Mucormycosis

Today, Dr. Trevor Rafferty presented an extremely interesting case of acute sinusitis in an uncontrolled diabetic that was rapidly progressive and progressed to include right facial edema, erythema, and numbness.

Management of acute sinusitis:

Acute sinusitis can be managed with supportive care in most cases as it is of viral etiology 98% of the time. Less than 2% are bacterial, with an extremely small percentage being of fungal etiology. If symptoms persist and/or worsen over 7-10 days or if the patient endorses a “double worsening” (symptoms getting better and then getting worse again), bacterial sinusitis should be suspected and antibiotics given. In immunocompromised hosts, antibiotics should be considered on a case by case basis and fungal sinusitis should always be on the differential diagnosis. In addition, one should always evaluate for signs of complications of sinusitis, which are orbital cellulitis, preseptal cellulitis, meningitis, abscesses, osteomyelitis, and infections of other adjacent structures. Worrisome signs and symptoms include nuchal rigidity, sepsis, proptosis, painful extraocular movements, diplopia, focal neurological deficit, eschar, and altered mental status.

Diagnosis of fungal sinusitis:

Diagnosis of fungal sinusitis is ONLY possible with a very high index of suspicion. It should be suspected in any and all patients with acute sinusitis in the setting of uncontrolled diabetes, organ transplant, chronic steroids, AIDS, IVDU, or other immunosuppressing medications or conditions. If the patient is in diabetic ketoacidosis or if there is clinical or imaging evidence of erosive disease, invasive fungal sinusitis must be ruled out surgically. Mucor especially thrives in patients with DKA due to an enzyme called ketone reductase, which causes it to thrive in environments rich in ketones. Mucor is angioinvasive and therefore, spreads quickly and produces eschar, necrosis, and frequently focal neurological deficits.

Endoscopic biopsies by ENT are the initial test of choice to obtain the histopathology and culture necessary to diagnosed fungal sinusitis, but are not very sensitive and if negative, do not rule out mucor. In most cases, more invasive exploration in the OR is necessary to look for necrotic tissue and eschar. B-D Glucan is not helpful in diagnosis as mucor does not have the cell wall components that make the test positive.

Treatment of mucormycosis:

The mainstay of treatment is surgical debridement, which often results in significant disfigurement. Multiple debridements are often necessary for source control. Antifungal treatment is also necessary and consists of amphotericin B initially and as a clinical responsive is observed, usually over several weeks, may be transitioned to posaconazole or isavuconazole. Mortality of sinus mucor is around 50%, pulmonary mucor 76%, and 96% for disseminated mucor. Antifungal treatment can be necessary for months and is usually continued until the period of immunosuppression can be stopped (if possible). For patients that cannot stop their immunosuppressing medications or diseases, they may required lifelong antifungal treatment. In addition to surgical debridement and antifungal therapy, aggressive treatment of predisoposing factors is necessary, including hyperglycemia, acidosis, and if possible, immunocompromise.

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