Ji Lianfu, Ji Zhaoming, Xiang Dandan, Qin Yuming, Yang Shiwei
Department of Cardiology, Children's Hospital of Nanjing Medical University, Nanjing, China.
Front Pediatr. 2022 Sep 9;10:856611. doi: 10.3389/fped.2022.856611. eCollection 2022.
Abernethy malformation is a rare abnormality of the hepatic portal vein system with non-specific and diverse clinical manifestations. Here, we described a case of abernethy malformation with hepatopulmonary syndrome in a 10-year-old girl. On physical examination, cyanosed lips and acropachy could be found. Her oxygen saturation fluctuated at 89-94%, and the fasting blood ammonia was 98 umol/L. Furthermore, there were abnormalities in the imaging. The microbubble test with contrast echocardiography was positive. Computer tomography angiography (CTA) showed the splenic vein, and the superior mesenteric drained directly into the inferior vena cave after confluence. The same result was also observed in delayed splenic arteriography. Then, we discovered a tiny branch of the intrahepatic portal vein by the inferior vena cava balloon occlusion test, which could also show the confluence of the splenic vein and superior mesenteric vein with the inferior venacave. According to the evidence above, we concluded that the girl was a patient of type II abernethy malformation. For the severe dysplasia of the portal vein, the girl accepted partial ligation of portosystemic shunt and Rex shunt, which improved her oxygen saturation and exercise tolerance.
阿伯内西畸形是一种罕见的肝门静脉系统异常，临床表现不特异且多样。在此，我们描述了一名10岁女孩患有阿伯内西畸形合并肝肺综合征的病例。体格检查时，可发现嘴唇发绀和杵状指。她的血氧饱和度波动在89% - 94%之间，空腹血氨为98 μmol/L。此外，影像学检查存在异常。超声心动图造影微泡试验呈阳性。计算机断层血管造影（CTA）显示脾静脉和肠系膜上静脉汇合后直接汇入下腔静脉。延迟脾动脉造影也得到相同结果。然后，通过下腔静脉球囊闭塞试验，我们发现了肝内门静脉的一个小分支，其也能显示脾静脉和肠系膜上静脉与下腔静脉的汇合情况。根据上述证据，我们得出该女孩为Ⅱ型阿伯内西畸形患者的结论。鉴于门静脉严重发育不良，该女孩接受了部分门体分流结扎术和雷克斯分流术，这改善了她的血氧饱和度和运动耐量。
