Budd-Chiari Syndrome in Children in Children

Budd-Chiari Syndrome in Children in Children

• There is limited data on pediatric Budd-Chiari syndrome (BCS), hence diagnosis may be missed.
• A high index of suspicion and adequate work-up is needed. ‹ Adolescents and infants represent unique groups.
• Complete prothrombotic work-up should be done in all, up to 50% have multiple causes.
• Anticoagulation is difficult as there exists no data on direct oral anticoagulant (DOAC) in BCS in children.
• Expertise in interventional radiology is required for the treatment of BCS in children. Innovations in technique and efforts to limit radiation exposure are needed. 
• Angioplasty of the hepatic vein (HV) is associated with a high failure rate. When feasible, HV stenting should be preferred over transjugular intrahepatic portosystemic shunt (TIPS).
• Inclusion of shear wave elastography (SWE) with color Doppler ultrasound (CDUS) examinations both in the pre-treatment evaluation and follow-up after RI helps indeciding therapy, monitoring response and detection of recurrence.
• Long-term monitoring for hepatopulmonary syndrome and liver SOL including HCC(including AFP) should be done.
• There is an urgent need for consensus guidelines on BCS in children.