Rhinosinusitis (Fungal)

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Rhinosinusitis (Fungal)

Rhinology
Author Credentials 

Sarah K. Wise, MD
Associate Professor of Otolaryngology
Emory University

Lauren J. Luk, MD
Rhinology Fellow
Emory University

With notable prior contribution from Berrylin FErguson, MD

Module Summary

Fungal rhinosinusitis must be categorized to optimize therapy and predict outcomes. This categorization is most dependent on the host’s immunologic status. Acute invasive disease occurs primarily in the immunocompromised patient and requires rapid recognition and rapid reversal of immunocompromised state if possible, systemic antifungals, and surgical debridement. The other forms of fungal sinusitis are usually chronic. CIFS is treated with long-term antifungals as well as surgical removal. Fungus balls may be asymptomatic and are usually cured with surgical removal in the host with a normal immune system. AFRS occurs in the allergic individual and is diagnosed by characteristic eosinophilic mucin plugs in which hyphae are present. Endoscopic sinus surgery with perioperative steroids are the mainstays of therapy. Recurrence is high and may be reduced with the use of postoperative immunotherapy to the relevant molds and topical steroid therapy.

Module Learning Objectives 
  1. Categorize fungal rhinosinusitis into acute [fulminant] invasive fungal sinusitis (AIFS), chronic invasive fungal sinusitis (CIFS), fungus ball, saprophytic, and allergic fungal rhinosinusitis (AFRS).
  2. Explain the pathophysiology of the various forms of fungal rhinosinusitis.
  3. Recognize that the five categorizations of fungal sinusitis are dependent on the immune status of the host.
  4. Recognize and diagnose the various manifestations of fungal rhinosinusitis on the basis of history, demographics, physical exam, radiologic, histologic, and laboratory findings.
  5. Cite the prognosis and optimal treatment for various forms fungal rhinosinusitis.

Anatomy

Learning Objectives 
  1. Understand how the anatomic deposition of inhaled fungal spores and entrapment of spores in a paranasal sinus can account for frequency of fungal involvement.
  2. Know the sinuses and nasal structures most frequently involved with various manifestations, e.g., invasive fungal sinusitis most commonly initiates on middle turbinate.
  3. Detail the complications of fungal rhinosinusitis relative to surrounding anatomy, particularly the orbit, brain, and cavernous sinus (in sphenoid sinus involvement).
References 
  1. Orlandi RR, Kingdom TT, Hwang PH, et al. International Consensus Statement on Allergy and Rhinology: Rhinosinusitis. Int Forum Allergy Rhinol. 2016;6 Suppl 1:S56, S100.
  2. Loftus PA, Wise SK. Allergic Fungal Rhinosinusitis: The Latest in Diagnosis and Management. Adv Otorhinolaryngol. 2016;79:13-20.
  3. Turner JH, Soudry E, Nayak JV, Hwang PH. Survival outcomes in acute invasive fungal sinusitis: a systematic review and quantitative synthesis of published evidence. Laryngoscope. 2013 May;123(5):1112-8.

