Cargando…

Segmental Absence of Intestinal Musculature in a Child with Type IV Ehlers–Danlos Syndrome

Patients with vascular Ehlers–Danlos syndrome (vEDS) have a defect in the formation of type III collagen. This defect puts patients at risk of vascular rupture, uterine rupture, and bowel perforations. The segmental absence of intestinal musculature is a rare histopathologic finding, wherein there i...

Descripción completa

Detalles Bibliográficos
Autores principales: Zeky, Nicole, Short, Celia, Keith, Brent, Craver, Randall D., Zagory, Jessica A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8393798/
https://www.ncbi.nlm.nih.gov/pubmed/34438571
http://dx.doi.org/10.3390/children8080680
_version_ 1783743807140921344
author Zeky, Nicole
Short, Celia
Keith, Brent
Craver, Randall D.
Zagory, Jessica A.
author_facet Zeky, Nicole
Short, Celia
Keith, Brent
Craver, Randall D.
Zagory, Jessica A.
author_sort Zeky, Nicole
collection PubMed
description Patients with vascular Ehlers–Danlos syndrome (vEDS) have a defect in the formation of type III collagen. This defect puts patients at risk of vascular rupture, uterine rupture, and bowel perforations. The segmental absence of intestinal musculature is a rare histopathologic finding, wherein there is a lack of a muscularis propria layer in the intestinal wall. Although typically documented in the literature in neonates or adults, it can be seen in children of other ages. This is a case report of a patient who exhibits both rare entities, which has not been described in the literature to date.
format Online
Article
Text
id pubmed-8393798
institution National Center for Biotechnology Information
language English
publishDate 2021
publisher MDPI
record_format MEDLINE/PubMed
spelling pubmed-83937982021-08-28 Segmental Absence of Intestinal Musculature in a Child with Type IV Ehlers–Danlos Syndrome Zeky, Nicole Short, Celia Keith, Brent Craver, Randall D. Zagory, Jessica A. Children (Basel) Case Report Patients with vascular Ehlers–Danlos syndrome (vEDS) have a defect in the formation of type III collagen. This defect puts patients at risk of vascular rupture, uterine rupture, and bowel perforations. The segmental absence of intestinal musculature is a rare histopathologic finding, wherein there is a lack of a muscularis propria layer in the intestinal wall. Although typically documented in the literature in neonates or adults, it can be seen in children of other ages. This is a case report of a patient who exhibits both rare entities, which has not been described in the literature to date. MDPI 2021-08-05 /pmc/articles/PMC8393798/ /pubmed/34438571 http://dx.doi.org/10.3390/children8080680 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Zeky, Nicole
Short, Celia
Keith, Brent
Craver, Randall D.
Zagory, Jessica A.
Segmental Absence of Intestinal Musculature in a Child with Type IV Ehlers–Danlos Syndrome
title Segmental Absence of Intestinal Musculature in a Child with Type IV Ehlers–Danlos Syndrome
title_full Segmental Absence of Intestinal Musculature in a Child with Type IV Ehlers–Danlos Syndrome
title_fullStr Segmental Absence of Intestinal Musculature in a Child with Type IV Ehlers–Danlos Syndrome
title_full_unstemmed Segmental Absence of Intestinal Musculature in a Child with Type IV Ehlers–Danlos Syndrome
title_short Segmental Absence of Intestinal Musculature in a Child with Type IV Ehlers–Danlos Syndrome
title_sort segmental absence of intestinal musculature in a child with type iv ehlers–danlos syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8393798/
https://www.ncbi.nlm.nih.gov/pubmed/34438571
http://dx.doi.org/10.3390/children8080680
work_keys_str_mv AT zekynicole segmentalabsenceofintestinalmusculatureinachildwithtypeivehlersdanlossyndrome
AT shortcelia segmentalabsenceofintestinalmusculatureinachildwithtypeivehlersdanlossyndrome
AT keithbrent segmentalabsenceofintestinalmusculatureinachildwithtypeivehlersdanlossyndrome
AT craverrandalld segmentalabsenceofintestinalmusculatureinachildwithtypeivehlersdanlossyndrome
AT zagoryjessicaa segmentalabsenceofintestinalmusculatureinachildwithtypeivehlersdanlossyndrome