Cholesteatoma-Pathogenesis and Cholesteatoma-Pathogenesis and Surgical ManagementSurgical Management

Grand Rounds PresentationGrand Rounds PresentationFebruary 24, 1999February 24, 1999

Kyle Kennedy, M.D.Kyle Kennedy, M.D.Jeffrey Vrabec, M.D.Jeffrey Vrabec, M.D.

IntroductionIntroduction

Cholesteatoma (keratoma)-essentially an Cholesteatoma (keratoma)-essentially an accumulation of skin in ME/mastoidaccumulation of skin in ME/mastoid

insidious natureinsidious nature variable symptoms depending on extent and variable symptoms depending on extent and

location of diseaselocation of disease primarily a surgical diseaseprimarily a surgical disease high rate of recidivistic diseasehigh rate of recidivistic disease long-term follow-up essentiallong-term follow-up essential

Introduction Introduction

Pathology and classificationPathology and classification Eustachian tube dysfunctionEustachian tube dysfunction PathogenesisPathogenesis Anatomic considerationsAnatomic considerations EvaluationEvaluation Surgical managementSurgical management Results of therapyResults of therapy Complications Complications

Pathology and ClassificationPathology and Classification

Non-neoplastic accumulation of keratinizing Non-neoplastic accumulation of keratinizing stratified squamous epithelium with stratified squamous epithelium with desquamated keratin debrisdesquamated keratin debris

Subepithelial fibroconnective tissueSubepithelial fibroconnective tissue Granulation tissueGranulation tissue Bone destruction possible Bone destruction possible Elaboration of collagenase and other Elaboration of collagenase and other

inflammatory mediators inflammatory mediators

Pathology and ClassificationPathology and Classification

Congenital cholesteatomaCongenital cholesteatoma Acquired cholesteatomaAcquired cholesteatoma Canal cholesteatomaCanal cholesteatoma

Congenital CholesteatomaCongenital Cholesteatoma

Cholesteatoma sac medial to an intact Cholesteatoma 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 not H/o prior episodes of OM does not

preclude its presencepreclude its presence

Acquired CholesteatomaAcquired Cholesteatoma

Usually found in posterosuperior Usually found in posterosuperior quadrant of TM with asso. retraction quadrant of TM with asso. retraction pocket or perforationpocket or perforation

Primary acquired cholesteatoma asso. Primary acquired cholesteatoma asso. with pre-existing retraction pocketwith pre-existing retraction pocket

Secondary acquired cholesteatoma Secondary acquired cholesteatoma arises in setting of persistent TM arises in setting of persistent TM perforationperforation

Canal CholesteatomaCanal Cholesteatoma

Found lateral to TMFound lateral to TM Idiopathic, post-traumatic, and Idiopathic, post-traumatic, and

iatrogenic variantsiatrogenic variants Must be distinguished from keratosis Must be distinguished from keratosis

obturansobturans

Eustachian Tube DysfunctionEustachian Tube Dysfunction

Important in pathogenesis of middle ear Important in pathogenesis of middle ear disease and cholesteatomadisease and cholesteatoma

Essential role in recurrent disease and Essential role in recurrent disease and surgical failuresurgical failure

Preoperative clinical assessment of tubal Preoperative clinical assessment of tubal patency mandatorypatency mandatory

Tubal function and ME aeration particularly Tubal function and ME aeration particularly important in postoperative hearing resultsimportant in postoperative hearing results

PathogenesisPathogenesis

Migratory nature of TM epithelium and Migratory 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 cell restsEmbryonic squamous epithelial cell rests

Anatomic ConsiderationsAnatomic Considerations

Tympanic cavity derived from Tympanic cavity derived from endodermally-lined first branchial pouchendodermally-lined first branchial pouch

Characteristic pathways of disease Characteristic pathways of disease spreadspread

Attic or epitympanum-Prussack’s spaceAttic or epitympanum-Prussack’s space Posterior mesotympanum-facial recess Posterior mesotympanum-facial recess

and sinus tympaniand sinus tympani

EvaluationEvaluation

History-long h/o ear complaints History-long h/o ear complaints Physical examination-otomicroscopyPhysical examination-otomicroscopy Audiology-CHLAudiology-CHL Imaging-assessment of mastoid Imaging-assessment of mastoid

disease, surgical road map, revision disease, surgical road map, revision cases, sensorineural hearing loss, cases, sensorineural hearing loss, vestibular symptomsvestibular symptoms

