Enteric nervous system, interstitial cells of cajal, and smooth muscle vacuolization in segmental dilatation of jejunum

Background/Purpose: The etiology of congenital segmental dilatation (CSD) of bowel remains elusive. Intestitial cell of Cajal plays a role in the pace making of the intestine. Its abnormality has been documented in a variety of conditions of abnormal intestinal motility. The current study attempts to evaluate the roles of intestitial cells of Cajal, enteric nervous system, and smooth muscle in segmental dilatation of small bowel. Methods: Resected specimen from a neonate with segmental dilatation of jejunum was stained with H&E, Alcian blue, Periodic Acid-Schiff (PAS), and immunostained with S100, Ret, MAP5, and c-kit antibodies using the standard immunohistochemical process. Results: The immunostaining of S100, Ret, MAP5 and c-kit of CSD specimen were positive. Localized vacuolization was, however, detected in the circular smooth muscle of the jejunum. The Alcian blue and PAS staining of the vacuolization were negative. Conclusions: CSD shows no abnormality in the enteric nervous system and pace makers. Localized vacuolization suggests myopathy to be a contributing factor to the disease. J Pediatr Surg 36:930-935. Copyright (C) 2001 by W.B. Saunders Company.