INTRACTABLE CONSTIPATION WITH A DECREASE IN SUBSTANCE P-IMMUNOREACTIVE FIBERS - IS IT A VARIANT OF INTESTINAL NEURONAL DYSPLASIA

After Hirschsprung's disease was ruled out for 25 children who had sev ere chronic constipation, the authors studied the distribution of immu noreactivity for substance P (SP) and vasoactive intestinal peptide (V IP) in the intestinal wall, using immunofluorescence. SP and VIP immun oreactivity identify excitatory and inhibitory nerve fibres, respectiv ely. Full-thickness rectal biopsy specimens were unsatisfactory, so se romuscular biopsies of the caecum, transverse colon, and sigmoid colon were obtained (by laparoscopy and laparotomy; n = 10 patients). SP-im munoreactive fibres were markedly reduced in seven, with concomitant r eduction of VIP-immunoreactive fibres in four. In two other patients, there was no obvious reduction in SP- or VIP-immunoreactive fibres. In a patient who subsequently was found to have multiple endocrine neopl asia type 2b, the myenteric plexus was markedly hyperplastic, with an increase in nerve cells and nerve fibres. VIP-immunoreactive fibres we re increased, but SP-immunoreactive fibres were markedly decreased. Su rgical options included proximal stoma. Malone operation, and subtotal colectomy with preservation of the rectum. Three children with subtot al colectomy have had improvement over short-term follow-up. The combi nation of seromuscular laparoscopic biopsies and immunofluorescence de monstration of neuropeptides may identify new variants of intestinal n euronal dysplasia than can be treated successfully with surgery. (C) 1 996 by W.B. Saunders Company