Cholesteatoma

1. Cholesteatoma Department of Otorhinolaryngoglogy the 2nd Hospital affliatted to Medical college Zhejiang University Xu Yaping

2. Overview • Definition • Classification and Theories • Management • Complications

3. Definition • Named by Johannes Mueller in 1838 1. Erroneous belief that one of the primarycomponents of the tumor was fat 2.“a pearly tumor of fat…among sheets of polyhedral cells” • More appropriate name has been suggested tobe keratomato describe tumor composition

4. Definition: Cholesteatomas are expanding lesions of the temporal bone that are composed of a stratified squamous epithelialouter lining and a desquamated keratin center.

5. including • Cystic content: desquamated keratin center • Matrix: keratinizing stratified squamousepithelium • Perimatrix: granulation tissue that secretesmultiple proteolyticenzymes capable of bone destruction • May develop anywhere within pneumatizedportions of the temporal bone • Most frequent locationsMiddle ear space Mastoid

6. Classification andTheories It can be classified as one of two different types: • Congenital • Acquired 􀂄Primary 􀂄Secondary

7. Congenital Cholesteatoma • Definition (Levenson, 1989). These criteria included • 􀂄White mass medial to normal tympanic membrane • 􀂄Normal pars flaccidaand pars tensa • 􀂄No prior history of otorrheaor perforations • 􀂄No prior otologicprocedures • 􀂄Prior bouts of otitismedia were not grounds for media exclusion as was the case in original definition

8. Twoprominent theories include 1. the failure of the involution of ectodermal epithelial thickening that is present during fetal development in proximity to the geniculate ganglion . 2. metaplasia of the middle ear mucosa.

9. ossicularerosion cholesteatoma

10. Acquired Cholesteatomas Common factor: keratinizing squamousepithelium has grown beyond its normal limits. Acquired cholesteatomas are subdivided into primary acquired and secondary acquiredcholesteatoma.

11. Primary Acquired Cholesteatomas • Ultimately form due to underlying Eustachian tube dysfunction that causes retraction of pars flaccida • Results in poor aeration of epitympanicspace which drawspars flaccidamedially on top of malleusneck, forming retraction pocket . • Normal migratory pattern of the tympanic membrane epithelium altered by retraction pocket. • Enhances potential accumulation of keratin.

12. Primary Acquired Cholesteatomas Pars flaccidaretraction Pars tensa retraction

13. Secondary Acquired Cholesteatomas • Implantation theory Squamous epithelium implanted in the middle ear as a result of surgery,foreign body, blast injury, etc. • Metaplasiatheory Desquamated epithelium is transformed to keratinized stratified squamousepithelium secondary to chronic or recurrent otitis media. • Epithelial invasion theory Squamous epithelium migrates along perforation edge medially alongundersurface of tympanic membrane destroying the columnar epithelium. • Papillary ingrowththeory Inflammatory reaction in Prussack’s space with an intact pars flaccida (likely secondary to poor ventilation) may cause break in basal membrane allowing cord of epithelial cells to start inward proliferation.

14. Cholesteatoma Spread Predictable in that they are channeled along characteristic pathways by: • 􀂄Ligaments • 􀂄Folds • 􀂄Ossicles

15. Common Sites of CholesteatomaOrigin • Posterior epitympanum • Posterior mesotympanum • Anterior epitympanum

16. Cholesteatoma Spread Posterior epitympaniccholesteatomapassing through superior incudalspace and aditus antrum

17. Posterior mesotympaniccholesteatomainvading the sinus tympani and facial recess

18. Anterior epitympaniccholesteatomawith extension to with geniculateganglion

19. Patient Evaluation • Detailed otologichistory • Hearing loss • Otorrhea: malodorous • Otalgia • Tinnitus • Vertigo Progressive unilateral hearing losswith a chronic foul smelling otorrhea should raise suspicion. • Previous history of middle ear disease • Chronic otitis media • Tympanic membrane perforation: Pars flaccida • Prior surgery

