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cholesteatoma
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24/05/60
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cholesteatoma
• Aural cholesteatomas
are epidermal inclusion
cysts of the middle ear or
mastoid
• They contain the
desquamated debris
(principally keratin) from
their keratinizing,
squamous epithelial
lining.
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Misnomer ?
•
"cholesteatoma" coined
by the German
physiologist Johannes
Müller in 1838, is a
misnomer
• because this entity does
not contain cholesterol
• the white-yellow keratin
flakes found within
cholesteatomas grossly
resemble cholesterol
crystals
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Congenital cholesteatomas
PATHOGENESIS
• originate : keratinizing
epithelium [epidermoid
cysts ] within the middle
ear cleft,
– "skin in the wrong place “
– pearl-like masses behind
an intact membrane
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Congenital cholesteatomas
• Most involve the
anterior superior
quadrant of the
middle ear
• DDX :
myringosclerosis by
pneumatic otoscopy
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Congenital cholesteatomas
• Potsic et al. staging system:
– stage I : one quadrant
– stage II : multiple quadrants
without ossicular involvement
– stage III, ossicular
involvement without mastoid
extension
– stage IV, mastoid involvement
– correlation between stage and
risk of residual disease; stage
IV carries a 67% risk of
residual cholesteatoma
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MANAGEMENT Congenital
cholesteatomas
• prompt surgical excision
• high-resolution CT scanning of
the temporal bone :
– extension into the attic and
mastoid
– Children with extensive
disease : second-stage 6 to 12
months after the initial surgery
• look for regrowth of
microscopic residual disease
• reconstruct the ossicular
chain.
– long-term follow-up : recurrent
disease.
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acquired cholesteatoma
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The pathogenesis of acquired
cholesteatoma
There are four basic theories :
(1) invagination of the tympanic membrane
(retraction pocket cholesteatoma)
(2) basal cell hyperplasia
(3) epithelial ingrowth through a perforation
(the migration theory)
(4)squamous metaplasia of middle ear epithelium
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Invagination of the tympanic
membrane
• The invagination : attic
cholesteatomas
• eustachian tube
dysfunction (or OME) with
resultant negative middle
ear pressure
• repeated inflammation
• The pars flaccida, being
less fibrous and less
resistant to displacement
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invagination of the tympanic
membrane
• retraction pocket
deepens
• Bacteria can infect
the keratin matrix
biofilm
• primary acquired
cholesteatoma
results
• in the posterosuperior
quadrant
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2. The epithelial invasion Theory
• perforation in the tympanic
membrane
• keratinizing squamous
epithelium migrates into the
middle ear from
• "contact inhibition."
• concept of increased migration
of the epithelium in those with
cholesteatoma
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2. The epithelial invasion Theory
• Cholesteatomas originating after temporal bone
fractures
• allow ingrowth of keratinizing epithelium by
contact guidance
• Dimethylbenzanthracene (DMBA)
– chemical carcinogen
– advancing of the keratinizing squamous epithelium
into or under the mucosal layer middle ear cavity and
eustachian tube
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3.Basal Cell Hyperplasia Theory
• epithelial cells (prickle
cells) of the pars
flaccida
• invade
– subepithelial tissue
– lamina propria
– basement membrane
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3.Basal Cell Hyperplasia Theory
• formation of
microcholesteatomas
• perforate secondarily
through the tympanic
membrane
• an attic
cholesteatoma.
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3.Basal Cell Hyperplasia Theory
• Substance in cholesteatoma matrix
– cytokeratin (CK) 10 ,13 16
– fibronectin and tenascin
– Action
• Hyperproliferation
• Migration
• altered differentiation of keratinocytes
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4. Squamous Metaplasia Theory
• inflammation
• simple squamous or
cuboidal epithelium of the
middle ear cleft
• metaplastic
transformation into
keratinizing epithelium.
• and contact with the
tympanic membrane.
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4. Squamous Metaplasia Theory
• infection and
inflammation,
• lysis of the tympanic
membrane and
perforation
• typical appearance of
an attic
cholesteatoma.
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Diagnosis acquired
cholesteatoma
• otoscopic
examination
– typical attic retraction :
posterosuperior
portion of tympanic
membrane
• surgical exploration
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Diagnosis acquired
cholesteatoma
• High-resolution CT
scanning temporal
bone
– is useful for operative
planning
– recommended for all
revision mastoid
operations
• MRI temporal bone
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symptoms of acquire
cholesteatoma
• Asymptomatic
• slowly progressive
conductive hearing
loss
• chronic otitis with
purulent otorrhea :
most
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acquired cholesteatoma
• malodorous otorrhea :
anaerobic bacteria
• misdiagnosed :
external otitis careful
follow-up canal
débridement of a
patient with otorrhea
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Middle ear atelectasis
• longstanding eustachian tube
dysfunction
• Negative pressure in middle
ear
• atelectasis develops
• tympanic membrane becomes
retracted onto the promontory
and the ossicles of the middle
ear
• tympanic membrane is not
adherent to the medial wall of
the middle ear
• mucosal lining of the middle
ear is intact.
