Neuronal intestinal and fibromuscular arterial dysplasias associated with intraluminal mucosal web.

A two-year-old boy presenting with the signs and symptoms of partial intestinal obstruction caused by a mucosal web in the ileum is presented. Histopathological evaluation following resection and primary anastomosis revealed neuronal intestinal dysplasia associated with fibromuscular arterial dysplasia limited to the segment proximal to the web and normal findings in the distal part. These findings and a brief review of the literature support the hypothesis that neuronal and fibromuscular dysplasias may develop on the basis of congenital obstructive lesions of the bowel.