Soo-Hoo Sarah, Porten Brandon R, Engstrom Bjorn I, Skeik Nedaa
Department of Vascular Medicine, Minneapolis Heart Institute, Minneapolis, MN, USA.
Department of Vascular and Interventional Radiology, Abbott Northwestern Hospital, Minneapolis, MN, USA.
Vasc Endovascular Surg. 2016 Apr;50(3):156-9. doi: 10.1177/1538574416627697. Epub 2016 Mar 13.
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.
埃勒斯-当洛综合征（EDS）是一组罕见的遗传性结缔组织疾病。血管型（IV型）对患者构成的风险最为严重。诊断通常很困难，尤其是在患者没有家族病史的情况下。诸如夹层或破裂等危及生命的血管急症可能是首发症状。由于病情紧急、组织脆弱以及缺乏明确的治疗建议，该病的治疗可能会带来临床挑战。我们报告了一例独特的病例，一名40岁男性，患有腹腔动脉破裂且有很强的EDS家族病史。该病例突出了治疗这种罕见且严重疾病所面临的困难和并发症。
