Application of Tunica Vaginalis Flap for Epispadias Repair in the Epispadias-Exstrophy Complex. To describe long-term outcomes and experience with the tunica vaginalis flap (TVF) as a tissue augment for complex epispadias repair. A prospectively maintained institutional database was reviewed for patients who underwent epispadias repair with TVF from 2010 to 2021. Evaluation of patient characteristics between those who developed dehiscence or UCF and those who did not was performed via Mann-Whitney U, Kruskal-Wallis, or Fisher's exact test, as appropriate. A total of 47 male patients were identified, of whom, 1 (2.1%) had cloacal exstrophy, 38 (80.9%) had classic bladder exstrophy or a variant, and 8 (17.0%) had epispadias as their primary exstrophy-epispadias complex diagnosis. The median
A Rocky Road: Bladder Stones in the Augmented Exstrophy-Epispadias Complex Patient. To determine the rate of stone formation amongst patients of the exstrophy-epispadias complex with augmentation cystoplasty. We hypothesize that bowel segment choice influences the rate of stone formation after bladder augmentation and the rate of complications from bladder stone surgery. An IRB approved institutional database of 1512 exstrophy-epispadias patients was reviewed retrospectively. Patients that had a history of bladder augmentation and were seen at our institution between 2003 and 2023 were included. Out of 259 patients, bladder stones developed in 21.6% (56), of which the bowel segment used was colon in 147 patients and ileum in 100. Stones formed in 19% of colon augments compared to 29% ileal
Assessment of Anorectal Function and Related Quality of Life of 27 Patients with Bladder Exstrophy or Epispadias After Kelly Radical Soft Tissue Mobilisation. The Radical soft-tissue mobilisation (RSTM) described by J.H. Kelly for bladder exstrophy repair implies a detachment of levator ani muscle insertions from the pelvic wall. The aim of this controlled study was to evaluate the impact
Bladder Neck Surgery is not Routinely Needed to Achieve Urinary Continence in Patients with Primary Epispadias. This study aims at investigating the continence outcome in primary epispadias patients treated at a tertiary center. The authors hypothesized that additional continence procedures following primary epispadias repair is not routinely needed. Patients treated for primary epispadias with family/patient if still incontinent. urinary continence. PFM biofeedback therapy, continence surgery, hydronephrosis. Type of epispadias, age at repair and follow-up presented as median was also reported. Thirty-three patients (29 males) were included. Twelve had penopubic epispadias, 13 glanular/penile, 4 duplicated urethra, 4 females. Median age at repair: 2 years (IQR 1-3), at follow-up: 8 years
Prenatal Presentation of a Covered Cloacal Exstrophy Variant; Early Diagnostic Challenges within the Broad Spectrum of the Exstrophy-Epispadias Complex. There is a broad range of variant phenotypes that can occur within the bladder exstrophy and epispadias complex spectrum. Accurate prenatal detection helps prepare families and to coordinate subspecialty resources. Here, we present the case of a patient with prenatally diagnosed patient with covered cloacal exstrophy variant along with four additional cases illustrating the nonlinear spectrum from isolated epispadias to cloacal exstrophy. Given the rarity of these variants overall and of each subtype within the spectrum, there is a need for long-term multi-institutional outcomes data to improve detection, characterization, and prognostication
Introduction of a modified single stage reconstruction technique of male penopubic epispadias. To represent the long-term outcomes of our modified single-stage technique for the reconstruction of isolated penopubic epispadias in male patients. Data from 113 patients were obtained from bladder-exstrophy-epispadias database of our tertiary center. A total of seven boys with isolated penopubic epispadias with no prior history of surgery and any other anomaly underwent our modified surgical approach from February 1997 to September 2019. The mean ± SD age at surgery was 6.5 ± 2.4 years. Volitional voiding status and cosmetic appearance were evaluated at each follow-up interval. Postoperative follow-up was performed at quarterly intervals in the first year and once a year in subsequent years
