title: Ehlers-Danlos Syndrome Type IV: A Case Report.
abstract: Ehlers-Danlos syndrome (EDS) encompasses a group of rare genetic connective tissue disorders. The vascular type (type IV) poses the most serious risk to patients. Diagnosis is usually difficult, especially if patients lack a family history. Life-threatening vascular emergency such as dissection or rupture can be the first presenting symptom. Management of the disease can pose a clinical challenge due to the emergency of presentation, tissue friability, and lack of clear management recommendations. We report a unique case of a 40-year-old man who presented with a ruptured celiac artery and a strong family history of EDS. This case highlights the difficulties and complications associated with treating this uncommon and serious disease.
authors.affiliation: Department of Vascular Medicine, Minneapolis Heart Institute, Minneapolis, MN, USA.
authors.forename: Sarah
authors.initials: S
authors.lastname: Soo-Hoo
authors.affiliation: Department of Vascular Medicine, Minneapolis Heart Institute, Minneapolis, MN, USA.
authors.forename: Brandon R
authors.initials: BR
authors.lastname: Porten
authors.affiliation: Department of Vascular and Interventional Radiology, Abbott Northwestern Hospital, Minneapolis, MN, USA.
authors.forename: Bjorn I
authors.initials: BI
authors.lastname: Engstrom
authors.affiliation: Department of Vascular Medicine, Minneapolis Heart Institute, Minneapolis, MN, USA nedaa.skeik@allina.com.
authors.forename: Nedaa
authors.initials: N
authors.lastname: Skeik
mesh_terms: D000328:Adult; D000784:Aortic Dissection / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery; D017541:Aneurysm, False / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery; D017542:Aneurysm, Ruptured / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery; D019917:Blood Vessel Prosthesis Implantation; D002445:Celiac Artery / Q000000981:diagnostic imaging / Q000601:surgery; D000072226:Computed Tomography Angiography; D018450:Disease Progression; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000175:diagnosis; D057510:Endovascular Procedures / Q000295:instrumentation; D006801:Humans; D008297:Male; D015607:Stents; D016896:Treatment Outcome
keywords: Ehlers-Danlos syndrome; arterial dissection and rupture; type IV