Increased submucosal nerve trunk caliber in aganglionosis: a "positive" and objective finding in suction biopsies and segmental resections in Hirschsprung's disease.
OBJECTIVE
To establish the diagnostic usefulness of submucosal hypertrophic nerve trunk morphology in Hirschsprung's disease as a quantifiable parameter supportive of aganglionosis on hematoxylin-eosin-stained sections.


METHODS
We retrospectively evaluated size and density of submucosal nerves on hematoxylin-eosin-stained sections and S100 protein-stained sections of resected segments from 13 patients with Hirschsprung's disease, and in sections of 20 aganglionic and 50 ganglionic rectal suction biopsies.


METHODS
All patients were seen at Childrens Hospital Los Angeles (Calif), a tertiary-care pediatric center; the age of patients at diagnosis or resection ranged between 2 days and 3 years.


RESULTS
Aganglionic segments contain many distinct nerve trunks greater than 40 microm in diameter. Ganglionic segments/biopsies showed no nerve trunk larger than this threshold value (P approximately .0000). Nerve trunks of such caliber are rarely encountered in pathologic transition zones and sites of colostomy.


CONCLUSIONS
Submucosal nerve trunks that are 40 microm or greater in diameter strongly correlate with abnormal innervation/aganglionosis. Use of this objective parameter in evaluating suction biopsies should be helpful in the morphologic diagnosis of Hirschsprung's disease in infancy and early childhood.
Department of Pathology and Laboratory Medicine, Childrens Hospital Los Angeles, University of Southern California School of Medicine, 90027, USA.
H
H
Monforte-Muñoz
I
I
Gonzalez-Gomez
J M
JM
Rowland
B H
BH
Landing
D001707:Biopsy, Needle; D002675:Child, Preschool; D003106:Colon / Q000737:chemistry / Q000294:innervation; D005724:Ganglia / Q000737:chemistry / Q000473:pathology*; D006627:Hirschsprung Disease / Q000473:pathology*; D006801:Humans; D006984:Hypertrophy; D007150:Immunohistochemistry; D007223:Infant; D007231:Infant, Newborn; D007413:Intestinal Mucosa / Q000737:chemistry / Q000294:innervation; D009474:Neurons / Q000737:chemistry / Q000473:pathology*; D012007:Rectum / Q000737:chemistry / Q000294:innervation; D012189:Retrospective Studies; D009418:S100 Proteins / Q000032:analysis
D009418:S100 Proteins