1. An Interesting Case of Sinusitis Jamie Tibbo PGY-4, Otolaryngology University of Ottawa

2. Case Presentation • 22 yr old male • Healthy non-smoker, no significant PMHx • Symptoms started 3-4 years ago • Nasal obstruction • Anosmia • Occasional rhinorhea • Alerting symptoms (March 2005) • RT peri-orbital swelling • RT blurry vision, headaches, bulging fore-head • Referred to an allergist • Referred to an Ophthalmologist • Ordered a CT Head

3. Case Presentation • Physical Examination: • Bulging of fore-head • RT peri-orbital swelling & exophthalmos • Good EOMs • No visual disturbances • Anterior Rhinoscopy: • Nasal polyps bilaterally • MRI was available for viewing

10. Case Presentation • Pt was scheduled for the OR within 3 weeks • Planned for combined approach with Neurosurgery • Frontal Craniotomy • Endoscopic Sinus Surgery • Abdominal fat graft to obliterate sinus • Use Stereotactic Neuronavigation

11. Case Presentation • A transcoronal incision was created • skin flap retracted inferiorly • drilled 3 holes and a frontal free bone flap was turned over

26. Diagnosis: Allergic Fungal Sinusitis

27. Fungal Sinusitis • Invasive • Acute Necrotizing Fungal Rhinosinusitis • Chronic Invasive Fungal Rhinosinusitis • Granulomatous invasive (indolent) Fungal Rhinosinusitis • Non-Invasive • Fungal Ball (sinus mycetoma) • Allergic Fungal Sinusitis (AFS)

28. History of Allergic Fungal Sinusitis • 1981- First reported as allergic aspergillosis of the paranasal sinuses by Miller et al. • 1983-Katzenstein performed retrospective histopathologic analysis on 113 consecutive sinus surgeries • 6% appeared identical to ABPA • Extramucosal eosinophil-rich allergic mucin • Termed the condition “Allergic Aspergillus Sinusitis”

29. Pathophysiology of AFS • An intense allergic/hypersensitivity reaction to a fungal antigen • Production of allergic mucin which may perpetuate the disease • Predisposing Factors: • Atopic Host • Mechanical Obstruction • Exposure to Fungus • Similar pathophysiology to Allergic Bronchopulmonary Aspergillosis (ABPA)

30. AFS Clinical Findings • Often young, atopic, immunocompetent individuals • Present with clinical findings suggestive of rhinosinusitis • Often have nasal polyposis • Often have had previous sinus surgery

31. AFS Clinical Findings • Most skin-test positive to common aeroallergens • 64% history of asthma or reactive airways • 75% history of nasal allergic mucin cast production (tan to dark-green, rubbery formed elements from the nose)

32. AFS Clinical and Lab Findings • Patients are immunocompetent • Eosinophils and ESR not usually elevated • Total serum IgE usually elevated • Fungal-specific IgE present in most cases • Type I hypersensitivity skin tests positive to the etiologic agent

33. CT Scan Findings • Heterogeneous densities • expanding the sinus • pushing nasal septum • “eroding” through various bony structures • Due to thick allergic mucin and fungal hyphae within it • May be present in other forms of fungal sinusitis

34. CT Scan Findings • Extrasinus extension caused by bone resorption from pressure from the expanding fungal mass- NOT from invasion of the fungus • Show evidence of chronic rhinosinusitis • May be unilateral or bilateral

35. Characteristics of AFS • Allergic mucin: • thick peanut-butter consistency • Color: from tan to dark green • H & E Stains: • Hypertrophic sinus mucosa • Edematous • Infiltrate of lymphocytes, plasma cells and eosinophils • Mucin: • Strongly staining masses of eosinophils • Often see Charcot-Leyden Crystals • GMS Staining: • Sparsely scattered fungal hyphae within the mucin but now within the mucosa

36. The Offending Agent • Aspergillus fumigatus was previously thought to be the primary mold • Most cases caused by other pigment-forming species including: • Bipolaris spicifera * • Exserohilum rostratum* • Curvularia lunata • Alternaria species • Manning and Holdman 1998 • * Most common

37. Alternaria • Taxonomic Classification • Kingdom: Fungi • Phylum: Ascomycota • Class: Euascomycetes • Order: Pleosporales • Family: Pleosporaceae • Genus: Alternaria

38. Alternaria Species • Ubiquitous • Commonly isolated from plants, soil, food and indoor air environment • Common laboratory contaminants • Produces melanin-like pigment • Genus contains 50 species • Alternaria alternata most common isolate from human infections • Same Phylum as Aspergillus

39. Alternata Species • Opportunistic pathogen • Bone marrow transplant patients • Onchymycosis • Ulcerative cutaneous infections • Osteomyelitis • Otitis media in agricultural field workers • Sinusitis

41. Epidemiology of AFS • Common among adolescents and young adults (mean age 22 yrs) • 5-10% of patients with chronic rhinosinusitis • Higher incidence in temperate regions with high humidity

42. Diagnosis of AFS • Surgically obtained thick mucin seen hisopathologically or grossly at surgery • Allergic mucin must be positive for fungal hyphae • No histopathologic evidence for mucosal fungal invasion, mucosal necrosis, granulomata or giant cells • Other fungal sinusitis disorders must be excluded

43. Treatment of AFS • Surgical intervention • FESS may have higher recurrence rates than more aggressive surgical procedures • Schubert 1998, 2000 • Post-operative Medical Treatment • Systemic Corticosteroids • Nasal Corticosteroids • Nasal Lavage • Allergen Immunotherapy • Anti-histamines, Anti-Leukotrienes • Systemic anti-fungals not indicated long-term

44. Treatment of AFS • Recurrent Symptoms? • Test IgE levels • May not be valuable (Sohail et al, 2004) • Start systemic corticosteroids • Urgent re-evaluation by ENT Surgeon

45. Conclusion • AFS is difficult to diagnose, thus, is often under-diagnosed • Management must involve both surgical and long-term medical management to prevent recurrences • Minimal research on efficacy of post surgical managment