Pathogenesis

Learning Objectives 
  1. Understand that the development of fungal sinusitis depends on the host’s immunologic status
    1. Atopy (type I IgE mediated hypersensitivity) – AFRS
    2. Immunocompetent – Fungus Ball
    3. Between immunocompetent and immunocompromised; sometimes no immunocompromise detectable – CIFS
    4. Immunocompromised – AIFS
  2. Understand that nasal cultures obtained with irrigation are positive for fungus in almost everyone and reflect mold counts. Positive cultures must be interpreted in relation to clinical presentation.
  3. Know typical histopathologic appearance of common fungi.
    1. Aspergillus – septate, narrow hyphae, 45 degree branching
    2. Mucor – non-septate broad hyphae, 90 degree branching
  4. Understand that AIFS occurs in the immunocompromised patient. Certain fungal organisms are more common in patients with particular etiologies of immunocompromise.
    1. Neutropenic: Aspergillus, Mucormycosis, Fusarium, etc.
    2. Diabetic (especially in ketoacidosis): Mucormycosis
      1. Mucor: Invades bloods vessels, results in ischemia and classic necrosis in late stages
  5. Know that CIFS has the following characteristics: Rare, chronic course of months to years, mild immunocompromise to immunocompetent
    1. Aspergillus species
      1. Chronic nongranulomatous associated with A. fumigatus
      2. Chronic granulomatous associated with A. flavus in Northern Africa, Sudan, East Asia
    2. Histology: tissue invasion but no acute tissue necrosis, may be accompanied by chronic inflammatory reaction including giant cells and granuloma formation
  6. Know that fungus balls have the following characteristics: No immunocompromised state; inhalation of spore, not cleared by mucociliary action, germinates and grows to form tangled mass of hyphae in one of the paranasal sinuses (generally maxillary antrum or sphenoid) without invasion.
    1. Aspergillus >> Pseudallescheria boydii, Alternaria
    2. If host becomes immunocompromised, invasive fungal sinusitis may develop.
  7. Understand the pathophysiology of saprophytic fungus: No immunocompromised state; inhalation of spore, germinates within the nasal cavity, and forms small fungus ball and is expelled with nose blowing. Know that there is no invasion. It is usually noted incidentally and may be seen in infected/colonized sinus cavities after surgery or in areas where crusting and/or stagnation provide environment for fungal growth.
  8. Understand the following pathophysiology of AFRS: Allergic patient (type I IgE-mediated hypersensitivity), sinus cavities contain classic allergic mucin (eosinophilic, non-invasive fungal hyphae, Charcot Leyden crystals).
    1. Most often involves dematiaceous (darkly pigmented) fungi. Bipolaris, Curvularia, Alternaria, Aspergillus, Fusarium, Penicillium, Deschslera, Exserohilium are common species in AFRS.
    2. AFRS characterized by a primarily T helper cell 2 response (Elevated IgE and IgG to fungus present in allergic mucin).
    3. Total IgE frequently elevated, but fluctuates with disease activity (not required for diagnosis).
    4. Inhalation of spore generates allergic response with secretion of eosinophilic “allergic mucus.” Spore germinates, increasing antigenic stimulation with further mucin production.
    5. Polyps almost always present; bony remodeling, proptosis common without histologic fungal invasion.
References 
  1. Orlandi RR, Kingdom TT, Hwang PH, et al. International Consensus Statement on Allergy and Rhinology: Rhinosinusitis. Int Forum Allergy Rhinol. 2016;6 Suppl 1:S132.
  2. Fokkens WJ, Lund VJ, Mullol J, et al. EPOS 2012: European position paper on rhinosinusitis and nasal polyps 2012. A summary for otorhinolaryngologists. Rhinology. 2012;50(1):1-12.
  3. Loftus PA, Wise SK. Allergic Fungal Rhinosinusitis: The Latest in Diagnosis and Management. Adv Otorhinolaryngol. 2016;79:13-20.
  4. Turner JH, Soudry E, Nayak JV, Hwang PH. Survival outcomes in acute invasive fungal sinusitis: a systematic review and quantitative synthesis of published evidence. Laryngoscope. 2013 May;123(5):1112-8.
  5. D'Anza B, Stokken J, Greene JS, Kennedy T, Woodard TD, Sindwani R. Chronic invasive fungal sinusitis: characterization and shift in management of a rare disease. Int Forum Allergy Rhinol. 2016;6(12):1294-1300.
  6. Duggal P, Wise SK. Chapter 8: Invasive fungal rhinosinusitis. Am J Rhinol Allergy. 2013 May-Jun;27 Suppl 1:S28-30.
  7. Mahdavinia M, Keshavarzian A, Tobin MC, Landay AL, Schleimer RP. A comprehensive review of the nasal microbiome in chronic rhinosinusitis (CRS). Clin Exp Allergy. 2016 Jan;46(1):21-41.
  8. Mirza N, Lanza D. Diagnosis and management of rhinosinusitis before scheduled immuno-suppression: a schematic approach to the prevention of acute fungal rhinosinusitis. Otolaryngol Clin North Am. 2000;33:313-22.

