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Show Neuro-ophthalmic Manifestations of Fungal Disease Associated With Posthurricane Environment Jayanth Sridhar, MD, Byron L. Lam, MD, Joshua Pasol, MD, Linda Sternau, MD Background: Allergic fungal sinusitis (AFS) is thought to represent an immunologic response to exposure to dema-tiaceous fungi. These fungi are known to cause disease more frequently in hot and humid climates and seasons. Methods: Three patients presented with unusual mani-festations of fungal disease after exposure to environ-ments recently affected by hurricanes. Results: Two patients had AFS, 1 with gradual painless visual loss from an AFS mass extending into the suprasellar region and 1 with orbital apex syndrome. Another patient had invasive fungal disease and developed orbital apex syndrome. Conclusions: These cases underscore the importance of clinical recognition of fungal disease in patients with sinus, orbital, or skull base involvement as well as its potential for causing permanent visual loss. This report suggests a po-tential association between fungal disease and tropical storm exposure. Journal of Neuro-Ophthalmology 2012;32:197-201 doi: 10.1097/WNO.0b013e3182268746 2012 by North American Neuro-Ophthalmology Society Fungal disease of the sinuses is classified into 4 subtypes: chronic noninvasive infection, chronic indolent in-fection, fulminant invasive disease, and allergic fungal si-nusitis (AFS) (1). AFS is the most recently described form, thought originally to be an allergic response to Aspergillus species (2). It is now known that dematiaceous fungi, such as Bipolaris and Drechslera, are the principle organisms involved (3). AFS is relatively common, accounting for up to 7%of sinus disease in patients undergoing sinus surgery (4). The exact pathogenesis of AFS is unknown but is thought to involve both type 1 (immediate, IgE-mediated) and type 3 (immune complex, IgG-mediated) immunologic reactions to fungal antigens after fungal colonization of the sinuses (5). AFS is usually seen in immunocompetent individuals with long-standing sinus disease symptoms (6). Most commonly, AFS occurs in hot and humid environments (7). The dematiaceous fungal species that cause AFS have been demonstrated to cause disease more frequently in warmer and more humid months as well (8). Despite being ‘‘noninvasive'' by definition, AFS is not benign and may cause destructive inflammation extending through the si-nuses into the orbit and cavernous sinus, leading to oph-thalmoplegia (9,10). We report 3 patients with fungal disease associated with exposure to posthurricane environments. Two patients had AFS, and 1 patient had invasive fungal infection. The cases underscore the importance of clinical recognition of fungal disease in patients with sinus, orbital, or skull base in-volvement as well as its potential for causing permanent visual loss. CASE REPORTS Case 1 A 25-year-old Miami schoolteacher presented with a 10-month history of painless vision loss in the left eye that worsened in the week prior to presentation. She had a his-tory of seasonal allergies and migraine headache. She was present in Miami when both Hurricane Katrina and Wilma passed through south Florida and worked in a hurricane-damaged classroom. Best-corrected visual acuity was 20/20, right eye, and 5/200, left eye, with a left relative afferent pupillary defect. Extraocular movements were full. The right fundus was normal, and the left optic disc had pallor and temporal cupping. Manual kinetic perimetry disclosed a left central scotoma, and optical coherence tomography was consistent with retinal nerve fiber layer thinning in the left eye. Bascom Palmer Eye Institute (JS, BLL, JP), University of Miami Miller School of Medicine, Miami, Florida; and Memorial Neuroscience Center (LS), Memorial Regional Hospital, Hollywood, Florida. Supported by National Institutes of Health center grant P30- EY014801 and Research to Prevent Blindness. The authors report no conflicts of interests. Address correspondence to Byron L. Lam, MD, Bascom Palmer Eye Institute, 900 NW 17th Street, Miami, FL 33136; E-mail: blam@ med.miami.edu Sridhar et al: J Neuro-Ophthalmol 2012; 32: 197-201 197 Original Contribution Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. MRI revealed a sphenoid sinus mass, hypointense on T2 images, extending superiorly displacing the pituitary gland and optic chiasm (Fig. 1). The lesion also extended pos-teriorly to displace and distend the prepontine cistern and compress the basilar artery. The patient underwent transsphenoidal surgery with the removal of a large necrotic mass with bony fragments from the left sphenoid