Primary Repair of Cloacal Exstrophy with a Small Bladder with a Bowel Patch: A Case Report

Abstract

Cloacal exstrophy (CE) is a group of congenital anomalies most commonly affecting the genitourinary, gastrointestinal and skeletal systems. Management of these anomalies requires multidisciplinary care and involves staged surgical reconstruction. We present the case of a newborn male referred due to a midline abdominal mass and imperforate anus noted upon delivery. Physical exam revealed an omphalocele, bladder exstrophy, bifid penis, and imperforate anus. Imaging revealed right renal agenesis and pubic diastasis. The patient underwent repair of cloacal exstrophy, bilateral ureteral stent, bilateral anterior osteotomy, primary repair of bladder with bowel augmentation to increase capacity, perineal urethrostomy, genital reconstruction and end colostomy.  He was maintained in a pelvic wrap for the osteotomy to heal. He was discharged well.  The surgical management of exstrophy is varied involving multiple approaches.  While most authors would do a staged repair of the exstrophy leaving the bladder augmentation later because of high failure rates.  Our approach to primarily address a relatively small bladder with a patch during closure in selected patients is feasible.  Although long term follow-up is needed to achieve good quality of fecal and urinary incontinence, early surgical bladder correction may suggest earlier achievement of continence among patients.  Patient is doing well after three years of this procedure with minimal soiling per urethrostomy.  Our future plans include repair of the imperforate anus and urodynamic studies to monitor bladder function.