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https://www.readbyqxmd.com/read/29150195/anatomic-findings-associated-with-epispadias-in-boys-implications-for-surgical-management-and-urinary-continence
#1
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
#2
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
https://www.readbyqxmd.com/read/29126155/isl1-mediates-mesenchymal-expansion-in-the-developing-external-genitalia-via-regulation-of-bmp4-fgf10-and-wnt5a
#3
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
https://www.readbyqxmd.com/read/28988809/commentary-on-primary-female-epispadias-perineal-approach-or-kelly-repair
#4
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
#5
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
https://www.readbyqxmd.com/read/28958802/cystectomy-in-the-pediatric-exstrophy-population-indications-and-outcomes
#6
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
https://www.readbyqxmd.com/read/28924534/image-of-the-month-clinical-features-in-a-newborn-with-covered-cloacal-exstrophy
#7
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
https://www.readbyqxmd.com/read/28892921/a-rare-case-of-genital-malformation-with-omphalocele-exstrophy-of-bladder-imperforate-anus-and-spinal-defect-complex-autopsy-findings
#8
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
https://www.readbyqxmd.com/read/28888749/complete-penile-disassembly-for-repair-of-epispadias-causes-erectile-tissue-alteration-through-tgf-%C3%AE-1-overexpression-in-rabbit-model
#9
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
https://www.readbyqxmd.com/read/28861290/mystery-and-realities-of-phalloplasty-a-systematic-review
#10
REVIEW
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
https://www.readbyqxmd.com/read/28721463/exstrophy-bladder-reconstruction-or-diversion-for-the-underprivileged
#11
REVIEW
Yogesh Kumar Sarin, Virender Sekhon
The surgical techniques for management of bladder exstrophy epispadias complex have evolved from staged reconstruction, complete primary repair to radical mobilization. Post-operative complications add to the multiplicity of surgical procedures at each step. The end results are variable with many achieving continence rates of 85-89% only after bladder augmentation and clean intermittent catheterization. The situation is further complicated in resource-poor settings, where illiteracy and poverty are the driving factors for choosing a single operative procedure for creation of low pressure reservoir aiming at upper tract preservation and good primary continence...
September 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28712888/is-concealed-epispadias-a-rare-variant
#12
Ahmed Shahat, Ahmed S Safwat, Ahmad Elderwy, Islam F Abdelkawi, Ahmad Elbadry I Abonnoor, Mohamed F Abdelhafez, Ahmed Moeen, Hisham Hammouda
OBJECTIVE: To present our experience with concealed epispadias and to estimate its actual share in the isolated male epispadias cases and its effect on the surgical outcome. MATERIALS AND METHODS: Consecutive patients with isolated male epispadias treated in our center between 2008 and 2015 were classified into concealed and classic epispadias. The 2 groups were compared regarding age at presentation, meatal location, incontinence, dorsal curvature, success rate, and complications...
November 2017: Urology
https://www.readbyqxmd.com/read/28689649/epispadias-repair-with-tunica-vaginalis-flap
#13
R S Zee, J H Makari, Fernando Ferrer, C D A Herndon
INTRODUCTION: Primary epispadias is a rare congenital malformation that afflicts about 1 in 80,000 children. The surgical repair, originally described by Cantwell over 100 years ago, is fundamentally sound and incorporates several important steps to achieve an optimal result. METHODS: A 9-month-old male with penopubic epispadias presented for surgical repair. Pre-operative imaging included a normal renal ultrasound and voiding cystourethrogram that demonstrated a moderate-sized bladder, partially competent bladder neck, and no vesicoureteral reflux...
October 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28652161/the-surgical-management-of-bladder-polyps-in-the-setting-of-exstrophy-epispadias-complex
#14
John Jayman, Ali Tourchi, Mahsa Shabaninia, Mahir Maruf, Heather DiCarlo, John P Gearhart
OBJECTIVE: To investigate the surgical course for treating bladder polyps in patients with exstrophy epispadias complex (EEC). EEC bladder polyps are unique pathologic entities, with a distinct difference between polyps discovered at birth and polyps developed after failed exstrophy closure. METHODS: A prospectively maintained database of 1300 patients with EEC was reviewed for bladder exstrophy patients with bladder polyps. The following data were obtained: patient demographics, polyp type, past medical and surgical history, and continence outcomes...
