VENOOCCLUSIVE DISEASE OF THE LIVER AFTER CHEMOTHERAPY FOR RHABDOMYOSARCOMA - CASE-REPORT WITH A REVIEW OF THE LITERATURE

We describe the case of a 3-year-old girl who developed veno-occlusive disease of the liver while receiving chemotherapy for parameningeal r habdomyosarcoma. After suffering lethargy and oral mucosal bleeding fo r one day, the patient exhibited a sudden weight gain and refractorine ss to platelet transfusions. Symptoms rapidly worsened with elevation of liver enzymes, bleeding diatheses, and respiratory failure. An ultr asound scan of the liver demonstrated reversed flow in the portal vein . Maximal supportive care, including tracheal intubation and mechanica l ventilation, was required. The patient gradually recovered with no r espiratory and minimal neurological sequelae. Veno-occlusive disease o f the liver should be considered in children receiving chemotherapy wh o develop weight gain, a sudden drop in platelet count and derangement of liver enzymes. Aggressive supportive measures should be instituted if necessary, as patients surviving the acute phase can expect to mak e a full recovery. (C) 1995 Wiley-Liss, Inc.