Abernethy畸形：临床病例

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先天性肝外门腔分流是与部分或全部门静脉血转入全身血液有关的罕见血管异常。先天性肝外门腔分流被称为Abernethy畸形。由于发病率低并临床表现多样，这种病理的识别是一个诊断问题。

本文描述了一个15岁患者的Ib型Abernethy畸形的临床病例，该患者长期有高血压、反复鼻出血、胸痛、头晕、呼吸困难、运动耐力低下、便血、上腹痛、恶心和瘙痒等病史。经过全面检查，患者被诊断为门静脉系统异常：门静脉导管扩张，直接流入下腔静脉。还发现了肝实质中的多个结节、扩张的心腔、心肌肥厚和肺动脉高压。鉴于症状的严重性以及分流的大小和类型，一个多学科科联合会诊建议进行肝移植。

我们在本文中讨论了门静脉系统异常的诊断算法和其他可能的治疗方案。

作者简介

Alexandra V. Panyukova

Lomonosov Moscow State University, Medical Research and Educational Center

Email: panyukovaalexandra@gmail.com
ORCID iD: 0000-0002-5367-280X
俄罗斯联邦, Moscow

Valentin E. Sinitsyn

Lomonosov Moscow State University, Medical Research and Educational Center

Email: vsini@mail.ru
ORCID iD: 0000-0002-5649-2193
SPIN 代码: 8449-6590

MD, Dr. Sci. (Med); Professor

俄罗斯联邦, Moscow

Elena A. Mershina

Lomonosov Moscow State University, Medical Research and Educational Center

Email: elena_mershina@mail.ru
ORCID iD: 0000-0002-1266-4926
SPIN 代码: 6897-9641

MD, Cand. Sci. (Med.), Assistant Professor

俄罗斯联邦, Moscow

Natalya A. Rucheva

V.I. Shumakov National Medical Research Center of Transplantology and Artificial Organs

编辑信件的主要联系方式.
Email: rna1969@yandex.ru
ORCID iD: 0000-0002-8063-4462

MD, Cand. Sci. (Med.)

俄罗斯联邦, Moscow

参考

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补充文件

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1. JATS XML
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2. 图1。造影剂计算机断层扫描，门静脉相，轴向投影：脾静脉（SV）扩张。

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3. Fig. 2. Contrast-enhanced CT, portal phase, coronal view. Splenic (SV) and superior mesenteric (SMV) veins fused together, forming a portal vein conduit (white arrow).

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4. Fig. 3. Contrast-enhanced CT, portal phase, axial view. Portal vein conduit (white arrow).

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5. Fig. 4. Contrast-enhanced CT, portal phase, coronal view. Portal vein conduit flowing directly into the IVC (white arrow), enlarged liver with heterogeneous parenchymal enhancement.

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6. 图5。肺部计算机断层扫描血管造影，轴向投影：肺动脉干扩张。

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7. 图6。肺部计算机断层扫描血管造影，轴向投影：心肌肥厚。

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8. 图7。门静脉的正常解剖和分流的分类：a——门静脉的正常解剖；b——先天性肝外门腔分流，Ia型；c——先天性肝外门腔分流，Ib型；d——先天性肝外门腔分流，II型。 注：IVC（inferior vena cava）——下腔静脉；PV（portal vein）——门静脉；SV（splenic vein）——脾静脉；SMV（superior mesenteric vein）——肠系膜上静脉；Shunt——分流。

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9. 图8。根据A.Baiges等人的研究，先天性肝外门腔分流患者的治疗模式[32]。

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