先天性肾动静脉瘘伴肾动脉多支畸形病例分析  

Congenital renal arteriovenous fistula complicated with multiple renal arteries malformation: case analysis

作　　者：于路平[1] 赵卫红 刘士军[1] 李清[1] 徐涛[1] YU Lu-ping1, ZHAO Wei-hong2 ,LIU Shi-jun1 ,LI Qing1, XU Tao1(1. Department of Urology, Peking University People＇ s Hospital, Beijing 100044, China;2. Department of Urology, Weinan City Center Hospital, Weinan 714000, Shaanxi, Chin)

机构地区：[1]北京大学人民医院泌尿外科,北京100044 [2]渭南市中心医院,陕西渭南714000

出　　处：《北京大学学报（医学版）》2018年第4期722-728,共7页Journal of Peking University:Health Sciences

摘　　要：肾多支动脉并发先天性肾动静脉瘘是一种罕见疾病,其出血速度快、出血量大,早期诊断困难,栓塞后并发症严重。疾病可通过B超、增强CT或肾动脉数字减影血管造影(digital subtraction angiography,DSA)确诊,膀胱镜、输尿管镜检查可明确出血位置、除外肿瘤、清除凝血块造成的输尿管梗阻。本文报告1例肾多支动脉并发先天性肾动静脉瘘患者,以探讨先天性肾动静脉瘘伴肾动脉多支畸形的发病情况、临床特点、诊断及治疗。患者为36岁女性,入院前5 d无诱因出现无痛性全程肉眼血尿,伴血块,血压90/70 mm Hg,血红蛋白60 g/L。泌尿系统CT提示右侧输尿管下段占位性病变可能性大,近端输尿管及肾积水;膀胱高密度影,考虑出血可能性大。入院后膀胱镜检查见膀胱内大量陈旧性凝血块,右侧输尿管口喷血,输尿管镜检查见右侧输尿管及肾盂内陈旧性凝血块,未见肿瘤,输尿管黏膜未见出血点,肾盂可见喷血,清除输尿管内凝血块并留置输尿管支架管。术后考虑右侧肾血管畸形所致出血可能,急诊行肾的动脉数字减影血管造影,显示右侧肾动脉双支畸形,右肾中部见团状迂曲杂乱血管,选择性插管提示两支肾动脉二级和三级分支均存在破裂出血,考虑"肾动静脉瘘"。用微导管分别超选择造影置管至右肾病变供血动脉,以微弹簧栓子进行栓塞,栓塞后造影,示原迂曲杂乱血管消失,肾静脉提前显影征象消失。患者术后4 d血尿缓解,术后5 d贫血改善,并恢复良好出院,随访4个月未出现血尿症状,复查B超右肾积水消失,肾动态显像提示右肾功能正常。因此,肾动脉DSA是诊断肾多支动脉并发先天性肾动静脉瘘的金标准,DSA时有必要先行腹主动脉造影明确肾动脉数目,避免漏诊,肾动脉栓塞治疗肾动静脉瘘安全、有效。Congenital renal arteriovenous fistula complicated with multiple renal arteries malformation is rare and hard to diagnose at early stage. Blood loss and complications after embolization are both severe. Some cases can be diagnosed by ultrasound,enhanced CT scan or digital subtraction angiography（DSA）. Cystoscopy and ureteroscopy can identify the location of bleeding,exclude tumors,and discharge ureteral obstruction. A case of congenital renal arteriovenous fistula complicated with multiple renal arteries malformation was reported to investigate the pathogenesis,clinical characteristics,diagnosis and treatment of congenital renal arteriovenous fistula with multiple renal arteries malformation. A 36-year-old female patient with congenital renal arteriovenous fistula with multiple renal arteries malformation was hospitalized in the Department of Urology of Peking University People＇s Hospital. Five days before admission,the patient experienced whole course painless gross hematuria for 5 days with many blood clots. The patient＇s blood pressure was 90/70 mm Hg,and hemoglobin was 60 g/L. The urinary CT scan showed a right hydronephrosis associated with dilatation of the upper ureter which was obstructed by space occupying lesion of the lower ureter. Many clots in the bladder could also be found in the CT scan. Cystoscopy showed many blood clots in the bladder and confirmed that the bleeding was fromthe right ureteral orifice. Ureteroscopy confirmed that the bleeding was from the right renal pelvis and many blood clots in the right ureter,and found no tumor in the right ureter and renal pelvis. We cleared the blood clots in the right ureter and inserted a ureteral stent. We thought that renal vascularmalformation of the right kidney might lead to the hematuria from right renal pelvis. DSA showed a double renal arteries malformation in the right kidney. The diagnosis of ＂renal arteriovenous fistula＂was considered with renal arteriovenous fistula in the right kidney. Selective arteriography revealed the presenc

关 键 词：肾动脉多支畸形 肾动静脉瘘 血尿 动脉数字减影血管造影 肾动脉栓塞 

分 类 号：R692.1[医药卫生—泌尿科学]