Abstract: Objective To elucidate the clinical characteristics and imaging findings associated with congenital intrahepatic portosystemic shunts in pediatric patients. Methods From June 2017 to June 2022, we examined the clinical and imaging features of 40 pediatric patients diagnosed with congenital intrahepatic portosystemic shunts at our hospital. Results In our cohort of 40 pediatric patients with congenital intrahepatic portosystemic shunts, a significant proportion(23 cases, 57.5%) had a history of hepatitis B cirrhosis. The predominant clinical classification was type III shunts(23 cases, 57.5%), with the shunt site most commonly identified at the right posterior branch of the portal vein inferior vena cava (17 cases, 42.5%). These patients exhibited alterations in liver function markers, including elevated alanine aminotransferase(ALT), glutamic oxaloacetic aminotransferase(AST), and total bilirubin(TBil) levels. CT imaging revealed that lesions were predominantly located in the right hepatic lobe(25 cases, 62.5%) and the left hepatic lobe(15 cases, 37.5%). A majority(33 cases, 82.5%) showed significant expansion of hepatic veins at the shunt site, whereas 7 cases (17.5%) did not. Ultrasound findings indicated that lesions were situated near the surface or lobe edge within the parenchyma, appearing as tubular, cystic, or irregular shaped structures without echo on two-dimensional ultrasound. Color Doppler ultrasound demonstrated non-pulsatile, alternating red and blue blood flow signals from the portal vein branch to the hepatic vein. Conclusion In pediatric patients, congenital intrahepatic portosystemic shunts exhibit distinct clinical and radiological profiles, often correlated with a history of hepatitis B cirrhosis. Predominatly classified as type III, these shunts frequently occur at the right posterior branch of the portal to the inferior vena cava, eliciting significant alterations in liver function parameters. These diagnostic features and their clinical implications offer valuable reference points for effective management in clinical settings.

Key words: Children, Congenital intrahepatic portosystemic shunt, Clinical characteristics, Imaging manifestations