A Rare Case of Extrahepatic Portal Venous Obstruction in a Nine-Year-Old Female and Its Management: A Case Report

Abstract

Extrahepatic portal vein obstruction (EHPVO) is a kind of liver vascular disease that causes structural abnormalities in the portal veins, including cavernomatous metamorphosis and obstruction. It is the most common cause of esophageal varices-related hematemesis in youngsters. Significant risk factors include congenital abnormalities, dehydration, sepsis, trauma, hypercoagulable conditions, and multiple transfusions. Acute extrahepatic portal vein blockage is often ignored because patients are usually asymptomatic. Subacute and chronic stages can cause symptoms including splenomegaly and hematemesis without hepatic decompensation. Imaging studies aid in the diagnosis; Doppler imaging is added to ultrasonography to visualize portal vein blood flow. MRI and CT scans are used to visualize portal vein blockage. Prevention of acute bleeding is the cornerstone in the management. Studies have shown that transhepatic thrombolysis is the preferred choice to avoid systemic side effects. Transjugular intrahepatic portosystemic shunt (TIPS) treats extrahepatic portal venous thrombosis and is typically followed by conservative variceal hemorrhage treatment. Liver transplantation is performed when other management measures fail. Here, we present a rare case of EHPVO in a nine-year-old female who was lost to follow-up for a long time and later showed signs of portal biliopathy and non-visualization of a surgically created splenorenal shunt. Re-shunting was performed after detailed conservative management, and the patient responded well to the treatment given.