Clinicopathologic Reports, Case Reports, and Small Case Series
September 2004

Allergic Fungal Sinusitis With Unilateral Eye Involvement

Author Affiliations



Copyright 2004 American Medical Association. All Rights Reserved.Applicable FARS/DFARS Restrictions Apply to Government Use.2004

Arch Ophthalmol. 2004;122(9):1390-1393. doi:10.1001/archopht.122.9.1390

We describe 2 patients with ocular signs and symptoms who were subsequentlydiagnosed as having allergic fungal sinusitis (AFS). This disease is characterizedprimarily by chronic rhinosinusitis, nasal polyposis, allergic mucin, andthe presence of fungal organisms according to a culture and/or histologicexamination.1 Clinical features are thoseassociated with chronic rhinosinusitis, which include facial pressure, nasalobstruction, and rhinorrhea.2 Proptosis,ptosis, and diplopia are the most common ocular symptoms; however, these conditionsrarely represent the initial manifestation of the disease. The nasal mucusspecimens of patients with AFS are designated as allergic mucin because ofthe abundance of eosinophils and their degradation products within the mucus.1 Although standardized treatment for AFS is notwell defined, surgical debridement and systemic corticosteroid therapy arecommonly recommended.

The typical patient with AFS is young and immunocompetent with a historyof asthma or atopy.2 Orbital involvementin AFS is caused by the direct extension of sinus inflammation and can resultin compressive ocular symptoms. Although well described in the medical literature,AFS has rarely been described with ophthalmic involvement. We found only 5such patients discussed in the ophthalmologic literature and take this opportunityto add our case series to this list.25

Report of Cases

Case 1. A 15-year-old African American boycame to our clinic with mild proptosis, pain and tearing of the left eye,and a history of having been "elbowed" in that eye 4 months previously (Figure 1). Medications included ibuprofen,fexofenadine hydrochloride, and an artificial tear preparation. Medical andfamily histories were noncontributory. His visual acuity was 20/20 OU. Slighthypoglobus and 5-mm proptosis were present in the left eye. The remainderof the ocular examination results were unremarkable. An otolaryngologic consultationprovided findings of erythematous nares, congestion, a deviated nasal septum,and polyps in the left nasal cavity. An orbital computed tomographic (CT)scan revealed extensive sinus soft tissue opacification with sinus expansionand multifocal erosion with lytic destruction of bone along the paranasalsinuses. In addition, a left frontal sinus soft tissue abnormality was notedextending into the left anterior cranial fossa and superior left orbit (Figure 2). Initial treatment included a dailynasal preparation with fluticasone propionate and a methylprednisolone taper.

Figure 1.
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A 15-year-old African Americanboy with proptosis, hypoglobus, and mild blepharoptosis of the left eye.

Figure 2.
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Coronal computed tomographic scanreveals opacification of the frontal, ethmoid, and maxillary sinuses withextension to the left anterior cranial fossa and superior left orbit, clearlyinvading the left cranial fossa and superior orbit as an expansive process.

Endoscopic sinus surgery included left polypectomy, ethmoidectomy, maxillaryantrostomy, frontal sinus trephination, and right frontal sinus exploration.The operation revealed thick, "claylike" fungal material and thick mucinoussecretions throughout the sinuses. The sinus contents were removed and submittedfor histologic examination. The patient was instructed to self-administersaline irrigation with a bulb syringe and to continue taking fluticasone.A 1-month postoperative evaluation showed resolution of periocular edema witha return of facial symmetry.

Histologic specimens revealed polypoid fragments of respiratory mucosaand bone admixed with sheets of hyaline eosinophilic material containing numerouscellular aggregates. The respiratory mucosa was largely denuded of epithelium,and the stroma was edematous and contained a chronic inflammatory infiltratecomposed predominantly of lymphocytes and plasma cells but also with patchyaggregates of eosinophils. The cellular aggregates within the mucus were composedalmost entirely of intact and degranulated eosinophils. Charcot-Leyden crystalswere abundant (Figure 3). Fungalelements were not visible on a routine hematoxylin-eosin stain, but numerousbranching septate hyphae with degenerative features were identified withinthe mucus on a Gomori methenamine silver stain (Figure 4). No invasive fungal elements were noted. Microbiologicalcultures of the specimens were not performed.

Figure 3.
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Case 1. Eosinophilic mucoid materialcontains clusters of eosinophils and associated Charcot-Leyden crystals (arrowheads)(hematoxylin-eosin; original magnification ×400).

Figure 4.
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Case 1. Degenerated branchingseptate fungal hyphae are present within the mucus (Gomori methenamine silver;original magnification ×400).

