Epispadias Repair Research Articles

Isolated Male Epispadias Repair: Long-Term Outcomes.

Isolated male epispadias is one of the most severe congenital genital anomalies that require surgical correction. The goals of the surgery are to reach good aesthetic and functional outcomes. The aim of this retrospective study was to analyze the long-term outcomes of surgical reconstruction of male epispadias. A total of 31 patients with a mean age of 17 years, who underwent surgical repair of isolated male epispadias from January 2000 to January 2015, were involved. The main outcome measures were defined as: aesthetic outcome, continence, postoperative complications, sexual function, and quality of life. The follow-up period ranged from 8 to 23 years, with an average of 14.4 years. Each patients underwent an average of 2.2 surgical procedures in this period. The most common postoperative complications were urethral fistula and residual curvature, in 22.6% and 12.9%, respectively. Satisfactory aesthetic outcome was reported in 71.4% of cases. The repair of male epispadias usually includes more than two procedures with satisfactory aesthetic outcome. Unsolved urinary incontinence remains a significant issue and has a high impact on the quality of life. Follow-up should be extended even after complete sexual maturity. Comprehensive long-term evaluation is necessary for proper treatment of isolated epispadias.

Male epispadias repair: Outcomes at three sites prior to the establishment of a multi-institutional collaboration

Epispadias, which occurs on the more mild end of the Bladder Exstrophy Epispadias Complex (BEEC) spectrum, presents still with a wide range of severity in boys, from mild glanular epispadias to penopubic epispadias with severe urethral and bladder neck defects. Surgical management ranges from isolated epispadias repair to epispadias repair with bladder neck reconstruction (BNR) with or without pelvic osteotomies. We aimed to evaluate outcomes in epispadias treated at three institutions prior to formation of a formal collaboration. In addition, we sought to delineate outcomes based on anatomic severity at time of diagnosis, and initial procedure performed in cases of penopubic epispadias. IRB approved databases were retrospectively queried at three institutions for patients who underwent repair of epispadias between 1/1993 and 1/2013. Degree of epispadias, age and technique at initial repair, and self-reported continence status at last follow-up were recorded. Continence was categorized as: wet, intermediate (dry 2-3h), or dry, while also distinguishing those who void and those who require clean intermittent catheterization (CIC). Those not seen since 1/1/2015, younger than 10 years at last follow up, or in whom continence data were not recorded were excluded. A total of 48 boys were identified; 36 met inclusion criteria. The epispadias cohort consisted of 8 glanular epispadias (GE) (22%); 8 penile epispadias (PE) (22%), and 20 penopubic epispadias (PPE) (56%) with a median follow-up of 11.3 years (3.2-26.2 years). Overall, 33 of 36 (92%) boys void per urethra. Within the group that voids, 19/33 (58%) are completely dry, while 6/33 (18%) are wet. Among patients who underwent initial epispadias repair without concurrent or subsequent bladder neck reconstruction, continence rates were: GE 63% (5/8); PE 75% (6/8); PPE 71% (5/7). Among the 9 boys with PPE who underwent initial epispadias repair with concurrent BNR, 22% (2/9) were dry with no further surgeries. Overall, 8/20 (40%) of boys with PPE void with complete dryness. This multi-center retrospective review of continence in epispadias demonstrates that even some boys with glanular and penile epispadias can have challenges with continence, and boys with penopubic epispadias may remain wet despite careful preoperative assessment of bladder neck functionality and concurrent BNR. Continence outcomes in boys with all degrees of epispadias can be variable. Even boys with more distal defects may have significant bladder neck deficiency. And those with the most severe form of epispadias may require bladder neck reconstruction to achieve continence.

