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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| Main Authors: | , , , , |
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| Format: | Book |
| Published: |
MDPI AG,
2021-08-01T00:00:00Z.
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| Online Access: | Connect to this object online. |
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| Summary: | 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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| Item Description: | 10.3390/children8080680 2227-9067 |