Abstract and Introduction
Abstract
Objectives We evaluated clinical features, treatment practices and early outcome in a multicentre cohort of children with cerebral sinovenous thrombosis (CSVT).
Methods Children with CSVT from 10 countries were enrolled from January 2003 to July 2007 in the International Paediatric Stroke Study. We analysed clinical symptoms, underlying conditions, antithrombotic treatment and neurological outcome at hospital discharge in 170 children.
Results Of 170 children enrolled, 60% were male; median age 7.2 years (IQR 2.9–12.4). Headache, altered consciousness, focal deficits and seizures were common presenting clinical features. Infarction affected 37% and intracranial haemorrhage 31%. Risk factors included chronic disease in 50%; acute systemic illness or head/neck disorders 41%; prothrombotic state 20% and other haematological abnormality 19%. Discharge neurological status was normal in 48%, abnormal in 43% and unknown in 5%. Antithrombotic therapy was common, most often low molecular weight heparin was common, with significant regional variation in treatment practices. Mortality was low (4%) and was associated with no anticoagulation but not underlying chronic disease, anatomic extent of thrombosis or intracranial haemorrhage. Abnormal neurological status at discharge or death was associated with decreased level of consciousness at presentation and the presence of an identified prothrombotic state.
Conclusions Our study extends the observations of previously published smaller studies in children with CSVT that this is a morbid disease with diverse underlying causes and risk factors. Divergent treatment practices among highly specialised centres as well as limited data on treatment efficacy and safety suggest that further study of this condition is warranted.
Introduction
Cerebral sinovenous thrombosis (CSVT), defined as blood clot in a vein carrying blood from the brain to the heart, affects 0.34–0.67/100 000 children annually, of which 27%–35% are neonates. Mortality is 3%–12%, and neurological sequelae affect up to 62% of survivors. Aetiology in the paediatric population includes a wide spectrum of underlying conditions and symptoms at clinical presentation. Recent studies suggest that haematological abnormalities, including prothrombotic disorders and iron deficiency, contribute to development of CSVT. Previously published cohort studies have described epidemiology, treatment practices and outcomes, but are limited due to small sample size or single-centre enrolment. The International Paediatric Stroke Study (IPSS) established a prospective multicentre registry to characterise epidemiology, treatment and outcome of infants and children with arterial ischaemic stroke (AIS) and CSVT. The objective of this paper is to analyse this large multicentre international data set for clinical presentation, underlying diseases and provoking conditions, treatment and outcome at hospital discharge among children with CSVT and to define risk factors for death and abnormal neurological status at hospital discharge.