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Infantile Onset Budd Chiari Syndrome: Challenges and Outcome of Liver Transplantation After Radiological
Aabha Nagral1, Samriddhi Poyekar1, Shailesh Sable2,3
1Department of Gastroenterology, Jaslok Hospital and Research Centre and Apollo Hospital, Mumbai, India.
Insights
Budd-Chiari syndrome (BCS) in infants is rare but treatable with radiological intervention (RI) and liver transplantation (LT). This study shows LT offers good long-term outcomes for pediatric BCS, with RI effectively managing complications.
Area of Science:
- Pediatric Hepatology
- Interventional Radiology
- Organ Transplantation
Background:
- Budd-Chiari syndrome (BCS) is a rare condition in infants, with limited data on liver transplantation (LT) outcomes.
- Radiological interventions (RI) like transjugular intrahepatic portosystemic shunt (TIPSS) and hepatic vein venoplasty are sometimes used before LT.
Purpose of the Study:
- To analyze the outcomes of liver transplantation (LT) in infants with Budd-Chiari syndrome (BCS).
- To evaluate the role of prior radiological interventions (RI) and the incidence of hepatopulmonary syndrome (HPS) in pediatric BCS patients undergoing LT.
Main Methods:
- Retrospective analysis of eight pediatric patients who underwent LT for BCS between 2017 and 2023.
- Review of pre-transplant radiological interventions, indications for LT, post-transplant complications, and survival rates.
Main Results:
- Seven of eight patients had prior RI (3 TIPSS, 4 venoplasty). Hepatopulmonary syndrome (HPS) occurred post-RI in 5 patients.
- Indications for LT included refractory ascites/bleeding (3) and HPS (5). Median age at transplant was 51 months.
- 1-year and 3-year survival rates were 75%. Vascular and biliary complications occurred in 37.5% and 25% of patients, respectively.
Conclusions:
- Liver transplantation (LT) combined with radiological intervention (RI) can effectively manage Budd-Chiari syndrome (BCS) in infants, leading to good long-term outcomes.
- Hepatopulmonary syndrome (HPS) is a significant concern post-TIPSS in pediatric BCS.
- Recurrent BCS and complications post-LT can be managed with RI, though high complication rates may be linked to HPS and prior interventions.
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.
