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https://www.readbyqxmd.com/read/29681696/technique-of-antireflux-procedure-without-creating-submucosal-tunnel-for-surgical-correction-of-vesicoureteric-reflux-during-bladder-closure-in-exstrophy
#1
Kanoujia Sunil, Archika Gupta, Digamber Chaubey, Anand Pandey, Shiv Narain Kureel, Ajay Kumar Verma
Aim: 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. Materials and Methods: 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...
April 2018: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/29657022/female-epispadias-single-stage-approach-a-technique-to-achieve-continence
#2
João Vitor Quadra Vieira Dos Santos, Fernando J Abreu, Luis Fernando C Castro, Patric M Tavares, Brasil S Neto, Tiago Elias Rosito
This video provides a case report of a 3 year old girl with epispadia and the highlights of the surgery. A cystoscopic guided bladder neck plication was performed to achieve continence. Key points include: (1) Skin incision planning; (2) Cutaneous flap liberation to create a new urethra; (3) Complete bladder neck release to allow a controlled plication; (4) Use of cystoscopy to achieve the ideal bladder neck closure; (5) Bladder neck manipulation to achieve continence.
March 15, 2018: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/29619236/evaluation-of-the-isl1-gene-in-the-pathogenesis-of-bladder-exstrophy-in-a-swedish-cohort
#3
Samara Arkani, Jia Cao, Johanna Lundin, Daniel Nilsson, Thomas Källman, Gillian Barker, Gundela Holmdahl, Christina Clementsson Kockum, Hans Matsson, Agneta Nordenskjöld
Bladder exstrophy is a congenital closure defect of the urinary bladder with a profound effect on morbidity. Although the malformation is usually sporadic, a genetic background is supported by an increased recurrence risk in relatives, higher concordance rates in monozygotic twins and several associated chromosomal aberrations. Recently, the ISL1 gene was presented as a candidate gene for bladder exstrophy and epispadias complex (BEEC) development in two different studies. In our study, we screened for genetic variants in the ISL1 gene in DNA from 125 Swedish patients using Sanger sequencing and array-CGH analysis...
2018: Human Genome Variation
https://www.readbyqxmd.com/read/29555156/achieving-urinary-continence-in-cloacal-exstrophy-the-surgical-cost
#4
Mahir Maruf, Matthew Kasprenski, John Jayman, Seth D Goldstein, Karl Benz, Timothy Baumgartner, John P Gearhart
INTRODUCTION: Cloacal exstrophy (CE) is a severe midline congenital abnormality that requires numerous surgical corrections to achieve an acceptable quality of life. Candidates for urinary continence undergo multiple procedures, most often continent bladder diversions, to become socially dry. Here, the authors investigate the number of genitourinary interventions that patients with CE undergo to attain urinary continence. MATERIALS AND METHODS: A retrospective review of a prospectively maintained database of 1311 exstrophy epispadias complex patients was performed...
February 24, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29516095/a-model-for-sustained-collaboration-to-address-the-unmet-global-burden-of-bladder-exstrophy-epispadis-complex-and-penopubic-epispadis-the-international-bladder-exstrophy-consortium
#5
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
https://www.readbyqxmd.com/read/29505857/genital-mobilization-of-intravesical-phallus-associated-with-covered-cloacal-exstrophy-a-case-report
#6
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
https://www.readbyqxmd.com/read/29504860/surgical-and-endoscopic-treatment-of-bladder-exstrophy-epispadias-complex-in-a-female-dog
#7
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
https://www.readbyqxmd.com/read/29502980/male-sexuality-fertility-and-urinary-continence-in-bladder-exstrophy-epispadias-complex
#8
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
https://www.readbyqxmd.com/read/29499975/the-double-dutch-technique-a-new-way-of-creating-an-ileocystoplasty-with-a-lengthy-catheterizable-ileal-tube
#9
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
https://www.readbyqxmd.com/read/29447946/bladder-re-augmentation-in-classic-bladder-exstrophy-risk-factors-and-prevention
#10
Karl S 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 (CBE). METHODS: A prospectively maintained institutional database of 1327 exstrophy-epispadias complex patients was reviewed for patients with CBE who underwent more than 1 augmentation cystoplasty (AC) procedure. Data regarding bladder capacities, complications following AC, and reasons for re-augmentation were evaluated. RESULTS: A total of 166 patients with CBE underwent AC...
February 12, 2018: Urology
https://www.readbyqxmd.com/read/29426576/primary-female-epispadias-perineal-approach-or-kelly-repair
#11
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
https://www.readbyqxmd.com/read/29370895/pelvic-and-lower-extremity-immobilization-for-cloacal-exstrophy-bladder-and-abdominal-closure-in-neonates-and-older-children
#12
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
https://www.readbyqxmd.com/read/29368874/preoperative-care-of-polypoid-exposed-mucosal-template-in-bladder-exstrophy-the-role-of-high-barrier-plastic-wraps-in-reducing-inflammation-and-polyp-size
#13
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
https://www.readbyqxmd.com/read/29318419/prolapsed-bladder-following-rupture-of-patent-urachal-cyst-mimicking-bladder-exstrophy-a-case-report-and-literature-review
#14
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
https://www.readbyqxmd.com/read/29150195/anatomic-findings-associated-with-epispadias-in-boys-implications-for-surgical-management-and-urinary-continence
#15
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
https://www.readbyqxmd.com/read/29126846/sexual-function-and-quality-of-life-in-adult-male-individuals-with-exstrophy-epispadias-complex-a-survey-of-the-german-cure-network
#16
Justina Traceviciute, Nadine Zwink, Ekkehart Jenetzky, Heiko Reutter, Karin Hirsch, Raimund Stein, Wolfgang H Rösch, Anne-Karoline 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 Urorectal Malformations (CURE-Net) were used. PATIENTS AND METHODS: Fifty-one male participants (≥18 years) recruited by CURE-Net between 2009 and 2012 were re-contacted per mail and asked to fill out 4 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, and sexual experience...
February 2018: Urology
https://www.readbyqxmd.com/read/29126155/isl1-mediates-mesenchymal-expansion-in-the-developing-external-genitalia-via-regulation-of-bmp4-fgf10-and-wnt5a
#17
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...
January 1, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/28988809/commentary-on-primary-female-epispadias-perineal-approach-or-kelly-repair
#18
Ranjiv I Mathews
No abstract text is available yet for this article.
September 19, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28986224/autophagy-apoptosis-and-cell-proliferation-in-exstrophy-epispadias-complex
#19
Mahsa Shabaninia, Ali Tourchi, Heather Di Carlo, John P 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 vesicoureteral reflux as control. PATIENTS AND METHODS: Primary cultures of bladder smooth muscle cells (SMCs) were established from patients with successful neonatal bladder closure (group 1, N = 5), delayed closure because of small bladder template (group 2, N = 5), and vesicoureteral reflux as control (group 3, N = 5)...
January 2018: Urology
https://www.readbyqxmd.com/read/28958802/cystectomy-in-the-pediatric-exstrophy-population-indications-and-outcomes
#20
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
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