Cardinal Signs of Sinusitis

  • ≥2 of these signs must be present to consider the diagnosis of chronic rhinosinusitis
    • Anterior and/or posterior mucopurulent drainage
    • Nasal obstruction
    • Facial pain, pressure, and/or fullness
    • Decreased sense of smell


Chronic Rhinosinusitis (CRS)

  • Defined as >12 weeks (despite medical management)
  • Associated conditions/risk factors: smoking, immunodeficiency (sNP), allergic rhinitis, AERD (cNP), ciliary dyskinesia, repeated sinus surgeries, CF, Wegener's granulomatosis, Churg-Strauss (cNP), sarcoidosis
  • Bacteria associated with CRS (with or without polyps):
    • CRS: S. pneumoniae, H. influenzae, M. catarrhalis, anaerobes; less commonly MRSA, gram-negative enteric bacteria
    • Acute exacerbation of CRS: H. influenzae, S. pneumoniae, M. catarrhalis, S. aureus, K. pneumoniae, and anaerobes.
    • Special populations:
      • Neutropenic: Fungi (Aspergillus commonly, also Mucor, Rhizopus, Alternaria) and Pseudomonas
      • Diabetes mellitus: Fungi, S. aureus, streptococci, gram-negative enteric bacteria
      • CF: Pseudomonas
      • HIV: Pseudomonas, S. aureus, streptococci, anaerobes, and fungi (Aspergillus, Cryptococcus, and Rhizopus). Rarely CMV, non-tuberculous mycobacteria (M. kansasii) and parasites (Microsporidium, Cryptosporidium and Acanthamoeba)
    • Sinuses house a diverse microbiome that is related to sinus health
      • Microbiome collapse is associated with CRS; loss of protective lactic acid bacteria is a hallmark of chronic disease
      • Corynebacterium tuberculostearicum is an overlooked pathogen in CRS
  • Unilateral sinusitis suggests
    • Dental abscess - usually in the maxillary sinus above the site of the abscess, foul-smelling due to microaerophilic Strep species infection, diagnosed by dental XR
    • Fungal sinusitis
    • Nasal polyp
    • Mucocele
    • Tumor of the sinus or nose (e.g. inverted papilloma)
    • Congenital aplasia, hypoplasia


With Nasal Polyps (cNP)
  • Usually bilateral; unilateral polyps should prompt consideration of alternative diagnoses (inverting papillomas, nasal tumors, etc.)
  • Usually arise from middle meatus and ethmoid cells (rarely from inferior turbinates)
Without Nasal Polyps (sNP)

CRScNP

Allergic Fungal Rhinosinusitis (AFRS)

CRSsNP

Diagnostic Criteria
  • 2 of the cardinal signs of sinusitis
    • Anosmia may be more frequent than in other types of CRS
  • Objective documentation with rhinoscopy or sinus CT
  • Bilateral nasal polyps in middle meatus
  • 2 of the cardinal signs of sinusitis
  • Objective documentation with rhinoscopy or sinus CT
  • Positive fungal stain (hyphae) or culture of allergic mucin (obtained via surgery)
  • IgE-mediated fungal allergy (via skin or in-vitro test)

Hamilos - AFRS should be suspected in an immunocompetent patient with the following:
  • 1 opacified sinus on CT despite therapy (antibiotics and oral steroids); least specific finding for AFRS
  • Characteristic CT hyperdensities within the opacified sinuses, which suggest accumulated allergic mucin
    • Not entirely specific for AFRS and are not required to make the diagnosis
    • Allergic mucin alone is neither highly sensitive/specific for AFRS and can be seen in other subtypes of CRS
  • Evidence of IgE-mediated allergy to fungus by skin test (prick or ID) and serum specific IgE
    • Most patients show allergy to >1 fungus, although sensitization to multiple fungi is not required for the diagnosis
  • Establishing the diagnosis almost always requires surgery to confirm allergic mucin which should be examined pathologically for degranulating eosinophils, fungi (by staining or culture); the pathology of sinus tissue should not show fungal invasion

