Embolization of a hepatoportal fistula in a patient with Ehlers-Danlos syndrome and colon perforation
1992
: 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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