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Rakesh S Joshi, Dhirendra Shrivastava, Richard Grady, Anjana Kundu, Jaishri Ramji, Pramod P Reddy, Joao Luiz Pippi-Salle, Jennifer R Frazier, Douglas A Canning, Aseem R Shukla
Importance: International collaboration to alleviate the massive burden of surgical disease is recognized by World Health Organization as an urgent need, yet the surgical mission model to treat reconstructive surgical challenges is often constrained in ensuring adequate patient follow-up, optimal outcomes, and sustainability. Objective: To determine whether a collaboration predicated on long-term commitment by surgeons returning to the same institution annually combined with an experienced host surgical team and infrastructure to ensure sustained patient follow-up could provide surgical care with acceptable outcomes to treat bladder exstrophy-epispadias complex (BE) and penopubic epispadias (PE)...
March 7, 2018: JAMA Surgery
Akiko Haruna, Eiji Hisamatsu, Taketoshi Nara, Yoshifumi Sugita
Cloacal exstrophy is the most severe form of the exstrophy-epispadias complex (EEC), occurring in approximately 1 of every 200,000 to 400,000 live births. Variant such as covered cloacal exstrophy presentations are only one-tenth as common. Although EECs include genital anomalies, intravesical phallus is very rare. We report an extremely rare case of intravesical phallus with covered cloacal exstrophy that was successfully treated by phallic mobilization.
March 2, 2018: Urology
Brittani Jones, Allyson C Berent, Chick W Weisse, Robert Hart, Leilani Alvarez, Anthony Fischetti, B David Horn, Douglas Canning
CASE DESCRIPTION A 14-week-old 7.7-kg (16.9-lb) sexually intact female Golden Retriever was evaluated because of urine dripping from the caudoventral aspect of the abdomen. CLINICAL FINDINGS Ultrasonography, radiography, excretory CT urography, and vaginocystourethroscopy were performed. Results indicated eversion of the bladder through the ventral abdominal wall with exposure of the ureterovesicular junctions, pubic diastasis, and an open vulva and clitoral fossa. Clinical findings were suggestive of bladder exstrophy, a rare congenital anomaly...
March 15, 2018: Journal of the American Veterinary Medical Association
Nelly Reynaud, Frédérique Courtois, Pierre Mouriquand, Nicolas Morel-Journel, Kathleen Charvier, Marina Gérard, Alain Ruffion, Jean-Etienne Terrier
BACKGROUND: The bladder exstrophy-epispadias complex is a rare congenital malformation associated with severe dysfunction of the genital and urinary tracts and requiring a staged surgical reconstruction. AIM: The primary aims of this study were to report the sexuality, infertility, and urinary incontinence outcomes in a cohort of men born with bladder exstrophy-epispadias complex. The secondary aim was to highlight some predictive factors of infertility in this population...
March 2018: Journal of Sexual Medicine
P D Polm, S Beyerlein, A J Klijn, T P V M de Jong, P Dik
INTRODUCTION: A tubularized conduit from an open 2-cm vascularized ileal segment is a frequently used technique to create a continent catheterizable channel in cases of an inappropriate or absent appendix. In the long term, many patients experience catheterization problems with the classic ileal segment tube, and even more with spiral or double-segment tubes. OBJECTIVE: The objective of this paper was to introduce an ileocystoplasty modification combined with a long ileal segment tube that has better support by surrounding tissue than other lengthy ileal segment tubes...
February 7, 2018: Journal of Pediatric Urology
Karl Benz, John Jayman, Karen Doersch, Mahir Maruf, Timothy Baumgartner, Matthew Kasprenski, John P Gearhart
OBJECTIVE: To characterize the causes of re-augmentation in patients with classic bladder exstrophy. METHODS: A prospectively-maintained institutional database of 1327 exstrophy-epispadias complex patients was reviewed for CBE patients who have undergone more than one AC procedure. Data regarding bladder capacities, complications following AC, and reasons for re-augmentation were evaluated. RESULTS: A total of 166 CBE patients underwent AC...
