To do or not to do: Large-dose steroid treatment for severe vision loss secondary to compressive inflammatory optic neuropathy in the setting of invasive fungal sinusitis

Purpose: Invasive fungal sinusitis (IFS) is associated with high rates of morbidity and mortality and often presents with orbital apex syndrome. Prompt diagnosis and management are crucial to prevent irreversible visual loss. We report a case of an immunosuppressed patient with rapidly progressive severe visual loss associated with frontal lobe cerebritis and leptomeningitis related to IFS, causing an adjacent compressive inflammatory optic neuropathy, which was treated successfully by large-dose corticosteroids.

Observations: A 29-year-old woman with acute myeloid leukemia status post chemotherapy presented with right-sided headaches and periorbital swelling. Her examination was significant for subjective red desaturation and trace right eyelid edema and ptosis. The remainder of her initial ocular examination was normal. Her labs demonstrated neutropenia and thrombocytopenia. Imaging of the brain and orbits was concerning for extensive sinus disease with intracranial extension. An urgent multi-sinus and optic nerve decompression was performed given concern for compressive optic neuropathy, and the biopsy was consistent with invasive fungal infection. Despite aggressive antifungal treatment, vision in her right eye decreased rapidly to counting fingers. No optic nerve abnormalities were observed on serial MRIs, but adjacent inferior frontal lobe enhancement was present. After a vigorous debate in a multidisciplinary meeting, her severe vision loss was attributed to cerebritis causing an adjacent compressive inflammatory optic neuropathy, and large-dose intravenous (IV) steroid treatment was initiated while maintaining systemic antifungal therapy. Remarkably, she had a full recovery of her vision.

Conclusions and importance: Severe vision loss in IFS can occur due to compressive inflammatory optic neuropathy without direct fungal invasion as a contributing factor. Timely and effective intervention is crucial in preventing vision loss. Large-dose steroid therapy may be a potential treatment option for immunocompromised patients with invasive fungal sinusitis and intracranial invasion, provided strict fungal infection control measures are in place.