Bladder exstrophy from childhood into adult life.

PubMed Central

Ben-Chaim, J; Docimo, S G; Jeffs, R D; Gearhart, J P

1996-01-01

Exstrophy of the bladder is rare and the incidence of bladder exstrophy is calculated to be from 1 per 30,000 to 50,000 live births with male to female ratio ranging from 1.5-5 to 1(1-4). It was found that persistence or overgrowth of the cloacal membrane on the lower anterior abdominal area, prevents normal mesenchymal ingrowth. This causes divergence of the lower abdominal muscular structures and forces the genital ridges to fuse caudal to the cloacal membrane. The stage of ingrowth of the urorectal septum at the time of rupture determines whether one will produce an exstrophic urinary tract alone (classic bladder exstrophy or epispadias) or cloacal exstrophy with the hindgut interposed between the hemibladders. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Figure 7 PMID:8709084

Experience with the artificial urinary sphincter in children and young adults.

PubMed

Mitchell, M E; Rink, R C

1983-12-01

The artificial urinary sphincter (model AS 791-792), American Medical Systems, Minnetonka, Minn.) has been implanted in 41 patients (mean age 13.9 years) who were incontinent in spite of intensive efforts with other modes of management. Neurogenic bladder dysfunction is present in 34 patients. Seven patients have non-neuropathic dysfunction of the bladder neck and urethra (3 with exstrophy/epispadias, 3 incontinent after multiple bladder and urethral procedures, and 1 incontinent after a pelvic fracture). Twenty-two patients have had intestinocystoplasty performed and 11 patients had previous urinary diversion. Mean follow-up for a given device is 23 months (range 6 to 47 months). Of these patients, 80.5% are totally or acceptably dry. Five patients (12.2%) were rated as fair, and three were failures (7.3%). Complications have been significant in that reoperation has been necessary in 16 patients. Indications for patient selection is emphasized.

Bladder calculi in the augmented bladder: a follow-up study of 160 children and adolescents.

PubMed

Kisku, S; Sen, S; Karl, S; Mathai, J; Thomas, R J; Barla, R

2015-04-01

Bladder augmentation (BA) has been used for various congenital and acquired conditions to create a low pressure, continent catheterizable reservoir. The prevalence of calculi within the BA have been reported to be from 3 to 52.5%. The present study reports the prevalence and risk factors of bladder calculi in patients with BA. A retrospective review of 160 patients was performed from January 1997 through December 2012. The various risk factors for the formation of bladder calculi such as the nature of the anatomical defect, presence of preoperative urinary calculi, type of bowel augmentation, addition of a mitrofanoff and/or bladder neck procedure, prevalence of post-operative urinary tract infections (UTIs), need for mitrofanoff revision due to stenosis/difficulty catheterization, postoperative significant hydronephrosis and bladder calculi were recorded for analysis. The children underwent open removal or endoscopic cystolithotripsy. One hundred and eight males and 52 females (average age 6.3 years) were followed up for a median of 70.5 months. All patients performed daily bladder irrigation with tap or drinking water. Post-operative bladder calculi were noted in 14 (8.8%) of 160 patients following BA. Median time to stone formation was 37.5 months (11-120 months). Recurrent febrile UTIs were noted in 16 of the 160 patients following BA. The various risk factors and their outcomes are summarized in table. Eight patients underwent open cystolithotomy and four patients were treated by cystolithotripsy. Post-operative recurrent bladder calculi were noted in 2 patients. Multivariate analysis revealed that exstrophy/epispadias (OR 17.2) and recurrent UTI (OR 55.4) were independent risk factors for developing postoperative calculi in bladder augmentations. All other risk factors did not achieve statistical significance. There seemed to be no difference in the prevalence of calculi in the ileal or colonic augmentations. Mucus secreted by the bowel segment blocks catheters leading to incomplete drainage, stagnation and UTIs. Our protocol consists of daily bladder irrigation till the effluents are clear of mucus. This is probably the key to the low prevalence of postoperative calculi (8.8%) in our patients. Bladder exstrophy/epispadias and UTIs are independent statistically significant risk factors for the formation of bladder calculi in BA patients. Other risk factors such as preoperative calculi, bladder neck procedures and the use of mitrofanoff though not statistically significant, may contribute to the overall risk. The performance of daily bladder irrigation is an important part of our management of mucus. Copyright © 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

Penile anomalies in adolescence.

PubMed

Wood, Dan; Woodhouse, Christopher

2011-03-07

This article considers the impact and outcomes of both treatment and underlying condition of penile anomalies in adolescent males. Major congenital anomalies (such as exstrophy/epispadias) are discussed, including the psychological outcomes, common problems (such as corporal asymmetry, chordee, and scarring) in this group, and surgical assessment for potential surgical candidates. The emergence of new surgical techniques continues to improve outcomes and potentially raises patient expectations. The importance of balanced discussion in conditions such as micropenis, including multidisciplinary support for patients, is important in order to achieve appropriate treatment decisions. Topical treatments may be of value, but in extreme cases, phalloplasty is a valuable option for patients to consider. In buried penis, the importance of careful assessment and, for the majority, a delay in surgery until puberty has completed is emphasised. In hypospadias patients, the variety of surgical procedures has complicated assessment of outcomes. It appears that true surgical success may be difficult to measure as many men who have had earlier operations are not reassessed in either puberty or adult life. There is also a brief discussion of acquired penile anomalies, including causation and treatment of lymphoedema, penile fracture/trauma, and priapism.

