Orbitomaxillary mucormycosis (zygomycosis) and the surgical approach to treatment: perspectives from a maxillofacial surgeon

Rhinocerebral or rhino-orbitocerebral (mucormycosis) zygomycosis (ROCZ) usually occurs among patients with poorly controlled diabetes mellitus (especially those with ketoacidosis), solid malignancies, iron overload or extensive burns, in patients undergoing treatment with glucocorticosteroid agents, or in patients with neutropenia related to haematologic malignancies. The disease process starts with inhalation of the fungus into the paranasal sinuses. The fungus may spread to invade the palate, sphenoid sinus, cavernous sinus, orbits or cranially to invade the brain. Pain and swelling precede oral ulceration and the resulting tissue necrosis can result in palatal perforation. Infection can sometimes extend from the sinuses into the mouth and produce painful, necrotic ulcerations of the hard palate. If untreated, infection usually spreads from the ethmoid sinus to the orbit, resulting in the loss of extraocular muscle function and proptosis. Surgical treatment includes the resection of involved tissues of the face, including skin and muscle, any skin of the nose that is involved, maxillary and ethmoid sinuses, necrotic tissue of the temporal area and infratemporal fossa, and orbital exenteration. The keys to successful therapy include suspicion of the diagnosis and early recognition of the signs and symptoms, correction of underlying medical disorders such as ketoacidosis, and aggressive medical and surgical intervention.