A 42 year-old man with a history of methamphetamine use, hypertension and uncontrolled type 2 diabetes (Hgb A1C 13.5%) presented with a dental abscess and hyperglycemia (>400) without DKA. Three teeth were extracted; cultures revealed candida spp. He received antibiotics and fluconazole. On the 4th day he developed right facial paralysis and central retinal artery occlusion with blindness. Brain MRI showed severe right ethmoid, sphenoid, and maxillary sinusitis with cavernous sinus thrombosis. Heparin and steroids were started. On the 9th day, he underwent drainage of the right maxillary sinus that was complicated by orbital cellulitis and ophthalmoplegia. Rush biopsies revealed “invasive fungal elements.” He was started on Amphotericin and referred to our institution for higher care.
At presentation, he had right facial edema, necrotic periorbital skin, a fixed, mid-position pupil, and facial paralysis with anesthesia. (Image 1) Labs showed WBC 43, 000 with left shift and thrombocytosis. He underwent right orbital exenteration (Image 2), extensive sinus debridement, and hyperbaric therapy. Mucormycosis was confirmed histopathologically; Posaconazole and Amphotericin (topical and intravenous) were initiated. Hospital stay was complicated by multiple embolic frontal lobe infarcts, hallucinations, and acute renal failure. He also developed left sided edema and vision loss, but refused debridement of the left orbit.
Eighty-seven days after his initial presentation, he underwent flap closure of his right orbital defect. Amphotericin was stopped and a prolonged course of posaconazole was continued.
Discussion:
Mucormycosis is a deadly, invasive fungal disease that requires aggressive debridement and antifungal therapy. Delayed amphotericin therapy increased mortality to 83% in one series, and survival after cavernous sinus thrombosis, especially with undebrided compromised tissue, as in our patient, is rare.
Our patient did not receive treatment with amphotericin until 10 days after presentation and 7 days after evidence of invasive sinus disease—instead, initial therapy was directed at bacterial pathogens and his positive culture for candida.
Possible contributors include:
-Availability bias: targeting usual, bacterial pathogens.
-Anchoring bias: failure to reconsider etiology after cavernous thrombosis / CRAO.
-Test obedience: targeting his positive culture for candida, although mucor is best diagnosed by pathology.
-Bad Rule: assuming diabetic patients have to have DKA to acquire mucor.
Conclusions:
– Rhinocerebral mucormycosis requires a high index of suspicion for diagnosis and can complicate uncontrolled diabetes without DKA.
– Early debridement minimizes tissue loss and mortality.
– Salvage of affected tissue is possible, but risks progressive disease.