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''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.''
''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==
[[File:Cerebral veins.jpg|thumbnail|Cerebral Veins]]
*Occlusion of venous sinus (most commonly superior sagittal and lateral sinuses) by thrombus<ref>Piazza G. Cerebral venous thrombosis. Circulation 2012;125:1704-1709.</ref>
*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.<ref name="lancet">Bousser MG, Ferro JM. Cerebral venous thrombosis: an update. Lancet Neurol 2007; 6:162-70.</ref>
*Median Age ~ 37 years<ref name="lancet"></ref>
*Female:Male ratio 3:1<ref name="lancet"></ref>


== Background ==
===Predisposing factors===
*Occlusion of venous sinus (most commonly superior sagittal and lateral sinuses) by thrombus or compression from mass
*Mainly a diverse group of hypercoagulable states
*No precise prevalence or incidence established due to rarity of condition
*Median Age ~ 37 years
*Female:Male ratio 3:1
*Predisposing factors
**Cancer
**Cancer
**Pregnancy
***Leukemia
**Local infections (otitis media, sinusitis, cellulitis)
***Adenocarcinoma
**Hypercoagulable states
**[[Pregnancy]] (up to 6 weeks post-partum)
**Trauma
**Polycythemia Vera
**Drugs (ecstasy, androgens, OCPs)
**Sickle cell
**Compression of venous sinus (tumor, abscess)
**Factor V Lediden
**Protein S & C deficiency
**Hyperhomocysteinemia
**Behcets syndrome
*Severe dehydration in infants and children, mainly in developing countries.
*COVID-19 vaccines (rare)<ref>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</ref>
**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 problems|dental infections]])
**Mastoiditis common cause in developing countries.
*Direct cranial [[Trauma]] & neurosurgical procedures
*Compression of venous sinus ([[brain tumor|tumor]], [[brain abscess|abscess]])


== Clinical Features ==
==Clinical Features==
*Clinical presentation varies depending on location, acuity, and severity of thrombosis
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  
**More gradual onset of symptoms or thrombosis allows for compensatory collateral venous system to develop  
===Common Symptoms===
**Common Symptoms:
''Symptoms are variable and may not all be present<ref name="NEJM>Stam J. Thrombosis of the cerebral veins and sinuses. N Engl J Med 2005;352:1791–8.</ref>
***Headache 74-92%
*[[Headache]] 74-92%
***Seizures 35-50%
**Usually progresses during a period of hours or days to severe aching or throbbing over the entire cranium, bifrontally, or at the vertex.
***Papilledema 28-45%
***Pain at the vertex of the cranium is infrequent but more specific.<ref>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</ref>
***Focal Neurologic sequelae (seizures, dizziness) 25-71%
**Coughing, bending over, or head movements tend to worsen headaches.
***Encephalopathy
*[[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]]
[[File:sinus thrombosis.jpg|thumbnail]]


== Diagnosis ==
===[[focal neuro deficits|Neurodefecits]]===
*Suspect in patients presenting with headache, signs of increased ICP, or focal neurologic deficits in setting of any of above predisposing factors
Although presentation can be highly variable, neuro deficits can be correlated with the location of the occlusion<ref name=NEJM></ref>
*'''Superior Sagital sinus'''
**[[weakness|motor deficits]]
***Paresis of one or both legs or hemiparesis from infarction in the frontoparietal regions surrounding the vein of Trolard<ref>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</ref>
**Seizures
*'''Left transverse sinus'''
**Aphasia and confusion from infarction in the temporal lobe surrounding the vein of Labbe<ref>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</ref>
*'''[[cavernous sinus thrombosis|Cavernous sinus]]'''
**[[eye pain|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.
*'''Deep venous sinus'''
**Drowsiness or stupor from bilateral thalamic dysfunction and obstructive hydrocephalus.


