A case of a closed partial cloacal septation defect with a patent urachus.
A boy with a closed partial cloacal septation defect with a patent urachus is reported. He had an intact abdominal wall, a patent urachus, a colovesical fistula, intact genitalia and urethra, imperforate anus, and a lipomyelocystocoele. Patients with similar constellation of findings have been reported as cloacal exstrophy variants. What distinguishes this case from the other reported variants is the intact abdominal wall with the patent urachus, the small and normally formed phallus and urethra, and the presence of a lipomyelocystocoele. We discuss the possible embryologic mechanism responsible for this boy's findings and possible relationship with the cloacal exstrophy spectrum. We also discuss new terminology for the epispadias-exstrophy spectrum. Furthermore this case reminds us that there is considerable variability within the epispadias-exstrophy spectrum.
Stoler
J M
JM
Embryology-Teratology Unit, Massachusetts General Hospital, Boston 02114.
Doody
D P
DP
Holmes
L B
LB
D000015:Abnormalities, Multiple / Q000235:genetics*; D001006:Anus, Imperforate / Q000235:genetics; D002988:Cloaca / Q000002:abnormalities*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007412:Intestinal Fistula / Q000235:genetics; D008297:Male; D008591:Meningomyelocele / Q000235:genetics; D013577:Syndrome; D014497:Urachus / Q000002:abnormalities*; D001747:Urinary Bladder Fistula / Q000235:genetics