[Reconstructive surgery of penile deformities and tissue deficiencies].

PubMed

Kelemen, Zsolt

2009-05-31

Penile deformities and tissue deficiencies can disturb sexual intercourse or make it impossible. The aim of the study is to summarize the different diseases according to their clinical appearance and pathological processes and to review operative methods and personal experiences. Surgical treatment of hypo- and epispadias is usually performed in childhood, but curvatures after unsuccessful operation can demand the reconstruction of urethra, skin and corpora cavernosa eventually. Peyronie's disease and curvature after penile fracture desire the reconstruction of tunica albuginea. Plaque surgery used to be performed with dermal, tunica vaginalis or venous grafts, but best results are obtained by shortening procedure on the contralateral side according to the Heinecke-Mikulitz principle. Tissue deficiencies and curvatures were observed after necrotic inflammatory processes, like Fournier's gangrene or chronic dermatitis. Skin defects were cured by flaps and grafts. Abscesses of penis, severe tissue defects and also curvatures were observed after intracavernous injection in cases of erectile dysfunction. Possibilities of reconstruction seem to be very poor. Oil granuloma of penis presents a new task for penile reconstruction. The best results of skin replacement were achieved by temporary embedding of the penis in scrotum.

Evaluation of sexual function in females with exstrophy-epispadias-complex: A survey of the multicenter German CURE-Net.

PubMed

Ebert, Anne-Karoline; Lange, Theresa; Reutter, Heiko; Jenetzky, Ekkehart; Stein, Raimund; Boemers, Thomas M; Hirsch, Karin; Rösch, Wolfgang H; Zwink, Nadine

2017-04-01

Standardized knowledge about genital function in adult female individuals with exstrophy-epispadias complex (EEC) is scarce. The aim of this study was to investigate sexual function using the standardized Female Sexual Function Index (FSFI), and to assess the influence of bladder and vaginal reconstruction and the presence of incontinence on FSFI results. Sixty-one females (aged ≥18 years) recruited by the German multicenter network for congenital uro-rectal malformations (CURE-Net) were asked to complete the FSFI and a self-designed semi-structured questionnaire assessing comprehensive medical data, gynecological, and psychosocial items. Twenty-one eligible females (34%) returned both questionnaires (mean ± standard deviation [SD] age of 26 ± 5.1 years). In 43% of participants, a staged or single-staged approach had been used for reconstruction, and these had their bladder in use. A primary or secondary urinary diversion (UD) after cystectomy had been performed in 38% of participants. Of the participants, 57% lived in a committed partnership, and 62% had sexual intercourse on a regular basis, with a further 19% experiencing pain or discomfort thereby. Introitus plasty was done in 43%. Mean total FSFI for all participants was 21.3 (SD 1.9). Most domain scores of patients after introitus plasty were similar compared with those without an operative vaginal approach, except for satisfaction (p = 0.057) and pain (p = 0.024). Comparing incontinent with continent patients, significant differences were found for desire (mean 4.6 vs. 3.5, p = 0.021), lubrication (mean 3.1 vs. 4.2, p = 0.049), and satisfaction (mean 1.6 vs. 3.6, p = 0.0065). In contrast pain was not significant between groups. Sexual activity rate in the present study was similar to that reported in the literature (81% vs. 89%), whereas dyspareunia rate was lower in our cohort (19% vs. 24%). The risk for sexual dysfunction seems to be lower in patients reconstructed with primary or secondary UD than patients with bladder in use. It is surprising that lubrification was better after UD than after bladder neck surgery. Incontinence and in some parts the history of an introitus plasty may play an additional role in development of sexual dysfunction in EEC. Although most of the female EEC patients lived in a committed partnership and had sexual intercourse, total FSFI values <26.55 clearly indicate a risk of sexual dysfunction. Although continence itself played a major role, females reconstructed with UD seem to have better sexual function. Further evaluation of sexual outcome and improvement of care for these patients is mandatory. Copyright © 2016. Published by Elsevier Ltd.

Functional and aesthetic results in hypospadias repair with Hinderer's techniques.

PubMed

Hinderer, U T

2000-01-01

In his editorial to the first issue of Aesthetic Plastic Surgery in 1976, the Managing Editor, Dr. Blair Rogers lays special emphasis on the publication of papers and reports dealing with the increasing role of Aesthetic Plastic Surgery as the final step in the overall rehabilitation of Reconstructive Surgery patients. In genitourinary malformations-hypospadias and epispadias-without any doubt a satisfactory sexual and urinary functional result is essential. However, also a normal aesthetic appearance, resembling a circumcised penis, and with the meatus at the tip of the glans, is becoming increasingly important, notably since the second half of the last century. An abnormal aesthetic appearance affects the patient's body image and has a negative influence on his self-esteem and sexual behaviour. Psychological stress is brought on from genital comparison with school-mates, in adulthood in gym changing rooms and, specifically, in sexual relations. In these days of greater sexual freedom, the knowledge of male genital anatomy and aesthetic appearance has considerably improved. Penile hypoplasia creates a psychological impact perhaps only comparable with that of female mammary hypoplasia. It is therefore unsurprising that not only normal aesthetic appearance after hypospadias surgery is essential, but also the demand for penile lengthening and girth augmentation has progressively increased over these past recent years.