Redo surgery to improve urinary function, sexual function and cosmesis in male patients with exstrophy–epispadias complex complications. Technical principles and pearls based on case scenarios

The bladder exstrophy-epispadias complex includes some of the most challenging conditions treated by pediatric urologists. They are associated with the need for multiple intricate reconstructive procedures, aimed at restoring the anatomy and function of the bladder, urethra and external genitalia. These patients often endure multiple redo reconstructive procedures to improve urinary function, sexual function and cosmesis throughout the first two decades of life. In this article, we present the 30-year experience of a single surgeon performing redo surgery for males born with epispadias and bladder exstrophy. Through detailed documentation of 6 clinical cases, we highlight technical aspects that may contribute to a successful surgical reconstruction in these patients. The article is focused specifically on patients undergoing redo epispadias repair with or without concomitant continence procedures. We make the case for complete penile disassembly with external rotation of the corpora to correct recurrent dorsal curvature; this approach also allows the surgeon to have access to the proximal urethra and bladder neck after opening the intersymphiseal scar/band. This is useful when additional procedures on the bladder, such as bladder neck tailoring, are necessary. We also highlight the importance of avoiding reverse Byars' flaps when performing skin closure, due to the resulting midline scar. Besides being associated with a poor cosmetic outcome, it can also contribute to recurrent dorsal curvature. The authors advocate for rotational skin flaps to cover the penile shaft. Correction of dorsal curvature and improved cosmesis obtained with complete penile disassembly sometimes comes at the expense of the urethra being left as a hypospadias (figure). This will require further surgeries (usually a 2-stage buccal mucosa graft), much like the treatment of proximal hypospadias. Redo epispadias surgery in males remains a challenge. The systematic approach offered by the case scenarios may help guide surgeons dealing with this difficult condition. Patient with complications after repair of classic bladder exstrophy. A) Stone retrieved from posterior urethra after complete penile disassembly. B) After opening the inter-symphiseal scar, the bladder has been opened and the bladder neck tailored. C) Complete penile disassembly has been completed with corporal bodies and urethra individualized. D,E,F) Final appearance of the repair; abdominal wall was closed with anterior rectus sheath flaps, penile skin was closed with rotational flaps and urethra ended up as a hypospadias.

A novel modified penile disassembly procedure for isolated male epispadias repair: preliminary outcomes.

This study aimed to evaluate the preliminary outcome of a novel modification of a penile disassembly procedure. We retrospectively reviewed the data of 15 patients with isolated male epispadias (IME) who underwent a modified penile disassembly procedure. This modification involved leaving the glans connected while dissecting the urethral plate from the corpus cavernosum. This approach reserves the bundles of the anastomosis at the glans, which can prevent ischemic changes. One of the first two patients experienced glans ischemic changes on the first day after the operation, which ultimately resulted in the loss of half of the hemiglans. Urethral plate shortening was observed in two patients and was addressed with a transverse preputial island flap. Two patients developed a penopubic urethral fistula, which was repaired 6 months postoperatively. Of the 15 patients, 10 (66%) were continent or social continent, while 4 were incontinent and needed bladder neck reconstruction. One patient had not yet reached the age for continence evaluation. Additionally, Two patients had a residual dorsal curvature of approximately 10-15° and were advised to undergo continuous follow-up. The modified penile disassembly procedure is a simple, effective, and safe method for IME repair with an acceptable penile appearance and complication rate.

Bladder Neck Surgery is not Routinely Needed to Achieve Urinary Continence in Patients with Primary Epispadias

BackgroundThis 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. MethodsPatients treated for primary epispadias at the authors’ institution between 2007 and 2019 and toilet trained, were identified from a prospective maintained database. Males underwent chordee correction, urethroplasty and glanuloplasty. Females underwent genitoplasty with reduction urethroplasty. If continence was not achieved by 4–5 years of age, pelvic floor muscle (PFM) biofeedback therapy was performed. Other continent procedures were discussed with family/patient if still incontinent. Primary outcome: urinary continence. Secondary outcomes: PFM biofeedback therapy, continence surgery, hydronephrosis. Type of epispadias, age at repair and follow-up presented as median was also reported. ResultsThirty-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 (IQR 6–10). Daytime continence: 100 % in penile/glanular; 33 % in penopubic and 75 % in duplicated urethra. Nighttime continence: respectively 92 %, 50 % and 100 %. 24 % of males were intermittently incontinent. All patients except one voided urethrally. One patient underwent bladder neck closure, ileocystoplasty and Mitrofanoff. One girl achieved daytime continence, 2 were intermittently incontinent, one continuously incontinent. All were enuretic. 38 % of boys and 100 % of girls had biofeedback therapy. None had hydronephrosis/renal impairment. ConclusionsMost children with primary epispadias can achieve social urinary continence spontaneously or with the support of PFM biofeedback therapy. Other continence procedures should be reserved for patients who do not attain satisfactory continence. Level of EvidenceTreatment study – level IV.

