Allergic Fungal Sinusitis Allergic Fungal Sinusitis William Reisacher, MD FACS FAAOA William Reisacher, MD FACS FAAOA Assistant Professor Assistant Professor Weill Cornell Medical College Weill Cornell Medical College NewYork- -Presbyterian Hospital Presbyterian Hospital NewYork
Background Background � Chronic Chronic rhinosinusitis rhinosinusitis (CRS) is reported by (CRS) is reported by � 1 approximately 35 million Americans 1 approximately 35 million Americans � Direct cost of CRS in the USA is $4.3 billion Direct cost of CRS in the USA is $4.3 billion � 2 annually 2 annually 3 30% of CRS patients have nasal polyps (NP) 3 � 25 25- -30% of CRS patients have nasal polyps (NP) � 4% of the general population 4 4 � NP are found in 1 NP are found in 1- -4% of the general population � 5 � Incidence slightly higher in males and elderly Incidence slightly higher in males and elderly 5 �
6 Classification of NP 6 Classification of NP CRS w NP CRS AFS CRS w/o NP
Background of AFS Background of AFS � Similarities with allergic Similarities with allergic bronchopulmonary bronchopulmonary � aspergillosis (ABPA) (ABPA) aspergillosis � Combination of Combination of Gell Gell and Coombs types 1 and 3 and Coombs types 1 and 3 � 7 hypersensitivities 7 hypersensitivities Bent and Kuhn diagnostic criteria (1994) 8 8 � Bent and Kuhn diagnostic criteria (1994) � – Type I hypersensitivity Type I hypersensitivity – – Nasal polyps Nasal polyps – – Characteristic radiologic findings Characteristic radiologic findings – – Eosinophilic Eosinophilic mucus without fungal invasion into sinus mucus without fungal invasion into sinus – tissue tissue – Positive fungal stain of sinus contents removed at Positive fungal stain of sinus contents removed at – surgery surgery
Demographic Features of AFS Demographic Features of AFS � Young, atopic and Young, atopic and immunocompetent immunocompetent � � Mean age of presentation is 28.9 Mean age of presentation is 28.9 � � Lower socioeconomic status Lower socioeconomic status � More common in southeastern United States 7 7 � More common in southeastern United States � 7 10% of all patient undergoing surgery for CRS 7 � 5 5- -10% of all patient undergoing surgery for CRS � 10 � Present in 51% of patients in Northern India with CRS Present in 51% of patients in Northern India with CRS 10 � � Wise et al. Wise et al. – – African Americans with AFS presented with African Americans with AFS presented with � mean age 11.7 years younger than Caucasians 11 11 mean age 11.7 years younger than Caucasians � Ghegan Ghegan et al. et al. – – African American males were 15x more African American males were 15x more � likely to have bone erosion than Caucasians and African likely to have bone erosion than Caucasians and African 12 American females combined 12 American females combined
Clinical Presentation of AFS Clinical Presentation of AFS � Headache Headache � � Unilateral nasal Unilateral nasal � congestion congestion � Diminished sense of Diminished sense of � smell and taste smell and taste � Postnasal drip Postnasal drip � � Refractory sinusitis Refractory sinusitis � � Proptosis Proptosis, , telecanthus telecanthus � � History of sinus History of sinus � surgery surgery
Differential Diagnosis of AFS Differential Diagnosis of AFS � � CRS with nasal polyposis CRS with nasal polyposis � � EMCRS EMCRS � � Invasive fungal sinusitis Invasive fungal sinusitis � � Mycetoma ( Mycetoma (“ “Fungus ball Fungus ball” ”) ) � � Antrochoanal polyp Antrochoanal polyp � � Neoplasm Neoplasm – Inverting papilloma papilloma – Inverting – – Carcinoma Carcinoma – Nasolacrimal duct cyst duct cyst – Nasolacrimal – Glioma, , dermoid dermoid lesions, lesions, encephaloceles encephaloceles – Glioma � Inflammatory / Autoimmune � Inflammatory / Autoimmune – Wegener’ ’s s granulomatosis granulomatosis – Wegener – Sarcoidosis – Sarcoidosis � Polypoid mucosal changes mucosal changes � Polypoid
Radiologic Findings of AFS Radiologic Findings of AFS CT scan T1 - MRI
Gross and Histologic Histologic Appearance of Appearance of Gross and AFS AFS � Thick, dark, tenacious Thick, dark, tenacious � mucus mucus � Sheets of necrotic and Sheets of necrotic and � degranulating degranulating eosinophils eosinophils � Charcot Charcot- -Leyden Leyden � crystals crystals � Scant fungal Scant fungal hyphae hyphae �
