儿童Abernethy畸形及并发症与合并畸形的超声诊断

Diagnosis of Abernethy malformation with the complications and associated malformations in children by ultrasound

目的提高超声对儿童Abernethy畸形的诊断准确率,并对其并发症、合并畸形进行更全面的超声评估。方法回顾性收集2020年3月至2023年12月于浙江大学医学院附属儿童医院行超声检查并经CT血管造影(CTA)和(或)经皮穿刺血管造影证实的Abernethy畸形患儿7例,同时进行心脏超声检查,并对低氧血症患儿行右心声学造影检查。总结临床表现,并对Abernethy畸形及其并发症、合并畸形的超声特征及检查思路进行归纳。结果7例患儿中,3例因体检、发热、外伤等偶然因素发现,4例有临床症状。7例患儿均由超声首先发现并诊断Abernethy畸形,3例已行手术治疗者超声分型准确。并发症:2例患儿并发肝脏局灶性结节增生;1例并发消化道出血和肾病综合征;1例并发肺动脉高压;2例有缺氧表现者右心声学造影显示右向左分流分别为Ⅱ级和Ⅲ级,诊断为并发肝肺综合征。合并畸形:4例合并先天性心脏病,其中1例为多脾综合征。治疗:1例行血管造影监视下分流血管封堵术,2例行肝脏移植,3例随访中,1例放弃治疗。结论超声对儿童Abernethy畸形有较高的诊断准确率并可定位门-体静脉分流血管及部位。除腹部脏器及血管超声外,对Abernethy畸形患儿建议常规行超声心动图检查,有缺氧表现患儿行右心声学造影可对肝肺综合征的诊断提供重要信息。

Objective To improve the accuracy of ultrasound for the diagnosis of Abernethy malformation in children,and to achieve a comprehensive ultrasound evaluation of its complications and associated malformations.MethodsThe data of 7 children who underwent ultrasound examination and confirmed with Abernethy malformation by computed tomography angiography(CTA)and/or percutaneous angiography in the Children′s Hospital of Zhejiang University School of Medicine from March 2020 to December 2023 were retrospectively collected.All the children received routine cardiac ultrasound examination,and those with hypoxemia also received right heart contrast echocardiography.The clinical manifestations were analyzed,and the ultrasound characteristics of Abernethy malformation,its complications,associated malformations and examination method were summarized.ResultsAbernethy malformation was detected by accident as physical examination,fever,and trauma in 3 of the 7 children.The other 4 cases presented with clinical symptoms.Abernethy malformation in all 7 children was first detected and diagnosed by ultrasound.In 3 cases,the surgical results confirmed that the type of Abernethy malformation determined by ultrasound was correct.The complications of Abernethy malformation included focal nodular hyperplasia in 2 cases,gastrointestinal bleeding and nephrotic syndrome in 1 case,and pulmonary hypertension in 1 case.The right heart contrast echocardiography results of 2 cases with hypoxia symptoms suggested that their right-to-left shunts were gradedⅡandⅢ,respectively.Therefore,2 cases were complicated by hepatopulmonary syndrome.Among these 7 cases,4 cases had congenital heart disease,one of whom had polysplenia syndrome.Shunt vascular occlusion under digital subtraction angiography monitoring was performed in 1 case.Two cases underwent liver transplantation.Three cases were being followed up.One case abandoned treatment.ConclusionsUltrasound has high accuracy in the diagnosis of Abernethy malformation in children and can locate portosystemic shunt vessels.In addition to abdominal organ and vascular ultrasound examination,routine echocardiography is recommended for children with Abernethy malformation.Right heart contrast echocardiography provides important information for the diagnosis of hepatopulmonary syndrome in children with hypoxia symptoms.