Cerebral Venous Thrombosis

Aka: Cerebral Venous Thrombosis, Thrombosis of Cerebral Vein, Thrombophlebitis of Cerebral Vein, Cerebral Vein Thrombophlebitis, Cerebral Infarction due to Cerebral Venous Thrombosis, Lateral Sinus Thrombosis, Transverse Sinus Thrombosis, Superior Sagittal Sinus Thrombosis, Sagittal Sinus Thrombosis ( C0338575), Sigmoid Sinus Thrombosis, Deep Venous Cerebral Thrombosis, Great Cerebral Vein Thrombosis, Cortical Vein Thrombosis, Superficial Cerebral Vein Thrombosis, Cerebral Sinus Thrombosis

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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. Images
    1. cnsVenousSinus.jpg
  3. As with other Venous Thromboembolism, virchow's triad applies to pathogenesis (stasis, vessel wall, Coagulopathy)
  4. Venous obstruction causes increased venous pressure and decreased capillary perfusion
    1. Initial compensation with venous dilation including collateral veins
    2. Blood brain barrier is disrupted
      1. Results in vasogenic edema and possible cerebral Hemorrhage
    3. Cerebrospinal fluid absorption may also be blocked, increasing Intracranial Pressure
      1. Results in decreased Cerebral Perfusion Pressure, cerebral ischemia and cytotoxic edema
      2. Hydrocephalus may develop, with increasing Intracranial Pressure and risk of Cerebral Herniation

IV. Risk Factors

  1. Precautions
    1. More than 85% of Cerebral Venous Thrombosis patients have at least one risk factor for thrombosis
  2. Hormonal
    1. Oral Contraceptives
    2. Tamoxifen
    3. Hormone Replacement Therapy
    4. Pregnancy or Postpartum State (up to 6 weeks after delivery)
      1. Most common cause in developed countries
  3. Medications
    1. Asparaginase
    2. Corticosteroids
    3. Methotrexate
    4. Cytotoxic drugs
  4. Hypercoagulable Conditions (Thrombophilia, present in 30% of CVT patients)
    1. See Thrombophilia
    2. Malignancy (esp. Hematologic Malignancy, myeloproliferative disorder)
    3. Vasculitis or Inflammation (e.g. Systemic Lupus Erythematosus, Inflammatory Bowel Disease, Behcet's Disease)
    4. Antiphospholipid Antibody Syndrome
    5. Hyperhomocysteinaemia (MTHFR gene mutation)
    6. Factor V Leiden Mutation
    7. Antithrombin Deficiency
    8. Protein C Deficiency
    9. Protein S Deficiency
    10. G20210A Mutation
  5. Hematologic Conditions
    1. Heparin-Induced Thrombocytopenia
    2. Essential Thrombocytosis
    3. Paroxysmal Nocturnal Hemoglobinuria
    4. Thrombotic Thrombocytopenic Purpura
  6. Head and Neck Disorders
    1. Head and Neck Infections (e.g. paramenigeal infections of the ear, sinus or oropharynx)
      1. Most common cause in developing countries
    2. Central Nervous System Infections
    3. Recent neurosurgery
    4. Closed Head Injury
    5. Dural AV Fistula or Arteriovenous Malformation
  7. Miscellaneous Conditions
    1. Dehydration
    2. Hyperthyroidism

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)
  3. Consider in atypical Cerebrovascular Accident (CVA)
    1. Young patients
    2. Infarcts that cross typical arterial distributions
    3. Multiple infarcts
    4. Associated atypical features (Altered Mental Status, Seizures, Headache)

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 (up to 30% of cases present with Seizure)
  5. Encephalopathy (esp. deep sinus involvement)
    1. Altered Mental Status (esp. in elderly)
  6. Intracranial Hemorrhage
    1. Present in up to 40% of CVT patients (secondary to Increased Intracranial Pressure)
    2. Bilateral parenchymal hemorrhagic lesions or Hemorrhages across multiple arterial territories

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. Labs

  1. See Cerebrovascular Accident
  2. Bedside Serum Glucose
  3. Complete Blood Count
  4. Basic Metabolic Panel
  5. Coagulation studies (INR, aPTT)
  6. Erythrocyte Sedimentation Rate or C-Reactive Protein
  7. Serum Troponin
  8. Thrombophilia labs
    1. Obtain before Anticoagulation initiated (consult neurology, hematology)
  9. Other testing
    1. Evaluate for other triggering events (systemic or CNS Infection)

XIV. Diagnostics

  1. Electrocardiogram
  2. Optic Nerve Sheath Diameter
    1. Increased diameter is a marker of Increased Intracranial Pressure (Papilledema)

XV. 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. CTV identifies filling defects
    3. Similar efficacy to MRV except in Altered Level of Consciousness or encephalopathy (parenchymal lesions)
  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

