Diagnosis → Neurology/Neurosurgery

Cerebral venous sinus thrombosis

Etiology

Cause [ac3]

• Thrombophilia:

  • Genetic: factor V Leiden mutation, prothrombin mutation, antithrombin III deficiency, protein C or S deficiency, and elevated factor VIII levels

  • Acquired: antiphospholipid antibody syndrome, hyperhomocysteinemia, heparin-induced thrombocytopenia, obesity, severe dehydration, estrogen exposure, pregnancy, nephrotic syndrome, and sickle cell disease

• Aseptic causes: Trauma, surgery, systemic inflammatory disease

• Drugs: Corticosteroids, L-asparaginase, chemotherapy

• Infections primarily within the nasolabial triangle:

  • Orbital cellulitis, pharyngitis, abscess formation, cellulitis, sphenoid or ethmoid sinusitis, otitis media, mastoiditis, dental infection (tooth extractions), maxillofacial surgery, meningitis

  • Agent: Staphylococcus aureus (66%), Streptococcus species (20%), pneumococcus (5%), Gram-negative species (Proteus, Haemophilus, Pseudomonas, Fusobacterium, and Bacteroides), gram-positive species (Corynebacterium and Actinomyces), non-bacterial (VZV, HIV, fungal mucormycosis)

Pathophysiology

1. Thrombotic stimuli (adjacent infection, embolization, thrombophilia) --> thrombosis

2. ↑ venous/capillary hydrostatic pressure upstream → vasogenic edema

3. If venous pressure approaches arterial pressure → ↓ arterial inflow → venous infarction

4. Anatomic subtypes:

   • Cortical and Deep cerebral venous thrombosis --> Intracranial venous system:

     Intracranial hypertension, venous infarction, and/or intracranial hemorrhage

   • Dural venous sinus thrombosis --> Meninges

   • Cavernous sinus thrombosis --> Facial structures:

     Facial and periorbital edema, ptosis, proptosis, chemosis, discomfort, pain with eye muscle movement, papilledema, retinal venous distension, and loss of vision

Risk factors [ac3]

• Facial infections

• Thrombophilia

• Malignancy

• Autoimmune/inflammatory disease

• Head trauma

• Dehydration

• Immunosuppression (diabetes mellitus, steroids, cancer, and chemotherapy)

• Pregnancy

• Estrogen exposure

Complications

• Intracranial hemorrhage

• Hemorrhagic venous infarction

• Stroke

• Seizures/epilepsy

• Visual loss

• Diplopia

• Return-to-work issues: headache/fatigue/cognitive issues

Epidemiology

Incidence per 100.000 [ac3][s6h][vgf][1d2]

Default

Approach

• Blood test: WBC, CRP, erythrocyte sedimentation rate (ESR), and D-dimer

• CT venography

• MR venography

• Lumbar puncture

Treatment

1. Stabilize ABCs [p2v]

2. Manage elevated ICP

3. Treat seizures

4. Therapeutic anticoagulation (LMWH, later oral) unless clear contraindication

5. Address provoking cause(s) immediately

   • Stop estrogen, treat infection, evaluate malignancy, hydration

6. Antibiotics for 3-4 weeks [ac3]

   • Anti-staphylococcal agent (Vancomycin)

   • Third-generation cephalosporin

   • Metronidazole (for anaerobic coverage)

   • Antifungal treatment (Amphotericin B)

7. Corticosteroid

8. Thrombolysis/thrombectomy in selected patients failing anticoagulation

9. Surgical intervention in selective patients:

   • Sphenoidectomy

   • Ethmoidectomy

   • Maxillary antrostomy

   • Mastoidectomy

   • Abscess drainage

   • Craniotomy for subdural empyema or brain abscess

   • Orbital decompression

   • Ventricular shunt placement

   • Extensive sinus debridement due to mucormycosis

Differential diagnoses

Aspergillosis, Brain tumor, Carotid artery dissection, Carotid cavernous fistula, Cellulitis, Intracranial hemorrhage, Meningioma, Meningitis, Metastatic bone tumors, Migraine, Sarcoidosis, Stroke, Subarachnoid hemorrhage, Syphilis, Tuberculosis