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.
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Zeky, Nicole; Short, Celia; Keith, Brent; Craver, Randall D.; 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.