Congenital portosystemic shunts: surgical treatment experience

Abstract

Congenital porto-caval shunts are rare and may have a different morphological structure (intra- and extrahepatic shunts, with or without portal blood flow). The main method of treating patients with this pathology is endovascular shunt occlusion. However, in some cases, this method is ineffective.

The article contains a description of six clinical examples of surgical treatment of congenital porto-systemic shunts in children. In the diagnosis of congenital portosystemic shunts, the leading role belongs to Doppler ultrasound, multislice computed tomography, and angiography. The indication for surgical treatment was the anatomical features of the shunt, which makes endovascular occlusion technically impossible. In one observation a wide Arantian duct was diagnosed, its open ligation was performed. In another case, the portal vein emptied directly into an aneurysmal dilatation, performed reconstructive plastic surgery on the vessels of the portal vein. In the next observation, a pronounced retrograde blood flow was determined along the dilated inferior mesenteric vein, blood was discharged through the sacral plexus into the internal iliac vein. The left internal iliac vein was isolated and ligated, the dysplastic inferior mesenteric vein was ligated and partially removed. In 2 patients, the portal vein flowed directly into the inferior vena cava in the area of aneurysmal expansion; an operation was performed — open ligation of the shunt. In one observation, a deep hypoplasia of the intrahepatic branches of the portal vein was diagnosed, and therefore the restoration of portal blood flow after the closure of the shunt is impossible. The child was sent to decide on a liver transplant.

Each case of congenital porto-caval shunts is unique. The surgeon determines the tactics directly during the operation, depending on the morphological structure of the organs, since the preoperative examination does not always give an unambiguous idea.

Keywords

case report / congenital portosystemic shunt / Abernathy malformation / surgical treatment / angiography / children

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