Incidence

Learning Objectives 
  1. Understand that incidence varies with the manifestation and actual epidemiology is unclear since data is extrapolated from comes from case series.
  2. AIFS - reported in literature between 1-3%; incidence is determined by prevalence of immunosuppressed patients (hematologic malignancy undergoing chemotherapy and bone marrow transplant; diabetic ketoacidosis)
    1. Pediatric: systematic review of the literature demonstrates (103 cases)
      1. Male predominance
      2. Median age 11 years old
      3. Absolute neutrophil count <600 for 2 weeks duration in majority of patients
      4. 44% patients with acute lymphocytic leukemia (ALL)
      5. Aspergillius sp. in 47% of cases
      6. 46% mortality
    2. Adults: systematic review of the literature demonstrates (807 cases)
      1. Slight male predominance of 57%
      2. 47.8% patients with diabetes with 23.1% in diabetic ketoacidosis
      3. 39% patients with hematologic malignancy (mainly acute myeloid leukemia)
      4. 50.3% mortality
  3. CIFS - exceedingly rare in the United States. More common arid regions such as India and Sudan.
    1. From few case studies available, appears to be more common in men.
    2. Patients may be immunocompetent or mildly immunocompromised.
    3. Systemic comorbidities reported included diabetes mellitus type 2, long term corticosteroid and immunosuppressant use for kidney transplant, liver cirrhosis. There is one case report of an association with illicit intranasal drug use.
    4. A subtype of CIFS is granulomatous CIFS which may have a less aggressive course and is seen mainly in India, Sudan, and North Africa.
  4. Fungus balls:
    1. Average age is 64 years, no pediatric cases reported
    2. Female predominance, unexplained
  5. AFRS - 6.9% incidence: Varies geographically in the United States and in international reports. Highest in United States along Mississippi River basin and in south/southeastern United States.
    1. Average age: 30 years; range: 3-74 years
    2. AFRS bone erosion/expansion/severity of disease seen more in males, African American and those with lower socioeconomic status
References 
  1. Wise SK, Ghegan MD, Gorham E, Schlosser RJ. Socioeconomic factors in the diagnosis of allergic fungal rhinosinusitis. Otolaryngol Head Neck Surg. 2008;138(1):38-42.
  2. Wise SK, Venkatraman G, Wise JC, DelGaudio JM. Ethnic and gender differences in bone erosion in allergic fungal sinusitis. Am J Rhinol. 2004;18(6):397-404.
  3. Smith A, Thimmappa V, Shepherd B, Ray M, Sheyn A, Thompson J. Invasive fungal sinusitis in the pediatric population: Systematic review with quantitative synthesis of the literature. Int J Pediatr Otorhinolaryngol. 2016 Nov;90:231-235.
  4. Turner JH, Soudry E, Nayak JV, Hwang PH. Survival outcomes in acute invasive fungal sinusitis: a systematic review and quantitative synthesis of published evidence. Laryngoscope. 2013 May;123(5):1112-8.
  5. D'Anza B, Stokken J, Greene JS, Kennedy T, Woodard TD, Sindwani R. Chronic invasive fungal sinusitis: characterization and shift in management of a rare disease. Int Forum Allergy Rhinol. 2016 Dec;6(12):1294-1300.
  6. Pekala KR, Clavenna MJ, Shockley R, Weiss VL, Turner JH. Chronic invasive fungal sinusitis associated with intranasal drug use. Laryngoscope. 2015 Dec;125(12):2656-9.
  7. Ferguson BJ. Fungus balls of the paranasal sinuses. Otolaryngol Clin North Am. 2000;33:389-98.

