HEPATIC VENOOCCLUSIVE DISEASE IN WILMS-TUMOR

Invasive surgical procedures will be avoided by recognition of veno-oc clusive disease (VOD) as a clinical syndrome which sometimes leads to serious complications in children receiving Actinomycin D for Wilms' t umor. In order to document the unusually frequent occurrence of VOD fa r beyond the observations of others, a prospective study was undertake n in 13 consecutive Actinomycin D-treated children. There were 9 child ren 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 righ t side. The criteria used for VOD were painful hepatomegaly and abdomi nal distension accompanied by weight gain, ascites, hemoglobin and pla telet drop, with or without elevated bilirubin level found in all pati ents developing VOD. Supportive management of these patients should at tempt to preserve respiratory and renal function, generally resulting in a favorable outcome.