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
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。
Transcatheter closure of rare type Ⅱ Abernethy malformation with pulmonary hypertension in children: A case report
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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。
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关键词:
- 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.-
Key words:
- Abernethy Malformation /
- Diagnosis /
- Interventional Closure /
- Management
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