Infantile Onset Budd Chiari Syndrome: Challenges and Outcome of Liver Transplantation After Radiological Interventions

Abstract

Introduction

Budd-Chiari syndrome (BCS) in infants is rare, and there is limited published literature on liver transplantation (LT).

Methods

Eight children who underwent LT for BCS from 2017 to 2023 were analyzed.

Results

Seven out of the eight children had radiological intervention (RI) prior to LT (3 had transjugular intrahepatic portosystemic shunt [TIPSS], and 4 had hepatic vein venoplasty). Hepatopulmonary syndrome (HPS) was seen post-TIPSS in 3 and postvenoplasty in 2 children, respectively. The indication for LT was refractory ascites or recurrent upper gastrointestinal bleed in 3 patients, HPS in 5 patients, respectively. The median age and weight of children at transplant was 51 months (IQR 26–82 months) and 11.35 kg (IQR 10.05–18 kg), respectively. The median duration from onset of symptoms to LT was 42 months (IQR 18.5–75 months). HPS resolved in the 4 patients alive, after a median period of 25 days (IQR 15.5–60 days). The median duration of post-transplant follow-up is 4 years 9 months (IQR 3 year 5 months–4 years 11 months). Vascular complications were seen in 37.5% patients which were amenable to RI. Biliary complications were seen in 25% of children. The 1-year and 3-year survival rates both were 75%.

Conclusion

BCS in infants can be managed effectively with RI followed by LT. LT has shown good long-term outcomes in children with BCS. HPS seems to be common after TIPSS in pediatric BCS. Recurrent BCS post-LT can be salvaged using RI. High biliary and vascular complications are likely related to HPS and previous TIPSS/RI.