NEONATAL INTESTINAL PERFORATION SECONDARY TO CONGENITAL SEGMENTAL AGENESIS OF INTESTINAL MUSCULARIS

Congenital segmental absence of intestinal musculature is a rare disor der of unknown etiology. It is a rare cause of intestinal obstruction or perforation in newborns which may resemble necrotizing enterocoliti s (NEC). The diagnosis is confirmed histologically by segmental absenc e of the muscularis propria, normal intact mucosa, submucosa and seros a. The absence of inflammation, hemorrhage and necrosis helps to disti nguish it from NEC. A case of an infant boy with intestinal perforatio n secondary to segmental absence of intestinal musculature is presente d, the literature is reviewed and possible etiologies are discussed.