目的 探讨数字减影血管造影（Digital Subtraction Angiography，DSA）在儿童先天性肝外门 体静脉分流的临床分型及治疗中的重要价值。方法 回顾性分析15例先天性肝外门体静脉分流患儿 的DSA结果并与临床资料进行对比。结果 15例患儿经过DSA检查得到明确诊断及临床分型，Ⅰ型 2例，1例Ⅰ型患儿行肝移植，1例随访中；Ⅱ型13例，Ⅱ型中除1例随访外，其余12例均得到有效 治疗，其中4例在DSA下对异常侧枝血管进行plug封堵器封堵，8例在确诊后因无法封堵而进行及 时有效的外科门体静脉断流术。治疗后患儿临床症状明显得到改善。结论 DSA是确诊先天性门-体 静脉分流类型一种敏感而准确的方法，尤其能检测到易被常规CT/MRI漏诊的肝内细小的门静脉分 支而纠正分型，从而争取及时有效的治疗机会。

Objective This paper aimed to explore the value of digital subtraction angiography (DSA) in the clinical classification and treatment of children's congenital extrahepatic portosystemic shunts (CEPS). Methods The DSA results of 15 cases of congenital hepatic portal vein were analyzed retrospectively as well as being compared with the clinical data. Results Fifty cases were diagnosed and classified by DSA, which contained 2 cases of typeⅠand 13 cases of typeⅡ. For the 13 cases of type Ⅱ, 12 cases were treated effectively except 1 case was followed-up. 4 cases with abnormal sido veins were plugged under DSA, the other 8 cases could not undergone the ligating the portosystemic shunts surgery effectively because they could not be plugged well. The clinical symptoms of the children were significantly improved after treatment. Conclusion DSA is a sensitive and accurate way to make a definite diagnosis for CEPS, especially in modifying the classification of patients which are always misdiagnosed by routine CT/MRI due to not detecting tiny branch of intrahepatic portal vein, and provides timely and effective treatment opportunities for these patients.