Budd Chiari Syndrome in Children: Experience From a Single Center

Background: Budd Chiari syndrome (BCS) is obstruction of venous outflow at any level from the small hepatic veins to right atrium. The etiologies BCS are multifactorial, prothrombotic conditions most common. Ultrasonography with doppler, CT and MR angiography diagnostic. Treatment modalities anticoagulants, angioplasty, transjugular intrahepatic portosystemic shunt (TIPS), thrombectomy liver transplantation. Aims: To analyze demographic, clinical, therapeutic interventional details along outcome all patients. Methods: This was a retrospective observational study data between 2007 2021. parameters studied were clinical presentation like jaundice, ascites, hepatosplenomegaly, hepatopulmonary (HPS); extent involvement vein (HV), inferior vena- cava (IVC) alone both HV+IVC; etiological workup including thrombophilia profile genetics. Intervention noted use anticoagulation, TIPS or transplant. Results: Total 18 patients identified mean age 8.6 years males 55.5%(n=10). common ascites (83.3%), hepatosplenomegaly (77.7% & 72.2%) HPS 38.8%(n=7). HV involved in 38.8%(n=7), IVC 16.6%(n= 3) 44.4%(n=8). etiology found 6(33.3%) cases, which 11.1%(n=2 each) had Antiphospholipid antibodies antithrombin-III deficiency, 5.5%(n=1 protein C S paroxysmal nocturnal hemoglobinuria, methylenetetrahydrofolate reductase gene JAK-2 mutation. Portal thrombosis co-existed 16.6%(n=3). Fourteen transplant, 4.4%(14/317) total. One year survival 78.5%(n=11) 83.3% HPS. Two successful stenting, 1 lost follow up. Conclusion: Thrombophilia evaluation usually inconclusive late presentation. Outcome good if referred early as intervention feasible. Liver transplant has shown promising even