Optimal management of cloacal exstrophy

Classic management of cloacal exstrophy has emphasized primary closure of the omphalocele, end ileostomy, and delayed genitourinary reconstruction. The resultant mortality from "short-bowel syndrome" and urinary sepsis, and morbidity from inappropriate gender assignment has prompted us to reexamine the operative approach to this problem. Our experience involves 15 cases from 2 days to 21 years after repair. All had initial closure of their omphalocele. Nine of the 15 had closure of the vesicointestinal fistula with preservation of the distal colon segment and creation of an end colostomy; 2 had an initial ileostomy with later conversion to an end colostomy; and 2 have permanent ileostomies. Nine of the 15 were genetic males; six were converted to female, five during the newborn period and one at 9 months. The hemibladders were approximated in 13 of 15 and two had a primary turn-in of the exstrophied bladder. Three had bladder closure with iliac osteotomies delayed beyond the newborn period. Urinary diversion has been utilized in 11 patients, 7 ileal conduits, 1 vesicostomy, 2 cutaneous ureterostomies, and 2 temporary pyelostomies; only one patient remains with an exstrophied bladder. Three patients with closure of the bladder remain incontinent. Of our patients, 13 of 15 are alive and well. One died at age 2 days because of renal agenesis and one was 19 years old when found dead of an unknown cause. There have been no deaths due to short-bowel syndrome or sepsis, and no patients have had upper urinary-tract deterioration. These results prompt us to recommend the following therapy for cloacal exstrophy: (1) early closure of the omphalocele; (2) closure of the vesicointestinal fistula with creation of an end colostomy at the distal end of the blind pouch; (3) reapproximation of the hemibladders; and (4) gender assignment to female. Subsequent procedures in the stable infant would include bladder closure and later reconstruction for continence if the bladder is suitable or appropriate permanent diversion.