Balloon venoplasty as primary modality of treatment in children with Budd-Chiari syndrome.

INTRODUCTION: Budd-Chiari syndrome (BCS) is a rare condition affecting children. It is characterized by occlusion of venous outflow from liver at the level of hepatic veins (HV) or inferior vena cava (IVC). The management of BCS in children revolves around forming new collaterals for venous outflow or by elimination of blockage in the venous outflow tracts. These can be achieved by balloon venoplasty (BV), transjugular intrahepatic portosystemic shunting (TIPSS) or open shunt surgeries.

AIM: The aim of this study is to evaluate the management of BCS in children with balloon venoplasty as primary modality of treatment.

MATERIALS AND METHODS: This is a retrospective study which includes children diagnosed with BCS managed by balloon venoplasty by a single surgeon at a single institute. Once confirmed, the child was posted for balloon venoplasty and liver biopsy. When venoplasty was successful, child was subsequently heparinized and dose titrated. Routine follow up was mandated and dose adjustments were continued during follow up. In case of unsuccessful venoplasty, depending on the liver biopsy report, shunt procedure or liver transplantation is offered to patients. An algorithm was then designed for management of BCS in children.

RESULTS: A total of 35 children who underwent evaluation of symptoms associated with Budd-Chiari syndrome were included in the study. Of all the children, 14 are alive and symptom free, 9 are deceased and 12 lost to follow up. Hepatic vein was the most common site of obstruction (85%), followed by both IVC and HV (15%). Overall, in 35 children, 26 had a successful balloon venoplasty, in 3 venoplasty was not done (2 spontaneous resolution and 1 died awaiting), in 6 it was unsuccessful (3 technical failures: 1 underwent TIPSS and 2 lost to follow up, 3 clinical failures: portocaval shunt for failed venoplasty).

CONCLUSION: Budd-Chiari syndrome is a rare condition affecting children. Balloon venoplasty as a primary modality of treatment for BCS is a promising option for management in children. Early and aggressive use of radiological intervention can help achieve recanalization in children.

TYPE OF STUDY: Clinical research paper.

LEVEL OF EVIDENCE: Level IV.