nose.

PubMed

Morovic, Carmen Gloria; Cutting, Court

2005-11-01

Since 1990, primary bilateral cleft nasal reconstruction has been focused on placing the lower lateral cartilages into normal anatomical position. Of the four major techniques in this class, the Cutting (i.e., retrograde) method and the Mulliken method have been most successful. The retrograde method makes no external nasal incisions, but requires either preoperative or postoperative nasal molding to achieve maximum benefit. Mulliken's technique does not require molding, but leaves the footplates of the medial crura in the depression above the projecting premaxilla associated with the diminutive anterior nasal spine. Leaving the footplates in place also prevents adequate approximation of the alar bases. In this article, the two methods are combined to achieve the benefits of both. We report our experience with the retrograde nasal approach associated with marginal rim incisions (Mulliken method) in a series of 25 consecutive bilateral cleft lip cases simultaneous with lip repair. We performed a retrograde approach through membranous septum incisions elevating a prolabial-columellar flap. To facilitate alar cartilage manipulation we added bilateral marginal rim incisions. Nasal width, columella length and width, tip projection, and nasolabial angle were analyzed after a minimum of 2 years after surgery. These were compared with a normal, age-matched, control group. We also examined nostril symmetry and marginal nostril scars. Columellar length was not statistically significantly different from that of the control group (p = 0.122442). Nasal width, columellar width, tip projection, and nasolabial angle were all significantly greater in the cleft group than normal (p < 0.001). No hypertrophied scars were found associated with the marginal rim scar. Adding the Mulliken approach allows alar cartilage manipulation to be performed more easily than when using the retrograde approach alone. Tip projection and alar base narrowing are facilitated using the combined technique rather than the Mulliken approach alone. Prolabial flap manipulation is safe using this combined approach, even in cases with a severely projected premaxilla. We believe that the combined approach is safe and yields better long-term results than either technique alone.

Complete second branchial cleft anomaly presenting as a fistula and a tonsillar cyst: an interesting congenital anomaly.

PubMed

Thottam, Prasad John; Bathula, Samba S; Poulik, Janet M; Madgy, David N

2014-01-01

Branchial cleft anomalies make up 30% of all pediatric neck masses, but complete second branchial cleft anomalies are extremely rare. We report an unusual case of a complete second branchial cleft anomaly that presented as a draining neck fistula and a tonsillar cyst in an otherwise healthy 3-month-old girl. At the age of 7 months, the patient had been experiencing feeding difficulties, and there was increasing concern about the risk of persistent infections. At that point, the anomaly was excised in its entirety. Our suspicion that the patient had a complete second branchial cleft anomaly was confirmed by imaging, surgical excision, and histopathologic analysis.

Prevalence of cleft lip and/or palate in children from Lodz between years 1981-2010.

PubMed

Antoszewski, Bogusław; Fijałkowska, Marta

2016-03-01

Congenital malformations constitute a serious problem of both medical and social nature. Cleft lip and/or palate represent the most common congenital anomaly of the face that is why it is essential to know the real frequency of the described phenomenon. The aim of this paper is to determine the frequency of cleft lip and/or palate and the types of malformations that occurred in Lodz city between the years 1981-2010. Our clinic has been carrying on the studies concerning the incidence of cleft lip and/or palate since 1981. The Polish Registry of Congenital Malformations has been operating in Poland since 1 April 1997. The team has managed to obtain data, from the Registry, concerning the total number of all live born infants and the number of children with cleft lip and/or palate, who were born in Lodz, between 1998 and 2010. In years 1981-2010, 319 children, in 210 952 live born infants, were born with cleft lip and/or palate in Lodz. The isolated cleft palate was observed more frequently in girls and the unilateral cleft of lip and palate in boys. In all three decades palate clefts are more common whereas bilateral lip, alveolus and palate clefts are more infrequent. A small tendency to decrease in actual cleft lip and/or palate frequency among children, in the period of 30 years, is observed in Lodz. Over the years it has still been observed that the isolated cleft palate is the most common type of defect. © 2015 Japanese Teratology Society.

Maxillary Anterior Segmental Distraction Osteogenesis to Correct Maxillary Deficiencies in a Patient With Cleft Lip and Palate.

PubMed

Kageyama-Iwata, Asuka; Haraguchi, Seiji; Iida, Seiji; Aikawa, Tomonao; Yamashiro, Takashi

2017-07-01

This report describes a case of successful orthodontic treatment using maxillary anterior segmental distraction osteogenesis with an internal maxillary distractor and bilateral sagittal split ramus osteotomy in a girl with cleft lip and palate. A 16-year-old girl with unilateral cleft lip and palate exhibited midface retrusion because of growth inhibition of the maxillary complex and mandibular excess. After the presurgical orthodontic treatment, 6.0-mm advancement of the maxillary anterior segment and 4.0-mm set back of the mandible were performed. After a retention period, the patient's midface convexity was greatly improved and the velopharyngeal competence was preserved without relapse.

Skeletal and soft tissue changes and stability in cleft lip and palate patients after distraction osteogenesis using a new intraoral maxillary device.

PubMed

Ansari, Edward; Tomat, Catherine; Kadlub, Natacha; Diner, Patrick A; Bellocq, Thomas; Vazquez, Marie-Paule; Picard, Arnaud

2015-04-01

The authors have recently reported on the use of an internal maxillary distraction device. In this study, we report on the hard and soft tissue movements achieved with this intraoral distraction device, and the stability changes after distraction osteogenesis for maxillary hypoplasia in patients with cleft lip and palate. Ten male patients with severe hypoplasia of the maxilla, with complete uni- or bilateral cleft lip and palate were included. The mean age of the patients at the time of operation was 11.91 years (±3.41). To evaluate the distraction process and stability, superimpositions on the preoperative lateral cephalograms were performed. The mean follow-up (FU) was 15.42 months (±3.94). Cephalometric measurements at all of the maxillary hard and soft tissue points improved significantly. Maxillary point A was advanced by 8.25 mm (±3.17; P < 0.001). After distraction soft tissue point A' had advanced 7.10 mm (±2.69; P < 0.001). The soft tissue to hard tissue ratio at point A was 0.86:1 after distraction. Maxillary horizontal relapse at point A was 14.1% at FU. Vertical relapse was not significant. This rigid intraoral distraction device can be successfully used in the correction of severe maxillary hypoplasia. The marked aesthetic improvement and low psychological encumbrance make this device viable for the treatment of cleft-related hypoplasia of the maxilla. Copyright © 2014 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Factors contributing to hearing impairment in patients with cleft lip/palate in Malaysia: A prospective study of 346 ears.

PubMed

Cheong, Jack Pein; Soo, Siew Shuin; Manuel, Anura Michelle

2016-09-01

To determine the factors contributing towards hearing impairment in patients with cleft lip/palate. A prospective analysis was conducted on 173 patients (346 ears) with cleft lip and palate (CL/P) who presented to the combined cleft clinic at University Malaya Medical Centre (UMMC) over 12 months. The patients' hearing status was determined using otoacoustic emission (OAE), pure tone audiometry (PTA) and auditory brainstem response (ABR). These results were analysed against several parameters, which included age, gender, race, types of cleft pathology, impact and timing of repair surgery. The patients' age ranged from 1-26 years old. They comprised 30% with unilateral cleft lip and palate (UCLP), 28% with bilateral cleft lip and palate (BCLP), 28% with isolated cleft palate (ICP) and 14% with isolated cleft lip (ICL). Majority of the patients (68.2%) had normal otoscopic findings. Out of the 346 ears, 241 ears (70%) ears had passed the hearing tests. There was no significant relationship between patients' gender and ethnicity with their hearing status. The types of cleft pathology significantly influenced the outcome of PTA and ABR screening results (p < 0.001). There was no significant difference between the repaired and unrepaired cleft groups and the outcome of hearing tests. However, hearing improvement occurred when palatal repair was performed at the age of <1year old (OR = 2.37, CI 1.2 = 4.6, p = 0.01). Majority of the cleft patients had normal hearing (70%). Hearing threshold varied significantly between the different types of cleft pathology. Surgery conferred no significant impact on the hearing outcome unless surgery was performed at the age of <1 year old. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Anophthalmia with cleft palate and micrognathia: a new syndrome?

PubMed Central

Phadke, S R; Sharma, A K; Agarwal, S S

1994-01-01

A 5 day old male with bilateral anophthalmos, hypospadias, bifid scrotum, micrognathia, and cleft palate with normal chromosomes is described. There have been two case reports with similar clinical manifestations but associated with interstitial deletion of 14q (q22q23). We propose that either our patient represents a new syndrome or has a microdeletion which could not be detected on routine cytogenetics. High quality cytogenetics and FISH may show microdeletions in patients presenting with this clinical picture and normal chromosomes. Images PMID:7891380

Simple solution for difficult face mask ventilation in children with orofacial clefts.

PubMed

Veerabathula, Prardhana; Patil, Manajeet; Upputuri, Omkar; Durga, Padmaja

2014-10-01

Significant air leak from the facial cleft predisposes to difficult mask ventilation. The reported techniques of use of sterile gauze, larger face mask and laryngeal mask airway after intravenous induction have limited application in uncooperative children. We describe the use of dental impression material molded to the facial contour to cover the facial defect and aid ventilation with an appropriate size face mask in a child with a bilateral Tessier 3 anomaly. © 2014 John Wiley & Sons Ltd.

Prevalence of dental anomalies of number in different subphenotypes of isolated cleft palate.

PubMed

Schwartz, João Paulo; Somensi, Daniele Salazar; Yoshizaki, Priscila; Reis, Luciana Laís Savero; de Cássia Moura Carvalho Lauris, Rita; da Silva Filho, Omar Gabriel; Dalbén, Gisele; Garib, Daniela Gamba

2014-01-01

This study aimed at carrying out a radiographic analysis on the prevalence of dental anomalies of number (agenesis and supernumerary teeth) in permanent dentition, in different subphenotypes of isolated cleft palate preadolescent patients. Panoramic radiographs of 300 patients aged between 9 and 12 years, with cleft palate and enrolled in a single treatment center, were retrospectively analyzed. The sample was divided into two groups according to the extension/severity of the cleft palate: complete and incomplete. The chi-square test was used for intergroup comparison regarding the prevalence of the investigated dental anomalies (P<0.05). Agenesis was found in 34.14% of patients with complete cleft palate and in 30.27% of patients with incomplete cleft palate. Supernumerary teeth were found in 2.43% of patients with complete cleft palate and in 0.91% of patients with incomplete cleft palate. No statistically significant difference was found between groups with regard to the prevalence of agenesis and supernumerary teeth. There was no difference in cleft prevalence between genders within each study group. The prevalence of dental anomalies of number in preadolescents with cleft palate was higher than that reported for the general population. The severity of cleft palate did not seem to be associated with the prevalence of dental anomalies of number.

[Inconformity between soft tissue defect and bony defect in incomplete cleft palate].

PubMed

Zhou, Xia; Ma, Lian

2014-12-01

To evaluate the inconformity between soft tissue defect and bony defect by observing the cleft extent of palate with complete secondary palate bony cleft in incomplete cleft palate patient. The patients with incomplete cleft palate treated in Hospital of Stomatology Peking University from July 2012 to June 2013 were reviewed, of which 75 cases with complete secondary palate bony cleft were selected in this study. The CT scan and intraoral photograph were taken before operation. The patients were classified as four types according to the extent of soft tissue defect. Type 1: soft tissue defect reached incisive foremen region, Type 2 was hard and soft cleft palate, Type 3 soft cleft palate and Type 4 submucous cleft palate. Type 1 was defined as conformity group (CG). The other three types were defined as inconformity group (ICG) and divided into three subgroups (ICG-I), (ICG-II) and (ICG-III). Fifty-seven patients were in ICG group, and the rate of inconformity was 76% (57/75). The percentage of ICG-I, ICG-II and ICG-III was 47% (27/57), 23% (13/57) and 30% (17/57), respevtively. There are different types of soft tissue deformity with complete secondary palate bony cleft. The inconformity between soft tissue and hard tissue defect exits in 3/4 of isolated cleft palate patients.

Facial tissue depths in children with cleft lip and palate.

PubMed

Starbuck, John M; Ghoneima, Ahmed; Kula, Katherine

2015-03-01

Cleft lip and palate (CLP) is a craniofacial malformation affecting more than seven million people worldwide that results in defects of the hard palate, teeth, maxilla, nasal spine and floor, and maxillodental asymmetry. CLP facial soft-tissue depth (FSTD) values have never been published. The purpose of this research is to report CLP FSTD values and compare them to previously published FSTD values for normal children. Thirty-eight FSTDs were measured on cone beam computed tomography images of CLP children (n = 86; 7-17 years). MANOVA and ANOVA tests determined whether cleft type, age, sex, and bone graft surgical status affect tissue depths. Both cleft type (unilateral/bilateral) and age influence FSTDs. CLP FSTDs exhibit patterns of variation that differ from normal children, particularly around the oronasal regions of the face. These differences should be taken into account when facial reconstructions of children with CLP are created. © 2014 American Academy of Forensic Sciences.

Fourth-dimensional changes in nasolabial dimensions following rotation-advancement repair of unilateral cleft lip.

PubMed

Mulliken, John B; LaBrie, Richard A

2012-02-01

Repair of unilateral cleft lip requires three-dimensional craftsmanship and understanding four-dimensional changes. Ninety-nine children with unilateral complete or incomplete cleft lip were measured by direct anthropometry following rotation-advancement repair (intraoperatively) and again in childhood. Changes in heminasal width, labial height, and labial width were analyzed and compared measures depending on whether the cleft was incomplete/complete or involved left/right side. Average heminasal width (sn-al) was set 1 mm less on the cleft side and measured only 0.7 mm less at 6 years. Labial height (sn-cphi) was slightly greater on the cleft side at repair and matched the noncleft side at follow-up. Vertical dimension (sbal-cphi) was slightly less at operation; the percent change was the same on both sides. Transverse labial width (cphi-ch) was set short on the cleft side and lengthened disproportionately, resulting in less than 1 mm difference at 6 years. All anthropometric dimensions grew less in complete cleft lips compared with incomplete forms; however, only labial height and width were significantly different. There were no disparities in nasolabial growth between left- and right-sided cleft lips. Cleft side alar base drifts laterally and should be positioned slightly more medial and secured to nasalis or periosteum. Growth in labial height lags and, therefore, the repaired side should be equal to or slightly greater than on the normal side, particularly in a complete labial cleft. Transverse labial width grows more on the cleft side; thus, lateral Cupid's bow peak point can be marked closer to the commissure to match the labial height on the noncleft side. Therapeutic, IV.

Esthetic, Functional, and Everyday Life Assessment of Individuals with Cleft Lip and/or Palate

PubMed Central

Papamanou, Despina A.; Karamolegkou, Marina; Dorotheou, Domna

2015-01-01

Objectives. To evaluate the level of satisfaction of individuals with cleft lip and/or palate (CLP) and their parents concerning the esthetic and functional treatment outcomes, the impact of the cleft on everyday life, and potential associations with treatment outcome satisfaction. Subjects and Methods. The sample consisted of 33 patients (7 CP, 20 unilateral CLP, and 6 bilateral CLP; median age: 17.1, range: 9.0–33.1 years) and 30 parents, who responded to a questionnaire in an interview-guided session. All participants received their orthodontic treatment at the Department of Orthodontics in the University of Athens. Results. Patients and their parents were quite satisfied with esthetics and function. Patients with UCLP primarily were concerned about nose esthetics (BCLP about lip esthetics and CP about speech). Increased satisfaction was associated with decreased influence of the cleft in everyday life (0.35 < rho < 0.64, P < 0.05). Parents reported significant influence of the cleft on family life, while patients did not. Conclusions. Despite the limited sample size of subgroups, the main concerns of patients with different cleft types and the importance of satisfying lip, nose, and speech outcomes for an undisturbed everyday life were quite evident. Thus, the need for targeted treatment strategies is highlighted for individuals with cleft lip and/or palate. PMID:26064918

Esthetic, Functional, and Everyday Life Assessment of Individuals with Cleft Lip and/or Palate.

PubMed

Gkantidis, Nikolaos; Papamanou, Despina A; Karamolegkou, Marina; Dorotheou, Domna

2015-01-01

To evaluate the level of satisfaction of individuals with cleft lip and/or palate (CLP) and their parents concerning the esthetic and functional treatment outcomes, the impact of the cleft on everyday life, and potential associations with treatment outcome satisfaction. The sample consisted of 33 patients (7 CP, 20 unilateral CLP, and 6 bilateral CLP; median age: 17.1, range: 9.0-33.1 years) and 30 parents, who responded to a questionnaire in an interview-guided session. All participants received their orthodontic treatment at the Department of Orthodontics in the University of Athens. Patients and their parents were quite satisfied with esthetics and function. Patients with UCLP primarily were concerned about nose esthetics (BCLP about lip esthetics and CP about speech). Increased satisfaction was associated with decreased influence of the cleft in everyday life (0.35 < rho < 0.64, P < 0.05). Parents reported significant influence of the cleft on family life, while patients did not. Despite the limited sample size of subgroups, the main concerns of patients with different cleft types and the importance of satisfying lip, nose, and speech outcomes for an undisturbed everyday life were quite evident. Thus, the need for targeted treatment strategies is highlighted for individuals with cleft lip and/or palate.

Three-dimensional assessment of facial asymmetry in preschool patients with orofacial clefts after neonatal cheiloplasty.

PubMed

Moslerová, Veronika; Dadáková, Martina; Dupej, Ján; Hoffmannova, Eva; Borský, Jiří; Černý, Miloš; Bejda, Přemysl; Kočandrlová, Karolína; Velemínská, Jana

2018-05-01

To evaluate facial asymmetry changes in pre-school patients with orofacial clefts after neonatal cheiloplasty and to compare facial asymmetry with age-matched healthy controls. The sample consisted of patients with unilateral cleft lip (UCL), unilateral cleft lip and palate (UCLP), and bilateral cleft lip and palate (BCLP). The patients were divided in two age groups with a mean age of 3 years (n = 51) and 4.5 years (n = 45), respectively, and 78 age-matched individuals as controls. Three-dimensional (3D) facial scans were analyzed using geometric morphometry and multivariate statistics. Geometric morphometry showed positive deviations from perfect symmetry on the right side of the forehead in the intervention groups and the controls. The UCL groups showed the greatest asymmetric nasolabial area on the cleft-side labia and the contralateral nasal tip. The UCLP group showed, moreover, asymmetry in buccal region due to typical maxillar hypoplasia, which was accentuated in the older group. The BCLP groups showed slightly similar but greater asymmetry than the control groups, except for the philtrum region. Asymmetry of each of the cleft groups significantly differed from the controls. Except for the buccal region in the UCLP and BCLP groups, asymmetry did not significantly increase with age. Copyright © 2018 Elsevier B.V. All rights reserved.

Social life aspects of young adults with cleft lip and palate: grounded theory approach.

PubMed

Chetpakdeechit, Woranuch; Hallberg, Ulrika; Hagberg, Catharina; Mohlin, Bengt

2009-01-01

The findings of many questionnaire and inventory studies suggest that people with cleft lip and/or palate report a decreased quality of life. Common problems include dissatisfaction with the external appearance of the lips and nose, speech problems, depression, and anxiety. This qualitative study aimed to explore the subjective perceptions and values of young adults with clefts, particularly with regard to their social lives. Twelve persons participated in an in-depth interview. Among those, seven had a repaired isolated cleft palate involving only the hard/soft palate. Five had a repaired bilateral cleft lip and palate that had been a continuous lesion of the lip, the alveolar process, and the palate. A grounded theory approach was used to conduct and analyze the interviews. The study revealed seven important categories--hoping to be like other people, being treated differently from others, experiencing deviation from others, regarding oneself as being different from others, lack of recognition, low self-esteem, and receiving recognition from significant others--with hoping to be like other people as the core category. Young adults with either cleft lip and palate or isolated cleft palate who received recognition from significant others reported increased self-esteem and greater ability to cope with their social lives.

Transverse mucoperiosteal flap inset by rotation for cleft palate repair: technique and outcomes.

PubMed

Black, Jonathan S; Gampper, Thomas J

2014-01-01

Cleft palate is a relatively common deformity with various techniques described for its repair. Most techniques address the hard palate portion of the cleft with bilateral mucoperiosteal flaps transposed to the midline. This results in superimposed, linear closure layers directly over the cleft and may predispose the repair to oronasal fistula formation. This report details an alternative technique of flap rotation with an outcome analysis. A retrospective chart analysis was performed of all patients having undergone primary palatoplasty for cleft palate. Demographics and cleft Veau type were recorded. Postoperative speech outcomes were assessed by standardized speech evaluation performed by 2 speech language pathologists. The presence and location of oronasal fistulae was assessed and recorded by the surgeon and speech language pathologists in follow-up evaluations. The study revealed an overall incidence of velopharyngeal insufficiency of 5.7% using this surgical technique. It also revealed a fistula rate of 8.6%. Secondary surgery has been successful in those patients in which it was indicated. Eleven (31%) patients were diagnosed with Robin sequence. This technique demonstrates excellent early outcomes in a difficult subset of cleft patients including a high proportion of those with Pierre Robin sequence. The technique addresses the inherent disadvantages to a linear closure over the bony cleft. The variability in its design provides the surgeon another option for correction of this deformity.

[Presurgical nasoalveolar molding in infants with cleft lip and palate: analysis of 29 cases].

PubMed

Hou, Yu-xia; Ren, Zhan-ping; Li, Jin-feng; Li, Xiang-lin; Ma, Si-wei; Cao, Hui-qin

2011-12-01

The objective of this study was to treat the cleft lip and alveolus, nasal deformity with presurgical nasoalveolar molding (PNAM), to elucidate the problems and treatment methods, which may be helpful for the use of PNAM in clinic. Twenty nine infants with cleft lip and palate (CLP) were treated with PNAM in our center. There were 19 unilateral and 10 bilateral CLP patients. The initial visit time was 3 to 150 days after birth. Treatment time ranged from 2.5 to 3 months. The appliance was modified at 2-week interval. According to the evaluation standards, 17 infants were treated successfully with the closure of cleft lip and alveolar processes, reposition of the deformed nasal cartilages, and increased length of columella. The lip and nasal deformities of 9 infants were corrected partly, which were helpful for surgery. There were 3 infants giving up PNAM. There were five important facts for the successful treatment, including initial visit time, impression of the intraoral cleft defect, modification of the plate and the nasal stent, and use of nasal splints. Orthodontics and plastic surgeons should have the same views for PNAM in infants, which will advance the treatment level for cleft lip and palate.

Premaxillary characteristics in complete bilateral cleft lip and palate: A predictor for treatment outcome

PubMed Central

El-Kassaby, Marwa Abdelwahab; Abdelrahman, Noha Ibrahim; Abbass, Islam Tarek

2013-01-01

Objectives: The aim of the current study was to investigate how bilateral cleft lip and palate (BCLP) cases responded differently to presurgical orthopedics (PSO) and primary lip repair (LR) based on premaxillary characteristics. We suggest a clinically oriented descriptive classification for BCLP based on premaxillary characteristics. Design and Setting: A retrospective longitudinal comparative study where available records of all non-syndromic patients with complete BCLP attending the Cleft Clinic, affiliated to the Oral and Maxillofacial Surgery department, Ain-Shams University, Cairo, Egypt were assessed. Sample Population and Methodology: Twenty-two cases were collected over a 4-years period from 2008 to 2011 (15 boys and 7 girls). Model assessment was performed for serial models representing four stages of treatment; M1: Prior to start of PSO, M2: At the end of PSO, M3: One month after LR, M4: Three months after LR. The premaxillary and vomerine widths were measured on M1. Models (M1-M4) were assessed for changes in anteroposterior projection, anterior arch width, intercanine width and posterior arch width and results were statistically analyzed. Intra-and postoperative surgical findings during and after primary LR were recorded. The sample was divided into two groups based on the premaxillary size and characteristics; Group R: Rudimentary premaxilla and Group P: Prominent premaxilla. Results: There was a highly significant difference in premaxillary width between the two groups (P = 0.00), changes in anteroposterior projection of the premaxilla were significant one and three months after LR. Changes in maxillary anterior arch width, intercanine and posterior arch widths were non-significant between groups. Mean age difference between the two groups was only statistically significant at the stage of LR. Surgical differences were noted between the two groups. Postoperatively as compared to group R; group P showed more premaxillary bulge and show at rest, as well as more prolabial stretching. In addition, facial profile was more convex in group P. Conclusion: The two types of BCLP outlined in this study are different from several aspects, and hence management should be modified according to each case. This descriptive classification provides a useful tool for evaluation and planning of patients with BCLP. PMID:23662253

A Survey of Congenital Heart Disease and Other Organic Malformations Associated with Different Types of Orofacial Clefts in Eastern China

PubMed Central

Sun, Ting; Tian, Hua; Wang, Changqian; Yin, Ping; Zhu, Yaqin; Chen, Xianghua; Tang, Zhengde

2013-01-01

Background A high incidence of orofacial clefts is reported in China, but no data has shown the relation between cleft types and the incidence of other defects so far. The aim of this study is to assess the incidence of congenital heart diseases and other organic defects associated with different types of orofacial clefts. Methodology and Principal Findings All children with orofacial clefts, which were sought out from the Health Information System of Shanghai Ninth People's Hospital between 1st Jan 2009 and 30th Dec 2011, were enrolled in this study. All subjects underwent a thorough examination and grouped by the cleft phenotype. The numbers and types of other organic defects were recorded and analyzed statistically using SPSS 17.0. Of 2180 cases reported as having orofacial clefts, 657 (30.1%) had other congenital abnormalities, which were significantly more common in cleft palate (47.9% (329/687)) than that in cleft lip (10.6% (80/755)) or cleft lip and palate (33.6% (248/738)) (P<0.01). In subgroups, unilateral cleft lip and palate had a statistically higher incidence of associated abnormalities than bilateral cleft lip and palate (P<0.01). The most common malformation was congenital heart disease, which counted 45.1% (296/657) of all malformations. Disorders of the central nervous system (14.3%(94/657)) and Skeletal anomalies (13.1%(86/657)) were also frequently associated. Additionally, the most common defect in heart was atrial septal defect, which was 39.7% (118/296) of all congenital heart diseases. Conclusions and Significance As the high incidence of heart defects and other organic abnormalities in the children with cleft palate in Eastern China, special attention should be paid to them and echocardiography should be a proposed examination in the evaluation of children with cleft palate before any surgical correction being executed. PMID:23349958

A survey of congenital heart disease and other organic malformations associated with different types of orofacial clefts in Eastern China.

PubMed

Sun, Ting; Tian, Hua; Wang, Changqian; Yin, Ping; Zhu, Yaqin; Chen, Xianghua; Tang, Zhengde

2013-01-01

A high incidence of orofacial clefts is reported in China, but no data has shown the relation between cleft types and the incidence of other defects so far. The aim of this study is to assess the incidence of congenital heart diseases and other organic defects associated with different types of orofacial clefts. All children with orofacial clefts, which were sought out from the Health Information System of Shanghai Ninth People's Hospital between 1(st) Jan 2009 and 30(th) Dec 2011, were enrolled in this study. All subjects underwent a thorough examination and grouped by the cleft phenotype. The numbers and types of other organic defects were recorded and analyzed statistically using SPSS 17.0. Of 2180 cases reported as having orofacial clefts, 657 (30.1%) had other congenital abnormalities, which were significantly more common in cleft palate (47.9% (329/687)) than that in cleft lip (10.6% (80/755)) or cleft lip and palate (33.6% (248/738)) (P<0.01). In subgroups, unilateral cleft lip and palate had a statistically higher incidence of associated abnormalities than bilateral cleft lip and palate (P<0.01). The most common malformation was congenital heart disease, which counted 45.1% (296/657) of all malformations. Disorders of the central nervous system (14.3%(94/657)) and Skeletal anomalies (13.1%(86/657)) were also frequently associated. Additionally, the most common defect in heart was atrial septal defect, which was 39.7% (118/296) of all congenital heart diseases. As the high incidence of heart defects and other organic abnormalities in the children with cleft palate in Eastern China, special attention should be paid to them and echocardiography should be a proposed examination in the evaluation of children with cleft palate before any surgical correction being executed.

Functional Analysis of SPECC1L in Craniofacial Development and Oblique Facial Cleft Pathogenesis

PubMed Central

Gfrerer, Lisa; Shubinets, Valeriy; Hoyos, Tatiana; Kong, Yawei; Nguyen, Christina; Pietschmann, Peter; Morton, Cynthia C.; Maas, Richard L.; Liao, Eric C.

2015-01-01

Background Oblique facial clefts, also known as Tessier clefts, are severe orofacial clefts, the genetic basis of which is poorly understood. Human genetics studies revealed that disruption in SPECC1L resulted in oblique facial clefts, demonstrating that oblique facial cleft malformation has a genetic basis. An important step toward innovation in treatment of oblique facial clefts would be improved understanding of its genetic pathogenesis. The authors exploit the zebrafish model to elucidate the function of SPECC1L by studying its homolog, specc1lb. Methods Gene and protein expression analysis was carried out by reverse-transcriptase polymerase chain reaction and immunohistochemistry staining. Morpholino knockdown, mRNA rescue, lineage tracing and terminal deoxynucleotidyl transferase-mediated dUTP nick end-labeling assays were performed for functional analysis. Results Expression of specc1lb was detected in epithelia juxtaposed to chondrocytes. Knockdown of specc1lb resulted in bilateral clefts between median and lateral elements of the ethmoid plate, structures analogous to the frontonasal process and the paired maxillary processes. Lineage tracing analysis revealed that cranial neural crest cells contributing to the frontonasal prominence failed to integrate with the maxillary prominence populations. Cells contributing to lower jaw structures were able to migrate to their destined pharyngeal segment but failed to converge to form mandibular elements. Conclusions These results demonstrate that specc1lb is required for integration of frontonasal and maxillary elements and convergence of mandibular prominences. The authors confirm the role of SPECC1L in orofacial cleft pathogenesis in the first animal model of Tessier cleft, providing morphogenetic insight into the mechanisms of normal craniofacial development and oblique facial cleft pathogenesis. PMID:25357034

Evaluation of the results of simultaneous open rhinoplasty and Abbe flap for the reconstruction of the secondary bilateral cleft and nasal deformity.

PubMed

Eski, Muhitdin; Aykan, Andac; Alhan, Dogan; Zor, Fatih; Isik, Selcuk

2015-06-01

We aimed to evaluate the results of simultaneous rhinoplasty and Abbe flap for the reconstruction of the secondary bilateral cleft and nasal deformity by means of a newly developed software-based method. A total of 16 patients with the bilateral cleft lip nasal deformity received Abbe flap and simultaneous open rhinoplasty between 2004 and 2010. The mean age of the patients was 21 years, and the average follow-up time was 2.4 years. After the open rhinoplasty procedure, the upper lip was reconstructed with the Abbe flap. Preoperative and postoperative photographs that had been taken laterally were evaluated by using Adobe Photoshop(®) CS4 and Adobe InDesign(®) software. The length of the columella and the relative changes of the most anterior point of the upper lip to the vertical plane tangent to the most anterior point of the lower lip were measured in pixels on standardized preoperative and postoperative images. The differences were calculated and compared as a percentage (%). There was no flap loss and associated problems. The measurements of columellar length revealed an average increase of 51.8 ± 11.3%, while the relative change of the most anterior point of the upper lip revealed an average increase of 68.6 ± 11.2%. The results of the treatment modality were successfully evaluated by a newly developed software-based method. Copyright © 2015 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

Apert Syndrome: Molecularly Confirmed C.758C>G (P.Pro253Arg) in FGFR2

DOE Office of Scientific and Technical Information (OSTI.GOV)

Cha Gon, Lee, E-mail: leechagon@eulji.ac.kr

A 5-day-old girl was referred to our clinic for evaluation of congenital malformations. She was identified with a pathogenic mutation c.758C>G (p.Pro253Arg) in FGFR2 gene using targeted exome sequencing. The de novo mutation was confirmed with Sanger sequencing in the patient and her parents. She showed occipital plagiocephaly with frontal bossing (Figure A and B). Skull frontal and lateral radiography revealed fusion of most of the sutures except coronal suture, with convolutional markings (Figure D and E). She had complete cleft palate (Figure C). Her fused bilateral hands showed type II syndactyly with complete syndactyly between the ring and themore » little fingers (Figure F1-F3). Both toes were simple syndactyly with side-to-side fusion of skin (Figure G1-)« less

Isolated cleft palate requires different surgical protocols depending on cleft type.

PubMed

Elander, Anna; Persson, Christina; Lilja, Jan; Mark, Hans

2017-08-01

A staged protocol for isolated cleft palate (CPO), comprising the early repair of the soft palate at 6 months and delayed repair of the eventual cleft in the hard palate until 4 years, designed to improve maxillary growth, was introduced. CPO is frequently associated with additional congenital conditions. The study evaluates this surgical protocol for clefts in the soft palate (CPS) and for clefts in the hard and soft palate (CPH), with or without additional malformation, regarding primary and secondary surgical interventions needed for cleft closure and for correction of velopharyngeal insufficiency until 10 years of age. Of 94 consecutive children with CPO, divided into four groups with (+) or without (-) additional malformations (CPS + or CPS - and CPH + or CPH-), hard palate repair was required in 53%, performed with small local flaps in 21% and with bilateral mucoperiosteal flaps in 32%. The total incidence of soft palate re-repair was 2% and the fistula repair of the hard palate was 5%. The total incidence of secondary velopharyngeal surgery was 17% until 10 years, varying from 0% for CPS - and 15% for CPH-, to 28% for CPS + and 30% for CPH+. The described staged protocol for repair of CPO is found to be safe in terms of perioperative surgical results, with comparatively low need for secondary interventions. Furthermore, the study indicates that the presence of a cleft in the hard palate and/or additional conditions have a negative impact on the development of the velopharyngeal function.

Genitourinary malformations: an under-recognized feature of ectrodactyly, ectodermal dysplasia and cleft lip/palate syndrome.

PubMed

Hyder, Zerin; Beale, Victoria; O'Connor, Ruth; Clayton-Smith, Jill

2017-04-01

The ectodermal dysplasia and cleft lip/palate (EEC) syndrome describes the association of ectrodactyly, ectodermal dysplasia and orofacial clefting. As with many autosomal dominant disorders, there is variability in expression and not all of these three core features are present in every individual with the condition. Moreover, there may be additional associated features, which are under-recognized. One of these is the presence of genitourinary anomalies, some of which cause significant morbidity. This report details a further two patients with EEC syndrome and genitourinary involvement, including flaccid megacystis with detrusor muscle failure, bilateral hydronephrosis and megaureter, requiring significant renal and urological involvement during their childhood. We go on to review the literature on the diagnosis and management of genitourinary malformations in EEC syndrome.

Bilateral pharyngoceles (branchial cleft anomalies?) and endoscopic surgical considerations.

PubMed

Chang, Christopher Y; Furdyna, Julia A

2005-07-01

A case report of bilateral pharyngoceles without a history of elevated intrapharyngeal pressures is used to support the hypothesis that pharyngoceles may be an adult manifestation of an internal branchial sinus anomaly. The development of a pharyngocele from a branchial sinus origin would suggest a predictable relationship to the hypoglossal, glossopharyngeal, and superior laryngeal nerves, which may influence the choice of surgical approach (open versus endoscopic) and the counseling of patients who are considering surgical correction.

Hospital care of children with a cleft in England

PubMed Central

Fitzsimons, Kate J; Copley, Lynn P; Deacon, Scott A; van der Meulen, Jan H

2013-01-01

Objective To analyse hospital admissions in the first 2 years of life among children with cleft lip and/or palate in England. Design Analysis of national administrative data of hospital admissions. Setting National Health Service hospitals. Patients Patients born alive between 1997 and 2008 who underwent surgical cleft repair. Outcome measures Number of admissions, including the birth episode, and days spent in hospital were examined. Children were analysed according to cleft type and whether or not they had additional congenital anomalies. Results 10 892 children were included. In their first 2 years, children without additional anomalies (n=8482) had on average 3.2 admissions and 13.2 days in hospital, which varied from 2.6 admissions and 9.2 days with cleft lip to 4.7 admissions and 19.7 days with bilateral cleft lip and palate (BCLP). Children with additional anomalies (n=2410) had on average 6.7 admissions and 51.4 days in hospital, which varied from 6.4 admissions and 48.5 days with cleft palate to 8.8 admissions and 67.5 days with BCLP. The mean number and duration of cleft-related admissions was similar in children without (1.6 admissions and 6.4 days) and in those with additional anomalies (1.5 admissions and 8.5 days). 35.2% of children without additional anomalies had at least one emergency admission, whereas the corresponding figure was 67.3% with additional anomalies. Conclusions The burden of hospital care in the first 2 years of life varied according to cleft type and presence of additional anomalies. However, cleft-specific hospital care did not differ between children with and without additional anomalies. PMID:23968774

Ventral C1 Fracture Combined with Congenital Posterior Cleft: What to Do?

PubMed

Gembruch, Oliver; Dammann, Philipp; Schoemberg, Tobias; Ahmadipour, Yahya; Payer, Michael; Sure, Ulrich; Tessitore, Enrico; Özkan, Neriman

2018-01-01

 We present a treatment approach for a rare condition of patients with a ventral C1 fracture and a congenital cleft in the posterior arch (half-ring Jefferson fracture) with an intact transverse atlantal ligament. Our technique aims to achieve stability of the atlanto-occipital and atlantoaxial joints while preserving mobility of the upper cervical spine.  Two male patients, 43 years and 29 years of age, respectively, were admitted to our hospital due to a fracture of the ventral arch of the atlas with no damage of the transverse atlantal ligament. Both men also presented a congenital cleft of the posterior arch. Initial conservative management with a halo-thoracic vest was performed in one case and failed. As a result, surgical treatment was performed in both cases using bilateral C1 mass screws and a transverse connector.  The patients showed no neurologic deficits on follow-up examination 4 weeks after surgery with a full range of head and neck motion. Computed tomography (CT) showed no dislocation of the implanted material with good dorsal alignment and a stable ventral fracture distance. Follow-up CT showed osseous stability in both cases with the beginning of bony ossification of the bone graft.  Isolated instable fractures of the ventral arch of the atlas with a congenital cleft of the posterior arch with no damage of the transverse atlantal ligament can be stabilized using bilateral C1 mass screws and a transverse connector preserving upper cervical spine mobility. Georg Thieme Verlag KG Stuttgart · New York.

Dentofacial morphology in adolescent or early adult patients with cleft lip and palate after a treatment regimen that included vomer flap surgery and pushback palatal repair.

PubMed

Friede, H; Lilja, J

1994-06-01

Dentofacial morphology was evaluated in 94 adolescent or early adult patients born with unilateral or bilateral cleft lip and palate. As well as lip closure, the primary treatment included vomer flap surgery and pushback palatal repair. Roentgencephalometric measurements as well as classification of the patients into different classes of dentofacial deformity indicated development of bimaxillary retrognathia with severe midfacial deficiency in about a quarter of the cases. Our results were similar to those reported by other teams who used similar surgical regimen.

Orthognathic surgery in the young cleft patient: preliminary study on subsequent facial growth.

PubMed

Wolford, Larry M; Cassano, Daniel Serra; Cottrell, David A; El Deeb, Mohamed; Karras, Spiro C; Goncalves, Joao Roberto

2008-12-01

This study evaluated the long-term effects of orthognathic surgery on subsequent growth of the maxillomandibular complex in the young cleft patient. We evaluated 12 young cleft patients (9 male and 3 female patients), with a mean age of 12 years 6 months (range, 9 years 8 months to 15 years 4 months), who underwent Le Fort I osteotomies, with maxillary advancement, expansion, and/or downgrafting, by use of autogenous bone or hydroxyapatite grafts, when indicated, for maxillary stabilization. Five patients had concomitant osteotomies of the mandibular ramus. All patients had presurgical and postsurgical orthodontic treatment to control the occlusion. Radiographs taken at initial evaluation (T1) and presurgery (T2) were compared to establish the facial growth vector before surgery, whereas radiographs taken immediately postsurgery (T3) and at longest follow-up (T4) were used to determine postsurgical growth. Each patient's lateral cephalograms were traced, and 16 landmarks were identified and used to compute 11 measurements describing presurgical and postsurgical growth. Before surgery, all patients had relatively normal growth. After surgery, cephalograms showed statistically significant growth changes from T3 to T4, with the maxillary depth decreasing by -3.3 degrees +/- 1.8 degrees , Sella-nasion-point A by -3.3 degrees +/- 1.8 degrees, and point A-nasion-point B by -3.6 degrees +/- 2.8 degrees. The angulation of the maxillary incisors increased by 9.2 degrees +/- 11.7 degrees. Of 12 patients, 11 showed disproportionate postsurgical jaw growth. Maxillary growth occurred predominantly in a vertical vector with no anteroposterior growth, even though most patients had shown anteroposterior growth before surgery. The distance increased in the linear measurement from nasion to gnathion by 10.3 +/- 7.9 mm. Four of 5 patients operated on during the mixed dentition phase had teeth that erupted through the cleft area. A variable impairment of postoperative growth was seen with the 2 types of grafting material used. No significant difference was noted in the effect on growth in patients with unilateral clefts versus those with bilateral clefts. The presence of a pharyngeal flap was noted to adversely affect growth, whereas simultaneous mandibular surgery did not. After surgery, 11 of 12 patients tended toward a Class III end-on occlusal relation. Orthognathic surgery may be performed on growing cleft patients when mandated by psychological and/or functional concerns. The surgeon must be cognizant of the adverse postsurgical growth outcomes when performing orthognathic surgery on growing cleft patients with the possibility for further surgery requirements. Performing maxillary osteotomies on cleft patients would be more predictable after completion of facial growth.

Early Cleft Lip Repair Revisited: A Safe and Effective Approach Utilizing a Multidisciplinary Protocol

PubMed Central

Imahiyerobo, Thomas A.; Liang, Fan; Fahradyan, Artur; Urbinelli, Leo; Lau, Jennifer; Matar, Marla; Magee, William; Urata, Mark

2017-01-01

Background: The optimal timing for cleft lip repair has yet to be established. Advances in neonatal anesthesia, along with a growing body of literature, suggesting benefits of earlier cleft lip and nasal repair, have set the stage for a reexamination of current practices. Methods: In this prospective study, cleft lip and nasal repair occurred on average at 34.8 days (13–69 days). Nasal correction was achieved primarily through molding the nasal cartilage without the placement of nasal sutures at the time of repair. A standardized anesthetic protocol aimed at limiting neurotoxicity was utilized in all cases. Anesthetic and postoperative complications were assessed. A 3-dimensional nasal analysis compared pre- and postoperative nasal symmetry for unilateral clefts. Surveys assessed familial response to repair. Results: Thirty-two patients were included (27 unilateral and 5 bilateral clefts). In this study, the overall complication rate was 3.1%. Anthropometric measurements taken from 3-dimensional-image models showed statistically significant improvement in ratios of nostril height (preoperative mean, 0.59; postoperative mean, 0.80), nasal base width (preoperative mean, 1.96; postoperative mean, 1.12), columella length (preoperative mean, 0.62; postoperative mean, 0.89; and columella angle (preoperative mean, 30.73; postoperative mean, 9.1). Survey data indicated that families uniformly preferred earlier repair. Conclusions: We present evidence that early cleft lip and nasal repair can be performed safely and is effective at improving nasal symmetry without the placement of any nasal sutures. Utilization of this protocol has the potential to be a paradigm shift in the treatment of cleft lip and nasal deformity. PMID:28740766

Maxillary dental anomalies in patients with cleft lip and palate: a cone beam computed tomography study.

PubMed

Celikoglu, M; Buyuk, S K; Sekerci, A E; Cantekin, K; Candirli, C

2015-01-01

To compare the frequency of maxillary dental anomalies in patients affected by unilateral (UCLP) and bilateral (BCLP) cleft lip with palate and to determine whether statistical differences were present or not between cleft and normal sides in UCLP group by using cone beam computed tomography (CBCT). In addition, the frequency of those dental anomalies was compared with previous studies presenting the same population without cleft Study Design: Fifty non-syndromic patients affected by UCLP (28 patients) and BCLP (22 patients) were selected for analysis of dental anomalies by means of CBCT. The frequency of maxillary dental anomalies including tooth agenesis, microdontia of lateral incisor, ectopic eruption and impaction of canine and supernumerary tooth were examined. Pearson chi-square and Fisher's exact tests were performed for statistical comparisons. All patients affected by UCLP and BCLP were found to have at least one maxillary dental anomaly. The most frequently observed dental anomaly was tooth agenesis (92.5% and 86.4%, respectively) in UCLP and BCLP groups. Tooth agenesis and canine impaction were observed more commonly in the cleft side (75.0% and 35.7%, respectively) than in the normal side (57.1% and 14.3%, respectively) in UCLP group (p≯0.05). All dental anomalies were found to be higher in both cleft groups than in general populations not affected by cleft. Since patients affected by UCLP and BCLP had at least one dental anomaly and higher dental anomaly frequency as compared to patients without cleft, those patients should be examined carefully prior to orthodontic treatment.

Cleft lip and palate treatment of 530 children over a decade in a single centre.

PubMed

Vlastos, I M; Koudoumnakis, E; Houlakis, M; Nasika, M; Griva, M; Stylogianni, E

2009-07-01

We sought to evaluate the process of care and the outcomes of cleft lip and palate operations carried by a multidisciplinary team at a centre of craniofacial anomalies with a high patients' volume. A retrospective review of all cleft lips and/or palates cases treated in the centre from 1995 to 2007 was performed. Direct and long term complication rates, clinical, audiologic, speech intelligibility and dental arch assessments were analyzed. A total of 530 children have been operated this period in the centre (64 isolated cleft lip closures). A detailed presentation of the outcomes is performed in relation to the various types of cleft lip and palates. The majority of parents (70%) reported very good or excellent results 2-5 years after the lip closure with the Millard technique, although those with bilateral clefts were significantly less satisfied (P<0.002). Forty-two percent of children with cleft palate and otitis media with effusion were self-improved 2-8 months after palate reconstruction and 83.3% of children treated with the two flaps palatoplasty technique had a rather high or very high intelligibility score. Muscles' retropositioning had a significant effect on intelligibility (P=0.04). Children with cleft lips and palates have a variety of conditions and functional limitations even after the surgical correction of their problem that need to be evaluated and treated by several specialists. The treatment protocol utilized by the multidisciplinary team of our centre is efficient with a relative low percentage of complications and unfavorable results.

Early Cleft Lip Repair Revisited: A Safe and Effective Approach Utilizing a Multidisciplinary Protocol.

PubMed

Hammoudeh, Jeff A; Imahiyerobo, Thomas A; Liang, Fan; Fahradyan, Artur; Urbinelli, Leo; Lau, Jennifer; Matar, Marla; Magee, William; Urata, Mark

2017-06-01

The optimal timing for cleft lip repair has yet to be established. Advances in neonatal anesthesia, along with a growing body of literature, suggesting benefits of earlier cleft lip and nasal repair, have set the stage for a reexamination of current practices. In this prospective study, cleft lip and nasal repair occurred on average at 34.8 days (13-69 days). Nasal correction was achieved primarily through molding the nasal cartilage without the placement of nasal sutures at the time of repair. A standardized anesthetic protocol aimed at limiting neurotoxicity was utilized in all cases. Anesthetic and postoperative complications were assessed. A 3-dimensional nasal analysis compared pre- and postoperative nasal symmetry for unilateral clefts. Surveys assessed familial response to repair. Thirty-two patients were included (27 unilateral and 5 bilateral clefts). In this study, the overall complication rate was 3.1%. Anthropometric measurements taken from 3-dimensional-image models showed statistically significant improvement in ratios of nostril height (preoperative mean, 0.59; postoperative mean, 0.80), nasal base width (preoperative mean, 1.96; postoperative mean, 1.12), columella length (preoperative mean, 0.62; postoperative mean, 0.89; and columella angle (preoperative mean, 30.73; postoperative mean, 9.1). Survey data indicated that families uniformly preferred earlier repair. We present evidence that early cleft lip and nasal repair can be performed safely and is effective at improving nasal symmetry without the placement of any nasal sutures. Utilization of this protocol has the potential to be a paradigm shift in the treatment of cleft lip and nasal deformity.

Bilateral respiratory epithelial adenomatoid hamartoma of the olfactory cleft penetrating into the endocranium.

PubMed

Mladina, Ranko; Skitarelić, Neven; Poje, Gorazd; Vuković, Katarina

2011-09-01

Respiratory epithelial adenomatoid hamartomas (REAHs) of the nose and paranasal sinuses are relatively rare. These tumors usually do not extend over the boundaries of the nose and sinuses. The authors presented a 65-year-old man experiencing progressive hyposmia, followed by intermittent stubborn headache. The symptoms lasted for almost 2 years and were getting worse very slowly. Fiberendoscopy showed relatively discrete polypoid tissue occupying the olfactory cleft bilaterally. The computed tomography and magnetic resonance imaging suggested the possible lack of the cribriform plate and the unity and uniformity of the tissues located both in the endocranium and high in the nasal cavity. The clinical picture resembled very much a esthesineuroblastoma.The patient underwent endoscopic sinus surgery under the general hypotensive anesthesia. Frozen sections during the surgery showed REAH. The entire tumor was removed in a piece meal way, including both olfactory bulbs because they were involved within the pathologic tissue as well.This case showed that REAH could also be a locally aggressive process, penetrating even into the endocranium.

Nasoalveolar molding in cleft care: is it efficacious?

PubMed

Abbott, Megan M; Meara, John G

2012-09-01

In the era of evidence-based medicine, new treatment protocols and interventions should be routinely evaluated for their efficacy by reviewing the available evidence. In the cleft literature, nasoalveolar molding has garnered attention over the last decade as a new option for improving nasal form and symmetry before primary surgical repair. Systematic review of the evidence is, however, currently lacking. This review evaluates whether nasoalveolar molding can improve nasal symmetry and form toward the norm, as well as whether nasoalveolar molding demonstrates advantages over other protocols in achieving this goal. A literature search of five databases plus relevant reference lists retrieved 98 articles regarding nasoalveolar molding, 21 of which reported objective outcome measures of nasal symmetry and form, and six of which were able to be given evidence level ratings, all in the unilateral cleft population. Statistical analysis was not possible given the range of techniques and outcomes. Studies of bilateral cleft were not given evidence level ratings, given the inability to separate the effects of nasoalveolar molding from other primary nasal interventions in studies that would have otherwise been rated. In unilateral cleft lip-cleft palate, there was some evidence that nasoalveolar molding may improve nasal outcomes, though comparison with other techniques was limited. Despite a relative paucity of high-level evidence, nasoalveolar molding appears to be a promising technique that deserves further study.

Complete Midline Cleft of Lower Lip, Mandible, Tongue, Floor of Mouth with Neck Contracture: A Case Report and Review of Literature

PubMed Central

Rao, Anantheswar Y. N.

2015-01-01

Midline cleft of the lower lip and mandible is an extremely rare condition. Since 1819, when the first case was reported by Couronne, fewer than 80 cases have been described in the world literature so far. The cleft has also been described as facial cleft no. 30 by Paul Tessier. The condition varies in severity from a mild variety in which there is a submucous cleft and notching in the lower lip to a severe variety, involving the tongue, floor of the mouth, mandible, absent hyoid, atrophic neck muscles, and sternum. In this case report, a female child having complete midline cleft of the lower lip and mandible, with bifid tongue stuck to the floor of the mouth, absent hyoid bone and flexion contracture band extending from the confluence of the tip of the tongue, floor of the mouth, cleft mandible to the manubrium sterni is described, with special emphasis on surgical planning and management. PMID:26576245

[The effect of pre-surgical orthodontics on secondary alveolar bone grafting in the patients with complete cleft lip and palate].

PubMed

Jia, Yi-lin; Fu, Min-kui; Ma, Lian

2004-05-01

To examine the effect of pre-surgical orthodontics on the outcome of the secondary alveolar bone grafting in the patients with complete cleft lip and palate. Sixteen complete cleft lip and palate patients (9 males and 7 females) with collapsed upper arch or severe mal-positioned upper incisors were selected. The cleft was not easily grafted because of the poor access. The total cleft sites were 22 (10 patients with UCLP and 6 patients with BCLP). The age range of the patients was from 8 to 22 years. Pre-surgical orthodontic treatment was mainly to expand the collapsed upper arch and correct the mal-positioned upper incisors. After the secondary alveolar bone grafting, the patients were followed up and anterior occlusal radiograph/intraoral panograph were taken regularly. The observation period was from 6 months to 4 years. Bergland criteria were used to evaluate the interdental septal height. Upper arch expansion and the correction of the mal-positioned upper incisors done by the orthodontic treatment made the bone grafting procedure easier. The clinically successful rate reached 86%. The severe upper arch collapse and mal-positioned upper incisors in the patients with complete cleft lip and palate should be corrected orthodontically before the secondary alveolar bone grafting.

A novel computer system for the evaluation of nasolabial morphology, symmetry and aesthetics after cleft lip and palate treatment. Part 2: Comparative anthropometric analysis of patients with repaired unilateral complete cleft lip and palate and healthy individuals.

PubMed

Pietruski, Piotr; Majak, Marcin; Pawlowska, Elzbieta; Skiba, Adam; Antoszewski, Boguslaw

2017-04-01

The aim of this study was to use a novel system, 'Analyse It Doc' (A.I.D.) for a complex anthropometric analysis of the nasolabial region in patients with repaired unilateral complete cleft lip and palate and in healthy individuals. A set of standardized facial photographs in frontal, lateral and submental view have been taken in 50 non-cleft controls (mean age 20.6 years) and 42 patients with repaired unilateral complete cleft and palate (mean age 19.57 years). Then, based on linear, angular and area measurements taken from the digital photographs with the aid of the A.I.D. system, a photogrammetric analysis of intergroup differences in nasolabial morphology and symmetry was conducted. Patients with cleft lip and palate differed from the controls in terms of more than half of analysed angular measurements and proportion indices derived from linear and area measurements of the nasolabial region. The findings presented herein imply that despite primary surgical repair, patients with unilateral complete cleft lip and palate still show some degree of nasolabial dysmorphology. Furthermore, the study demonstrated that the novel computer system is suitable for a reliable, simple and time-efficient anthropometric analysis in a clinical setting. Copyright © 2017 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Identifying Genetic Sources of Phenotypic Heterogeneity in Orofacial Clefts by Targeted Sequencing.

PubMed

Carlson, Jenna C; Taub, Margaret A; Feingold, Eleanor; Beaty, Terri H; Murray, Jeffrey C; Marazita, Mary L; Leslie, Elizabeth J

2017-07-17

Orofacial clefts (OFCs), including nonsyndromic cleft lip with or without cleft palate (NSCL/P), are common birth defects. NSCL/P is highly heterogeneous with multiple phenotypic presentations. Two common subtypes of NSCL/P are cleft lip (CL) and cleft lip with cleft palate (CLP) which have different population prevalence. Similarly, NSCL/P can be divided into bilateral and unilateral clefts, with unilateral being the most common. Individuals with unilateral NSCL/P are more likely to be affected on the left side of the upper lip, but right side affection also occurs. Moreover, NSCL/P is twice as common in males as in females. The goal of this study is to discover genetic variants that have different effects in case subgroups. We conducted both common variant and rare variant analyses in 1034 individuals of Asian ancestry with NSCL/P, examining four sources of heterogeneity within CL/P: cleft type, sex, laterality, and side. We identified several regions associated with subtype differentiation: cleft type differences in 8q24 (p = 1.00 × 10 -4 ), laterality differences in IRF6, a gene previously implicated with wound healing (p = 2.166 × 10 -4 ), sex differences and side of unilateral CL differences in FGFR2 (p = 3.00 × 10 -4 ; p = 6.00 × 10 -4 ), and sex differences in VAX1 (p < 1.00 × 10 -4 ) among others. Many of the regions associated with phenotypic modification were either adjacent to or overlapping functional elements based on ENCODE chromatin marks and published craniofacial enhancers. We have identified multiple common and rare variants as potential phenotypic modifiers of NSCL/P, and suggest plausible elements responsible for phenotypic heterogeneity, further elucidating the complex genetic architecture of OFCs. Birth Defects Research 109:1030-1038, 2017. © 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

Invasive cervical resorption and the oro-facial cleft patient: a review and case series.

PubMed

O'Mahony, A; McNamara, C; Ireland, A; Sandy, J; Puryer, J

2017-05-12

Introduction Invasive cervical resorption (ICR) has an unknown aetiology, yet it exhibits very aggressive behaviour compared with typical external root resorption, posing a high risk of tooth loss.Aim To investigate the number of patients at the Dublin Cleft Prosthodontic Department with an oro-facial cleft who experienced ICR and to identify any possible aetiological factors.Materials and method A retrospective investigation of all oro-facial cleft patients treated at the Dublin Cleft Prosthodontic Department, St James's Hospital, Dublin. All patients' clinical and radiological records were reviewed. Patients where tooth loss became inevitable due to Class 4 ICR were analysed.Results From 588 oro-facial cleft patients, 14 (2.38%) patients with ICR were identified. Of these eight (57%) were female and six (43%) were male. Mean age at diagnosis was 28 years (range = 16-49 years). Cleft type: six (42.1%) unilateral cleft lip and palate, eight (57.9%) bilateral cleft lip and palate. Seventeen ICR affected teeth in total, with eleven (65%) maxillary central incisors, two (12%) maxillary lateral incisors, four (23%) maxillary canines, and one (7%) central, lateral and canine affected. Some, (N = 10, 71.4%) presented with ICR resulting in immediate tooth loss. Other patients (N = 4, 28.6%) developed ICR during or following prosthodontic treatment at the Cleft Centre. Tooth loss for this cohort, though not immediate, was inevitable. All had undergone fixed orthodontic appliance treatment and twelve had received dento-alveolar bone grafts. A number (N = 7, 50%) had undergone osteotomy, two (14%) had received night guard vital dental whitening and one had a history of trauma.Conclusions ICR, given its aggressive nature and ill-understood aetiology, poses significant treatment challenges. The most severe form of ICR (Class 4) leads inevitably to tooth loss. The slow-moderate progression of ICR may explain the late presentation found in this study, reinforcing the importance of long-term follow-up of this special dental care group.

Tessier 3 cleft with bilateral anophthalmia: case report and surgical treatment.

PubMed

Sesenna, Enrico; Anghinoni, Marilena L; Modugno, Alessandra C; Magri, Alice S

2012-12-01

Tessier clefts type 3 and 4 are rare. In this paper the authors report on the management of a wide Tessier 3 cleft. There is no standardized protocol or timing of the surgical procedures in this rare disfiguring condition. Generally speaking, the aim is to preserve the function of important anatomical structures (e.g., a seeing eye.) and reconstruct, as best as possible, harmonic facial features. The authors present a "step by step" solution of the malformation pointing out the limitations of the surgical procedures they used and the goals they wanted to obtain. Despite of the uniqueness and the complexity of the pathology, the authors think they obtained reasonable results both in term of function and aesthetics, permitting the patient to be accepted in the social environment. Copyright © 2012 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Repair of complete bilateral cleft lip with severely protruding premaxilla performing a premaxillary setback and vomerine ostectomy in one stage surgery

PubMed Central

Sanchez-Sanchez, Marta; Iglesias-Martin, Fernando; Garcia-Perla-Garcia, Alberto; Belmonte-Caro, Rodolfo; Gonzalez-Perez, Luis-Miguel

2015-01-01

Background The authors present a technique for selected cases of CBCL. The primary repair of the CBCL with a severely protruding premaxilla in one stage surgery is very difficult, essentially because a good muscular apposition is difficult, forcing synchronously to do a premaxillary setback to facilitate subsequent bilateral lip repair and, thus, achieving satisfactory results. We achieve this by a reductive ostectomy on the vomero-premaxillary suture. Material and Methods 4 patients with CBCL and severely protruding premaxilla underwent premaxillary setback by vomerine ostectomy at the same time of lip repair in the past 24 months. The extent of premaxillary setback varied between 9 and 16 mm. The required amount of bone was removed anterior to the vomero-premaxillary suture. The authors did an additional simultaneous gingivoperiosteoplasty in all patients, achieving an enough stability of the premaxilla in its new position, to be able to close the alveolar gap bilaterally. The authors have examined the position of premaxilla and dental arch between 6 and 24 months. We did not do the primary nose correction, because this increased the risk of impairment of the already compromised vascularity of the philtrum and premaxilla. Results The follow-up period ranged between 6 and 24 months. None of the patients had any major complication. During follow-up, the premaxilla was minimally mobile. We achieved a good lip repair in all cases: adequate muscle repair, symmetry of the lip, prolabium and Cupid’s bow, as well as good scars. Conclusions To our knowledge, there are few reports of one stage surgery with vomerine ostectomy to repair CBCL with severely protruding premaxilla. Doing this vomerine ostectomy, we don’t know how it will affect the subsequent growth of the premaxila and restrict the natural maxillary growth. Applying this alternative treatment for children with CBCL and protruded premaxilla without any preoperative orthopedic, we can successfully perform, in a single-stage surgery, a good primary lip repair at our center. Further confirmations of this surgery with follow up and anthropometric studies of these patients during childhood and adolescence are required. Key words: Protruding premaxilla, bilateral cleft lip, vomerine ostectomy, one stage surgery, Millard II technique. PMID:26034932

Asymmetric Anterior Distraction for Transversely Distorted Maxilla and Midfacial Anteroposterior Deficiency in a Patient With Cleft Lip/Palate: Two-Stage Surgical Approach.

PubMed

Hirata, Kae; Tanikawa, Chihiro; Aikawa, Tomonao; Ishihama, Kohji; Kogo, Mikihiko; Iida, Seiji; Yamashiro, Takashi

2016-07-01

The present report describes a male patient with a unilateral cleft lip and palate who presented with midfacial anteroposterior and transverse deficiency. Correction involved a two-stage surgical-orthodontic approach: asymmetric anterior distraction of the segmented maxilla followed by two-jaw surgery (LeFort I and bilateral sagittal splitting ramus osteotomies). The present case demonstrates that the asymmetric elongation of the maxilla with anterior distraction is an effective way to correct a transversely distorted alveolar form and midfacial anteroposterior deficiency. Furthermore, successful tooth movement was demonstrated in the new bone created by distraction.

Proper size of endotracheal tube for cleft lip and palate patients and intubation outcomes.

PubMed

Abdollahifakhim, Shahin; Sheikhzadeh, Dariush; Shahidi, Nikzad; Nojavan, Gholamreza; Bayazian, Gholamreza; Aleshi, Hamideh

2013-05-01

The aim of the current study was to identify the proper size of endotracheal tube for intubation of cleft lip and palate patients and intubation outcomes in these patients. In this analytic cross-sectional study, 60 nonsyndromic cleft lip and palate patients were selected who had surgery between April 2010 and April 2012 at Pediatrics Hospital, Tabriz University of Medical Sciences, Iran. Demographic findings, previous admissions, and surgical history were registered. The proper tube size was measured by normal children formulas. Then tube size was confirmed by patients' minimum resistance to intubation, proper ventilation reported by anesthesiologist, and appropriate air leakage at an airway pressure of 15-20 cm H₂O. If intubation was unsuccessful then smaller size of endotracheal tube would be tried. Frequency of intubation trials and the biggest endotracheal tube size were recorded. Their average age, weight and height were 21.39 ± 4.95 months, 9.97 ± 1.18 kg and 74.30 ± 26.61 cm, respectively. The average tracheal tube size and frequency of intubation trials were 4.34 ± 0.78 and 1.63 ± 0.80, respectively. Seven cases required an endotracheal tube size smaller than the recommended size for that age including one case in unilateral cleft palate, three cases in unilateral cleft lip, one case in unilateral cleft lip and palate, and two cases in bilateral cleft lip and palate. Findings proved that considering subglottic stenosis incidence in these children, it is reasonable to determine the tube size for nonsyndromic cleft lip and palate patients by applying the currently available standards for normal children. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

Bilateral buphthalmia in a 4-month-old Texas longhorn steer.

PubMed

Jones, M L; Beck, A P; Dubielzig, R R

2013-01-01

Congenital ocular disease occurs uncommonly in cattle, with multiple abnormalities reported only sporadically in the literature. This report describes a case of anterior segment dysgenesis resulting in glaucoma in a 4-month-old Texas Longhorn steer. On clinical exam, bilateral buphthalmia was present and intraocular pressures exceeded 47 mm Hg in both eyes. On histopathologic examination, the iridocorneal angle and filtration apparatus were distorted due to collapse of the ciliary cleft and anterior displacement of the anterior portion of the ciliary body. No evidence of inflammation or other causes of glaucoma were recognized.

Changing strategy and implementation of a new treatment protocol for cleft palate surgery in "Maria Sklodowska Curie" (MSC) Children's Hospital, Bucharest, Romania.

PubMed

Spataru, Radu; Mark, Hans

2014-12-01

In "Maria Sklodowska Curie" (MSC) Children's Hospital, Bucharest, Romania, cleft palate repair has been performed according to von Langenbeck since 1984. The speech was good in most patients but wide clefts had a high percentage of fistulas, abnormal speech due to short length and limited mobility of the soft palate. In 2009, the protocol was changed to Gothenburg Delayed Hard Palate Closure, (DHPC) technique. The present evaluation was performed to study the implementation of this technique. One hundred and sixty-eight patients with cleft palate were admitted, 89 isolated cleft palate (ICP), 53 unilateral (UCLP) and 26 bilateral (BCLP). In these, 228 surgical interventions were performed. Soft Palate Repair (SPR) and Hard Palate Repair (HPR) were performed with the DHPC procedure. The transfer to this technique was successfully performed in three steps: one team visit to Gothenburg by a surgeon from MSC and two visits by surgeons from Gothenburg to the MSC. Patients with SPR and HPR were operated on without major complications and there were no differences in results between Gothenburg surgeons and MSC surgeons. The interventions with SPR and HPR technique were proven to be easy to teach and learn and successfully performed without major complications. For cleft patients at MSC hospital it has meant earlier surgery, less re-operations and complications. This report shows a successful change of strategy for palatal repair with improved outcome regarding surgery. In future, speech and growth will be followed on a regular basis and will be compared with results from the Gothenburg Cleft Team.

Incisor and molar overjet, arch contraction, and molar relationship in the mixed dentition in repaired complete unilateral cleft lip and palate: A qualitative and quantitative appraisal.

PubMed

Disthaporn, Suteeta; Suri, Sunjay; Ross, Bruce; Tompson, Bryan; Baena, Diogenes; Fisher, David; Lou, Wendy

2017-07-01

To compare the mixed dentition incisor and molar overjet, severity of contraction of the dental arch, and the sagittal molar relationship on the cleft side vs the noncleft side in children with repaired complete unilateral cleft of the lip and palate (UCLP). Orthodontic records taken prior to orthodontic preparation for alveolar bone grafting were screened to select study casts from patients with nonsyndromic repaired complete UCLP who did not have mandibular skeletal or dental asymmetry. The study sample comprised dental casts from 74 children aged 8.9 ± 1 years. Standardized digital photographs were acquired at 1:1 magnification. A coordinate system was developed using digital image-processing software (Photoshop CS4 and Adobe Illustrator). Incisor and molar overjet, Angle's classification, and arch contraction were recorded. Descriptive statistics, paired t-tests, and kappa statistics were used to compare the cleft and noncleft sides. A negative overjet of -1 to -5 mm was often present at the incisors, with greater frequency and magnitude on the cleft side. Class II molar relation was more frequent on the cleft side (61.1%) than on the noncleft side (47.2%). Significantly greater contraction of the cleft side deciduous canine and deciduous first molar was noted, while the difference was very minor at the first permanent molar. Cleft side maxillary arch contraction was most severe in the deciduous canine and first deciduous molar region and progressively less severe in the posterior region of the arch. A greater frequency and severity of negative overjet and Class II molar relationship was seen on the cleft side.