Lower urinary tract development and disease

PubMed Central

Rasouly, Hila Milo; Lu, Weining

2013-01-01

Congenital Anomalies of the Lower Urinary Tract (CALUT) are a family of birth defects of the ureter, the bladder and the urethra. CALUT includes ureteral anomalies such as congenital abnormalities of the ureteropelvic junction (UPJ) and ureterovesical junction (UVJ), and birth defects of the bladder and the urethra such as bladder-exstrophy-epispadias complex (BEEC), prune belly syndrome (PBS), and posterior urethral valves (PUV). CALUT is one of the most common birth defects and is often associated with antenatal hydronephrosis, vesicoureteral reflux (VUR), urinary tract obstruction, urinary tract infections (UTI), chronic kidney disease and renal failure in children. Here, we discuss the current genetic and molecular knowledge about lower urinary tract development and genetic basis of CALUT in both human and mouse models. We provide an overview of the developmental processes leading to the formation of the ureter, bladder, and urethra, and different genes and signaling pathways controlling these developmental processes. Human genetic disorders that affect the ureter, bladder and urethra and associated gene mutations are also presented. As we are entering the post-genomic era of personalized medicine, information in this article may provide useful interpretation for the genetic and genomic test results collected from patients with lower urinary tract birth defects. With evidence-based interpretations, clinicians may provide more effective personalized therapies to patients and genetic counseling for their families. PMID:23408557

Technique of Antireflux Procedure without Creating Submucosal Tunnel for Surgical Correction of Vesicoureteric Reflux during Bladder Closure in Exstrophy.

PubMed

Sunil, Kanoujia; Gupta, Archika; Chaubey, Digamber; Pandey, Anand; Kureel, Shiv Narain; Verma, Ajay Kumar

2018-01-01

To report the clinical application of the new surgical technique of antireflux procedure without creating submucosal tunnel for surgical correction of vesicoureteric reflux during bladder closure in exstrophy. Based on the report of published experimental technique, the procedure was clinically executed in seven patients of classic exstrophy bladder with small bladder plate with polyps, where the creation of submucosal tunnel was not possible, in last 18 months. Ureters were mobilized. A rectangular patch of bladder mucosa at trigone was removed exposing the detrusor. Mobilized urteres were advanced, crossed and anchored to exposed detrusor parallel to each other. Reconstruction included bladder and epispadias repair with abdominal wall closure. The outcome was measured with the assessment of complications, abolition of reflux on cystogram and upper tract status. At 3-month follow-up cystogram, reflux was absent in all. Follow-up ultrasound revealed mild dilatation of pelvis and ureter in one. The technique of extra-mucosal ureteric reimplantation without the creation of submucosal tunnel is simple to execute without risk and complications and effectively provides an antireflux mechanism for the preservation of upper tract in bladder exstrophy. With the use of this technique, reflux can be prevented since the very beginning of exstrophy reconstruction.

Experience with the artificial urinary sphincter model AS800 in 148 patients.

PubMed

Fishman, I J; Shabsigh, R; Scott, F B

1989-02-01

The latest version of the artificial urinary sphincter, AS800, was used in 148 patients with urinary incontinence of different etiologies. Followup ranged from 3 to 37 months, with an average of 20.8 months. There were 112 (76 per cent) male and 36 (24 per cent) female patients. The cuff was implanted around the bladder neck in 78 patients (53 per cent) and around the bulbar urethra in 70 (47 per cent). Socially acceptable urinary control was achieved in 90 per cent of the 139 patients with active devices in place. It was necessary to remove the sphincter in 11 patients (7.4 per cent). The reasons for removal were infection and erosion in 8 patients (5.4 per cent), infection without erosion in 2 (1.3 per cent), and erosion due to excess pressure and poor tissues in 1 (0.7 per cent). Comparison of success and failure rates associated with incontinence of different etiologies revealed that patients with incontinence after failure of a conventional antistress incontinence operation and those with incontinence after transurethral resection or radical prostactectomy had the highest success rate, and that patients with incontinence secondary to pelvic fracture or exstrophy and epispadias had the highest failure rates. The deactivation feature (the lock) of the new artificial sphincter model was beneficial for primary deactivation, urethral catheterization or cystoscopy, or for elective nocturnal decompression of the bladder neck or urethral tissues.

Psychosocial screening at paediatric BEEC clinics: a pilot evaluation study.

