Zhang Bihui, Zou Yinghua, Yang Min, Niu Guochen
Department of Interventional Radiology and Vascular Surgery, Peking University First Hospital, Peking University, Beijing, China.
Medicine (Baltimore). 2017 Nov;96(47):e8858. doi: 10.1097/MD.0000000000008858.
Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder characterized by cafe au lait macules and neurofibromatosis. Renal artery aneurysms are relatively uncommon. Endovascular techniques are effective in treating renal aneurysms but successful cases are rarely reported in NF-1 adults.
The patient was one 23-year-old female presented with hypertension, multiple café-au-lait spots ≥15 mm, and plexiform neurofibroma. Renal artery aneurysms were found by ultrasound.
NF-1 was diagnosed based on clinical manifestations and confirmed by gene test. Renal artery aneurysms were diagnosed based on computed tomography.
Bilateral renal artery angiography was performed and 3 aneurysms were found sequentially on the left anterior superior segmental artery. Microcoil embolization of aneurysms was undertaken.
The patient's blood pressure decreased after the procedure with reduction of medicine. A 3-month follow-up unilateral selective renal angiogram demonstrated little change in size of aneurysms, and no opacification of the aneurysmal sac was found. Serum creatinine remained in normal range at 3-month.
Successful endovascular treatment for NF-1 related renal artery aneurysms in adults is reported for the 1st time with preserved renal function and improved hypertension. Endovascular procedure is considered to be feasible and effective for renal artery aneurysms induced by NF-1.
1型神经纤维瘤病（NF-1）是一种常染色体显性疾病，其特征为牛奶咖啡斑和神经纤维瘤病。肾动脉动脉瘤相对少见。血管内技术在治疗肾动脉瘤方面有效，但在NF-1成年患者中成功病例鲜有报道。
该患者为一名23岁女性，表现为高血压、多个直径≥15毫米的牛奶咖啡斑以及丛状神经纤维瘤。超声检查发现肾动脉动脉瘤。
根据临床表现诊断为NF-1，并经基因检测确诊。根据计算机断层扫描诊断为肾动脉动脉瘤。
进行双侧肾动脉血管造影，在左前上段动脉依次发现3个动脉瘤。对动脉瘤进行微线圈栓塞。
术后患者血压下降，药物用量减少。3个月的随访单侧选择性肾血管造影显示动脉瘤大小变化不大，未发现动脉瘤囊显影。3个月时血清肌酐仍在正常范围内。
首次报道了对成年NF-1相关肾动脉动脉瘤进行成功的血管内治疗，肾功能得以保留，高血压情况有所改善。血管内手术被认为对NF-1所致肾动脉动脉瘤可行且有效。