The Development of the Cleft Aesthetic Rating Scale: A New Rating Scale for the Assessment of Nasolabial Appearance in Complete Unilateral Cleft Lip and Palate Patients.

PubMed

Mosmuller, David G M; Mennes, Lisette M; Prahl, Charlotte; Kramer, Gem J C; Disse, Melissa A; van Couwelaar, Gijs M; Niessen, Frank B; Griot, J P W Don

2017-09-01

  The development of the Cleft Aesthetic Rating Scale, a simple and reliable photographic reference scale for the assessment of nasolabial appearance in complete unilateral cleft lip and palate patients.   A blind retrospective analysis of photographs of cleft lip and palate patients was performed with this new rating scale.   VU Medical Center Amsterdam and the Academic Center for Dentistry of Amsterdam.   Complete unilateral cleft lip and palate patients at the age of 6 years.   Photographs that showed the highest interobserver agreement in earlier assessments were selected for the photographic reference scale. Rules were attached to the rating scale to provide a guideline for the assessment and improve interobserver reliability. Cropped photographs revealing only the nasolabial area were assessed by six observers using this new Cleft Aesthetic Rating Scale in two different sessions.   Photographs of 62 children (6 years of age, 44 boys and 18 girls) were assessed. The interobserver reliability for the nose and lip together was 0.62, obtained with the intraclass correlation coefficient. To measure the internal consistency, a Cronbach alpha of .91 was calculated. The estimated reliability for three observers was .84, obtained with the Spearman Brown formula.   A new, easy to use, and reliable scoring system with a photographic reference scale is presented in this study.

Evaluation of secondary alveolar bone grafting outcomes performed after canine eruption in complete unilateral cleft lip and palate.

PubMed

Lorenzoni, Diego Coelho; Janson, Guilherme; Bastos, Juliana Cunha; Carvalho, Roberta Martinelli; Bastos, José Carlos; de Cássia Moura Carvalho Lauris, Rita; Henriques, José Fernando Castanha; Ozawa, Terumi Okada

2017-01-01

Evaluate the results of secondary alveolar bone grafting (SABG) in patients with complete unilateral cleft lip and palate (UCLP), operated after permanent canine eruption (CE). Seventy-four periapical radiographs from patients with complete UCLP (mean age 14 years) submitted to SABG were retrospectively analyzed for the amount of bone in the cleft site through the Bergland and Chelsea scales. Of the cases, 47.3 % was classified as Bergland type I and Chelsea type A, 35.2 % as type II/C, 6.7 % as type III/D, and 10.8 % as type IV/failure. When the canine was moved into the grafted area, the success rate (type I/A) was 56.8 %; for cases in which the space was maintained for an implant or prosthetic finishing, the index was 45.8 %; however, this difference was not statistically significant. Even in advanced ages, after permanent canine eruption, SABG can be considered a highly successful procedure. This research shows good results of secondary alveolar bone grafting performed in patients with unilateral complete cleft lip and palate, even when it was performed after eruption of the permanent canine in the cleft area.

Characterization of subtle brain abnormalities in a mouse model of Hedgehog pathway antagonist-induced cleft lip and palate.

PubMed

Lipinski, Robert J; Holloway, Hunter T; O'Leary-Moore, Shonagh K; Ament, Jacob J; Pecevich, Stephen J; Cofer, Gary P; Budin, Francois; Everson, Joshua L; Johnson, G Allan; Sulik, Kathleen K

2014-01-01

Subtle behavioral and cognitive deficits have been documented in patient cohorts with orofacial clefts (OFCs). Recent neuroimaging studies argue that these traits are associated with structural brain abnormalities but have been limited to adolescent and adult populations where brain plasticity during infancy and childhood may be a confounding factor. Here, we employed high resolution magnetic resonance microscopy to examine primary brain morphology in a mouse model of OFCs. Transient in utero exposure to the Hedgehog (Hh) signaling pathway antagonist cyclopamine resulted in a spectrum of facial dysmorphology, including unilateral and bilateral cleft lip and palate, cleft of the secondary palate only, and a non-cleft phenotype marked by midfacial hypoplasia. Relative to controls, cyclopamine-exposed fetuses exhibited volumetric differences in several brain regions, including hypoplasia of the pituitary gland and olfactory bulbs, hyperplasia of the forebrain septal region, and expansion of the third ventricle. However, in affected fetuses the corpus callosum was intact and normal division of the forebrain was observed. This argues that temporally-specific Hh signaling perturbation can result in typical appearing OFCs in the absence of holoprosencephaly--a condition classically associated with Hh pathway inhibition and frequently co-occurring with OFCs. Supporting the premise that some forms of OFCs co-occur with subtle brain malformations, these results provide a possible ontological basis for traits identified in clinical populations. They also argue in favor of future investigations into genetic and/or environmental modulation of the Hh pathway in the etiopathogenesis of orofacial clefting.

Correction of cleft lip nose deformity with rib cartilage.

PubMed

Hafezi, Farhad; Naghibzadeh, Bijan; Ashtiani, Abbas Kazemi; Mousavi, S Jaber; Nouhi, Amir Hossein; Naghibzadeh, Ghazal

2013-07-01

Correction of cleft lip nasal deformities (CLND) is often unsatisfactory because of problems resulting from cartilage weakness and strong soft tissue forces. Therefore, strong cartilaginous support, such as rib cartilage, is mandatory. The authors describe placement of rib cartilage grafts to create a more symmetric and aesthetically acceptable repair of CLND with improved nasal air flow. Two groups of patients, including those with unilateral and bilateral CLND, underwent operations with different sources of autologous cartilage. Group 1 received grafts from the septum and ear, whereas group 2 received grafts from the septum and ribs. Results were evaluated by 2 independent physicians who rated improvement between pre- and postoperative photographs. There were significant differences in postoperative improvement between patients who received septal/ear cartilage grafts and those who received septal/rib cartilage grafts in both unilateral and bilateral cases (P = .028 and P = .043, respectively). The authors' results demonstrate that rib cartilage has a positive effect on the aesthetic outcome of CLND operations and provides a strong support structure for correcting this deformity with minimal postoperative complications.

Photometric Evaluation in Adolescence of Patients With Bilateral Cleft Lip and Palate Treated With Nasoalveolar Molding and Primary Columella Lengthening.

PubMed

Meazzini, Maria Costanza; Chiavenna, Carlo; Autelitano, Luca; Garattini, Giovanna; Brusati, Roberto

2018-04-01

Nasal stigma in patients with bilateral cleft lip and palate (BCLP) are a short columella and a flattened nasal tip. The aim of this study was to evaluate the aesthetics of adolescents with BCLP, operated with a modified Cutting primary columella lengthening technique, associated to a modified Grayson orthopedic nasoalveolar molding (NAM). 72 BCLP patients were operated with this approach. Standardized photographic records were taken every 2 years. A group of 23 patients between 12 and 13 years of age was compared through normalized photogrammetry to a matched control of 23 noncleft adolescents. Nasal protrusion and length of the columella were very close to normal. On the other hand, nasolabial angle and interalar width were still excessively wide compared to the noncleft sample. NAM and primary columella lengthening in BCLP has allowed to avoid traditional secondary columella lengthening at 5 to 6 years of age and given the patients a more pleasing, near-normal nasolabial appearance until adolescence. Some of the patients will require correction of the nasal width at a later stage.

First branchial cleft anomaly, a case for misdiagnosis.

PubMed

Lanisnik, Bostjan; Didanovic, Vojko; Cizmarevic, Bogdan

2004-01-01

First branchial cleft anomaly is a rare condition that is often misdiagnosed and falsely mistreated before complete and definitive surgical treatment. Its origin is uncertain and the presence of ectodermal and sometimes also mesodermal elements has led some authors to the conclusion that it represents buried nests of cells forming the first branchial cleft and the underlying mesoderm. First branchial cleft anomaly can be presented as a cystic lesion, fistula or sinus extending towards the membranous external ear canal. The sinus tract runs through the parotid gland in close association with the facial nerve. There is no imaging method capable of identifying a first branchial cleft anomaly with certainty. The danger of facial nerve injury during surgery and the failure to identify the sinus tract running to the external ear canal are the main reasons for incomplete excision. The facial nerve must be identified and preserved and the lesion completely excised. Facial nerve injury is more common in attempts to remove recurrent branchial cleft lesions.

Branchial cleft and pouch anomalies in childhood: a report of 50 surgical cases.

PubMed

Spinelli, C; Rossi, L; Strambi, S; Piscioneri, J; Natale, G; Bertocchini, A; Messineo, A

2016-05-01

Branchial abnormalities occur when there is disturbance in the maturation of the branchial apparatus during fetal development. Branchial anomalies are congenital lesions usually present in childhood, even if they can be diagnosed later for enlargement or infection. A correct diagnosis will lead to proper management: complete surgical excision is the treatment of choice. The purpose of this article is to present clinical features, diagnostic methods and surgical treatment of branchial anomalies in childhood, based on a series of 50 patients. We conducted a retrospective analysis of a total of 50 pediatric patients operated from June 2005 to June 2014 for the presence of branchial cleft anomalies. 27 cases (54 %) presented a second branchial cleft fistula and 11 cases (22 %) a second branchial cleft cyst and one case (2 %) presented both cyst and sinus of the second branchial cleft; four cases (8 %) presented first branchial cleft cyst whereas four cases (8 %) a first branchial cleft sinus and two cases (4 %) a first branchial cleft fistula; one case (2 %) presented a piriform sinus fistula (third branchial cleft). None of our patients presented anomalies of the fourth branchial cleft. All patients underwent surgical treatment and lesions have been removed by excision or fistulectomy. No post-surgical complication occurred. The rate of recurrence was 4 %. Pre-operative diagnosis supplies important information to the surgeon for a proper therapy: a complete excision of the lesion without inflammatory signs is essential to avoid re-intervention and to achieve a good outcome.

Use of autologous platelet-rich plasma in complete cleft palate repair.

PubMed

El-Anwar, Mohammad Waheed; Nofal, Ahmed Abdel Fattah; Khalifa, Mohamed; Quriba, Amal Saeed

2016-07-01

Evaluate the effect of topical application of autologous platelet-rich plasma (PRP) in primary repair of complete cleft palate and then compare the result with another group of patients using the same surgical technique, without application of PRP with regard to the incidence of oronasal fistula, velopharyngeal closure, and grade of nasality. Case control study. This study was carried on 44 children with complete cleft palate with age range from 12 to 23 months. The children were divided into two age- and gender-matched groups: All children were subjected to the same technique of V-Y pushback repair of the complete cleft palate. In group A (22 children), the PRP prepared from the patient was topically applied between the nasal and oral mucosa layer during palatoplasty, whereas in group B (22 children) the PRP was not applied. All cases were recovered smoothly without problems. In group A, no oronasal fistula was reported, whereas in group B three patients (13.6%) had postoperative fistulae and two patients (9.1%) needed revision palatoplasty. At 6 months postoperative assessment, group A (with PRP application) showed significantly better grade of nasality (P = 0.024) and better endoscopic velopharyngeal closure (P = 0.016) than group B. Usage of autologous PRP in complete cleft palate repair is simple; effective; can decrease the incidence of oronasal fistula; and also significantly improves the grade of nasality and velopharyngeal closure, which decreases the need of further surgical intervention in cleft palate patients. 3b. Laryngoscope, 126:1524-1528, 2016. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

[Bilateral labio-maxillo-palatal clefts. Therapeutic evaluation].

PubMed

Raphaël, B; Morand, B; Bettega, G; Lesne, V; Lesne, C; Lebeau, J

2001-06-01

The wide diversity of bilateral facial clefts makes it most difficult to assess surgical success, particularly in terms of long-term outcome. The aim of this work was to examine the rationale for the current protocol used for cleft surgery at the Grenoble University Hospital. In a first group of 28 children, a 3-step surgical protocol was applied. The first two steps were performed between 4 and 8 months with at least 3 months between each procedure. Skoog's unilateral cheilo-rhino-uranoplasty was used, associated with a periosteal tibial graft. The third step, performed between 10 and 12 months, was for staphylorraphy. Outcome was analyzed at 15 years and evidenced the deleterious effect of excessive and asymmetrical premaxillary scars, of the 2-step cheiloplasty and of columella lengthenings from the lip. The frequency of secondary revision of the superior labial vestibule and the medial labial tubercule (43%) was considered to be high; this procedure should be re-examined as should be osteotomy (32% revision). Palatine closure, acquired in 82% of the cases and premaxillary stability, achieved in 86%, would appear to favor use of the periosteal tibial graft. The osteogenic capacity of this graft tissue was less satisfactory after a second harvesting (from the same tibia three months later). These results have led us to modify our protocol, favoring early and total closure of the bony palate and continued use of the periosteal tibial graft. We now use the following operative protocol: premaxillary alignment using an active orthopedic plate at 2 months, lip adhesion associated with staphylorraphy and passive palatine contention plate at 3 months, definitive bilateral cheilo-uranoplasty associated with a single periosteal graft at 7 months. The preliminary results with this protocol in a group of 12 children have shown better quality scars, more harmonious maxillary arches, an excellent occlusion of the deciduous dentition, and preservation of the positive results obtained with the periosteal tibial graft.

Intraflagellar transport 88 (IFT88) is crucial for craniofacial development in mice and is a candidate gene for human cleft lip and palate.

PubMed

Tian, Hua; Feng, Jifan; Li, Jingyuan; Ho, Thach-Vu; Yuan, Yuan; Liu, Yang; Brindopke, Frederick; Figueiredo, Jane C; Magee, William; Sanchez-Lara, Pedro A; Chai, Yang

2017-03-01

Ciliopathies are pleiotropic human diseases resulting from defects of the primary cilium, and these patients often have cleft lip and palate. IFT88 is required for the assembly and function of the primary cilia, which mediate the activity of key developmental signaling pathways. Through whole exome sequencing of a family of three affected siblings with isolated cleft lip and palate, we discovered that they share a novel missense mutation in IFT88 (c.915G > C, p.E305D), suggesting this gene should be considered a candidate for isolated orofacial clefting. In order to evaluate the function of IFT88 in regulating craniofacial development, we generated Wnt1-Cre;Ift88fl/fl mice to eliminate Ift88 specifically in cranial neural crest (CNC) cells. Wnt1-Cre;Ift88fl/flpups died at birth due to severe craniofacial defects including bilateral cleft lip and palate and tongue agenesis, following the loss of the primary cilia in the CNC-derived palatal mesenchyme. Loss of Ift88 also resulted in a decrease in neural crest cell proliferation during early stages of palatogenesis as well as a downregulation of the Shh signaling pathway in the palatal mesenchyme. Importantly, Osr2KI-Cre;Ift88fl/flmice, in which Ift88 is lost specifically in the palatal mesenchyme, exhibit isolated cleft palate. Taken together, our results demonstrate that IFT88 has a highly conserved function within the primary cilia of the CNC-derived mesenchyme in the lip and palate region in mice and is a strong candidate as an orofacial clefting gene in humans. © The Author 2017. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Novel GREM1 Variations in Sub-Saharan African Patients With Cleft Lip and/or Cleft Palate.

PubMed

Gowans, Lord Jephthah Joojo; Oseni, Ganiyu; Mossey, Peter A; Adeyemo, Wasiu Lanre; Eshete, Mekonen A; Busch, Tamara D; Donkor, Peter; Obiri-Yeboah, Solomon; Plange-Rhule, Gyikua; Oti, Alexander A; Owais, Arwa; Olaitan, Peter B; Aregbesola, Babatunde S; Oginni, Fadekemi O; Bello, Seidu A; Audu, Rosemary; Onwuamah, Chika; Agbenorku, Pius; Ogunlewe, Mobolanle O; Abdur-Rahman, Lukman O; Marazita, Mary L; Adeyemo, A A; Murray, Jeffrey C; Butali, Azeez

2018-05-01

Cleft lip and/or cleft palate (CL/P) are congenital anomalies of the face and have multifactorial etiology, with both environmental and genetic risk factors playing crucial roles. Though at least 40 loci have attained genomewide significant association with nonsyndromic CL/P, these loci largely reside in noncoding regions of the human genome, and subsequent resequencing studies of neighboring candidate genes have revealed only a limited number of etiologic coding variants. The present study was conducted to identify etiologic coding variants in GREM1, a locus that has been shown to be largely associated with cleft of both lip and soft palate. We resequenced DNA from 397 sub-Saharan Africans with CL/P and 192 controls using Sanger sequencing. Following analyses of the sequence data, we observed 2 novel coding variants in GREM1. These variants were not found in the 192 African controls and have never been previously reported in any public genetic variant database that includes more than 5000 combined African and African American controls or from the CL/P literature. The novel variants include p.Pro164Ser in an individual with soft palate cleft only and p.Gly61Asp in an individual with bilateral cleft lip and palate. The proband with the p.Gly61Asp GREM1 variant is a van der Woude (VWS) case who also has an etiologic variant in IRF6 gene. Our study demonstrated that there is low number of etiologic coding variants in GREM1, confirming earlier suggestions that variants in regulatory elements may largely account for the association between this locus and CL/P.

Analysis of primary gingivoperiosteoplasty in alveolar cleft repair. Part I: Facial growth.

PubMed

Henkel, K O; Gundlach, K K

1997-10-01

The primary gingivoperiosteoplasty by Millard consists of presurgical active orthognathic treatment ('Latham device') of the alveolar margins at the age of 3 months and of surgical closure of the alveolar cleft with local gingivoperiosteal flaps at the age of 5 months. The aim of this investigation was to analyse the facial growth following this treatment. The following material was studied: lateral head X-rays and plaster casts from 146 patients with unilateral (UCLP) and bilateral (BCLP) clefts of lip and palate from birth to 16 years of age. Ninety-one of these patients formed the control group, who received neither gingivoperiosteoplasty nor pre-surgical active orthognathic treatment. The same surgeon and orthodontist treated all 146 patients. A three-dimensional growth disturbance after gingivoperiosteoplasty was observed: 42% patients with UCLP and 40% patients with BCLP had an 'open bite' following closure of the alveolar cleft (control group 5%/10%). The length of the upper jaw in patients who underwent gingivoperiosteoplasty was shorter than in the control group. The frequency of posterior cross bite was also higher in the gingivoperiosteoplasty group. These results demonstrate that treatment with a 'Latham device' disturbs facial growth. Therefore, this treatment should be abandoned.

Bilateral oblique facial clefts and extremity anomaly in an infant after intrauterine efavirenz exposure and review of its teratogenic risk.

PubMed

Shanske, Alan L

2012-09-10

Congenital anomalies may be caused by genetic or environmental factors or a combination of both. Oblique facial clefts are very rare congenital deformities. The occurrence of facial clefts and an extremity anomaly suggests a common underlying cause. Lateral oro-ocular clefts do not occur along normal developmental planes and may be part of the amnion disruption complex sequence. Our objective was to report a case of this very event, which also followed an unusual intrauterine exposure and review the literature on the teratogenic risk of efavirenz. We report a case of amniotic rupture sequence after fetal HIV and antiretroviral exposure. Teratogenic exposure has been rarely reported and never after antiretroviral exposure. By reporting and registering more cases, we will be able to better assess the risks such medications pose to the developing fetus. The publication of a single case report has the potential to contribute to our knowledge of the significance of prenatal exposure to antiretrovirals and other medications for common HIV-associated disorders. It also generates a hypothesis that can be tested with further clinical data, animal models and epidemiologic studies.

Van der Woude syndrome: Management in the mixed dentition.

PubMed

Agarwal, Sonahita; Dinesh, M R; Dharma, R M; Amarnath, B C

2013-01-01

This article presents the case of a patient with Van der Woude syndrome treated with orthodontic and orthopedic intervention in the mixed dentition stage. The patient had a bilateral cleft of the lip and alveolus accompanied by lip pits on the lower lip. Intra-orally, there was bilateral anterior and posterior cross-bite with a collapsed maxilla. The maxillary transverse deficiency was managed with orthopedic expansion and the second phase of treatment involved secondary alveolar bone grafting followed by retention with functional regulator-3. The mild maxillary retrognathia and deficient lip support was managed with dental compensation.

Van der Woude syndrome: Management in the mixed dentition

PubMed Central

Agarwal, Sonahita; Dinesh, M. R.; Dharma, R. M.; Amarnath, B. C.

2013-01-01

This article presents the case of a patient with Van der Woude syndrome treated with orthodontic and orthopedic intervention in the mixed dentition stage. The patient had a bilateral cleft of the lip and alveolus accompanied by lip pits on the lower lip. Intra-orally, there was bilateral anterior and posterior cross-bite with a collapsed maxilla. The maxillary transverse deficiency was managed with orthopedic expansion and the second phase of treatment involved secondary alveolar bone grafting followed by retention with functional regulator-3. The mild maxillary retrognathia and deficient lip support was managed with dental compensation. PMID:23853466

Correction of complete maxillary crossbite with severe crowding using Hyrax expansion and fixed appliance.

PubMed

Wangsrimongkol, Tasanee; Manosudprasit, Montian; Pisek, Poonsak; Leelasinjaroen, Pornnapha

2013-09-01

An 18-year-old Thai man who presented with a secondary cleft palate, maxillary hypoplasia and severe crowding was treated by rapid maxillary expansion and fixed orthodontic appliances. Initial assessment found skeletal Class III malrelationship and dental Class II malocclusion with anterior and bilateral posterior crossbites. Camouflage orthodontic treatment was planned using a rapid maxillary expansion appliance and correcting crowding with extraction all four premolar teeth. A Hyrax appliance and vertical loop arch wire were placed for maxillary arch expansion of 9.5 mms at first molars and canines, and 5.5 mms at the premolars and obtained positive overjet. Both acceptable skeletal and soft tissue relationships and satisfactory occlusion were produced. After 14 months of postoperative follow-up, the occlusal result was stable and no skeletal reversals could be detected.

Presurgical Unilateral Cleft Lip Anthropometrics and the Presence of Dental Anomalies.

PubMed

Antonarakis, Gregory S; Fisher, David M

2015-07-01

To investigate associations between cleft lip anthropometrics and dental anomalies in the permanent dentition in unilateral cleft lip patients. Retrospective cross-sectional study. Children with unilateral clefts of the lip, with or without cleft palate. Anthropometric lip measurements, made immediately prior to lip repair, were available for each patient. The presence of dental anomalies in the permanent dentition was assessed radiographically. The presence of associations between anthropometric lip measurements and prevalence rates of different dental anomalies were determined using logistic regression analyses. In the 122 included patients, the cleft lateral lip element was deficient in height in 80% and in transverse length in 84% of patients. Patients with more deficient cleft side lateral lip height and less deficient cleft side lateral lip transverse length were more likely to present with cleft side maxillary lateral incisor agenesis. On the other hand, patients with a less deficient cleft side lateral lip height and more deficient cleft side lateral lip transverse length were more likely to present with a cleft side supernumerary maxillary lateral incisor. When looking only at incomplete clefts, the cleft side lateral lip transverse length deficiency was more predictive of the presence of supernumerary maxillary lateral incisors (P = .030), while for complete clefts, the cleft side lateral lip height deficiency was more predictive of the presence of maxillary lateral incisor agenesis (P = .035). In patients with unilateral clefts, cleft lip anthropometrics have a predictive role in determining the occurrence of dental anomalies.

Nasoalveolar molding improves long-term nasal symmetry in complete unilateral cleft lip-cleft palate patients.

PubMed

Barillas, Ingrid; Dec, Wojciech; Warren, Stephen M; Cutting, Court B; Grayson, Barry H

2009-03-01

Nasoalveolar molding was developed to improve dentoalveolar, septal, and lower lateral cartilage position before cleft lip repair. Previous studies have documented the long-term maintenance of columella length and nasal dome form and projection. The purpose of the present study was to determine the effect of presurgical nasoalveolar molding on long-term unilateral complete cleft nasal symmetry. A retrospective review of 25 consecutively presenting nonsyndromic complete unilateral cleft lip-cleft palate patients was conducted. Fifteen patients were treated with presurgical nasoalveolar molding for 3 months before surgical correction, and 10 patients were treated by surgical correction alone. The average age at the time of follow-up was 9 years. Four nasal anthropometric distances and two angular relationships were measured to assess nasal symmetry. All six measurements demonstrated a greater degree of nasal symmetry in nasoalveolar molding patients compared with the patients treated with surgery alone. Five symmetry measurements were significantly more symmetric in the nasoalveolar molding patients and one measurement demonstrated a nonsignificant but greater degree of symmetry compared with the patients treated with surgery alone. The data demonstrate that the lower lateral and septal cartilages are more symmetric in the nasoalveolar molding patients compared with the surgery-alone patients. Furthermore, the improved symmetry observed in nasoalveolar molding-treated noses during the time of the primary surgery is maintained at 9 years of age.

Evaluation of Stress Distribution of Mini Dental Implant-Supported Overdentures in Complete Cleft Palate Models: A Three-Dimensional Finite Element Analysis Study.

PubMed

Soğancı, Gökçe; Yazıcıoğlu, Hüseyin

2016-01-01

Mini dental implants could be an alternative treatment method for prosthetic treatment of edentulous cleft palate. The aim of this study was to analyze stress distribution around the cortical bone and different plans using a varied number of mini dental implants in edentulous unilateral complete cleft palates. Three edentulous maxillary models were modified to create unilateral complete cleft palates. Mini dental implants (2.4 × 15 mm) were located as two mini implants at the premolar region, four mini implants at the premolar and molar region, and six mini implants at the first premolar, second premolar, and first molar regions in the models, respectively. Mucosa, o-ring/ball attachments, and overdentures were simulated. Vertical and horizontal loads of 100 N were applied on both the right and left molar teeth of the overdenture for each model. Maximum and minimum principal stress values and the distribution at cortical bone around the implants and cleft palates were evaluated by finite element analysis. Stress values under vertical loads were lower than values under horizontal loadings for all models. Stress values were found to be lower in the first model than in the second and third models. The highest stress values were found around implants in the second model. The unilateral feature of a complete cleft pattern affected the stress distribution. Stresses occured mostly around implants when the overdenture was supported by six implants; however, the stress distribution around implants was low with two implants because of tissue support.

[Diagnosis and surgical operation for fourth branchial cleft anomalies].

PubMed

Zhu, Ting; Hua, Qingquan

2011-11-01

To explore diagnosis and surgical operation through analyzing clinical features of the fourth branchial cleft anomalies. Clinical materials of 10 patients with the fourth branchial cleft anomalies were retrospectively analyzed, and literatures were studied to explore the diagnosis, differential diagnosis and treatment methods of surgical operation; lesions of 10 patients were completely removed by surgical operation, and internal sinus was properly handled. All 10 cases were cured, no recurrence were observed during a follow-up of 1-3 years. 1 patient appeared low voice, and drinking cough, back to normality after 2 weeks; 1 patient appeared paralysis of left hypoglossal nerves, back to normality after 3 months. Recurrent deep neck abscess and chronic sinus infections of anterior area in the lower part of neck should be considered with the diagnosis of the fourth branchial cleft anomalies. Enhanced neck CT scan and barium sulfate meal examination aid to diagnosis, pathological examination can be confirmed. Complete surgical removal of lesions is an effective treatment of fourth branchial cleft anomalies, knowing of the courses of internal sinus and spread of infection, and use of principle of selective neck dissection is the key to ensure complete removal of lesions.

Bony defect of palate and vomer in submucous cleft palate patients.

PubMed

Ren, S; Ma, L; Zhou, X; Sun, Z

2015-01-01

The aim of this study was to visualize bony defects of the palate and vomer in submucous cleft palate patients (SMCP) by three-dimensional (3D) computed tomography (CT) reconstruction and to classify the range of bony defects. Forty-eight consecutive non-operated SMCP patients were included. Diagnosis was based on the presence of at least one of three classical signs of SMCP: bifid uvula, a translucent zone in the midline of the soft palate, and a palpable 'V' notch on the posterior border of the bony palate. Patients were imaged using spiral CT. 3D reconstruction models were created of the palate and vomer. The sagittal extent of the bony cleft in SMCP was classified into four types: type I, no V-shaped hard palate cleft (8.3%); type II, cleft involving the partial palate (43.8%); type III, cleft involving the complete palate and extending to the incisive foramen (43.8%); type IV, cleft involving the complete palate and the alveolar bone (4.2%). The extent of the vomer defect was classified into three types: type A, vomer completely fused with the palate (8.3%); type B, vomer partially fused with the palate (43.8%); type C, vomer not fused with the palate up to the incisive foramen (47.9%). Significant variability in hard palate defects in SMCP is the rule rather than the exception. The association of velopharyngeal insufficiency with anatomical malformations may be complex. Copyright © 2014 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Presurgical cleft lip anthropometrics and dental arch relationships in patients with complete unilateral cleft lip and palate.

PubMed

Antonarakis, Gregory S; Adibfar, Alex; Tompson, Bryan D; Paedo, D; Daskalogiannakis, John; Fisher, David M

2015-05-01

To investigate associations between anthropometric lip measurements and dental arch relationships in patients with complete unilateral cleft lip and palate (CUCLP). Retrospective cross-sectional study. Children with CUCLP. Anthropometric lip measurements, made immediately prior to lip repair, were available for each patient. The dental arch relationships were evaluated on dental study casts (8.6 ± 0.9 years) taken prior to any orthodontic treatment and prior to alveolar bone graft, using the modified Huddart and Bodenham (MHB) scoring system. The presence of associations between anthropometric lip measurements and dental arch relationships was determined using linear regression analysis. In the 63 patients included in the study, the cleft lateral lip element was deficient in height in 87% and in transverse width in 86% of patients. Patients with more deficient cleft-side lateral lip height were more likely to present with more negative MHB scores (r = .443; P < .001). Conversely, patients with more deficient cleft-side lateral lip transverse width more often presented with more positive MHB scores (r = .281; P = .025). In patients with CUCLP, there is a wide variability in the degree of deficiency of the cleft-side lateral lip element, both in the vertical and in the transverse dimension. The extent of this deficiency may, in part, predict the resulting dental arch relationships.

Kids' Medical Dictionary

MedlinePlus

... Chronic Word! Cleft Lip Word! Cleft Palate Word! Cochlea Word! Complete Blood Count (CBC) Word! Cone Word! ... Word! Palpitations Word! Pancreas Word! Papillae Word! Peak Flow Meter Word! Pediatric Endocrinologist Word! Pediatrician Word! Peritonitis ...

The Soft Palate Friendly Speech Bulb for Velopharyngeal Insufficiency.

PubMed

Kahlon, Sukhdeep Singh; Kahlon, Monaliza; Gupta, Shilpa; Dhingra, Parvinder Singh

2016-09-01

Velopharyngeal insufficiency is an anatomic defect of the soft palate making palatopharyngeal sphincter incomplete. It is an important concern to address in patients with bilateral cleft lip and palate. Speech aid prosthesis or speech bulbs are best choice in cases where surgically repaired soft palate is too short to contact pharyngeal walls during function but these prosthesis have been associated with inadequate marginal closure, ulcerations and patient discomfort. Here is a case report of untreated bilateral cleft lip and palate associated with palatal insufficiency treated by means of palate friendly innovative speech bulb. This modified speech bulb is a combination of hard acrylic and soft lining material. The hard self-curing acrylic resin covers only the hard palate area and a permanent soft silicone lining material covering the soft palate area. A claw-shaped wire component was extended backwards from acrylic and was embedded in soft silicone to aid in retention and approximation of two materials. The advantage of adding the soft lining material in posterior area helped in covering the adequate superior extension and margins for maximal pharyngeal activity. This also improved the hypernasality, speech, comfort and overall patient acceptance.

[Morphological classification and velopharyngeal function analysis of submucous cleft palate patients].

PubMed

Heng, Yin; Chunli, Guo; Bing, Shi; Yang, Li; Jingtao, Li

2016-10-01

To enhance the accuracy in diagnosis and management of submucous cleft palate via a thorough analysis of its anatomical and functional details. Two hundred seventy-six submucous cleft palate cases from 2008 to 2014 were retrospectively investigated. Subgroup analysis were performed on the basis of preoperative velopharyngeal function, palatal morphology, cleft lip concurrence, and patient motives for treatment. Among the included cases, 96 (34.78%) were presented as velopharyngeal competence (VPC), 151 (54.71%) as velopharyngeal insufficiency (VPI), and 29 (10.51%) as marginal VPI (MVPI). Eighty cases (28.99%) also demonstrated cleft lip deformity, and 196 cases (71.01%) were merely submucous cleft palate. Compared with patients with submucous cleft palate only, those with cleft lips exhibited higher rates of complete velopharyngeal closure. The pathological spectrum of submucous cleft palate varied significantly. Only 103 (37.32%) cases met all the three diagnostic criteria proposed by Calnan. Given that the velopharyngeal closure rate varies among the subgroups, the factors analyzed in this study should be considered in the personalized manage-ment of submucous cleft palate.

Correlations between Cervical Vertebral Maturation (CVM) and Dental Development in Thai Cleft Patients.

PubMed

Chongcharueyskul, Pathomporn; Wangsrimonkol, Tasanee; Pisek, Poonsak; Pisek, Araya; Manosudprasit, Montian

2015-08-01

To examine correlations between cervical vertebral maturation stages (CVMs) and dental development stages, and cervical vertebral maturation (CVM) stage 6 and completion of root formation of mandibular third molar in Thai cleft patients. Lateral cephalograms of 366 cleft subjects aged 7-9 years were assessed for CVMs using Baccetti method. Calcication stages of all left mandibular teeth within each CVMs were assessed from panoramic films using Demirjian method. Spearman rank correlation coefficients comparing CVMs and teeth were 0.51-0.79 (p<0.001). Second molar had the highest and central incisor had the lowest correlations. In CVMs 6, 2.9% of third molars had completed root formation. However, only CVMs 6 could be predicted from third molar stage G that had a high likelihood ratio (30.94). Dental development was highly correlated with CVM in clefts. Third molar stage G could predict completed growth of mandible in individual patients, but it should be combined with other maturation indicators.

Tbx1 regulates oral epithelial adhesion and palatal development

PubMed Central

Funato, Noriko; Nakamura, Masataka; Richardson, James A.; Srivastava, Deepak; Yanagisawa, Hiromi

2012-01-01

Cleft palate, the most frequent congenital craniofacial birth defect, is a multifactorial condition induced by the interaction of genetic and environmental factors. In addition to complete cleft palate, a large number of human cases involve soft palate cleft and submucosal cleft palate. However, the etiology of these forms of cleft palate has not been well understood. T-box transcriptional factor (Tbx) family of transcriptional factors has distinct roles in a wide range of embryonic differentiation or response pathways. Here, we show that genetic disruption of Tbx1, a major candidate gene for the human congenital disorder 22q11.2 deletion syndrome (Velo-cardio-facial/DiGeorge syndrome), led to abnormal epithelial adhesion between the palate and mandible in mouse, resulting in various forms of cleft palate similar to human conditions. We found that hyperproliferative epithelium failed to undergo complete differentiation in Tbx1-null mice (Tbx1−/−). Inactivation of Tbx1 specifically in the keratinocyte lineage (Tbx1KCKO) resulted in an incomplete cleft palate confined to the anterior region of the palate. Interestingly, Tbx1 overexpression resulted in decreased cell growth and promoted cell-cycle arrest in MCF7 epithelial cells. These findings suggest that Tbx1 regulates the balance between proliferation and differentiation of keratinocytes and is essential for palatal fusion and oral mucosal differentiation. The impaired adhesion separation of the oral epithelium together with compromised palatal mesenchymal growth is an underlying cause for various forms of cleft palate phenotypes in Tbx1−/− mice. Our present study reveals new pathogenesis of incomplete and submucous cleft palate during mammalian palatogenesis. PMID:22371266

Cleft palate caused by congenital teratoma.

PubMed

Veyssière, Alexis; Streit, Libor; Traoré, Hamady; Bénateau, Hervé

2017-02-01

A cleft palate results from incomplete fusion of the lateral palatine processes, the median nasal septum and the median palatine process. This case report describes a rare case of congenital teratoma originating from the nasal septum that may have interfered with the fusion of the palatal shelves during embryonic development, resulting in a cleft palate. An infant girl was born at 40 weeks of gestation weighing 3020 g with a complete cleft palate associated with a large central nasopharyngeal tumour. Computed tomography (CT) of the head showed a well defined mass of mixed density. The tumour was attached to the nasal septum in direct contact with the cleft palate. A biopsy confirmed the teratoma. Tumour resection was performed at 5 months, soft palate reconstruction at 7 months and hard palate closure at 14 months. There was no sign of local recurrence 1 year later. Most teratomas are benign and the prognosis is usually good. However, recurrence is not rare if germ cell carcinomatous foci are present within the teratoma. For these reasons, we advocate the use of a two-stage procedure in which closure of the cleft palate is postponed until histological examination confirms complete excision of the teratoma.

Local Wound Care for Primary Cleft Lip Repair: Treatment and Outcomes With use of Topical Hydrogen Peroxide.

PubMed

Strong, Amy L; Nauta, Allison C; Kuang, Anna A

2015-12-01

This study highlights and validates a peroxide-based wound healing strategy for treatment of surgically closed facial wounds in a pediatric population. The authors identified pediatric patients undergoing primary cleft lip repair as a specific population to evaluate the outcomes of such a protocol. Through analysis of defined outcome measures, a reliable and reproducible protocol for postoperative wound care following primary cleft lip repair with favorable results is described. This retrospective study analyzes wound healing outcomes in pediatric patients undergoing primary cleft lip repair from 2006 to 2011 at a tertiary academic center. The wound healing protocol was used in both primary unilateral and bilateral repairs. One hundred fortysix patients between the ages of 0 and 4 years underwent primary cleft lip repair and cleft rhinoplasty by a single, fellowship-trained craniofacial surgeon. Postoperatively, wounds were treated with half-strength hydrogen peroxide and bacitracin, as well as scar massage. Incisional dehiscence, hypertrophic scar formation, discoloration, infection, and reoperation were studied. Outcomes were evaluated in light of parent compliance, demographics, preoperative nasoalveolar molding (PNAM), and diagnosis. The authors identified 146 patients for inclusion in this study. There was no wound or incisional dehiscence. One hundred twenty-four patients demonstrated favorable cosmetic outcome. Only 3 (2%) of patients who developed suboptimal outcomes underwent secondary surgical revision (> 1 year after surgery). Demographic differences were not statistically significant, and PNAM treatment did not influence outcomes. These data validate the use of halfstrength hydrogen peroxide and bacitracin as part of a wound healing strategy in pediatric incisional wounds. The use of hydrogen peroxide produced comparable outcomes to previously published studies utilizing other wound healing strategies and, therefore, these study findings support the further use of this regimen for this particular population.

Gingival esthetics and oral health-related quality of life in patients with cleft lip and palate.

PubMed

Stelzle, F; Rohde, M; Oetter, N; Krug, K; Riemann, M; Adler, W; Neukam, F W; Knipfer, C

2017-08-01

While the oral health-related quality of life (OHRQoL) is known to be reduced in patients with cleft lip and palate (CLP), its inter-dependency with the soft tissue characteristics of the CLP area remains unclear. This study aimed to evaluate the soft tissue characteristics in the treated cleft area in order to investigate whether gingival esthetics correlate with OHRQoL. Thirty-six patients with unilateral or bilateral CLP (46 cleft areas) were investigated after secondary/tertiary alveolar bone grafting and orthodontic/prosthetic implant treatment using an adapted score to rate gingival esthetics (clinical esthetic score, CES). The patient's OHRQoL was determined using the German short version of the Oral Health Impact Profile questionnaire (OHIP-G14). The results showed a significantly better rating in patients with their own teeth in situ (12.05±1.10) than in patients with implants (6.95±4.78) or prosthetics (4.00±3.58). The best OHRQoL values were achieved by patients with their own teeth integrated into the cleft area (1.32±2.31), followed by patients with implants (2.33±2.33) and prosthetics (3.75±5.87). A significant (P=0.017) correlation was found between OHIP-G14 and CES scores, suggesting an increased OHRQoL in cases with higher oral esthetics in the cleft area. The therapeutic strategy contributes to both gingival esthetics and OHRQoL. The patient's subjective perception of OHRQoL can be attributed to objective gingival esthetic ratings. Copyright © 2017 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Postoperative alar base symmetry in complete unilateral cleft lip and palate:A prospective study.

PubMed

Vyloppilli, Suresh; Krishnakumar, K S; Sayd, Shermil; Latheef, Sameer; Narayanan, Saju V; Pati, Ajit

2017-11-01

In the evolution of cleft lip repair, there have been continuous attempts to minimize local trauma and to improve lip and nasal appearances. In order to obtain an aesthetically balanced development of midface, the primary surgical correction of the nasolabial area is of paramount importance. In this study, the importance of a back-cut extending cephalically above the inferior turbinate at the mucocutaneous junction which elevates the nostril floor on the cleft side for the purpose of achieving symmetry of the alar bases are analyzed by pre and postoperative photographic anthropometry. This study comprised of fifty cases of the unilateral complete cleft lip. At the time of surgery, the patient age ranged from 3-9 months. The surgeries, performed by a single surgeon, employed the standard Millard technique, incorporating Mohler modifications of lip repair. Anthropometric analysis revealed that the preoperative mean difference between the normal side and the cleft side was 0.2056 with a standard deviation of 0.133. In the postoperative analysis, the mean difference was reduced to 0.0174 with a standard deviation of 0.141. The paired t-test showed that the p-value is <0.001, indicating high statistical significance. To conclude, in complete unilateral cleft lip and palate, the geometrically placed nasal back-cut incision has a definite role in the correction of the alar base symmetry during primary surgery. Copyright © 2017 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

Anatomical variations of the facial nerve in first branchial cleft anomalies.

PubMed

Solares, C Arturo; Chan, James; Koltai, Peter J

2003-03-01

To review our experience with branchial cleft anomalies, with special attention to their subtypes and anatomical relationship to the facial nerve. Case series. Tertiary care center. Ten patients who underwent resection for anomalies of the first branchial cleft, with at least 1 year of follow-up, were included in the study. The data from all cases were collected in a prospective fashion, including immediate postoperative diagrams. Complete resection of the branchial cleft anomaly was performed in all cases. Wide exposure of the facial nerve was achieved using a modified Blair incision and superficial parotidectomy. Facial nerve monitoring was used in every case. The primary outcome measurements were facial nerve function and incidence of recurrence after resection of the branchial cleft anomaly. Ten patients, 6 females and 4 males,with a mean age of 9 years at presentation, were treated by the senior author (P.J.K.) between 1989 and 2001. The lesions were characterized as sinus tracts (n = 5), fistulous tracts (n = 3), and cysts (n = 2). Seven lesions were medial to the facial nerve, 2 were lateral to the facial nerve, and 1 was between branches of the facial nerve. There were no complications related to facial nerve paresis or paralysis, and none of the patients has had a recurrence. The successful treatment of branchial cleft anomalies requires a complete resection. A safe complete resection requires a full exposure of the facial nerve, as the lesions can be variably associated with the nerve.

Analysis of the correlative factors for velopharyngeal closure of patients with cleft palate after primary repair.

PubMed

Chen, Qi; Li, Yang; Shi, Bing; Yin, Heng; Zheng, Guang-Ning; Zheng, Qian

2013-12-01

The objective of this study was to analyze the correlative factors for velopharyngeal closure of patients with cleft palate after primary repair. Ninety-five nonsyndromic patients with cleft palate were enrolled. Two surgical techniques were applied in the patients: simple palatoplasty and combined palatoplasty with pharyngoplasty. All patients were assessed 6 months after the operation. The postoperative velopharyngeal closure (VPC) rate was compared by χ(2) test and the correlative factors were analyzed with logistic regression model. The postoperative VPC rate of young patients was higher than that of old patients, the group with incomplete cleft palate was higher than the group with complete cleft palate, and combined palatoplasty with pharyngoplasty was higher than simple palatoplasty. Operative age, cleft type, and surgical technique were the contributing factors for postoperative VPC rate. Operative age, cleft type, and surgical technique were significant factors influencing postoperative VPC rate of patients with cleft palate. Copyright © 2013 Elsevier Inc. All rights reserved.

Branchial cleft cyst: A case report and review of literature.

PubMed

Chavan, Surekha; Deshmukh, Revati; Karande, Prasad; Ingale, Yeshwant

2014-01-01

First branchial cleft anomaly is a rare disease of the head and neck. Because of its rarity, first branchial cleft anomaly is often misdiagnosed and results in inappropriate management. In this article, we present a case of type II first branchial cleft anomaly. A middle-aged woman who had suffered from swelling on lower jaw visited our department with the chief complaint of a swelling. She underwent complete excision of the lesion with preservation of the facial nerve. The patient recovered well and had no recurrence at 1-year of follow up.

First branchial cleft sinus presenting with cholesteatoma and external auditory canal atresia.

PubMed

Yalçin, Sinasi; Karlidağ, Turgut; Kaygusuz, Irfan; Demirbağ, Erhan

2003-07-01

First branchial cleft abnormalities are rare. They may involve the external auditory canal and middle ear. We describe a 6-year-old girl with congenital external auditory canal atresia, microtia, and cholesteatoma of mastoid and middle ear in addition to the first branchial cleft abnormalities. Clinical features of the patient are briefly described and the embryological relationship between first branchial cleft anomaly and external auditory canal atresia is discussed. The surgical management of these lesions may be performed, both the complete excision of the sinus and reconstructive otologic surgery.

Self-esteem, coping styles, and quality of life in polish adolescents and young adults with unilateral cleft lip and palate.

PubMed

Pisula, Ewa; Lukowska, Ewa; Fudalej, Piotr S

2014-05-01

Objectives : To evaluate self-esteem, coping styles, and health-related quality of life and their relationships in Polish adolescents and young adults with unilateral complete cleft lip and palate and related sex differences. Design and Participants : Self-report questionnaires measuring self-esteem (Multidimensional Self-Esteem Inventory), coping styles (Coping Inventory for Stressful Situations), and health-related quality of life (WHOQOL-BREF) were completed by 48 participants with cleft lip and palate (age, 16 to 23 years; 31 males, 17 females) and 48 controls without cleft lip and palate (age, 16 to 23 years; 28 males, 20 females) matched for age, place of residence, and socioeconomic status. Results : Regarding self-esteem, individuals with cleft lip and palate scored higher on body functioning (P < .01) and defensive self-enhancement (P < .05). Self-control showed an interaction effect: Females with cleft lip and palate scored higher than controls, but males did not differ between groups (P < .05). Males with cleft lip and palate scored lower than controls in personal power but higher in body functioning (P < .05); females showed no differences between groups. The groups did not differ with regard to coping styles or quality of life, but several correlations were found between self-esteem and coping styles, and quality of life (P < .01). Conclusions : Late adolescents and young adults with and without cleft lip and palate differed little in terms of psychological adjustment measures. The higher scores in defensive self-enhancement of individuals with cleft lip and palate suggest the need for instruments measuring social approval in psychosocial adjustment research involving this group.

Anophthalmia, cleft lip/palate, absent vomer bone, nystagmus, and mental-motor retardation: a new syndrome or Fryns "anophthalmia-plus" syndrome?

PubMed

Ozçelik, Derya; Sağlam, Ibrahim; SIlan, Fatma; Sezen, Gülbin; Unveren, Toygar

2008-05-01

We report that a 4-year-old boy presented with right unilateral complete cleft lip and palate, right anophthalmos, left congenital nystagmus, absence of the vomer bone, mental-motor retardation, and normal lymphocyte karyotype (46, XY). For reconstruction of the deformities, we performed cleft lip repair by Millard's rotation-advancement technique and planned cleft palate repair. This combination of cleft lip and palate, anophthalmos, congenital nystagmus, absent vomer bone, and mental-motor retardation has not, to our knowledge, previously been described. We suggest that this represents either another case of the rare Fryns "anophthalmia-plus" syndrome or a new syndrome.

First branchial cleft anomalies: avoiding the misdiagnosis.

PubMed

Kumar, Rajeev; Sikka, Kapil; Sagar, Prem; Kakkar, Aanchal; Thakar, Alok

2013-07-01

First branchial cleft anomalies are a very rare entities accounting for less than 1 % of all branchial cleft malformations. They are often misdiagnosed for other cystic lesions occurring in parotid gland and inadequately treated (incision and drainage or incomplete excision) leading to multiple recurrences. We report a series of four patients who were previously operated (incision and drainage) for misdiagnosed first branchial cleft anomalies with subsequent recurrences. All patients underwent superficial parotidectomy with complete tract excision using facial nerve monitoring to prevent iatrogenic injury because of extensive fibrosis. We discuss the literature pertaining to first branchial cleft anomalies, their varied presentations and their relationship to facial nerve in parotid gland and importance of facial nerve monitoring in revision surgery.

Selected Case From the Arkadi M. Rywlin International Pathology Slide Seminar: Benign Warthin Tumor of the Thyroid.

PubMed

Peckova, Kvetoslava; Daum, Ondrej; Michal, Michael; Curcikova, Radmila; Michal, Michal

2016-09-01

We report on an exceedingly rare lesion of the thyroid probably of a branchial cleft origin, which was not published in the world literature before. A 58-year-old woman underwent a total thyroidectomy for bilateral goiter. Grossly, there was one yellowish nodule sized 15 mm in the largest dimension found in the right lobe. Microscopically, the thyroid parenchyma showed signs of Hashimoto thyroiditis. The nodule in the right lobe was composed of a part of solid cell nests appearance, another part resembling a branchial cleft cyst, and a part resembling Warthin tumor. This lesion may belong to the histogenetically similar group of entities in the head and neck region which are derived from branchial cleft derivatives and which, under the inflammatory influence, have the ability to a cystic dilatation and proliferation of the epithelial component. The epithelium can afterwards become papillary and may undergo oncocytic transformation, thus gaining features that impart the resemblance of a Warthin tumor. Club members generally agreed with a submitted diagnosis of benign Warthin tumor of the thyroid.

[Risk factors for teeth aplasia and hypoplasia in cleft lip and palate children].

PubMed

Korolenkova, M V; Starikova, N V; Ageeva, L V

2016-01-01

The aim of the study was to assess the significance of environmental risk factors for teeth aplasia and hypoplasia in cleft lip and palate children. Two hundred and forty-seven cleft lip and palate (CLP) children were enrolled in the study including 105 (42.5%) with bilateral CLP and 57.5% with unilateral CLP. The mean age was 11.2±4.9 years. Teeth condition was assessed clinically and radiologically. The impact of risk factors for teeth anomalies was analyzed by retrospective data obtained from computer database (absence of preoperative orthopedic treatment, palatal defects after primary palatoplasty and type of primary procedures). Surgical trauma by early periosteoplasty (at the age of 3-4 months), excessive scarring and tissue traction due to absence of early orthopedic treatment and palatal defect were associated with significantly higher incidence of incisors hypoplasia (both developmental enamel defects and microdentia) and aplasia of central incisors not seen in the other study subgroups. Incisors aplasia and hypoplasia in CLP patients do not always have disembryogenic origin but may depend on external environmental factors, including surgical trauma.

19q13.32 microdeletion syndrome: three new cases.

PubMed

Castillo, Angela; Kramer, Nancy; Schwartz, Charles E; Miles, Judith H; DuPont, Barbara R; Rosenfeld, Jill A; Graham, John M

2014-01-01

A previous report described a unique phenotype associated with an apparently de novo 732 kb 19q13.32 microdeletion, consisting of intellectual disability, facial asymmetry, ptosis, oculomotor abnormalities, orofacial clefts, cardiac defects, scoliosis and chronic constipation. We report three unrelated patients with developmental delay and dysmorphic features, who were all found to have interstitial 19q13.32 microdeletions of varying sizes. Both the previously reported patient and our Patient 1 with a larger, 1.3-Mb deletion have distinctive dysmorphic features and medical problems, allowing us to define a recognizable 19q13.32 microdeletion syndrome. Patient 1 was hypotonic and dysmorphic at birth, with aplasia of the posterior corpus callosum, bilateral ptosis, oculomotor paralysis, down-slanting palpebral fissures, facial asymmetry, submucosal cleft palate, micrognathia, wide-spaced nipples, right-sided aortic arch, hypospadias, bilateral inguinal hernias, double toenail of the left second toe, partial 2-3 toe syndactyly, kyphoscoliosis and colonic atony. Therefore, the common features of the 19q13.32 microdeletion syndrome include facial asymmetry, ptosis, oculomotor paralysis, orofacial clefting, micrognathia, kyphoscoliosis, aortic defects and colonic atony. These findings are probably related to a deletion of some combination of the 20-23 genes in common between these two patients, especially NPAS1, NAPA, ARHGAP35, SLC8A2, DHX34, MEIS3, and ZNF541. These candidate genes are expressed in the brain parenchyma, glia, heart, gastrointestinal tract and musculoskeletal system and likely play a fundamental role in the expression of this phenotype. This report delineates the phenotypic spectrum associated with the haploinsufficiency of genes found in 19q13.32. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

Reading in Subjects with an Oral Cleft: Speech, Hearing and Neuropsychological Skills

PubMed Central

Conrad, Amy L.; McCoy, Thomasin E.; DeVolder, Ian; Richman, Lynn C.; Nopoulos, Peg

2014-01-01

Objective Evaluate speech, hearing, and neuropsychological correlates to reading among children, adolescents and young adults with non-syndromic cleft of the lip and/or palate (NSCL/P). Method All testing was completed in one visit at a Midwestern university hospital. Subjects in both the NSCL/P (n = 80) and control group (n = 62) ranged in age from 7 to 26 years (average age = 17.60 and 17.66, respectively). Subjects completed a battery of standardized tests evaluating intelligence, neuropsychological skills, and word reading. Subjects with NSCL/P also underwent speech assessment and past audiology records were evaluated. Results After controlling for age and SES, subjects with cleft performed significantly worse on a test of word reading. For subjects with cleft, word reading deficits were not associated with measures of speech or hearing, but were correlated with impairments in auditory memory. Conclusions These findings show poorer reading among subjects with NCL/P compared to those without. Further work needs to focus on correlates of reading among subjects with cleft to allow early identification and appropriate intervention/accommodation for those at risk. PMID:24188114

Study of relationship between clinical factors and velopharyngeal closure in cleft palate patients

PubMed Central

Chen, Qi; Zheng, Qian; Shi, Bing; Yin, Heng; Meng, Tian; Zheng, Guang-ning

2011-01-01

BACKGROUND: This study was carried out to analyze the relationship between clinical factors and velopharyngeal closure (VPC) in cleft palate patients. METHODS: Chi-square test was used to compare the postoperative velopharyngeal closure rate. Logistic regression model was used to analyze independent variables associated with velopharyngeal closure. RESULTS: Difference of postoperative VPC rate in different cleft types, operative ages and surgical techniques was significant (P=0.000). Results of logistic regression analysis suggested that when operative age was beyond deciduous dentition stage, or cleft palate type was complete, or just had undergone a simple palatoplasty without levator veli palatini retropositioning, patients would suffer a higher velopharyngeal insufficiency rate after primary palatal repair. CONCLUSIONS: Cleft type, operative age and surgical technique were the contributing factors influencing VPC rate after primary palatal repair of cleft palate patients. PMID:22279464

Doubtful descent, dilemma and diagnosis: a case of Kallmann syndrome.

PubMed

Chakraborty, P P; Chowdhury, S R; Mandal, S K; Bandyopadhyay, D

2007-03-01

A 16-year-old boy with a diagnosis of bilateral cryptorchidism was referred for preoperative evaluation. He had diminished hearing and difficulty in vision since birth, with inattentiveness, poor school performance and delayed milestones. He was previously operated on for cleft lip. General survey revealed bilateral short fourth metacarpals and an operative scar mark over the left nostril and upper lip. He had a micropenis, small soft testes with anosmia, and sensory-motor deafness. The hormonal assay was consistent with hypogonadotrophic hypogonadism. Magnetic resonance imaging of the brain and computed tomography cisternography revealed almost hypoplastic olfactory bulb with an ill-defined olfactory tract and sulci, supporting the clinical diagnosis of Kallmann syndrome.

Branchial cleft cyst: A case report and review of literature

PubMed Central

Chavan, Surekha; Deshmukh, Revati; Karande, Prasad; Ingale, Yeshwant

2014-01-01

First branchial cleft anomaly is a rare disease of the head and neck. Because of its rarity, first branchial cleft anomaly is often misdiagnosed and results in inappropriate management. In this article, we present a case of type II first branchial cleft anomaly. A middle-aged woman who had suffered from swelling on lower jaw visited our department with the chief complaint of a swelling. She underwent complete excision of the lesion with preservation of the facial nerve. The patient recovered well and had no recurrence at 1-year of follow up. PMID:24959062

Measuring Symmetry in Children With Unrepaired Cleft Lip: Defining a Standard for the Three-Dimensional Midfacial Reference Plane.

PubMed

Wu, Jia; Heike, Carrie; Birgfeld, Craig; Evans, Kelly; Maga, Murat; Morrison, Clinton; Saltzman, Babette; Shapiro, Linda; Tse, Raymond

2016-11-01

  Quantitative measures of facial form to evaluate treatment outcomes for cleft lip (CL) are currently limited. Computer-based analysis of three-dimensional (3D) images provides an opportunity for efficient and objective analysis. The purpose of this study was to define a computer-based standard of identifying the 3D midfacial reference plane of the face in children with unrepaired cleft lip for measurement of facial symmetry.   The 3D images of 50 subjects (35 with unilateral CL, 10 with bilateral CL, five controls) were included in this study.   Five methods of defining a midfacial plane were applied to each image, including two human-based (Direct Placement, Manual Landmark) and three computer-based (Mirror, Deformation, Learning) methods.   Six blinded raters (three cleft surgeons, two craniofacial pediatricians, and one craniofacial researcher) independently ranked and rated the accuracy of the defined planes.   Among computer-based methods, the Deformation method performed significantly better than the others. Although human-based methods performed best, there was no significant difference compared with the Deformation method. The average correlation coefficient among raters was .4; however, it was .7 and .9 when the angular difference between planes was greater than 6° and 8°, respectively.   Raters can agree on the 3D midfacial reference plane in children with unrepaired CL using digital surface mesh. The Deformation method performed best among computer-based methods evaluated and can be considered a useful tool to carry out automated measurements of facial symmetry in children with unrepaired cleft lip.

Orthodontic Treatment Combined with Orthognathic Surgery and Simultaneous Alveolar Bone Graft of a Unilateral Complete Cleft Lip and Palate Patient: A Case Report.

PubMed

Pisek, Poonsak; Manosudprasit, Montian; Wangsrimongkol, Tasanee; Jinaporntham, Suthin; Wiwatworakul, Opas

2015-08-01

This article aimed to present a case of 22 year-old Thai female with cleft lip and palate who had malocclusion developed from dental problems, skeletal disharmony and unrepaired alveolar cleft. The treatment was orthodontic combined with one-stage surgical correction which corrected skeletal discrepancy and alveolar cleft in single operation. After treatment, the patient had improved in facial esthetics, attaining good occlusal function and continuous maxillary dental arch. This procedure can reduce morbidity, preclude a second hospitalization and the cost of two-stage surgical correction. However this is only an alternative treatment for adult cleft patients who need late alveolar bone graft and orthognathic surgery.

Arrangement of the orbicularis oris muscle in different types of cleft lips.

PubMed

Wijayaweera, C J; Amaratunga, N A; Angunawela, P

2000-05-01

A thorough knowledge of the anatomy of the labial region, especially the arrangement of the muscle fibers, is essential for the success of primary repair of the cleft lip. Pared lateral and medial edges from 20 unilateral incomplete cleft lips and 25 unilateral complete cleft lips were obtained during primary surgery. Three specimens of normal lips were taken from unclaimed infant cadavers as the controls. They were prepared for routine histological studies and were examined to study the direction of muscle fibers. Intrinsic and extrinsic bundles were identified in both lateral and medial sides of specimens of both cleft types. The intrinsic bundle was not displaced but was interrupted by the cleft. The extrinsic bundle in the lateral side of both cleft types ran upward along the lateral cleft margin, whereas in the medial side it ran horizontally to terminate close to the medial cleft margin. The extrinsic bundle is the retractor, which is associated with facial expression, whereas the intrinsic bundle is the constrictor of the mouth. Because there are two functional components in the orbicularis oris muscle, identifying and repairing them separately will enable each of them to accomplish their distinctive functions.

Computer-assisted preoperative simulation for positioning and fixation of plate in 2-stage procedure combining maxillary advancement by distraction technique and mandibular setback surgery.

PubMed

Suenaga, Hideyuki; Taniguchi, Asako; Yonenaga, Kazumichi; Hoshi, Kazuto; Takato, Tsuyoshi

2016-01-01

Computer-assisted preoperative simulation surgery is employed to plan and interact with the 3D images during the orthognathic procedure. It is useful for positioning and fixation of maxilla by a plate. We report a case of maxillary retrusion by a bilateral cleft lip and palate, in which a 2-stage orthognathic procedure (maxillary advancement by distraction technique and mandibular setback surgery) was performed following a computer-assisted preoperative simulation planning to achieve the positioning and fixation of the plate. A high accuracy was achieved in the present case. A 21-year-old male patient presented to our department with a complaint of maxillary retrusion following bilateral cleft lip and palate. Computer-assisted preoperative simulation with 2-stage orthognathic procedure using distraction technique and mandibular setback surgery was planned. The preoperative planning of the procedure resulted in good aesthetic outcomes. The error of the maxillary position was less than 1mm. The implementation of the computer-assisted preoperative simulation for the positioning and fixation of plate in 2-stage orthognathic procedure using distraction technique and mandibular setback surgery yielded good results. Copyright © 2016 The Author(s). Published by Elsevier Ltd.. All rights reserved.

[Effects of maxillary distraction osteogenesis on the velopharyngeal configuration of cleft palate patients].

PubMed

Wang, Xiao-xia; Wang, Xing; Yi, Biao; Li, Zi-li; Liang, Cheng

2005-12-18

To study the effects of internal maxillary distraction osteogenesis(DO) on the velopharyngeal configuration of cleft palate patients. Ten patients with severe maxillary hypoplasia secondary to cleft lip and palate patients (7 males and 3 females, average age 20.1 years old) had undertaken high step LeFort I osteotomy, and internal maxillary distraction devices were applied to advance the maxilla. Before surgery, when DO was completed and 6 months after DO was completed, oriented lateral cephalograms at rest position of each patient were taken, and 6 measure indexes of velopharyngeal configuration were collected and analyzed. All patients had successfully accomplished maxillary DO and the maxilla had been averagely advanced 11.3 mm. PNS-PhW, C-PhW, UL and ANS-PNS-T had all significantly increased, and UD had significantly decreased when DO was completed and 6 months after DO was completed as compared with pre-surgery. No significant linear correlation was found between maxilla advancement distance and velopharyngeal configuration changes. Correction of maxillary hypoplasia secondary to cleft palate surgery by using internal maxillary DO can increase the velopharyngeal cavity depth, and may impair velopharyngeal competence, but the compensatory changes of velopharyngeal soft tissue can alleviate this impairment to certain extent.

Correlations between initial cleft size and dental anomalies in unilateral cleft lip and palate patients after alveolar bone grafting.

PubMed

Jabbari, Fatima; Reiser, Erika; Thor, Andreas; Hakelius, Malin; Nowinski, Daniel

2016-01-01

Objective To determine in individuals with unilateral cleft lip and palate the correlation between initial cleft size and dental anomalies, and the outcome of alveolar bone grafting. Methods A total of 67 consecutive patients with non-syndromic unilateral complete cleft lip and palate (UCLP) were included from the cleft lip and palate-craniofacial center, Uppsala University Hospital, Sweden. All patients were operated by the same surgeon and treated according to the Uppsala protocol entailing: lip plasty at 3 months, soft palate closure at 6 months, closure of the residual cleft in the hard palate at 2 years of age, and secondary alveolar bone grafting (SABG) prior to the eruption of the permanent canine. Cleft size was measured on dental casts obtained at the time of primary lip plasty. Dental anomalies were registered on radiographs and dental casts obtained before bone grafting. Alveolar bone height was evaluated with the Modified Bergland Index (mBI) at 1 and 10-year follow-up. Results Anterior cleft width correlated positively with enamel hypoplasia and rotation of the central incisor adjacent to the cleft. There was, however, no correlation between initial cleft width and alveolar bone height at either 1 or 10 years follow-up. Conclusions Wider clefts did not seem to have an impact on the success of secondary alveolar bone grafting but appeared to be associated with a higher degree of some dental anomalies. This finding may have implications for patient counseling and treatment planning.

The characteristics and distribution of dental anomalies in patients with cleft.

PubMed

Wu, Ting-Ting; Chen, Philip K T; Lo, Lun-Jou; Cheng, Min-Chi; Ko, Ellen Wen-Ching

2011-01-01

Dental anomalies associated with different severities of cleft lip and palate have been rarely reported. This retrospective study investigates the characteristics of dental anomalies associated with different types of cleft, and compares the dental anomaly traits based on sex and severity of cleft. Cleft patients born in 1995 with qualified diagnostic records from 7 to 11 years were included for evaluation. Records were retrieved from database of Chang Gung Craniofacial Center, including panoramic radiographs and intraoral photographs. In total, 196 patients with complete records were included in the evaluation. This study compares the dental anomalies associated with each type of cleft. The frequency of dental anomalies in the maxillary incisor area in the cleft palate (CP) group (20%) was significantly lower than that in other groups. The frequency of missing maxillary lateral incisors (MLIs) increased as the cleft severity increased. Supernumerary teeth and missing lower incisors exhibited the opposite trend. No sexual dimorphism appeared in terms of the frequencies of peg laterals and missing MLIs. The distribution patterns of missing MLIs and peg laterals in males, but not in females, were consistent for the three types of unilateral clefts. Regarding the characteristics of dental anomalies among the three unilateral clefts, missing MLIs, supernumerary teeth, and missing lower incisors were found to be related to cleft severity. The maxillary lateral incisor was the most affected tooth in the cleft area. The frequency of missing MLIs and peg laterals was not sexual dimorphic, but the distribution pattern was different between the sexes.

A congenital true teratoma with cleft lip, palate, and columellar sinus.

PubMed

Işken, Tonguç; Alagöz, M Sahin; Günlemez, Ayla; Unal, Ciğdem; Sen, Cenk; Onyedi, Murat; Işil, Eda; Izmirli, Hakki; Yücel, Ergin

2007-09-01

Teratomas, the most common extragonadal germ cell tumor of childhood, involve at least two of the ectodermal, mesodermal, and endodermal layers. Of the teratomas seen in the first 2 months of life, 82% are sacrococcygeal. The head and neck region is the second most common location for teratomas in early infancy, accounting for five (14%) of those cases. We describe a female neonate with a teratoma of the nasopharyngeal area, bilateral cleft palate/lip, and columellar sinus pathologies. The mass, which was 8 x 5 x 7 cm and soft in consistency, blocked the airway and prevented oral feeding. On macroscopic examination of the excised mass, there was a notable typical cilia arrangement and lower eyelid appearance. The patient, who was diagnosed with a well-differentiated teratoma after the pathologic examination, did not have any complications in the postoperative period.

[The paternal origin of an extra chromosome 13 from the patient with patau syndrome.].

