Diagnosis of Abernethy malformation with the complications and associated malformations in children by ultrasound
10.3760/cma.j.cn131148-20240409-00220
- VernacularTitle:儿童Abernethy畸形及并发症与合并畸形的超声诊断
- Author:
Bin XU
1
;
Zhisu LYU
;
Jin YU
;
Jingjing YE
Author Information
1. 浙江大学医学院附属儿童医院超声科 国家儿童健康与疾病临床医学研究中心，杭州 310000
- Keywords:
Ultrasonography;
Abernethy malformation;
Portosystemic Shunts;
Children;
Hepatopulmonary syndrome
- From:
Chinese Journal of Ultrasonography
2024;33(10):891-898
- CountryChina
- Language:Chinese
-
Abstract:
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.Methods:The 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.Results:Abernethy 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 graded Ⅱ and Ⅲ, 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.Conclusions:Ultrasound 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.
