Primary myenteric plexus alterations as a cause of megacolon in Von Recklinghausen's disease.

This is a case report of a megacolon in a 26 years old male patient having typical von Recklinghausen's disease. Exploratory laparotomy shows no neurofibroma on digestive tract. Myenteric plexus study of colic resection specimen following B. Smith technique reveals marked alterations. These alterations are both quantitative (hyperhypoganglionosis) and qualitative (ganglion cells are irregular in shape and size, dendritic processes are hypertrophic, irregular; schwannosis; no inflammatory infiltration). The patient suffers no more transit disorders after adequate resection of this megacolon.