| Autor: |
Zeky N; Department of Pediatrics, Division of Gastroenterology, Louisiana State University Health Sciences Center, New Orleans, LA 70112, USA., Short C; Department of Surgery, Louisiana State University Health Sciences Center, New Orleans, LA 70112, USA., Keith B; Department of Pediatrics, Division of Gastroenterology, Louisiana State University Health Sciences Center, New Orleans, LA 70112, USA., Craver RD; Department of Pathology, Louisiana State University Health Sciences Center, New Orleans, LA 70112, USA., Zagory JA; Department of Surgery, Louisiana State University Health Sciences Center, New Orleans, LA 70112, USA.; Department of Surgery, Division of Pediatric Surgery, Children's Hospital New Orleans, 200 Henry Clay Avenue, New Orleans, LA 70118, USA. |
| Abstrakt: |
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. |
