The Extent of the Transition Zone in Hirschsprung Disease.
Aganglionosis
Constipation
Hirschsprung
Hypertrophic nerve trunk
Hypoganglionosis
Pull-through
Transition zone
Journal
Journal of pediatric surgery
ISSN: 1531-5037
Titre abrégé: J Pediatr Surg
Pays: United States
ID NLM: 0052631
Informations de publication
Date de publication:
Nov 2019
Nov 2019
Historique:
received:
25
11
2018
revised:
09
03
2019
accepted:
17
04
2019
pubmed:
14
5
2019
medline:
23
2
2020
entrez:
14
5
2019
Statut:
ppublish
Résumé
Retained transition zone is a leading cause of obstructive symptoms after pull-through operation in Hirschsprung's disease. We aimed to evaluate the extent of the histological transition zone in patients with Hirschsprung's disease. We performed an observational study. DAB+ immunohistochemistry for Protein Gene Product 9.5 was used to evaluate the neuronal networks in serial sections of pull-through specimens obtained from children with Hirschsprung's disease (n = 12). Reference ranges for ganglion size/density and nerve trunk diameter were statistically determined using healthy controls obtained from colostomy specimens from children with anorectal malformations (n = 8). The transition zone was defined as ganglionic bowel exhibiting ganglion hypoplasia, hypertrophic nerve trunks, or partial circumference aganglionosis. The mean submucosal nerve trunk diameter in controls was 19.56 μm +/- 3.87 μm. The median age at pull-through for Hirschsprung's disease was 5 months (3-14 months). The median length of the transition zone across the population was 8 cm (4-22 cm). Median transition zone extent was significantly longer in patients with long-segment aganglionosis (n = 6) compared to rectosigmoid aganglionosis (n = 6, 13 cm vs 6 cm, p = 0.041). Due to the age of the patients enrolled, long-term follow-up of bowel function is not yet available. Our data suggest that, in children with rectosigmoid Hirschsprung's disease, the transition zone can extend for up to 13 cm. In children with long-segment disease, a longer transition zone is possible. Extended resection at a minimum 5 cm beyond the most distal ganglionic intra-operative biopsy and intra-operative histological examination of the proximal resection margin are required to minimize transition zone pull-through. 2.
Sections du résumé
BACKGROUND
BACKGROUND
Retained transition zone is a leading cause of obstructive symptoms after pull-through operation in Hirschsprung's disease.
OBJECTIVE
OBJECTIVE
We aimed to evaluate the extent of the histological transition zone in patients with Hirschsprung's disease.
DESIGN
METHODS
We performed an observational study. DAB+ immunohistochemistry for Protein Gene Product 9.5 was used to evaluate the neuronal networks in serial sections of pull-through specimens obtained from children with Hirschsprung's disease (n = 12). Reference ranges for ganglion size/density and nerve trunk diameter were statistically determined using healthy controls obtained from colostomy specimens from children with anorectal malformations (n = 8). The transition zone was defined as ganglionic bowel exhibiting ganglion hypoplasia, hypertrophic nerve trunks, or partial circumference aganglionosis.
RESULTS
RESULTS
The mean submucosal nerve trunk diameter in controls was 19.56 μm +/- 3.87 μm. The median age at pull-through for Hirschsprung's disease was 5 months (3-14 months). The median length of the transition zone across the population was 8 cm (4-22 cm). Median transition zone extent was significantly longer in patients with long-segment aganglionosis (n = 6) compared to rectosigmoid aganglionosis (n = 6, 13 cm vs 6 cm, p = 0.041). Due to the age of the patients enrolled, long-term follow-up of bowel function is not yet available.
CONCLUSION
CONCLUSIONS
Our data suggest that, in children with rectosigmoid Hirschsprung's disease, the transition zone can extend for up to 13 cm. In children with long-segment disease, a longer transition zone is possible. Extended resection at a minimum 5 cm beyond the most distal ganglionic intra-operative biopsy and intra-operative histological examination of the proximal resection margin are required to minimize transition zone pull-through.
LEVEL OF EVIDENCE
METHODS
2.
Identifiants
pubmed: 31079866
pii: S0022-3468(19)30314-8
doi: 10.1016/j.jpedsurg.2019.04.017
pii:
doi:
Types de publication
Journal Article
Observational Study
Langues
eng
Sous-ensembles de citation
IM
Pagination
2318-2324Informations de copyright
Copyright © 2019 Elsevier Inc. All rights reserved.