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    CHOLESTEATOMA

    Dept of Otorhinolaryngology Head and Neck Surgery

     Padjadjaran University

    Hasan Sadikin General Hospital

    20! 1

    "e#ryanti P Sari

    Pe$#i$#ing Uta$a%dr& Sally 'ahdiani( ')es( Sp&*H*+),

    Literature Reading

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      Overvie-

    Definition

    .nato$y

    /lassification and *heories

    'anage$ent

    /o$plications

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    Definition

    Na$ed #y ohannes 'ueller in 11

      & 3rroneous #elief that one of the pri$ary co$ponents

      of the tu$or -as fat

      2&4a pearly tu$or of fat5a$ong sheets of polyhedral

    cells6

    'ore appropriate na$e has #een suggested to #ekerato$a to descri#e tu$or co$position

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      Definition%

      /holesteato$as are e7panding lesions of the

    te$poral #one that are co$posed of a stratified

    s8ua$ous epithelial outer lining and a des8ua$ated

    keratin center&

    3pide$iology %

    + incidence of ac8uired cholesteato$a ranges fro$

    appro7i$ately 9 to 2&! cases per 00(000 adults+ to : cases per 00(000 children . $ale

    predo$inance of &; % in cholesteato$a incidence  .

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    including

    & /ystic content% des8ua$ated keratin center 

    2& 'atri7% keratini

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    Middle Ear Anatomy

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    Tympanic Membrane

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    TULANG-TULANG

    PENDENGARAN

    Ossicles

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    Epitympanum

    MesotympanumHipotympanum

    *y$panic /avity

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    EPITYMPANUM

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    /lassification and *heories

    =t can #e classified as one of t-o different types%

    /ongenital

    .c8uired

      >Pri$ary

      >Secondary

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    /ongenital /holesteato$a

    Definition ?,evenson( 919@& *hese criteria included

    & >Ahite $ass $edial to nor$al ty$panic $e$#rane

    2& >Nor$al pars flaccida and pars tensa

    & >No prior history of otorrhea or perforations

    ;& >No prior otologic procedures

    :& >Prior #outs of otitis $edia -ere not grounds for

    $edia e7clusion as -as the case in original definition

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      *-o pro$inent theories include

    & the failure of the involution of ectoder$al epithelial

    thickening that is present during fetal develop$ent

    in pro7i$ity to the geniculate ganglion &

    2& $etaplasia of the $iddle ear $ucosa&

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      .c8uired /holesteato$as

      /o$$on factor%

    keratini

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    Pri$ary .c8uired /holesteato$as

      Ulti$ately for$ due to underlying 3ustachian tu#e

    dysfunction that causes retraction of pars flaccida

      Besults in poor aeration of epity$panic space

    -hich dra-s pars flaccida $edially on top of

    $alleus neck( for$ing retraction pocket & 

      Nor$al $igratory pattern of the ty$panic

    $e$#rane epitheliu$ altered #y retraction pocket&   3nhances potential accu$ulation of keratin&

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      Pri$ary .c8uired /holesteato$as

      Pars flaccida retraction Pars tensa retraction

     

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    Secondary .c8uired /holesteato$as

    =$plantation theory

      S8ua$ous epitheliu$ i$planted in the $iddle ear as a result of surgery(

    foreign #ody( #last injury( etc&

    'etaplasia theory

      Des8ua$ated epitheliu$ is transfor$ed to keratini

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    3pithelial invasion theory

      S8ua$ous epitheliu$ $igrates along perforation edge

    $edially along undersurface of ty$panic $e$#rane destroying

    the colu$nar epitheliu$&

    Papillary ingro-th theory

      =nfla$$atory reaction in PrussackCs space -ith an intact pars

    flaccida

      ?likely secondary to poor ventilation@ $ay cause #reak in

    #asal $e$#rane

      allo-ing cord of epithelial cells to start in-ard proliferation

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    /holesteato$a Spread

      Predicta#le in that they are channeled along

    characteristic path-ays #y%

      >,iga$ents

      >"olds

      >Ossicles

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      /o$$on Sites of /holesteato$a Origin

