Rhinocerebral Mucormycosis: Evolution of the Disease and Treatment Options

Abstract

Rhinocerebral mucormycosis is recognized as a potentially aggressive and commonly fatal fungal infection. The classic presentation is involvement of nasal mucosa with invasion of the paranasal sinuses and orbit. Mucormycosis is most commonly seen in association with diabetic ketoacidosis, but disease demographics have changed with the onset of AIDS and the advent of powerful immunosuppressive drugs. Treatment includes aggressive debridement, systemic antifungal therapy, and control of underlying comorbid factors. Although surgical intervention remains essential, advances in medical therapy have permitted a more limited surgical approach to minimize functional loss without compromising survival. We present the UCLA experience with rhinocerebral mucormycosis from 1955 to 1995, with emphasis on the evolution of disease presentation and alternative treatment options.

Use of posaconazole in the treatment of infective rhinocerebral mucormycosis

“…Mucormycosis can manifest as six different syndromes: rhinocerebral, pulmonary, gastrointestinal, central nervous system, subcutaneous and disseminated. 3 Rhinocerebral mucormycosis falls under the category of acute fulminant invasive sinusitis. 1,2 This particular form is characterised by a painless, necrotic septal ulcer, sinusitis and rapid intracranial spread with a high mortality rate.…”

“…The syndrome develops most commonly in individuals with poorly controlled diabetes, as in our patient. [1][2][3][4][10][11][12] Immunocompromise is the main predisposing factor, and this may be congenital, acquired (e.g. acquired immunodeficiency syndrome) or drug-induced (e.g.…”

“…12 Other known predisposing factors include haematological malignancy, severe burns, renal disease, iron overload and the use of desferoxamine. [1][2][3]10,11 Acidosis and hyperglycaemia are ideal environments for fungal growth. 3 The infection can spread from the nasal mucosa into the paranasal sinuses (specifically the ethmoid and maxillary sinuses) and the brain.…”

“…[1][2][3][4] Four of the fourteen families are pathogenic, with mucoraceae being most significant. Patients with mucor commonly have organisms related to this genus, including rhizopus, rhizomucor and absidia.…”

“…Patients with mucor commonly have organisms related to this genus, including rhizopus, rhizomucor and absidia. 3 These organisms are found in soil and are important in the decay of organic material. The presence of such opportunistic pathogens suggests a significant underlying predisposing factor.…”