PDF(Pubmed)
Abstract：
We report a case of a 63-year-old woman diagnosed with vascular Ehlers-Danlos syndrome (vEDS) who survived two prophylactic surgeries for the dilatation of a thoracoabdominal aortic aneurysm. She initially developed acute type B aortic dissection at the age of 44 years. Five years later, her dissected descending aorta was enlarged to 54 mm; thus, the descending aorta was replaced as the first surgery. Fortunately, the intra- and post-operative courses were uneventful. Fourteen years post her first surgery, the dissected thoracoabdominal aorta distal to the graft expanded to 53 mm; however, no anastomotic leakage was observed. Genetic testing revealed a COL3A1 abnormality, confirming the diagnosis of vEDS. Thoracoabdominal aorta replacement using deep hypothermia circulatory arrest was performed because of the high risk of aortic aneurysm rupture. The second surgery was performed without complications, and no complications were observed 13 months post-surgery. The major reason for a successful surgery in this patient was the relatively low vascular fragility associated with vEDS. This case demonstrates that there may be considerable individual differences in vascular fragility in patients with vEDS. Thus, surgical repair, along with endovascular therapy, might still be a beneficial option for patients with vEDS having large aortic aneurysms and a high risk of rupture.
UNASSIGNED: Prophylactic surgery for vascular lesions in Ehlers-Danlos syndrome (vEDS) is generally not recommended because of its high vascular fragility. However, if a patient with vEDS has an aortic aneurysm that is at a very high risk of rupture, aggressive treatment is a plausible option as there may be considerable individual differences in vascular fragility among patients with vEDS.
UNASSIGNED: Prophylactic surgery for vascular lesions in Ehlers-Danlos syndrome (vEDS) is generally not recommended because of its high vascular fragility. However, if a patient with vEDS has an aortic aneurysm that is at a very high risk of rupture, aggressive treatment is a plausible option as there may be considerable individual differences in vascular fragility among patients with vEDS.
摘要：
我们报告了一例63岁的女性，被诊断患有血管Ehlers-Danlos综合征（vEDS），她在两次预防性手术中幸存下来，以扩张胸腹主动脉瘤。她最初在44岁时发展为急性B型主动脉夹层。五年后,她解剖的降主动脉扩大到54毫米；因此，作为第一次手术，降主动脉被替换。幸运的是,术中和术后的课程都很顺利.第一次手术14年后，在移植物远端解剖的胸腹主动脉扩张至53毫米；然而，未观察到吻合口漏。基因检测显示COL3A1异常,确认vEDS的诊断。由于主动脉瘤破裂的风险很高，因此使用深低温停循环进行了胸腹主动脉置换。第二次手术没有并发症,术后13个月无并发症发生。该患者手术成功的主要原因是与vEDS相关的血管脆性相对较低。此病例表明vEDS患者的血管脆性可能存在相当大的个体差异。因此，手术修复,随着血管内治疗，对于有大型主动脉瘤和高破裂风险的vEDS患者，可能仍然是一个有益的选择。
■通常不建议对Ehlers-Danlos综合征（vEDS）的血管病变进行预防性手术，因为它具有很高的血管脆性。然而,如果vEDS患者的主动脉瘤破裂风险很高，积极治疗是一个合理的选择,因为vEDS患者的血管脆性可能存在相当大的个体差异.
■通常不建议对Ehlers-Danlos综合征（vEDS）的血管病变进行预防性手术，因为它具有很高的血管脆性。然而,如果vEDS患者的主动脉瘤破裂风险很高，积极治疗是一个合理的选择,因为vEDS患者的血管脆性可能存在相当大的个体差异.