Long term evaluation of continence after complete primary bladder exstrophy repair

Continence after bladder exstrophy (BE) repair remains a major debatable challenge to pediatric urologists, together with the lack of standard definitions and long-term results in large series. We assessed the long-term urinary continence in 142 toilet-trained cases after one (1-) stage of complete primary repair of bladder exstrophy (CPRE) and consequent procedures to achieve this goal in a single tertiary referral center. The current retrospective study included 123 boys and 19 girls with BE that were repaired by (1-) stage CPRE. The Mean age at (BE) repair was 9.5±2.6 weeks. Complete penile disassembly (CPD) was used for epispadias repair in 42 (34.1%) and modified Cantwell-Ransley repair (MCR) was used in 81 (65.9%) boys. Bilateral anterior transverse innominate osteotomies (ATIO) were applied in all. Urinary continence was expressed in terms of the dry interval (DI). Continence procedures were afforded if CPRE failed to achieve DI≥3h (hrs.), those were in the form of endoscopic bladder neck injection (BNI), bladder neck reconstruction (BNR), and bladder neck closure (BNC) with catheterizable stoma. The mean age at follow up was 12.1±5.2 years. DI≥3h was gained in 23 (16.2%) after CPRE alone, while complementary post-CPRE continence procedures were required to reach this goal in the remaining patients. Deflux injection was reported in 10 (7%), CIC in 8 (5.6%), BNR in 32 (22.5), and BNC with catheterizable stoma alone in 37 (26.1%), or with Charleston pouch in 32 (22.5%). We think that ≥3h DI with voiding represents an appropriate definition of continence after BE repair. According to the results in the current series, we think that successful anatomical closure of BE is achievable, but the functional outcome in terms of continence and its evaluation is tricky. Results of continence were reported to change with age of the child, and it is difficult to evaluate both before toilet training age and long-term follow up. Long-term follow up of CPRE with bilateral ATIO alone or with BNI results in ≥3h DI in a few cases; BNR after CPRE can provide a good chance for continence; otherwise, BNC with catheterizable stoma is a valid option.

Significant extravasation does not preclude conservative management of spontaneous perforation of the augmented bladder

BackgroundPerforation of the augmented bladder is a serious and well-known complication of bladder augmentation. The traditional treatment has been emergent surgical exploration and repair of the bladder perforation due to the risk of peritonitis, sepsis and mortality. Some studies have reported successful conservative management by maximizing bladder drainage in patients with stable hemodynamics and limited peritoneal extravasation. Herein, we report the successful conservative management of spontaneous perforation of augmented bladder in a 5-year-old boy with exstrophy-epispadias complex following epispadias repair with significant extravasation.Case presentationA 5-year-old boy had augmentation ileocystoplasty to facilitate repair of failed bladder exstrophy closure. Modified penile disassembly was used for epispadias repair with a transurethral catheter draining the bladder. The patient presented on postoperative day 2 with a non-draining catheter, fever, repeated vomiting and abdominal distension. Leukocytosis and elevated creatinine were evident on laboratory work-up. CT cystogram confirmed the diagnosis of spontaneous perforation of the augmented bladder with significant intraperitoneal extravasation. Conservative management was successful by inserting a suprapubic catheter and an intraperitoneal drain. The patient subsequently underwent creation of Mitrofanoff appendicovesicostomy and bladder neck closure to achieve dryness without recurrence of perforation on follow-up.ConclusionsPerforation of the augmented bladder can be managed conservatively even in the presence of significant peritoneal extravasation by maximizing bladder drainage and insertion of an image-guided intraperitoneal drain.

