Introduction : Hepatic vein occlusive disease (HVOD) is a rare complication after liver transplantation but it often takes a life-threatening course. HVOD is characterized by nonthrombotic, fibrous obliteration of the small centrilobular hepatic veins by connective tissue and centrilobular necrosis in zone 3 of the acini. We describe, for the first time, our experience with defibrotide administered to HVOD after living donor liver transplantation.

Methods : 39-year-old female patient was diagnosed as primary biliary cirrhosis (PBC). PBC was managed with several medications including azathioprine. Living donor Liver transplantation was considered as her condition was getting worse. We underwent living donor liver transplantation (LDLT) with extended right lobe graft. Clinical acute rejection was considered as liver enzyme and bilirubin was elevated after post-operative 4th day. Although we treated acute rejection, patient’ condition was getting worse. Clinical symptoms were ascites, pleural effusion, weight gain, jaundice, recurrent RUQ pain.

Results : We performed transjugular liver biopsy and transferred to ICU for management of fluid retention at POD 14. A liver biopsy was confirmed the diagnosis of HVOD. we searched published studies for treatment. we searched published studies for treatment of VOD after transplantation. Several studies showed that defibrotide was considered as effective treatment for VOD. We administered defibrotide(6.25 mg/kg) at POD 25 for 21 days and then patient was slowly recovered from jaundice.

Conclusions : While differentiating VOD on clinical backgrounds in each recipient, the diagnosis of VOD is required as early as possible, as the outcome of the disease can be considerably affected by the start of specific treatments.