Fatal course of veno-occlusive disease of the liver (Endophlebitis hepatica obliterans) in a preterm infant

We describe the fatal course of a preterm infant of 34 weeks' gestation wit h veno-occlusive disease of the liver and refractory ascites. Despite aggre ssive medical management, the baby died twenty-two hours post partum becaus e of cerebral haemorrhage before potentially life-saving organ transplantat ion could take place. At autopsy, paucity of lymphoid tissue in lymph nodes , thymus, spleen, and gastrointestinal tract were also seen. To our knowled ge, this is the youngest infant with veno-occlusive disease of the liver re ported in the literature.