MP49-07 THE UGANDAN BLADDER EXSTROPHY-EPISPADIAS COMPLEX INITIATIVE: A PROGRAM FOR TRANSFERENCE OF COMPLEX SURGICAL RECONSTRUCTIVE SKILLS

You have accessJournal of UrologyCME1 Apr 2023MP49-07 THE UGANDAN BLADDER EXSTROPHY-EPISPADIAS COMPLEX INITIATIVE: A PROGRAM FOR TRANSFERENCE OF COMPLEX SURGICAL RECONSTRUCTIVE SKILLS Anthony Schaeffer, Pamela Artigas, Amanya Colline, Marlo Eldridge, Janet Gibson, Emily Haddad, Martin Situma (Posthumous), Stephen Watya, and Ranjiv Mathews Anthony SchaefferAnthony Schaeffer More articles by this author , Pamela ArtigasPamela Artigas More articles by this author , Amanya CollineAmanya Colline More articles by this author , Marlo EldridgeMarlo Eldridge More articles by this author , Janet GibsonJanet Gibson More articles by this author , Emily HaddadEmily Haddad More articles by this author , Martin Situma (Posthumous)Martin Situma (Posthumous) More articles by this author , Stephen WatyaStephen Watya More articles by this author , and Ranjiv MathewsRanjiv Mathews More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000003297.07AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Patients with the bladder exstrophy-epispadias (BE) complex in resource limited countries lack access to readily available state-of-the-art BE care. We aim to describe a collaboration between US-based BE experts and Ugandan care providers to address this unmet need. METHODS: Described below. RESULTS: Need: Despite 43 million citizens, Uganda has only 5 pediatric surgeons and 1 pediatric urologist. Ugandans have sparse access to BE surgical care, and if unable to obtain adequate BE care have limited educational and/or employment prospects. Advanced training in state-of-the-art BE operative and perioperative care for healthcare providers is also limited. Response: As the largest BE patient/parent support group, the Association for the Bladder Exstrophy Community (A-BE-C) has partnered with US-based BE experts, a Ugandan hospital, and local healthcare providers to address this gap. The mission of this 5-year program is to provide hands-on BE skills training to Ugandan surgeons, nurses, and social workers and facilitate BE knowledge transfer through an intensive didactic symposium. This is accomplished using the same care team each year. The goal is to develop a self-sustaining program to provide optimal, cost effective BE care in local facilities. This program also benefits urologists, nurses, and social workers who are members of the College of Surgeons of East Central and Southern Africa. Outcomes: An initial planning visit occurred in 2018. The first of 5 yearly, 7 day long symposia commenced in October 2019 when 18 patients received care (1 delayed closure w/ osteotomy, 1 epispadias repair, 16 cystoscopies for treatment planning). 2 females attained urinary continence with new catheterization regimens. Social workers, assigned to each individual patient, are absolutely critical to ensure ongoing patient communication, assist with travel and supply delivery, and liaise with BE providers in Kampala and the US. 7 talks were given to symposia attendees from many East African countries. Challenges: Challenges identified include: the need for pediatric orthopedists with BE experience; selection of the most suitable urinary continence operation; medical supply access/payment/delivery; program cancellations due to global (2020, 2021) and locoregional disease outbreaks (2022). CONCLUSIONS: Partnering with nonprofit organizations and identifying a supportive local facility committed to providing short- and long-term management has been a crucial step in starting to advance BE care in Uganda. Source of Funding: Schaeffer: NIH K08DK119535 © 2023 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 209Issue Supplement 4April 2023Page: e680 Advertisement Copyright & Permissions© 2023 by American Urological Association Education and Research, Inc.MetricsAuthor Information Anthony Schaeffer More articles by this author Pamela Artigas More articles by this author Amanya Colline More articles by this author Marlo Eldridge More articles by this author Janet Gibson More articles by this author Emily Haddad More articles by this author Martin Situma (Posthumous) More articles by this author Stephen Watya More articles by this author Ranjiv Mathews More articles by this author Expand All Advertisement PDF downloadLoading ...

