II. Epidemiology

  1. Accounts for 0.5 to 1% of all Cerebrovascular Accidents
  2. Female gender in two thirds of cases
  3. Younger patients (mean age 33 years old)

III. Pathophysiology

  1. See Cerebral Sinus
  2. As with other Venous Thromboembolism, virchow's triad applies to pathogenesis (stasis, vessel wall, Coagulopathy)
  3. Venous obstruction causes increased venous pressure and decreased capillary perfusion
    1. Initial compensation with venous dilation including collateral veins
    2. Progresses to vasogenic edema and decreased Cerebral Perfusion Pressure
    3. Cerebrospinal fluid absorption may also be blocked, increasing Intracranial Pressure
  4. Images
    1. cnsVenousSinus.jpg

IV. Risk Factors

  1. Hormonal
    1. Oral Contraceptives
    2. Hormone Replacement Therapy
    3. Pregnancy or Postpartum State (up to 6 weeks after delivery)
  2. Hypercoagulable Conditions or Stasis
    1. Malignancy (esp. Hematologic Malignancy, myeloproliferative disorder)
    2. Inflammatory Conditions (e.g. Systemic Lupus Erythematosus, Inflammatory Bowel Disease, Behcet's Disease)
    3. Thyroid Disease
    4. Antiphospholipid Antibody Syndrome
    5. Hyperhomocysteinaemia (MTHFR gene mutation)
    6. Dehydration
  3. Inflammatory or Structural Conditions
    1. Head and Neck Infections
    2. Central Nervous System Infections
    3. Recent neurosurgery
    4. Closed Head Injury
    5. Dural AV Fistula
    6. Cytotoxic drugs

V. Precautions

  1. Early diagnosis is key to good prognosis
  2. Frequently missed diagnosis
    1. Mimics other acute neurologic conditions
    2. Requires specific testing in most cases (venogram)

VI. Findings: General

  1. Findings are specific to venous sinus involved
  2. Headache (90% of cases, and only symptom in 25% of cases)
    1. New or different Headache
    2. Acute to insidious onset progressive over hours to days
    3. Provoked when Increased Intracranial Pressure (e.g. valsalva, coughing)
  3. Increased Intracranial Pressure
    1. Papilledema
    2. Visual changes
    3. Nausea or Vomiting
  4. Focal Findings
    1. Motor deficits
    2. Seizure
  5. Encephalopathy (esp. deep sinus involvement)
    1. Altered Mental Status

VII. Findings: Transverse Sinus Thrombosis

  1. Accounts for 44 to 73% of cases
  2. Isolated, noninfectious unilateral thrombosis
    1. Symptoms may be mild (e.g. Headache) if no infarction
    2. Seizures
    3. Contralateral Hemiparesis, hyperreflexia, or spasticity (pyramidal symptoms) may be present
  3. Left Transverse Sinus (with venous infarction, occluded vein of Labbe)
    1. Aphasia
  4. Contiguous Sinus extension (e.g. Superior Sagittal Sinus)
    1. Increased Intracranial Pressure (Intracranial Hypertension)
    2. Altered Level of Consciousness
    3. Cranial Nerve Palsy (CN 9-12)
  5. Cerebral Vein Extension
    1. Headache
    2. Vomiting
    3. Ataxia
    4. Limb Incoordination

VIII. Findings: Superior Sagittal Sinus Thrombosis

  1. Accounts for 39-62% of cases
  2. Increased Intracranial Pressure (Intracranial Hypertension)
    1. Isolated in many cases
  3. Focal venous infarction related symptoms
    1. Headache
    2. Blurred Vision, Vision Loss or Hemianopsia (Visual Field deficit)
    3. Nausea or Vomiting
    4. Cranial Nerve Palsy
    5. Aphasia
    6. Hemiparesis or hemi-sensory loss
    7. Seizures

