Rapidly progressive orbital intravascular papillary endothelial hyperplasia in a newborn

Intravascular papillary endothelial hyperplasia (IPEH), also known as "vegetant intravascular haemangioendothelioma", is a rare benign proliferation of vascular endothelial cells secondary to intravascular thrombosis and thrombus organisation. It can develop from vascular lesions such as haemangiomas, pyogenic granulomas or varicose veins. This vascular tumour of the skin and subcutaneous tissue may exhibit rapid and progressive growth, and is usually located in the neck or head. Ocular presentation is unusual and orbital involvement is even rarer. We report a case of a preterm newborn male with a rapidly growing left orbital mass that was histologically diagnosed as intravascular papillary endothelial hyperplasia. He was successfully managed with total excision of the tumour and propranolol therapy and remains recurrence free after an eight-year follow-up.