Cloacal Exstrophy Closure Without Osteotomy and Immobilization: A Recipe for Failure.

BACKGROUND: Cloacal exstrophy (CE) remains one of the most severe birth defects compatible with life with a constellation of anomalies involving the bladder, genitalia, hindgut, and spinal cord. Pelvic osteotomy and immobilization have been utilized to facilitate bladder closure, yet their role as adjuncts remains a topic of debate. The authors sought to evaluate the outcomes of CE closure without the use of osteotomy or lower extremity (LE)/pelvic immobilization. METHODS: An institutional database of 173 CE patients was reviewed for patients closed without osteotomy and/or limb immobilization. Patient records were reviewed for continence procedures, reclosure operations, and continence outcomes. RESULTS: A total of 59 closure surgeries that met inclusion criteria were identified in 56 unique patients. Thirty-seven closure procedures developed eventual failure (63%) with secondary closure events also resulting in failure. Most closures did not use an osteotomy, 93.2%. LE immobilization-only was used in most closures (43/59), of which only 37% were successful. Failures were attributed to dehiscence (14/37), bladder prolapse (19/37), or both dehiscence and prolapse (4/37). The median age at closure was 3 days old (1-18.5 IQR) with the majority of closure events (47) closure events taking place in the newborn period. Median diastasis prior to primary closure was 6 cm (4.8-8 cm IQR). The median number of closure attempts needed to close the bladder was 2 (1-2 IQR). Of the 56 patients, 31 have >3 h of daytime continence, with the entirety of these patients catheterizing a stoma or below. CONCLUSION: These results highlight the critical role of osteotomy and lower limb immobilization in successful closure of the bladder and abdominal wall in CE. TYPE OF STUDY: Treatment Study. LEVEL OF EVIDENCE: Level III.

A Rocky Road: Bladder Stones in the Augmented Exstrophy-Epispadias Complex Patient.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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 augments, however, this was not statistically significant (P = .07). The most common primary stone component was dahllite, followed by struvite for all augments (Table 1). The median time to stone treatment after augmentation was 4.14 years (0.75-31). Seventy-four percentage of patients had a recurrence that required a second surgery. The median time from first to second surgery and second to third surgery was 1.4 years and 2.22 years, respectively. Bladder stone surgery complications occurred in 14% of patients, vesicocutaneous fistula being the most common, and complications did not differ by augment type. Median follow-up after first stone intervention was 6.07 years (0-19.5). CONCLUSION: The treatment of bladder stones in the exstrophy-epispadias complex remains challenging. Interventions to prevent recurrence are crucial as the majority of patients will require 2 or more stone surgeries in their lifetime.

Perioperative management of primary classic bladder exstrophy: A single institutional pathway to success.

PURPOSE: Appropriate perioperative management is crucial in patients undergoing classic bladder exstrophy closure (CBE). Therefore, the authors sought to review their intra and postoperative management of patients with CBE undergoing primary closure and examine the impact of this pathway on patient outcomes. METHOD: A prospectively maintained institutional approved exstrophy-epispadias complex database was reviewed for patients with CBE who had undergone primary closure between 2016 and 2022 and whose closure was performed within one year of age. Electronic medical records for eligible patients were retrospectively reviewed to examine patient demographics, use of pelvic osteotomy, immobilization status, pediatric intensive care unit (PICU) admission and management, perioperative analgesia and sedation, nutritional support, drainage tubes, blood transfusions, antibiotic coverage, hospital length of stay, postoperative complications, and closure failure. RESULTS: A total of 25 patients were identified, 22 with CBE and 3 with variant CBE. Closure was performed at a median age of 84 days with patients ranging in age from 9 to 351 days. All patients underwent osteotomy and immobilization with modified Buck's traction and external fixation for a median duration of 41 days. A suprapubic tube was placed in all patients for a median duration of 46.5 days. All patients underwent PICU admission following closure for a median duration of 8 days. Ventilator support was required in 68 % of patients for a median of 3 days. Epidural analgesia was used in all patients and catheters were maintained for a median duration of 19 days. All patients received a blood transfusion over the course of their admission. Patient-controlled analgesia was used in most patients as an adjunct for a median duration of 38.5 days. Other commonly used analgesic adjuncts included acetaminophen, diazepam, clonidine, and dexmedetomidine. TPN was used in 80 % of patients for a median of 7 days with a return of oral feeding thereafter. Overall, the closure success rate in this cohort of patients was 100 %. DISCUSSION: The outcome of primary bladder closure can have inauspicious consequences that can affect a child's continence for years. The incidence of failed bladder closure can be minimized with the implementation of a detailed plan for immobilization, analgesia, and nutrition guided by an experienced multi-disciplinary team. CONCLUSION: We have identified several guiding principles for perioperative success in exstrophy patients at our center including Buck's traction with external fixation, provision of adequate postoperative analgesia and sedation, aggressive nutritional support, renal and bladder drainage, and robust antibacterial support. Our high success rate in managing this complex pathology demonstrates its validity and use as a pathway to success.

Robotic Female Radical Cystectomy.

Radical cystectomy is the standard of care for patients with nonmetastatic high-risk bladder cancer. Robotic approach to radical cystectomy has been developed to reduce perioperative morbidities and enhance postoperative recovery while maintaining oncologic control. Classically, radical cystectomy in female patient entails anterior pelvic exenteration with removal of the bladder, uterus, fallopian tubes, ovaries, anterior vaginal wall, and urethra. Pelvic organ-sparing radical cystectomy has been adopted in carefully selected patients to optimize postoperative sexual and urinary function, especially in those undergoing orthotopic urinary diversion. In this article, we describe our techniques of both classical and organ-sparing robot-assisted laparoscopic radical cystectomy in female patients. We also review patient selection criteria, perioperative management, and alternative approaches to improve operative outcomes in female patients.

Approaches to Eliminate Radiation Exposure in the Management of Pediatric Urolithiasis.

PURPOSE OF REVIEW: Traditional management of pediatric urolithiasis has been associated with a significant amount of radiation exposure. The steady increase in pediatric urolithiasis in recent years has raised concerns of long-term consequences in this special population. This review seeks to highlight the newer insights towards eliminating radiation exposure in pediatric urolithiasis from contemporary literature. RECENT FINDINGS: Establishing a clinical care pathway restricting usage of computed tomography in emergency rooms in suspected pediatric urolithiasis can eliminate unnecessary radiation exposure. Ultrasound can successfully replace fluoroscopy at ureteroscopy and percutaneous nephrolithotomy. Accredited technicians who use optimized settings combined with dose monitoring can significantly reduce fluoroscopic radiation. Radiation exposure in pediatric urolithiasis can be significantly reduced and potentially eliminated by employing standard protocols during workup, intervention, and follow-ups. Larger studies can support the feasibility of routinely performing ultrasound-guided surgeries instead of fluoroscopy. There is a need for development of consensus towards standardization of the management of pediatric urolithiasis.