title: Rupture of the abdominal aorta in patients with Ehlers-Danlos syndrome.
abstract: Ehlers-Danlos syndrome (EDS) is a heterogeneous inherited disorder of collagen synthesis. Type IV is frequently associated with major vascular catastrophes and challenges the vascular surgeon with its varied clinical presentation and the difficulty of vascular repair. Rupture of the abdominal aorta is one of the most serious complications and is associated with nearly 100% mortality rate. We describe here three patients with type IV EDS.
authors.affiliation: Department of Surgery, Royal Preston Hospital, Preston, UK.
authors.forename: C D
authors.initials: CD
authors.lastname: Karkos
authors.forename: V
authors.initials: V
authors.lastname: Prasad
authors.forename: U
authors.initials: U
authors.lastname: Mukhopadhyay
authors.forename: G J
authors.initials: GJ
authors.lastname: Thomson
authors.forename: A R
authors.initials: AR
authors.lastname: Hearn
mesh_terms: D000328:Adult; D001012:Aorta, Abdominal / Q000293:injuries* / Q000601:surgery*; D017544:Aortic Aneurysm, Abdominal / Q000150:complications* / Q000601:surgery; D004535:Ehlers-Danlos Syndrome / Q000150:complications*; D017809:Fatal Outcome; D005260:Female; D006488:Hemostasis, Surgical; D006801:Humans; D007431:Intraoperative Complications; D008297:Male; D012421:Rupture