Deficient neurogenic innervation of the myenteric plexus with normal submucous plexus involving the entire small and large bowel.

A newborn presented with a picture of intestinal obstruction. Multiple biopsies of the small and large bowel showed an unusual neurogenic innervation. The myenteric plexus of Auerbach was severely depleted of ganglion cells and nerve fibers, while the submucous plexus of Meissner was normally innervated. An ileostomy failed to function and extended trial with experimental smooth muscle stimulant (Cisapride) was equally ineffective. The patient was finally treated by a myectomy from the duodenum to the descending colon with a sigmoid colostomy. This procedure, coupled with a Nissen fundoplication, stopped the vomiting and allowed normal defecation through the colostomy. The patient is presently taking increasing increments of oral fluids with a concomitant decrease in the volume of parenteral nutrition. The myectomy initiated marked hypertrophy of the muscularis mucosa. Could this muscular hypertrophy account for the improvement in bowel function? Possible etiology will be discussed. We caution that rectal submucosal suction biopsy alone may be misleading if normal ganglion cells and nerve fibers are found, yet the patient's clinical symptoms fail to improve. A full thickness bowel wall biopsy is then recommended.