Embolization of a hepatoportal fistula in a patient with Ehlers-Danlos syndrome and colon perforation.
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.
Sherry
R M
RM
Department of Surgery, Kaiser Permanente Medical Center, San Francisco, Calif.
Fisch
A
A
Grey
D P
DP
Lubbock
C A
CA
D000293:Adolescent; D000792:Angiography; D003108:Colonic Diseases / Q000150:complications / Q000601:surgery*; D003125:Colostomy; D004535:Ehlers-Danlos Syndrome / Q000150:complications*; D004621:Embolization, Therapeutic; D005402:Fistula / Q000150:complications / Q000628:therapy*; D006499:Hepatic Artery / Q000000981:diagnostic imaging; D006801:Humans; D007416:Intestinal Perforation / Q000150:complications / Q000601:surgery*; D008297:Male; D014057:Tomography, X-Ray Computed; D014652:Vascular Diseases / Q000150:complications / Q000628:therapy