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DDX acquired cholesteatoma
• adhesive otitis media
– middle ear space is totally
obliterated
– tympanic membrane is
adherent to the ossicles
and promontory
– mucosal surfaces are not
present
– erosion of the long process
of the incus and the stapes
suprastructure
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four stages of tympanic membrane
retraction
stage I, retracted
tympanic membrane
stage II, retraction with
contact onto the incus
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four stages of tympanic membrane
retraction
stage III, middle ear
atelectasis
stage IV, adhesive otitis
media
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Management middle ear
atelectasis
• ventilating tubes
• Myringoplasty
– hearing loss
– ossicular erosion
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secondary acquired
cholesteatoma
• keratinizing
epithelium has
migrated through a
perforation into the
middle ear
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secondary acquired
cholesteatoma
"aural polyp."
• An infected cholesteatoma
• granulation tissue at the
junction between an eroding
cholesteatoma and bone.
• The presence of an aural polyp
in a chronically infected ear
should be considered to be a
cholesteatoma until proven
otherwise.
• cholesteatoma cannot be
seen otoscopically but will be
discovered during
tympanomastoid surgery.
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secondary acquired
cholesteatoma
• Cholesteatomas are prone to
– recurrent infection
– erode the bone of the ossicles and the otic capsule.
– exhibit typical growth patterns into the temporal bone.
• The pars flaccida may invaginate into
– Lateral most portion of the epitympanum (Prussak's
space)
– recesses of the epitympanum posteriorly, lateral to the
body of the incus, inferiorly into the middle ear by way
of the pouch of von Tröltsch
– anteriorly into the protympanum
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Prussak
space
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• Prussak’s space connected to Posterior
Pouch von Troeltsch which opens up into
Mesotympanum
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The Prussak’s space
connects
• anterior pouch von
Troeltsch and the
mesotympanum
(Anterior Tympanic
Space)
• Posterior Pouch Von
Troeltsch connections
to the Posterior
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Posterior mesotympanic
cholesteatoma
• retraction of the
posterior portion of
pars tensa
• invades
– sinus tympani
– facial recess
– Extension to mastoid
occurs medial to
ossicle heads
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Anterior epitympanic
cholesteatoma
• Invagination of
epitympanum anterior to
malleus head and neck
creates a cholesteatoma
sac
• threatens horizontal facial
nerve and geniculate
ganglion.
• Forward extension into
supratubal recess is
common
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BACTERIOLOGY OF INFECTED
CHOLESTEATOMAS
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Complications
• bone erosion
– Ossicles : most incus
– otic capsule : most
lateral semicircular
canal
– facial nerve canal
– tegmen tympani
– tegmen mastoideum
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Complications
– Sensorineural hearing loss :
• secondary suppurative labyrinthitis
• cochlear hair cell loss adjacent to cholesteatoma
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Extratemporal (extracranial)
complications
Subperiosteal abscess
• Cholesteatoma can block the
aditus ad antrum
• preventing communication of
the infected contents of the
mastoid with the middle ear
space and the eustachian tube
• This obstruction increases the
possibility of infectious
decompression through the
mastoid cortex.
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Subperiosteal abscess
• systemic symptoms
– fever and malaise
• local signs
– protruding auricle that is
laterally and inferiorly
displaced
– fluctuant, erythematous,
tender area behind the ear.
• CT scan can demonstrate
abscess
– cortical defect in the
mastoid
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Subperiosteal abscess
• abscess is drained
• mastoidectomy is
performed and the
cholesteatoma
matrix is removed in
the standard manner.
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Bezold's abscess
• cervical abscess deep to
the sternocleidomastoid
• mastoid cortex is violated
at its tip, as opposed to
its lateral cortex
• Partway :
– direct extension
– mastoid vein phlebitis
• more common AOM with
mastoiditis
• complication of COM with
cholesteatoma
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Bezold's abscess
• A contrasted CT scan of
the neck and mastoid :
– rim-enhancing abscess
with surrounding
inflammation
– bony dehiscence in the tip
of the mastoid,
• Management :
– drainage of the cervical
abscess through a
transcervical approach
– combined with a cortical
mastoidectomy
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Labyrinthine fistulae
• most common
complications of chronic
otitis with associated
cholesteatoma
• The risk of significant
sensorineural hearing
loss as a result of surgical
manipulation makes the
open labyrinth
– pressure from the
cholesteatoma
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Labyrinthine fistulae
• horizontal
semicircular canal is
the most common
• Erosion of the bone of
the otic capsule
– activated mediators
from the matrix
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Diagnosis Labyrinthine fistulae
• subjective vertigo : 62% to 64% of patients
• positive fistula test on examination : 32% to
50% of patients
• sensorineural hearing loss is found in most
of these patients (68%),
• this classic picture is not sensitive in the
preoperative identification of a fistula
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Labyrinthine fistulae
• the ability to detect
fistulae accurately on
preoperative CT has
been reported as 57% to
60%
• in current reports CT
scans are no more
sensitive than history and
physical examination in
detecting labyrinthine
fistulae.
