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A 57-year-old man with leukocytosis and sphenoid sinus disease
Digital Journal of Ophthalmology 2020
Volume 26, Number 2
April 24, 2020
DOI: 10.5693/djo.03.2019.09.003
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Ansuya P. Deosaran, MD | Department of Ophthalmology, Louisiana State University, New Orleans, Louisiana
Ahmaida Zeglam, MD | Department of Ophthalmology, University of Florida, Gainesville, Florida
Mary K. Wilson, BS | College of Medicine, University of Florida, Gainesville, Florida
Andres Gonzalez, MD | Department of Ophthalmology, University of Florida, Gainesville, Florida
Matthew J. Gray, MD | Department of Ophthalmology, University of Florida, Gainesville, Florida
Diagnosis and Discussion
Mucormycosis is the third most common invasive fungal infection, following Aspergillosis and Candidiasis, and has high morbidity and mortality.(4) Rhino-cerebral-orbito mucormycosis (RCOM) is associated with diabetes mellitus, hematological malignancies, hematopoietic stem cell transplantation, and solid organ transplant.(5) High-dose glucocorticoids, chronic antibiotics, long-term neutropenia, deroxamine, chronic renal failure, major burns, severe trauma, and acquired immune deficiency syndrome predispose to RCOM.(6,7)

Although rare, RCOM in immunocompetent individuals has been reported. An analysis of the literature of mucormycosis in immunocompetent, otherwise healthy individuals found 81 of 212 patients (38.2%) presenting with RCOM.(7) The present report reveals the need to have a high index of suspicion for RCOM in immunocompetent patients, especially if they are clinically deteriorating despite appropriate antibiotics.

Early diagnosis, removal or reversal of risk factors, prompt antifungals, and surgical debridement of devitalized tissue optimize RCOM outcomes.(8) Additionally, early evaluation and diagnosis of sinus mucormycosis helps prevent orbital invasion. Recognizing early findings such as fever, facial pain with swelling, nasal mucosal ulceration/necrosis, sinusitis, decreased visual acuity, and headache assists in timely diagnosis.(6,9) Amphotericin B is associated with increased response rates and survival.(8) Prompt amphotericin B initiation is recommended because a delay of >6 days is associated with increased mortality.(10) Early surgical debridement of necrotic tissue is fundamental. Necrotic tissue can decrease antifungal penetration, decreasing drug effectiveness.(11)

Tissue cultures are not necessarily reliable, because samples often fail to grow Mucor. Debridement biopsies help identify Mucor on tissue preparations using direct microscopy. Improved outcomes are associated with therapeutic decisions made based on frozen tissue sections.(12) Furthermore, Mucor tissue infections may manifest without a black necrotic eschar, as in our case. Mucormycosis must be considered irrespective of tissue presentation.

Mortality rates of RCOM range from 30% to 69%.(4) Despite following standard practice guidelines, our patient’s prognosis was poor. We hypothesize that intranasal steroids may have predisposed our immunocompetent patient. Local steroids likely suppressed immune responses, rendering the nasal mucosa susceptible to fungal colonization. An intensive literature search found no other reports of local steroid therapy with RCOM.

Rapidly deteriorating RCOM may occur in both immunocompromised and immunocompetent individuals. A high index of suspicion must be held in patients with signs and symptoms suspicious for mucormycosis sinusitis, especially if an immunocompetent individual is on intranasal steroids. Prompt administration of antifungal medications and debridement of necrotic tissues are recommended in all patients with RCOM.

Literature Search
PubMed was searched on July 21, 2016, without date restriction, for English-language results, using the following terms: rhino-orbital-cerebral mucormycosis, mucor, and immunocompetent.