The complete primary repair of bladder exstrophy refinements through collaboration within the Multi-Institutional Bladder Exstrophy Consortium (MIBEC)

Abstract

Objective: To provide a step-by-step presentation of the complete primary repair of bladder exstrophy (CPRE). Design: Video presentation. Setting: Children's Hospital of Philadelphia, within the Multi-Institutional Bladder Exstrophy Collaboration Patient(s): A 3-month-old boy with the bladder exstrophy (BE) undergoing a CPRE after the family provided informed consent. Results: A ten-hour procedure, including a bilateral pelvic osteotomy and epidural catheter placement was completed. There was no intraoperative complication. The child was immobilized with a spica cast for 6 weeks. His bladder was cycled via the suprapubic tube until he could tolerate clamping for 24 h with no evidence of upper tract dilation. Conclusions: Through collaboration, we have significantly refined the CPRE technique for BE repair. We believe that for rare congenital anomalies such as bladder exstrophy, collaboration across institutions increases volume exposure, which in turn, correlates with improved outcomes. We continue to carefully follow treated patients for the long-term to observe these outcomes, and measure the burden of BE on families in terms of stigma, quality of life and psychosocial impacts.

Keywords

• Bladder exstrophy
• Reconstructive surgical procedures
• Pediatrics
• Complete primary repair of exstrophy (CPRE)
• Consortium