Yu L P, Zhao W H, Liu S J, Li Q, Xu T
Department of Urology, Peking University People's Hospital, Beijing 100044, China.
Department of Urology, Weinan City Center Hospital, Weinan 714000, Shaanxi, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2018 Aug 18;50(4):722-728.
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 mmHg, 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 vascular malformation 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 presence of tortuous, coiled, dilated, and multichannelled vessels in the middle of the right kidney. With stainless steel coils, we embolized the vessels which supplied the fistula. Four days after the procedure, gross hematuria disappeared. Five days after the procedure, the patient's anemia improvedand the patient was discharged in good condition. Four months after the procedure, gross hematuria did not recur. The Doppler showed that the right kidney was normal and the renal dynamic showed that the right kidney function was normal. So DSA is the golden standard for diagnosis of congenital renal arteriovenous fistula complicated with multiple renal arteries malformation. Confirming the number of renal arteries by abdominal aorta angiography is necessary to avoid missed diagnosis. Renal arterial embolization is safe and effective.
先天性肾动静脉瘘合并多条肾动脉畸形较为罕见,早期难以诊断。栓塞术后失血和并发症都很严重。部分病例可通过超声、增强CT扫描或数字减影血管造影(DSA)进行诊断。膀胱镜检查和输尿管镜检查可明确出血部位,排除肿瘤,并解除输尿管梗阻。本文报道1例先天性肾动静脉瘘合并多条肾动脉畸形的病例,以探讨先天性肾动静脉瘘合并多条肾动脉畸形的发病机制、临床特点、诊断及治疗方法。1例36岁先天性肾动静脉瘘合并多条肾动脉畸形的女性患者入住北京大学人民医院泌尿外科。入院前5天,患者全程无痛肉眼血尿5天,伴有大量血凝块。患者血压为90/70 mmHg,血红蛋白为60 g/L。泌尿系统CT扫描显示右肾积水伴上段输尿管扩张,下段输尿管占位性病变导致梗阻。CT扫描还发现膀胱内有许多血凝块。膀胱镜检查显示膀胱内有许多血凝块,证实出血来自右输尿管口。输尿管镜检查证实出血来自右肾盂,右输尿管内有许多血凝块,且在右输尿管和肾盂未发现肿瘤。我们清除了右输尿管内的血凝块并置入输尿管支架。我们认为右肾血管畸形可能导致右肾盂血尿。DSA显示右肾双肾动脉畸形。考虑诊断为右肾肾动静脉瘘。选择性动脉造影显示右肾中部存在迂曲、盘绕、扩张和多通道的血管。我们用不锈钢圈栓塞了供应瘘管的血管。术后4天,肉眼血尿消失。术后5天,患者贫血改善,康复出院。术后4个月,肉眼血尿未复发。多普勒检查显示右肾正常,肾动态检查显示右肾功能正常。因此,DSA是诊断先天性肾动静脉瘘合并多条肾动脉畸形的金标准。通过腹主动脉造影确定肾动脉数量对于避免漏诊很有必要。肾动脉栓塞术安全有效。