Justin H. Kelly and his procedure for bladder exstrophy and epispadias. The operations involved in the repair of complete bladder exstrophy (CBE), familiarly known as the Kelly procedure, evolved over more than 100 years. Through repeated cycles of trial and error, some of the most prominent urologists in the world developed techniques that addressed each of the formidable surgical challenges presented by CBE and epispadias. A key figure is Justin H. Kelly of the Royal Children's Hospital, Melbourne, who made surgery for CBE his life's work. He took the lessons of his surgical predecessors, giants like: Friedrich Trendelenburg, Hugh Hampton Young, John Dees, and Guy Leadbetter, applied techniques for anorectal anomalies from his contemporary Alberto Peña, and saw his procedures improved
Exstrophy-Epispadias Complex With Isolated Ectopic Bowel Segment: A Case Series and Literature Review. To present a case series of the exstrophy-epispadias complex (EEC) with isolated ectopic bowel segment (IEBS) with the literature review, highlighting the clinical findings and treatments. We present 3 cases of bladder exstrophy (BE) with IEBS in our institute and reviewed the literature in PubMed with the terms "("bladder exstrophy" OR "epispadias") AND ("visceral sequestration" OR "sequestered" OR "ectopic bowel")." There were 2 males and 1 female. The IEBS was detected by physical examination in 2 cases and by ultrasonography in another one. All cases were BE accompanying with lower abdominal mass which adhered to the bladder wall but was separated from the digestive system. All cases
Pelvic Floor Anatomical Variations in Children With Exstrophy-Epispadias Complex Using Magnetic Resonance Imaging. To quantitatively measure the anatomical variations of the pelvic floor in children with exstrophy-epispadias complex using magnetic resonance imaging. Six cases of classic bladder exstrophy (CBE), 5 cases of penile epispadias (PE) and 11 cases of penopubic epispadias (PPE) were
Dextranomer Endoscopic Injections for the Treatment of Urinary Incontinence in Bladder Exstrophy Epispadias Complex. Since bladder neck dextranomer/hyaluronic acid copolymer (Dx/Ha, Deflux) injections can improve urinary incontinence of various etiologies, we hypothesized that incontinent children with Bladder Exstrophy-Epispadias Complex (BEEC) would benefit from Dx/Ha. We aimed to analyze Dx , bladder emptying mode and bladder capacity were reported. Failure-free survival was estimated by Kaplan Meier models. Altogether 58 patients (27 male epispadias, 9 female epispadias, 22 bladder exstrophy) underwent 105 injections at median age of 8.5 (interquartile range 6.1-11) years. Previous bladder neck reconstruction (BNR) had been performed in 38 (66%) and 33 (57%) received multiple injections
Development of Late Continence in Bladder Exstrophy and Epispadias Patients. To evaluate to what extend urinary continence develops during puberty in patients with classic bladder exstrophy (CBE) and epispadias. The operative database was reviewed for surgical procedures and urinary continence in all 65 CBE and epispadias patients born in 1976-2005. Excluded were 2 patients who had insufficient incontinence (P = .04); 3/9 males with CBE, 10/13 males with epispadias, 3/5 females with CBE, and 0/4 females with epispadias (P = .25 between sex and P = .48 between diagnosis). Of the 15 patients remaining incontinent, 10 had daily and 5 had rare incontinence episodes. The latest control was at the median age of 19 years (interquartile range 17-21 years). Twenty-five patients (40% of the whole material
Utility of Skin Grafting and Tissue Expansion in Penile Reconstruction for the Exstrophy-Epispadias Complex. To describe the use of additional tissue recruited for coverage after penile lengthening in male exstrophy-epispadias complex patients using either local skin from tissue expansion (TE) or extragenital skin with a skin graft (SG) and report their respective outcomes. An institutionally approved database of exstrophy-epispadias complex patients was retrospectively reviewed for male patients who received penile reconstruction. This included a penile lengthening procedure and the subsequent use of TE and/or a full thickness skin graft to provide cutaneous coverage of gained corporal length. A total of 50 patients (mean age 18.1 years) underwent penile reconstruction. TE was used in 27