Patient Evaluation

Learning Objectives 
  1. Understand that the clinical context is critical to determining the necessity of rapid diagnosis.
  2. Know that in the immunocompromised patient (especially with ANC <500), an endoscopic evaluation should be done immediately if the patient has fever associated with imaging abnormality of the paranasal sinuses or symptoms of facial numbness, pain, purulence, or nasal congestion. Suspicion for AIFS should be high in these patients.
    1. Perform biopsy of suspicious areas with immediate frozen section.
  3. Know that fungus balls are usually discovered when a patient, with symptoms of rhinosinusitis refractory to standard medical therapy, undergoes sinus surgery. Understand that 20% of patients are asymptomatic. Fungus balls may also be suspected based upon radiologic characteristics in symptomatic or asymptomatic patients.
  4. Know that patients with AFRS often have the following characteristics: Long-standing history of nasal symptoms; 50% of patients have unilateral disease. Sinus computed tomography (CT), endoscopic harvest of mucin for pathologic evaluation including fungal stains and culture, allergy testing. Disease burden and clinical progression tends to be more severe than regular CRSwNP.

Measurement of Functional Status

Learning Objectives 

Several validated surveys of rhinosinusitis are available, some of which have been applied to AFRS management.

Imaging

Learning Objectives 
  1. Understand that a sinus CT is the most useful imaging modality.
  2. List the findings in the following diseases:
    1. AIFS: Early signs include mucosal thickening of nasal septum, lateral nasal wall, nasal floor (typically not seen in chronic rhinosinusitis), and turbinates. Usually unilateral. Late signs include bone erosion, orbital involvement, facial soft tissue swelling, retroantral fat thickening.
    2. CIFS: sinus thickening or opacification, bone erosion, orbital involvement, dural involvement
    3. Fungus balls: Often hyperdense material present in an opacified or partially opacified sinus seen in 62%, heterogenous microcalcifications seen in 20% predominantly affecting maxillary sinus (84%)
    4. AFRS: Areas of increased opacity representing allergic fungal mucin surrounded by the less dense mucosal hypertrophy and polyps, often described with “serpiginous areas of increased attenuation”, bony erosion/expansion is common but spares dura and periorbita (20-90% of cases)
  3. Role of MRI
    1. AIFS, CIFS: MRI with gadolinium – loss of mucosal contrast enhancement predicts disease involvement, also can be used to evaluate for intracranial disease and to follow patients after initial debridement
    2. AFRS: MRI may show signal void in areas of allergic fungal mucin
    3. Fungus ball: MRI demonstrates a signal void in 50%
References 
  1. Bent JP, 3rd, Kuhn FA. Diagnosis of allergic fungal sinusitis. Otolaryngol Head Neck Surg. 1994;111(5):580-588.
  2. DelGaudio JM, Swain RE, Jr., Kingdom TT, Muller S, Hudgins PA. Computed tomographic findings in patients with invasive fungal sinusitis. Arch Otolaryngol Head Neck Surg. 2003;129(2):236-240.
  3. Kim JH, Kang BC, Lee JH, Jang YJ, Lee BJ, Chung YS. The prognostic value of gadolinium-enhanced magnetic resonance imaging in acute invasive fungal rhinosinusitis. J Infect. 2015;70(1):88-95.
  4. DelGaudio JM, Clemson LA. An early detection protocol for invasive fungal sinusitis in neutropenic patients successfully reduces extent of disease at presentation and long term morbidity. Laryngoscope. 2009;119(1):180-183.
  5. Nicolai P, Lombardi D, Tomenzoli D, et al. Fungus ball of the paranasal sinuses: experience in 160 patients treated with endoscopic surgery. Laryngoscope. 2009;119(11):2275-2279.