Bladder exstrophy-epispadias complex

A 2-year-old male, with the bladder exstrophy-epispadias complex since birth presents to the department of surgical pediatrics for surgical management. Exposed, everted bladder template is clearly obvious immediately below umbilicus; left and right corpora cavernosa are visible beneath and alongside urethral plate; the scrotum is caudally displaced. A spectrum of congenital defects epispadias, typical bladder exstrophy, and cloacal exstrophy, make up the exstrophy-epispadias complex (EEC). Nelson et al. calculated the total incidence of EEC at 2.15 per 100,000 live births, with an equal male-to-female ratio. Normal invagination of the urogenital system begins at the end of the third week of pregnancy in the intermediate layer of mesoderm, while the lateral plate mesoderm contributes to the formation of the primitive gut tube. EEC develops because of disruption in this interaction, which may be caused by an overgrowth of the cloacal membrane that prevents mesenchymal tissue from migrating medially. The severity of the resulting condition depends on the point at which the interaction between the mesodermal layers is disturbed. Depending on the form and degree of the defect, different surgical procedures are required to rectify it; most neonates, however, require bladder and abdominal wall closure, epispadias repair, ureteral reimplantation, and bladder neck repair.

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 age at epispadias repair was 13 months (IQR 11 - 19). The overall rate of fistula or dehiscence development was 19.1% (9 patients), of whom, 7 developed urethrocutaneous fistulae, 1 dehiscence, and 1 both. Notably, 0 of the 5 patients who had undergone prior epispadias repair and 0 of the 8 patients with a diagnosis of isolated epispadias developed a UCF or dehiscence after repair with TVF. Differences in age at repair, primary EEC diagnosis, prior epispadias repair, pre-repair testosterone, fibrin sealant utilization, closure layers, stent removal time, and bladder capacity at repair were not statistically significant (P>.05) CONCLUSION: Our expanded evaluation indicates that utilization of the tunica vaginalis as an adjunct to epispadias repair may provide durable protection against fistula development in EEC patients who have undergone prior epispadias repair and in primary repair of patients with isolated epispadias.

SEPA Based Flap for Covering Defect after Adult Exstrophy Epispadias Repair

INTRODUCTION
 Bladder exstrophy is a complex, rare disorder that affects the foetus in utero. Here the abdominal wall is not fully formed, leaving the pubic bones separated and the bladder exposed in the lower abdominal wall.
 METHODOLOGY
 Single-stage reconstructive and repair surgery was undertaken in adults diagnosed with bladder exstrophy epispadias complex. The superficial external pudendal artery flap was considered for covering the defect after extensive reviews and discussion.
 CONCLUSION
 An uneventful surgery with recovery and a two-year follow-up post-surgery did not account for any complications following the procedure. This shows that the superficial external pudendal artery flap can be considered a favourable option for urologists in the repair of bladder exstrophy epispadias complex when undertaken in a single sitting surgery, especially in adults.
 MeSH Terms
 Bladder Exstrophy, epispadias repair, superficial external pudendal artery, axial-pattern flap.

Kidney function outcomes in patients after complete primary repair of bladder exstrophy and penopubic epispadias: Results from the international bladder exstrophy consortium

