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Fungal Rhinosinusitis Niyada Teerasuwanajug. Cumming otolaryngology head and neck surgery 5 th edition Bailey otolaryngology head and neck surgery 4 th.

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Presentation on theme: "Fungal Rhinosinusitis Niyada Teerasuwanajug. Cumming otolaryngology head and neck surgery 5 th edition Bailey otolaryngology head and neck surgery 4 th."— Presentation transcript:

1 Fungal Rhinosinusitis Niyada Teerasuwanajug

2 Cumming otolaryngology head and neck surgery 5 th edition Bailey otolaryngology head and neck surgery 4 th edition The Otolaryngologic Clinics of North America 2000 SIPAC 2003

3 Incidence Rare NoninvasiveNoninvasive fungal rhinosinusitis is more common. AFS4-7% of Sx cases for chronic inflammatory sinonasal dis. are AFS. fungal ball.A review by Ferreiro et al. 1984-1994  3.7% of Sx cases for inflammatory sinus dis. were fungal ball. CIFSCIFS: very rare in the United States  only case reports & a few small series. –More common in region; Sudan & India. SIPAC 2003

4 Incidence AIFSIncidence rates of AIFS for leukemia, CA, or bone marrow transplant = 1-2% seems to be increasing.The incidence of fungal sinus disease seems to be increasing. SIPAC 2003

5 1. practitioner awareness. 2.Technical advanced >> mycology, serology, histopathology and radiology. 3.Growth of immunocompromised population. 4.Inappropriate use of anti-bacterial ATBs. 5. incidence of atopic dis. in the United States. Factor Contribute to Increase Incidence

6 Increased Susceptibility to Invasive Fungal Infections Disease of the sinuses: Dx & Mx. 2001: 180

7 Classification of Fungal Rhinosinusitis Noninvasive / ExtramucosalNoninvasive / Extramucosal –Saprophytic colonization / Superficial sinonasal mycosis –Fungal ball –Allergic fungal rhinosinusitis (AFS) InvasiveInvasive –Chronic invasive (indolent) fungal rhinosinusitis (CIFS) –Acute invasive (fulminant) fungal rhinosinusitis (AIFS) Disease of the sinuses: Dx & Mx. 2001: 179

8 Am J Surg Pathol Volume 30, Number 6, June 2006

9 Hypersensitivity Host response to fungus Immunocompromised AFRS FungusBallChronicInvasive Acuteinvasive Non-Invasive Invasive Fungal sinus dis. manifestations: by host’s immune response & tissue invasion. SIPAC 2003 HostDefense FungalForm Immunocompromised Invasive Immunocompetent Fungus ball Saprophytic Granulomatous Atopic AFRS The Otolaryngologic Clinics of North America 2000 Fungal manifestion : by patient’s immunologic status

10 Signs & Symptoms Seen with Fungal Infections Disease of the sinuses: Dx & Mx. 2001: 180

11 Endoscopic Findings Present During Fungal Infection Disease of the sinuses: Dx & Mx. 2001: 180

12 Endoscopic Findings Present During Fungal Infection

13 Microbiology Fungal form: 1. Mould Multicellular colonies Hyphae Cause most fungal rhinosinusitis 2. Yeast Unicellular Most reproduction by asexual budding However, some species >> dimorphic depending upon environmental conditions. SIPAC 2003

14 Microbiology Common fungi in fungal rhinosinusitis Category Disease Genera Zygomycetes (Mucoraceae) Acute invasive Absidia Cunninghamella Mucor Rhizomucor Rhizopus Hyaline moulds Fungas ball Aspergillus Acute invasive Fusarium Chronic invasive Pseudallescheria Dematiacious moulds Allergic fungal Alternaria Bipolaris Cladosporium Curoularia Exserohilum SIPAC 2003

15 Microscopic examination 10%, 20% KOH –Light microscopy –limit in thick specimen KOH – calcufluor white stain –Fluorescence microscope

16 Gram stain : not common use H & E (hematoxylin and eosin) GMS (Gomori methenamine silver) PAS (periodic acid schiff) Microscopic examination GMS and PAS superior to H&E

17 Culture identification – Media : Sabouraud’ s agar (glucose + beef extract; PH 5 ) Serology – specific IgE and IgG  detected by serum radioimmunoassay

18 Noninvasive Fungal Rhinosinusitis

19 Saprophytic colonization Fungal ball Allergic fungal rhinosinusitis

20 Saprophytic Colonization Extramucosal sinonasal fungi promote inflammation fungal spores on mucous crustsPresence of fungal spores on mucous crusts within nose & paranasal sinus Detected grossly on examination. Perhaps one could consider this an early form of a fungus ball. Common fungal agent: Aspergillus species Disease of the sinuses: Dx & Mx. 2001: 180-182