IX. Findings: Sigmoid Sinus Thrombosis

  1. Accounts for 40-47% of cases
  2. Mastoid region pain
  3. Cranial Nerve Deficit (CBN 6-8)

X. Findings: Deep Venous Cerebral Thrombosis (e.g. Great Cerebral Vein of Galen)

  1. Accounts for 10-11% of cases
  2. Altered Mental Status (encephalopathy to coma)
  3. Motor deficits
    1. Fluctuating or alternating paresis (or bilateral)

XI. Findings: Cortical Vein Thrombosis (Superficial Cerebral Vein Thrombosis)

  1. Accounts for 3-17% of cases
  2. Thrombosis involving superficial veins (superficial middle and anastomotic cerebral veins)
  3. Seizures
  4. Focal neurologic deficits depending on distribution of thrombosis

XII. Findings: Cavernous Sinus Thrombosis

  1. See Cavernous Sinus Thrombosis
  2. Accounts for 1-2% of cases
  3. Headache
  4. Eye Pain
  5. Chemosis
  6. Proptosis
  7. Cranial Nerve Palsy (CN 3, 4, and 6, as well as opthalmic branch CN 5)
  8. Fever (if septic Thrombophlebitis)

XIII. Imaging

  1. Non-Contrast Head CT
    1. Low Test Sensitivity for Cerebral Venous Thrombosis (~33%)
    2. Findings consistent with Cerebral Venous Thrombosis
      1. Delta Sign
        1. Posterior Superior Sagittal Sinus hyperdensity
      2. Venous Cerebral Infarction
        1. Infarct spans more than one arterial perfusion regions
      3. Diffuse cerebral edema
      4. Hydrocephalus
      5. Subarachnoid Hemorrhage (secondary)
  2. CT Venogram (with CT Head)
    1. Gold standard study for venous cerebral thrombosis
    2. Similar efficacy to MRV except in Altered Level of Consciousness or encephalopathy
  3. Magnetic Resonance Venogram (MRI/MRV)
    1. As with CT Venogram, gold standard for Cerebral Venous Thrombosis diagnosis
    2. MRV is preferred over CT venogram for patients with Altered Level of Consciousness or encephalopathy
      1. Suggests possible deep cerebral vein thrombosis (better visualized on MRV)
    3. General Findings
      1. DWI hyperintense
      2. Cerebral venous wall enhancement
      3. Thrombosed sinuses with decreased or absent flow
    4. Findings vary by timing from onset
      1. Week 1: T1W/T2W isointense to hypointense
      2. Week 2: T1W/T2W hyperintense

XIV. Management

  1. Consult Neurosurgery and Stroke Neurology
  2. Initiate Low Molecular Heparin (e.g. Lovenox) which is preferred over Unfractionated Heparin
    1. Initiate Anticoagulation with Warfarin with INR target 2-3 for 3-12 months
      1. Limited evidence for DOACs in CVT as of 2020, and therefore Warfarin is preferred
    2. First episode of CVT: 3-6 months (6-12 months if no known risk factor)
    3. Continue Anticoagulation lifelong for recurrent CVT
  3. Endovascular intervention considered in decompensating or refractory cases
  4. Manage Increased Intracranial Pressure
    1. Acute monitoring by neurosurgery if risk of Increased Intracranial Pressure
  5. Manage Seizures
    1. Antiepileptic drugs are indicated for clinical evidence of Seizures and are continued for >=1 year

XV. Prognosis

  1. Recurrent Venous Thrombosis risk off of Anticoagulation
    1. Recurrent Cerebral Venous Thrombosis: 2-7% per year
    2. Recurrent Venous Thromboembolism (e.g. DVT, PE): 4-7% per year

XVI. References

  1. Marcolini and Swaminathan in Herbert (2021) EM:Rap 21(2): 5-7
  2. Ulivi (2020) Pract Neurol 20:356-67 [PubMed]

Images: Related links to external sites (from Bing)

