Abstract • 139

Fourty seven children with CNS thrombosis (males: 27, females: 20) aged 8 days - 14 years (mean age 6 years) were diagnosed between 1990 and July 1998. Twenty out 47 children (42%) had ischaemic infarcts 16 (34%) arterial thrombosis and 11 (23,4%) sinus thrombosis. Thrombosis was documented with one or more imaging techniques (CTscan, MRI, MRI angiography, transcranial doppler). Extensive thrombosis was observed in 4 (8.5%), thrombosis in multiple sites in 7 (14.9%) and recurrent thrombosis in three cases (6.4%). Underlying disease was documented in 20 patients (42.5%) and a triggering event in seven (14.8%). Although the laboratory investigation for predisposing factors to thrombosis was not complete in all patients, an abnormality was found in 20 cases, six of which were congenital (FV Leiden: 2, APCR: 2, mutant FII: 1, homozygous homocystinaemia: 1). Reevaluation of children away from the thrombotic event showed that the deficiency in ATIII, PC, PS or Pig identified in 13 patients was transient. All but five children with CNS infarcts or arterial thrombosis were managed conservatively, whereas 6/11 children with sinus thrombosis were effectively treated with rTPA or/and heparin followed by warfarin. Four out of 49 patients (8.5%) succumbed, three as a result of thrombosis. The clinical outcome was good in 13/47 cases (27.7%), while 18/47 patients (38.3%) showed neurological sequelae (intracranial hypertension: 3, movement disorders or and convulsions: 15).The clinical outcome was unknown in 12 patients. In radiological re-evaluation of 21 children, complete lysis of thrombus was documented in 13 cases (61,9%) partial lysis in two(9.5%) end no change in 6 cases (28.5%).