介入封堵治疗小儿Ⅱ型Abernethy畸形合并肺动脉高压1例报告

张恒; 江斌; 方志成; 孟忠吉; 杜恩辅

doi:10.3969/j.issn.1001-5256.2023.11.022

介入封堵治疗小儿Ⅱ型Abernethy畸形合并肺动脉高压1例报告

DOI: 10.3969/j.issn.1001-5256.2023.11.022

张恒^a, , ,,
江斌^b,
方志成^a,
孟忠吉^c,
杜恩辅^d

a.
湖北医药学院附属太和医院，湖北省肝癌精准诊疗临床医学研究中心急诊医学科，湖北十堰 442000
b.
湖北医药学院附属太和医院，湖北省肝癌精准诊疗临床医学研究中心肝胆胰腺外科，湖北十堰 442000
c.
湖北医药学院附属太和医院，湖北省肝癌精准诊疗临床医学研究中心感染科，湖北十堰 442000
d.
湖北医药学院附属太和医院，湖北省肝癌精准诊疗临床医学研究中心介入放射科，湖北十堰 442000

基金项目:

湖北陈孝平科技发展基金会基金项目 (CXPJJH11800001-2018246)

伦理学声明：本例报告已获得患者家属知情同意。

利益冲突声明：本文不存在任何利益冲突。

作者贡献声明：张恒负责课题设计，资料分析，撰写论文；方志成、孟忠吉、杜恩辅参与收集数据，修改论文；江斌负责拟定写作思路，指导撰写文章并最后定稿。

详细信息

通信作者:
张恒， zhangheng9987@163.com （ORCID： 0000-0003-2243-9279）

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出版历程
- 收稿日期: 2023-06-27
- 录用日期: 2023-07-28
- 出版日期: 2023-11-28

Transcatheter closure of rare type Ⅱ Abernethy malformation with pulmonary hypertension in children： A case report

a.
Department of Emergency Medicine, Hubei University of Medicine，Hubei Provincial Clinical Research Center for Precision Diagnosis and Treatment of Liver Cancer，Shiyan，Hubei 442000，China
b.
Department of Hepatobiliary and Pancreatic Surgery，Hubei University of Medicine，Hubei Provincial Clinical Research Center for Precision Diagnosis and Treatment of Liver Cancer，Shiyan，Hubei 442000，China
c.
Department of Infectious Diseases，Hubei University of Medicine，Hubei Provincial Clinical Research Center for Precision Diagnosis and Treatment of Liver Cancer，Shiyan，Hubei 442000，China
d.
Department of Interventional Radiology，Taihe Hospital，Hubei University of Medicine，Hubei Provincial Clinical Research Center for Precision Diagnosis and Treatment of Liver Cancer，Shiyan，Hubei 442000，China

Research funding:

Fund Project of Hubei Chen Xiaoping Science and Technology Development Foundation (CXPJJH11800001-2018246)

More Information

Corresponding author: ZHANG Heng， zhangheng9987@163.com （ORCID： 0000-0003-2243-9279）

摘要

摘要: Abernethy又称先天性门体静脉分流畸形，临床罕见，截至2019年全球文献报道不足300例，该病可合并肺动脉高压、肝脏肿瘤、肝衰竭等严重并发症，总体预后极差，早期诊断、积极有效治疗可降低和延缓并发症发生。本文通过门静脉造影，结合球囊阻断，将原本发育不良、纤细未显影的门静脉显影，从而将原本误诊为Ⅰ型Abernethy畸形的患儿明确为Ⅱ型Abernethy畸形，进而经介入封堵治疗成功救治患儿，现报道如下。
- Abernethy畸形 /
- 诊断 /
- 介入封堵 /
- 治疗
Abstract: Abernethy malformation， also known as congenital portosystemic shunts， is rare in clinical practice， with less than 300 cases reported in the global literature up to 2019. The disease can have serious complications such as pulmonary hypertension， liver tumor， and liver failure and tends to have an extremely poor prognosis， and early diagnosis and active and effective treatment can reduce and delay the onset of complications. In this case， portography combined with balloon occlusion helped to display the underdeveloped slender portal vein with dysplasia， so that the child who was formerly misdiagnosed with type Ⅰ Abernethy malformation was diagnosed with type Ⅱ Abernethy malformation， and then the child was successfully treated by transcatheter closure. This article gives a detailed report of this case.
- Abernethy Malformation /
- Diagnosis /
- Interventional Closure /
- Management

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图 1 腹部增强CT 检查

注：：a，冠状位；b，矢状位；c，动脉期；d，门静脉期；e，延迟期。

Figure 1. The enhanced CT images

下载: 全尺寸图片幻灯片

图 2 下腔静脉、门静脉造影术及试验性球囊封堵术

注： a，下腔静脉、门静脉造影；b，肝静脉造影；c，球囊封堵后门静脉造影；d，门静脉主干与髂内静脉交通支。

Figure 2. The portography and experimental balloon occlusion

下载: 全尺寸图片幻灯片

图 3 术后门静脉CT检查

注： a，术后VR图像；b，术后MIP图像。

Figure 3. Postoperative portal vein CT images

下载: 全尺寸图片幻灯片

参考文献(18)

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