Cloacal Exstrophy Repair

Abstract

Cloacal exstrophy is a birth defect in which part of the abdomen is open, and some of the abdominal contents (such as the bladder and intestines) are exposed. It is the most severe birth defect within the exstrophy-epispadias complex and can occur as part of the OEIS complex, which is characterized by omphalocele, exstrophy, imperforate anus, and spinal defects. It is a rare congenital malformation, occurring in 1/200,000-400,000 births, and is diagnosed by prenatal ultrasound. In cloacal exstrophy, there are two exstrophied hemibladders separated by a foreshortened cecum or hindgut, often characterized by a blind end, resulting in an imperforate anus. There is significant diastasis of the pubic symphysis, and the phallus is separated into two halves along with the scrotum. In males, the penis is usually flat and short, with the exposed inner surface of the urethra on top, and split into a right and left half. In females, the clitoris is split and there may be two vaginal openings. This condition is also highly associated with other birth defects, especially spina bifida, which coexists in up to 75% of cases. Multidisciplinary care followed by surgical management should begin immediately following the birth of the patient. Closure of the meningocele and omphalocele, as well as adaptation of the bladder halves, should be started in the neonatal period, followed by a multi-stage approach for bladder, bowel, and genital reconstruction (including a pelvic osteotomy) at a later time. Here, we present a patient who was diagnosed with cloacal exstrophy by prenatal ultrasound. The urinary bladder and intestines were noted to be outside of the body and associated with a closed myelomeningocele. The exstrophied cloaca was repaired and reduced back into the abdominal cavity. Urinary and fecal diversions were created, and a leg casting was placed for pelvic osteotomy.