A 24-year-old Barber With Proptosis and Seizures

Abstract

Diagnosis: Allergic Fungal Rhinosinusitis Secondary to Bipolaris hawaiiensis and Curvularia lunata. The patient underwent a bicoronal craniotomy with neurosurgery for the resection of a presumptive tumor. Intraoperatively, the mass was consistent with a multiloculated lobular mucocele extending from the sinuses, and the dura was intact. Otolaryngology was called in to assist with the case and provided extensive sinus debridement. The radiographic imaging is suggestive of bone invasion [Figure 1]. However, in surgery, there was only thinning of the sinus wall and calcification caused by the expansion of the mucocele over a prolonged period of time. There was no evidence of bone destruction secondary to invasive disease. The expansion has been present for more than 2 years, since the patient reported facial changes consistent with proptosis and hypertelorism for 2 years prior to admission. The tissue pathology demonstrated eosinophilic deposition and Charcot-Leyden crystals, and no vascular or bony infiltration. Cultures from the mucocele and sinus tissue were positive for Bipolaris hawaiiensis, seen in Figure 2, and Curvularia lunata (not shown). Figure 2 also demonstrates the fungal-stained hyphae in the surgically removed tissue. Collectively, the findings are most likely consistent with allergic fungal rhinosinusitis (AFRS). Fungal rhinosinusitis is divided into invasive and noninvasive forms distinguished by histopathological findings. The noninvasive subtypes are saprophytic fungal infestation, mycetoma, and eosinophil-related fungal rhinosinusitis, which includes AFRS [1]. AFRS is classically characterized by the presence of a thick inspissated mucus with eosinophils and fungal hyphae [2]. The 5 diagnostic criteria of AFRS are: (1) radiographical evidence of sinusitis, (2) allergic mucin within the sinus, (3) absence of fungal invasion of the submucosa, blood vessels, or bone, and (5) absence of immunodeficiency disease, diabetes, or recent treatment with immunosuppressive drugs. The most common causes of AFRS are the dematiaceous molds, including bipolaris, curvularia, and pseudallescheria, and the hyaline molds, most commonly aspergillus and fusarium [3]. Eosinophils and fungi are typically present in all forms of chronic fungal rhinosinusitis. It is thought that fungi activate nasal/sinus epithelial cells, which leads to the degranulation of eosinophils, resulting in a complex localized eosinophilic reaction [4]. Persistent fungi continuously stimulate locally destructive immune responses, and this process can extend to adjacent sinuses. Over time, the sinuses may expand and bony erosion may occur. Patients may present with local or more extensive symptoms, including facial dysmorphic changes, complete nasal obstruction, and acute visual loss, all of which were present in our patient [1]. Sinus debridement is the initial treatment to provide drainage for sinus patency, as well as the removal of nasal polyps, which are usually present. Surgery is followed with oral and intranasal corticosteroids [5]. Frequent sinus irrigation with warm isotonic saline and aggressive allergy management along with corticosteroid use help to reduce the need for additional surgical debridements. However, despite these additive efforts, recurrence rates remain high [6]. Controversy lies in the role of antifungal therapy. There is a clear role for systemic antifungal therapy in invasive disease, but the role of topical antifungals in noninvasive disease is still unknown. Reducing the fungal load, which is thought to be the activator of inflammation, seems to be the obvious treatment modality. However, multiple studies