Portal Vein Reconstruction in Children Undergoing Living Donor Liver Transplantation for Biliary Atresia

BACKGROUND: Biliary atresia (BA), a fibroinflammatory disease of the extra- and intra-hepatic bile ducts, is associated with hypoplasia±sclerosis of the portal vein (PV) due to obliterative portal venopathy (OPV). It renders the native PV not suitable for reconstruction during living donor liver transplantation (LDLT). We aimed to predict preoperatively who would require portal vein replacement during LDLT. METHODS: A retrospective review of children who underwent LDLT for BA from January 2019 to June 2024 was performed. Patient demographics, operative characteristics, and post-LDLT outcomes were analyzed. Preoperative factors predictive of the need to replace PV were identified, and a predictive model was developed. RESULTS: In the study period, 152 children who underwent LDLT for BA were included. Median (IQR) age and weight were 9 (7-12) months and 6.9 (5.9-8) kg, respectively. Primary LDLT was performed in 54 children, while 98 underwent LDLT after Kasai portoenterostomy (KP). Branch patch anastomosis was the most common type of PV reconstruction. Replacement of the native PV was required in 42 recipients, including 22 branch patch anastomoses, which were converted to vein conduit reconstruction due to thrombosis. There was no significant difference in the need for PV replacement in patients with and without prior KP. Preoperative body mass index (BMI) and mid-portal vein (mPVA) area were independently predictive of the usability of native PV. CONCLUSIONS: In children who underwent LDLT for BA, OPV caused significant PV narrowing, necessitating its replacement. Its etiology remains unknown and is not related to KP. The model, which incorporates BMI and mPVA, predicts the usability of native PV for anastomosis.