Long-term survival in rhinocerebral mucormycosis. Case report

Abstract

Mucormycosis refers to a group of rapidly progressive infections caused by fungi belonging to the order Mucorales. Infection most often develops in individuals with immunological or metabolic compromise, although patients without underlying abnormalities have been affected. Specific clinical manifestations are associated with various predisposing factors. Rhinocerebral mucormycosis is the most common form and most frequently develops in individuals with poorly controlled diabetes mellitus. The extent of anatomical involvement and clinical course are unpredictable, depending on the intrinsic factors of the host. Over the past 20 years the prognosis for patients with rhinocerebral mucormycosis, once considered to be a uniformly fatal disease, has improved. Coordinated medical and surgical treatment, including rapid diagnosis, the advent of systemic antifungal agents, aggressive surgical debridement, and control of the underlying disease process, have been credited with its successful management. The range of survival rates recorded with the regimen of combined therapies is wide because the number of patients reported is limited and anatomical involvement is diverse. Survival with intracerebral abscess is rare. The authors describe the successful management of a patient who developed a bifrontal fungal abscess during treatment for rhinocerebral mucormycosis associated with ketoacidosis and diabetes mellitus. The patient remains without radiographic or clinical evidence of infection more than 2 years after treatment. The authors review the characteristic clinical, radiographic, and pathological features of previously reported infections and emphasize the importance of early detection and aggressive treatment in the management of this frequently fulminant and fatal disease.