Segmental Absence of Intestinal Musculature in a Child With Type IV Ehlers-Danlos Syndrome

Authors

Nicole Zeky, LSU Health Sciences Center- New OrleansFollow
Celia Short, LSU Health Sciences Center- New OrleansFollow
Brent Keith, LSU Health Sciences Center- New Orleans
Randall Craver, LSU Health Sciences Center- New OrleansFollow
Jessica A. Zagory, LSU Health Sciences Center- New OrleansFollow

Document Type

Article

Publication Date

8-3-2021

Publication Title

Children

Abstract

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.

First Page

1

Last Page

6

Volume

8

Issue

8

Publisher

MDPI

Creative Commons License

This work is licensed under a Creative Commons Attribution 4.0 International License.

Recommended Citation

Zeky, Nicole; Short, Celia; Keith, Brent; Craver, Randall; and Zagory, Jessica A., "Segmental Absence of Intestinal Musculature in a Child With Type IV Ehlers-Danlos Syndrome" (2021). School of Medicine Faculty Publications. 249.
https://digitalscholar.lsuhsc.edu/som_facpubs/249
10.3390/children8080680

File Format

pdf

File Size

1440 KB