DEVELOPMENT OF A SUBDURAL VEIN-THROMBOSIS FOLLOWING AGGRESSIVE FACTOR-VII REPLACEMENT FOR POSTNATAL INTRACRANIAL HEMORRHAGE IN A HOMOZYGOUSFACTOR VII-DEFICIENT INFANT

Congenital factor VII deficiency is a rare (1:500000) autosomally rece ssive coagulopathy with variable expression and high penetration. In i nfants the most devastating presentation is that of intracranial haemo rrhage. An infant is described with severe factor VII deficiency who d eveloped postnatal intracranial haemorrhage. The baby was treated with factor VII concentrate (ImmunoA.G., Vienna, Austria). Three weeks aft er the haemorrhage he developed a dural venous sinus thrombosis. Altho ugh factor VII-deficient patients may need treatment with factor VII c oncentrate, this needs to be carefully monitored because of the thromb otic risk.