Science.gov

Sample records for branchial cleft cyst

  1. Branchial Cleft-Like Cysts Involving 3 Different Organs

    PubMed Central

    Nakazawa, Tadao; Kondo, Tetsuo; Oishi, Naoki; Tahara, Ippei; Kasai, Kazunari; Inoue, Tomohiro; Mochizuki, Kunio; Katoh, Ryohei

    2015-01-01

    Abstract Branchial cleft cysts (BCCs) are also named lateral cervical cysts and widely acknowledged as being derived from embryonic remnants. Lymphoepithelial cysts (LECs) generally show microscopic features that are identical to those of BCCs, and rarely occur at unusual sites or organs. A case of multiple cysts arising in both lobes of the thyroid gland, thymus, and right parotid gland in a 41-year-old man is reported. Clinically, the patient presented with Hashimoto's thyroiditis for about 20 years and had past histories of idiopathic thrombocytopenic purpura and severe respiratory infection. This case is unusual in that multiple cysts arose synchronously and/or heterochronously and grew, increasing their sizes in these different organs. Microscopic examinations revealed that all of the cysts were composed of squamous epithelium, dense lymphoid tissue with germinal centers, and a fibrous capsule. These findings corresponded to those of BCCs or LECs. It is notable that the histopathological features were nearly the same in the individual organs. A review of the literature disclosed no previous such reported cases. The etiology is unknown. However, based upon the similar histopathological features of all the excised specimens, common immune and/or hematopoietic disorders may have contributed to their occurrence and development in association with putative genetic abnormalities. PMID:26496296

  2. 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

  3. [Intraparotid first branchial arch cyst: complex diagnostic and therapeutic process].

    PubMed

    Gilabert Rodríguez, R; Berenguer, B; González Meli, B; Marín Molina, C; de Tomás Palacios, E; Buitrago Weiland, G; Aguado del Hoyo, A

    2013-01-01

    First branchial arch cysts are uncommon. Therefore, together with its variable clinical and age presentation they are often misdiagnosed at first. The treatment is surgical, requiring a correct procedure to avoid future recurrences. In this paper we describe a typical case of first branchial arch cyst in which as described in other reports, we first made several misdiagnoses and therefore an inadequate treatment and lastly, with the correct diagnosis, we performed a meticulous complete excision under facial nerve monitoring. PMID:23833927

  4. Intrasphenoidal rathke cleft cyst.

    PubMed

    Megdiche-Bazarbacha, H; Ben Hammouda, K; Aicha, A B; Sebai, R; Belghith, L; Khaldi, M; Touibi, S

    2006-05-01

    Symptomatic Rathke cleft cysts (RCC) are reported in the sellar and suprasellar regions, but no case of sphenoidal RCC has been reported. We report a case of sphenoidal RCC in a 41-year-old man. The lesion was revealed by headaches and diplopia. Symptoms disappeared transiently after a spontaneous rhinorrhea but relapsed 4 months later. MR imaging showed a cystic sphenoidal lesion, isointense on T1-weighted images (WI) with peripheral gadolinium enhancement and hyperintense on T2 WI. The patient underwent surgery through a transrhinoseptal approach. The wall of the sphenoid sinus was paper-thin. The cyst contained a motor-oil-like fluid and communicated widely with the nasal fossa. Its wall was partially extracted. Symptoms and signs ceased after surgery. MR imaging performed 1 year later showed the disappearance of the sphenoidal cyst. Embryological origin of RCCs is discussed. The hypothesis of a continuum between the different epithelial cystic lesions of the sellar and parasellar region is discussed. Imaging has an important impact on the diagnosis; nevertheless, the specific characterization remains difficult. PMID:16687551

  5. First Branchial Cleft Fistula Associated with External Auditory Canal Stenosis and Middle Ear Cholesteatoma

    PubMed Central

    Abdollahi fakhim, Shahin; Naderpoor, Masoud; Mousaviagdas, Mehrnoosh

    2014-01-01

    Introduction: First branchial cleft anomalies manifest with duplication of the external auditory canal. Case Report: This report features a rare case of microtia and congenital middle ear and canal cholesteatoma with first branchial fistula. External auditory canal stenosis was complicated by middle ear and external canal cholesteatoma, but branchial fistula, opening in the zygomatic root and a sinus in the helical root, may explain this feature. A canal wall down mastoidectomy with canaloplasty and wide meatoplasty was performed. The branchial cleft was excised through parotidectomy and facial nerve dissection. Conclusion: It should be considered that canal stenosis in such cases can induce cholesteatoma formation in the auditory canal and middle ear. PMID:25320705

  6. Surgical Approaches to First Branchial Cleft Anomaly Excision: A Case Series.

    PubMed

    Quintanilla-Dieck, Lourdes; Virgin, Frank; Wootten, Chistopher; Goudy, Steven; Penn, Edward

    2016-01-01

    Objectives. First branchial cleft anomalies (BCAs) constitute a rare entity with variable clinical presentations and anatomic findings. Given the high rate of recurrence with incomplete excision, identification of the entire tract during surgical treatment is of paramount importance. The objectives of this paper were to present five anatomic variations of first BCAs and describe the presentation, evaluation, and surgical approach to each one. Methods. A retrospective case review and literature review were performed. We describe patient characteristics, presentation, evaluation, and surgical approach of five patients with first BCAs. Results. Age at definitive surgical treatment ranged from 8 months to 7 years. Various clinical presentations were encountered, some of which were atypical for first BCAs. All had preoperative imaging demonstrating the tract. Four surgical approaches required a superficial parotidectomy with identification of the facial nerve, one of which revealed an aberrant facial nerve. In one case the tract was found to travel into the angle of the mandible, terminating as a mandibular cyst. This required en bloc excision that included the lateral cortex of the mandible. Conclusions. First BCAs have variable presentations. Complete surgical excision can be challenging. Therefore, careful preoperative planning and the recognition of atypical variants during surgery are essential. PMID:27034873

  7. Surgical Approaches to First Branchial Cleft Anomaly Excision: A Case Series

    PubMed Central

    Quintanilla-Dieck, Lourdes; Virgin, Frank; Wootten, Chistopher; Goudy, Steven; Penn, Edward

    2016-01-01

    Objectives. First branchial cleft anomalies (BCAs) constitute a rare entity with variable clinical presentations and anatomic findings. Given the high rate of recurrence with incomplete excision, identification of the entire tract during surgical treatment is of paramount importance. The objectives of this paper were to present five anatomic variations of first BCAs and describe the presentation, evaluation, and surgical approach to each one. Methods. A retrospective case review and literature review were performed. We describe patient characteristics, presentation, evaluation, and surgical approach of five patients with first BCAs. Results. Age at definitive surgical treatment ranged from 8 months to 7 years. Various clinical presentations were encountered, some of which were atypical for first BCAs. All had preoperative imaging demonstrating the tract. Four surgical approaches required a superficial parotidectomy with identification of the facial nerve, one of which revealed an aberrant facial nerve. In one case the tract was found to travel into the angle of the mandible, terminating as a mandibular cyst. This required en bloc excision that included the lateral cortex of the mandible. Conclusions. First BCAs have variable presentations. Complete surgical excision can be challenging. Therefore, careful preoperative planning and the recognition of atypical variants during surgery are essential. PMID:27034873

  8. Thymopharyngeal duct cyst: MR imaging of a third branchial arch anomaly in a neonate.

    PubMed

    Vade, A; Griffiths, A; Hotaling, A; Eisenbeis, J F; Husain, A N

    1994-01-01

    Third branchial arch anomalies are rare. The authors present a case report of a neonate with a rapidly growing neck mass due to cystic dilation of a persistent thymopharyngeal duct, which is a derivative of the third branchial arch. The presence of thyroid and thymic tissue in the cyst wall, the communication of the cyst with the piriform sinus, and the relationship of the cyst to carotid vessels and the sternomastoid muscle were consistent with the features of a thymopharyngeal duct cyst embedded in the thyroid gland. PMID:7949690

  9. A Type-II First Branchial Cleft Anomaly Presenting as a Post-Auricular Salivary Fistula: A Rare Presentation.

    PubMed

    Jain, S; Deshmukh, Pt; Gupta, M; Shukla, S

    2014-01-01

    First branchial cleft anomalies are rare with the average age of presentation as 19 years. There is an average delay of 3.5 years between initial presentation and adequate treatment due to diagnostic dilemma. A very rare variant of first branchial cleft anomaly presenting as a post-auricular salivary fistula is reported. A 12-year-old girl presented with a history of intermittent watery discharge, more so at the time of meals from a right post-auricular opening for last 3 years. Computed tomography sialography revealed a fistulous tract connecting the sub segmental duct of the parotid gland extending along the pre-tragus region in subcutaneous plane up to mastoid tip after passing inferior to external auditory canal. Superficial parotidectomy with identification of facial nerve branches was carried out for excision of the tract. Histopathology revealed sinus tract comprising of ectodermal components and acini of the parotid gland. We classified our case into work's type-2 based on anatomical location at an angle of mandible, its relationship to parotid gland and facial nerve and previous history of ear discharge. To the best of our knowledge, this is the first case of its type to be reported. PMID:24669347

  10. Rathke's cleft cysts: review of natural history and surgical outcomes.

    PubMed

    Han, Seunggu J; Rolston, John D; Jahangiri, Arman; Aghi, Manish K

    2014-04-01

    Rathke's cleft cysts (RCCs), also known as pars intermedia cysts, represent benign lesions formed from remnants of the embryologic Rathke's pouch. Commonly asymptomatic, they are identified in nearly 1 in 6 healthy volunteers undergoing brain imaging. When symptomatic, they can cause headaches, endocrine dysfunction, and, rarely, visual disturbances. A systematic review of the published English literature was performed focusing on large modern case series of RCCs to describe their natural history, clinicopathologic features, radiographic features, and surgical outcomes, including rates of recurrence. The natural history of asymptomatic RCCs is one of slow growth, suggesting that observation through serial magnetic resonance imaging is appropriate for smaller asymptomatic RCCs. Symptomatic RCCs can be treated by surgical resection with low morbidity, usually through an endonasal transsphenoidal corridor using either a microscope or an endoscope. Surgical treatment frequently provides symptomatic relief of headaches and visual disturbances, and sometimes even improves endocrine dysfunction. Rates of recurrence after surgical treatment range from 16 to 18 % in large series, and higher rates of recurrence are associated with suprasellar location, inflammation and reactive squamous metaplasia in the cyst wall, superinfection of the cyst, and use of a fat graft into the cyst cavity. PMID:24146189

  11. The Coexistence of Rathke Cleft Cyst and Pituitary Adenoma.

    PubMed

    Gao, Mingtong; An, Yanyan; Huang, Zhihong; Niu, Jianyi; Yuan, Xunhui; Bai, Yun'an; Guo, Liemei

    2016-03-01

    Both of Pituitary adenoma (PA) and Rathke cleft cyst (RCC) are the most common and benign sellar lesions. Generally, the origin of RCC is considered to be derived from remnants of Rathke punch, while PA is formed by proliferation of the anterior wall of Rathke pouch. Although they have a possibility to share a common embryological origin, the coexistence of PA and RCC is extremely rare. Here, the authors report a 50-year-old male patient who was found to have a large cystic sellar lesion, and surgical resection revealed components of a RCC coexisting with a PA. This collision reminded us of the possibility of RCC coexisting with PA. Furthermore, a clinicopathologic relation of them were reviewed and investigated. PMID:26845092

  12. Unusual case of concomitant occurrence of Tessier's number 7 cleft and dentigerous cyst

    PubMed Central

    Issar, Yuvraj; Kaushal, Nitin; Goomer, Pallvi

    2014-01-01

    Dentigerous cysts are commonly encountered associated with impacted teeth; however the exact histogenesis of these cysts is not known even though numerous theories have been proposed. Majority of the maxillary cysts arise as a result of defect in the embryonal development, which can be either abnormal fusion of facial processes or as a result of abnormality in the development of dental follicle. Congenital Tessier's number 7 unilateral facial cleft is a rare anomaly, which arises as a result of defect in the fusion of facial processes. We report an unusual case of concomitant occurrence of Tesssier's number 7 cleft and maxillary dentigerous cyst in 11-year-old child. PMID:25191083

  13. Transient thyrotoxicosis accompanied by panhypopituitarism caused by ruptured Rathke's cleft cyst.

    PubMed

    Chung, Jin Ook; Cho, Dong Hyeok; Chung, Dong Jin; Chung, Min Young

    2011-01-01

    We describe a rare case of transient thyrotoxicosis secondary to painless thyroiditis accompanied by panhypopituitarism caused by ruptured Rathke's cleft cyst. A 32-year-old man presented with vomiting and diarrhea. Laboratory data showed that he had transient hypercalcemia, primary thyrotoxicosis due to painless thyroiditis and panhypopituitarism. The sellar magnetic resonance imaging showed cystic macroadenoma. He underwent surgical exploration. Histological examination showed a ruptured Rathke's cleft cyst. Our case suggests that, although rare, it is important to recognize the possibility of coexistence of hypopituitarism in patients with primary thyrotoxicosis. PMID:22185992

  14. Endoscopic Endonasal Approach in the Management of Rathke’s Cleft Cysts

    PubMed Central

    Solari, Domenico; Cavallo, Luigi Maria; Somma, Teresa; Chiaramonte, Carmela; Esposito, Felice; Del Basso De Caro, Marialaura; Cappabianca, Paolo

    2015-01-01

    Objective Rathke’s cleft cysts (RCCs) are quite uncommon sellar lesions that can extend or even arise in the suprasellar area. The purpose of this study is to evaluate the effectiveness of both standard and extended endoscopic endonasal approaches in the management of different located RCCs. Methods We retrospectively analyzed a series of 29 patients (9 males, 20 females) complaining of a RCC, who underwent a standard or an extended endoscopic transsphenoidal approach at the Division of Neurosurgery, Department of Neurosciences and Reproductive and Odontostomatological Sciences, of the Università degli Studi di Napoli "Federico II”. Data regarding patients’ demographics, clinical evaluation, cyst characteristics, surgical treatments, complications and outcomes were extracted from our electronic database (Filemaker Pro 11, File Maker Inc., Santa Clara, California, USA). Results A standard transsphenoidal approach was used in 19 cases, while the extended variation of the approach in 10 cases (5 purely suprasellar and 5 intra-suprasellar RCC). Cysts contents was fully drained in all the 29 cases, whilst a gross total removal, that accounts on the complete cyst wall removal, was achieved in an overall 55,1% of patients (16/29), specifically 36,8% (7/19) that received standard approach and 90% (9/10) of those that underwent to extended approach. We reported a 56.2% of recovery from headache, 38.5% of complete recovery and 53.8% of improvement from visual field defect and an overall 46.7% of improvement of the endocrine functions. Postoperative permanent DI rate was 10.3%, overall post-operative CSF leak rate 6.9%; recurrence/regrowth occurred in 4 patients (4/29, 13.8%), but only one required a second surgery. Conclusion The endoscopic transsphenoidal approach for the removal of a symptomatic RCC offers several advantages in terms of visualization of the surgical field during both the exposure and removal of the lesion. The “extended” variation of the

  15. EpCAM (CD326) is differentially expressed in craniopharyngioma subtypes and Rathke's cleft cysts.

    PubMed

    Thimsen, Vivian; Hölsken, Annett; Buchfelder, Michael; Flitsch, Jörg; Fahlbusch, Rudolf; Stefanits, Harald; Losa, Marco; Jones, David T W; Buslei, Rolf

    2016-01-01

    The epithelial cell adhesion molecule (EpCAM) is a type I glycoprotein located on the surface of epithelial cells. It is strongly expressed in many neoplasms and already used in the diagnosis and distinction of various tumour subtypes. Comparative studies about EpCAM expression in cystic sellar lesions are lacking. Therefore, we analysed its distribution pattern in adamantinomatous (aCP) and papillary (pCP) craniopharyngiomas (CP) and Rathke's Cleft Cysts (RCC) using immunohistochemistry and gene expression profiling. Whereas the protein was not detectable in pCP (n = 10), all aCP (n = 64) showed distinct staining patterns. The vast majority of RCC (n = 10) also appeared positive, but these displayed notably lower labeling scores. Additionally, significantly higher mRNA levels were detectable in aCP (n = 19) when compared to pCP (n = 10) (p = 9.985(-8)). Furthermore, pediatric aCP cases, in general, exhibited stronger EpCAM staining levels compared to adult ones (p = 0.015). However, we were not able to verify this result on mRNA level. In summary, our findings demonstrate that EpCAM can be used as an additional distinction-marker for cystic lesions of the sellar region. Its unknown function in aCP and the presence of an approved monoclonal bispecific trifunctional antibody for cancer therapy are interesting starting points for further studies. PMID:27431859

  16. GH-Producing Pituitary Adenoma and Concomitant Rathke's Cleft Cyst: A Case Report and Short Review.

    PubMed

    Tamura, Ryota; Takahashi, Satoshi; Emoto, Katsura; Nagashima, Hideaki; Toda, Masahiro; Yoshida, Kazunari

    2015-01-01

    Concomitant pituitary adenoma (PA) and Rathke's cleft cyst (RCC) are rare. In some cases, such PA is known to produce pituitary hormones. A 53-year-old man was admitted to our hospital with a diagnosis of lacunar infarction in the left basal ganglia. Magnetic resonance imaging (MRI) incidentally showed a suprasellar mass with radiographic features of RCC. When he consulted with a neurosurgical outpatient clinic, acromegaly was suspected based on his appearance. A diagnosis of growth hormone- (GH-) producing PA was confirmed from hormonal examinations and additional MRI. Retrospectively, initial MR images also showed intrasellar mass that is compatible with the diagnosis of PA other than suprasellar RCC. The patient underwent endonasal-endoscopic removal of the PA. Since we judged that the RCC of the patient was asymptomatic, only the PA was completely removed. The postoperative course of the patient was uneventful and GH levels gradually normalized. Only 40 cases of PA with concomitant RCC have been reported to date, including 13 cases of GH-producing PA. In those 13 cases, RCC tended to be located in the sella turcica, and suprasellar RCC like this case appears rare. In a few cases, concomitant RCCs were fenestrated, but GH levels normalized postoperatively as in the cases without RCC fenestration. If radiographic imaging shows typical RCC, and PA is not obvious at first glance, the possibility of concomitant PA still needs to be considered. In terms of treatment, removal of the RCC is not needed to achieve hormone normalization. PMID:25883817

  17. [A case of Rathke's cleft cyst in association with anterior communicating artery aneurysm presenting a rare visual field defect].

    PubMed

    Yuki, K; Katsuzo, K; Ikawa, F; Takeshita, S; Hamasaki, O; Tohru, U

    1996-05-01

    We report a case of Rathke's cleft cyst associated with anterior communicating artery aneurysm. The patient was 60-year-old woman who developed visual disturbance two months before admission to our hospital. Visual acuity on the right was 0.06 and on the left was 0.08. The visual fields showed a complete temporal hemianopsia on the left eye and an incomplete temporal hemianopsia of the right eye with a central defect of the temporal visual field. CT and MR imagings showed an intra- and suprasellar mass lesion with no enhancement. Angiography showed bilateral A1 elevations and anterior communicating artery aneurysm. The operation was performed through interhemispheric approach. Suprasellar cystic mass compressed upward the optic nerves and chiasm, and aneurysmal dome stuck in the central region of chiasm. This anatomical disorders affected to the optic chiasm resulted in a rare visual field defect. Neck clipping of aneurysm and opening of the cyst were performed. A diagnosis of Rathke's cleft cyst was made. Following surgery, her visual fields resolved but she suffered from diabetes insipidus. PMID:8672308

  18. Branchial Anomalies: Diagnosis and Management

    PubMed Central

    Azeez, Arun; Thada, Nikhil Dinaker; Rao, Pallavi; Prasad, Kishore Chandra

    2014-01-01

    Objective. To find out the incidence of involvement of individual arches, anatomical types of lesions, the age and sex incidence, the site and side of predilection, the common clinical features, the common investigations, treatment, and complications of the different anomalies. Setting. Academic Department of Otolaryngology, Head and Neck Surgery. Design. A 10 year retrospective study. Participants. 30 patients with clinically proven branchial anomalies including patients with bilateral disease totaling 34 lesions. Main Outcome Measures. The demographical data, clinical features, type of branchial anomalies, and the management details were recorded and analyzed. Results and Observations. The mean age of presentation was 18.67 years. Male to female sex ratio was 1.27 : 1 with a male preponderance. Of the 34 lesions, maximum incidence was of second arch anomalies (50%) followed by first arch. We had two cases each of third and fourth arch anomalies. Only 1 (3.3%) patients of the 30 presented with lesion at birth. The most common pathological type of lesions was fistula (58.82%) followed by cyst. 41.18% of the lesions occurred on the right side. All the patients underwent surgical excision. None of our patients had involvement of facial nerve in first branchial anomaly. All patients had tracts going superficial to the facial nerve. Conclusion. Confirming the extent of the tract is mandatory before any surgery as these lesions pass in relation to some of the most vital structures of the neck. Surgery should always be the treatment option. injection of dye, microscopic removal and inclusion of surrounding tissue while excising the tract leads to a decreased incidence of recurrence. PMID:24772172

  19. Visual Deterioration and Herniation of the Anterior Cerebral Artery: Unusual Presentation of an Empty Sella Syndrome Complicating Decompression of a Rathke Cleft Cyst.

    PubMed

    Sivaraju, Laxminadh; Thakar, Sumit; Hegde, Alangar S

    2016-06-01

    Clinical manifestations of empty sella syndrome include hypopituitarism cerebrospinal fluid rhinorrhea, headache, and visual abnormalities. A 21-year-old woman reported a 6-month history of worsening vision 3 years after decompression of a sellar-suprasellar Rathke cleft cyst. Her magnetic resonance imaging (MRI) showed a well-defined recurrent cyst in the sellar-suprasellar region causing chiasmatic compression. She underwent an endonasal, endoscopic decompression of the cyst, with subsequent improvement in her vision. A postoperative computed tomography confirmed good decompression of the cyst. Ten days after surgery, she reported sudden loss of vision in both eyes. MRI revealed an empty sella with herniation of both anterior cerebral arteries and optic chiasm into the sella. She underwent transnasal packing of the sellar floor with fat graft and bone plaques, and experienced gradual improvement in vision in her right eye. PMID:26828843

  20. EpCAM (CD326) is differentially expressed in craniopharyngioma subtypes and Rathke’s cleft cysts

    PubMed Central

    Thimsen, Vivian; Hölsken, Annett; Buchfelder, Michael; Flitsch, Jörg; Fahlbusch, Rudolf; Stefanits, Harald; Losa, Marco; Jones, David T. W.; Buslei, Rolf

    2016-01-01

    The epithelial cell adhesion molecule (EpCAM) is a type I glycoprotein located on the surface of epithelial cells. It is strongly expressed in many neoplasms and already used in the diagnosis and distinction of various tumour subtypes. Comparative studies about EpCAM expression in cystic sellar lesions are lacking. Therefore, we analysed its distribution pattern in adamantinomatous (aCP) and papillary (pCP) craniopharyngiomas (CP) and Rathke’s Cleft Cysts (RCC) using immunohistochemistry and gene expression profiling. Whereas the protein was not detectable in pCP (n = 10), all aCP (n = 64) showed distinct staining patterns. The vast majority of RCC (n = 10) also appeared positive, but these displayed notably lower labeling scores. Additionally, significantly higher mRNA levels were detectable in aCP (n = 19) when compared to pCP (n = 10) (p = 9.985−8). Furthermore, pediatric aCP cases, in general, exhibited stronger EpCAM staining levels compared to adult ones (p = 0.015). However, we were not able to verify this result on mRNA level. In summary, our findings demonstrate that EpCAM can be used as an additional distinction-marker for cystic lesions of the sellar region. Its unknown function in aCP and the presence of an approved monoclonal bispecific trifunctional antibody for cancer therapy are interesting starting points for further studies. PMID:27431859

  1. The Intraprofessional Continuum and Cleft.

    PubMed

    Jensen, Clyde B

    2016-08-01

    The continuum cleft is a costly and precarious gap that divides professions on the health professions' continuum. It is an interprofessional phenomenon that is encouraged because health care professions protect their members in professional silos and isolate competing professions in professional cysts. This article uses case studies of the allopathic, osteopathic, naturopathic, and chiropractic professions to contemplate the existence, consequences, and possible mitigation of intraprofessional silos, cysts, and clefts. PMID:27574493

  2. Cleft Lip and Cleft Palate

    MedlinePlus

    ... Age Support Resources Books for Kids and Adults Cleft Lip/Palate & Craniofacial Specialists in Your Area FAQs for ... Conference: For Patients and Families Glossary of Terms Cleft lip and cleft palate comprise the most common birth ...

  3. A Case Report: A Third/Fourth Branchial Pouch Anomaly Presented by Solid Thyroid and Lateral Cervical Neck Masses.

    PubMed

    Nasreldin, Magda H A; Ibrahim, Eman A; Saad El-Din, Somaia A

    2016-01-01

    Branchial pouch-derived anomalies may arise from remnants of the first, second, or third/fourth branchial arches. Branchial pouch-related structures are found within the thyroid gland in the form of solid cell rests, epithelial lined cyst with or without an associated lymphoid component, thymic and/or parathyroid tissue, and less commonly in the form of heterotopic cartilage. We present a rare case of left solid thyroid swelling nearby two cervical nodules in a seven-year-old female with a clinical diagnosis suggestive of malignant thyroid tumor with metastasis to the cervical lymph nodes. Histopathological examination revealed that it was compatible with third/fourth branchial pouch-derived anomaly composed of mature cartilage and thymic and parathyroid tissues for clinical and radiological correlations. PMID:26819565

  4. Ectopic congenital bronchogenic cyst accompanied by infection appearing in the cervical region of an elderly female patient: A case report

    PubMed Central

    LIU, ZHONGLONG; TIAN, ZHUOWEI; ZHANG, CHENPING; HE, YUE

    2016-01-01

    Bronchogenic cysts (BCs) are rare congenital cystic lesions arising as a development malformation in the trachea-bronchial system during the embryonic period. The cysts mostly occur in the mediastinum, with an extremely low morbidity rate in the oromaxillofacial-head and neck region. The age distribution of patients who present with BCs shows a significant predominance towards young individuals compared with the elderly. The current study reports the case a 70-year-old female who presented with a painful mass in the right cervical region. Pre-operative computed tomography (CT) and ultrasound showed a heterogeneous hypodense cystic lesion accompanied by infection. A mass of gaseous density was observed on repeat CT following antibiotic therapy. The pathological examination after complete resection revealed a BC, which can also be easily misdiagnosed as common congenital cervical cysts, such as a branchial cleft cyst or a thyroglossal duct cyst, among others. There was no recurrence or any discomfort during the 34-month follow-up period. PMID:26893692

  5. Congenital midline cervical cleft.

    PubMed

    Agag, Richard; Sacks, Justin; Silver, Lester

    2007-01-01

    Congenital midline cervical cleft (CMCC) is a rare disorder of the ventral neck that is clinically evident at birth and must be differentiated from the more common thyroglossal duct cyst. The case of CMCC presented here was associated with chromosomes 13/14 de novo Robertsonian translocations as well as midline deformities including a sacral tuft and a minor tongue-tie. The case is presented as well as discussion of histopathology, embryology, and surgical treatment. PMID:17214531

  6. Cleft Lip and Palate

    MedlinePlus

    ... Got Homework? Here's Help White House Lunch Recipes Cleft Lip and Cleft Palate KidsHealth > For Kids > Cleft Lip ... to the back of your mouth. What's a Cleft Lip or Cleft Palate? The word cleft means a ...

  7. Distribution of branchial anomalies in a paediatric Asian population

    PubMed Central

    Teo, Neville Wei Yang; Ibrahim, Shahrul Izham; Tan, Kun Kiaang Henry

    2015-01-01

    INTRODUCTION The objective of the present study was to review the distribution and incidence of branchial anomalies in an Asian paediatric population and highlight the challenges involved in the diagnosis of branchial anomalies. METHODS This was a retrospective chart review of all paediatric patients who underwent surgery for branchial anomalies in a tertiary paediatric hospital from August 2007 to November 2012. The clinical notes were correlated with preoperative radiological investigations, intraoperative findings and histology results. Branchial anomalies were classified based on the results of the review. RESULTS A total of 28 children underwent surgery for 30 branchial anomalies during the review period. Two children had bilateral branchial anomalies requiring excision. Of the 30 branchial anomalies, 7 (23.3%) were first branchial anomalies, 5 (16.7%) were second branchial anomalies, 3 (10.0%) were third branchial anomalies, and 4 (13.3%) were fourth branchial anomalies (one of the four patients with fourth branchial anomalies had bilateral branchial anomalies). In addition, seven children had 8 (26.7%) branchial anomalies that were thought to originate from the pyriform sinus; however, we were unable to determine if these anomalies were from the third or fourth branchial arches. There was inadequate information on the remaining 3 (10.0%) branchial anomalies for classification. CONCLUSION The incidence of second branchial anomalies appears to be lower in our Asian paediatric population, while that of third and fourth branchial anomalies was higher. Knowledge of embryology and the related anatomy of the branchial apparatus is crucial in the identification of the type of branchial anomaly. PMID:25917471

  8. Cleft Lip and Cleft Palate

    MedlinePlus

    ... refers to a cleft in the lip only accounting for 20 percent of all clefts. What causes ... permission. Oral Cavity Get Involved Professional Development Practice Management ENT Careers Marketplace Privacy Policy Terms of Use ...