Related Studies

Ontology: Thrombosis of cerebral veins (C0151945)

Concepts Disease or Syndrome (T047)
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English THROMBOSIS CEREBRAL VEIN, cerebral vein thrombosis (diagnosis), cerebral vein thrombosis, Thrombosis cerebral vein, cerebral thrombosis venous, cerebral venous thrombosis, Cerebral venous thrombosis, Thrombosis of cerebral veins, Cerebral vein thrombosis, Thrombosis of cerebral veins (disorder)
Italian Trombosi venosa cerebrale, Trombosi di vena cerebrale
Dutch trombose cerebrale vene, cerebrale veneuze trombose
French Thrombose d'une veine cérébrale, THROMBOPHLEBITE CEREBRALE, Thrombose veineuse cérébrale
German Thrombose der Gehirnvene, HIRNVENENTHROMBOSE, zerebrale Venenthrombose
Portuguese Trombose venosa cerebral, TROMBOSE VENOSA CEREBRAL, Flebotrombose cerebral
Spanish Trombosis de la vena cerebral, TROMBOSIS VENOSA CEREBRAL, trombosis de las venas cerebrales (trastorno), trombosis de las venas cerebrales, trombosis venosa cerebral, Trombosis venosa cerebral
Japanese 大脳静脈血栓症, ダイノウジョウミャクケッセンショウ
Czech Trombóza mozkové žíly, Venózní mozková trombóza
Hungarian Cerebralis venás thrombosis, Agyi venás thrombosis

Ontology: Thrombophlebitis of cerebral vein (C0235502)

Concepts Disease or Syndrome (T047)
SnomedCT 95461006
English THROMBOPHLEBITIS CEREBRAL VEIN, Thrombophlebitis cerebral vein, Thrombophlebitis of cerebral vein, Thrombophlebitis of cerebral vein (disorder), cerebral; thrombophlebitis, thrombophlebitis; cerebral
Dutch tromboflebitis van cerebrale vene, cerebraal; tromboflebitis, tromboflebitis; cerebraal
French Thrombophlébite de la veine cérébrale, THROMBOPHLEBITE CEREBRALE
German Thrombophlebitis der Gehirnvene, HIRNVENENTHROMBOPHLEBITIS
Italian Tromboflebite di vena cerebrale
Portuguese Tromboflebite cerebral, TROMBOFLEBITE DA VEIA CEREBRAL
Spanish Tromboflebitis de la vena cerebral, TROMBOFLEBITIS VENOSA CEREBRAL, tromboflebitis de vena cerebral (trastorno), tromboflebitis de vena cerebral
Japanese 大脳血栓性静脈炎, ダイノウケッセンセイジョウミャクエン
Czech Tromboflebitida mozkové žíly
Hungarian Agyi venás thrombophlebitis

Ontology: Lateral Sinus Thrombosis (C0270639)

