Rare condition in pediatric population: 0.34–0.67 cases/100.000/year
- Affects primarily neonates
- Results in neurologic impairment or death in approximately half the cases
- Occurrence of venous infarcts or seizures portends a poor outcome
- Increase in diagnostic frequency due to more sensitive and safe radiological exams
- Greater clinical awareness of the condition necessary
Predisposing Factor Is Often Present
- Infections, dehydration, anemia, fever, hypoxic-ischemic injury
- Head and neck infections (otitis media and mastoiditis, meningitis, sinusitis)
- Head injury, post intracranial surgery
- Heart disease, nephrotic syndrome, malignancy
- Drugs (corticosteroids, L-asparaginase, oral contraceptives)
Clinical Features: Varying Symptoms
- Depressed level of consciousness and coma
- Nausea and vomiting
- Visual impairment
- Neurological deficits
- Neonates: seizures and diffuse neurologic signs
- Diagnosis: Lack of flow in the cerebral veins
- Methods of choice for investigation: CT venography or MRI with venography
- Well tolerated by children and neonates (in the absence of any contraindication).
- During the acute phase, anticoagulation is probably effective in reducing the risk of death and sequelae.
- Anticoagulation is also effective in reducing the risk of recurrence.
- Duration of anticoagulation needs to be individually tailored.
- Prolonged treatment over 3-6 mo is justified according to individual factors.
Pretreatment intracranial hemorrhage: requires more careful consideration
- Pathophysiology of hemorrhage in venous infarction involves venous/capillary hypertension and erythrocyte diapedesis or frank hemorrhage.
- By preventing new thrombus formation, anticoagulation enables unopposed fibrinolysis to dissolve thrombi, relieving venous congestion.
- Therefore, the potential ability of anticoagulant therapy to reduce intracranial hemorrhage caused by severe or persistent thrombosis may balance the risks of anticoagulant therapy dependent bleeding.
- deVeber G, Andrew M, Adams C, et al. Cerebral sinovenous thrombosis in children. N Engl J Med 2001;345:417-23.
- Barnes C, Newall F, Furmedge J, et al. Cerebral sinus venous thrombosis in children. J Pediatr Child Health 2004;40:53-5.
- Grunt S, Wingeier K,Wehrli E, et al. Cerebral sinus venous thrombosis in Swiss children. Dev Med Child Neurol 2010;52:1145-50.
- deVeber G, Chan A, Monagle P, et al. Anticoagulation Therapy in Pediatric Patients With Sinovenous Thrombosis. Arch Neurol 1998;55:1533-1537.
- Dlamini N, Billinghurst L, Kirkham FJ. Cerebral Venous Sinus (Sinovenous) Thrombosis in Children. Neurosurg Clin N Am 2010;21:511-527
- Guenther G, Arauz A. Cerebral venous thrombosis: A diagnostic and treatment update. Neurologia. 2011;26:488–98.
- Moharir M, Shroff M, Stephens D. Anticoagulants in Pediatric Cerebral Sinovenous Thrombosis. A Safety and Outcome Study. Ann Neurol 2010;67:590-599
Patient Case Video