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CholesteatomaCholesteatoma--Pathogenesis andPathogenesis andSurgical ManagementSurgical Management
Grand Rounds PresentationGrand Rounds Presentation
February 24, 1999February 24, 1999
Kyle Kennedy, M.D.Kyle Kennedy, M.D.
Jeffrey Vrabec, M.D.Jeffrey Vrabec, M.D.
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IntroductionIntroduction
Cholesteatoma (keratoma)Cholesteatoma (keratoma)--essentiallyessentially
an accumulation of skin in ME/mastoidan accumulation of skin in ME/mastoid
insidious natureinsidious nature
variable symptoms depending on extentvariable symptoms depending on extent
and location of diseaseand location of disease
primarily a surgical diseaseprimarily a surgical disease
high rate of recidivistic diseasehigh rate of recidivistic disease
longlong--term followterm follow--up essentialup essential
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IntroductionIntroduction
Pathology and classificationPathology and classification
Eustachian tube dysfunctionEustachian tube dysfunction
PathogenesisPathogenesis
Anatomic considerationsAnatomic considerations
EvaluationEvaluation
Surgical managementSurgical management
Results of therapyResults of therapy
ComplicationsComplications
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Pathology and ClassificationPathology and Classification
NonNon--neoplastic accumulation ofneoplastic accumulation of
keratinizing stratified squamouskeratinizing stratified squamous
epithelium with desquamated keratinepithelium with desquamated keratindebrisdebris
Subepithelial fibroconnective tissueSubepithelial fibroconnective tissue
Granulation tissueGranulation tissue Bone destruction possibleBone destruction possible
Elaboration of collagenase and otherElaboration of collagenase and other
inflammatory mediatorsinflammatory mediators
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Pathology and ClassificationPathology and Classification
Congenital cholesteatomaCongenital cholesteatoma
Acquired cholesteatomaAcquired cholesteatoma
Canal cholesteatomaCanal cholesteatoma
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Congenital CholesteatomaCongenital Cholesteatoma
Cholesteatoma sac medial to an intactCholesteatoma sac medial to an intact
tympanic membranetympanic membrane
Normal pars flaccida and tensaNormal pars flaccida and tensa
No h/o TM perforation or otorrheaNo h/o TM perforation or otorrhea
No h/o otologic trauma or surgeryNo h/o otologic trauma or surgery
H/o prior episodes of OM does notH/o prior episodes of OM does not
preclude its presencepreclude its presence
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Acquired CholesteatomaAcquired Cholesteatoma
Usually found in posterosuperiorUsually found in posterosuperior
quadrant of TM with asso. retractionquadrant of TM with asso. retraction
pocket or perforationpocket or perforation Primary acquired cholesteatoma asso.Primary acquired cholesteatoma asso.
with prewith pre--existing retraction pocketexisting retraction pocket
Secondary acquired cholesteatomaSecondary acquired cholesteatomaarises in setting of persistent TMarises in setting of persistent TM
perforationperforation
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Canal CholesteatomaCanal Cholesteatoma
Found lateral to TMFound lateral to TM
Idiopathic, postIdiopathic, post--traumatic, andtraumatic, and
iatrogenic variantsiatrogenic variants
Must be distinguished from keratosisMust be distinguished from keratosis
obturansobturans
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Eustachian Tube DysfunctionEustachian Tube Dysfunction
Important in pathogenesis of middle earImportant in pathogenesis of middle ear
disease and cholesteatomadisease and cholesteatoma
Essential role in recurrent disease andEssential role in recurrent disease andsurgical failuresurgical failure
Preoperative clinical assessment ofPreoperative clinical assessment of
tubal patency mandatorytubal patency mandatory Tubal function and ME aerationTubal function and ME aeration
particularly important in postoperativeparticularly important in postoperative
hearing results
hearing results
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PathogenesisPathogenesis
Migratory nature of TM epithelium andMigratory nature of TM epithelium and
cholesteatomacholesteatoma
Iatrogenic implantationIatrogenic implantation
Invasion of squamous epitheliumInvasion of squamous epithelium
Invagination theoryInvagination theory
Basal cell proliferationBasal cell proliferation
MetaplasiaMetaplasia
Embryonic squamous epithelial cellEmbryonic squamous epithelial cell
restsrests
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Anatomic ConsiderationsAnatomic Considerations
Tympanic cavity derived fromTympanic cavity derived from
endodermallyendodermally--lined first branchial pouchlined first branchial pouch
Characteristic pathways of diseaseCharacteristic pathways of diseasespreadspread
Attic or epitympanumAttic or epitympanum--Prussacks spacePrussacks space
Posterior mesotympanumPosterior mesotympanum--facial recessfacial recessand sinus tympaniand sinus tympani
