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A rare combination of arterio-portal fistula and portosystemic shunt. Endovascular treatment
- Authors: Garbuzov R.V.1, Polyaev Y.A.2, Mylnikov I.3
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Affiliations:
- Russian Children's Clinical Hospital of Pirogov Russian National Research Medical University
- Russian Children’s Clinical Hospital
- Russian Children's Clinical Hospital (RDCB) — branch of the Federal State Educational Institution of Higher Education named after N.I. Pirogov of the Ministry of Health of the Russian Federation
- Section: CASE REPORTS
- Submitted: 23.09.2024
- Accepted: 11.02.2025
- Published: 16.02.2025
- URL: https://jps-nmp.ru/jour/article/view/807
- DOI: https://doi.org/10.17816/ps807
- ID: 807
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Abstract
Background: Portosystemic shunts (PSS) are rare malformations in which there is an abnormal connection between the portal system and the systemic circulation. Arterio-portal fistulas (APF) are arterio-venous connections between the arteries of the ventral trunk system and the portal vein. Hepatopulmonary syndrome (HPS) is defined by respiratory dysfunction due to a defect in arterial oxygenation induced by dilatation of intrapulmonary vessels associated with liver disease.
Matherials and Methods: In our clinic, we performed endovascular treatment of a 10.5-year-old patient with a rare combination of congenital portosystemic shunt (CPSS) and congenital APF complicated by HPS. The treatment was performed in several stages. The first stage was APF occlusion. The second stage was a balloon occlusion test. The third stage was PSS occlusion. In the postoperative period, signs of portal hypertension (PH) were observed and anticoagulant therapy was administered, resulting in regression of PH symptoms.
Conclusion: In combination of arterio-portal fistula and congenital portosystemic shunt, it is necessary to first occlude the APF, then perform a balloon occlusion test, and in the presence of sufficiently developed intrahepatic branches of the portal vein, it is possible to perform occlusion of the PSS. In the postoperative period, it is necessary to prescribe anticoagulant therapy for preventive purposes.
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About the authors
R. V. Garbuzov
Russian Children's Clinical Hospital of Pirogov Russian National Research Medical University
Email: 9369025@mail.ru
ORCID iD: 0000-0002-5287-7889
д-р мед. наук, заведующий отделением рентгенохирургических методов диагностики и лечения
РоссияYu. A. Polyaev
Russian Children’s Clinical Hospital
Email: polyaev@inbox.ru
ORCID iD: 0000-0002-9554-6414
д-р мед. наук, профессор, врач отделения рентгенохирургических методов диагностики и лечения
РоссияIvan Mylnikov
Russian Children's Clinical Hospital (RDCB) — branch of the Federal State Educational Institution of Higher Education named after N.I. Pirogov of the Ministry of Health of the Russian Federation
Author for correspondence.
Email: dr.mylnikov@mail.ru
ORCID iD: 0009-0005-0898-5355
pediatric surgeon, interventional radiology
РоссияReferences
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