21 Saprophytic Colonization Clinical presentation: immunocompetent asymptomatic an odor in nose crusts of debris on nose blowing nasal endoscope: a tuft of fungal material is seen growing on nasal crusts, much like mold growing on old bread Disease of the sinuses: Dx & Mx. 2001: 180-182

22 Saprophytic Colonization Patient undergone previous endoscopic sinus surgery: –disrupt mucocilary transport pathway –dry nasal passageways / have some mucus stasis Fungal crusts may appear in areas of high airflow - anterior edge of turbinates, but can also appear in surgical widened sinus cavities. Imaging : not seen on imaging Disease of the sinuses: Dx & Mx. 2001: 180-182

23 Saprophytic Colonization Treatment: Debridement the involved region  endoscopic cleaning. Disease of the sinuses: Dx & Mx. 2001: 180-182

24 Saprophytic Colonization Treatment: Saline irrigation if it accumulate. Minimizing overuse of drying agents: antihistamine, topical nasal steriod. Rx underlying bacterial infection. Antifungal agent: not used. Disease of the sinuses: Dx & Mx. 2001: 180-182

25 Fungal Ball

26 Terms: –Mycetoma –Sinus mycetoma –Aspergilloma –Simple sinus aspergillosis A true mycetoma:  a suppurative & granulomatous subcutaneous fungal infection with draining sinus tracts.  refer to an invasive fungal infection of the feet. SIPAC 2003

27 Epidemiology: The average age reported in an american 29 cases = 64 yr (28-86 yr). A review by deShazo et al. >> similar age range, the youngest = 18 yr. Common in older No pediatric. Female predominant Fungal Ball Fungus ball of paranasal sinus: The Otolaryngologic Clinics of North America 2000

28 Often found unexpectedly during Rx of chronic bacterial sinusitis Hx : months to yearsMay be present for months to years. Nonspecific chronic sinusitis symptoms: –Nasal obstruction –Facial pressure –Postnasal drainage **Hx of symptoms refractory to common medical Rx: –ATBs –Antihistamines –Nasal steroids Fungal Ball SIPAC 2003

29 Fungal Ball Diagnosis No evidence of immunocompromise + No incidence of atopy *** A solitory maxillary/ sphenoid sinus May also in frontal & ethmoid sinuses May involve contiguous sinuses In asymptomatic patients  often detect only after imaging for other conditions SIPAC 2003

30 Fungal Ball PE: A single sinus 40% of patients: purulent d/c from involved sinus 10% of patients: polyps SIPAC 2003 F. Pagella et al.: Paranasal sinus fungus ball: Dx & Mx. Mycoses (2007), 50, 451–456

31 Fungal Ball Hx PE Imaging Histopathologic exam. Culture

32 Fungal Ball - Hyperattenuating material filling the Rt. maxillary sinus with central calcific areas of increased attenuation (long arrow). - The circumferential thickening of the osseous walls of sinus (short arrows). (short arrows). Unenhanced CT scan

33 Fungal Ball hyperattenuating fungus ball with calcific foci in Lt. maxillary sinusThe typical hyperattenuating fungus ball with calcific foci in Lt. maxillary sinus (long arrow). thickening of the osseous wallsThe sclerotic thickening of the osseous walls of sinus (short arrows) from chronic sinus inflammation. Axial unenhanced CT scan

34 Fungal Ball

35 Pathology: Gross –Lesions: vary from soft, wet-appearing bundles of debris to firm, gritty & crumbly balls –Color: white, yellow, green, tan, brown & black

36 Fungal Ball Granville et al: Fungal sinusitis, HUMAN PATHOLOGY Volume 35, No. 4 (April 2004)

37 Fungal Culture Usually –ve In Klossek's review (109 patients), only 31% of cases had positive cultures. All of these : Aspergillus fumigatus Usual pathogen: Aspergillus species But Pseudallescheria, Alternaria sp, and other species have been reported. SIPAC 2003

38 Fungal Ball Pathogenesis Unknown Persistence of fungal spores within nasal cavity into maxillary/ other sinus.  When fungal spore is not cleared. (in warm dark recesses of a sinus)  germination & growth. Saprophytic colonization  obstruct sinus ostium& lead to episodes of acute sinusitis and result in a fungal ball.

