Rosen & Barkin's 5-Minute Emergency Medicine Consult (139 page)

BASICS

• 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
  

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
  

• 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
    
• 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

• 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
    

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
  

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

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

• 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
    

• 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
  

TREATMENT

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

• 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)
  

• 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)
  

• Broad-spectrum antibiotics
  
• Anticoagulation
  

• Dexamethasone or hydrocortisone IV
  
• Endovascular thrombolytics in selected cases
  

FOLLOW-UP

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

None

Neurologic and neurosurgical consultation

• 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
  

• 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.