Budd Chiari Syndrome in Children: Clinical Features, Radiological Intervention and Outcome Presidential Poster

Abstract

Purpose: “Radiological intervention” is now the first line of therapy in adults with Budd-Chiari syndrome (BCS). Published literature on pediatric BCS is scarce. We evaluated the clinical profile and role of therapeutic radiological intervention in children with BCS. Methods: 46 children (29 boys, median age 10.5 [2-16] y) diagnosed as BCS were evaluated. Standard medical therapy was given to all patients. Radiological intervention {angioplasty (hepatic vein [HV]-3), stenting (HV-18, inferior vena cava [IVC]-5), transjugular intrahepatic portosystemic shunt [TIPS] (n-3)} was done in 25 cases. All patients subjected to radiological intervention were started on long-term anticoagulation. Clinical, biochemical and radiological follow-up was done. Results: Doppler ultrasonography was diagnostic in 96% cases. All patients had chronic BCS, with hepatomegaly in 84.8%, ascites in 82.6%, splenomegaly in 69.6%, prominent abdominal veins in 69.6%, variceal bleed in 34.8% and jaundice in 19.6% cases. Hepatic vein (all three) was the commonest site of block (n=33, 72%) followed by combined HV and IVC block (n=11, 24%) and isolated IVC block (n=2, 4%). 8/12 (75%) cases had abnormal procoagulant workup. There was no difference in the clinical presentation and liver functions of the patients given only medical therapy as compared to those who underwent radiological intervention except for higher bilirubin in the nonintervention group. Radiological intervention was technically successful in 100%. Clinical and biochemical improvement was seen in the intervention group. Complications included neck hematoma and haemorrhagic ascites in 1 patient each. One child in the intervention group (post TIPS sudden cardiac event) and 2 in non-intervention group (end stage liver disease-1, head injury-1) died. Stent was patent in 15/20 (75%) children in follow-up [median 6.5mo (15 days to 7.2 y)]. Two of the 5 cases with re-stenosis have undergone repeat radiological procedures succesfully. Only 2/21 (10%) patients in the nonintervention group showed improvement. Conclusion: Hepatic vein block is the commonest site of block seen in 72% cases. Therapeutic radiological intervention is a technically feasible, safe and effective therapeutic modality for children with BCS.