• The definitive diagnosis :
intraoperatively
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fistula (arrow) of lateral semicircular canal
from cholesteatoma
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Management Labyrinthine
fistulae
A tympanomastoidectomy
• the most appropriate approach to the
fistula is to perform a canal wall down
mastoidectomy
• remove the bulk of the cholesteatoma,
and leave the fistula covered with the
matrix exteriorizing it into the cavity
• then re-operation 9-12 months after first
operation
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Labyrinthine fistulae
• In small fistula
(<2mm) 2 ,
cholesteatoma matrix
gently removed from
fistula +repaired with
soft tissue graft (eg,
fascia,
Intraoperativepicture
perichondrium), bone of a horizontal canal fistula
pate
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Facial paralysis
• usually occurs with a dehiscent fallopian
canal within its tympanic segment,
• allowing direct contact of inflammatory
mediators
• dehiscent nerve,
• bony erosion. slowly progressive facial
paralysis Diagnosis
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Facial paralysis
• The diagnosis facial paralysis
– COM, or cholesteatoma
– CT scan is not required, it can be useful in
therapeutic planning and patient counseling
– cholesteatoma involves the fallopian canal
ass.
• labyrinth
• tegmen
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Facial paralysis
• Coronal CT scan
demonstrating
involvement of
tympanic facial nerve
(arrow) from
cholesteatoma (arrow
heads) causing lateral
semicircular canal
fistula
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Management
•
mastoidectomy is performed to
remove the cholesteatoma or
granulation tissue that is
contacting the facial nerve.
•
The nerve is approached on both
sides of the involvement
•
diamond burr is used to expose
the epineurium on either side of
the diseased segment.
•
Once the proximal and distal
segments of the fallopian canal
have been opened
•
blunt dissection is used to remove
the disease from the epineurium.
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Congenital cholesteatoma of the
temporal bone ?
• divided into four anatomic groups:
1. middle ear DDX congenital auditory
canal cholesteatomas
2. perigeniculate area
3. petrous apex
4. cerebellopontine angle.
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Perigeniculate and petrous apex
cholesteatomas
• present
– insidious or rapidly
progressive facial nerve
paralysis
– Sensorineural hearing loss
from labyrinthine or internal
auditory canal erosion is
common
– conductive hearing loss
also may result from
cholesteatoma extension
into the middle ear or
blockage of the eustachian
tube.
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Perigeniculate and petrous apex
cholesteatomas
– Facial twitching may occur
in the presence of
congenital
cholesteatomas and with
facial nerve neuromas.
– Vestibular dysfunction may
complete the symptom
complex.
– the cholesteatoma may
erode into the middle or
posterior fossa and expand
markedly before producing
symptoms
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Perigeniculate and petrous apex
cholesteatomas
• left cerebellopontine cistern
congenital epidermoid cyst
• A, Axial computed tomography
(CT). Displacement of the pons
DDX :arachnoid cyst
• B, Axial magnetic resonance
imaging T1-weighted, no
contrast.
– This slightly higher signal on
T1-weighted images is helpful
in separating epidermoid cysts
from arachnoid cysts..
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Perigeniculate and petrous apex
cholesteatomas
• MRI will provide further definition
– congenital cholesteatomas
• almost always slightly higher signal T1-weighted images
• moderately high signal on T2-weighted images
– bone marrow fat
• high signal on T1-weighted images
• fade dramatically on T2-weighted images.
– Typical effusions
• low signal on T1-weighted MRI
• high signal on T2-weighted MRI.
– Giant cholesterol cysts :show capsular enhancement.
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Surgical management of a
congenital cholesteatoma
• complete removal of the cholesteatoma
matrix
• The isolated middle ear cholesteatoma
– removed transtympanically
– Routine middle ear reconstructive techniques
may be used if ossicles are eroded or
removed or if the tympanic membrane is
sacrificed.
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Perigeniculate and petrous apex
cholesteatomas
• congenital cholesteatomas of the
perigeniculate area or petrous apex
– Transmastoid
– middle cranial fossa approach
– the transsphenoidal approach
– combination of these procedures
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Cholesteatoma
vs.
Temporal bone fracture
There are four pathogenic mechanisms for
posttraumatic cholesteatoma formation:
(1) epithelial entrapment in the fracture line
(2) ingrowth of epithelium through the unhealed
fracture line or a rent in the tympanic
membrane
(3) traumatic implantation of tympanic membrane
skin into the middle ear
(4) trapping of epithelium medial to a stenosis of
the EAC.
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posttraumatic cholesteatoma
formation
• The typical location : epitympanum and the
antrum. The fracture line along the posterior
superior canal wall and scutum expands and
then closes
• Traumatic implantation of tympanic membrane
skin will result in cholesteatoma formation
within the mesotympanum.
• Blast injuries can result in the displacement of
keratinizing stratified squamous epithelium into
the mastoid air cells, the mesotympanum, the
epitympanum, and even intracranially
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Management posttraumatic
cholesteatoma formation
• careful follow-up
– Débridement
– stenting when narrowing progresses : ear
mold
– canal is adequately dilated : Oto-Wicks
(Meditronic Xomed, Jacksonville, Fl)
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Management posttraumatic
cholesteatoma formation
• canalplasty
– Complete stenosis
– dilation is not possible
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