ABSTRACT
Cloacal exstrophy is an extremely rare congenital disorder first described by Littre in 1709. The exact embryology and etiology of cloacal exstrophy are still poorly understood, but several theories have been postulated. With the advent of prenatal imaging, more patients are now being diagnosed prenatally either by ultrasound or with the aid of fetal magnetic resonance imaging (MRI). Several criteria have been proposed for diagnosis. Major criteria include nonvisualization of the bladder, a large midline infraumbilical anterior wall defect or cystic anterior wall structure, omphalocele, and/or lumbosacral anomalies. The most common gastrointestinal (GI) anomalies include omphalocele, exstrophy of the cecal plate, blind-ending hindgut, and imperforate anus. Females with cloaca exstrophy should be raised as females. Gender assignment in male patients with cloacal exstrophy remains a complex and controversial issue. Initial surgical management should be undertaken in the newborn period. Close postoperative monitoring in the neonatal intensive care unit is necessary to ensure adequate pain control, immobilization, and nutritional support.