February 12, 2018: Urology
Marc-David Leclair, Sébastien Faraj, Thierry Villemagne, Julie Carrouget, Alexis Arnaud, Yves Heloury
OBJECTIVES: Primary female epispadias encompasses a spectrum of disease, presenting with a variable degree of incontinence. We hypothesized that although perineal urethrocervicoplasty can be a successful first-line procedure in patients with normal bladder, a more radical reconstruction was necessary to achieve continence in cases lying at the most severe end of the spectrum. Our aim was to assess the results of a surgical management using perineal approach in girls with normal bladder capacity, and Kelly radical soft-tissue mobilization (RSTM) in patients with inadequate bladder, based on the assumption that bladder capacity (BC) is a reliable marker of epispadias severity...
October 9, 2017: Journal of Pediatric Urology
Karl S Benz, John Jayman, Mahir Maruf, Timothy Baumgartner, Matthew C Kasprenski, Daniel A Friedlander, Heather N Di Carlo, Paul D Sponseller, John P Gearhart
INTRODUCTION: Successful bladder closure in cloacal exstrophy (CE) is best accomplished through a multidisciplinary team and attention to pre- and postoperative technique. This study from a high volume exstrophy center investigates outcomes and complications of primary and reoperative bladder closures in patients immobilized with spica cast or patients with external fixation (EF) and skin traction. METHODS: The authors reviewed an institutionally approved and daily updated database of 1311 patients with exstrophy-epispadias complex and identified patients with cloacal exstrophy born between 1975 and 2015 who had undergone primary or reoperative bladder closures...
December 27, 2017: Journal of Pediatric Surgery
Nastaran Sabetkish, Shabnam Sabetkish, Abdol-Mohammad Kajbafzadeh
OBJECTIVE: 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. MATERIALS AND METHODS: 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...
January 26, 2018: International Braz J Urol: Official Journal of the Brazilian Society of Urology
Kasemsri Srisupundit, Phitsanu Mahawong, Cholaros Charoenratana, Theera Tongsong
A 26-year-old pregnant woman was diagnosed with fetal bladder prolapse following rupture of a patent urachus/urachal cyst, based on the finding of cyst disappearance with replacement with an infra-umbilical, extra-abdominal solid soft-tissue mass, mimicking bladder exstrophy. The neonatal findings confirmed the prenatal diagnosis. The baby was healthy and had a successful surgical correction. This report provides clues to differentiating ruptured bladder prolapse from bladder exstrophy as follows: (1) well-documented urachal cyst with urine-filled mass in the early gestation, (2) development of solid soft-tissue mass shortly after disappearance of the urachal cyst, and (3) no other structural abnormalities (bladder exstrophy is usually associated with abnormal genitalia, epispadias, or pubic diastasis)...
January 9, 2018: Journal of Medical Ultrasonics
M Cendron, P S Cho, M Pennison, I Rosoklija, D A Diamond, J G Borer
INTRODUCTION: Pre-operative physical examination of male epispadias allows for classification of epispadias level as glanular (GE), penile (PE) or penopubic (PPE), and for delineation of anatomic anomalies. The incidence of associated extragenital abnormalities, such as vesicoureteral reflux (VUR), bladder neck (BN) abnormality and abnormal pubic diastasis (PD), and their impact on urinary continence has not yet been systematically studied. OBJECTIVE: The goal of this study was to evaluate whether the more proximal level of epispadias correlated with associated extragenital anatomic anomalies seen on initial imaging or endoscopic evaluation, and whether these pre-operative findings contributed to subsequent surgical management and impacted on achieving urinary continence...
October 26, 2017: Journal of Pediatric Urology
J Traceviciute, N Zwink, E Jenetzky, H Reutter, K Hirsch, R Stein, W H Rösch, A K Ebert
OBJECTIVE: To investigate sexual function and quality of life (QoL) in adult male individuals with exstrophy-epispadias complex (EEC). Data from the German network for congenital uro-rectal malformations (CURE-Net) were used. PATIENTS AND METHODS: 51 males (≥18 years) recruited by CURE-Net between 2009 and 2012 were re-contacted per mail and asked to fill out four questionnaires including International Index of Erectile Function (IIEF-5), Cologne assessment of Erectile Dysfunction (KEED), the Short-Form 36 (SF-36) and one self-designed questionnaire about their medical history, current health status, sexual experience...
November 7, 2017: Urology
Saunders T Ching, Carlos R Infante, Wen Du, Amnon Sharir, Sungdae Park, Douglas B Menke, Ophir D Klein
Genital malformations are among the most common human birth defects, and both genetic and environmental factors can contribute to these malformations. Development of the external genitalia in mammals relies on complex signaling networks, and disruption of these signaling pathways can lead to genital defects. Islet-1 (ISL1), a member of the LIM/Homeobox family of transcription factors, has been identified as a major susceptibility gene for classic bladder exstrophy in humans, a common form of the bladder exstrophy-epispadias complex (BEEC), and is implicated in a role in urinary tract development...
