Pseudoexstrophy associated with congenital pouch colon.
Pseudoexstrophy or covered exstrophy is a rare exstrophy variant. The authors report a case of covered exstrophy that presented as a newborn with widely separated pubic bones and rectus muscles, a low-set umbilicus, and a subcutaneous bladder. The anal opening was absent, and there was a complex malformation of the external genitalia consisting of a small, laterally displaced penis and a right-sided ectopic hemiscrotum. Micturition and urinary continence were normal. The child also had a high anorectal malformation with a coexistent type IV congenital pouch colon (CPC) malformation. Both kidneys were normal. Preliminary surgery consisted of a divided sigmoid colostomy proximal to the colonic pouch. The literature is reviewed and the embryogenesis of pseudoexstrophy and its associated malformations are discussed.
Chadha
R
R
Department of Paediatric Surgery, Lady Hardinge Medical College and Kalawati Saran Children's Hospital, New Delhi, India.
Sharma
A
A
Bagga
D
D
Mahajan
J K
JK
D000015:Abnormalities, Multiple; D001003:Anal Canal / Q000002:abnormalities*; D001746:Bladder Exstrophy / Q000150:complications*; D003106:Colon / Q000002:abnormalities*; D003125:Colostomy; D005837:Genitalia, Male / Q000002:abnormalities*; D006801:Humans; D007231:Infant, Newborn; D008297:Male