Howard Ryan, Osborne Nicholas
Department of Surgery, Michigan Medicine, Ann Arbor, Mich.
Section of Vascular Surgery, Michigan Medicine, Ann Arbor, Mich.
J Vasc Surg Cases Innov Tech. 2020 Feb 12;6(1):71-74. doi: 10.1016/j.jvscit.2019.11.013. eCollection 2020 Mar.
Vascular Ehlers-Danlos syndrome (vEDS), also known as type IV Ehlers-Danlos syndrome, is a rare inherited connective tissue disease that affects 1 in 50,000 to 250,000 individuals. It is characterized by catastrophic vascular complications and hollow viscus rupture; 80% of patients with vEDS experience a vascular complication by the age of 40 years, and median life expectancy is 40 to 50 years. The central vasculature and visceral vasculature are most commonly affected; peripheral involvement is much less common. We describe the case of a 40-year-old woman with vEDS previously complicated by ruptured splenic and posterior tibial artery aneurysms who presented with a ruptured left ulnar artery aneurysm resulting in compartment syndrome.
血管型埃勒斯-当洛综合征（vEDS），也称为IV型埃勒斯-当洛综合征，是一种罕见的遗传性结缔组织疾病，每50000至250000人中就有1人患病。其特征为灾难性的血管并发症和中空脏器破裂；80%的vEDS患者在40岁前会经历血管并发症，中位预期寿命为40至50岁。中央脉管系统和内脏脉管系统最常受累；外周受累则较为少见。我们报告了一例40岁患有vEDS的女性病例，该患者既往有脾动脉瘤和胫后动脉瘤破裂史，此次因左尺动脉瘤破裂导致骨筋膜室综合征前来就诊。
