emDOCs Podcast – Episode 56: Lemierre’s Syndrome and Retropharyngeal Abscess

Today on the emDOCs cast with Brit Long, MD (@long_brit) and Rachel Bridwell, MD (@rebridwell), we cover Lemierre’s syndrome and retropharyngeal abscess.

Epidemiology:

• Suppurative thrombophlebitis of the internal jugular vein
• Incidence of 3.6 cases per 1 million persons
  • Predominantly in 15-24 year old
• Resurgence in Group A streptococcus negative rapid testing
• Pathophysiology: hematogenous spread of bacteria from commonly an oropharyngeal infection via tonsillar vein vs. lymphangitis
  • Cases can occur after tonsillitis (37%), pharyngitis (30%)
  • More rare sources include:
    • Mandibular fracture
    • Bartholin gland abscess with hematogenous spread
•  Mortality was previously 90%, but more recent rates are 5-10% with early antibiotics
• Increased incidence with antibiotic resistance, decreased tonsillectomies, and use of rapid Strep pharyngitis testing
• Named for Anton Lemierre who published a 20 patient case series of this disease—18 patients in that cohort died

Microbiology:

• Oral flora:
  • Fusobacterium necrophorum (classically—accounts for 33%)
  • Fusobacterium nucleatum
  • Streptococcus
  • Staphylococcus
  • Klebsiella pneumoniae

Evaluation:

• Assess ABCs and begin resuscitation
  • May present with toxic appearance
    • Shock is unsurprisingly associated with increased mortality
  • Up to 97% will experience a cough, which can be due to septic pulmonary emboli
  • Abdominal symptoms to include nausea, vomiting, and abdominal pain occur in 50% of patients
  • 83% of patients demonstrate fever
• Classic triad: pharyngitis, anterior neck tenderness/swelling, and non-cavitary pulmonary infiltrates
• Patient may be leaning forward to optimize diameter of the airway
• Perform a complete physical examination
  • ENT: Unilateral neck pain aggravated by movement
  • Integumentary: Overlying erythema, edema, or tenderness on ipsilateral neck may be present in 52% of patients
  • Neck: Bilateral cervical lymphadenopathy (LAD) whereas mononucleosis often has unilateral cervical LAD, torticollis
  • Pulmonary: Wheezes, rhonchi, or diminished breath sounds due to septic pulmonary emboli
  • MSK: Assess for evidence of arthralgias indicating septic arthritis
  • Neuro: may demonstrate cranial nerve palsies (seen in 6% of patients)
• Laboratory evaluation:
  • Leukocytosis with left shift may occur as well as thrombocytopenia
  • 1/3 of patients will demonstrate hyperbilirubinemia with elevation in liver associated enzymes
    • 11-49% of these patients will demonstrate jaundice
    • Splenomegaly and hepatomegaly seen in patients with large septic thromboembolic burden

Treatment:

• ABCs—Sit upright and resuscitate appropriately, very likely to be septic
  • Tachycardic, febrile
    • Hypoxia if septic pulmonary emboli
•  Antibiotics: Ampicillin-sulbactam, piperacillin-tazobactam, or a carbapenem
  • Median duration of antibiotics is 4-6 weeks
• Consider anticoagulation in consultation with hematology and infectious disease
  • No consensus statement and no randomized control trials available to assess safety, efficacy, or mortality
    • Recent meta-analysis did not demonstrate reduced mortality with anticoagulation though confounded by anticoagulation rate varied widely based on side and clot burden
  • Pulmonary system is the most common site of septic emboli
    • Other sites include liver, muscle, pericardium, brain, and skin
• Consider surgery if abscess noted or if persistence of disease/worsening clinical picture despite maximal medical therapy
• 3% of cases are complicated by meningitis

Pearls

• The classic triad includes pharyngitis, anterior neck tenderness/swelling, and non-cavitary pulmonary infiltrates, though only 52% of patients have neck tenderness or swelling on exam
• Diagnosis includes CT with IV contrast of the neck
• Management includes resuscitation/stabilization and antibiotics

Retropharyngeal Abscess

Epidemiology: 

• Increasing incidence over past 20 years, with 4.10 cases per 100,000 patients under the age of 20 years
• Life-threatening infection between prevertebral fascia and posterior pharyngeal wall which can spread via potential space to mediastinum
• More common in children and younger adults due to larger retropharyngeal lymph nodes which can develop into an abscess
• Often occurs after pharyngitis or posterior pharyngeal trauma (fall with pen or toothbrush), recent ENT procedures
• Can also occur in patients with caustic substance ingestions

Microbiology

• Often polymicrobial, and predominant bacteria include:
  • Streptococcus pyogenes (group A strep), Staphylococcus aureus (MSSA, MRSA)
  • Mouth/respiratory bacteria: Fusobacteria, Prevotella, and Veillonella species
  • Rare gram negatives: Eikenella corrodens, Bartonella henselae, Mycobacterium tuberculosis

Evaluation:

• Patients present with:
  • Fever
  • Respiratory distress
  • Odynophagia
  • Decreased oral intake
  • Cri du canard (duck quack)
  • Voice change
  • Retractions
  • Chest pain if mediastinal spread has occurred
• Presentation is typically slower than epiglottitis
• Perceived neck stiffness in discomfort in extension may mimic meningitis

Examination:

• Prefer to lie supine with neck in extension
• Normal oropharyngeal examination
• Pain with tracheal rock
• Cervical LAD
• Torticollis
• Trismus

Laboratory analysis

• May demonstrate:
  • Leukocytosis
    • 91% of patients demonstrate white blood cell count >12,000
    • Neutrophil to lymphocyte ratio (NLR) >5.4 suggests deep neck space infection as compared to pharyngitis
  • Elevated acute phase reactants
    • C-reactive protein >100 mcg/mL correlated with increased hospitalization duration
  • Dehydration due to odynophagia

Imaging

• Lateral neck radiograph—performed in extension on inspiration to prevent pseudo-enlargement
• Widened prevertebral soft tissue
  • Anteroposterior (AP) diameter of soft tissues along anterior bodies of C1- 4 should be less than 40% of the AP diameter of the vertebral body behind it or C2</= 7mm
• Computed Tomography (CT) with IV contrast of the neck
  • Sensitivity of CT approaches up to 100%
  • Demonstrate phlegmon versus abscess
  • Location for operative planning
    • 21% of patients require second CT to assess progression and aid management

Treatment:

• Position of optimal patient comfort is key
• Airway management may require adjuncts
• Antibiotics:
  • Ampicillin-sulbactam or clindamycin
  • If concerned about MRSA, add vancomycin or linezolid (600 mg IV BID)
• Consider addition of dexamethasone
• Analgesia
• Consult ENT for possible surgical drainage though medical management is becoming more popular
  • Factors suggesting surgical management include abscess with cross-sectional measurement > 2cm² for more than 2 days of symptoms

Pearls

• New data demonstrate decreased rates of drainage when steroids used
• Consider in toxic child preferring to lie flat with normal oropharyngeal exam and painful tracheal rock
• Lateral neck radiographs can be used initially, many patients require neck CT for operative management