Eosinophilic Mucin Rhinosinusitis - basically AFRS with negative fungal stain and culture
  • 2 of the cardinal signs of sinusitis
    • Facial pressure/pain may be more prominent than in in other types of CRS
  • Objective documentation with rhinoscopy or sinus CT
Clinical Features
  • 20-33% of CRS
  • Associated with CF (<1%, neutrophilic polyps with pediatric onset), AERD (40%), primary cililary dyskinesia (neutrophilic), Churg-Strauss
  • Immunodeficiency rarely found
  • 8-12% of CRS
  • Advanced disease with complications of bony erosion and facial dysmorphia
  • Immunodeficiency and AERD rare
  • Associated fungi
    • Dematiaceous fungi most common: Bipolaris spicifera, Curvularia lunata, Exserohilum rostratum, and Alternaria spp.
    • Less common: Aspergillus fumigatus, other Aspergillus species, Epicoccum nigrum, Fusarium solani
  • 60-65% of CRS
  • 12% with defective/deficient antibody production
  • AERD rarely found
Evaluation
  • Sinus CT (multiplanar)
    • Consider obtaining before initial treatment of CRS because history has low predictive value
    • May demonstrate mucosal thickening, ostial obstruction, polyps, and opacification
      • Many anatomical variants (e.g., a deviated septum with a readily visualizable middle turbinate) are often misconstrued to be a polyp
      • Significant hyperplastic disease observed on a sinus CT can strongly support the diagnosis of polyps
    • Allergic mucin has characteristic ‘‘hyperdense’’ appearance and is not specific to AFRS
    • If isolated sphenoid disease or sinus wall distortion obtain MRI
  • Sinus MRI
    • Further delineates allergic mucin in opacified sinus (hypoattenuated areas on T2 images)
    • Identifies multiple polyps crowded together with mucus (which have same density on CT)
  • Rhinoscopy/endoscopyabpa_vs_afs.png
    • Document purulent mucus from the middle meatus, edematous/hyperemic mucosa, polypoid changes or frank polyps. If negative exam consider imaging.
  • Bacterial culture - obtain from sinus ostia only to document unusual or drug-resistant organisms
  • Fungal culture of allergic mucin - due to technical difficulty may be negative despite presence of fungal hyphae on stain
  • Allergy evaluation - aeroallergen sensitization found in 60% with CRS (vs. 30-40% general population)
  • Immunodeficiency evaluation - immunodeficiency found in 12% with CRSsNP
    • Immunoglobulin levels, Ig subclasses, specific antibody response to pneumococcus
  • Evaluation for systemic disease
    • CBC/diff, ESR
    • ANCA - found in 40-60% with Churg-Strauss, 60-90% with Wegener's
    • Serum angiotensic converting enzyme (ACE) level - elevated in 75% of untreated patients with sarcoidosis
  • Total IgE and fungal IgG precipitins (for AFRS)
    • Neither total IgE levels nor fungus-specific IgG precipitins are as strikingly elevated in AFRS as in ABPA, and neither is required to establish the diagnosis
    • Total IgE often >1000 IU/mL
    • Precipitins to the implicated fungus present in most (not all) cases
Treatment
(Hamilos)
  • Nasal lavage >200 mL each nostril QD to multiple times daily
  • Intranasal steroids
    • Nasal spray (Flonase, Nasonex, Rhinocort) up to 2 sprays each nostril BID
    • If symptoms persist: Budesonide respules 0.5 mg/2mL vial + 5 mL saline (7 mL total) each nostril followed by head-positioning QD
  • LTRA - adjunct to intranasal steroids, may be more effective in patients with AERD
  • Medical polypectomy - oral corticosteroids for initial treatment of nasal blockage or anosmia
    • Prednisone 20 mg PO BID x 5 days then 10 mg PO BID x 5 days, then 10 mg PO QD x 5 days, then stop
  • Maintenance treatment - intranasal steroids
  • Surgical polypectomy - considered if steroids do not reduce polyps sufficiently (persistent obstruction or anosmia)
  • Antibiotics
    • Doxycycline 200 mg PO on day 1 then 100 mg PO QD x 20 days - small reduction in polyp size
  • Aspirin desensitization for AERD
  • Omalizumab - in a small study of patients with comorbid asthma (+/- atopy), omalizumab x 4 months significantly decreased polyp size
  • Cigarette smoke cessation
  • Optimal treatment of allergic rhinitis (if present)

Controversial treatments:
  • Oral/topical antifungals suggested for persistent mucosal thickening and mucus production with no evidence of bacterial infection, who have failed intranasal steroids
    • Itraconazole (adults) 200 mg PO BID x 3 mos with AST/ALT qMo. If good response, continue with topical amphotericin B or itraconazole.
  • Surgery - remove inspissated mucus and maximize sinus ventilation and drainage
  • Post-surgery:
    • Prednisone 0.5 mg/Kg PO QD tapered over a few weeks to ~10 mg PO QD, then dose is slowly reduced by 1-2.5 mg/week to the lowest dose necessary to maintain control symptoms.
    • When mucosa has healed following surgery: Budesonide respules 0.5 mg/2mL vial + 5 mL saline (7 mL total) each nostril followed by head-positioning QD
    • Itraconazole 200 mg PO BID x 3-6 months (monitor LFTs QMo), then wean and maintain on topical steroid irrigation
    • Schubert: Prednisone 0.5 mg/Kg/d PO x 2 wks, then 0.5 mg/Kg PO QOD x 2 weeks, then taper to 5-7.5 mg/day PO over 3 months, and then continue on this dose for up to a year. Use short bursts of prednisone for any intercurrent exacerbation. If AFS requires repeat surgery restart prednisone protocol.
  • Cigarette smoke cessation
  • Optimal treatment of allergic rhinitis

