Management of Cloacal Exstrophy

Abstract

The exstrophy complex represents a spectrum of congenital abnormalities that includes the following conditions: epispadias, classic bladder exstrophy, and cloacal exstrophy. These three deformations comprise a continuum with epispadias representing the least severe, classic bladder exstrophy the most common, and cloacal exstrophy the most severe. Cloacal exstrophy is extremely rare, occurring in approximately 1 in every 200,000 to 400,000 live births (Ziegler et al, 1986). Although primarily a ventral abdominal wall defect, cloacal exstrophy is associated with varying degrees of abnormalities in the genitourinary tract, bowel, spine, and lower extremities. Until recently, most children with cloacal exstrophy did not survive infancy because of the complex multiple system abnormalities resulting in fluid and nutritional defects. In 1960, Rickham reported the first survivor with cloacal exstrophy (Rickham, 1960). Primarily due to advances in perinatal care and total parenteral nutrition, survival is now feasible in greater than 90% of patients born with cloacal exstrophy. Thus, the focus is now directed at quality of life issues in these complex patients.