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Key Features

  • Rare; includes both rhinocerebral mucormycosis (Mucor, Absidia, and Rhizopus sp.) and other invasive fungal infections, such as Aspergillus

  • Fungus spreads rapidly through vascular channels and may be lethal if not detected early

  • Prognosis is guarded even with early diagnosis and immediate appropriate intervention

    • – Mortality rate is about 20% in persons with diabetes mellitus

    • – Mortality rate is over 50% if kidney disease is present or develops

    • – Mortality rate approaches 100% in patients with AIDS or hematologic malignancy with neutropenia

Clinical Findings

  • Mucormycosis

    • Classic finding is a black eschar on the middle turbinate; however, this finding is not universal and may not be apparent if the infection is deep or high within the nasal bones

    • Seen in patients who have a contributing factor that results in some degree of immunocompromise, such as diabetes mellitus, long-term corticosteroid therapy, or end-stage renal disease

    • Also seen in patients who are profoundly immunocompromised, eg, undergoing treatment for hematologic malignancies

    • Occasional cases have been reported in patients with AIDS, though Aspergillus sp. is more common in this setting

  • Initial symptoms may be similar to those of acute bacterial rhinosinusitis, although facial pain is often more severe

  • Nasal drainage is typically clear or straw-colored, rather than purulent

  • Visual symptoms may be noted at presentation in the absence of significant nasal findings

  • Mucosa may appear normal or simply pale and dry

Diagnosis

  • Nasal biopsy with silver stains reveals broad nonseptate hyphae within tissues and necrosis with vascular occlusion

  • Because CT or MRI may initially show only soft tissue changes, biopsy and ultimate debridement should be based on the clinical setting rather than radiographic demonstration of bony destruction or intracranial changes

Treatment

  • Invasive fungal sinusitis represents a medical and surgical emergency

  • Prompt wide surgical debridement and amphotericin B by intravenous infusion are indicated for patients with reversible immune deficiency

  • Lipid-based amphotericin B (Ambisome) may be used in patients who have kidney disease or in those in whom it develops secondary to nephrotoxic doses of nonlipid amphotericin

  • Other antifungals, including voriconazole and caspofungin, may be appropriate therapy depending on the fungus

  • Aggressive management with surgery should be carefully considered

    • While necessary for any possibility of cure, it often results in tremendous disfigurement and functional deficits

    • Disease specific survival is only about 57%

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