Hepatic haemangioendothelioma: a vertical association with biliary atresia?

Haemangioendotheliomas (HAE) are the commonest vascular tumours of the liver in infancy and are characterised by solitary or multifocal lesions often arising in conjunction with cutaneous haemangiomas. The authors report on a 4-month-old infant with massive hepatomegaly and hypothyroidism due to multifocal HAE whose mother had been born with biliary atresia. As the incidence of biliary atresia in the UK is about 1 in 16 000 live births, the association reported here seems more than co-incidental. This report reviews the literature and discusses potential aetiologies to explain this rare association.