Development of a subdural vein thrombosis following aggressive factor VII replacement for postnatal intracranial haemorrhage in a homozygous factor VII-deficient infant.

Congenital factor VII deficiency is a rare (1:500,000) autosomally recessive coagulopathy with variable expression and high penetration. In infants the most devastating presentation is that of intracranial haemorrhage. An infant is described with severe factor VII deficiency who developed postnatal intracranial haemorrhage. The baby was treated with factor VII concentrate (ImmunoA.G., Vienna, Austria). Three weeks after the haemorrhage he developed a dural venous sinus thrombosis. Although factor VII-deficient patients may need treatment with factor VII concentrate, this needs to be carefully monitored because of the thrombotic risk.