PubMed

Zhou, Qin; Cui, Ying-Xia; Wang, Yong-Mei; Huang, Yu-Feng

2005-05-01

A boy was born at 38 week's gestation by Cesarean delivery . Affected newborn was characteristically bilateral cleft lip and severe cleft palate. The sternum was malformation. The sacrospinal bifida and the talipes varus were found and the testes were not descended. The boy died after delivery within 10 minutes owing to respiratory failure. Cytogenetic analysis of his peripheral lymphocyte by G banding showed a karyotype 47,XY,+13 , which was also confirmed by fluorescence in situ hybridization (FISH). The locus D13S317 was detected by the peripheral blood from the boy and the parents. Three alleles were found from the boy in locus D13S317 and two from the father. The extra chromosome 13 was from the nondisjunction during the first meiotic division of the father.

Cleft closure and undersizing annuloplasty improve mitral repair in atrioventricular canal defects

PubMed Central

Padala, Muralidhar; Vasilyev, Nikolay V.; Owen, James W.; Jimenez, Jorge H.; Dasi, Lakshmi P.; del Nido, Pedro J.; Yoganathan, Ajit P.

2009-01-01

Objective Reoperation rates to correct left atrioventricular valve regurgitation after primary repair of atrioventricular canal defects remain relatively high. The causes of valvular regurgitation are likely multifactorial, and simple cleft closure is often insufficient to prevent recurrence. Methods To elucidate the mechanisms leading to regurgitation, we conducted hemodynamic studies using isolated native mitral valves. Anatomy of these valves was altered to mimic atrioventricular canal type valves and studied under pediatric hemodynamic conditions. The impact of subvalvular geometry, cleft closure, annular dilatation, and annular undersizing on regurgitation were investigated. Results Papillary muscle position did not have a significant effect on regurgitation. Cleft closure had a significant impact on valvular competence, with reduction in regurgitation volume with increased cleft closure. Regurgitation volume decreased from 12.5 ± 2.4 mL/beat for an open cleft to 4.9 ± 1.9 mL/beat for a partially closed cleft and to 1.4 ± 1.6 mL/beat when the cleft was completely closed. Annular dilatation had a significant impact on regurgitation even after cleft closure. A 40% increase in annular size increased regurgitation by 59% for a partially closed cleft and by 84% for a fully closed cleft. Reducing the annular size by 20% from the physiologic level decreased the regurgitation volume by 12% for a fully open cleft and by 58% for the partially closed cleft case. Conclusions Annular dilatation after primary repair has a potentially significant role in the recurrence of atrioventricular valve regurgitation. Reducing the annular size and restricting dilatation as an adjunct to cleft closure is a promising surgical approach in such valve anatomies. PMID:19026810

Influence of different palate repair protocols on facial growth in unilateral complete cleft lip and palate.

PubMed

Xu, Xue; Kwon, Hyuk-Jae; Shi, Bing; Zheng, Qian; Yin, Heng; Li, Chenghao

2015-01-01

To address the question of whether one- or two-stage palatal treatment protocol has fewer detrimental effects on craniofacial growth in patients aged 5 years with unilateral complete cleft lip and palate. Forty patients with non-syndromic unilateral complete cleft lip and palate (UCCLPs) who had received primary cleft lip repair at age 6-12 months and cleft palate repair at age 18-30 months were selected in this study. Eighteen UCCLP patients who received two-stage palate repair were selected as group 1, and 22 UCCLP patients who received one-stage palate repair were selected as group 2. The control group consisted of 20 patients with unilateral incomplete cleft lip (UICL patients) whose age and gender matched with UCCLP patients. A one-sample Kolmogorov-Smirnov test was used to analyze the nature of data distribution. Bonferroni test and Kruskal-Wallis H tests were used for multiple comparisons. Both case groups showed reduced maxillary sagittal length (ANS-PMP, A-PM, p < 0.05) and retrusion of the maxilla (S-Ptm, p < 0.05), A point and ANS point (Ba-N-A, Ba-N-ANS, p < 0.05). Patients treated with two-stage palate repair had a reduced posterior maxillary vertical height (R-PMP, p < 0.05). Our results indicated that maxillary sagittal length and position could be perturbed by both one- and two-stage palate repair. Vomer flap repair inhibited maxilla vertical growth. Delayed hard palate repair showed less detrimental effects on maxillary growth compared to early hard palate repair in UCCLP patients aged 5 years. Copyright © 2014 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

A Comparative Study of Oral Microbiota in Infants with Complete Cleft Lip and Palate or Cleft Soft Palate

PubMed Central

Tanasiewicz, Marta

2017-01-01

Few reports have been published on the early microbiota in infants with various types of cleft palate. We assessed the formation of the oral microbiota in infants with complete cleft lip and palate (CLP n = 30) or cleft soft palate (CSP n = 25) in the neonatal period (T1 time) and again in the gum pad stage (T2 time). Culture swabs from the tongue, palate, and/or cleft margin at T1 and T2 were taken. We analysed the prevalence of the given bacterial species (the percentage) and the proportions in which the palate and tongue were colonised by each microorganism. At T1, Streptococcus mitis (S. mitis) were the most frequently detected in subjects with CLP or CSP (63% and 60%, resp.). A significantly higher frequency of methicillin-sensitive Staphylococcus aureus (S. aureus MSSA) was observed in CLP compared to the CSP group. At T2, significantly higher percentages of S. mitis, S. aureus MSSA, Staphylococcus epidermidis, and members of the Enterobacteriaceae family were noted in CLP infants compared to the CSP. S. mitis and Streptococcus sanguinis appeared with the greatest frequency on the tongue, whereas Streptococcus salivarius was predominant on the palate. The development of the microbiota in CLP subjects was characterised by a significant increase in the prevalence of pathogenic bacteria. PMID:28393073

Infant orthopedics has no effect on maxillary arch dimensions in the deciduous dentition of children with complete unilateral cleft lip and palate (Dutchcleft).

PubMed

Bongaarts, Catharina A M; van 't Hof, Martin A; Prahl-Andersen, Birte; Dirks, Iris V; Kuijpers-Jagtman, Anne M

2006-11-01

Evaluation of the effect of infant orthopedics on maxillary arch dimensions in the deciduous dentition in patients with unilateral cleft lip and palate. Prospective two-arm randomized controlled clinical trial with three participating cleft palate centers. Cleft palate centers of the Radboud University Nijmegen Medical Center, Academic Center of Dentistry Amsterdam, and University Medical Center Rotterdam, the Netherlands. Children with complete unilateral cleft lip and palate (n = 54) were included. Patients were randomly divided into two groups. Half of the patients (IO+) had a presurgical orthopedic plate until surgical closure of the soft palate at the age of 52 weeks; the other half (IO-) did not undergo presurgical orthopedics. Maxillary arch dimensions were assessed on dental casts at 4 and 6 years of age with measurements for arch width, arch depth, arch length, arch form, and the vertical position of the lesser segment. Contact and collapse were assessed also. There were no clinically significant differences found between IO+ and IO- for any of the variables. Infant orthopedics had no observable effect on the maxillary arch dimensions or on the contact and collapse scores in the deciduous dentition at the ages of 4 and 6 years. Considering the Dutchcleft results to date, there is no need to perform infant orthopedics for unilateral cleft lip and palate patients.

A Comparative Study of Oral Microbiota in Infants with Complete Cleft Lip and Palate or Cleft Soft Palate.

PubMed

Machorowska-Pieniążek, Agnieszka; Mertas, Anna; Skucha-Nowak, Małgorzata; Tanasiewicz, Marta; Morawiec, Tadeusz

2017-01-01

Few reports have been published on the early microbiota in infants with various types of cleft palate. We assessed the formation of the oral microbiota in infants with complete cleft lip and palate (CLP n = 30) or cleft soft palate (CSP n = 25) in the neonatal period (T1 time) and again in the gum pad stage (T2 time). Culture swabs from the tongue, palate, and/or cleft margin at T1 and T2 were taken. We analysed the prevalence of the given bacterial species (the percentage) and the proportions in which the palate and tongue were colonised by each microorganism. At T1, Streptococcus mitis (S. mitis) were the most frequently detected in subjects with CLP or CSP (63% and 60%, resp.). A significantly higher frequency of methicillin-sensitive Staphylococcus aureus ( S. aureus MSSA) was observed in CLP compared to the CSP group. At T2, significantly higher percentages of S. mitis , S. aureus MSSA, Staphylococcus epidermidis , and members of the Enterobacteriaceae family were noted in CLP infants compared to the CSP. S. mitis and Streptococcus sanguinis appeared with the greatest frequency on the tongue, whereas Streptococcus salivarius was predominant on the palate. The development of the microbiota in CLP subjects was characterised by a significant increase in the prevalence of pathogenic bacteria.

Branchial cleft cyst encircling the hypoglossal nerve

PubMed Central

Long, Kristin L.; Spears, Carol; Kenady, Daniel E.

2013-01-01

Branchial cleft anomalies are a common cause of lateral neck masses and may present with infection, cyst enlargement or fistulas. They may affect any of the nearby neck structures, causing compressive symptoms or vessel thrombosis. We present a case of a branchial cleft cyst in a 10-year-old boy who had been present for 1year. At the time of operation, the cyst was found to completely envelop the hypoglossal nerve. While reports of hypoglossal nerve palsies due to external compression from cysts are known, we believe this to be the first report of direct nerve involvement by a branchial cleft cyst. PMID:24963902

Reconstruction of Drug-induced Cleft Palate Using Bone Marrow Mesenchymal Stem Cell in Rodents.

PubMed

Amalraj, Julie Christy; Gangothri, Manasa; Babu, Hari

2017-01-01

Triamcinolone acetonide (TAC) (Kenacort*) is a commonly used synthetic glucocorticoid in today's medical practice. The drug is also a potential agent in inducing cleft palates in rats. This drug has been used to induce cleft palate in the fetus of the pregnant rats to bring out a suitable animal model for human cleft lip and palate. The drug was given intraperitoneally to induce congenital cleft palate in pregnant mother rats. The aim of this study is to induce congenital cleft palate in pregnant Wister albino rats and reconstruct the defect with bone marrow mesenchymal stem cells (BMSCs) isolated from the same species along with PLGA (poly lactic co glycolic acid) scaffold. Twenty female animals were divided into two groups. Each group contains 10 animals. The animals were allowed to mate with male rat during the esterase period and the day, in hich vaginal plug was noticed was taken to be day 0. The pregnant rats were given triamcinolone acetonide (Kenacort* 10 mg/1 ml intramuscularly/intravenous [IM/IV] injections) injection intraperitoneally at two different dosages as the existing literature. The injection was given on the 10, 12, and 14 th day of gestation. The clinical changes observed were recorded, and the change in the body weight was noted carefully. Group 1 which received 0.5 mg/kg body weight of TAC had many drug toxic effects. Group 2 which received 0.05 mg/kg body weight produced cleft palate in rat pups. The pups were divided into three groups. Group A control group without cell transplant, the cleft was allowed to close by itself. Group B containing palate reconstructed with plain PLGA scaffold (Bioscaffold, Singapore) without BMSC, Group C containing BMSC and PLGA scaffold (Bioscaffold, Singapore), Group C operated for the cleft palate reconstruction using BMSCs and PLGA scaffold. There was faster and efficient reconstruction of bone in the cleft defect in Group C while there was no defect closure in Group A and B. There was complete reconstruction of the cleft palate in the group of rat pups which received BMSCs along with PLGA scaffold. Bone growth in the cleft defect was faster; complete fusion of the defect was achieved. The dosage of drug used for inducing cleft palate was standardized in rodents for a definitive congenital cleft palate model. The cleft palate induced was reconstructed using BMSCs and PLGA scaffold. This was compared with a control group and the other group with plain PLGA used for reconstruction of the palate. This study will invite future research in the effect of the drug on human beings, especially on pregnant mothers.

Practical Repair Method for Unilateral Cleft Lips: Straight-Line Advanced Release Technique.

PubMed

Baek, Rong-Min; Choi, Jun-Ho; Kim, Baek-Kyu

2016-04-01

Straight-line closure repair of unilateral cleft lips was first introduced in the 1840s, and since then, many different techniques have been attempted for cleft repair. However, these methods have several disadvantages and are difficult to adopt. In this study, we describe our novel technique, known as Straight-Line Advanced Release Technique (StART), and its application in treating several cases of unilateral cleft lip. The preoperative design of the surgical method is drawn on the skin, the vermilion, and the oral mucosa. A total of 13 points are marked (points 0-12). The A flap, B flap, triangular flap, M (medial mucosal) flap, and L (lateral mucosal) flap are designed. After completion of the preoperative marking, the wide dissection is performed to separate the orbicularis oris muscle completely from the abnormally inserted bony structure and the enveloped skin-mucosal flap. The freed orbicularis oris muscle is then reconstructed with full width. After all planes of the lip wound are closed, a straight vertical skin suture line is achieved without any unnecessary transverse scar. Unilateral cleft lip repair using StART was conducted in 145 patients between 1993 and 2012. Cases of microform cleft lip were excluded. A total of 21 patients (14%) required a secondary operation on the lip after the first unilateral cheiloplasty. In all patients, satisfactory surgical outcomes were obtained with an indistinct straight-lined scar and a well-aligned lip contour. To acquire a natural and balanced shape in unilateral cleft lip repair, we recommend the novel StART.

Video-imaging assessment of nasal morphology in individuals with complete unilateral cleft lip and palate.

PubMed

Russell, K A; Waldman, S D; Lee, J M

2000-11-01

The purpose of this study was to develop a video-imaging mathematical method to assess nostril morphology. This retrospective study involved two age-matched groups: 28 subjects with complete unilateral cleft lip and palate (CUCLP) and 19 noncleft controls. Nose casts were reproducibly oriented in a jig such that the casts could be rotated about the coronal axis. Video images of the nostrils were captured and then analyzed for area, perimeter, centroid, principal axis, moments about the major and minor axes (I11, I22), anisometry, bulkiness, lateral offset, internostril angle, and rotational angle. All parameters identified nostril asymmetry in both groups. The results of the analyses using anisometry, I11, and I22 showed that, in both groups, one nostril was rounder and one was more elliptical. This asymmetry, however, differed between the two groups, and the difference was primarily based on the degree of ellipticity of the nostrils. Maximum dimension, perimeter, lateral offset, I11, and I22 were more asymmetric in the cleft group. In the control group, the right nostril was more elliptical and had a greater perimeter, and the left-side nostril had a greater bulkiness (enfolding). The method developed was validated for assessment of nasal morphology in cleft and noncleft samples. Nostril morphology was asymmetric in both groups but more asymmetric in the cleft group than the control group. The dominant influence of the cleft resulted in more elliptical noncleft nostrils and greater nostril shape asymmetry in the cleft group. The validated video-imaging method can now be used to assess the efficacy of treatment on nasal morphology.

Long-term follow-up of early cleft maxillary distraction.

PubMed

Park, Young-Wook; Kwon, Kwang-Jun; Kim, Min-Keun

2016-12-01

Most of cleft lip and palate patients have the esthetic and functional problems of midfacial deficiencies due to innate developmental tendency and scar tissues from repeated operations. In these cases, maxillary protraction is required for the harmonious facial esthetics and functional occlusion. A 7-year old boy had been diagnosed as severe maxillary constriction due to unilateral complete cleft lip and palate. The author tried to correct the secondary deformity by early distraction osteogenesis with the aim of avoiding marked psychological impact from peers of elementary school. From 1999 to 2006, repeated treatments, which consisted of Le Fort I osteotomy and face mask distraction, and complementary maxillary protraction using miniplates were performed including orthodontics. But, final facial profile was not satisfactory, which needs compromising surgery. The result of this study suggests that if early distraction treatment is performed before facial skeletal growth is completed, an orthognathic surgery or additional distraction may be needed later. Maxillofacial plastic and reconstructive surgeons should notify this point when they plan early distraction treatment for cleft maxillary deformity.

Preoperative evaluation of micro-organisms in non-operated cleft in soft palate: impact on use of antibiotics.

PubMed

Roode, G J; Bütow, K-W; Naidoo, S

2017-02-01

To identify the pathogenic micro-organisms that had colonised preoperatively in clefts in the soft palate and oro-nasopharynx, we retrospectively studied the preoperative microbiological profiles of 200 infants who had had primary repair of all types of cleft in the soft palate. Data from a private practice that specialises in the repair of facial clefts were extracted randomly from patients' files. We analysed the results of the culture of preoperative swabs taken from clefts in the soft palate and oro-nasopharynx, and the resistance profile of organisms towards various antibiotics. A total of 23 different pathogenic micro-organisms were isolated from 115 (57%) of the sample. Klebsiella pneumoniae most commonly colonised clefts in the lip, alveolus, and palate. This was considerably higher than in other groups. The second most common micro-organism was Staphylococcus aureus, which was found most often in patients with isolated clefts in the hard palate. Those with complete cleft lip and palate presented with more pathogenic micro-organisms in preoperative cultures than those with other types of cleft. We need to find a way to control pathogenic micro-organisms in the oral and oro-nasopharyngeal region preoperatively to limit postoperative complications. Copyright © 2016 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

The impact of cleft lip and palate repair on maxillofacial growth.

PubMed

Shi, Bing; Losee, Joseph E

2015-03-23

Surgical correction is central to current team-approached cleft treatment. Cleft surgeons are always concerned about the impact of their surgical maneuver on the growth of the maxilla. Hypoplastic maxilla, concaved mid-face and deformed dental arch have constantly been reported after cleft treatments. It is very hard to completely circumvent these postoperative complications by current surgical protocols. In this paper, we discussed the factors that inhibit the maxillofacial growth on cleft patients. These factors included pre-surgical intervention, the timing of cleft palate and alveolae repair, surgical design and treatment protocol. Also, we made a review about the influence on the maxillary growth in un-operated cleft patients. On the basis of previous researches, we can conclude that most of scholars express identity of views in these aspects: early palatoplasty lead to maxilla growth inhibition in all dimensions; secondary alveolar bone graft had no influence on maxilla sagittal growth; cleft lip repair inhibited maxilla sagittal length in patients with cleft lip and palate; Veau's pushback palatoplasty and Langenbeck's palatoplasty with relaxing incisions were most detrimental to growth; Furlow palatoplasty showed little detrimental effect on maxilla growth; timing of hard palate closure, instead of the sequence of hard or soft palate repair, determined the postoperative growth. Still, scholars hold controversial viewpoints in some issues, for example, un-operated clefts have normal growth potential or not, pre-surgical intervention and pharyngoplasty inhibited maxillofacial growth or not.

Maternal report of fever from cold or flu during early pregnancy and the risk for noncardiac birth defects, National Birth Defects Prevention Study, 1997-2011.

PubMed

Waller, Dorothy Kim; Hashmi, Syed Shahrukh; Hoyt, Adrienne T; Duong, Hao T; Tinker, Sarah C; Gallaway, Michael Shayne; Olney, Richard S; Finnell, Richard H; Hecht, Jacqueline Tauber; Canfield, Mark A

2018-03-01

As maternal fever affects approximately 6-8% of early pregnancies, it is important to expand upon previous observations of an association between maternal fever and birth defects. We analyzed data from the National Birth Defects Prevention Study, a multistate, case-control study of major structural birth defects. Telephone interviews were completed by mothers of cases (n = 17,162) and controls (n = 10,127). Using multivariable logistic regression, we assessed the association between maternal self-report of cold or flu with fever and cold or flu without fever during early pregnancy and 30 categories of non-cardiac birth defects. Maternal report of cold or flu with fever was significantly associated with 8 birth defects (anencephaly, spina bifida, encephalocele, cleft lip with or without cleft palate, colonic atresia/stenosis, bilateral renal agenesis/hypoplasia, limb reduction defects, and gastroschisis) with elevated adjusted odds ratios ranging from 1.2 to 3.7. Maternal report of cold or flu without fever was not associated with any of the birth defects studied. This study adds to the evidence that maternal fever during early pregnancy is associated with an increased risk for selected birth defects. Elevated associations were limited to mothers who reported a fever, suggesting that it is fever that contributes to the excess risk rather than illnesses associated with it. However, fever may also serve as a marker for more severe infections. © 2017 Wiley Periodicals, Inc.

Preoperative Cleft Lip Measurements and Maxillary Growth in Patients With Unilateral Cleft Lip and Palate.

PubMed

Antonarakis, Gregory S; Tompson, Bryan D; Fisher, David M

2016-11-01

Maxillary growth in patients with cleft lip and palate is highly variable. The authors' aim was to investigate associations between preoperative cleft lip measurements and maxillary growth determined cephalometrically in patients with complete unilateral cleft lip and palate (cUCLP). Retrospective cross-sectional study. Children with cUCLP. Preoperative cleft lip measurements were made at the time of primary cheiloplasty and available for each patient. Maxillary growth was evaluated on lateral cephalometric radiographs taken prior to any orthodontic treatment and alveolar bone grafting (8.5 ± 0.7 years). The presence of associations between preoperative cleft lip measurements and cephalometric measures of maxillary growth was determined using regression analyses. In the 58 patients included in the study, the cleft lateral lip element was deficient in height in 90% and in transverse width in 81% of patients. There was an inverse correlation between cleft lateral lip height and transverse width with a β coefficient of -0.382 (P = .003). Patients with a more deficient cleft lateral lip height displayed a shorter maxillary length (β coefficient = 0.336; P = .010), a less protruded maxilla (β coefficient = .334; P = .008), and a shorter anterior maxillary height (β coefficient = 0.306; P = .020) than those with a less deficient cleft lateral lip height. Patients with cUCLP present with varying degrees of lateral lip hypoplasia. Preoperative measures of lateral lip deficiency are related to later observed deficiencies of maxillary length, protrusion, and height.

Developmental Toxicity Potential of Nitroguanidine in Rats

DTIC Science & Technology

1988-02-01

shape in which the body was short and thick, particularly through the neck. In the 316 mg/kg/day group, one fetus had bilateral anophthalmia , hypoplastic... Anophthalmia 1/1 Hypoplastic pinnae 1/1 Lowr jaw absent 1/1 Variations Hematoma / /i/ Lips scalloped at edge 1/1 A C.ngle fetus may have more than one...as malformations included cleft palate, malformed mandible, extra vertebrae, anasarca, anophthalmia , abnormal heart, marked enlargement of the

Bone-borne palatal distraction to correct the constricted cleft maxilla.

PubMed

Vyas, Raj M; Jarrahy, Reza; Sisodia, Manisha; Jourabchi, Natanel; Wasson, Kristy L; Bradley, James P

2009-05-01

Transverse maxillary hypoplasia, with or without cleft deformity, is typically treated with orthodontic expansion. However, the management of those patients who present later in life with severe uncorrected deformities or who have failed prior orthodontic care remains controversial. Tooth-borne palatal expanders have limitations in this subset of patients with scarred, constricted cleft palate. In this study, we assess the efficacy and safety of using a bone-borne palatal distraction device as an alternative treatment for this difficult subset of patients. Older children with a constricted maxilla who previously had unilateral or bilateral cleft lip and palate repairs and failed orthodontic expansion were included in the study (n = 15). Patients underwent Le Fort I corticotomy with placement of the bone-borne distraction device, expansion at a rate of 0.5 mm/d, and subsequent alveolar bone grafting. Preoperative and follow-up maxillary impressions were compared with assessed improvements in intermolar distance, intercanine distance, alveolar cleft width, and total palatal area. In addition, a small group of noncleft patients with constricted maxillas was treated with bone-borne palatal distraction without a corticotomy (n = 4). The mean amount of distraction in all patients was 14.1 mm, with a follow-up period of 19 months (range, 8-30 months). The average increase in intermolar distance was 8.4 mm, intercanine distance increased by an average of 9.5 mm, and palate surface areas were increased by a mean of 28.9 mm2. Relapse was between 4% and 7%, and all patients underwent subsequent alveolar bone grafting. In addition, the noncleft patients successfully underwent bone-borne palatal distraction without a corticotomy, with a 4% to 5% relapse. Rapid palatal expansion using a bone-borne distraction device in cleft patients with transverse maxillary hypoplasia who have failed nonsurgical orthodontic expansion provides adequate expansion necessary for alveolar bone grafting and stable results.

Speech evaluation after intravelar veloplasty. How to use Borel-Maisonny classification in the international literature?

PubMed

Kadlub, N; Chapuis Vandenbogaerde, C; Joly, A; Neiva, C; Vazquez, M-P; Picard, A

2018-04-01

Comparing functional outcomes after velar repair appeared to be difficult because of the absence of international standardized scale. Moreover most of the studies evaluating speech after cleft surgery present multiple biases. The aim of our study was to assess speech outcomes in a homogeneous group of patients, and to define an equivalence table between different speech scales. Patients with isolated cleft lip and palate (CLP), operated in our unit by the same senior surgeon were included. All patient were operated according to the same protocol (cheilo-rhinoplasty and intravelar veloplasty at 6 months, followed by a direct closure of the hard palate at 15 months). Speech evaluation was performed after 3 year-old and before the alveolar cleft repair. Borel-Maisonny scale and nasometry were used for speech evaluation. Twenty-four patients were included: 17 unilateral CLP and 7 bilateral CLP. According to the Borel-Maisonny classifications, 82.5% were ranged phonation 1, 1-2 or 2b. Nasometry were normal in almost 60% of cases. This study showed the efficiency of our protocol, and intravelar veloplasty. Moreover we proposed an equivalence table for speech evaluation scale. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Behavioral Signs of (Central) Auditory Processing Disorder in Children With Nonsyndromic Cleft Lip and/or Palate: A Parental Questionnaire Approach.

PubMed

Ma, Xiaoran; McPherson, Bradley; Ma, Lian

2016-03-01

Objective Children with nonsyndromic cleft lip and/or palate often have a high prevalence of middle ear dysfunction. However, there are also indications that they may have a higher prevalence of (central) auditory processing disorder. This study used Fisher's Auditory Problems Checklist for caregivers to determine whether children with nonsyndromic cleft lip and/or palate have potentially more auditory processing difficulties compared with craniofacially normal children. Methods Caregivers of 147 school-aged children with nonsyndromic cleft lip and/or palate were recruited for the study. This group was divided into three subgroups: cleft lip, cleft palate, and cleft lip and palate. Caregivers of 60 craniofacially normal children were recruited as a control group. Hearing health tests were conducted to evaluate peripheral hearing. Caregivers of children who passed this assessment battery completed Fisher's Auditory Problems Checklist, which contains 25 questions related to behaviors linked to (central) auditory processing disorder. Results Children with cleft palate showed the lowest scores on the Fisher's Auditory Problems Checklist questionnaire, consistent with a higher index of suspicion for (central) auditory processing disorder. There was a significant difference in the manifestation of (central) auditory processing disorder-linked behaviors between the cleft palate and the control groups. The most common behaviors reported in the nonsyndromic cleft lip and/or palate group were short attention span and reduced learning motivation, along with hearing difficulties in noise. Conclusion A higher occurrence of (central) auditory processing disorder-linked behaviors were found in children with nonsyndromic cleft lip and/or palate, particularly cleft palate. Auditory processing abilities should not be ignored in children with nonsyndromic cleft lip and/or palate, and it is necessary to consider assessment tests for (central) auditory processing disorder when an auditory diagnosis is made for this population.

Orthodontically guided bone transport in the treatment of alveolar cleft: A case report

PubMed Central

Gómez, Elena; Otero, Marta; Berraquero, Rosario; Wucherpfennig, Begona; Hernández-Godoy, Juan; Guiñales, Jorge; Vincent, Germán; Burgueño, Miguel

2016-01-01

Introduction Conventional treatments are sometimes not possible in certain alveolar cleft cases due to the severity of the gap which separates the fragments. Various management strategies have been proposed, including sequential surgical interventions or delaying treatment until adulthood to then carry out maxillary osteotomies. A further alternative approach has also been proposed, involving the application of bone transport techniques to mobilise the osseous fragments and thereby reduce the gap between lateral fragments and the premaxilla. Case Report We introduce the case of a 10-year-old patient who presented with a bilateral alveolar cleft and a severe gap. Stable occlusion between the premaxilla and the mandible was achieved following orthodontic treatment, making it inadvisable to perform a retrusive osteotomy of the premaxilla in order to close the alveolar clefts. Faced with this situation, it was decided we would employ a bone transport technique under orthodontic guidance using a dental splint. This would enable an osseous disc to be displaced towards the medial area and reduce the interfragmentary distance. During a second surgical intervention, closure of the soft tissues was performed and the gap was filled in using autogenous bone. Conclusions The use of bone transport techniques in selected cases allows closure of the osseous defect, whilst also preserving soft tissues and reducing the amount of bone autograft required. In our case, we were able to respect the position of the premaxilla and, at the same time, generate new tissues at both an alveolar bone and soft tissue level with results which have remained stable over the course of time. Key words:Alveolar cleft, bone transport, graft. PMID:26855699

Unilateral cleft nasal deformity correction using conchal cartilage lily flower graft.

PubMed

Hwang, Kun; Kim, Han Joon; Paik, Moo Hyun

2012-11-01

We present a conchal cartilage lily flower graft for correcting depressed and laterally displaced alar cartilage for correction of unilateral cleft nasal deformity.After making a V incision at the base of the columellar and then marginal incisions, the alar cartilages were exposed. A fusiform-shaped cartilage larger than 2.5 cm in length and 1 cm in width was obtained. The midline long axis was scored with a No. 15 knife, and the lateral one third was split. Two-thirds length portions were folded in half, and they became straightened in the shape of a stalk of a lily flower. Two symmetrical one-third length portions were fanned out bilaterally in the shape of the leaf of a lily flower. The stalk portion was positioned in a pocket between the medial crura, and the 2 leaf portions were placed on the dome of the alar cartilages. The marked points of the cleft side and contralateral side were secured with sutures. The V incision at the base of the columellar and the marginal incisions were closed with a V-Y shape. In this technique, the 2 leaf portions were placed on the dome of the alar cartilages and sutured; therefore, the suture holds the dome of the cleft side to the contralateral side without peaking.Thirteen patients (6 male and 7 female subjects; age range, 13-30 years) were operated. Among them, 6 patients were very satisfied, and 5 patients were satisfied with the results. Two patients felt they were improved.We think the conchal cartilage lily flower graft might be a good method for correction of depressed and laterally displaced alar cartilage in unilateral cleft nasal deformity.

Maxillary reaction patterns identified by three-dimensional analysis of casts from infants with unilateral cleft lip and palate.

PubMed

Neuschulz, J; Schaefer, I; Scheer, M; Christ, H; Braumann, B

2013-07-01

In order to visualize and quantify the direction and extent of morphological upper-jaw changes in infants with unilateral cleft lip and palate (UCLP) during early orthodontic treatment, a three-dimensional method of cast analysis for routine application was developed. In the present investigation, this method was used to identify reaction patterns associated with specific cleft forms. The study included a cast series reflecting the upper-jaw situations of 46 infants with complete (n=27) or incomplete (n=19) UCLP during week 1 and months 3, 6, and 12 of life. Three-dimensional datasets were acquired and visualized with scanning software (DigiModel®; OrthoProof, The Netherlands). Following interactive identification of landmarks on the digitized surface relief, a defined set of representative linear parameters were three-dimensionally measured. At the same time, the three-dimensional surfaces of one patient series were superimposed based on a defined reference plane. Morphometric differences were statistically analyzed. Thanks to the user-friendly software, all landmarks could be identified quickly and reproducibly, thus, allowing for simultaneous three-dimensional measurement of all defined parameters. The measured values revealed that significant morphometric differences were present in all three planes of space between the two patient groups. Patients with complete UCLP underwent significantly larger reductions in cleft width (p<0.001), and sagittal growth in the complete UCLP group exceeded sagittal growth in the incomplete UCLP group by almost 50% within the first year of life. Based on patients with incomplete versus complete UCLP, different reaction patterns were identified that depended not on apparent severities of malformation but on cleft forms.

Computer-Assisted Orthognathic Surgery for Patients with Cleft Lip/Palate: From Traditional Planning to Three-Dimensional Surgical Simulation

PubMed Central

Lonic, Daniel; Pai, Betty Chien-Jung; Yamaguchi, Kazuaki; Chortrakarnkij, Peerasak; Lin, Hsiu-Hsia; Lo, Lun-Jou

2016-01-01

Background Although conventional two-dimensional (2D) methods for orthognathic surgery planning are still popular, the use of three-dimensional (3D) simulation is steadily increasing. In facial asymmetry cases such as in cleft lip/palate patients, the additional information can dramatically improve planning accuracy and outcome. The purpose of this study is to investigate which parameters are changed most frequently in transferring a traditional 2D plan to 3D simulation, and what planning parameters can be better adjusted by this method. Patients and Methods This prospective study enrolled 30 consecutive patients with cleft lip and/or cleft palate (mean age 18.6±2.9 years, range 15 to 32 years). All patients received two-jaw single-splint orthognathic surgery. 2D orthodontic surgery plans were transferred into a 3D setting. Severe bony collisions in the ramus area after 2D plan transfer were noted. The position of the maxillo-mandibular complex was evaluated and eventually adjusted. Position changes of roll, midline, pitch, yaw, genioplasty and their frequency within the patient group were recorded as an alternation of the initial 2D plan. Patients were divided in groups of no change from the original 2D plan and changes in one, two, three and four of the aforementioned parameters as well as subgroups of unilateral, bilateral cleft lip/palate and isolated cleft palate cases. Postoperative OQLQ scores were obtained for 20 patients who finished orthodontic treatment. Results 83.3% of 2D plans were modified, mostly concerning yaw (63.3%) and midline (36.7%) adjustments. Yaw adjustments had the highest mean values in total and in all subgroups. Severe bony collisions as a result of 2D planning were seen in 46.7% of patients. Possible asymmetry was regularly foreseen and corrected in the 3D simulation. Conclusion Based on our findings, 3D simulation renders important information for accurate planning in complex cleft lip/palate cases involving facial asymmetry that is regularly missed in conventional 2D planning. PMID:27002726

Computer-Assisted Orthognathic Surgery for Patients with Cleft Lip/Palate: From Traditional Planning to Three-Dimensional Surgical Simulation.

PubMed

Lonic, Daniel; Pai, Betty Chien-Jung; Yamaguchi, Kazuaki; Chortrakarnkij, Peerasak; Lin, Hsiu-Hsia; Lo, Lun-Jou

2016-01-01

Although conventional two-dimensional (2D) methods for orthognathic surgery planning are still popular, the use of three-dimensional (3D) simulation is steadily increasing. In facial asymmetry cases such as in cleft lip/palate patients, the additional information can dramatically improve planning accuracy and outcome. The purpose of this study is to investigate which parameters are changed most frequently in transferring a traditional 2D plan to 3D simulation, and what planning parameters can be better adjusted by this method. This prospective study enrolled 30 consecutive patients with cleft lip and/or cleft palate (mean age 18.6±2.9 years, range 15 to 32 years). All patients received two-jaw single-splint orthognathic surgery. 2D orthodontic surgery plans were transferred into a 3D setting. Severe bony collisions in the ramus area after 2D plan transfer were noted. The position of the maxillo-mandibular complex was evaluated and eventually adjusted. Position changes of roll, midline, pitch, yaw, genioplasty and their frequency within the patient group were recorded as an alternation of the initial 2D plan. Patients were divided in groups of no change from the original 2D plan and changes in one, two, three and four of the aforementioned parameters as well as subgroups of unilateral, bilateral cleft lip/palate and isolated cleft palate cases. Postoperative OQLQ scores were obtained for 20 patients who finished orthodontic treatment. 83.3% of 2D plans were modified, mostly concerning yaw (63.3%) and midline (36.7%) adjustments. Yaw adjustments had the highest mean values in total and in all subgroups. Severe bony collisions as a result of 2D planning were seen in 46.7% of patients. Possible asymmetry was regularly foreseen and corrected in the 3D simulation. Based on our findings, 3D simulation renders important information for accurate planning in complex cleft lip/palate cases involving facial asymmetry that is regularly missed in conventional 2D planning.

Cleft Palate Fistula Closure Utilizing Acellular Dermal Matrix.

PubMed

Emodi, Omri; Ginini, Jiriys George; van Aalst, John A; Shilo, Dekel; Naddaf, Raja; Aizenbud, Dror; Rachmiel, Adi

2018-03-01

Fistulas represent failure of cleft palate repair. Secondary and tertiary fistula repair is challenging, with high recurrence rates. In the present retrospective study, we review the efficacy of using acellular dermal matrix as an interposition layer for cleft palate fistula closure in 20 consecutive patients between 2013 and 2016. Complete fistula closure was obtained in 16 patients; 1 patient had asymptomatic recurrent fistula; 2 patients had partial closure with reduction of fistula size and minimal nasal regurgitation; 1 patient developed a recurrent fistula without changes in symptoms (success rate of 85%). We conclude that utilizing acellular dermal matrix for cleft palate fistula repair is safe and simple with a high success rate.

Cleft Palate Fistula Closure Utilizing Acellular Dermal Matrix

PubMed Central

Emodi, Omri; van Aalst, John A.; Shilo, Dekel; Naddaf, Raja; Aizenbud, Dror; Rachmiel, Adi

2018-01-01

Summary: Fistulas represent failure of cleft palate repair. Secondary and tertiary fistula repair is challenging, with high recurrence rates. In the present retrospective study, we review the efficacy of using acellular dermal matrix as an interposition layer for cleft palate fistula closure in 20 consecutive patients between 2013 and 2016. Complete fistula closure was obtained in 16 patients; 1 patient had asymptomatic recurrent fistula; 2 patients had partial closure with reduction of fistula size and minimal nasal regurgitation; 1 patient developed a recurrent fistula without changes in symptoms (success rate of 85%). We conclude that utilizing acellular dermal matrix for cleft palate fistula repair is safe and simple with a high success rate. PMID:29707449

Fryns anophthalmia-plus syndrome in an 18-week-old fetus.

PubMed

Jayasinghe, Caren; Gembruch, Ulrich; Kuchelmeister, Klaus; Körber, Friederike; Müller, Annette M

2012-01-01

Fryns anophthalmia-plus syndrome is a very rare condition initially described by Fryns and colleagues in 1995 in a pair of siblings of nonconsanguineous parents. Since that time, only a few cases have been reported, most of them in newborns and young children. Clinical presentation is variable and includes anophthalmia/microphthalmia, cleft lip/palate, and other facial deformities. Furthermore, skeletal, central nervous system, and endocrine anomalies have been described. We report the case of a male fetus of 18 weeks of gestation with normal karyotype and findings matching Fryns anophthalmia-plus syndrome. Pregnancy was terminated because of sonographically proven facial midline defects and a marked cerebral ventriculomegaly. Macroscopic and histological findings obtained at autopsy showed extreme bilateral microphthalmia, unilateral cleft palate, unilateral nasal deformity, and low-set ears. Skeletal anomalies included 13 pairs of ribs, premature ossification of the calcaneus, and talipes.

Soft tissue nasal asymmetry as an indicator of orofacial cleft predisposition.

PubMed

Zhang, Charles; Miller, Steven F; Roosenboom, Jasmien; Wehby, George L; Moreno Uribe, Lina M; Hecht, Jacqueline T; Deleyiannis, Frederic W B; Christensen, Kaare; Marazita, Mary L; Weinberg, Seth M

2018-06-01

The biological relatives of offspring with nonsyndromic orofacial clefts have been shown to exhibit distinctive facial features, including excess asymmetry, which are hypothesized to indicate the presence of genetic risk factors. The significance of excess soft tissue nasal asymmetry in at-risk relatives is unclear and was examined in the present study. Our sample included 164 unaffected parents from families with a history of orofacial clefting and 243 adult controls. Geometric morphometric methods were used to analyze the coordinates of 15 nasal landmarks collected from three-dimensional facial surface images. Following generalized Procrustes analysis, Procrustes ANOVA and MANOVA tests were applied to determine the type and magnitude of nasal asymmetry present in each group. Group differences in mean nasal asymmetry were also assessed via permutation testing. We found that nasal asymmetry in both parents and controls was directional in nature, although the magnitude of the asymmetry was greater in parents. This was confirmed with permutation testing, where the mean nasal asymmetry was significantly different (p < .0001) between parents and controls. The asymmetry was greatest for midline structures and the nostrils. When subsets of parents were subsequently analyzed and compared (parents with bilateral vs. unilateral offspring; parents with left vs. right unilateral offspring), each group showed a similar pattern of asymmetry and could not be distinguished statistically. Thus, the side of the unilateral cleft (right vs. left) in offspring was not associated with the direction of the nasal asymmetry in parents. © 2018 Wiley Periodicals, Inc.

#Cleft: The use of Social Media Amongst Parents of Infants with Clefts.

PubMed

Khouri, Joseph S; McCheyne, Melisande J; Morrison, Clinton S

2018-01-01

Introduction Many societies and organizations are using social media to reach their target audience. The extent to which parents of patients with craniofacial anomalies use social media has yet to be determined. The goal of this study is to characterize and describe the use of social media by the parents of children with cleft lip and palate as it pertains to the care of their child. Materials and Methods Parents or guardian of all patients presenting for initial consultation regarding a child's congenital cleft anomaly were contacted by phone or mail to complete a survey regarding their use of social media vis-à-vis their child's cleft anomaly. Participants were asked to answer a 19-question survey. Results Thirty-two families were contacted and 25 surveys were completed. Ninety-two percent of respondents used social media to learn about their child's diagnosis. Facebook (76%) and blogs (24%) were the most commonly accessed social media outlets, followed by Instagram (8%). Education about the diagnosis and treatment of cleft pathology (87%) was the most common reason for accessing social media, followed by companionship and support (56%), and advice about perioperative care (52%). Almost half (43%) of parents used social media to obtain information on their caregiver and treatment team, and 26% of parents used information gained on social media to guide their decision on where to seek care. Conclusion Social media is a readily available resource, one that will certainly shape the experiences of our patients and families for years to come.

Esthetic composition of smile in individuals with cleft lip, alveolus, and palate: visibility of the periodontium and the esthetics of smile.

PubMed

Esper, Luis Augusto; Sbrana, Michyele Cristhiane; Cunha, Mércia Jussara da Silva; Moreira, Guilherme Santos; de Almeida, Ana Lúcia Pompéia Fraga

2012-01-01

Objective. To evaluate characteristics of smile related to visibility in individuals with cleft lip, alveolus, and palate. Design. Cross-sectional. Setting. HRAC/USP, Brazil. Patients. Individuals with repaired complete unilateral cleft lip and palate (n = 45), aged 15-30 years. Interventions. Frontal facial photographs were obtained in natural and forced smiles (n = 135). Six specialists in periodontics evaluated the photographs as to the smile line, thickness, and curve of the upper lip. Main Outcome Measures. The cleft area was compared with the contralateral region. Results were expressed as percentages and means. The findings were compared between groups of periodontists. Results. Statistically significant relationship was observed in the smile line between examiners and between natural and forced smiles, regardless of the association with the cleft side. The lip was thicker at rest and thinner in the forced smile, as also evaluated by the group not experienced with cleft care. The curve of the upper lip in natural and forced smiles was considered as close to straight by both groups, regardless of the cleft. Conclusion. The smile in individuals with clefts was regarded as average for both cleft and noncleft sides. The thickness was characterized as average to thin, being thinner in forced smile and when analyzed by the group not experienced with cleft care. In the average, the curve of the upper lip was considered as straight. The present study elucidates some characteristics related to the smile in individuals with repaired unilateral cleft lip, alveolus, and palate.

Esthetic Composition of Smile in Individuals with Cleft Lip, Alveolus, and Palate: Visibility of the Periodontium and the Esthetics of Smile

PubMed Central

Esper, Luis Augusto; Sbrana, Michyele Cristhiane; Cunha, Mércia Jussara da Silva; Moreira, Guilherme Santos; de Almeida, Ana Lúcia Pompéia Fraga

2012-01-01

Objective. To evaluate characteristics of smile related to visibility in individuals with cleft lip, alveolus, and palate. Design. Cross-sectional. Setting. HRAC/USP, Brazil. Patients. Individuals with repaired complete unilateral cleft lip and palate (n = 45), aged 15–30 years. Interventions. Frontal facial photographs were obtained in natural and forced smiles (n = 135). Six specialists in periodontics evaluated the photographs as to the smile line, thickness, and curve of the upper lip. Main Outcome Measures. The cleft area was compared with the contralateral region. Results were expressed as percentages and means. The findings were compared between groups of periodontists. Results. Statistically significant relationship was observed in the smile line between examiners and between natural and forced smiles, regardless of the association with the cleft side. The lip was thicker at rest and thinner in the forced smile, as also evaluated by the group not experienced with cleft care. The curve of the upper lip in natural and forced smiles was considered as close to straight by both groups, regardless of the cleft. Conclusion. The smile in individuals with clefts was regarded as average for both cleft and noncleft sides. The thickness was characterized as average to thin, being thinner in forced smile and when analyzed by the group not experienced with cleft care. In the average, the curve of the upper lip was considered as straight. The present study elucidates some characteristics related to the smile in individuals with repaired unilateral cleft lip, alveolus, and palate. PMID:23227326

The impact of cleft lip and palate repair on maxillofacial growth

PubMed Central

Shi, Bing; Losee, Joseph E

2015-01-01

Surgical correction is central to current team-approached cleft treatment. Cleft surgeons are always concerned about the impact of their surgical maneuver on the growth of the maxilla. Hypoplastic maxilla, concaved mid-face and deformed dental arch have constantly been reported after cleft treatments. It is very hard to completely circumvent these postoperative complications by current surgical protocols. In this paper, we discussed the factors that inhibit the maxillofacial growth on cleft patients. These factors included pre-surgical intervention, the timing of cleft palate and alveolae repair, surgical design and treatment protocol. Also, we made a review about the influence on the maxillary growth in un-operated cleft patients. On the basis of previous researches, we can conclude that most of scholars express identity of views in these aspects: early palatoplasty lead to maxilla growth inhibition in all dimensions; secondary alveolar bone graft had no influence on maxilla sagittal growth; cleft lip repair inhibited maxilla sagittal length in patients with cleft lip and palate; Veau's pushback palatoplasty and Langenbeck's palatoplasty with relaxing incisions were most detrimental to growth; Furlow palatoplasty showed little detrimental effect on maxilla growth; timing of hard palate closure, instead of the sequence of hard or soft palate repair, determined the postoperative growth. Still, scholars hold controversial viewpoints in some issues, for example, un-operated clefts have normal growth potential or not, pre-surgical intervention and pharyngoplasty inhibited maxillofacial growth or not. PMID:25394591

[Dental anomalies in Chinese children with complete unilateral cleft lip and palate].

PubMed

Fan, Xin-xin; Li, Jing; Ge, Li-hong; Ma, Lian

2011-05-01

To investigate the prevalence of dental anomalies in Chinese children with complete unilateral cleft lip and palate (UCLP). Dental histories and radiographs of 244 Chinese children with UCLP were collected. The diagnosis of dental anomalies was based on panoramic radiographs before alveolar bone grafting. All patients were over 8 years old. In the UCLP group, 66.8% of the patients was presented with hypodontia. The maxillary lateral incisors were the most common teeth affected, followed by maxillary second premolars, mandibular incisors and mandibular second premolars. A total of 33.6% the patients was presented with dental malformation, most were microdontic laterel incisors. A total of 4.9% the patients was presented with hyperdontia. The supernumerary teeth were more frequently found in the cleft region. The prevalence of missing maxillary lateral incisor in the noncleft side was statistically different between genders, which was higher in male (P < 0.050). This group of Chinese children with UCLP demonstrated significantly higher prevalence of hypodontia, hyperdontia, and malformation on the cleft side than on the noncleft side (P < 0.01). Hypodontia is the most common type of dental anomalies. The prevalence of dental anomalies is higher in the UCLP patients than in the normal population. Dental anomalies occur more frequently on the cleft side than on the noncleft side.

Differences in the Tensor Veli Palatini Between Adults With and Without Cleft Palate Using High-Resolution 3-Dimensional Magnetic Resonance Imaging.

PubMed

George, Thomas N; Kotlarek, Katelyn J; Kuehn, David P; Sutton, Bradley P; Perry, Jamie L

2018-05-01

To investigate the dimensions of the tensor veli palatini (TVP) muscle in adults with and without cleft palate. Prospective study. There were a total of 14 adult participants, 8 noncleft and 6 with cleft palate. Analysis and comparison of the TVP muscle and surrounding structures was completed using 3D MRI data and Amira 5.5 Visualization Modeling software. TVP muscle volume, hamular process distance, mucosal thickness, TVP muscle length, and TVP muscle diameter were used for comparison between participant groups based upon previous research methods. Mann-Whitney U tests revealed a significantly smaller ( U < .001, P = .002) TVP muscle volume in the cleft palate group (median = 536.22 mm 3 ) compared to individuals in the non-cleft palate group (median = 895.19 mm 3 ). The TVP muscle was also significantly shorter ( U = 1.00, P = .003) in the cleft palate group (median = 18.04 mm) versus the non-cleft palate (median = 21.18 mm). No significant differences were noted for the other measured parameters. Significant differences in the TVP muscle volume and length among the noncleft participants found in this study may insights into the reported increased incidence of otitis media with effusion (OME) seen in the cleft population. Results from this study contribute to our understanding of the underlying anatomic differences among individuals with cleft palate.

The Effect of Cleft Size in Infants With Unilateral Cleft Lip and Palate on Mixed Dentition Dental Arch Relationship.

PubMed

Russell, Lisa M; Long, Ross E; Romberg, Elaine

2015-09-01

To determine the relationship between infant cleft size and dental arch relationship in the mixed dentition in patients with complete unilateral cleft lip and palate. Retrospective analysis of mixed longitudinal records. A total of 29 consecutively enrolled patients with unilateral cleft lip and palate participated in a longitudinal study that included dental casts prior to lip surgery (T1: age 1 month), prior to palate surgery (T2: age 10 months), and in mixed dentition (T3: age 9 years). All infants were managed with lip repair (2.5 months), hard palate repair (12 months), and soft palate repair (16 months) but without any presurgical orthopedic treatment and no orthodontic intervention prior to mixed dentition records. The outcome measures included determination of an infant cleft severity ratio, defined as the ratio of palatal cleft area to palatal surface area, at both T1 and T2, and the 9-year-old (T3) dental arch relationship as determined using the GOSLON Yardstick. The correlation between the infant cleft severity ratio at T1 and T2 and the later GOSLON Yardstick score at T3 was determined using Pearson r. The intrarater reliability of the infant cleft severity ratio was assessed with Pearson r and the interrater reliability of the GOSLON Yardstick ratings, by weighted kappa. Reliability for the infant cleft severity ratio method was r = .92 to .95, and for GOSLON ratings κ = .81 to .91. There was no significant correlation between 1-month infant cleft severity ratio and GOSLON (r = .3) and 10-month infant cleft severity ratio and GOSLON (r = .1). Cleft size versus the amount of palatal tissue available for repair and concern over more scarring with a greater infant cleft severity ratio were not factors in affecting the eventual dental arch relationship.

Coping With Cleft: A Conceptual Framework of Caregiver Responses to Nasoalveolar Molding

PubMed Central

Sischo, Lacey; Broder, Hillary L.; Phillips, Ceib

2014-01-01

Objective To present a conceptual framework of caregiver coping and adaptation to early cleft care using nasoalveolar molding. Design In-depth interviews were conducted at three time points with caregivers of infants with cleft lip or cleft lip and palate whose children had nasoalveolar molding to treat their cleft. Qualitative data were analyzed using modified grounded theory. Results Most caregivers expressed initial apprehension and anxiety about the responsibilities of care associated with nasoalveolar molding (e.g., changing and positioning tapes, cleaning the appliance). In subsequent interviews, caregivers often reported positive feelings related to their active participation in their child’s treatment for cleft. These positive feelings were associated with increased self-esteem and feelings of empowerment for the caregivers. Although caregivers also identified burdens associated with nasoalveolar molding (e.g., stress related to lip taping, concerns about the appliance causing sores in their child’s mouth, travel to weekly appointments), they tended to minimize the impact of these issues in comparison with the perceived benefits of nasoalveolar molding. Conclusions Despite the increased burden of care, many caregivers of infants with cleft used nasoalveolar molding as a problem-focused coping strategy to deal with their child’s cleft. Completing nasoalveolar molding was often associated with positive factors such as increased empowerment, self-esteem, and bonding with their infant. PMID:25225840

Coping With Cleft: A Conceptual Framework of Caregiver Responses to Nasoalveolar Molding.

PubMed

Sischo, Lacey; Broder, Hillary L; Phillips, Ceib

2015-11-01

To present a conceptual framework of caregiver coping and adaptation to early cleft care using nasoalveolar molding. In-depth interviews were conducted at three time points with caregivers of infants with cleft lip or cleft lip and palate whose children had nasoalveolar molding to treat their cleft. Qualitative data were analyzed using modified grounded theory. Most caregivers expressed initial apprehension and anxiety about the responsibilities of care associated with nasoalveolar molding (e.g., changing and positioning tapes, cleaning the appliance). In subsequent interviews, caregivers often reported positive feelings related to their active participation in their child's treatment for cleft. These positive feelings were associated with increased self-esteem and feelings of empowerment for the caregivers. Although caregivers also identified burdens associated with nasoalveolar molding (e.g., stress related to lip taping, concerns about the appliance causing sores in their child's mouth, travel to weekly appointments), they tended to minimize the impact of these issues in comparison with the perceived benefits of nasoalveolar molding. Despite the increased burden of care, many caregivers of infants with cleft used nasoalveolar molding as a problem-focused coping strategy to deal with their child's cleft. Completing nasoalveolar molding was often associated with positive factors such as increased empowerment, self-esteem, and bonding with their infant.

Complex Correction of Complete Cleft Lip with Severe Prominent Premaxilla using Lip Adhesion and Nasoalveolar Molding Device.

PubMed

Seo, Bin Na; Park, Su Han; Yang, Jeong Yeol; Son, Kyung Min; Cheon, Ji Seon

2015-04-01

Nasoalveolar molding (NAM) device is an effective treatment for protruding maxilla in infants with cleft palate. However, only a few studies have investigated the effect of NAM devices on the treatment of protruding maxilla in infants with cleft lip only. We have designed a combination treatment using NAM devices prior to cheiloplasy for cleft lip-only patients with severe anterior protrusion of the premaxilla. Three cleft lip-only infants with 1-cm or more of premaxilla protrusion were included. Definitive cheiloplasty was performed at 6 months of age without any preoperative correction in infant 1. Cheiloplasty was performed in conjunction with the use of NAM device and lip adhesion in infants 2 and 3. Postoperative columella length and anterior-posterior dimension of the protruding premaxilla were compared amongst the infants. We were able to obtain satisfactory postoperative columella length and general nasal appearance.

A Type I first branchial cleft cyst masquerading as a parotid tumor.

PubMed

Krishnamurthy, Arvind; Ramshanker, Vijayalakshmi

2014-01-01

Branchial cleft anomalies are caused by incomplete regression of the cervical sinus of "His" during the 6(th) and 7(th) weeks of embryologic development. Although congenital in origin, first branchial cleft cysts (FBCCs) can present later in life. FBCCs are rare causes of parotid swellings, accounting for <1% of all the branchial cleft abnormalities. The diagnosis of FBCCs is a clinical challenge; the condition is often overlooked and mismanaged. We report a case of Type 1 FBCC in a 22-year-old female with an asymptomatic 3.5 cm × 2.5 cm sized cystic mass. It was removed completely under the impression of a cystic tumor of the parotid. On histopathology, the cyst had a squamous epithelium-lined wall with lymphoid aggregation which was characteristic of a branchial cleft cyst. A good understanding of the regional anatomy and embryology can lead to an early diagnosis and thereby effective management of FBCC.

Speech and phonology in Swedish-speaking 3-year-olds with unilateral complete cleft lip and palate following different methods for primary palatal surgery.

PubMed

Klintö, Kristina; Svensson, Henry; Elander, Anna; Lohmander, Anette

2014-05-01

Objective : To describe and compare speech and phonology at age 3 years in children born with unilateral complete cleft lip and palate treated with three different methods for primary palatal surgery. Design : Prospective study. Setting : Primary care university hospitals. Participants : Twenty-eight Swedish-speaking children born with nonsyndromic unilateral complete cleft lip and palate. Interventions : Three methods for primary palatal surgery: two-stage closure with soft palate closure between 3.4 and 6.4 months and hard palate closure at mean age 12.3 months (n = 9) or 36.2 months (n = 9) or one-stage closure at mean age 13.6 months (n = 10). Main Outcome Measures : Based on independent judgments performed by two speech-language pathologists from standardized video recordings: percent correct consonants adjusted for age, percent active cleft speech characteristics, total number of phonological processes, number of different phonological processes, hypernasality, and audible nasal air leakage. The hard palate was unrepaired in nine of the children treated with two-stage closure. Results : The group treated with one-stage closure showed significantly better results than the group with an unoperated hard palate regarding percent active cleft speech characteristics and total number of phonological processes. Conclusions : Early primary palatal surgery in one or two stages did not result in any significant differences in speech production at age 3 years. However, children with an unoperated hard palate had significantly poorer speech and phonology than peers who had been treated with one-stage palatal closure at about 13 months of age.

Combined approach branchial sinusectomy: a new technique for excision of second branchial cleft sinus.

PubMed

Olusesi, A D

2009-10-01

Branchial cleft anomalies are well described, with the second arch anomaly being the commonest. Following surgical excision, recurrence occurs in 2 to 22 per cent of cases, and is believed to be due largely to incomplete resection. This report aims to describe a simple surgical technique for treatment of second branchial cleft sinus in the older paediatric age group and adults. An 11-year-old girl underwent surgical excision of a second branchial sinus. Prior to surgery, she was assessed by means of an imaging sonogram, and by direct methylene blue dye injection into the sinus on the operating table, followed by insertion of a metallic probe. Dissection was of the 'step ladder' incision type, but the incision was completed via an oropharyngeal approach. Histological examination of the lesion after excision established the diagnosis. No recurrence had been observed at the time of writing. Although they are congenital lesions, second branchial cleft abnormalities usually present in the older paediatric age group or even in adulthood. In the case reported, a simple combined approach ensured completeness of resection.

Type II first branchial cleft anomaly.

PubMed

Al-Mahdi, Akmam H; Al-Khurri, Luay E; Atto, Ghada Z; Dhaher, Ameer

2013-01-01

First branchial cleft anomaly is a rare disease of the head and neck. It accounts for less than 8% of all branchial abnormalities. It is classified into type I, which is thought to arise from the duplication of the membranous external ear canal and are composed of ectoderm only, and type II that have ectoderm and mesoderm. Because of its rarity, first branchial cleft anomaly is often misdiagnosed and results in inappropriate management. A 9-year-old girl presented to us with fistula in the submandibular region and discharge in the external ear. Under general anesthesia, complete surgical excision of the fistula tract was done through step-ladder approach, and the histopathologic examination confirmed the diagnosis of type II first branchial cleft anomaly.

A Comparative Cost Analysis of Cleft Lip Adhesion and Nasoalveolar Molding before Formal Cleft Lip Repair.

PubMed

Shay, Paul L; Goldstein, Jesse A; Paliga, J Thomas; Wink, Jason; Jackson, Oksana A; Low, David; Bartlett, Scott P; Taylor, Jesse A

2015-12-01

Patients with complete cleft lip and palate may benefit from cleft lip adhesion or nasoalveolar molding before formal cleft lip repair. The authors compared the relative costs to insurers of these two treatment modalities and the burden of care to families. A retrospective analysis was performed of cleft lip and palate patients treated with nasoalveolar molding or cleft lip adhesion at The Children's Hospital of Philadelphia between January of 2007 and June of 2012. Demographic, appointment, and surgical data were reviewed; surgical, inpatient hospital, and orthodontic charges and costs were obtained. Multivariate linear regression and two-sample, two-tailed independent t tests were performed to compare cost and appointment data between groups. Forty-two cleft adhesion and 35 nasoalveolar molding patients met inclusion criteria. Mean costs for nasoalveolar molding were $3550.24 ± $667.27. Cleft adhesion costs, consisting of both hospital and surgical costs, were $9370.55 ± $1691.79. Analysis of log costs demonstrated a significant difference between the groups, with the mean total cost for nasoalveolar molding significantly lower than that for adhesion (p < 0.0001). Nasoalveolar molding patients had significantly more made, cancelled, no-show, and missed visits and a higher missed percentage than adhesion patients (p < 0.0001) for all except no-show appointments, (p = 0.0199), indicating a higher burden of care to families. Nasoalveolar molding may cost less before formal cleft lip repair treatment than cleft lip adhesion. Third-party payers who cover adhesion and not nasoalveolar molding may not be acting in their own best interest. Nasoalveolar molding places a higher burden of care on families, and this fact should be considered in planning treatment.

Three-dimensional morphologic nasal surface characteristics that predict the extremes of esthetics in patients with repaired cleft lip and palate.

PubMed

Russell, Kathy A; Milne, Andrew D; Varma, Devesh; Josephson, Keith; Lee, J Michael

2011-01-01

The purposes of this study were (1) to develop imaging methods and objective numeric parameters to describe nose morphology, and (2) to correlate those parameters with nasal esthetics for patients with clefts. A total of 28 patients with repaired complete unilateral cleft lip and palate (CUCLP) and 20 age- and gender-matched individuals without clefts were identified. A panel of orthodontists rated and ranked nasal esthetics from nose casts for the cleft group. Best and worst esthetic cleft groups were established from the cast assessments. Three-dimensional surface coordinates of the casts were digitally mapped with an electromagnetic tracking device. Digitized nasal images were oriented, voxelated, sliced, and mathematically curve-fitted. Maximum difference, percent area difference, and maximum and minimum derivative differences between cleft and noncleft and between right and left nose sides were calculated. Differences in parameters between groups were assessed with the use of analysis of variance (ANOVA) and t tests, and correlations with esthetics were assessed with the Spearman rank correlation test. Differences were seen between cleft and noncleft and best and worst esthetic groups for all four parameters (p < .05). The best esthetic cleft group had (1) lower percent area difference (p < .0001), (2) lower maximum difference (p < .001), and (3) smaller differences in slope of the nose in the coronal plane (p < .0001) than the worst esthetic cleft group. Maximum difference and maximum derivative difference and, to a lesser degree, percent area difference can be used to identify differences between cleft and noncleft nasal morphology and to assess levels of nasal esthetics for patients with CUCLP.

Branchial Cleft Anomalies

PubMed Central

McPhail, Neil; Mustard, Robert A.

1966-01-01

The embryology, anatomy and pathology of branchial cleft anomalies are discussed and 87 cases reviewed. The most frequent anomaly was branchial cleft cyst, of which there were 77 cases. Treatment in all cases consisted of complete excision. There were five cases of external branchial sinus and five cases of complete branchial fistula. Sinograms were helpful in demonstrating these lesions. Excision presented little difficulty. No proved case of branchiogenic carcinoma has been found in the Toronto General Hospital. Five cases are described in which the original diagnosis was branchiogenic carcinoma—in four of these a primary tumour has already been found. The authors believe that the diagnosis of branchiogenic carcinoma should never be accepted until repeated examinations over a period of at least five years have failed to reveal a primary tumour. ImagesFig. 1Fig. 2Fig. 3Fig. 4Fig. 5 PMID:5901161

The Role of Smile Train and the Partner Hospital Model in Surgical Safety, Collaboration, and Quality in the Developing World.

PubMed

Purnell, Chad A; McGrath, Jennifer L; Gosain, Arun K

2015-06-01

The partner hospital model identifies hospitals in the developing world to educate and enable local surgeons to deliver effective cleft care. This study aimed to determine the outcomes of this model on safety, education, and quality of surgical care. Twelve partner hospitals, sponsored by Smile Train for 5 or more years and distributed over 4 continents, were selected. Activities at each institution were evaluated using cleft surgical data, and surveys were completed by hospital leadership. A mean of 82% of cleft patients at partner hospitals underwent sponsored surgeries. After partnership, all 12 hospitals implemented preoperative checklists for cleft surgery, and 5 implemented checklists for other surgeries. All hospitals had personnel who received safety training as a result of partnership. There was no change in 30-day reoperations or readmissions. Follow-up rate increased by 18% (P = 0.03). Facilities recruited 1.8 additional cleft surgeons (P < 0.01) and increased the number of cleft surgical trainees by a mean of 13.3 annually (P = 0.012); 2.5 ± 1.7 additional ancillary services were added, resulting in 75% of partner hospitals having a basic multidisciplinary cleft team (surgery, speech, and dental) compared with 25% prior to partnership (P < 0.01). Total cleft surgeries, alveolar bone grafts, and percentage of secondary surgeries increased significantly as length of partnership progressed (P < 0.01). Smile Train's partner hospital model increases both the volume and quality of cleft care delivered at these institutions. Safety initiatives for cleft care demonstrate effects extending to global surgical care delivered at partner hospitals.

Oral health-related quality of life in Dutch children with cleft lip and/or palate.

PubMed

Bos, Annemieke; Prahl, Charlotte

2011-09-01

To investigate the oral health-related quality of life (OH-RQoL) of Dutch cleft lip and palate patients. It was hypothesized that (1) there is no difference between cleft patients' and their parents' reports of patients' OH-RQoL; (2) there are no gender differences; (3) there are no differences in OH-RQoL between cleft patients with regard to their symptoms; and (4) there is no difference between patients above and below 12 years of age. The sample consisted of 122 patients with clefts (age range, 8-15 years) and their parents. Respondents were recruited from the cleft palate team of Amsterdam. They completed the Child Oral Health-Related Quality of Life questionnaire (COHIP). Items were divided into five different subscales, and scores on all subscales were compared between and within groups. Patients' and parents' perceptions differed significantly on three of the five subscales. Girls and boys did not differ significantly with regard to their perception of reported OH-RQoL. The cleft lip and cleft lip and alveolus [CL(A)] subgroup scored significantly higher on the functional well-being subscale. The cleft patients aged 12 years and older scored significantly lower on the emotional well-being and oral symptoms subscales when compared with their younger peers. Only the second hypothesis was not rejected in this study. This means that parents are not interchangeable with regard to reporting on their children's perceptions related to OH-RQoL, that OH-RQoL changes with age, and that it is important that subgroups are respected when investigating OH-RQoL in cleft patients.

Nasal Obstruction in Children With Cleft Lip and Palate: Results of a Cross-Sectional Study Utilizing the NOSE Scale.

PubMed

Zhang, Rosaline S; Lin, Lawrence O; Hoppe, Ian C; Jackson, Oksana A; Low, David W; Bartlett, Scott P; Swanson, Jordan W; Taylor, Jesse A

2018-01-01

To characterize the epidemiology and risk factors for nasal obstruction among subjects with cleft lip and/or cleft palate (CL/P) utilizing the well-validated Nasal Obstruction Symptom Evaluation (NOSE) survey. Retrospective cross-sectional study. Cleft Lip and Palate Program, Children's Hospital of Philadelphia. Patients, Subjects: One thousand twenty-eight surveys obtained from 456 subjects (mean age: 10.10 (4.48) years) with CL/P evaluated between January 2015 and August 2017 with at least 1 completed NOSE survey. Nasal Obstruction Symptom Evaluation surveys completed at each annual visit. Composite NOSE and individual symptom scores. Sixty-seven percent of subjects had nasal obstruction at some point during the study period, with 49% reporting nasal obstruction at latest follow-up. subjects aged 14 years and older reported the most severe symptoms ( P = .002). Subjects with cleft lip and alveolus (CL+A) and unilateral cleft lip and palate (CLP) reported more severe nasal blockage than other phenotypes ( P = .021). subjects with a history of either posterior pharyngeal flap (PPF) or sphincter pharyngoplasty (SP) had significantly higher NOSE scores than subjects with no history of speech surgery ( P = .006). There was no significant difference ( P > .050) in NOSE scores with regard to history of primary tip rhinoplasty, nasal stent use, or nasoalveolar molding. There are more severe nasal obstructive symptoms among subjects older than 14 years of age, with CL+A or unilateral CLP, and with a history of PPF or SP. Future studies utilizing the NOSE are needed to evaluate and address this prevalent morbidity in the CLP population.

