Cerebral venous thrombosis
(Redirected from Cerebral venous sinus thrombosis)
The cavernous sinus is one of the several cerebral veins and cavernous sinus thrombosis is a specific type of cerebral venous (sinus) thrombosis. See that article for a discussion of that specific clinical entity.
Background
- Occlusion of venous sinus (most commonly superior sagittal and lateral sinuses) by thrombus[1]
- No precise prevalence or incidence established due to rarity of condition. However the disease is more prevalent in patients with thrombophilia, oral contraceptive use, and during pregnancy.[2]
- Median Age ~ 37 years[2]
- Female:Male ratio 3:1[2]
Predisposing factors
- Mainly a diverse group of hypercoagulable states
- Cancer
- Leukemia
- Adenocarcinoma
- Pregnancy (up to 6 weeks post-partum)
- Polycythemia Vera
- Sickle cell
- Factor V Lediden
- Protein S & C deficiency
- Hyperhomocysteinemia
- Behcets syndrome
- Cancer
- Severe dehydration in infants and children, mainly in developing countries.
- COVID-19 vaccines (rare)[3]
- 1 per 1,000,000 for the Ad26.COV2.S (Johnson & Johnson And Janssen brands)
- 1 per 100,000 for the ChAdOx1 nCoV-19 (AstraZeneca brand)
- Local infections (otitis media, sinusitis, cellulitis, dental infections)
- Mastoiditis common cause in developing countries.
- Direct cranial Trauma & neurosurgical procedures
- Compression of venous sinus (tumor, abscess)
Clinical Features
Clinical presentation varies depending on location, acuity, and severity of thrombosis. More gradual onset of symptoms or thrombosis allows for compensatory collateral venous system to develop
Common Symptoms
Symptoms are variable and may not all be present[4]
- Headache 74-92%
- Usually progresses during a period of hours or days to severe aching or throbbing over the entire cranium, bifrontally, or at the vertex.
- Pain at the vertex of the cranium is infrequent but more specific.[5]
- Coughing, bending over, or head movements tend to worsen headaches.
- Usually progresses during a period of hours or days to severe aching or throbbing over the entire cranium, bifrontally, or at the vertex.
- Seizures 35-50%
- Usually focal convulsions
- Papilledema 28-45%
- Focal neurologic sequelae
- Occurs hours to days after headache in about half of patients
- dizziness) 25-71%
- Encephalopathy
Neurodefecits
Although presentation can be highly variable, neuro deficits can be correlated with the location of the occlusion[4]
- Superior Sagital sinus
- motor deficits
- Paresis of one or both legs or hemiparesis from infarction in the frontoparietal regions surrounding the vein of Trolard[6]
- Seizures
- motor deficits
- Left transverse sinus
- Aphasia and confusion from infarction in the temporal lobe surrounding the vein of Labbe[7]
- Cavernous sinus
- ocular pain, proptosis, oculomotor palsies
- CN palsy of 3,4, and 6 as well as the ophthalmic and maxillary divisions of the 5th cranial nerve.
- ocular pain, proptosis, oculomotor palsies
- Deep venous sinus
- Drowsiness or stupor from bilateral thalamic dysfunction and obstructive hydrocephalus.
Differential Diagnosis
Headache
Common
Killers
- Meningitis/encephalitis
- Myocardial ischemia
- Retropharyngeal abscess
- Intracranial Hemorrhage (ICH)
- SAH / sentinel bleed
- Acute obstructive hydrocephalus
- Space occupying lesions
- CVA
- Carbon monoxide poisoning
- Basilar artery dissection
- Preeclampsia
- Cerebral venous thrombosis
- Hypertensive emergency
- Depression
Maimers
- Giant cell arteritis of temporal artery (temporal arteritis)
- Idiopathic intracranial hypertension (Pseudotumor Cerebri)
- Acute Glaucoma
- Acute sinusitis
- Cavernous sinus thrombosis or cerebral sinus thrombosis
- Carotid artery dissection
Others
- Mild traumatic brain injury
- Trigeminal neuralgia
- TMJ pain
- Post-lumbar puncture headache
- Dehydration
- Analgesia abuse
- Various ocular and dental problems
- Herpes zoster ophthalmicus
- Herpes zoster oticus
- Cryptococcosis
- Febrile headache (e.g. pyelonephritis, nonspecific viral infection)
- Ophthalmoplegic migraine
- Superior Vena Cava Syndrome
Aseptic Meningitis
- Viral
- Tuberculosis
- Lyme disease
- Syphilis
- Leptospirosis
- Fungal (AIDS, transplant, chemotherapy, chronic steroid use)
- Noninfectious
Evaluation
Suspect in patients with headache, signs of increased ICP, or focal neurologic deficits, especially if any of above predisposing factors are present
Imaging
- MRI and MRV are considered diagnostic study of choice[8]
- CT venography is a reasonable alternative if there is a contraindication to MRV and may have a similar sensitivity to MRV in recent studies[8][9]
- May see "Empty delta sign" dense triangle in superior sagittal sinus[10]
- Cord sign: thrombus in the cerebral sinus may appear as a hyperattenuated foci. It is homogenous in nature and appears linear or round based on the affected sinus. This is most commonly seen in the first week. [11]
- Vein Sign: After two weeks, the thrombus becomes hypoattenuated. When the thrombus is located in the deep vein it is referred to as the vein sign. [12]
- Non contrast CT possesses insufficient sensitivity or specificity to be of diagnostic value in the setting of high clinical suspicion
- May see "dense delta sign" (hyperattenuation consistent with thrombus in the posterior sagittal sinus)
Labs
- D-Dimer is not a reliable test to rule out a cerebral venous thrombosis[13][14]
- In patients with a concern for meningitis then pursue diagnosis via standard workup which includes a CT before lumbar puncture
Management
Anticoagulation
- Heparin or low molecular weight heparin (Grade 1C)
- Of note, heparin initial bolus is 3000-5000U, lower than the dosing for PE/DVT
- Following the acute phase, patients should transition to oral anticoagulation for a 3-6 month duration
- Warfarin is recommended as oral anticoagulation of choice
- There is a controversy regarding the use of direct oral anticoagulants. However, findings from the RE-SPECT CVT trial which was published recently and compared warfarin to dabigatran, suggest that both agents have similar effectiveness and safety for preventing recurrent CVT.