Pathology

Learning Objectives 
  1. Understand that the diagnosis of fungal sinusitis depends on histopathology (hematoxylin and eosin) supplemented with special fungal stains (silver, nitroblue tetrazolium violet, Grocott stain)
  2. Understand that a fungal culture is required to determine causative organism in all manifestations. Know that fungal cultures are frequently negative in fungus balls or if patient has been on systemic antifungal agents. Understand that a positive culture alone does not make a diagnosis of AFRS, nor does a negative culture rule it out.
  3. Know that frozen section for AIFS has a high positive predictive value but a low negative predictive value and therefore high clinical suspicion is important.
  4. List the characteristics of the following diagnoses:
    1. AIFS: fungal tissue and/or angioinvasion present
    2. CIFS: fungal tissue/angioinvasion with or without granulomatous reaction
    3. Fungus ball: Tangled fungal hyphae without invasion
    4. AFRS: Eosinophilic mucin with Charcot-Leyden crystals, scattered hyphae and eosinophils with epithelial debris
References 
  1. Montone KT. Pathology of Fungal Rhinosinusitis: A Review. Head Neck Pathol. 2016 Mar;10(1):40-6. 
  2. Papagiannopoulos P, Lin DM, Al-Khudari S, Rajan K, Reddy S, Gattuso P, Tajudeen B, Batra PS. Utility of intraoperative frozen sections in surgical decision making for acute invasive fungal rhinosinusitis. Int Forum Allergy Rhinol. 2017 May;7(5):502-507.

Medical Therapies

Learning Objectives 
  1. Be able to individualize therapy based on the particular fungal manifestation and fungal culture.
  2. AIFS
    1. Know that with AIFS, therapy is futile if the patient is irreversibly immunocompromised, e.g., bone marrow engraftment failure.
      1. Systemic antifungals directed by fungal culture - usually amphotericin B initially; may be changed to newer antifungal agents when culture results available
      2. Reversal of the source of immunocompromise
    2. Know factors that predict worse survival in AIFS – underlying disease (diabetes, hematologic malignancy), elevated CRP > 5.5, neutropenia, facial swelling, nasal septal involvement, shorter symptom duration
    3. In children with AIFS, higher absolute neutrophil count is a predictor of improved survival
  3. CIFS
    1. Stage 1 - sinonasal disease - surgery + itraconazole or voriconazole
    2. Stage 2 - spread to orbit/palate - surgery + itraconazole or voriconazole
    3. Stage 3 - extensive disease - surgery + voriconazole
    4. Amphotericin not recommended
  4. Fungal Ball treatment is surgical only, no need for antifungals
  5. AFRS
    1. Preoperative steroids - recommend use - oral steroids 1 mg/kg prednisone 1-3 days prior to surgery has been shown to decrease mucosal edema on CT and endoscopy for CRSwNP
    2. Postoperative steroids - recommend use in topical preparations to minimize systemic side effects
    3. Steroid complications - Short term use - weight gain, psychosis, insomnia, elevated blood glucose level, hypertension, gastric upset/peptic ulcer disease. Long term use - avascular necrosis of the hip, cataracts and glaucoma, adrenal insufficiency, Cushing’s, osteoporosis.
    4. Oral antifungals - low quality of evidence; benefit balanced by harm; most common side effect is elevated liver enzymes, may be used as an option in selected postoperative AFS cases - itraconazole 200-400 mg PO daily in divided doses has shown benefit in level 4 studies.
    5. Immunotherapy - low quality of evidence; cost is high but benefits and harms are equal. There is consistency in low level studies for clinical effectiveness and may be considered as an option postoperatively. Can be started as soon as 6 weeks after surgery to allow for appropriate mucosal healing.

Pharmacology

Learning Objectives 

See below (AFRS case study)