Definition (MSH) Formation or presence of a blood clot (THROMBUS) in the LATERAL SINUSES. This condition is often associated with ear infections (OTITIS MEDIA or MASTOIDITIS) without antibiotic treatment. In developed nations, lateral sinus thrombosis can result from CRANIOCEREBRAL TRAUMA; BRAIN NEOPLASMS; NEUROSURGICAL PROCEDURES; THROMBOPHILIA; and other conditions. Clinical features include HEADACHE; VERTIGO; and increased intracranial pressure.
Concepts Disease or Syndrome (T047)
MSH D020227
SnomedCT 21258007, 192761004
English Cerebral ven thromb of lat sin, Thromboses, Transverse Sinus, Transverse Sinus Thrombosis, Transverse Sinus Thromboses, Thrombosis, Transverse Sinus, Sinus Thromboses, Transverse, cerebral vein thrombosis of lateral sinus, cerebral vein thrombosis of lateral sinus (diagnosis), Transverse sinus thrombosis, Lateral Sinus Thromboses, Lateral Sinus Thrombosis, Thromboses, Lateral Sinus, Thrombosis, Lateral Sinus, Lateral Sinus Thrombosis [Disease/Finding], transverse sinus thrombosis, lateral sinus thrombosis, sinus transverse thrombosis, Thrombosis of lateral venous sinus, Cerebral venous thrombosis of lateral sinus, Thrombosis transverse sinus, Thrombosis of transverse sinus, Thrombosis of lateral venous sinus (disorder), Thrombosis transverse sinus (disorder)
Swedish Lateral sinustrombos
Japanese オウジョウミャクドウケッセンショウ, 横静脈洞敗血症性静脈炎, 血栓症-横静脈洞, 静脈炎-横静脈洞敗血症性, 敗血症性静脈炎-横静脈洞, 血栓-横静脈洞, 横静脈洞血栓, 横静脈洞血栓症
Czech sinus lateralis - trombóza, Trombóza sinus transversus
Portuguese Trombose do Seio Transverso, Trombose de Seio Lateral, Trombose dos Seios Transversos, Trombose de Seio Transverso, Trombose dos Seios Laterais, Trombose do seio transverso, Trombose do Seio Lateral
Spanish Trombosis de Seno Lateral, Trombosis del Seno Transverso, trombosis del seno venoso lateral (trastorno), trombosis de seno venoso lateral (trastorno), trombosis del seno transverso, trombosis de seno venoso lateral, trombosis del seno venoso lateral, Trombosis de los Senos Laterales, Trombosis de los Senos Transversos, Trombosis de Seno Transverso, trombosis de seno transverso (trastorno), trombosis de seno transverso, Trombosis del seno transverso, Trombosis del Seno Lateral
Finnish Lateraaliveriviemärin tromboosi
Russian LATERAL'NOGO SINUSA SEPTICHESKII FLEBIT, TROMBOZ LATERAL'NOGO SINUSA, LATERAL'NOGO SINUSA TROMBOZ, ЛАТЕРАЛЬНОГО СИНУСА СЕПТИЧЕСКИЙ ФЛЕБИТ, ЛАТЕРАЛЬНОГО СИНУСА ТРОМБОЗ, ТРОМБОЗ ЛАТЕРАЛЬНОГО СИНУСА
Italian Trombosi del seno trasverso, Trombosi del seno laterale
Polish Zakrzepica zatoki bocznej
Hungarian Sinus transversus thrombosis
Norwegian Sinus lateralis-trombose
Dutch sinus transversus trombose, Sinus-lateralistrombose, Trombose van de laterale sinus, Trombose, sinus-lateralis-
French Thrombose du sinus transverse, Thrombose du sinus latéral
German Thrombose des Sinus transversus, Sinus-lateralis-Thrombose, Thrombose, Sinus lateralis

Ontology: Sagittal Sinus Thrombosis (C0338575)

