105.18 Perioperative Management of Primary Classic Bladder Exstrophy: Managing the Leaks and Peaks

A. Haffar1, A. Hirsch1, C. Morrill1, T. G. Harris1, C. Crigger1, A. Garcia1, V. Maxon1, H. N. Di Carlo1, C. Monitto2, J. P. Gearhart1, J. B. Hunsberger2  1The Johns Hopkins University School Of Medicine, Division Of Pediatric Urology, Baltimore, MD, USA 2The Johns Hopkins University School Of Medicine, Division Of Pediatric Anesthesia, Baltimore, MD, USA

Introduction:  Appropriate postoperative management is crucial in patients undergoing classic bladder exstrophy closure (CBE). Therefore, the authors sought to review the postoperative pathway in patients with CBE following primary closure.  

Methods:  A prospectively maintained institutional approved exstrophy-epispadias complex database of 1504 patients was reviewed for patients with CBE who had undergone primary closure between 2016-2022 whose closure was performed within one year of age. Migration of electronic health records prior to 2016 limited data collection during that time.    

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. Suprapubic tube is placed in all patients for a median duration of 46.5 days. All patients underwent PICU placement 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 frequently placed in the thoracic region (60%). Catheters are typically threaded caudally and tunneled to the patient’s flank and maintained for a median duration of 19 days. 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%.  

Conclusion: The outcome of primary bladder closure can have inauspicious consequences that can affect attaining their future continence. Successful primary closure of bladder exstrophy requires a detailed plan for immobilization, analgesia, and nutrition in addition to an experienced multi-disciplinary team. We have identified several guiding principles for perioperative success in exstrophy patients at our center including Buck’s traction with external fixation, adequate postoperative analgesia and sedation when needed, 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.