Candidate Gene/Loci Studies in Cleft Lip/Palate and Dental Anomalies Finds Novel Susceptibility Genes for Clefts

PubMed Central

Vieira, Alexandre R.; McHenry, Toby G.; Daack-Hirsch, Sandra; Murray, Jeffrey C.; Marazita, Mary L.

2009-01-01

We revisited 42 families with two or more cleft affected siblings that participated in previous studies and collected complete dental information. Genotypes from 1489 single nucleotide polymorphism (SNP) markers located in 150 candidate genes/loci were reanalyzed. Two sets of association analyses were carried out. First we ran the analysis solely on the cleft status. Second we assigned affection to any cleft or dental anomaly (tooth agenesis, supernumerary teeth, and microdontia), and repeated the analysis. Significant over-transmission was seen for a SNP in ANKS6 (rs4742741, 9q22.33; p=0.0004) when a dental anomaly phenotype was included in the analysis. Significant over-transmission was also seen for a SNP in ERBB2 (rs1810132, 17q21.1; p=0.0006). In the clefts only data, the most significant result was also for ERBB2 (p=0.0006). Other markers with suggestive p-values included IRF6 and 6q21-q23 loci. In contrast to the above results, suggestive over-transmission of markers in GART, DPF3, and NRXN3 were seen only when the dental anomaly phenotype was included in the analysis. These findings support the hypothesis that some loci may contribute to both clefts and congenital dental anomalies. Thus, including dental anomalies information in the genetics analysis of cleft lip and palate will provide new opportunities to map susceptibility loci for clefts. PMID:18978678

[Psychosocial adjustment in children with a cleft lip and/or palate].

PubMed

Hoek, Ineke H C; Kraaimaat, Floris W; Admiraal, Ronald J C; Kuijpers-Jagtman, Anne Marie; Verhaak, Christianne M

2009-01-01

To gain insight into the psychosocial health of children aged 9 to 12 years with a cleft lip and/or palate; to determine the relation between their health and the nature and severity of the cleft as well as other individual characteristics. Descriptive, cross-sectional study. Questionnaires completed by parents, teachers and children were used to obtain information about the psychosocial health, nature and severity of the cleft lip and/or palate, and individual characteristics of 80 children. The interrelationship between these parameters was assessed using chi-square tests, single-factor analysis of variance and correlational analysis. In general, the psychosocial health of children with a cleft lip and/or palate did not differ from that of the norm groups. Parents of children with a cleft lip/and or palate reported more withdrawn or depressive behaviour in their child than parents from the norm groups. Children with a cleft lip and/or palate exhibited less rule-breaking behaviour. Teachers reported relatively more social problems. One-third of the children had learning problems. A better psychosocial health was associated with fewer speech problems but not with a more or less abnormal physical appearance. Self-image showed a negative correlation with psychosocial health problems, while learning problems showed a positive correlation. In general, the psychosocial health of children with a cleft lip and/or palate does not differ from children without this condition. However, children with a cleft lip and/or palate do exhibit more learning problems.

Teaching Palatoplasty Using a High-Fidelity Cleft Palate Simulator.

PubMed

Cheng, Homan; Podolsky, Dale J; Fisher, David M; Wong, Karen W; Lorenz, H Peter; Khosla, Rohit K; Drake, James M; Forrest, Christopher R

2018-01-01

Cleft palate repair is a challenging procedure for cleft surgeons to teach. A novel high-fidelity cleft palate simulator has been described for surgeon training. This study evaluates the simulator's effect on surgeon procedural confidence and palatoplasty knowledge among learners. Plastic surgery trainees attended a palatoplasty workshop consisting of a didactic session on cleft palate anatomy and repair followed by a simulation session. Participants completed a procedural confidence questionnaire and palatoplasty knowledge test immediately before and after the workshop. All participants reported significantly higher procedural confidence following the workshop (p < 0.05). Those with cleft palate surgery experience had higher procedural confidence before (p < 0.001) and after (p < 0.001) the session. Palatoplasty knowledge test scores increased in 90 percent of participants. The mean baseline test score was 28 ± 10.89 percent and 43 ± 18.86 percent following the workshop. Those with prior cleft palate experience did not have higher mean baseline test scores than those with no experience (30 percent versus 28 percent; p > 0.05), but did have significantly higher scores after the workshop (61 percent versus 35 percent; p < 0.05). All trainees strongly agreed or agreed that the simulator should be integrated into training and they would use it again. This study demonstrates the effective use of a novel cleft palate simulator as a training tool to teach palatoplasty. Improved procedural confidence and knowledge were observed after a single session, with benefits seen among trainees both with and without previous cleft experience.

Team approach concept in management of oro-facial clefts: a survey of Nigerian practitioners.

PubMed

Akinmoladun, Victor I; Obimakinde, Obitade S

2009-05-10

Cleft palate craniofacial teams have evolved across the globe in the last 20 years in compliance with the interdisciplinary concept of management of oro-facial clefts. An interdisciplinary care allows a coordinated treatment protocol for the patient. The objective of this study was to evaluate oro-facial cleft care in Nigeria with particular emphasis on the compliance of the practitioners to the team approach concept. A snapshot survey was conducted among specialists that attended the Pan African Congress on Cleft Lip and Palate, at the International Institute of Tropical Agriculture, Nigeria in February 2007. Sixty three respondents successfully completed and returned the questionnaire for analysis. Mean age of respondents was 43.5 years and the range was 38-62 years.Male to female ratio was 2.7:1. Oral and Maxillofacial Surgeons and Plastic Surgeons constituted the majority of respondents (38.1% and 22.2%) respectively. Only 47.6% (n = 30) of the specialists belonged to cleft teams. Majority of Oral and Maxillofacial Surgeons and Plastic Surgeons belonged to cleft teams (70% and 63.3% respectively) while speech pathologists and orthodontists were less represented (20% and 36.7% respectively) in teams. Findings from this study suggests that interdisciplinary care for the cleft patient does not appear to have been fully embraced in Nigeria. This may be a result of several reasons ranging from non availability of the requisite specialists, the relatively young age of cleft care practice in this part of the world to the poor state of infrastructure.

Secondary Alveolar Bone Grafting in Patients Born With Unilateral Cleft Lip and Palate: A 20-Year Follow-up.

PubMed

Jabbari, Fatemeh; Wiklander, Laila; Reiser, Erika; Thor, Andreas; Hakelius, Malin; Nowinski, Daniel

2018-02-01

To identify factors of oral health important for the final outcome, after secondary alveolar bone grafting in patients born with unilateral cleft lip and palate and compare occlusal radiographs with cone beam computed tomography (CBCT) in assessment of alveolar bone height. Observational follow-up study. Cleft Lip and Palate Team, Craniofacial Center, Uppsala University Hospital, Sweden. 40 nonsyndromic, Caucasian patients with unilateral complete cleft lip and palate. Clinical examination, CBCT, and occlusal radiographs. Alveolar bone height was evaluated according to Bergland index at a 20-year follow-up. The alveolar bone height in the cleft area was significantly reduced compared to a previously reported 10-year follow-up in the same cohort by total ( P = .045) and by subgroup with dental restoration ( P = .0078). This was positively correlated with the gingival bleeding index (GBI) ( r = 0.51, P = .0008) and presence of dental restorations in the cleft area ( r = 0.45, P = .0170). There was no difference in the Bergland index generated from scoring the alveolar bone height on occlusal radiographs as with the equivalent index on CBCT. Patients rehabilitated with complex dental restoration seems to be at higher risk for progression of bone loss in the cleft area. Supportive periodontal therapy should be implemented after complex dental restorations in cleft patients. Conventional occlusal radiographs provide an adequate image for evaluating postoperative bone height in clinical follow-up.

Assessment of complete unilateral cleft lip and palate treatment outcome using EUROCRAN index and associated factors.

PubMed

Arshad, Anas Imran; Alam, Mohammad Khursheed; Khamis, Mohd Fadhli

2017-09-01

Assessment of treatment outcome is the only non-invasive approach to identify the effects of cleft lip and palate repair and modify management accordingly. Here the aim is to assess the outcome of complete unilateral cleft lip and palate (CUCLP) patients using EUROCRAN index and to check whether there are any factors associated with the treatment outcome. It is a retrospective cross sectional study. Dental models were collected from archives of two cleft referral centers in Pakistan. Five blinded examiners scored 101 models twice at two week interval. The primary outcome was mean EUROCRAN scores based on dental arch relationships and palatal surface morphology. A mean(SD) score of 2.72 (0.76) and 2.20 (0.73) was determined based on dental arch relationships and palatal surface morphology, respectively. According to the final logistic regression model, modified Millard technique (cheiloplasty) and Veau-Wardill-Kilners' method (palatoplasty) had higher odds of producing unfavorable treatment outcome. Present study determined a fair and a fair to poor treatment outcome based on dental arch relationships and palatal surface morphology, respectively. Our study suggests a significant association between treatment outcome and primary surgical techniques for lip and palate. These findings could warrant a modification of management protocols to ensure improvement in future cleft outcomes. Copyright © 2017 Elsevier B.V. All rights reserved.

Prevalence, cause, and location of palatal fistula in operated complete unilateral cleft lip and palate: retrospective study.

PubMed

de Agostino Biella Passos, Vivian; de Carvalho Carrara, Cleide Felício; da Silva Dalben, Gisele; Costa, Beatriz; Gomide, Marcia Ribeiro

2014-03-01

To evaluate the prevalence of fistulas after palate repair and analyze their location and association with possible causal factors. Retrospective analysis of patient records and evaluation of preoperative initial photographs. Tertiary craniofacial center. Five hundred eighty-nine individuals with complete unilateral cleft lip and palate that underwent palate repair at the age of 12 to 36 months by the von Langenbeck technique, in a single stage, by the plastic surgery team of the hospital, from January 2003 to July 2007. The cleft width was visually classified by a single examiner as narrow, regular, or wide. The following regions of the palate were considered for the location: anterior, medium, transition (between hard and soft palate), and soft palate. Descriptive statistics and analysis of association between the occurrence of fistula and the different parameters were evaluated. Palatal fistulas were observed in 27% of the sample, with a greater proportion at the anterior region (37.11%). The chi-square statistical test revealed statistically significant association (P ≤ .05) between the fistulas and initial cleft width (P = .0003), intraoperative problems (P = .0037), and postoperative problems (P = .00002). The prevalence of palatal fistula was similar to mean values reported in the literature. Analysis of causal factors showed a positive association between palatal fistulas with wide and regular initial cleft width and intraoperative and postoperative problems. The anterior region presented the greatest occurrence of fistulas.

Use of three-dimensional computer graphic animation to illustrate cleft lip and palate surgery.

PubMed

Cutting, C; Oliker, A; Haring, J; Dayan, J; Smith, D

2002-01-01

Three-dimensional (3D) computer animation is not commonly used to illustrate surgical techniques. This article describes the surgery-specific processes that were required to produce animations to teach cleft lip and palate surgery. Three-dimensional models were created using CT scans of two Chinese children with unrepaired clefts (one unilateral and one bilateral). We programmed several custom software tools, including an incision tool, a forceps tool, and a fat tool. Three-dimensional animation was found to be particularly useful for illustrating surgical concepts. Positioning the virtual "camera" made it possible to view the anatomy from angles that are impossible to obtain with a real camera. Transparency allows the underlying anatomy to be seen during surgical repair while maintaining a view of the overlaying tissue relationships. Finally, the representation of motion allows modeling of anatomical mechanics that cannot be done with static illustrations. The animations presented in this article can be viewed on-line at http://www.smiletrain.org/programs/virtual_surgery2.htm. Sophisticated surgical procedures are clarified with the use of 3D animation software and customized software tools. The next step in the development of this technology is the creation of interactive simulators that recreate the experience of surgery in a safe, digital environment. Copyright 2003 Wiley-Liss, Inc.

Maxillary movement in distraction osteogenesis using internal devices in cleft palate patients.

PubMed

Tomita, Daisuke; Omura, Susumu; Ozaki, Shusaku; Shimazaki, Kazuo; Fukuyama, Eiji; Tohnai, Iwai; Torikai, Katsuyuki

2011-03-01

The purpose of this cephalometric study was to compare the actual movement with the planned movement of the maxilla by using internal maxillary distraction in cleft lip and palate patients. Twelve patients, including eight with unilateral and four with bilateral cleft lip and palate, underwent maxillary advancement with internal maxillary distractors. Lateral cephalometric radiographs obtained preoperatively, predistraction, and postdistraction were used for analysis. The movement of the maxilla, angular change of the internal devices and rotation of the mandible were measured at each stage, and the planned vector of advancement predicted from the placement vector of the distractors was compared with the actual vector. Internal maxillary distractors were rotated in a clockwise direction during the distraction period. The angular change of the distractors was 7.7°. The amount of actual advancement at anterior nasal spine with distraction was 6.3 mm, which represented about 70% of the distance of activation of distraction. The actual advanced vector at anterior nasal spine was 9.7° smaller than the planned vector. The mandible underwent a clockwise rotation of 3.5°. In the internal distraction technique, the maxilla was advanced inferiorly to the planned vector and with a slight clockwise rotation. These results are useful for surgical planning when using internal distractors.

RapidNAM: generative manufacturing approach of nasoalveolar molding devices for presurgical cleft lip and palate treatment.

PubMed

Bauer, Franz Xaver; Schönberger, Markus; Gattinger, Johannes; Eblenkamp, Markus; Wintermantel, Erich; Rau, Andrea; Güll, Florian Dieter; Wolff, Klaus-Dietrich; Loeffelbein, Denys J

2017-08-28

Nasoalveolar molding (NAM) is an accepted treatment strategy in presurgical cleft therapy. The major drawbacks of the treatment listed in the literature relate to the time of the treatment and the coordination of the required interdisciplinary team of therapists, parents, and patients. To overcome these limitations, we present the automated RapidNAM concept that facilitates the design and manufacturing process of NAM devices, and that allows the virtual modification and subsequent manufacture of the devices in advance, with a growth prediction factor adapted to the patient's natural growth. The RapidNAM concept involves (i) the prediction of three trajectories that envelope the fragmented alveolar segments with the goal to mimic a harmonic arch, (ii) the extrusion from the larger toward the smaller alveolar segment along the envelope curves toward the harmonic upper alveolar arch, and (iii) the generation of the NAM device with a ventilation hole, fixation pin, and fixation points for the nasal stents. A feasibility study for a vector-based approach was successfully conducted for unilateral and bilateral cleft lip and palate (CLP) patients. A comparison of the modified target models with the reference target models showed similar results. For further improvement, the number of landmarks used to modify the models was increased by a curve-based approach.

First branchial cleft anomalies: presentation, variability and safe surgical management.

PubMed

Magdy, Emad A; Ashram, Yasmine A

2013-05-01

First branchial cleft (FBC) anomalies are uncommon. The aim of this retrospective clinical study is to describe our experience in dealing with these sporadically reported lesions. Eighteen cases presenting with various FBC anomalies managed surgically during an 8-year period at a tertiary referral medical institution were included. Ten were males (56 %) and eight females (44 %) with age range 3-18 years. Anomaly was right-sided in 12 cases (67 %). None were bilateral. Nine patients (50 %) had prior abscess incision and drainage procedures ranging from 1 to 9 times. Two also had previous unsuccessful surgical excisions. Clinical presentations included discharging tract openings in external auditory canal/conchal bowl (n = 9), periauricular (n = 6), or upper neck (n = 4); cystic postauricular, parotid or upper neck swellings (n = 5); and eczematous scars (n = 9). Three distinct anatomical types were encountered: sinuses (n = 7), fistulas (n = 6), and cysts (n = 5). Complete surgical excision required superficial parotidectomy in 11 patients (61 %). Anomaly was deep to facial nerve (FN) in three cases (17 %), in-between its branches in two (11 %) and superficial (but sometimes adherent to the nerve) in remaining cases (72 %). Continuous intraoperative electrophysiological FN monitoring was used in all cases. Two cases had postoperative temporary lower FN paresis that recovered within 2 months. No further anomaly manifestation was observed after 49.8 months' mean postoperative follow-up (range 10-107 months). This study has shown that awareness of different presentations and readiness to identify and protect FN during surgery is essential for successful management of FBC anomalies. Intraoperative electrophysiological FN monitoring can help in that respect.

Segmental maxillary distraction with a novel device for closure of a wide alveolar cleft

PubMed Central

Bousdras, Vasilios A.; Liyanage, Chandra; Mars, Michael; Ayliffe, Peter R

2014-01-01

Treatment of a wide alveolar cleft with initial application of segmental distraction osteogenesis is reported, in order to minimise cleft size prior to secondary alveolar bone grafting. The lesser maxillary segment was mobilised with osteotomy at Le Fort I level and, a novel distractor, facilitated horizontal movement of the dental/alveolar segment along the curvature of the maxillary dental arch. Following a latency period of 4 days distraction was applied for 7 days at a rate of 0.5 mm twice daily. Radiographic, ultrasonographic and clinical assessment revealed new bone and soft tissue formation 8 weeks after completion of the distraction phase. Overall the maxillary segment did move minimising the width of the cleft, which allowed successful closure with a secondary alveolar bone graft. PMID:24987601

Segmental maxillary distraction with a novel device for closure of a wide alveolar cleft.

PubMed

Bousdras, Vasilios A; Liyanage, Chandra; Mars, Michael; Ayliffe, Peter R

2014-01-01

Treatment of a wide alveolar cleft with initial application of segmental distraction osteogenesis is reported, in order to minimise cleft size prior to secondary alveolar bone grafting. The lesser maxillary segment was mobilised with osteotomy at Le Fort I level and, a novel distractor, facilitated horizontal movement of the dental/alveolar segment along the curvature of the maxillary dental arch. Following a latency period of 4 days distraction was applied for 7 days at a rate of 0.5 mm twice daily. Radiographic, ultrasonographic and clinical assessment revealed new bone and soft tissue formation 8 weeks after completion of the distraction phase. Overall the maxillary segment did move minimising the width of the cleft, which allowed successful closure with a secondary alveolar bone graft.

Use of cartilage grafts for closure of cleft palate fistulae.

PubMed

Jeffery, S L; Boorman, J G; Dive, D C

2000-10-01

We describe the results of using a free cartilage graft in the closure of cleft palate fistulae in 14 patients with a mean follow-up of 8.6 months. Complete closure of the fistula was achieved in 11 patients (79%), with partial closure in the remaining three patients. This technique is simple, causes relatively little discomfort, involves little tissue dissection and can be performed as a day-case procedure. The success rate is comparable with or better than other methods, and we consider it the treatment of choice for small cleft palate fistulae. Copyright 2000 The British Association of Plastic Surgeons.

Toxicological Assessment of ABATE (Tradename) (0,0,0’,0’-Tetramethyl-0,0’-Thio-Di-P-Phenylene Phosphorothioate) Administered Orally and Dermally to Mated and Nonmated Female Rabbits, April 1983.

DTIC Science & Technology

1983-11-02

such as gastroschisis, exencephaly, cleft palate ) are classified as abnormalities. Anomelies are considered to be minor varients from the normal, such as...and femurs not completely formed 1 Group VIII (IP-6-AN) 107 spina bifida 1 m microopthalmia 5 l cleft palate 3 0 talipes equinovarus 5 m... palate 1 m crantoschisis I m gastroschisis 1 webbed feet 1 m fused ribs 1 118 microopthalmia 3 m cleft palate 2 0 tallpes equtnovarus 1 0 120

A Case of First Branchial Cleft Fistula Presenting with an External Opening on the Root of the Helical Crus

PubMed Central

2018-01-01

Background First branchial cleft anomalies (FBCA) are rare clinical entities of the head and neck. Typically, the tract of the FBCA begins in the external auditory canal and ends in the postauricular or submandibular region. Case Presentation We present a case of a 23-year-old man who had a first branchial cleft fistula with atypical opening on the root of the helical crus. Complete excision of the tract, including the cuff of surrounding cartilage, was performed. Histopathology revealed a fistular tract lined with squamous epithelium. To our knowledge, this is the first case to be reported of type I FBCA with an opening on the root of the helical crus. The low incidence and varied presentation often result in misdiagnosis and inappropriate treatment. Conclusions In the patients with FBCA, careful recognition of atypical variants is essential for complete excision. PMID:29560006

Electromyographic Activity of the Masseter and Temporal Muscles in Patients With Nonsyndromic Complete Unilateral Cleft Lip and Palate: 2-Stage Versus 1-Stage Palate Repair.

PubMed

Sabbag, Anelise; Denadai, Rafael; Raposo-Amaral, Cesar Augusto; Buzzo, Celso Luiz; Raposo-Amaral, Cassio Eduardo; Nagae, Mirian H

2018-05-14

To assess the electromyographic activity of the masseter and temporal muscles in cleft patients who underwent 1-stage palate repair versus 2-stage palate repair. Thirty-two patients with nonsyndromic complete unilateral cleft lip and palate operated by 2 different protocols for palate repair, 1-stage (group 1, n = 16) versus 2-stage with delayed hard palate closure (group 2, n = 16) were available in the retrospective longitudinal study. Standardized electromyographic records of the masseter and anterior portion of temporal muscles were obtained with 2 repetitions during mastication and rest. No statistically significant (all P > 0.05) differences were observed in the electromyographic data between the groups 1 and 2. There were similar electromyographic activity of masseter and temporal muscles during mastication and at rest after 1- and 2-stage palate closure.

A Type I first branchial cleft cyst masquerading as a parotid tumor

PubMed Central

Krishnamurthy, Arvind; Ramshanker, Vijayalakshmi

2014-01-01

Branchial cleft anomalies are caused by incomplete regression of the cervical sinus of “His” during the 6th and 7th weeks of embryologic development. Although congenital in origin, first branchial cleft cysts (FBCCs) can present later in life. FBCCs are rare causes of parotid swellings, accounting for <1% of all the branchial cleft abnormalities. The diagnosis of FBCCs is a clinical challenge; the condition is often overlooked and mismanaged. We report a case of Type 1 FBCC in a 22-year-old female with an asymptomatic 3.5 cm × 2.5 cm sized cystic mass. It was removed completely under the impression of a cystic tumor of the parotid. On histopathology, the cyst had a squamous epithelium-lined wall with lymphoid aggregation which was characteristic of a branchial cleft cyst. A good understanding of the regional anatomy and embryology can lead to an early diagnosis and thereby effective management of FBCC. PMID:25298726

Effect of Maxillary Osteotomy on Speech in Cleft Lip and Palate: Perceptual Outcomes of Velopharyngeal Function

ERIC Educational Resources Information Center

Pereira, Valerie J.; Sell, Debbie; Tuomainen, Jyrki

2013-01-01

Background: Abnormal facial growth is a well-known sequelae of cleft lip and palate (CLP) resulting in maxillary retrusion and a class III malocclusion. In 10-50% of cases, surgical correction involving advancement of the maxilla typically by osteotomy methods is required and normally undertaken in adolescence when facial growth is complete.…

Team approach concept in management of oro-facial clefts: a survey of Nigerian practitioners

PubMed Central

Akinmoladun, Victor I; Obimakinde, Obitade S

2009-01-01

Background Cleft palate craniofacial teams have evolved across the globe in the last 20 years in compliance with the interdisciplinary concept of management of oro-facial clefts. An interdisciplinary care allows a coordinated treatment protocol for the patient. The objective of this study was to evaluate oro-facial cleft care in Nigeria with particular emphasis on the compliance of the practitioners to the team approach concept. Methods A snapshot survey was conducted among specialists that attended the Pan African Congress on Cleft Lip and Palate, at the International Institute of Tropical Agriculture, Nigeria in February 2007. Result Sixty three respondents successfully completed and returned the questionnaire for analysis. Mean age of respondents was 43.5 years and the range was 38–62 years. Male to female ratio was 2.7:1. Oral and Maxillofacial Surgeons and Plastic Surgeons constituted the majority of respondents (38.1% and 22.2%) respectively. Only 47.6% (n = 30) of the specialists belonged to cleft teams. Majority of Oral and Maxillofacial Surgeons and Plastic Surgeons belonged to cleft teams (70% and 63.3% respectively) while speech pathologists and orthodontists were less represented (20% and 36.7% respectively) in teams. Conclusion Findings from this study suggests that interdisciplinary care for the cleft patient does not appear to have been fully embraced in Nigeria. This may be a result of several reasons ranging from non availability of the requisite specialists, the relatively young age of cleft care practice in this part of the world to the poor state of infrastructure. PMID:19426559

Nasal Duplication Combined with Cleft Lip and Palate: Surgical Correction and Long-Term Follow-Up.

PubMed

Long, Kanharith; Yamaguchi, Kazuaki; Lonic, Daniel; Long, Vanna; Chhoeurn, Vuthy; Lo, Lun-Jou

2017-10-01

Diprosopus dirrhinus, or nasal duplication, is a rare entity of partial craniofacial duplication. The case we present is the first report of diprosopus dirrhinus associated with complete cleft lip and palate. The baby was born in Cambodia at full term by normal vaginal delivery with no significant perinatal and family history. Physical examination revealed significant facial deformity due to the duplicated nose and the left complete cleft lip/palate on the right subset. There were 4 nostrils; both medial apertures including the cleft site were found to be 10-15 mm deep cul-de-sac structures without communication to the nasopharynx. The upper third of the face was notable for hypertelorism with a duplication of the soft-tissue nasion and glabella. Between the 2 nasal dorsums, there was a small cutaneous depression with a lacrimal fistula in the midline. Surgical treatment included the first stage of primary lip and nose repair and the second stage of palatoplasty. The patient was followed up at the age of 10 years showing satisfactory results for both aesthetic and functional aspects. Further management in the future will be required for the hypertelorism and nasal deformity.

Nasal Duplication Combined with Cleft Lip and Palate: Surgical Correction and Long-Term Follow-Up

PubMed Central

Long, Kanharith; Yamaguchi, Kazuaki; Lonic, Daniel; Long, Vanna; Chhoeurn, Vuthy

2017-01-01

Background: Diprosopus dirrhinus, or nasal duplication, is a rare entity of partial craniofacial duplication. Methods: The case we present is the first report of diprosopus dirrhinus associated with complete cleft lip and palate. The baby was born in Cambodia at full term by normal vaginal delivery with no significant perinatal and family history. Physical examination revealed significant facial deformity due to the duplicated nose and the left complete cleft lip/palate on the right subset. Results: There were 4 nostrils; both medial apertures including the cleft site were found to be 10–15 mm deep cul-de-sac structures without communication to the nasopharynx. The upper third of the face was notable for hypertelorism with a duplication of the soft-tissue nasion and glabella. Between the 2 nasal dorsums, there was a small cutaneous depression with a lacrimal fistula in the midline. Surgical treatment included the first stage of primary lip and nose repair and the second stage of palatoplasty. Conclusions: The patient was followed up at the age of 10 years showing satisfactory results for both aesthetic and functional aspects. Further management in the future will be required for the hypertelorism and nasal deformity. PMID:29184738

Rehabilitative treatment of cleft lip and palate: experience of the Hospital for Rehabilitation of Craniofacial Anomalies - USP (HRAC-USP) - Part 2: Pediatric Dentistry and Orthodontics

PubMed Central

FREITAS, José Alberto de Souza; GARIB, Daniela Gamba; OLIVEIRA, Thais Marchini; LAURIS, Rita de Cássia Moura Carvalho; de ALMEIDA, Ana Lúcia Pompéia Fraga; NEVES, Lucimara Teixeira; TRINDADE-SUEDAM, Ivy Kiemle; YAEDÚ, Renato Yassutaka Faria; SOARES, Simone; PINTO, João Henrique Nogueira

2012-01-01

The aim of this article is to present the pediatric dentistry and orthodontic treatment protocol of rehabilitation of cleft lip and palate patients performed at the Hospital for Rehabilitation of Craniofacial Anomalies - University of São Paulo (HRAC-USP). Pediatric dentistry provides oral health information and should be able to follow the child with cleft lip and palate since the first months of life until establishment of the mixed dentition, craniofacial growth and dentition development. Orthodontic intervention starts in the mixed dentition, at 8-9 years of age, for preparing the maxillary arch for secondary bone graft procedure (SBGP). At this stage, rapid maxillary expansion is performed and a fixed palatal retainer is delivered before SBGP. When the permanent dentition is completed, comprehensive orthodontic treatment is initiated aiming tooth alignment and space closure. Maxillary permanent canines are commonly moved mesially in order to substitute absent maxillary lateral incisors. Patients with complete cleft lip and palate and poor midface growth will require orthognatic surgery for reaching adequate anteroposterior interarch relationship and good facial esthetics. PMID:22666849

Mandibulofacial Dysostosis in a Patient with a de novo 2;17 Translocation that Disrupts the HOXD Gene Cluster

PubMed Central

Stevenson, David A.; Bleyl, Steven B.; Maxwell, Teresa; Brothman, Arthur R.; South, Sarah T.

2011-01-01

Treacher Collins syndrome is the prototypical mandibulofacial dysostosis syndrome, but other mandibulofacial dysostosis syndromes have been described. We report an infant with mandibulofacial dysostosis and an apparently balanced de novo 2;17 translocation. She presented with severe lower eyelid colobomas requiring skin grafting, malar and mandibular hypoplasia, bilateral microtia with external auditory canal atreasia, dysplastic ossicles, hearing loss, bilateral choanal stenosis, cleft palate without cleft lip, several oral frenula of the upper lip/gum, and micrognathia requiring tracheostomy. Her limbs were normal. Chromosome analysis at the 600-band level showed a 46,XX,t(2;17)(q24.3;q23) karyotype. Sequencing of the entire TCOF1 coding region did not show evidence of a sequence variation. High-resolution genomic microarray analysis did not identify a cryptic imbalance. FISH mapping refined the breakpoints to 2q31.1 and 17q24.3–25.1 and showed the 2q31.1 breakpoint likely affects the HOXD gene cluster. Several atypical findings and lack of an identifiable TCOF1 mutation suggest that this child has a provisionally unique mandibulofacial dysostosis syndrome. The apparently balanced de novo translocation provides candidate loci for atypical and TCOF1 mutation negative cases of Treacher Collins syndrome. Based on the agreement of our findings with one previous case of mandibulofacial dysostosis with a 2q31.1 transocation, we hypothesize that misexpression of genes in the HOXD gene cluster produced the described phenotype in this patient. PMID:17431905

Mandibulofacial dysostosis in a patient with a de novo 2;17 translocation that disrupts the HOXD gene cluster.

PubMed

Stevenson, David A; Bleyl, Steven B; Maxwell, Teresa; Brothman, Arthur R; South, Sarah T

2007-05-15

Treacher Collins syndrome (TCS) is the prototypical mandibulofacial dysostosis syndrome, but other mandibulofacial dysostosis syndromes have been described. We report an infant with mandibulofacial dysostosis and an apparently balanced de novo 2;17 translocation. She presented with severe lower eyelid colobomas requiring skin grafting, malar and mandibular hypoplasia, bilateral microtia with external auditory canal atreasia, dysplastic ossicles, hearing loss, bilateral choanal stenosis, cleft palate without cleft lip, several oral frenula of the upper lip/gum, and micrognathia requiring tracheostomy. Her limbs were normal. Chromosome analysis at the 600-band level showed a 46,XX,t(2;17)(q24.3;q23) karyotype. Sequencing of the entire TCOF1 coding region did not show evidence of a sequence variation. High-resolution genomic microarray analysis did not identify a cryptic imbalance. FISH mapping refined the breakpoints to 2q31.1 and 17q24.3-25.1 and showed the 2q31.1 breakpoint likely affects the HOXD gene cluster. Several atypical findings and lack of an identifiable TCOF1 mutation suggest that this child has a provisionally unique mandibulofacial dysostosis syndrome. The apparently balanced de novo translocation provides candidate loci for atypical and TCOF1 mutation negative cases of TCS. Based on the agreement of our findings with one previous case of mandibulofacial dysostosis with a 2q31.1 transocation, we hypothesize that misexpression of genes in the HOXD gene cluster produced the described phenotype in this patient.

[Presurgical alveolar molding using computer aided design in infants with unilateral complete cleft lip and palate].

PubMed

Zgong, Xin; Yu, Quan; Yu, Zhe-yuan; Wang, Guo-min; Qian, Yu-fen

2012-04-01

To establish a new method of presurgical alveolar molding using computer aided design(CAD) in infants with complete unilateral cleft lip and palate (UCLP). Ten infants with complete UCLP were recruited. A maxillary impression was taken at the first examination after birth. The study model was scanned by a non-contact three-dimensional laser scanner and a digital model was constructed and analyzed to simulate the alveolar molding procedure with reverse engineering software (RapidForm 2006). The digital geometrical data were exported to produce a scale model using rapid prototyping technology. The whole set of appliances was fabricated based on these solid models. The digital model could be viewed and measured from any direction by the software. By the end of the NAM treatment before surgical lip repair, the cleft was narrowed and the malformation of alveolar segments was aligned normally, significantly improving nasal symmetry and nostril shape. Presurgical NAM using CAD could simplify the treatment procedure and estimate the treatment objective, which enabled precise control of the force and direction of the alveolar segments movement.

Sports-Related Groin Pain Secondary to Symphysis Pubis Disorders: Correlation Between MRI Findings and Outcome After Fluoroscopy-Guided Injection of Steroid and Local Anesthetic.

PubMed

Byrne, Caoimhe A; Bowden, Dermot J; Alkhayat, Abdullah; Kavanagh, Eoin C; Eustace, Stephen J

2017-08-01

The objective of our study was to correlate patterns of injury on preprocedural MRI with outcome after targeted fluoroscopy-guided steroid and local anesthetic injection of the symphysis pubis and its muscular attachments in a group of athletes with chronic groin pain. Forty-five patients with chronic sports-related groin pain underwent MRI of the pelvis and a targeted fluoroscopy-guided symphyseal corticosteroid and local anesthetic injection. Preprocedural MRI was reviewed. The presence or absence of a "superior cleft" sign (i.e., rectus abdominis-adductor longus attachment microtearing), "secondary cleft" sign (i.e., short adductor [gracilis, adductor brevis, and pectineus muscles] attachment microtearing), osteitis pubis, and extrasymphyseal pelvic abnormalities was recorded. Patients were followed up a mean time of 23 months after the procedure, and outcome was recorded. Correlation was made between preprocedural MRI findings and outcome. Forty-two percent of the patients had an isolated superior cleft sign, 7% had an isolated secondary cleft sign, and 11% had isolated osteitis pubis. Thirty-one percent of patients had a more complex injury, and 9% had a normal symphysis pubis. Overall, 89% of the patients experienced an improvement in symptoms. The response was sustained after a minimum of 6 months in 58% of the patients. The presence of the superior cleft sign was more frequently associated with a complete recovery. Fluoroscopy-guided corticosteroid symphyseal injection is a safe and effective treatment of sports-related groin pain. It is more frequently associated with a complete recovery in patients who display an isolated superior cleft sign on MRI. MRI not only is useful in characterizing groin injuries but also may be helpful in predicting response to therapeutic injection.

Evolution of my philosophy in the treatment of unilateral cleft lip and palate.

PubMed

Brusati, Roberto

2016-08-01

At the end of 50-year-long clinical activity, the evolution of my approach to the treatment of unilateral cleft of the lip and palate is discussed. I had several teachers in this field (Rusconi, Reherman, Perko, Delaire, Talmant, Sommerlad and others) and I introduced in my approach what I considered to be improvements from all of them. My current protocol is related to the anatomy of the cleft: for wide clefts a two-stage protocol is applied (1° step: soft palate and lip and nose repair; 2° step: hard palate repair with gingivoalveoloplasty); for narrow cleft (less than 1 cm at the posterior border of hard palate) an "all in one" protocol is performed with or without gingivoalveoloplasty (in accordance to the presence or absence of contact between the stumps at alveolar level). The most important details regarding surgery of the lip and palate are discussed. Robust data collection on speech and skeletal growth is still needed to determine whether the "all in one" approach can be validated as the treatment of choice for unilateral complete lip and palate cleft in selected cases. Copyright © 2016. Published by Elsevier Ltd.

Segmental Maxillary Distraction Osteogenesis With a Hybrid-Type Distractor in the Management of Wide Alveolar Cleft.

PubMed

Zhang, Jianfei; Zhang, Wenbin; Shen, Steve Guofang

2018-01-01

To investigate segmental maxillary distraction osteogenesis (DO) with hybrid-type distractor in the management of wide alveolar cleft. Six patients underwent segmental DO with a hybrid-type distractor. After the success of DO and 3-month consolidation period, removal of the distractor was accompanied by alveolar bone graft with iliac bone. Panoramic radiograph and computed tomography scanning were taken preoperatively (T0) and the day after distractor removal (T1). The crest distance between the long axis of cleft nearby teeth was measured. All patients completed the DO period, and the succeeding alveolar bone graft healing was uneventful. The mean cleft distance decrease was 12.05 mm (range: 10.1-13.5 mm). As for the mobility degree record of abutment tooth in the transport segment recorded, 6 patients were grading I° at T0, while 5 patients were grading I° and 1 patient was grading II° at T1. Segmental maxillary DO with the hybrid-type distractor is successful to reduce the cleft width in these cases, and it is promising in the treatment of wide dental alveolar cleft, especially for the adult patient.

Coping with a cleft II: Factors associated with psychosocial adjustment of adolescents with a cleft lip and palate and their parents.

PubMed

Berger, Zoe E; Dalton, Louise J

2011-01-01

TO explore the factors that predict psychosocial adjustment in young people with a cleft and their parents. DESIGN, PARTICIPANTS, SETTING: The study used a cross-sectional postal questionnaire design involving young people aged between 11 and 16 and their parents from two cleft services. Data are presented for 91 adolescents and their mothers. Participants completed measures of psychological adjustment, coping, social experiences, satisfaction with appearance, stressful life events, cleft-related factors, and demographic information. Psychosocial adjustment in adolescents was predicted by their social experiences and maternal well-being. Satisfaction with appearance, perceived speech problems, and the use of avoidant coping strategies were also important factors relating to their adjustment. For mothers, adjustment was predicted by use of coping strategies such as self-blame, venting, and acceptance, in addition to perceived problems with their child's hearing and the number of stressful life events experienced. The findings are discussed in relation to the concepts of adjustment, coping, satisfaction with appearance, and maternal mental health. Directions for future research are outlined, and a number of opportunities and challenges for cleft services regarding the provision of timely interventions for this age group and their families are discussed.

Prenatal diagnosis of orofacial clefts: association with maternal satisfaction, team care, and treatment outcomes.

PubMed

Robbins, James M; Damiano, Peter; Druschel, Charlotte M; Hobbs, Charlotte A; Romitti, Paul A; Austin, April A; Tyler, Margaret; Reading, J Alex; Burnett, Whitney

2010-09-01

Prenatal diagnosis of an orofacial cleft is thought to allow mothers greater opportunity to become prepared for the special needs of an infant with a cleft and plan for the care of their child. Using a population-based sample, we determined which children were more likely to be diagnosed prenatally, and whether early diagnosis was associated with maternal satisfaction and treatment outcomes. Interviews were completed with 235 (49% of eligible) mothers of children ages 2 to 7 with orofacial clefts initially enrolled in the National Birth Defects Prevention Study from the Arkansas, Iowa, and New York sites. Maternal satisfaction with information, support, and treatment outcomes was compared between women who received a prenatal diagnosis and those who did not. Of 235 infants with clefts, 46 (19.6%) were identified prenatally. One third of mothers were somewhat or not satisfied with information provided by medical staff. Satisfaction did not vary by timing of the diagnosis. Infants diagnosed prenatally were no more likely to have received care provided by a recognized multidisciplinary cleft team (76%) than were infants diagnosed at birth (78%). Speech problems and facial appearance as rated by the mother did not vary by timing of the diagnosis. Timing of the cleft diagnosis did not alter maternal satisfaction with information, whether care was provided by a designated cleft team, or maternal perception of facial appearance or speech. Further research should determine whether prenatal diagnoses alter maternal anxiety or influence postnatal morbidity.

Prospective Patient-Related Outcome Evaluation of Secondary Cleft Rhinoplasty Using a Validated Questionnaire.

PubMed

Sawyer, Adam R; Robinson, Stephen; Cadier, Michael

2017-07-01

To evaluate patient satisfaction and quality of life following secondary cleft rhinoplasty. Prospective consecutive patient, single unit, single surgeon study. Spires Cleft Centre, Salisbury, Wilshire, United Kingdom, and private practice. 56 (27 secondary cleft rhinoplasty) patients completed evaluation forms preoperatively and 3 to 6 months postoperatively. Subjective assessment was performed using a validated Rhinoplasty Outcomes Evaluation (ROE) questionnaire. This instrument comprises six questions that capture three quality-of-life domains: physical, mental/emotional, and social. Rhinoplasty outcomes evaluation scores were calculated (range = 0 to 100) to indication satisfaction with rhinoplasty outcomes. Average age was 28 years (range = 18 to 59 years). There was a significant subjective improvement in the total ROE evaluation scores from 28 ± 10 to 80 ± 11 (P < .01) in secondary cleft rhinoplasty. Similar results were achieved in noncleft rhinoplasty 34 ± 9 to 84 ± 9 (P < .01). Specific scores for nasal aesthetic appearance improved from 0.3 ± 0.2 to 3.2 ± 0.3 (P < .01) in secondary cleft rhinoplasty. No significant change was seen in breathing capacity in secondary cleft rhinoplasty (from 2.7 ± 0.3 to 3.2 ± 0.2; P = .29). All patients said they would undergo the procedure again. Our results demonstrate high patient satisfaction after cleft rhinoplasty with particular regard to cosmetic appearance. These results are similar to those for noncleft rhinoplasty. We would recommend the use of this simple and quick validated outcome tool with all rhinoplasty patients.

Anophthalmia-plus syndrome with unusual findings. A clinical report and review of the literature.

PubMed

Cayir, A; Tasdemir, S; Eroz, R; Yuce, I; Orbak, Z; Tatar, A

2013-01-01

We present a male child at 3 years old with Anophthalmia-Plus Syndrome (APS). He has asymmetry of the face and head, left choanal atresia, a sunken facial appearance, microphthalmia in the right eye, severe microphthalmia in the left eye, bilateral low-set ears, scarring from cleft palate surgery. Magnetic resonance imaging (MRI) sections revealed decreased right globe volume, an undeveloped left globe, decreased left optical nerve thickness, Chiari type 2 malformation, left choanal atresia and cleft palate. Echocardiography and abdominal ultrasonography were normal. The patient has a 45 dB conductive hearing loss in the left ear. Repeated thyroid function tests were evaluated as compatible with central hypothyroidism. We report a Fryns Anophthalmia-Plus Syndrome in a child with unusual findings including central hypothyroidism, chiari type 2 malformation, conductive hearing loss and developmental regression. Summary of the features reported in the present case and all 14 previous cases that might be defined as APS.

Evaluation of 5-year-old children with complete cleft lip and palate: Multicenter study. Part 1: Lip and nose aesthetic results.

PubMed

Dissaux, Caroline; Bodin, Frédéric; Grollemund, Bruno; Picard, Arnaud; Vazquez, Marie-Paule; Morand, Béatrice; James, Isabelle; Kauffmann, Isabelle; Bruant-Rodier, Catherine

2015-12-01

Cleft surgery is marked by all the controversies and the multiplication of protocols, as it has been shown by the Eurocleft study. The objective of this pilot study is to start a comparison and analyzing procedure between primary surgical protocols in French centers. Four French centers with different primary surgical protocols for cleft lip and palate repair, have accepted to be involved in this retrospective study. In each center, 20 consecutive patients with complete cleft lip and palate (10 UCLP and 10 BCLP per center), non syndromic, have been evaluated at a mean age of 5 [4,6]. In this first part, the aesthetic results of nose and lip repair were assessed based on the scale established by Mortier et al. (1997). Considering nose outcome, primary cleft repair surgery including a nasal dissection gives a statistically significant benefit in terms of septum deviation. Considering lip result, muscular dehiscence rate is significantly higher in BCLP patients with a two-stage lip closure. The centers using Millard one-stage lip closure do not have uniform results. For UCLP patients, the quality of scar is not statistically different between Skoog and Millard techniques. Primary results based on a simple, reproducible evaluation protocol. Extension to other centers required. Therapeutic study. Level III/retrospective multicenter comparative study. Copyright © 2015 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

First Branchial Cleft Malformation with Duplication of External Auditory Canal

PubMed Central

Parida, Pradipta Kumar; Raja, Kalairasi; Surianarayanan, Gopalakrishnan; Ganeshan, Sivaraman

2013-01-01

First branchial cleft anomalies are uncommon, accounting for less than 10% of all branchial abnormalities. Their rare occurrence and varied presentation have frequently led to misdiagnosis and inadequate and inappropriate treatment of these conditions leading to repeated recurrences and secondary infection. In this paper, a case of 11-year girl with type 2 first branchial cleft defect is described. She first presented with a nonhealing ulcer of upper neck from childhood. Diagnosis had previously been missed and treated as tubercular ulcer. We confirmed the correct diagnosis by history and computerized tomography fistulogram. The lesion was completely excised with no further recurrence. PMID:24312740

Preoperative non-surgical over-correction of cleft lip nasal deformity.

PubMed

Matsuo, K; Hirose, T

1991-01-01

Alar cartilage, which is elastic like auricular cartilage, is correctable in the early neonatal period. Taking advantage of this correctability, we have performed preoperative non-surgical over-correction for cleft lip nasal deformity of incomplete and complete cleft lips with a Simonart's band. The device for this correction was made by processing a nostril retainer into a nostril over-corrector which utilises a spring of silicone rubber, works like a tissue expander and is supported by the nostril floor. Twenty cases are reviewed whose follow-up lasted more than 19 months. The earlier the non-surgical over-correction began, the more satisfactory were the results that were obtained.

Uneven Reassembly of Tense, Telicity and Discourse Features in L2 Acquisition of the Chinese "shì…de" Cleft Construction by Adult English Speakers

ERIC Educational Resources Information Center

Mai, Ziyin; Yuan, Boping

2016-01-01

This article reports an empirical study investigating L2 acquisition of the Mandarin Chinese "shì…de" cleft construction by adult English-speaking learners within the framework of the Feature Reassembly Hypothesis (Lardiere, 2009). A Sentence Completion task, an interpretation task, two Acceptability Judgement tasks, and a felicity…

Atypical findings in three patients with Pai syndrome and literature review.

PubMed

Lederer, Damien; Wilson, Brian; Lefesvre, Pierre; Poorten, Vincent Vander; Kirkham, Nigel; Mitra, Dipayan; Verellen-Dumoulin, Christine; Devriendt, Koenraad

2012-11-01

Pai syndrome is a rare disorder characterized by congenital nasal or facial polyp, midline cleft lip, pericallosal lipoma, ocular anomalies, and normal neuropsychological development. Here, we report on three patients with Pai syndrome and atypical findings: temporal triangular alopecia, posterior lenticonus, bilateral palatal pits, bifid uvula, hypospadias, sacral dimple, true tracheal bronchus, and epilepsy. Thirty-three cases of Pai syndrome have been described so far. We present a review of the previously reported cases and suggest modified diagnostic criteria for Pai syndrome. Copyright © 2012 Wiley Periodicals, Inc.

Secondary repair of cleft lip deformity.

PubMed

Tessier, P; Tulasne, J F

1984-10-01

A considerable number of procedures have been described for secondary improvements of the lip. They involve the scars, the Cupid's bow, the philtrum, the alar base, the vestibulum and the columella. In fact, these procedures may give a good aesthetic result in a static position, but the truly successful results occur with normal movements. A normal motion of the lip is impossible without a proper dissection and reorientation of the muscles. Satisfactory lip motion cannot be realized with a defective alveolus or piriform aperture that retracts the alar base. Consequently, a good lip or nasal base cannot be achieved without bony restoration of the alveolus and piriform aperture. A transversely short lip never has normal mobility, and it must be widened by an Abbé flap. A tight, retruded lip on a retruded maxilla cannot be improved by a prosthetic vestibular plate because a greater tension will restrict movements. The lip must be advanced with the maxilla, then widened if necessary by an Abbé flap. A thick prolapsed lip never has normal movement; it must be raised by an infranasal excision. A cleft lip has nothing to gain from being displayed under a short nose, or, still worse, under a upturned nose. The nose must be kept long enough to cast a shadow on the lip. For the same reason, a bone graft is often necessary in bilateral clefts because the nose is short and retruded.

Genomewide Scan for Nonsyndromic Cleft Lip and Palate in Multigenerational Indian Families Reveals Significant Evidence of Linkage at 13q33.1-34

PubMed Central

Radhakrishna, Uppala; Ratnamala, Uppala; Gaines, Mathew; Beiraghi, Soraya; Hutchings, David; Golla, Jeffrey; Husain, Syed A.; Gambhir, Prakash S.; Sheth, Jayesh J.; Sheth, Frenny J.; Chetan, Ghati K.; Naveed, Mohammed; Solanki, Jitendra V.; Patel, Uday C.; Master, Dilipkumar C.; Memon, Rafiq; Antonarakis, Gregory S.; Antonarakis, Stylianos E.; Nath, Swapan K.

2006-01-01

Nonsyndromic cleft lip with or without cleft palate (CL-P) is a common congenital anomaly with incidence ranging from 1 in 300 to 1 in 2,500 live births. We analyzed two Indian pedigrees (UR017 and UR019) with isolated, nonsyndromic CL-P, in which the anomaly segregates as an autosomal dominant trait. The phenotype was variable, ranging from unilateral to bilateral CL-P. A genomewide linkage scan that used ∼10,000 SNPs was performed. Nonparametric linkage (NPL) analysis identified 11 genomic regions (NPL>3.5; P<.005) that could potentially harbor CL-P susceptibility variations. Among those, the most significant evidence was for chromosome 13q33.1-34 at marker rs1830756 (NPL=5.57; P=.00024). This was also supported by parametric linkage; MOD score (LOD scores maximized over genetic model parameters) analysis favored an autosomal dominant model. The maximum LOD score was 4.45, and heterogeneity LOD was 4.45 (α=100%). Haplotype analysis with informative crossovers enabled the mapping of the CL-P locus to a region of ∼20.17 cM (7.42 Mb) between SNPs rs951095 and rs726455. Thus, we have identified a novel genomic region on 13q33.1-34 that harbors a high-risk variant for CL-P in these Indian families. PMID:16909398

The Effect of Nasoalveolar Molding on Nasal Airway Anatomy: A 9-Year Follow-up of Patients With Unilateral Cleft Lip and Palate.

PubMed

Massie, Jonathan P; Bruckman, Karl; Rifkin, William J; Runyan, Christopher M; Shetye, Pradip R; Grayson, Barry; Flores, Roberto L

2018-04-01

To determine the effects of nasoalveolar molding (NAM) on nasal airway architecture. Retrospective case-control study of patients with unilateral cleft lip treated with NAM vs without NAM. Tertiary referral center specializing in cleft and craniofacial care. Patients, Participants, and Interventions: Thirty-six patients with complete unilateral cleft lip and alveolus: 19 with NAM therapy and 17 without NAM therapy. Cone beam computed tomography (CBCT) scans were compared in multiple coronal sections and were evaluated for linear and angular septal deviation, inferior turbinate hypertrophy, and linear and 2-dimensional airway area. There were no significant differences in linear or angular septal deviation, inferior turbinate area, linear stenosis, or airway area between NAM- and non-NAM-treated patients. NAM effectively molds the external nasal cartilage and structures but may have limited effects on internal nasal structures.

Economic Valuation of the Global Burden of Cleft Disease Averted by a Large Cleft Charity.

PubMed

Poenaru, Dan; Lin, Dan; Corlew, Scott

2016-05-01

This study attempts to quantify the burden of disease averted through the global surgical work of a large cleft charity, and estimate the economic impact of this effort over a 10-year period. Anonymized data of all primary cleft lip and cleft palate procedures in the Smile Train database were analyzed and disability-adjusted life years (DALYs) calculated using country-specific life expectancy tables, established disability weights, and estimated success of surgery and residual disability probabilities; multiple age weighting and discounting permutations were included. Averted DALYs were calculated and gross national income (GNI) per capita was then multiplied by averted DALYs to estimate economic gains. 548,147 primary cleft procedures were performed in 83 countries between 2001 and 2011. 547,769 records contained complete data available for the study; 58 % were cleft lip and 42 % cleft palate. Averted DALYs ranged between 1.46 and 4.95 M. The mean economic impact ranged between USD 5510 and 50,634 per person. This corresponded to a global economic impact of between USD 3.0B and 27.7B USD, depending on the DALY and GNI values used. The estimated cost of providing these procedures based on an average reimbursement rate was USD 197M (0.7-6.6 % of the estimated impact). The immense economic gain realized through procedures focused on a small proportion of the surgical burden of disease highlights the importance and cost-effectiveness of surgical treatment globally. This methodology can be applied to evaluate interventions for other conditions, and for evidence-based health care resource allocation.

Perceptions of team members working in cleft services in the United kingdom: a pilot study.

PubMed

Scott, Julia K; Leary, Sam D; Ness, Andy R; Sandy, Jonathan R; Persson, Martin; Kilpatrick, Nicky; Waylen, Andrea E

2015-01-01

Cleft care provision in the United Kingdom has been centralized over the past 15 years to improve outcomes for children born with cleft lip and palate. However, to date, there have been no investigations to examine how well these multidisciplinary teams are performing. In this pilot study, a cross-sectional questionnaire surveyed members of all health care specialties working to provide cleft care in 11 services across the United Kingdom. Team members were asked to complete the Team Work Assessment (TWA) to investigate perceptions of team working in cleft services. The TWA comprises 55 items measuring seven constructs: team foundation, function, performance and skills, team climate and atmosphere, team leadership, and team identity; individual constructs were also aggregated to provide an overall TWA score. Items were measured using five-point Likert-type scales and were converted into percentage agreement for analysis. Responses were received from members of every cleft team. Ninety-nine of 138 cleft team questionnaires (71.7%) were returned and analyzed. The median (interquartile range) percentage of maximum possible score across teams was 75.5% (70.8, 88.2) for the sum of all items. Team performance and team identity were viewed most positively, with 82.0% (75.0, 88.2) and 88.4% (82.2, 91.4), respectively. Team foundation and leadership were viewed least positively with 79.0% (72.6, 84.6) and 76.6% (70.6, 85.4), respectively. Cleft team members perceive that their teams work well, but there are variations in response according to construct.

Electromyographic analysis of superior orbicularis oris muscle function in children surgically treated for unilateral complete cleft lip and palate.

PubMed

Szyszka-Sommerfeld, Liliana; Woźniak, Krzysztof; Matthews-Brzozowska, Teresa; Kawala, Beata; Mikulewicz, Marcin

2017-09-01

The aim of this study was to assess the electrical activity of the superior orbicularis oris muscle in children surgically treated for unilateral complete cleft lip and palate (UCCLP). The sample comprised 45 patients 6.38-12.68 years of age with UCCLP and 40 subjects 6.61-11.71 years of age with no clefts. Electromyographical (EMG) recordings were taken with a DAB-Bluetooth Instrument (Zebris Medical GmbH, Germany) in the rest position and during saliva swallowing, lip protrusion and reciprocal compression of the lips, as well as while producing the phonemes /p/, /b/, and /m/ combined with the vowel /a/. The electrical activity of the upper lip during saliva swallowing and lip compression was significantly greater in the cleft group. Similar resting level activity was observed in both groups. During the production of the /p/, /b/, and /m/ phonemes combined with the vowel /a/ the results showed no significant differences in the EMG activity between children with UCCLP and noncleft subjects. Patients with UCCLP have abnormal upper lip function characterized by increased activity of the superior orbicularis oris muscle during saliva swallowing and lip compression, and this may affect facial morphology. Copyright © 2017 The Author(s). Published by Elsevier Ltd.. All rights reserved.

3-D shape analysis of palatal surface in patients with unilateral complete cleft lip and palate.

PubMed

Rusková, Hana; Bejdová, Sárka; Peterka, Miroslav; Krajíček, Václav; Velemínská, Jana

2014-07-01

Facial development of patients with unilateral complete cleft lip and palate (UCLP) is associated with many problems including deformity of the palate. The aim of this study was to evaluate palatal morphology and variability in patients with UCLP compared with Czech norms using methods of geometric morphometrics. The study was based on virtual dental cast analysis of 29 UCLP patients and 29 control individuals at the age of 15 years. The variability of palatal shape in UCLP patients was greater than that in nonclefted palates. Only 24% of clefted palates fell within the variability of controls. The palatal form of UCLP patients (range from 11.8 to 17.2 years) was not correlated with age. Compared with control palates, palates of UCLP patients were narrower, more anteriorly than posteriorly. Apart from the praemaxilla region, they were also shallower, and the difference increased posteriorly. The UCLP palate was characterised by the asymmetry of its vault. The maximum height of the palatal vault was anterior on the clefted side, whereas it was posterior on the nonclefted side. The slope of the UCLP palate was more inclined compared with the control group. The praemaxilla was therefore situated more inferiorly. Copyright © 2013 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

A Comparative Analysis of Recombinant Human Bone Morphogenetic Protein-2 with a Demineralized Bone Matrix versus Iliac Crest Bone Graft for Secondary Alveolar Bone Grafts in Patients with Cleft Lip and Palate: Review of 501 Cases.

PubMed

Hammoudeh, Jeffrey A; Fahradyan, Artur; Gould, Daniel J; Liang, Fan; Imahiyerobo, Thomas; Urbinelli, Leo; Nguyen, JoAnna T; Magee, William; Yen, Stephen; Urata, Mark M

2017-08-01

Alveolar cleft reconstruction using iliac crest bone graft is considered standard of care for children with complete cleft lip and palate at the time of mixed dentition. Harvesting bone may result in donor-site morbidity and additional operating time and length of hospitalization. Recombinant human bone morphogenetic protein (rhBMP)-2 with a demineralized bone matrix is an alternative bone source for alveolar cleft reconstruction. The authors investigated the outcomes of rhBMP-2/demineralized bone matrix versus iliac crest bone graft for alveolar cleft reconstruction by reviewing postoperative surgical complications and cleft closure. A retrospective chart review was conducted for 258 rhBMP-2/demineralized bone matrix procedures (mean follow-up, 2.9 years) and 243 iliac crest bone graft procedures (mean follow-up, 4.1 years) on 414 patients over a 12-year period. The authors compared complications, canine eruption, and alveolar cleft closure between the two groups. In the rhBMP-2/demineralized bone matrix group, one patient required prolonged intubation because of intraoperative airway swelling not thought to be caused by rhBMP-2, 36 reported facial swelling and one required outpatient steroids as treatment, and 12 had dehiscence; however, half of these complications resolved without intervention. Twenty-three of the 228 rhBMP-2/demineralized bone matrix patients and 28 of the 242 iliac crest bone graft patients required repeated surgery for alveolar cleft repair. Findings for canine tooth eruption into the cleft site through the graft were similar between the groups. The rhBMP-2/demineralized bone matrix appears to be an acceptable alternative for alveolar cleft repair. The authors found no increase in serious adverse events with the use of this material. Local complications, such as swelling and minor wound dehiscence, predominantly improved without intervention. Therapeutic, III.

Two-stage palatoplasty, is it still a valuable treatment protocol for patients with a cleft of lip, alveolus, and palate?

PubMed

Gundlach, Karsten K H; Bardach, Janusz; Filippow, Daniel; Stahl-de Castrillon, Franka; Lenz, Jan-Hendrik

2013-01-01

Speech development is of utmost importance and requires early closure of a palatal cleft. On the other hand, it is well known that all types and timings of surgical repair of facial clefts are detrimental to maxillary growth. Nevertheless, these days one is more and more confronted with a world-wide tendency in favour of the one-in-all operation to close clefts of the lip, alveolus, and palate. Therefore, a three-centre study was performed for testing - once more - the value of two-stage palatoplasty as a means to reduce the detrimental effects of surgery on palatal growth and at the same time to also enable early speech development. Plaster casts from 85 patients have been re-evaluated. All of them had a complete unilateral cleft of lip, alveolus, and palate. They had been treated according to the old therapy protocols followed in either one of the three different cleft centres many years ago, namely in Hamburg, (Western) Germany, Iowa City, IO, USA, and Rostock, (in those days still Eastern) Germany. The impressions had been taken already in 1987 from patients being either 8 years (36 pts.) or 16 years of age (49 pts.). Three different treatment protocols had been followed for these patients in those centres in those days: The main difference was that in centres A and B the palates were closed in two stages whilst in centre C palatoplasty was performed in just one operation. The most interesting results regarding the palatal growth were that: 1. In centre C (one-stage palatoplasty) the patients had more constricted palates. 2. In centre A (two-stage palatoplasty) the patients had least often an anterior cross-bite. It appears that it was possible to show once more that closing the palate in one stage at age 1 year or less is interfering most with maxillary growth. This study leads us to conclude that two-stage palatoplasty is still a valuable treatment protocol for patients with a complete unilateral cleft of lip, alveolus, and palate, especially as apparently good guidance of speech development can lead to satisfactory speech for cleft patients in whom the hard palate was closed at a later age. Copyright © 2012 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Rehabilitative treatment of cleft lip and palate: experience of the Hospital for Rehabilitation of Craniofacial Anomalies/USP (HRAC/USP) - Part 4: Oral Rehabilitation

PubMed Central

FREITAS, José Alberto de Souza; de ALMEIDA, Ana Lúcia Pompéia Fraga; SOARES, Simone; NEVES, Lucimara Teixeira das; GARIB, Daniela Gamba; TRINDADE-SUEDAM, Ivy Kiemle; YAEDÚ, Renato Yassutaka Faria; LAURIS, Rita de Cássia Moura Carvalho; OLIVEIRA, Thais Marchini; PINTO, João Henrique Nogueira

2013-01-01

Treatment of patients with cleft lip and palate is completed with fixed prostheses, removable, total, implants and aims to restore aesthetics, phonetics and function and should be guided by the basic principles of oral rehabilitation, such as physiology, stability, aesthetics, hygiene and the expectations of the patient. In order to obtain longevity of a prosthetic rehabilitation, the periodontal and dental tissue as well as the biomechanics of the prosthesis are to be respected. The purpose of this article is to describe the types of prosthetics treatment, which are performed at HRAC/USP for the rehabilitation of cleft area in adult patients. PMID:23857655

Rehabilitative treatment of cleft lip and palate: experience of the Hospital for Rehabilitation of Craniofacial Anomalies/USP (HRAC/USP) - Part 4: oral rehabilitation.

PubMed

Freitas, José Alberto de Souza; Almeida, Ana Lúcia Pompéia Fraga de; Soares, Simone; Neves, Lucimara Teixeira das; Garib, Daniela Gamba; Trindade-Suedam, Ivy Kiemle; Yaedú, Renato Yassutaka Faria; Lauris, Rita de Cássia Moura Carvalho; Oliveira, Thais Marchini; Pinto, João Henrique Nogueira

2013-01-01

Treatment of patients with cleft lip and palate is completed with fixed prostheses, removable, total, implants and aims to restore aesthetics, phonetics and function and should be guided by the basic principles of oral rehabilitation, such as physiology, stability, aesthetics, hygiene and the expectations of the patient. In order to obtain longevity of a prosthetic rehabilitation, the periodontal and dental tissue as well as the biomechanics of the prosthesis are to be respected. The purpose of this article is to describe the types of prosthetics treatment, which are performed at HRAC/USP for the rehabilitation of cleft area in adult patients.

Nonunion of the external maxillary distraction in cleft lip and palate: analysis of possible reasons.

PubMed

He, Dongmei; Genecov, David G; Barcelo, Raul

2010-10-01

Distraction osteogenesis (DO) is an effective way to treat severe maxillary deficiency in patients with cleft lip and palate. It has been reported to have long-term stability and low relapse rate compared with conventional maxillary osteotomy in large maxillary movement. However, complications such as nonunion are seldom reported. While developing our external distraction techniques, we had 4 nonunion cleft lip and palate (CLP) patients. The aim of this study is to analyze the possible reasons for these conditions by comparing them to patients successfully treated at 1 craniofacial institute. Fifty-six patients with CLP deformities treated with external maxillary distraction from 2000 to 2006 in 1 craniofacial center were reviewed. Among them, 17 had full records and follow-up time of more than 1 year. They had rigid external halo distraction with dental splint after a high Le Fort I osteotomy. Distraction was started at 5 days with a rate of 1 mm per day until the maxilla acquired the planned correction. Consolidation time ranged from 4 weeks to 3 months. Patients' general information, DO protocol, and cephalometric radiograph measurements before DO, immediately after DO, and follow-ups were evaluated. Thirteen of 17 patients successfully completed DO, whereas 4 others developed dental occlusion relapse 2 to 3 months after device removal. Later jaw surgery showed nonunion at the distraction sites. These were our first patients treated with external distraction. Their consolidation time was from 4 to 6 weeks with a mean of 5.2 weeks. After increasing the consolidation time up to 12 weeks, all of the other 13 patients had successful results without nonunion. Their mean consolidation time was 9.8 weeks. The nonunion group had significantly shorter consolidation time, older age, and larger distraction osteogenesis (DO) advancement than the successful group statistically. There was also a greater percentage of bilateral CLP patients in the nonunion group than in the successful treatment group. Among the 4 nonunion patients, 2 had failed alveolar bone graft before DO, and 3 did not have face mask retention therapy after device removal. The horizontal relapse rate in the 13 successful patients was 1 to 3 mm (mean, 11.9%) after 1 to 3 years of follow-up. All of these patients had good occlusion in the short-term follow-up (1 yr). Five developed end-to-end or Class III malocclusion in the long-term follow-up (1-3 yrs) because of maxillary relapse and continued mandibular growth that required jaw surgery later. A short consolidation time can cause nonunion, and should be lengthened to 12 weeks or more for a stable maxilla. Older age, bilateral CLP, large DO advancement (>15 mm), and compromised bone healing are the risk factors for nonunion. Copyright © 2010 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

Modification of Perioral Stiffness in Patients With Repaired Cleft Lip and Palate

PubMed Central

Barlow, Steven M.; Trotman, Carroll-Ann; Chu, Shin-Ying; Lee, Jaehoon

2013-01-01

Objective To measure and compare the perioral stiffness among three groups of pediatric subjects: a group of patients with a repaired cleft lip (and palate) who had a secondary lip revision surgery (revision), another group of patients with repaired cleft lip (and palate) who did not have secondary surgery (nonrevision), and a group of noncleft “normal” patients (noncleft). Design A parallel, three-group, nonrandomized clinical trial. Participants A total of 16 patients with repaired cleft lip/palate who did not have lip revision, 13 patients with repaired cleft lip/palate who had lip revision surgery and were tested at 18 to 24 months postsurgery, and 27 noncleft patients. Analysis Nonparticipatory perioral stiffness was sampled using a recently developed face-referenced measurement technology known as OroSTIFF. Perioral stiffness, derived as a quotient from resultant force and interangle lip span, was modeled with multilevel regression techniques. Real-time calculation of the perioral stiffness function demonstrated a significant quadratic relation between imposed interangle stretch and resultant force for each of the three groups. Results This nonlinear stiffness growth function was significantly elevated in the nonrevision patients compared with the noncleft controls and is likely due to the presence of scar tissue in the upper lip; it was significantly lower among patients with cleft lip/palate who completed lip revision surgery. Conclusion This study demonstrates the efficacy of applying an objective measurement to map differences in perioral tissue biomechanics among patients born with orofacial clefts. PMID:21247345

Bilateral cleft lip and palate: A morphometric analysis of facial skeletal form using cone beam computed tomography.

