Living donor liver transplantation on pediatric patient with Budd-Chiari syndrome: Case report and literature review.

INTRODUCTION: The management of Budd-Chiari syndrome is determined on the basis of the severity of the disease. There are no standard guidelines regarding the management of Budd-Chiari syndrome in children, particularly in cases of liver transplantation. Therefore, we present a case of a pediatric patient with Budd-Chiari syndrome treated with liver transplantation.

CASE PRESENTATION: A female patient aged 1 year and 8 months presented to the hospital with an enlarged stomach in the last 1.5 months before admission. The patient was moderately ill, malnourished, and jaundiced. Liver biopsy revealed fibrosis in the portal area and confluent necrosis caused by vascular disorders. Magnetic resonance imaging (MRI) revealed a nutmeg liver and ascites due to the stenosis of the inferior vena cava at the level of the liver and the middle and left hepatic veins. The patient underwent living-donor liver transplantation. The occlusion of the proximal right hepatic vein due to the presence of a membrane was identified as the cause of Budd-Chiari syndrome in this case. Postoperatively, the patient's condition was stable and there was no sepsis or any other complications.

CLINICAL DISCUSSION: Prothrombotic factors are often the underlying cause of more than 80 % of Budd-Chiari syndrome cases. Protein C deficiency is suspected to be a prothrombotic factor that triggers Budd-Chiari syndrome in patients. Liver transplantation is the treatment of choice for patients with Budd-Chiari syndrome who were not treated with anticoagulation therapy, angioplasty, and TIPS.

CONCLUSION: Living-donor liver transplantation has a good outcome in the management of pediatric patients with Budd-Chiari syndrome.