Segmental absence of intestinal musculature: A rare cause of intestinal obstruction in infants

Segmental absence of intestinal musculature (SAIM) is a rare entity of unknown cause. Presenting features vary from intestinal obstruction, necrotizing enterocolitis, volvulus, and intussusception to spontaneous intestinal perforations. In majority of the cases, SAIM is a histological diagnosis. Here, we present a case of a 1-month female baby who presented with fever, abdominal distension, and vomiting. Resected segment of intestine histologically showed focal segmental absence of the muscularis propria. To the best of our knowledge, fewer than 50 cases of SAIM have been reported in the literature so far.