Narayan Pradeep, Alwair Hazaim, Bryan Alan J
Department of Cardio-thoracic Surgery, Bristol Royal Infirmary, Bristol Heart Institute, Bristol BS2 8HW, UK.
Interact Cardiovasc Thorac Surg. 2004 Jun;3(2):346-8. doi: 10.1016/j.icvts.2004.02.001.
Wiskott-Aldrich syndrome is a rare X-linked disorder. Vasculitis and aneurysm formation are extremely rare but potentially fatal complications of the disease. Aortitis and aneurysm formation seem to be progressive in patients with Wiskott-Aldrich syndrome. The risk of death from aneurysmal rupture in patients with Wiskott-Aldrich syndrome is high and hence surgery is required for resection of aneurysms. We report a case where a successful resection of a descending thoracic aneurysm was carried out in a patient who previously had undergone a composite root replacement for aortic root dilatation.
威斯科特-奥尔德里奇综合征是一种罕见的X连锁疾病。血管炎和动脉瘤形成是该疾病极为罕见但可能致命的并发症。在威斯科特-奥尔德里奇综合征患者中，主动脉炎和动脉瘤形成似乎呈进行性发展。威斯科特-奥尔德里奇综合征患者因动脉瘤破裂而死亡的风险很高，因此需要进行手术切除动脉瘤。我们报告了一例病例，该患者此前因主动脉根部扩张接受了复合根部置换手术，此次成功切除了降胸段动脉瘤。
