This presentation describes cholesteatoma and its treatment

1. Cholesteatoma
Dr. T. Balasubramanian M.S.
D.L.O.
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2. Definition
• It is defined as a cystic bag like structure
filled with desquamated squamous debris
lying on fibrous matrix.
• In lay terms it is defined as “skin in wrong
place”
• It is also known as keratoma
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3. History
1. The term cholesteatoma was coined by Johannes Muller in
1838.
2. Virchow, in 1855, considered cholesteatoma to be a tumor
arising from the metaplasia of mesenchymal cells to
epidermal cells, growing then as tumoral cells.
3. Politzer, in 1869, assumed that cholesteatoma was a
glandular neoplasm of middle ear mucosa.
4. Bezold and Habermann, in 1889, considered cholesteatoma
to be the result of migration of the external ear canal
epidermis into the tympanic cavity via a marginal perforation
after acute or chronic otitis.
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4. Histology
• Expansile lesion of temporal bone
composed of:
1. Cystic content: Desquamated keratin center
2. Matrix: Keratinizing stratified squamous epithelium
3. Perimatrix: granulation tissue that secretes multiple proteolytic
enzymes capable of bone destruction. (Lie in contact with bone).
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5. Classification
• Congenital
• Acquired:
1. Primary
2. Secondary
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6. Congenital cholesteatoma
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7. Congenital cholesteatoma
Levenson’s definition:
• White mass behind the normal ear drum
• Normal pars flaccida and pars tensa
• No prior history of perforation / Otorrhoea
• No previous otological procedures
• Prior bouts of otitis media were not grounds for exclusion
as was the case in original definition
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8. Theories “Congenital
cholesteatoma”
• Teed’s epithelial cell rest theory
• Friedberg’s implantation theory
• Ruedi’s invagination theory
• Aimi’s epithelial migration theory
• Michael’s epidermoid formation theory
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9. Teed’s theory
• Proposed in 1936
• Persistence of squamous cell rests in the
temporal bone led to the formation of
congenital cholesteatoma.
• Presence of squamous cell rests in the
temporal bone – fairly common. They
involute at a later date.
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10. Implantation theory
• Friedberg observed viable squamous cells
in the amniotic fluid present in the middle
ear of neonates.
• Could they be a cause for congenital
cholesteatoma?
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11. Invagination theory
• First proposed by
Ruedi
• Suggested inutero
infection of ear drum
causing it to
invaginate into the
middle ear cavity.
• These invaginations
usually predispose to
cholesteatoma
formation
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12. Epithelial migration theory
• First proposed by Aimi
• He suggested that ectoderm of external
canal managed to grow / migrate in to the
middle ear cavity somehow overcoming
the restraining influence of the annulus.
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13. Epidermoid formation theory
• First proposed by Michael
• He observed nests of squamous
epithelium in the lateral wall of tympanic
cavity below the level of pars flaccida
• These nests normally involute
• Failure of this involution process can
possibly cause cholesteatoma
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14. Features of congenital
cholesteatoma
• Origin remains uncertain
• Usually starts from the antero superior
quadrant
• Spreads through the posterior superior
quadrant, attic and finally into the mastoid
cavity
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15. Primary acquired cholesteatoma
• Etiology unknown
• Various theories have
been proposed
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16. Theories of primary acquired
cholesteatoma
• Cawthrone theory
• Tumarkin’s theory
• Toss theory of invagination
• Wendt’s metaplastic theory
• Habermann’s epithelial invasion theory
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17. Cawthrone theory
• Suggested by Cawthrone in 1963
• He suggested that cholesteatoma
originated from congenital embryonic cell
rests present in various areas of temporal
bone
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18. Tumarkin’s theory
• Tumarkin believed that cholesteatomas occur
due to migration of squamous epithelium from
the deep portion of the external canal in to the
middle ear cleft through a marginal perforation /
total perforation of ear drum.
• Total perforation is seen in acute necrotizing
otitis media
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19. Toss theory of invagination
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20. Toss theory (contd)
• Toss postulated that persistent negative
pressure in the attic region causes
invagination of pars flaccida thus forming a
retraction pocket.
• This retraction pocket later becomes filled
with desquamated squamous epithelial
debris. These debris form a nidus for
bacterial infections.
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21. Toss’s grades of retraction pockets
• Grade I : The pars flaccida is retracted, but is not
in contact with the neck of the malleus.
• Grade II : The retracted pars flaccida is in
contact with the neck of the malleus clothing it.
• Grade III : Here in addition to grade II features
there is minimal erosion of the outer attic wall
• Grade IV : In this condition, in addition to grade
III features the outer attic wall is drastically
eroded.
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22. Metaplastic theory
• This was first proposed by Wendt in 1873
• The attic area of the middle ear cavity is
lined by pavement epithelium.
• According to Wendt, this pavement
epithelium undergoes squamous
metaplasia in response to infection thus
forming a nidus for cholesteatoma
formation.
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23. Epithelial invasion theory
• This theory was first proposed by
Habermann
• He suggested that attic perforation caused
squamous epithelium to migrate into the
middle ear cavity
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24. Secondary acquired cholesteatoma
• Commonly caused in patients with acute
necrotizing otitis media
• There is associated destruction of the
annulus of the ear drum
• The annulus prevents epithelial migration
from the external canal to the middle ear
cavity.
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25. Features of acute necrotizing otitis
media
• Caused by exanthematous fever like
measles.
• Should be suspected in cases with early
spontaneous perforation, aural secretions
devoid of mucin, secretions with foul odor,
and presence of extensive hearing loss.
