Primary Intraventricular Phaeohyphomycosis: An Atypical Presentation of Neurotropic Fungus.

Cerebral fungal infections are usually reported secondary to contiguous spread from paranasal sinuses or orbit, hematogenous spread, traumatic brain injury, or immunocompromised conditions. Primary isolated intraventricular phaeohyphomycosis is rare. We report a 29-year-old man who presented with acute symptomatic unilateral hydrocephalus with an intraventricular lesion. Intraventricular endoscopy demonstrated 3 lesions along the choroid plexus with turbid cerebrospinal fluid. The lesions were yellowish in appearance. Excision of all lesions was done along with septostomy. The histopathology of the lesion as well as cerebrospinal fluid showed thin, septate, pigmented hyphae suggestive of phaeohyphomycosis. The patient initially responded to oral voriconazole but later developed acute symptoms and died 3 months after surgery despite continuous antifungal treatment. Primary intraventricular phaeohyphomycosis is uncommon and may have a dismal prognosis even with early diagnosis and prompt treatment.