Granulomatous conditions in ENT are rare conditions that we come in contact with, we tend to overlook them because they are so rare, however some of the conditions like TB and syphillis and Mucormycosis of the Nose and PNS are seen in our clinics
this is a good summary from scotts brown chapter
2. Granuloma formation
• Response of immune system to an indigestible agent
• Usually neutrophils remove agents by phagocytosis &
digestion
• Macrophages phagocytose indigestible agents, loose their
mobility & accumulate at site of injury
• Undergo structural changes: Larger with more cytoplasm
epitheloid cells
• Epitheloid cells fuse Multinucleated giant cells
• Nuclei arrange in horse shoe pattern Langerhans giant
cells
• All these cells are surrounded by a collar of lymphocytes
4. Granuloma
• Noncaseating or Caseating
– Central area of necrosis
– “Cheese-like” appearance
– Often visible macroscopically
5. Granulomatous diseases
• Infective
• Inflammatory
• Neoplastic
• Forms part of a spectrum of conditions in which
vasculitides play a role
• Many are systemic conditions
– Preferentially target or present in the upper
respiratory tract
8. Wegener’s Granulomatosis
• Relatively common disease of upper airway
• Friedrick Wegerner
– 1939: Necrotizing granulomas and vasculitis of upper
& lower airway, occurring either together or as
separate components
• Coexistence of vasculitis & granulomas
• Classically involves triad:
– Airway
– Lung
– Renal disease
10. • 3 main forms of WG
– Type 1
• Limited form
• Symptoms of URTI persisting for weeks & not responding to
A/b
• Pain over the dorsum of the nose
• Epistaxis & variable degree of nasal obstruction
• Very large crusting of the nasal cavities & nasopharynx
bilaterally
• When crusts are removed – mucosa is very friable
• Septal perforations & eventual nasal collapse in advanced
disease
Wegener’s Granulomatosis
14. Wegener’s Granulomatosis
• End result of advanced
disease
– Infiltration of orbit
– Sclerosis and opacification of
nasal cavity
– Septal erosion
15. Wegener’s Granulomatosis
• End result of advanced
disease
– Infiltration of orbit
– Septal destruction
– Hyperostosis
– Opacification of sinuses
16. • 3 main forms of WG
– Type 2
• Sicker patient with more systemic symptoms
• Pulmonary involvement
– Cough & pleuritic pain
– Haemoptysis & cavitating lesions on CXR
– Encapsulated lung abscess
Wegener’s Granulomatosis
17. • 3 main forms of WG
– Type 3
• Widely disseminated with involvement of multiple organs
– Airway
» Short segment of subglottic/upper tracheal stenosis
– Pulmonary
– Renal
» Hematuria & abn urinary sediment & segmental/diffuse
glomerulonephritis
– Cutaneous
» Tick-bite like lesions on the distal limbs
– Oral
» Hyperplastic granular lesion of gingiva; ulcerative stomatitis
– Otological
» OME ± mastoiditits & profound SNHL
– Eye
» Conjunctivitis; Dacrocystitis; Episcleritis; Orbital pseudotumour
Wegener’s Granulomatosis
21. Wegener’s Granulomatosis
• Main histological features:
– Vasculitis involving medium & small vessels
– Granulomatous necrosis (can be non-necrotic);
Large epitheloid cells; lined with histiocytes
– Scattered multinucleated giant cells
22. Wegener’s Granulomatosis
• Treatment:
– Prednisone 1mg/kg/day + Cyclophosphamide 2mg/kg/day x
1/12
• Prednisone tapered to alternate days x 2/12 & then discontinued once
complete response achieved
• Cyclophosphamide continued x 6/12 to 1 year & then tapered over a
few months
– Other
• Azathioprine
• Methotrexate
• Plasma exchange Ig infusion
– Nose
• Topical steroids, nasal douching & irrigation
– Surgical reconstruction
• Wait until disease has been in remission for some time
23. Sarcoidosis
• Systemic condition of unknown aetiology
• Can involve any organ including many H&N structures
• Incidence = 64/100,000 in Scandinavia
• Condition of young adults between 3rd & 5th decade
• Female preponderance = 2:1
• Ratio of black to Caucasian = 12:1
• Nasal manifestation almost always part of multisystem
sarcoid which have been present for some years
25. Sarcoidosis
• Histology
– Epitheloid cells surrounded by lymphocytes &
fibroblasts, but devoid of caseation
– Crystalline or calcified inclusion bodies sometimes
seen (Schaumann bodies)
39. Churg-Strauss Syndrome
• Nasal involvement
– Usual polyposis
– May also have crusting & septal perforation
– Doesn’t display diffuse mucosal destruction like
with WG
41. Giant cell Granuloma
• “giant cell reparative granuloma” or “giant cell
reaction of bone”
• Benign condition of jaws & other craniofacial
sites
• Commonly occur in children & young adults
42. Giant cell Granuloma
• Clinical features
– Maxilla & mandible most commonly affected
– sphenoid bone temporal bone
