An 88 year-old patient presented with two months of left cheek numbness but no pain or sinus complaints. CT imaging revealed opacification of the left maxillary sinus with massive bony erosion and extension into the ethmoids and inferior orbit. Orbitotomy was performed leading to the diagnosis of sino-orbital fungal sinusitis. The unusual manifestation, differential and management are presented.
We report an interesting and unusual case of an elderly male with allergic fungal sinusitis (AFS). This case is remarkable because of the patient’s advanced age, the clinical presentation as a sino-orbital mass, presenting symptoms of left cheek numbness over a few months, and the patient’s residence in a geographic location quite atypical for AFS. This report adhered to the tenets of the Declaration of Helsinki and has been Health Insurance Portability and Accountability Act compliant.
While the precise etiology of allergic fungal sinusitis is still debated, the process is characterized by a chronic, non-invasive reaction to multiple fungal species that is likely autoimmune in nature . It most commonly presents painlessly in immunocompetent younger patients with a history of atopy, nasal casts, and polyposis . Multiple but unilateral sinus involvement is typical. One third of patients have positive hypersensitivity skin tests, increased IgE, and allergic sinusitis [1-3]. Usually, AFS arises in hot, humid climates characteristic of the southern and southeastern United States . Interestingly, sinus symptoms, while common, are not always present, whereas bone erosion is common. It is noteworthy that bone erosion does not indicate invasive disease [4-6].
An 88 year-old male and long-term resident of Colorado Springs, CO (high desert climate, 6035 feet elevation) presented with two months of left cheek numbness. He reported periodic and mostly seasonal clear rhinorrhea, but no pain, fever, diplopia, headaches, sinus pressure, or new visual complaints. He denied trauma, but had previously undergone left dacryocystorhinostomy for tearing problems. A few years earlier, he had undergone a head MRI to evaluate headache symptoms and numbness in the left upper and lower extremities. These imaging studies were remarkable only for asymptomatic left maxillary sinus disease that had not been diagnosed previously and went untreated.
He had poor right vision (hand motion) from age-related macular degeneration. The left had 20/30 distance vision, limited supraduction, and lower eyelid retraction. The anterior segment was remarkable for mild injection and conjunctival chemosis. There was no proptosis which is a relatively common finding in immunocompetent patients in whom fungal sinusitis develops . CT showed complete opacification of the left maxillary sinus, erosive changes of the maxillary walls, partial opacification of the left anterior ethmoid sinuses, and calcification changes (Figures 1-2).
For diagnosis, the patient underwent an anterior orbitotomy which revealed a large orbital floor defect with edge fibrosis that, upon release, expressed a thick, green material with peanut butter consistency. No neoplastic mass was seen. Biopsy and cultures of the maxillary mucosa showed chronic inflammation (eosinophils/neutrophils), necrosis, and branching uniform septate hyphae consistent with Aspergillus species. Fungal cultures were negative.
Subsequent nasal endoscopic partial ethmoidectomy and debridement of the left maxillary sinus were necessary to clear the disease. Very thick, inspissated mucus consistent with allergic mucin, along with thick green “peanut butter” consistency debris was evacuated. Polyps were debrided from the middle meatus. The left maxillary sinus ostium was already widened by the erosive disease process; therefore, an antrostomy was not necessary.
Postoperatively, the patient was seen in follow-up frequently to perform in-office debridements evacuating any reaccumulation of mucin and debris. He was maintained on aggressive saline sinus irrigations. Systemic steroids were avoided due to his advanced age and risks. His disease was controlled by frequent debridement and aggressive saline irrigations.
Repair of the orbital floor defect with an implant was later performed. Over time, his initially poor upward gaze improved dramatically.
Four categories describe the clinical classification of fungal sinusitis: acute invasive; chronic invasive; chronic non-invasive (“fungus ball”); and allergic fungal sinusitis (AFS) [1-6]. Acute invasive fungal sinusitis, caused most commonly by Mucor and Aspergillus species , is feared by ophthalmologists because of its high morbidity. It may present with cellulitis, ophthalmoplegia, and even blindness. Characteristic tissue invasion requires aggressive debridement, systemic antifungal medications, and management of underlying comorbidities. Diabetics and immunocompromised patients are most at risk .
