Cholesteatoma 2

8/6/2019 Cholesteatoma 2

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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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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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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

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Cholesteatoma 2