PubMed

Starbuck, John M; Ghoneima, Ahmed; Kula, Katherine

2015-07-01

Bilateral cleft lip and palate (BCLP) is caused by a lack of merging of maxillary and nasal facial prominences during development and morphogenesis. BCLP is associated with congenital defects of the oronasal facial region that can impair ingestion, mastication, speech, and dentofacial development. Using cone beam computed tomography (CBCT) images, 7- to 18-year old individuals born with BCLP (n = 15) and age- and sex-matched controls (n = 15) were retrospectively assessed. Coordinate values of three-dimensional facial skeletal anatomical landmarks (n = 32) were measured from each CBCT image. Data were evaluated using principal coordinates analysis (PCOORD) and Euclidean Distance Matrix Analysis (EDMA). PCOORD axes 1-3 explain approximately 45% of the morphological variation between samples, and specific patterns of morphological differences were associated with each axis. Approximately, 30% of facial skeletal measures significantly differ by confidence interval testing (α = 0.10) between samples. While significant form differences occur across the facial skeleton, strong patterns of differences are localized to the lateral and superioinferior aspects of the nasal aperture. In conclusion, the BCLP deformity significantly alters facial skeletal morphology of the midface and oronasal regions of the face, but morphological differences were also found in the upper facial skeleton and to a lesser extent, the lower facial skeleton. This pattern of strong differences in the oronasal region of the facial skeleton combined with differences across the rest of the facial complex underscores the idea that bones of the craniofacial skeleton are integrated. © 2015 Wiley Periodicals, Inc.

Comparative effectiveness studies examining patient-reported outcomes among children with cleft lip and/or palate: a systematic review.

PubMed

Ranganathan, Kavitha; Vercler, Christian J; Warschausky, Seth A; MacEachern, Mark P; Buchman, Steven R; Waljee, Jennifer F

2015-01-01

Health care policy makers are increasingly encouraging comparative effectiveness research. Little is known regarding comparative studies among children with cleft lip and/or palate. Cleft lip and/or palate profoundly influences self-perception and social functioning, and patient-reported outcomes provide a unique perspective on the success of reconstruction. The purpose of this study was to systematically review the literature regarding patient-reported outcomes among patients with cleft lip and/or palate. The authors reviewed articles from MEDLINE, Embase, and PsycInfo that examined the use of patient-reported outcome instruments for cleft lip and/or palate. Studies of patients with cleft lip and/or palate across any age that described the use of patient-completed measures in patient and control populations were included. A research librarian confirmed the search, and two independent, blinded reviewers performed full-text review. The authors identified 1979 articles and selected 30 for inclusion. Forty-two different assessment tools were used to analyze factors such as self-esteem, behavior, and social support. The Strengths and Difficulties Questionnaire was most commonly used (n = 7), followed by the Childhood Experience Questionnaire (n = 5), and the Satisfaction with Appearance survey (n = 4). Barriers to analysis included lack of standardization of survey administration, effect of publication bias, and variations in patient populations between individual studies. Comparative studies of patient-reported outcomes among patients with cleft lip and/or palate are infrequent. Many instruments exist to measure patient-reported outcomes in this population, but no specific standard exists. Identifying efficient and targeted forms of instrument selection and administration will enhance comparative studies among children with cleft lip and/or palate. Diagnostic, III.

Injection and adhesion palatoplasty: a preliminary study in a canine model.

PubMed

Martínez-Álvarez, Concepción; González-Meli, Beatriz; Berenguer-Froehner, Beatriz; Paradas-Lara, Irene; López-Gordillo, Yamila; Rodríguez-Bobada, Cruz; González, Pablo; Chamorro, Manuel; Arias, Pablo; Hilborn, Jöns; Casado-Gómez, Inmaculada; Martínez-Sanz, Elena

2013-08-01

Raising mucoperiosteal flaps in traditional palatoplasty impairs mid-facial growth. Hyaluronic acid-based hydrogels have been successfully tested for minimally invasive craniofacial bone generation in vivo as carriers of bone morphogenetic protein-2 (BMP-2). We aimed to develop a novel flapless technique for cleft palate repair by injecting a BMP-2 containing hydrogel. Dog pups with congenital cleft palate were either non-treated (n=4) or treated with two-flap palatoplasty (n=6) or with the proposed injection/adhesion technique (n=5). The experimental approach was to inject a hyaluronic acid-based hydrogel containing hydroxyapatite and BMP-2 subperiosteally at the cleft palate margins of pups aged six weeks. At week ten, a thin strip of the medial edge mucosa was removed and the margins were closed directly. Occlusal photographs and computed tomography (CT) scans were obtained up to week 20. Four weeks after the gel injection the cleft palate margins had reached the midline and engineered bone had enlarged the palatal bones. Removal of the medial edge mucosa and suturing allowed complete closure of the cleft. Compared to traditional palatoplasty, the injection/adhesion technique was easier, and the post-surgical recovery was faster. CT on week 20 revealed some overlapping or "bending" of palatal shelves in the two-flap repair group, which was not observed in the experimental nor control groups. A minimally invasive technique for cleft palate repair upon injectable scaffolds in a dog model of congenital cleft palate is feasible. Results suggest better growth of palatal bones. This represents an attractive clinical alternative to traditional palatoplasty for cleft palate patients. Copyright © 2013 Elsevier Inc. All rights reserved.

Interdisciplinary evaluation of consecutive patients with unilateral cleft lip and palate at age 6, 15, and 25 years: a concurrent standardized procedure and documentation by plastic surgeon; speech and language pathologist; ear, nose, and throat specialist; and orthodontist.

PubMed

Tindlund, Rolf S; Holmefjord, Anders; Eriksson, Jens-Christian Haug; Johnson, Gunnar E; Vindenes, Hallvard

2009-09-01

To evaluate surgical results, speech, hearing, and craniofacial morphology after primary cleft repair performed from 1973 to 1979. During the years 1972 to 1985, all primary cleft surgeries were performed by 1 plastic surgeon, using Tennison lip closure combined with a periosteoplasty on the clefted alveolus at age 3 months. By mobilizing mucoperiosteal flaps, bony bridges were induced in the alveolar process in approximately 60% of the cases. All patients had the soft palate closed at age 24 months by a pushback technique. All children with complete unilateral clefts without soft tissue bands (unilateral cleft lip and palate) primary operated on 1973 to 1979 were included in the material, except 3 patients with a syndrome and 2 patients of foreign ethnicity. The material involved 30 consecutive patients with unilateral cleft lip and palate (20 boys and 10 girls; 16 left-sided and 14 right-sided clefts) who were operated on. Standardized records including photos, radiographs, lateral cephalograms, plaster model, and recording of speech and hearing were collected according to the treatment protocol at age 6, 15, and approximately 25 years. Evaluation included craniofacial descriptive cephalometric analysis, dentoalveolar morphology, dentofacial aesthetics, speech concerning articulation and nasality, and hearing status. Number of surgical interventions after primary surgery was recorded. Secondary revisions and candidates for orthognathic surgery are reported. Ten consecutive patients (case nos. 11-20) are demonstrated as clinical reports. : This longitudinal study shows how a multidisciplinary evaluation adequate for intercenter comparison can be performed when standardized procedures, registrations, and documentations are available.

A Comparison of the Need for Speech Therapy After 2 Palatal Repair Techniques.

PubMed

Yen, Debra W; Nguyen, Dennis C; Skolnick, Gary B; Naidoo, Sybill D; Patel, Kamlesh B; Grames, Lynn Marty; Woo, Albert S

2017-03-01

Reconstruction of the levator musculature during cleft palate repair has been suggested to be important in long-term speech outcomes. In this study, we compare the need for postoperative speech therapy between 2 intravelar veloplasty techniques. Chart review was performed for patients with nonsyndromic cleft palate who underwent either primary Kriens or overlapping intravelar veloplasty before 18 months of age. All subjects completed a follow-up visit at approximately 3 years of age. Data obtained included documentation of ongoing or recommended speech therapy at age 3 years and reasons for speech therapy, which were categorized as cleft-related and non-cleft-related by a speech-language pathologist. One surgeon performed all Kriens procedures (n = 81), and the senior author performed all overlapping procedures (n = 25). Mean age at surgery (Kriens = 13.5 ± 1.4 months; overlapping = 13.1 ± 1.5 months; P = 0.188) and age at 3-year follow-up (Kriens = 3.0 ± 0.5 years; overlapping = 2.8 ± 0.5 years; P = 0.148) were equivalent in both groups. Cleft severity by Veau classification (P = 0.626), prepalatoplasty pure tone averages, (P = 0.237), pure tone averages at 3-year follow-up (P = 0.636), and incidence of prematurity (P = 0.190) were also similar between the 2 groups. At 3 years of age, significantly fewer overlapping intravelar veloplasty patients required cleft-related speech therapy (Kriens = 47%; overlapping = 20%; P = 0.015). The proportions of patients requiring non-cleft-related speech therapy were equivalent (P = 0.906). At 3 years of age, patients who received overlapping intravelar veloplasty were significantly less likely to need cleft-related speech therapy compared with patients who received Kriens intravelar veloplasty. Cleft severity, hearing loss, and prematurity at birth did not appear to explain the difference found in need for speech therapy.

Assisted eruption of impacted teeth into an alveolar bone graft in a patient with cleft lip and palate.

PubMed

Peamkaroonrath, Chonthicha; Manosudprasit, Montien; Godfrey, Keith

2008-11-01

To assist the eruption of impacted upper teeth into an alveolar bone graft in a patient with a unilateral cleft lip and palate. An 8-year-old Thai boy with left unilateral complete cleft lip and palate had the chief complaint of anterior crossbite. He presented with a mild skeletal 3, dental Class III subdivision malocclusion, anterior crossbite, left unilateral posterior crossbite, moderate crowding in the upper arch with impaction of upper the left lateral incisor (tooth 22) and canine (tooth 23). In the first phase of treatment the posterior crossbite was corrected with a removable appliance with a 3-way screw. In the second phase the impacted teeth were surgically exposed, moved into the alveolar bone graft and the teeth aligned with fixed appliances. The upper left lateral incisor was extracted because of its questionable longevity. The orthodontic treatment resulted in normal overjet, overbite and an acceptable facial profile. A prosthesis replaced tooth 22. Forced eruption of impacted teeth can be carried out successfully in the cleft patients after an appropriate treatment plan has been formulated and following preparation of alveolar bone graft in the cleft site.

Denture reconstruction of the edentulous upper jaw in cleft palate using implants--clinical report.

PubMed

Dostálová, T; Holakovský, J; Bartonová, M; Seydlová, M; Smahel, Z

2007-01-01

Early prosthodontic therapy (usually at around 18 years of age) often leads to early loss of teeth and in extreme cases to complete loss of dentition at between 40 and 50 years of age. This report describes the clinical features of two middle-aged cleft patients. Edentulous maxilla with cleft defect was treated with 6 implants supported by fixed appliance. Treatment of the whole dental arch on the basis of implants is currently frequently used as it provides a possibility of thorough functional and aesthetic therapy to a patient. The biomechanics of the reconstruction enables individual adjustment of the shape of the dental arch. The problem in cleft patients involves other diameter relations in the dental arch caused by the defect alone or also by affecting the growth of the maxillary segment by surgery. A potentially removable framework is therefore the main method of choice because the position of the implants must be prosthetically modified.

Metastatic squamous cell carcinoma of the gingiva appearing as a solitary branchial cyst carcinoma: diagnostic role of PET/CT.

PubMed

Zhang, Xiong-Xin; Zhao, Kui; Zhou, Shui-Hong; Wang, Qin-Ying; Liu, Jian-Hua; Lu, Zhong-Jie

2014-01-01

We herein present a case of a left cervical cystic mass, for which the initial pathological diagnosis was branchial cleft cyst carcinoma (following complete mass excision). Thorough postoperative examinations, including with FDG positron emission tomography/computed tomography (PET/CT), revealed a primary tumor in the retromolar region of the left mandible. A 52-year-old female presented with a 2-month history of a painless, progressively enlarged left-sided neck mass. Fine-needle aspiration biopsy suggested a branchial cleft cyst. Physical examination revealed a 3 × 3-cm smooth, tender mass in the upper-left neck and anterior border of the sternocleidomastoid muscle. Examination using nasendoscopy and a strobolaryngoscope revealed no abnormalities of the nasal cavity, nasopharynx, oropharynx, hypopharynx or larynx. MRI of the neck revealed a solitary, round, cystic mass under the left parotid gland. The mass was excised completely. Pathologic results indicated a branchial cleft cyst carcinoma. According to the diagnostic criteria for a branchial cleft cystic carcinoma, PET/CT was performed to detect the occult primary site. PET/CT revealed high FDG uptake in the tooth root of the left mandible. Frozen sections of the mass were indicative of moderate, differentiated squamous cell carcinoma. The carcinoma in the retromolar region of the left mandible was locally excised under general anesthesia. A partial left maxillectomy, partial mandibulectomy, and left radical neck dissection were performed. The patient received postoperative concurrent chemoradiotherapy, and was disease-free at the 8-month follow-up. True branchial cleft cyst carcinoma is rare: once diagnosed, it should be distinguished from metastatic cystic cervical lymph and occult primary carcinoma. FDG PET/CT is useful in the identification of occult primary tumor.

Long-Term Follow-Up Study of Young Adults Treated for Unilateral Complete Cleft Lip, Alveolus, and Palate by a Treatment Protocol Including Two-Stage Palatoplasty: Speech Outcomes

PubMed Central

Bittermann, Dirk; Janssen, Laura; Bittermann, Gerhard Koendert Pieter; Boonacker, Chantal; Haverkamp, Sarah; de Wilde, Hester; Van Der Heul, Marise; Specken, Tom FJMC; Koole, Ron; Kon, Moshe; Breugem, Corstiaan Cornelis; Mink van der Molen, Aebele Barber

2017-01-01

Background No consensus exists on the optimal treatment protocol for orofacial clefts or the optimal timing of cleft palate closure. This study investigated factors influencing speech outcomes after two-stage palate repair in adults with a non-syndromal complete unilateral cleft lip and palate (UCLP). Methods This was a retrospective analysis of adult patients with a UCLP who underwent two-stage palate closure and were treated at our tertiary cleft centre. Patients ≥17 years of age were invited for a final speech assessment. Their medical history was obtained from their medical files, and speech outcomes were assessed by a speech pathologist during the follow-up consultation. Results Forty-eight patients were included in the analysis, with a mean age of 21 years (standard deviation, 3.4 years). Their mean age at the time of hard and soft palate closure was 3 years and 8.0 months, respectively. In 40% of the patients, a pharyngoplasty was performed. On a 5-point intelligibility scale, 84.4% received a score of 1 or 2; meaning that their speech was intelligible. We observed a significant correlation between intelligibility scores and the incidence of articulation errors (P<0.001). In total, 36% showed mild to moderate hypernasality during the speech assessment, and 11%–17% of the patients exhibited increased nasalance scores, assessed through nasometry. Conclusions The present study describes long-term speech outcomes after two-stage palatoplasty with hard palate closure at a mean age of 3 years old. We observed moderate long-term intelligibility scores, a relatively high incidence of persistent hypernasality, and a high pharyngoplasty incidence. PMID:28573094

Facial attractiveness of patients with unilateral cleft lip and palate and of controls assessed by laypersons and professionals.

PubMed

Eichenberger, Martina; Staudt, Christine B; Pandis, Nikolaos; Gnoinski, Wanda; Eliades, Theodore

2014-06-01

The aim of the study was to identify differences in the aesthetic evaluation of profile and frontal photographs of (1) patients treated for complete left-sided cleft lip and palate and (2) control patients by laypeople and professionals. Left-side profile and frontal photographs of 20 adult patients treated for complete left-sided cleft lip and palate (10 men, 10 women, mean age: 20.5 years) and of 10 control patients with a class I occlusion (five men, five women, mean age: 22.1 years) were included in the study. The post-treatment photographs were evaluated by 15 adult laypeople, 14 orthodontists, and 10 maxillofacial surgeons. Each photograph was judged on a modified visual analogue scale (VA S, 0-10; 0 'very unattractive' to 10 'very attractive'). A four-level mixed model was fitted in which the VA S score was the dependent variable; cases, profession, view, and rater were independent variables. Compared with laypersons, orthodontists gave higher VA S scores (+0.69, 95% confidence interval (CI) [0.53, 0.84]; P < 0.001), followed by surgeons (+0.21, 95% CI [0.03, 0.38], P = 0.02). Controls were given significantly higher scores than patients with clefts for profile and frontal photographs (+1.97, 95% CI [1.60; 2.35], P < 0.001). No significant difference was found between the scores for the frontal and lateral views (P = 0.46). All the different rater panels were less satisfied with the facial aesthetics of patients with clefts compared with that of control patients. Further research should evaluate whether these findings correlate with patients' self-perception and to what extent it affects the patients' psychosocial well-being.

Transoral robotic-assisted laryngeal cleft repair in the pediatric patient.

PubMed

Leonardis, Rachel L; Duvvuri, Umamaheswar; Mehta, Deepak

2014-09-01

To assess the feasibility of performing robotic-assisted laryngeal cleft repair in the pediatric population. Retrospective chart review at a tertiary academic children's hospital. All patients underwent transoral robotic-assisted laryngeal cleft repair from March 2011 to June 2013. Demographics, robotic docking time, operative time, and postoperative course and swallowing function were collected and analyzed. Five children, three male and two female, underwent successful transoral robotic-assisted laryngeal cleft repair for closure of a type I laryngeal cleft. Mean age at time of surgery was 21.6 months (standard deviation 6.1 months; range, 15-29 months). From case 1 to case 5, robotic docking time (18-10 minutes), robotic operative time (102-36 minutes), and total operating room time (173-105 minutes) decreased. There were no complications with time until extubation (range, 2-3 days), length of intensive care unit stay (range, 3-4 days), and total hospital stay (range, 3-5 days) within acceptable range following laryngeal cleft repair. Modified barium swallow (two patients) or fiberoptic endoscopic evaluation of swallowing (three patients) was performed postoperatively, with all patients showing complete resolution of penetration and aspiration. In addition, all patients experienced subjective resolution of dysphagia and/or choking with feeds postoperatively. Transoral robotic-assisted laryngeal cleft repair may offer specific advantages over a traditional endoscopic approach. In our experience, the procedure was well tolerated and associated with definitive surgical cure in all patients. The scope of robotic technology continually expands and should be considered a feasible tool at an institution-based level. © 2014 The American Laryngological, Rhinological and Otological Society, Inc.

Sclerosteosis involving the temporal bone: histopathologic aspects.

PubMed

Nager, G T; Hamersma, H

1986-01-01

Sclerosteosis is a rare, potentially lethal, autosomal recessive, progressive craniotubular sclerosing bone dysplasia with characteristic facial and skeletal features. The temporal bone changes include a marked increase in overall size, extensive sclerosis, narrowing of the external auditory canal, and severe constriction of the internal auditory meatus, fallopian canal, eustachian tube, and middle ear cleft. Attenuation of the bony canals of the 9th, 10th, and 11th cranial nerves, reduction in size of the internal carotid artery, and severe obliteration of the sigmoid sinus and jugular bulb also occur. Loss of hearing, generally bilateral, is a frequent symptom. It often manifests in early childhood and initially is expressed as sound conduction impairment. Later, a sensorineural hearing loss and loss of vestibular nerve function often develop. Impairment of facial nerve function is another feature occasionally present at birth. In the beginning, a unilateral intermittent facial weakness may occur which eventually progresses to a bilateral permanent facial paresis. The histologic examination of the temporal bones from a patient with sclerosteosis explains the mechanisms involved in the progressive impairment of sound conduction and loss of cochlear, vestibular, and facial nerve function. There is a decrease of the arterial blood supply to the brain and an obstruction of the venous drainage from it. The histopathology reveals the obstacles to decompression of the middle ear cleft, ossicular chain, internal auditory and facial canals, and the risks, and in many instances the contraindications, to such procedures. On the other hand, decompression of the sigmoid sinus and jugular bulb should be considered as an additional life-saving procedure in conjunction with the prophylactic craniotomy recommended in all adult patients.

The development of the nursing care system for patients with cleft lip-palate and craniofacial deformities at Tawanchai Cleft Center, Srinagarind Hospital, Khon Kaen, Thailand.

PubMed

Pradubwong, Suteera; Pongpagatip, Sumalee; Volrathongchai, Kanittha; Chowchuen, Bowornsilp

2012-11-01

The highest incidence of cleft lip-palate and craniofacial deformities in Thailand occur in the Northeastern Region. There is the necessity for an interdisciplinary care team as well as the specialized care center with systematic coordinated care, thus "Tawanchai Cleft Center" is becoming a superior medical center for patients with cleft lip-palate and craniofacial deformities. Therefore, the development of the nursing care system for patients with cleft lip-palate and craniofacial deformities at Tawanchai Cleft Center, Srinagarind Hospital is extremely important and necessary. To develop the nursing care system appropriate for a super tertiary hospital (Tawanchai Cleft Center). It is a participation study which has 3 steps as follows, 1) Analyzing the situations and collecting the opinions of the 22 Out-patient Surgery Department staff and Tawanchai Cleft Center staff by using 6 questions, 2) Summarizing of the situation analysis from the meetings and the questionnaires, then using such summary as the guidelines for developing the nursing care system from January 2011 onwards, 3) evaluating the satisfaction after the 4 month development period (May-August 2011) with 106 caregivers by using 8 questions and being analyzed by the average value, percentage and standard deviation. 1) The nursing care system consisted of psychosocial care, breast feeding, counseling and other assistance as required. This various assistance responded to the patient/family problems by following the treatment guideline of the multidisciplinary team which uses the continuous evaluation processes for the holistic patient/family care. 2) The patients with complete cleft lip-palate were the most common type, found in 44 cases or 41.53 percent. The highest number of caregivers were mothers which were 68 percent; the average age of those mothers was 36 years old. The highest number of them finished elementary school at 43 percent and 40 percent were farmers. The satisfaction for the services of Tawanchai Cleft Center showed the average for each satisfaction subject all at very good level (x = 3.56, SD = 0.13). The care for patients with CLP and craniofacial deformities at Tawanchai Cleft Center Srinagarind Hospital has been developed in order to have the appropriate nursing care system to provide superior quality care, which provides patient-holistic care, as well as improving effective accessibility to the services. Thus, the patients/caregivers who are satisfied with given services, get continuing monitoring and treatment and are able to live their lives in the society happily.

[A case of diprosopus in the cat].

PubMed

Aharon, D C; Wouda, W; van Weelden, E

1986-06-15

A case of diprosopus in a spontaneously delivered live-born kitten is reported. All facial components were completely duplicated. Fusion of the skulls had occurred in the temporal region; a single ear was present at the site of fusion. Additional defects were a cleft lip in one face and cleft palates in both faces. The cerebral hemispheres and arterior portions of the brain stem were completely duplicated, whereas the cerebellum and caudal brain stem were partially duplicated. The pathogenesis and aetiology are discussed. It is believed that disprosopus originates during the (pre)gastrulation stage of embryonic development, either by coalescence of two embryonic fields following a double process of gastrulation or by bifurcation of the axial mesoderm during a single gastrulation.

Mechanical coupling in myosin V: a simulation study

PubMed Central

Ovchinnikov, Victor; Trout, Bernhardt L.

2009-01-01

Myosin motor function depends on the interaction between different domains that transmit information from one part of the molecule to another. The inter-domain coupling in myosin V is studied with Restrained Targeted Molecular Dynamics (RTMD) using an all-atom representation in explicit solvent. To elucidate the origin of the conformational change due to the binding of ATP, targeting forces are applied to small sets of atoms (the forcing sets, FS) in the direction of their displacement from the rigor conformation, which has a closed actin-binding cleft, to the post-rigor conformation, in which the cleft is open. The ‘minimal’ FS that results in extensive structural changes in the overall myosin conformation is comprised of the ATP, Switch 1, and the nearby HF, HG and HH helices. Addition of switch 2 to the forcing set is required to achieve a complete opening of the actin-binding cleft. The RTMD simulations reveal the mechanical coupling pathways between (i) the nucleotide-binding pocket (NBP) and the actin-binding cleft, (ii) the NBP and the converter, and (iii) the actin-binding cleft and the converter. Closing of the NBP due to ATP binding is tightly coupled to the opening of the cleft, and leads to the rupture of a key hydrogen bond (F441N/A684O) between switch 2 and the SH1 helix. The actin-binding cleft may mediate the rupture of this bond via a connection between the HW helix, the Relay helix, and Switch 2. The findings are consistent with experimental studies and a recent normal mode analysis. The present method is expected to be useful more generally in studies of inter-domain coupling in proteins. PMID:19853615

Mechanical coupling in myosin V: a simulation study.

PubMed

Ovchinnikov, Victor; Trout, Bernhardt L; Karplus, Martin

2010-01-29

Myosin motor function depends on the interaction between different domains that transmit information from one part of the molecule to another. The interdomain coupling in myosin V is studied with restrained targeted molecular dynamics using an all-atom representation in explicit solvent. To elucidate the origin of the conformational change due to the binding of ATP, targeting forces are applied to small sets of atoms (the forcing sets, FSs) in the direction of their displacement from the rigor conformation, which has a closed actin-binding cleft, to the post-rigor conformation, in which the cleft is open. The "minimal" FS that results in extensive structural changes in the overall myosin conformation is composed of ATP, switch 1, and the nearby HF, HG, and HH helices. Addition of switch 2 to the FS is required to achieve a complete opening of the actin-binding cleft. The restrained targeted molecular dynamics simulations reveal the mechanical coupling pathways between (i) the nucleotide-binding pocket (NBP) and the actin-binding cleft, (ii) the NBP and the converter, and (iii) the actin-binding cleft and the converter. Closing of the NBP due to ATP binding is tightly coupled to the opening of the cleft and leads to the rupture of a key hydrogen bond (F441N/A684O) between switch 2 and the SH1 helix. The actin-binding cleft may mediate the rupture of this bond via a connection between the HW helix, the relay helix, and switch 2. The findings are consistent with experimental studies and a recent normal mode analysis. The present method is expected to be useful more generally in studies of interdomain coupling in proteins.

The Cleft Care UK study. Part 4: perceptual speech outcomes

PubMed Central

Sell, D; Mildinhall, S; Albery, L; Wills, A K; Sandy, J R; Ness, A R

2015-01-01

Structured Abstract Objectives To describe the perceptual speech outcomes from the Cleft Care UK (CCUK) study and compare them to the 1998 Clinical Standards Advisory Group (CSAG) audit. Setting and sample population A cross-sectional study of 248 children born with complete unilateral cleft lip and palate, between 1 April 2005 and 31 March 2007 who underwent speech assessment. Materials and methods Centre-based specialist speech and language therapists (SLT) took speech audio–video recordings according to nationally agreed guidelines. Two independent listeners undertook the perceptual analysis using the CAPS-A Audit tool. Intra- and inter-rater reliability were tested. Results For each speech parameter of intelligibility/distinctiveness, hypernasality, palatal/palatalization, backed to velar/uvular, glottal, weak and nasalized consonants, and nasal realizations, there was strong evidence that speech outcomes were better in the CCUK children compared to CSAG children. The parameters which did not show improvement were nasal emission, nasal turbulence, hyponasality and lateral/lateralization. Conclusion These results suggest that centralization of cleft care into high volume centres has resulted in improvements in UK speech outcomes in five-year-olds with unilateral cleft lip and palate. This may be associated with the development of a specialized workforce. Nevertheless, there still remains a group of children with significant difficulties at school entry. PMID:26567854

The Cleft Care UK study. Part 4: perceptual speech outcomes.

PubMed

Sell, D; Mildinhall, S; Albery, L; Wills, A K; Sandy, J R; Ness, A R

2015-11-01

To describe the perceptual speech outcomes from the Cleft Care UK (CCUK) study and compare them to the 1998 Clinical Standards Advisory Group (CSAG) audit. A cross-sectional study of 248 children born with complete unilateral cleft lip and palate, between 1 April 2005 and 31 March 2007 who underwent speech assessment. Centre-based specialist speech and language therapists (SLT) took speech audio-video recordings according to nationally agreed guidelines. Two independent listeners undertook the perceptual analysis using the CAPS-A Audit tool. Intra- and inter-rater reliability were tested. For each speech parameter of intelligibility/distinctiveness, hypernasality, palatal/palatalization, backed to velar/uvular, glottal, weak and nasalized consonants, and nasal realizations, there was strong evidence that speech outcomes were better in the CCUK children compared to CSAG children. The parameters which did not show improvement were nasal emission, nasal turbulence, hyponasality and lateral/lateralization. These results suggest that centralization of cleft care into high volume centres has resulted in improvements in UK speech outcomes in five-year-olds with unilateral cleft lip and palate. This may be associated with the development of a specialized workforce. Nevertheless, there still remains a group of children with significant difficulties at school entry. © The Authors. Orthodontics & Craniofacial Research Published by John Wiley & Sons Ltd.

Evaluating the Use of Cleft Lip and Palate 3D-Printed Models as a Teaching Aid.

PubMed

AlAli, Ahmad B; Griffin, Michelle F; Calonge, Wenceslao M; Butler, Peter E

Visualization tools are essential for effective medical education, to aid students understanding of complex anatomical systems. Three dimensional (3D) printed models are showing a wide-reaching potential in the field of medical education, to aid the interpretation of 2D imaging. This study investigates the use of 3D-printed models in educational seminars on cleft lip and palate, by comparing integrated "hands-on" student seminars, with 2D presentation seminar methods. Cleft lip and palate models were manufactured using 3D-printing technology at the medical school. Sixty-seven students from two medical schools participated in the study. The students were randomly allocated to 2 groups. Knowledge was compared between the groups using a multiple-choice question test before and after the teaching intervention. Group 1 was the control group with a PowerPoint presentation-based educational seminar and group 2 was the test group, with the same PowerPoint presentation, but with the addition of a physical demonstration using 3D-printed models of unilateral and bilateral cleft lips and palate. The level of knowledge gained was established using a preseminar and postseminar assessment, in 2 different institutions, where the addition of the 3D-printed model resulted in a significant improvement in the mean percentage of knowledge gained (44.65% test group; 32.16%; control group; p = 0.038). Student experience was assessed using a postseminar survey, where students felt the 3D-printed model significantly improved the learning experience (p = 0.005) and their visualization (p = 0.001). This study highlights the benefits of the use of 3D-printed models as visualization tools in medical education and the potential of 3D-printing technology to become a standard and effective tool in the interpretation of 2D imaging. Copyright © 2018 Association of Program Directors in Surgery. Published by Elsevier Inc. All rights reserved.

Evaluation of 5-year-old children with complete cleft lip and palate: Multicenter study. Part 2: Functional results.

PubMed

Dissaux, Caroline; Grollemund, Bruno; Bodin, Frédéric; Picard, Arnaud; Vazquez, Marie-Paule; Morand, Béatrice; James, Isabelle; Kauffmann, Isabelle; Bruant-Rodier, Catherine

2016-02-01

Cleft surgery is marked by all the controversies and the multiplication of protocols, as it has been shown by the Eurocleft study. The objective of this pilot study is to start a comparison and analyzing procedure between primary surgical protocols in French centers. Four French centers with different primary surgical protocols for cleft lip and palate repair, have accepted to be involved in this retrospective study. In each center, 20 consecutive patients with complete cleft lip and palate (10 UCLP, 10 BCLP per center), non syndromic, have been evaluated at a mean age of 5 [range, 4-6]. In this second part, maxillary growth and palatine morphology were assessed on clinical examination and on dental casts (Goslon score). Speech was also evaluated clinically (Borel-maisonny classification) and by Aerophonoscope. Veau-Wardill-Killner palatoplasty involves a higher rate of transversal maxillary deficiency and retromaxillary. The fistula rate is statistically lower with tibial periosteum graft hard palate closure but this technique seems to give retromaxillary. Malek and Talmant two-stage-palatoplasty techniques reach Goslon scores of 1 or 2. Considering speech, Sommerlad intravelar veloplasty got higher outcomes. Primary results. Extension to other centers required. The two-stage palatoplasty, including a Sommerlad intravelar veloplasty seems to have the less negative impact on maxillary growth, and to give good speech outcomes. Therapeutic study. Level III/retrospective multicenter comparative study. Copyright © 2015 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Complete occipitalization of the atlas with bilateral external auditory canal atresia.

PubMed

Dolenšek, Janez; Cvetko, Erika; Snoj, Žiga; Meznaric, Marija

2017-09-01

Fusion of the atlas with the occipital bone is a rare congenital dysplasia known as occipitalization of the atlas, occipitocervical synostosis, assimilation of the atlas, or atlanto-occipital fusion. It is a component of the paraxial mesodermal maldevelopment and commonly associated with other dysplasias of the craniovertebral junction. External auditory canal atresia or external aural atresia is a rare congenital absence of the external auditory canal. It occurs as the consequence of the maldevelopment of the first pharyngeal cleft due to defects of cranial neural crest cells migration and/or differentiation. It is commonly associated with the dysplasias of the structures derived from the first and second pharyngeal arches including microtia. We present the coexistence of the occipitalization of the atlas and congenital aural atresia, an uncommon combination of the paraxial mesodermal maldevelopment, and defects of cranial neural crest cells. The association is most probably syndromic as minimal diagnostic criteria for the oculoariculovertebral spectrum are fulfilled. From the clinical point of view, it is important to be aware that patients with microtia must obtain also appropriate diagnostic imaging studies of the craniovetebral junction due to eventual concomitant occipitalization of the atlas and frequently associated C1-C2 instability.

[Surgical treatment of first branchial cleft anomaly].

PubMed

Xiao, Hongjun; Kong, Weijia; Gong, Shusheng; Wang, Jibao; Liu, Shiying; Shi, Hong

2005-10-01

To identify the clinical and anatomical presentations and to discuss the guidelines for surgical management of anomalies of the first branchial cleft. Twenty-one patients with first branchial cleft anomalies were treated in our department between January 1994 and December 2004, their clinical data were retrospectively analysed. Surgery was performed on all patients. Among them 13 were males and 8 females, ranging in age from 1.5 to 33 years with an average of 15 years. Anatomically, 3 types of first branchial cleft anomalies were identified: fistulas (n = 17), cysts (n = 2), and fistula combined with cyst (n = 2). Before definitive surgery, soma patients (n = 4) underwent incision and drainage for infection owing to the difficulties in diagnosing this anomaly. Methylthioninium Chloride was used in almost all cases for tracking the fistulous during operation. Wide exposure is necessary in many cases,and a standard parotidectomy incision allows adequate exposure of the anomaly and preservation of the facial nerve. Complete removal without complications depends on a good understanding of regional embryogenesis, an awareness of the different anatomical presentations, and a readiness to identify and protect the facial nerve during resection.

Long-term stability of intra-oral maxillary distraction in unilateral cleft lip and palate: a case report.

PubMed

Nevzatoğlu, Sirin; Küçükkeleş, Nazan; Güzel, Zeki

2013-11-01

This case report presents short and long-term treatment results of a unilateral cleft lip and palate patient treated with a modified intra-oral tooth-bone borne distraction appliance. The chief complaints of a 16 year-old, unilateral cleft lip and palate patient were poor facial aesthetics, crowding and a fistula. Severe maxillary retrognathism was treated via distraction osteogenesis of the maxilla and performed using an intra-oral tooth-bone borne appliance. Treatment continued to completion with a multibracket system. At an eight-year review following the distraction procedure, the short and long-term results were determined cephalometrically. Following the distraction, A-point advanced 7 mm, 2 mm of which relapsed during fixed appliance treatment. At the end of the active treatment, the patient's skeletal and dental Class III relationship improved to Class I, which was preserved at the long-term review. The profile was markedly improved by the distraction osteogenesis. In cases of severe maxillary retrognathism as a result of a cleft lip and palate, maxillary distraction osteogenesis provides a viable alternative to orthognathic surgery.

Sacrococcygeal teratoma in a female newborn with clinical features of trisomy 13: a case report from Central Africa.

PubMed

Lubala, Toni Kasole; Mukuku, Olivier; Shongo, Mick Pongombo; Mutombo, Augustin Mulangu; Lubala, Nina; Luboya, Oscar Numbi; Lukusa-Tshilobo, Prosper

2015-01-01

The objective of this report is to describe the first patient presenting clinical features of trisomy 13 in association with a sacrococcygeal teratoma. We present the case of a Congolese female infant born with bilateral cleft lip and palate, hypotelorism, microcephaly, and capillary hemangioma on her face. She presented with a large sacrococcygeal mass (15.0 cm ×12.0 cm ×5.0 cm) with a cystic consistency and a positive transillumination. This observation suggests that overexpression of certain genes on chromosome 13 may lead to tumor formation from remnant cells of Hensen's node.

A rare case of complete second arch branchial fistula in a 7-year-old child.

PubMed

Shankar, Venkateswara Gomathi; Babu, Thirunavukkarasu Arun; Swami, Hartimath Basavanand

2012-07-01

Branchial fistulae are formed due to the abnormal persistence of the embryonic branchial clefts. Complete branchial fistula with internal and external opening is extremely rare. We report a rare case of complete second arch branchial fistulae in a 7-year-old boy, which was confirmed by a fistulogram. The tract was completely excised and the patient was successfully treated.

Congenital Abnormalities of the Mouth

PubMed Central

Lindsay, W. K.

1976-01-01

The spectrum of congenital malformations in the mouth is broad. The incidence of any one condition is not great, with the exception of the anomalies of cleft lip and cleft palate. Most of these oral malformations can produce urgent problems in neonatal management for the physician, including the care of the distraught parents. Considerable plastic and reconstructive surgery is involved in the subsequent management. The modern key to the complete successful management of these patients is the participation of a multidiscipline team. ImagesFig. 1p65-aFig. 2Fig. 3Fig. 4Fig. 5 PMID:21308081

Disruption of cleft palate repair following the use of the laryngeal mask airway.

PubMed

Somerville, N S; Fenlon, S; Boorman, J; Abbot, M

2004-04-01

A 55-year-old man was admitted for routine examination of ears with insertion of grommets under general anaesthesia. At 2 years of age he had undergone successful repair of cleft lip and palate. A reinforced laryngeal mask airway was employed to maintain the airway. Postoperatively, it was evident he had suffered complete disruption of the soft palate repair, leading to velopharyngeal insufficiency with nasal regurgitation of fluids. We discuss the possible aetiology, having found no such reported injury pattern documented in the literature.

Mechanisms of palatal epithelial seam disintegration by Transforming Growth Factor (TGF)-β3

PubMed Central

Ahmed, Shaheen; Liu, Chang-Chih; Nawshad, Ali

2007-01-01

TGFβ3 signaling initiates and completes sequential phases of cellular differentiation that is required for complete disintegration of the palatal medial edge seam, that progresses between 14 to 17 embryonic days in the murine system, which is necessary in establishing confluence of the palatal stroma. Understanding the cellular mechanism of palatal MES disintegration in response to TGFβ3 signaling will result in new approaches to defining the causes of cleft palate and other facial clefts that may result from failure of seam disintegration. We have isolated MES primary cells to study the details of MES disintegration mechanism by TGFβ3 during palate development using several biochemical and genetic approaches. Our results demonstrate a novel mechanism of MES disintegration where MES, independently yet sequentially, undergoes cell cycle arrest, cell migration and apoptosis to generate immaculate palatal confluency during palatogenesis in response to robust TGFβ3 signaling. The results contribute to a missing fundamental element to our base knowledge of the diverse roles of TGFβ3 in functional and morphological changes that MES undergo during palatal seam disintegration. We believe that our findings will lead to more effective treatment of facial clefting. PMID:17698055

Craniofacial orthodontics and postgraduate orthodontic training in Nigeria.

PubMed

Isiekwe, G I; Oguchi, C O; daCosta, O O; Utomi, I L

2016-01-01

Craniofacial orthodontics has been shown to be a critical component of the care of patients with craniofacial anomalies such as cleft lip and palate. Thus, the purpose of this study was to assess the perceptions and clinical experience in cleft and craniofacial care, of orthodontic residents in Nigeria. Questionnaires were sent out to orthodontic residents in the six Postgraduate Orthodontic Training Centers in the country at that time. The questionnaires were self-administered and covered areas in beliefs in cleft care and the clinical experience and challenges faced by the residents in the provision of craniofacial orthodontic care at their various institutions. Thirty-three respondents returned completed questionnaires, with a response rate of 97%. All the respondents believed that residents should be involved in cleft and craniofacial care. Postnatal counseling was the clinical procedure in which the residents reported the highest level of clinical experience (47.4%). The least clinical experience was recorded in pre-bone graft orthodontics (7.4%) and orthodontic preparation for orthognathic surgery (5.5%). Some of the challenges highlighted by the residents were low patients turn out for orthodontic care and the absence of multidisciplinary treatment for craniofacial patients in their centers. Orthodontic residents in Nigeria believe that they should be involved in the management of patients with craniofacial anomalies and cleft lip and palate. However, majority of the residents have limited clinical experience in the management of these patients. A lot more needs to be done, to expose orthodontic residents in training, to all aspects of the orthodontic and multidisciplinary team care required for the cleft/craniofacial patient.

Three-Dimensional Soft Tissue Nasal Changes After Nasoalveolar Molding and Primary Cheilorhinoplasty in Infants With Unilateral Cleft Lip and Palate.

PubMed

Mancini, Laura; Gibson, Travis L; Grayson, Barry H; Flores, Roberto L; Staffenberg, David; Shetye, Pradip R

2018-01-01

To quantify 3-dimensional (3D) nasal changes in infants with unilateral cleft lip with or without cleft palate (UCL±P) treated by nasoalveolar molding (NAM) and cheilorhinoplasty and compare to noncleft controls. Retrospective case series of infants treated with NAM and primary cheilorhinoplasty between September, 2012 and July, 2016. Infants were included if they had digital stereophotogrammetric records at initial presentation (T1), completion of NAM (T2), and following primary cheilorhinoplasty (T3). Images were oriented in 3dMD Vultus software, and 16 nasolabial points identified. Twenty consecutively treated infants with UCL±P. Nasoalveolar molding and primary cheilorhinoplasty. Anthropometric measures of nasal symmetry and morphology were compared in the treatment group between time points using paired Student t tests. Postsurgical nasal morphology was compared to noncleft controls. Nasal tip protrusion increased, and at T3 was 2.64 mm greater than noncleft controls. Nasal base width decreased on the cleft side by 4.01 mm after NAM and by 6.73 mm after cheilorhinoplasty. Columellar length of the noncleft to cleft side decreased from 2:1 to 1:1 following NAM. Significant improvements in subnasale, columella, and nasal tip deviations from midsagittal plane were observed. Treatment improved symmetry of the alar morphology angle and the nasal base-columella angle between cleft and noncleft sides. Three-dimensional analysis of UCL±P patients demonstrated significant improvements in nasal projection, columella length, nasal symmetry, and nasal width. Compared to noncleft controls, nasal form was generally corrected, with overcorrection of nasal tip projection, columella angle, and outer nasal widths.

Assessment and Management of Psychosocial Needs: Social Work Utilization in Comprehensive Cleft Team Care.

PubMed

Kaye, Alison; Lybrand, Sandra; Chew, William L

2018-01-01

To determine family-reported psychosocial stressors and social worker assessments and interventions within a comprehensive cleft team. Single-institution prospective provider-completed survey. Four hundred one families seen by cleft team social worker over a 7-month period. Most families (n = 331; 83%) participated in the team social work assessment. At least 1 active psychosocial stressor was reported by 238 (72%) families, with 63 (19%) families reported 3 or more stressors. There were 34 types of stressors reported. Most common were financial strain, young age of patient, new cleft diagnosis, and distance from clinic (57% of families live over an hour away). Family structure and home environment were assessed in detail for 288 (87%) families. Detailed assessments for access to care and behavioral/developmental issues also figured prominently. Social work interventions were provided in 264 (80%) of the visits, of which 91 were for families of new patients with over half who had infants less than 3 months old. Of the 643 interventions provided, the most frequent were parent mental health screens and counseling, early intervention referrals, transportation assistance, securing local hotel discounts, orthodontic referrals, and orthodontic cost coverage. Approximately 10% of encounters required follow-up contact related to the psychosocial concerns identified in clinic. The inclusion of a cleft team social worker is a critical component of comprehensive cleft team care as evidenced by the large proportion of families who required assistance. Ongoing social work assessments are recommended for each patient to help address the variety of psychosocial stressors families face.

Caregiver responses to early cleft palate care: A mixed method approach.

PubMed

Sischo, Lacey; Clouston, Sean A P; Phillips, Ceib; Broder, Hillary L

2016-05-01

This study sought to understand caregivers' (CGs') responses to early cleft lip/palate care for their infants. A prospective, mixed methods multicenter longitudinal study was conducted among CGs (N = 118) seeking treatment for their infants' cleft lip and palate or cleft lip only at 1 of 6 cleft treatment centers in the United States. Participants were in 1 of 2 treatment groups: traditional care only or nasoalveolar molding (NAM) plus traditional care. The CGs completed semistructured interviews and standardized questionnaires assessing psychosocial well-being and family impact at 3 time points: the beginning of treatment (∼1 month of age), prelip surgery (∼3-5 months of age), and postpalate surgery (∼12-13 months of age). Multilevel modeling was used to longitudinally assess CGs' psychosocial outcomes. Although the first year was demanding for all CGs, NAM onset and the child's lip surgery were particularly stressful times. CGs used optimism, problem-solving behavior, and social support to cope with this stress. Qualitatively, CGs' ability to balance cleft treatment demands with their psychosocial resources and coping strategies influenced family adaptation. Qualitative and quantitative results indicated CGs of NAM-treated infants experienced more rapid declines in anxiety and depressive symptoms and better coping skills over time than CGs whose infants had traditional care. CGs of NAM-treated infants experienced more positive psychosocial outcomes than CGs whose infants had traditional care. Results from the mixed model support the family adjustment and adaptation response model as used in pediatric chronic condition research. (c) 2016 APA, all rights reserved).

The effect of various factors on the dental arch relationship in non-syndromic unilateral cleft lip and palate children assessed by new approach: a retrospective study.

PubMed

Haque, Sanjida; Alam, Mohammad Khursheed; Khamis, Mohd Fadhli

2017-05-06

Cleft lip and palate (CLP) is one of the most common birth defects. Multiple factors are believed to be responsible for an unfavorable dental arch relationship in CLP. Facial growth (maxillary) retardation, which results in class III malocclusion, is the primary challenge that CLP patients face. Phenotype factors and postnatal treatment factors influence treatment outcomes in unilateral cleft lip and palate (UCLP) children, which has led to a great diversity in protocols and surgical techniques by various cleft groups worldwide. The aim of this study was to illustrate the dental arch relationship (DAR) and palatal morphology (PM) of UCLP in Bangladeshi children and to explore the various factors that are responsible for poor DAR and PM. Dental models of 84 subjects were taken before orthodontic treatment and alveolar bone grafting. The mean age was 7.69 (SD 2.46) years. The DAR and PM were assessed blindly by five raters using the EUROCRAN index (EI). Kappa statistics was used to evaluate the intra- and inter-examiner agreement, chi square was used to assess the associations, and logistic regression analysis was used to explore the responsible factors that affect DAR and PM. The mean EUROCRAN scores were 2.44 and 1.93 for DAR and PM, respectively. Intra- and inter-examiner agreement was moderate to very good. Using crude and stepwise backward regression analyses, significant associations were found between the modified Millard technique (P = 0.047, P = 0.034 respectively) of cheiloplasty and unfavorable DAR. Complete UCLP (P = 0.017) was also significantly correlated with unfavorable DAR. The PM showed a significant association with the type of cleft, type of cheiloplasty and type of palatoplasty. This multivariate study determined that the complete type of UCLP and the modified Millard technique of cheiloplasty had significantly unfavorable effects on both the DAR and PM.

Speech outcome after early repair of cleft soft palate using Furlow technique.

PubMed

Abdel-Aziz, Mosaad

2013-01-01

The earlier closure of palatal cleft is the better the speech outcome and the less compensatory articulation errors, however dissection on the hard palate may interfere with facial growth. In Furlow palatoplasty, dissection on the hard palate is not needed and surgery is usually limited to the soft palate, so the technique has no deleterious effect on the facial growth. The aim of this study was to assess the efficacy of Furlow palatoplasty technique on the speech of young infants with cleft soft palate. Twenty-one infants with cleft soft palate were included in this study, their ages ranged from 3 to 6 months. Their clefts were repaired using Furlow technique. The patients were followed up for at least 4 years; at the end of the follow up period they were subjected to flexible nasopharyngoscopy to assess the velopharyngeal closure and speech analysis using auditory perceptual assessment. Eighteen cases (85.7%) showed complete velopharyngeal closure, 1 case (4.8%) showed borderline competence, and 2 cases (9.5%) showed borderline incompetence. Normal resonance has been attained in 18 patients (85.7%), and mild hypernasality in 3 patients (14.3%), no patients demonstrated nasal emission of air. Speech therapy was beneficial for cases with residual hypernasality; no cases needed secondary corrective surgery. Furlow palatoplasty at a younger age has favorable speech outcome with no detectable morbidity. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

A rare case of complete second arch branchial fistula in a 7-year-old child

PubMed Central

Shankar, Venkateswara Gomathi; Babu, Thirunavukkarasu Arun; Swami, Hartimath Basavanand

2012-01-01

Branchial fistulae are formed due to the abnormal persistence of the embryonic branchial clefts. Complete branchial fistula with internal and external opening is extremely rare. We report a rare case of complete second arch branchial fistulae in a 7-year-old boy, which was confirmed by a fistulogram. The tract was completely excised and the patient was successfully treated. PMID:23833506

A Scandcleft randomised trials of primary surgery for unilateral cleft lip and palate: 1. Planning and management.

PubMed

Semb, Gunvor; Enemark, Hans; Friede, Hans; Paulin, Gunnar; Lilja, Jan; Rautio, Jorma; Andersen, Mikael; Åbyholm, Frank; Lohmander, Anette; Shaw, William; Mølsted, Kirsten; Heliövaara, Arja; Bolund, Stig; Hukki, Jyri; Vindenes, Hallvard; Davenport, Peter; Arctander, Kjartan; Larson, Ola; Berggren, Anders; Whitby, David; Leonard, Alan; Neovius, Erik; Elander, Anna; Willadsen, Elisabeth; Bannister, R Patricia; Bradbury, Eileen; Henningsson, Gunilla; Persson, Christina; Eyres, Philip; Emborg, Berit; Kisling-Møller, Mia; Küseler, Annelise; Granhof Black, Birthe; Schöps, Antje; Bau, Anja; Boers, Maria; Andersen, Helene Søgaard; Jeppesen, Karin; Marxen, Dorte; Paaso, Marjukka; Hölttä, Elina; Alaluusua, Suvi; Turunen, Leena; Humerinta, Kirsti; Elfving-Little, Ulla; Tørdal, Inger Beate; Kjøll, Lillian; Aukner, Ragnhild; Hide, Øydis; Feragen, Kristin Billaud; Rønning, Elisabeth; Skaare, Pål; Brinck, Eli; Semmingsen, Ann-Magritt; Lindberg, Nina; Bowden, Melanie; Davies, Julie; Mooney, Jeanette; Bellardie, Haydn; Schofield, Nina; Nyberg, Jill; Lundberg, Maria; Karsten, Agneta Linder-Aronson; Larson, Margareta; Holmefjord, Anders; Reisæter, Sigvor; Pedersen, Nina-Helen; Rasmussen, Therese; Tindlund, Rolf; Sæle, Paul; Blomhoff, Reidunn; Jacobsen, Gry; Havstam, Christina; Rizell, Sara; Enocson, Lars; Hagberg, Catharina; Najar Chalien, Midia; Paganini, Anna; Lundeborg, Inger; Marcusson, Agneta; Mjönes, Anna-Britta; Gustavsson, Annica; Hayden, Christine; McAleer, Eilish; Slevan, Emma; Gregg, Terry; Worthington, Helen

2017-02-01

Longstanding uncertainty surrounds the selection of surgical protocols for the closure of unilateral cleft lip and palate, and randomised trials have only rarely been performed. This paper is an introduction to three randomised trials of primary surgery for children born with complete unilateral cleft lip and palate (UCLP). It presents the protocol developed for the trials in CONSORT format, and describes the management structure that was developed to achieve the long-term engagement and commitment required to complete the project. Ten established national or regional cleft centres participated. Lip and soft palate closure at 3-4 months, and hard palate closure at 12 months served as a common method in each trial. Trial 1 compared this with hard palate closure at 36 months. Trial 2 compared it with lip closure at 3-4 months and hard and soft palate closure at 12 months. Trial 3 compared it with lip and hard palate closure at 3-4 months and soft palate closure at 12 months. The primary outcomes were speech and dentofacial development, with a series of perioperative and longer-term secondary outcomes. Recruitment of 448 infants took place over a 9-year period, with 99.8% subsequent retention at 5 years. The series of reports that follow this introductory paper include comparisons at age 5 of surgical outcomes, speech outcomes, measures of dentofacial development and appearance, and parental satisfaction. The outcomes recorded and the numbers analysed for each outcome and time point are described in the series. ISRCTN29932826.

Speech and Communication Disorders

MedlinePlus

... to being completely unable to speak or understand speech. Causes include Hearing disorders and deafness Voice problems, ... or those caused by cleft lip or palate Speech problems like stuttering Developmental disabilities Learning disorders Autism ...

Engineering out motion: introduction of a de novo disulfide bond and a salt bridge designed to close a dynamic cleft on the surface of cytochrome b5.

PubMed

Storch, E M; Daggett, V; Atkins, W M

1999-04-20

A previous molecular dynamics (MD) simulation of cytochrome b5 (cyt b5) at 25 degrees C displayed localized dynamics on the surface of the protein giving rise to the periodic formation of a cleft that provides access to the heme through a protected hydrophobic channel [Storch and Daggett (1995) Biochemistry 34, 9682]. Here we describe the production and testing of mutants designed to prevent the cleft from opening using a combination of experimental and theoretical techniques. Two mutants have been designed to close the surface cleft: S18D to introduce a salt bridge and S18C:R47C to incorporate a disulfide bond. The putative cleft forms between two separate cores of the protein: one is structural in nature and can be monitored through the fluorescence of Trp 22, and the other binds the heme prosthetic group and can be tracked via heme absorbance. An increase in motion localized to the cleft region was observed for each protein, except for the disulfide-containing variant, in MD simulations at 50 degrees C compared to simulations at 25 degrees C. For the disulfide-containing variant, the cleft remained closed. Both urea and temperature denaturation curves were nearly identical for wild-type and mutant proteins when heme absorbance was monitored. In contrast, fluorescence studies revealed oxidized S18C:R47C to be considerably more stable based on the midpoints of the denaturation transitions, Tm and U1/2. Moreover, the fluorescence changes for each protein were complete at approximately 50 degrees C and a urea concentration of approximately 3.9 M, significantly below the temperature and urea concentration (62 degrees C, 5 M urea) required to observe heme release. In addition, solvent accessibility based on acrylamide quenching of Trp 22 was lower in the S18C:R47C mutant, particularly at 50 degrees C, before heme release [presented in the accompanying paper (58)]. The results suggest that a constraining disulfide bond can be designed to inhibit dynamic cleft formation on the surface of cyt b5. Located near the heme, the native dynamics of the cleft may be functionally important for protein-protein recognition and/or complex stabilization.

Surgical repair of a congenital sternal cleft in a cat.

PubMed

Schwarzkopf, Ilona; Bavegems, Valerie C A; Vandekerckhove, Peter M F P; Melis, Sanne M; Cornillie, Pieter; de Rooster, Hilde

2014-07-01

To describe the clinical findings, diagnosis, and treatment of an incomplete cleft of the 5th-8th sternebra and a cranioventral abdominal wall hernia in a 2 month old Ragdoll kitten and to evaluate the short- and long-term outcome. Clinical report. Ragdoll cat (n = 1), 2 months old. Sternal cleft was confirmed by thoracic radiographs. Computed tomography (CT) was used to plan an optimal surgical approach. A ventral median incision was made, starting at the 3rd sternebra and extended into the abdomen. Ostectomy of the proximal part of the 5th left sternebra was performed. Lateral periosteal flaps were created, unfolded, and absorbable monofilament sutures preplaced to facilitate closure and the repair was reinforced by 2 peristernal sutures. A bone graft was applied, and the free margin of the omentum was sutured to the cranial aspect of the wound. No major complications occurred. At 3 weeks, CT scan confirmed approximation of the hemisternebrae and at 10 months, complete fusion of the hemisternebrae had not occurred, but a strong connection of the sternal bars was present. Sternal cleft is a rare congenital abnormality that can be corrected surgically with favorable outcome. © Copyright 2014 by The American College of Veterinary Surgeons.

Cleft Lip and Palate Repair Using a Surgical Microscope.

PubMed

Kato, Motoi; Watanabe, Azusa; Watanabe, Shoji; Utsunomiya, Hiroki; Yokoyama, Takayuki; Ogishima, Shinya

2017-11-01

Cleft lip and palate repair requires a deep and small surgical field and is usually performed by surgeons wearing surgical loupes. Surgeons with loupes can obtain a wider surgical view, although headlights are required for the deepest procedures. Surgical microscopes offer comfort and a clear and magnification-adjustable surgical site that can be shared with the whole team, including observers, and easily recorded to further the education of junior surgeons. Magnification adjustments are convenient for precise procedures such as muscle dissection of the soft palate. We performed a comparative investigation of 18 cleft operations that utilized either surgical loupes or microscopy. Paper-based questionnaires were completed by staff nurses to evaluate what went well and what could be improved in each procedure. The operating time, complication rate, and scores of the questionnaire responses were statistically analyzed. The operating time when microscopy was used was not significantly longer than when surgical loupes were utilized. The surgical field was clearly shared with surgical assistants, nurses, anesthesiologists, and students via microscope-linked monitors. Passing surgical equipment was easier when sharing the surgical view, and preoperative microscope preparation did not interfere with the duties of the staff nurses. Surgical microscopy was demonstrated to be useful during cleft operations.

Distraction osteogenesis versus orthognathic surgery for the treatment of maxillary hypoplasia in cleft lip and palate patients: a systematic review.

PubMed

Austin, S L; Mattick, C R; Waterhouse, P J

2015-05-01

To compare the effectiveness of distraction osteogenesis to orthognathic surgery for the treatment of maxillary hypoplasia in individuals with cleft lip and palate. A systematic review of prospective randomized, quasi-randomized or controlled clinical trials. MEDLINE, EMBASE, Scopus, Web of Science, CINAHL, CENTRAL, trial registers and grey literature were searched. Hand searching of five relevant journals was completed. Two reviewers independently completed inclusion assessment. Data extraction and risk of bias assessment were completed by a single reviewer and checked by a second reviewer. Five publications all reporting different outcomes of a single randomized controlled trial are included within the review. The quality of the evidence was low with a high risk of bias. Both surgical interventions produce significant soft tissue improvement. Horizontal relapse of the maxilla was statistically significantly greater following orthognathic surgery. There was no statistically significant difference in speech and velo-pharyngeal function between the interventions. Maxillary distraction initially lowered social self-esteem, but this improved with time resulting in higher satisfaction with life in the long term. The low quality of evidence included within the review means there is insufficient evidence to conclude whether there is a difference in effectiveness between maxillary distraction and osteotomy for the treatment of cleft-related maxillary hypoplasia. There is a need for further high-quality randomized controlled trials to allow conclusive recommendations to be made. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Psychosocial Wellbeing of Parents of Children with Oral Clefts

PubMed Central

Nidey, Nichole; Moreno Uribe, Lina M.; Marazita, Mary M.; Wehby, George L.

2016-01-01

Background Parents of children with oral clefts may be impacted psychosocially in several ways, but empirical evidence remains relatively sparse. The aim of this study was to identify predictors of psychosocial wellbeing of parents of affected children. Methods The study included a total sample of 287 parents (171 mothers, 116 fathers) of children with oral clefts. Parents completed validated psychosocial instruments to measure social avoidance and distress, fear of negative evaluation scale, self-esteem and interpersonal support. Regression analysis was used to evaluate how selected child, parent, and household characteristics relate to psychosocial outcomes focusing on child’s cleft type, sex, and age, differences between mothers and fathers, marital status, and household income. Results Fathers had higher self-esteem than mothers (p=0.01) and lower concern of being negatively judged by others (p<0.0001) but also had lower perception of having someone to talk to about their problems (p=0.01). High household income was associated with greater self-esteem and perception of social support (<0.05). Parents of male affected children had greater perception of social support than parents of female affected children (p=0.04). No significant differences in parental psychosocial status measures were found by cleft type. Similarly, there is little evidence of changes with child age, except for an increase in parental distress and decline in self-esteem during mid adolescence (age 15–17 years). Conclusions The results indicate that mothers and fathers of children with oral clefts may differ in their psychosocial adjustment and that mothers may overall experience more psychosocial problems than fathers. Also, parents from less wealthy households may be at greater risk. Parental psychosocial status should be considered in holistic family-based treatment approaches to reduce burden on affected families and improve their wellbeing. PMID:26302988

Comparative Study of Folic Acid and α-Naphthoflavone on Reducing TCDD-Induced Cleft Palate in Fetal Mice.

PubMed

Yuan, Xingang; He, Xiaomeng; Zhang, Xuan; Liu, Cuiping; Wang, Chen; Qiu, Lin; Pu, Wei; Fu, Yuexian

2017-03-01

  Tocompare the effect of folic acid (FA) and α-naphthoflavone on 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD)-induced cleft palate in fetal mice.   Pregnant mice were randomly divided into seven groups. The mice treated with corn oil were used as a negative control. The mice in the other six groups were given a single dose of 28 μg/kg TCDD on GD 10 by gavage. For FA treatment, TCDD-treated mice were also dosed with 5, 10, and 15 mg/kg FA on GD 10, while for α-naphthoflavone treatment, the mice received a single dose of 50 μg/kg or 5 mg/kg α-naphthoflavone on GD 10.   Fetal mice palates were imaged using light and scanning electron microscopy on GD 13.5, GD 14.5, and GD 15.5, and cleft palate were recorded on GD 17.5. The expression of guanosine diphosphate dissociation inhibitor (GDI) in fetal mice palate on GD 15.5 was examined by immunohistochemistry.   TCDD successfully induced cleft palate. Ten mg/ml FA and 5 mg/ml α-naphthoflavone significantly reduced TCDD-induced cleft palate. FA and α-naphthoflavone partly reduced TCDD-induced cleft palate but did not affect the expression of Rho GDI.   FA and α-naphthoflavone may reduce the generation of reactive oxygen species, inhibit MEE apoptosis through anti-oxidation, and increase filopodia and MEE movement. This may result in restoration of the ultrastructure of the palatal surface to a normal state, leading to the fusion and formation of complete palate in TCDD-treated fetal mice.

Clefting of the Alveolus: Emphasizing the Distinction from Cleft Palate.

PubMed

Wirtz, Nicholas; Sidman, James; Block, William

2016-05-01

Oral clefting is one of the most common significant fetal abnormalities. Cleft lip and cleft palate have drastically different clinical ramifications and management from one another. A cleft of the alveolus (with or without cleft lip) can confuse the diagnostic picture and lead to a false assumption of cleft palate. The cleft alveolus should be viewed on the spectrum of cleft lip rather than be associated with cleft palate. This is made evident by understanding the embryological development of the midface and relevant terminology. Cleft alveolus carries significantly different clinical implications and treatment options than that of cleft palate. Accurately distinguishing cleft alveolus from cleft palate is crucial for appropriate discussions regarding the patient's care. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Cleft Lip and Palate

MedlinePlus

... Safe Videos for Educators Search English Español Cleft Lip and Cleft Palate KidsHealth / For Kids / Cleft Lip and Cleft Palate ... the back of your mouth. What's a Cleft Lip or Cleft Palate? The word cleft means a gap or split ...

Bartsocas-Papas syndrome with variable expressivity in an Egyptian family.

PubMed

Zaki, M S; Kamel, A K; Effat, L K; El-Ruby, M O

2012-01-01

Bartsocas-Papas syndrome (BPS) is an autosomal recessively inherited sublethal popliteal pterygium condition characterized by intrauterine or neonatal death, severe popliteal webbing, oligosyndactyly, ankyloblepharon, orofacial clefts, intraoral filiform bands and genital anomalies. Internal organ involvement has seldom been identified. We report on a 3 years old female patient of healthy first cousin parents with BPS. She presented with orofacial clefting, severe popliteal webs, club feet, oligosyndactyly of the toes, hypogenitalism and normal hands and internal organs. Ankyloblepharon and filiform bands between the alveolar ridges were evident at birth. Pedigree analysis revealed a more severely affected female sib, who died a few minutes after birth with additional manifestations including near complete lip fusion without oral cleft, complete syndactyly in both hands and an omphalocele. Linkage was excluded to the IRF6 gene; a candidate gene implicated in the Van der Woude and popliteal pterygium syndromes, with overlapping features with BPS. To our knowledge, this is the 5th surviving patient with this syndrome in the literature. In this report, we also discuss the proposed pathogenetic mechanisms for BPS and compare our patients with similarly described cases as well as overlapping spectrum of other popliteal pterygium syndromes. Our findings provide further evidence of intrafamilial clinical heterogeneity in families with BPS.

Correlation between the cumulative analgesic effect of electroacupuncture intervention and synaptic plasticity of hypothalamic paraventricular nucleus neurons in rats with sciatica☆

PubMed Central

Xu, Qiuling; Liu, Tao; Chen, Shuping; Gao, Yonghui; Wang, Junying; Qiao, Lina; Liu, Junling

2013-01-01

In the present study, a rat model of chronic neuropathic pain was established by ligation of the sciatic nerve and a model of learning and memory impairment was established by ovariectomy to investigate the analgesic effect of repeated electroacupuncture stimulation at bilateral Zusanli (ST36) and Yanglingquan (GB34). In addition, associated synaptic changes in neurons in the paraventricular nucleus of the hypothalamus were examined. Results indicate that the thermal pain threshold (paw withdrawal latency) was significantly increased in rats subjected to 2-week electroacupuncture intervention compared with 2-day electroacupuncture, but the analgesic effect was weakened remarkably in ovariectomized rats with chronic constrictive injury. 2-week electroacupuncture intervention substantially reversed the chronic constrictive injury-induced increase in the synaptic cleft width and thinning of the postsynaptic density. These findings indicate that repeated electroacupuncture at bilateral Zusanli and Yanglingquan has a cumulative analgesic effect and can effectively relieve chronic neuropathic pain by remodeling the synaptic structure of the hypothalamic paraventricular nucleus. PMID:25206591

Postoperative evaluation of palatoplasty using the modified Kaplan method.

PubMed

Isago, Tsukasa; Nozaki, Motohiro; Honda, Takashi; Kikuchi, Yuji

2005-01-01

Bilateral buccal mucosal flaps were used for a pushback operation of the nasal mucosa as well as the oral mucosa. Two flaps from the buccal mucosal areas were inserted into the nasal mucosal raw surface at the hard and soft palatal border to achieve nasal mucosal pushback. Forty-four children with cleft palates were retrospectively studied to investigate the velopharyngeal function, articulation, and craniofacial morphology following primary palatoplasty using the modified Kaplan method. 72.7% showed good velopharyngeal function. Articulation disorders were found in 26 of the 44 subjects (59.1%). A craniofacial morphological analysis revealed no major changes in the SNA (sella-nasion point A angle) and SNB (sella-nasion point B angle) and no cases of a marked loss of maxillary mandibular balance. In addition, no significant differences were observed in the maxillary length and width. The results of these palatoplasties by the modified Kaplan method indicated that the use of bilateral buccal mucosal flaps for the pushback of the nasal mucosa was effective in preventing the contraction of the maxillary and mandibular corpal lengths.

