Journal of clinical and experimental hepatology · 2025
Infantile Onset Budd Chiari Syndrome: Challenges and Outcome of Liver Transplantation After Radiological Interventions.
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Nagral Aabha, Poyekar Samriddhi, Sable Shailesh, Bagde Abhijit, Verma Rahul, Shah Ketul, Vasant Suresh, Sawant Ambreen, Mirza Darius
Аннотация
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.
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.