PubMed

Hurrell, Ruth A; Fullwood, Catherine; Keys, Joni; Dickson, Alan P; Fishwick, Janet; Whitnall, Beverley; Cervellione, Raimondo M

2015-04-01

Bladder Exstrophy and Epispadias Complex (BEEC) is associated with an increased risk of impaired mental health, quality of life, and psychosocial functioning. Therefore, screening patients to help identify and evaluate potential psychosocial difficulty is arguably an important consideration for BEEC Services. To screen paediatric BEEC patients for a range of general psychosocial difficulties in a multi-disciplinary out-patient clinic setting. This cross-sectional evaluation was conducted between April 2012 and July 2013. Families attending BEEC multi-disciplinary out-patient clinics were asked to complete a range of standardised psychosocial questionnaires, including the Paediatric Quality of Life Inventory (PedsQL 4.0 Generic Core and Family Impact Module), the Strengths and Difficulties Questionnaire (SDQ), the Paediatric Index of Emotional Distress (PI-ED), and the Hospital Anxiety and Depression Scale (HADS). 108 children attended clinic of which 80 (74.1%) patients and their parents/carers completed some or all of the questionnaires. The mean patient age was 8.41 years (SD = 4.46, range = 1-18 years). There were more boys (N = 50, 62.5%) and the majority had a diagnosis of classic bladder exstrophy (N = 51, 63.8%), followed by primary epispadias (N = 22, 27.5%) and cloacal exstrophy (N = 7, 8.7%). Mean total scores fell within the average/normal range on all questionnaires used (See table below). However, variation around these means was high. Age, gender and diagnosis were found to significantly influence certain questionnaire responses with older-age groups, males, and those with classic bladder exstrophy particularly at risk across some domains. The children/adolescents self-reported better health related quality of life (HRQoL) scores than published results for a range of paediatric chronic health conditions. Differences between parent and child responses on both the PedsQL and SDQ favoured a more positive response on the child self-report questionnaire but were not statistically significant. Mean scores on the measures used suggest a relatively optimistic picture of general psychosocial well-being, especially for HRQoL, in the BEEC population studied. Positive HRQoL outcomes have recently been reported for BEEC paediatric populations. Our results reflect this trend with better mean HRQoL scores than paediatric patients with a range of other chronic health conditions. However, this optimism is cautious given the limitations of this evaluation study and the high variation around the means. Limitations included the small sample size (especially for patients with cloacal exstrophy), the lack of a control group, the limited sensitivity of generic questionnaires in respect of BEEC-specific issues, and the low mean age of patients in the study. Future screening programmes may wish to consider measuring BEEC-specific variables (e.g. satisfaction with genital appearance/function); collecting information on medical aspects, such as continence, pubertal stage and frequency/timing of medical intervention; and asking both parents/carers (where possible) to complete the questionnaires. Screening questionnaire responses were used in conjunction with clinical psychology consultations to evaluate a range of psychosocial aspects in BEEC paediatric patients. Whilst mean scores on the measures used suggest a relatively optimistic picture, certain individual scores did fall within the clinical ranges, highlighting the potential need for further assessment. Developmentally tailored consultations with a clinical psychologist can provide detailed information around questionnaire responses and further assess BEEC specific aspects. Copyright © 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

The importance of social media for patients and families affected by congenital anomalies: A Facebook cross-sectional analysis and user survey.

PubMed

Jacobs, Robyn; Boyd, Leanne; Brennan, Kirsty; Sinha, C K; Giuliani, Stefano

2016-11-01

We aimed to define characteristics and needs of Facebook users in relation to congenital anomalies. Cross-sectional analysis of Facebook related to four congenital anomalies: anorectal malformation (ARM), congenital diaphragmatic hernia (CDH), congenital heart disease (CHD) and hypospadias/epispadias (HS/ES). A keyword search was performed to identify relevant Groups/Pages. An anonymous survey was posted to obtain quantitative/qualitative data on users and their healthcare needs. 54 Groups and 24 Pages were identified (ARM: 10 Groups; CDH: 9 Groups, 7 Pages; CHD: 32 Groups, 17 Pages; HS/ES: 3 Groups), with 16,191 Group members and 48,766 Page likes. 868/1103 (79%) of respondents were parents. Male:female ratio was 1:10.9. 65% of the users were 26-40years old. Common reasons for joining these Groups/Pages included: seeking support, education, making friends, and providing support to others. 932/1103 (84%) would like healthcare professionals (HCPs) to actively participate in their Group. 31% of the respondents felt that they did not receive enough support from their healthcare system. 97% of the respondents would like to join a Group linked to their primary hospital. Facebook Groups/Pages related to congenital anomalies are highly populated and active. There is a need for HCPs and policy makers to better understand and participate in social media to support families and improve patient care. Copyright © 2016 Elsevier Inc. All rights reserved.

Prevalence of preoperative penile abnormalities among voluntary male medical circumcision patients in Swaziland.