  9. Cleft rhinoplasty

    PubMed Central

    Baskaran, M.; Packiaraj, I.; Arularasan, S. Gidean; Divakar, T. K.

    2015-01-01

    It is universally accepted that correction of cleft lip nose deformity remains a formidable challenge for any cleft surgeon. The nose is a prominent part of the face, and hence a masterly executed cleft lip repair directs the beholders’ eyes from the deformed lip to the deformed nose. A deformed nose that results from unilateral cleft of the lip and palate is likened to a tent whose one side is depressed. Many investigators believe that the deformity of the nose is produced by the malpositioning of essentially normal structures, on the other hand some cleft surgeons contend that it is the intrinsic defects in nasal structures that result in cleft nasal deformity. Depressed and hypoplastic bony scaffolding is the most important aspect of cleft nose deformity and addressing this aspect of cleft nose deformity is the secret of success of a perfect secondary rhinoplasty. Controversy still exists on timing of cleft nasal deformity. Proponents of delayed nasal repair suggest that altering the cartilages in early nasal repair at the time of lip repair would complicate future corrective nasal surgeries if the primary repair would prove unsatisfactory. The correction of nasal deformity could be performed with closed or open technique. This paper highlights one such challenging unilateral cleft lip nasal deformity in a adult patient treated by secondary rhinoplasty by open technique. PMID:26538945

  10. Cleft rhinoplasty.

    PubMed

    Baskaran, M; Packiaraj, I; Arularasan, S Gidean; Divakar, T K

    2015-08-01

    It is universally accepted that correction of cleft lip nose deformity remains a formidable challenge for any cleft surgeon. The nose is a prominent part of the face, and hence a masterly executed cleft lip repair directs the beholders' eyes from the deformed lip to the deformed nose. A deformed nose that results from unilateral cleft of the lip and palate is likened to a tent whose one side is depressed. Many investigators believe that the deformity of the nose is produced by the malpositioning of essentially normal structures, on the other hand some cleft surgeons contend that it is the intrinsic defects in nasal structures that result in cleft nasal deformity. Depressed and hypoplastic bony scaffolding is the most important aspect of cleft nose deformity and addressing this aspect of cleft nose deformity is the secret of success of a perfect secondary rhinoplasty. Controversy still exists on timing of cleft nasal deformity. Proponents of delayed nasal repair suggest that altering the cartilages in early nasal repair at the time of lip repair would complicate future corrective nasal surgeries if the primary repair would prove unsatisfactory. The correction of nasal deformity could be performed with closed or open technique. This paper highlights one such challenging unilateral cleft lip nasal deformity in a adult patient treated by secondary rhinoplasty by open technique. PMID:26538945

  11. Submucous Clefts

    MedlinePlus

    ... properly and the individual is at risk for speech problems, middle ear disease, and swallowing difficulties. However, ... for a submucous cleft palate is abnormal nasal speech. Other symptoms may include persistent middle ear disease ...

  12. Cleft lip and palate

    MedlinePlus

    Cleft palate; Craniofacial defect ... way to the base of the nose. A cleft palate can be on 1 or both sides of ... nose, and palate confirms a cleft lip or cleft palate. Medical tests may be done to rule out ...

  13. Cleft Lip and Cleft Palate

    MedlinePlus

    ... virus and pregnancy Microcephaly Medicine safety and pregnancy Birth defects prevention Learn how to help reduce your risk ... of all races about the same. Can other birth defects happen along with oral clefts? Yes. There are ...

  14. Ovarian cysts

    MedlinePlus

    Physiologic ovarian cysts; Functional ovarian cysts; Corpus luteum cysts; Follicular cysts ... cyst often contains a small amount of blood. Ovarian cysts are more common in the childbearing years between ...

  15. Lymphoepithelial cyst of the submandibular gland

    PubMed Central

    Ahamed, A. Saneem; Kannan, V. Sadesh; Velaven, K.; Sathyanarayanan, G. R.; Roshni, J.; Elavarasi, E.

    2014-01-01

    Lymphoepithelial cysts are benign, slowly growing unilocular or multilocular lesions that appear in the head and neck. They are also called Branchial cyst. The head and neck sites are the salivary glands(more commonly parotid and rarely submandibular gland) and the oral cavity (usually the floor of the mouth). there are various methods of investigation available today, of which Fine needle aspiration cytology (FNAC) can be used to provide an immediate diagnosis of a lymphoepithelial cyst. The other investigations include, Ultrasonogram,and Computed tomography. It usually occurs due to the process of lymphocyte-induced cystic ductular dilatation and the confirmatory diagnosis is always made postoperatively by histopathological examination. The mainstay in the treatment of a lymphoepithelial cyst remains the surgical approach, which includes complete enucleation of the cyst along with total excision of the involved salivary gland. This is a report of a lymphoepithelial cyst involving the submandibular salivary gland and its management. PMID:25210369

  16. Laryngotracheoesophageal clefts.

    PubMed

    Strychowsky, Julie E; Rahbar, Reza

    2016-06-01

    Laryngotracheoesophageal clefts are rare congenital anomalies of the aerodigestive tract. Patients may present with airway and/or swallowing impairments. An approach to evaluation and management is presented. Important pearls for conservative and surgical management are discussed. Open versus endoscopic surgical techniques are reviewed. PMID:27301597

  17. 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 ...

  18. 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.com/professionals/14332_1210.asp ...

  19. Ovarian Cysts

    MedlinePlus

    f AQ FREQUENTLY ASKED QUESTIONS FAQ075 GYNECOLOGIC PROBLEMS Ovarian Cysts • What is an ovarian cyst? • What are the symptoms of ovarian cysts? • How are ovarian cysts diagnosed? • How are ovarian ...

  20. Cleft lip and palate repair

    MedlinePlus

    Orofacial cleft; Craniofacial birth defect repair; Cheiloplasty; Cleft rhinoplasty; Palatoplasty; Tip rhinoplasty ... A cleft lip is a birth defect: A cleft lip may be just a small notch in the lip. It may also be a complete split in the ...

  1. Cleft Lip and Palate

    MedlinePlus

    ... ear infections, hearing loss, and problems with their teeth. Often, surgery can close the lip and palate. Cleft lip surgery is usually done before age 12 months, and cleft palate surgery is done ...

  2. Cleft Palate Foundation

    MedlinePlus

    ... for a list of 2016 funded research projects. Cleft Palate Foundation 1504 East Franklin Street, Suite 102 Chapel ... to order bottles Order CPF publications © Copyright 2014 Cleft Palate Foundation. Website by Mixer Creative Follow us on ...

  3. Cleft lip and palate

    MedlinePlus

    Cleft lip and palate are birth defects that affect the upper lip and the roof of the mouth. ... There are many causes of cleft lip and palate. Problems with genes ... viruses, or other toxins can all cause these birth defects. ...

  4. Ovarian Cysts

    MedlinePlus

    ... new cysts. A health problem that may involve ovarian cysts is polycystic ovary syndrome (PCOS). Women with ... male hormones, irregular or no periods and small ovarian cysts. Dept. of Health and Human Services Office ...

  5. BAKER'S CYST

    PubMed Central

    Demange, Marco Kawamura

    2015-01-01

    Baker's cysts are located in the posteromedial region of the knee between the medial belly of the gastrocnemius muscle and semimembranosus tendon. In adults, these cysts are related to intra-articular lesions, which may consist of meniscal lesions or arthrosis. In children, these cysts are usually found on physical examination or imaging studies, and they generally do not have any clinical relevance. Ultrasound examination is appropriate for identifying and measuring the popliteal cyst. The main treatment approach should focus on the joint lesions, and in most cases there is no need to address the cyst directly. Although almost all knee cysts are benign (Baker's cysts and parameniscal cysts), presence of some signs makes it necessary to suspect malignancy: symptoms disproportionate to the size of the cyst, absence of joint damage (e.g. meniscal tears) that might explain the existence of the cyst, unusual cyst topography, bone erosion, cyst size greater than 5 cm and tissue invasion (joint capsule). PMID:27027065

  6. Ovarian cysts

    MedlinePlus

    ... cysts due to hormone-related conditions such as polycystic ovary syndrome . Symptoms Ovarian cysts often cause no symptoms. An ... You may need other treatments if you have polycystic ovary syndrome or another disorder that can cause cysts. Outlook ( ...

  7. BRANCHIAL ELIMINATION OF SUPERHYDROPHOBIC ORGANIC COMPOUNDS BY RAINBOW TROUT (ONCORHYNCHUS MYKISS)

    EPA Science Inventory

    The branchial elimination of pentachloroethane and four congeneric polychlorinated bephenyls by rainbow trout was measured using a fish respirometer-metabolism chamber and an adsorption resin column. Branchial elimination was characterized by calculating a set of apparent in vivo...

  8. First Branchial Arch Fistula: A Rarity and a Surgical Challenge

    PubMed Central

    Rajkumar, J.S.; Anirudh, J.R.; Akbar, S.; Joshi, Niraj

    2016-01-01

    Although 2nd Branchial arch fistulae (from incomplete closure of Cervical sinus of His) are well known, 1st arch fistulae are much rarer (<10%) and are usually not tackled comprehensively. We present a case of a rare first branchial arch fistula of the type II Arnot classification, which presented with two external openings of more than 20 years duration. Patient had a successful resection of all the concerned fistulous tract. Review of literature and the surgical challenges of the procedure are presented herewith.

  9. 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. PMID:26906186

  10. Arachnoid Cysts

    MedlinePlus

    ... Awards Enhancing Diversity Find People About NINDS NINDS Arachnoid Cysts Information Page Synonym(s): Intracranial Cysts Table of Contents ( ... Clinical Trials Organizations Publicaciones en Español What are Arachnoid Cysts? Arachnoid cysts are cerebrospinal fluid-filled sacs that ...

  11. 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;…

  12. Physiological and molecular ontogeny of branchial and extra-branchial urea excretion in posthatch rainbow trout (Oncorhynchus mykiss).

    PubMed

    Zimmer, Alex M; Wood, Chris M

    2016-02-01

    All teleost fish produce ammonia as a metabolic waste product. In embryos, ammonia excretion is limited by the chorion, and fish must detoxify ammonia by synthesizing urea via the ornithine urea cycle (OUC). Although urea is produced by embryos and larvae, urea excretion (J(urea)) is typically low until yolk sac absorption, increasing thereafter. The aim of this study was to determine the physiological and molecular characteristics of J(urea) by posthatch rainbow trout (Oncorhynchus mykiss). Following hatch, whole body urea concentration decreased over time, while J(urea) increased following yolk sac absorption. From 12 to 40 days posthatch (dph), extra-branchial routes of excretion accounted for the majority of J(urea), while the gills became the dominant site for J(urea) only after 55 dph. This represents the most delayed branchial ontogeny of any process studied to date. Urea transporter (UT) gene expression in the gills and skin increased over development, consistent with increases in branchial and extra-branchial J(urea). Following exposure to 25 mmol/l urea, the accumulation and subsequent elimination of exogenous urea was much greater at 55 dph than 12 dph, consistent with increased UT expression. Notably, UT gene expression in the gills of 55 dph larvae increased in response to high urea. In summary, there is a clear increase in urea transport capacity over posthatch development, despite a decrease in OUC activity. PMID:26608657

  13. Cleft Lip and Palate Surgery

    MedlinePlus

    ... The experts in face, mouth and jaw surgery. Cleft Lip / Palate and Craniofacial Surgery This type of surgery ... to correct a physical defect caused by a cleft lip or cleft palate, which occur once in every ...

  14. Cleft Lip Nose.

    PubMed

    Sykes, Jonathan M; Tasman, Abel-Jan; Suárez, Gustavo A

    2016-01-01

    All patients with a cleft lip deformity have an associated nasal deformity that varies in degree of severity. A three-dimensional understanding of the anatomy of the cleft nose aids surgeons in selecting the proper technique for repair. Analysis and performance of orthognathic surgery should be done before nasal surgery to optimize the overall result. Goals of the secondary rhinoplasty include relief of nasal obstruction, creation of symmetry and definition of the nasal base and tip, and management of nasal scarring and webbing. Septal reconstruction in the cleft nose is a key maneuver in cleft rhinoplasty. PMID:26616710

  15. Renal Cysts

    MedlinePlus

    ... kidneys. They are usually characterized as “simple” cysts, meaning they have a thin wall and contain water- ... of the time, they are simple kidney cysts, meaning they have a thin wall and only water- ...

  16. Kidney Cysts

    MedlinePlus

    ... are two types of kidney cysts. Polycystic kidney disease (PKD) runs in families. In PKD, the cysts ... failure, dialysis or kidney transplants. Acquired cystic kidney disease (ACKD) usually happens in people who are on ...

  17. Vaginal cysts

    MedlinePlus

    ... or is protruding from the vagina. It is important to contact your provider for an exam for any cyst or mass you notice. Alternative Names Inclusion cyst; Gartner duct cyst Images Female reproductive anatomy Uterus Normal uterine anatomy (cut section) References Baggish ...

  18. Myxoid Cyst

    MedlinePlus

    ... question & answer discussion forum widgets for professionals dermatology education rash and rashes clinical tools ... These cysts form in the middle aged and the elderly. Overview A myxoid cyst, also known as a digital mucous cyst or pseudocyst, is a growth usually ...

  19. Epidermoid Cyst

    MedlinePlus

    ... epidermis) grows into the middle layer of the skin (dermis). This may occur due to injury or blocked hair follicles. The lesion may be ... the cyst. However, this is a temporary measure. After this treatment, a cyst will refill with the cheesy contents because the lining of the cyst has not been removed. ... Jean L., ed. Dermatology , pp. ...

  20. Cleft lip repair - series (image)

    MedlinePlus

    ... in the middle of the upper lip. A cleft palate is an opening in the roof of the ... Cleft lip repair and cleft palate repair are indicated for: Repair of physical deformity Nursing, feeding, or speech problems resulting from cleft lip or palate

  1. Cleft-orthognathic surgery.

    PubMed

    Posnick, Jeffrey C; Ricalde, Pat

    2004-04-01

    For the cleft patient presenting in adolescence with a jaw discrepancy and malocclusion, misinformation and limited available surgical and dental expertise often prevents a favorable facial reconstruction and dental rehabilitation. A major advantage of the modified Le Fort I osteotomy is its ability to simultaneously close cleft dental gap(s), resolve oronasal fistulas, manage skeletal defects, stabilize dentoalveolar segments, and correct jaw deformities. When a thoughtful staging of reconstruction is undertaken, individuals born with cleft lip and palate can reach adolescence after undergoing only a limited number of operations and interventions, without negative attention being drawn to their original malformation. PMID:15145672

  2. Molecular contribution to cleft palate production in cleft lip mice

    PubMed Central

    Sasaki, Yasunori; Taya, Yuji; Saito, Kan; Fujita, Kazuya; Aoba, Takaaki; Fujiwara, Taku

    2014-01-01

    Cleft palate following cleft lip may include a developmental disorder during palatogenesis. CL/Fr mice fetuses, which develop cleft lip and palate spontaneously, have less capability for in vivo cell proliferation in palatal mesenchyme compared with CL/Fr normal fetuses. In order to know the changes of signaling molecules contributing to cleft palate morphogenesis following cleft lip, the mRNA expression profiles were compared in palatal shelves oriented vertically (before elevation) in CL/Fr fetuses with or without cleft lip. The changes in mRNA profile of cleft palate morphogenesis were presented in a microarray analysis, and genes were restricted to lists contributing to cleft palate development in CL/Fr fetuses with cleft lip. Four candidate genes (Ywhab, Nek2, Tacc1 and Frk) were linked in a gene network that associates with cell proliferation (cell cycle, MAPK, Wnt and Tgf beta pathways). Quantitative real-time RT-PCR highlighted the candidate genes that significantly changed in CL/Fr fetuses with cleft lip (Ywhab, Nek2 and Tacc1). The results of these molecular contributions will provide useful information for a better understanding of palatogenesis in cleft palate following cleft lip. Our data indicated the genetic contribution to cleft palate morphogenesis following cleft lip. PMID:24206222

  3. Molecular contribution to cleft palate production in cleft lip mice.

    PubMed

    Sasaki, Yasunori; Taya, Yuji; Saito, Kan; Fujita, Kazuya; Aoba, Takaaki; Fujiwara, Taku

    2014-05-01

    Cleft palate following cleft lip may include a developmental disorder during palatogenesis. CL/Fr mice fetuses, which develop cleft lip and palate spontaneously, have less capability for in vivo cell proliferation in palatal mesenchyme compared with CL/Fr normal fetuses. In order to know the changes of signaling molecules contributing to cleft palate morphogenesis following cleft lip, the mRNA expression profiles were compared in palatal shelves oriented vertically (before elevation) in CL/Fr fetuses with or without cleft lip. The changes in mRNA profile of cleft palate morphogenesis were presented in a microarray analysis, and genes were restricted to lists contributing to cleft palate development in CL/Fr fetuses with cleft lip. Four candidate genes (Ywhab, Nek2, Tacc1 and Frk) were linked in a gene network that associates with cell proliferation (cell cycle, MAPK, Wnt and Tgf beta pathways). Quantitative real-time RT-PCR highlighted the candidate genes that significantly changed in CL/Fr fetuses with cleft lip (Ywhab, Nek2 and Tacc1). The results of these molecular contributions will provide useful information for a better understanding of palatogenesis in cleft palate following cleft lip. Our data indicated the genetic contribution to cleft palate morphogenesis following cleft lip. PMID:24206222

  4. Urethroid cyst.

    PubMed

    Paslin, D

    1983-01-01

    Cysts found on the ventral surface of the penis have been pathogenetically ascribed to defective embryologic closure of the median raphe, to anomalous developmental rests of the periurethral glands of Littre, and to the ectopic development of apocrine cystadenoma in the penile skin. This case reports a previously unrecognized but compelling pathogenesis for at least some ventral penile cysts, namely the anomalous congenital outgrowth of the entodermal urethral lining, giving rise to what is here designated as a urethroid cyst. PMID:6849573

  5. Management of Midline Facial Clefts.

    PubMed

    Mishra, Sobhan; Sabhlok, Samrat; Panda, Pankaj Kumar; Khatri, Isha

    2015-12-01

    Median or midline facial clefts are rare anomalies of developmental origin, etiology of whose occurrence is still unknown precisely. The most basic presentation of midline facial clefts is in the form of a Median cleft lip which is defined as any congenital vertical cleft through the centre of the upper lip. First described by Bechard in 1823, it is the most common amongst all atypical clefts reported. The incidence is about 1:10,00,000 births. This may occur as a sporadic event or as a part of an inherited sequence of anomalies. It arises embryologically from incomplete fusion of the medial nasal prominences. The authors present a series of eight cases with varying degrees of midline facial clefts. This review article aims to give a broad idea on the various classifications used for further understanding of midline facial clefts and a brief idea about the various surgical management techniques used in the repair of these facial clefts. PMID:26604459

  6. The canonical Wnt signaling activator, R-spondin2, regulates craniofacial patterning and morphogenesis within the branchial arch through ectodermal-mesenchymal interaction

    PubMed Central

    Jin, Yong-Ri; Turcotte, Taryn J.; Crocker, Alison L.; Han, Xiang Hua; Yoon, Jeong Kyo

    2011-01-01

    R-spondins are a recently characterized family of secreted proteins that activate Wnt/β-catenin signaling. Herein, we determine R-spondin2 (Rspo2) function in craniofacial development in mice. Mice lacking a functional Rspo2 gene exhibit craniofacial abnormalities such as mandibular hypoplasia, maxillary and mandibular skeletal deformation, and cleft palate. We found that loss of the mouse Rspo2 gene significantly disrupted Wnt/β-catenin signaling and gene expression within the first branchial arch (BA1). Rspo2, which is normally expressed in BA1 mesenchymal cells, regulates gene expression through a unique ectoderm-mesenchyme interaction loop. The Rspo2 protein, potentially in combination with ectoderm-derived Wnt ligands, up-regulates Msx1 and Msx2 expression within mesenchymal cells. In contrast, Rspo2 regulates expression of the Dlx5, Dlx6, and Hand2 genes in mesenchymal cells via inducing expression of their upstream activator, Endothelin1 (Edn1), within ectodermal cells. Loss of Rspo2 also causes increased cell apoptosis, especially within the aboral (or caudal) domain of the BA1, resulting in hypoplasia of the BA1. Severely reduced expression of Fgf8, a survival factor for mesenchymal cells, in the ectoderm of Rspo2−/− embryos is likely responsible for increased cell apoptosis. Additionally, we found that cleft palate in Rspo2−/− mice is not associated with defects intrinsic to the palatal shelves. A possible cause of cleft palate is a delay of proper palatal shelf elevation that may result from the small mandible and a failure of lowering the tongue. Thus, our study identifies Rspo2 as a mesenchyme-derived factor that plays critical roles in regulating BA1 patterning and morphogenesis through ectodermal-mesenchymal interaction and a novel genetic factor for cleft palate. PMID:21237142

  7. Dissection and Flat-mounting of the Threespine Stickleback Branchial Skeleton.

    PubMed

    Ellis, Nicholas A; Miller, Craig T

    2016-01-01

    The posterior pharyngeal segments of the vertebrate head give rise to the branchial skeleton, the primary site of food processing in fish. The morphology of the fish branchial skeleton is matched to a species' diet. Threespine stickleback fish (Gasterosteus aculeatus) have emerged as a model system to study the genetic and developmental basis of evolved differences in a variety of traits. Marine populations of sticklebacks have repeatedly colonized countless new freshwater lakes and creeks. Adaptation to the new diet in these freshwater environments likely underlies a series of craniofacial changes that have evolved repeatedly in independently derived freshwater populations. These include three major patterning changes to the branchial skeleton: reductions in the number and length of gill raker bones, increases in pharyngeal tooth number, and increased branchial bone lengths. Here we describe a detailed protocol to dissect and flat-mount the internal branchial skeleton in threespine stickleback fish. Dissection of the entire three-dimensional branchial skeleton and mounting it flat into a largely two-dimensional prep allows for the easy visualization and quantification of branchial skeleton morphology. This dissection method is inexpensive, fast, relatively easy, and applicable to a wide variety of fish species. In sticklebacks, this efficient method allows the quantification of skeletal morphology in genetic crosses to map genomic regions controlling craniofacial patterning. PMID:27213248

  8. The branchial arches and HGF are growth-promoting and chemoattractant for cranial motor axons.

    PubMed

    Caton, A; Hacker, A; Naeem, A; Livet, J; Maina, F; Bladt, F; Klein, R; Birchmeier, C; Guthrie, S

    2000-04-01

    During development, cranial motor neurons extend their axons along distinct pathways into the periphery. For example, branchiomotor axons extend dorsally to leave the hindbrain via large dorsal exit points. They then grow in association with sensory ganglia, to their targets, the muscles of the branchial arches. We have investigated the possibility that pathway tissues might secrete diffusible chemorepellents or chemoattractants that guide cranial motor axons, using co-cultures in collagen gels. We found that explants of dorsal neural tube or hindbrain roof plate chemorepelled cranial motor axons, while explants of cranial sensory ganglia were weakly chemoattractive. Explants of branchial arch mesenchyme were strongly growth-promoting and chemoattractive for cranial motor axons. Enhanced and oriented axon outgrowth was also elicited by beads loaded with Hepatocyte Growth Factor (HGF); antibodies to this protein largely blocked the outgrowth and orientation effects of the branchial arch on motor axons. HGF was expressed in the branchial arches, whilst Met, which encodes an HGF receptor, was expressed by subpopulations of cranial motor neurons. Mice with targetted disruptions of HGF or Met showed defects in the navigation of hypoglossal motor axons into the branchial region. Branchial arch tissue may thus act as a target-derived factor that guides motor axons during development. This influence is likely to be mediated partly by Hepatocyte Growth Factor, although a component of branchial arch-mediated growth promotion and chemoattraction was not blocked by anti-HGF antibodies. PMID:10725250

  9. Tarlov Cysts

    MedlinePlus

    ... the herpes simplex virus, which thrives in an alkaline environment, can cause Tarlov cysts to become symptomatic. Making the body less alkaline, through diet or supplements, may lessen symptoms. Microsurgical ...

  10. Kidney Cysts

    MedlinePlus

    ... fluid-filled sac. There are two types of kidney cysts. Polycystic kidney disease (PKD) runs in families. In PKD, the ... place of the normal tissue. They enlarge the kidneys and make them work poorly, leading to kidney ...

  11. Ovarian Cysts

    MedlinePlus

    ... information Endometriosis fact sheet Ovarian cancer fact sheet Polycystic ovary syndrome fact sheet The javascript used in this widget ... ovaries make many small cysts. This is called polycystic ovary syndrome (PCOS). PCOS can cause problems with the ovaries ...

  12. Congenital Midline Cervical Cleft

    PubMed Central

    Villanueva-Meyer, Javier; Glastonbury, Christine; Marcovici, Peter

    2015-01-01

    Congenital midline cervical cleft is a rare anomaly that typically presents in the neonatal period as a thin suprasternal vertical band of erythematous skin with a nipple-like projection superiorly, which may exude fluid. We present the clinical and pathophysiologic features and the imaging findings of this uncommon, and rarely described entity in a newborn girl. PMID:25926928

  13. Closing the cleft over a throbbing heart: neonatal sternal cleft

    PubMed Central

    J, Ashok Raja; G, Mathevan; K, Mathiarasan; P, Ramasubramaniam

    2014-01-01

    Sternal cleft is a rare anomaly comprising 0.5% of chest wall malformations. We present a case of a neonate with a ‘V’-shaped upper partial sternal cleft at birth. A hyperpigmented cutaneous nevi was present over the cleft. Primary approximation and closure of the defect was performed at 1 week of life. We discuss the presentation and management, and review the literature. PMID:25100810

  14. Choledochal cysts

    PubMed Central

    Singham, Janakie; Yoshida, Eric M.; Scudamore, Charles H.

    2009-01-01

    Much about the etiology, pathophysiology, natural course and optimal treatment of cystic disease of the biliary tree remains under debate. Gastroenterologists, surgeons and radiologists alike still strive to optimize their roles in the management of choledochal cysts. To that end, much has been written about this disease entity, and the purpose of this 3-part review is to organize the available literature and present the various theories currently argued by the experts. In part 1, we discuss the background of the disease, describing the etiology, classification, pathogenesis and malignant potential of choledochal cysts. PMID:19865581

  15. Clefting in pumpkin balloons

    NASA Astrophysics Data System (ADS)

    Baginski, F.; Schur, W.

    NASA's effort to develop a large payload, high altitude, long duration balloon, the Ultra Long Duration Balloon, focuses on a pumpkin shape super-pressure design. It has been observed that a pumpkin balloon may be unable to pressurize into the desired cyclically symmetric equilibrium configuration, settling into a distorted, undesired stable state instead. Hoop stress considerations in the pumpkin design leads to choosing the lowest possible bulge radius, while robust deployment is favored by a large bulge radius. Some qualitative understanding of design aspects on undesired equilibria in pumpkin balloons has been obtained via small-scale balloon testing. Poorly deploying balloons have clefts, but most gores away from the cleft deploy uniformly. In this paper, we present models for pumpkin balloons with clefts. Long term success of the pumpkin balloon for NASA requires a thorough understanding of the phenomenon of multiple stable equilibria and means for quantitative assessment of measures that prevent their occurrence. This paper attempts to determine numerical thresholds of design parameters that distinguish between properly deploying designs and improperly deploying designs by analytically investigating designs in the vicinity of criticality. Design elements which may trigger the onset undesired equilibria and remedial measures that ensure deployment are discussed.

  16. Genetic Factors and Orofacial Clefting

    PubMed Central

    Lidral, Andrew C.; Moreno, Lina M.; Bullard, Steven A.

