Deadly Sphenoid Fungus-Isolated Sphenoid Invasive Fungal Rhinosinusitis: A Case Report.

Introduction: Acute invasive fungal rhinosinusitis (AIFRS) is a potentially fatal infection, usually affecting immunocompromised patients. Isolated sphenoid sinus involvement is rare and has been reported in only a few cases. We discuss the clinical characteristics, histopathologic features, and differential diagnosis of AIFRS of the sphenoid sinus.

Case Presentation: A 57-year-old man with a history of refractory non-Hodgkin lymphoma and neutropenia presented with a 1-week duration of left-sided headache and ipsilateral cheek paresthesia. Nasal endoscopy showed mucoid drainage from the sphenoethmoidal recess. Magnetic resonance imaging demonstrated left sphenoid mucosal thickening and enhancement along the adjacent skull base. The patient underwent endoscopic sinus surgery with extended sphenoidotomy and débridement. The lateral wall and recess of the left sphenoid sinus demonstrated pale mucosa and fungal debris. Pathologic analysis demonstrated necrotic tissue and fungal hyphae with angioinvasion. Microbiology studies isolated Aspergillus fumigatus. The right maxillary sinus contained a synchronous fungal ball, which was removed during surgery; there was no evidence of tissue necrosis or invasive fungus in the maxillary sinus. He was treated with long-term voriconazole therapy, and 6-month follow-up showed disease resolution.

Discussion: AIFRS should be considered in the differential diagnosis of immunocompromised patients with nonspecific sinonasal symptoms. Usually, AIFRS is diffuse with multiple sinus involvement; however, isolated sphenoid AIFRS can occur. This is one of the few cases of AIFRS demonstrating isolated sphenoid involvement and is thought to be the first case showing a synchronous noninvasive fungal ball of another sinus cavity. Prompt recognition and surgical treatment may be curative and lifesaving.