PubMed

Oddo, Anthony R; Ruedrich, Elizabeth; Zust, Christopher; Marugg, Lindsey; VanderWal, Echo; VanderWal, Harry; Sartori, Rebekah; Markert, Ronald; McCarthy, Mary C

2017-08-01

Circumcision has been found to be an effective strategy for lowering the transmission of HIV in Africa. The Luke Commission, a mobile hospital outreach program, has used this information to decrease the rate of HIV in Swaziland by performing voluntary male medical circumcisions throughout the country. During many of these circumcisions, genital medical conditions and penile abnormalities are simultaneously discovered and corrected. The goal of our study was to evaluate the prevalence of penile abnormalities discovered and treated during voluntary male medical circumcisions performed by The Luke Commission (TLC) throughout rural Swaziland. We completed a retrospective analysis of all male patients who underwent voluntary male medical circumcision performed by TLC during a period from June-August, 2014. The penile abnormalities included: phimosis, paraphimosis, epispadias, hypospadias, ulcers, balanitis, torsion, and foreskin adherent to the glans. Of 929 total circumcisions, 771 (83%) patients had at least one pre-existing penile abnormality identified during their examinations and procedures, totaling 1110 abnormalities. Three specific abnormalities were detected - phimosis, adherent foreskin, and hypospadias. The 6-12 and 13-19 age groups had adequate sample sizes to yield precise estimates of prevalence (age group 6-12: 87% (95% confidence interval [CI]=84-90%; age group 13-19: 79% (95% CI=74-84%). The Luke Commission is improving the lives of children and adults with limited access to healthcare through regular preoperative evaluations during male circumcision, and the organization is setting an example for other international healthcare groups. Type of Study: Prognostic Study, Level II. Copyright © 2017 Elsevier Inc. All rights reserved.

Microduplications at 22q11.21 are associated with non-syndromic classic bladder exstrophy.

PubMed

Draaken, Markus; Reutter, Heiko; Schramm, Charlotte; Bartels, Enrika; Boemers, Thomas M; Ebert, Anne-Karoline; Rösch, Wolfgang; Schröder, Annette; Stein, Raimund; Moebus, Susanne; Stienen, Dietlinde; Hoffmann, Per; Nöthen, Markus M; Ludwig, Michael

2010-01-01

The exstrophy-epispadias complex (EEC) comprises a spectrum of urogenital anomalies in which part or all of the distal urinary tract fails to close. The present study aimed to identify microaberrations characterized by loss or gain of genomic material that contribute to the EEC at a genome-wide level. Molecular karyotyping, utilizing 549,839 single nucleotide polymorphisms (SNPs) with an average spacing of 5.7 kilobases, was performed to screen an initial cohort of 16 patients with non-syndromic EEC. A de novo microduplication involving chromosomal region 22q11.21 was identified in one patient with classic exstrophy of the bladder (CBE). Subsequent multiplex ligation-dependent probe amplification (MLPA) analysis was performed with an MLPA 22q11 kit in a further 50 non-syndromic EEC cases. We identified one CBE patient with an overlapping 22q11.21 duplication in whom the duplication had been transmitted from the unaffected mother. Chromosomal region 22q11 is well known for its susceptibility to genomic rearrangements, and these are associated with various syndromes including the velo-cardio-facial/DiGeorge syndrome (VCFS/DGS), the der(22) syndrome, and the cat-eye syndrome. Duplications in this region result in a wide and variable spectrum of clinical presentations that include features of the VCFS/DGS, while some carriers present with a completely normal phenotype. Our findings extend the phenotypic spectrum of the 22q11.2 duplication syndrome, and indicate that this aberration predisposes to CBE with incomplete penetrance. Copyright 2009 Elsevier Masson SAS. All rights reserved.

Combined Bladder Neck Reconstruction and Continent Stoma Creation as a Suitable Alternative for Continence in Bladder Exstrophy: a Preliminary Report.

PubMed

Kasprenski, Matthew; Benz, Karl; Jayman, John; Lue, Kathy; Maruf, Mahir; Baumgartner, Timothy; Gearhart, John

2018-05-25

To explore the use of concomitant BNR and creation of a continent stoma (CS) in patients that are not quite eligible for BNR, but still strongly desire volitional voiding. The authors retrospectively reviewed an institutional database of EEC patients for those who underwent BNR-CS between 2000 and 2015. Indications for a BNR-CS, perioperative outcomes, and continence status were evaluated. Method of voiding and continence status were analyzed for patients with greater than six months of follow-up after the BNR-CS. A total of 24 EEC patients (15 male and 9 female) underwent BNR-CS at a median age of 8.9 years (range 5.4-17.4). This included, 18 patients with classic bladder exstrophy (CBE), 5 with epispadias, and 1 with a cloacal exstrophy variant. There were 5 (20.1%) surgical complications following the BNR-CS, including 3 febrile urinary tract infections, 1 superficial wound infection, and 1 urethrocutaneous fistula. The median follow-up time from the time of BNR-CS was 1.1 years (range 0.1-14.1). Seventeen of 24 patients (71%) had a follow-up greater than 6 months and were evaluated for continence. Twelve patients (71%) were completely dry for intervals greater than 3 hours following BNR-CS. Five (29%) did not achieve continence with BNR-CS. Of those 5 patients, 3 (60%) underwent subsequent bladder neck transection. Combined BNR and CS is a suitable alternative to achieve urinary continence in patients that are not ideal candidates for BNR alone. This approach can offer a select group of patients the opportunity for volitional voiding. Copyright © 2018. Published by Elsevier Inc.

Preoperative care of Polypoid exposed mucosal template in bladder exstrophy: the role of high-barrier plastic wraps in reducing inflammation and polyp size.