    2008-01-01

    Cleft lip with or without cleft palate is the most common facial birth defect and it is caused by a complex interaction between genetic and environmental factors. The purpose of this review is to provide an overview of the spectrum of the genetic causes for cleft lip and cleft palate using both syndromic and nonsyndromic forms of clefting as examples. Although the gene identification process for orofacial clefting in humans is in the early stages, the pace is rapidly accelerating. Recently, several genes have been identified that have a combined role in up to 20% of all clefts. While this is a significant step forward, it is apparent that additional cleft causing genes have yet to be identified. Ongoing human genome-wide linkage studies have identified regions in the genome that likely contain genes that when mutated cause orofacial clefting, including a major gene on chromosome 9 that is positive in multiple racial groups. Currently, efforts are focused to identify which genes are mutated in these regions. In addition, parallel studies are also evaluating genes involved in environmental pathways. Furthermore, statistical geneticists are developing new methods to characterize both gene-gene and gene-environment interactions to build better models for pathogenesis of this common birth defect. The ultimate goal of these studies is to provide knowledge for more accurate risk counseling and the development of preventive therapies. PMID:19492008

  17. Biliary cysts.

    PubMed Central

    Flanigan, P D

    1975-01-01

    This review brings the total number of biliary cysts reported in the world literature to 955. Eighty-one per cent of patients are females and 61% were discovered before age ten. The classical triad of right upper quadrant pain, right upper quandrant mass, and juandice is present in 38% of cases. The duration of symptoms prior to diagnosis ranged from less than one week to more than 40 years. The etiology is multifaceted and evidence of the existence of both acquired and congenital cysts is presented. The most useful diagnostic tool is fiberoptic endoscopy with retrograde contrast injection of the common bile duct and pancreatic duct. The incidence of biliary carcinoma in patients with biliary cysts is found to be 2.5%; 24 cases have been reported. Considerable controversy has existed concerning the best operative procedure for biliary cysts; no treatment or medical treatment yielding a 97% mortality rate. In an analysis of 235 patients presented since 1968 with an average followup of 5.2 years, the best procedure appears to be excision with either choledochocholedocostomy or Roux-en-Y hepaticojejunostomy. The operative mortality for all procedures is now 3 to 4%. PMID:1103760

  18. Lip Prints and Inheritance of Cleft Lip and Cleft Palate

    PubMed Central

    CJ, Manasa Ravath; HC, Girish; Hegde, Ramesh B; JK, Savita

    2014-01-01

    Background: Labial mucosa has elevations and depressions forming a pattern called ‘Lip Prints’. Parents of patients with cleft lip &/or palate are known to have a particular lip print pattern. Objectives: Analysis of lip prints and relationship between Cheiloscopy and inheritance of cleft lip &/or cleft palate. Methodology: The study included 100 subjects [study groupparents with children having cleft lip &/or cleft palate, 50 fathers and 50 mothers) and 50 subjects (control group-parents having children without cleft lip &/or cleft palate, 25 fathers and 25 mothers. The lip prints of the subjects were obtained using the cellophane method and analysed using Suzuki & Tsuchihashi classification of lip prints. The data was subjected to Chi- Square test, Fisher Exact test and Student t-test [two tailed, independent]. Results: A new whorl pattern was present in the study group. The groove count was higher in the fathers’ than in the mothers’ prints in the upper lip and vice versa in the lower lip. Conclusion: The new pattern was present in the study group in a significant number of cases. The groove count was significantly high in the study group. These two parameters can be of significant value to similar future studies. PMID:25177633

  19. 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…

  20. Molecular basis of cleft palates in mice

    PubMed Central

    Funato, Noriko; Nakamura, Masataka; Yanagisawa, Hiromi

    2015-01-01

    Cleft palate, including complete or incomplete cleft palates, soft palate clefts, and submucosal cleft palates, is the most frequent congenital craniofacial anomaly in humans. Multifactorial conditions, including genetic and environmental factors, induce the formation of cleft palates. The process of palatogenesis is temporospatially regulated by transcription factors, growth factors, extracellular matrix proteins, and membranous molecules; a single ablation of these molecules can result in a cleft palate in vivo. Studies on knockout mice were reviewed in order to identify genetic errors that lead to cleft palates. In this review, we systematically describe these mutant mice and discuss the molecular mechanisms of palatogenesis. PMID:26322171

  1. Management of the alveolar cleft.

    PubMed

    Santiago, Pedro E; Schuster, Lindsay A; Levy-Bercowski, Daniel

    2014-04-01

    Orthopedic and orthodontic management of patients born with clefts of the lip, alveolus and palate is based on the application of basic biomechanical principles adapted to the individualized cleft anatomy. This article focuses on orthopedic and orthodontic preparation for 2 stages of interdisciplinary orthodontic/surgical cleft care: presurgical infant orthopedics (nasoalveolar molding) for lip/alveolus/nasal surgical repair and maxillary arch preparation for secondary alveolar bone grafting. These preparatory stages of orthopedic/orthodontic therapy are undertaken with the goal of restoring normal anatomic relationships to assist the surgeon in providing the best possible surgical care. PMID:24607190

  2. Reassessing the Dlx code: the genetic regulation of branchial arch skeletal pattern and development

    PubMed Central

    Depew, Michael J; Simpson, Carol A; Morasso, Maria; Rubenstein, John LR

    2005-01-01

    The branchial arches are meristic vertebrate structures, being metameric both between each other within the rostrocaudal series along the ventrocephalic surface of the embryonic head and within each individual arch: thus, just as each branchial arch must acquire a unique identity along the rostrocaudal axis, each structure within the proximodistal axis of an arch must also acquire a unique identity. It is believed that regional specification of metameric structures is controlled by the nested expression of related genes resulting in a regional code, a principal that is though to be demonstrated by the regulation of rostrocaudal axis development in animals exerted by the nested HOM-C/Hox homeobox genes. The nested expression pattern of the Dlx genes within the murine branchial arch ectomesenchyme has more recently led to the proposal of a Dlx code for the regional specification along the proximodistal axis of the branchial arches (i.e. it establishes intra-arch identity). This review re-examines this hypothesis, and presents new work on an allelic series of Dlx loss-of-function mouse mutants that includes various combinations of Dlx1, Dlx2, Dlx3, Dlx5 and Dlx6. Although we confirm fundamental aspects of the hypothesis, we further report a number of novel findings. First, contrary to initial reports, Dlx1, Dlx2 and Dlx1/2 heterozygotes exhibit alterations of branchial arch structures and Dlx2−/− and Dlx1/2−/− mutants have slight alterations of structures derived from the distal portions of their branchial arches. Second, we present evidence for a role for murine Dlx3 in the development of the branchial arches. Third, analysis of compound Dlx mutants reveals four grades of mandibular arch transformations and that the genetic interactions of cis first-order (e.g. Dlx5 and Dlx6), trans second-order (e.g. Dlx5 and Dlx2) and trans third-order paralogues (e.g. Dlx5 and Dlx1) result in significant and distinct morphological differences in mandibular arch development

  3. Neonate with VACTERL Association and a Branchial Arch Anomaly without Hydrocephalus

    PubMed Central

    Velazquez, Danitza; Pereira, Elaine; Havranek, Thomas

    2015-01-01

    VACTERL (vertebral anomalies, anal atresia, cardiac defect, tracheoesophageal fistula, renal anomaly, limb anomalies) is an association of anomalies with a wide spectrum of phenotypic expression. While the majority of cases are sporadic, there is evidence of an inherited component in a small number of patients as well as the potential influence of nongenetic risk factors (maternal diabetes mellitus). Presence of hydrocephalus has been reported in VACTERL patients (VACTERL-H) in the past, with some displaying branchial arch anomalies. We report the unique case of an infant of diabetic mother with VACTERL association and a branchial arch anomaly—in the absence of hydrocephalus. PMID:26929876

  4. Neonate with VACTERL Association and a Branchial Arch Anomaly without Hydrocephalus.

    PubMed

    Velazquez, Danitza; Pereira, Elaine; Havranek, Thomas

    2016-03-01

    VACTERL (vertebral anomalies, anal atresia, cardiac defect, tracheoesophageal fistula, renal anomaly, limb anomalies) is an association of anomalies with a wide spectrum of phenotypic expression. While the majority of cases are sporadic, there is evidence of an inherited component in a small number of patients as well as the potential influence of nongenetic risk factors (maternal diabetes mellitus). Presence of hydrocephalus has been reported in VACTERL patients (VACTERL-H) in the past, with some displaying branchial arch anomalies. We report the unique case of an infant of diabetic mother with VACTERL association and a branchial arch anomaly-in the absence of hydrocephalus. PMID:26929876

  5. IRF6 Sequencing in Interrupted Clefting.

    PubMed

    Cuddapah, Sanmati R; Kominek, Selma; Grant, John H; Robin, Nathaniel H

    2016-05-01

    In a retrospective review of patients seen at the University of Alabama at Birmingham Cleft and Craniofacial Center, four patients with rare interrupted clefting were identified who had undergone genetic testing. Each of these patients had a typical cleft lip, with intact hard palate and cleft of the soft palate. Given this picture of mixed clefting, IRF6 sequencing was done and was negative for mutations in all four patients. As genetic testing for single-gene mutations and exome sequencing become clinically available, it may be possible to identify novel mutations responsible for this previously unreported type of interrupted clefting. PMID:26090788

  6. PCB126 Exposure Disrupts ZebraFish Ventricular and Branchial but Not Early Neural Crest Development

    PubMed Central

    Grimes, Adrian C.; Erwin, Kyle N.; Stadt, Harriett A.; Hunter, Ginger L.; Gefroh, Holly A.; Tsai, Huai-Jen; Kirby, Margaret L.

    2008-01-01

    We have used zebrafish and 3,3′,4,4′,5-pentachlorobiphenyl (PCB126) to investigate the developmental toxicity of polychlorinated biphenyls (PCBs) that exert their effects through the aryl hydrocarbon receptor (AHR). We found that cardiac and neural crest (NC)–derived jaw and branchial cartilages are specifically targeted early in development. The suite of malformations, which ultimately leads to circulatory failure, includes a severely dysmorphic heart with a reduced bulbus arteriosus and abnormal atrioventricular and outflow valve formation. Early NC migration and patterning of the jaw and branchial cartilages was normal. However, the jaw and branchial cartilages failed to grow to normal size. In the heart, the ventricular myocardium showed a reduction in cell number and size. The heart and jaw/branchial phenotype could be rescued by pifithrin-α, a blocker of p53. However, the function of pifithrin-α in this model may act as a competitive inhibitor of PCB at the AHR and is likely independent of p53. Morpholinos against p53 did not rescue the phenotype, nor were zebrafish with a mutant p53-null allele resistant to PCB126 toxicity. Morpholino knockdown of cardiac troponin T, which blocks the onset of cardiac function, prevented the PCB126-induced cardiac dysmorphogenesis but not the jaw/branchial phenotype. The cardiovascular characteristics appear to be similar to hypoplastic left heart syndrome (HLHS) and introduce the potential of zebrafish as a model to study this environmentally induced cardiovascular malformation. HLHS is a severe congenital cardiovascular malformation that has previously been linked to industrial releases of dioxins and PCBs. PMID:18660518

  7. Comparison of the structure and composition of the branchial filters in suspension feeding elasmobranchs.

    PubMed

    Misty Paig-Tran, E W; Summers, A P

    2014-04-01

    The four, evolutionarily independent, lineages of suspension feeding elasmobranchs have two types of branchial filters. The first is a robust, flattened filter pad akin to a colander (e.g., whale sharks, mantas and devil rays) while the second more closely resembles the comb-like gill raker structure found in bony fishes (e.g., basking and megamouth sharks). The structure and the presence of mucus on the filter elements will determine the mechanical function of the filter and subsequent particle transport. Using histology and scanning electron microscopy, we investigated the anatomy of the branchial filters in 12 of the 14 species of Chondrichthyian filter-feeding fishes. We hypothesized that mucus producing cells would be abundant along the filter epithelium and perform as a sticky mechanism to retain and transport particles; however, we found that only three species had mucus producing goblet cells. Two of these (Mobula kuhlii and Mobula tarapacana) also had branchial cilia, indicating sticky retention and transport. The remaining filter-feeding elasmobranchs did not have a sticky surface along the filter for particles to collect and instead must employ alternative mechanisms of filtration (e.g., direct sieving, inertial impaction or cross-flow). With the exception of basking sharks, the branchial filter is composed of a hyaline cartilage skeleton surrounded by a layer of highly organized connective tissue that may function as a support. Megamouth sharks and most of the mobulid rays have denticles along the surface of the filter, presumably to protect against damage from large particle impactions. Basking sharks have branchial filters that lack a cartilaginous core; instead they are composed entirely of smooth keratin. PMID:24443216

  8. The hyal and ventral branchial muscles in caecilian and salamander larvae: homologies and evolution.

    PubMed

    Kleinteich, Thomas; Haas, Alexander

    2011-05-01

    Amphibians (Lissamphibia) are characterized by a bi-phasic life-cycle that comprises an aquatic larval stage and metamorphosis to the adult. The ancestral aquatic feeding behavior of amphibian larvae is suction feeding. The negative pressure that is needed for ingestion of prey is created by depression of the hyobranchial apparatus as a result of hyobranchial muscle action. Understanding the homologies of hyobranchial muscles in amphibian larvae is a crucial step in understanding the evolution of this important character complex. However, the literature mostly focuses on the adult musculature and terms used for hyal and ventral branchial muscles in different amphibians often do not reflect homologies across lissamphibian orders. Here we describe the hyal and ventral branchial musculature in larvae of caecilians (Gymnophiona) and salamanders (Caudata), including juveniles of two permanently aquatic salamander species. Based on previous alternative terminology schemes, we propose a terminology for the hyal and ventral branchial muscles that reflects the homologies of muscles and that is suited for studies on hyobranchial muscle evolution in amphibians. We present a discussion of the hyal and ventral branchial muscles in larvae of the most recent common ancestor of amphibians (i.e. the ground plan of Lissamphibia). Based on our terminology, the hyal and ventral branchial musculature of caecilians and salamanders comprises the following muscles: m. depressor mandibulae, m. depressor mandibulae posterior, m. hyomandibularis, m. branchiohyoideus externus, m. interhyoideus, m. interhyoideus posterior, m. subarcualis rectus I, m. subarcualis obliquus II, m. subarcualis obliquus III, m. subarcualis rectus II-IV, and m. transversus ventralis IV. Except for the m. branchiohyoideus externus, all muscles considered herein can be assigned to the ground plan of the Lissamphibia with certainty. The m. branchiohyoideus externus is either apomorphic for the Batrachia (frogs

  9. 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…

  10. Understanding Cleft and Craniofacial Team Care

    MedlinePlus

    ... Donor Spotlight Fundraising Ideas Vehicle Donation Volunteer Efforts Cleft Lip/Palate & Craniofacial Specialists in Your Area skip to submenu Parents & Individuals Cleft Lip/Palate & Craniofacial Specialists in Your Area Team Disclaimer ...

  11. [Rare location of arachnoid cysts. Extratemporal cysts].

    PubMed

    Martinez-Perez, Rafael; Hinojosa, José; Pascual, Beatriz; Panaderos, Teresa; Welter, Diego; Muñoz, María J

    2016-01-01

    The therapeutic management of arachnoid cysts depends largely on its location. Almost 50% of arachnoid cysts are located in the temporal fossa-Sylvian fissure, whereas the other half is distributed in different locations, sometimes exceptional. Under the name of infrequent location arachnoid cysts, a description is presented of those composed of 2 sheets of arachnoid membrane, which are not located in the temporal fossa, and are primary or congenital. PMID:26725189

  12. Cleft lip: The historical perspective.

    PubMed

    Bhattacharya, S; Khanna, V; Kohli, R

    2009-10-01

    The earliest documented history of cleft lip is based on a combination of religion, superstition, invention and charlatanism. While Greeks ignored their existence, Spartans and Romans would kill these children as they were considered to harbour evil spirits. When saner senses prevailed Fabricius ab Aquapendente (1537-1619) was the first to suggest the embryological basis of these clefts. The knowledge of cleft lip and the surgical correction received a big boost during the period between the Renaissance and the 19th century with the publication of Pierre Franco's Petit Traite and Traite des Hernies in which he described the condition as "lievre fendu de nativite" (cleft lip present from birth). The first documented Cleft lip surgery is from China in 390 BC in an 18 year old would be soldier, Wey Young-Chi. Albucasis of Arabia and his fellow surgeons used the cautery instead of the scalpel and Yperman in 1854 recommended scarifying the margins with a scalpel before suturing them with a triangular needle dipped in wax. The repair was reinforced by passing a long needle through the two sides of the lip and fixing the shaft of the needle with a figure-of-eight thread over the lip. Germanicus Mirault can be credited to be the originator of the triangular flap which was later modified by C.W. Tennison in 1952 and Peter Randall in 1959. In the late 50s, Ralph Millard gave us his legendary 'cut as you go' technique. The protruding premaxilla of a bilateral cleft lip too has seen many changes throughout the ages - from being discarded totally to being pushed back by wedge resection of vomer to finally being left to the orthodontists. PMID:19884680

  13. Oral cleft prevention program (OCPP)

    PubMed Central

    2012-01-01

    Background Oral clefts are one of the most common birth defects with significant medical, psychosocial, and economic ramifications. Oral clefts have a complex etiology with genetic and environmental risk factors. There are suggestive results for decreased risks of cleft occurrence and recurrence with folic acid supplements taken at preconception and during pregnancy with a stronger evidence for higher than lower doses in preventing recurrence. Yet previous studies have suffered from considerable design limitations particularly non-randomization into treatment. There is also well-documented effectiveness for folic acid in preventing neural tube defect occurrence at 0.4 mg and recurrence with 4 mg. Given the substantial burden of clefting on the individual and the family and the supportive data for the effectiveness of folic acid supplementation as well as its low cost, a randomized clinical trial of the effectiveness of high versus low dose folic acid for prevention of cleft recurrence is warranted. Methods/design This study will assess the effect of 4 mg and 0.4 mg doses of folic acid, taken on a daily basis during preconception and up to 3 months of pregnancy by women who are at risk of having a child with nonsyndromic cleft lip with/without palate (NSCL/P), on the recurrence of NSCL/P. The total sample will include about 6,000 women (that either have NSCL/P or that have at least one child with NSCL/P) randomly assigned to the 4 mg and the 0.4 mg folic acid study groups. The study will also compare the recurrence rates of NSCL/P in the total sample of subjects, as well as the two study groups (4mg, 0.4 mg) to that of a historical control group. The study has been approved by IRBs (ethics committees) of all involved sites. Results will be disseminated through publications and presentations at scientific meetings. Discussion The costs related to oral clefts are high, including long term psychological and socio-economic effects. This study provides an opportunity for

  14. How to Feed Cleft Patient?

    PubMed Central

    Khan, Saima Yunus

    2013-01-01

    ABSTRACT Cleft lip and palate patients have all rights like other normal individuals, to enjoy the benefits of nourishment. Knowledge has to be there about the different feeding positions like straddle, dancer hand position along with the use of specially designed bottles and nipples. Parent's should be trained about the correct positions of feeding, in extreme of the cases in which parents are not able to follow these instructions, feeding obturators can be given. How to cite this article: Jindal MK, Khan SY. How to Feed Cleft Patient? Int J Clin Pediatr Dent 2013;6(2):100-103. PMID:25206201

  15. Dermoid Cysts of the Floor of the Mouth: Two Case Reports

    PubMed Central

    Makos, Christos; Noussios, George; Peios, Marinos; Gougousis, Spyridon; Chouridis, Pantelis

    2011-01-01

    Dermoid cysts in the floor of the mouth may be congenital or acquired. The congenital form, according to the main theory, originates from embryonic cells of the 1st and 2nd branchial arch. The acquired form may be due to traumatic or iatrogenic causes and as a result of the occlusion of a sebaceous gland duct. Its occurrence is less and is estimated to be from 1.6 to 6.4% of the dermoid cysts of the body in adults and 0.29% of the head and neck tumors occurring in children. They may also be classified as anatomical and histological. Anatomically, they are divided into median genioglossal, median geniohyoid, and lateral cysts, while histologically they are divided into epidermoid, dermoid cysts and teratomas. Clinically, a distinction between supra and inferior type as well as between central and lateral type is proposed in relation to themylohyoidmuscle and themidline, respectively. Histologically, an estimation of dermoid, epidermoid, and teratoid cysts is reported. Enucleation via intraoral and/or extraoral approach is the method of treatment. Two case reports of dermoid cysts in the floor of the mouth are presented in this paper, and an evaluation with regard to pathology, clinical findings, differential diagnosis, and treatment is discussed. PMID:21922020

  16. Extradural Giant Multiloculated Arachnoid Cyst Causing Spinal Cord Compression in a Child

    PubMed Central

    Kahraman, Serdar; Anik, Ihsan; Gocmen, Selcuk; Sirin, Sait

    2008-01-01

    Background: Spinal extradural arachnoid cysts are rare expanding lesions in the spinal canal. Enlargement may cause progressive signs and symptoms caused by spinal cord compression. They are associated with trauma, surgery, arachnoiditis, and neural tube defects. Most nontraumatic spinal extradural arachnoid cysts are thought to be congenital. Design: Case report and literature review. Findings: A 9-year-old boy with mild paraparesis was found to have an extradural multiloculated arachnoid cyst with fibrous septa at T4-L3 levels and anterior compression and displacement of the spinal cord. Conclusions: Definitive treatment of arachnoid cyst entails radical cyst removal and dura cleft repair. Formation of a postoperative cerebrospinal fluid fistula may require external lumbar drainage. PMID:18795482

  17. Baker’s cyst

    MedlinePlus

    Popliteal cyst; Bulge-knee ... Baker's cyst is caused by swelling in the knee. The swelling is due to an increase in the fluid that lubricates the knee joint (synovial fluid). When pressure builds up, fluid ...

  18. Branchial effects of epinephrine in the seawater-adapted mullet. II. Na+ and Cl- extrusion.

    PubMed

    Pic, P; Mayer-Gostan, N; Maetz, J

    1975-02-01

    Injection of epinephrine into Mugil capito adapted to seawater is followed by a 40-60% inhibition of the Na and Cl effluxes. Simultaneously the Na influx is decreased by 30%, the overall result being a reduction of the net sodium extrusion rate by the gill. The change in Na influx is in part explained by a 75-80% decrease of the oral ingestion of seawater. This branchial adrenergic response is sensitive to alpha-blockade by phentolamine and tolazoline and insensitive to beta-blockade by propranolol. Both alpha-blockers are ineffective when injected alone. Propranolol injected alone mimics epinephrine while simultaneous injection of phentolamine blocks the response to propranolol. Rapid transfer experiments suggest that epinephrine inhibits the branchial Cl pump and its associated Na/K exchange mechanism. The leak pathway for these ions remains insensitive to epinephrine. PMID:1119569

  19. Simple Kidney Cysts

    MedlinePlus

    ... Organizations​​ . (PDF, 345 KB)​​​​​ Alternate Language URL Simple Kidney Cysts Page Content On this page: What are simple ... Points to Remember Clinical Trials What are simple kidney cysts? Simple kidney cysts are abnormal, fluid-filled sacs ...

  20. Beware the Tarlov cyst.

    PubMed

    Hirst, Jane E; Torode, Hugh; Sears, William; Cousins, Michael J

    2009-01-01

    Tarlov cysts are sacral perineural cysts. This case report describes the clinical course after biopsy of a very large Tarlov cyst via laparoscopy, which was thought preoperatively to be an adnexal mass. It serves as a warning against attempting biopsy or resection of these lesions. PMID:19110185

  1. Simulating clefts in pumpkin balloons

    NASA Astrophysics Data System (ADS)

    Baginski, Frank; Brakke, Kenneth

    2010-02-01

    The geometry of a large axisymmetric balloon with positive differential pressure, such as a sphere, leads to very high film stresses. These stresses can be significantly reduced by using a tendon re-enforced lobed pumpkin-like shape. A number of schemes have been proposed to achieve a cyclically symmetric pumpkin shape, including the constant bulge angle (CBA) design, the constant bulge radius (CBR) design, CBA/CBR hybrids, and NASA’s recent constant stress (CS) design. Utilizing a hybrid CBA/CBR pumpkin design, Flight 555-NT in June 2006 formed an S-cleft and was unable to fully deploy. In order to better understand the S-cleft phenomenon, a series of inflation tests involving four 27-m diameter 200-gore pumpkin balloons were conducted in 2007. One of the test vehicles was a 1/3-scale mockup of the Flight 555-NT balloon. Using an inflation procedure intended to mimic ascent, the 1/3-scale mockup developed an S-cleft feature strikingly similar to the one observed in Flight 555-NT. Our analysis of the 1/3-scale mockup found it to be unstable. We compute asymmetric equilibrium configurations of this balloon, including shapes with an S-cleft feature.

  2. Orthokeratinised odontogenic cyst mimicking periapical cyst

    PubMed Central

    Rajalakshmi, R; Sreeja, C; Vijayalakshmi, D; Leelarani, V

    2013-01-01

    Orthokeratinised odontogenic cyst (OOC) denotes the odontogenic cyst that microscopically has an orthokeratinised epithelial lining. OOC is characterised by a less-aggressive behaviour and a low rate of recurrence. This report describes a case of OOC involving posterior part of the mandible that mimicked periapical cyst in a 14-year-old boy. The initial clinical diagnosis was given as periapical cyst based on the clinical and radiographical features. Enucleation of the cyst was performed and the specimen was sent for histopathological examination. A definite diagnosis of OOC was made by histopathological examination of the biopsy specimen. This case emphases on including OOC in the differential diagnosis of radiolucencies occurring in the periapical region of non-vital tooth. PMID:24099763

  3. Clinical Approach to a Suspected Case of First Branchial Arch Syndrome

    PubMed Central

    Nakamura, Shiho; Nishigaki, Soichiro; Yanagita, Ken-ichi; Nonaka, Kazuaki

    2014-01-01

    First branchial arch syndrome is a congenital disorder characterized by a wide spectrum of anomalies in the first branchial arch, mainly affecting the lower jaw, ear, or mouth, during early embryonic development. We sought to confirm a suspected case of this syndrome by making differential diagnosis and taking an intensive clinical approach. A 12-year-6-month-old girl with a horizontally impacted left canine in the maxilla had the history of digital fusion in her hands and feet and has been suffering from hearing impairment of her left ear. To diagnose this case and make her careful treatment plan, we further carried out cephalometric analysis and mutation analysis. Her face looks like asymmetry and is not apparently symmetric by cephalometric analysis. Mutation analysis of the patient was conducted by direct DNA sequencing of the goosecoid gene, which is an excellent candidate for determination of hemifacial microsomia, but no changes in this gene were identified. We could not precisely diagnose this case as first branchial arch syndrome. However, certain observations in this case, including hearing impairment of the left ear, allow us to suspect this syndrome. PMID:24523735

  4. Semimembranosus ganglion cyst

    PubMed Central

    Kannadath, Bijun Sai; Soundamourthy, Sandosh; Subramanian, Aruna; Sinhasan, Sankappa P.; Bhat, Ramachandra V.

    2014-01-01

    Ganglion cysts are tumor-like lesions in the soft tissues, generated by mucoid degeneration of the joint capsule, tendon or tendon sheaths on the dorsum of hand, wrist and foot. However, an intratendinous origin for a ganglion cyst is extremely rare. During dissection of the popliteal fossa, a cyst of 2.5 cm×2 cm×0.5 cm was observed in the tendon of right semimembranosus, 3.5 cm above the insertion of the muscle. Contrast X-ray revealed the cyst as not communicating with the knee joint or any adjacent bursae. Histopathological examination confirmed the diagnosis of ganglion cyst. PMID:25276481

  5. Imaging alterations due to squamous metaplasia in intracranial neurenteric cysts: A report of two cases.

    PubMed

    Watanabe, Nobuyuki; Akasaki, Yasuharu; Fujigasaki, Junko; Mori, Ryosuke; Aizawa, Daisuke; Ikeuchi, Satoshi; Murayama, Yuichi

    2016-06-01

    Intracranial neurenteric cysts are rare congenital abnormalities with a broad imaging spectrum, and therefore are occasionally mistaken for other common intracranial cysts such as epidermoid and arachnoid cysts. We report two cases of neurenteric cysts in the posterior cranial fossa that were initially mistaken for other types of cysts. They exhibited signal intensity alterations in magnetic resonance imaging with significant volume expansion during their long-term observation. Both cases received surgical treatment because of clinical deterioration. Histologically, the cysts were lined by flattened or cuboidal epithelium, occasionally showing squamous metaplasia. Xanthogranulomatous inflammation and accumulation of cholesterol clefts, dry keratin and proteinaceous substance were observed in the cysts. These findings may indicate that chronic inflammation in neurenteric cysts induces squamous metaplasia, keratinization and high proteinaceous content, and causes MRI signal intensity alterations and volume expansion. We propose that MRI signal intensity alterations in neurenteric cysts may be a warning sign of their volume expansions, and thus require closer follow-up imaging and eventually surgical treatment. PMID:27009777

  6. Baker’s Cyst

    PubMed Central

    Frush, Todd J.; Noyes, Frank R.