Anophthalmia-plus syndrome: a clinical report and review of the literature.

PubMed

Makhoul, Imad R; Soudack, Michalle; Kochavi, Orna; Guilburd, Joseph N; Maimon, Shimon; Gershoni-Baruch, Ruth

2007-01-01

We describe a term male infant of healthy non-consanguineous parents, born with congenital malformations, including bilateral cleft palate and lip, mild microphthalmia with iris coloboma and glaucoma of the right eye, and blepharophimosis with severe microphthalmia of the left eye. Spine radiograph and MRI showed first sacral hemivertebra with spina bifida, and agenesis of the 2nd, 3rd, 4th, and 5th sacral vertebrae and coccyx. Spine MRI showed caudal tethering of spinal cord at L(3) level, filum terminalis lipoma and a syringomyelia. Brain ultrasound and MRI showed hypoplasia of corpus callosum with mild dilatation of the lateral ventricles. Orbital MRI showed bilateral microphthalmia-distorted small left eyeball with posteriorly located lens, and a split vitreous body in the right eye, suggestive of primary hyperplastic vitreous. The karyotype was normal. Summary of the findings in nine cases (our case and eight published cases) support the notion that anophthalmia-plus syndrome (APS) is a distinct syndrome. Gene locus of APS is yet to be identified. (c) 2006 Wiley-Liss, Inc.

Clival Malformations in CHARGE Syndrome.

PubMed

Mahdi, E S; Whitehead, M T

2018-06-01

CHARGE syndrome is a multisystemic congenital disorder, most commonly including coloboma, heart malformations, choanal atresia, developmental delay, and genital and ear anomalies. The diagnostic criteria for CHARGE syndrome have been refined with time. However, limited reports describe skull base and craniocervical junction abnormalities. Recently, a coronal clival cleft has been identified in association with CHARGE syndrome. The aim of our study was to assess the prevalence of clival pathology in CHARGE syndrome. In this retrospective study, the CT/MR imaging data base at a single academic children's hospital was queried for the phrase "CHARGE syndrome" during a 17-year period (2001-2017). Electronic medical records were reviewed to confirm the diagnosis. Images were assessed for skull base anomalies, specifically clival hypoplasia and dysplasia. The search yielded 42 examinations (21 CTs and 21 MRIs) from 15 distinct patients (mean age, 4.1 ± 5.6 years; range, 2 days to 19 years). CHARGE syndrome diagnosis was confirmed either by clinical and genetic testing ( n = 6) or by clinical diagnosis only ( n = 9). A coronal clival cleft was identified in 87% of patients (37 examinations, n = 13 patients), either partial (53%) or complete (33%). Clival hypoplasia without clefting was present in all 5 examinations from the remaining 2 patients. Clival pathology is universal in CHARGE syndrome. Coronal clival clefts are extremely common, representing a useful additional diagnostic finding. Detection of a clival cleft should alert the radiologist to examine the palate, choana, eyes, ears, and olfactory centers for other signs of CHARGE syndrome. © 2018 by American Journal of Neuroradiology.

3D Printed Models of Cleft Palate Pathology for Surgical Education.

PubMed

Lioufas, Peter A; Quayle, Michelle R; Leong, James C; McMenamin, Paul G

2016-09-01

To explore the potential viability and limitations of 3D printed models of children with cleft palate deformity. The advantages of 3D printed replicas of normal anatomical specimens have previously been described. The creation of 3D prints displaying patient-specific anatomical pathology for surgical planning and interventions is an emerging field. Here we explored the possibility of taking rare pediatric radiographic data sets to create 3D prints for surgical education. Magnetic resonance imaging data of 2 children (8 and 14 months) were segmented, colored, and anonymized, and stereolothographic files were prepared for 3D printing on either multicolor plastic or powder 3D printers and multimaterial 3D printers. Two models were deemed of sufficient quality and anatomical accuracy to print unamended. One data set was further manipulated digitally to artificially extend the length of the cleft. Thus, 3 models were printed: 1 incomplete soft-palate deformity, 1 incomplete anterior palate deformity, and 1 complete cleft palate. All had cleft lip deformity. The single-material 3D prints are of sufficient quality to accurately identify the nature and extent of the deformities. Multimaterial prints were subsequently created, which could be valuable in surgical training. Improvements in the quality and resolution of radiographic imaging combined with the advent of multicolor multiproperty printer technology will make it feasible in the near future to print 3D replicas in materials that mimic the mechanical properties and color of live human tissue making them potentially suitable for surgical training.

Psychological status as a function of residual scarring and facial asymmetry after surgical repair of cleft lip and palate.

PubMed

Millar, Keith; Bell, Aileen; Bowman, Adrian; Brown, Denise; Lo, Tsz-Wai; Siebert, Paul; Simmons, David; Ayoub, Ashraf

2013-03-01

Objective : Objective measure of scarring and three-dimensional (3D) facial asymmetry after surgical correction of unilateral cleft lip (UCL) and unilateral cleft lip (UCLP). It was hypothesized that the degree of scarring or asymmetry would be correlated with poorer psychological function. Design : In a cross-sectional design, children underwent 3D imaging of the face and completed standardized assessments of self-esteem, depression, and state and trait anxiety. Parents rated children's adjustment with a standard scale. Setting : Glasgow Dental School, School of Medicine, College of Medical, Veterinary and Life Sciences. Patients : Fifty-one children aged 10 years with UCLP and 43 with UCL were recruited from the cohort treated with the surgical protocol of the CLEFTSIS managed clinical network in Scotland. Methods : Objective assessment to determine the luminance and redness of the scar and facial asymmetry. Depression, anxiety, and a self-esteem assessment battery were used for the psychological analysis. Results : Cleft cases showed superior psychological adjustment when compared with normative data. Prevalence of depression matched the population norm. The visibility of the scar (luminance ratio) was significantly correlated with lower self-esteem and higher trait anxiety in UCLP children (P  =  .004). Similar but nonsignificant trends were seen in the UCL group. Parental ratings of poorer adjustment also correlated with greater luminance of the scar. Conclusions : The objectively defined degree of postoperative cleft scarring was associated with subclinical symptoms of anxiety, depression, and low self-esteem.

A cross-sectional survey of 5-year-old children with non-syndromic unilateral cleft lip and palate: the Cleft Care UK study. Part 1: background and methodology.

PubMed

Persson, M; Sandy, J R; Waylen, A; Wills, A K; Al-Ghatam, R; Ireland, A J; Hall, A J; Hollingworth, W; Jones, T; Peters, T J; Preston, R; Sell, D; Smallridge, J; Worthington, H; Ness, A R

2015-11-01

We describe the methodology for a major study investigating the impact of reconfigured cleft care in the United Kingdom (UK) 15 years after an initial survey, detailed in the Clinical Standards Advisory Group (CSAG) report in 1998, had informed government recommendations on centralization. This is a UK multicentre cross-sectional study of 5-year-olds born with non-syndromic unilateral cleft lip and palate. Children born between 1 April 2005 and 31 March 2007 were seen in cleft centre audit clinics. Consent was obtained for the collection of routine clinical measures (speech recordings, hearing, photographs, models, oral health, psychosocial factors) and anthropometric measures (height, weight, head circumference). The methodology for each clinical measure followed those of the earlier survey as closely as possible. We identified 359 eligible children and recruited 268 (74.7%) to the study. Eleven separate records for each child were collected at the audit clinics. In total, 2666 (90.4%) were collected from a potential 2948 records. The response rates for the self-reported questionnaires, completed at home, were 52.6% for the Health and Lifestyle Questionnaire and 52.2% for the Satisfaction with Service Questionnaire. Response rates and measures were similar to those achieved in the previous survey. There are practical, administrative and methodological challenges in repeating cross-sectional surveys 15 years apart and producing comparable data. © 2015 The Authors. Orthodontics & Craniofacial Research Published by John Wiley & Sons Ltd.

Three-dimensional development of the upper dental arch in unilateral cleft lip and palate patients after early neonatal cheiloplasty.

PubMed

Hoffmannova, E; Moslerová, V; Dupej, J; Borský, J; Bejdová, Š; Velemínská, J

2018-06-01

This prospective morphometric study evaluated the growth of the upper dental arch in UCLP patients after early neonatal cheiloplasty and compared the selected dimensions with published data on non-cleft controls and on later operation protocol patients. The sample comprised 36 Czech children with nonsyndromic complete UCLP (cUCLP) and 20 Czech children with nonsyndromic incomplete UCLP (UCLP + b). 2-D and 3-D analyses of palatal casts were made at two time points: before neonatal cheiloplasty at the mean age of 3 days (±1 day), and 10 months after surgery at the mean age of 10 months (±1 month). The upper dental arch of cUCLP and UCLP + b patients showed similar developmental changes, but the cleft type influenced growth significantly. The initial high shape variability in cUCLP patients diminished after 10 months, and approached the variability in UCLP + b patients. Both the width and length dimensions increased after surgery. Important growth concerned the anterior ends of both segments. The width and length dimensions illustrated similar growth trends with non-cleft controls and UCLP patients who underwent later cheiloplasty. Early neonatal cheiloplasty caused no reduction in the length or width dimensions during the first year of life. Our data suggest a reconstructed lip has a natural formative effect on the actively growing anterior parts of upper dental arch segments, which cause narrowing of the alveolar cleft. Copyright © 2018 Elsevier B.V. All rights reserved.

Effect of early correction of nasal septal deformity in unilateral cleft lip and palate on inferior turbinate hypertrophy and nasal patency.

PubMed

Pinto, Valentina; Piccin, Ottavio; Burgio, Luca; Summo, Valeria; Antoniazzi, Elisa; Morselli, Paolo G

2018-05-01

A relatively neglected aspect of cleft lip nasal deformity is the effect of septal deviation and inferior turbinate hypertrophy (ITH) on the functional airway. In particular, ITH in the noncleft side can be especially problematic, because it reduces the healthy nasal area, creating bilateral nasal obstruction that might affect the growth of the maxillofacial skeleton. Although these anatomic and functional changes are documented, few recommendations have been developed regarding the proper approach to ITH. The aim of the present study was to asses the ITH severity and determine the degree of nasal airway patency in patients who have undergone primary correction of the nasal septum during lip repair compared to patients operated on without primary septal correction. The study population included two groups. One group consisted of twenty unilateral cleft lip palate UCLP patients who have previously undergone primary rhinoseptoplasty as part of their treatment plan. The control group consisted of twenty UCLP patients operated on without rhinoseptal correction. The Nasal Obstructive Symptom Evaluation (NOSE) scale and nasal endoscopy were used to assess nasal obstruction. The overall untreated group reported severe symptoms across all NOSE scale dimensions more frequently than children who have undergone primary rhinoseptoplasty. The difference was statistically significant for each dimensions (p < 0.05). The mean NOSE score for group A and group B was 21.4 ± 9.4 and 70.8 ± 17.2 respectively (p < 0.0001). In group A turbinate size decreased significantly (p < 0.05) compared to pre-operative data. Comparing the two groups a statistically significant difference in turbinate size was observed (p < 0.0001). The results of the present study confirm that there is a significant degree of ITH and nasal airway dysfunction in patients with UCLP. Early septal repositioning during primary cleft lip repair results in a statistically significant reduction in IT size and improvement of nasal patency. Copyright © 2018 Elsevier B.V. All rights reserved.

Maxillary distraction complications in cleft patients.

PubMed

Jeblaoui, Y; Morand, B; Brix, M; Lebeau, J; Bettega, G

2010-06-01

Cleft lip and palate (CLP) patients often present with a class III malocclusion in connection with a three dimensional maxillary hypoplasia. Twenty-five to 60% of these patients need maxillary advancement. Two solutions are possible: orthognathic surgery and maxillary distraction. The purpose of this study was to evaluate the complications of maxillary distraction in CLP patients. Data was collected from the records of patients treated in our surgery unit between 2000 and 2007. Among the eight patients (four male and four female), five presented with a bilateral CLP, two with a unilateral CLP, and one with a unilateral cleft lip associated to a soft palate cleft. The average age at surgery was 17 years. All underwent a Le Fort I osteotomy with a pterygomaxillary disjunction. An external distractor was used for the first two patients and an internal distractor for the six following patients. After a seven-day latency, activation was implemented at a rate of 1mm twice a day. The average period of consolidation was four months. Maxillary advancement ranged between 7 and 19mm, with an average of 12.6mm. The average follow-up was four years. Complications were noted in seven patients: one intra-operative hemorrhage, one avulsion of a tooth anchored at the pterygoid process during osteotomy, three cases of device dysfunction, two cases of significant pain during activation, one loosening of the orthodontic arch in an external system, two cases of labial ulceration, and one maxillary sinusitis due to migration of a wisdom tooth. Complications of maxillary distraction in CLP patients were very frequent. Most were related to the device and did not interfere with the final result. This must be taken into account when indicating distraction and choosing the device. Two types of complications can occur during distraction: those related to the osteotomy and those related to the device. The complications related to the osteotomy are linked to the cicatricial ground of previous surgery. They are not specific to distraction. The comfort of the internal device is undeniable, but the design of some models must be reviewed to improve their tolerance. Copyright 2010 Elsevier Masson SAS. All rights reserved.

[Maxillary distraction complications in cleft patients].

PubMed

Jeblaoui, Y; Morand, B; Brix, M; Lebeau, J; Bettega, G

2008-09-01

Cleft lip and palate (CLP) patients often present with a class III malocclusion in connection with a three dimensional maxillary hypoplasia. Twenty-five to 60% of these patients require a maxillary advancement. Two solutions are possible: orthognathic surgery and maxillary distraction. The purpose of this study was to evaluate the complications of the maxillary distraction in CLP patients. Data was collected from the records of patients treated at our Surgery Unit between 2000 and 2007. Among the eight patients (four male and four female), five presented a bilateral CLP, two a unilateral CLP and one a unilateral cleft lip associated to a soft palate cleft. The average age at surgery was 17 years old. All had a Le Fort I osteotomy with a pterygomaxillary disjunction. The first two patients had external distractors and the six following internal ones. After a seven-day latency, activation was led to the rate of 1mm per day twice. The period of consolidation was four months on average. The maxillary advancement varied between 7 and 19 mm with an average of 12.6mm. The average follow-up was four years. We encountered difficulties and/or complications in seven patients: one intraoperatively haemorrhage, one avulsion of a tooth fixed at the pterygoid process during the osteotomy, three device failures, two cases of significant pains during activation, one dissociation of the dental anchorage of an external system, two labial ulcerations and one maxillary sinusitis by migration of the 18. Difficulties of maxillary distraction in CLP patients are very frequent. The majority is related to the distractors and did not interfere with the final result. But this frequency must be taken into account in the indication and in the choice of the material. Two types of complications can occur during distraction: those related to the osteotomy and those related to the material. The complications related to the osteotomy are in connection with the cicatricial ground of the CLP. They are not specific of the distraction. We especially managed complications related to the material. The social benefit of the internal distractors is undeniable, but the design of certain models must be reexamined to improve their tolerance.

Cystic Dilation of the Aqueductus Sylvii in Case of Trisomy 17p11.2—pter with the Deletion of the Terminal Portion of the Chromosome 6

PubMed Central

Horváth, Emese; Sikovanyecz, János; Pál, Attila; Kaiser, László; Bálint, Bálint L.; Szilárd, Póliska; Kozinszky, Zoltán; Szabó, János

2010-01-01

Since the 1970s, about 30 cases of partial or complete trisomy 17p have been presented in the literature. Partial trisomies of the short arm of chromosome 17 are somewhat more common, but complete trisomy is quite rare. Most of these cases were described in infants and newborns; and to our knowledge only 3 cases of trisomy 17p have been detected intrauterine. Phenotypic features of trisomy 17p in fetuses are intrauterine growth retardation, ventriculomegaly, cleft lip and cleft palate, micrognathia, horseshoe kidneys, single umbilical artery, and congenital heart defects. The sonographic and foetopathologic findings of a pregnancy trisomy 17p11.2—pter with the deletion of the terminal portion of the chromosome 6 due to paternal balanced translocation are described in this case report. PMID:21274281

International confederation for cleft lip and palate and related craniofacial anomalies task force report: holistic outcomes.

PubMed

Broder, Hillary L

2014-11-01

Objective : This paper describes the process and outcomes of the 2013 American Cleft Palate-Craniofacial Association task force on Holistic Outcomes. The goals and membership of the task force are presented. Methods : Using internet communication, the group introduced themselves, shared ideas and information related to holistic assessment and implementation of using a validated holistic measure, the Child Oral Health Impact Profile (COHIP) at participating international sites. Results : Data from the sites were analyzed using descriptive statistics. Administration of the COHIP was successful. It varied from self-completion as well as verbal presentation due to language differences and a function of the short time period to complete collection. Additionally qualitative comments were reported by the task force site directors. Conclusions : Future directions for holistic assessment and communication among task force members and sites were discussed at the Congress and are presented in this report.

Breeding experiments and genome-wide association analysis elucidate two genetically different forms of non-syndromic congenital cleft lip and jaw in Vorderwald × Montbéliarde cattle.

PubMed

Reinartz, S; Distl, O

2017-10-01

Non-syndromic congenital cleft lip and jaw (CLJ) is a condition reported in Vorderwald × Montbéliarde cattle. The objective of the present study was to perform a genome-wide association study (GWAS) for 10 CLJ-affected and 50 unaffected Vorderwald × Montbéliarde cattle using the bovine Illumina high density bead chip to identify loci for this condition. Phenotypic classification of CLJ was based on a detailed recording of orofacial structures using computed tomography. A breeding experiment among CLJ-affected Vorderwald × Montbéliarde cattle and CLJ-affected Vorderwald × Montbéliarde cattle with unaffected Holsteins confirmed recessive inheritance and different loci for bilateral or left-sided versus right-sided CLJ. The GWAS for the five cases with right-sided CLJ gave a genome-wide signal on bovine chromosome (BTA) 29 at 16 Mb. For the four left-sided and one bilateral CLJ case, a genome-wide significant association was identified on BTA4 at 32 Mb. Two different loci are very likely to be involved in CLJ in Vorderwald × Montbéliarde cattle because experimental matings among affected cows and bulls with different types of CLJ did not result in CLJ-affected progeny, and in addition, two different loci were also found through GWAS and mapped on two different bovine chromosomes. Validation in 346 Vorderwald × Montbéliarde cattle for the highly associated SNPs on BTA4 and 29 gave ratios of 33/346 (0.095, BTA4) and 6/346 (0.017, BTA29) homozygous mutant genotypes. Further studies should elucidate the responsible mutations underlying the different types of CLJ in Vorderwald × Montbéliarde cattle. © 2017 Stichting International Foundation for Animal Genetics.

Maxillary protraction in adult cleft lip and palate by a rigid external distraction device with dentoskeletal anchorage.

PubMed

Akarsu, Bengisu; Taner, Tulin; Tuncbilek, Gokhan; Mavili, M Emin

2012-04-01

The objective is to evaluate the effects of maxillary distraction osteogenesis (DO) in an adult patient with unilateral cleft lip and palate (UCLP) by using a rigid external distraction (RED) device with dentoskeletal anchorage. 31-year-old male patient with UCLP with severe maxillary hypoplasia, dolichofacial growth pattern, negative overjet and 1.5 mm openbite. After pre-surgical orthodontic treatment, an intra-oral appliance was modified to prevent extrusion of the molars and clockwise rotation of the mandible. Stainless steel plates were soldered bilaterally to the intra oral appliance at the level of canines. During surgery, miniplates were inserted in the maxillary segment and fixed to the plates of the intra oral appliance with screws. The mean distraction length was 12 mm immediately after DO. SNA increased from 73o to 82o after distraction. A significant advancement of the maxilla and correction of the sagittal Class III skeletal relationship was achieved. The vertical position of the mandible and the face was kept stable, and the soft tissue profile became more balanced. This intra oral appliance design achieved desired skeletal changes during maxillary protraction with RED device in dolichofacial CLP patient. Occlusion and facial profile changes was found to be stable in 1-year follow-up.

Maxillary protraction in adult cleft lip and palate by a rigid external distraction device with dentoskeletal anchorage

PubMed Central

Akarsu, Bengisu; Taner, Tulin; Tuncbilek, Gokhan; Mavili, M. Emin

2012-01-01

Objective: The objective is to evaluate the effects of maxillary distraction osteogenesis (DO) in an adult patient with unilateral cleft lip and palate (UCLP) by using a rigid external distraction (RED) device with dentoskeletal anchorage. Method: 31-year-old male patient with UCLP with severe maxillary hypoplasia, dolichofacial growth pattern, negative overjet and 1.5 mm openbite. After pre-surgical orthodontic treatment, an intra-oral appliance was modified to prevent extrusion of the molars and clockwise rotation of the mandible. Stainless steel plates were soldered bilaterally to the intra oral appliance at the level of canines. During surgery, miniplates were inserted in the maxillary segment and fixed to the plates of the intra oral appliance with screws. Results: The mean distraction length was 12 mm immediately after DO. SNA increased from 73o to 82o after distraction. A significant advancement of the maxilla and correction of the sagittal Class III skeletal relationship was achieved. The vertical position of the mandible and the face was kept stable, and the soft tissue profile became more balanced. Conclusion: This intra oral appliance design achieved desired skeletal changes during maxillary protraction with RED device in dolichofacial CLP patient. Occlusion and facial profile changes was found to be stable in 1-year follow-up. PMID:22509125

Anterior maxillary segmental distraction for correction of maxillary hypoplasia and dental crowding in cleft palate patients: a preliminary report.

PubMed

Wang, X-X; Wang, X; Li, Z-L; Yi, B; Liang, C; Jia, Y-L; Zou, B-S

2009-12-01

To evaluate the feasibility of anterior maxillary segmental distraction (AMSD) to correct maxillary hypoplasia and severe dental crowding in cleft lip and palate (CLP) patients, 7 patients (average age 16.4 years) with maxillary hypoplasia, shortened maxillary dental arch length and severe anterior dental crowding secondary to CLP were selected for this study. After anterior maxillary segmental osteotomy, 3 patients were treated using bilateral internal distraction devices, and 4 patients were treated using rigid external distraction devices. Photographs and radiographs were taken to review the improvement in facial profile and occlusion after distraction. An average 10.25 mm anterior maxillary advancement was obtained in all patients after 10-23 days of distraction and 9-16 weeks of consolidation. The sella-nasion-point A (SNA) angle increased from 69.5 degrees to 79.6 degrees. Midface convexity was greatly improved and velopharyngeal competence was preserved. The maxillary dental arch length was greatly increased by 10.1 mm (P<0.01). Dental crowding and malocclusion were corrected by orthodontic treatment. These results show that AMSD can effectively correct the hypoplastic maxilla and severe dental crowding associated with CLP by increasing the midface convexity and dental arch length while preserving velopharyngeal function, and dental crowding can be corrected without requiring tooth extraction.

Congenital cervical cysts, sinuses, and fistulae in pediatric surgery.

PubMed

LaRiviere, Cabrini A; Waldhausen, John H T

2012-06-01

Congenital cervical anomalies are essential to consider in the clinical assessment of head and neck masses in children and adults. These lesions can present as palpable cystic masses, infected masses, draining sinuses, or fistulae. Thyroglossal duct cysts are most common, followed by branchial cleft anomalies and dermoid cysts. Other lesions reviewed include median ectopic thyroid, cervical teratomas, and midline cervical clefts. Appropriate diagnosis and management of these lesions requires a thorough understanding of their embryology and anatomy. Correct diagnosis, resolution of infectious issues before definitive therapy, and complete surgical excision are imperative in the prevention of recurrence. Copyright © 2012 Elsevier Inc. All rights reserved.

The Epidemiology of Cleft Lip and Palate in Canada, 1998 to 2007.

PubMed

Matthews, Jennifer L K; Oddone-Paolucci, Elizabeth; Harrop, Robertston A

2015-07-01

To examine the birth prevalence, gender distribution, and pattern of surgical intervention for clefts in Canada (1998 to 2007). Also to highlight the difficulties associated with studying the epidemiology of clefts using the current data collection mechanisms. Epidemiologic data acquired from the Canadian Institute for Health Information. Population-based study in Canada 1998 to 2007. All live births with an International Classification of Diseases (9th or 10th revision) diagnostic code for cleft palate or for cleft lip with or without cleft palate or with a surgical intervention code for repair of cleft lip or cleft palate. Birth prevalence, gender distribution, and pattern of surgical intervention. There were 3,015,325 live births in Canada (1998 to 2007). The mean birth prevalence was 0.82 per 1000 live births for cleft lip with or without cleft palate and 0.58 per 1000 live births for cleft palate. The birth prevalence of cleft lip with or without cleft palate was significantly higher in boys, with a stable boy to girl ratio of 1.75:1. Cleft palate was significantly greater in girls; however, the boy to girl ratio decreased from 0.97:1 in 1998 to 0.59:1 in 2007. The median age of repair in Canada from 1998 to 2007 was 4.7 months for cleft lip and 11.6 months for cleft palate. Thirty percent of patients underwent cleft palate repair after age 1. The birth prevalence of cleft palate and cleft lip with or without cleft palate is stable in Canada. An increasing birth prevalence of cleft palate in girls is suggested. The timing of surgical intervention is consistent with current standards. The challenges associated with collecting these data in Canada are discussed.

Quantitative 3D maxillary arch evaluation of two different infant managements for unilateral cleft lip and palate.

PubMed

Prasad, C N; Marsh, J L; Long, R E; Galic, M; Huebener, D V; Bresina, S J; Vannier, M W; Pilgram, T K; Mazaheri, M; Robison, S; Bartell, T

2000-11-01

A two-institution retrospective study was undertaken to determine whether two different prepalatoplasty protocols quantitatively affect maxillary arch morphology in infants with complete unilateral cleft lip and palate (UCLP). Serial maxillary dental casts, obtained at regular intervals through the first 18 months of life from preintervention until palatoplasty were evaluated quantitatively using computer-assisted three-dimensional digitization and analysis for three populations: institution 1 (protocol 1), institution 2 (protocol 2), and unaffected individuals (neither cleft nor treatment). Sequential UCLP patients from institution 1 were matched for age and initial alveolar cleft width, sex and cleft side having been demonstrated to be nonsignificant, with UCLP patients from institution 2 and to unaffected individuals for age for the analysis. Both treatment institutions are well-established regional interdisciplinary cleft centers. Institution 1 is located in a tertiary, academic children's hospital in a metropolis within a primarily agrarian region of the Midwest; institution 2 is a freestanding private clinic located in a small city within a primarily agrarian region of an eastern state; the unaffected population is a historic archive acquired in the 1930s. Data acquisition (model digitization) and computer processing were performed at institution 1. Eighty-five casts of 28 infants from institution 1, 106 casts of 31 infants from institution 2, and 68 casts of 29 unaffected infants were analyzed. All infants had alginate impressions taken prior to intervention and at several additional 6-month intervals after that, consistent with each institution's treatment protocol. At institution 1, patients with UCLP underwent lip adhesion and placement of a passive alveolar molding plate at 7 weeks of age, definitive cheiloplasty at 7 months of age, and one-stage palatoplasty at 14 months of age. At institution 2, patients with UCLP underwent definitive cheiloplasty at 3 months of age, had no maxillary orthopedics, and had vomer flap hard palate repair at 12 months of age and soft palate repair at 18 months of age. The outcome measures included directly digitized (cleft segment and hemialveolar ridge lengths) and derived (alveolar base width, alveolar cleft gap, maxillary frenum-alveolar base perpendicular angle, and rates of change over time of digitized cleft segment and hemialveolar ridge lengths) features. The data were assessed by comparing simple linear regression lines and an unpaired, two-tailed t test. Prior to initiating therapy, there were no statistically significant differences between the two populations with clefts. However, both populations with clefts differed significantly from unaffected individuals (p < .001), with increased maxillary base widths and larger perpendicular/frenum angles. At the time of palatoplasty, the two populations with clefts had statistically significant differences between them in the maxillary base width (p < .01) and the cleft gap distance (p < .05). The base width of institution 1 did not differ significantly from that of widths of unaffected children, and that of institution 2 was significantly less, although the latter had already received first-stage palate repair. Alveolar segment growth rates were similar for the greater and lesser segments, respectively, and the left side hemialveolus of both groups. The growth rate for the noncleft side hemialveolus of institution 2 exceeded (p < .05) that of both institution 1 and unaffected patients. Two different regimens for the initial management of UCLP can significantly affect maxillary alveolar arch growth with respect to the treatment used and in comparison with unaffected controls.

Effects of early and late cheiloplasty on anterior part of maxillary dental arch development in infants with unilateral complete cleft lip and palate.

PubMed

Valentová-Strenáčiková, Silvia; Malina, Radovan

2016-01-01

Objectives. The objective of this study is to compare the impact of early and late reconstruction of complete unilateral cleft lip and palate on the growth and development of the front of the dentoalveolar arch. Methods. This study was carried out in the years 2012-2015 at the Clinic of Plastic, Reconstructive and Aesthetic Surgery in Banska Bystrica. Infants with unilateral complete cleft lip and palate were divided into 2 groups according to the timing of lip reconstruction. Group A consisted of infants with early lip reconstruction-realised in the first 14 days of life. Group B consisted of infants with later lip reconstruction-realised in the third month of age. Maxillary dental casts were obtained for each child in four periods-in the first 14 days of life, in the third month, in the sixth month and in the age of one year. These were followed by the identification, measurement and evaluation of anthropometric parameters. Results. Significant differences were occurred after the reconstruction of the lips in linear and angle measurements between infants in the A and B groups. Conclusion. The early surgical reconstruction of the lips in the first 14 days of life has a positive effect on the growth and development of the anterior segment of the dentoalveolar arch. Early lip reconstruction forms a continuous pressure on the frontal segment, resulting in the earlier remedy of anatomical properties and creates appropriate conditions for the best development of this area.

Effects of early and late cheiloplasty on anterior part of maxillary dental arch development in infants with unilateral complete cleft lip and palate

PubMed Central

2016-01-01

Objectives. The objective of this study is to compare the impact of early and late reconstruction of complete unilateral cleft lip and palate on the growth and development of the front of the dentoalveolar arch. Methods. This study was carried out in the years 2012–2015 at the Clinic of Plastic, Reconstructive and Aesthetic Surgery in Banska Bystrica. Infants with unilateral complete cleft lip and palate were divided into 2 groups according to the timing of lip reconstruction. Group A consisted of infants with early lip reconstruction–realised in the first 14 days of life. Group B consisted of infants with later lip reconstruction–realised in the third month of age. Maxillary dental casts were obtained for each child in four periods–in the first 14 days of life, in the third month, in the sixth month and in the age of one year. These were followed by the identification, measurement and evaluation of anthropometric parameters. Results. Significant differences were occurred after the reconstruction of the lips in linear and angle measurements between infants in the A and B groups. Conclusion. The early surgical reconstruction of the lips in the first 14 days of life has a positive effect on the growth and development of the anterior segment of the dentoalveolar arch. Early lip reconstruction forms a continuous pressure on the frontal segment, resulting in the earlier remedy of anatomical properties and creates appropriate conditions for the best development of this area. PMID:26893957

An alternative clinical approach to achieve greater anterior than posterior maxillary expansion in cleft lip and palate patients.

PubMed

Oliveira, Dauro Douglas; Bartolomeo, Flávia Uchôa Costa; Cardinal, Lucas; Figueiredo, Daniel Santos Fonseca; Palomo, Juan Martin; Andrade, Ildeu

2014-11-01

Cleft lip and palate patients commonly present maxillary constriction, particularly in the anterior region. The aim of this case report was to describe an alternative clinical approach that used a smaller Hyrax screw unconventionally positioned to achieve greater anterior than posterior expansion in patients with complete unilateral cleft lip and palate. The idea presented here is to take advantage of a reduced dimension screw to position it anteriorly. When only anterior expansion was needed (patient 1), the appliance was soldered to the first premolar bands and associated to a transpalatal arch cemented to the first molars. However, when overall expansion was required (patient 2), the screw was positioned anteriorly, but soldered to the first molar bands. Intercanine, premolar, and first molar widths were measured on dental casts with a digital caliper. Pre-expansion and postexpansion radiographs and tomographies were also evaluated. A significant anterior expansion and no intermolar width increase were registered in the first patient. Although patient 2 also presented a greater anterior than posterior expansion, a noteworthy expansion occurred at the molar region. The alternative approach to expand the maxilla in cleft patients reported here caused greater anterior than posterior expansion when the Mini-Hyrax was associated to a transpalatal arch, and its reduced dimension also minimized discomfort and facilitated hygiene.

Centralisation of services for children with cleft lip or palate in England: a study of hospital episode statistics

PubMed Central

2012-01-01

Background In 1998, a process of centralisation was initiated for services for children born with a cleft lip or palate in the UK. We studied the timing of this process in England according to its impact on the number of hospitals and surgeons involved in primary surgical repairs. Methods All live born patients with a cleft lip and/or palate born between April 1997 and December 2008 were identified in Hospital Episode Statistics, the database of admissions to English National Health Service hospitals. Children were included if they had diagnostic codes for a cleft as well as procedure codes for a primary surgical cleft repair. Children with codes indicating additional congenital anomalies or syndromes were excluded as their additional problems could have determined when and where they were treated. Results We identified 10,892 children with a cleft. 21.0% were excluded because of additional anomalies or syndromes. Of the remaining 8,606 patients, 30.4% had a surgical lip repair only, 41.7% a palate repair only, and 28.0% both a lip and palate repair. The number of hospitals that carried out these primary repairs reduced from 49 in 1997 to 13, with 11 of these performing repairs on at least 40 children born in 2008. The number of surgeons responsible for repairs reduced from 98 to 26, with 22 performing repairs on at least 20 children born in 2008. In the same period, average length of hospital stay reduced from 3.8 to 3.0 days for primary lip repairs, from 3.8 to 3.3 days for primary palate repairs, and from 4.6 to 2.6 days for combined repairs with no evidence for a change in emergency readmission rates. The speed of centralisation varied with the earliest of the nine regions completing it in 2001 and the last in 2007. Conclusions Between 1998 and 2007, cleft services in England were centralised. According to a survey among patients’ parents, the quality of cleft care improved in the same period. Surgical care became more consistent with current recommendations. However, key outcomes, including facial appearance and speech, can only be assessed many years after the initial surgical treatment. PMID:22682355

Cost management of cleft lips under the Universal Health Coverage Program of the Tawanchai Cleft Center, Srinagarind Hospital, Faculty of Medicine, Khon Kaen University.

PubMed

Odton, Cheewarat; Rittirod, Theera; Pradubwong, Suteera; Chowchuen, Bowornsilp

2014-10-01

The study ofcost management with regard to cleft lip patients under the Universal Health Coverage Program at Tawanchai Cleft Center Srinagarind Hospital, Faculty of Medicine, Khon Kaen University, was conducted in order to provide fundamental information for the administrative team on how best to administrate and manage the organization. To study the cost management of cleft lip patients under the Universal Health Coverage Program. To compare individual patient management costs and costs from the National Health Security Office (NHSO), and to offer proper guidelines for cost management to the organization. The study was performed retrospectively. The data were collected by reviewing secondary sources of information from patients with cleft lips who consistently underwent treatment at Tawanchai Cleft Center. As for the provider prospects, the cost management did not address the other expenses. The study analyzed the comparison between cost management and income from the Universal Health Coverage Program, which it receivedfrom the National Health Security Office (NHSO). The study was conducted over 2 years (October 1, 2010 to 30 September, 2013). There were 21patients in this study. Microsoft excel was the instrument used to calculate the cost ofmanagement. (1) Total costs were lower than real payments because this cost did not take into account the total cost of the operation room, patient room, common bed, and costs of the medical equipment. Moreover the information regarding the building's price and the facility were not clear enough. The database of materials and equipment was also not yet complete. (2) The average cost ofpatient management was 12,025.14 Bahtperperson, but the compensation receivedfrom the National Health Security Office (NHSO) averaged 10,527.63 Bahtperperson, which was 87.55% ofthe total cost management. The department with the largest expenses was Anesthesia (36.42%). This study indicated that the cost of patient management is lower than usual due to the lack of clear cost information. The cost of medical care, which was received from the National Health Security Office (NHSO), was only 87.55%; the department with the highest costs was Anesthesia (36.42%).

A cross-sectional survey of 5-year-old children with non-syndromic unilateral cleft lip and palate: the Cleft Care UK study. Part 1: background and methodology

PubMed Central

Persson, M; Sandy, J R; Waylen, A; Wills, A K; Al-Ghatam, R; Ireland, A J; Hall, A J; Hollingworth, W; Jones, T; Peters, T J; Preston, R; Sell, D; Smallridge, J; Worthington, H; Ness, A R

2015-01-01

Structured Abstract Objectives We describe the methodology for a major study investigating the impact of reconfigured cleft care in the United Kingdom (UK) 15 years after an initial survey, detailed in the Clinical Standards Advisory Group (CSAG) report in 1998, had informed government recommendations on centralization. Setting and Sample Population This is a UK multicentre cross-sectional study of 5-year-olds born with non-syndromic unilateral cleft lip and palate. Children born between 1 April 2005 and 31 March 2007 were seen in cleft centre audit clinics. Materials and Methods Consent was obtained for the collection of routine clinical measures (speech recordings, hearing, photographs, models, oral health, psychosocial factors) and anthropometric measures (height, weight, head circumference). The methodology for each clinical measure followed those of the earlier survey as closely as possible. Results We identified 359 eligible children and recruited 268 (74.7%) to the study. Eleven separate records for each child were collected at the audit clinics. In total, 2666 (90.4%) were collected from a potential 2948 records. The response rates for the self-reported questionnaires, completed at home, were 52.6% for the Health and Lifestyle Questionnaire and 52.2% for the Satisfaction with Service Questionnaire. Conclusions Response rates and measures were similar to those achieved in the previous survey. There are practical, administrative and methodological challenges in repeating cross-sectional surveys 15 years apart and producing comparable data. PMID:26567851

Presurgical nasoalveolar molding for cleft lip and palate: the application of digitally designed molds.

PubMed

Shen, Congcong; Yao, Caroline A; Magee, William; Chai, Gang; Zhang, Yan

2015-06-01

The authors present a novel nasoalveolar molding protocol by prefabricating sets of nasoalveolar molding appliances using three-dimensional technology. Prospectively, 17 infants with unilateral complete cleft lip and palate underwent the authors' protocol before primary cheiloplasty. An initial nasoalveolar molding appliance was created based on the patient's first and only in-person maxillary cast, produced from a traditional intraoral dental impression. Thereafter, each patient's molding course was simulated using computer software that aimed to narrow the alveolar gap by 1 mm each week by rotating the greater alveolar segment. A maxillary cast of each predicted molding stage was created using three-dimensional printing. Subsequent appliances were constructed in advance, based on the series of computer-generated casts. Each patient had a total three clinic visits spaced 1 month apart. Anthropometric measurements and bony segment volumes were recorded before and after treatment. Alveolar cleft widths narrowed significantly (p < 0.01), soft-tissue volume of each segment expanded (p < 0.01), and the arc of the alveolus became more contiguous across the cleft (p < 0.01). One patient required a new appliance at the second visit because of bleeding and discomfort. Eleven patients had mucosal irritation and two experienced minor mucosal ulceration. Three-dimensional technology can precisely represent anatomic structures in pediatric clefts. Results from the authors' algorithm are equivalent to those of traditional nasoalveolar molding therapies; however, the number of required clinic visits and appliance adjustments decreased. As three-dimensional technology costs decrease, multidisciplinary teams may design customized nasoalveolar molding treatment with improved efficiency and less burden to medical staff, patients, and families. Therapeutic, IV.

Effects of pre-surgical nasoalveolar moulding on maxillary arch and nasal form in unilateral cleft lip and palate before lip surgery.

PubMed

Fuchigami, T; Kimura, N; Kibe, T; Tezuka, M; Amir, M S; Suga, H; Takemoto, Y; Hashiguchi, M; Maeda-Iino, A; Nakamura, N

2017-11-01

To investigate the effects of pre-surgical nasoalveolar moulding (PNAM) on the maxillary arch and nasal form in patients with unilateral cleft lip and palate (UCLP). This is a retrospective case series study. The subjects were infants with complete UCLP who were treated with PNAM (n = 18) at Kagoshima University Medical and Dental Hospital (Japan) between 2006 and 2013. Maxillary dental casts and facial photographs were taken at the time of the first visit and immediately prior to lip surgery to evaluate the maxillary arch and nasal form changes. The dental casts were scanned with a laser scanner, and changes in the 3-Dimensional coordinates of anatomical landmarks and alveolar cleft width were analysed. Moreover, we investigated the correlation between the changes in the maxillary alveolar arch and nasal form. Regarding the maxillary alveolar arch form, the anterior points of the major segment had moved significantly to the cleft side just prior to the time of lip repair, and the alveolar cleft width was significantly decreased. For nasal form, the inclination and displacement of the columella were significantly improved. The improvement of columella inclination was moderately correlated with the posterior movement of the anterior points of the major segment. These findings indicate that PNAM for infants with UCLP enhanced symmetry in the maxillary alveolar arch and nasolabial form. In addition, the posterior movement of the anterior points of the maxillary alveolar arch was correlated with the improvement of columella deformation. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Dominant role of local dipolar interactions in phosphate binding to a receptor cleft with an electronegative charge surface: equilibrium, kinetic, and crystallographic studies.

PubMed

Ledvina, P S; Tsai, A L; Wang, Z; Koehl, E; Quiocho, F A

1998-12-01

Stringent specificity and complementarity between the receptor, a periplasmic phosphate-binding protein (PBP) with a two-domain structure, and the completely buried and dehydrated phosphate are achieved by hydrogen bonding or dipolar interactions. We recently found that the surface charge potential of the cleft between the two domains that contains the anion binding site is intensely electronegative. This novel finding prompted the study reported here of the effect of ionic strength on the equilibrium and rapid kinetics of phosphate binding. To facilitate this study, Ala197, located on the edge of the cleft, was replaced by a Trp residue (A197W PBP) to generate a fluorescence reporter group. The A197W PBP-phosphate complex retains wild-type Kd and X-ray structure beyond the replacement residue. The Kd (0.18 microM) at no salt is increased by 20-fold at greater than 0.30 M NaCl. Stopped-flow fluorescence kinetic studies indicate a two-step binding process: (1) The phosphate (L) binds, at near diffusion-controlled rate, to the open cleft form (Po) of PBP to produce an intermediate, PoL. This rate decreases with increasing ionic strength. (2) The intermediate isomerizes to the closed-conformation form, PcL. The results indicate that the high specificity, affinity, and rate of phosphate binding are not influenced by the noncomplementary electronegative surface potential of the cleft. That binding depends almost entirely on local dipolar interactions with the receptor has important ramification in electrostatic interactions in protein structures and in ligand recognition.

3D Printed Models of Cleft Palate Pathology for Surgical Education

PubMed Central

Lioufas, Peter A.; Quayle, Michelle R.; Leong, James C.

2016-01-01

Objective: To explore the potential viability and limitations of 3D printed models of children with cleft palate deformity. Background: The advantages of 3D printed replicas of normal anatomical specimens have previously been described. The creation of 3D prints displaying patient-specific anatomical pathology for surgical planning and interventions is an emerging field. Here we explored the possibility of taking rare pediatric radiographic data sets to create 3D prints for surgical education. Methods: Magnetic resonance imaging data of 2 children (8 and 14 months) were segmented, colored, and anonymized, and stereolothographic files were prepared for 3D printing on either multicolor plastic or powder 3D printers and multimaterial 3D printers. Results: Two models were deemed of sufficient quality and anatomical accuracy to print unamended. One data set was further manipulated digitally to artificially extend the length of the cleft. Thus, 3 models were printed: 1 incomplete soft-palate deformity, 1 incomplete anterior palate deformity, and 1 complete cleft palate. All had cleft lip deformity. The single-material 3D prints are of sufficient quality to accurately identify the nature and extent of the deformities. Multimaterial prints were subsequently created, which could be valuable in surgical training. Conclusion: Improvements in the quality and resolution of radiographic imaging combined with the advent of multicolor multiproperty printer technology will make it feasible in the near future to print 3D replicas in materials that mimic the mechanical properties and color of live human tissue making them potentially suitable for surgical training. PMID:27757345

Influence of the primary cleft palate closure on the future need for orthognathic surgery in unilateral cleft lip and palate patients.

PubMed

Broome, Martin; Herzog, George; Hohlfeld, Judith; de Buys Roessingh, Anthony; Jaques, Bertrand

2010-09-01

The aim of the study was to determine the influence of the dissection of the palate during primary surgery and the type of orthognathic surgery needed in cases of unilateral total cleft. The review concerns 58 children born with a complete unilateral cleft lip and palate and treated between 1994 and 2008 at the appropriate age for orthognathic surgery. This is a retrospective mixed-longitudinal study. Patients with syndromes or associated anomalies were excluded. All children were treated by the same orthodontist and by the same surgical team. Children are divided into 2 groups: the first group includes children who had conventional primary cleft palate repair during their first year of life, with extensive mucoperiosteal undermining. The second group includes children operated on according to the Malek surgical protocol. The soft palate is closed at the age of 3 months, and the hard palate at 6 months with minimal mucoperiosteal undermining. Lateral cephalograms at ages 9 and 16 years and surgical records were compared. The need for orthognathic surgery was more frequent in the first than in the second group (60% vs 47.8%). Concerning the type of orthognathic surgery performed, 2- or 3-piece Le Fort I or bimaxillary osteotomies were also less required in the first group. Palate surgery following the Malek procedure results in an improved and simplified craniofacial outcome. With a minimal undermining of palatal mucosa, we managed to reduce the amount of patients who required an orthognathic procedure. When this procedure was indicated, the surgical intervention was also greatly simplified.

A novel technique for presurgical nasoalveolar molding using computer-aided reverse engineering and rapid prototyping.

PubMed

Yu, Quan; Gong, Xin; Wang, Guo-Min; Yu, Zhe-Yuan; Qian, Yu-Fen; Shen, Gang

2011-01-01

To establish a new method of presurgical nasoalveolar molding (NAM) using computer-aided reverse engineering and rapid prototyping technique in infants with unilateral cleft lip and palate (UCLP). Five infants (2 males and 3 females with mean age of 1.2 w) with complete UCLP were recruited. All patients were subjected to NAM before the cleft lip repair. The upper denture casts were recorded using a three-dimensional laser scanner within 2 weeks after birth in UCLP infants. A digital model was constructed and analyzed to simulate the NAM procedure with reverse engineering software. The digital geometrical data were exported to print the solid model with rapid prototyping system. The whole set of appliances was fabricated based on these solid models. Laser scanning and digital model construction simplified the NAM procedure and estimated the treatment objective. The appliances were fabricated based on the rapid prototyping technique, and for each patient, the complete set of appliances could be obtained at one time. By the end of presurgical NAM treatment, the cleft was narrowed, and the malformation of nasoalveolar segments was aligned normally. We have developed a novel technique of presurgical NAM based on a computer-aided design. The accurate digital denture model of UCLP infants could be obtained with laser scanning. The treatment design and appliance fabrication could be simplified with a computer-aided reverse engineering and rapid prototyping technique.

Cleft Lip and Palate

MedlinePlus

Cleft lip and cleft palate are birth defects that occur when a baby's lip or mouth do not form properly. They happen early during ... A baby can have a cleft lip, a cleft palate, or both. A cleft lip happens if the ...

Cross-Cultural Attitudes toward Speech Disorders.

ERIC Educational Resources Information Center

Bebout, Linda; Arthur, Bradford

1992-01-01

University students (n=166) representing English-speaking North American culture and several other cultures completed questionnaires examining attitudes toward four speech disorders (cleft palate, dysfluency, hearing impairment, and misarticulations). Results showed significant group differences in beliefs about the emotional health of persons…

Hearing thresholds and ventilation tube treatment in children with unilateral cleft lip and palate.

PubMed

Tengroth, Birgitta; Hederstierna, Christina; Neovius, Erik; Flynn, Traci

2017-06-01

Children with cleft lip and palate have a high prevalence of otitis media with effusion (OME) which is often associated with a fluctuating, conductive hearing loss in the low and mid-frequencies and a risk for permanent hearing loss in the higher frequencies. Although common, there is no consensus on the treatment of OME with ventilation tubes. The aim of this study is to document if the risk for permanent hearing loss and acquired cholesteatoma increases due to treatment with ventilation tubes (VT treatments) during childhood in a group of children with cleft lip and palate. A retrospective medical chart review of 33 children (25 boys and 8 girls) born with unilateral cleft lip and palate (UCLP) was completed. Audiological data (results of hearing sensitivity tests, the total number of hearing tests, and number of VT treatments) were extracted from medical records from when the children were 4-7 and >7-10 years of age. The hearing thresholds in the speech frequencies improved with age (p < 0,05) but a minority of the children continued to present with elevated hearing thresholds in the higher frequencies at >7-10 years of age. There were no significant correlations between number of VT treatments and hearing thresholds at >7-10 years. Four of the 33 children presented with complications: two children exhibited perforations of the ear drum (6.1%) and two children developed unilateral cholesteatoma (6.1%). In the current study, the hearing sensitivity of children with cleft lip and palate improved with age. However, this improvement was not seen in the higher frequencies. Twelve percent of the children experienced complications following VT treatments. Due to these complications, it is recommended that all children with cleft palate should have routine follow-ups by an ENT doctor and audiologist. As part of the routine follow-up care, hearing assessments should be performed before and after VT treatments. Copyright © 2017 Elsevier B.V. All rights reserved.

Bone morphogenetic protein type I receptor inhibition induces cleft palate associated with micrognathia and cleft lower lip in mice.

PubMed

Lai, Yongzhen; Xie, Changfu; Zhang, Shixian; Gan, Guowu; Wu, Di; Chen, Weihui

2016-07-01

Gain-of- and loss-of-function studies have demonstrated that changes in bone morphogenetic protein (BMP) signaling during embryo development cause craniofacial malformations, including cleft palate. It remains uncertain whether BMP signaling could be targeted pharmacologically to affect craniofacial morphogenesis. Pregnant C57Bl/6J mice were treated with the BMP type I receptor inhibitor LDN-193189 at the dose of 3, 6, or 9 mg/kg twice a day by intraperitoneal injection from embryonic day 10.5 (E10.5) to E15.5. At E16.5, embryos were investigated by facial measurement analysis and histology to determine the optimal concentration for malformation. Subsequent embryonic phenotypes were analyzed in detail by histology, whole-mount skeletal staining, micro-computed tomography, and palatal organic culture. We further used immunohistochemistry to analyze protein expression of the BMP-mediated canonical and noncanonical signaling components. The optimal concentration of LDN-193189 was determined to be 6 mg/kg. In utero, LDN-193189 exposures induced partial clefting of the anterior palate or complete cleft palate, which was attributed to a reduced cell proliferation rate in the secondary palate, and delayed palatal elevation caused by micrognathia. Analysis of signal transduction in palatal shelves at E12.5 and E13.5 identified a significant reduction of BMP/Smad signaling (p-Smad1/5/8) and unchanged BMP noncanonical signaling (p-p38, p-Erk1/2) after treatment with LDN-193189. The results of this study indicate that LDN-193189 can be used to manipulate BMP signaling by selectively targeting the BMP/Smad signaling pathway to affect palatal morphogenesis and produce phenotypes mimicking those caused by genetic mutations. This work established a novel mouse model for teratogen-induced cleft palate. Birth Defects Research (Part A) 106:612-623, 2016. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

Oral health-related quality of life in youth receiving cleft-related surgery: self-report and proxy ratings.

PubMed

Broder, Hillary L; Wilson-Genderson, Maureen; Sischo, Lacey

2017-04-01

This paper evaluated the impact of cleft-related surgery on the oral health-related quality of life (OHRQoL) of youth with cleft over time. Data were derived from a 5-year, multi-center, prospective, longitudinal study of 1196 youth with cleft lip and/or palate and their caregivers. Eligible youth were between 7.5 and 18.5 years old, spoke English or Spanish, and were non-syndromic. During each observational period, which included baseline, and 1- and 2-year post-baseline follow-up visits, youths and their caregivers completed the Child Oral Health Impact Profile, a validated measure of OHRQoL. Multilevel mixed-effects models were used to analyze the effects of receipt of craniofacial surgery on OHRQoL over time. During the course of this study a total of 516 patients (43 %) received at least one surgery. Youth in the surgery recommendation group had lower self- (β = -2.18, p < 0.05) and proxy-rated (β = -2.92, p < 0.02) OHRQoL when compared to non-surgical self- and proxy-rated OHRQoL at baseline. Both surgical and non-surgical youth (β = 3.73, p < 0.001) and caregiver (β = 1.91, p < 0.05) ratings of OHRQoL improved over time. There was significant incremental improvement (time × surgery interaction) in self-reported OHRQoL for youth postsurgery (β = 1.04, p < 0.05), but this postsurgery increment was not seen in the caregiver proxy ratings. Surgical intervention impacts OHRQoL among youth with cleft. Youth who were surgical candidates had lower baseline self- and caregiver-rated OHRQoL when compared to non-surgical youth. Youth who underwent cleft-related surgery had significant incremental improvements in self-rated but not caregiver (proxy)-rated OHRQoL after surgery.

Association of single nucleotide polymorphisms in WNT genes with the risk of nonsyndromic cleft lip with or without cleft palate.

PubMed

Rafighdoost, Houshang; Hashemi, Mohammad; Asadi, Hossein; Bahari, Gholamreza

2018-01-22

Nonsyndromic cleft lip with or without cleft palate is a common congenital deformity worldwide with multifaceted etiology. Interaction of genes and environmental factors has been indicated to be related with susceptibility to nonsyndromic cleft lip with or without cleft palate. Some WNT genes which are involved in craniofacial embryogenesis may play a key role in the pathogenesis of nonsyndromic cleft lip with or without cleft palate. In the present study, we aimed to inspect the relationship between WNT3 (rs3809857 and rs9890413), WNT3A (rs752107 and rs3121310), and WNT10a rs201002930 (c.392 C>T) polymorphisms and nonsyndromic cleft lip with or without cleft palate in an Iranian population. The present case-control study was carried out on 120 unrelated nonsyndromic cleft lip with or without cleft palate patients and 112 healthy subjects. The variants were genotyped by polymerase chain reaction-restriction fragment length polymorphism method. The findings suggest that the rs3809857 polymorphism significantly decreased the risk of nonsyndromic cleft lip with or without cleft palate in codominant (odds ratio = 0.16, 95% confidence interval = 0.03-0.75, P = 0.020, TT vs GG), recessive (odds ratio = 0.16, 95% confidence interval = 0.03-0.72, P = 0.009, TT vs GG + GT) inheritance models. The rs9890413 variant marginally decreased the risk of nonsyndromic cleft lip with or without cleft palate in codominant (odds ratio = 0.41, 95% confidence interval = 0.17-0.99, P = 0.047, AG vs AA) model. Regarding C392T variant, the findings revealed that this variant significantly decreased the risk of nonsyndromic cleft lip with or without cleft palate in codominant (odds ratio = 0.24, 95% confidence interval = 0.10-0.58, P = 0.002, CT vs CC) and allele (odds ratio = 0.26, 95% confidence interval = 0.11-0.62, P = 0.002, T vs C) models. No significant association was observed between the rs752107 and rs3121310 variants and risk/protection of nonsyndromic cleft lip with or without cleft palate. Stratified analysis showed that WNT10a rs201002930 (c.392 C>T) significantly decreased the risk of cleft lip with cleft palate and cleft palate only. In summary, the results suggest an association between WNT genes polymorphisms and the risk nonsyndromic cleft lip with or without cleft palate in a sample of the southeast Iranian population. © 2018 Japanese Teratology Society.

Cleft palate - resources

MedlinePlus

Resources - cleft palate ... The following organizations are good resources for information on cleft palate : Cleft Palate Foundation -- www.cleftline.org March of Dimes -- www.marchofdimes.org/complications/cleft-lip-and- ...

Assessment of patients' level of satisfaction with cleft treatment using the Cleft Evaluation Profile.

PubMed

Noor, Siti Noor Fazliah Mohd; Musa, Sabri

2007-05-01

Determination of the psychosocial status and assessment of the level of satisfaction in Malaysian cleft palate patients and their parents. Cross-sectional study. Sixty cleft lip and palate patients (12 to 17 years of age) from Hospital Universiti Sains Malaysia and their parents were selected. The questionnaires used were the Child Interview Schedule, the Parents Interview Schedule, and the Cleft Evaluation Profile (CEP), administered via individual interviews. Patients were teased because of their clefts and felt their self-confidence was affected by the cleft condition. They were frequently teased about cleft-related features such as speech, teeth, and lip appearance. Parents also reported that their children were being teased because of their clefts and that their children's self-confidence was affected by the clefts. Both showed a significant level of satisfaction with the treatment provided by the cleft team. There was no significant difference between the responses of the patients and their parents. The features that were found to be most important for the patients and their parents, in decreasing order of priority, were teeth, nose, lips, and speech. Cleft lip and/or palate patients were teased because of their clefts, and it affected their self-confidence. The Cleft Evaluation Profile is a reliable and useful tool to assess patients' level of satisfaction with treatment received for cleft lip and/or palate and can identify the types of cleft-related features that are most important for the patients.

Cleft Palate; A Multidiscipline Approach.

ERIC Educational Resources Information Center

Stark, Richard B., Ed.

Nineteen articles present a multidisciplinary approach to the management of facial clefts. The following subjects are discussed: the history of cleft lip and cleft palate surgery; cogenital defects; classification; the operation of a cleft palate clinic; physical examination of newborns with cleft lip and/or palate; nursing care; anesthesia;…

The Primary Care Pediatrician and the Care of Children With Cleft Lip and/or Cleft Palate.

PubMed

Lewis, Charlotte W; Jacob, Lisa S; Lehmann, Christoph U

2017-05-01

Orofacial clefts, specifically cleft lip and/or cleft palate (CL/P), are among the most common congenital anomalies. CL/P vary in their location and severity and comprise 3 overarching groups: cleft lip (CL), cleft lip with cleft palate (CLP), and cleft palate alone (CP). CL/P may be associated with one of many syndromes that could further complicate a child's needs. Care of patients with CL/P spans prenatal diagnosis into adulthood. The appropriate timing and order of specific cleft-related care are important factors for optimizing outcomes; however, care should be individualized to meet the specific needs of each patient and family. Children with CL/P should receive their specialty cleft-related care from a multidisciplinary cleft or craniofacial team with sufficient patient and surgical volume to promote successful outcomes. The primary care pediatrician at the child's medical home has an essential role in making a timely diagnosis and referral; providing ongoing health care maintenance, anticipatory guidance, and acute care; and functioning as an advocate for the patient and a liaison between the family and the craniofacial/cleft team. This document provides background on CL/P and multidisciplinary team care, information about typical timing and order of cleft-related care, and recommendations for cleft/craniofacial teams and primary care pediatricians in the care of children with CL/P. Copyright © 2017 by the American Academy of Pediatrics.

Delayed detection of cleft palate: an audit of newborn examination.

PubMed

Habel, A; Elhadi, N; Sommerlad, B; Powell, J

2006-03-01

To identify prevalence of delayed detection of cleft palate, and associated factors that could lead to improved identification at neonatal clinical examination. Audit of hospital notes, parental questionnaire incorporating open ended questions, and telephone questionnaire of junior doctors in the referring hospitals incorporating fixed choice questions. Of 344 cleft palate patients without cleft lip or submucous cleft palate, the day the cleft was detected was recorded in 92%. Delayed detection, after the first day, was 28% overall, distributed as 37% with isolated cleft palate and 23% with syndromic cleft palate. Narrow V shaped clefts were more likely to be delayed in detection compared with broad U shaped clefts, as were soft palate clefts compared with hard palate clefts. Five with isolated cleft palates were not detected until after the first year. Babies born at home were unlikely to be detected on day 1. Symptoms were significantly increased in the delayed detection group for feeding problems and nasal regurgitation. A telephone questionnaire of trainee paediatricians in referring units revealed that digital examination was more commonly practised than visual inspection, and few recalled receiving specific instruction on examination of the palate. Delayed detection of cleft palate was not uncommon, and the features of those more likely to be missed suggested digital examination was related. Trainee doctors and midwives should be instructed to inspect visually using a light and tongue depressor, then digitally if submucous cleft palate is suspected.

Unique association of hypochondroplasia with craniosynostosis and cleft palate in a Mexican family.

PubMed

González-Del Angel, Ariadna; Caro-Contreras, Alan; Alcántara-Ortigoza, Miguel Angel; Ramos, Sandra; Cruz-Alcívar, Roberto; Moyers-Pérez, Paola

2018-01-01

Hypochondroplasia (HCH) is a skeletal dysplasia caused by an abnormal function of the fibroblast growth factor receptor 3. Although believed to be relatively common, its prevalence and phenotype are not well established owing to its clinical, radiological, and genetic heterogeneity. Here we report on a molecularly proven HCH family with an affected father and two children. The siblings (male and female) with HCH also had craniosynostosis and cleft palate, respectively. The present report supports the conclusion that the full clinical spectrum of HCH is not completely delineated. It also suggests that secondary, as yet unknown, modifying factors can influence the final phenotype. © 2017 Wiley Periodicals, Inc.

"With Some Families, Nothing Goes Right."

ERIC Educational Resources Information Center

Exceptional Parent, 1986

1986-01-01

A case history describes the effects on a family of a child whose cleft palate required frequent medical attention. Adjustments required by a completion of surgery are noted to have unsettling effects on the 10-year-old brother and his mother and father. (CL)

Clinical diagnostic exome evaluation for an infant with a lethal disorder: genetic diagnosis of TARP syndrome and expansion of the phenotype in a patient with a newly reported RBM10 alteration.

PubMed

Powis, Zöe; Hart, Alexa; Cherny, Sara; Petrik, Igor; Palmaer, Erika; Tang, Sha; Jones, Carolyn

2017-06-02

Diagnostic Exome Sequencing (DES) has been shown to be an effective tool for diagnosis individuals with suspected genetic conditions. We report a male infant born with multiple anomalies including bilateral dysplastic kidneys, cleft palate, bilateral talipes, and bilateral absence of thumbs and first toes. Prenatal testing including chromosome analysis and microarray did not identify a cause for the multiple congenital anomalies. Postnatal diagnostic exome studies (DES) were utilized to find a molecular diagnosis for the patient. Exome sequencing of the proband, mother, and father showed a previously unreported maternally inherited RNA binding motif protein 10 (RBM10) c.1352_1353delAG (p.E451Vfs*66) alteration. Mutations in RBM10 are associated with TARP syndrome, an X-linked recessive disorder originally described with cardinal features of talipes equinovarus, atrial septal defect, Robin sequence, and persistent left superior vena cava. DES established a molecular genetic diagnosis of TARP syndrome for a neonatal patient with a poor prognosis in whom traditional testing methods were uninformative and allowed for efficient diagnosis and future reproductive options for the parents. Other reported cases of TARP syndrome demonstrate significant variability in clinical phenotype. The reported features in this infant including multiple hemivertebrae, imperforate anus, aplasia of thumbs and first toes have not been reported in previous patients, thus expanding the clinical phenotype for this rare disorder.

Bright Promise for Your Child with Cleft Lip and Cleft Palate. Revised Edition.

ERIC Educational Resources Information Center

McDonald, Eugene T.; Berlin, Asa J.

Intended for parents of children with cleft lip and cleft palate, the booklet provides an overview of the condition. Addressed are the following topics (sample subtopics in parentheses): prenatal development and birth defects (facial development); possible causes of cleft lip/cleft palate (common misconceptions, genetic factors, environmental…

First branchial cleft anomalies have relevance in otology and more.

PubMed

Tham, Y S; Low, W K

2005-05-01

First branchial cleft anomalies account for less than 8% of all branchial abnormalities. Their rarity and diverse presentations have frequently led to misdiagnosis and inappropriate treatment. In a trend towards specialisation/subspecialisation, first branchial cleft duplication anomalies, with their varied clinical manifestations, may possibly present to an Otology, Head and Neck Surgery, Paediatric Otolaryngology, Maxillofacial or even a General Paediatric and General Surgery practice. There is a need to highlight the clinical features which can aid in accurate diagnosis. A case of an adult with Work Type 2 first branchial cleft duplication anomaly presenting as a collaural fistula is described. It first presented as a recurrent upper neck abscess in childhood. The diagnosis had previously been missed although the patient was able to clearly establish a correlation between digging of the ipsilateral ear and precipitation of the abscess. Instead of an epidermal web, a myringeal lesion in the form of a fibrous band-like was present. The lesion was completely excised with no further recurrence. This case highlights useful diagnostic features both from the history and physical examination. The specialist/subspecialist must be aware of this condition and be mindful of its possible cross specialty/subspecialty symptoms and signs. Together with a good understanding of the regional embryology and anatomy, the lesion can be diagnosed early at initial presentation with the potential for best treatment outcomes.

Hypertrophied tonsils impair velopharyngeal function after palatoplasty.

PubMed

Abdel-Aziz, Mosaad

2012-03-01

When tonsillar hypertrophy obstructing the airway is encountered in a child with a repaired cleft palate and velopharyngeal insufficiency, the surgeon may opt for tonsillectomy to relieve the airway obstruction, with possible effects on velopharyngeal closure. The aim of this study was to assess the impact of hypertrophied tonsils on velopharyngeal function in children with repaired cleft palate and to measure the effect of tonsillectomy on velopharyngeal closure and speech resonance. Case series. Twelve children with repaired cleft palate and tonsillar hypertrophy underwent tonsillectomy to relieve airway obstruction. Preoperative and postoperative evaluation of velopharyngeal function was performed. Auditory perceptual assessment of speech and nasalance scores were measured, and velopharyngeal closure was evaluated by flexible nasopharyngoscopy. Preoperative impairment of velopharyngeal function was detected. However, significant postoperative improvement of speech parameters (hypernasality, nasal emission of air, and weak pressure consonants measured with auditory perceptual assessment) was achieved, and the overall postoperative nasalance score was improved significantly for nasal and oral sentences. Reduction of velopharyngeal gap size was detected after removal of hypertrophied tonsils. Although the improvement of velopharyngeal closure was not significant, three cases demonstrated complete postoperative closure with no gap. Hypertrophied tonsils may impair velopharyngeal function in children with repaired cleft palate, and tonsillectomy is beneficial for such patients as it can improve the velopharyngeal closure and speech resonance. Secondary corrective surgery may be avoided in some cases after tonsillectomy. Copyright © 2012 The American Laryngological, Rhinological, and Otological Society, Inc.

Legal considerations in the management of cleft lip and palate.

