目的 先天性门-体静脉分流(CPSS)是一种罕见的畸形.影像学检查是诊断CPSS的重要手段.本文旨在探讨彩色多普勒超声在儿童先天性门-体静脉分流的表现及诊断价值.方法 选取我院收治CPSS患者14例,男性7例,女性7例,均行超声及DSA检查.回顾性分析本组患者的临床资料及超声影像资料,根据Bicetre外科学分型,以DSA作为金标准,将超声与DSA检查结果进行比较,评估超声诊断的准确性.结果 本组病例中EHPS型1例、PC型9例(ESPC 1例、SSPC 7例、HPC 1例)、PH型3例、PDV 1例;误诊1例为SSPC型;漏诊2例(1例为EHPS型,1例为PH型).与DSA检查结果比较,超声准确率为78.6％,其中EHPS、PC、PH和PDV的准确率分别为0.0％、88.9％、66.7％和100.0％,PC型的3个亚型ESPC、SSPC和HPC的准确率分别为100.0％、85.7％和100.0％.对于评估肝内门静脉分支发育情况,超声与DSA比较的准确率为85.7％.结论 彩色多普勒超声在先天性门-体静脉分流的诊断和分类中具有较高的准确性,并且能直观地显示分流的位置和初步评估肝内门静脉分支的发育情况,具有重要的影像学价值.

Background ：Abernethy malformation is a rare congenital abnormality. Imaging examination is an important method for the diagnosis. The purpose of this study was to demonstrate manifestations of multi-slice computed tomography (MSCT) in Abernethy malformation and its diagnostic accuracy. Methods ：Fourteen children with Abernethy malformation were admitted to our center in China between July 2011 and January 2018. All 14 patients (eight males and six females) received MSCT and digital subtraction angiography (DSA) while eight patients also received ultrasound. The patients’ age ranged from 1 to 14 (median age 8 years old). The clinical records of the patients were retrospectively reviewed. MSCT raw data were transferred to an Advantage Windows 4.2 or 4.6 workstation (General Electric Medical Systems, Waukesha, WI). We compared the findings of MSCT with DSA and surgical results in order to ascertain diagnostic accuracy. Results ：Three cases had type Ib Abernethy malformation and eleven cases had type II. Two cases of type II Abernethy malformation were misdiagnosed as type Ib using MSCT. Comparing the findings of MSCT with DSA and surgical results, the accuracy of MSCT was 85.7% (12/14), in which 100.0% (3/3) were type Ib and 81.8% (9/11) were type II. Clinical information included congenital heart disease, pulmonary hypertension, diffuse pulmonary arteriovenous fistula, abnormal liver function, hepatic nodules, elevated blood ammonia, and hepatic encephalopathy. Eleven cases were treated after diagnosis. One patient with Abernethy malformation type Ib(1/3) underwent liver transplantation. Seven patients with Abernethy malformation type II (7/11) were treated by shunt occlusion, received laparoscopy, or were treated with open surgical ligation. Another three patients (3/11) with Abernethy malformation type II were treated by interventional portocaval shunt occlusion under DSA. Conclusion ：MSCT attains excellent capability in diagnosing type II Abernethy malformation and further shows the location of the portacaval shunt. DSA can help when it is hard to determine diagnosis between Abernethy type Ib and II using MSCT. Key words: Abernethy malformation; Angiography; Tomography