    2015-01-01

    Context: Popliteal synovial cysts, also known as Baker’s cysts, are commonly found in association with intra-articular knee disorders, such as osteoarthritis and meniscus tears. Histologically, the cyst walls resemble synovial tissue with fibrosis evident, and there may be chronic nonspecific inflammation present. Osteocartilaginous loose bodies may also be found within the cyst, even if they are not seen in the knee joint. Baker’s cysts can be a source of posterior knee pain that persists despite surgical treatment of the intra-articular lesion, and they are routinely discovered on magnetic resonance imaging scans of the symptomatic knee. Symptoms related to a popliteal cyst origin are infrequent and may be related to size. Evidence Acquisition: A PubMed search was conducted with keywords related to the history, diagnosis, and treatment of Baker’s cysts—namely, Baker’s cyst, popliteal cyst, diagnosis, treatment, formation of popliteal cyst, surgical indications, and complications. Bibliographies from these references were also reviewed to identify related and pertinent literature. Study Design: Clinical review. Level of Evidence: Level 4. Results: Baker’s cysts are commonly found associated with intra-articular knee disorders. Proper diagnosis, examination, and treatment are paramount in alleviating the pain and discomfort associated with Baker’s cysts. Conclusion: A capsular opening to the semimembranosus–medial head gastrocnemius bursa is a commonly found normal anatomic variant. It is thought that this can lead to the formation of a popliteal cyst in the presence of chronic knee effusions as a result of intra-articular pathology. Management of symptomatic popliteal cysts is conservative. The intra-articular pathology should be first addressed by arthroscopy. If surgical excision later becomes necessary, a limited posteromedial approach is often employed. Other treatments, such as arthroscopic debridement and closure of the valvular mechanism

  7. Laryngo-tracheo-oesophageal clefts

    PubMed Central

    2011-01-01

    A laryngo-tracheo-esophageal cleft (LC) is a congenital malformation characterized by an abnormal, posterior, sagittal communication between the larynx and the pharynx, possibly extending downward between the trachea and the esophagus. The estimated annual incidence of LC is 1/10,000 to 1/20,000 live births, accounting for 0.2% to 1.5% of congenital malformations of the larynx. These incidence rates may however be underestimated due to difficulty in diagnosing minor forms and a high mortality rate in severe forms. A slightly higher incidence has been reported in boys than in girls. No specific geographic distribution has been found. Depending on the severity of the malformation, patients may present with stridor, hoarse cry, swallowing difficulties, aspirations, cough, dyspnea and cyanosis through to early respiratory distress. Five types of laryngo-tracheo-esophageal cleft have been described based on the downward extension of the cleft, which typically correlates with the severity of symptoms: Type 0 laryngo-tracheo-esophageal cleft to Type 4 laryngo-tracheo-esophageal cleft. LC is often associated with other congenital abnormalities/anomalies (16% to 68%), mainly involving the gastro-intestinal tract, which include laryngomalacia, tracheo-bronchial dyskinesia, tracheo-bronchomalacia (mostly in types 3 and 4), and gastro-esophageal reflux disease (GERD). The syndromes most frequently associated with an LC are Opitz/BBB syndrome, Pallister Hall syndrome, VACTERL/VATER association, and CHARGE syndrome. Laryngeal clefts result from failure of fusion of the posterior cricoid lamina and abnormal development of the tracheo-esophageal septum. The causes of the embryological developmental anomalies leading to LC are not known but are thought to be multifactorial. LC appears to be mostly sporadic although some familial cases with suspected autosomal dominant transmission have been reported. The age of diagnosis depends mainly on the severity of the clinical symptoms and

  8. Face facts: Genes, environment, and clefts

    SciTech Connect

    Murray, J.C.

    1995-08-01

    Cleft lip and/or palate provides an ideal, albeit complex, model for the study of human developmental anomalies. Clefting disorders show a mix of well-defined syndromic causes (many with single-gene or environmental etiologies) coupled with their more common presentation in the nonsyndromic form. This summary presents some insight into the genetic causes of, etiology of and animal models for cleft lip and/or palate. 79 refs.

  9. A Model Humanitarian Cleft Mission: 312 Cleft Surgeries in 7 Days

    PubMed Central

    Gill, Nauman Ahmad; Ishaq, Irfan; Ganatra, Muhammad Ashraf; Mahmood, Farrakh; Kashif, Muhammad; Alam, Iftikhar; Chen, Philip Kuo-Ting; Lo, Lun-Jou; Laub, Donald Rudolph

    2015-01-01

    Background: There are many countries in the world where patients with cleft lip and palate cannot get access to specialized cleft care units. Cleft missions play an important role in providing surgical care to the areas of the world with limited resources. This article presents a model of cleft missions that can be adopted in many countries where expertise is available but resources are limited. Through proper utilization of local human resource, this type of mission can be a cost-effective and robust way of treating patients with cleft in countries with approximately 52% of the world’s population. Methods: We present a case series of patients of one of our cleft missions carried out in Khairpur, Pakistan, in March 2014 over a period of 7 days. Specific details concerning the organization of mission, gathering of patients, preparation for surgery, and carrying out surgical procedures in a safe and swift manner are presented. Results: A total of 312 patients were operated on in 7 days. There were 145 patients with cleft lip and 167 patients with cleft palate. There were 187 male and 125 female patients with mean age of 7 years. Contemporary operative techniques were utilized to repair different types of cleft lip and palate. Of 167 patients, only 16 developed fistula. Conclusion: A locoregional cleft team can be more effective to care for the patients with cleft in countries where surgical and other expertise can be utilized by proper organization of cleft missions on a national level. PMID:25878924

  10. 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…

  11. Improving Informed Consent for Cleft Palate Repair

    ClinicalTrials.gov

    2015-11-02

    Cleft Palate; Jaw Abnormalities; Maxillofacial Abnormalities; Mouth Abnormalities; Congenital Abnormalities; Jaw Diseases; Musculoskeletal Diseases; Craniofacial Abnormalities; Musculoskeletal Abnormalities; Stomatognathic Diseases; Stomatognathic System Abnormalities

  12. Splenic cysts in children.

    PubMed

    Ho, Y H; Sheih, C P; Horng, S S; Liao, Y J; Lu, W T; Li, Y W; Kao, S P

    1997-01-01

    Splenic cysts were found, incidentally, in eight children during the past nine years (1987-1995) in Taipei Municipal Women's and Children's Hospital. Five of the children were boys and three were girls. The age at diagnosis ranged from 8 to 15 years. Evidence of possible splenic cyst development was found initially by ultrasonography; six patients received further evaluation with computerized tomography (CT); one patient received radionuclide scanning. The cysts ranged from 2 cm to 14 cm in diameter. Four of the patients received surgical treatment (three partial splenectomy and one total splenectomy) because of huge splenic cysts (diameter > 10 cm). Subsequent pathological examination revealed that all cysts had epithelial cell lining in the cyst wall, meaning they were all congenital in origin. The remaining four cases were followed up at the Out-patient Clinic here. All cases had a benign clinical course. PMID:9066189

  13. Periodontal Status Among Patients With Cleft Lip (CL), Cleft Palate (CP) and Cleft Lip, Alveolus and Palate (CLAP) In Chennai, India. A Comparative Study

    PubMed Central

    John, Joseph

    2015-01-01

    Background: Long term health of the stomatognathic system as well as esthetic aspects is the therapeutic goals in patients with oro facial clefts. Aim: The aim of this study was to assess and compare the periodontal status of patients with cleft lip (CL), cleft palate (CP) and cleft lip, alveolus and palate (CLAP) reporting to a hospital in Chennai, India. Materials and Methods: The study group consisted of 80 cleft patients. Subjects were divided into three groups. Group 1: patients with cleft lip (CL), Group 2: subjects with cleft palate (CP) and Group 3: subjects with cleft lip alveolus and palate (CLAP). Community Periodontal Index for Treatment needs CPITN Index was recorded. Results: Among the 80 study subjects, 51 (63.8%) were males and 29 (36.2%) were females. Among the 26 study subjects with cleft lip, 10 (38.5%) had healthy periodontium, 4 (15.4%) had bleeding on probing and 12 (46.1%) had calculus. Mean number of sextants coded for healthy and bleeding was maximum among the subjects with cleft palate. Mean number of sextants coded for calculus was maximum among the subjects with cleft lip alveolus and palate. Prevalence of periodontal disease is high among patients with cleft lip, alveolus and palate (35%) than in Cleft lip (32.5%) and Cleft Palate (32.5%). Conclusion: Gingivitis and Calculus is predominantly high in patients with Cleft Palate and Cleft Lip respectively. PMID:25954706

  14. Intrathoracic extrapulmonary hydatid cysts

    PubMed Central

    Atoini, Fouad; Ouarssani, Aziz; Hachimi, Moulay Ahmed; Aitlhou, Fatima; Rguibi, Mustapha Idrissi; Hommadi, Abdelaziz

    2012-01-01

    Hydatid disease caused by echinococcus granulosus is still a serious problem in both underdeveloped and developing countries. Clinical signs of the disease are not specific. Most patients have a few symptoms when a hydatid cyst is discovered. Symptoms depend on its location, size and complications. Parasite can settle in every organ and tissue in the human body. We report two cases with intrathoracic extrapulmonary hydatid cyst with multiple cysts. Pathophysiology of the mode of dissemination, and surgery are discussed. PMID:23308314

  15. Intravesical hydatid cyst.

    PubMed

    Sallami, Sataa; Nouira, Yassine; Kallel, Yousri; Gargouri, Mourad; Horchani, Ali

    2005-11-01

    A case of intravesical hydatid cyst is reported. The cyst was completely evacuated cystoscopically with intravesical instillation of a scolicidal agent (hydrogen peroxide) to destroy scolices and daughter cysts. The postoperative course was uneventful, and follow-up did not show evidence of recurrence. Because this is the first case, to our knowledge, to be reported, little is known about the nonoperative management of such hydatid localization. A recommendation is made, however, to adopt this minimally invasive procedure. PMID:16286147

  16. Pilonidal cyst resection

    MedlinePlus

    ... abscess; Pilonidal dimple; Pilonidal disease; Pilonidal cyst; Pilonidal sinus ... asked to stop taking aspirin, ibuprofen (Advil, Motrin), naproxen (Aleve, Naprosyn), vitamin E, clopidogrel (Plavix), warfarin (Coumadin), ...

  17. Spinal Extradural Arachnoid Cyst

    PubMed Central

    Choi, Seung Won; Seong, Han Yu

    2013-01-01

    Spinal extradural arachnoid cyst (SEAC) is a rare disease and uncommon cause of compressive myelopathy. The etiology remains still unclear. We experienced 2 cases of SEACs and reviewed the cases and previous literatures. A 59-year-old man complained of both leg radiating pain and paresthesia for 4 years. His MRI showed an extradural cyst from T12 to L3 and we performed cyst fenestration and repaired the dural defect with tailored laminectomy. Another 51-year-old female patient visited our clinical with left buttock pain and paresthesia for 3 years. A large extradural cyst was found at T1-L2 level on MRI and a communication between the cyst and subarachnoid space was illustrated by CT-myelography. We performed cyst fenestration with primary repair of dural defect. Both patients' symptoms gradually subsided and follow up images taken 1-2 months postoperatively showed nearly disappeared cysts. There has been no documented recurrence in these two cases so far. Tailored laminotomy with cyst fenestration can be a safe and effective alternative choice in treating SEACs compared to traditional complete resection of cyst wall with multi-level laminectomy. PMID:24294463

  18. Penile epidermal inclusion cyst.

    PubMed

    Saini, Pradeep; Mansoor, M N; Jalali, Sanjay; Sharma, Abhishek

    2010-07-01

    We report a case of epidermal inclusion cyst of penis in a five-year-old boy, who had presented to the outpatient department of our hospital. Epidermal inclusion cysts are benign lesions that can develop in any part of the body. However, the finding of an epidermal inclusion cyst in the penis is rare. The child was operated and discharged uneventfully. The objective of reporting this case is to highlight the rare possibility of an inclusion cyst arising from penis as a late complication of circumcision. PMID:20589475

  19. Pineal cysts in children.

    PubMed

    Lacroix-Boudhrioua, V; Linglart, A; Ancel, P Y; Falip, C; Bougnères, P F; Adamsbaum, C

    2011-12-01

    OBJECTIVE: To describe the prevalence and characteristics of pineal cysts found on MRI in children. METHODS: This is a retrospective monocentric study of all brain magnetic resonance imaging (MRI) examinations performed under the same technical conditions for checking the idiopathic nature of short stature (ISS group, n = 116) and for the investigation of central precocious puberty (CPP) over a 3-year period (n = 56). Dimensions, wall and septal thickness, number of locules, signal intensity, and the presence of a solid component were analysed. Ten of 19 cysts were re-evaluated (follow-up interval 4-28 months). The prevalence of the pineal cysts was compared between the two groups using χ2 and Fisher's exact tests, and a significance threshold of p < 0.05. RESULTS: The prevalence of cysts was comparable in the two groups, CPP (10.7%) and ISS (11.2%). Cyst characteristics were similar in the two groups and 74% had thin septations. None of the cysts changed on follow-up. None of the children with pineal cysts exhibited neurological signs. CONCLUSION: Benign pineal cysts are a common finding in young children. High-resolution MRI demonstrates that these cysts are often septated. This pattern is a normal variant and does not require follow-up MR imaging or IV contrast media. PMID:22347985

  20. Development of oral and branchial muscles in lancelet larvae of Branchiostoma japonicum.

    PubMed

    Yasui, Kinya; Kaji, Takao; Morov, Arseniy R; Yonemura, Shigenobu

    2014-04-01

    The perforated pharynx has generally been regarded as a shared characteristic of chordates. However, there still remains phylogenetic ambiguity between the cilia-driven system in invertebrate chordates and the muscle-driven system in vertebrates. Giant larvae of the genus Asymmetron were reported to develop an orobranchial musculature similar to that of vertebrates more than 100 years ago. This discovery might represent an evolutionary link for the chordate branchial system, but few investigations of the lancelet orobranchial musculature have been completed since. We studied staged larvae of a Japanese population of Branchiostoma japonicum to characterize the developmental property of the orobranchial musculature. The larval mouth and the unpaired primary gills develop well-organized muscles. These muscles function only as obturators of the openings without antagonistic system. As the larval mouth enlarged posteriorly to the level of the ninth myomere, the oral musculature was fortified accordingly without segmental patterning. In contrast, the iterated branchial muscles coincided with the dorsal myomeric pattern before metamorphosis, but the pharynx was remodeled dynamically irrespective of the myomeric pattern during metamorphosis. The orobranchial musculature disappeared completely during metamorphosis, and adult muscles in the oral hood and velum, as well as on the pterygial coeloms developed independently. The lancelet orobranchial musculature is apparently a larval adaptation to prevent harmful intake. However, vestigial muscles appeared transiently with the secondary gill formation suggest a bilateral ancestral state of muscular gills, and a segmental pattern of developing branchial muscles without neural crest and placodal contributions is suggestive of a precursor of vertebrate branchiomeric pattern. PMID:24301696

  1. Levels of arsenic, cadmium, lead and mercury in the branchial plate and muscle tissue of mobulid rays.

    PubMed

    Ooi, Michelle S M; Townsend, Kathy A; Bennett, Michael B; Richardson, Anthony J; Fernando, Daniel; Villa, Cesar A; Gaus, Caroline

    2015-05-15

    Mobulid rays are targeted in fisheries for their branchial plates, for use in Chinese medicine. Branchial plate and muscle tissue from Mobula japanica were collected from fish markets in Sri Lanka, and muscle tissue biopsies from Manta alfredi in Australia. These were analysed for arsenic, cadmium, lead and mercury and compared to maximum levels (MLs) set by Food Standards Australia and New Zealand (FSANZ), European Commission (EC) and Codex Alimentarius Commission. The estimated intake for a vulnerable human age group was compared to minimal risk levels set by the Agency for Toxic Substances and Disease Registry. The mean inorganic arsenic concentration in M. japanica muscle was equivalent to the FSANZ ML while cadmium exceeded the EC ML. The mean concentration of lead in M. alfredi muscle tissue exceeded EC and Codex MLs. There were significant positive linear correlations between branchial plate and muscle tissue concentrations for arsenic, cadmium and lead. PMID:25792120

  2. Reliability of esthetic ratings of cleft impairment.

    PubMed

    Tobiasen, J M; Hiebert, J M

    1988-07-01

    The decision to seek secondary treatment for facial clefts is often the result of concerns about the esthetic acceptability of appearance. There are no standard techniques to assess cleft impairment for esthetic acceptability. Therefore, it is not possible to evaluate objectively either the need for or the benefits of treatment. If it could be shown that people agree closely on how they rate the esthetic appearance of cleft impairments that vary in severity, then esthetic measures of cleft impairment could be developed with human judgment as the yardstick. The goals of this study were: (1) to examine the reliability with which children express their preferences for cleft impairments that vary in severity, (2) to determine if other facial characteristics influence the reliability of children's preferences for cleft impairments, and (3) to evaluate if age and gender of children influence preferences for cleft impairments. Based on preratings, eight types of photographic slides were created that varied in severity of cleft impairment and global facial attractiveness. A second sample of subjects then rated the slides on the esthetic acceptability of appearance. Children ranked the photographic types consistently. They least preferred the photographic types depicting severe impairment or low facial attractiveness, or both, and most preferred faces with no impairment or moderate attractiveness, or both. There were also developmental effects in that younger children tended to have less consensus in their ratings of appearance than older children. Finally, boys displayed greater consensus than girls. PMID:3168276

  3. Pilonidal sinus cyst of the penis: a rare manifestation of a common disease

    PubMed Central

    Al-Qassim, Zubair; Reddy, Kiran; Khan, Zeb; Reddy, Ippala Sreenath

    2013-01-01

    Pilonidal sinus is a chronic inflammatory condition owing to the subcutaneous trapping of hair. Most commonly it is found in the sacrococcygeal region (natal cleft) but rarely it is found on the penis with very few cases reported in the literature worldwide. We are reporting a case of a pilonidal sinus growing on the distal penile shaft with the sinus opening to the mucosal layer of the foreskin, in a young and fit patient. The cyst was removed with a circumcision and found to contain hair. This was confirmed by histology as a pilonidal sinus cyst. PMID:23697453

  4. International Task Force on Volunteer Cleft Missions.

    PubMed

    Yeow, Vincent K L; Lee, Seng-Teik T; Lambrecht, Thomas J; Barnett, John; Gorney, Mark; Hardjowasito, Widanto; Lemperle, Gottfried; McComb, Harold; Natsume, Nagato; Stranc, Mirek; Wilson, Libby

    2002-01-01

    The International Task Force on Volunteer Cleft Missions was set up to provide a report to be presented at the Eighth International Congress of Cleft Palate and Associated Craniofacial Anomalies on September 12, 1997, in Singapore. The aim of the report was to provide data from a wide range of different international teams performing volunteer cleft missions and, thereafter, based on the collected data, to identify common goals and aims of such missions. Thirteen different groups actively participating in volunteer cleft missions worldwide were selected from the International Confederation of Plastic and Reconstructive Surgery's list of teams actively participating in volunteer cleft missions. Because of the time frame within which the committee had to work, three groups that did not respond by the stipulated deadline were omitted from the committee. The represented members and their respective institutions have undertaken more than 50 volunteer cleft missions to underdeveloped nations worldwide within the last 3 years. They have visited over 20 different countries, treating more than 3,500 patients worldwide. Based on the data collected and by consensus, the committee outlined recommendations for future volunteer cleft missions based on 1) mission objectives, 2) organization, 3) personal health and liability, 4) funding, 5) trainees in volunteer cleft missions, and 6) public relations. The task force believed that all volunteer cleft missions should have well-defined objectives, preferably with long-term plans. The task force also decided that it was impossible to achieve a successful mission without good organization and close coordination. All efforts should be made, and care taken, to ensure that there is minimal morbidity and no mortality. Finally, as ambassadors of goodwill and humanitarian aid, the participants must make every effort to understand and respect local customs and protocol. The main aims are to provide top-quality surgical service, train local

  5. Ambulatory cleft lip surgery: A value analysis

    PubMed Central

    Arneja, Jugpal S; Mitton, Craig

    2013-01-01

    BACKGROUND: Socialized health systems face fiscal constraints due to a limited supply of resources and few reliable ways to control patient demand. Some form of prioritization must occur as to what services to offer and which programs to fund. A data-driven approach to decision making that incorporates outcomes, including safety and quality, in the setting of fiscal prudence is required. A value model championed by Michael Porter encompasses these parameters, in which value is defined as outcomes divided by cost. OBJECTIVES: To assess ambulatory cleft lip surgery from a quality and safety perspective, and to assess the costs associated with ambulatory cleft lip surgery in North America. Conclusions will be drawn as to how the overall value of cleft lip surgery may be enhanced. METHODS: A value analysis of published articles related to ambulatory cleft lip repair over the past 30 years was performed to determine what percentage of patients would be candidates for ambulatory cleft lip repair from a quality and safety perspective. An economic model was constructed based on costs associated with the inpatient stay related to cleft lip repair. RESULTS: On analysis of the published reports in the literature, a minority (28%) of patients are currently discharged in an ambulatory fashion following cleft lip repair. Further analysis suggests that 88.9% of patients would be safe candidates for same-day discharge. From an economic perspective, the mean cost per patient for the overnight admission component of ambulatory cleft surgery to the health care system in the United States was USD$2,390 and $1,800 in Canada. CONCLUSIONS: The present analysis reviewed germane publications over a 30-year period, ultimately suggesting that ambulatory cleft lip surgery results in preservation of quality and safety metrics for most patients. The financial model illustrates a potential cost saving through the adoption of such a practice change. For appropriately selected patients, ambulatory

  6. Splenic epidermoid cysts.

    PubMed Central

    Robbins, F G; Yellin, A E; Lingua, R W; Craig, J R; Turrill, F L; Mikkelsen, W P

    1978-01-01

    Four patients with splenic masses were operated upon and found to have epidermoid cysts of the spleen, a rare lesion comprising less than 10% of benign, nonparasitic splenic cysts. The patients were young and had vague, non-specific symptoms which were related to the size of the slowly enlarging splenic mass. Three patients had palpable masses. Contrast gastrointestinal studies and intravenous urography will help exclude mass lesions of the gastrointestinal or genitourinary tract. Sonar scan may confirm the cystic nature of the lesion and localize it to the spleen. A review of 42,327 autopsy records at the Los Angeles County--University of Southern California Medical Center revealed 32 benign splenic cysts found incidentally at autopsy. Hemorrhage, infection, rupture, and rarely, malignant change are complications of splenic cysts. Splenectomy is recommended to eliminate the symptoms produced by the cyst and prevent the potential complications. Images Fig. 1. Fig. 2. Fig. 3. Fig. 4. Fig. 5. Fig. 6. PMID:637577

  7. Arachnoid cyst spontaneous rupture.

    PubMed

    Marques, Inês Brás; Vieira Barbosa, José

    2014-01-01

    Arachnoid cysts are benign congenital cerebrospinal fluid collections, usually asymptomatic and diagnosed incidentally in children or adolescents. They may become symptomatic after enlargement or complications, frequently presenting with symptoms of intracranial hypertension. We report an unusual case of progressive refractory headache in an adult patient due to an arachnoid cyst spontaneous rupture. Although clinical improvement occurred with conservative treatment, the subdural hygroma progressively enlarged and surgical treatment was ultimately needed. Spontaneous rupture is a very rare complication of arachnoid cysts. Accumulation of cerebrospinal fluid accumulation in the subdural space causes sustained intracranial hypertension that may be life-threatening and frequently requires surgical treatment. Patients with arachnoid cysts must be informed on their small vulnerability to cyst rupture and be aware that a sudden and severe headache, especially if starting after minor trauma or a Valsalva manoeuvre, always requires medical evaluation. PMID:24581205

  8. Treatment of Ganglion Cysts

    PubMed Central

    Fung, B.; Lung, C. P.

    2013-01-01

    Ganglion cysts are soft tissue swellings occurring most commonly in the hand or wrist. Apart from swelling, most cysts are asymptomatic. Other symptoms include pain, weakness, or paraesthesia. The two main concerns patients have are the cosmetic appearance of the cysts and the fear of future malignant growth. It has been shown that 58% of cysts will resolve spontaneously over time. Treatment can be either conservative or through surgical excision. This review concluded that nonsurgical treatment is largely ineffective in treating ganglion cysts. However, it advised to patients who do not surgical treatment but would like symptomatic relief. Compared to surgery, which has a lower recurrence rate but have a higher complication rate with longer recovery period. It has been shown that surgical interventions do not provide better symptomatic relief compared to conservative treatment. If symptomatic relief is the patient's primary concern, a conservative approach is preferred, whilst surgical intervention will decrease the likelihood of recurrence. PMID:24967120

  9. Review of secondary alveolar cleft repair

    PubMed Central

    Cho-Lee, Gui-Youn; García-Díez, Eloy-Miguel; Nunes, Richard-Agostinho; Martí-Pagès, Carles; Sieira-Gil, Ramón; Rivera-Baró, Alejandro

    2013-01-01

    Introduction: The alveolar cleft is a bony defect that is present in 75% of the patients with cleft lip and palate. Although secondary alveolar cleft repair is commonly accepted for these patients, nowadays, controversy still remains regarding the surgical technique, the timing of the surgery, the donor site, and whether the use of allogenic materials improve the outcomes. The purpose of the present review was to evaluate the protocol, the surgical technique and the outcomes in a large population of patients with alveolar clefts that underwent secondary alveolar cleft repair. Materials and Methods: A total of 109 procedures in 90 patients with alveolar cleft were identified retrospectively after institutional review board approval was obtained. The patients were treated at a single institution during a period of 10 years (2001-2011). Data were collected regarding demographics, type of cleft, success parameters of the procedure (oronasal fistulae closure, unification of the maxillary segments, eruption and support of anterior teeth, support to the base of the nose, normal ridge form for prosthetic rehabilitation), donor site morbidity, and complications. Pre- and postoperative radiological examination was performed by means of orthopantomogram and computed tomography (CT) scan. Results: The average patient age was 14.2 years (range 4–21.3 years). There were 4 right alveolar-lip clefts, 9 left alveolar-lip clefts, 3 bilateral alveolar-lip clefts, 18 right palate-lip clefts, 40 left palate-lip clefts and 16 bilateral palate-lip clefts. All the success parameters were favorable in 87 patients. Iliac crest bone grafts were employed in all cases. There were three bone graft losses. In three cases, allogenic materials used in a first surgery performed in other centers, underwent infection and lacked consolidation. They were removed and substituted by autogenous iliac crest bone graft. Conclusions: The use of autogenous iliac crest for secondary alveolar bone grafting

  10. Oesophageal duplication cyst mimicking hydatid cyst in endemic areas

    PubMed Central

    Akin, Melih; Yildiz, Abdullah; Karadag, Cetin Ali; Sever, Nihat; Dokucu, Ali Ihsan

    2015-01-01

    The cystic appearance of both oesophageal duplications and pulmonary hydatid cysts can cause a misdiagnosis very easily due to rarity of cystic oesophageal duplications beside the higher incidence of hydatid cyst, especially in endemic areas. Here we report a 7-year-old girl with an oesophageal duplication cyst on the left side misdiagnosed as a hydatid cyst. The aim of the study is to report rare oesophageal duplications in the differential diagnosis of intrathoracic cysts. PMID:26702290

  11. Management of adult choledochal cysts.

    PubMed Central

    Powell, C S; Sawyers, J L; Reynolds, V H

    1981-01-01

    A review of the English literature reveals a total of 1,337 patients with choledochal cysts. Improved diagnostic techniques to visualize the biliary system are demonstrating an increasing number of unsuspected choledochal cysts in adult patients. Either choledochal cysts remain clinically silent until adulthood or may develop in later life. Experience is reported with adult patients having type I, II, III, and IV choledochal cysts. Type I cysts are preferably managed by excision but cyst anatomy may necessitate choledochoenteric drainage. Type II cysts are treated by excision except for those located within the pancreatic portion of the common bile duct. These are best managed by transduodenal cystoduodenostomy. The type III cyst (choledochocele) should be excised carefully, identifying and preserving the common bile and pancreatic ducts. Type IV cysts include a combination of any one of the first three types of cyst plus the presence of intrahepatic cyst or cysts. Treatment of these cysts is dictated by the type and location of the extrahepatic cyst. Since choledochal cysts are being recognized with increased frequency in adults, surgeons need to be aware of the diagnostic and treatment modalities available for each type of biliary cyst. Images Fig. 3. Fig. 4. Fig. 6. Fig. 7. Fig. 9. Fig. 10. PMID:7235770

  12. Genetics of Nonsyndromic Orofacial Clefts

    PubMed Central

    Rahimov, Fedik; Jugessur, Astanand; Murray, Jeffrey C.

    2011-01-01

    With an average worldwide prevalence of approximately 1.2/1000 live births, orofacial clefts are the most common craniofacial birth defects in humans. Like other complex disorders, these birth defects are thought to result from the complex interplay of multiple genes and environmental factors. Significant progress in the identification of underlying genes and pathways has benefited from large populations available for study, increased international collaboration, rapid advances in genotyping technology, and major improvements in analytic approaches. Here we review recent advances in genetic epidemiological approaches to complex traits and their applications to studies of nonsyndromic orofacial clefts. Our main aim is to bring together a discussion of new and previously identified candidate genes to create a more cohesive picture of interacting pathways that shape the human craniofacial region. In future directions, we highlight the need to search for copy number variants that affect gene dosage and rare variants that are possibly associated with a higher disease penetrance. In addition, sequencing of protein-coding regions in candidate genes and screening for genetic variation in non-coding regulatory elements will help advance this important area of research. PMID:21545302

  13. [Intraventricular arachnoid cyst].

