Embolization of a hepatoportal fistula in a patient with Ehlers-Danlos syndrome and colon perforation.

Sherry, R M; Fisch, A; Grey, D P; Lubbock, C A · Surgery · 1992

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.

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