Background: Budd Chiari Syndrome(BCS) characterised most commonly by obstruction of blood flow in 2 or more hepatic veins, invariably requires radiologic intervention with venoplasty/ stenting/ TIPS , besides anticoagulation, for relief of symptoms. Here we report 5 children with Celiac Disease and BCS at presentation. The BCS resolved completely in all cases on gluten free diet and short administration of anticoagulation.
Methods: A retrospective review of children with CeD & BCS seen in our Celiac Disease Clinic. A ll relevant details were extracted from clinic files of the included patients.
Results: There were 3 girls and 2 boys aged 7, 6, 11, 10 & 13 years. One child was a known Celiac Disease patient who was non compliant. Two children had chronic, 2 sub acute and 1 acute presentation. Four presented with features of hepatic venous outflow tract obstruction(HVOTO) and Celiac Disease was diagnosed simultaneously. No definite evidence of thrombophilia was found in any child. However all children had iron deficiency and thrombocytosis. Liver biopsy done in all 5 was consistent with HVOTO and excluded other causes of CD associated liver disease.The symptoms of HVOTO resolved in all 5 only on GFD and short periods of anticoagulation of 6-24 months. No child required radiologic intervention. Both children with varices at presentation had normal endoscopy for the same within 1 year.One child with established chronic liver disease has proven recompensation documented at 11 years of follow up.
Conclusions: Celiac disease may cause a HVOTO like phenotype which responds well to GFD and brief anticoagulation.