摘要本文报道1例反复漏诊的先天性左肾动静脉瘘(RAVF)伴左肾多支动脉合并椎骨侧弯畸形病例。患者因反复血尿1年于2020年8月29日入院。外院行两次输尿管软镜检查、入院后的影像学和实验室检查均未明确血尿病因。通过仔细阅读增强CT检查图片发现肾窦下极血管结构紊乱，血管迂曲成团，肾盂周围也可见扩张迂曲血管，考虑血尿原因为肾脏血管畸形引起。为查明病因，行肾动脉数字减影血管造影(DSA)检查，结果提示先天性左肾RAVF伴左肾3支供养动脉，行动静脉瘘供养动脉栓塞治疗。对于常规影像学和实验室检查排除肿瘤、结石、结核、凝血功能异常等病因的大量肉眼血尿患者，应考虑先天性肾脏血管畸形可能，需行DSA检查。血管内栓塞是治疗先天性RAVF的有效方法。

abstractsA recurrent misdiagnosed case of congenital left renal arteriovenous fistula (RAVF) with multiple left renal arteries and scoliosis was reported. The patient was admitted to hospital on 29 August 2020 due to repeated hematuria for one year. No abnormality was found in two flexible ureteroscope examinations, imaging and laboratory examinations after admission. It was found that the structure of blood vessels in the inferior pole of renal sinus was disordered, and the blood vessels were tortuous and clustered through careful reading of CT enhancement films. The dilated tortuous blood vessels were also seen around the renal pelvis, and hematuria was considered to be caused by renal vascular malformation. In order to confirm the etiology, digital subtraction angiography (DSA) of renal artery was performed. DSA showed a congenital left renal RAVF with three renal arteries, and the arteriovenous fistula of renal arteries was embolized. For patients presenting with severe gross hematuria, if tumor, stone, tuberculosis, or coagulation abnormalities were excluded by conventional imaging and/or laboratory examination the possibility of congenital renal vascular malformation should be suspected, and DSA examination should be performed. Endovascular embolization is an effective treatment for congenital RAVF.