PubMed

Berbert-Campos, Cláudia

2007-03-01

To inform professionals providing care to individuals with cleft lip and palate on the legal aspects and organizations available to protect individuals with this alteration, and to advocate that cleft lip and palate should be considered a handicap, even though it is provisional and may be rehabilitated, to assure afflicted individuals basic rights and complete personal, social, and economic welfare. Literature review on the issue, including assessment of national and international laws, doctrines, and jurisprudences; conceptual analysis of the word "handicapped" in dictionaries. Analysis included the federal constitutions of Brazil, France, Argentina, Spain, Cuba, Italy, China, Portugal, Japan, Great Britain, and Colombia, regarding the protection of handicapped people. Constitutional protection of handicapped people is a recent issue that has been addressed only in the last few decades in some countries such as Brazil, Italy, Spain, China, and Portugal. The Brazilian Federal Constitution of 1988 addresses the protection of handicapped people to assure them access to social and individual rights, freedom, security, welfare, development, equality, and justice as supreme values of a fraternal, pluralist, and prejudice-free society. Individuals with cleft lip and palate should be included in national policies for integration of handicapped people, in agreement with programs of human rights, establishing a collaborative action between state and society. This would assure their inclusion in the socioeconomic and cultural context and equal opportunities in society, without privileges or paternalism.

Pitfalls and solutions in virtual design of nasoalveolar molding plates by using CAD/CAM technology--A preliminary clinical study.

PubMed

Ritschl, Lucas M; Rau, Andrea; Güll, Florian D; diBora, Benjamin; Wolff, Klaus-Dietrich; Schönberger, Markus; Bauer, Franz X; Wintermantel, Erich; Loeffelbein, Denys J

2016-04-01

Computer-assisted design and computer-aided manufacturing (CAD/CAM) technology in nasoalveolar molding (NAM) should save time and manpower and reduce family input in cases of cleft lip and palate. Intraoral casts from 12 infants with complete unilateral cleft lip and palate were taken immediately after birth (T1) and after (T2) NAM treatment, digitalized, and transformed into STL data. The infants were randomized into Group 1 (n = 6) receiving conventional NAM treatment or Group 2 receiving CAD/CAM NAM (n = 6). We analyzed the following variables by using Geomagic software: intersegmental alveolar distance (ISAD); intersegmental lip distance (ISLD); nostril height cleft/noncleft (NHc/nc); nasal width cleft/noncleft (NWn/nc); and columella deviation angle (CDA). In both groups, all variables except NHnc and NWnc were changed significantly between T1 and T2. The analysis of the mean differences of the variables in Group 1 and 2 showed no significant differences, with a comparable incidence of clinical alterations such as skin or mucosal irritations. NAM plates can be produced virtually by using CAD/CAM technology. The CAD/CAM NAM results show no significant differences from the conventional technique. We present our clinically usable virtual CAD/CAM workflow for producing a basic NAM plate. Copyright © 2016 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Associations between maternal periconceptional exposure to secondhand tobacco smoke and major birth defects.

PubMed

Hoyt, Adrienne T; Canfield, Mark A; Romitti, Paul A; Botto, Lorenzo D; Anderka, Marlene T; Krikov, Sergey V; Tarpey, Morgan K; Feldkamp, Marcia L

2016-11-01

While associations between secondhand smoke and a few birth defects (namely, oral clefts and neural tube defects) have been noted in the scientific literature, to our knowledge, there is no single or comprehensive source of population-based information on its associations with a range of birth defects among nonsmoking mothers. We utilized data from the National Birth Defects Prevention Study, a large population-based multisite case-control study, to examine associations between maternal reports of periconceptional exposure to secondhand smoke in the household or workplace/school and major birth defects. The multisite National Birth Defects Prevention Study is the largest case-control study of birth defects to date in the United States. We selected cases from birth defect groups having >100 total cases, as well as all nonmalformed controls (10,200), from delivery years 1997 through 2009; 44 birth defects were examined. After excluding cases and controls from multiple births and whose mothers reported active smoking or pregestational diabetes, we analyzed data on periconceptional secondhand smoke exposure-encompassing the period 1 month prior to conception through the first trimester. For the birth defect craniosynostosis, we additionally examined the effect of exposure in the second and third trimesters as well due to the potential sensitivity to teratogens for this defect throughout pregnancy. Covariates included in all final models of birth defects with ≥5 exposed mothers were study site, previous live births, time between estimated date of delivery and interview date, maternal age at estimated date of delivery, race/ethnicity, education, body mass index, nativity, household income divided by number of people supported by this income, periconceptional alcohol consumption, and folic acid supplementation. For each birth defect examined, we used logistic regression analyses to estimate both crude and adjusted odds ratios and 95% confidence intervals for both isolated and total case groups for various sources of exposure (household only; workplace/school only; household and workplace/school; household or workplace/school). The prevalence of secondhand smoke exposure only across all sources ranged from 12.9-27.8% for cases and 14.5-15.8% for controls. The adjusted odds ratios for any vs no secondhand smoke exposure in the household or workplace/school and isolated birth defects were significantly elevated for neural tube defects (anencephaly: adjusted odds ratio, 1.66; 95% confidence interval, 1.22-2.25; and spina bifida: adjusted odds ratio, 1.49; 95% confidence interval, 1.20-1.86); orofacial clefts (cleft lip without cleft palate: adjusted odds ratio, 1.41; 95% confidence interval, 1.10-1.81; cleft lip with or without cleft palate: adjusted odds ratio, 1.24; 95% confidence interval, 1.05-1.46; cleft palate alone: adjusted odds ratio, 1.31; 95% confidence interval, 1.06-1.63); bilateral renal agenesis (adjusted odds ratio, 1.99; 95% confidence interval, 1.05-3.75); amniotic band syndrome-limb body wall complex (adjusted odds ratio, 1.66; 95% confidence interval, 1.10-2.51); and atrial septal defects, secundum (adjusted odds ratio, 1.37; 95% confidence interval, 1.09-1.72). There were no significant inverse associations observed. Additional studies replicating the findings are needed to better understand the moderate positive associations observed between periconceptional secondhand smoke and several birth defects in this analysis. Increased odds ratios resulting from chance (eg, multiple comparisons) or recall bias cannot be ruled out. Copyright © 2016 Elsevier Inc. All rights reserved.

Prevalence of cleft lip and cleft palate in rural north-central guatemala.

PubMed

Matute, Jorge; Lydick, Elaine A; Torres, Olga R; Owen, Karen K; Jacobsen, Kathryn H

2015-05-01

To estimate the number of new cases of cleft lip and cleft palate in the department (state) of Alta Verapaz, Guatemala, in 2012. Cross-sectional survey of midwives from communities identified through a two-stage cluster-sampling process. Midwives were asked how many babies they had delivered in the past year and how many of those newborns had various types of birth defects, as illustrated in pictures. Indigenous Mayan communities in rural north-central Guatemala. Midwives (n = 129) who had delivered babies in the previous year. Reports of babies born with cleft lip and cleft palate. A 1-year prevalence rate of 18.9 per 10,000 for cleft lip and 4.7 per 10,000 for cleft palate was estimated for Alta Verapaz. None of the cases of cleft lip also had cleft palate. The indigenous communities in north-central Guatemala might have a relatively high cleft lip prevalence rate compared with the global average.

Ectrodactyly-ectodermal dysplasia clefting syndrome (EEC syndrome).

PubMed

Koul, Monika; Dwivedi, Rahul; Upadhyay, Vinod

2014-01-01

Ectrodactyly-ectodermal dysplasia- clefting syndrome (also k/a. split hand- split foot malformation /split hand-split foot ectodermal dysplasia- cleft syndrome/ectodermal dysplasia cleft lip/cleft palate syndrome) a rare form of ectodermal dysplasia, is an autosomal dominant disorder inherited as a genetic trait and characterized by a triad of (i) ectrodactyly, (ii) ectodermal dysplasia and, (iii) & facial clefts.

Cleft Lip and Cleft Palate

MedlinePlus

... to obese women may have increased risk of cleft lip and palate. Males are more likely to have a cleft ... more common in females. In the United States, cleft lip and palate are reportedly most common in Native Americans and ...

Laterality of Oral Clefts and Academic Achievement.

PubMed

Gallagher, Emily R; Collett, Brent R; Barron, Sheila; Romitti, Paul; Ansley, Timothy; Wehby, George L

2017-02-01

Children with isolated oral clefts have lower academic performance when compared with unaffected peers, yet few studies have examined specific attributes of clefts that may modify this risk. Oral clefts have nonrandom laterality, with left-sided clefts being more common than right-sided clefts, a pattern that may be genetically or environmentally influenced. The objective of this study was to evaluate the association between cleft laterality and academic achievement in a population-based sample of children with and without isolated oral clefts. The study included 292 children with isolated unilateral cleft lip with or without cleft palate identified by using the Iowa Registry for Congenital and Inherited Disorders matched with 908 unaffected classmates. This group provided 1953 child-grade observations for cases and 6829 for classmates. Academic achievement was evaluated by using high-quality standardized test data on multiple academic domains as well as use of special education. We found that children with right-sided clefts had similar achievement scores and usage of special education services compared with their unaffected classmates. Children with left-sided clefts had lower reading scores than children with right-sided clefts by nearly 7 percentiles (P < .05). They also had lower scores on all evaluated domains by 4 to 6 percentiles and greater use of special education services by 6 percentage points than their classmates. Children with left-sided clefts had poorer academic performance than their classmates or children with right-sided clefts, who showed similar academic achievement compared with their unaffected classmates. Copyright © 2017 by the American Academy of Pediatrics.

Centre-based statistics of cleft lip with/without alveolus and palate as well as cleft palate only patients in Aden, Yemen.

PubMed

Esmail, Ahlam Hibatulla Ali; Abdo, Muhgat Ahmed Ali; Krentz, Helga; Lenz, Jan-Hendrik; Gundlach, Karsten K H

2014-06-01

The purpose of the study was to report the types and patterns of cleft lip with/without cleft alveolus and palate as well as cleft palate only as seen in Aden, Yemen. Retrospective, centre-based study conducted at the Cleft Lip and Palate Centre, Aden University, Yemen. Statistical evaluation of the data from all cleft patients who were registered at or referred to this centre during the years 2005-2011. A total of 1110 cleft patients were seen during the period studied (2005-2011). Amongst these there were 183 (16.48%) with a cleft lip and 144 (12.98) with a cleft of lip and alveolus, 228 (20.54%) had a cleft palate, and 555 (50%) had a combination of cleft lip, alveolus, and palate. The clefts were found more often in males than in females (56.5% boys versus 43.5% girls). This difference was statistically significant (p ≤ 0.001). Statistically significant sex differences were also noted when evaluating the various cleft types. Isolated cleft palates were found most often in females. Among the cleft palate cases there were 102 (9.2%) with a cleft soft palate only. The ages of the patients were between one day and 40 years. Two hundred and one children (18%) had a positive family history of clefts. Among the risk factors considered in this study, consanguineous marriages among cousins were found most frequently (in 48% of the cases). In contrast to this, only 10% of the mothers had reported to have been taking medication directly prior to or during the first trimester of their pregnancy. On average the mothers were neither very young nor very old. The prevalence rate of orofacial cleft types among this Yemeni sample was similar to prevalence rates previously reported in white Caucasians. The present study did neither find many cases with medication before, nor during, pregnancy; there were few young or very old mothers; and the incidence of positive family histories was similar to those found in other studies on clefts. However, consanguineous marriages were encountered quite often. Copyright © 2013 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Medical care for 4- to 5-year-olds with cleft lip/palate at the Tawanchai Center: geoinformatics study.

PubMed

Pattaranit, Rumpan; Songsung, Chainarong; Simmalee, Krisda; Pradubvong, Suteera; Thanapaisal, Chaiwit

2012-11-01

The incidence of cleft lip and cleft palate in Thailand is based on reports from three hospitals in Northeastern Thailand. There are 2.49 new babies born with cleft lip and cleft palate and craniofacial deformities for every 1,000 babies the highest incidence in the world. Area 7 Office of the National Health Security Office (NHSO) comprises Khon Kaen, Roi Et, Mahasarakam and Kalasin. The number of newborns with cleft lip and palate is an estimated 114 cases/year. A retrospective geoinformatics study was conducted using the medical records of 4- to 5-year-olds with cleft lip and cleft palate registered with the Tawanchai Center Srinagarind Hospital. To study the distribution of 4- to 5-year-olds with cleft lip and palate using geoinformatics. This retrospective study was conducted using data from the medical records, which contains the records of 4- to 5-year-olds with cleft lip and cleft palate admitted to Srinagarind Hospital. The 54-patient sample groups were registered at the Tawanchai Center Faculty of Medicine, Khon Kaen University, under the aegis of the NHSO (Area 7-Khon Kaen). The data were collected using self-administered questionnaires between May and June 2012 and geoinformatics, to study the distribution of patients' vis-a-vis the various parameters. Among the 27 male and 27femalepatients, 51.85% had cleft lip with cleft palate. Most of the cleft lip patients (7 cases; 41.18%) were in Khon Kaen, while 4 (44.44%) of the cleft palate patients were in Roi-Et and 11 (39.29%) of the cleft lip with palate patients were in Khon Kaen. Most cleft lip patients were in Roi-Et province: 1 case in each of four districts. Similarly, most cases of cleft palate were in Roi-Et province: 1 case in each of four districts. Finally, most cases (5; 50%) of cleft lip with cleft palate were found in Roi-Et province, mostly in the central (Muang) district. The use of Arc View software as an instrument for geoinformatics, spatial data collection helped to clarify the district level distribution of patients with CLP; this information is needed for policy planning and resource allocation.

Prevalence of orofacial clefts and risks for nonsyndromic cleft lip with or without cleft palate in newborns at a university hospital from West Mexico.

PubMed

Corona-Rivera, Jorge Román; Bobadilla-Morales, Lucina; Corona-Rivera, Alfredo; Peña-Padilla, Christian; Olvera-Molina, Sandra; Orozco-Martín, Miriam A; García-Cruz, Diana; Ríos-Flores, Izabel M; Gómez-Rodríguez, Brian Gabriel; Rivas-Soto, Gemma; Pérez-Molina, J Jesús

2018-02-19

We determined the overall prevalence of typical orofacial clefts and the potential risks for nonsyndromic cleft lip with or without cleft palate in a university hospital from West México. For the prevalence, 227 liveborn infants with typical orofacial clefts were included from a total of 81,193 births occurred during the period 2009-2016 at the "Dr. Juan I. Menchaca" Civil Hospital of Guadalajara (Guadalajara, Jalisco, Mexico). To evaluate potential risks, a case-control study was conducted among 420 newborns, including only those 105 patients with nonsyndromic cleft lip with or without cleft palate (cases), and 315 infants without birth defects (controls). Data were analyzed using multivariable logistic regression analysis expressed as adjusted odds ratio with 95% confidence intervals . The overall prevalence for typical orofacial clefts was 28 per 10,000 (95% confidence interval: 24.3-31.6), or 1 per 358 live births. The mean values for the prepregnancy weight, antepartum weight, and pre-pregnancy body mass index were statistically higher among the mothers of cases. Infants with nonsyndromic cleft lip with or without cleft palate had a significantly higher risk for previous history of any type of congenital anomaly (adjusted odds ratio: 2.7; 95% confidence interval: 1.4-5.1), history of a relative with cleft lip with or without cleft palate (adjusted odds ratio: 19.6; 95% confidence interval: 8.2-47.1), and first-trimester exposures to progestogens (adjusted odds ratio: 6.8; 95% CI 1.8-25.3), hyperthermia (adjusted odds ratio: 3.4; 95% confidence interval: 1.1-10.6), and common cold (adjusted odds ratio: 3.6; 95% confidence interval: 1.1-11.9). These risks could have contributed to explain the high prevalence of orofacial clefts in our region of Mexico, emphasizing that except for history of relatives with cleft lip with or without cleft palate, most are susceptible of modification. © 2018 Japanese Teratology Society.

Improved Early Cleft Lip and Palate Complications at a Surgery Specialty Center in the Developing World.

PubMed

Park, Eugene; Deshpande, Gaurav; Schonmeyr, Bjorn; Restrepo, Carolina; Campbell, Alex

2018-01-01

To evaluate complication rates following cleft lip and cleft palate repairs during the transition from mission-based care to center-based care in a developing region. We performed a retrospective review of 3419 patients who underwent cleft lip repair and 1728 patients who underwent cleft palate repair in Guwahati, India between December 2010 and February 2014. Of those who underwent cleft lip repair, 654 were treated during a surgical mission and 2765 were treated at a permanent center. Of those who underwent cleft palate repair, 236 were treated during a surgical mission and 1491 were treated at a permanent center. Two large surgical missions to Guwahati, India, and the Guwahati Comprehensive Cleft Care Center (GCCCC) in Assam, India. Overall complication rates following cleft lip and cleft palate repair. Overall complication rates following cleft lip repair were 13.2% for the first mission, 6.7% for the second mission, and 4.0% at GCCCC. Overall complication rates following cleft palate repair were 28.0% for the first mission, 30.0% for the second mission, and 15.8% at GCCCC. Complication rates following cleft palate repair by the subset of surgeons permanently based at GCCCC (7.2%) were lower than visiting surgeons ( P < .05). Our findings support the notion that transitioning from a mission-based model to a permanent facility-based model of cleft care delivery in the developing world can lead to decreased complication rates.

Diprosopus (partially duplicated head) associated with anencephaly: a case report.

PubMed

al Muti Zaitoun, A; Chang, J; Booker, M

1999-01-01

Craniofacial duplication (diprosopus) is a rare form of conjoined twin. A 16 year old mother with a twin pregnancy delivered one normally formed baby boy and one diprosopus male. The malformed baby was 33 weeks of gestation with a single trunk, normal limbs and various degrees of facial duplication. Of the following structures there were two of each: noses, eyes, ears (and one dimple), mouths, tongues and, with bilateral central cleft lips and cleft palates. This was associated with holoprosencephaly and craniorachischisis. Internal organs showed no duplication. There were multiple congenital anomalies including diaphragmatic hernia, small lungs, two lobes of the right lung, ventricular septal defect, small adrenal gland and small left kidney with short ureter. The body also had a short neck, small chest cavities and kyphosis. X-ray revealed duplication of the vertebral column. The case presented here represents a type II of diprosopia of Rating (1933) and is the least common type reported. We also reviewed 22 recently reported cases of diprosopus. In addition to facial duplication, anencephaly, neural tube defect and cardiac malformations represent the more common congenital abnormalities associated with diprosopus. The pathogenesis of diprosopus is not well understood. Factors that play a role in diprosopus are probably similar to those factors (genetic, environmental and abnormal placental circulation) which affect monozoygotic twins as observed in this case report. Early ultrasonography diagnosis of diprosopus permits one to consider a vaginal therapeutic abortion.

Oral health-related quality of life, and satisfaction with treatment and treatment outcomes of adolescents/young adults with cleft lip/palate: an exploration.

PubMed

Munz, S M; Edwards, S P; Inglehart, M R

2011-08-01

The objectives of this correlational study were to explore whether the oral health-related quality of life (ohrqol) of adolescents/young adults with cleft lip/palate (CL/P) relates to their own and their parents' satisfaction with treatment and treatment outcomes. Data were collected using mailed surveys from 30 parents and 27 patients who completed CL/P treatment. Patients' ohrqol was assessed with the Michigan Oral Health-Related Quality of Life (MOHRQoL) Scale, treatment satisfaction with Kiyak's Post-Surgical Patient Satisfaction Questionnaire, and satisfaction with treatment outcomes with Noor and Musa's Cleft Evaluation Profile. Overall, patients reported a positive ohrqol. Their satisfaction scores ranged from low to high. Patient and parent treatment satisfaction was related, while their outcome satisfaction did not correlate. Patients' MOHRQoL scores correlated with parent and patient treatment satisfaction and parents' outcome satisfaction. Patients' MOHRQoL scores did not correlate with patients' outcome satisfaction. The patients' level of discomfort was strongly correlated with patients' and parents' treatment satisfaction and parents' outcome satisfaction. In conclusion, regardless of outcome satisfaction, young patients with CL/P report a quite positive ohrqol after treatment completion. There is a clear relationship between the ohrqol of these patients with CL/P and their own as well as their parents' treatment satisfaction assessments. Copyright © 2011 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Speech outcome in unilateral complete cleft lip and palate patients: a descriptive study.

PubMed

Rullo, R; Di Maggio, D; Addabbo, F; Rullo, F; Festa, V M; Perillo, L

2014-09-01

In this study, resonance and articulation disorders were examined in a group of patients surgically treated for cleft lip and palate, considering family social background, and children's ability of self monitoring their speech output while speaking. Fifty children (32 males and 18 females) mean age 6.5 ± 1.6 years, affected by non-syndromic complete unilateral cleft of the lip and palate underwent the same surgical protocol. The speech level was evaluated using the Accordi's speech assessment protocol that focuses on intelligibility, nasality, nasal air escape, pharyngeal friction, and glottal stop. Pearson product-moment correlation analysis was used to detect significant associations between analysed parameters. A total of 16% (8 children) of the sample had severe to moderate degree of nasality and nasal air escape, presence of pharyngeal friction and glottal stop, which obviously compromise speech intelligibility. Ten children (10%) showed a barely acceptable phonological outcome: nasality and nasal air escape were mild to moderate, but the intelligibility remained poor. Thirty-two children (64%) had normal speech. Statistical analysis revealed a significant correlation between the severity of nasal resonance and nasal air escape (p ≤ 0.05). No statistical significant correlation was found between the final intelligibility and the patient social background, neither between the final intelligibility nor the age of the patients. The differences in speech outcome could be explained with a specific, subjective, and inborn ability, different for each child, in self-monitoring their speech output.

Musculo-nasomucosal unit with complete lateral bony freeing and medial rotation for ideal C-shape restoration and retropositioning of the levator veli palatini.

PubMed

El-Shazly, Mohamed

2012-11-01

No definitive procedure for cleft repair has been identified yet as the gold standard. Accordingly, this work tried to appraise the hypothesis that if the bony detachment and full retropositioning of the levator veli palatini muscle can ideally present an anatomical C-shape muscular sling restoration and if this is accompanied with pushback palatoplasty, would this present a better result in terms of tissue fistulation and phonetic impairment? A series of 74 different degrees of palatal clefts were operated by pushback palatoplasty combined with a modified approach of the levator vili palatini. This muscle was dissected only from the oral mucosa while kept attached to the nasal one as a musculo-nasomucosal unit. This unit was completely detached from the bony margin of the hard palate and then medially rotated and retropositioned in a typical C-shape mobile sling. Evaluations included suture line assessment and fistula development, and following the child's need for speech therapy. There were no intraoperative complications. Definite anterior fistulae with nasal air and foot leakage were observed in 2 cases. Four cases had postoperative velopharyngeal incompetence with a need for speech therapy. Tension-free closure, lower risk of fistula, good restoration of velopharyngeal functions, ability to be performed on all cleft types, ability to provide a good intraoperative exposure, and being a single stage seem to be the most important advantages of this unpublished technique.

Dental Care for a Child with Cleft Lip and Palate

MedlinePlus

... Volunteer Efforts Dental Care for a Child with Cleft Lip and Palate skip to submenu What We Do Cleft & Craniofacial ... version of this factsheet, click here How does cleft lip/palate affect the teeth? A cleft of the lip, ...

Clinical Study of Second Branchial Cleft Anomalies.

PubMed

Lee, Dong Hoon; Yoon, Tae Mi; Lee, Joon Kyoo; Lim, Sang Chul

2018-03-30

The objective of this study was to review the clinical characteristics and surgical treatment outcomes of second branchial cleft anomalies, and to evaluate the usefulness and accuracy of preoperative fine-needle aspiration cytology (FNAC) in the diagnosis of branchial cleft cysts. A retrospective chart review was performed at Chonnam National University Hwasun Hospital from January 2010 to December 2016. Among 25 patients with second branchial cleft anomalies, in 23 patients (92.0%), these anomalies presented as cysts, and in the remaining 2 patients (8.0%), these anomalies presented as fistulas. Fine-needle aspiration cytology had a diagnostic sensitivity of 100%, a positive-predictive value of 100%, and accuracy of 100% for diagnosing second branchial cleft cyst. All patients of second branchial cleft anomalies were treated surgically under general anesthesia. No recurrence of second branchial cleft anomalies was observed. Branchial cleft cysts were the most common type of second branchial cleft anomalies. Preoperative FNAC is a useful and accurate method for preoperative evaluation of branchial cleft cysts. Surgical excision of second branchial cleft anomalies is the treatment of choice without any complications and with no recurrence.

Identification of mutations in TCOF1: use of molecular analysis in the pre- and postnatal diagnosis of Treacher Collins syndrome.

PubMed

Dixon, Jill; Ellis, Ian; Bottani, Armand; Temple, Karen; Dixon, Michael James

2004-06-15

Treacher Collins syndrome (TCS) is an autosomal dominant disorder of facial development, which results from mutations in TCOF1. TCS comprises conductive hearing loss, hypoplasia of the mandible and maxilla, downward sloping palpebral fissures and cleft palate. Although, there is usually a reasonable degree of bilateral symmetry, a high degree of both inter- and intrafamilial variability is characteristic of TCS. The wide variation in the clinical presentation of different patients, together with the fact that more than 60% of cases arise de novo, can complicate the diagnosis of mild cases and genetic counselling. In the current study, we describe how molecular techniques have been used to facilitate pre- and postnatal disease diagnoses in 13 TCS families. Copyright 2004 Wiley-Liss, Inc.

The Correlation between Maternal Exposure to Air Pollution and the Risk of Orofacial Clefts in Infants: a Systematic Review and Meta-Analysis

PubMed Central

Rao, Ajit; Ahmed, Mairaj K.; Taub, Peter J.

2016-01-01

ABSTRACT Objectives The authors performed a systematic review and meta-analysis to investigate the possible correlation between ambient air pollution and orofacial cleft anomalies in newborns. Material and Methods A literature search was performed using the PubMed and Google Scholar, using the keywords “air pollution”, “cleft lip”, “cleft palate”, “carbon monoxide”, “ozone”, “sulfur dioxide”, “nitrogen oxide”, “nitrogen dioxide”, and “aerodynamic diameter”. Eight epidemiologic articles met the criteria of correlating either carbon monoxide (CO), ozone (O3), nitrogen oxides (NOx), airborne particulate matter of less than 10 µm in diameter (PM10), or sulfur dioxide (SO2) exposures with clefting of the palate alone, clefting of the lip alone, or clefting of the lip and palate. Odds ratios were extracted from the eight studies and tabulated in this meta-analysis. Quality analysis showed six high quality, one medium quality, and one low quality study. Results Meta-analysis of the combined data confirmed the association of O3 exposure and risk of orofacial cleft anomalies (OR = 1.08; P = 0.02). NOx was consistently associated with decreased risk of cleft lip with or without palate and cleft palate. Conclusions Ozone showed the strongest correlation with cleft lip and cleft palate anomalies. However, the studies overall showed an inconsistent correlation between orofacial clefts and air pollutants. PMID:27099696

Morphological evaluation of clefts of the lip, palate, or both in dogs.

PubMed

Peralta, Santiago; Fiani, Nadine; Kan-Rohrer, Kimi H; Verstraete, Frank J M

2017-08-01

OBJECTIVE To systematically characterize the morphology of cleft lip, cleft palate, and cleft lip and palate in dogs. ANIMALS 32 client-owned dogs with clefts of the lip (n = 5), palate (23), or both (4) that had undergone a CT or cone-beam CT scan of the head prior to any surgical procedures involving the oral cavity or face. PROCEDURES Dog signalment and skull type were recorded. The anatomic form of each defect was characterized by use of a widely used human oral-cleft classification system on the basis of CT findings and clinical images. Other defect morphological features, including shape, relative size, facial symmetry, and vomer involvement, were also recorded. RESULTS 9 anatomic forms of cleft were identified. Two anatomic forms were identified in the 23 dogs with cleft palate, in which differences in defect shape and size as well as vomer abnormalities were also evident. Seven anatomic forms were observed in 9 dogs with cleft lip or cleft lip and palate, and most of these dogs had incisive bone abnormalities and facial asymmetry. CONCLUSIONS AND CLINICAL RELEVANCE The morphological features of congenitally acquired cleft lip, cleft palate, and cleft lip and palate were complex and varied among dogs. The features identified here may be useful for surgical planning, developing of clinical coding schemes, or informing genetic, embryological, or clinical research into birth defects in dogs and other species.

Hearing outcomes in patients with cleft lip/palate.

PubMed

Skuladottir, Hildur; Sivertsen, Ase; Assmus, Jorg; Remme, Asa Rommetveit; Dahlen, Marianne; Vindenes, Hallvard

2015-03-01

Objective : Children with cleft lip and palate or cleft palate only have a high incidence of conductive hearing loss from otitis media with effusion. Studies demonstrating longitudinal results are lacking. This study was undertaken to investigate long-term longitudinal hearing outcomes of children with cleft lip and/or cleft palate and cleft palate only. Design : Retrospective chart review. Setting : Clinical charts of patients born with cleft lip and palate or cleft palate only in 1985 to 1994 who were referred to the cleft team in Bergen, Norway. Study findings include 15 years of follow-up. Participants : The study population consisted of 317 children of whom 159 had nonsyndromic cleft lip and palate and 158 had nonsyndromic cleft palate. Main Outcome Measures : Pure tone average calculated from pure tone audiometry at ages 4, 6, and 15 years. Results : The median pure tone average significantly improved with increasing age. For the cleft lip and palate group, the median pure tone average at ages 4, 6, and 15 years was 16 dB hearing level (HL), 13 dB HL, and 9 dB HL, respectively (P ≤ .001). In the cleft palate group the median pure tone average at ages 4, 6, and 15 years was 15 dB HL, 12 dB HL, and 9 dB HL, respectively (P ≤ .001). There was no significant difference in the hearing levels between the two groups. Patients who had surgical closure of the palate at age 18 months had a significantly better pure tone average outcome at age 15 compared with patients who had surgery at 12 months. Conclusions : Hearing improves significantly from childhood to adolescence in patients with cleft lip and palate and cleft palate only.

Description of Mexican Cleft Surgeons' Experience With Foreign Surgical Volunteer Missions in Mexico.

PubMed

Schoenbrunner, Anna R; Kelley, Kristen D; Buckstaff, Taylor; McIntyre, Joyce K; Sigler, Alicia; Gosman, Amanda A

2018-05-01

Mexican cleft surgeons provide multidisciplinary comprehensive cleft lip and palate care to children in Mexico. Many Mexican cleft surgeons have extensive experience with foreign, visiting surgeons. The purpose of this study was to characterize Mexican cleft surgeons' domestic and volunteer practice and to learn more about Mexican cleft surgeons' experience with visiting surgeons. A cross-sectional validated e-mail survey tool was sent to Mexican cleft surgeons through 2 Mexican plastic surgery societies and the Asociación Mexicana de Labio y Paladar Hendido y Anomalías Craneofaciales, the national cleft palate society that includes plastic and maxillofacial surgeons who specialize in cleft surgery. We utilized validated survey methodology, including neutral fact-based questions and repeated e-mails to survey nonresponders to maximize validity of statistical data; response rate was 30.6% (n = 81). Mexican cleft surgeons performed, on average, 37.7 primary palate repairs per year with an overall complication rate of 2.5%; 34.6% (n = 28) of respondents had direct experience with patients operated on by visiting surgeons; 53.6% of these respondents performed corrective surgery because of complications from visiting surgeons. Respondents rated 48% of the functional outcomes of visiting surgeons as "acceptable," whereas 43% rated aesthetic outcomes of visiting surgeons as "poor"; 73.3% of respondents were never paid for the corrective surgeries they performed. Thirty-three percent of Mexican cleft surgeons believe that there is a role for educational collaboration with visiting surgeons. Mexican cleft surgeons have a high volume of primary cleft palate repairs in their domestic practice with good outcomes. Visiting surgeons may play an important role in Mexican cleft care through educational collaborations that complement the strengths of Mexican cleft surgeons.

Anterior segment dysgenesis associated with Williams-Beuren syndrome: a case report and review of the literature.

PubMed

Todorova, Margarita G; Grieshaber, Matthias C; Cámara, Rafael J A; Miny, Peter; Palmowski-Wolfe, Anja M

2014-05-21

Williams-Beuren syndrome is characterized by mild mental retardation, specific neurocognitive profile, hypercalcemia during infancy, distinctive facial features and cardiovascular diseases. We report on complete ophthalmologic, sonographic and genetic evaluation of a girl with a clinical phenotype of Williams-Beuren syndrome, associated with unilateral anterior segment dysgenesis and bilateral cleft of the soft and hard palate. These phenotypic features have not been linked to the haploinsufficiency of genes involved in the microdeletion. A term born girl presented at the initial examination with clouding of the right cornea. On ultrasound biomicroscopy the anterior chamber structures were difficult to differentiate, showing severe adhesions from the opacified cornea to the iris with a kerato-irido-lenticular contact to the remnant lens, a finding consistent with Peters' anomaly. Genetic analyses including FISH confirmed a loss of the critical region 7q11.23, usually associated with the typical Williams-Beuren syndrome. Microsatellite analysis showed a loss of about 2.36 Mb. A diagnosis of Williams-Beuren syndrome was made based on the microdeletion of 7q11.23. The unique features, including unilateral microphthalmia and anterior segment dysgenesis, were unlikely to be caused by the microdeletion. Arguments in favor of the latter are unilateral manifestation, as well as the fact that numerous patients with deletions of comparable or microscopically visible size have not shown similar manifestations.

Evidence-Based Medicine: Cleft Palate.

PubMed

Woo, Albert S

2017-01-01

After studying this article, the participant should be able to: 1. Describe the incidence of cleft palate and risk factors associated with development of an orofacial cleft. 2. Understand differences among several techniques to repair clefts of both the hard and soft palates. 3. Discuss risk factors for development of postoperative fistulas, velopharyngeal insufficiency, and facial growth problems. 4. Establish a treatment plan for individualized care of a cleft palate patient. Orofacial clefts are the most common congenital malformations of the head and neck region, and approximately three-quarters of these patients have some form of cleft palate deformity. Cleft palate repair is generally performed in children between 6 and 12 months of age. The goals of palate repair are to minimize the occurrence of fistulas, establish a normal velopharyngeal mechanism, and optimize facial growth. This Maintenance of Certification review discusses the incidence and epidemiology associated with cleft palate deformity and specifics associated with patient care, including analgesia, surgical repair techniques, and complications associated with repair of the cleft palate.

Maxillary growth in a congenital cleft palate canine model for surgical research.

PubMed

Paradas-Lara, Irene; Casado-Gómez, Inmaculada; Martín, Conchita; Martínez-Sanz, Elena; López-Gordillo, Yamila; González, Pablo; Rodríguez-Bobada, Cruz; Chamorro, Manuel; Arias, Pablo; Maldonado, Estela; Ortega, Ricardo; Berenguer, Beatriz; Martínez-Álvarez, Concepción

2014-01-01

We have recently presented the Old Spanish Pointer dog, with a 15-20% spontaneous congenital cleft palate rate, as a unique experimental model of this disease. This study aimed to describe the cleft palate of these dogs for surgical research purposes and to determine whether congenital cleft palate influences maxillofacial growth. Seven newborn Old Spanish Pointer dogs of both sexes, comprising a cleft palate group (n = 4) and a normal palate group (n = 3), were fed using the same technique. Macroscopic photographs and plaster casts from the palate, lateral radiographs and computer tomograms of the skull were taken sequentially over 41 weeks, starting at week 5. The cleft morphology, the size and the tissue characteristics in these dogs resembled the human cleft better than current available animal models. During growth, the cleft width varies. Most of the transverse and longitudinal measures of the palate were statistically lower in the cleft palate group. The cleft palate group showed hypoplasia of the naso-maxillary complex. This model of congenital cleft palate seems suitable for surgical research purposes. A reduced maxillofacial pre- and post-natal development is associated to the congenital cleft palate in the Old Spanish Pointer dog. Copyright © 2013 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Maternal underweight and obesity and risk of orofacial clefts in a large international consortium of population-based studies.

PubMed

Kutbi, Hebah; Wehby, George L; Moreno Uribe, Lina M; Romitti, Paul A; Carmichael, Suzan; Shaw, Gary M; Olshan, Andrew F; DeRoo, Lisa; Rasmussen, Sonja A; Murray, Jeffrey C; Wilcox, Allen; Lie, Rolv T; Munger, Ronald G

2017-02-01

Evidence on association of maternal pre-pregnancy weight with risk of orofacial clefts is inconsistent. Six large case-control studies of orofacial clefts from Northern Europe and the USA were included in analyses pooling individual-level data. Cases included 4943 mothers of children with orofacial clefts (cleft lip only: 1135, cleft palate with cleft lip: 2081, cleft palate only: 1727) and controls included 10 592 mothers of unaffected children. Association of orofacial cleft risk with pre-pregnancy maternal weight classified by level of body mass index (BMI, kg/m 2 ) was evaluated using logistic regression adjusting for multiple covariates. Cleft palate, both alone and with cleft lip (CP+/-CL), was associated with maternal class II+ pre-pregnancy obesity (≥ 35)compared with normal weight [adjusted odds ratio (aOR) = 1.36; 95% confidence interval (CI) = 1.16, 1.58]. CP+/-CL was marginally associated with maternal underweight (aOR = 1.16; 95% CI = 0.98, 1.36). Cleft lip alone was not associated with BMI. In this largest population-based study to date, we found an increased risk of cleft palate, with or without cleft lip, in class II+ obese mothers compared with normal-weight mothers; underweight mothers may also have an increased risk, but this requires further study. These results also suggest that extremes of weight may have a specific effect on palatal development. © The Author 2016; all rights reserved. Published by Oxford University Press on behalf of the International Epidemiological Association

CIRPLAST: Cleft Lip and Palate Missions in Peru.

PubMed

Navarro, Carlos E

2015-06-01

The author presents a 20-year experience leading cleft lip and palate surgical volunteer missions in Peru for CIRPLAST, a nonprofit volunteer plastic surgery goodwill program that has provided free surgery for patients with cleft lip and palate deformities in remote areas of Peru. Surgical procedures were performed by the author, together with a group of experienced plastic surgeons, under the auspices of the Peruvian Plastic Surgery Society, and local health authorities. CIRPLAST missions are scheduled annually in different locations around Peru. Selected patients for surgery after adequate screening are photographed, and their cleft deformity is recorded. Scheduled patients or their parents, when they are minors, sign an informed consent form. Patients operated on in any given day are examined and photographed 1 day after surgery, before discharge. Between 30 and 35 patients are operated on at each mission site. About 2 weeks after the mission, patients are checked and photographed, and the outcome of surgery is recorded. Complications that may occur are recorded and treated by the CIRPLAST team as soon as possible. Almost all operations are performed under general endotracheal anesthesia coupled by local anesthesia containing a vasoconstrictor, to reduce bleeding and facilitate tissue dissection. All wounds of the lip and palate are closed with absorbable sutures, to avoid the need for suture removal. After cleft lip surgery, patients go to the recovery room for monitoring by nurses until they recover completely. A total of 6108 cleft lip and palate repairs, primary and secondary, were performed by CIRPLAST in 141 missions, between May 12, 1994, and October 15, 2014. The medical records of the 5162 patients (84.5%) who returned for follow-up (ranging from 12 days to 9 years) were reviewed retrospectively. Between 45% and 70% of the patients operated on a mission have returned for early follow-up and some the following year. There were 3176 males (51.9%) and 2932 females (48.1%). The incidence of isolated lip clefts was 1546 patients (25.3%); of isolated palate clefts, 2223 patients (36.4%); and combined defects, 2339 patients (38.3%). Of the 5162 patients who returned for follow-up, 377 patients (7.3%) had complications. Lip wound dehiscence was present in 58 patients (15.4). Palate fistula formation in 33 patients (8.8%): 24 (6.4%) after primary palate closure, and 9 (2.4%) after previous fistula closure. Infection occurred in 37 cleft lip patients (9.8%). Hypertrophic lip scars were seen in 56 patients (14.9%). Bleeding occurred in the recovery room after palatoplasty in 48 patients (12.7%), and in most cases, it was contained by applying pressure. No blood transfusions were used. Residual deformities of varying degree of the nose and/or lip occurred in 145 patients (38.5%). All required reoperation for correction. There were no intraoperative deaths in this series. During the past 20 years, the CIRPLAST team has offered free surgery with good outcomes and few complications, to more than 6000 cleft lip and/or palate patients in remote areas of Peru.

What does magnetic resonance imaging add to the prenatal ultrasound diagnosis of facial clefts?

PubMed

Mailáth-Pokorny, M; Worda, C; Krampl-Bettelheim, E; Watzinger, F; Brugger, P C; Prayer, D

2010-10-01

Ultrasound is the modality of choice for prenatal detection of cleft lip and palate. Because its accuracy in detecting facial clefts, especially isolated clefts of the secondary palate, can be limited, magnetic resonance imaging (MRI) is used as an additional method for assessing the fetus. The aim of this study was to investigate the role of fetal MRI in the prenatal diagnosis of facial clefts. Thirty-four pregnant women with a mean gestational age of 26 (range, 19-34) weeks underwent in utero MRI, after ultrasound examination had identified either a facial cleft (n = 29) or another suspected malformation (micrognathia (n = 1), cardiac defect (n = 1), brain anomaly (n = 2) or diaphragmatic hernia (n = 1)). The facial cleft was classified postnatally and the diagnoses were compared with the previous ultrasound findings. There were 11 (32.4%) cases with cleft of the primary palate alone, 20 (58.8%) clefts of the primary and secondary palate and three (8.8%) isolated clefts of the secondary palate. In all cases the primary and secondary palate were visualized successfully with MRI. Ultrasound imaging could not detect five (14.7%) facial clefts and misclassified 15 (44.1%) facial clefts. The MRI classification correlated with the postnatal/postmortem diagnosis. In our hands MRI allows detailed prenatal evaluation of the primary and secondary palate. By demonstrating involvement of the palate, MRI provides better detection and classification of facial clefts than does ultrasound alone. Copyright © 2010 ISUOG. Published by John Wiley & Sons, Ltd.

Ear Infection in Isolated Cleft Lip: Etiological Implications.

PubMed

Ruegg, Teresa A; Cooper, Margaret E; Leslie, Elizabeth J; Ford, Matthew D; Wehby, George L; Deleyiannis, Frederic W B; Czeizel, Andrew E; Hecht, Jacqueline T; Marazita, Mary L; Weinberg, Seth M

2017-03-01

  Chronic ear infections are a common occurrence in children with orofacial clefts involving the secondary palate. Less is known about the middle ear status of individuals with isolated clefts of the lip, although several studies have reported elevated rates of ear infection in this group. The purpose of this retrospective study was to test the hypothesis that chronic ear infections occur more frequently in isolated cleft lip cases (n = 94) compared with controls (n = 183).   A questionnaire was used to obtain information on history of chronic ear infection. The association between ear infection status (present/absent) and cleft lip status (cleft lip case/control) was tested using both chi-square and logistic regression.   The reported occurrence of chronic ear infection was significantly greater in cleft lip cases (31%) compared with unaffected controls (11%). After adjusting for age and sex, having a cleft lip increased the odds of being positive for ear infection by a factor greater than 3 (odds ratio = 3.698; 95% confidence interval = 1.91 to 7.14). Within cleft lip cases, there was no difference in the occurrence of ear infection by defect laterality or by the type of clefting present in the family history. Although velopharyngeal insufficiency was present in 18.4% of our cleft lip sample, there was no statistical association between ear infection and abnormal speech patterns. These results may have potential implications both for the clinical management of isolated cleft lip cases and for understanding the etiology of orofacial clefting.

Ear Infection in Isolated Cleft Lip: Etiological Implications

PubMed Central

Ruegg, Teresa A.; Cooper, Margaret E.; Leslie, Elizabeth J.; Ford, Matthew D.; Wehby, George L.; Deleyiannis, Frederic W.B.; Czeizel, Andrew E.; Hecht, Jacqueline T.; Marazita, Mary L.; Weinberg, Seth M.

2015-01-01

Background and Hypothesis Chronic ear infections are a common occurrence in children with orofacial clefts involving the secondary palate. Less is known about the middle ear status of individuals with isolated clefts of the lip, although several studies have reported elevated rates of ear infection in this group. The purpose of this retrospective study was to test the hypothesis that chronic ear infections occur more frequently in isolated cleft lip cases (n=94) compared with controls (n=183). Methods A questionnaire was used to obtain information on history of chronic ear infection. The association between ear infection status (present/absent) and cleft lip status (cleft lip case/control) was tested using both chi-square and logistic regression. Results and Conclusions The reported occurrence of chronic ear infection was significantly greater in cleft lipcases (31%) compared to unaffected controls (11%). After adjusting for age and sex, having a cleft lip increased the odds of being positive for ear infection by a factor greater than three (OR=3.698; 95%CI=1.91–7.14). Within cleft lipcases, there was no difference in the occurrence of ear infection by defect laterality or by the type of clefting present in the family history. Although velopharyngeal insufficiency was present in 18.4% of our cleft lip sample, there was no statistical association between ear infection and abnormal speech patterns. These results may have potential implications both for the clinical management of isolated cleft lip cases and for understanding the etiology of orofacial clefting. PMID:26153759

Lower lip deformity in patients with cleft and non-cleft Class III malocclusion before and after orthognathic surgery.

PubMed

Park, Joo Seok; Koh, Kyung S; Choi, Jong Woo

2015-10-01

Orthognathic surgery does not yield the same cosmetic benefits in patients with Class III jaw deformities associated with clefts as for patients without clefts. Preoperative upper lip tightness caused by cleft lip repair may not fully explain this difference, suggesting that a lower lip deformity is present. The study compared the outcomes of orthognathic surgery in patients with cleft and non-cleft Class III malocclusion, focusing on lip relationship. The surgical records of 50 patients with Class III malocclusion, including 25 with and 25 without clefts, who had undergone orthognathic surgery, were retrospectively analyzed. Lateral cephalometric tracings, preoperatively and at 6 months postoperatively, were superimposed to analyze the soft tissue changes at seven reference points. At 6 months after surgery, there were no significant differences in skeletal location, whereas the soft tissues of the lower lip differed significantly between patients with and without cleft (p=0.002), indicating the persistence of a lower lip deformity in cleft patients. Moreover, the soft tissues of the lower lip receded in non-cleft patients and protruded in cleft patients after orthognathic surgery. Lower lip deformity and upper lip tightness may result in an unsatisfactory relationship between the upper and lower lips of patients with cleft-related jaw deformity after orthognathic surgery. Other factors were less important than the pathology of the lower lip. Copyright © 2015 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

RISK FACTORS AND CLINICAL SIGNIFICANCE OF PRECHOROIDAL CLEFT IN NEOVASCULAR AGE-RELATED MACULAR DEGENERATION.

PubMed

Kim, Jong Min; Kang, Se Woong; Son, Dae Yong; Bae, Kunho

2017-11-01

To investigate the risk factors associated with prechoroidal cleft occurrence after treatment for neovascular age-related macular degeneration (nAMD) and to elucidate its clinical significance. Two hundred thirty-four subjects who were treated for neovascular age-related macular degeneration were assessed to identify prechoroidal cleft on optical coherence tomography. Clinical variables were compared between patients manifesting a cleft (cleft group) and patients who did not (control group). Prechoroidal cleft was detected in 29 of 234 patients (8.1%). Although the baseline visual acuity was not different between the 2 groups, logMAR visual acuity at final visit was 0.89 ± 0.74 (with approximate Snellen equivalent of 20/160) in the cleft group and 0.65 ± 0.69 (with approximate Snellen equivalent of 20/100) in controls (P < 0.05). Within cleft group, the early-onset (<6 months) subgroup had even worse visual outcomes than the late-onset subgroup (P < 0.05). Multiple logistic regression analyses revealed that the incidence of prechoroidal cleft was positively correlated with having received intravitreal gas injection to displace a submacular hemorrhage and a diagnosis of retinal angiomatous proliferation and typical neovascular age-related macular degeneration (P < 0.05). Diagnosis of retinal angiomatous proliferation and typical neovascular age-related macular degeneration, and a submacular hemorrhage treated by pneumatic displacement were the independent risk factors for development of prechoroidal cleft. Eyes with a cleft, especially clefts that develop early, generally had worse prognoses than eyes without clefts.

Maternal exposure to ozone and PM{sub 2.5} and the prevalence of orofacial clefts in four U.S. states

DOE Office of Scientific and Technical Information (OSTI.GOV)

Zhou, Ying, E-mail: yzhou2@cdc.gov

Background: While there is some evidence that maternal exposure to ambient air pollution is associated with orofacial clefts in offspring, the epidemiologic studies have been largely equivocal. We evaluated whether maternal exposure to elevated county-level ambient fine particulate matter with aerodynamic diameter ≤2.5 µm (PM{sub 2.5}) and ozone during early gestation was associated with a higher prevalence of orofacial clefts. Methods: Birth data consisting of 4.7 million births from 2001 to 2007 were obtained from National Birth Defects Prevention Network for four states — Arizona, Florida, New York (excluding New York City), and Texas. The air pollution exposure assessment formore » gestational weeks 5–10 was based on county-level average concentrations of PM{sub 2.5} and ozone data generated using a Bayesian fusion model available through CDC's Environmental Public Health Tracking Network. Two outcomes were analyzed separately: cleft lip with or without cleft palate, cleft palate alone. In logistic regression analyses, we adjusted for factors that were suspected confounders or modifiers of the association between the prevalence of orofacial clefts and air pollution, i.e., infant sex, race-ethnicity, maternal education, smoking status during pregnancy, whether this was mother's first baby, maternal age. Results: Each 10 µg/m{sup 3} increase in PM{sub 2.5} concentration was significantly associated with cleft palate alone (OR =1.43, 95% CI: 1.11–1.86). There was no significant association between PM{sub 2.5} concentration and cleft lip with or without cleft palate. No associations were observed between ozone exposure and the two outcomes of orofacial clefts. Conclusions: Our study suggests that PM{sub 2.5} significantly increased the risk of cleft palate alone, but did not change the incidence of cleft lip with or without palate. Ozone levels did not correlate with incidence of orofacial clefts. - Highlights: • This is a large study with about 4.7 million births and 7000 orofacial cleft cases. • PM{sub 2.5} significantly increased the risk of cleft palate alone. • PM{sub 2.5} did not change the incidence of cleft lip with or without palate. • Ozone levels did not correlate with incidence of orofacial clefts.« less

Psychometric findings and normative values for the CLEFT-Q based on 2434 children and young adult patients with cleft lip and/or palate from 12 countries.

PubMed

Klassen, Anne F; Riff, Karen Wy Wong; Longmire, Natasha M; Albert, Asteria; Allen, Gregory C; Aydin, Mustafa Asim; Baker, Stephen B; Cano, Stefan J; Chan, Andrew J; Courtemanche, Douglas J; Dreise, Marieke M; Goldstein, Jesse A; Goodacre, Timothy E E; Harman, Karen E; Munill, Montserrat; Mahony, Aisling O; Aguilera, Mirta Palomares; Peterson, Petra; Pusic, Andrea L; Slator, Rona; Stiernman, Mia; Tsangaris, Elena; Tholpady, Sunil S; Vargas, Federico; Forrest, Christopher R

2018-04-16

Patients with cleft lip and/or palate can undergo numerous procedures to improve appearance, speech, dentition and hearing. We developed a cleft-specific patient-reported outcome instrument to facilitate rigorous international measurement and benchmarking. Data were collected from patients aged 8-29 years with cleft lip and/or palate at 30 hospitals in 12 countries between October 2014 and November 2016. Rasch measurement theory analysis was used to refine the scales and to examine reliability and validity. Normative CLEFT-Q values were computed for age, sex and cleft type. Analysis led to the refinement of an eating and drinking checklist and 12 scales measuring appearance (of the face, nose, nostrils, teeth, lips, jaws and cleft lip scar), health-related quality of life (psychological, social, school, speech distress) and speech function. All scales met the requirements of the Rasch model. Analysis to explore differential item functioning by age, sex and country provided evidence to support the use of a common scoring algorithm for each scale for international use. Lower (worse) scores on CLEFT-Q scales were associated with having a speech problem, being unhappy with facial appearance, and needing future cleft-related treatments, providing evidence of construct validity. Normative values for age, sex and cleft type showed poorer outcomes associated with older age, female sex and having a visible cleft. The CLEFT-Q represents a rigorously developed instrument that can be used internationally to collect and compare evidence-based outcomes data from patients aged 8-29 years of age with cleft lip and/or palate. © 2018 Joule Inc. or its licensors.

Psychometric findings and normative values for the CLEFT-Q based on 2434 children and young adult patients with cleft lip and/or palate from 12 countries

PubMed Central

Riff, Karen WY Wong; Longmire, Natasha M.; Albert, Asteria; Allen, Gregory C.; Aydin, Mustafa Asim; Baker, Stephen B.; Cano, Stefan J.; Chan, Andrew J.; Courtemanche, Douglas J.; Dreise, Marieke M.; Goldstein, Jesse A.; Goodacre, Timothy E.E.; Harman, Karen E.; Munill, Montserrat; Mahony, Aisling O.; Aguilera, Mirta Palomares; Peterson, Petra; Pusic, Andrea L.; Slator, Rona; Stiernman, Mia; Tsangaris, Elena; Tholpady, Sunil S.; Vargas, Federico; Forrest, Christopher R.

2018-01-01

BACKGROUND: Patients with cleft lip and/or palate can undergo numerous procedures to improve appearance, speech, dentition and hearing. We developed a cleft-specific patient-reported outcome instrument to facilitate rigorous international measurement and benchmarking. METHODS: Data were collected from patients aged 8–29 years with cleft lip and/or palate at 30 hospitals in 12 countries between October 2014 and November 2016. Rasch measurement theory analysis was used to refine the scales and to examine reliability and validity. Normative CLEFT-Q values were computed for age, sex and cleft type. RESULTS: Analysis led to the refinement of an eating and drinking checklist and 12 scales measuring appearance (of the face, nose, nostrils, teeth, lips, jaws and cleft lip scar), health-related quality of life (psychological, social, school, speech distress) and speech function. All scales met the requirements of the Rasch model. Analysis to explore differential item functioning by age, sex and country provided evidence to support the use of a common scoring algorithm for each scale for international use. Lower (worse) scores on CLEFT-Q scales were associated with having a speech problem, being unhappy with facial appearance, and needing future cleft-related treatments, providing evidence of construct validity. Normative values for age, sex and cleft type showed poorer outcomes associated with older age, female sex and having a visible cleft. INTERPRETATION: The CLEFT-Q represents a rigorously developed instrument that can be used internationally to collect and compare evidence-based outcomes data from patients aged 8–29 years of age with cleft lip and/or palate. PMID:29661814

Is gravidity 4+ a risk factor for oral clefts? A case-control study in eight South american countries using structural equation modeling.

PubMed

Gili, Juan Antonio; Poletta, Fernando Adrián; Campaña, Hebe; Comas, Belén; Pawluk, Mariela; Rittler, Monica; López-Camelo, Jorge Santiago

2013-09-01

Background : There is disagreement about the association between cleft lip with or without cleft palate and multigravidity, which could be explained by differences of adjusting for maternal age, Amerindian ancestry, and socioeconomic status. Objective : The aim was to evaluate gravidity 4+ (four or more gestations) as a risk factor for cleft lip with or without cleft palate in South America. Design : We used a matched (1:1) case-control study with structural equation modeling for related causes. Data were obtained from 1,371,575 consecutive newborn infants weighing ≥500 g who were born in the hospitals of the Estudio Colaborativo Latinoamericano de Malformaciones Congénitas (ECLAMC) network between 1982 and 1999. There were a total of 1,271 cases with cleft lip with or without cleft palate (excluding midline and atypical cleft lip with or without cleft palate). A total of 1,227 case-control pairs were obtained, matched by maternal age, newborn gender, and year and place of birth. Potential confounders and intermediary variables were analyzed with structural equation modeling. Results : The crude risk of gravidity 4+ was 1.41 and the 95% confidence interval was 1.14 to 1.61. When applying structural equation modeling, the effect of multigravidity on the risk of cleft lip with or without cleft palate was 1.22 and the 95% confidence interval was 0.91 to 1.39. Conclusions : Multigravid mothers (more than four gestations) showed no greater risk of bearing children who had cleft lip with or without cleft palate than mothers with two or three births. Therefore, the often observed and reported association between multigravidity and oral clefts likely reflects the effect of other risk factors related to low socioeconomic status in South American populations.

Stem cell therapy for reconstruction of alveolar cleft and trauma defects in adults: A randomized controlled, clinical trial.

PubMed

Bajestan, Mona N; Rajan, Archana; Edwards, Sean P; Aronovich, Sharon; Cevidanes, Lucia H S; Polymeri, Angeliki; Travan, Suncica; Kaigler, Darnell

2017-10-01

Stem cell therapy with bone marrow-derived mesenchymal stem cells is a promising tissue engineering strategy to promote regeneration of craniofacial bone. To determine whether cell therapy with ex vivo expanded stem cell populations would be safe and efficacious in the regeneration of large alveolar defects in patients with a history of cleft palate or craniofacial trauma. Eighteen patients (10 patients with traumatic injury and 8 patients with cleft palate) presenting with missing teeth associated with horizontal alveolar bone deficiencies were included in this randomized controlled clinical trial. Patients were randomized to receive either conventional autogenous block grafts or stem cell therapy. After a healing period of 4 months the treated sites were re-entered and the bone width re-assessed prior to implant placement. Implant stability was evaluated through torque testing of the implant upon insertion and at 6 months postloading. The mean gain in bone width was 1.5 ± 1.5 mm in the stem cell therapy group and 3.3 ± 1.4 mm in the control group. Overall, bone gain was higher in trauma patients as compared to patients with cleft palate, for both the control and the stem cell therapy groups. Most postoperative complications were wound dehiscences and incision line openings. Implants were placed successfully in 5 out of 10 patients in the stem cell therapy group and in all 8 patients in the control group. One implant from the control/cleft palate group failed before loading, while the rest of the implants were loaded successfully and remained stable at 6 months. The patients who did not receive implants were re-treated with autogenous block bone graft. The ability of stem cells to treat large alveolar defects is safe, yet, their ability to completely reconstitute large alveolar defects is limited. This approach requires further optimization to meet the outcomes seen using current methods to treat large defects, particularly those resultant of cleft palate. © 2017 Wiley Periodicals, Inc.

Maternal Consumption of Coffee and Caffeine-containing Beverages and Oral Clefts: A Population-based Case-Control Study in Norway

PubMed Central

Wilcox, Allen J.; Lie, Rolv T.; Andersen, Lene F.; Drevon, Christian A.

2009-01-01

A large, population-based case-control study of facial clefts was carried out in Norway between 1996 and 2001. The study included 573 cases—377 with cleft lip with or without cleft palate and 196 with cleft palate only—and 763 randomly selected controls. Maternal consumption of coffee and other caffeine-containing beverages in early pregnancy was recorded shortly after birth. Compared with that for no coffee consumption, the adjusted odds ratios for cleft lip with or without cleft palate were 1.39 (95% confidence interval: 1.01, 1.92) for less than 3 cups a day and 1.59 (95% confidence interval: 1.05, 2.39) for 3 cups or more. Coffee consumption was not associated with risk of cleft palate only (for ≥3 cups vs. none, adjusted odds ratio = 0.96, 95% confidence interval: 0.55, 1.67). Tea consumption was associated with a reduced odds ratio of both cleft lip with or without cleft palate and cleft palate only. There was little evidence of an association between caffeine exposure and clefts when all sources of caffeine were considered. Adjustment for known confounding factors in general had minor effects on risk estimates. Still, the authors could not rule out the possibility of uncontrolled confounding by factors associated with the habit of drinking coffee. PMID:19342400

The use and reliability of SymNose for quantitative measurement of the nose and lip in unilateral cleft lip and palate patients.

PubMed

Mosmuller, David; Tan, Robin; Mulder, Frans; Bachour, Yara; de Vet, Henrica; Don Griot, Peter

2016-10-01

It is essential to have a reliable assessment method in order to compare the results of cleft lip and palate surgery. In this study the computer-based program SymNose, a method for quantitative assessment of the nose and lip, will be assessed on usability and reliability. The symmetry of the nose and lip was measured twice in 50 six-year-old complete and incomplete unilateral cleft lip and palate patients by four observers. For the frontal view the asymmetry level of the nose and upper lip were evaluated and for the basal view the asymmetry level of the nose and nostrils were evaluated. A mean inter-observer reliability when tracing each image once or twice was 0.70 and 0.75, respectively. Tracing the photographs with 2 observers and 4 observers gave a mean inter-observer score of 0.86 and 0.92, respectively. The mean intra-observer reliability varied between 0.80 and 0.84. SymNose is a practical and reliable tool for the retrospective assessment of large caseloads of 2D photographs of cleft patients for research purposes. Moderate to high single inter-observer reliability was found. For future research with SymNose reliable outcomes can be achieved by using the average outcomes of single tracings of two observers. Copyright © 2016 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Transverse dental arch relationship at 9 and 12 years in children with unilateral cleft lip and palate treated with infant orthopedics: a randomized clinical trial (DUTCHCLEFT).

PubMed

Noverraz, R L M; Disse, M A; Ongkosuwito, E M; Kuijpers-Jagtman, A M; Prahl, C

2015-12-01

A long-term evaluation to assess the transverse dental arch relationships at 9 and 12 years of age in unilateral cleft lip and palate treated with or without infant orthopedics (IO). The hypothesis is that IO has no effect on the transverse dental arch relationship. A prospective two-arm randomized controlled trial (DUTCHCLEFT) in three academic cleft palate centers (Amsterdam, Nijmegen and Rotterdam, the Netherlands). Fifty-four children with complete unilateral cleft lip and palate and no other malformations were enrolled in this evaluation. One group wore passive maxillary plates (IO+) during the first year of life, and the other group did not (IO-). Until the age of 1.5, all other interventions were the same. Hard palate was closed simultaneously with bone grafting according to protocol of all teams. Orthodontic treatment was performed when indicated. The transverse dental arch relationship was assessed on dental casts using the modified Huddart/Bodenham score to measure the maxillary arch constriction at 9 and 12 years of age. No significant differences were found between the IO+ and IO- groups. Differences between the centers increased from 9 to 12 years of age. Transverse dental arch relationships at 9 and 12 years of age do not differ between children with UCLP treated with or without IO. There is no orthodontic need to perform IO as applied in this study in children with UCLP.

Orthodontic and orthopedic treatment for a growing patient with Tessier number 0 cleft

PubMed Central

Park, Yoon-Hee; Chung, Jee Hyeok; Kim, Sukwha; Choi, Jin-Young

2018-01-01

The purpose of this case report was to introduce the concept of orthodontic and orthopedic treatment for a growing patient with Tessier number 0 cleft. A 5-year-old boy patient with Tessier number 0 cleft presented congenitally missing maxillary central incisors (MXCI), a bony defect at the premaxilla, a constricted maxillary arch, an anterior openbite, and maxillary hypoplasia. His treatment was divided into three stages: management of the bony defect at the premaxilla and the congenitally missing MXCIs using a fan-type expansion plate, iliac bone grafting, and eruption guidance of the maxillary lateral incisors into the graft area for substitution of MXCIs; management of the maxillary hypoplasia using sequential facemask therapy with conventional and skeletal anchorage; and management of the remaining occlusal problems using fixed orthodontic treatment. The total treatment duration was 15 years and 10 months. Class I canine and Class II molar relationships and normal overbite and overjet were achieved at the end of treatment. Although the long-term use of facemask therapy resulted in significant protraction of the retrusive maxilla, the patient exhibited Class III profile because of continued mandibular growth. However, the treatment result was well maintained after 2 years of retention. The findings from this case suggest that interdisciplinary and customized approaches are mandatory for successful management of maxillary hypoplasia, bony defect, and dental problems in Tessier number 0 cleft. Moreover, considering the potential of orthognathic surgery or distraction osteogenesis, meticulous monitoring of mandibular growth until growth completion is important. PMID:29564221

Centre-level variation in behaviour and the predictors of behaviour in 5-year-old children with non-syndromic unilateral cleft lip: The Cleft Care UK study. Part 5.

PubMed

Waylen, A; Mahmoud, O; Wills, A K; Sell, D; Sandy, J R; Ness, A R

2017-06-01

The aims of this study were to describe child behavioural and psychosocial outcomes associated with appearance and speech in the Cleft Care UK (CCUK) study. We also wanted to explore centre-level variation in child outcomes and investigate individual predictors of such outcomes. Two hundred and sixty-eight five-year-old children with non-syndromic unilateral cleft lip and palate (UCLP) recruited to CCUK. Parents completed the Strengths and Difficulties questionnaire (SDQ) and reported their own perceptions of the child's self-confidence. Child facial appearance and symmetry were assessed using photographs, and intelligibility of speech was derived from audio-visual speech recordings. Centre-level variation in behavioural and psychosocial outcomes was examined using hierarchical models, and associations with clinical outcomes were examined using logit regression models. Children with UCLP had a higher hyperactive difficulty score than the general population. For boys, the average score was 4.5 vs 4.1 (P=.03), and for girls, the average score was 3.8 vs 3.1 (P=.008). There was no evidence of centre-level variation for behaviour or parental perceptions of the child's self-confidence. There is no evidence of associations between self-confidence and SDQ scores and either facial appearance or behaviour. Children born with UCLP have higher levels of behaviour problems than the general population. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

CLEFT PALATE. FOUNDATIONS OF SPEECH PATHOLOGY SERIES.

ERIC Educational Resources Information Center

RUTHERFORD, DAVID; WESTLAKE, HAROLD

DESIGNED TO PROVIDE AN ESSENTIAL CORE OF INFORMATION, THIS BOOK TREATS NORMAL AND ABNORMAL DEVELOPMENT, STRUCTURE, AND FUNCTION OF THE LIPS AND PALATE AND THEIR RELATIONSHIPS TO CLEFT LIP AND CLEFT PALATE SPEECH. PROBLEMS OF PERSONAL AND SOCIAL ADJUSTMENT, HEARING, AND SPEECH IN CLEFT LIP OR CLEFT PALATE INDIVIDUALS ARE DISCUSSED. NASAL RESONANCE…

Open conformation of a substrate-binding cleft: sup 19 F NMR studies of cleft angle in the D-galactose chemosensory receptor

DOE Office of Scientific and Technical Information (OSTI.GOV)

Luck, L.A.; Falke, J.J.

1991-07-02

The Escherichia coli D-galactose and D-glucose receptor is a two-domain structure with a sugar-binding site at the interface between domains. The structure of the closed cleft containing bound D-glucose has been determined crystalloghraphically, but the open cleft remains to be characterized. The present study illustrates a generalizable approach that is used to detect and analyze both the open- and closed-cleft conformations in solution. A {sup 19}F nucleus located inside the cleft is monitored by {sup 19}F NMR. When the cleft is occupied by D-glucose, the {sup 19}F nucleus is found to be inaccessible to the aqueous paramagnetic probe Gd{center dot}EDTA,more » verifying that the occupied cleft is closed in solution and inaccessible to bulk solvent. When the cleft is empty, the {sup 19}F nucleus becomes accessible to the paramagnet such that the distance of closest approach is r {le} 10 {angstrom}, indicating that the empty cleft opens at least transiently by an angle {theta} {ge} 18 {plus minus} 3{degrees}.« less

Clinical directors' views of centralisation and commissioning of cleft services in the U.K.

PubMed

Searle, Aidan; Scott, Julia K; Sandy, Jonathan; Ness, Andrew; Waylen, Andrea

2015-01-22

To determine the views of Clinical Directors working in the United Kingdom (U.K.) Cleft Service with regard to centralisation, commissioning and impact on cleft service provision. In-depth qualitative interviews were conducted with 11 Clinical Directors representing regional cleft services. Interviews were transcribed verbatim, a coding frame was developed by two researchers and transcripts were coded using a thematic, 'interpretive' approach. Clinical Directors perceived the commissioning of cleft services in the U.K. to be dependent upon historical agreements and individual negotiation despite service centralisation. Furthermore, Clinical Directors perceived unfairness in the commissioning and funding of cleft services and reported inconsistencies in funding models and service costs that have implications for delivering an equitable cleft service with an effective Multidisciplinary Team. National Health Service (NHS) commissioning bodies can learn lessons from the centralisation of cleft care. Clinical Directors' accounts of their relationships with specialist commissioning bodies and their perspectives of funding cleft services may serve to increase parity and improve the commissioning of cleft services in the U.K.

