Ileal atresia with segmental defect of intestinal musculature.

An unusual case of segmental defect of ileal musculature with terminal ileal atresia and ventricular septal defect is reported. Clinically the patient was diagnosed as ileal atresia. On exploration, there was type I atresia at ileocaecal junction with attendant ileal dilatation and an adjoining 5 cm x 4 cm segment resembling segmental dilatation of ileum, about 15 cm proximal to ileocaecal junction. Pathological examination of segmentally dilated ileum revealed focal absence of circular and longitudinal muscle coat without any evidence of necrosis or inflammation. The possible etiology and its relationship with congenital segmental dilatation of intestine is discussed.