Department of Radiology, The Raymond and Ruth Perelman School of Medicine of the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Am J Med Genet A. 2011 Dec;155A(12):3090-4. doi: 10.1002/ajmg.a.34332. Epub 2011 Nov 7.
Life expectancy in vascular Ehlers-Danlos syndrome (EDS) is shortened due to spontaneous rupture of arteries, the colon and the gravid uterus. Two adolescent males with vascular EDS illustrate rapid progression of arterial aneurysms, dissections, and rupture. Radiologic imaging played an important role in initially diagnosing and monitoring the evolution of arterial involvement. Both prophylactic and emergency management remain largely ineffective in this connective tissue disorder; however, noninvasive imaging may provide important prognostic information.
血管型埃勒斯-当洛斯综合征（EDS）患者的预期寿命会因动脉、结肠和妊娠子宫的自发性破裂而缩短。本文报告了两名血管型 EDS 青少年男性，他们的动脉瘤、夹层和破裂迅速进展。放射影像学在最初诊断和监测动脉受累的演变方面发挥了重要作用。在这种结缔组织疾病中，预防性和紧急性的治疗管理大多效果不佳；然而，非侵入性成像可能提供重要的预后信息。