Tobacco smoking and oral clefts: a meta-analysis.

PubMed Central

Little, Julian; Cardy, Amanda; Munger, Ronald G.

2004-01-01

OBJECTIVE: To examine the association between maternal smoking and non-syndromic orofacial clefts in infants. METHODS: A meta-analysis of the association between maternal smoking during pregnancy was carried out using data from 24 case-control and cohort studies. FINDINGS: Consistent, moderate and statistically significant associations were found between maternal smoking and cleft lip, with or without cleft palate (relative risk 1.34, 95% confidence interval 1.25-1.44) and between maternal smoking and cleft palate (relative risk 1.22, 95% confidence interval 1.10-1.35). There was evidence of a modest dose-response effect for cleft lip with or without cleft palate. CONCLUSION: The evidence of an association between maternal tobacco smoking and orofacial clefts is strong enough to justify its use in anti-smoking campaigns. PMID:15112010

A chronicle of Tessier no. 0 and 1 facial cleft and its surgical management.

PubMed

Jhamb, Aakarsh; Mohanty, Sujata

2009-06-01

Description and successful management of a patient with Tessier no. 0 and 1 facial cleft is being presented. This bizarre coexistence of two of the rare facial clefts in a single patient deserves a place in the cleft database. Appropriate evaluation was done to rule out the presence of median cleft face syndrome. Lip cleft was repaired by straight line technique with staggering at the vermilion border. Nasal cleft was reconstructed by replacing the missing lower lateral cartilage with conchal cartilage via endonasal approach. Satisfactory outcome was achieved for this singular deformity by conforming to the basic tenets of plastic surgery.

Bilateral neuroretinitis associated with mumps.

PubMed

Khubchandani, Raju; Rane, Tej; Agarwal, Premlata; Nabi, Fazal; Patel, Phiroze; Shetty, Avinash K

2002-10-01

Involvement of the optic nerve is a rare complication of mumps infection. To report a case of bilateral neuroretinitis complicating a mumps infection and to review 5 previously reported cases. Case report and literature review. Tertiary hospital. A 7-year-old girl had sudden-onset blindness due to bilateral neuroretinitis. Approximately 3 weeks prior to the initial examination, she developed a self-limited febrile illness with parotid swelling and subsequent meningoencephalitis. Mumps was determined to be the underlying cause of the meningoencephalitis and bilateral optic neuritis because of the exposure history in this nonvaccinated child, the typical clinical signs and symptoms, and the positive serologic test results. Recovery of visual function was gradual but nearly complete. Physicians should be aware that optic nerve involvement may be a manifestation of mumps infection. The delayed onset of optic neuritis, the bilateral involvement, and the near complete recovery suggest an immune-mediated pathogenesis.

The financial impact of multidisciplinary cleft care: an analysis of hospital revenue to advance program development.

PubMed

Deleyiannis, Frederic W-B; TeBockhorst, Seth; Castro, Darren A

2013-03-01

The purpose of this study was to determine the financial impact of cleft care on the hospital and to evaluate trends in reimbursement over the past 6 years. Medical and accounting records of 327 consecutive infants undergoing cleft repair between 2005 and 2011 were reviewed. Charges, payments, and direct cost data were analyzed to illustrate hospital revenue and margins. Hospital payments for all inpatient services (cleft and noncleft) during the first 24 months of life were $9,483,168. Mean hospital payment varied from $5525 (Medicaid) to $10,274 (managed care) for a cleft lip repair (p < 0.0001) and from $6573 (Medicaid) to $12,933 (managed care) for a cleft palate repair (p < 0.0001). Hospital charges for a definitive lip or palate repair to both Medicaid and managed care more than doubled between 2005 and 2011 (p < 0.0001). Overall, mean hospital margins were $3904 and $3520, respectively, for a cleft lip repair and cleft palate repair. Medicaid physician payments for cleft lip and palate were, respectively, $588 and $646. From 2005 to 2006, 2007 to 2008, and 2009 to 2010, 41 percent, 43 percent, and 63 percent of patients, respectively, were enrolled in Medicaid. Cleft care generates substantial revenue for the hospital. For their mutual benefit, hospitals should join with their cleft teams to provide administrative support. Bolstered reimbursement figures, based on the overall value of cleft care to the hospital system, would better attract and retain skilled clinicians dedicated to cleft care. This may become particularly important if Medicaid enrollment continues to increase.

Dermatoglyphics and Cheiloscopy as Key Tools in Resolving the Genetic Correlation of Inheritance Patterns in Cleft Lip and Palate Patients: An Assessment of 160 Patients.

PubMed

Singh, Priyankar; Nathani, Dipesh B

2017-09-01

  The objective of this study was to correlate dermatoglyphics and cheiloscopy with genetic inheritance in cleft lip and cleft palate patients.   This was a case-control study to look for asymmetry in finger and lip print patterns. All of the participants were divided into two equal groups (40 mothers and 40 fathers in each group). The data were analyzed by three evaluators who were blind to the study to avoid any chances of error.   A sample of 160 sporadic participants were identified and evaluated. Group A was composed of 80 healthy parents not affected by cleft lip and cleft palate but had at least one child born with nonsyndromic cleft. Group B consisted of 80 healthy parents not affected by cleft lip and cleft palate and had healthy children without cleft lip and cleft palate.   Main outcome measures were marked dermatoglyphic asymmetry and specific lip print pattern in the study group.   We found marked asymmetry in various fingerprints and specific type II and type III lip print in the study group when compared with the control group. It was observed that groove count on the lip was significantly more frequent in study group parents.   Our study determined that there is a significant correlation between increased dermatoglyphic asymmetry and specific type II and type III lip print pattern in parents of children born with cleft. This could act as an important screening marker for the prediction of cleft lip and cleft palate inheritance.

Cleft Palate

PubMed Central

Kosowski, Tomasz R.; Weathers, William M.; Wolfswinkel, Erik M.; Ridgway, Emily B.

2012-01-01

Our understanding of cleft palates has come a long way over the last few decades. A better understanding of the long-term consequences of a cleft palate and its effect on speech development challenges surgeons to not only effectively repair the cleft, but to also restore function of the palate for adequate speech. Coordination with speech pathologists is integral for effective management of cleft palate patients, particularly as children begin to develop language. In this article, the authors review and summarize the various challenges and goals of cleft palate management. PMID:24179449

Cranio-facial clefts in pre-hispanic America.

PubMed

Marius-Nunez, A L; Wasiak, D T

2015-10-01

Among the representations of congenital malformations in Moche ceramic art, cranio-facial clefts have been portrayed in pottery found in Moche burials. These pottery vessels were used as domestic items during lifetime and funerary offerings upon death. The aim of this study was to examine archeological evidence for representations of cranio-facial cleft malformations in Moche vessels. Pottery depicting malformations of the midface in Moche collections in Lima-Peru were studied. The malformations portrayed on pottery were analyzed using the Tessier classification. Photographs were authorized by the Larco Museo.Three vessels were observed to have median cranio-facial dysraphia in association with midline cleft of the lower lip with cleft of the mandible. ML001489 portrays a median cranio-facial dysraphia with an orbital cleft and a midline cleft of the lower lip extending to the mandible. ML001514 represents a median facial dysraphia in association with an orbital facial cleft and a vertical orbital dystopia. ML001491 illustrates a median facial cleft with a soft tissue cleft. Three cases of midline, orbital and lateral facial clefts have been portrayed in Moche full-figure portrait vessels. They represent the earliest registries of congenital cranio-facial malformations in ancient Peru. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

The Fetal Cleft palate: V. Elucidation of the Mechanism of Palatal Clefting in the Congenital Caprine Model

USDA-ARS?s Scientific Manuscript database

Maternal ingestion of Nicotiana glauca from gestation days 32 through 41 results in a high incidence of cleft palate in Spanish goats. This caprine cleft palate model was used to evaluate the temporal sequence of palatal shelf fusion throughout the period of cleft induction with the poisonous plant...

Maxillary Nine-year Molars: Prevalence and Associated Dental and Medical Conditions.

PubMed

Klein, Ulrich; Alford, Jacqueline A; Allshouse, Amanda A; Sain, Joel

2016-01-01

The purpose of this study was to determine, from a large number of panoramic images taken in the clinic of a pediatric dentistry residency program, the prevalence of maxillary nine-year molars (9YM), associated medical conditions (MCs), and the presence of coexisting tooth anomalies (TAs). Pantomograms from 13,140 patients were searched for 9YM. Radiographs from identified subjects were examined for coexisting TAs, and charted MCs were noted. For identified 9YM, Nolla's stage of tooth formation was determined and the subjects' dental ages were calculated. Among 53 subjects (68 percent females), 21 unilateral and 32 bilateral cases of 9YM occurred. Females and males were of similar age (7.98 versus 7.91 years old). Overall dental and chronological ages were significantly different: dental development in bilateral cases was delayed by 4.1 months in females and 2.9 months in both sexes (P=.003). In unilateral cases, it was accelerated by 3.6 months in females and 3.5 months in males. The specific tooth age for all 9YM was 4.95 years for females and 5.56 years for males (P=.018). The average count of associated findings (e.g., missing teeth, peg laterals, other TAs, and other eruption delays) was 0.62 in unilateral and 1.44 in bilateral cases (P=.02). Commonly associated MCs included clefting (N equals five), and Down syndrome (n equals three). The prevalence of nine-year molars was one in 248 (0.4 percent), and the average delay in dental development was 2.7 years.

Radiology of Cleft Lip and Palate: Imaging for the Prenatal Period and throughout Life.

PubMed

Abramson, Zachary R; Peacock, Zachary S; Cohen, Harris L; Choudhri, Asim F

2015-01-01

Recent advances in prenatal imaging have made possible the in utero diagnosis of cleft lip and palate and associated deformities. Postnatal diagnosis of cleft lip is made clinically, but imaging still plays a role in detection of associated abnormalities, surgical treatment planning, and screening for or surveillance of secondary deformities. This article describes the clinical entities of cleft lip with or without cleft palate (CLP) and isolated cleft palate and documents their prenatal and postnatal appearances at radiography, ultrasonography (US), magnetic resonance (MR) imaging, and computed tomography (CT). Imaging protocols and findings for prenatal screening, detection of associated anomalies, and evaluation of secondary deformities throughout life are described and illustrated. CLP and isolated cleft palate are distinct entities with shared radiologic appearances. Prenatal US and MR imaging can depict clefting of the lip or palate and associated anomalies. While two- and three-dimensional US often can depict cleft lip, visualization of cleft palate is more difficult, and repeat US or fetal MR imaging should be performed if cleft palate is suspected. Postnatal imaging can assist in identifying associated abnormalities and dentofacial deformities. Dentofacial sequelae of cleft lip and palate include missing and supernumerary teeth, oronasal fistulas, velopharyngeal insufficiency, hearing loss, maxillary growth restriction, and airway abnormalities. Secondary deformities can often be found incidentally at imaging performed for other purposes, but detection is necessary because they may have considerable implications for the patient. (©)RSNA, 2015.

Oral clefting in china over the last decade: 205,679 patients.

PubMed

Kling, Rochelle R; Taub, Peter J; Ye, Xiaoqian; Jabs, Ethylin Wang

2014-10-01

China is the most populated country and has one of the highest prevalences of oral clefting. The present study reports the epidemiology and surgical procedures performed on the largest reported cohort of individuals with clefting in China. A retrospective review of patients who received cleft repair through Smile Train in China from 2000 to 2011 was conducted. Data on demographics, cleft characteristics, associated malformations, pregnancy and family history, and surgical technique were analyzed using SPSS (IBM, Chicago, Ill.). A total of 205,679 patients underwent 209,169 cleft procedures. Cleft lip and palate (42.7%) was most common followed by isolated cleft palate (32.4%) and isolated cleft lip (24.9%). Males accounted for 63.5% of cases. The average age at initial surgery was 6.12 years. By 2011, this decreased to 1.8 years of age for lip repair and to 5.9 years of age for palate repair. The preferred techniques were rotation-advancement (55%) for unilateral lip repair and Von-Langenbeck (38%) and pushback (39%) for palate repair. The percentages of cases with associated anomalies and surgical complications were 12.8% and 0.36%, respectively. This study provides insight into cleft care in China as it reports the largest cohort of cleft patients treated by surgeons to date. Our results generally follow trends previously reported in China and developed countries. The male:female ratio for cleft palate patients was higher than expected. The average age at primary repair is higher than recommended, but seems to be decreasing.

Prevalence and evaluation of environmental risk factors associated with cleft lip and palate in a central Indian population.

PubMed

Kalaskar, Ritesh; Kalaskar, Ashita; Naqvi, Fatama Sana; Tawani, Gopal S; Walke, Damayanti R

2013-01-01

Cleft lip and palate, the most common developmental deformity seen worldwide, may be either genetic or environmental in origin. Recent research clearly shows the inter-relationship between environmental risk factors and development of oral clefts. The purpose of this study was to determine the prevalence of cleft lip and palate in the Nagpur region of India and to evaluate environmental risk factors associated with the occurrence of this orofacial abnormality. The parents of infants born with or without cleft lip and palate were subjected to questionnaires that elicited sociodemographic profiles and histories of maternal dietary, medical, and environmental risk factors as well family histories of cleft. A multifactorial comparison of environmental risk factors associated with this deformity was performed. The prevalence of cleft lip and palate and cleft palate was found to be 0.66% and 0.27%, respectively, in the Nagpur region. The results demonstrated a positive association between cleft lip and palate and the environmental risk factors of nutritional deficiency, anemia, and self-administered medications. Several environmental risk factors appear to play an important role in the development of cleft lip and palate in a Central Indian population of low socioeconomic status.

The comparative study of resonance disorders for Vietnamese and Korean cleft palate speakers using nasometer.

PubMed

Shin, Yu-Jeong; Kim, Yongsoo; Kim, Hyun-Gi

2017-12-01

Nasalance is used to evaluate the velopharyngeal incompetence in clinical diagnoses using a nasometer. The aim of this study is to find the nasalance differences between Vietnamese cleft palate children and Korean cleft palate children by measuring the nasalance of five oral vowels. Ten Vietnamese cleft palate children after surgery, three Vietnamese children for the control group, and ten Korean cleft palate children after surgery with the same age participated in this experimentation. Instead of Korean control, the standard value of Korean version of the simplified nasometric assessment procedures (kSNAP) was used. The results are as follows: (1) the highest nasalance score among the Vietnamese normal vowels is the low vowel /a/; however, that of Korean normal vowels is the high vowel /i/. (2) The average nasalance score of Korean cleft palate vowels is 18% higher than that of Vietnamese cleft palate vowels. There was a nasalance score of over 45% among the vowels /e/ and /i/ in Vietnamese cleft palate patients and /i/, /o/, and /u/ in Korean cleft palate patients. These different nasalance scores of the same vowels seem to cause an ethnic difference between Vietnamese and Korean cleft palate children.

Short mandible - a possible risk factor for cleft palate with/without a cleft lip.

PubMed

Hermann, N V; Darvann, T A; Ersbøll, B K; Kreiborg, S

2014-05-01

To estimate the influence of a short mandible on the risk of developing a cleft palate with/without a cleft lip (CP). The retrospective sample consisted of 115 2-month-old Danish infants with CP, and 70 control infants with unilateral incomplete cleft lip (UICL). Cephalometric X-rays were obtained. Mandibular length (Lm ) was measured and corrected for body length (Lb ) to remove influence of varying body length in the sample. Logistic regression was applied to the corrected mandibular length (Lmc ) to calculate the risk of having a cleft palate. The mean mandibular length in the group with CP was about 4 mm shorter than in the control group. Odds ratio (OR) was calculated to be 0.58 (95% confidence interval 0.48-0.68), implying that an individual's risk of cleft palate with/without a cleft lip increases about 50% per mm decrease in mandibular length. A special facial type including a short mandible is a possible risk factor for cleft palate, and it was found that the risk of cleft palate increases 58% per mm decreases in mandibular length. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Initial Nutritional Assessment of Infants With Cleft Lip and/or Palate: Interventions and Return to Birth Weight.

PubMed

Kaye, Alison; Thaete, Kristi; Snell, Audrey; Chesser, Connie; Goldak, Claudia; Huff, Helen

2017-03-01

  To assess and quantify cleft team practices with regard to nutritional support in the neonatal period Design :  Retrospective review.   Tertiary pediatric hospital.   One hundred consecutive newborn patients with a diagnosis of cleft lip and/or cleft palate between 2009 and 2012.   Birth weight, cleft type, initial cleft team weight measurements, initial feeding practices, recommended nutritional interventions, and follow-up nutritional assessments.   All patients in the study were evaluated by a registered dietitian and an occupational feeding therapist. Average birth weight and average age at the first cleft team visit were similar for each cleft type: cleft lip (CL), cleft lip and palate (CLP), and cleft palate (CP). The calculated age (in days) for return to birth weight was significantly different between cleft types: CL = 13.58 days, CLP = 15.88 days, and CP = 21.93 days. Exclusive use of breast milk was 50% for patients with CL, 30.3% for patients with CLP, and 21.4% for patients with CP. Detailed nutritional interventions were made for 31 patients at the first visit: two with CL, 14 with CLP, and 15 with CP.   Distinct differences were seen in neonatal weight gain between cleft types. There was significantly greater total weight gain for patients with CL at their first visit and significantly slower return to birth weight for patients with isolated CP. Patients with CL required far fewer interventions at the initial assessment and were more likely to be provided breast milk exclusively or in combination with formula. Infants with CP were far less likely to receive any breast milk. Patients with CLP and CP required frequent nutritional interventions.

Incidence of cleft Lip and palate in the state of Andhra Pradesh, South India

PubMed Central

Reddy, Srinivas Gosla; Reddy, Rajgopal R.; Bronkhorst, Ewald M.; Prasad, Rajendra; Ettema, Anke M.; Sailer, Hermann F.; Bergé, Stefaan J.

2010-01-01

Objective: To assess the incidence of cleft lip and palate defects in the state of Andhra Pradesh, India. Design Setting: The study was conducted in 2001 in the state of Andhra Pradesh, India. The state has a population of 76 million. Three districts, Cuddapah, Medak and Krishna, were identified for this study owing to their diversity. They were urban, semi-urban and rural, respectively. Literacy rates and consanguinity of the parents was elicited and was compared to national averages to find correlations to cleft births. Type and side of cleft were recorded to compare with other studies around the world and other parts of India. Results: The birth rate of clefts was found to be 1.09 for every 1000 live births. This study found that 65% of the children born with clefts were males. The distribution of the type of cleft showed 33% had CL, 64% had CLP, 2% had CP and 1% had rare craniofacial clefts. Unilateral cleft lips were found in 79% of the patients. Of the unilateral cleft lips 64% were left sided. There was a significant correlation of children with clefts being born to parents who shared a consanguineous relationship and those who were illiterate with the odds ratio between 5.25 and 7.21 for consanguinity and between 1.55 and 5.85 for illiteracy, respectively. Conclusion: The birth rate of clefts was found to be comparable with other Asian studies, but lower than found in other studies in Caucasian populations and higher than in African populations. The incidence was found to be similar to other studies done in other parts of India. The distribution over the various types of cleft was comparable to that found in other studies. PMID:21217978

Early Mother-Child Interaction and Later Quality of Attachment in Infants With an Orofacial Cleft Compared to Infants Without Cleft.

PubMed

Habersaat, Stephanie; Monnier, Maryline; Peter, Camille; Bolomey, Luce; Borghini, Ayala; Despars, Josée; Pierrehumbert, Blaise; Müller-Nix, Carole; Ansermet, François; Hohlfeld, Judith

2013-04-23

Objective :  The main objective of this study was to assess mother-child patterns of interaction in relation to later quality of attachment in a group of children with an orofacial cleft compared with children without cleft. Design :  Families were contacted when the child was 2 months old for a direct assessment of mother-child interaction and then at 12 months for a direct assessment of the child's attachment. Data concerning socioeconomical information and posttraumatic stress symptoms in mothers were collected at the first appointment. Participants :  Forty families of children with a cleft and 45 families of children without cleft were included in the study. Families were recruited at birth in the University Hospital of Lausanne. Results :  Results showed that children with a cleft were more difficult and less cooperative during interaction at 2 months of age with their mother compared with children without a cleft. No significant differences were found in mothers or in dyadic interactive styles. Concerning the child's attachment at 12 months old, no differences were found in attachment security. However, secure children with a cleft were significantly more avoidant with their mother during the reunion episodes than secure children without cleft. Conclusion :  Despite the facial disfigurement and the stress engendered by treatment during the first months of the infant's life, children with cleft and their mothers are doing as well as families without cleft with regard to the mothers' mental health, mother-child relationships, and later quality of attachment. A potential contribution for this absence of difference may be the pluridisciplinary support that families of children with cleft benefit from in Lausanne.

Early mother-child interaction and later quality of attachment in infants with an orofacial cleft compared to infants without cleft.

PubMed

Habersaat, Stephanie; Monnier, Maryline; Peter, Camille; Bolomey, Luce; Borghini, Ayala; Despars, Josée; Pierrehumbert, Blaise; Müller-Nix, Carole; Ansermet, François; Hohlfeld, Judith

2013-11-01

Objective : The main objective of this study was to assess mother-child patterns of interaction in relation to later quality of attachment in a group of children with an orofacial cleft compared with children without cleft. Design : Families were contacted when the child was 2 months old for a direct assessment of mother-child interaction and then at 12 months for a direct assessment of the child's attachment. Data concerning socioeconomical information and posttraumatic stress symptoms in mothers were collected at the first appointment. Participants : Forty families of children with a cleft and 45 families of children without cleft were included in the study. Families were recruited at birth in the University Hospital of Lausanne. Results : Results showed that children with a cleft were more difficult and less cooperative during interaction at 2 months of age with their mother compared with children without a cleft. No significant differences were found in mothers or in dyadic interactive styles. Concerning the child's attachment at 12 months old, no differences were found in attachment security. However, secure children with a cleft were significantly more avoidant with their mother during the reunion episodes than secure children without cleft. Conclusion : Despite the facial disfigurement and the stress engendered by treatment during the first months of the infant's life, children with cleft and their mothers are doing as well as families without cleft with regard to the mothers' mental health, mother-child relationships, and later quality of attachment. A potential contribution for this absence of difference may be the pluridisciplinary support that families of children with cleft benefit from in Lausanne.

A randomized controlled trial comparing two techniques for unilateral cleft lip and palate: Growth and speech outcomes during mixed dentition.

PubMed

Ganesh, Praveen; Murthy, Jyotsna; Ulaghanathan, Navitha; Savitha, V H

2015-07-01

To study the growth and speech outcomes in children who were operated on for unilateral cleft lip and palate (UCLP) by a single surgeon using two different treatment protocols. A total of 200 consecutive patients with nonsyndromic UCLP were randomly allocated to two different treatment protocols. Of the 200 patients, 179 completed the protocol. However, only 85 patients presented for follow-up during the mixed dentition period (7-10 years of age). The following treatment protocol was followed. Protocol 1 consisted of the vomer flap (VF), whereby patients underwent primary lip nose repair and vomer flap for hard palate single-layer closure, followed by soft palate repair 6 months later; Protocol 2 consisted of the two-flap technique (TF), whereby the cleft palate (CP) was repaired by two-flap technique after primary lip and nose repair. GOSLON Yardstick scores for dental arch relation, and speech outcomes based on universal reporting parameters, were noted. A total of 40 patients in the VF group and 45 in the TF group completed the treatment protocols. The GOSLON scores showed marginally better outcomes in the VF group compared to the TF group. Statistically significant differences were found only in two speech parameters, with better outcomes in the TF group. Our results showed marginally better growth outcome in the VF group compared to the TF group. However, the speech outcomes were better in the TF group. Copyright © 2015 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Comparison of two nasoalveolar molding techniques in unilateral complete cleft lip patients: a randomized, prospective, single-blind trial to compare nasal outcomes.

PubMed

Chang, Chun-Shin; Wallace, Christopher Glenn; Pai, Betty Chien-Jung; Chiu, Yu-Ting; Hsieh, Yuh-Jia; Chen, I-Ju; Liao, Yu-Fang; Liou, Eric Jen-Wein; Chen, Philip Kuo-Ting

2014-08-01

Nasoalveolar molding became increasingly popular in the 1990s as a means of easing surgery and improving nasal outcomes for cleft lip repairs. In the late 1990s, three orthodontists from our center underwent nasoalveolar molding training: two at the Rush Craniofacial Center, in Chicago; and one at New York University Craniofacial Center. They brought two different nasoalveolar molding techniques back to Chang Gung Craniofacial Center: the modified Figueroa and the modified Grayson techniques. Outcomes following use of these techniques have not previously been compared prospectively. Between May of 2010 and March of 2013, a randomized, prospective, single-blind trial was conducted to compare the number of clinical visits, total costs, complications, and nasal symmetry between the two nasoalveolar molding techniques in 30 patients with unilateral complete cleft lip. There were no differences between nasoalveolar molding techniques in the number of clinical visits, total costs, nostril height, or nostril area ratio. Preoperatively but after nasoalveolar molding, the nostril width ratio was wider for the Figueroa group than for the Grayson group. Six months after surgical correction, there were no differences in nostril height, nostril width, nasal sill height, or nostril area ratio between nasoalveolar molding methods. Alveolar ulceration occurred more frequently in the Grayson group. The modified Grayson technique reduced nostril width more efficiently, but alveolar ulceration was more frequent and no differences in nostril width were found following surgery. Overall, the two nasoalveolar molding techniques produced similar nasal outcomes. Therapeutic, II.

Maternal exposure to ozone and PM2.5 and the prevalence of orofacial clefts in four U.S. states

PubMed Central

Zhou, Ying; Gilboa, Suzanne M.; Herdt, Michele L.; Lupo, Philip J.; Flanders, W. Dana; Liu, Yang; Shin, Mikyong; Canfield, Mark A.; Kirby, Russell S.

2017-01-01

Background While there is some evidence that maternal exposure to ambient air pollution is associated with orofacial clefts in offspring, the epidemiologic studies have been largely equivocal. We evaluated whether maternal exposure to elevated county-level ambient fine particulate matter with aerodynamic diameter ≤2.5 μm (PM2.5) and ozone during early gestation was associated with a higher prevalence of orofacial clefts. Methods Birth data consisting of 4.7 million births from 2001 to 2007 were obtained from National Birth Defects Prevention Network for four states — Arizona, Florida, New York (excluding New York City), and Texas. The air pollution exposure assessment for gestational weeks 5–10 was based on county-level average concentrations of PM2.5 and ozone data generated using a Bayesian fusion model available through CDC's Environmental Public Health Tracking Network. Two outcomes were analyzed separately: cleft lip with or without cleft palate, cleft palate alone. In logistic regression analyses, we adjusted for factors that were suspected confounders or modifiers of the association between the prevalence of orofacial clefts and air pollution, i.e., infant sex, race-ethnicity, maternal education, smoking status during pregnancy, whether this was mother's first baby, maternal age. Results Each 10 μg/m3 increase in PM2.5 concentration was significantly associated with cleft palate alone (OR =1.43, 95% CI: 1.11–1.86). There was no significant association between PM2.5 concentration and cleft lip with or without cleft palate. No associations were observed between ozone exposure and the two outcomes of orofacial clefts. Conclusions Our study suggests that PM2.5 significantly increased the risk of cleft palate alone, but did not change the incidence of cleft lip with or without palate. Ozone levels did not correlate with incidence of orofacial clefts. PMID:27888746

Treatment of alveolar cleft with distraction osteogenesis using anchorage with a tooth-microimplant joint in a dog model.

PubMed

Huang, Dai-Ying; Zhang, Ji-Bing; Li, Xiang; Chen, Song-Ling

2012-10-01

Our aim was to investigate the efficacy of correction of an alveolar cleft with distraction osteogenesis using anchorage with a tooth-microimplant joint in a canine model, which was established in 12 adult mongrel dogs that were subsequently randomised into two groups (n=6 in each). The first group comprised dogs that had osteogenesis using anchorage with a tooth (tooth group), while in the second, anchorage with tooth-microimplant joint (microimplant group) was used. All animals were killed one month after completion of distraction. Samples were collected for gross observation and histological examination. There was a significant difference in the degree of movement of the anchorage teeth in the transport discs between the 2 groups (p<0.01). There was less prominent inclination and shift of the natural teeth in the transport disc and less bony resorption around the root in the microimplant group than in the tooth group. These changes were less remarkable in the microimplant group. Treatment of alveolar cleft by distraction osteogenesis using anchorage with a tooth-microimplant joint is practical, and yields better results. Copyright © 2011 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Suggestion of an oral hygiene program for orthodontic patients with cleft lip and palate: findings of a pilot study.

PubMed

Brasil, Juliana Marcelina Plácido; de Almeida Pernambuco, Renata; da Silva Dalben, Gisele

2007-11-01

To evaluate the efficacy of an oral hygiene program for orthodontic patients with cleft lip and palate. Retrospective pilot study. Hospital for Rehabilitation of Craniofacial Anomalies, Bauru, Brazil. One hundred twenty-two patients with complete cleft lip and palate undergoing orthodontic treatment. Orientation on toothbrushing and flossing, plaque disclosure, and scoring according to an especially designed index. Statistical comparison of variation in plaque index between sessions; correlation of intervals between sessions and variation in plaque index. Mean scores were reduced significantly, from 2.17 to 1.75 between first and second, 2.18 to 1.62 between first and third, and 1.93 to 1.62 between second and third sessions. Plaque reduction was inversely proportional to the time interval. The program demonstrated a significant plaque reduction. The highest reduction between the first and second sessions reveals the need to reinforce the initial instructions at all sessions. The greatest reduction observed at shorter intervals highlights the need for regular follow up. More controlled studies on larger samples should be encouraged to evaluate the validity of the index and the efficacy of similar programs worldwide.

The American Cleft Palate Association: its first 36 years.

PubMed

Wells, C G

1979-01-01

History involves memory, and sometimes memory is faulty. History requires documentation, and sometimes documents cannot be found. History demands winnowing, and sometimes the wheat and the chaff cannot be completely separated. History is an assembly of details, and details may become burdensome. History is a challenge, to which the historian tries to respond. Surely, earlier Historians of the American Cleft Palate Association must have encountered this challenge. The present Historian acknowledges the work of her predecessors: George H. Foster, W. J. Robinson, and William Harkins from dentistry and Gretchen M. Phair and Asa J. Berlin from speech pathology. In preparing this review of events that took place from 1943 to 1978, in the United States and in other countries, during times of war and times of relative peace, through difficulties and accomplishments, I have attempted to be as accurate as possible. If I have distorted facts and if I have omitted people and happenings that should have been included, I have not done so intentionally. As Historian of the American Cleft Palate Association, I have appreciated the opportunity to learn about the organization and the people, past and present, who have built and maintained it. Thank you for giving me this assignment.

Dental Care for a Child with Cleft Lip and Palate

MedlinePlus

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Cleft Lip and Cleft Palate--What to Know and Who Can Help

ERIC Educational Resources Information Center

Apel, Laura

2008-01-01

Craniofacial defects such as cleft lip and cleft palate are among the most common of all birth defects in the United States, with one in every 600 newborns affected. Cleft lip and/or palate can occur as an isolated condition or may be one component of an inherited disease or syndrome. Dealing with the condition is an extremely difficult and…

Congenital Spondylolytic Spondylolisthesis of C2 Vertebra Associated With Atlanto-Axial Dislocation, Chiari Type I Malformation, and Anomalous Vertebral Artery: Case Report With Review Literature.

PubMed

Sardhara, Jayesh; Pavaman, Sindgikar; Das, Kuntal; Srivastava, Arun; Mehrotra, Anant; Behari, Sanjay

2016-11-01

Congenital spondylolytic spondylolisthesis of C2 vertebra resulting from deficient posterior element of the axis is rarely described in the literature. We describe a unique case of agenesis of posterior elements of C2 with craniovertebral junction anomalies consisting of osseous, vascular, and soft tissue anomalies. A 26-year-old man presented with symptoms of upper cervical myelopathy of 12 months' duration. A computed tomography scan of the cervical spine including the craniovertebral junction revealed spondylolisthesis of C2 over C3, atlantoaxial dislocation, occipitalization of the atlas, hypoplasia of the odontoid, and cleft posterior C1 arch. Additionally, the axis vertebra was found devoid of its posterior elements except bilaterally rudimentary pedicles. Magnetic resonance imaging revealed tonsilar herniation, suggesting associated Chiari type I malformation. CT angiogram of the vertebral arteries displayed persistent bilateral first intersegmental arteries crossing the posterior aspect of the C1/2 facet joint. This patient underwent foramen magnum decompression, C3 laminectomy with occipito-C3/C4 posterior fusion using screw and rod to maintain the cervical alignment and stability. We report this rare constellation of congenital craniovertebral junction anomaly and review the relevant literature. Copyright © 2016 Elsevier Inc. All rights reserved.

[A retrospective study of orthodontic treatment of children with clefts].

PubMed

Brin, I; Bar-Abudi, R; Abed, Y; Ben-Bassat, Y; Harari, D; Zilberman, Y

2003-04-01

To evaluate the population of cleft patients treated in a Department of Orthodontics and the types of treatment modalities provided. Demographic, cleft related and treatment related data existing in the patients' files were supplemented by questionnaires. Comparisons were conducted among 3 cleft groups: cleft lip (CL), cleft lip and palate (CLP) and cleft palate (CP). The response rate was 36% (n = 152). The distribution of the patients in the 3 cleft groups, the sidedness, the male predominance and association with additional anomalies were similar to the reports in the literature. Most of the patients were the 3rd born (or more) and were of normal birth weight. Consanguity in the cleft families was at least 2.5 times more prevalent than that of the Israeli population and 30% reported on additional cleft in the family. Low birth weight and additional anomalies were found mainly in the CP group. Orthodontic involvement spanned 3 developmental periods: immediate postnatal presurgical treatment, phase I between the ages 6-8 years and full orthodontic treatment at a later age. Up to the age of 5 years only one lip operation was performed for 60% of the lip-affected children and one palate operation for 65% of the palate affected patients. 1. The distribution of the various cleft-related parameters in this retrospective study was similar to the findings in the literature. 2. The high prevalence of additional anomalies found emphasizes the need for a thorough examination of the cleft babies. 3. Orthodontic treatment was rendered in one and two phase protocols in addition to immediate postnatal pre-surgical intervention.

Dental anomalies associated with cleft lip and palate in Northern Finland.

PubMed

Lehtonen, V; Anttonen, V; Ylikontiola, L P; Koskinen, S; Pesonen, P; Sándor, G K

2015-12-01

Despite the reported occurrence of dental anomalies of cleft lip and palate, little is known about their prevalence in children from Northern Finland with cleft lip and palate. The aim was to investigate the prevalence of dental anomalies among patients with different types of clefts in Northern Finland. Design and Statistics: patient records of 139 subjects aged three years and older (with clefts treated in Oulu University Hospital, Finland during the period 1996-2010 (total n. 183) were analysed for dental anomalies including the number of teeth, morphological and developmental anomalies and their association with the cleft type. The analyses were carried out using Chi-square test and Fisher's exact test. Differences between the groups were considered statistically significant at p values < 0.05. More than half of the patients had clefts of the hard palate, 18% of the lip and palate, and 13% of the lip. At least one dental anomaly was detected in 47% of the study population. Almost one in three (26.6%) subjects had at least one anomaly and 17.9% had two or three anomalies. The most common type of anomaly in permanent teeth were missing teeth followed by supernumerary teeth. Supernumerary teeth were significantly more apparent when the lip was involved in the cleft compared with palatal clefts. Missing teeth were less prevalent among those 5 years or younger. The prevalence of different anomalies was significantly associated with the cleft type in both age groups. Dental anomalies are more prevalent among cleft children than in the general population in Finland. The most prevalent anomalies associated with cleft were missing and supernumerary teeth.

Oral Clefting in China Over the Last Decade: 205,679 Patients

PubMed Central

Kling, Rochelle R.; Taub, Peter J.; Ye, Xiaoqian

2014-01-01

Background: China is the most populated country and has one of the highest prevalences of oral clefting. The present study reports the epidemiology and surgical procedures performed on the largest reported cohort of individuals with clefting in China. Methods: A retrospective review of patients who received cleft repair through Smile Train in China from 2000 to 2011 was conducted. Data on demographics, cleft characteristics, associated malformations, pregnancy and family history, and surgical technique were analyzed using SPSS (IBM, Chicago, Ill.). Results: A total of 205,679 patients underwent 209,169 cleft procedures. Cleft lip and palate (42.7%) was most common followed by isolated cleft palate (32.4%) and isolated cleft lip (24.9%). Males accounted for 63.5% of cases. The average age at initial surgery was 6.12 years. By 2011, this decreased to 1.8 years of age for lip repair and to 5.9 years of age for palate repair. The preferred techniques were rotation-advancement (55%) for unilateral lip repair and Von-Langenbeck (38%) and pushback (39%) for palate repair. The percentages of cases with associated anomalies and surgical complications were 12.8% and 0.36%, respectively. Conclusions: This study provides insight into cleft care in China as it reports the largest cohort of cleft patients treated by surgeons to date. Our results generally follow trends previously reported in China and developed countries. The male:female ratio for cleft palate patients was higher than expected. The average age at primary repair is higher than recommended, but seems to be decreasing. PMID:25426353

Three-dimensional morphology of first molars in relation to ethnicity and the occurrence of cleft lip and palate.

PubMed

Echtermeyer, Sandra; Metelmann, Philine H; Hemprich, Alexander; Dannhauer, Karl-Heinz; Krey, Karl-Friedrich

2017-01-01

This study aims to describe morphological peculiarities of maxillary and mandibular first molars in Europeans, Asians and Europeans with cleft lip and palate. Reflex microscopy was used to obtain three-dimensional morphometric landmarks from 40 models (11 Europeans and 13 Asians without cleft lip and palate, 16 Europeans with unilateral cleft lip and palate). The cases were examined using traditional morphometry and geometric morphometry, and visualized using thin-plate splines. Classic morphometry showed no right/left differences in the study groups and no significant differences with regard to the cleft side in patients with cleft lip and palate. In Asians, a significantly greater mesiodistal width was found. Geometric morphometry showed an enlarged centroid size in Asians (maxilla and mandible). In cleft patients, the cleft site did not appear to impact the morphology of first molars. Unilateral clefting did not affect the size and shape of molars; however, characteristic ethnicity-based differences were in fact identified. The results are relevant for orthodontic treatment with preadjusted appliances, and prosthetic CAD/CAM restorations.

Cleft lip and palate: an adverse pregnancy outcome due to undiagnosed maternal and paternal coeliac disease.

PubMed

Arakeri, Gururaj; Arali, Veena; Brennan, Peter A

2010-07-01

Development of orofacial component involves a complex series of events. Any insult to this significant event can lead to various orofacial cleft defects. The main categories among orofacial clefts are isolated cleft palate and cleft lip with or without cleft palate. There have been many factors implicated in the development of the anomaly. The environmental factors which contribute and the genes which predispose to the condition remain obscure despite decades of research. Though it is generally agreed that folic acid deficiency is a contributory factor for non-syndromic cleft lip and palate, fewer concerns are directed towards the role for maternal/paternal nutrition in orofacial cleft origin. However, previously undescribed, here we consider the potential influence of maternal and paternal coeliac disease on the etiology of non-syndromic cleft lip and palate as an unfavorable pregnancy outcome. We postulated this relationship based on our observation, study and an empirical survey, and could be due either to (I) folic acid mal absorption (II) a genetically mediated genomic imprinting system. Copyright 2010 Elsevier Ltd. All rights reserved.

Biological Effects of Orthodontic Tooth Movement Into the Grafted Alveolar Cleft.

PubMed

Sun, Jian; Zhang, Xiaoyue; Li, Renmei; Chen, Zhengxi; Huang, Yuanliang; Chen, Zhenqi

2018-03-01

Functional stimulus during orthodontic tooth movement into the grafted bone can lead to better alveolar bone grafting outcomes. The aim of this study was to analyze the biological effects of orthodontic tooth movement into the grafted alveolar cleft area with histologic staining, fluorescence staining, and real-time polymerase chain reaction (PCR). An animal model of orthodontic tooth movement into the grafted alveolar cleft area was established in 8-week-old Sprague-Dawley rats. The animals were divided into the experimental group and the control group. Four checkpoints were observed: before orthodontic stimuli, day 1 after orthodontic stimuli, day 3 after orthodontic stimuli, and day 5 after orthodontic stimuli. The cleft bone formation conditions, including the collagen fibers and the activities of the osteoclasts and osteoblasts, were evaluated by histologic staining. The expression of tartrate-resistant acid phosphatase (TRAP), receptor activator nuclear factor κB ligand, and Runt-related transcription factor 2 was detected by real-time PCR in both groups. Hematoxylin-eosin staining showed that the remodeling process of iliac autografts was completed when the orthodontic stress was applied, whereas the bone tissues first showed osteoclastogenesis and then osteogenesis. On the basis of TRAP staining, the osteoclasts increased to the maximal amount on day 3 and decreased thereafter. Evidence from tetracycline fluorescence staining indicated that no obvious changes in osteoblast activity were detected at the early stage; however, it gradually increased, especially in the region close to the root surface. According to real-time PCR, the expression of TRAP increased in both the early and middle stages, that of receptor activator nuclear factor κB ligand increased in the early stage, and that of Runt-related transcription factor 2 increased in the late stage. Moreover, the results showed significant differences between the experimental and control groups. Orthodontic tooth movement into the alveolar cleft bone graft area promoted bone remodeling of embedded bone, thus inducing bone resorption and subsequent deposition. Copyright © 2017. Published by Elsevier Inc.

Is It Safe to Re-Harvest the Anterior Iliac Crest to Manage Le Fort I Interpositional Defects in Young Adults With a Repaired Cleft?

PubMed

Posnick, Jeffrey C; Gray, James A

2015-12-01

Re-harvesting the anterior iliac crest to obtain autogenous bone grafts is a controversial practice. The purpose of this study was to assess the feasibility and associated disability of re-harvesting the anterior iliac crest. To address the research purpose, the authors executed a retrospective case series study. The sample consisted of young adult patients with cleft (<26 yr old) with prior harvesting of the anterior iliac crest during mixed dentition for management of an alveolar cleft(s) and then re-harvesting of the same donor site for management of interpositional defects after Le Fort I advancement. Wound-healing parameters were reviewed at the donor and recipient sites. A survey questionnaire was provided after completion of treatment to document patient perception of early and any long-term donor-site disability. Descriptive statistics were computed for the variables. The sample was composed of 27 patients with a mean age at re-harvesting of 17 years (range, 14 to 25 yr). Patients underwent on average 7.4-mm horizontal advancement, 2.4-mm lengthening, and 2.6-mm transverse expansion of the maxilla. Adequate bone graft was re-harvested to accomplish objectives in all cases. There were no perioperative complications at the donor or recipient sites. Twenty-six of the 27 patients (97%) had fewer donor-site recovery difficulties at the time of re-harvesting compared with the first time graft was taken. There were no cases of lateral femoral cutaneous nerve injury and no long-term discomfort with walking, running, or other activities. The study confirms the safety and efficacy of re-harvesting corticocancellous bone from the anterior iliac crest for management of interpositional defects associated with Le Fort I advancement in young adults with a repaired cleft. Copyright © 2015 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

Long-term follow-up of large maxillary advancements with distraction osteogenesis in growing and non-growing cleft lip and palate patients.

PubMed

Meazzini, Maria Costanza; Basile, Valentina; Mazzoleni, Fabio; Bozzetti, Alberto; Brusati, Roberto

2015-01-01

Maxillary distraction osteogenesis (DO) in cleft lip and palate patients has been described by several authors, but most studies have a relatively short follow-up and do not clearly separate growing patients from non-growing patients. The records of 22 consecutive patients affected by cleft lip and palate, who underwent Le Fort I osteotomy and maxillary distraction with a rigid external distractor (RED), were reviewed. The sample was subdivided into a growing and a non-growing group. All patients had pre-DO cephalometric records, immediately post DO, 12 months post DO and long-term records with a long-term follow-up of >5 years (range 5-13 years). As a control sample for the growing group, cleft children with a negative overjet not subjected to distraction or any protraction treatment during growth were followed up until the completion of growth. The average maxillary advancement in the growing group was 22.2 ± 5.5 mm (range: 15-32 mm); in the non-growing group, it was 17.7 ± 6.6 mm (range: 6-25 mm). Excellent post-surgical stability was recorded in the adult sample. On the other hand, growing children had an average 16% relapse in the first year post DO and an additional 26% relapse in the long-term follow-up. This study seems to point out that early Le Fort I DO allows for the correction of very severe deformities. It is followed by a relatively high amount of true skeletal relapse in children with cleft lip and palate. Prognosis should be discussed in depth with the family and true aesthetic and psychological needs assessed. Copyright © 2014 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

Three-Dimensional Cone Beam Computed Tomography Volumetric Outcomes of rhBMP-2/Demineralized Bone Matrix versus Iliac Crest Bone Graft for Alveolar Cleft Reconstruction.

PubMed

Liang, Fan; Yen, Stephen L-K; Imahiyerobo, Thomas; Sanborn, Luke; Yen, Leia; Yen, Daniel; Nazarian, Sheila; Jedrzejewski, Breanna; Urata, Mark; Hammoudeh, Jeffrey

2017-10-01

Recent studies indicate that recombinant human bone morphogenetic protein-2 (rhBMP-2) in a demineralized bone matrix scaffold is a comparable alternative to iliac bone autograft in the setting of secondary alveolar cleft repair. Postreconstruction occlusal radiographs demonstrate improved bone stock when rhBMP-2/demineralized bone matrix (DBM) scaffold is used but lack the capacity to evaluate bone growth in three dimensions. This study uses cone beam computed tomography to provide the first clinical evaluation of volumetric and density comparisons between these two treatment modalities. A prospective study was conducted with 31 patients and 36 repairs of the alveolar cleft over a 2-year period. Twenty-one repairs used rhBMP-2/DBM scaffold and 14 repairs used iliac bone grafting. Postoperatively, occlusal radiographs were obtained at 3 months to evaluate bone fill; cone beam computed tomographic images were obtained at 6 to 9 months to compare volumetric and density data. At 3 months, postoperative occlusal radiographs demonstrated that 67 percent of patients receiving rhBMP-2/DBM scaffold had complete bone fill of the alveolus, versus 56 percent of patients in the autologous group. In contrast, cone beam computed tomographic data showed 31.6 percent (95 percent CI, 24.2 to 38.5 percent) fill in the rhBMP-2 group compared with 32.5 percent (95 percent CI, 22.1 to 42.9 percent) in the autologous population. Density analysis demonstrated identical average values between the groups (1.38 g/cc). These data demonstrate comparable bone regrowth and density values following secondary alveolar cleft repair using rhBMP-2/DBM scaffold versus autologous iliac bone graft. Cone beam computed tomography provides a more nuanced understanding of true bone regeneration within the alveolar cleft that may contribute to the information provided by occlusal radiographs alone. Therapeutic, II.

Prosthetic Rehabilitation of Completely Blind Subject with Bilateral Customised Ocular Prosthesis: A Case Report

PubMed Central

Pawah, Salil; Singh, Gurpreet; Yadav, Ila; Kundra, Shrey

2017-01-01

The special sensory organs play significant role in our daily lives. Until one of them is totally or partially lost, its real value is not fully appreciated. The most tragic, yet common loss of sensory organ is that of an eye. Restoration of unilateral ocular defects is very challenging for a maxillofacial prosthodontist and requires great skill to mimic bilateral eye. Restoration of bilateral defects using customized eye prosthesis presents even more difficult task for clinician especially in communication with patient, impression procedures, and patient education regarding insertion, removal and maintenance of prosthesis. Such loss of eyes may have a tremendous emotional and psychological impact on the patient. This article presents a technique for fabrication of bilateral ocular prosthesis of completely blind patient. PMID:28274064

Tessier 3 Cleft in a Pre-Hispanic Anthropomorphic Figurine in El Salvador, Central America.

PubMed

Aleman, Ramon Manuel; Martinez, Maria Guadalupe

2017-03-01

In 1976, Paul Tessier provided a numerical classification system for rare facial clefts, numbered from 0 to 14. The Tessier 3 cleft is a rare facial cleft extending from the philtrum of the upper lip through the wing of the nostril, and reaches the medial canthus of the eye. The aim of this document was to describe a pre-Hispanic anthropomorphic figurine dating from the classic period (200 A.D.-900 A.D.), which has a Tessier 3 cleft. We also discuss the documented pre-Hispanic beliefs about facial clefts.

Epidemiological and genetic study in 207 cases of oral clefts in Alsace, north-eastern France.

PubMed Central

Stoll, C; Alembik, Y; Dott, B; Roth, M P

1991-01-01

The epidemiology of oral clefts was studied in the geographical area covered by our registry of congenital malformations. For each of the 207 new cases studied during the period 1979 to 1987, more than 50 factors were compared in probands and controls. The incidence of oral clefts was 1.75 per 1000, with cleft lip/palate (CL(P] 0.98 and cleft palate only (CP) 0.77 per 1000. A total of 8.2% of cleft cases were stillbirths and 5.3% were induced abortions. The more common types of associated malformations in the 76 affected cases (36.7%) with at least one anomaly other than oral cleft were neural tube defects and skeletal malformations. At birth, infants with oral clefts and other malformations were smaller, weighed less, and their head circumference was lower than in controls. Placental weight was also lower than in controls. Pregnancies with oral clefts were more often complicated by threatened abortion, polyhydramnios, and arterial hypertension. There was a significant association between clefting and consanguinity; heritability of CL(P) was 81% and first degree relatives of probands had more than three times the prevalence of noncleft malformations as controls. These results are of relevance to genetic counselling. PMID:1842671

Variations in Velopharyngeal Structure in Adults With Repaired Cleft Palate.

PubMed

Perry, Jamie L; Kotlarek, Katelyn J; Sutton, Bradley P; Kuehn, David P; Jaskolka, Michael S; Fang, Xiangming; Point, Stuart W; Rauccio, Frank

2018-01-01

The purpose of this study was to examine differences in velopharyngeal structures between adults with repaired cleft palate and normal resonance and adults without cleft palate. Thirty-six English-speaking adults, including 6 adults (2 males and 4 females) with repaired cleft palate (M = 32.5 years of age, SD = 17.4 years) and 30 adults (15 males and 15 females) without cleft palate (M = 23.3 years of age, SD = 4.1 years), participated in the study. Fourteen velopharyngeal measures were obtained on magnetic resonance images and compared between groups (cleft and noncleft). After adjusting for body size and sex effects, there was a statistically significant difference between groups for 10 out of the 14 velopharyngeal measures. Compared to those without cleft palate, participants with repaired cleft palate had a significantly shorter hard palate height and length, shorter levator muscle length, shorter intravelar segment, more acute levator angles of origin, shorter and thinner velum, and greater pharyngeal depth. Although significant differences were evident in the cleft palate group, individuals displayed normal resonance. These findings suggest that a wide variability in velopharyngeal anatomy can occur in the presence of normal resonance, particularly for those with repaired cleft palate. Future research is needed to understand how anatomic variability impacts function, such as during speech.

Correction of the bilateral question mark ear deformity using double-opposing z-plasty and a chondrocutaneous flap.

PubMed

Bastidas, Nicholas; Taylor, Jesse A

2013-03-01

To describe an alternative technique for correcting a rare and complex ear deformity. The question mark ear deformity has been described as a congenital cleft between the helix and the lobule in addition to a prominent ear. Here we describe a novel technique using double-opposing Z-plasty and a chondrocutaneous flap to correct the deformity without significant reduction in auricle height or creation of a donor-site defect. An anterior/posterior double-opposing Z-plasty and a superiorly based chondrocutaneous flap were designed at the level of the cleft. Flaps were raised full thickness, and Mustarde and Furnas sutures were placed to reduce the prominence. The flaps were then transposed and reapproximated. Of note, all anterior and posterior skin flaps were used in the repair to minimize diminution of ear height. Pre- and postoperative measurements and photographs of the ears were taken. Postoperatively, all flaps were viable, and there was no evidence of skin necrosis. The total length of the left ear was reduced by only 3 mm (53 to 50 mm), and the right ear was reduced by 1 mm (52 to 51 mm). The patient's pain was successfully managed as an outpatient, and there were no infectious complications. The resultant scars healed aesthetically and are minimally visible. The patient and her parents are satisfied with the results. Our technique addresses all components of the question mark ear deformity and does not create a visible donor-site deformity.

[Cloverleaf skull and bilateral facial clefts].

PubMed

Alvarez-Manassero, Denisse; Manassero-Morales, Gioconda

2015-01-01

Cloverleaf skull syndrome, or Kleeblattschädel syndrome, is a rare malformation in which the skull has a cloverleaf appearance. It is caused by the premature closure of several sutures, being evident before birth. To present our experience in a case of cloverleaf skull syndrome, and update the information from the literature. A female infant of 5 months of age, diagnosed at birth with cleft lip and palate and hydrocephaly. A peritoneal ventricle valve was implanted at 30 days of life, and an ocular enucleation was performed due to an infectious process. The patient was followed-up in Genetics, where it confirmed a macrocephaly and craniosynostosis type cloverleaf skull. The 46XX cytogenetic study and echocardiography were normal. The brain CT scan showed multiple anomalies associated with hydrocephaly and non-specific malformations. Cloverleaf skull may be present in isolated form or associated with other congenital abnormalities, leading to various craniosynostosis syndromes, such as Crouzon, Pfeiffer or Carpenter. It may also be a component of the amniotic rupture sequence or to different dysplasias, such as campomelic dysplasia, thanatophoric dysplasia type 2, or the asphyxiating thoracic dystrophy of Jeune. The case presented does not fulfil all the characteristics needed to be included within a specific syndrome, and on not having a family history that suggests a hereditary pattern or chromosome abnormalities, it is concluded that it is a case of a congenital anomaly of sporadic presentation. Copyright © 2015 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

Incidence of cleft pathology in Greater New Orleans before and after Hurricane Katrina.

PubMed

Goenjian, Haig A; Chiu, Ernest S; Alexander, Mary Ellen; St Hilaire, Hugo; Moses, Michael

2011-11-01

Reports after the 2005 Hurricane Katrina have documented an increase in stress reactions and environmental teratogens (arsenic, mold, alcohol). To assess the incidence of cleft pathology before and after the hurricane, and the distribution of cleft cases by gender and race. Retrospective chart review of cleft lip with or without cleft palate (CL/P) and cleft palate (CP) cases registered with the Cleft and Craniofacial Team at Children's Hospital of New Orleans, the surgical center that treated cleft cases in Greater New Orleans between 2004 and 2007. Live birth data were obtained from the Louisiana State Center for Health Statistics. The incidence of cleft cases, beginning 9 months after the hurricane (i.e., June 1, 2006) was significantly higher compared with the period before the hurricane (0.80 versus 1.42; p = .008). Within racial group comparisons showed a higher incidence among African Americans versus whites (0.42 versus 1.22; p = .01). The distribution of CL/P and CP cases by gender was significant (p = .05). The increase in the incidence of cleft cases after the hurricane may be attributable to increased stress and teratogenic factors associated with the hurricane. The increase among African Americans may have been due to comparatively higher exposure to environmental risk factors. These findings warrant further investigation to replicate the results elsewhere in the Gulf to determine whether there is a causal relationship between environmental risk factors and increased cleft pathology.

The Cleft Aesthetic Rating Scale for 18-Year-Old Unilateral Cleft Lip and Palate Patients: A Tool for Nasolabial Aesthetics Assessment.

PubMed

Mulder, F J; Mosmuller, D G M; de Vet, H C W; Mouës, C M; Breugem, C C; van der Molen, A B Mink; Don Griot, J P W

2018-01-01

Objective To develop a reliable and easy-to-use method to assess the nasolabial appearance of 18-year-old patients with unilateral cleft lip and palate (CLP). Design Retrospective analysis of nasolabial aesthetics using a 5-point ordinal scale and newly developed photographic reference scale: the Cleft Aesthetic Rating Scale (CARS). Three cleft surgeons and 20 medical students scored the nasolabial appearance on standardized frontal photographs. Setting VU University Medical Center, Amsterdam. Patients Inclusion criteria: 18-year-old patients, unilateral cleft lip and palate, available photograph of the frontal view. history of facial trauma, congenital syndromes affecting facial appearance. Eighty photographs were available for scoring. Main Outcome Measures The interobserver and intraobserver reliability of the CARS for 18-year-old patients when used by cleft surgeons and medical students. Results The interobserver reliability for the nose and lip together was 0.64 for the cleft surgeons and 0.61 for the medical students. There was an intraobserver reliability of 0.75 and 0.78 from the surgeons and students, respectively, on the nose and lip together. No significant difference was found between the cleft surgeons and medical students in the way they scored the nose ( P = 0.22) and lip ( P = 0.72). Conclusions The Cleft Aesthetic Rating Scale for 18-year-old patients has a substantial overall estimated reliability when the average score is taken from three or more cleft surgeons or medical students assessing the nasolabial aesthetics of CLP patients.

The impact of congenital cardiovascular malformations on the assessment and surgical management of infants with cleft lip and/or palate.

PubMed

Harry, Brian L; TeBockhorst, Seth; Deleyiannis, Frederic W-B

2013-05-01

The purpose of this study was to assess the cardiac evaluation of cleft lip and/or palate patients, characterize their cardiovascular malformations, and determine the impact of cardiovascular malformations on surgical management. A single-institution retrospective study of 329 consecutive cleft patients was performed. Cardiovascular malformations were categorized according to involvement of cardiac septa, vasculature, and valves. Their impact on the need for cardiac surgery, timing of cleft repair, need for subacute bacterial endocarditis (SBE) prophylaxis, and the perioperative experience was evaluated. Ten percent (33/329) of cleft patients had a cardiovascular malformation, and 3% underwent cardiac surgery prior to cleft repair. Malformations of the septa, vasculature, and valves were present in 9%, 6%, and 2% of cleft infants, respectively. Murmur as a sign of structural cardiovascular disease was 79% sensitive and 97% specific. Cleft palate repair was delayed by 2 months in patients with a cardiovascular malformation (P = .001). Subacute bacterial endocarditis prophylaxis was recommended, not recommended, or not specified by cardiology in 18%, 33%, and 48% of cleft patients with a cardiovascular malformation, respectively. Postoperative stay and surgical complications were not associated with cardiovascular malformation. Even in the absence of a murmur, echocardiographic screening should be considered in infants with nonspecific signs of cardiovascular disease. Greater awareness of the guidelines for SBE prophylaxis is needed. Most cleft patients with a cardiovascular malformation do not require cardiac surgery and do not experience an increased rate of complications associated with cleft surgery.

Contractile properties of single permeabilized muscle fibers from congenital cleft palates and normal palates of Spanish goats.

PubMed

Hanes, Michael C; Weinzweig, Jeffrey; Kuzon, William M; Panter, Kip E; Buchman, Steven R; Faulkner, John A; Yu, Deborah; Cederna, Paul S; Larkin, Lisa M

2007-05-01

Analysis of the composition of muscle fibers constituent to a cleft palate could provide significant insight into the cause of velopharyngeal inadequacy. The authors hypothesized that levator veli palatini muscle dysfunction inherent to cleft palates could affect the timing and outcome of cleft palate repair. Single, permeabilized muscle fibers from levator veli palatini muscles of three normal (n = 19 fibers) and three chemically induced congenital cleft palates (n = 21 fibers) of 14-month-old goats were isolated, and contractile properties were evaluated. The maximum isometric force and rate constants of tension redevelopment (ktr) were measured, and the specific force and normalized power were calculated for each fiber. The ktr measures indicate that cleft fibers are predominantly fast-fatigable; normal fibers are slow fatigue-resistant: after a 10-minute isometric contraction, fibers from cleft palates had a loss of force 16 percent greater than that from normal palates (p = 0.0001). The cross-sectional areas of the fibers from cleft palates (2750 +/- 209 microm2) were greater (p = 0.05) than those from normal palates (2226 +/- 143 microm2). Specific forces did not differ between the two groups. Maximum normalized power of fibers from cleft palates (11.05 +/- 1.82 W/l) was greater (p = 0.0001) than fibers from normal palates (1.60 +/- 0.12 W/l). There are clear physiologic differences in single muscle fibers from cleft palates and normal palates: cleft palate fibers are physiologically fast, have greater fatigability, and have greater power production. Detection of functional and/or fiber type differences in muscles of cleft palates may provide preoperative identification of a patient's susceptibility to velopharyngeal inadequacy and permit early surgical intervention to correct this clinical condition.

Remote Digital Preoperative Assessments for Cleft Lip and Palate May Improve Clinical and Economic Impact in Global Plastic Surgery.

PubMed

Hughes, Christopher; Campbell, Jacob; Mukhopadhyay, Swagoto; McCormack, Susan; Silverman, Richard; Lalikos, Janice; Babigian, Alan; Castiglione, Charles

2017-09-01

Reconstructive surgical care can play a vital role in the resource-poor settings of low- and middle-income countries. Telemedicine platforms can improve the efficiency and effectiveness of surgical care. The purpose of this study is to determine whether remote digital video evaluations are reliable in the context of a short-term plastic surgical intervention. The setting for this study was a district hospital located in Latacunga, Ecuador. Participants were 27 consecutive patients who presented for operative repair of cleft lip and palate. We calculated kappa coefficients for reliability between in-person and remote digital video assessments for the classification of cleft lip and palate between two separate craniofacial surgeons. We hypothesized that the technology would be a reliable method of preoperative assessment for cleft disease. Of the 27 (81.4%) participants, 22 received operative treatment for their cleft disorder. Mean age was 11.1 ± 8.3 years. Patients presented with a spectrum of disorders, including cleft lip (24 of 27, 88.9%), cleft palate (19 of 27, 70.4%), and alveolar cleft (19 of 27, 70.4%). We found a 95.7% agreement between observers for cleft lip with substantial reliability (κ = .78, P < .01). There was an 82.6% agreement between observers for cleft palate, with a moderate interrater reliability (κ = .55, P = .01). We found only a 47.8% agreement between observers for alveolar cleft with a nonsignificant, weak kappa agreement (κ = .06, P = .74). Remote digital assessments are a reliable way to preoperatively diagnose cleft lip and palate in the context of short-term plastic surgical interventions in low- and middle-income countries. Future work will evaluate the potential for real-time, telemedicine assessments to reduce cost and improve clinical effectiveness in global plastic surgery.

Self-reports of psychosocial functioning among children and young adults with cleft lip and palate.

PubMed

Hunt, Orlagh; Burden, Donald; Hepper, Peter; Stevenson, Mike; Johnston, Chris

2006-09-01

A cross-sectional study was employed to determine the psychosocial effects of cleft lip and/or palate among children and young adults, compared with a control group of children and young adults without cleft lip and palate. The study comprised 160 children and young adults with cleft lip and/or palate and 113 children and young adults without cleft lip and/or palate. All participants were between 8 and 21 years of age. Psychological functioning (anxiety, self-esteem, depression, and behavioral problems) was assessed using validated psychological questionnaires. Happiness with facial appearance was rated using a visual analog scale. Social functioning, including experience of teasing/bullying and satisfaction with speech, was assessed using a semistructured interview. Participants with cleft lip and/or palate reported greater behavioral problems (p < .001) and more symptoms of depression (p < .01); they were teased more often (p < .001) and were less happy with their facial appearance (p < .01) and speech (p < .001), compared with controls. There were no significant difference between subjects with cleft lip and/or palate and subjects without cleft lip and/or palate in terms of anxiety (p > .05) or self-esteem (p > .05). Having been teased was a significant predictor of poor psychological functioning, more so than having a cleft lip and/or palate per se (p < .001). Teasing was greater among participants who had cleft lip and/ or palate and it was a significant predictor of poorer psychosocial functioning. Children and young adults with cleft lip and/or palate require psychological assessment, specifically focusing on their experience of teasing, as part of their routine cleft care.

Active Presurgical Infant Orthopedics for Unilateral Cleft Lip and Palate: Intercenter Outcome Comparison of Latham, Modified McNeil, and Nasoalveolar Molding.

PubMed

Kornbluth, Michelle; Campbell, Richard E; Daskalogiannakis, John; Ross, Elizabeth J; Glick, Patricia H; Russell, Kathleen A; Doucet, Jean-Charles; Hathaway, Ronald R; Long, Ross E; Sitzman, Thomas J

2018-05-01

To compare dental arch relationship, craniofacial form, and nasolabial aesthetic outcomes among cleft centers using distinct methods of presurgical infant orthopedics (PSIO). Retrospective cohort study. Four cleft centers in North America. One hundred ninety-one children with repaired complete unilateral cleft lip and palate (CUCLP). Dental arch relationship was assessed using the GOSLON Yardstick. Craniofacial form was assessed by 12 cephalometric measurements. Nasolabial aesthetics were assessed using the Asher-McDade system. Assessments were performed between 6 and 12 years of age. The center that used no PSIO achieved the most favorable dental arch relationship and maxillomandibular relationship, with a median GOSLON score of 2.3 ( P < .01) and an ANB angle of 5.1° ( P < .05). The proportion of children assigned a GOSLON score of 4 or 5, predictive of the need for orthognathic surgery in adolescence, was 16% at the center that used no PSIO and no secondary surgery, compared to 76% at the centers that used the Latham appliance and early secondary lip and nose surgery ( P < .01). The center that used no PSIO and no secondary surgery achieved significantly less favorable nasolabial aesthetic outcomes than the centers using Latham appliance or nasoalveolar molding (NAM) ( P < .01). Effects of active PSIO are multifaceted and intertwined with use of revision surgery. In our study, centers using either the Latham appliance combined with early revision surgery or the NAM appliance without revision surgery achieved better nasolabial aesthetic outcomes but worse maxillary growth, compared to a center using no PSIO and secondary surgery.

Teachers' Perspectives on the Impact of Cleft Lip and/or Palate During the School Years.

PubMed

Stock, Nicola Marie; Ridley, Matthew; Guest, Ella

2018-01-01

Cleft lip and/or palate (CL/P) poses many challenges for those affected throughout the school years, with recent studies pointing to a trend in poor educational outcomes. Previous research has highlighted the important role that teachers play in supporting young people with chronic health conditions yet studies in the field of CL/P suggest that teachers may lack knowledge of the condition, and underestimate pupils' academic ability. Qualitative investigation of the impact of CL/P from the teacher's perspective may provide additional insight as well as potential suggestions for improving the school experience for affected pupils. Twenty UK preschool, primary, and secondary school teachers with experience of teaching pupils with CL/P completed an open-ended survey, eliciting qualitative data. Qualitative content analysis was performed. Data were presented under 5 key headings: Knowledge of CL/P and its effects, perceived impact of CL/P on education, teachers' training needs, delivery of training, and impact of CL/P on teachers. Teachers were able to identify a number of potential social, emotional, and treatment-related challenges within the school setting, yet did not believe that CL/P would have a long-term impact on pupils' educational achievement. Participating teachers reported that cleft-specific training and access to relevant resources would be highly beneficial. Acting as a platform for future research, this exploratory study provides insight into the challenges faced by teachers and offers suggestions for how to improve teachers' understanding of CL/P, and their confidence in dealing with cleft-related issues.