Park Yang Jin, Park Keun Myoung, Oh Jiyoung, Park Hong Suk, Kim Jung-Sun, Kim Young-Wook
Division of Vascular Surgery, Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
J Korean Surg Soc. 2012 Apr;82(4):261-5. doi: 10.4174/jkss.2012.82.4.261. Epub 2012 Mar 27.
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.
I型神经纤维瘤病（NF-1）是一种罕见的常染色体显性遗传病，发病率为三千分之一。血管病变在NF-1患者中鲜有报道。在此，我们报告一例49岁患有NF-1的男性患者，在接受血管内动脉瘤修复术后发生复发性主动脉假性动脉瘤的病例。在成功进行主动脉假性动脉瘤开放手术修复后的第六天，他因肠系膜动脉分支延迟破裂而出现血腹。对此采用血管内弹簧圈栓塞治疗。我们报告了这种罕见血管疾病的临床特征和组织学发现，并对相关文献进行了综述。
