选择性肾动脉栓塞治疗结节性硬化症相关肾AML七例分析

Clinical Analysis of Selective Arterial Embolization in Treatment of Renal Angiomyolipoma 7 Patients with Tuberous Sclerosis Complex

目的探讨选择性肾动脉栓塞治疗结节性硬化症(tuberous sclerosis complex,TSC)相关肾血管平滑肌脂肪瘤(angiomyolipoma,AML)的有效性和安全性。方法选取2010年1月—2016年1月我院7例行选择性肾动脉栓塞治疗的TSC相关肾AML患者,治疗前及治疗后1周、3个月、6个月、1年复查血肌酐及腹部CT或磁共振尿路造影(MRU)评估患者肾功能、肾脏病灶的变化和手术疗效及安全性。结果本组均为双侧、多发病变,平均瘤径8.5 cm。均选择股动脉入路行肾动脉栓塞,动脉造影明确肿瘤血供或破裂出血点后酌情选择栓塞剂,对有较大动静脉瘘或需栓塞较粗动脉者,加用合适的弹簧钢圈或聚乙烯醇微球。术后平均随访32个月,除1例并肺淋巴管肌瘤病致大咯血死亡外,6例存活;1例半年后再出血行2次栓塞治疗;3例术后应用西罗莫司或依维莫司治疗,肿瘤最大径显著缩小;7例术后及随访期血肌酐水平与术前比较均无明显升高(P>0.05),腹部CT或MRU检查示栓塞侧肾皮质无明显萎缩。7例围术期未发生严重并发症,4例术后3 d内出现栓塞后综合征,予对症治疗2周,症状消失。结论选择性肾动脉栓塞是治疗破裂出血或有高出血风险TSC相关肾AML的一种安全、有效方法,对患者肾功能影响较小。

Objective To investigate safety and efficacy of selective arterial embolization in treatment of renal angiomyolipoma （AML） in patients with tuberous sclerosis complex （TSC）. Methods Changes of renal function and renal lesions, surgical effect and safety were analyzed for 7 renal AML patients with TSC during January 2010 and January 2016 undergoing selective arterial embolization by serum ereatinine testing, abdominal CT or magnetic resonance urography （MRU） before and 1 week, 3 months, 6 and 12 months after embolization. Results All patients had bilateral multiple renal angiomyolipomas, and the mean tumor diameter was 8.5 cm. Selective arterial embolization was performed by femoral artery approach, and emboliaztion agents were given selectively after confirming blood supply of tumor or breaking bleeding point by arteriography, and patients with large arteriovenous fistula or need of big emboliaztion agents were treated with suitable spring steel cycle or Polyvinyl Alcohol microballoons. During average 32 months of follow-up, one patient died of serious hemoptysis induced by combination of pulmonary lymphangiomyomatosis, the other 6 patients were survival; 1 patient received second selective arterial embolization because of bleeding 6 months after the first embolization ; 3 patients were treated with Sirolimus or Everofimus after embolization, and the largest tumor diameter was significantly reduced. During postoperative follow-up, no significant in- creasing was found in serum creatinine levels of 7 patients before and after surgery （ P 〉 0.05） , and no obvious atrophy was found in renal substantia corticalis near the embolism by abdominal CT or MRU. No serious complications were found during operation, and 4 patients had post embolization syndrome within postoperative 3 d, and symptoms were disappeared within 2 weeks after symptomatic treatment. Conclusion Selective arterial embolization is a safe and effective method in treatment of TSC-AML with bleeding or high risk of bleeding, and its effect on renal function is little.