Controversial treatments:
  • Topical amphotericin B or itraconazole should be considered optional unproved treatments for AFRS
  • Allergen immunotherapy for fungus (based on positive SPT or sIgE) - may be safe and reduce disease recurrence
  • Nasal saline lavage >200 mL each nostril QD to multiple times daily
  • Intranasal topical steroids
    • Nasal spray (Flonase, Nasonex, Rhinocort) up to 2 sprays each nostril BID
    • If symptoms persist: Budesonide respules 0.5 mg/2mL vial + 5 mL saline (7 mL total) each nostril followed by head-positioning QD
    • LTRA - adjunct to intranasal steroids
  • Intensive medical treatment - initial approach to patient with CRSsNP
    • Prednisone 20 mg PO BID x 5 days, then 20 mg PO QD x 5 days
    • Oral antibiotics x 3-4 weeks; extend up to 6 weeks if symptoms not entirely resolved at end of course (or continue antibiotics for 1 week after symptoms cleared)
      • Antibiotic choice guided by culture from middle meatus or other sinus ostium
      • Empiric antibiotics to cover common organisms:
        • Amox-clav 500 mg PO TID, 875 mg PO BID, OR 1000 mg ER PO BID
        • Clindamycin 450 mg PO TID
        • Moxifloxacin 400 mg PO QD
        • If MRSA suspected: Clindamycin, or metronidazole plus TMP-SMX or linezolid
        • If refractory: Metronidazole plus cefuroxime, cefdinir, cefpodoxime, levofloxacin (adults), azithromycin, clarithromycin, or TMP-SMX
    • Alternative to prednisone/antibiotics is long term macrolides x 3 months
    • In a study of children (6-17 years old), the following regimen for intensive medical treatment of CRSsNP was effective and well-tolerated:
      • Amox/clav at 45/6.4 mg/kg/d (maximum, 2000/285 mg/d) x 30 days
      • Methylprednisolone PO taper x first 15 days according to the following schedule: 1 mg/kg/d (maximum, 40 mg/d) for 10 days, 0.75 mg/kg/d for 2 days, 0.5 mg/kg/d for 2 days, and 0.25 mg/kg/d for 1 day
  • Additional course of intensive medical treatment for incomplete improvement in symptoms/CT findings if no clear indication for surgery and no alternative diagnosis. If second course fails then sinus surgery is logical next step.
  • Maintenance treatment: intranasal steroids + nasal saline lavage
  • Cigarette smoke cessation
  • Optimal treatment of allergic rhinitis (if present)

Controversial treatments:
  • Topical itraconazole or amphotericin-B: suggested for persistent mucosal thickening and mucus production with no evidence of bacterial infection, who have failed intranasal steroids

PRACTALL (2013)

Practall CRScNP.png

Practall CRSsNP.png

CRS Surgical Treatment (Children)

  • Most supported surgical approach to the child with CRS who has failed maximal medical therapy probably consists of an initial attempt at an adenoidectomy with a maxillary sinus wash plus/minus balloon dilation followed by FESS in case of recurrence of symptoms
  • In children with CF, nasal polyposis, antrochoanal polyposis, or AFS, FESS to decrease disease burden is the initial favored surgical option



Chronic Bacterial Rhinitis (Kaliner)

  • Not currently recognized as a specific disease

Clinical Features

  • Frequently a child with frequent URI, nasal congestion, facial pain/pressure/headache, runny nose with crusting green nasal drainage, post nasal drainage causing throat clearing cough
  • No anosmia
  • Normal CT
  • Culture positive for Staph or Strep
  • Exam may demonstrate contact points (septal spurs, septum/turbinate contact)

Treatment

  • Mupirocin (Bactroban 2%) ointment
    • Instill locally with finger or Q-tip and massage back, vs.
    • Add 1/2-1" strip in 1 oz. of hot water in sinus rinse bottle, shake until dissolved, fill to 4 or 8 oz., add salt (or salt packet), shake and perform nasal saline sinus rinse procedure




Patient Resources and Handouts










References