November 8, 2017: Human Molecular Genetics
Ranjiv I Mathews
No abstract text is available yet for this article.
September 19, 2017: Journal of Pediatric Urology
Mahsa Shabaninia, Ali Tourchi, Heather Di Carlo, John Gearhart
OBJECTIVE: To investigate the state of autophagy and its interactions with apoptosis and cell proliferation in patients who underwent successful early closure or delayed closure of exstrophy. They compared those outcomes with cell culture samples from patients with VUR as control. METHODS: Primary cultures of bladder smooth muscle cells were established from patients with successful neonatal bladder closure (group 1, N=5), delayed closure due to small bladder template (group 2, N=5) and vesicoureteral reflux as control (group3, N=5)...
October 3, 2017: Urology
Joan Ko, Kathy Lue, Daniel Friedlander, Timothy Baumgartner, Peter Stuhldreher, Heather N DiCarlo, John P Gearhart
OBJECTIVE: To explore the long-term outcomes and indications for cystectomy in bladder exstrophy patients. Although rare, cystectomy is the final surgical alternative to bladder repair among these patients with a poor quality bladder template. METHODS: A prospectively maintained database of 1298 patients with exstrophy-epispadias complex was reviewed for patients who underwent cystectomy between 1970 and 2015 at the author's institution. Demographic data, indication for cystectomy, surgical history, post-operative outcomes, and continence status were collected...
September 25, 2017: Urology
Alejandra Vilanova-Sánchez, Christina B Ching, Alessandra C Gasior, Karen Diefenbach, Richard J Wood, Marc Levitt
Cloacal exstrophy is the most severe type of anorectal malformations that belongs to the bladder-exstrophy-epispadias complex of genitourinary malformations. Interestingly, its variant, the covered cloacal exstrophy, is often missed. The clinical findings of this variant may include an imperforate anus, low lying umbilicus, thick pubic bone, and pubic diastasis but with an intact abdominal wall. We present an interesting case of covered cloacal exstrophy with a side-by-side duplicated bladder and discuss important considerations for the time of colostomy creation in the newborn period...
January 2017: European Journal of Pediatric Surgery Reports
K Mamatha, B R Yelikar, Varsha R Deshpande, B S Disha
Omphalocele, Exstrophy of cloaca, Imperforate anus and Spinal defects (OEIS) is a severe manifestation of exstrophy-epispadias sequence with a combination of defects including OEIS. It results from improper closure of anterior abdominal wall and defective development of cloaca and urogenital septum due to defect in blastogenesis during the 4(th) week of gestation. Identification of this complex is important through foetal autopsy as this condition can recur in siblings. Prenatal diagnosis also helps to prevent foetal death with appropriate management in the less severe cases...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
Ali Tourchi, Mahsa Shabaninia, Meghan Stewart, Hiroshi Miyamoto, Heather Di Carlo, John Gearhart
OBJECTIVE: To investigate corporal tissue viability and changes in endothelial content following current techniques used for epispadias repair in animal model MATERIALS AND METHODS: Sixty rabbits were allocated into three groups: Sham operation (penile degloving), complete disassembly model, and Cantwell-Ransley model. On weeks 2, 4, 12, and 24 post-op, the penile tissue was harvested and processed for (i)Masson's trichrome staining for smooth muscle cell(SMC)/collagen ratios (ii)immunohistochemical staining for endothelial factor(CD31) and TGF-β1 (iii)TUNEL assay to detect apoptosis...
September 6, 2017: Urology
Selçuk Sarıkaya, David John Ralph
The neophallus creation is still a mystery and it remains challenging even today. In this article, we performed a comprehensive review of the literature regarding phalloplasty and penile reconstructive surgery between January 2008 and May 2016. In this review, we have included 15 research articles and the results of 276 patients were examined. Studies revealed several indications and when indications were reviewed, 191 patients were female-to-male transgender, 9 patients had disorder of sex development/micropenis, 16 had penile amputation/trauma, 9 had ambiguus genitalia, 40 had exstrophy and/or epispadias, 11 had other problems...
September 2017: Turkish Journal of Urology
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