20. Otologicexamination • Otomicroscopyis essential in evaluating the extent of disease • Clean ear thoroughly of otorrheaand debris with cottonand cotton-tipped applicators or suction • Culture wet, infected ears and treat with topical and/or oral antibiotics • Pneumatic otoscopyshould be performed in every patient with cholesteatoma • Positive fistula (pneumatic otoscopywill result in nystagmusand vertigo) response suggests erosion of the semicircular canals orcochlea

21. Hearing evaluation • conductive hearing loss • Pure tone audiometrywith air and bone conduction • Speech reception thresholds • Word recognition • 512Hz tuning fork exam 􀂄Always correlate with audiometryresults • Tympanometry 􀂄May suggest decreased compliance or TM perforation

22. The degree of conductive loss will vary considerably depending on the extent of disease.

23. Preoperative imaging with computed tomographies(CTs ) of temporal bones (2mm ) section without contrast in axial and coronalplanes. • Allows for evaluation of anatomy • May reveal evidence of the extent • Screen for asymptomatic complications

24. CholesteatomaManagement

25. Preventative Management • Tympanostomytube for early retraction pockets • Surgical exploration for retraction persistence

26. Treated surgically with primary goal of total eradication of cholesteatomato obtain a safe to and dry ear • Canal-wall -down procedures (CWD) • Canal-wall -up procedure (CWU) • Transcanalanterior atticotomy • Bondymodified radical procedure

27. Prior to the advent of the tympanoplasty, allcholesteatomasurgery was performed using CWD surgery approach procedure involves: 􀂄Taking down posterior canal wall to level of vertical facial nerve 􀂄Exteriorizing the mastoid into external auditory canal

28. Classic CWD operation is the modified radical mastoidectomyin which middle ear space is preserved • Radical mastoidectomy is CWD operation in which: 􀂄 Middle ear space is eliminated 􀂄Eustachian tube is plugged • Meatoplastyshould be large enough to allow good aeration of mastoid cavity and permit easy visualization to facilitate postoperative care and self cleaning

29. Indications for CWD approach: • Cholesteatomain an only hearing ear • Significant erosion of the posterior bony canal wall • History of vertigo suggesting a labyrinthine fistula • Recurrent cholesteatomaafter canal-wall -up surgery • Poor eustachiantube function • Sclerotic mastoid with limited access to epitympanum

30. Advantages: 􀂄Residual disease is easily detected 􀂄Recurrent disease is rare 􀂄Facial recess is exteriorized • Disadvantages: 􀂄Open cavity created Takes longer to heal 􀂄Mastoid bowl maintenance can be a lifelong problem 􀂄Shallow middle ear space makes OCR (Ossicular Chain Reconstruction) difficult 􀂄Dry ear precautions are essential

31. Canal-Wall -Down

32. Canal -Wall -Up • CWU procedure developed to avoid problems and maintenance necessary with CWD procedures • CWU consists of preservation of posterior bony external auditory canal wall during simple mastoidectomywith or without a posterior with tympanotomy • Staged procedure often necessary with a scheduled second look operation at 6 to 18 months for: 􀂄Removal of residual cholesteatoma 􀂄Ossicularchain reconstruction if necessary • Procedure should be adapted to extent of disease as well as skill of otologist

33. CWU may be indicated in patients with large pneumatizedmastoid and well aerated middle space 􀂄Suggests good eustachiantube function • CWU procedures are contraindicated in: 􀂄Only hearing ear 􀂄Patients with labyrinthine fistula 􀂄Long-standing ear disease 􀂄Poor eustachiantube function

34. Canal-Wall -Up • Advantages: 􀂄Rapid healing time 􀂄Easier long-term care 􀂄Hearing aids easier to fit 􀂄No water precautions • Disadvantages: 􀂄Technically more difficult 􀂄Staged operation often necessary 􀂄Recurrent disease possible 􀂄Residual disease harder to detect