Chronic invasive fungal sinusitis shows tissue invasion despite immunocompetence. It occurs mostly in dry climate areas outside the United States and is most commonly due to Aspergillus species. Treatment is primarily debridement and sinus re-aeration, with or without systemic antifungal medications for up to a year [2, 3].
Chronic non-invasive fungal sinusitis “fungus ball,” is also seen in immunocompetent patients but does not show tissue invasion or bone erosion. Treatment is surgical evacuation and sinus re-aeration as medical treatment alone has not shown to be effective .
AFS is primarily a disease of younger patients. Ophthalmic findings are common and include proptosis, tear drain obstruction, ptosis, globe displacement, visual loss, and diplopia [1, 2]. Involved orbital tissue generally shows no inflammation as compared to bacterial or invasive fungal infections.3 Patients typically are healthy with a history of recurrent sinus disease presumed secondary to bacterial sinusitis that showed poor response to antibiotics. Diagnosis is made per the Bent and Kuhn criteria noted below and is based on history, histopathological studies and characteristic radiologic findings [1-4, 6]. AFS classically has thick green material of peanut butter consistency which, upon special staining, shows the branching hyphae crucial for diagnosis . CT shows a unilateral, asymmetric process with heterogeneity, often with areas of calcification. The ethmoid sinuses are most frequently involved, and over half show bone erosion into the orbit [3, 6]. MRI findings provide high specificity showing T1 central hypointensity and T2 central void with peripheral enhancement .
Treatment strategies may include allergen avoidance and control , surgery to remove the allergic mucin, and drainage with re-aeration of the sinuses . Oral corticosteroids are important and may be used for up to a year [2, 3, 6]. This may be followed by allergen immunotherapy targeting fungal/non-fungal allergens . Thus far, desensitization is not supported by high level evidence . Antifungal medications have no role in the management of AFS [2, 3].
Bent and Kunh have established the diagnostic criteria for AFS . They are as follows:
- 1. Type 1 hypersensitivity by history, skin testing, or serology;
- 2. History of nasal polyposis;
- 3. Characteristic CT signs as noted above;
- 4. Eosinophilic mucus without fungal invasion into sinus tissue;
- 5. Positive fungal stain of sinus contents removed during surgery.
Our patient met each of these criteria as well as many characteristic but not diagnostically essential findings in patients with AFS. He was immune-competent and had unilateral disease. Abundant thick, green mucous material with peanut butter consistency, which was fungal culture negative, was removed from the sinuses. It is noteworthy that a positive fungal culture is desirable but not necessary for diagnosis.
He was type I hypersensitive by history, but no serology was done prior to diagnosis. Nasal polyposis was confirmed intra-operatively. His CT findings noted above were absolutely characteristic of AFS. Furthermore, the presence of bony erosion in our patient helped to eliminate the diagnosis of chronic non-invasive fungal sinusitis (fungus ball) that would have been more typical for his age. Biopsy and cultures of the maxillary mucosa showed chronic inflammation (eosinophils/neutrophils), necrosis, and branching uniform septate hyphae consistent with Aspergillus species. Our patient was unique in being elderly, residing in an atypical climate and region for AFS, and having a relative absence of prior sinus issues.
The differential of sino-orbital disease is broad and includes fungal infection, sarcoidosis, granulomatosis with polyangiitis (Wegener's), idiopathic orbital inflammation, syphilis, tuberculosis, vasculitis, and malignancies.3 Prompt sino-orbital imaging and referral to appropriate specialists should therefore be sought.
John R. Burroughs: intellectual contribution and manuscript preparation; Kathlene R. Mondanaro: intellectual contribution and manuscript preparation; James B. Hsueh: manuscript preparation; Barton Knox: intellectual contribution and manuscript preparation.
We acknowledge a reviewer for comments on the article.
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