PubMed

Sabetkish, Nastaran; Sabetkish, Shabnam; Kajbafzadeh, Abdol-Mohammad

2018-01-26

To assess the role of high-barrier plastic wrap in reducing the number and size of polyps, as well as decreasing the inflammation and allergic reactions in exstrophy cases, and to compare the results with the application of low-barrier wrap. Eight patients with bladder exstrophy-epispadias complex (BEEC) that had used a low density polyethylene (LDPE) wrap for coverage of the exposed polypoid bladder in preoperative care management were referred. The main complaint of their parents was increase in size and number of polyps. After a period of 2 months using the same wrap and observing the increasing pattern in size of polyps, these patients were recommended to use a high-barrier wrap which is made of polyvinylidene chloride (PVdC), until closure. Patients were monitored for the number and size of polyps before and after the change of barriers. The incidence of para-exstrophy skin infection/inflammation and skin allergy were assessed. Biopsies were taken from the polyps to identify histopathological characteristics of the exposed polyps. The high barrier wrap was applied for a mean ± SD duration of 12±2.1 months. Polyps' size and number decreased after 12 months. No allergic reaction was detected in patients after the usage of PVdC; three patients suffered from low-grade skin allergy when LDPE was applied. Also, pre-malignant changes were observed in none of the patients in histopathological examination after the application of PVdC. Polyps' size and number and skin allergy may significantly decrease with the use of a high-barrier wrap. Certain PVdC wraps with more integrity and less evaporative permeability may be more "exstrophy-friendly". Copyright® by the International Brazilian Journal of Urology.

History of hypospadias: Lost in translation.

PubMed

Hadidi, Ahmed T

2017-02-01

The evolution of hypospadias surgery can be classified under 3 periods that were closely related to advances in surgical instruments, introduction of anesthesia, and newer suture materials. Stretching dominated the early period, tunneling during the Middle Ages, and flaps during the modern period. Suture materials have included at various time silver wires, horsehair, and stainless steel. Examination and translation of the original manuscripts showed that Galen recommended stretching and suturing of glanular hypospadias and not amputation or partial penectomy as has been currently reported. The term chordee was first introduced in the 18th century in relation to gonorrhea and was defined as "painful imperfect erection of the penis during gonorrhea, with downward incurvation." This was a common complication of gonorrhea before the introduction of antibiotics. Mettauer, Duplay, Mayo, and others used the terms incurvation, ventral deformity, ventral curvature, and others. Clinton Smith in 1938 was the first surgeon to use the term congenital chordee in direct relation to hypospadias. The use of prepuce for urethroplasty, popular now, was first reported by Liston in 1838, Rochet in 1899, Russell in 1900, and Mayo in 1901. The two stage repair performed in the early 20th century differed from that in the early 21st century in that urethroplasty was performed in the first stage and only anastomosis to the original meatus was performed in the second stage. The two-stage repair, currently known as Bracka's two-stage repair, was first described in 1962 by Cloutier. The use of the urethral plate in epispadias was first described by Liston in 1838, Thiersch in 1869 and by Anger & Duplay in hypospadias in 1874. Partially epithelialized urethroplasty using the urethral plate was described by Duplay in 1880, Russell 1915, Denis Browne 1940, Reddy 1975, Orkiszewski 1987, Rich 1989, and Snodgrass in 1994. V. Copyright © 2017 Elsevier Inc. All rights reserved.

Congenital renal anomalies in cloacal exstrophy: Is there a difference?

PubMed

Suson, K D; Inouye, B; Carl, A; Gearhart, J P

2016-08-01

Cloacal exstrophy (CE) is the most severe manifestation of the epispadias-exstrophy spectrum. Previous studies have indicated an increased rate of renal anomalies in children with classic bladder exstrophy (CBE). Given the increased severity of the CE defect, it was hypothesized that there would be an even greater incidence among these children. The primary objective was to characterize renal anatomy in CE patients. Two secondary objectives were to compare these renal anatomic findings in male and female patients, and female patients with and without Müllerian anomalies. An Institutional Review Board-approved retrospective review of 75 patients from an institutional exstrophy database. Data points included: age at analysis, sex, and renal and Müllerian anatomy. Abnormal renal anatomy was defined as a solitary kidney, malrotation, renal ectopia, congenital cysts, duplication, and/or proven obstruction. Abnormal Müllerian anatomy was defined as uterine or vaginal duplication, obstruction, and/or absence. The Summary Table presents demographic data and renal anomalies. Males were more likely to have renal anomalies. Müllerian anomalies were present in 65.7% of female patients. Girls with abnormal Müllerian anatomy were 10 times more likely to have renal anomalies than those with normal Müllerian anatomy (95% CI 1.1-91.4, P = 0.027). Patients with CE had a much higher rate of renal anomalies than that reported for CBE. Males and females with Müllerian anomalies were at greater risk than females with normal uterine structures. Mesonephric and Müllerian duct interaction is required for uterine structures to develop normally. It has been proposed that women with both Müllerian and renal anomalies be classified separately from other uterine malformations on an embryonic basis. In these patients, an absent or dysfunctional mesonephric duct has been implicated as potentially causal. This provided an embryonic explanation for uterine anomalies in female CE patients. There were also clinical implications. Women with renal agenesis and uterine anomalies were more likely to have endometriosis than those with isolated uterine anomalies, but were also more likely to have successful pregnancies. Males may have had an analogous condition with renal agenesis and seminal vesicle cysts. Future research into long-term kidney function in this population, uterine function, and possible male sexual duct malformation is warranted. Congenital renal anomalies occurred frequently in children with CE. They were more common in boys than in girls. Girls with abnormal Müllerian anatomy were more likely to have anomalous renal development. Mesonephric duct dysfunction may be embyologically responsible for both renal and Müllerian maldevelopment. Copyright © 2016 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

