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.