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.
Visnesky
P M
PM
Department of Surgery, Southern Illinois University School of Medicine, Springfield.
Texter
J H
JH
Galle
P C
PC
Walker
G G
GG
McRae
M A
MA
D000015:Abnormalities, Multiple / Q000473:pathology / Q000601:surgery; D000293:Adolescent; D001746:Bladder Exstrophy / Q000473:pathology* / Q000601:surgery; D002988:Cloaca / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D009095:Mullerian Ducts / Q000473:pathology / Q000601:surgery*; D014599:Uterus / Q000473:pathology / Q000601:surgery*