Urological, Sexual, and Quality of Life Evaluation of Adult Patients With Exstrophy-Epispadias Complex: Long-term Results From a Dutch Cohort. To assess urological function, sexual function, and quality of life in patients with exstrophy or epispadias. Little is known regarding these outcomes in adult patients; our aim is to determine where improvements are needed for long-term management . For sexual function, no difference was found between those in the general population and our participants except for the domain "pain" in Female Sexual Function Index. Adult patients with exstrophy or epispadias have a high rate of incontinence and lower urinary tract symptoms with relatively low to some degree of bother. When compared with the general population, quality of life, and sexual function
Single-stage Abdominoplasty Using Groin Flaps Without Osteotomies: Management of Exstrophy-epispadias Complex. The optimal treatment for children born with exstrophy-epispadia complex is still a matter of debate. We demonstrate the Single-Stage Abdominoplasty using Groin Flap technique to close the abdominal wall of children with classic bladder exstrophy (CBE) without osteotomy neither radical
ASSOCIATION BETWEEN EXSTROPHY-EPISPADIAS COMPLEX AND CONGENITAL ANOMALIES: A GERMAN MULTICENTER STUDY. To further investigate associated anomalies in exstrophy-epispadias complex (EEC) patients congenital uro-rectal malformations network (CURE-Net) database was systematically screened. In literature the EEC comprises a spectrum of anomalies, mainly occurring "isolated" without additional
The Role of Human Acellular Dermis in Preventing Fistulas after Bladder Neck Transection in the Exstrophy-Epispadias Complex. To evaluate human acellular dermis (HAD) as an adjunct during bladder neck transection (BNT) by comparing surgical outcomes with other types of tissue interposition. A prospectively maintained institutional database of exstrophy-epispadias complex (EEC) patients was reviewed for those who underwent a BNT with at least 6 months follow-up. The primary outcome was the occurrence of BNT-related fistulas. In total, 147 EEC patients underwent a BNT with a mean follow-up time of 6.9 years (range 0.52-23.35 years). There were 124 (84.4%) classic exstrophy patients, 22 (15.0%) cloacal exstrophy patients, and 1 (0.7%) penopubic epispadias patient. A total of 12 (8.2%) BNTs
Male Sexuality, Fertility, and Urinary Continence in Bladder Exstrophy-Epispadias Complex. 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. 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. We conducted a descriptive, cross-sectional study of men diagnosed with classic presentations of bladder exstrophy or epispadias. Patients were asked to complete 4 validated questionnaires: the International Index of Erectile Function (IIEF)-5
Surgical Management of Complete Procidentia in a Female Patient with Bladder Exstrophy-epispadias Complex: Case Report and Literature Review We herein describe the operative approach of a postmenopausal woman with a history of surgically corrected congenital bladder exstrophy-epispadias who presented with long-standing complete procidentia. The patient was initially treated by laparoscopic sacral , a successful colpocleisis was performed. This case report emphasizes the complexity of pelvic organ prolapse (POP) in the context of a bladder exstrophy-epispadias complex. These women are more likely to fail the more conventional current surgical treatments for POP, coercing to revert to colpocleisis.
Variant Presentations of the Exstrophy-Epispadias Complex: A 40 Year Experience. To investigate the diagnosis, surgical management, and outcomes in patients with variant EEC. Variant presentations of the exstrophy-epispadias complex (EEC) span a wide range of abnormalities. The rarity and diversity of EEC variants can lead to challenges in the diagnosis and subsequent management of this population. The authors reviewed an institutional database of 1336 EEC patients from 1975 to 2018 for variant presentations of EEC. Variant presentations included those with skin covered bladder exstrophy (BE), duplicate bladders, superior vesical fistula, and epispadias with major bladder prolapse. Surgical management and outcomes were assessed. In total, 44 EEC variants were identified. Nineteen (43