A rare combination of arterio-portal fistula and portosystemic shunt. Endovascular treatment

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Abstract

BACKGROUND: Portosystemic shunts (PSS) is a rare malformation 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) is defined by the respiratory dysfunction due to the defect in arterial oxygenation induced by the dilatation of intrapulmonary vessels associated with liver disease.

CLINICAL CASE DESCRIPTION: In our clinic, we treated endovascularly a 10.5-y.o. patient with a rare combination of congenital portosystemic shunt (CPSS) and congenital APF complicated by Hepatopulmonary syndrome. 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, there were signs of portal hypertension (PH), that is why the anticoagulant therapy was administered which resulted in PH symptoms regression.

CONCLUSION: In case of the combined arterio-portal fistula and congenital portosystemic shunt, it is necessary, first, to occlude APF, then to perform the balloon occlusion test, and then -if there are well developed intrahepatic branches of the portal vein- to perform PSS occlusion. In the postoperative period, anticoagulant therapy is recommended for preventive purposes.

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About the authors

Roman V. Garbuzov

Russian Children’s Clinical Hospital — Pirogov Russian National Research Medical University

Email: 9369025@mail.ru
ORCID iD: 0000-0002-5287-7889
SPIN-code: 7590-2400

MD, Dr. Sci. (Medicine)

Россия, Moscow

Yuri A. Polyaev

Russian Children’s Clinical Hospital — Pirogov Russian National Research Medical University

Email: polyaev@inbox.ru
ORCID iD: 0000-0002-9554-6414
SPIN-code: 7587-9843

MD, Dr. Sci. (Medicine), Professor

Россия, Moscow

Ivan A. Mylnikov

Russian Children’s Clinical Hospital — Pirogov Russian National Research Medical University

Author for correspondence.
Email: dr.mylnikov@mail.ru
ORCID iD: 0009-0005-0898-5355
Россия, Moscow

References

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2. Fig. 1. Schematic representation of anomalies of liver vessels structure: 1 — aorta, 2 — hepatic artery, 3 — inferior vena cava, 4 — portal vein, 5 — segmental branch of the left hepatic artery, 6 — portosystemic shunt, 7 — segmental branch of the left branch of the portal vein.

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3. Fig. 2. Selective angiography of the hepatic artery: 1 — hepatic artery, 2 — segmental branch of the left hepatic artery, 3 — portosystemic shunt, 4 — segmental branch of the left branch of the portal vein.

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4. Fig. 3. Upper mesentericoportography: 1 — portosystemic shunt, 2 — basic trunk of portal vein, 3 — vena mesenterica superior.

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5. Fig. 4. Selective angiography of the hepatic artery, after occlusion of the arterio-venous shunt: 1 — hepatic artery, 2 — right branch of the hepatic artery, 3 — spiral occluder installed in the segmental branch of the left hepatic artery, 4 — left branch of the hepatic artery.

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6. Fig. 5. Test balloon occlusion: 1 — portal vein, 2 — right branch of the portal vein, 3 — portosystemic shunt, 4 — balloon catheter.

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7. Fig. 6. Installation of an occluder in a port-system shunt: 1 — occluder (Amplatzer™ Vascular Plug II 22 mm. USA), 2 — spiral occluder installed in the segmental branch of the left hepatic artery.

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