Cerebral Venous Sinus Thrombosis: Risk Factors, Diagnosis, and Outcome

Abstract

Source: Sébire G, Tabarki B, Saunders DE, et al. Cerebral venous sinus thrombosis in children: risk factors, presentation, diagnosis and outcome. Brain. 2005;128:477–489.Clinical presentations, predisposing risk factors, laboratory and radiological findings, and predictors of outcome were studied in 42 consecutive children (ages 3 weeks to 13 years [median 5.75 years]) with cerebral venous sinus thrombosis (CVST) seen between 1990 and 2002 at 5 European pediatric neurology centers with stroke registries, the majority at Great Ormond Street Hospital, London, and at Birmingham and Southampton hospitals, United Kingdom. Neonatal cases were excluded. The majority (83%) presented acutely with seizures, focal signs, and symptoms of raised intracranial pressure; 17% had a subacute onset. CVST was demonstrated on CT or MR venography. Pre-existing chronic illness in 17 (40%) patients included 4 with surgical procedures, 8 with recent infections (otitis in 3), 4 with dehydration, and 2 with lupus erythematosus. Recent infection and dehydration were triggers for CVST in 25 (59%) previously well children. Anemia (Hgb <2 SD below the mean for age) was present in 22 (52%). Seventeen anemic children had microcytosis compatible with a diagnosis of iron deficiency. A risk factor for thrombophilia was diagnosed in 18 of 29 (62%) screened, most commonly high factor VIII. Iron deficiency, parietal infarction, and lack of caudate involvement independently predicted CVST rather than arterial stroke. Follow-up ranged from 0.5 to 10 years. Five patients died, and 26 had sequelae, including pseudotumor in 12, cognitive and/or behavioral disorders in 14, epilepsy in 3, and hemiparesis in 2. Of 19 patients with follow-up MR venography, 3 had persistent occlusion associated with anemia. Independent predictors of a good cognitive outcome were older age group, use of anticoagulation therapy (18 patients), and lateral and/or sigmoid sinus involvement.Dr. Millichap has disclosed no financial relationships relevant to this commentary.CVST was once a common complication of many diseases and infections; reports in the 1930s found that one-half of cases were due to mastoid or other cranial infection, 5% were secondary to surgery, and the remainder, usually in infants, were associated with cyanotic congenital heart disease.1,2 In cases not secondary to infection, the superior longitudinal (sagittal) sinus was usually involved. After the 1960s, when infectious causes were largely preventable, CVST was diagnosed almost exclusively in infants with severe congenital heart disease.3In the above large European series, infection has resurfaced as a significant factor in CVST, while cardiac disease accounted for only 4% of patients. Infection and dehydration, anemia, iron deficiency, and prothrombotic disorders were frequent triggers or predisposing causes. In a further series of 17 cases reported recently from the Netherlands, infection was the most common cause of CVST, with mastoiditis in 10, and malignancies in 4.4 Periorbital bruising, misdiagnosed as due to assault, occurred in 2 young female adults with CVST who presented with severe generalized headache, papilledema, and raised intracranial pressure.5CVST should be suspected in children with acute neurologic symptoms, especially in patients presenting with headache who are anemic, have iron deficiency, or are suffering from recent infection, dehydration, or poor nutrition. The authors recommend early anticoagulation therapy except in comatose patients. Pseudotumor cerebri is a common sequel of CVST and may be underdiagnosed in young children7 (see AAP Grand Rounds, October 2001;6:45).