Definition (MSH) Formation or presence of a blood clot (THROMBUS) in the SUPERIOR SAGITTAL SINUS or the inferior sagittal sinus. Sagittal sinus thrombosis can result from infections, hematological disorders, CRANIOCEREBRAL TRAUMA; and NEUROSURGICAL PROCEDURES. Clinical features are primarily related to the increased intracranial pressure causing HEADACHE; NAUSEA; and VOMITING. Severe cases can evolve to SEIZURES or COMA.
Concepts Disease or Syndrome (T047)
MSH D020225
SnomedCT 70607008, 192760003
English Cerebral ven thromb of sag sin, Thrombosis sup.longit.sinus, cerebral vein thrombosis of superior sagittal sinus, cerebral vein thrombosis of superior sagittal sinus (diagnosis), Superior sagittal sinus thrombosis, Sagittal Sinus Thromboses, Sagittal Sinus Thrombosis, Sinus Thromboses, Sagittal, Sinus Thrombosis, Sagittal, Thromboses, Sagittal Sinus, Thrombosis, Sagittal Sinus, Sagittal Sinus Thrombosis [Disease/Finding], sagittal sinus thrombosis, sagittal sinus superior thrombosis, Thrombosis of superior sagittal sinus, Cerebral venous thrombosis of sagittal sinus, Thrombosis of superior longitudinal sinus, Sagittal sinus thrombosis, Thrombosis of superior longitudinal sinus (disorder), Thrombosis of superior sagittal sinus (disorder)
Italian Trombosi del seno sagittale superiore, Trombosi del seno sagittale
Dutch sinus sagittalis trombose, sinus sagittalis superior trombose, Sinus-sagittalistrombose, Trombose van de sinus sagittalis, Trombose, sinus-sagittalis-
German Thrombose des Sinus sagittalis, Thrombose des Sinus sagittalis superior, Sinus-sagittalis-Thrombose, Thrombose, Sinus sagittalis
Portuguese Trombose do seio sagital, Trombose do seio sagital superior, Trombose do Seio Sagital
Spanish Trombosis del seno sagital, trombosis de seno sagital superior, trombosis de seno sagital superior (trastorno), trombosis del seno sagital superior (trastorno), trombosis del seno sagital superior, trombosis del seno longitudinal superior (trastorno), trombosis del seno longitudinal superior, Trombosis del seno sagital superior, Trombosis del Seno Sagital
Japanese 上矢状洞血栓症, 矢状洞血栓症, シジョウドウケッセンショウ, ウエシジョウドウケッセンショウ, ジョウシジョウドウケッセンショウ, 血栓症-矢状静脈洞, 矢状静脈洞敗血症性静脈炎, 敗血症性静脈炎-矢状静脈洞, 矢状静脈洞血栓, 血栓-矢状静脈洞, 矢状静脈洞血栓症
Swedish Sagittal sinustrombos
Czech sinus sagittalis - trombóza, Trombóza sinus sagittalis superior, Trombóza sinus sagittalis
Finnish Nuoliveriviemärin tromboosi
Russian SAGITTAL'NOGO SINUSA TROMBOZ, FLEBIT SEPTICHESKII SAGITTAL'NOGO SINUSA, SAGITTAL'NOGO SINUSA FLEBIT SEPTICHESKII, TROMBOZ SAGITTAL'NOGO SINUSA, САГИТТАЛЬНОГО СИНУСА ТРОМБОЗ, САГИТТАЛЬНОГО СИНУСА ФЛЕБИТ СЕПТИЧЕСКИЙ, ТРОМБОЗ САГИТТАЛЬНОГО СИНУСА, ФЛЕБИТ СЕПТИЧЕСКИЙ САГИТТАЛЬНОГО СИНУСА
Polish Zakrzepica zatoki strzałkowej
Hungarian Sinus sagittalis superior thrombosis, Sinus sagittalis thrombosis
Norwegian Sinus sagittalis-trombose
French Thrombose du sinus sagittal supérieur, Thrombose du sinus sagittal

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English Cerebral ven thromb of sig sin, Cerebral venous thrombosis of sigmoid sinus, Cerebral venous thrombosis of sigmoid sinus (disorder)
Spanish trombosis venosa cerebral de seno sigmoide, trombosis venosa cerebral del seno sigmoide (trastorno), trombosis venosa cerebral de seno sigmoide (trastorno), trombosis venosa cerebral del seno sigmoide

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Spanish trombosis venosa cerebral de vena cortical (trastorno), trombosis venosa cerebral de vena cortical

Ontology: Cerebral venous thrombosis of great cerebral vein (C0338581)

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English Cereb ven thromb grt cer vein, Cerebral venous thrombosis of great cerebral vein, Cerebral venous thrombosis of great cerebral vein (disorder)
Spanish trombosis venosa cerebral de vena cerebral mayor (trastorno), trombosis venosa cerebral de vena cerebral mayor

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