Risk of adverse birth outcomes in populations living near landfill sites

PubMed Central

Elliott, Paul; Briggs, David; Morris, Sara; de Hoogh, Cornelis; Hurt, Christopher; Jensen, Tina Kold; Maitland, Ian; Richardson, Sylvia; Wakefield, Jon; Jarup, Lars

2001-01-01

Objective To investigate the risk of adverse birth outcomes associated with residence near landfill sites in Great Britain. Design Geographical study of risks of adverse birth outcomes in populations living within 2 km of 9565 landfill sites operational at some time between 1982 and 1997 (from a total of 19 196 sites) compared with those living further away. Setting Great Britain. Subjects Over 8.2 million live births, 43 471 stillbirths, and 124 597 congenital anomalies (including terminations). Main outcome measures All congenital anomalies combined, some specific anomalies, and prevalence of low and very low birth weight (<2500 g and <1500 g). Results For all anomalies combined, relative risk of residence near landfill sites (all waste types) was 0.92 (99% confidence interval 0.907 to 0.923) unadjusted, and 1.01 (1.005 to 1.023) adjusted for confounders. Adjusted risks were 1.05 (1.01 to 1.10) for neural tube defects, 0.96 (0.93 to 0.99) for cardiovascular defects, 1.07 (1.04 to 1.10) for hypospadias and epispadias (with no excess of surgical correction), 1.08 (1.01 to 1.15) for abdominal wall defects, 1.19 (1.05 to 1.34) for surgical correction of gastroschisis and exomphalos, and 1.05 (1.047 to 1.055) and 1.04 (1.03 to 1.05) for low and very low birth weight respectively. There was no excess risk of stillbirth. Findings for special (hazardous) waste sites did not differ systematically from those for non-special sites. For some specific anomalies, higher risks were found in the period before opening compared with after opening of a landfill site, especially hospital admissions for abdominal wall defects. Conclusions We found small excess risks of congenital anomalies and low and very low birth weight in populations living near landfill sites. No causal mechanisms are available to explain these findings, and alternative explanations include data artefacts and residual confounding. Further studies are needed to help differentiate between the various possibilities. What is already known on this topicVarious studies have found excess risks of certain congenital anomalies and low birth weight near landfill sitesRisks up to two to three times higher have been reportedThese studies have been difficult to interpret because of problems of exposure classification, small sample size, confounding, and reporting biasWhat this study addsSome 80% of the British population lives within 2 km of known landfill sites in Great BritainBy including all landfill sites in the country, we avoided the problem of selective reporting, and maximised statistical powerAlthough we found excess risks of congenital anomalies and low birth weight near landfill sites in Great Britain, they were smaller than in some other studiesFurther work is needed to differentiate potential data artefacts and confounding effects from possible causal associations with landfill PMID:11509424

Research progress in muscle-derived stem cells: Literature retrieval results based on international database.

