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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., Competing Interests: Conflict of interest None of the authors have conflicts of interest to disclose., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: 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., Objective: We aim to evaluate the risk of upper urinary tract deterioration or chronic kidney disease after the complete primary repair of exstrophy (CPRE)., Study Design: 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., Results: 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 128 ml/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 >3 h, 9 (13%) patients have dry intervals of 1-3 h and 44 (61%) patients had dry intervals <1 h during follow-up. We found that kidney function outcomes (i.e., eGFR and microalbuminuria) were not associated with continence status (p = 0.3)., Discussion: 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., Conclusions: 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., Competing Interests: Conflict of interest The authors have no financial or personal relationships with other people or organizations that could inappropriately influence their work., (Copyright © 2022. Published by Elsevier Ltd.)

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)., Design, Setting, and Participants: In this prospective, observational study, long-term collaboration was created and based at a public hospital in Ahmedabad, India, between January 2009 and January 2015. The entire postoperative cohort was recalled in January 2016 for comprehensive examination, measurement of continence outcomes, and assessment of surgical complications. Seventy-six percent of patients (n = 57) who underwent complete primary repair of exstrophy during the study interval returned for annual follow-up in 2016 and formed the study cohort: 23 patients with primary BE, 19 patients with redo BE, and 11 patients with PE repair., Main Outcomes and Measures: Demographics, operative techniques, and perioperative complications were recorded. A postoperative protocol outlining procedures to ensure monitoring of study participants was followed including removal of ureteral stents, urethral catheter, external fixators, imaging, and patient discharge., Results: Of the 57 patients, 4 were excluded because they underwent ureterosigmoidostomy. Median age at time of surgery was 3 years (primary BE), 7 years (redo BE), and 10 years (PE), with median follow-up of 3 years, 5 years and 3 years, respectively; boys made up more than 70% of each cohort (n = 17 for primary BE, n = 15 for redo BE, and n = 9 for PE). All BE and 3 PE repairs (27%) were completed with concurrent anterior pubic osteotomies. Seventeen of 53 patients (32%) experienced complications. Only 1 patient with BE (4%) had a bladder dehiscence and was repaired the following year., Conclusions and Relevance: A unique surgical mission model consisting of an international collaborative focused on treating the complex diagnoses of BE and PE offers outcomes comparable with those in high-income countries, demonstrating a significant patient retention rate and an opportunity to rigorously study outcomes over an accelerated interval owing to the high burden of disease in India. Postoperative care following a systematized algorithm and rigorous follow-up is mandatory to ensure safety and optimal outcomes.

Purpose: We review our results with isolated male epispadias repair, comparing the Cantwell-Ransley and Mitchell-Bagli procedures in regard to continence status and postoperative complications., Materials and Methods: We reviewed the charts of 33 patients presenting with isolated epispadias (glanular in 3, penile in 9, penopubic in 21) between 1994 and 2005. Of the 21 patients with penopubic epispadias Cantwell-Ransley repair was performed in 14 and Mitchell-Bagli repair was done in 7., Results: Mean patient age at epispadias repair was 16.8 months (range 12 to 24) and 19.3 months (9 to 42) for the Cantwell-Ransley and Mitchell-Bagli operations, respectively. After epispadias repair in the Mitchell-Bagli group 4 of 6 patients (67%) became continent vs 0 of 13 in the Cantwell-Ransley group (p <0.01). Bladder neck repair was performed in 11 of 13 children (85%) who underwent a Cantwell-Ransley repair. Of these 11 boys 6 became continent. Hence, 3 of the 5 patients who failed the first bladder neck repair underwent a second bladder neck repair with augmentation cystoplasty to achieve dryness (dry more than 4 hours). The other 2 patients are on clean intermittent catheterization and have 2 to 4-hour dry intervals. After undergoing a Mitchell-Bagli epispadias repair 2 patients (33%) remained incontinent and went on to receive endoscopic injection of bulking agent to become dry. Mean followup was 70 months (range 10 to 120) for Mitchell-Bagli repair and 80 months (21 to 144) for Cantwell-Ransley repair. At last followup 13 of 17 children (76%) with penopubic epispadias were completely dry or had dry intervals greater than 4 hours. Among the 21 patients 10 (48%) had complications, of whom 8 were in the Cantwell-Ransley group (57%) and 2 were in the Mitchell-Bagli group (29%)., Conclusions: Similar urinary continence rates can be achieved for male penopubic epispadias with both surgical techniques, at the expense of more bladder neck repairs following the Cantwell-Ransley procedure.

Purpose: Long-term data suggest that a significant number of women with the exstrophy-epispadias complex (EEC) report dissatisfaction with the cosmetic appearance of the genitalia. We developed a new technique of mons plasty in an attempt to improve the overall cosmetic appearance of the external genitalia in females with EEC., Materials and Methods: Nine consecutive females with a mean age of 6.4 years (range 2 days to 12.2 years) previously diagnosed with EEC underwent simplified mons plasty concomitantly with various other urological reconstructive procedures. Briefly, a simplified mons plasty was performed by approximating the bifid hemiclitoris and subsequently incising the lateral sulci between the labia majora and minora, which allowed the superomedial rotation of the labia majora and underlying peripubic adipose tissue, thus, recreating the mons., Results: There were no perioperative complications. With a mean followup of 6 months (range 4 to 12) all patients had improved cosmesis with respect to a more prominent mons, more appropriately situated labia and adequately recessed introitus., Conclusions: Simplified mons plasty improves cosmesis in females with external genital abnormalities secondary to EEC. Although our followup is admittedly short, we believe that this technique should be considered in the armamentarium of the reconstructive surgeon during the operative treatment of this complex group of patients.

Objective: To describe a technical modification that facilitates dorsal skin closure, improves cosmesis and eliminates chordee recurrence secondary to contracture of the dorsal penile skin in the repair of epispadias., Patients and Methods: Eleven patients with penopubic epispadias (mean age 1.8 years) had the epispadias repaired using a modified ventral penile skin flap. Four patients had isolated epispadias and seven had had a previous primary closure of bladder exstrophy. Nine patients underwent the Cantwell-Ransley technique, leaving the meatus in a glanular position. Two patients were repaired using the penile disassembly technique of Mitchell and Bägli, because they had a short urethral plate. A ventral island skin flap was fashioned, starting at the base of the penis. Dissection was carried ventrally into the scrotum to allow for adequate dorsal flap transposition. The flap was rotated laterally to shift the suture line from the midline and to cover the dorsal aspect of the penis with untouched penile shaft skin. Redundant ventral foreskin was discarded., Results: All patients had an uneventful course after surgery. Dorsal penile skin was viable in every case and no patient developed recurrence of chordee or a urethrocutaneous fistula. The cosmetic result was excellent in all patients., Conclusions: Dorsal skin closure using lateral rotation of ventral penile skin flap improves cosmesis after epispadias repair and eliminates the recurrence of chordee secondary to midline dorsal scarring.