Embolization of a hepatoportal fistula in a patient with Ehlers-Danlos syndrome and colon perforation.
case_report · Level V
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Abstract
Patients with type IV Ehlers-Danlos syndrome (EDS) have defective collagen synthesis, which places them at risk for spontaneous vascular rupture and other clinical sequelae. After treatment with resection and colostomy, a hepatoportal fistula developed in a 16-year-old boy with type IV EDS. The fistula was embolized angiographically by transcatheter coil occlusion. Management options for patients with type IV EDS and these sequelae are reviewed.
Medical subject headings
- Colonic Diseases
- Ehlers-Danlos Syndrome
- Embolization, Therapeutic
- Fistula
- Hepatic Artery
- Intestinal Perforation