    PubMed

    Rico-Cotelo, María; Diaz-Cabanas, Lucía; Allut, Alfredo G; Gelabert-Gonzalez, Miguel

    2013-07-01

    INTRODUCTION. Intracranial arachnoids cysts are considered benign developmental anomalies that occur within the arachnoid membrane and generally contain clear and colourless fluid resembling cerebrospinal fluid. The prevalence of these cysts is higher in the first two decades of life, and the incidence is widely quoted as approximately 1% of all space-occupying intracranial lesions. Arachnoids cysts in the elderly person are a rare occurrence. We report the unusual presentation of a woman with an intraventricular arachnoid cyst treated with endoscopic technique. CASE REPORT. A 75-year-old woman presented with progressive hemiparesis of two years duration. Cranial MR imaging showed a right parieto-occipital intraventricular cyst with local mass effect and moderate dilatation of lateral ventricles. A right-sided burr hole was made and the arachnoids cyst was reached and cysto-ventricle shunting was realized. This was followed by a septum pellucidum fenestration. There were no complications during the surgery and the patient presented no symptoms at time of discharge. CONCLUSIONS. The neuroendoscopic approach to intraventricular arachnoid cysts was effective with few complications. PMID:23799598

  14. Tarlov Cyst and Infertility

    PubMed Central

    Singh, Pankaj Kumar; Singh, Vinay Kumar; Azam, Amir; Gupta, Sanjeev

    2009-01-01

    Background/Objective: Tarlov cysts or spinal perineurial cysts are uncommon lesions. These are mostly incidental findings on magnetic resonance imaging or myelograms. The objectives of this study were to describe Tarlov cysts of the sacral region as a potential cause for retrograde ejaculations and review available management options. Methods: Case report and literature review. Results: A 28-year-old man presented with back pain and retrograde ejaculations resulting in infertility. After microsurgical excision of large perineurial cysts, back pain resolved, but semen quality showed only marginal improvement. Later, the couple successfully conceived by intrauterine insemination. To the best of our knowledge, this is the first reported case of Tarlov cyst associated with retrograde ejaculation and infertility. Conclusions: Despite being mostly asymptomatic and an incidental finding, Tarlov cyst is an important clinical entity because of its tendency to increase in size with time. Tarlov cysts of the sacral and cauda equina region may be a rare underlying cause in otherwise unexplained retrograde ejaculations and infertility. Microsurgical excision may be a good option in a select group of patients. PMID:19569467

  15. Implementing the Brazilian Database on Orofacial Clefts

    PubMed Central

    Monlleó, Isabella Lopes; Fontes, Marshall Ítalo Barros; Ribeiro, Erlane Marques; de Souza, Josiane; Leal, Gabriela Ferraz; Félix, Têmis Maria; Fett-Conte, Agnes Cristina; Bueno, Bruna Henrique; Magna, Luis Alberto; Mossey, Peter Anthony; Gil-da Silva-Lopes, Vera

    2013-01-01

    Background. High-quality clinical and genetic descriptions are crucial to improve knowledge of orofacial clefts and support specific healthcare polices. The objective of this study is to discuss the potential and perspectives of the Brazilian Database on Orofacial Clefts. Methods. From 2008 to 2010, clinical and familial information on 370 subjects was collected by geneticists in eight different services. Data was centrally processed using an international system for case classification and coding. Results. Cleft lip with cleft palate amounted to 198 (53.5%), cleft palate to 99 (26.8%), and cleft lip to 73 (19.7%) cases. Parental consanguinity was present in 5.7% and familial history of cleft was present in 26.3% subjects. Rate of associated major plus minor defects was 48% and syndromic cases amounted to 25% of the samples. Conclusions. Overall results corroborate the literature. Adopted tools are user friendly and could be incorporated into routine patient care. The BDOC exemplifies a network for clinical and genetic research. The data may be useful to develop and improve personalized treatment, family planning, and healthcare policies. This experience should be of interest for geneticists, laboratory-based researchers, and clinicians entrusted with OC worldwide. PMID:23577250

  16. Ectrodactyly-ectodermal dysplasia clefting syndrome (EEC syndrome)

    PubMed Central

    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. PMID:25737931

  17. 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. PMID:25737931

  18. Distal regeneration involves the age dependent activity of branchial sac stem cells in the ascidian Ciona intestinalis

    PubMed Central

    2014-01-01

    Abstract Tunicates have high capacities for regeneration but the underlying mechanisms and their relationship to life cycle progression are not well understood. Here we investigate the regeneration of distal structures in the ascidian tunicate Ciona intestinalis. Analysis of regenerative potential along the proximal−distal body axis indicated that distal organs, such as the siphons, their pigmented sensory organs, and the neural complex, could only be replaced from body fragments containing the branchial sac. Distal regeneration involves the formation of a blastema composed of cells that undergo cell proliferation prior to differentiation and cells that differentiate without cell proliferation. Both cell types originate in the branchial sac and appear in the blastema at different times after distal injury. Whereas the branchial sac stem cells are present in young animals, they are depleted in old animals that have lost their regeneration capacity. Thus Ciona adults contain a population of age‐related stem cells located in the branchial sac that are a source of precursors for distal body regeneration. PMID:25893097

  19. The endocrine spectrum of intracranial cysts in childhood and review of the literature.

    PubMed

    Savas Erdeve, Senay; Ocal, Gonul; Berberoglu, Merih; Siklar, Zeynep; Hacihamdioglu, Bulent; Evliyaoglu, Olcay; Fitoz, Suat

    2011-01-01

    Intracranial cysts (ICC) may cause a wide spectrum of endocrinological disorders. We evaluated 27 patients who were diagnosed with ICC during investigation for neuroendocrine dysfunctions and reviewed the relevant literature. The types of ICC in the patients were arachnoid cysts (n = 13); Rathke cleft cysts (n = 7); pineal cysts (n = 5); an ependymal cyst (n = 1) and a cavum septum pellucidum cyst (n = 1). The neuroendocrine dysfunctions of the patients were obesity (n = 7), isolated growth hormone deficiency (n = 6), central precocious puberty (n = 6), multiple pituitary hormone deficiency (n = 3), central diabetes insipidus (n = 1), growth hormone deficiency and central precocious puberty (n = 1), obesity and galactorrhea (n = 1), obesity and hypogonadotropic hypogonadism (n = 1) and growth hormone neurosecretory dysfunction (n = 1). Only three patients, who had arachnoid cysts, showed neurologic symptomatology. Although three patients underwent surgery, no improvements in endocrinological dysfunctions were observed. ICC should be considered when evaluating patients with endocrinological problems and patients with coincidental ICC should be recommended for follow-up. PMID:22308834

  20. Use of Biphasic Continuous Positive Airway Pressure in Premature Infant with Cleft Lip–Cleft Palate

    PubMed Central

    George, Lovya; Jain, Sunil K.

    2015-01-01

    Preterm infants (PIs) often require respiratory support due to surfactant deficiency. Early weaning from mechanical ventilation to noninvasive respiratory support decreases ventilation-associated irreversible lung damage. This wean is particularly challenging in PIs with cleft lip and cleft palate due to anatomical difficulties encountered in maintaining an adequate seal for positive pressure ventilation. PI with a cleft lip and palate often fail noninvasive respiratory support and require continued intubation and mechanical ventilation. We are presenting the first case report of a PI with cleft lip and palate who was managed by biphasic nasal continuous positive airway pressure. PMID:26495158

  1. Use of Biphasic Continuous Positive Airway Pressure in Premature Infant with Cleft Lip-Cleft Palate.

    PubMed

    George, Lovya; Jain, Sunil K

    2015-10-01

    Preterm infants (PIs) often require respiratory support due to surfactant deficiency. Early weaning from mechanical ventilation to noninvasive respiratory support decreases ventilation-associated irreversible lung damage. This wean is particularly challenging in PIs with cleft lip and cleft palate due to anatomical difficulties encountered in maintaining an adequate seal for positive pressure ventilation. PI with a cleft lip and palate often fail noninvasive respiratory support and require continued intubation and mechanical ventilation. We are presenting the first case report of a PI with cleft lip and palate who was managed by biphasic nasal continuous positive airway pressure. PMID:26495158

  2. Dental anomalies inside the cleft region in individuals with nonsyndromic cleft lip with or without cleft palate

    PubMed Central

    Sá, Jamile; Araújo, Luana; Guimarães, Laís; Maranhão, Samário; Lopes, Gabriela; Medrado, Alena; Coletta, Ricardo

    2016-01-01

    Background Individuals with non syndromic cleft lip with or without cleft palate (NSCL±P) present high frequency of dental anomalies, which may represent complicating factors for dental treatment. The aim of this study was to investigate the prevalence of dental anomalies inside cleft area in a group of Brazilians with NSCL±P. Material and Methods Retrospective analysis of 178 panoramic radiographs of patients aged from 12 to 45 years old and without history of tooth extraction or orthodontic treatment was performed. Association between cleft type and the prevalence of dental anomalies was assessed by chi-square test with a significance level set at p≤ 0.05. Results Dental anomalies were found in 88.2% (n=157) of the patients. Tooth agenesis (47.1%), giroversion (20%) and microdontia (15.5%) were the most common anomalies. Individuals with unilateral complete cleft lip and palate (CLP, p<0.0001), bilateral complete CLP (p=0.0002) and bilateral incomplete CLP (p< 0.0001) were more affected by tooth agenesis than individuals with other cleft types. The maxillary lateral incisors were the most affected teeth (p<0.0001). Conclusions The present study revealed a high frequency of dental anomalies inside cleft region in NSCL±P patients, and further demonstrated that patients with unilateral complete CLP and bilateral incomplete CLP were frequently more affected by dental anomalies. Moreover, our results demonstrate that dental anomalies should be considered during dental treatment planning of individuals affected by NSCL±P. Key words:Nonsyndromic cleft lip with or without palate, dental anomaly, tooth agenesis, microdontia. PMID:26615505

  3. Fibrosis and Simple Cysts

    MedlinePlus

    ... lobular) Lobular carcinoma in situ (LCIS) Adenosis Fibroadenomas Phyllodes tumors Intraductal papillomas Granular cell tumors Fat necrosis ... caused by fibrosis and/or cysts, which are benign changes in breast tissue that happen in many ...

  4. Pelvic aneurysmal bone cyst

    PubMed Central

    Sharifah, MIA; Nor Hazla, MH; Suraya, A; Tan, SP

    2011-01-01

    This paper describes an extremely rare case of a huge aneurysmal bone cyst (ABC) in the pelvis, occurring in the patient’s 5th decade of life. The patient presented with a history of painless huge pelvic mass for 10 years. Plain radiograph and computed tomography showed huge expansile lytic lesion arising from the right iliac bone. A biopsy was performed and histology confirmed diagnosis of aneurysmal bone cyst. Unfortunately, the patient succumbed to profuse bleeding from the tumour. PMID:22279501

  5. Testicular epidermoid cyst

    PubMed Central

    Çakıroglu, Basri; Sönmez, Nurettin Cem; Sinanoğlu, Orhun; Ateş, Lora; Aksoy, Süleyman Hilmi; Özcan, Faruk

    2015-01-01

    Epidermoid cyst of the testis is a benign, non-teratomatous tumour. It is often possible to make the diagnosis pre-operatively, combining typical sonographic features with normal biochemical tumour markers. The accurate pre-operative diagnosis will allow for testis-sparing surgery and prevent unnecessary orchiectomy. An 11-year-old boy with testicular epidermoid cyst who presented with pain in testis was presented in this report. PMID:25659561

  6. Lacrimal duct cyst abscess.

    PubMed

    Dharmasena, Aruna; Sobajo, Cassandra; Irion, Luciane; Ataullah, Sajid

    2014-12-01

    Cystic dilatation within the lacrimal gland is thought to be related to chronic inflammation and scarring of the lacrimal gland ductules. We review the literature and discuss a case and of lacrimal duct cyst suppuration presenting with visual loss, external ophthalmoplegia, proptosis and ptosis. To our knowledge, only one other report of a lacrimal ductal cyst abscess has been reported in the literature so far. PMID:25208223

  7. Sonic hedgehog is required for the assembly and remodeling of branchial arch blood vessels.

    PubMed

    Kolesová, Hana; Roelink, Henk; Grim, Milos

    2008-07-01

    Sonic hedgehog (Shh) is a morphogen involved in many developmental processes. Injection of cells (5E1) that produce a Shh-blocking antibody causes an attenuation of the Shh response, and this causes vascular malformations and impaired remodeling characterized by hemorrhages and protrusions of the anterior cardinal vein and outflow tract, delayed fusion of the dorsal aortae, impaired branching of the internal carotid artery, and delayed remodeling of the aortic arches. Distribution of smooth muscle cells in the vessel wall is unchanged. In 5E1-injected embryos, we also observed impaired assembly of endothelial cells into vascular tubes, particularly in the sixth branchial arch, around the anterior cardinal vein and around the dorsal aorta. In 5E1-treated embryos, increased numbers of macrophage-like cells, apoptotic cells, and a decreased level of proliferation were observed in head mesenchyme. Together, these observations show that Shh signaling is required at multiple stages for proper vessel formation and remodeling. PMID:18570256

  8. Technique in Cleft Rhinoplasty: The Foundation Graft.

    PubMed

    Gassner, Holger G; Schwan, Franziska; Haubner, Frank; Suárez, Gustavo A; Vielsmeier, Veronika

    2016-04-01

    Secondary cleft rhinoplasty represents a particular surgical challenge. The authors have identified the deficit in skeletal projection of the cleft-sided piriform rim as an important contributor to the pathology. A graft is described to augment the piriform crest on the cleft side. This foundation graft is suture fixated to the piriform crest after complete release of all soft tissue attachments to the alar base. The foundation graft is articulated with a long alar strut graft, which allows for powerful projection of the cleft-sided nasal tip. An advancement flap of vestibular skin is described to correct the vestibular stenosis. A transplant of diced cartilage in fascia is added to augment maxillary soft tissue volume. Subjective and objective measures of form and function are presented in a retrospective series of five cases, illustrating the efficacy of the techniques described. PMID:27097143

  9. Cleft Lip and Palate (For Parents)

    MedlinePlus

    ... and breathing, overbites/underbites, and appearance. Dental and Orthodontic Treatment Maintaining healthy teeth and preventing cavities is ... Kids with cleft lip and palate may begin orthodontic treatment as early as 6 years of age. ...

  10. Pancreas and cyst segmentation

    NASA Astrophysics Data System (ADS)

    Dmitriev, Konstantin; Gutenko, Ievgeniia; Nadeem, Saad; Kaufman, Arie

    2016-03-01

    Accurate segmentation of abdominal organs from medical images is an essential part of surgical planning and computer-aided disease diagnosis. Many existing algorithms are specialized for the segmentation of healthy organs. Cystic pancreas segmentation is especially challenging due to its low contrast boundaries, variability in shape, location and the stage of the pancreatic cancer. We present a semi-automatic segmentation algorithm for pancreata with cysts. In contrast to existing automatic segmentation approaches for healthy pancreas segmentation which are amenable to atlas/statistical shape approaches, a pancreas with cysts can have even higher variability with respect to the shape of the pancreas due to the size and shape of the cyst(s). Hence, fine results are better attained with semi-automatic steerable approaches. We use a novel combination of random walker and region growing approaches to delineate the boundaries of the pancreas and cysts with respective best Dice coefficients of 85.1% and 86.7%, and respective best volumetric overlap errors of 26.0% and 23.5%. Results show that the proposed algorithm for pancreas and pancreatic cyst segmentation is accurate and stable.

  11. Juxtafacet Spinal Synovial Cysts

    PubMed Central

    2016-01-01

    Study Design This was a retrospective study. Purpose To study the surgical outcome of synovial cysts of the lumbar spine through posterior laminectomy in combination with transpedicular screw fixation. Overview of Literature Synovial cysts of the lumbar spine contribute significantly to narrowing of the spinal canal and lateral thecal sac and nerve root compression. Cysts form as a result of arthrotic disruption of the facet joint, leading to degenerative spondylolisthesis in up to 40% of patients. Methods Retrospective data from 6 patients, treated during the period of March 2007 to February 2011, were analyzed. All preoperative and postoperative manifestations, extension/flexion radiographs, magnetic resonance imaging, and computed tomography records were reviewed. All underwent surgery for synovial cysts with excision and decompression combined with posterior fixation. The result of surgery was evaluated with Macnab's classification. An excellent or good outcome was considered as satisfactory. Japanese Orthopedic Association Scale was used for evaluation of back pain. Results All patients included in this study had excellent outcomes as regarding to improvement of all preoperative manifestations and returning to normal daily activities. Only 2 cases developed postoperative transient cerebro-spinal fluid leak and were treated conservatively and improved during the follow up period. Conclusions Although this study included a small number of cases and we could not have statistically significant results, the good outcome of decompression of synovial cysts combined with posterior fixation and fusion encouraged us to recommend this approach for patients with juxtafacet synovial cysts. PMID:26949457

  12. Acromioclavicular joint cyst formation.

    PubMed

    Hiller, Andrew D; Miller, Joshua D; Zeller, John L

    2010-03-01

    Acromioclavicular joint (ACJ) cysts are an uncommon and unusual sequela associated with shoulder pathophysiology. The majority of literature on ACJ cysts consists of individual case reports with no definitive literature review currently available. In addition to a comprehensive literature review, four clinical cases are presented in this report. First described by Craig (1984), a total of 41 cases have been previously reported in the literature. Of these cases, five occurred with the rotator cuff musculature intact. The remaining 36 cases of ACJ cysts occurred in patients with a complete tear/avulsion of the rotator cuff. Previous attempts at compiling a complete record of all reported cases have combined several distinct conditions into a single category. This article presents two distinct etiologies for the pathogenesis of ACJ cyst formation. In the presence of an intact rotator cuff, a Type 1 cyst can form superficially and be limited to the ACJ. Following a massive or traumatic tear of the rotator cuff, mechanical instability of the humeral head can cause a deterioration of the inferior acromioclavicular capsule (cuff tear arthropathy) and an overproduction of synovial fluid. Overtime, a "geyser" of fluid can form between the glenohumeral and the ACJ, forming a Type 2 cyst. This differentiation and categorization is essential for appropriate classification and treatment. PMID:20069645

  13. [Choledochal cysts: surgical treatment].

    PubMed

    Gogolja, D; Visnjić, S; Milić, Z; Tomić, K; Car, A; Roić, G; Fattorini, I

    2000-03-01

    The excision of the choledochal cyst with bile drainage through intestinal conduit is a standard operative procedure in the surgical management of choledochal cysts. During the last eight years five patients have been treated with this operation at the University Children's Hospital in Zagreb. All the patients were girls aged from two months to twelve years. The classical triad of pain, jaundice and abdominal mass was observed in only one patient, an eight-year-old girl. The only symptom in infancy was jaundice. Diagnosis was made by abdominal ultrasound, bibliography, CT scan with hepatotropic contrast and in older children by ERCP. Four cysts were type Todani I, and one cyst was Todani type II. The complete excision of the choledochal cyst with the Roux-Y jejunal conduit without antireflux valve was performed. There was neither operative morbidity nor mortality. Three months postoperatively the control ultrasonography and liver laboratory tests were without abnormalities. The routine control which followed did not show episodes of cholangitis, lithiasis, lipid malabsorption, blood clotting abnormalities or growth failure. The complete excision of the cyst with Roux-Y hepaticoenterostomy is an operative treatment with good results in infancy and childhood. PMID:10932533

  14. [Laser navigation guided cleft lip repair].

    PubMed

    Bing, Shi

    2016-06-01

    A new method using the ideal mid-facial line as the navigating reference was introduced to improve the outcome of cleft lip repair. Using the verticle coordinate crossing the middle point of the intercanthus line, surgeons could observe and correct the distortion of the fine structures in labial-nasal area. This laser projecting mid-facial-line navigation was repeatable, while not interfere the operating. In conclusion, generalizing laser navigation is a valuable supplementary for cleft lip repair. PMID:27526442

  15. Growing Hemorrhagic Choroidal Fissure Cyst

    PubMed Central

    Gelal, Fazıl; Gurkan, Gokhan; Feran, Hamit

    2016-01-01

    Choroidal fissure cysts are often incidentally discovered. They are usually asymptomatic. The authors report a case of growing and hemorrhagic choroidal fissure cyst which was treated surgically. A 22-year-old female presented with headache. Cranial MRI showed a left-sided choroidal fissure cyst. Follow-up MRI showed that the size of the cyst had increased gradually. Twenty months later, the patient was admitted to our emergency department with severe headache. MRI and CT showed an intracystic hematoma. Although such cysts usually have a benign course without symptoms and progression, they may rarely present with intracystic hemorrhage, enlargement of the cyst and increasing symptomatology. PMID:26962426

  16. Choledochal cysts: diagnosis and treatment.

    PubMed

    Popova-Jovanovska, R; Genadieva-Dimitrova, M; Trajkovska, M; Serafimoski, V

    2012-01-01

    The aim of this study is to show the different diagnostic procedures and treatment in patients diagnosed with congenital choledochal cysts. Choledochal cysts are congenital anomalies of the bile ducts and include cystic dilatation of the extrahepatic and intrahepatic biliary ducts or both. The study shows ten patients diagnosed as having choledochal cysts. Diagnosis was established by clinical and radiographic findings including: ultrasound (US), magnetic resonance cholangiopancreatograpy (MRCP), endoscopic retrograde cholangiopancreatography (ERCP), percutaneous transhepatic cholangiography (PTC) and cytological examination of the bile juice. In the study choledochal cysts were classified according to the Todani classification. Most common cysts were type I (six cases); type III (one case), type IVa (one case) and two patients were type V cysts (Caroli disease). The most frequent symptoms were abdominal pain, jaundice and cholangitis. US findings were sensitive for the preliminary diagnosis of choledochal cysts in all the patients. MRCP accurately defined the cyst anatomy and the site of the biliary origin in all the cases with extrahepatic cysts. In three cases ERCP clearly demonstrated the cyst and by PTC smaller cysts were well defined. Cytological examination of the bile juice obtained during the PTC procedure showed malignant cells in one case. Therefore pancreaticoduodenectomy was performed and pathological examination showed associated cholangiocarcinoma. Five years after the operation the patient was well and free of the disease. Five patients underwent surgical treatment with a total cyst excision and Roux-en-Y hepaticojejunostomy while the surgical approach in two patients was partial cyst excision and cystojejunostomy. Patients with Caroli disease were conservatively treated and 3 with interventional endoscopic procedures. Despite US evidence suggesting choledochal cyst diagnosis, other supportive radiographic imaging modalities such as MRCP, ERCP and

  17. Preservation of protein clefts in comparative models

    PubMed Central

    Piedra, David; Lois, Sergi; de la Cruz, Xavier

    2008-01-01

    Background Comparative, or homology, modelling of protein structures is the most widely used prediction method when the target protein has homologues of known structure. Given that the quality of a model may vary greatly, several studies have been devoted to identifying the factors that influence modelling results. These studies usually consider the protein as a whole, and only a few provide a separate discussion of the behaviour of biologically relevant features of the protein. Given the value of the latter for many applications, here we extended previous work by analysing the preservation of native protein clefts in homology models. We chose to examine clefts because of their role in protein function/structure, as they are usually the locus of protein-protein interactions, host the enzymes' active site, or, in the case of protein domains, can also be the locus of domain-domain interactions that lead to the structure of the whole protein. Results We studied how the largest cleft of a protein varies in comparative models. To this end, we analysed a set of 53507 homology models that cover the whole sequence identity range, with a special emphasis on medium and low similarities. More precisely we examined how cleft quality – measured using six complementary parameters related to both global shape and local atomic environment, depends on the sequence identity between target and template proteins. In addition to this general analysis, we also explored the impact of a number of factors on cleft quality, and found that the relationship between quality and sequence identity varies depending on cleft rank amongst the set of protein clefts (when ordered according to size), and number of aligned residues. Conclusion We have examined cleft quality in homology models at a range of seq.id. levels. Our results provide a detailed view of how quality is affected by distinct parameters and thus may help the user of comparative modelling to determine the final quality and

  18. Congenital Mucous Retention Cyst of the Anterior Hard Palate! the First Case Report

    PubMed Central

    Priyadarshini, Smita; Pati, Abhishek Ranjan; Bhuyan, Sanat Kumar; Panigrahi, Rajat G

    2014-01-01

    Children may be born with birth defects, the most common being oro-facial clefts and fissural cysts. A well circumscribed pedunculated soft tissue growth that occurs congenitally is known as congenital epulis of the newborn or ‘Neuman’s Tumour’ as described in the literature. It is a rare lesion and the diagnosis has to be confirmed histologically. We present a rare case of a 7-year-old child with a congenital growth in the pre-maxillary region of the anterior hard palate clinically diagnosed as congenital epulis however, histologically confirmed as a mucous retention cyst. An elaborate clinical differential diagnosis is discussed. The anterior hard palate is devoid of salivary glands and the presence of a mucous retention cyst in the area is suggestive of ectopic salivary gland tissue and in a child manifesting at birth is probably the first case to be reported in the English literature. PMID:25478467

  19. Thyroglossal cyst: an unusual presentation.

    PubMed

    Karmakar, Subhamay; Saha, A M; Mukherjee, Dhrubyajyoti

    2013-07-01

    To highlight the difference in symptoms, clinical features and management of an intralingual thyroglossal cyst from a classical thyroglossal cyst. We present here the case of a 10 year old boy, who presented to us with the chief complaint of difficulty in speech for 2 years. A marble shaped swelling was seen on the base of the tongue. It was diagnosed as an intralingual thyroglossal cyst. He underwent a Sistrunk operation and the cyst was removed from the base of the tongue. Literature search revealed the rarity of this intralingual thyroglossal cyst, its atypical presentation and difference in way of management. A case report and review of literature regarding this unusual unusual entity is presented. An intralingual thyroglossal cyst is the rarest form of a thyroglossal cyst, and differs from a classical thyroglossal cyst totally in presentation and management. PMID:24427642

  20. Fat Necrosis and Oil Cysts

    MedlinePlus

    ... Previous Topic Granular cell tumors Next Topic Mastitis Fat necrosis and oil cysts Fat necrosis happens when ... lumpy area if it becomes bothersome. How do fat necrosis and oil cysts affect your risk for ...

  1. Treatment for Adults (with Cleft Lip and Palate)

    MedlinePlus

    ... throat, hearing, dentistry, speech, oral surgery, nursing, and psychology among others. You can obtain the names of ... of the cleft team, particularly the psychologist and social worker. Interaction with other adults with clefts, through ...

  2. Bone cysts: unicameral and aneurysmal bone cyst.

    PubMed

    Mascard, E; Gomez-Brouchet, A; Lambot, K

    2015-02-01

    Simple and aneurysmal bone cysts are benign lytic bone lesions, usually encountered in children and adolescents. Simple bone cyst is a cystic, fluid-filled lesion, which may be unicameral (UBC) or partially separated. UBC can involve all bones, but usually the long bone metaphysis and otherwise primarily the proximal humerus and proximal femur. The classic aneurysmal bone cyst (ABC) is an expansive and hemorrhagic tumor, usually showing characteristic translocation. About 30% of ABCs are secondary, without translocation; they occur in reaction to another, usually benign, bone lesion. ABCs are metaphyseal, excentric, bulging, fluid-filled and multicameral, and may develop in all bones of the skeleton. On MRI, the fluid level is evocative. It is mandatory to distinguish ABC from UBC, as prognosis and treatment are different. UBCs resolve spontaneously between adolescence and adulthood; the main concern is the risk of pathologic fracture. Treatment in non-threatening forms consists in intracystic injection of methylprednisolone. When there is a risk of fracture, especially of the femoral neck, surgery with curettage, filling with bone substitute or graft and osteosynthesis may be required. ABCs are potentially more aggressive, with a risk of bone destruction. Diagnosis must systematically be confirmed by biopsy, identifying soft-tissue parts, as telangiectatic sarcoma can mimic ABC. Intra-lesional sclerotherapy with alcohol is an effective treatment. In spinal ABC and in aggressive lesions with a risk of fracture, surgical treatment should be preferred, possibly after preoperative embolization. The risk of malignant transformation is very low, except in case of radiation therapy. PMID:25579825

  3. Perioperative complications in infant cleft repair

    PubMed Central

    Fillies, Thomas; Homann, Christoph; Meyer, Ulrich; Reich, Alexander; Joos, Ulrich; Werkmeister, Richard

    2007-01-01

    Background Cleft surgery in infants includes special risks due to the kind of the malformation. These risks can be attributed in part to the age and the weight of the patient. Whereas a lot of studies investigated the long-term facial outcome of cleft surgery depending on the age at operation, less is known about the complications arising during a cleft surgery in early infancy. Methods We investigated the incidence and severity of perioperative complications in 174 infants undergoing primary cleft surgery. The severity and the complications were recorded during the intraoperative and the early postoperative period according to the classification by Cohen. Results Our study revealed that minor complications occurred in 50 patients. Severe complications were observed during 13 operations. There was no fatal complication in the perioperative period. The risk of complications was found to be directly correlated to the body weight at the time of the surgery. Most of the problems appeared intraoperatively, but they were also followed by complications immediately after the extubation. Conclusion In conclusion, cleft surgery in infancy is accompanied by frequent and sometimes severe perioperative complications that may be attributed to this special surgical field. PMID:17280602

  4. Relationship between spatially restricted Krox-20 gene expression in branchial neural crest and segmentation in the chick embryo hindbrain.