Surgical Therapies

Learning Objectives 
  1. AIFS
    1. Surgical biopsy for diagnosis – in early invasive fungal sinusitis - tissue may appear non-necrotic but inflamed (should biopsy)
    2. Serial surgical debridement of necrotic tissue
    3. Indications for orbital extenteration unclear – recent studies show this procedure does not change outcome for most patients
  2. CIFS – conservative endoscopic resection guided by intraoperative findings until bleeding tissue encountered with conservation of vital structures (intraorbital structures, cranial nerves, dural involvement) despite apparent involvement on MRI
  3. For fungus balls, surgical removal is usually curative.
  4. For AFRS, endoscopic removal of all allergic mucin, systemic steroids perioperatively and topical steroid therapy for maintenance
    1. Surgical goals: widely marsupialize sinus cavities, extended sinusotomies may be helpful in accessing disease and maintaining long term patency
    2. High recurrence and persistence of disease in most series otherwise
References 
  1. Orlandi RR, Kingdom TT, Hwang PH, et al. International Consensus Statement on Allergy and Rhinology: Rhinosinusitis. Int Forum Allergy Rhinol. 2016 Feb;6 Suppl 1:S22-209.
  2. Gan EC, Thamboo A, Rudmik L, Hwang PH, Ferguson BJ, Javer AR. Medical management of allergic fungal rhinosinusitis following endoscopic sinus surgery: an evidence-based review and recommendations. Int Forum Allergy Rhinol. 2014;4(9):702-715.
  3. Cho HJ, Jang MS, Hong SD, Chung SK, Kim HY, Dhong HJ. Prognostic factors for survival in patients with acute invasive fungal rhinosinusitis. Am J Rhinol Allergy. 2015;29(1):48-53.
  4. Zuniga MG, Turner JH. Treatment outcomes in acute invasive fungal rhinosinusitis. Curr Opin Otolaryngol Head Neck Surg. 2014;22(3):242-248.
  5. Green KK, Barham HP, Allen GC, Chan KH. Prognostic Factors in the Outcome of Invasive Fungal Sinusitis in a Pediatric Population. Pediatr Infect Dis J. 2016;35(4):384-386.
  6. D'Anza B, Stokken J, Greene JS, Kennedy T, Woodard TD, Sindwani R. Chronic invasive fungal sinusitis: characterization and shift in management of a rare disease. Int Forum Allergy Rhinol. 2016;6(12):1294-1300.
  7. Rupa V, Maheswaran S, Ebenezer J, Mathews SS. Current therapeutic protocols for chronic granulomatous fungal sinusitis. Rhinology. 2015;53(2):181-186.
  8. Fokkens WJ, Lund VJ, Mullol J, et al. EPOS 2012: European position paper on rhinosinusitis and nasal polyps 2012. A summary for otorhinolaryngologists. Rhinology. 2012;50(1):1-12.
  9. Ferguson BJ. Fungus balls of the paranasal sinuses. Otolaryngol Clin North Am. 2000;33:389-98.
  10. Landsberg R, Segev Y, DeRowe A, Landau T, Khafif A, Fliss DM. Systemic corticosteroids for allergic fungal rhinosinusitis and chronic rhinosinusitis with nasal polyposis: a comparative study. Otolaryngol Head Neck Surg. 2007;136(2):252-257.
  11. Konstantinidis I, Constantinidis J. Medial maxillectomy in recalcitrant sinusitis: when, why and how? Curr Opin Otolaryngol Head Neck Surg. 2014;22(1):68-74.
  12. http://www.american-rhinologic.org/videos (Surgical dissection videos on the ARS website, for members. ARS membership is FREE for residents.)

Staging

Learning Objectives 
  1. CIFS (as above in Treatment)
  2. AFRS - clinical severity staging system based on CT scan and location of bony remodeling on a scale of 0 - 24.
References 
  1. Wise SK, Rogers GA, Ghegan MD, Harvey RJ, Delgaudio JM, Schlosser RJ. Radiologic staging system for allergic fungal rhinosinusitis (AFRS). Otolaryngol Head Neck Surg. 2009 May;140(5):735-40.

