Modern treatment of cloacal exstrophy

We have treated 12 newborns with cloacal exstrophy (10 classical and 2 variants) since 1980. Eleven infants underwent repair and they are all surviving; the one nonoperated infant died of pulmonary hypoplasia. There are six genetic females and six genetic males, five of whom are being raised as females. The initial operation consisted of separating the bowel from the bladder to create an intestinal stoma; closing the omphalocele; and reapproximating (5), closing (4), or leaving the exstrophied bladder undisturbed (2). The importance of creating a “tailgutostomy” instead of an ileostomy to prevent problems with diarrhea, dehydration, and acidosis is emphasized. There have been 71 subsequent operations in these 11 patients (28 general surgical, 25 urological, 9 neurosurgical, and 9 orthopedic). Quality of life, rather than survival, is now the major issue facing patients with cloacal exstrophy. All of these patients except one are neurologically normal. The defect is closed in all patients. Three patients wear no appliances, 7 wear one (gastrointestinal [GI]), and one wears two (GI and genitourinary [GU]). We developed a scoring system to analyze bowel and bladder continence: voluntary control = 3; control with an enema program or intermittent catheterization = 2; incontinence with a well-functioning stoma = 1; and incontinence without a stoma = 0. The best continence score is 6 (GU + GI). We currently have 7 patients with a continence score of 1 (colostomy + incontinent bladder); 1 with a score of 2 (ileostomy + incontinent ureteroenterostomy); 2 with a score of 4 (enema program + continent urinary diversion); and 1 with a score of 5 (enema program + continent bladder). It is anticipated that most patients will obtain a score of at least 3 (GI stoma + continent urinary diversion). Additional procedures are planned in the future to enhance the quality of life of these patients as they mature.