Case 2. A 22-year-old man came to our otolaryngologyclinic with a 2-year history of progressive symptoms of left eye fullnessand nasal airway obstruction. His medical history was significant for mildasthma, nasal airway obstruction, and nasal polyps requiring multiple endoscopicsinus operations since age 17 years. No double vision or other visual abnormalitieswere noted. Medication consisted of an over-the-counter inhaler. An examinationrevealed mild left proptosis and slight hypoglobus. An intranasal examinationrevealed polyps filling both nasal cavities with extension into the nasalvestibule. The remaining physical examination findings were normal. A coronalCT scan of the head, without contrast, revealed extensive sinusitis with softtissue densities of the frontal, ethmoid, and maxillary sinuses (Figure 5). The medial walls of the maxillaryantra were obscured, and involvement of the sphenoid sinuses was also noted.Focal areas within the soft tissue densities suggested the possibility offungal disease. The patient was given 40 mg/d of prednisone for 5 days priorto endoscopic sinus surgery.

Figure 5.
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Coronal view soft tissue windowof an expansive/destructive process originating from pansinusitis and causingsecondary ptosis and hypoglobus.

Surgery consisted of bilateral maxillary antrostomy, bilateral totalethmoidectomy, and left frontal sinus trephination. The operation revealedthick, "claylike" fungal material and edematous polypoid mucosa. The sinusmucosa and its contents were submitted for pathologic examination. The patientwas instructed to self-administer saline irrigation using a bulb syringe.Three weeks postoperatively, he noted a resolution of discharge and facialpressure symptoms. An endoscopic evaluation showed mild residual sinus edema,and treatment was instituted with a regimen of oral corticosteroids for 10days.

The results of histopathologic examination of the sinus contents andexcised mucosa were identical to those in case 1; namely, edematous respiratorymucosa and mucoid sinus contents, both containing numerous eosinophils, andfungal hyphae within the sinus contents on a Gomori methenamine silver stain(Figure 6 and Figure 7). Microbiological cultures were not performed.

Figure 6.
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Case 2. Edematous polypoid respiratorymucosa (bottom) and eosinophilic intraluminal mucoid material (top) comprisethe pathologic specimen (hematoxylin-eosin; original magnification ×100).

Figure 7.
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Case 2. Eosinophils predominatein the lamina propria of the respiratory mucosa (hematoxylin-eosin; originalmagnification ×200).


Allergic fungal sinusitis was originally described by A. L. Katzensteinin 1983 and was categorized as allergic Aspergillus sinusitis.1 The dermatiaceous family of fungi has been recognizedas the most common etiology of AFS.6 Thisfamily includes Bipolaris species, Curvularia lunata, and Alternaria species.5 The mucus specimens of patients with AFS are designatedas allergic mucosa owing to the marked presence of eosinophils and their degradationproducts, which include Charcot-Leyden crystals and a major basic protein.The hypothesized pathophysiologic mechanism of AFS is the presence of an eosinophilicreaction to fungal exposure of the sinuses.1 ElevatedIgE levels have been noted in less than 33% of patients with AFS.

Allergic fungal sinusitis has occurred in patients ranging from age8 to 56 years with a mean age of 26 years.2 Thisdisease often occurs in patients with a marked allergic diathesis such asatopy and the presence of nasal polyps.3 Blunttrauma has been identified as an inciting factor for AFS.5 Arecent study identified AFS in 93% of patients with chronic rhinosinusitis,suggesting that the disease may be more prevalent than originally estimated.1

Clinical features are those associated with chronic rhinosinusitis,which include facial pressure, nasal obstruction, and rhinorrhea.2 Proptosis, ptosis, and diplopia are the most commonocular symptoms; however, these seem to occur late and rarely represent theinitial manifestation of the disease.2,5 Epiphorahas been noted, and loss of vision is rare.2

Diagnosis of AFS is made primarily with a combination of characteristicfindings on a CT scan or magnetic resonance imaging (MRI) that correlate withthe presence of allergic mucin and positive fungi according to a culture orhistologic examination. Classic sinus CT scan findings in AFS include centralareas of hyperattenuation that correspond to a hypointense signal on T1-weightedMRI and a signal void with T2-weighted MRI. Sinus expansion and bony erosionwith evidence of remodeling have also been reported.7 Resultsof fungal cultures are often negative and may be due to sampling difficulty.

Although no standard treatment for AFS has been defined, surgical debridementand systemic corticosteroid therapy are commonly recommended. Fungal desensitizationwith immunotherapy injection is finding an increasing role in the treatmentof this disease.8 Corticosteroid therapyis based on similarities between the pathophysiologic mechanisms of AFS andthose of allergic bronchopulmonary aspergillosis.5 Antifungalagents generally are not recommended, even in cases with intracranial extension.6

This study was supported in part by unrestricted grants from Researchto Prevent Blindness, New York, NY, and the Pat and Willard Walker Eye ResearchCenter, Little Rock, Ark.

Correspondence: Dr Westfall, Department of Ophthalmology, Mail Slot523, 4301 W Markham St, Little Rock, AR 72205-7199 (

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