Percutaneous hepatic vein recanalization in pediatric Budd-Chiari syndrome - 10 years' experience from a tertiary center

Background: Budd - Chiari syndrome (BCS) due to hepatic venous outflow obstruction is a rare cause of liver disease with dismal outcome, often amenable to catheter intervention.

Materials and methods: This retrospective single-center study analyzed the clinical profile and medium-term outcome of interventional treatment with balloon angioplasty ± stenting in all pediatric BCS over a 10-year period. Clinical, laboratory, imaging, and interventional data were retrieved. Transhepatic (TH) access was utilized in the recent 3 years.

Results: We included a total of 27 patients. Acute and subacute BCS comprised 93% of subjects. Ascites was the most common symptom. COVID-19 infection and Takayasu arteritis were two novel etiologies in our study. There was isolated hepatic vein (HV) narrowing in 11 (41%), isolated inferior vena cava obstruction in 4, and combined occlusion in 12 (44%). Intervention was successful in 22 (82%) patients. Stenting was required in 14 (64%) patients and the rest underwent balloon angioplasty. The immediate outcome was better with stenting than balloon (91% vs. 64%). Transhepatic access in 6 patients allowed HV cannulation in all and achieved patency in five patients. Two patients from the balloon group (25%) and 9 from the stent group (64%) are alive with patent veins at a median follow-up of 60 months, indicating a high attrition rate.

Conclusion: Catheter interventions restored physiological blood flow in pediatric BCS. TH route improved cannulation of occluded HV compared to other accesses. Immediate and medium-term outcomes were better after stenting with lower rates of reinterventions than balloon angioplasty. Life-long surveillance is required as mortality is high on follow-up.