    PubMed Central

    Nieto, M A; Sechrist, J; Wilkinson, D G; Bronner-Fraser, M

    1995-01-01

    Previous studies have suggested that the rostrocaudal patterning of branchial arches in the vertebrate embryo derives from a coordinate segmental specification of gene expression in rhombomeres (r) and neural crest. However, expression of the Krox-20 gene is restricted to neural crest cells migrating to the third branchial arch, apparently from r5, whereas this rhombomere contributes cells to both the second and third arches. We examined in the chick embryo how this spatially restricted expression is established. Expression occurs in precursors in both r5 and r6, and we show by cell labelling that both rhombomeres contribute to Krox-20-expressing neural crest, emigration occurring first from r6 and later caudally from r5. Krox-20 transcripts are not detected in some precursors in rostral r5, presaging the lack of expression in cells migrating rostrally from this rhombomere. After transposition of r6 to the position of r4 or r5, many Krox-20-expressing cells migrate rostral to the otic vesicle, whereas when r5 is transplanted to the position of r4, only a small number of migrating cells express Krox-20. These results indicate that, in the chick, Krox-20 expression in branchial neural crest does not correlate with rhombomeric segmentation, and that there may be intrinsic differences in regulation between the r5 and r6 Krox-20-expressing populations. Images PMID:7537662

  5. Spectrographic measures of the speech of young children with cleft lip and cleft palate.

    PubMed

    Casal, C; Domínguez, C; Fernández, A; Sarget, R; Martínez-Celdrán, E; Sentís-Vilalta, J; Gay-Escoda, C

    2002-01-01

    Twenty-two consecutive children with repaired cleft lip and/or palate [isolated cleft lip (CL) 6, isolated cleft palate (CP) 7, unilateral cleft lip and palate (UCLP) 7, and bilateral cleft lip and palate 2] with a mean age of 27 months underwent spectrographic measures of tape-recorded speech (DSP Sona-Graph digital unit). Controls were 22 age- and sex-matched noncleft children. Data analyzed included (1) the Spanish vocalic variables [a, i, u, e, o]: first formant, second formant, duration, and context; (2) obstruent variables [p, t, k]: burst, voice onset time, and duration, and (3) nasal variables [m]: first formant, second formant, and duration. Statistically significant differences were observed between the CL group and the control group in the first formant of [e] and in the increase of the frequency of the [t] burst. Comparison between UCLP and controls showed differences in the second formant of [a], in the first formant of [o], and in the second formant of [o]. These results suggest a small but significant influence of either the cleft lip or its repair on lip rounding for [o] and [u]. In addition, tongue position differences were most likely responsible for the differences seen with [a] and [e]. Spectrographic differences in the current patients did not contribute to meaningful differences in speech sound development. Individualized care (orthodontics, surgery, speech therapy) in children with cleft lip and/or palate attended at specialized craniofacial units contributes to normalization of speech development. PMID:12378036

  6. Correlation of vermilion symmetry to alveolar cleft defect in unilateral cleft lip repair.

    PubMed

    Bonanthaya, K; Rao, D D; Shetty, P; Uguru, C

    2016-06-01

    Asymmetry is a major problem in repaired unilateral cleft lip (UCL). One of the important manifestations of this is the asymmetry of the vermilion. The aim of this study was to correlate the severity of the asymmetry in the vermilion to the size of the alveolar defect. Twenty patients aged between 6 and 18 months with complete unilateral cleft lip, alveolus, and palate were included. An impression of each patient's alveolus at the time of cheiloplasty was taken using silicon rubber base material, and a study cast was prepared. The width of the cleft alveolus was measured on these casts using a transparent grid. Frontal photographs were taken at 6 months postoperative and vermilion symmetry was measured as the ratio between the cleft and non-cleft sides. The results obtained in this study showed a direct correlation between the size of the alveolar defect and the vermilion symmetry in repaired UCL. The wider the cleft alveolus and greater the antero-posterior discrepancy, the greater is the vermilion asymmetry. The asymmetry of the vermilion in UCL after repair is directly dependent on the size of the alveolar defect. The alveolar discrepancy causes 'in-rolling' of the vermilion on the cleft side and affects the vermilion symmetry. PMID:26754270

  7. Risk of Oral Clefts (Cleft Lip and/or Palate) in Infants Born to Mothers Taking Topamax (Topiramate)

    MedlinePlus

    ... topiramate labels are being updated with the new information describing the increased risk of oral clefts. Q8. Does FDA have post marketing adverse event reports of oral clefts with topiramate? ...

  8. The Cleft Lip Nose: Primary and Secondary Treatment.

    PubMed

    Wolfe, Stephen Anthony; Nathan, Nirmal R; MacArthur, Ian R

    2016-01-01

    This article presents an overview of the cleft lip nasal deformity and its treatment. The complex pathologic changes to normal nasal anatomy are described, and treatment strategies for both unilateral and bilateral cleft lip patients are presented. The surgical technique for management of the cleft lip nasal deformity is discussed as it pertains to both primary and secondary correction. PMID:26616709

  9. Colloid cyst: a case report.

    PubMed

    Grasu, Beatrice L; Alberico, Anthony M

    2011-01-01

    Colloid cysts are a rare clinical finding with a unique clinical presentation: non-specific paroxysmal headaches. The current recommended treatment is microsurgery, which poses the greatest risk to the patient but allows complete removal of the cyst to prevent recurrence. A 41-year old man presented with a colloid cyst located in the foramen of Monro causing obstructive hydrocephalus. He had paroxysmal headaches and memory and personality changes. Transcortical transventricle microsurgery was performed to remove the entire cyst. A temporary shunt was placed to prevent post-operative hydrocephalus. Normal neurological function returned upon cyst removal. PMID:22034805

  10. [The adolescent with cleft lip and palate].

    PubMed

    Chapados, C

    1998-02-01

    Research studies afford an in-depth look at the problems experienced by adolescents born with a cleft lip, a cleft palate or cleft lip and palate. If not addressed, their problems can multiply with each successive stage of development. An interdisciplinary team, where the adolescents themselves play an important role, is one of the best means of helping them come to terms with the consequences of the abnormality. The nurse should be a key member of the team. To help adolescents cope with their situation now and in the future, the author promotes a holistic and humanistic approach. Here, she sees nurses creating therapeutic alliances as educators. She recommends that nurses assert themselves more as professionals and as essential resources. Her doctoral thesis proposes a training model to this effect. PMID:9573899

  11. Bilateral optic disc pit with maculopathy in a patient with cleft lip and cleft palate

    PubMed Central

    Seth, Anisha; Gupta, Rajat; Gupta, Anika; Raina, Usha K; Ghosh, Basudeb

    2015-01-01

    Optic disc pit (ODP) is small, gray-white, oval depression found at the optic nerve head. It is a congenital defect that occurs due to imperfect closure of superior edge of the embryonic fissure. Cleft lip and palate are also congenital midline abnormalities occurring due to defect in the fusion of frontonasal prominence, maxillary prominence and mandibular prominence. There is only one case report describing the occurrence of ODP in a young patient with cleft lip and palate who also had basal encephalocele. We describe a 52-year-old patient with congenital cleft lip and palate with bilateral ODP with maculopathy but without any other midline abnormality. PMID:26044478

  12. Hoxa2 Selectively Enhances Meis Binding to Change a Branchial Arch Ground State

    PubMed Central

    Amin, Shilu; Donaldson, Ian J.; Zannino, Denise A.; Hensman, James; Rattray, Magnus; Losa, Marta; Spitz, François; Ladam, Franck; Sagerström, Charles; Bobola, Nicoletta

    2015-01-01

    Summary Hox transcription factors (TFs) are essential for vertebrate development, but how these evolutionary conserved proteins function in vivo remains unclear. Because Hox proteins have notoriously low binding specificity, they are believed to bind with cofactors, mainly homeodomain TFs Pbx and Meis, to select their specific targets. We mapped binding of Meis, Pbx, and Hoxa2 in the branchial arches, a series of segments in the developing vertebrate head. Meis occupancy is largely similar in Hox-positive and -negative arches. Hoxa2, which specifies second arch (IIBA) identity, recognizes a subset of Meis prebound sites that contain Hox motifs. Importantly, at these sites Meis binding is strongly increased. This enhanced Meis binding coincides with active enhancers, which are linked to genes highly expressed in the IIBA and regulated by Hoxa2. These findings show that Hoxa2 operates as a tissue-specific cofactor, enhancing Meis binding to specific sites that provide the IIBA with its anatomical identity. PMID:25640223

  13. Branchial versus intestinal silver toxicity and uptake in the marine teleost Parophrys vetulus.

    PubMed

    Grosell, M; Wood, C M

    2001-10-01

    Exposure to elevated waterborne silver as AgNO3 (4.07 microM=448 microg l(-1)) in seawater resulted in osmoregulatory disturbance in the lemon sole (Parophrys vetulus). The main effects were increased plasma Na+ and Cl- concentrations which translated into increased plasma osmolality. Plasma Mg2+ levels were also slightly increased after 96 h exposure. Using radioisotopic flux measurements, a 50% reduction in branchial unidirectional Na+ extrusion was observed after 48 h silver exposure. By applying an intestinal perfusion approach, we were able to separate and thus quantify the intestinal contribution to the observed silver-induced physiological disturbance and internal silver accumulation. This analysis revealed that the intestinal contribution to silver-induced ionoregulatory toxicity was as high as 50-60%. In marked contrast, internal silver accumulation (in liver and kidney) was found to be derived exclusively from uptake across the gills. Drinking of silver-contaminated seawater resulted in substantial silver accumulation in the intestinal tissue (but apparently not silver uptake across the intestine), which probably explains the intestinal contribution to silver-induced physiological disturbance. PMID:11686617

  14. The Fetal Cleft palate: V. Elucidation of the Mechanism of Palatal Clefting in the Congenital Caprine Model

    Technology Transfer Automated Retrieval System (TEKTRAN)

    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...

  15. [Atrioventricular septal defect in an adult patient: There are 'clefts' and clefts].

    PubMed

    Moreno, Nuno; Almeida, Jorge; Amorim, Mário Jorge

    2016-03-01

    In this report, we present the case of an adult male with severe mitral regurgitation due to an atrioventricular septal defect. Anatomical assessment by two- and three-dimensional transesophageal echocardiography was essential for detailed morphological characterization and surgical planning. The different features of a 'cleft' in an atrioventricular septal defect compared to an anterior leaflet cleft in an otherwise normal mitral valve are here discussed. PMID:26947378

  16. 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

  17. Folic Acid and Orofacial Clefts: A Review of the Evidence

    PubMed Central

    Wehby, George

    2010-01-01

    Orofacial clefts are common and burdensome birth defects with a complex genetic and environmental etiology. The contribution of nutritional factors and supplements to the etiology of orofacial clefts has long been theorized and studied. Multiple studies have evaluated the role of folic acid in the occurrence and recurrence of orofacial clefts, using observational and non-randomized interventional designs. While preventive effects of folic acid on orofacial clefts are commonly reported, the evidence remains generally inconsistent. This paper reviews the findings of the main studies of the effects of folic acid on orofacial clefts, summarize study limitations, and discuss research needs with a focus on studying the effects of high dosage folic acid on the recurrence of oral clefts using a randomized clinical trial design. The role of folic acid in the prevention of neural tube defects is also briefly summarized and discussed as a reference model for orofacial clefts. PMID:20331806

  18. Parental Reactions to Cleft Palate Children.

    ERIC Educational Resources Information Center

    Vanpoelvoorde, Leah; Shaughnessy, Michael F.

    1991-01-01

    This paper reviews parents' emotional reactions following the birth of a cleft lip/palate child. It examines when parents were told of the deformity and discusses the duties of the speech-language pathologist and the psychologist in counseling the parents and the child. (Author/JDD)

  19. Parental Reactions to Cleft Palate Children.

    ERIC Educational Resources Information Center

    Vanpoelvoorde, Leah

    This literature review examines parental reactions following the birth of a cleft lip/palate child, focusing primarily on the mother's reactions. The research studies cited have explored such influences on maternal reactions as her feelings of lack of control over external forces and her feelings of guilt that the deformity was her fault. Delays…

  20. A case of optic-nerve hypoplasia and anterior segment abnormality associated with facial cleft

    PubMed Central

    Miyake, Tomoko; Kojima, Shota; Sugiyama, Tetsuya; Ueki, Mari; Sugasawa, Jun; Oku, Hidehiro; Tajiri, Kensuke; Shigemura, Yuka; Ueda, Koichi; Harada, Atsuko; Yamasaki, Mami; Yamanaka, Takumi; Utsunomiya, Hidetsuna; Ikeda, Tsunehiko

    2016-01-01

    Introduction The incidence of facial cleft is rare and ranges between 1.43 and 4.85 per 100,000 births. To date, there have been few reports of detailed ophthalmologic examinations performed in cases of facial cleft. Here, we report a case of optic-nerve hypoplasia and anterior segment abnormality associated with facial cleft. Case report A 9-day-old female infant was delivered by cesarian section at 34 weeks of gestational age (the second baby of twins) and weighed 2,276 g upon presentation. She had a facial cleft and ectrodactyly at birth. Right eye-dominant blepharophimosis was obvious. Examination of the right eye revealed inferior corneal opacity with vascularization, downward corectopia, and optic-nerve hypoplasia. The corneal diameter was 8 mm in both eyes, and tonometry by use of a Tono-Pen® XL (Reichert Technologies, Depew, NY, USA) handheld applanation tonometer revealed that her intraocular pressure was 11–22 mmHg (Oculus Dexter) and 8 mmHg (Oculus Sinister). B-mode echo revealed no differences in axial length between her right and left eyes. When she was 15–16 months old, we attempted to examine her eyes before she underwent plastic surgery under general anesthesia. She had a small optic disc in both eyes and the right-eye disc was tilted. After undergoing canthotomy, gonioscopy and ultrasound biomicroscopy revealed that almost all directions were open except for the peripheral anterior synechia. Since magnetic resonance imaging revealed ventriculomegaly associated with an interhemispheric cyst at birth, a ventriculoperitoneal shunt was inserted at 12 days of age. At 25 months of age, her condition suddenly deteriorated due to occlusion of the ventricular shunt catheter, and she died 5 days later. In this patient, amniotic band syndrome was presumed to be the primary cause due to the clinical findings. Conclusion We experienced a case of optic-nerve hypoplasia and anterior segment abnormality that occurred with facial cleft. The cause of these

  1. Two rare cases of simultaneous Tessier number 3 cleft, contralateral cleft lip, and signs of amniotic band syndrome.

    PubMed

    Xu, Yi; Mu, Yue; Chen, Renji; Zheng, Zongmei; Zhang, Wenjing

    2016-06-01

    The Tessier number 3 cleft is rare. In this paper, we report two extremely rare cases of simultaneous Tessier number 3 cleft, contralateral cleft lip, and signs of amniotic band syndrome. In the two cases, we confirmed that amniotic bands were the probable cause of the Tessier number 3 cleft, where swallowed fibrous strands of amniotic bands entangle a typical cleft lip and cause the more severe Tessier number 3 cleft. In this study, Z-plasty was performed for one case, and a straight-line method was used for the other. Postoperatively, the appearance of both patients was satisfactory, as expected. Consequently, treatment for the Tessier number 3 cleft should be designed individually based on the severity of deformity. PMID:27052637

  2. Angiomyolipoma With Epithelial Cysts.

    PubMed

    LeRoy, Michael A; Rao, Priya

    2016-06-01

    Angiomyolipoma with epithelial cysts is a rare mesenchymal tumor of the kidney that enters in the differential diagnosis of adult cystic renal neoplasms. These tumors demonstrate a slight female predominance and can present either incidentally or with symptoms, commonly flank pain and hematuria. Unlike conventional angiomyolipoma, this variant is characterized grossly by both solid and cystic areas, and histologically by the presence of single or multiple cysts lined by epithelial cells, a subepithelial "cambium-like" layer of small stromal cells with a prominent capillary vasculature, and a thick exterior wall composed of poorly formed fascicles of smooth muscle and thick-walled dysplastic blood vessels. Tumors show a distinct immunohistochemical profile and are often reactive for melanocytic markers (HMB-45 and Melan-A), as well as estrogen receptor and progesterone receptor. These tumors have an indolent clinical course, with no reports of progression or metastasis in reported cases thus far. PMID:27232352

  3. [Arachnoid cysts: Embriology and pathology].

    PubMed

    García-Conde, Mario; Martín-Viota, Lucia

    2015-01-01

    There is still great controversy surrounding the origin of the arachnoid cyst. The most accepted theory in the case of congenital cysts explains how they are formed from an anomalous development of the arachnoid membrane, which is unfolded allowing the accumulation of cerebrospinal fluid inside and creating a cyst. This theory seems to explain the origin of convexity and sylvian cistern arachnoid cysts, whereas those in other locations might be due to other mechanisms. In the anatomopathological analysis, the arachnoid cyst wall can be seen as having few differences from normal, although thickened due to an increase quantity of collagenous material. A description of the embryological development of the arachnoid layer and cyst formation is presented, describing the main anatomopathological findings. PMID:25866380

  4. Functional characterization of neuroendocrine regulation of branchial carbonic anhydrase induction in the euryhaline crab Callinectes sapidus.

    PubMed

    Mitchell, Reed T; Henry, Raymond P

    2014-12-01

    Carbonic anhydrase (CA) plays an essential role as a provider of counterions for Na(+)/H(+) and Cl(-)/HCO3 (-) exchange in branchial ionic uptake processes in euryhaline crustaceans. CA activity and gene expression are low in crabs acclimated to full-strength seawater, with transfer to low salinity resulting in large-scale inductions of mRNA and subsequent enzyme activity in the posterior ion-regulating gills (e.g., G7). In the green crab Carcinus maenas, CA has been shown to be under inhibitory neuroendocrine control by a putative hormone in the x-organ-sinus gland complex (XOSG), located in the eyestalk. This study characterizes the neuroendocrine regulation of CA induction in the blue crab Callinectes sapidus, a commonly used experimental organism for crustacean osmoregulation. In crabs acclimated to full-strength seawater, eyestalk ligation (ESL) triggered a 1.8- and 100-fold increase in CA activity and mRNA, respectively. Re-injection with eyestalk homogenates abolished increases in CA activity and fractionally reduced CA gene expression. ESL also enhanced CA induction by 33% after 96 h in crabs transferred to 15 ppt salinity. Injection of eyestalk homogenates into intact crabs transferred from 35 to 15 ppt diminished by 43% the CA induction stimulated by low salinity. These results point to the presence of a repressor hormone in the eyestalk. Separate injections of medullary tissue (MT) and sinus gland (SG), two components of the eyestalk, reduced salinity-stimulated CA activity by 22% and 49%, suggesting that the putative repressor is localized to the SG. Crabs injected with SG extract harvested from crabs acclimated to 5 ppt showed no decrease in CA activity, demonstrating that the hormone is down-regulated at low salinity. Our results show the presence in the XOSG of an inhibitory compound that regulates salinity-stimulated CA induction. PMID:25572216

  5. Osmoregulation and branchial plasticity after acute freshwater transfer in red drum, Sciaenops ocellatus.

    PubMed

    Watson, Caroline J; Nordi, Wiolene M; Esbaugh, Andrew J

    2014-12-01

    Red drum, Sciaenops ocellatus, is an estuarine-dependent fish species commonly found in the Gulf of Mexico and along the coast of the southeastern United States. This economically important species has demonstrated freshwater tolerance; however, the physiological mechanisms and costs related to freshwater exposure remain poorly understood. The current study therefore investigated the physiological response of red drum using an acute freshwater transfer protocol. Plasma osmolality, Cl⁻, Mg²⁺ and Ca²⁺ were all significantly reduced by 24h post-transfer; Cl⁻ and Mg²⁺ recovered to control levels by 7days post-transfer. No effect of transfer was observed on muscle water content; however, muscle Cl⁻ was significantly reduced. Interestingly, plasma and muscle Na⁺ content was unaffected by freshwater transfer. Intestinal fluid was absent by 24h post-transfer indicating cessation of drinking. Branchial gene expression analysis showed that both CFTR and NKCC1 exhibited significant down-regulation at 8 and 24h post-transfer, respectively, although transfer had no impact on NHE2, NHE3 or Na⁺, K⁺ ATPase (NKA) activity. These general findings are supported by immunohistochemical analysis, which revealed no apparent NKCC containing cells in the gills at 7days post transfer while NKA cells localization was unaffected. The results of the current study suggest that red drum can effectively regulate Na⁺ balance upon freshwater exposure using already present Na⁺ uptake pathways while also down-regulating ion excretion mechanisms. PMID:25152533

  6. Conjunctival cysts in anophthalmic orbits.

    PubMed Central

    Smit, T J; Koornneef, L; Zonneveld, F W

    1991-01-01

    Five out of 149 patients (3%) who received an intraorbital implant to prevent or treat the disfiguring symptoms associated with the postenucleation socket syndrome developed intraorbital conjunctival cysts. All five patients had received a secondary implant two 14 months previously. After excision of the cysts four patients required additional surgery for lack of conjunctiva and/or recurrent cyst formation. The clinical findings, mechanism of development, and management of this rare but serious complication of socket surgery are described. Images PMID:2043576

  7. Giant adrenal cyst displacing the right kidney

    PubMed Central

    Chodisetti, Subbarao; Boddepalli, Yogesh; Kota, Malakondareddy

    2016-01-01

    Adrenal cysts are rare and should be considered in the differential diagnosis of retroperitoneal cysts. We present a case of a huge adrenal cyst displacing the right kidney anteriorly toward the left side in a young female. PMID:26941503

  8. Syntax and Discourse in Near-Native French: Clefts and Focus

    ERIC Educational Resources Information Center

    Donaldson, Bryan

    2012-01-01

    This study examines aspects of the syntax-discourse interface in near-native French. Two cleft structures--"c'est" clefts and "avoir" clefts--are examined in experimental and spontaneous conversational data from 10 adult Anglophone learners of French and ten native speakers of French. "C'est" clefts mark focus, and "avoir" clefts introduce new…

  9. 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. PMID:26010214

  10. Definition of Critical Periods for Hedgehog Pathway Antagonist-Induced Holoprosencephaly, Cleft Lip, and Cleft Palate

    PubMed Central

    Heyne, Galen W.; Melberg, Cal G.; Doroodchi, Padydeh; Parins, Kia F.; Kietzman, Henry W.; Everson, Joshua L.; Ansen-Wilson, Lydia J.; Lipinski, Robert J.

    2015-01-01

    The Hedgehog (Hh) signaling pathway mediates multiple spatiotemporally-specific aspects of brain and face development. Genetic and chemical disruptions of the pathway are known to result in an array of structural malformations, including holoprosencephaly (HPE), clefts of the lip with or without cleft palate (CL/P), and clefts of the secondary palate only (CPO). Here, we examined patterns of dysmorphology caused by acute, stage-specific Hh signaling inhibition. Timed-pregnant wildtype C57BL/6J mice were administered a single dose of the potent pathway antagonist vismodegib at discrete time points between gestational day (GD) 7.0 and 10.0, an interval approximately corresponding to the 15th to 24th days of human gestation. The resultant pattern of facial and brain dysmorphology was dependent upon stage of exposure. Insult between GD7.0 and GD8.25 resulted in HPE, with peak incidence following exposure at GD7.5. Unilateral clefts of the lip extending into the primary palate were also observed, with peak incidence following exposure at GD8.875. Insult between GD9.0 and GD10.0 resulted in CPO and forelimb abnormalities. We have previously demonstrated that Hh antagonist-induced cleft lip results from deficiency of the medial nasal process and show here that CPO is associated with reduced growth of the maxillary-derived palatal shelves. By defining the critical periods for the induction of HPE, CL/P, and CPO with fine temporal resolution, these results provide a mechanism by which Hh pathway disruption can result in “non-syndromic” orofacial clefting, or HPE with or without co-occurring clefts. This study also establishes a novel and tractable mouse model of human craniofacial malformations using a single dose of a commercially available and pathway-specific drug. PMID:25793997

  11. [Cleft rhinoplasty, from primary to secondary surgery].

    PubMed

    Talmant, Jean-Claude; Talmant, Jean-Christian

    2014-12-01

    Despite fifty years of statistics, congresses, publications, the cleft nose remains an enigma to the great majority of cleft specialists. Most of the published papers give recipes to camouflage the cleft deformity, very few are concerned by the functional anatomy and its relation with facial growth. The complexity of the matter, the results frequently disappointing, the lack of awareness of the necessity of early nasal breathing, and the academic condemnation of any imperfect attempt to correct the nose at the time of the first operation have led to resignation. For the last forty years, we have been involved in a careful and obstinate research about the early correction of the cleft nose deformity. We wish to present our conclusions in this chapter with at least 17 years of follow-up. They are as following: in cleft patients the nasal cartilages are only deformed. We can achieve sub periosteal and sub perichondrial dissections by 6 months of age without being harmful for facial and nasal growth. Repositioning accurately the nasal structures is enough if we are able to control the healing process and prevent endonasal wound contraction. We have not to do any compromise and favor one function with regard to the others, nasal ventilation being the most important for a good facial growth. In a word, nasal pediatric surgery is necessary at the time of the first operation from 6 months of age and should be carried on with a double demand, aesthetic and functional. To achieve this goal, we must have a sound knowledge of the cleft nose deformity, of the adequate surgical techniques and of the logic chronology to reach the best result. The nose repair cannot be limited to the nasal cartilages. The whole nasal structure is concerned especially its bony framework, the width of which at the level of the piriform orifice and the nasal floor depends on the outcomes of any surgical step that it would relate to the lip, palate or alveolar closure. Interaction of all these factors

  12. Megalencephalic Leukoencephalopathy with Subcortical Cysts (MLC)

    MedlinePlus

    ... new treatments for the disease. Are there other names for Megalencephalic Leukoencephalopathy with subcortical Cysts (MLC)? Other names for Megalencephalic Leukoencephalopathy with subcortical Cysts (MLC) include: ...

  13. Otofaciocervical syndrome and metachondromatosis in a girl: Presentation of a novel association and remarks on clinical variability of branchial-arch disorders.

    PubMed

    Salinas-Torres, Victor M; Salinas-Torres, Rafael A

    2016-06-01

    Otofaciocervical syndrome (OFCS) is a rare disorder characterized by facial, ear, branchial, and musculoskeletal anomalies, along with hearing loss and mild intellectual disability. Clinically, its distinction from branchiootorenal syndrome can be difficult. To date, the coexistence of OFCS and metachondromatosis has not been reported. Here, we describe a sporadic patient with both OFCS and metachondromatosis. This novel association prompts us to do some remarks on the clinical variability of branchial-arch disorders; in fact, our observations are consistent with the highly variable expressivity of OFCS and illustrate the need of a more accurate characterization of these branchial-arch disorders. In the meantime, involvement of clavicles, scapulae and shoulders remains a distinctive feature of OFCS. PMID:27240490

  14. Pacific Craniofacial Team and Cleft Prevention Program.

    PubMed

    Tolarová, Marie M; Poulton, Donald; Aubert, Maryse M; Oh, HeeSoo; Ellerhorst, Thomas; Mosby, Terezie; Tolar, Miroslav; Boyd, Robert L

    2006-10-01

    There is no doubt modern genetics have greatly influenced our professional and personal lives during the last decade. Uncovering genetic causes of many medical and dental pathologies is helping to narrow the diagnosis and select a treatment plan that would provide the best outcome. Importantly, having an understanding of multifactorial etiology helps direct our attention toward prevention. We now understand much better our own health problems. In some cases, we can modify our lifestyle and diet in order to prevent "environmental factors" from triggering the mutated genes inherited from our parents. Good examples are diabetes and cardiovascular diseases. If we realize we might have inherited genes for cardiovascular problems from several ancestors who had heart attacks, we already know that these genes will make us only "susceptible" for disease. Those who exercise, watch one's weight, diet, and carefully monitor one's lifestyle will very likely--though possessing "susceptibility genes"--stay healthier and, maybe, will never experience any cardiovascular problems. In principle, the same applies for craniofacial anomalies, especially for nonsyndromic cleft lip and palate. One needs to understand genetic and environmental causes of nonsyndromic orofacial clefts in order to prevent them. With all this in mind, the Pacific Craniofacial Team and Cleft Prevention Program have been established at the Department of Orthodontics, University of the Pacific Arthur A. Dugoni School of Dentistry in San Francisco. A partnership with Rotaplast International, Inc., has made it possible for the faculty, orthodontic residents, and students to participate in 27 multidisciplinary cleft medical missions in underdeveloped and developing countries by donating professional and educational services, and, last but not least, by collecting valuable data and specimens to further research. A significant number of research studies, including 15 master of science theses, have been accomplished in

  15. FOXE1 association with both isolated cleft lip with or without cleft palate, and isolated cleft palate

    PubMed Central

    Moreno, Lina M.; Mansilla, Maria Adela; Bullard, Steve A.; Cooper, Margaret E.; Busch, Tamara D.; Machida, Junichiro; Johnson, Marla K.; Brauer, David; Krahn, Katherine; Daack-Hirsch, Sandy; L'Heureux, Jamie; Valencia-Ramirez, Consuelo; Rivera, Dora; López, Ana Maria; Moreno, Manuel A.; Hing, Anne; Lammer, Edward J.; Jones, Marilyn; Christensen, Kaare; Lie, Rolv T.; Jugessur, Astanand; Wilcox, Allen J.; Chines, Peter; Pugh, Elizabeth; Doheny, Kim; Arcos-Burgos, Mauricio; Marazita, Mary L.; Murray, Jeffrey C.; Lidral, Andrew C.

