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.


Lemierre’s Syndrome

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
  • Imaging:
    • Computed tomography (CT) neck with contrast is initial study of choice
      • Allows for imaging of clot and potential abscess formation
      • May demonstrate intraluminal filling defect, peripheral rim enhancement
      • Thrombus can measure 10-20cm and CT allows for evaluation of complete occlusion
      • Can extend to chest to visualize septic pulmonary emboli
    • Ultrasound (US)
      • POCUS allows for rapid identification, though reduced sensitivity for recently formed thrombus as well as deeper infections and areas not amenable to US (e.g., mandibular)
    • Chest plain radiograph may demonstrate septic pulmonary emboli

 

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

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