ManagementManagement

Surgical diseaseSurgical disease Patient age (I.e. pediatric cholesteatoma Patient age (I.e. pediatric cholesteatoma

generally considered more aggressive)generally considered more aggressive) Primary goal is eradication of disease with Primary goal is eradication of disease with

hearing preservation or improvement hearing preservation or improvement secondarysecondary

Final therapeutic decisions often made at Final therapeutic decisions often made at surgerysurgery

Non-surgical ManagementNon-surgical Management

Office management of limited disease in Office management of limited disease in elderly patients with comorbidities elderly patients with comorbidities

Topical antibiotic preparations including Topical antibiotic preparations including those containing steroids sometimes those containing steroids sometimes useful preoperativelyuseful preoperatively

Surgical ManagementSurgical Management

No consensus regarding optimal No consensus regarding optimal surgical strategysurgical strategy

Principal controversy concerning intact Principal controversy concerning intact canal wall vs. canal wall down canal wall vs. canal wall down mastoidectomymastoidectomy

Therapy must be individualized on Therapy must be individualized on case-by-case basiscase-by-case basis

Preoperative Patient CounselingPreoperative Patient Counseling

Surgical goalsSurgical goals Risks of surgery including facial paralysis, Risks of surgery including facial paralysis,

tinnitus, vertigo, worsening of hearingtinnitus, vertigo, worsening of hearing Possible need for staged procedurePossible need for staged procedure Chronic nature of disease process with Chronic nature of disease process with

need for long-term follow-upneed for long-term follow-up Routine aural toilet if mastoid bowl created Routine aural toilet if mastoid bowl created

Tympanostomy Tube InsertionTympanostomy Tube Insertion

Alleviation of early TM retraction in setting Alleviation of early TM retraction in setting of ETDof ETD

Arrest pathologic process prior to Arrest pathologic process prior to irreversible changes such as atelectasis, irreversible changes such as atelectasis, deep retraction pocket formation, TM deep retraction pocket formation, TM perforation, or cholesteatoma formationperforation, or cholesteatoma formation

Assist in maintenance of ME aeration after Assist in maintenance of ME aeration after tympanoplasty or tympanomastoidectomytympanoplasty or tympanomastoidectomy

Tympanomeatal Tympanomeatal Flap/TympanoplastyFlap/Tympanoplasty

Smaller congenital cholesteatomas of Smaller congenital cholesteatomas of involving TM or MEinvolving TM or ME

Acquired cholesteatomas limited to Acquired cholesteatomas limited to mesotympanummesotympanum

Intact Canal Wall MastoidectomyIntact Canal Wall Mastoidectomy

Preservation of posterior canal wall during Preservation of posterior canal wall during simple mastoidectomy with or without posterior simple mastoidectomy with or without posterior tympanotomy (facial recess approach)tympanotomy (facial recess approach)

Cholesteatomas of attic, antrum, post. Cholesteatomas of attic, antrum, post. mesotympanum with adequate ME and mesotympanum with adequate ME and mastoid aerationmastoid aeration

Staging necessary with ME mucosal Staging necessary with ME mucosal abnormalities, ossicular erosion, residual abnormalities, ossicular erosion, residual diseasedisease

Canal Wall Down Canal Wall Down MastoidectomyMastoidectomy

Removal of post. canal wall to level of vertical Removal of post. canal wall to level of vertical facial nervefacial nerve

Creation of mastoid cavity with exteriorization of Creation of mastoid cavity with exteriorization of mastoid into EACmastoid into EAC

Scutum removed with obliteration of epitympanum Scutum removed with obliteration of epitympanum and removal of malleus head and incusand removal of malleus head and incus

MRM ME space maintained while radical mastoid MRM ME space maintained while radical mastoid eliminates ME space and obliterates eustachian eliminates ME space and obliterates eustachian tubetube

Canal Wall Down Canal Wall Down MastoidectomyMastoidectomy

Surgery in an only-hearing earSurgery in an only-hearing 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

AtticotomyAtticotomy

Removal of scutumRemoval of scutum Limited attic diseaseLimited attic disease Scutal reconstruction with autologous Scutal reconstruction with autologous

cartilagecartilage

Bondy ProcedureBondy Procedure

Removal of scutum and posterior canal Removal of scutum and posterior canal wall with preservation of ossicles and wall with preservation of ossicles and ME spaceME space

