TEACHING POINTS
■ Rhinocerebral mucormycosis is a rare fungal infection that historically is seen in the immunocompromised.
■ Well-recognized risk factors for the disease include diabetes mellitus, leukemia, aplastic anemia, myelodysplastic syndrome, blood dyscracias, immunosuppressive therapy in organ transplantation, renal disease, sepsis, and severe burns.
■ The infection progresses in three stages and usually manifests as an acute sinus infection. If not treated early, it can lead to serious complications such as cavernous sinus thrombosis and vascular invasion.
■ Current therapy for the invasive disease includes treating the underlying predisposing factors, antifungal therapy, and surgical debridement of the affected tissues.
■ Early recognition of the disease and treating the underlying cause of mucormycosis are key to improving outcomes.
CASE
A 37-year-old male presented to his primary care physician with acute onset periorbital edema, tinnitus, and nasal congestion. He attributed his symptoms to ongoing chronic sinusitis. On physical examination, the patient was afebrile and had ptosis of the right eyelid with periorbital edema and erythema. Edema and hypertrophy of the nasal turbinates and posterior pharynx were also present. His history included hypertension and chronic anxiety. The patient denied smoking but admitted to drinking alcohol socially. His physician made the primary diagnosis of periorbital cellulitis and admitted him to the hospital for treatment, where he was started on clindamycin, vancomycin, and piperacillin-tazobactam.
Two days after admission, the patient exhibited altered mental status. On neurologic examination, he had facial asymmetry due to complete ptosis and significant edema of the right eyelid. Extraocular movements were intact but limited during upward gaze in the right eye. CT of the brain showed cavernous sinus thrombosis with similar changes to the orbit, a semiacute right frontal lobe infarct, and diffuse sinusitis (Figure 1). Heparin was started in conjunction with ceftriaxone for the cavernous sinus thrombosis, and the patient's previous antibiotic regimen was discontinued. Based on an elevated hemoglobin A1C level of 7.8% and random blood glucose level of 260 mg/dL, a presumptive diagnosis of type 2 diabetes mellitus was made.
On hospital day 3, the patient's neurologic condition began to deteriorate acutely. He exhibited some posturing with blindness of the right eye; the right pupil became dilated and nonreactive. CT of the head showed hemorrhage in the area where the right frontal lobe infarct had been (Figure 2). Left pupillary response was intact. Heparin was stopped, and the patient was intubated because of concern that the airway was compromised. The following day, repeat CT of the head showed a large frontal bleed with surrounding edema and mass effect as well as subarachnoid hemorrhage (Figure 3).
The patient underwent a frontal lobectomy with removal of the blood clot. A complete right ethmoidectomy, right maxillary sinus antrostomy, frontal sinusotomy, and sphenoidectomy were also performed. Intraoperatively, significant devitalized mucous membranes were seen within the nasal cavity that were suspicious for an acute fungal process. The area was debrided, and pathology was consistent with mucormycosis. The patient was started on IV amphotericin B and anidulafungin. Multiple debridements of the right nasal cavity were subsequently performed. Bilateral myringotomy with insertion of a tympanostomy was also performed, and he was started on hyperbaric oxygen therapy (HBO). His condition started to improve dramatically, and he was subsequently discharged to an outpatient rehabilitation program on posaconazole after 21 days of treatment with amphotericin B, anidulafungin, and ceftriaxone.
