1型神经纤维瘤病患者的自发性主动脉破裂。

Journal of the Korean Surgical Society Pub Date : 2012-04-01 Epub Date: 2012-03-27 DOI:10.4174/jkss.2012.82.4.261
Yang Jin Park, Keun Myoung Park, Jiyoung Oh, Hong Suk Park, Jung-Sun Kim, Young-Wook Kim
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引用次数: 14

摘要

神经纤维瘤病I型(NF-1)是一种罕见的常染色体显性遗传疾病,发病率为1 / 3000。血管病变在NF-1患者中很少报道。在这里,我们报告一例49岁的NF-1男性患者在血管内动脉瘤修复后复发的主动脉假性动脉瘤。术后第六天,手术成功修复了主动脉假性动脉瘤,由于肠系膜动脉分支延迟破裂,患者出现腹膜出血。采用血管内线圈栓塞治疗。我们报告这种罕见的血管疾病的临床特征和组织学表现,并回顾相关文献。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Spontaneous aortic rupture in a patient with neurofibromatosis type 1.

Spontaneous aortic rupture in a patient with neurofibromatosis type 1.

Spontaneous aortic rupture in a patient with neurofibromatosis type 1.

Spontaneous aortic rupture in a patient with neurofibromatosis type 1.

Neurofibromatosis type I (NF-1) is a rare autosomal dominant genetic disorder occurring in 1 in 3,000 individuals. Vasculopathy is a rarely reported finding in patients with NF-1. Here, we report a case of recurrent aortic pseudoaneurysm after endovascular aneurysm repair in a 49-year-old male patient with NF-1. On the sixth postoperative day following a successful open surgical repair of an aortic pseudoaneurysm, he developed hemoperitoneum due to a delayed rupture of the mesenteric artery branch. This was treated with endovascular coil embolization. We report the clinical features and histologic findings of this rare vascular disorder with a review of the relevant literature.

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