Larger attic cholesteatomas lateral to Larger attic cholesteatomas lateral to ossicles in pt with sclerotic mastoidossicles in pt with sclerotic mastoid

Intact Canal Wall AdvantagesIntact Canal Wall Advantages

More rapid healingMore rapid healing Easier long-term postoperative careEasier long-term postoperative care No water precautions necessary No water precautions necessary

(particularly important in children)(particularly important in children) More options available for hearing aid, if More options available for hearing aid, if

necessarynecessary

Intact Canal Wall DisadvantagesIntact Canal Wall Disadvantages

Epitympanum/mastoid not accessible to Epitympanum/mastoid not accessible to postop inspectionpostop inspection

Supratubal space not easily accessible Supratubal space not easily accessible unless malleus head and incus removedunless malleus head and incus removed

Both residual and recurrent disease more Both residual and recurrent disease more likelylikely

Greater number of procedures usually Greater number of procedures usually required for disease eradicationrequired for disease eradication

Canal Wall Down AdvantagesCanal Wall Down Advantages

Easy detection of residual diseaseEasy detection of residual disease Recurrent cholesteatoma rareRecurrent cholesteatoma rare Fewer procedures necessary for Fewer procedures necessary for

eradication of diseaseeradication of disease

Canal Wall Down DisadvantagesCanal Wall Down Disadvantages

Longer healing timeLonger healing time Special cavity care often necessary for Special cavity care often necessary for

proper healingproper healing Periodic cleaning necessaryPeriodic cleaning necessary Accumulation of debris may occur with Accumulation of debris may occur with

increased risk of infectionincreased risk of infection Water precautions necessaryWater precautions necessary

Results of TherapyResults of Therapy

Rosenberg et al. examined variables with Rosenberg et al. examined variables with regard to residual-recurrent disease regard to residual-recurrent disease (retrospective)(retrospective)

232 children with cholesteatoma (244 ears)232 children with cholesteatoma (244 ears) Ossicular erosion asso. with residual-Ossicular erosion asso. with residual-

recurrent disease (necessitates 2nd look)recurrent disease (necessitates 2nd look) Recidivism 61% at 6 years (necessitates Recidivism 61% at 6 years (necessitates

long-term F/U)long-term F/U)

Results of TherapyResults of Therapy

Dodson et al. examined cases of 66 Dodson et al. examined cases of 66 children with cholesteatoma (73 ears) children with cholesteatoma (73 ears) retrospectively with ave. F/U 37.7 mos.retrospectively with ave. F/U 37.7 mos.

ICW-41% recidivism and CWD-12% ICW-41% recidivism and CWD-12% recidivismrecidivism

Postop SRT less than 30 dB in 75% of Postop SRT less than 30 dB in 75% of ICW and 72% of CWDICW and 72% of CWD

Prefer ICW with 2nd stage Prefer ICW with 2nd stage

Results of TherapyResults of Therapy

Hirsch et al. retro. reviewed 164 cases of Hirsch et al. retro. reviewed 164 cases of ped. chol. (116 avail. for 5 year F/U)ped. chol. (116 avail. for 5 year F/U)

Majority of pts required CWD procedureMajority of pts required CWD procedure Recidivism 11% for tympanoplasty, 19% Recidivism 11% for tympanoplasty, 19%

for ICW, 5% for MRM, and 0% for radical for ICW, 5% for MRM, and 0% for radical mastoidmastoid

Also reported fewer revisions and better Also reported fewer revisions and better hearing results with CWDhearing results with CWD

ComplicationsComplications

Conductive hearing lossConductive hearing loss Labyrinthine fistulaLabyrinthine fistula Facial nerve paresis or paralysisFacial nerve paresis or paralysis Intratemporal or intracranial Intratemporal or intracranial

complicationscomplications EncephaloceleEncephalocele

ConclusionsConclusions

Exact pathogenesis not entirely clearExact pathogenesis not entirely clear Important anatomic considerations in Important anatomic considerations in

managementmanagement Eradication of disease primary goalEradication of disease primary goal No universally accepted surgical strategyNo universally accepted surgical strategy High rate of recidivism with long-term F/U High rate of recidivism with long-term F/U

essentialessential Maintain vigilance for complicationsMaintain vigilance for complications