Ehlers-Danlos Syndrome Type IV: A Case Report. (April 2016)

• Record Type: Journal Article
• Authors: Soo-Hoo, Sarah; Porten, Brandon R.; Engstrom, Bjorn I.; Skeik, Nedaa
• Is Part Of: Vascular & endovascular surgery. Volume 50:Number 3(2016)
• Page Start: 156
• Page End: 159
• Publication Date: 2016-04
• Subjects: Ehlers-Danlos syndrome -- type IV -- arterial dissection and rupture; Blood-vessels -- Surgery -- Periodicals; Blood-vessels -- Endoscopic surgery -- Periodicals; Vascular Surgical Procedures -- Periodicals; Angioplasty -- Periodicals; Surgical Procedures, Minimally Invasive -- Periodicals; Vascular Diseases -- surgery -- Periodicals; Vaisseaux sanguins -- Chirurgie -- Périodiques; Vaisseaux sanguins -- Chirurgie endoscopique -- Périodiques; 617.41
• DOI: 10.1177/1538574416627697
• Languages: English
• ISSNs: 1538-5744

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.