== Work-Up ==
==Differential Diagnosis==
*Imaging
{{Headache DDX}}
**MRI/MRV considered diagnostic study of choice, now considered gold standard
**CT venography is a reasonable alternative
***Has been found to have similar sensitivity to MRV in recent studies
**Non contrast CT possesses insufficient sensitivity or specificity to be of diagnostic value in the setting of high clinical suspicion
***May see "Delta sign" dense triangle in superior sagittal sinus
*Labs
**D-Dimer has been proposed as a possible screening tool in low risk patients, but still needs further investigation


== DDx ==
==Evaluation==
[[Headache DDX]]
[[File:Sagital sinus thrombus.JPG|thumbnail|Sagital sinus thrombosis on CT]]
''Suspect in patients with headache, signs of increased ICP, or focal neurologic deficits, especially if any of above predisposing factors are present''
===Imaging===
*[[Brain MRI|MRI]] and MRV are considered diagnostic study of choice<ref name="rads">Khandelwal N et al. Comparison of CT venography with MR venography in cerebral sinovenous thrombosis. AJR Am J Roentgenol 2006;187:1637–1643.</ref>
*CT venography is a reasonable alternative if there is a contraindication to MRV and may have a similar sensitivity to MRV in recent studies<ref name="rads"></ref><ref>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.</ref>
**May see "Empty delta sign" dense triangle in superior sagittal sinus<ref>Lee Emil J. Y. “The Empty Delta Sign.” Radiology. 224(3). 2002. 788-789.</ref>
**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. <ref>Ram K. P. Vijay. "The Cord Sign."Radiology. 2006; 240:299-300.</ref>
**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. <ref>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. </ref>
*Non contrast [[head CT|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)


== Treatment ==
===Labs===
*Due to the low incidence of disease, evidence-based recommendations on treatment are lacking
*[[D-Dimer]] is not a reliable test to rule out a cerebral venous thrombosis<ref>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.</ref><ref>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</ref>
*Currently accepted that treatment of choice is anticoagulation with heparin or low molecular weight heparin, with eventual transition to oral anticoagulation for a 3-6 month duration
*In patients with a concern for [[meningitis]] then pursue diagnosis via standard workup which includes a [[CT before lumbar puncture]]
*Seizure prophylaxis should be considered in patients with CVT presenting with seizures
*Supportive care and close monitoring for increased ICP with consideration of hemicraniectomy for acute decompensation
*Intravascular thrombolytics can be considered in patients who acutely decompensate, have symptoms of increased ICP, or decreased level of consciousness


== Disposition ==
==Management==
*All patients with confirmed CVT should be admitted to a level of care capable of frequent neurologic monitoring
===Anticoagulation===
*Patients should be started on anticoagulation if there are no contraindications, and transitioned to oral therapy and continued for 3-6 months
*[[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]]<ref>Ferro JM et al. Early seizures in cerebral vein and dural sinus thrombosis: risk factors and role of antiepileptics. Stroke 2008;39:1152–1158.</ref>
*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<ref>38.Dentali F et al. Safety of thrombolysis in cerebral venous thrombosis: a systematic review of the literature. Thromb Haemost 2010;104:1055–1062.</ref>
 
==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==
==See Also==
*[[Headache]]
*[[Headache]]
*[[Head CT]]
*[[Head CT]]
*[[Cavernous sinus thrombosis]]


== Source ==
==References==
*Rosen's
<references/>
*Uptodate
[[Category:Neurology]]
 
[[Category:Neuro]]

Latest revision as of 20:32, 29 July 2021

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

Cerebral Veins
  • 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
  • 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.
  • 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
Sinus thrombosis.jpg

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
  • 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.
  • Deep venous sinus
    • Drowsiness or stupor from bilateral thalamic dysfunction and obstructive hydrocephalus.

Differential Diagnosis

Headache

Common

Killers

Maimers

Others

Aseptic Meningitis

Evaluation

Sagital sinus thrombosis on CT

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

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

  1. Piazza G. Cerebral venous thrombosis. Circulation 2012;125:1704-1709.
  2. 2.0 2.1 2.2 Bousser MG, Ferro JM. Cerebral venous thrombosis: an update. Lancet Neurol 2007; 6:162-70.
  3. 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. 4.0 4.1 Stam J. Thrombosis of the cerebral veins and sinuses. N Engl J Med 2005;352:1791–8.
  5. 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
  6. 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
  7. 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. 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.
  9. 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.
  10. Lee Emil J. Y. “The Empty Delta Sign.” Radiology. 224(3). 2002. 788-789.
  11. Ram K. P. Vijay. "The Cord Sign."Radiology. 2006; 240:299-300.
  12. 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.
  13. 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.
  14. 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
  15. Ferro JM et al. Early seizures in cerebral vein and dural sinus thrombosis: risk factors and role of antiepileptics. Stroke 2008;39:1152–1158.
  16. 38.Dentali F et al. Safety of thrombolysis in cerebral venous thrombosis: a systematic review of the literature. Thromb Haemost 2010;104:1055–1062.