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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...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2021
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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 |
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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 |
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