先天性肾动静脉畸形自然消退的血管造影及临床分析

Angiography and clinical analysis of spontaneous regression of congenital renal arteriovenous malformation

目的探讨先天性肾动静脉畸形(rAVM)自然消退的血管造影(DSA)表现及其原因。方法详细报道1例先天性rAVM的患者,由于持续性血尿于第一次DSA检查后7d行第二次DSA检查,病灶消失,出现自然消退,并结合文献报道的病例(3例)对血尿时间、分型等临床资料及DSA表现进行总结分析。结果本病例第一次肾动脉DSA显示明显的畸形血管和动静脉瘘的rAVM征象,第二次肾动脉DSA显示上述征象消失,超选择DSA时显示一条出血小血管并顺利行肾动脉栓塞术,随访30个月无复发血尿和相关并发症,CT检查未见异常。包括文献报道共4例先天性rAVM出现自然消退,均以DSA明确诊断,其共同临床特点包括静脉曲张型、大量血尿、间歇性血尿、DSA后血尿消失或改善。结论 DSA是诊断rAVM的最敏感及了解rAVM动态变化最直接的方法,大多数先天性rAVM仍需介入或外科治疗,其自然消退的具体原因尚未完全明确,需进一步深入研究。

Objective To investigate DSA manifestations and possible causes of spontaneous regression of congenital renal arteriovenous malformation (rAVM).Methods One patient with congenital rAVM was reported in detail that there was no DSA manifestation in the second angiography,even though there were typical DSA features of AVM in the first procedure 7 days ago.DSA manifestations and clinical data of spontaneous regression of rAVM were collected and discussed with the related literature.Results The first renal DSA of this case demonstrated obviously abnormal small vessels and arteriovenous fistula.The second renal DSA did not find any evidence of rAVM.Superselective renal DSA showed a bleeding small vessel and transcatheter arterial embolization was performed successfully in it.During the follow-up of 30 months,no hematuria recurred and no complications were found.And there were no CT findings of rAVM.There were 4 cases of spontaneous regression of rAVM in all including precious reported 3 cases.Their diagnosis and spontaneous regression were confirmed by DSA.And their common clinical features including cirsoid type,massive hematuria,intermittent hematuria,resolved or improved hematuria after renal DSA and so on.Conclusions Renal DSA remains the most sensitive modality to diagnose the rAVM and the most straightforward method of its dynamic development.Most symptomatic congenital rAVMs still require interventional or surgical therapy.The natural history of spontaneous regression of rAVM remains unknown and needs further studies.