title: Intractable constipation with a decrease in substance P-immunoreactive fibres: is it a variant of intestinal neuronal dysplasia?
abstract: After Hirschsprung's disease was ruled out for 25 children who had severe chronic constipation, the authors studied the distribution of immunoreactivity for substance P (SP) and vasoactive intestinal peptide (VIP) in the intestinal wall, using immunofluorescence. SP and VIP immunoreactivity identify excitatory and inhibitory nerve fibres, respectively. Full-thickness rectal biopsy specimens were unsatisfactory, so seromuscular biopsies of the caecum, transverse colon, and sigmoid colon were obtained (by laparoscopy and laparotomy; n = 10 patients). SP-immunoreactive fibres were markedly reduced in seven, with concomitant reduction 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 neoplasia type 2b, the myenteric plexus was markedly hyperplastic, with an increase in nerve cells and nerve fibres. VIP-immunoreactive fibres were increased, but SP-immunoreactive fibres were markedly decreased. Surgical options included proximal stoma, Malone operation, and subtotal colectomy with preservation of the rectum. Three children with subtotal colectomy have had improvement over short-term follow-up. The combination of seromuscular laparoscopic biopsies and immunofluorescence demonstration of neuropeptides may identify new variants of intestinal neuronal dysplasia than can be treated successfully with surgery.
authors.affiliation: F. Douglas Stephens Surgical Research Unit, Royal Children's Hospital, Melbourne, Australia.
authors.forename: J M
authors.initials: JM
authors.lastname: Hutson
authors.forename: C W
authors.initials: CW
authors.lastname: Chow
authors.forename: J
authors.initials: J
authors.lastname: Borg
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000473:pathology / Q000601:surgery; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003082:Colectomy; D003106:Colon / Q000294:innervation / Q000473:pathology / Q000601:surgery; D003112:Colonic Pseudo-Obstruction / Q000473:pathology* / Q000601:surgery; D003248:Constipation / Q000473:pathology* / Q000601:surgery; D005260:Female; D005455:Fluorescent Antibody Technique; D006627:Hirschsprung Disease / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008297:Male; D009412:Nerve Fibers / Q000473:pathology; D013373:Substance P / Q000032:analysis*; D014660:Vasoactive Intestinal Peptide / Q000032:analysis
chemical_list: D013373:Substance P; D014660:Vasoactive Intestinal Peptide