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Case report: Rare abernethy malformation with hepatopulmonary syndrome in a pediatric patient
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...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9500375/ https://www.ncbi.nlm.nih.gov/pubmed/36160790 http://dx.doi.org/10.3389/fped.2022.856611 |
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author | Ji, Lianfu Ji, Zhaoming Xiang, Dandan Qin, Yuming Yang, Shiwei |
author_facet | Ji, Lianfu Ji, Zhaoming Xiang, Dandan Qin, Yuming Yang, Shiwei |
author_sort | Ji, Lianfu |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-9500375 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-95003752022-09-24 Case report: Rare abernethy malformation with hepatopulmonary syndrome in a pediatric patient Ji, Lianfu Ji, Zhaoming Xiang, Dandan Qin, Yuming Yang, Shiwei Front Pediatr Pediatrics 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. Frontiers Media S.A. 2022-09-09 /pmc/articles/PMC9500375/ /pubmed/36160790 http://dx.doi.org/10.3389/fped.2022.856611 Text en Copyright © 2022 Ji, Ji, Xiang, Qin and Yang. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Pediatrics Ji, Lianfu Ji, Zhaoming Xiang, Dandan Qin, Yuming Yang, Shiwei Case report: Rare abernethy malformation with hepatopulmonary syndrome in a pediatric patient |
title | Case report: Rare abernethy malformation with hepatopulmonary syndrome in a pediatric patient |
title_full | Case report: Rare abernethy malformation with hepatopulmonary syndrome in a pediatric patient |
title_fullStr | Case report: Rare abernethy malformation with hepatopulmonary syndrome in a pediatric patient |
title_full_unstemmed | Case report: Rare abernethy malformation with hepatopulmonary syndrome in a pediatric patient |
title_short | Case report: Rare abernethy malformation with hepatopulmonary syndrome in a pediatric patient |
title_sort | case report: rare abernethy malformation with hepatopulmonary syndrome in a pediatric patient |
topic | Pediatrics |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9500375/ https://www.ncbi.nlm.nih.gov/pubmed/36160790 http://dx.doi.org/10.3389/fped.2022.856611 |
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