Prune belly syndrome with pouch colon and absent dermatome

Abstract

Prune belly syndrome (PBS) is a rare congenital constellation of defects in pediatric surgical practice. Although anorectal anomalies have been reported in association with PBS, only few case of pouch colon with PBS has been reported.[1] In addition, our patient had deficient abdominal wall with absent dermatome in left upper quadrant, which has never been reported in the
English literature. This association with abdominal wall deÞ ciency and absent dermatome not only strengthens the theory of mesodermal arrest in the etiology of PBS but also points towards a defect in the ectodermal
development.

Key words: Prune belly, pouch colon, anorectal malformation