35. Canal-Wall -Up

36. Novel Techniques In 2005 Gantz. al. reported 130 cases of canal wall reconstruction tympanomastoidectomy with mastoid obliteration 􀂄No evidence of recurrence = 98.5% 􀂄Recurrence treated with CWD (1.5%) 􀂄Second look ossiculoplastyin 78% 􀂄Post-operative wound infection was 14.3% for first42 patients Decreased rate to 4.5% in last 88 patients with 2 days of postpost-operative IV antibiotics

37. Novel Techniques • Canal Wall Reconstruction technique 􀂄Complete cortical mastoidectomywith opening of with facial recess and removal of incus and malleushead 􀂄Posterior canal wall skin elevated, annulus elevated 􀂄Microsagittalsaw used to cut posterior canal wall 􀂄Cholesteatomaremoved 􀂄Posterior canal wall bone replaced 􀂄Cortical bone chips used to block attic and mastoid from tympanum 􀂄Bone pate’ holds bone chips in place

38. Complications

39. The expansion of cholesteatomas Infection,otorrhea,bone destruction 1. extracranial complications • Hearing loss • Facial nerve paresis or paralysis • Labyrinthine fistula: semicirculai canal erosion • extradural or perisinus abscess • serous or suppurative labyrinthitis

40. 2. Intracranial complications potentially life-threatening • Periostealabscess • Lateral sinus thrombosis: sigmoid sinus • Thrombosis/phlebitis • Meningitis • Epidural ,subdural, or parenchymal brain abscess

41. Hearing Loss • Conductive hearing loss: ossicularchain erosion (30%) 􀂄1. Erosion oflenticularprocess and/or stapes superstructure process may produce 50dB conductive hearing loss 􀂄2. Hearing loss varies despite disease extent (natural myringostapediopexy, transmission of sound through , cholesteatomasac) • Sensorineuralhearing loss: involvement of labyrinth • Following surgery, 30% have further impairment due to: 􀂄Extent of disease present 􀂄Complications in healing process

42. Labyrinthine Fistula • Incidence: as high as 10% • Symptom: Sensorineuralhearing loss and/or vertigo induced by noise or pressure change Absence of a positive fistula test does not rule out this complication. • Common site: horizontal semicircular canal, basal turn of cochlea • Diagnosis: Fine cut temporal bone CT (1mm) • Management: modified radical mastoidectomywith management of matrix overlying fistula

43. Facial Paralysis • May develop: 􀂄Acutely secondary to infection 􀂄Slowly from chronic expansion of cholesteatoma • Temporal bone CT: localize the nerve involvement • Most common site: geniculateganglion due to disease in the anterior epitympanum • Management: Needs immediate surgery • Removal of cholesteatomaand infected material with decompression of the nerve (mastoidectomy, middle , fossaapproach) • Administration of intravenous antibiotics and high-dose steroids • Iatrogenic injury to the nerve during surgery should be immediately repaired with decompression of nerve proximal and distal to site of injury

44. Intracranial Complications • Potentially life-threatening • Incidence: as high as 1% • Complications • Periostealabscess • Lateral sinus thrombosis • Intracranial abscess • Meningitis • Symptom: • Suppurativemalodorous otorrhea • Chronic headache • Fever • Otalgia

45. Management: 􀂄Presence of mental status changes with nuchalrigidityor cranial neuropathies warrant consultation with urgent intervention 􀂄Epidural abscess, subduralempyema, meningitis and , cerebral abscesses should be treated immediatelyprior to definitive otologicmanagement of ear disease.

46. Conclusions • Pathogenesis of cholesteatomaremains uncertain • Essential to possess basic knowledge of the important anatomic and functional characteristics of the middle ear for successful management of cholesteatomas • Careful and thorough evaluations are the key to early diagnosis and treatment • Treatment is surgical with primary goal to eradicate disease andprovide a safe and dry ear • Surgical approaches must be customized to each patient depending on extent of disease • Surgeon must be aware of serious and potentially life-threateningcomplications of cholesteatomas

47. Thanks