sinus. Histopathologic examination re-vealed septated fungal hyphae and several eosinophils mixed with mucus and necrotic material (Fig. 2). Fungal culture grew Drechslera species. The patient was diagnosed with AFS and placed on intravenous methylprednisolone and oral voriconazole. The patient's kinetic visual fields improved over the next few months, with near resolution of the left central scotoma. Repeat MRI 9 months after the surgery revealed persistent AFS, but the patient did not follow-up thereafter. Case 2 A 58-year-old Caribbean woman was referred with a 6-month history of left-sided headache with recent de-velopment of diplopia. Medical history was remarkable for hypertension and hypothyroidism managed with oral medication. About 2 years prior to presentation, she worked in a hurricane-damaged hotel in the Cayman Islands and was involved in cleaning the damaged rooms. Best-corrected visual acuity was 20/25, right eye, and 20/ 20, left eye, with no relative afferent pupillary defect. The patient had a left sixth nerve palsy and decreased sensation in the ophthalmic branch (V1) of the left trigeminal nerve. Ophthalmoscopic examination was unremarkable. MRI showed left sphenoid and posterior ethmoid sinus expansion extending around the left internal carotid artery into the cavernous sinus. The sinuses appeared to be filled with a central nonenhancing material demonstrating pe-ripheral mucosal enhancement (Fig. 3). The patient underwent emergent left transsphenoidal decompression, with the removal of a large necrotic mass. Histopathology revealed numerous eosinophils and septate hyphal fragments consistent with AFS. The patient was placed on a short course of intravenous methylprednisolone and a 1-year course of voriconazole. One month later, the patient's sixth nerve palsy and decreased V1 sensation had resolved. At the 2-year follow-up, the patient was asymptomatic, with no headache or sinus congestion. MRI showed residual left sphenoid sinus disease. Case 3 A 64-year-old Federal Emergency Management Agency (FEMA) inspector was referred with a 3-month history of vision loss in the right eye as well as right ptosis and ophthalmoplegia. He had been previously evaluated else-where for severe right-sided temporal headache associated with weight loss and was treated briefly with oral cortico-steroids for presumed giant cell arteritis. Over the next 3 months, he experienced rapid deterioration of vision in the right eye followed by diplopia and ptosis. The patient was diabetic, and in the year prior to presentation, he had been working without any respiratory protection as an inspector of mold-infested trailers in the areas of Louisiana affected by Hurricane Katrina. Visual acuity was no light perception (NLP), right eye, and 20/25, left eye. The right pupil demonstrated both an afferent and an efferent pupillary defect. There was a complete right upper lid ptosis with near-complete ophthalmoplegia with only minimal abduction of the right eye. The fundi were normal as was function of the trigeminal and facial nerves. MRI revealed an enhanced area involving the right optic nerve and cavernous sinus (Fig. 4). The patient underwent FIG. 1. Case 1. Coronal MRI shows a sphenoid sinus mass extending superiorly, displacing the pituitary gland and displacing the chiasm (arrows) upward. The mass shows low signal on T2 image (A) and contrast enhancement on T1 image (B). 198 Sridhar et al: J Neuro-Ophthalmol 2012; 32: 197-201 Original Contribution Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. transsphenoidal biopsy the following day that revealed fungal hyphae. He was treated with oral cortisone, oral voriconazole, and intravenous micafungin. Two months later, the area of infection appeared more extensive on MRI and a right orbital exenteration was considered. The patient declined surgery, and medical therapy was changed to micafungin and voriconazole, both given intravenously for an additional 10 months. The pa-tient's headache and ophthalmoplegia gradually improved with full extraocular movements at 1 year following the initial presentation. Visual acuity remained NLP, right eye. The patient had stable neuroimaging studies during the 3 years of follow-up. DISCUSSION As our first 2 cases demonstrate, AFS must be considered in the differential diagnosis of patients who have sinus disease and ophthalmic symptoms and signs (11). As many as 17% of patients can present with orbital symptoms (12). Although proptosis is the most common orbital sign, diplopia and visual loss in the absence of proptosis and pain have been reported as presenting complaints (6,13). The pathophysiology of AFS involves destructive inflammation