Historically, repair of bladder exstrophy (BE) is associated with compromise to the upper tracts; the single stage repair of BE was considered to exacerbate risks of kidney impairment. We aim to evaluate the risk of upper urinary tract deterioration or chronic kidney disease after the complete primary repair of exstrophy (CPRE). As part of the U.S.-India Multi-institutional Bladder Exstrophy Collaboration, we prospectively performed data collection on all patients managed at the Civil Hospital, Ahmedabad from 2010 to 2020. All patients who underwent primary or redo BE or primary penopubic epispadias (PE) repair using CPRE were included. Data on annual VCUG and DMSA, serum creatinine and cystatin-C, urinary albumin, and creatinine were aggregated. 72/104 patients who underwent CPRE at a median age of 1.7 years (IQR: 1.1-4.6) were included: 43(60%) patients with primary BE, 17(24%) with redo BE, and 12(17%) with primary PE. At a median follow-up of 4 years (IQR: 3-6), the overall median eGFR was 105 for BE, and 128ml/min for PE. 14(19%) patients had eGFR<90, and 22(31%) had microalbuminuria. 21(30%) patients had kidney scarring in DMSA and 31(44%) had VUR. Multivariate analysis showed that neither kidney scarring nor VUR could predict the presence of eGFR<90 or microalbuminuria. Of 72 patients, 2 (3%) patients had dry intervals >3h, 9 (13%) patients have dry intervals of 1-3h and 44 (61%) patients had dry intervals <1h during follow-up. We found that kidney function outcomes (i.e., eGFR and microalbuminuria) were not associated with continence status (p=0.3). In this series, we report a 5% incidence of CKD stage 2 or above that was not impacted by continence status. Furthermore, a 40% incidence of VUR and a 30% incidence of kidney scarring during follow-up was observed within this cohort, neither of which had a significant impact on renal function deterioration (i.e, decline in eGFR), but underscores the need for close kidney surveillance in children that have undergone bladder exstrophy repair. Modern CPRE technique for the repair of BE may increase the risk of kidney scarring in the intermediate-term follow-up, however, this finding does not correlate with low eGFR and presence of albuminuria inpatients. Therefore, close follow-up with serial kidney function measurements is warranted and necessary after CPRE.

Single-stage repair for female epispadias with urinary incontinence: A case report and literature review.

BackgroundEpispadias is a rare condition. Epispadias in females is two times less common than in males. Female epispadias range from 1 in 160,000 to 480,000 live births. Epispadias can be diagnosed through careful physical examination of the genital. Surgery is the management of epispadias. Surgical management of epispadias is quite tricky and requires expertise. The literature that discusses female epispadias is challenging to be found. In this paper, we would like to report surgical management of isolated female epispadias in Cipto Mangunkusumo Hospital, Jakarta.Case presentationA 7-year-old girl was admitted with a chief complaint of urinary incontinence since birth, during daytime and nighttime. Urinary incontinence was not induced by activities nor worsened by eating/drinking. Physical examination showed that the patient's external genitalia has underdeveloped labia minora, patulous urethra, bifid clitoris, and multiple hypopigmentation lesions. Laboratory results were in the normal range. The voiding cystourethrography (VCU) result revealed urine leaks during the filling phase. The bladder wall was normal, and no vesicoureteral reflux (VUR) appeared. The urethrocystoscopy shows a more vertical OUE, a wide-open bladder neck, and a urethral length of 1.5 cm. The patient underwent single-stage surgical procedures that consist of epispadias repair and bladder neck reconstruction through a subsymphyseal perineal approach. No complications occurred intra-operative and post-operative. At the 1-week and 6-months follow-up, the patient achieved urinary continence, and the surgical wound healed normally.DiscussionEpispadias is a rare condition that could occur in various degrees, from mild to severe degrees. To a severe degree, there is a split at the entire urethral and involves the bladder neck causing constant incontinence for the patient. Epispadias cases are quite challenging to diagnose. The physician needs to separate the labia majora and perform the physical examination carefully. The objective goals of surgical management of epispadias are to achieve urinary continence, restoration of anatomy, function, and cosmetic appearance of urethra and genitalia. Single-stage reconstruction is the current surgical method used to repair isolated female epispadias.ConclusionFemales epispadias is a rare congenital anomaly that is quite often to miss diagnosed. The single-stage technique is the recommended surgical technique for isolated female epispadias. This patient has achieved urinary continence, and no complications occurred during and post-surgery.