Management of AFS Management of AFS � Medical management Medical management � – Anti Anti- -inflammatory medication inflammatory medication – – Anti Anti- -fungal therapy fungal therapy – � Surgery � Surgery � Immunotherapy � Immunotherapy
Medical Management Medical Management � Anti Anti- -inflammatory inflammatory � – Systemic corticosteroids (pre Systemic corticosteroids (pre- -op/post op/post- -op) op) – – Topical corticosteroids Topical corticosteroids – – Leukotriene Leukotriene receptor antagonists receptor antagonists – – Macrolide Macrolide antibiotics antibiotics – � Anti Anti- -fungal fungal � – Systemic Systemic – 13 , Huang et al. 2004) 13 – Topical (Jen, Topical (Jen, Kacker Kacker, Huang et al. 2004) –
Surgical Management Surgical Management � Prior radical procedures replaced by complete, but Prior radical procedures replaced by complete, but � conservative endoscopic surgery. conservative endoscopic surgery. � Follow the polyps to the Follow the polyps to the mucin mucin � � Image Image- -guidance if available guidance if available � � Don Don’ ’t forget to send the t forget to send the mucin mucin for fungal staining for fungal staining � � Post Post- -operative debridement and irrigation operative debridement and irrigation � � Kupferberg Kupferberg staging system staging system � – Stage 0: NED Stage 0: NED – – Stage 1: Edematous mucosa/allergic Stage 1: Edematous mucosa/allergic mucin mucin – – Stage 2: Stage 2: Polypoid Polypoid mucosa/allergic mucosa/allergic mucin mucin – – Stage 3: Polyps and fungal debris Stage 3: Polyps and fungal debris – 14 � Need for revision surgery is about 30% Need for revision surgery is about 30% 14 �
Immunotherapy for AFS Immunotherapy for AFS � Mabry, et al. 1995 Mabry, et al. 1995 – – 9 AFS patients with no 9 AFS patients with no � controls. Less crust formation and post controls. Less crust formation and post and no adverse effects. 15 15 operative mucin mucin and no adverse effects. operative 14 � Bassichis Bassichis, et al. 2006 , et al. 2006 14 � – 60 patient with AFS 60 patient with AFS – – 24 patients, no IT. 36 patients, IT 24 patients, no IT. 36 patients, IT – – Average follow Average follow- -up was 48.5 months up was 48.5 months – – Significant reduction of office visits Significant reduction of office visits – – Decrease in need for re Decrease in need for re- -operation from 33% to 11% operation from 33% to 11% –
Dematiaceous Fungi Fungi Dematiaceous � “ “Darkly pigmented Darkly pigmented” ” � � Present in 87% of Present in 87% of � culture positive- -cases cases culture positive 16 of AFS 16 of AFS – Alternaria Alternaria – – Bipolaris Bipolaris – – Curvularia Curvularia – – Helminthosporium Helminthosporium – – Fusarium Fusarium – � 13% were 13% were aspergillus aspergillus �
Fungus and NP Fungus and NP � � Ponikau et al. from Mayo Clinic suggested that NP may arise from Ponikau et al. from Mayo Clinic suggested that NP may arise from immune hyper responsiveness to fungi that commonly colonize the immune hyper responsiveness to fungi that commonly colonize the 17 nose of patients with CRS 17 nose of patients with CRS � Gosepath et al. demonstrated et al. demonstrated Alternaria Alternaria DNA in 100% of surgical DNA in 100% of surgical � Gosepath 18 polyp specimens from CRS patients 18 polyp specimens from CRS patients 19 � Sabirov et al. studied the role of local et al. studied the role of local IgE IgE specific for Alternaria specific for Alternaria 19 � Sabirov – – CRS w NP (N= 21) vs. CRS w/o NP (N= 13) and healthy controls (N= 8) CRS w NP (N= 21) vs. CRS w/o NP (N= 13) and healthy controls (N= 8) – Serum levels of Alternaria Alternaria- -specific specific IgE IgE were no different between were no different between – Serum levels of groups groups – Alternaria- -specific specific IgE IgE in polyps was significantly higher than in the in polyps was significantly higher than in the – Alternaria nasal tissues of the other two groups nasal tissues of the other two groups � � An association was present between alternaria An association was present between alternaria- -specific specific IgE IgE and and 19 increased ECP and eosinophil eosinophil levels in patients with nasal polyps levels in patients with nasal polyps 19 increased ECP and
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