INTESTINAL NEURONAL DYSPLASIA IS A POSSIBLE CAUSE OF PERSISTENT BOWELSYMPTOMS AFTER PULL-THROUGH OPERATION FOR HIRSCHSPRUNGS-DISEASE

The proximal margin of the resected bower specimens from 33 consecutiv ely treated patients undergoing a definitive pull-through operation fo r Hirschsprung's disease (HD) and control specimens consisting of suct ion rectal biopsy specimens obtained from 24 age-matched patients eval uated for constipation (and proven not to have HD) were examined using conventional HandE staining and acetylcholinesterase (AChE) histochem istry. Complete resection of the aganglionic segment was confirmed in 31 patients. In one patient, the proximal margin was found to be agang lionic; in another, the proximal margin was in a transitional zone. In both patients, frozen sections at the time of surgery were interprete d as having ganglion cells. In 10 of 31 patients, intestinal neuronal dysplasia was demonstrated in the proximal margin of the resected bowe l. The abnormalities included hyperplasia of the submucous plexus, gia nt ganglia (with >7 ganglion cells), and ectopic ganglion cells (all 1 0 patients) and increased AChE activity in the lamina propria (5 patie nts). All ten patients with IND had persistent bower problems after th e definitive operation for HD, such as enterocolitis, soiling, or cons tipation. Only four of the other 21 patients had persistent bowel symp toms. This study suggests that IND is commonly associated with HD. It also emphasizes the impor tance of histochemical examination of the re sected segment to predict postoperative bowel function in patients wit h HD. Copyright (C) 1995 by W.B. Saunders Company