Genital outflow tract obstruction in an adolescent with cloacal exstrophy

The combination of congenital anomalies in patients with cloacal exstrophy presents a difficult management problem. Experience with these problems is limited because of the infrequent occurrence of the disorder and because few patients survive to adulthood. The present paper describes a 14-year-old female who presented with hyperchloremic metabolic acidosis, secondary to retained urine in a redundant ileal conduit, and symptoms of müllerian outflow tract obstruction. The literature is reviewed on genetic females with cloacal exstrophy, revealing a high incidence of müllerian fusion defects. Data are presented showing that women with cloacal exstrophy have a high potential incidence of genital outflow tract obstruction. These patients must be followed closely during adolescence so that diagnosis and treatment may be initiated early.