XVI. Management

  1. Consult Neurosurgery and Stroke Neurology
  2. Consult hematology in suspected Thrombophilia
  3. Initiate Low Molecular Heparin (e.g. Lovenox) which is preferred over Unfractionated Heparin
    1. Use Unfractionated Heparin in unstable patients who may require invasive procedure
    2. 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
    3. First episode of CVT: 3-6 months (6-12 months if no known risk factor)
    4. Continue Anticoagulation lifelong for recurrent CVT
  4. Endovascular intervention considered in decompensating or refractory cases
  5. Manage Increased Intracranial Pressure
    1. See Acute Severe Intracranial Pressure Management
    2. Acute monitoring by neurosurgery if risk of Increased Intracranial Pressure
    3. Cerebral Herniation is the most common cause of death in acute Cerebral Venous Thrombosis
  6. Manage Seizures
    1. Antiepileptic drugs are indicated for clinical evidence of Seizures and are continued for >=1 year

XVII. Complications

  1. Persistent Focal Neurologic Deficits
  2. Hydrocephalus and Increased Intracranial Pressure
  3. Cerebral Herniation
  4. Seizures
    1. Seen in up to 44% of cases in early Cerebral Venous Thrombosis (up to 30% present with Seizure)
    2. Seizure does not predict prognosis or mortality
    3. Kalita (2012) Seizure 21(8): 639-42 +PMID:22840965 [PubMed]

XVIII. Prognosis

  1. Recurrent Venous Thrombosis risk off of Anticoagulation
    1. Recurrent Cerebral Venous Thrombosis (>50% who recurr, do so in first year)
      1. Within first year: Up to 15% recurrence rate
      2. After first year: 2-7% recurrence per year
    2. Recurrent Venous Thromboembolism (e.g. DVT, PE): 4-7% per year
  2. Poor prognostic factors
    1. Age >35 years old
    2. Fever
    3. Altered Level of Consciousness or Coma
    4. Increased inracranial pressure or Papilledema
    5. Focal Neurologic deficits
    6. Banakar (2017) J Neurosci Rural Pract 8(2):204-8 +PMID:28479793 [PubMed]

XIX. References

  1. Alfalasi (2022) Crit Dec Emerg Med 36(3): 3-6
  2. Marcolini and Swaminathan in Herbert (2021) EM:Rap 21(2): 5-7
  3. Ulivi (2020) Pract Neurol 20:356-67 [PubMed]

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Related Studies

Ontology: Thrombosis of cerebral veins (C0151945)

Concepts Disease or Syndrome (T047)
SnomedCT 95455008
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, ﾀﾞｲﾉｳｼﾞ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
Derived from the NIH UMLS (Unified Medical Language System)

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, ﾀﾞｲﾉｳｹｯｾﾝｾｲｼﾞｮｳﾐｬｸｴﾝ
Czech Tromboflebitida mozkové žíly
Hungarian Agyi venás thrombophlebitis
Derived from the NIH UMLS (Unified Medical Language System)

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 ｵｳｼﾞｮｳﾐｬｸﾄﾞｳｹｯｾﾝｼｮｳ, 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
Derived from the NIH UMLS (Unified Medical Language System)

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, ｼｼﾞｮｳﾄﾞｳｹｯｾﾝｼｮｳ, ｳｴｼｼﾞｮｳﾄﾞｳｹｯｾﾝｼｮｳ, ｼﾞｮｳｼｼﾞｮｳﾄﾞｳｹｯｾﾝｼｮｳ, 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
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Cerebral venous thrombosis of sigmoid sinus (C0338577)

Concepts Disease or Syndrome (T047)
SnomedCT 230721009
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
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Cerebral venous thrombosis of cortical vein (C0338580)

Concepts Disease or Syndrome (T047)
SnomedCT 230722002
English Cerebr ven thromb of cort vein, cerebral vein thrombosis cortical, cerebral vein thrombosis cortical (diagnosis), cortical vein thrombosis, Cerebral venous thrombosis of cortical vein, Cerebral venous thrombosis of cortical vein (disorder)
Spanish trombosis venosa cerebral de vena cortical (trastorno), trombosis venosa cerebral de vena cortical
Derived from the NIH UMLS (Unified Medical Language System)

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

Concepts Disease or Syndrome (T047)
SnomedCT 230723007
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
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Cerebral infarction due to cerebral venous thrombosis (C3536606)

Concepts Disease or Syndrome (T047)
SnomedCT 433961000124100
English Cerebral infarction due to cerebral venous thrombosis, Cerebral infarction due to cerebral venous thrombosis (disorder), cerebral infarction due to cerebral venous thrombosis (diagnosis)
Derived from the NIH UMLS (Unified Medical Language System)

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