    Posterior epity$panu$

    Posterior $esoty$panu$

    .nterior epity$panu$

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    /holesteato$a Spread

    Posterior epity$panic cholesteato$a passing through

    superior incudal space and aditus antru$

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    Posterior $esoty$panic cholesteato$a invading the sinus

    ty$pani and facial recess

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    .nterior epity$panic cholesteato$a -ith e7tension to -ith

    geniculate ganglion

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    Patient 3valuation

    Detailed otologic history

    & Hearing loss

    2& Otorrhea% $alodorous

    & Otalgia

    ;& *innitus

    :& ertigo

      Progressive unilateral hearing loss-ith a chronic foul s$elling

    otorrhea should raise suspicion&

    Previous history of $iddle ear disease

    & /hronic otitis $edia

    2& *y$panic $e$#rane perforation% Pars flaccida

    & Prior surgery

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    Otologic e7a$ination

    Oto$icroscopy is essential in evaluating the e7tent of disease

    /lean ear  thoroughly of otorrhea and de#ris -ith cotton and

    cotton+tipped applicators or suction

    /ulture -et( infected ears and treat -ith topical andEor oral

    anti#iotics

    Pneu$atic otoscopy should #e perfor$ed in every patient -ith

    cholesteato$a

    Positive fistula ?pneu$atic otoscopy -ill result in nystag$us

    and vertigo@ response suggests erosion of the se$icircular

    canals or cochlea

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    Hearing evaluation

     conductive hearing loss

    &  Pure tone audio$etry -ith air and #one conduction

    2&  Speech reception thresholds

    &   Aord recognition

    :2H< tuning fork e7a$

      >.l-ays correlate -ith audio$etry results

    *y$pano$etry

      >'ay suggest decreased co$pliance or *' perforation

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    *he degree of conductive loss -ill vary considera#ly

    depending on the e7tent of disease&

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    Preoperative i$aging -ith co$puted to$ographies

    ?/*s @ of te$poral #ones ?$$ @ section -ithout

    contrast in a7ial and coronal planes&

    & .llo-s for evaluation of anato$y

    2& 'ay reveal evidence of the e7tent

    & Screen for asy$pto$atic co$plications

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      /holesteato$a 'anage$ent

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    Preventative 'anage$ent

    *y$panosto$y tu#e for early retraction pockets

    Surgical e7ploration for retraction persistence

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    *reated surgically -ith rimar! goal of total

    eradication of cholesteato$a to o#tain a safe to and

    dry ear 

    & /anal+-all +do-n procedures ?/AD@

    2& /anal+-all +up procedure ?/AU@

    & *ranscanal anterior atticoto$y

    ;& Fondy $odified radical procedure

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    Prior to the advent of the ty$panoplasty(

    all cholesteato$a surgery -as perfor$ed using

    /AD surgery approach procedure involves%

      >*aking do-n posterior canal -all to level ofvertical facial nerve

      >37teriori

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    /lassic /AD operation is the $odified radical $astoidecto$y

    in -hich $iddle ear space is preserved

    Badical $astoidecto$y is /AD operation in -hich%

      > 'iddle ear space is eli$inated

    > 3ustachian tu#e is plugged

    'eatoplasty should #e large enough to allo- good aeration of

    $astoid cavity and per$it easy visuali

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    =ndications for /AD approach%

    /holesteato$a in an only hearing ear  Significant erosion of the posterior #ony canal -all

    History of vertigo suggesting a la#yrinthine fistula

    Becurrent cholesteato$a after canal+-all +up

    surgery

    Poor eustachian tu#e function

    Sclerotic $astoid -ith li$ited access to

    epity$panu$

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     "#$antages%

      >Besidual disease is easily detected

      >Becurrent disease is rare

      >"acial recess is e7terioriOpen cavity created

      *akes longer to heal

      >'astoid #o-l $aintenance can #e a lifelong pro#le$

      >Shallo- $iddle ear space $akes O/B ?Ossicular /hain

    Beconstruction@ difficult

      >Dry ear precautions are essential

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      /anal+Aall +Do-n

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    /anal +Aall +Up

    /AU procedure developed to avoid pro#le$s and $aintenance

    necessary -ith /AD procedures

    /AU consists of preservation of posterior #ony e7ternal

    auditory canal -all during si$ple $astoidecto$y -ith or

    -ithout a posterior -ith ty$panoto$y

    Staged procedure often necessary -ith a scheduled second

    look operation at ! to 1 $onths for%

      >Be$oval of residual cholesteato$a

      >Ossicular chain reconstruction if necessary

    Procedure should #e adapted to e7tent of disease as -ell as

    skill of otologist

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    /AU $ay #e indicated in patients -ith large pneu$atiSuggests good eustachian tu#e function

