Acute orbital apex syndrome and rhino-orbito-cerebral mucormycosis

Abstract

PurposeTo demonstrate the successful clinical identification and management of rhino-orbital mucormycosis, a fungal infection with a high mortality rate.Patients and methodsA diabetic male patient with a headache and orbital apex syndrome in the right eye was examined using computed tomography (CT) and magnetic resonance imaging (MRI) for a possible fungal infection. Endoscopic surgical resection was performed and a pathology sample was taken. Specimens were prepared with Gömöri methenamine silver and hematoxylin and eosin staining. The patient was treated with liposomal amphotericin B 400 mg daily, followed by posaconazole 400 mg twice daily.ResultsCT and MRI revealed a mass of the right sphenoid spreading into the orbit, indicative of a fungal infection. The biopsy confirmed the diagnosis of mucormycosis. Complete recovery of eyelid and oculomotor function was achieved after 10 months of treatment, although the patient continues to suffer from irreversible blindness in the right eye due to optic nerve atrophy. He has been without signs or symptoms of recurrence.ConclusionPatients with rhino-orbito-cerebral mucormycosis need extensive surgical and medical treatment to maximize outcomes. Success requires multidisciplinary management.

Fungal infections of the orbit

“…[ 20 ] ROCZ can also present less commonly as a painless orbital apex syndrome without any signs of orbital cellulitis. [ 21 22 ] From the orbit, the infection can spread to brain through cribriform plate and orbital apex. Invasion of the cavernous sinus and cavernous part of carotid artery can lead to carotid occlusion, cerebral infarction, intracranial aneurysm/hemorrhage, fungal meningitis, mycotic abscess, and, eventually, death.…”

Rhino-orbital Mucormycosis Manifesting as Orbital Apex Syndrome with CRAO in An Immunocompetent Patient

“…4 Hyperglycemic status and metabolic acidosis nourish fungal colonies by interference with neutrophilic phagocytosis and increase in serum unbound iron. 5 This report emphasizes the rare occurrence of ROM in an immunocompetent diabetic patient with good metabolic control. The inciting event in ROM is inhalation of fungal spores.…”

“…Orbital involvement often presents as an orbital apex syndrome (OAS). 5 The exact pathogenesis of Rhino-Orbito-Cerebral Mucormycosis (ROCM) is not known. It is speculated to be due to spread from the paranasal sinuses to pterygopalatine fossa, from where it spreads to the cranium via orbital apex, orbital vessels or cribriform plate.…”

“…This thrombosing vasculitis causes emboli and vascular obstruction, culminating in tissue necrosis. 5 The ominous manifestations like Central Retinal Artery Occlusion (CRAO), cavernous sinus thrombosis and endophthalmitis have been variably reported. The unfortunate eventuality of CRAO bears an incidence of 16%-20%.…”

“…8 The prognosis depends on the extent of infection, immune status of the host and response to treatment. 5 There is ample evidence of high success rates with local amphotericin B and sino-nasal debridement, when the disease is restricted to sino-nasal or rhino-orbital sites. In case of cerebral involvement, surgical debridement provides no survival benefit and imminent mortality is inevitable.…”