PubMed

Zhang, Li; Wang, Wei

2012-04-05

To identify global research trends of muscle-derived stem cells (MDSCs) using a bibliometric analysis of the Web of Science, Research Portfolio Online Reporting Tools of the National Institutes of Health (NIH), and the Clinical Trials registry database (ClinicalTrials.gov). We performed a bibliometric analysis of data retrievals for MDSCs from 2002 to 2011 using the Web of Science, NIH, and ClinicalTrials.gov. (1) Web of Science: (a) peer-reviewed articles on MDSCs that were published and indexed in the Web of Science. (b) Type of articles: original research articles, reviews, meeting abstracts, proceedings papers, book chapters, editorial material and news items. (c) Year of publication: 2002-2011. (d) Citation databases: Science Citation Index-Expanded (SCI-E), 1899-present; Conference Proceedings Citation Index-Science (CPCI-S), 1991-present; Book Citation Index-Science (BKCI-S), 2005-present. (2) NIH: (a) Projects on MDSCs supported by the NIH. (b) Fiscal year: 1988-present. (3) ClinicalTrials.gov: All clinical trials relating to MDSCs were searched in this database. (1) Web of Science: (a) Articles that required manual searching or telephone access. (b) We excluded documents that were not published in the public domain. (c) We excluded a number of corrected papers from the total number of articles. (d) We excluded articles from the following databases: Social Sciences Citation Index (SSCI), 1898-present; Arts & Humanities Citation Index (A&HCI), 1975-present; Conference Proceedings Citation Index - Social Science & Humanities (CPCI-SSH), 1991-present; Book Citation Index - Social Sciences & Humanities (BKCI-SSH), 2005-present; Current Chemical Reactions (CCR-EXPANDED), 1985-present; Index Chemicus (IC), 1993-present. (2) NIH: (a) We excluded publications related to MDSCs that were supported by the NIH. (b) We limited the keyword search to studies that included MDSCs within the title or abstract. (3) ClinicalTrials.gov: (a) We excluded clinical trials that were not in the ClinicalTrials.gov database. (b) We excluded clinical trials that dealt with stem cells other than MDSCs in the ClinicalTrials.gov database. (1) Type of literature; (2) annual publication output; (3) distribution according to journals; (4) distribution according to country; (5) distribution according to institution; (6) top cited authors over the last 10 years; (7) projects financially supported by the NIH; and (8) clinical trials registered. (1) In all, 802 studies on MDSCs appeared in the Web of Science from 2002 to 2011, almost half of which derived from American authors and institutes. The number of studies on MDSCs has gradually increased over the past 10 years. Most papers on MDSCs appeared in journals with a particular focus on cell biology research, such as Experimental Cell Research, Journal of Cell Science, and PLoS One. (2) Eight MDSC research projects have received over US$6 billion in funding from the NIH. The current project led by Dr. Johnny Huard of the University of Pittsburgh-"Muscle-Based Tissue Engineering to Improve Bone Healing"-is supported by the NIH. Dr. Huard has been the most productive and top-cited author in the field of gene therapy and adult stem cell research in the Web of Science over last 10 years. (3) On ClinicalTrials.gov, "Muscle Derived Cell Therapy for Bladder Exstrophy Epispadias Induced Incontinence" Phase 1 is registered and sponsored by Johns Hopkins University and has been led by Dr. John P. Gearhart since November 2009. From our analysis of the literature and research trends, we found that MDSCs may offer further benefits in regenerative medicine.

Sexual function in adult patients with classic bladder exstrophy: A multicenter study.

PubMed

Park, Weon; Zwink, Nadine; Rösch, Wolfgang H; Schmiedeke, Eberhard; Stein, Raimund; Schmidt, Dominik; Noeker, Meinolf; Jenetzky, Ekkehart; Reutter, Heiko; Ebert, Anne-Karoline

2015-06-01

The bladder exstrophy-epispadias complex (BEEC) comprises a spectrum of congenital anomalies that represents the severe end of urorectal malformations, and has a profound impact on continence as well as sexual and renal functions. The relation between severity of BEEC and its associated functional impairments, on one hand, and the resulting restrictions in quality of life and potential psychopathology determine the patients' outcome. It is important for improving further outcome to identify BEEC-related sources of distress in the long term. Genital function and sexuality becomes an important issue for adolescent and adult BEEC individuals. Hence, the present study focused on sexual function and psychological adaption in patients with BEEC. In a multicenter study 52 patients (13 females, 39 males) with classic bladder exstrophy (BE) with their bladders in use were assessed by a self-developed questionnaire about sexual function, and psychosexual and psychosocial outcome. The patients were born between 1948 and 1994 (median age 31 years). Twelve of 13 (92%) females and 25 of 39 (64%) males with classic BE had answered the questions on sexual function. Of these, 50% females and 92% males answered that they masturbated. Females had sexual intercourse more frequently. Six (50%) females affirmed dyspareunia whereas only two (8%) males reported pain during erection. Eight (67%) females specified having orgasms. Eighteen (72%) males were able to ejaculate. Two males and none of the females lived in a committed partnership (Figure). Two (15%) females and 13 (33%) males answered all psychosocial questions. The majority of these patients had concerns about satisfactory sexuality and lasting, happy partnerships. A minority of patients of both sexes were willing to answer psychosocial questions. Sexual activity and relationships of many adult BE patients seems to be impaired. Not surprisingly, sexual activity and awareness were different in males and females even in a multi-organ anomaly. To date, one of the main goals of the medical treatment of BEEC/BE patients is to enable normal sexual life and fertility. However, only a few outcome studies have focused on these issues with contradicting results, most of them not using standardized outcome measures. In accordance with other studies, our female BE patients have dyspareunia and most of our male BE patients were able to ejaculate. But the question of normal force of ejaculation, ejaculated volume, or semen analysis remains unanswered. Despite partial confirmation of previous findings, there is inconsistency referring to the outcome measured by the available studies. This might in part be explained by the fact that, other than this study, most previous studies are the result of single-institution experience. Thus, selection bias in the patient sampling due to different a clinical collective in different hospitals may be the consequence. Furthermore, patients' honesty and self-reflection in answering difficult questions regarding their sexual and cosmetic impairments is questionable. In addition, studies include a wide range of age groups and are connected with this life period. Fears and condition-specific anxieties might change over time. Hence, the strengths of this study are the nationwide and treating physician-independent data acquisition as well as the large sample size of adult patients with a very rare congenital malformation. Unfortunately, more detailed analyses on sexual function and current psychosocial situation, for example correlation of data with clinical symptoms such as continence status, was not possible as data were mainly not answered by patients. To improve the quality of life of patients with BEEC/BE, treatment and follow-up should emphasize physical but also psychological care in these patients. Physicians should further re-evaluate their preconceptions and should take care of the patients throughout their lives. Copyright © 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.