Urological aspects of cloacal malformations

Cloacal malformations occur in a spectrum of severity. In general the urethra (sometimes short or absent), vagina (often 2) and rectum converge in the urogenital sinus. Immediate colostomy is necessary. However, after colostomy the urinary tract assumes prime importance because of life-threatening obstruction and/or reflux. I treated 72 patients with complex cloacal malformations, 33 as primary and 39 as secondary cases. In 40 patients there was severe reflux with severe upper tract damage in more than half of the cases: 19 had prior urinary diversions (4 ileal loop, 5 loop ureterostomy and 10 vesicostomy). In nearly all secondary cases a definitive rectal operation had been done but the more life-threatening urinary tract had not been repaired. Some patients had other urological and nonurological malformations. Review of these cases emphasizes several points. Each case should be evaluated early endoscopically and radiographically to guide immediate and later treatment. Rectal reconstruction (now done via the posterior sagittal approach) should not be done as the initial procedure, which often was practiced in the past. In some patients the urinary tract should be repaired first when there is obstruction or massive reflux to prevent upper tract deterioration. In most cases it is feasible to repair all aspects of the anomaly in a single operation, that is the urinary tract, genital tract and rectum. With a well planned and coordinated approach to all 3 organ systems a satisfactory outcome is possible in most cases.