    2009-01-01

    Nonsyndromic orofacial clefts are a common complex birth defect caused by genetic and environmental factors and/or their interactions. A previous genome-wide linkage scan discovered a novel locus for cleft lip with or without cleft palate (CL/P) at 9q22–q33. To identify the etiologic gene, we undertook an iterative and complementary fine mapping strategy using family-based CL/P samples from Colombia, USA and the Philippines. Candidate genes within 9q22–q33 were sequenced, revealing 32 new variants. Concurrently, 397 SNPs spanning the 9q22–q33 2-LOD-unit interval were tested for association. Significant SNP and haplotype association signals (P = 1.45E − 08) narrowed the interval to a 200 kb region containing: FOXE1, C9ORF156 and HEMGN. Association results were replicated in CL/P families of European descent and when all populations were combined the two most associated SNPs, rs3758249 (P = 5.01E − 13) and rs4460498 (P = 6.51E − 12), were located inside a 70 kb high linkage disequilibrium block containing FOXE1. Association signals for Caucasians and Asians clustered 5′ and 3′ of FOXE1, respectively. Isolated cleft palate (CP) was also associated, indicating that FOXE1 plays a role in two phenotypes thought to be genetically distinct. Foxe1 expression was found in the epithelium undergoing fusion between the medial nasal and maxillary processes. Mutation screens of FOXE1 identified two family-specific missense mutations at highly conserved amino acids. These data indicate that FOXE1 is a major gene for CL/P and provides new insights for improved counseling and genetic interaction studies. PMID:19779022

  16. Neurenteric cysts of the spine.

    PubMed

    Savage, Jesse J; Casey, James N; McNeill, Ian T; Sherman, Jonathan H

    2010-01-01

    Neurenteric cysts account for 0.7-1.3% of spinal axis tumors. These rare lesions result from the inappropriate partitioning of the embryonic notochordal plate and presumptive endoderm during the third week of human development. Heterotopic rests of epithelium reminiscent of gastrointestinal and respiratory tissue lead to eventual formation of compressive cystic lesions of the pediatric and adult spine. Histopathological analysis of neurenteric tissue reveals a highly characteristic structure of columnar or cuboidal epithelium with or without cilia and mucus globules. Patients with symptomatic neurenteric cysts typically present in the second and third decades of life with size-dependent myelopathic and/or radicular signs. Magnetic resonance imaging and computed tomography are essential diagnostic tools for the delineation of cyst form and overlying osseous architecture. A variety of approaches have been employed in the treatment of neurenteric cysts each with a goal of total surgical resection. Although long-term outcome analyses are limited, data available indicate that surgical intervention in the case of neurenteric cysts results in a high frequency of resolution of neurological deficit with minimal morbidity. However, recurrence rates as high as 37% have been reported with incomplete resection secondary to factors such as cyst adhesion to surrounding structure and unclear dissection planes. Here we present a systematic review of English language literature from January 1966 to December 2009 utilizing MEDLINE with the following search terminology: neurenteric cyst, enterogenous cyst, spinal cord tumor, spinal dysraphism, intraspinal cyst, intramedullary cyst, and intradural cyst. In addition, the references of publications returned from the MEDLINE search criteria were surveyed in order to examine other pertinent reports. PMID:20890417

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

    PubMed Central

    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. PMID:26923345

  18. 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

    2016-03-01

    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. PMID:26923345

  19. Arachnoid cyst slit valves: the mechanism for arachnoid cyst enlargement.

    PubMed

    Halani, Sameer H; Safain, Mina G; Heilman, Carl B

    2013-07-01

    Arachnoid cysts are common, accounting for approximately 1% of intracranial mass lesions. Most are congenital, clinically silent, and remain static in size. Occasionally, they increase in size and produce symptoms due to mass effect or obstruction. The mechanism of enlargement of arachnoid cysts is controversial. One-way slit valves are often hypothesized as the mechanism for enlargement. The authors present 4 cases of suprasellar prepontine arachnoid cysts in which a slit valve was identified. The patients presented with hydrocephalus due to enlargement of the cyst. The valve was located in the arachnoid wall of the cyst directly over the basilar artery. The authors believe this slit valve was responsible for the net influx of CSF into the cyst and for its enlargement. They also present 1 case of an arachnoid cyst in the middle cranial fossa that had a small circular opening but lacked a slit valve. This cyst did not enlarge but surgery was required because of rupture and the development of a subdural hygroma. One-way slit valves exist and are a possible mechanism of enlargement of suprasellar prepontine arachnoid cysts. The valve was located directly over the basilar artery in each of these cases. Caudad-to-cephalad CSF flow during the cardiac cycle increased the opening of the valve, whereas cephalad-to-caudad CSF flow during the remainder of the cardiac cycle pushed the slit opening against the basilar artery and decreased the size of the opening. Arachnoid cysts that communicate CSF via circular, nonslit valves are probably more likely to remain stable. PMID:23662935

  20. Cerebral arachnoid cysts in children

    PubMed Central

    Harrison, M. J. G.

    1971-01-01

    The case histories of 14 children are described in which hydrocephalus was found on investigation to be associated with a cyst of the posterior fossa or subarachnoid cisterns. The neuroradiological and histological findings are described. The cysts are considered to be developmental in origin. Their recognition and management are discussed. Images PMID:5315217

  1. Epidermal and urethroid penile cyst.

    PubMed

    Claudy, A L; Dutoit, M; Boucheron, S

    1991-01-01

    The authors describe a 74-year-old man who presented with a 2-cm nodule on the ventral face of the penis, showing histologically a cyst lined by both epidermal and urethroid epithelium. The authors discuss the various histological forms of raphe median cysts of the penis. PMID:1676220

  2. Epidermal cyst of median raphe.

    PubMed

    LaNasa, J A

    1976-10-01

    Cysts of the penis are rare and references to them in standard textbooks are sketchy. A case report of a congenital epidermal cyst of the median raphe of the penis is presented; therapy involved excision of the mass. Review of the literature is given. PMID:973298

  3. Median Facial Cleft in Amniotic Band Syndrome

    PubMed Central

    Das, Debabrata; Das, Gobinda; Gayen, Sibnath; Konar, Arpita

    2011-01-01

    Amniotic band syndrome manifests at birth with a variety of malformations ranging from constriction ring to defects incompatible to life, in various parts of the body. Although some theories have been proposed for the development of this syndrome, the exact cause remains unknown. The median facial cleft is an extremely rare manifestation of amniotic band syndrome with a relative paucity of reports available in the literature. Here, we report one such case. PMID:21731335

  4. Multiple infected cerebral hydatid cysts.

    PubMed

    Gana, R; Skhissi, M; Maaqili, R; Bellakhdar, F

    2008-05-01

    We report an unusual patient with multiple infected cerebral hydatid cysts. A 20-year-old man presented with a 2-month history of headache and progressive left-sided hempiparesis. A cerebral CT scan showed a large and heterogeneous parieto-occipital lesion. During surgery an infected hydatid cyst was discovered with multiple daughter vesicles. Post-operatively the patient was treated with albendazol, cefotaxime and metronidazole. The clinical course was good with total recovery of the hemiparesis. A follow-up CT scan showed persistence of some small deep-seated cysts. Multiple infected cerebral hydatid cyst is uncommon and can be confused with other cystic brain lesions. The aim of surgery is to remove the cyst unruptured and this should be followed by antihelminthic and antibiotic treatment in order to avoid recurrences. PMID:18342511

  5. Odontogenic Keratocyst Mimicking Paradental Cyst

    PubMed Central

    Borgonovo, Andrea Enrico; Bernardini, Luigi; Francinetti, Paola

    2014-01-01

    Objective. The aim of this paper is to present an uncommon clinical and radiographic aspect of odontogenic keratocyst (OKC) mimicking paradental cyst. Methods. A 32-year-old female patient showed a well-delimited radiolucent lesion connected with the root of the left third molar with close anatomical relationship with the mandibular canal. The clinical, radiographic, and anamnestic features lead us to diagnose a paradental cyst that was treated by enucleation after extraction of the partially impacted tooth. Results. Histological analysis showed typical histological features of PKC such as the presence of a lining of stratified squamous epithelium with a well-defined basal layer of palisading columnar of cuboidal cells. Conclusion. Initial X-ray analysis and the position of the lesion related to the third mandibular tooth caused us to mistakenly diagnose a paradental cyst. We were only able to identify the cyst as an PKC rather than a paradental cyst after histological analysis. PMID:25114809

  6. Evaluation of impacted Brazilian estuaries using the native oyster Crassostrea rhizophorae: Branchial carbonic anhydrase as a biomarker.

    PubMed

    Azevedo-Linhares, Maristela; Freire, Carolina A

    2015-12-01

    In this study, we investigated the use of branchial carbonic anhydrase activity in a sessile filter feeding species, the oyster Crassostrea rhizophorae, as a biomarker. The oysters were collected in three human impacted Brazilian estuaries, following a crescent latitudinal gradient: in Pernambuco state (Itamaracá), in Espírito Santo state (Piraquê), and in Paraná state (Paranaguá), in August/2003 (Winter in the southern hemisphere) and February/2004 (Summer). Three sites were chosen in each estuary for oyster sampling: Reference (R), Contaminated 1 (C1, close to industrial/harbor contamination), and Contaminated 2 (C2, near to sewage discharges). Comparing to values in oysters sampled in reference sites, there was apparent inhibition in carbonic anhydrase activity (CAA) in gills of oysters from C1 of Itamaracá and from C2 of Piraquê, both cases in Summer. On the other hand, increased CAA was noted in C2 oysters of Itamaracá in winter, and of Paranaguá, in both seasons. Branchial CAA in C. rhizophorae was thus very responsive to coastal contamination. Data are consistent with its usefulness as a supporting biomarker for inexpensive and rapid analysis in the assessment of estuaries using a sessile osmoconformer species, but preferably allied to other biomarkers and with knowledge on the suite of contaminants present. PMID:26410193

  7. Effects of sea water and stanniectomy on branchial Ca(2+) handling and drinking rate in eel (Anguilla anguilla L.).

    PubMed

    van der Heijden AJ; Verbost; Bijvelds; Atsma; Wendelaar Bonga SE; Flik

    1999-01-01

    We examined the effects of seawater adaptation and extirpation of the Stannius corpuscles on branchial Ca(2+) flows, gill plasma membrane Ca(2+) transporters and drinking rate of European eels, Anguilla anguilla. Transepithelial Ca(2+) inflow in the gills increased 2 weeks after transfer of the eels from fresh water to sea water and after stanniectomy. Neither of these treatments changed the membrane density or the affinity of the Ca(2+)-extrusion mechanisms (Ca(2+)-ATPase and Na(+)/Ca(2+)-exchanger) in the gill cells, as measured in basolateral plasma membrane vesicles. We conclude that the increase in the Ca(2+)-transporting capacity observed in the gills of fish exposed to the larger transepithelial Ca(2+) fluxes, resulting from exposure to sea water or stanniectomy, involves an increase in number and/or size of the Ca(2+)-transporting cells, but not in the membrane density of Ca(2+) transporters. Branchial Ca(2+) outflow was higher in stanniectomised than in sham-operated fish. Changes in electrochemical driving forces as well as plasma stanniocalcin or teleocalcin levels may be the basis for the observed differences. Stanniectomy enhanced drinking in stanniectomised eels. Drinking was not further affected by transfer to sea water. These observations suggest that the corpuscles of Stannius are involved in the water balance. PMID:10460737

  8. Simple Correction of the Congenital Cleft Earlobe.

    PubMed

    Karaci, Selman; Köse, Rüştü

    2016-07-01

    The appearance of the ear is an important component of the facial characteristics. Lower auricular malformations are less frequent than total or upper auricular malformations. The patients are affected unilaterally in general. Cleft earlobe is frequently encountered among earlobe anomalies. The presented case may be classified as longitudinal type according to Kitayama (Jpn J Plast Reconstr Surg 11:663-670, 1980). Many of the correction methods may lead to patient discomfort due to possible conspicuous scar. The patient was a 5 year old girl. In the presented case, a simple method has been performed. Satisfactory outcome is achieved. As a simple method applying longitudinal division and rotation procedure does not have marginal excision. Furthermore there is no additional incision outside the cleft margin. Local flap and graft are not applied. Conservative approach was maintained with respect to scar occurrence. This method is not favourable in the case of acquired split earlobe deformities due to the wide cleft surface. Postoperative 3rd-month appearance demonstrated adequate correction. PMID:27408464

  9. The Evolution of Human Genetic Studies of Cleft Lip and Cleft Palate

    PubMed Central

    Marazita, Mary L.

    2013-01-01

    Orofacial clefts (OFCs)—primarily cleft lip and cleft palate—are among the most common birth defects in all populations worldwide, and have notable population, ethnicity, and gender differences in birth prevalence. Interest in these birth defects goes back centuries, as does formal scientific interest; scientists often used OFCs as examples or evidence during paradigm shifts in human genetics, and have also used virtually every new method of human genetic analysis to deepen our understanding of OFC. This review traces the evolution of human genetic investigations of OFC, highlights the specific insights gained about OFC through the years, and culminates in a review of recent key OFC genetic findings resulting from the powerful tools of the genomics era. Notably, OFC represents a major success for genome-wide approaches, and the field is poised for further breakthroughs in the near future. PMID:22703175

  10. Corticosteroid use during pregnancy and risk of orofacial clefts

    PubMed Central

    Hviid, Anders; Mølgaard-Nielsen, Ditte

    2011-01-01

    Background The association between the risk of orofacial clefts in infants and the use of corticosteroids during pregnancy is unclear from the available evidence. We conducted a nationwide cohort study of all live births in Denmark over a 12-year period. Methods We collected data on all live births in Denmark from Jan. 1, 1996, to Sept. 30, 2008. We included live births for which information was available from nationwide health registries on the use of corticosteroids during pregnancy, the diagnosis of an orofacial cleft and possible confounders. Results There were 832 636 live births during the study period. Exposure to corticosteroids during the first trimester occurred in 51 973 of the pregnancies. A total of 1232 isolated orofacial clefts (i.e., cleft lip, cleft palate, or cleft lip and cleft palate) were diagnosed within the first year of life, including 84 instances in which the infant had been exposed to corticosteroids during the first trimester of pregnancy. We did not identify any statistically significant increased risk of orofacial clefts associated with the use of corticosteroids: cleft lip with or without cleft palate, prevalence odds ratio (OR) 1.05 (95% confidence interval [CI] 0.80–1.38]; cleft palate alone, prevalence OR 1.23 (95% CI 0.83–1.82). Odds ratios for risk of orofacial clefts by method of delivery (i.e., oral, inhalant, nasal spray, or dermatologic and other topicals) were consistent with the overall results of the study and did not display significant heterogeneity, although the OR for cleft lip with or without cleft palate associated with the use of dermatologic corticosteroids was 1.45 (95% CI 1.03–2.05). Interpretation Our results add to the safety information on a class of drugs commonly used during pregnancy. Our study did not show an increased risk of orofacial clefts with the use of corticosteroids during pregnancy. Indepth investigation of the pattern of association between orofacial clefts and the use of dermatologic

  11. Presurgical Dentofacial Orthopedic Management of the Cleft Patient.

    PubMed

    Smith, Kevin S; Henry, Byron T; Scott, Michelle A

    2016-05-01

    Over the last decade, presurgical orthopedic molding for the patient with cleft lip and palate has become much more common; it is even reasonable to assume it may be the standard of care for those wide unilateral and bilateral clefts with substantial dentofacial deformities. In 2013, there was a comparative study of nasoalveolar molding methods, comparing the Grayson-NAM device and DynaCleft. The results showed the 2 to be equivocal with both methods significantly reducing the cleft width and improving the nasal asymmetry. PMID:27150304

  12. Rehabilitation of Cleft Palate: Parents and Professionals, A Unifying Model

    ERIC Educational Resources Information Center

    Pannbacker, Mary; Schneiderman, Carl

    1977-01-01

    Described are commonalities and differences between parents of children with cleft palate and rehabilitation professionals, and offered are suggestions to increase communication and joint effectiveness. (DB)

  13. Ectrodactyly-ectodermal dysplasia-cleft lip and palate syndrome.

    PubMed

    Dhar, Reema Sharma; Bora, Amitava

    2014-01-01

    Ectrodactyly-ectodermal dysplasia-cleft (EEC) syndrome is an autosomal dominant disorder characterized by the triad of ectrodactyly-ectodermal dysplasia, and facial clefting along with some associated features. Presence of all the three major features in a single individual is extremely rare. We report a case of 4 year 11 months old child with EEC syndrome having ectodermal dysplasia-cleft lip and cleft palate and ectrodactyly with some associated features. Clinical features, diagnosis and role of a dentist in the multidisciplinary treatment approach have been elaborated in this case report. PMID:25231046

  14. Cleft Care UK study. Part 5: child psychosocial outcomes and satisfaction with cleft services

    PubMed Central

    Waylen, A; Ness, A R; Wills, A K; Persson, M; Rumsey, N; Sandy, J R

    2015-01-01

    Structured Abstract Objectives To describe the impact of cleft service centralization on parental perceptions of child outcomes and satisfaction with care from the Cleft Care UK (CCUK) study and compare them to the Clinical Standards Advisory Group (CSAG) study that took place 15 years earlier. Setting and Sample Population A subgroup of respondents from a UK multicentre cross-sectional study (CCUK) of 5-year-olds born with non-syndromic unilateral cleft lip and palate. Materials and Methods Data on parents’ perceptions of child self-confidence and their satisfaction with treatment outcomes and service provision were collected via self-report questionnaires. Data were compared with findings from the 1998 CSAG study. Results Fewer parents in the CCUK study perceived their children as having poor self-confidence than in the 1998 CSAG study (8 and 19%, respectively). At least 81% of parents report satisfaction with the child’s facial features after surgery and 98% report being satisfied with the care received. These results are similar to those reported in 1998. There is no evidence of an adverse impact on families’ ability to attend appointments at the cleft clinic following centralization. Levels of reported problems (around 30%) with attendance were similar to those reported by CSAG. Conclusion Centralization of cleft services appears to have improved parental perceptions of some child outcomes but has made little difference to already high levels of parental satisfaction with cleft care services. Centralization is not associated with an increase in the proportion of families who find it difficult to attend appointments. PMID:26567855

  15. Penile Epidermal Cyst: A Case Report.

    PubMed

    Kumaraguru, Veerapandian; Prabhu, Ravi; Kannan, Narayanasamy Subbaraju

    2016-05-01

    Epidermal cysts also known as epidermoid cysts, is one of the common benign tumours presenting anywhere in the body. However, epidermal cyst in the penis is very rare. This condition in children is usually congenital due to abnormal embryologic closure of the median raphe; hence, it is termed as median raphe cysts (MRCs). Penile epidermal cysts may occur in adults following trauma or surgery due to epidermal elements being trapped within closed space. During wound healing, trapped squamous epithelium, undergoing keratinisation leads to cyst formation. Here, we report a rare case of patient with a penile epidermoid cyst whose main complaints was discomfort during coitus. PMID:27437298

  16. Penile Epidermal Cyst: A Case Report

    PubMed Central

    Kumaraguru, Veerapandian; Prabhu, Ravi

    2016-01-01

    Epidermal cysts also known as epidermoid cysts, is one of the common benign tumours presenting anywhere in the body. However, epidermal cyst in the penis is very rare. This condition in children is usually congenital due to abnormal embryologic closure of the median raphe; hence, it is termed as median raphe cysts (MRCs). Penile epidermal cysts may occur in adults following trauma or surgery due to epidermal elements being trapped within closed space. During wound healing, trapped squamous epithelium, undergoing keratinisation leads to cyst formation. Here, we report a rare case of patient with a penile epidermoid cyst whose main complaints was discomfort during coitus. PMID:27437298

  17. Artemia cyst production in Russia

    NASA Astrophysics Data System (ADS)

    Litvinenko, Liudmila I.; Litvinenko, Aleksandr I.; Boiko, Elena G.; Kutsanov, Kirill

    2015-11-01

    In Western Siberia (Russia) there are about 100 Artemia lakes with total area over 1 600 km2. Geographically these lakes are located between 51°-56°N and 61°-82°E. In general these lakes are shallow (depth less than 1.5 m), small or medium size (0.1 to 10 km2); they are chloride; their total salinity is from 40 to 250 g/L. The harvesting of cysts per year is only in 20-40 lakes. In Russia 550 tons of dry Artemia cysts (14%-18% of the world production) were harvested annually. This includes about 350 tons in the Altai region and 200 tons in other regions. During our regular 20-year study period the cyst harvest was: 95 tons in Kurgan; 65 tons in Omsk, 20 tons in Novosibirsk, 20 tons in Tyumen. Ways of increasing cyst harvest in Russia are considered in this article. During the last 30 years the harvest of cysts in Russia has increased from 7-20 to 500-600 tons. A significant influence of dryness of the year was found on productivity in selected lakes, but taken for all the lakes together, the relationship was not significant. The optimal salinity for productivity of cysts in the lakes was determined. Analysis of productivity of the lakes and the harvesting results showed that the stocks of cysts are underutilized by approximately 1.7 times.

  18. Spinal perineurial and meningeal cysts.

    PubMed

    Tarlov, I M

    1970-12-01

    Perineurial cysts may be responsible for clinical symptoms and a cure effected by their removal. They do not fill on initial myelography but may fill with Pantopaque some time, days or weeks, after Pantopaque has been instilled into the subarachnoid space. Perineurial cysts arise at the site of the posterior root ganglion. The cyst wall is composed of neural tissue. When initial myelography fails to reveal an adequate cause for the patient's symptoms and signs referable to the caudal nerve roots, then about a millilitre of Pantopaque should be left in the canal for delayed myelography which may later reveal a sacral perineurial cyst or, occasionally, a meningeal cyst. Meningeal diverticula occur proximal to the posterior root ganglia and usually fill on initial myelography. They are in free communication with the subarachnoid space and are rarely in my experience responsible for clinical symptoms. Meningeal diverticula and meningeal cysts appear to represent a continuum. Pantopaque left in the subarachnoid space may convert a meningeal diverticulum into an expanding symptomatic meningeal cyst, as in the case described. Many cases described as perineurial cysts represent abnormally long arachnoidal prolongations over nerve roots or meningeal diverticula. In general, neither of the latter is of pathological significance. Perineurial, like meningeal cysts and diverticula, may be asymptomatic. They should be operated upon only if they produce progressive or disabling symptoms or signs clearly attributable to them. When myelography must be done, and this should be done only as a preliminary to a probable necessary operation, then patient effort should be made to remove the Pantopaque. PMID:5531903

  19. The role of an ancestral hyperpolarization-activated cyclic nucleotide-gated K+ channel in branchial acid-base regulation in the green crab, Carcinus maenas.

    PubMed

    Fehsenfeld, Sandra; Weihrauch, Dirk

    2016-03-01

    Numerous electrophysiological studies on branchial K(+) transport in brachyuran crabs have established an important role for potassium channels in osmoregulatory ion uptake and ammonia excretion in the gill epithelium of decapod crustaceans. However, hardly anything is known of the actual nature of these channels in crustaceans. In the present study, the identification of a hyperpolarization-activated cyclic nucleotide-gated potassium channel (HCN) in the transcriptome of the green crab Carcinus maenas and subsequent performance of quantitative real-time PCR revealed the ubiquitous expression of this channel in this species. Even though mRNA expression levels in the cerebral ganglion were found to be approximately 10 times higher compared with all other tissues, posterior gills still expressed significant levels of HCN, indicating an important role for this transporter in branchial ion regulation. The relatively unspecific K(+)-channel inhibitor Ba(2+), as well as the HCN-specific blocker ZD7288, as applied in gill perfusion experiments and electrophysiological studies employing the split gill lamellae revealed the presence of at least two different K(+)/NH4(+)-transporting structures in the branchial epithelium of C. maenas. Furthermore, HCN mRNA levels in posterior gill 7 decreased significantly in response to the respiratory or metabolic acidosis that was induced by acclimation of green crabs to high environmental PCO2 and ammonia, respectively. Consequently, the present study provides first evidence that HCN-promoted NH4(+) epithelial transport is involved in both branchial acid-base and ammonia regulation in an invertebrate. PMID:26787479

  20. Oxygen utilization and the branchial pressure gradient during ram ventilation of the shortfin mako, Isurus oxyrinchus: is lamnid shark-tuna convergence constrained by elasmobranch gill morphology?

    PubMed

    Wegner, Nicholas C; Lai, N Chin; Bull, Kristina B; Graham, Jeffrey B

    2012-01-01

    Ram ventilation and gill function in a lamnid shark, the shortfin mako, Isurus oxyrinchus, were studied to assess how gill structure may affect the lamnid-tuna convergence for high-performance swimming. Despite differences in mako and tuna gill morphology, mouth gape and basal swimming speeds, measurements of mako O(2) utilization at the gills (53.4±4.2%) and the pressure gradient driving branchial flow (96.8±26.1 Pa at a mean swimming speed of 38.8±5.8 cm s(-1)) are similar to values reported for tunas. Also comparable to tunas are estimates of the velocity (0.22±0.03 cm s(-1)) and residence time (0.79±0.14 s) of water though the interlamellar channels of the mako gill. However, mako and tuna gills differ in the sites of primary branchial resistance. In the mako, approximately 80% of the total branchial resistance resides in the septal channels, structures inherent to the elasmobranch gill that are not present in tunas. The added resistance at this location is compensated by a correspondingly lower resistance at the gill lamellae accomplished through wider interlamellar channels. Although greater interlamellar spacing minimizes branchial resistance, it also limits lamellar number and results in a lower total gill surface area for the mako relative to tunas. The morphology of the elasmobranch gill thus appears to constrain gill area and, consequently, limit mako aerobic performance to less than that of tunas. PMID:22162850

  1. The effects of cortisoland actinomycin D injections on choloride cells and branchial N+---K+-ATPase in rainbow trout (Salmo gairdneri)

    USGS Publications Warehouse

    Eib, D.W.; Hossner, K.L.

    1985-01-01

    Injections of cortisol, actinomycin D, or combined administration of the hormone and the antiobiotic did not effect rainbow trout (Salmo gairdneri) branchial Na+K+-ATPase activity. Numbers of chloride cells also did not change following cortisol and actinomycin D treatment. These results are discussed in light of a similar report concerning Atlantic salmon (Salmo salar).

  2. [Tarlov cyst and symptomatic bladder disfuction].

    PubMed

    Ruibal Moldes, M; Sánchez Rodríguez-Losada, J; López García, D; Casas Agudo, V; Janeiro País, J M; González Martín, M

    2008-01-01

    Tarlov cysts or perineural cyst are lesions of the nerve roots located at the sacral level and uncertain aetiology. Most of these cysts remain asymptomatic with no clinical relevance. The symptomatic cysts are uncommon and the usual symptoms are pain or radiculopathy. We report the case of a 53-year-old woman witha symptomatic cyst (with a history of frequency and urgency syndrom), that disappears after surgery. PMID:19143297

  3. [Microsurgical treatment of intracraneal arachnoid cysts].

    PubMed

    Saura Rojas, J Enrique; Horcajadas Almansa, Ángel; Ros López, Bienvenido

    2016-01-01

    Craniotomy and fenestration of membranes is one of the main treatment options for symptomatic arachnoid cysts. Open surgery advantages include, direct inspection of the cyst, biopsy sampling, fenestration in multilocular cysts and, in certain locations, cyst communication to basal cisterns. The aim of this paper is to review the advantages and disadvantages of this treatment modality for arachnoid cysts taking into account the different anatomical locations. PMID:25891259

  4. [Neurosurgical aspects of arachnoid cysts].

