Cloacal exstrophy: experience with 20 cases

Abstract

Exstrophy of the cloaca, a rare anomaly, is among the most complex malformations of infancy. Features include omphalocele, imperforate anus, and exstrophy of two hemibladders, between which lies the everted cecum. A small colon ends blindly in the pelvis, and the terminal ileum often prolapses out of the exposed cecum. Formerly, most of these infants died. From 1974 to 1992, 20 of these patients were treated, the majority in the past decade. Six were managed primarily; 14 were referred after prior surgery elsewhere. Current treatment of a newborn with cloacal exstrophy includes closure of the omphalocele, separation of the gastro-intestinal tract from the hemibladders, and closure of the two hemibladders as a single viscus. The colon can be pulled through in some cases, either immediately or later (6 cases). The bladder must be augmented in all cases to provide adequate volume and compliance; stomach is ideal for this (10 cases). A bladder continence mechanism must be constructed by narrowing the outlet (9 cases), or inserting a reversed small bowel nipple (7 cases). Intermittent catheterization is needed for the patient to empty the reconstructed bladder. Although two thirds of these patients are genetic males, they should be raised as females because they lack adequate tissue to construct a phallus. Magnetic resonance imaging of the spine has shown tethering of the cord in all patients; most were released neurosurgically. Lessons provided by these cases demonstrate that a satisfactory surgical and social outcome can be achieved today in most children with cloacal exstrophy.