Epispadias: recent techniques

Aim: This paper presents the latest surgical approaches for epispadias treatment in the pediatric population, as well as those for adolescent and adult populations after initial failed repair in childhood. Methods: The retrospective study was conducted between March 2005 and May 2020 and included 18 patients with the mean age of 21 months (range 11-48 months) (Group A), who underwent primary epispadias repair and 15 patients with the mean age of 18 years (range 13-29 years) (Group B), who underwent redo surgery after failed epispadias repair in childhood. In Group A, the surgery was performed as a one-stage procedure using complete penile disassembly technique, while, in Group B, the surgery was done as a two-stage procedure and included complete straightening and lengthening of the penis, followed by urethral reconstruction. Penile straightening and lengthening were achieved by tunica albuginea incision and grafting. In Group A, the urethral plate was mobilized, transposed ventrally, and tubularized and augmented with vascularized preputial skin flap where needed. In Group B, the urethra was reconstructed either using the buccal mucosa graft and genital skin flaps or with tubularization of genital skin flaps. Successful treatment was defined as a functional and esthetically acceptable penis without complications. Results: The mean follow-up was 88 months (range 15-197 months). Satisfactory results were achieved in 26/33 patients. Urethral fistula occurred in 4/18 patients from Group A and in 3/15 patients in Group B and was surgically repaired after four months. Skin dehiscence occurred in eight patients, five from Group A and three from Group B. Recurrent penile curvature was observed in 2/18 patients from Group A and required surgical correction and in 2/15 patients from Group B and was mild and did not need surgical repair. Eleven patients from Group B who filled out the International Index for Erectile Function reported satisfying erectile function, sexual desire, intercourse, and overall satisfaction. Conclusion: Primary or redo epispadias repair is challenging even for experienced reconstructive urologists. Only radical surgical approach can lead to complete correction of all deformities and provide successful outcome.

Bladder exstrophy: Modern staged repair experience in our institution.

Introduction:Bladder exstrophy is a major malformation in paediatric urology. The treatment results are not still completely satisfactory, and their management is an enormous problem in Sub-Saharan Africa. While outlining our challenges, we report our management experience to improve our results.Subjects and Methods:We retrospectively reviewed the records of patients undergoing surgical repair of classic bladder exstrophy at our department between January 2010 and December 2019 (10 years). Epidemiological, clinical, therapeutic and evolution data were analysed.Results:Twenty-five children with classic bladder exstrophy were treated. Our series included 16 boys and 9 girls with a sex ratio of 1.7. Age ranged from 0 day to 6 years. Twenty-five bladder closures were performed, associated to pelvic osteotomy in 11 cases. Epispadias repair was performed on nine boys. Eight cases of bladder neck reconstruction and three cases of bladder enlargement were performed. We observed six bladder fistulas, four wound dehiscence, of which three partial, two parietal suppurations and six cases of urinary tract infection. Eight children had a continence of 1–2 h.Conclusion:The treatment of bladder exstrophy in our context is still limited because of financial difficulties encountered by the population and the insufficient technical platform in our country.

Outcomes of Cystectomy with MAINZ Pouch II and Epispadias Repair in Exstrophy Epispadias Complex in Adults: A Single-centre Experience from Pakistan.

To evaluate surgical outcomes and renal functions after cystectomy + MAINZ Pouch II and epispadias repair as a staged procedure in adult patients with exstrophy epispadias complex (EEC). Descriptive study. Department of Urology, Sindh Institute of Urology and Transplantation (SIUT), Karachi, from January 2004 to December 2020. A total of 33 patients with EEC were treated. Out of which, 20 underwent cystectomy + MAINZ Pouch II with epispadias repair as a staged procedure. Out of these, 17 had a follow-up period of more than a year and were included in the study. The patients were followed up after 6 weeks of surgery, at 6 months, one year, and at the end of follow-up. The assessed variables included the patients' renal function tests, malignancy potential, morning erections, ejaculations, night emissions, day-and-night-time urinary frequency, and overall happiness of patients at the end of follow-up. The mean age was 25.1 ± 7.5 years and mean follow-up duration was 7.8 ± 5.2 years. Postoperatively, there was a rise in blood urea from 27.8 to 35.08 mg/dl with a concurrent fall in serum bicarbonate from a mean of 23.5 to 20.2 mEq/dl. All patients were continent during the day-time postoperatively; whereas, two patients experienced nocturnal enuresis. All male patients exhibited good erections and ejaculations, but there was persistence of dorsal chordae in 4 (23.3%) patients. All were delighted, happy and pleased with the surgical outcomes and had returned to normal life. No rectal or sigmoid malignancy was observed. In adult EEC patients, cystectomy + MAINZ Pouch II and epispadias repair is safe and effective. Key Words: Adults, Exstrophy epispadias complex, MAINZ pouch II, Pakistan.

