Batagini Nayara Cioffi, Gornik Heather, Kirksey Lee
Vascular Surgery Department, The Cleveland Clinic, Cleveland, OH, USA
Vascular Medicine Department, The Cleveland Clinic, Cleveland, OH, USA.
Vasc Endovascular Surg. 2015 Jul-Aug;49(5-6):152-4. doi: 10.1177/1538574415602783. Epub 2015 Aug 30.
Vascular Ehlers-Danlos Syndrome (VEDS) is a rare autosomal dominant collagen vascular disorder. Different from other Ehler-Danlos Syndrome subtypes, VEDS has poor prognosis due to severe fragility of connective tissues and association with life-threatening vascular and gastrointestinal complications. Spontaneous splenic rupture is a rare but hazardous complication related to this syndrome. To date, only 2 cases have been reported in the literature. Here we present another case of this uncommon complication, occurring in a 54-year-old woman in clinical follow-up for VEDS who presented with sudden onset of abdominal pain and hypotension.
血管性埃勒斯-当洛综合征（VEDS）是一种罕见的常染色体显性遗传性胶原血管疾病。与其他埃勒斯-当洛综合征亚型不同，VEDS由于结缔组织严重脆弱以及与危及生命的血管和胃肠道并发症相关，预后较差。自发性脾破裂是与该综合征相关的一种罕见但危险的并发症。迄今为止，文献中仅报道了2例。在此，我们报告另一例这种罕见并发症的病例，发生在一名54岁患有VEDS的女性患者身上，该患者在临床随访中突然出现腹痛和低血压。
