Khirurgiia (Mosk). 2026;(6):121-126. doi: 10.17116/hirurgia2026061121.
ABSTRACT
Congenital portosystemic shunts (CPSS) are rare vascular anomalies with portal blood bypassing hepatic filtration and subsequent various metabolic and developmental complications. Early diagnosis and timely intervention are crucial to prevent severe complications. We describe a 3-month-old boy with persistent jaundice and developmental delays. Laboratory tests revealed hyperbilirubinemia and elevated transaminases. Doppler ultrasound and contrast-enhanced computed tomography revealed a large intrahepatic PSS up to 25 mm in diameter with hypoplasia of the right liver lobe. Endovascular embolization was performed via transjugular approach. Postoperative migration of one coil into pulmonary artery required endovascular procedure. Gradual normalization of liver function tests, restoration of portal blood flow, regeneration of liver parenchyma and age-appropriate development were achieved. This case demonstrates technical feasibility and effectiveness of endovascular closure of large intrahepatic CPSS, even in infants. Key factors for successful outcomes include careful anatomical assessment, individual treatment plan and meticulous postoperative monitoring.
PMID:42296325 | DOI:10.17116/hirurgia2026061121