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EvaluationEvaluation
HistoryHistory--longh/o ear complaintslongh/o ear complaints
Physical examinationPhysical examination--otomicroscopyotomicroscopy
AudiologyAudiology--CHLCHL
ImagingImaging--assessment of mastoidassessment of mastoid
disease, surgical road map, revisiondisease, surgical road map, revision
cases, sensorineuralhearing loss,cases, sensorineuralhearing loss,vestibular symptomsvestibular symptoms
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ManagementManagement
Surgical diseaseSurgical disease
Patient age (I.e. pediatricPatient age (I.e. pediatric
cholesteatoma generally consideredcholesteatoma generally consideredmore aggressive)more aggressive)
Primary goal is eradication of diseasePrimary goal is eradication of disease
withhearing preservation orwithhearing preservation orimprovement secondaryimprovement secondary
Final therapeutic decisions often madeFinal therapeutic decisions often made
at surgeryat surgery
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Surgical ManagementSurgical Management
No consensus regarding optimalNo consensus regarding optimal
surgical strategysurgical strategy
Principal controversy concerning intactPrincipal controversy concerning intactcanal wall vs. canal wall downcanal wall vs. canal wall down
mastoidectomymastoidectomy
Therapy must be individualized onTherapy must be individualized oncasecase--byby--case basiscase basis
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Preoperative Patient CounselingPreoperative Patient Counseling
Surgical goalsSurgical goals
Risks of surgery including facialRisks of surgery including facial
paralysis, tinnitus, vertigo, worsening ofparalysis, tinnitus, vertigo, worsening ofhearinghearing
Possible need for staged procedurePossible need for staged procedure
Chronic nature of disease process withChronic nature of disease process withneed for longneed for long--term followterm follow--upup
Routine aural toilet if mastoid bowlRoutine aural toilet if mastoid bowl
createdcreated
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Tympanostomy Tube InsertionTympanostomy Tube Insertion
Alleviation of early TM retraction inAlleviation of early TM retraction in
setting of ETDsetting of ETD
Arrest pathologic process prior toArrest pathologic process prior toirreversible changes such asirreversible changes such as
atelectasis, deep retraction pocketatelectasis, deep retraction pocket
formation, TM perforation, orformation, TM perforation, orcholesteatoma formationcholesteatoma formation
Assist in maintenance of ME aerationAssist in maintenance of ME aeration
after tympanoplasty orafter tympanoplasty or
t m anomastoidectomt m anomastoidectom
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TympanomeatalTympanomeatal
Flap/TympanoplastyFlap/Tympanoplasty
Smaller congenital cholesteatomas ofSmaller congenital cholesteatomas of
involving TM or MEinvolving TM or ME
Acquired cholesteatomas limited toAcquired cholesteatomas limited tomesotympanummesotympanum
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Intact Canal Wall MastoidectomyIntact Canal Wall Mastoidectomy
Preservation of posterior canal wallPreservation of posterior canal wall
during simple mastoidectomy with orduring simple mastoidectomy with or
without posterior tympanotomy (facialwithout posterior tympanotomy (facialrecess approach)recess approach)
Cholesteatomas of attic, antrum, post.Cholesteatomas of attic, antrum, post.
mesotympanum with adequate ME andmesotympanum with adequate ME andmastoid aerationmastoid aeration
Staging necessary with ME mucosalStaging necessary with ME mucosal
abnormalities, ossicular erosion,abnormalities, ossicular erosion,
residual diseaseresidual disease
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Canal Wall DownCanal Wall Down
MastoidectomyMastoidectomy
Removal of post. canal wall to level ofRemoval of post. canal wall to level of
vertical facial nervevertical facial nerve
Creation of mastoid cavity withCreation of mastoid cavity withexteriorization of mastoid into EACexteriorization of mastoid into EAC
Scutum removed with obliteration ofScutum removed with obliteration of
epitympanum and removal of malleusepitympanum and removal of malleushead and incushead and incus
MRM ME space maintained whileMRM ME space maintained while
radical mastoid eliminates ME spaceradical mastoid eliminates ME space
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Canal Wall DownCanal Wall Down
MastoidectomyMastoidectomy
Surgery in an onlySurgery in an only--hearing earhearing ear
Poor anesthetic riskPoor anesthetic risk
Poor pt compliance with unreliable F/UPoor pt compliance with unreliable F/U
Poor tubal function and ME aerationPoor tubal function and ME aeration
Sclerotic mastoidSclerotic mastoid
Extensive canal wall defectExtensive canal wall defect
Labyrinthine fistulaLabyrinthine fistula
Meatoplasty and mastoid obliterationMeatoplasty and mastoid obliteration
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AtticotomyAtticotomy
Removal of scutumRemoval of scutum