V03-09 ANATOMICAL REPAIR OF EPISPADIAS

INTRODUCTION AND OBJECTIVE:Most boys with penopubic epispadias have varying degree of incontinence. We describe the technique of anatomical repair of epispadias through this short video presentatio...

The impact of classic bladder exstrophy-epispadias repair in adult patients without continence

Background: Bladder exstrophy-epispadias complex is a rare congenital anomaly. Very few patients grow with the condition intact in their adulthood which lead to compromise in the results of repair. We herein report our experience of correction of the defects in the adult patients in single stage without doing osteotomy andurinary diversion. Methods: The sample size was 13 patients over ten years. All were treated in single stage. Bladder neck repair was done using paraurethral tissue, primary abdominal closure was done without flap, epispadias repair was done using modified Cantwell-Ransley procedure and hernial repair was done without mesh. No osteotomy was done. All the patients were followed regularly. Minimum period of follow-up was one year. Urinary continence, change in habits and subjective assessment of well-being were also noted.Results: 86.4% patients were totally incontinent. Bladder and anterior abdominal wall were closed in all the patients. Complete epispadias was possible in15.3% patients and rest were converted into coronal hypospadias. Hernia was repaired in 23% patients. All the patients were satisfied with their cosmetic results and they were able to interact socially. Conclusions: Surgical correction of the exstrophy-epispadias complex is possible in adulthood in the hands of experts in single stage without osteotomy. Regular cystoscopic examination and urine cytology yearly are recommended to diagnose the malignant changes in the native bladder as early as possible. Psychological, social and self-level of satisfaction is high among these patients after the surgical correction.

Care of child having bladder exstrophy and epispadias

The bladder exstrophy-epispadias-cloacal exstrophy complex is caused by a developmental abnormality that occurs 4-5 weeks after conception in which the cloacal membrane is not replaced by tissue that will form the abdominal muscles. The treatment includes Bladder closure and abdominal wall repair operation, Kelly procedure, Bladder Augmentation, and Epispadias repair.

Modified partial penile disassembly repair for improved functional and cosmetic outcome in isolated male epispadias

Introduction and objectiveThe objective of the study was to evaluate the functional and cosmetic outcome of single-stage modified partial penile disassembly repair in isolated male epispadias. Materials and methodsA retrospective analysis of 15 cases of primary epispadias repair, from June 2015 to December 2018, was performed. Patients were classified by the type of epispadias, urinary incontinence, chordee, and rotation. Surgical techniquePenile degloving with the mobilization of the urethral plate from the ventral to the dorsal aspect with the preservation of blood supply at both ends, distally up to the level of mid-glans and proximally up to the pubic symphysis is done. Tubularization of urethral plate followed by spongioplasty, corporoplasty with medial rotation of corporeal bodies, and glanuloplasty with meatoplasty was done to bring the meatus ventrally. The skin cover is done by the rotation of the ventral flaps and the z-plasty whenever required. ResultsAge of the patients varied from 4 months to 21 years with a mean of 11 years. Thirteen patients had excellent cosmetic outcome while two patients had minimal residual chordee but did not require any surgery in a follow-up. Five patients with partial incontinence in the study group achieved continence after surgery. None of the patients developed complications such as fistula or stricture. All five male patients in the post-pubertal group reported normal erections and successful ejaculations after the surgery. Follow-up ranged from 3 months to 18 months. ConclusionsModified partial penile disassembly incorporates all the benefits of Cantwell Ransley repair and needs less extensive dissection than total penile disassembly. Both functional and cosmetic results are good with a low complication rate.