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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...

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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
Descripción
Sumario: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.