    PubMed

    Maier, F; Steube, D; Hamm, K D

    1986-01-01

    After an analysis of the patients treated in the last five years, a report is given on 9 cases of arachnoid cysts as a rare form of intracranial space occupation. The etiology of the arachnoid cysts has not been fully cleared up yet, but the semipermeability of the cyst membrane appears to be an important pathogenetic factor. Today, the diagnosis of the disease is verified by CT techniques. In case of the occurrence of clinical symptoms the treatment should always be carried out in the form of an operation. PMID:3564765

  5. Splenic hydatid cyst attacking retroperitoneum.

    PubMed

    Kaya, Bülent; Uçtum, Yalım; Kutanış, Rıza

    2010-01-01

    Hydatid disease most commonly affects the liver and lungs but no organ is immune. Splenic hydatid cyst is a rare clinical entity. Although the patients are usually asymptomatic, the disease may present with secondary infection, adhesion to adjacent organs with fistulisation or rupture into abdominal cavity. We present a 67 year old women with splenic hydatid cyst. Severe adhesions and tumorlike growth were found in the retroperitoneal region. To our knowledge, retroperitoneal invasion with a splenic hydatid cyst is a very rare clinical condition. Total splenectomy was performed without complication. PMID:21391192

  6. Nasopalatine canal cyst: often missed

    PubMed Central

    Srivastava, Saurabh; Misra, Neeta; Agarwal, Rashmi; Pandey, Praveen

    2013-01-01

    Nasopalatine canal cyst may occur within the nasopalatine canal or in the soft tissues of the palate, at the opening of the canal, where it is called the ‘cyst of the palatine papilla’. These are normally asymptomatic, constituting casual radiological findings. We present a case report of a male patient with infected nasopalatine canal cyst seen clinically as swelling over palate. Radiographic interpretation should be thoroughly performed for maxillary anterior region and any occlusal radiograph in routine radiography to screen this entity and must be distinguished from other maxillary anterior radiolucencies by the clinician. PMID:23536642

  7. Choroid plexus cysts and aneuploidy.

    PubMed Central

    Peleg, D; Yankowitz, J

    1998-01-01

    The association of choroid plexus cysts with fetal aneuploidy, particularly trisomy 18, was first noted in 1986. Through the years there have been numerous reports on this subject, but no consensus has been reached with regard to chromosomal risk. In this review, we attempt to summarise published reports on second trimester choroid plexus cysts, with an emphasis on the strengths and weaknesses of each report. Based on these reports, additional malformations are a significant risk factor for aneuploidy and an indication for determination of fetal karyotype. The management of isolated choroid plexus cysts remains controversial. PMID:9678699

  8. Renal hydatid cyst treatment: retroperitoneoscopic "closed cyst" pericystectomy.

    PubMed

    Ozden, Ender; Bostanci, Yakup; Mercimek, Mehmet Necmettin; Yakupoglu, Yarkin Kamil; Yilmaz, Ali Faik; Sarıkaya, Saban

    2011-03-01

    Cystic hydatid disease is an endemic disease caused by the larval form of Echinococcus spp. Isolated renal involvement is extremely rare. The treatment methods for renal hydatid disease require some form of intervention, ranging from traditional open techniques to laparoscopic techniques. Herein, we present a large hydatid cyst in the lower pole of the left kidney in a 43-year-old male patient who was treated by the "closed cyst" method via the retroperitoneal laparoscopic approach to prevent soiling of the peritoneal cavity. To our knowledge, this is the first case of a renal hydatid cyst treated by preserving the renal parenchyma by pericystectomy via the retroperitoneoscopic laparoscopic approach in an adult patient. No complications occurred during the perioperative and postoperative periods. After 9 months of follow up, the patient was asymptomatic with no evidence of clinical recurrence. Retroperitoneoscopic laparoscopic closed cyst pericystectomy can be an alternative minimally invasive treatment technique for the treatment of renal hydatid disease. PMID:21226768

  9. (Uncommon) Mechanisms of Branchial Ammonia Excretion in the Common Carp (Cyprinus carpio) in Response to Environmentally Induced Metabolic Acidosis.

    PubMed

    Wright, Patricia A; Wood, Chris M; Hiroi, Junya; Wilson, Jonathan M

    2016-01-01

    Freshwater fishes generally increase ammonia excretion in acidic waters. The new model of ammonia transport in freshwater fish involves an association between the Rhesus (Rh) protein Rhcg-b, the Na(+)/H(+) exchanger (NHE), and a suite of other membrane transporters. We tested the hypothesis that Rhcg-b and NHE3 together play a critical role in branchial ammonia excretion in common carp (Cyprinus carpio) chronically exposed to a low-pH environment. Carp were exposed to three sequential environmental treatments-control pH 7.6 water (24 h), pH 4.0 water (72 h), and recovery pH 7.6 water (24 h)-or in a separate series were simply exposed to either control (72 h) or pH 4.0 (72 h) water. Branchial ammonia excretion was increased by ∼2.5-fold in the acid compared with the control period, despite the absence of an increase in the plasma-to-water partial pressure NH3 gradient. Alanine aminotransferase activity was higher in the gills of fish exposed to pH 4 versus control water, suggesting that ammonia may be generated in gill tissue. Gill Rhcg-b and NHE3b messenger RNA levels were significantly elevated in acid-treated relative to control fish, but at the protein level Rhcg-b decreased (30%) and NHE3b increased (2-fold) in response to water of pH 4.0. Using immunofluorescence microscopy, NHE3b and Rhcg-b were found to be colocalized to ionocytes along the interlamellar space of the filament of control fish. After 72 h of acid exposure, Rhcg-b staining almost disappeared from this region, and NHE3b was more prominent along the lamellae. We propose that ammoniagenesis within the gill tissue itself is responsible for the higher rates of branchial ammonia excretion during chronic metabolic acidosis. Unexpectedly, gill Rhcg-b does not appear to be important in gill ammonia transport in low-pH water, but the strong induction of NHE3b suggests that some NH4(+) may be eliminated directly in exchange for Na(+). These findings contrast with previous studies in larval zebrafish

  10. A Glance at Methods for Cleft Palate Repair

    PubMed Central

    Tavakolinejad, Sima; Ebrahimzadeh Bidskan, Alireza; Ashraf, Hami; Hamidi Alamdari, Daryoush

    2014-01-01

    Context: Cleft palate is the second most common birth defect and is considered as a challenge for pediatric plastic surgeons. There is still a general lack of a standard protocol and patients often require multiple surgical interventions during their lifetime along with disappointing results. Evidence Acquisition: PubMed search was undertaken using search terms including 'cleft palate repair', 'palatal cleft closure', 'cleft palate + stem cells', 'cleft palate + plasma rich platelet', 'cleft palate + scaffold', 'palatal tissue engineering', and 'bone tissue engineering'. The found articles were included if they defined a therapeutic strategy and/or assessed a new technique. Results: We reported a summary of the key-points concerning cleft palate development, the genes involving this defect, current therapeutic strategies, recently novel aspects, and future advances in treatments for easy and fast understanding of the concepts, rather than a systematic review. In addition, the results were integrated with our recent experience. Conclusions: Tissue engineering may open a new window in cleft palate reconstruction. Stem cells and growth factors play key roles in this field. PMID:25593724

  11. COMPREHENSIVE EVALUATIVE TECHNIQUES FOR THE CHILD WITH A CLEFT PALATE.

    ERIC Educational Resources Information Center

    BENSEN, JACK F.; WHITE, FRAZER D.

    A MULTIDISCIPLINARY APPROACH TO CHILDREN WITH CLEFT PALATES IS DESCRIBED. THE SOUTH FLORIDA CLEFT PALATE CLINIC, REPRESENTING NINE PROFESSIONAL SPECIALTIES, MEETS WEEKLY TO SEE SIX OR SEVEN CASES. SPEECH PERFORMANCE IS RECORDED ON SIX DIAGNOSTIC, DATA COLLECTING FORMS WHICH PROVIDE A BASIS FOR RECORDING CLINICAL JUDGMENTS. PROGNOSIS AND…

  12. Feeding Techniques for Children Who Have Cleft Lip and Palate.

    ERIC Educational Resources Information Center

    Klein, Marsha Dunn

    This pamphlet on feeding techniques for children who have cleft lip and palate emphasizes the role of the parent as part of a team involving many specialists. The pamphlet begins with explanations of complete and incomplete separations of the lip, unilateral and bilateral cleft lips, corrective surgical procedures, etc. The importance of weight…

  13. Single-Word Intelligibility in Speakers with Repaired Cleft Palate

    ERIC Educational Resources Information Center

    Whitehill, Tara; Chau, Cynthia

    2004-01-01

    Many speakers with repaired cleft palate have reduced intelligibility, but there are limitations with current procedures for assessing intelligibility. The aim of this study was to construct a single-word intelligibility test for speakers with cleft palate. The test used a multiple-choice identification format, and was based on phonetic contrasts…

  14. Early Speech Production of Children with Cleft Palate.

    ERIC Educational Resources Information Center

    Estrem, Theresa; Broen, Patricia A.

    1989-01-01

    The study comparing word-initial target phonemes and phoneme production of five toddlers with cleft palate and five normal toddlers found that the cleft palate children tended to target more words with word-initial nasals, approximants, and vowels and fewer words with word-initial stops, fricatives, and affricates than normal children. (Author/DB)

  15. Smile Train: The ascendancy of cleft care in India

    PubMed Central

    Singh, Subodh Kumar

    2009-01-01

    Though India has an estimated population of one million untreated cleft patients, facilities for its treatment have been limited and are not evenly distributed across the country. Furthermore, a paucity of committed cleft surgeons in fewer hospitals to provide quality surgical treatment to these patients, poverty, illiteracy, superstitions and poor connectivity in some remote regions severely limit the chances of an average cleft lip patient born in India from receiving rational and effective comprehensive treatment for his/her malady. The Smile Train Project with its singular focus on cleft patients started its philanthropic activities in India in the year 2000. It made hospitals and included clefts surgeon equal partners in this programme and helped them treat as many cleft patients as they possibly could. The Project encouraged improvement of the training and infrastructure in various centres across the length and breadth of the region. The Project received an unprecedented success in terms of growth of number of centres, cleft surgeons and quantum of cleft patients reporting for treatment. The G S Memorial Hospital is one such partner hospital. It started innovative outreach programmes and took a holistic view of the needs of these patients and their families. With the support of the Smile Train, it has not only succeeded in providing treatment to more than 14,500 patients in 5 years, but has also devised innovative outreach programmes and seamlessly incorporated salient changes in the hospital system to suit the needs of the target population. PMID:19884676

  16. Aneurysmal bone cyst.

    PubMed

    Rapp, Timothy B; Ward, James P; Alaia, Michael J

    2012-04-01

    Aneurysmal bone cysts are rare skeletal tumors that most commonly occur in the first two decades of life. They primarily develop about the knee but may arise in any portion of the axial or appendicular skeleton. Pathogenesis of these tumors remains controversial and may be vascular, traumatic, or genetic. Radiographic features include a dilated, radiolucent lesion typically located within the metaphyseal portion of the bone, with fluid-fluid levels visible on MRI. Histologic features include blood-filled lakes interposed between fibrous stromata. Differential diagnosis includes conditions such as telangiectatic osteosarcoma and giant cell tumor. The mainstay of treatment is curettage and bone graft, with or without adjuvant treatment. Other management options include cryotherapy, sclerotherapy, radionuclide ablation, and en bloc resection. The recurrence rate is low after appropriate treatment; however, more than one procedure may be required to completely eradicate the lesion. PMID:22474093

  17. [Cilia and renal cysts].

    PubMed

    Paces-Fessy, Mélanie

    2014-11-01

    Advances in genomics, bioinformatics and the creation of model organisms have identified many genes associated with polycystic kidney diseases. Historically, these genes were not necessarily associated with ciliopathies, but it appeared that many connections can be made between the cystic kidney disease and function of the primary cilium. Indeed, the proteins encoded by these genes are localized to the cilium itself, to the basal body or are known to regulate the expression and localization of ciliary proteins. The goal of this article is to describe the multiple cellular processes that may lead to the development of renal cysts if they are deregulated. These include changes in proliferation rate, cell polarity or signaling pathways involved in embryonic kidney development. To highlight the role of the primary cilium in cystogenesis, I will discuss several studies investigating the function of ciliary genes and cilia in the kidneys of different model organisms. PMID:25388585

  18. The caries prevalence of oral clefts in eastern China

    PubMed Central

    Xiao, Wen-Lin; Zhang, Dai-Zun; Xu, Yao-Xiang

    2015-01-01

    Little information is available concerning the prevalence of caries among patients with oral clefts in Eastern China. Consecutive patients aged 6-18 with oral clefts were recruited. Patients were stratified into 2 groups according to their ages, namely Group I with aged 6-12 and Group II with aged 13-18. For each age group, the children were further divided into three subgroups according to the types of oral clefts they had: cleft lip/cleft lip and alveolus (CL), cleft palate only (CP), and cleft lip and palate (CLP). Dental caries were examined by using the decayed, missing, and filled index for primary teeth (dmft) and Decay, Missing and Filled index for Permanent teeth (DMFT) according to criteria of the World Health Organization. 268 eligible patients with oral clefts were included in the study. The mean DMFT for Group I was 1.77 (SD2.58) while that for Group II was 6.96 (SD4.35). The mean DMFT was statistically significant different between the age group I and age group II (t=12.21, P<0.05). In Group I, the dmft scores was 4.68 (SD3.67) for CL group, while that for the CP group was 7.36 (SD3.93), and that for the CLP group was 5.72 (SD 3.87). The mean dmft was no statistically significant different among cleft types (F=3.13, P>0.05). Also in Group I, the mean DMFT was 1.56 (SD2.18) for CL group, while that for the CP group was 1.24 (SD 1.81) and that for the CLP group was 2.08 (SD2.96). There were no statistically significant different in mean DMFT among different cleft types (F=1.09, P>0.05). In Group II, the mean DMFT was 6.06 (SD3.97) for CL group while that for the CP group was 7.71 (SD 4.94) and that for the CLP group was 7.05 (SD4.32). No significant difference was shown in the mean DMFT among different cleft groups (CL, CP, and CLP) (F=0.55, P>0.05). During assess the prevalence of dental caries among Eastern Chinese with oral clefts; the study confirmed that the prevalence of caries was increased with increasing age for oral clefts patients. It was

  19. Epidemiologic Research on Malformations Associated with Cleft Lip and Cleft Palate in Japan

    PubMed Central

    Koga, Hiroshi; Iida, Koichi; Maeda, Tomoki; Takahashi, Mizuho; Fukushima, Naoki; Goshi, Terufumi

    2016-01-01

    To investigate malformations associated with cleft lip and cleft palate, we conducted surveys at neonatal intensive care units (NICUs) and other non-NICU facilities and to determine whether there are differences among facilities. The regional survey investigated NICU facilities located in Oita Prefecture, including 92 patients with cleft lip and palate (CLP) or cleft palate (CP) that occurred between 2004 and 2013, and the national survey investigated oral surgery, plastic surgery, and obstetrics and gynecology facilities located in Japan, including 16,452 patients with cleft lip (CL), CLP, or CP that occurred since 2000. The incidence per 10,000 births was 4.2, 6.2, and 2.8 for CL, CLP, and CP, respectively, according to the national survey, and 6.3 and 2.9 for CLP and CP, respectively according to the regional survey. These results indicated comparable incidences between the two surveys. In contrast, when the survey results on malformations associated with CLP and CP according to the ICD-10 classification were compared between the national survey conducted at oral surgery or plastic surgery facilities and the regional survey conducted at NICU facilities, the occurrence of associated malformations was 19.8% vs. 41.3% for any types of associated malformation, 6.8% vs. 21.7% for congenital heart disease, and 0.5% vs. 16.3% for chromosomal abnormalities. These results indicated that the incidences of all of these associated malformations were significantly greater in the survey conducted at NICU facilities and similar to the findings from international epidemiological surveys. When comparing the survey conducted at obstetrics facilities vs. NICU facilities, the occurrence of associated malformations was similar results as above. The incidence of CLP and CP was not different between surveys conducted at NICU facilities vs. non-NICU facilities; however, when conducting surveys on associated malformations, it is possible to obtain accurate epidemiological data by

  20. Malonylcarnitine in Newborns with Non-syndromic Cleft Lip with or without Cleft Palate

    PubMed Central

    Hozyasz, Kamil Konrad; Oltarzewski, Mariusz; Dudkiewicz, Zofia

    2010-01-01

    Aim Malonyl-CoA is regarded as a key signaling molecule in mammalian cells. It is converted to acetyl-CoA, and to a lesser extent, to malonyl acid and malonylcarnitine (C3DC). Availability of carnitine has been reported to be essential for the developing fetus. The objectives of the present study were to analyze associations of malonylcarnitine, acetylcarnitine (C2), and free carnitine (C0) in subjects with orofacial clefts. Methodology We performed a retrospective analysis of carnitine concentration obtained from a newborn screening program carried out in our institution. Concentrations of whole blood malonylcarnitine, acetylcarnitine, and free carnitine were measured using tandem mass spectrometry. The study group consisted of 51 children with non-syndromic cleft lip with or without cleft palate. In total, 106 healthy children without congenital anomalies served as controls. Cut-off points were established using likelihood ratio values. Results The mean concentration of malonylcarnitine in the cleft group was lower than that of the control group, 0.048 μmol·L−1 vs. 0.058 μmol·L−1, respectively (P=0.009). In patients with orofacial cleft, low malonylcarnitine levels (≤0.047 μmol·L−1) were 1.7 times more predominant than in healthy individuals (P=0.03). The mean concentration of acetylcarnitine was also lower in affected newborns in comparison to controls, 33.8 μmol·L−1 vs. 37.8 μmol·L−1, respectively (P=0.026). After analysis of acetylcarnitine and free carnitine concentrations, the likelihood ratio test did not indicate valuable cut-off points. Conclusion The study provides initial data indicating a potential association between decreased malonylcarnitine and abnormal palatogenesis. PMID:21125791

  1. Ten-Year Cleft Surgery in Nepal: Achievements and Lessons Learned for Better Cleft Care Abroad

    PubMed Central

    Pape, Hans-Dieter; Koch, Heribert; Koller, Michael

    2016-01-01

    Background: Cleft lip and palate surgery abroad is devoid of global consensus regarding standards of therapy, follow-up, and outcome. Cleft surgery in Nepal during a 10-year sustained program provided the opportunity to inform on the need for such standards. Methods: Medical records were evaluated from the cleft clinic at Sushma Koirala Memorial Hospital, Sankhu, Kathmandu, Nepal, from 1997 to 2007. Four groups were identified for analysis: total cohort, total surgical cohort (TSC), primary program patients (PPP; patients had not been operated on before), and nonprimary program patients (non-PPP; patients operated on elsewhere before). Patient demographics, diagnostic, primary and secondary surgery (corrective surgery), and follow-up were evaluated. Results: One thousand forty-five patients were eligible for surgery. Three hundred twenty-three of 1,045 patients (30.9%) did not seek treatment, although scheduled for surgery. One thousand two hundred one procedures were performed in 722 patients [TSC; 845 PPP (70.4%); 356 non-PPP (29.64%)]. Corrective procedures were performed in 257 of 1,201 [3.5% (30 of 845 procedures in 509 patients) PPP vs 63.7% (227 of 356 procedures in 213 patients) non-PPP]. One hundred six lips were completely reoperated on (1 PPP vs 105 non-PPP), and 42 palates underwent a total revision (5 PPP vs 37 non-PPP). The surgical outcome of the TSC group in terms of complication rate was similar to the one in developed countries. Conclusions: The high rate of corrective surgery reveals the need for global regulatory mechanisms and the need for nongovernmental organizations to introduce strategies for delivering sustained cleft care until achieving full rehabilitation. The World Health Organization should establish standards for cleft care delivered in less developed countries. PMID:27579235

  2. Surgical repair of the isolated incomplete median cleft lip.

    PubMed

    Topkara, A; Özkan, A; Özcan, R H; Öksüz, M

    2016-02-01

    Median cleft lip refers to a vertical cleft on the midline of the upper lip. It is a rare congenital craniofacial anomaly brought about by a fusion failure in the medial nasal prominence. A novel surgical approach to median cleft lips and their repair is reported herein, with reference to a clinical case. The patient had a cleft in the lower half of the upper lip. There were no other craniofacial anomalies in this patient other than the cleft. Within the framework of the patient's surgical treatment, a functional and cosmetically satisfactory result was achieved by performing a V-Y advancement flap on the columella base, Z-plasty in the vermillion zone, and Z pattern muscular tissue repair, without having to resort to any tissue excisions. PMID:26364580

  3. The Repair of International Clefts in the Current Surgical Landscape.

    PubMed

    Persing, Sarah; Patel, Anup; Clune, James E; Steinbacher, Derek M; Persing, John A

    2015-06-01

    Cleft lip and palate (CLP) constitute a significant global disease burden. There are two general models that exist to deliver cleft care: surgical missions and comprehensive cleft centers (CCC). While surgical missions offer high quality surgical care to patients who would be unlikely to ever receive treatment, they may fail to provide sustainable solutions. The development of CCC is growing in popularity worldwide. CCC are permanent centers that offer a multidisciplinary team approach to the treatment of cleft lip and palate. Operation Smile has adopted the concept of specialized surgical care centers. These centers are shown to be safe, cost-effective, and provide sustainable solutions for cleft care. The authors discuss some of the benefits and drawbacks of the classic mission-based model and highlight why there may be a paradigm shift towards CCC. PMID:26080140

  4. The gingival Stillman’s clefts: histopathology and cellular characteristics

    PubMed Central

    Cassini, Maria Antonietta; Cerroni, Loredana; Ferlosio, Amedeo; Orlandi, Augusto; Pilloni, Andrea

    2015-01-01

    Summary Aim of the study Stillman’s cleft is a mucogingival triangular-shaped defect on the buccal surface of a root with unknown etiology and pathogenesis. The aim of this study is to examine the Stillman’s cleft obtained from excision during root coverage surgical procedures at an histopathological level. Materials and method Harvesting of cleft was obtained from two periodontally healthy patients with a scalpel and a bevel incision and then placed in a test tube with buffered solution to be processed for light microscopy. Results Microscopic analysis has shown that Stillman’s cleft presented a lichenoid hand-like inflammatory infiltration, while in the periodontal patient an inflammatory fibrous hyperplasia was identified. Conclusion Stillman’s cleft remains to be investigated as for the possible causes of such lesion of the gingival margin, although an inflammatory response seems to be evident and active from a strictly histopathological standpoint. PMID:26941897

  5. Flap Necrosis after Palatoplasty in Patients with Cleft Palate

    PubMed Central

    Rossell-Perry, Percy

    2015-01-01

    Palatal necrosis after palatoplasty in patients with cleft palate is a rare but significant problem encountered by any cleft surgeon. Few studies have addressed this disastrous complication and the prevalence of this problem remains unknown. Failure of a palatal flap may be attributed to different factors like kinking or section of the pedicle, anatomical variations, tension, vascular thrombosis, type of cleft, used surgical technique, surgeon's experience, infection, and malnutrition. Palatal flap necrosis can be prevented through identification of the risk factors and a careful surgical planning should be done before any palatoplasty. Management of severe fistulas observed as a consequence of palatal flap necrosis is a big challenge for any cleft surgeon. Different techniques as facial artery flaps, tongue flaps, and microvascular flaps have been described with this purpose. This review article discusses the current status of this serious complication in patients with cleft palate. PMID:26273624

  6. Recent cusp and cleft results from interball

    NASA Astrophysics Data System (ADS)

    Sandahl, Ingrid

    The Interball project has given important contributions to our understanding of the morphology and the physical processes in the cusp and cleft. Interball Tail and Magion-4 have performed more extensive measurements in the high altitude cusp than any previous spacecraft. Interball has also been a part in the ISTP program and data have been used in many multipoint studies. In this paper recent cusp and cleft studies based entirely or partly on Interball data will be reviewed. Interball data show that processes at high latitudes are very important for plasma entry into the magnetosphere. A case study for southward IMF conditions agrees with a model in which the mantle is populated via entry along open high-latitude field lines. A statistical study of events dominated by IMF B y shows that merging in anti-parallel fields, rather than subsolar point reconnection, populates the mantle. Plasma entry also takes place through the turbulent boundary layer, TBL, a region of strong, Alfvenic ULF turbulence above the cusp and cleft. The TBL is almost always present. It extends tailward from the cusp and is proposed to be related to the magnetospheric sash. For the overall magnetosheath plasma entry into the magnetosphere the magnetotail boundary is probably more important than the cusp. The position of the cusp is controlled by the solar wind in a similar way as the low altitude cusp. The mid-altitude cusp was found to maintain its fine structure over periods of the order of one hour. A suprathermal proton population not previously described has been detected in the mid-altitude cusp.

  7. Cleft Lip Repair: The Hybrid Subunit Method.

    PubMed

    Tollefson, Travis T

    2016-04-01

    The unilateral cleft lip repair is one of the most rewarding and challenging of plastic surgery procedures. Surgeons have introduced a variety of straight line, geometric, and rotation-advancement designs, while in practice the majority of North American surgeons have been using hybrids of the rotation-advancement techniques. The anatomic subunit approach was introduced in 2005 by Fisher and has gained popularity, with early adopters of the design touting its simplicity and effectiveness. The objectives of this article are to summarize the basic tenets of respecting the philtral subunit, accurate measurement and planning, and tips for transitioning to this subunit approach. PMID:27097136

  8. Genetics of Cleft Palate and Velopharyngeal Insufficiency

    PubMed Central

    Sweeney, Walter M.; Lanier, Steve T.; Purnell, Chad A.; Gosain, Arun K.

    2015-01-01

    Velopharyngeal insufficiency (VPI) can occur in the setting of an unrepaired or repaired cleft lip and palate. The rate of VPI has been documented as high as 33% in some studies with higher rates of recurrences following surgery associated with genetic syndromes such as 22q11.2 deletions. The primary cause of VPI in these groups is still identified as the anatomic abnormalities of the velum. In this review, the anatomy and physiology of the velum are discussed along with genetic mutations associated with VPI.

  9. Studies with WNT Genes and Nonsyndromic Cleft Lip and Palate

    PubMed Central

    Menezes, Renato; Letra, Ariadne; Kim, Ana H.; Kuchler, Erika C.; Day, Alicia; Tannure, Patricia N.; da Motta, Luise Gomes; Paiva, Katiucia Batista; Granjeiro, Jose M.; Vieira, Alexandre R.

    2010-01-01

    Background Clefts of the lip and/or palate (cleft lip/palate) are notable for their complex etiology. The WNT pathway regulates multiple developmental processes including craniofacial development and may play a role in cleft lip/palate and other defects of craniofacial development such as tooth agenesis. Variations in WNT genes have been recently associated with cleft lip/palate in humans. In addition, two WNT genes, Wnt3 and Wnt9B, are located in the clf1 cleft locus in mice. Methods We investigated 13 SNPs located in WNT3A, WNT5A, WNT8A, WNT11, WNT3 and WNT9B genes, for association with cleft lip/palate subphenotypes in 500 cleft cases and 500 unrelated controls. Genotyping of selected polymorphisms was carried out using Taqman assays. PLINK 1.06 software was used to test for differences in allele frequencies of each polymorphism between affected and unaffected individuals. Haplotype analysis was also performed. Results Individuals carrying variant alleles in WNT3 presented an increased risk for cleft lip/palate (P=0.0003; OR=1.61 95% C.I: 1.29 -2.02) in the population studied. Conclusion Our results continue to support a role for WNT genes in the pathogenesis of cleft lip/palate. Although much remains to be learned about the function of individual WNT genes during craniofacial development, additional studies should focus in the identification of potentially functional variants in these genes as contributors to human clefting. PMID:20890934

  10. Presurgical nasoalveolar moulding in unilateral cleft lip and palate

    PubMed Central

    Zuhaib, Mohammed; Bonanthaya, Krishnamurthy; Parmar, Renu; Shetty, Pritham N.; Sharma, Pradeep

    2016-01-01

    Context: Presurgical nasoalveolar moulding (PNAM) is a non-surgical method of reshaping the cleft lip, alveolus, palate and the nose to minimize the severity of the cleft deformity, before primary cheiloplastyand palatoplasty. In this context, PNAM proves to be an invaluable asset in the management of unilateral cleft lip and palate. Aims: The study was conducted to evaluate the efficacy of PNAM in the management of unilateral cleft lip and palate with the following objectives: (1) To assess and compare the degree of reduction in the size of cleft palate and alveolus (pre-PNAM and post-PNAM). (2) To evaluate and compare the improvement in columellar length and correction of columellar deviation (pre-PNAM and post-PNAM). (3) To assess the changes in the position of the alar base and the alar cartilages. Settings and Design: Prospective study. Subjects and Methods: A prospective study consisting of, which included 20 patients with complete unilateral cleft lip and palate was conducted. The age at the start of PNAM treatment of the infants ranged from 2 to 44 days of age reporting to our institute between December 2011 and August 2013. All the patients underwent PNAM therapy before primary cheiloplasty at 6 months of age; clinical parameters were assessed pre- and post-therapy using photographs and dental study models of the maxilla. Statistical Analysis Used: Student's t-test for paired comparisons. Results: Results of the study showed a promising reduction in the cleft size before the surgery, significant improvement in nasal symmetry, including the columellar length on the cleft side. Conclusions: PNAM is a valuable adjunct to our surgical armamentarium in dealing with the challenges of primary closure of unilateral cleft lip and palate thereby enhancing the overall surgical outcome. The advantages of this method include the simplicity of the procedure and improving the quality of surgical repair, particularly in obtaining tension free muscle closure in unilateral