Rhinocerebral mucormycosis: changing patterns of disease

Eleven cases of rhinocerebral mucormycosis (RM) encountered over a 13-year period were reviewed. Predisposing factors included leukemia (36%), diabetes mellitus (27%), aplastic anemia (9%), myelodysplastic syndrome (9%), and treatment with immunosuppressive medications necessary to maintain solid organ or bone marrow graft viability (64%). Two patients had no predisposing factors. Clinical findings included headache (73%), fever (55%), black nasal eschar (45%), orbitofacial cellulitis (36%), cranial nerve palsy (36%), altered sensorium (36%), and hemiparesis (27%). Seven patients presented with destruction of the paranasal sinuses and local invasion; three with direct extension to the frontal or temporal lobes. Four patients displayed hematogenous dissemination to the cerebrum, brain stem, and cerebellum from a primary pulmonary focus. The seven patients with sinus involvement were treated with aggressive surgical debridement. Two patients with focal intracerebral lesions underwent either open craniotomy or stereotactic biopsy. Amphotericin B was administered intravenously to all patients. Local irrigation via a percutaneous catheter was performed in the seven patients with sinus disease and in one case of intracranial abscess. All seven patients with intracranial infection died, in contrast to four patients that survived with infection localized to the sinuses and orbits. All survivors had been treated with a combination of surgery and amphotericin B therapy. This review demonstrates that RM is increasingly affecting patients with sources of immunosuppression other than diabetes mellitus. Early aggressive therapy to prevent cerebral involvement by this severe infection provides the best chance for a good outcome.