– Pain & swelling over affected bone
– Diplopia; frontal headache; hearing loss; vertigo;
tinnitus
43. Giant cell Granuloma
• Imaging
– Expansile lytic lesions
– “Soap bubble” centre
– Well demarcated edges
46. Cholesterol granuloma
• Granulomatous reaction to cholesterol crystals
precipitated in tissues
• Presumed 2ᴼ to haemorrhage and/or trauma
• Lesions may affect maxilla, frontal sinus,
temporal bone
• Produce expansion of bone, cosmetic
deformity & displacement of adjacent
structures
47. Cholesterol granuloma
• Imaging
– Cyst-like expansion of bone/sinus
– Opaque on CT & not contrast enhancing
• Histo
– Granulation tissue of giant cells surrounding clefts
created by cholesterol granulation
• Treatment
– Surgical excision & complete removal of granulation
49. Rhinoscleroma
• Chronic granulomatous disease of the resp tract
– Nose; larynx; trachea & bronchi
• Klebsiella rhinoscleromatis
• Occur @ any age in either sex
– > common in middle aged females
• Central & South-eastern Europe; North Africa;
Indian subcontinent; Indonesia; South America
50. Rhinoscleroma
• 3 stages
– Catarrhal stage
• Foul-smelling purulent rhinorrhoea (weeks – months)
– Atrophic stage
• Large, foul-smelling nasal plaques or crusts
• Same as lesions in atrophic rhinitis
– Granulomatous or proliferative stage
• Multiple granulomatous, non-ulcerative nodules throughout
nose, pharynx, larynx, trachea & bronchi
• Bluish red & rubbery and later paler & harder
– Cicatrizing stage
• Scaring, adhesions, stenosis & distortion of normal anatomy
52. Rhinoscleroma
• Histo
– Scattered large foam cells (Mikulicz cells)
• Vacuolated cells with a central nucleus
• Contain the indigestible bacilli & Russel bodies
• Diagnosis
– Clinical features
– Microbiology
– Histological features
53. Rhinoscleroma
• Treatment
– Treatment must be intense & prolonged
– Streptomycin 1g/day + Tetracycline 2g/day x 4/52
– Continued till 2 x consecutive negative cultures
– Rifampicin, Bactrim & Ciprofloxacin
– Surgical debridement prior to A/b in granulomatous
stage
– Surgery to “core out” scar tissue in Cicatrizing phase
55. Tuberculosis
• Nasal involvement
– Lupus Vulgaris
• Begins in vestibule, then extends to adjoining skin &
mucosa
• Apple jelly nodules (Does not blanch)
• May form ulcers with undermined edges
57. Tuberculosis
• Nasal involvement
– Lupus Vulgaris
• Begins in vestibule, then extends to adjoining skin & mucosa
• Apple jelly nodules (Does not blanch)
• May form ulcers with undermined edges
– Ulcerative form
• Involves cartilaginous nasal septum or inferior turbinate
• Nasal floor is spared
• Nasal obstruction, pain, discharge, crusting, epistaxis
• Septal perforation, but not dorsal saddling
– Sinus granuloma
• Soft tissue mass with/without bone destruction
59. Tuberculosis
• Laryngeal involvement
– Occurs in association with pulm TB
– Dysphonia, Dysphagia, Otalgia
– Diffusely oedematous & reddened larynx
• Predominantly affects post ⅓ of larynx
• May have ulceration (confused with Sq cell CA)
70. Leprosy
• Hansen’s disease
• AFB: Myrobacterium leprae
• Epidemiology
– 12 – 15 million people affected world wide
– Brazil, India, Indonesia, Myanmar, Nigeria: 82% of
cases
– Commonly manifests ages 10-20 years
– Nasal d/c = principal route of transmission
71. Leprosy
• 7th WHO Expert Committee on Leprosy
– Hypopigmented or reddish skin lesion w definite
loss of sensation
– Involvement of peripheral nerves (thickening or
loss of sensation
– Skin smear + for AFB,s
72. Leprosy
• 2 distinct polar forms of disease w spectrum in
between
– Tuberculoid Leprosy:
• Strong host resistance
• Non-infective
• Localized
– Lepromatous Leprosy
• Poor host resistance
• Infective
• Systemic with widespread involvement of tissues
88. Aspergillus/Mucor
• Non-invasive fungal sinusitis / rhinosinusitis
– Fungus ball
• Only described in adults in the literature
• Immunocompetent patients
• Aspergillus most common
• Tangled mats of hyphae in one or more sinuses
• Extramucosal disease
• Maxillary sinus sphenoid sinus other sinuses
• Symptom = Post-nasal d/c , asymptomatic
• CT: heterogeneous opacification
• Surgical removal by endonasal under endoscopic guidance
96. T/NK cell Lymphoma
• “midline destructive granuloma”
• Classical destruction of the midface
• Similar appearance of aggressive midfacial
destruction by cocaine abuse
• All decades of life (mean = 50years)
• Both sexes
• Association with EBV
97. T/NK cell Lymphoma
• 3 stages:
– Prodromal
• Persistent nasal obstruction & rhinorrhoea
– Second stage
• Necrosis around nasal cavity
• Purulent nasal d/c, crusting, tissue loss
– Terminal stage
• Haemorrhage & gross mutilation of face
• Eventually lead to death