■血管Ehlers-Danlos综合征由于出血性并发症而具有很高的死亡率。
■我们报告一例因多发性腹腔动脉瘤破裂而诊断为血管型Ehlers-Danlos综合征。患者是一名25岁的日本男子,有乙状结肠穿孔史。由于腹痛,他被送往附近的医院。在住院的第9天,病人经历了休克。增强腹部计算机断层扫描显示肝动脉瘤和腹腔出血,病人被转移到我们医院。紧急腹部血管造影显示腹腔多发动脉瘤,普通,和右肝动脉.右肝动脉被认为是负责任的并且被栓塞。患者具有该综合征的特征性身体检查结果,帮助确认COL3A1基因异常的遗传分析。
■幼年性结肠穿孔和腹腔动脉破裂是怀疑血管型Ehlers-Danlos综合征的关键发现。
UNASSIGNED: Vascular Ehlers-Danlos syndrome has a high mortality rate due to hemorrhagic complications.
UNASSIGNED: We report a
case of vascular-type Ehlers-Danlos syndrome diagnosed due to rupture of multiple celiac aneurysms. The patient was a 25-year-old Japanese man with a history of a sigmoid perforation. He was admitted to a nearby hospital because of abdominal pain. On day 9 of hospitalization, the patient experienced shock. Enhanced abdominal computed tomography revealed a hepatic aneurysm and intra-abdominal bleeding, and the patient was transferred to our hospital. Emergency abdominal angiography revealed multiple aneurysms in the celiac, common, and right hepatic arteries. The right hepatic artery was considered responsible and was embolized. The patient had characteristic physical findings of the syndrome, aiding in confirming the genetic analysis of COL3A1 gene abnormality.
UNASSIGNED: Juvenile-onset colonic perforation and rupture of the celiac arteries are key findings in the suspicion of vascular-type Ehlers-Danlos syndrome.