39 Fungal Ball F. Pagella et al.: Paranasal sinus fungus ball: Dx & Mx. Mycoses 2007, 50, 451–456

40 Fungal Ball Treatment The gold  removal of the hyphal mass + re-establishment of drainage from involved sinus. Antifungal Rx : unnecessary

41 Fungal Ball Postoperative care Saline irrigation & endoscopic debridementsSaline irrigation & endoscopic debridements are indicated until complete healing. No further Rx is required & widely aerated sinus should quickly return to normal. Recurrence  rare

42 Fungal Ball Treatment : Should an asymptomatic patient undergo surgery for an opacified sinus without evidence of bony erosion? This is controversial, and following the patient for symptoms & repeated imaging to assess for progression is also a reasonable path. Fungus ball of paranasal sinus: The Otolaryngologic Clinics of North America 2000

43 Allergic Fungal Rhinosinusitis Epidemiology The most common form of fungal rhinosinusitis Age: –mostly in young adults –Average age at Dx >> 23-26 yr. –Range 7-62 yr. Sex: –Male:Female >> 6:1 SIPAC 2003

44 Allergic Fungal Rhinosinusitis Epidemiology AR: –63% of AFS patients give Hx of AR  allergy testing  70-90% show evidence of atopy. Asthma: –About 50% of patients have asthma. (33-54%) Geography: –More common in the warm humid climates of the southern United States and along the Mississippi River. SIPAC 2003

45 Allergic Fungal Rhinosinusitis Clinical features: Hx Onset: difficult to pintpoint. Symptoms progress slowly (mo./yr. prior to Dx.) Typically presented with prolonged Hx of rhinosinusitis sym.: –Nasal congestion & obstruction –Anosmia –Postnasal drainage SIPAC 2003

46 Allergic Fungal Rhinosinusitis Clinical features: Hx Despite prolong medical Rx (repeated courses of ATB)  fail to improved. May multiple sinus procedures without benefit if overlooked the Dx. SIPAC 2003

47 Allergic Fungal Rhinosinusitis Clinical features: PE By the time of Dx >> advanced  PE findings reflect this. GA: nasal widening, proptosis Incidence of proptosis 20% Reversible blindness from sphenoid involved (several case reports) fungal mucocele formation SIPAC 2003

48 Allergic Fungal Rhinosinusitis Clinical features: PE Intranasal exam. Polyposis -predominantly unilateral, maybe bilat. -often massive & visible at nasal vestibule ** Allergic mucin -mucin: rubbery -difficult to suction out -often visibly nestle within the polyps SIPAC 2003 Sinus Surgery Endoscopic & Microscopic Approaches, Howard L. Leveine 2005

49 Allergic Fungal Rhinosinusitis Laboratory May provide evidence of atopy, but not usually required for Dx CBC: peripharal eosinophilia (7-15%) Elevated total IgE level: mean 668 IU/ml (normal= <125 IU/ml) (radioallergosorbent test for quantifying antigen-specific IgE)RAST (radioallergosorbent test for quantifying antigen-specific IgE) : +ve to multiple fungi. Skin test: +ve to multiple fungi. SIPAC 2003 Sinus Surgery Endoscopic & Microscopic Approaches, Howard L. Leveine 2005

50 Allergic Fungal Rhinosinusitis Laboratory Total IgE fluctuates with disease activity. In a review of 67 patients in Arizona, Schubert & Goetz found: –Total serum IgE correlated significantly with severity of disease. –Importantly, an increase >=10% in total serum IgE during F/U >> strong predictor of recurrence & need for Sx. Schubert MS, Goetz DW. Evaluation & Rx of ARS. J Allergy Clin Immunol. 1998

51 Allergic Fungal Rhinosinusitis Pathology Gross - The distinctive pathology of AFS >> tenacious inspissated mucin / “peanut butter-like” - Thick yellow, brown, or green debris fills the involved sinuses.  similar to fungal ball grossly

52 Allergic Fungal Rhinosinusitis Histopathology Microscope: - Within allergic mucin : “onionskin lamination” / cluster of necrotic & degranulation of eosinophil - Charcot-Leyden crystal. -Hyphal fragments scattered -No fungal tissue invasion Sinus Surgery Endoscopic & Microscopic Approaches, Howard L. Leveine 2005

53 Allergic Fungal Rhinosinusitis Histopathology Special stains for fungus: GMS >> hyphae Sinus Surgery Endoscopic & Microscopic Approaches, Howard L. Leveine 2005

54 Histopathology GMS >> scatter hyphae Periodic acid-Schiff, ×520

55 Allergic Fungal Rhinosinusitis Fungal culture Positive 70-80% of patients diagnosed with AFS. Dematiaceous fungi : the most common based on C/S data  84% of the total positive C/S The most common fungi = Bipolaris species

56 Allergic Fungal Rhinosinusitis Fungal culture Aspergillus species 13% of all fungal C/S

57 Allergic Fungal Rhinosinusitis Fungal culture Appear to be geographic variability in incidence of AFS & in fungal organism –Dematiaceous fungi : most common in the United states –Aspergillus species : most cases reported in the Middle East.

