Submucosal Supernumerary Smooth Muscle Coat: A Common Histologic Finding in Mowat-Wilson Syndrome With or Without Hirschsprung Disease.
Hirschsprung disease
Mowat–Wilson syndrome
ZEB2
pseudo-obstruction
smooth muscle
supernumerary muscle
Journal
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
ISSN: 1615-5742
Titre abrégé: Pediatr Dev Pathol
Pays: United States
ID NLM: 9809673
Informations de publication
Date de publication:
Historique:
pubmed:
30
5
2020
medline:
3
8
2021
entrez:
30
5
2020
Statut:
ppublish
Résumé
Mowat-Wilson syndrome (MWS) is a multiorgan system disorder caused by Rectal biopsies and rectosigmoidectomy specimens from MWS patients were identified by retrospective reviews of surgical pathology records. Routinely prepared glass slides were examined to determine whether any smooth muscle structural alteration was present. Clinical information was obtained by chart review. Six MWS patients were identified. A supernumerary smooth muscle coat in the submucosa was present in 3 of them, including 2 of the 4 patients with HSCR. The structural anomaly, termed submucosal supernumerary smooth muscle coat, is not a syndrome-specific pathological feature. However, it appears to be more common than expected in MWS and is consistent with contemporary models for the roles of
Sections du résumé
BACKGROUND
BACKGROUND
Mowat-Wilson syndrome (MWS) is a multiorgan system disorder caused by
METHOD
METHODS
Rectal biopsies and rectosigmoidectomy specimens from MWS patients were identified by retrospective reviews of surgical pathology records. Routinely prepared glass slides were examined to determine whether any smooth muscle structural alteration was present. Clinical information was obtained by chart review.
RESULTS
RESULTS
Six MWS patients were identified. A supernumerary smooth muscle coat in the submucosa was present in 3 of them, including 2 of the 4 patients with HSCR.
CONCLUSION
CONCLUSIONS
The structural anomaly, termed submucosal supernumerary smooth muscle coat, is not a syndrome-specific pathological feature. However, it appears to be more common than expected in MWS and is consistent with contemporary models for the roles of
Identifiants
pubmed: 32469269
doi: 10.1177/1093526620925960
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM