Cavernous Sinus Thrombosis, Emergency Medicine

Basics

Description

Thrombosis of a branch of the major intracerebral venous drainage system
Most commonly infectious
Spreads from facial, odontogenic, or sinus infection
Less frequently occurs with hypercoagulable state

Anatomy
3 primary sites of thrombosis:

Cavernous sinus-Most common:
- Drainage from superficial venous system
Superolateral to the sphenoid sinus and surrounds the sella:
- Cranial nerves (CN) III, IV, V1, and V2 traverse the lateral wall of the sinus.
- CN VI and the internal carotid artery occupy the medial portion of the sinus.
Can also involve transverse sinus and superficial sagittal sinus

Pathophysiology

Hematogenous spread of facial, otic, or neck infection into venous drainage system
Contiguous spread directly from infected sinus cavities (sphenoid, ethmoid > frontal)
Bacterial overgrowth leads to inflammation and coagulation, resulting in thrombosis.
Venous engorgement of cavernous sinus can affect adjacent structures:
- Ophthalmoplegia from inflammation of CN III, IV, or VI
- Pupillary fixation from CN III
- Sensory deficits or paresthesia of forehead or cheek from CN V1 and V2

Etiology

Septic:
- Staphylococcus aureus accounts for 70%
- Streptococcus pneumoniae, gram-negative bacilli, and anaerobes also seen
- Fungi less common; include Aspergillus and Rhizopus species
Aseptic:
- Less common
- Granulomatous conditions (TB)
- Inflammatory disorders
- From mass effect (tumors at base of skull, aneurysms)
Hypercoagulable states

Children may present with nonspecific symptoms such as decreased energy, vomiting, fever.
Have high level of suspicion for any child with recent otitis or pharyngitis with worsening symptoms, declining mental status, or signs of increased intracranial pressure (ICP):
- HTN, bradycardia, lethargy, vomiting, gait instability
More common in the neonatal period, when diagnosis can be extremely difficult to make

Diagnosis

Signs and Symptoms

Symptoms:
- Headache occurs in 90% of patients.
- Fever
- Ocular or retrobulbar pain
- Facial swelling
- Visual disturbance
- Facial dysesthesia
- Lethargy or altered mental status
Signs:
- Periorbital edema is earliest sign
- Chemosis with retinal vein engorgement
- Ptosis, proptosis
- Ophthalmoplegia
- CN palsies:
  - Lateral gaze palsy (CN VI)
  - Hypo/hyperesthesia of V1 and V2 (CN V)
- Meningismus
- Altered level of consciousness or coma
- Seizures
- Sepsis with cardiovascular instability or collapse

History
High-risk historical factors include:

A history of trauma
Previous ear/nose/throat (ENT) or neurosurgical instrumentation
History of central face furuncle that was manipulated
Diabetes or immunocompromised state (HIV, steroid use, cancer) may increase risk

Essential Workup

Clinical diagnosis: Venous engorgement, ocular symptoms, unilateral symptoms that become bilateral, rapidly progressive
Labs nonspecific
Imaging findings can be subtle

Diagnosis Tests & Interpretation

Lab

Neither sensitive nor specific
CBC:
- Leukocytosis
- Bandemia
PT/PTT/INR
ESR and D-dimer usually elevated
Lumbar puncture/CSF: Parameningeal inflammation or frank mengingitis

Imaging

CT scan:
- Can be normal early in disease course
- Noncontrast can show increased density
- Contrast can show filling defect
  - May identify original source of infection (e.g., sinusitis)
  - Dilated superior ophthalmic vein
  - Associated intracranial hemorrhage
  - Signs of increased ICP: Small ventricles, loss of sulci
MRI with MR angiography (MRA)/MR venography (MRV):
- Diagnostic modality of choice
- Direct visualization of intracranial vessels and sinuses
- Capable of visualizing thrombus at any stage

Differential Diagnosis

Meningitis/encephalitis
Intracranial abscess
Periorbital and orbital cellulitis
Internal carotid artery aneurysm or fistula
Pseudotumor cerebri
Acute angle-closure glaucoma
Intracranial hemorrhage
Tolosa-Hunt syndrome: Rare granulomatous inflammation of cavernous sinus

Extremely difficult diagnosis to make.
Maintain a high level of suspicion in toxic-appearing patients with recent ENT infections or in patients with refractory headache and risk factors for hypercoagulability or intracranial infection.