58 Allergic Fungal Rhinosinusitis Staging System Kupferberg et al. >> F/U patients: recurrent following surgery.

59 Allergic Fungal Rhinosinusitis Imaging CT: –Initial study of choice –An important roadmap before Sx >> MultiplesinusesMultiple opacified sinuses Predominantly unilateral, possibly bilateral Expanded sinuses Bone erosionBone erosion into orbit, cranium, or soft tissue of face. hyperattenuationfungal allergic mucinFocal areas within sinuses: hyperattenuation = fungal allergic mucin  irregular, speckled, or serpiginous SIPAC 2003

60 Allergic Fungal Rhinosinusitis Coronal sinus CTCoronal sinus CT Axial sinus CT Sinus Surgery Endoscopic & Microscopic Approaches, Howard L. Leveine 2005

61 Allergic Fungal Rhinosinusitis Imaging MRI –Sinus contents >> low T2 & isointense/hypointense T1 signal –Peripheral mucosa & polyps >> hyperintense on both T1 & T2

62 Allergic Fungal Rhinosinusitis Imaging Bony remodeling / erosionBony remodeling / erosion is common (90%)  from atrophy / the release of inflammatory mediators that dissolve bone, not due to fungal invasion While definitive Dx requires histological verification, the imaging findings are almost pathognomonic imaging findings are almost pathognomonic & facilitate pre-op planning. SIPAC 2003

63 Allergic Fungal Rhinosinusitis Diagnostic Criteria Several criteria have been proposed for the Dx. –Kartzenstein 1983 –Manning 1989 –Ence 1990 –Bent 1994 –deShazo 1995 –Kuferupferberg 1996 –Schubert 1998 –Ponikau 1999 –Schubert 2000 –McCann 2002 –Meltzer 2004 Singhal D et al. Medical interventions for post-surgical Mx of AFRS: The Cochrane Library 2008

64 Allergic Fungal Rhinosinusitis Diagnostic Criteria Presence of allergic mucinPresence of allergic mucin: fundamental criterion for the dis. Bent & Kuhn diagnostic criteria for allergic fungal rhinosinusitis 1.Type I hypersensitivity confirmed by Hx, skin test, or serology 2.Nasal polyposis 3.Characteristic CT scan findings 4.+ve fungal strain of sinus contents 5.Eosinophilic mucus without fungal invasion into sinus tissue Bent JP III, Kuhn FA: Dx of AFS. Otolaryngol Head Neck Surg 1994; 111: 580-588.

65 Pathophysiology & Natural Course Local Mucostasis Mucostasis Anatomic anomaly Anatomic anomalyEnvironmental Fungal exposure Fungal exposure Genetic Atopy Atopy T-lym susceptibility T-lym susceptibility Exposure Fungal proliferation Antigen exposure Anatomicfactors Bacterialinfection Edema Obstruction Obstruction Stasis Stasis Decreased Decreased ventilation ventilation Allergicmucin Inflammation Eosophillic inflammation (MBP, ECP, etc) Inflammatory trigger Gell & Coombs I/III Gell & Coombs I/III T-cell T-cell Other Other The UT Southwestern model of AFS pathogenesis. Local tissue, environ., & immunologic factors converge in pathogenesis of this disease. (Laryngoscope 2001; 111: 1006-1019)

66 Allergic Fungal Rhinosinusitis Natural Course Frequent recurrence Reported recurrent rate 10-100% SIPAC 2003

67 Allergic Fungal Rhinosinusitis Treatment : 1.Surgical –Required in almost all cases. –Goal: removing fungal mucin + widely marsupialize the involved sinuses. –Occasionally, the fungal mucin is so difficult to clear from the maxillary sinus & frontal sinus >> external approach / frontal sinus trephination may be necessary. Marple BF: AFRS: Current theories & Mx strategies. Laryngscope 2001; 111: 1006-1019

68 Allergic Fungal Rhinosinusitis 1.Surgical CT prior SxDis. may distort normal intranasal landmarks & erode important bony barriers to orbit/cranium.  CT prior Sx. Sx alone isn’t sufficient Rx for AFS, it is a crucial first step in Mx. SIPAC 2003

69 Allergic Fungal Rhinosinusitis 1.Surgical Goal : same as primary Sx; allergic mucin, nasal polyps & other sinus obstruction should be removed. Complete Sx to removed all fungal mucin is critical to reduce risk of recurrence.


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