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Updated: Dec 19 2019


  • Snapshot
    • A 50-year-old man is brought to the emergency room via ambulance from a fire. He has extensive burns over his arms, torso, and legs. His past medical history is significant for uncontrolled diabetes. On exam, he has periorbital swelling and rhinorrhea. There is a black eschar over his nares and palate. A CT of his sinuses show air fluid levels in his maxillary and frontal sinuses. Biopsy of the black eschar reveals nonseptate hyphae branching at wide angles.
  • Introduction
    • Classification
      • Mucor and Rhizopus spp.
        • fungi with irregular, broad, and nonseptate hyphae branching at wide or right angles
        • found in soil and decomposed material
        • transmission via inhalation of spores or direct inoculation (through trauma)
  • Epidemiology
    • Risk factors
      • ketoacidosis
      • uncontrolled diabetes
      • neutropenia
      • hematologic malignancies
      • immunocompromised status
      • trauma
      • burns
    • Pathogenesis
      • inhalation of spores causes germination in nares, sinuses, or lungs, and spread in blood vessel walls
      • invasion into blood vessels cause hemorrhage, thrombosis, and hematogenous spread
      • fungi can penetrate cribriform plate to enter the brain and sinuses
  • Presentation
    • Symptoms
      • headache
      • congestion
      • facial pain
      • sinus pressure
    • Physical exam
      • fevers
        • may be only presenting symptom in immunocompromised patients with pulmonary infections
      • soft tissue swelling
      • black necrotic eschar on face, particularly nares or palate
      • may affect cranial nerves
        • blurry vision
        • loss of ocular motility
      • may have erythematous and necrotic papules and plaques if skin is affected
  • Imaging
    • Computed tomography (CT) head/sinuses
      • indication
        • all patients
      • findings
        • air-fluid levels in sinuses
        • mucosal thickening
        • bony destruction
  • Studies
    • Labs
      • culture yield is often low and not useful
    • Biopsy of affected tissue
      • nonseptate hyphae with right-angle or wide-angle branching
    • Making the diagnosis
      • based on clinical presentation and demonstration of fungus in affected tissue
      • high level of suspicion in patients with sinusitis-like symptoms and appropriate risk factors who do not respond to broad-spectrum antibiotics
  • Differential
    • Bacterial sinusitis
      • distinguishing factor
        • does not present with black eschar or rapid progression to brain and cranial nerves
  • Treatment
    • Medical
      • amphotericin B
        • indication
          • first-line
      • isavuconazole
        • indication
          • second-line
    • Operative
      • surgical debridement
        • indications
          • all patients
          • in addition to antifungals
  • Complications
    • Death
  • Prognosis
    • High mortality
    • Rapidly progressive
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