Treatment

Pre-Hospital

Patients can be altered and unstable.
May require rapid assessment and stabilization of airway, breathing, and circulation (ABCs)

Initial Stabilization/Therapy

Careful assessment of mental status with intubation for airway protection as needed
Aggressive fluid resuscitation for cardiovascular instability

Ed Treatment/Procedures

Broad-spectrum antibiotics with multiple drug regimens:
- Cover for gram positives, gram negatives, as well as anaerobes.
- Nafcillin or vancomycin (for methicillin-resistant S. aureus [MRSA]) + ceftriaxone:
  - Add metronidazole or clindamycin in significant infections.
Heparin:
- Attenuates clot propagation and decreases morbidity/mortality.
- Controversial in transverse and sagittal thrombosis owing to higher risk of subsequent hemorrhage
- Administer only after ruling out bleed on CT scan.
- Questionable superiority of LMWH over IV heparin
- Endovascular TPA in severe refractory cases
Systemic steroids:
- Believed to be of benefit with concomitant pituitary insufficiency, and with infectious or inflammatory etiologies
Appropriate management of increased ICP as needed
Surgical consultation for drainage of primary site of infection (e.g., dental abscess or sinusitis)

Medication

Ceftriaxone: 2 g/d IV (peds: 80-100 mg/kg/d to q12h)
Clindamycin: 300-900 mg IV q6-12h (neonates: 10-20 mg/kg/24h IV divided q6-12h; peds: 25-40 mg/kg/24h divided q6-8h)
Metronidazole: 500 mg IV q6h (neonates: 7.5-30 mg/kg/24h IV divided q12-24h; peds: 30 mg/kg/24h IV divided q6h)
Nafcillin: 1-2 g IV q4h (peds: 50-75 mg/kg/24h IV divided q8-12h depending on age)
Vancomycin: 1 g IV q12h (peds: 10-20 mg/kg IV q8-12h depending on age)

First Line

Broad-spectrum antibiotics
Anticoagulation

Second Line

Dexamethasone or hydrocortisone IV
Endovascular thrombolytics in selected cases

Follow-Up

Disposition

Admission Criteria

All patients with sinus thrombosis warrant admission to a monitored setting.
Consider ICU admission.

Discharge Criteria
None

Followup Recommendations

Neurologic and neurosurgical consultation

Complications

Blindness
- 1/6 left with visual impairment
CN palsies
Meningitis or intracranial abscess
Seizures, especially in superior sagittal sinus thrombosis
Pituitary necrosis and insufficiency from local invasion
Septic emboli
Sepsis and shock
30% mortality

Pearls and Pitfalls

Diagnosis is made on clinical evaluation and confirmatory lab evidence. Maintain a high index of suspicion.
Noncontrast head CT is often negative or nonspecific. MRI/MRV is the diagnostic imaging modality of choice and should be pursued in high-risk individuals.
Administer IV antibiotics early, especially in any ill-appearing patient with ENT or neurologic complaints.
Hypercoagulable states result in both central and peripheral venous thrombosis. Workup and management decisions must include consideration of systemic thromboembolism.

Additional Reading

Cannon ML, Antonio BL, McCloskey JJ, et al. Cavernous sinus thrombosis complicating sinusitis. Pediatr Crit Care Med. 2004;5(1):86-88.
Carvalho KS, Garg BP. Cerebral venous thrombosis and venous malformations in children. Neurol Clin North Am. 2002;20:1061-1077.
Laupland KB. Vascular and parameningeal infections of the head and neck. Infect Dis Clin North Am. 2007;21(2):577-590, viii.
Misra UK, Kalita J, Bansal V. D-dimer is useful in the diagnosis of cortical venous sinus thrombosis. Neurol India. 2009;57(1):50-54.
Sztajunkrycer M, Jauch EC. The difficult diagnosis: Unusual headaches. Emerg Med Clin North Am. 1998;16(4):741-760.

Codes

ICD9

325 Phlebitis and thrombophlebitis of intracranial venous sinuses
437.6 Nonpyogenic thrombosis of intracranial venous sinus

ICD10

G08 Intracranial and intraspinal phlebitis and thrombophlebitis
I67.6 Nonpyogenic thrombosis of intracranial venous system

SNOMED

89980009 Thrombosis of cavernous venous sinus
192770001 Thrombophlebitis of cavernous sinus (disorder)
302879007 Septic thrombophlebitis of cavernous sinus
95794005 Tolosa-Hunt syndrome

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