November 2017: Urology
https://www.readbyqxmd.com/read/28615813/modified-cantwell-ransley-repair-for-isolated-continent-epispadias-in-adult-our-experience
#15
Venkat Arjunrao Gite, Hitesh Mahendra Jain, Sachin M Bote, Jayant Vijay Nikose
PURPOSE: We evaluate here our experience with modified Cantwell-Ransley technique described by Gearhart for correction of isolated continent epispadias in adults with respect to its long-term functional outcome and complications. INTRODUCTION: Isolated male epispadias is a rare anomaly with a reported incidence of 1 in 11,700 males. Many surgical techniques with various modifications described to correct epispadias as treatment are debatable and challenging. The majority of the cases are treated at childhood as clinical presentation is striking at birth; hence, presentation in adulthood is extremely rare...
January 2017: Indian Journal of Plastic Surgery: Official Publication of the Association of Plastic Surgeons of India
https://www.readbyqxmd.com/read/28534165/-the-german-research-network-cure-net-a-benefit-for-patients-with-rare-diseases
#16
REVIEW
A-K Ebert
With the help of the media, there is growing public awareness for the problems associated with rare diseases and their impact on the lives of those affected and their families. Bladder exstrophy-epispadias complex (BEEC) is also a part of the group of rare diseases within the urological field. The German network CURE-Net was founded in 2009 to systematically collect data regarding the epidemiological and molecular causes, and clinical and psychosocial effects of congenital urorectal malformations. With the help of self-help groups a national registry could be established for systematic data retrieval...
July 2017: Der Urologe. Ausg. A
https://www.readbyqxmd.com/read/28505946/re-clinical-pathway-for-early-discharge-after-complete-primary-repair-of-exstrophy-and-epispadias-by-using-a-spica-cast
#17
Douglas A Canning
No abstract text is available yet for this article.
June 2017: Journal of Urology
https://www.readbyqxmd.com/read/28401037/female-epispadias-presenting-as-urinary-incontinence
#18
Asmir Jonuzi, Nusret Popovic, Zlatan Zvizdic, Emir Milišic, Kenan Karavdic, Dewan Paddy
Isolated female epispadias without bladder exstrophy is a rare congenital anomaly affecting 1 in 484,000 females. The presenting features of female epispadias are urinary incontinence and abnormal anatomical features. A 6-year-old girl presented with primary urinary incontinence who on physical examination had a bifid clitoris and labia minora. The vagina and hymen were normal. Voiding cystourethrogram showed no reflux and a funnel shaped proximal urethra. With the diagnosis of isolated female epispadias, one-stage reconstruction of the urethra, bladder neck, labia minora and clitoris was performed...
March 2017: APSP Journal of Case Reports
https://www.readbyqxmd.com/read/28392008/long-term-follow-up-after-traditional-versus-modified-perineal-approach-in-the-management-of-female-epispadias
#19
F Alyami, N Fernandez, L Lee, P Metcalfe, A Lorenzo, J Pippi Salle
OBJECTIVE: Isolated female epispadias (IFE) is a rare congenital anomaly. The defect extends to the bladder neck, which is usually incompetent. The traditional surgical approach includes urethral and genital reconstruction in the first year, followed by bladder neck reconstruction (Young-Dees-Leadbetter cervicoplasty (YDL)) at the age of social continence. An alternative single-stage technique includes urethral, bladder neck and clitoris repair by a perineal approach. The aim of the present study was to describe long-term follow-up of patients who underwent the traditional vs alternative approach...
October 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28371167/long-term-sexual-health-outcomes-in-men-with-classic-bladder-exstrophy
#20
Timothy S Baumgartner, Kathy M Lue, Pokket Sirisreetreerux, Sarita Metzger, Ross G Everett, Sunil S Reddy, Ezekiel Young, Uzoma A Anele, Cameron E Alexander, Nilay M Gandhi, Heather N Di Carlo, John P Gearhart
OBJECTIVES: To identify the long-term sexual health outcomes and relationships in men born with classic bladder exstrophy (CBE). MATERIALS AND METHODS: A prospectively maintained institutional database comprising 1248 patients with exstrophy-epispadias was used. Men aged ≥18 years with CBE were included in the study. A 42-question survey was designed using a combination of demographic information and previously validated questionnaires. RESULTS: A total of 215 men met the inclusion criteria, of whom 113 (53%) completed the questionnaire...
September 2017: BJU International
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