Hepatic veno-occlusive disease in Wilms' tumor

Invasive surgical procedures will be avoided by recognition of veno-occlusive disease (VOD) as a clinical syndrome which sometimes leads to serious complications in children receiving Actinomycin D for Wilms' tumor. In order to document the unusually frequent occurrence of VOD far beyond the observations of others, a prospective study was undertaken in 13 consecutive Actinomycin D-treated children. There were 9 children experiencing 27 events of mostly mild VOD. Six of them were below 3 years of age having in 5 cases a unilateral kidney tumor on the right side. The criteria used for VOD were painful hepatomegaly and abdominal distension accompanied by weight gain, ascites, hemoglobin and platelet drop, with or without elevated bilirubin level found in all patients developing VOD. Supportive management of these patients should attempt to preserve respiratory and renal function, generally resulting in a favorable outcome.