Limited attic diseaseLimited attic disease
Scutal reconstruction with autologousScutal reconstruction with autologous
cartilagecartilage
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Bondy ProcedureBondy Procedure
Removal of scutum and posterior canalRemoval of scutum and posterior canal
wall with preservation of ossicles andwall with preservation of ossicles and
ME spaceME space Larger attic cholesteatomas lateral toLarger attic cholesteatomas lateral to
ossicles in pt with sclerotic mastoidossicles in pt with sclerotic mastoid
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Intact Canal WallAdvantagesIntact Canal WallAdvantages
More rapidhealingMore rapidhealing
Easier longEasier long--term postoperative careterm postoperative care
No water precautions necessaryNo water precautions necessary
(particularly important in children)(particularly important in children)
More options available forhearing aid, ifMore options available forhearing aid, if
necessarynecessary
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Intact Canal Wall DisadvantagesIntact Canal Wall Disadvantages
Epitympanum/mastoid not accessible toEpitympanum/mastoid not accessible to
postop inspectionpostop inspection
Supratubal space not easily accessibleSupratubal space not easily accessibleunless malleus head and incus removedunless malleus head and incus removed
Both residual and recurrent diseaseBoth residual and recurrent disease
more likelymore likely Greater number of procedures usuallyGreater number of procedures usually
required for disease eradicationrequired for disease eradication
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Canal Wall Down AdvantagesCanal Wall Down Advantages
Easy detection of residual diseaseEasy detection of residual disease
Recurrent cholesteatoma rareRecurrent cholesteatoma rare
Fewer procedures necessary forFewer procedures necessary for
eradication of diseaseeradication of disease
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Canal Wall Down DisadvantagesCanal Wall Down Disadvantages
Longerhealing timeLongerhealing time
Special cavity care often necessary forSpecial cavity care often necessary for
properhealingproperhealing
Periodic cleaning necessaryPeriodic cleaning necessary
Accumulation of debris may occur withAccumulation of debris may occur with
increased risk of infectionincreased risk of infection Water precautions necessaryWater precautions necessary
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Results of TherapyResults of Therapy
Rosenberg et al. examined variablesRosenberg et al. examined variables
with regard to residualwith regard to residual--recurrentrecurrent
disease (retrospective)disease (retrospective) 232 children with cholesteatoma (244232 children with cholesteatoma (244
ears)ears)
Ossicular erosion asso. with residualOssicular erosion asso. with residual--recurrent disease (necessitates 2ndrecurrent disease (necessitates 2nd
look)look)
Recidivism 61% at 6 yearsRecidivism 61% at 6 years--
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Results of TherapyResults of Therapy
Dodson et al. examined cases of 66Dodson et al. examined cases of 66
children with cholesteatoma (73 ears)children with cholesteatoma (73 ears)
retrospectively with ave. F/U37.7 mos.retrospectively with ave. F/U37.7 mos. ICWICW--41% recidivism andCWD41% recidivism andCWD--12%12%
recidivismrecidivism
Postop SRT less than 30 dB in 75% ofPostop SRT less than 30 dB in 75% ofICW and 72% ofCWDICW and 72% ofCWD
Prefer ICW with 2nd stagePrefer ICW with 2nd stage
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Results of TherapyResults of Therapy
Hirsch et al. retro. reviewed 164 casesHirsch et al. retro. reviewed 164 cases
of ped. chol. (116 avail. for 5 year F/U)of ped. chol. (116 avail. for 5 year F/U)
Majority of pts requiredCWD procedureMajority of pts requiredCWD procedure
Recidivism 11% for tympanoplasty, 19%Recidivism 11% for tympanoplasty, 19%
for ICW, 5% for MRM, and 0% forfor ICW, 5% for MRM, and 0% for
radical mastoidradical mastoidAlso reported fewer revisions and betterAlso reported fewer revisions and better
hearing results withCWDhearing results withCWD
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ComplicationsComplications
Conductive hearing lossConductive hearing loss
Labyrinthine fistulaLabyrinthine fistula
Facial nerve paresis or paralysisFacial nerve paresis or paralysis
Intratemporal or intracranialIntratemporal or intracranial
complicationscomplications
EncephaloceleEncephalocele
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ConclusionsConclusions
Exact pathogenesis not entirely clearExact pathogenesis not entirely clear
Important anatomic considerations inImportant anatomic considerations in
managementmanagement
Eradication of disease primary goalEradication of disease primary goal
No universally accepted surgicalNo universally accepted surgical
strategystrategy High rate of recidivism with longHigh rate of recidivism with long--termterm
F/UessentialF/Uessential
Maintain vigilance for complicationsMaintain vigilance for complications