Liver Transplantation Experience in Two Children Diagnosed with Abernethy Type 1B Congenital Extrahepatic Portosystemic Shunt

Congenital extrahepatic portosystemic shunt (CEPS), also known as Abernethy malformation, is a rare vascular anomaly characterized by diversion of portal venous blood away from the liver into the systemic circulation. Type 1 malformations are defined by the complete absence of intrahepatic portal venous flow and require liver transplantation as definitive treatment. We report two pediatric patients diagnosed with Abernethy type 1B malformation. The first patient presented with progressive cholestasis, growth retardation, and impaired liver synthetic function. Due to clinical deterioration and absence of intrahepatic portal flow, living donor liver transplantation was performed with portal vein reconstruction using an interposition graft. The second patient presented with hyperammonemia and neurocognitive impairment. Imaging confirmed type 1B CEPS, along with a focal hepatic lesion consistent with a benign regenerative nodule. The patient underwent successful living donor liver transplantation with standard portal reconstruction. Both patients had uneventful postoperative courses. During follow-up (18 and 24 months), liver function normalized and growth parameters improved, with no vascular complications observed. Liver transplantation remains the only definitive treatment for Abernethy type 1B malformation. Early diagnosis and meticulous surgical planning, particularly regarding portal inflow reconstruction, are essential for optimal outcomes.

Keywords: Abernethy malformation, liver transplantation, pediatric, portosystemic shunt