Cerebral venous sinus thrombosis in children: risk factors, presentation, diagnosis and outcome

Abstract

Neuroimaging and management advances require review of indications for excluding cerebral venous sinus (sinovenous) thrombosis (CSVT) in children. Our goals were to examine (i) clinical presentations of CSVT, (ii) prothrombotic risk factors and other predisposing events, (iii) clinical and radiological features of brain lesions in CSVT compared with arterial stroke, and (iv) predictors of outcome. We studied 42 children with CSVT from five European paediatric neurology stroke registries. Patients aged from 3 weeks to 13 (median 5.75) years (27 boys; 64%) presented with lethargy, anorexia, headache, vomiting, seizures, focal signs or coma and with CSVT on neuroimaging. Seventeen had prior chronic conditions; of the 25 previously well patients, 23 had recent infections, eight became dehydrated and six had both. Two children had a history compatible with prior CSVT. Anaemia and/or microcytosis (21 probable iron deficiency, five haemolytic, including two with sickle cell disease and one with beta-thalassaemia) was as common (62%) as prothrombotic disorder (13/21 screened). High factor VIII and homozygosity for the thermolabile methylene tetrahydrofolate reductase polymorphism were the commonest prothrombotic disorders. The superficial venous system was involved in 32 patients, the deep in six, and both in four. Data on the 13 children with bland infarction and the 12 with haemorrhage in the context of CSVT were compared with those from 88 children with ischaemic (AIS) and 24 with haemorrhagic (AHS) arterial stroke. In multiple logistic regression, iron deficiency, parietal infarction and lack of caudate involvement independently predicted CSVT rather than arterial disease. Five patients died, three acutely, one after recurrence and one after 6 months being quadriparetic and blind. Follow-up ranged from 0.5 to 10 (median 1) years. Twenty-six patients (62%) had sequelae: pseudotumour cerebri in 12 and cognitive and/or behavioural disabilities in 14, associated with epilepsy in three, hemiparesis in two and visual problems in two. Eighteen patients, including six with haemorrhage, were anticoagulated. Older age [odds ratio (OR) 1.54, 95% confidence limits (CI) 1.12, 2.13, P = 0.008], lack of parenchymal abnormality (OR 0.17, 95% CI 0.02, 1.56, P = 0.1), anticoagulation (OR 24.2, 95% CI 1.96, 299) and lateral and/or sigmoid sinus involvement (OR 16.2, 95% CI 1.62, 161, P = 0.02) were independent predictors of good cognitive outcome, although the last predicted pseudotumour cerebri. Death was associated with coma at presentation. Of 19 patients with follow-up magnetic resonance (MR) venography, three had persistent occlusion, associated with anaemia and longer prodrome. A low threshold for CT or MR venography in children with acute neurological symptoms is essential. Nutritional deficiencies may be modifiable risk factors. A paediatric anticoagulation trial may be required, after the natural history has been further established from registries of cases with and without treatment.

Cerebral sinovenous thrombosis in a child with homocystinuria

“…1,9 CSVT in our patient could have arisen from an interplay of dehydration, homocystinuria and anemia. Regular follow-up has been emphasized upon in order to detect recurrence of CSVT as well as development of other complications early.…”

“…Sé bire et al (2005) found that the incidence of pediatric CVST is at least 0.67 per 100,000 children per year. 1 We present an interesting case of a six-year old-boy with extensive CSVT who was diagnosed to be suffering from homocystinuria.…”

Venous sinus thrombosis leading to stroke in a patient with sickle cell disease on hydroxyurea and high hemoglobin levels: Treatment with thrombolysis

“…Two other cases one with HbSC and one with HbSS were reported as part of a registry of 42 patients with DVST [5]. A third case was reported in registry of 63 patients from North America [8].…”

“…Sickle cell disease (SCD) is increasingly recognized as a hypercoagulable state but has not been identified as a potential cause of cerebral dural venous sinus thrombosis (DVST) despite six well documented cases in the literature [1][2][3][4][5]. DVST often presents with nonspecific symptoms, the most frequent of which is severe headache, and the average delay from onset of symptoms to the diagnosis is 7 days.…”

Therapeutic approaches and advances in pediatric stroke

“…47 The most common risk factors for CSVT are dehydration and prothrombotic disorders with others including infection, trauma, cancer/ chemotherapy, and systemic disease. 47,48,49 …”