extending through the si-nuses and orbit into the cavernous sinus and resulting in ophthalmoplegia (9,10). The mechanism of visual loss is thought to be secondary to either orbital inflammation causing a contiguous inflammatory optic neuritis or optic nerve is-chemia secondary to increased intraorbital pressure (14). If the diagnosis of AFS is suspected clinically, neuro-imaging with attention to the paranasal sinuses and orbital structures is necessary. MRI classically reveals decreased T2 intensity in the involved sinus with high signal of the sur-rounding mucosa (15). This imaging finding is thought to be due to both the accumulation of desiccatedmucosal secretions and high concentrations of manganese, iron, and calcium in FIG. 2. Case 1. Histopathology of sphenoid sinus mass. A. Eosinophils mixed with mucus and necrotic material (hematoxylin and eosin, 340). B. Septated fungal hyphae (Gomori methenamine silver, 340). FIG. 3. Case 2. Initial contrast-enhanced T1 axial (A) and coronal (B) MRI with fat suppression reveals left sphenoid and posterior ethmoid sinus disease with left orbital apex involvement. Sridhar et al: J Neuro-Ophthalmol 2012; 32: 197-201 199 Original Contribution Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. the fungal concretions (16,17). Definitive diagnosis depends on positive fungal culture and histopathology of tissue re-vealing extensivemucin, eosinophils, and septate hyphae (18). Treatment of AFS consists of surgical removal of the fungal mass and postoperative use of systemic cortico-steroids to reduce risk of recurrence (19). There is no evi-dence to either support or discredit the use of intravenous and oral antifungal agents (19). In Case 1, the patient lived in Miami and began de-veloping symptoms 2 months after 2 major hurricanes affected the region. Ten months later, she presented with unilateral visual loss from an optic neuropathy secondary to suprasellar inflammatory process, an infrequently reported finding in AFS (6). Imaging is exceptionally helpful in guiding appropriate therapy because glucocorticoid treat-ment without surgery could have resulted in permanent vision loss (12). In Case 2, our patient cleaned hurricane-damaged hotel rooms in the Cayman Islands. Eighteen months later, she began having severe headache, and in another 5 months, she presented with a right cavernous sinus syndrome. This was confirmed on MRI, and treatment was made early enough to prevent any further clinical deterioration. Orbital apex syn-drome is a very uncommon manifestation of AFS, occurring in only 2% of AFS patients with orbital symptoms (9). In Case 3, the patient had a prolonged stay in Louisiana in the aftermath of Hurricane Katrina. Nine months after starting his job with FEMA, he began experiencing right-sided temporal headache followed by right-sided blindness, ptosis, and ophthalmoplegia. MRI revealed enhancement of the right orbital apex, which led to a transsphenoidal biopsy revealing fungal hyphae. The patient's ophthalmoplegia resolved with treatment but there was no improvement in vision. What factors might facilitate fungal disease occurring in the posthurricane environment? Soil-dwelling fungi that induce fungal disease and AFS have been shown to cause more disease in wetter and warmer months. It is known that a water-rich environment is conducive to the growth of mold species (8). Hurricanes can create such an environ-ment due to flooding and water damage. In the aftermath of Hurricane Katrina, aspergillus species were found to be more concentrated than normal in both general urban air quality studies and tests of the air in homes undergoing renovation (20,21). Six months after Hurricane Katrina, a direct correlation was demonstrated between exposure to water-damaged homes and respiratory symptoms (22). Although it is impossible to assign causality, our 3 pa-tients may all have developed fungal disease secondary to living and working in areas recently affected by inclement weather and flooding. They developed signs and symptoms over a 3- to 18-month period, consistent with the slow indolent course of AFS and fungal infection. In conclusion, fungal disease, including AFS, is an im-portant diagnostic consideration in the otherwise healthy patient presenting with symptoms, including visual loss, diplopia, facial numbness, and unilateral temporal head-ache. A potential association between tropical storm ex-posure and fungal disease, including AFS, requires further investigation. REFERENCES 1. Carter KD, Graham SM, Carpenter KM. Ophthalmic manifestations of allergic fungal sinusitis. Am J Ophthalmol. 1999;127:189-195. 2. 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