一名患有1型神经纤维瘤病的14岁男孩因多发肝动脉瘤继发梗阻性黄疸。

Obstructive jaundice secondary to multiple hepatic artery aneurysms in a 14-year-old boy with neurofibromatosis type 1.

作者信息

Morris Marvin E, Jones Riley G, Walker Sarah K, Yancey Andrea E, Dwivedi Amit J, Ross Charles B

机构信息

Department of Surgery, University of Louisville, Louisville, Kentucky, USA.

出版信息

Ann Vasc Surg. 2013 Jul;27(5):673.e1-4. doi: 10.1016/j.avsg.2012.09.010. Epub 2013 Apr 18.

DOI:10.1016/j.avsg.2012.09.010

PMID:23602432

Abstract

Neurofibromatosis type 1 is the most common inherited disorder of the nervous system, affecting approximately 1 in 3,000 people. A small but significant subset of these patients develop vasculopathies. We present the first reported case of neurofibromatosis type 1 presenting with obstructive jaundice secondary to multiple hepatic artery aneurysms. Therapy included staged coil embolization of the hepatic artery aneurysms and resection of a large retroperitoneal neurofibroma.

摘要

1型神经纤维瘤病是最常见的遗传性神经系统疾病，每3000人中约有1人受影响。这些患者中有一小部分但数量可观的人会发生血管病变。我们报告了首例因多发性肝动脉瘤继发梗阻性黄疸而出现的1型神经纤维瘤病病例。治疗方法包括对肝动脉瘤进行分期弹簧圈栓塞以及切除一个巨大的腹膜后神经纤维瘤。