    /AU procedures are contraindicated in%

      >Only hearing ear 

      >Patients -ith la#yrinthine fistula

      >,ong+standing ear disease

      >Poor eustachian tu#e function

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     /anal+Aall +Up

    .dvantages%

      >Bapid healing ti$e

      >3asier long+ter$ care

      >Hearing aids easier to fit

      >No -ater precautions

    Disadvantages%

      >*echnically $ore difficult

      >Staged operation often necessary

      >Becurrent disease possi#le  >Besidual disease harder to detect

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      /anal+Aall +Up

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    Novel *echni8ues

      =n 200: Gant

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    Novel *echni8ues

    /anal Aall Beconstruction techni8ue

    >/o$plete cortical $astoidecto$y -ith opening of -ith facial

    recess and re$oval of incus and $alleus head

    >Posterior canal -all skin elevated( annulus elevated

    >'icrosagittal sa- used to cut posterior canal -all

    >/holesteato$a re$oved

    >Posterior canal -all #one replaced

    >/ortical #one chips used to #lock attic and $astoid fro$

    ty$panu$

    >Fone pateC holds #one chips in place

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    /o$plications

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    *he e7pansion of cholesteato$as

    =nfection(otorrhea(#one destruction

    & e7tracranial co$plications

    Hearing loss

    "acial nerve paresis or paralysis

    ,a#yrinthine fistula% se$icircularis canal erosion

    e7tradural or perisinus a#scess

    serous or suppurative la#yrinthitis

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    2& =ntracranial co$plications

      potentially life+threatening

    Periosteal a#scess

    ,ateral sinus thro$#osis% sig$oid sinus

    *hro$#osisEphle#itis

    'eningitis

    3pidural (su#dural( or parenchy$al #rain a#scess

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    Hearing ,oss

    /onductive hearing loss% ossicular chain erosion ?0@

      >& 3rosion of lenticular process andEor stapes superstructure

    process $ay produce :0dF conductive hearing loss

      >2& Hearing loss varies despite disease e7tent ?natural

    $yringostapediope7y( trans$ission of sound through (

    cholesteato$a sac@

    Sensorineural hearing loss% involve$ent of la#yrinth

    "ollo-ing surgery( 0 have further i$pair$ent due to%

      >37tent of disease present

      >/o$plications in healing process

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    ,a#yrinthine "istula

    (nci#ence% as high as 0

    )!mtom% Sensorineural hearing loss andEor

    vertigo induced #y noise or pressure change

      .#sence of a positive fistula test does not rule out this

    co$plication&

    *ommon site% hori

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    "acial Paralysis

    +a! #e$elo%

      >.cutely secondary to infection

      >Slo-ly fro$ chronic e7pansion of cholesteato$a

    ,emoral 'one *, % locali

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     =ntracranial /o$plications

    Potentially life+threatening

    =ncidence% as high as

    *omlications

    & Periosteal a#scess

    2& ,ateral sinus thro$#osis

    & =ntracranial a#scess

    ;& 'eningitis

    )!mtom%

    & Suppurative $alodorous otorrhea2& /hronic headache

    & "ever 

    ;& Otalgia

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    'anage$ent%

      >Presence of $ental status changes -ith nuchal rigi#it!  or

    cranial neuropathies -arrant consultation -ith urgent

    intervention

      >3pidural a#scess( su#dural e$pye$a( $eningitis and (

    cere#ral a#scesses should #e treated i$$ediately prior to 

    definitive otologic $anage$ent of ear disease&

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    *onclusions

    Pathogenesis of cholesteato$a re$ains uncertain

    3ssential to possess #asic kno-ledge of the i$portant

    anato$ic and functional characteristics of the $iddle ear for

    successful $anage$ent of cholesteato$as

    /areful and thorough evaluations are the key to early diagnosisand treat$ent

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    *reat$ent is surgical -ith pri$ary goal to eradicate disease

    and provide a safe and dry ear  Surgical approaches $ust #e custo$i

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    Thanks