Pitfalls in the management of newborn cloacas

Clinicians caring for newborns with persistent cloaca face significant challenges in the newborn period. Avoiding key pitfalls during this time can have dramatic implications. We reviewed the medical records of 361 patients with cloaca operated on at our institution and analyzed sequelae that resulted from incorrect management in the newborn period. Of 361 patients, 282 underwent primary operations at our institution, and 79 patients were referred to us after a failed repair at other institutions. Pitfalls in management during the newborn period included the following: (1) Failure to recognize and manage hydrocolpos, which occurred in 46 patients. Of these, three patients developed pyocolpos (two progressed to vaginal perforation), and 43 suffered from persistent bilateral hydronephrosis, megaureters, recurrent urinary tract infections, persistent acidosis, or failure to thrive due to undrained hydrocolpos. They underwent unnecessary urinary drainage procedures (nephrostomy, ureterostomy, cystostomy, or vesicostomy) in the newborn period. When the vagina was finally decompressed, all of these symptoms disappeared. (2) Colostomy or vesicostomy problems, which occurred in 50 patients. These included incorrect placement of the colostomy (too distal, which interfered with the pull-through) in 24 and colostomy prolapse in 23. Incompletely diverting loop colostomies led to urinary tract infections in 49 patients. Vesicostomy prolapse occurred in three patients. (3) Clinical misdiagnosis, which occurred in 42 patients. Six were incorrectly diagnosed as "intersex" and 36 as "rectovaginal fistula." In this group only the rectum was repaired, and the patients were left with a urogenital sinus that required reoperation. Proper management of a newborn with cloaca includes drainage of a hydrocolpos, which avoids unnecessary urinary diversions and pyocolpos. Our preferred colostomy is one with separated stomas, adequate distal bowel for the pull-through, and use of a proper technique to avoid prolapse. Correct clinical diagnosis of cloaca avoids problems during the definitive repair.