Case Studies

  1. AIFS: A young woman in diabetic ketoacidosis is stabilized in the intensive care unit. Two days later she develops numbness of her cheek and a sinus CT shows mucosal thickening of the maxillary and ethmoid sinuses on that side. Endoscopic evaluation shows no purulence or necrosis but a small pale patch of mucosa on the anterior aspect of the middle turbinate.
    1. Biopsy of the middle turbinate is performed as well was endoscopic debridement of the maxillary and ethmoid sinuses on that side
    2. Frozen section shows broad, nonseptate hyphae in the tissue with vascular invasion
    3. Amphotericin B is initiated and patient is placed on an insulin drip for strict diabetic control
    4. Patient responds rapidly with resolution of numbness within 10 days
    5. She receives a total of 2.5 grams of amphotericin B over the next 4 weeks and recovers without recurrence.
  2. Fungus ball: An elderly man presents with a one-year history of unilateral parietal discomfort and increased postnasal discharge. Endoscopic evaluation is normal. Sinus CT shows an opacified sphenoid sinus on the side of clinical symptoms.
    1. Endoscopic sphenoidotomy reveals gritty gray, dark green material in sinus that is removed with the aid of irrigation.
    2. Histopathology shows a tangled mass of fungal hyphae; fungal culture is negative.
    3. Patient makes an uneventful recovery with resolution of parietal discomfort and postnasal discharge.
  3. AFRS: A 16-year-old boy presents with a three-month history of diplopia on leftward gaze, right-sided proptosis, and right-sided nasal obstruction. Endoscopic evaluation shows right-sided polyps with sticky green mucin present. Sinus CT shows opacification of right ethmoid, sphenoid, and maxillary sinuses with erosion of right lamina papyracea, and heterogeneity of soft tissue densities within the sinus cavities. Patient has symptoms of seasonal allergic rhinitis.
    1. Endoscopic sinus surgery with removal of polyps and large amounts of inspissated tenacious rubbery green mucin from the affected sinuses on right side.
    2. Histopathology with fungal stain shows eosinophilic mucin with non-invasive fungal elements and Charcot-Leyden crystals.
    3. Fungal culture grows Curvularia.
    4. Patient had resolution of diplopia and nasal obstruction postoperatively and was treated with a four-week tapering course of prednisone and saline nasal washes.
    5. He was lost to follow-up and returned six months later with nasal obstruction but no diplopia. Imaging showed maxillary opacification with heterogeneity and nasal polyps without sphenoid or ethmoid involvement.
    6. Revision endoscopic maxillary antrostomy consistent with AFRS, confirmed on repeat histology. Fungal culture showed Curvularia.
    7. Patient was again treated with perioperative steroids (four weeks) and immunotherapy to multiple molds including Curvularia, as well as other environmental allergens.
    8. Endoscopic evaluation every two months showed no recurrence. After three years on maintenance therapy, the allergy shots were discontinued.
  4. There are many scenarios other than these sometimes involving more than one manifestation, e.g., a fungus ball that becomes invasive after immunocompromise. Each scenario requires individual attention and careful consideration of diagnostic and treatment options.

Complications

Learning Objectives 
  1. Understand that complications depend primarily on the fungal manifestation, which is dependent on host defenses.
  2. Know that in the immunocompromised patient, the ultimate complication of AIFS is death, and this may occur if the disease is not recognized quickly with surgery to debride the necrotic tissue, institution of systemic antifungal agents, and reversal of immunocompromise.
  3. Understand that orbital involvement is not uncommon in AIFS. In the blind eye, orbital exeneration is often recommended but not always necessary.
  4. In AIFS, know that if the source of immunocompromised state cannot be reversed, therapy is usually futile and death may result.
  5. Understand that in fungus balls, bacterial superinfection, with the fungus ball acting as the “foreign body,” is possible. In sphenoid fungus balls intracerebral bleed and infarct may occur as a complication of surgical removal.
  6. Understand that in AFRS, blindness has been reported that was reversible with removal of the sphenoid mass causing compression of optic nerve; proptosis and intracranial extension with erosion of cranial vault are not uncommon. Encephaloceles may occur with removal of allergic fungal debris if a large cranial base bony defect is present. Endoscopic removal should avoid powered instrumentation in areas of bony dehiscence.
References 
  1. Orlandi RR, Kingdom TT, Hwang PH, et al. International Consensus Statement on Allergy and Rhinology: Rhinosinusitis. Int Forum Allergy Rhinol. 2016;6 Suppl 1:S22-209.

Review

Review Questions 
  1. What are the major categorizations of fungal sinusitis? What is the most important determinant of which categorization is manifested?
  2. List clinical characteristics of each fungal manifestation.
  3. Describe possible sinus CT imaging findings for each fungal manifestation.
  4. What are the three mainstays of treatment of invasive fungal sinusitis?
  5. Describe the treatment and prognosis for fungus balls and allergic fungal sinusitis.
Resources 

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  4. Surgical Videos: