Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

INITIAL

suspected CST

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1st line – 

empiric antibiotic therapy + supportive therapy

High-dose intravenous antibiotics should be instituted at the earliest suspicion of this diagnosis.[2]

Appropriate selection of empiric antibiotic regimens should be directed at the probable organisms implicated at the primary source of infection.

For empiric antibiotic therapy, the Infectious Diseases Society of America (IDSA) guideline recommends vancomycin for 4-6 weeks with or without rifampin.[75]​ Alternative options may include linezolid or trimethoprim/sulfamethoxazole.[75]​ However, these guidelines were published in 2011 and no evidence-based guidelines on empiric antibiotics for this indication have been published since. Some experts do not recommend vancomycin unless the patient is known to be colonized with MRSA. Other options, based on expert opinion, may include amoxicillin/clavulanate plus gentamicin, a third-generation cephalosporin, a fluoroquinolone, and the addition of metronidazole if brain abscess or dental or sinus infection is suspected.[6][76]​​[77][78]​​ Consult your local guidelines or infectious disease specialist for more information as this is a very specialized area with little evidence available to guide treatment decisions.

Antifungal therapy is required rarely and has been advocated only in cases of biopsy-confirmed invasive fungal infection.

As soon as the laboratory has reported sensitivities, empiric antibiotics can be switched to specific antibiotic therapy.

High doses of intravenous antibiotics are required. They also need to be administered over an extended period; for at least 3-4 weeks beyond the time of clinical resolution.[41]​​

Concurrent supportive therapy is necessary alongside antibiotic treatment, and includes resuscitation, oxygen support, and local eye care.

ACUTE

confirmed septic CST: without hemorrhagic complications

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targeted antibiotic therapy + supportive therapy

As soon as the laboratory has reported sensitivities, empiric antibiotics can be switched to specific antibiotic therapy.

High doses of intravenous antibiotics are required. They also need to be administered over an extended period; for at least 3-4 weeks beyond the time of clinical resolution.[41]​​

Antifungal therapy is required rarely and has been advocated only in cases of biopsy-confirmed invasive fungal infection. However, in at-risk patients, antifungal treatment should be considered as fungi may cause devastating neurologic complications beyond cerebral venous thrombosis.[79]

Concurrent supportive therapy is necessary alongside antibiotic treatment, and includes resuscitation, oxygen support, and local eye care.[4]

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Plus – 

consider heparin or argatroban

Treatment recommended for ALL patients in selected patient group

Considerable controversy exists concerning the efficacy of anticoagulation. Evidence concerning effects on mortality and morbidity has been inconsistent.[41] [ Cochrane Clinical Answers logo ]

There is a risk of hemorrhage but there is some evidence that it prevents propagation and contributes to recanalization of the thrombus.

Anticoagulation is contraindicated in intracerebral hemorrhage, subarachnoid hemorrhage, and bleeding diathesis. Some also consider it to be dangerous in patients with bilateral CST. Based on limited observation, it may be beneficial after exclusion of hemorrhagic complications by CT scan.[2][15][22]

Intravenous unfractionated heparin has been advocated in the early stages. This can be switched to longer-acting agents, such as warfarin, when the patient's condition has stabilized.[50]​ One systematic review and meta-analysis suggested that in patients with cerebral venous thrombosis, direct oral anticoagulants (DOACs), and warfarin may have comparable efficacy and safety.[84]​ The evidence for the use of DOACs for CVT is limited.[85]

Direct thrombin inhibitors, such as argatroban, can be considered as an alternate form of anticoagulation to heparin in patients with, or at risk of, heparin-induced thrombocytopenia.[97]

The required duration of anticoagulation has not been determined.[4][6]

Primary options

heparin: 80 units/kg intravenous bolus initially, followed by a 18 units/kg/hour intravenous infusion titrated to an activated partial thromboplastin time (aPTT) between 1.5 and 2.5

OR

argatroban: consult specialist for guidance on dose

Back
Consider – 

intravenous corticosteroids

Treatment recommended for SOME patients in selected patient group

The role of corticosteroids is controversial in many cases of CST. They are potentially harmful because of their immunosuppressive effects.

However, corticosteroids are absolutely indicated in cases of pituitary insufficiency. Corticosteroid use may have a critical role in patients with Addisonian crisis secondary to ischemia or necrosis of the pituitary that complicates CST.[91][92]

Although there would seem to be only empiric support for its anti-inflammatory properties, with real fear of generalized sepsis, the use of corticosteroids may be considered and prove helpful in reducing cranial nerve inflammation and secondary cranial nerve dysfunction and also in decreasing orbital edema.​[4]​​

There are only a few anecdotal reports documented concerning the use of corticosteroids but their efficacy cannot be confirmed by these reports because other treatments have been used at the same time.[15][39][93][94]

Primary options

hydrocortisone: 100 mg intravenously every 6 hours

OR

dexamethasone sodium phosphate: 10 mg intravenously every 6 hours

Back
Plus – 

surgical drainage post-stabilization

Treatment recommended for ALL patients in selected patient group

As soon as the patient's condition permits, prompt drainage of the primary site of infection (such as paranasal sinusitis or dental abscess) or other concurrent closed-space infection is advisable.​[7][15][96]​​ Surgical drainage of the cavernous sinus is almost never performed.[1]

In sinogenic CST, surgical drainage of the sinuses for all cases has been advocated.[11]

Different operations have been performed to decompress the sinuses, including transseptal sphenoidectomy, endoscopic sphenoidectomy and ethmoidectomy, as well as external fronto-ethmoidal-sphenoidectomy.

In cases of otogenic CST, mastoidectomy has been performed with decompression of sigmoid sinus thrombophlebitis.[42]

Back
Plus – 

switch to warfarin post-stabilization

Treatment recommended for ALL patients in selected patient group

When the patient has been stabilized, heparin or argatroban can be substituted with longer-acting anticoagulation such as warfarin.[50]​ One systematic review and meta-analysis suggested that in patients with cerebral venous thrombosis, direct oral anticoagulants (DOACs), and warfarin may have comparable efficacy and safety.[84]​ The evidence for the use of DOACs for CVT is limited.[85]

Primary options

warfarin: commenced following stabilization of the condition with heparin or argatroban; heparin or argatroban is discontinued as warfarin is commenced: see local specialist protocol for dosing guidance

confirmed septic CST: with hemorrhagic complications

Back
1st line – 

targeted antibiotic therapy + supportive therapy

As soon as the laboratory has reported sensitivities, empiric antibiotics can be switched to specific antibiotic therapy.

High doses of intravenous antibiotics are required. They also need to be administered over an extended period; for at least 3-4 weeks beyond the time of clinical resolution.[41]​​

Antifungal therapy is required rarely and has been advocated only in cases of biopsy-confirmed invasive fungal infection.

Concurrent supportive therapy is necessary alongside antibiotic treatment, and includes resuscitation, oxygen support, and local eye care.[4]

Back
Consider – 

intravenous corticosteroids

Treatment recommended for SOME patients in selected patient group

The role of corticosteroids is controversial in many cases of CST. They are potentially harmful because of their immunosuppressive effects.

However, corticosteroids are absolutely indicated in cases of pituitary insufficiency. Corticosteroid use may have a critical role in patients with Addisonian crisis secondary to ischemia or necrosis of the pituitary that complicates CST.[91][92]

Although there would seem to be only empiric support for its anti-inflammatory properties, with real fear of generalized sepsis, the use of corticosteroids may be considered and prove helpful in reducing cranial nerve inflammation and secondary cranial nerve dysfunction and also in decreasing orbital edema.​[4]​​

There are only a few anecdotal reports documented concerning the use of corticosteroids but their efficacy cannot be confirmed by these reports because other treatments have been used at the same time.[15][39][93][94]

Primary options

hydrocortisone: 100 mg intravenously every 6 hours

OR

dexamethasone sodium phosphate: 10 mg intravenously every 6 hours

Back
Plus – 

surgical drainage post-stabilization

Treatment recommended for ALL patients in selected patient group

As soon as the patient's condition permits, prompt drainage of the primary site of infection (such as para-nasal sinusitis or dental abscess) or other concurrent closed-space infection is advisable.​[7][15][96]​​ Surgical drainage of the cavernous sinus is almost never performed.[1]

In sinogenic CST, surgical drainage of the sinuses for all cases has been advocated.[11]

Different operations have been performed to decompress the sinuses, including transseptal sphenoidectomy, endoscopic sphenoidectomy and ethmoidectomy, as well as external fronto-ethmoidal-sphenoidectomy.

In cases of otogenic CST, mastoidectomy has been performed with decompression of sigmoid sinus thrombophlebitis.[42]

confirmed aseptic CST: without hemorrhagic complications

Back
1st line – 

supportive therapy

Concurrent supportive therapy is necessary and includes resuscitation, oxygen support, and local eye care.[4]

Back
Plus – 

heparin or argatroban

Treatment recommended for ALL patients in selected patient group

Considerable controversy exists concerning the efficacy of anticoagulation. Evidence concerning effects on mortality and morbidity has been inconsistent.[41] [ Cochrane Clinical Answers logo ]

There is a risk of hemorrhage but there is some evidence that it prevents propagation and contributes to recanalization of the thrombus.

Anticoagulation is contraindicated in intracerebral hemorrhage, subarachnoid hemorrhage, and bleeding diathesis. Some also consider it to be dangerous in patients with bilateral CST. Based on limited observation, it may be beneficial after exclusion of hemorrhagic complications by CT scan.[2][15][22]

Intravenous unfractionated heparin has been advocated in the early stages. This can be switched to longer-acting agents, such as warfarin, when the patient's condition has stabilized.[50]​ One systematic review and meta-analysis suggested that in patients with cerebral venous thrombosis, direct oral anticoagulants (DOACs), and warfarin may have comparable efficacy and safety.[84]​ The evidence for the use of DOACs for CVT is limited.[85]

Direct thrombin inhibitors, such as argatroban, can be considered as an alternate form of anticoagulation to heparin in patients with, or at risk of, heparin-induced thrombocytopenia.[97]

The required duration of anticoagulation has not been determined.

Primary options

heparin: 80 units/kg intravenous bolus initially, followed by a 18 units/kg/hour intravenous infusion titrated to an activated partial thromboplastin time (aPTT) between 1.5 and 2.5

OR

argatroban: consult specialist for guidance on dose

Back
Consider – 

endovascular therapy

Treatment recommended for SOME patients in selected patient group

If a patient is considered suitable for anticoagulation but deteriorates despite this therapy, they may be considered for endovascular treatment.[87][88][89] Although endovascular treatment is increasingly being used to treat patients with cerebral venous thrombosis, this treatment is not routinely recommended in all patients.[90]​​​ This therapy is usually reserved for progressive, aseptic CST and carries with it the risks of intracranial hemorrhage, stroke, and the inability to recanalize. It does not preclude corticosteroids.

Back
Consider – 

intravenous corticosteroids

Treatment recommended for SOME patients in selected patient group

The role of corticosteroids is controversial in many cases of CST. They are potentially harmful because of their immunosuppressive effects.

However, corticosteroids are absolutely indicated in cases of pituitary insufficiency. Corticosteroid use may have a critical role in patients with Addisonian crisis secondary to ischemia or necrosis of the pituitary that complicates CST.[91][92]

Although there would seem to be only empiric support for its anti-inflammatory properties, with real fear of generalized sepsis, the use of corticosteroids may be considered and prove helpful in reducing cranial nerve inflammation and secondary cranial nerve dysfunction and also in decreasing orbital edema.​[4]​​

There are only a few anecdotal reports documented concerning the use of corticosteroids but their efficacy cannot be proved by these reports because other treatments have been used at the same time.[15][39][93][94]

Primary options

hydrocortisone: 100 mg intravenously every 6 hours

OR

dexamethasone sodium phosphate: 10 mg intravenously every 6 hours

Back
Plus – 

switch to warfarin post-stabilization

Treatment recommended for ALL patients in selected patient group

When the patient has been stabilized, heparin or argatroban can be substituted with longer-acting anticoagulation such as warfarin.[50]​ One systematic review and meta-analysis suggested that in patients with cerebral venous thrombosis, direct oral anticoagulants (DOACs), and warfarin may have comparable efficacy and safety.[84]​ The evidence for the use of DOACs for CVT is limited.[85]

Primary options

warfarin: commenced following stabilization of the condition with heparin or argatroban; heparin or argatroban is discontinued as warfarin is commenced: see local specialist protocol for dosing guidance

confirmed aseptic CST: with hemorrhagic complications

Back
1st line – 

supportive therapy

Concurrent supportive therapy is necessary and includes resuscitation, oxygen support, and local eye care.[41]

Back
Consider – 

intravenous corticosteroids

Treatment recommended for SOME patients in selected patient group

The role of corticosteroids is controversial in many cases of CST. They are potentially harmful because of their immunosuppressive effects.

However, corticosteroids are absolutely indicated in cases of pituitary insufficiency. Corticosteroid use may have a critical role in patients with Addisonian crisis secondary to ischemia or necrosis of the pituitary that complicates CST.[91][92]

Although there would seem to be only empiric support for its anti-inflammatory properties, with real fear of generalized sepsis, the use of corticosteroids may be considered and prove helpful in reducing cranial nerve inflammation and secondary cranial nerve dysfunction and also in decreasing orbital edema.​[4]​​

There are only a few anecdotal reports documented concerning the use of corticosteroids but their efficacy cannot be confirmed by these reports because other treatments have been used at the same time.[15][39][93][94]

Primary options

hydrocortisone: 100 mg intravenously every 6 hours

OR

dexamethasone sodium phosphate: 10 mg intravenously every 6 hours

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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