Cloacal Exstrophy Repair
William Remley1; Howard Jen, MD2; Carl-Christian A. Jackson, MD2; Jeremy Wiygul, MD2
1Lake Erie College of Osteopathic Medicine
2Tufts Medical Center
Cloacal exstrophy is congenital malformation marked by an abdominal wall defect with open and exposed hindgut and bladder. It is the most severe birth defect within the exstrophy-epispadias complex, and when spinal defects are also present, it is called the OEIS (omphalocele, exstrophy, imperforate anus, and spinal defect) complex. Cloacal exstrophy is rare, occurring in 1/200,000–400,000 births, but it can be diagnosed on prenatal ultrasound. The defect results in two exstrophied hemibladders separated by an exposed cecal plate, with the distal hindgut being foreshortened and blind-ending, resulting in an imperforate anus. There is diastasis of the pubic symphysis, and the genitalia are separated. In males, the phallus is usually split in half, flattened and shortened, with the inner surface of the urethra exposed. In females, the clitoris is split, the labia are widely separated, and there may be two vaginal openings. Cloacal exstrophy is also highly associated with other birth defects, especially spina bifida, which coexist in up to 75% of cases. Multidisciplinary care followed by surgical management should begin immediately following the baby’s delivery.
Surgical goals in the neonatal period include closure of the meningocele and repair of the exstrophy and omphalocele, resulting in approximation of the bladder halves and repair of the hindgut defect with colostomy creation. Closure of the bladder, with positioning within the pelvis, can either occur at the initial operation or be staged to occur after a period of monitored growth, and is best performed with pelvic osteotomies to protect the closure from tension. Subsequent surgeries over several years will address genital reconstruction and colonic pull-through for fecal continence, if the patient is a candidate. Here, we present a patient diagnosed with OEIS complex by prenatal ultrasound, with a postnatal exam confirming the diagnosis and demonstrating a closed (covered) myelomeningocele. The cloacal exstrophy and omphalocele were repaired in one stage, with primary closure of the involved bowel and the bladder, facilitated by pelvic osteotomies.
