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

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.

• Authors: Nicole Zeky, Celia Short, Brent Keith, Randall D Craver, Jessica A Zagory
• Journal: Children (Basel, Switzerland) (Children (Basel)) Vol. 8 Issue 8 (Aug 05 2021) ISSN: 2227-9067 [Print] Switzerland
• PMID: 34438571 (Publication Type: Case Reports)