• Pneumococci type III have been
implicated
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26. Anatomic considerations
• Cholesteatomas in
attic start from the
Prussacks space
• This space lie
between pars flaccida
and handle of the
malleus
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27. Cholesteatoma spread
• Is determined by:
Ligaments
Mucosal folds
Ossicles
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28. Common sites of cholesteatoma
• Posterior
epitympanum
• Posterior
mesotympanum
• Anterior epitympanum
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29. Cholesteatoma spread
Posterior epitympanic
cholesteatoma
passes through
superior incudal
space to involve
aditus and antrum
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30. Cholesteatoma spread
Posterior mesotympanic cholesteatoma invades
the sinus tympani and facial recess
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31. Cholesteatoma
Anterior epitympanic
cholesteatoma can
involve the geniculate
ganglion of facial
nerve
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32. Evaluation
• History:
Detailed otologic history including
Hearing loss
• Otorrhea
• Otalgia
• Nasal obstruction
• Tinnitus
• Vertigo
• Previous history of middle ear disease:
CSOM
TM perforation
Previous surgery
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33. Evaluation
• Head and neck examination
• Otologic examination
Otomicroscopy is essential in evaluating the extent of
disease
– Clean ear thoroughly of Otorrhea and debris with cotton-tipped
applicators or suction
– Culture wet, infected ears and treat with topical and/or oral antibiotics
– Pneumatic otoscopy should be performed in every patient with
cholesteatoma
Positive fistula test (pneumatic otoscopy will result in nystagmus and vertigo)
response suggests erosion of the semicircular canals or cochlea
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34. Evaluation
• Hearing evaluation to access conductive hearing loss
Pure tone audiometry
Speech reception thresholds
Word recognition
• 512 Hz tuning fork examination
• Tympanometry
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35. Conductive hearing loss
• The degree of conductive hearing loss
may vary greatly:
Moderate hearing loss of more than 40 dB may
indicate ossicular discontinuity due to erosion of long
process of incus / head of stapes
Mild conductive deafness may be present with
extensive disease if cholesteatoma sac transmits sound
directly to stapes or footplate
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36. Role of imaging
CT scan of temporal bones 2mm sections
in both axial and coronal planes will help:
In evaluating the anatomy
Revealing the extent of the disease
Asymptomatic complications may become revealed.
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37. Indications for CT
• Revision cases where the landmarks may
be distorted
• In patients with suspected congenital
anamolies
• Cholesteatoma with s/n loss and
accompanying vestibular symptoms
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38. Management
Cholesteatoma is a surgical problem
Goals of surgery include:
To make the ear safe by eradicating the cholesteatoma and infection
To conserve residual hearing
Improvement of hearing when possible
To provide acceptable cosmetic appearance
To reconstruct the ear in a manner that reduces the chances of recurrence
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39. Surgeries
• Grommet insertion (to manage early
retraction pockets)
• Intact canal wall mastoidectomy
• Canal wall down mastoidectomy
• Transcanal anterior atticotomy
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40. Canal wall down procedure
• Most commonly used
• Good access
• Middle ear space reduced
• Large cavity (cavity problems)
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41. Canal wall down procedure
• Posterior canal wall is reduced up to the
level of vertical portion of facial nerve
• Middle ear, attic, antrum,and mastoid are
exteriorized and made into a single cavity
• Attic is obliterated
• Large meatoplasty is performed
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42. Indications of canal wall down
• Cholesteatoma in the only hearing ear
• Erosion of posterior canal wall
• H/o vertigo suggesting labyrinthine fistula
• Recurrent cholesteatoma after canal wall
up surgery
• Poor Eustachean tube function
• Sclerosed mastoid with poor access to
attic region
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43. Canal wall down surgery
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47. Canal wall up surgery
• More conservative procedure
• Posterior canal wall is retained
• Middle ear space is maintained
• Normal physiology is maintained
• Usually is performed as a staged
procedure
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48. Indications of canal wall up
procedure
• In patients with well pneumatized mastoid
• Patients with good Eustachean tube
function
• In patient’s with limited disease (confined
to the attic)
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49. Contraindications of CWU
• Patients with poor Eustachean tube
function
• Patient with labyrinthine involvement
• Long standing ear disease
• Patients with intra cranial complications
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51. Advantages of CWU
• Rapid healing time
• No cavity problems
• Hearing aids can be used without
problems
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52. Transcanal anterior atticotomy
Procedure involves:
– Elevation of tympanomeatal flap via endaural incision with
removal of scutum to limits of the cholesteatoma
– Aditus obliteration with muscle, fascia, cartilage or bone prior to
reconstruction of the middle ear space
– Reconstruction of lateral attic wall with bone or cartilage is
optional
• May lead to retraction disease and possible recurrence in patients
with poor Eustachian tube function
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53. Complications of cholesteatoma
• Infection
• Otorrhea
• Bone destruction
• Hearing loss
• Facial nerve paresis or paralysis
• Labyrinthine fistula
• Intracranial complications
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54. Conclusions
• Pathogenesis of cholesteatoma remains uncertain
• Essential to possess basic knowledge of the important anatomic and
functional characteristics of the middle ear for successful
management of cholesteatomas
• Careful and thorough evaluations are the key to early diagnosis and
treatment
• Treatment is surgical with primary goal to eradicate disease and
provide a safe and dry ear
• Surgical approaches must be customized to each patient depending
on extent of disease
• Surgeon must be aware of serious and potentially life-threatening
complications of cholesteatomas
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