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Cavernous Sinus Thrombosis, Emergency Medicine

Basics

Description

- 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

- Septic: - Staphylococcus aureus accounts for 70% - Streptococcus pneumoniae, gram-negative bacilli, and anaerobes also seen - Fungi less common; include Aspergillus and Rhizopus species

- 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

- 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

  • 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 VIPupillary fixation from CN IIISensory 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 seenFungi less common; include Aspergillus and Rhizopus species
  • Aseptic:Less commonGranulomatous conditions (TB)Inflammatory disordersFrom 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.FeverOcular or retrobulbar painFacial swellingVisual disturbanceFacial dysesthesiaLethargy or altered mental status
  • Signs:Periorbital edema is earliest signChemosis with retinal vein engorgementPtosis, proptosisOphthalmoplegiaCN palsies:Lateral gaze palsy (CN VI)Hypo/hyperesthesia of V1 and V2 (CN V)MeningismusAltered level of consciousness or comaSeizuresSepsis 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:
  • 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 courseNoncontrast can show increased densityContrast can show filling defectMay identify original source of infection (e.g., sinusitis)Dilated superior ophthalmic veinAssociated intracranial hemorrhageSigns of increased ICP: Small ventricles, loss of sulci
  • MRI with MR angiography (MRA)/MR venography (MRV):Diagnostic modality of choiceDirect visualization of intracranial vessels and sinusesCapable 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 hemorrhageAdminister only after ruling out bleed on CT scan.Questionable superiority of LMWH over IV heparinEndovascular 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

  • Blindness1/6 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.

See Also (Topic, Algorithm, Electronic Media Element)

Headache

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