MRV with finding of sigmoid sinus thrombosis
Cerebral Venous Sinus Thrombosis
Epidemiology
- Annual incidence ~ 1 per 100,000
- Female to male ratio of 3:1.
- Secondary to pregnancy (and the early postpartum period) and the prothrombotic effects of oral contraceptives and hormone replacement therapy.
Pathophysiology
- Cerebral vein and/or dural sinus thrombosis obstructs drainage of blood and CSF, which increases venous and capillary pressures and intracranial pressure.
- These increased pressures lead to reduced CSF reabsorption and blood-brain barrier breakdown with resultant venous infarcts, hemorrhage and cerebral edema (severe vasogenic edema, early, and cytotoxic edema with infarction).
Risk Factors and Causes
- Genetic Thrombophilias
- Prothrombin G20210A
- Factor V Leiden
- Antithrombin deficiency
- Protein C deficiency
- Protein S deficiency
- Hyperhomocysteinemia (MTHFR gene mutation for methylenetetrahydrofolate reductase)
- Acquired Thrombophilias
- Pregnancy and the early postpartum period
- Oral contraceptives and hormone replacement therapy
- Malignancy
- Infection
- Traumatic causes (uncommon)
- Direct head trauma
- Lumbar puncture
Clinical Presentation
Highly varied onset: acute, subacute, or chronic
- Headache
- Commonly the presenting symptom
- Can be gradual or sudden in onset
- Associated intracranial hypertension
- Intracranial hypertension
- Headache that worsens with Valsalva and supine position
- Visual obscuration
- Physical exam findings of papilledema and reduction in blind spots
- Cognitive dysfunction
- Personality changes
- Delirium
- Isolated abnormalities (eg, language deficits)
- Focal motor/sensory/visual deficits
- Seizures
- Encephalopathy
Imaging Characteristics
Head CT
- Intracranial hemorrhage and edema
- Dense clot sign (aka cord sign)
- Thrombosed cortical vein
- Dense triangle sign
- Posterior superior sagittal sinus contains a triangular or round shape hyperdensity.
Contrast enhancement
- Empty delta sign
- Posterior superior sagittal sinus contains a central region that lacks contrast enhancement.
MRI
- Focal areas venous infarction
- Absence of flow void
- Infarcts may include intracerebral hemorrhage
- Atypical infarction (crosses typical arterial boundaries)
- Superior sagittal sinus thrombosis produces small (< 2 cm) juxtacortical hemorrhages
- Diffuse vasogenic edema
MRI Venography or CT Venography
- Dural thrombus
- Cortical vein thrombosis
Laboratory Studies
- Routine studies
- CBC (looking for infection or thrombocytosis)
- BMP
- PT/INR, PTT (looking for coagulopathy)
- Hypercoagulopathy evaluation
- Evaluation as clinically appropriate
- D-dimer (positive test may support the diagnosis)
- CSF Studies
- Routine studies to assess for meningitis
- Opening pressure to assess for intracranial hypertension
- Contraindicated when there is increased risk of herniation.
Treatment
- Anticoagulation therapy
- Whether or not hemorrhage is present, it is recommended to give low molecular weight heparin (LMWH) or intravenous heparin.
- Minimum duration in all patients is 3 months but duration ultimately depends on clinical circumstance and range may be 3 months to lifelong, depending on the clinical scenario.
- Treat any increased intracranial pressure
- Antiepileptic therapy, if appropriate
MRV and Head CT Images of sigmoid sinus thrombosis from:
- P Thomas, A Keightley, R Kamble, N Gunasekera, M R Johnson. Sigmoid sinus thrombosis presenting with posterior alexia in a patient with Behcet's disease and polycythaemia: a case report. Journal of Medical Case Reports 2008, 2:175 (23 May 2008)
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