Seizure prophylaxis
- Recommended for patients with seizure at presentation PLUS focal cerebral lesion (edema, infarction or hemorrhage on CT/MRI) (Grade 1B)
- Only required if the patient has a seizure[15]
- Prophylaxis with antiepileptic is NOT required if the patient has a single seizure with no signs of supratentorial cerebral lesion.
Supportive care
- Frequent neurologic checks and clinical monitoring for increased ICP
- Neurology or neurosurgical consultation depending on institutional resources
Acute Decompensation
- If persistent or severe elevated intracranial pressure a hemicraniectomy may be necessary
- Intravascular thrombolytics (either catheter guided or systemic) in coordination with neurology may be required if the patient experiences rapidly progressing decompensation[16]
Disposition
- Admission
- To a level of care capable of frequent neurologic monitoring. Inpatient, the patient should also have a evaluation for a coagulopathy.
See Also
References
- ↑ Piazza G. Cerebral venous thrombosis. Circulation 2012;125:1704-1709.
- ↑ 2.0 2.1 2.2 Bousser MG, Ferro JM. Cerebral venous thrombosis: an update. Lancet Neurol 2007; 6:162-70.
- ↑ Ropper, A. H., & Klein, J. P. (2021). Cerebral Venous Thrombosis. New England Journal of Medicine, 385(1), 59–64. https://doi.org/10.1056/nejmra2106545
- ↑ 4.0 4.1 Stam J. Thrombosis of the cerebral veins and sinuses. N Engl J Med 2005;352:1791–8.
- ↑ Ropper, A. H., & Klein, J. P. (2021). Cerebral Venous Thrombosis. New England Journal of Medicine, 385(1), 59–64. https://doi.org/10.1056/nejmra2106545
- ↑ Ropper, A. H., & Klein, J. P. (2021). Cerebral Venous Thrombosis. New England Journal of Medicine, 385(1), 59–64. https://doi.org/10.1056/nejmra2106545
- ↑ Ropper, A. H., & Klein, J. P. (2021). Cerebral Venous Thrombosis. New England Journal of Medicine, 385(1), 59–64. https://doi.org/10.1056/nejmra2106545
- ↑ 8.0 8.1 Khandelwal N et al. Comparison of CT venography with MR venography in cerebral sinovenous thrombosis. AJR Am J Roentgenol 2006;187:1637–1643.
- ↑ Rodallec MH, Krainik A, Feydy A et-al. Cerebral venous thrombosis and multidetector CT angiography: tips and tricks. Radiographics. 2006;26 Suppl 1 (suppl_1): S5-18.
- ↑ Lee Emil J. Y. “The Empty Delta Sign.” Radiology. 224(3). 2002. 788-789.
- ↑ Ram K. P. Vijay. "The Cord Sign."Radiology. 2006; 240:299-300.
- ↑ Linn J, Pfefferkorn T. "Noncontrast CT in Deep Cerebral Venous Thrombosis and Sinus Thrombosis: Comparison of Its Diagnostic Value for Both Entities."AJNR Am J Neuroradiology. 2010; 30: 728-735.
- ↑ Crassard I, Soria C, Tzourio C, Woimant F, Drouet L, Ducros A, Bousser MG. A negative D-dimer assay does not rule out cerebral venous thrombosis: a series of seventy-three patients. Stroke 2005;36:1716 –1719.
- ↑ Kristoffersen ES, Harper CE, Vetvik KG, Faiz KW. Cerebral venous thrombosis – epidemiology, diagnosis and treatment. Cerebral venetrombose – forekomst, diagnostikk og behandling. Tidsskr Nor Laegeforen. 2018;138(12):10.4045/tidsskr.17.1047. Published 2018 Aug 20. doi:10.4045/tidsskr.17.1047
- ↑ Ferro JM et al. Early seizures in cerebral vein and dural sinus thrombosis: risk factors and role of antiepileptics. Stroke 2008;39:1152–1158.
- ↑ 38.Dentali F et al. Safety of thrombolysis in cerebral venous thrombosis: a systematic review of the literature. Thromb Haemost 2010;104:1055–1062.