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Cholesteatoma CME

Cholesteatoma - Theories and Pathogenesis

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Cholesteatoma CME

  1. 1. Theories & Pathogenesis and Overview of Cholesteatoma Tengku Ezulia CME | ENT HRPZ II 25/8/2015
  2. 2. Definition  (Abramson et al, 1977) Points to mention: [viva]  3 dimensional epidermal and connective tissue structure  Forming a sac composed of a stratified squamous epithelial outer lining and a desquamated keratin center  Conforming the middle ear cleft ( middle ear, attic, mastoid)  Capacity for progressive and independent growth involving the underlying bone and replacing the middle ear mucosa  Tendency to recur
  3. 3. Classification  Congenital  Acquired Primary Secondary
  4. 4. Congenital Cholesteatoma  Definition (Levenson, 1989) – 5 points White mass medial to normal tympanic membrane Normal pars flaccida and pars tensa No prior history of otorrhea or perforations No prior otologic procedures Prior bouts of otitis media not grounds for exclusion
  5. 5. Theories “Congenital cholesteatoma”  Epithelial cell rest theory  Squamous metaplasia theory  Epidermoid formation theory  Invagination theory  ESEI
  6. 6. Teed’s theory – Failure of involution of embryonic cell rest  Proposed in 1936  The embryonic ectodermal epithelial cell rests that is present during fetal development in proximity to the geniculate ganglion fails to involute.  Persistence of embryonic squamous cell rests in the temporal bone led to the formation of congenital cholesteatoma.  Presence of squamous cell rests in the temporal bone – fairly common. Usually they involute at a later date to become mature middle ear lining
  7. 7. Wendt’s Squamous Metaplastic theory  This was first proposed by Wendt in 1873  The attic area of the middle ear cavity is lined by pavement epithelium.  According to Wendt, this pavement epithelium undergoes squamous metaplasia in response to infection thus forming a nidus for cholesteatoma formation.
  8. 8. Michael’s epidermoid formation theory  Michaels in 1980s – fetal human temporal bones  Identified squamous cell tuft present from 10-33 wk of gestation.  This “epidermoid formation” was noted in anterosuperior wall of ME cleft.  Failure of involution could be basis of cholesteatoma in anterosuperior mesotympanum
  9. 9. Reudi’s invagination theory  First proposed by Ruedi  Suggested in-utero infection of TM causing it to invaginate into the middle ear cavity and produce stratified squamous epithelium.  These invaginations predispose to cholesteatoma formation.
  10. 10. Congenital Cholesteatoma  Origin remains uncertain  Usually starts from the antero superior quadrant  Spreads through the posterior superior quadrant, attic and finally into the mastoid cavity  Mean age of presentation is 4.5 yo  M:F ratio is 3:1  Incidence is 0.12 per 100,000 people
  11. 11.  Anterosuperior quadrant > Posterosuperior quadrant
  12. 12. Acquired Cholesteatoma:  subdivided into primary acquired and secondary acquired cholesteatoma.
  13. 13. Primary Acquired Cholesteatoma  Definition –  Cholesteatomas that arise from retraction pockets  Implies that infection has not given rise to the cholesteatoma.
  14. 14. Pathogenesis Invagination theory:  Sterile OM → fibrosis and thickening of tympanic tissue→ attic block → localized negative pressure → localized small retraction of pars flaccida - Attic block and fibrosis also prevents normal pneumatisation of epitympanum and mastoid  Long standing Eustachian Tube Dysfunction - Long standing OME or other eustachian tube disease → ETD - Negative middle ear pressure → retraction of pars flaccida – Invagination (retraction pocket formation) → accumulation of desquamated debris in the retraction pocket → alteration to normal migration of epithelium→accumulation of keratin
  15. 15. Primary Acquired Cholesteatomas  Results in poor aeration of epitympanic space which draws pars flaccida medially on top of malleus neck, forming retraction pocket.  Normal migratory pattern of the tympanic membrane epithelium altered by retraction pocket  Enhances potential accumulation of keratin
  16. 16. Primary Acquired Cholesteatomas Pars flaccida retraction Pars tensa retraction
  17. 17. Secondary Acquired Cholesteatomas Definition:  Cholesteatomas that arise from a perforation in the TM – Implies a previous ear infection
  18. 18. Pathogenesis: SEPI  Implantation theory – Squamous epithelium implanted in the middle ear as a result of surgery (iatrogenic), foreign body, blast injury etc.  Squamous metaplasia theory – Chronic otitis media / recurrent otitis media → desquamated epithelium transformation to keratinized stratified squamous epithelium  Epithelial migration theory – Squamous epithelium migrates along perforation edge medially along undersurface of tympanic membrane destroying the columnar epithelium. – Secondary to ventilation tube / myringotomy insertion, tympanoplasty  Papillary ingrowth / invasion theory – Inflammatory reaction in Prussack’s space with an intact pars flaccida causes break in basal membrane → marginal perforation → skin from EAC wall migrates into the middle ear→ loss of contact inhibition with the middle ear mucosa (destroyed by infection) – Posterior superior TM
  19. 19. Common Sites of Acquired Cholesteatoma Origin  Posterior epitympanum  Posterior mesotympanum  Anterior epitympanum
  20. 20. Cholesteatoma Spread Posterior epitympanic cholesteatoma passing through superior incudal space and aditus antrum
  21. 21. Posterior mesotympanic cholesteatoma invading the sinus tympani and facial recess
  22. 22. Anterior epitympanic cholesteatoma with extension to with geniculate ganglion
  23. 23. Management History: 1. Hearing loss 2. Otorrhea: malodorous 3. Otalgia 4. Tinnitus 5. Vertigo Progressive unilateral hearing loss with a chronic foul smelling otorrhea should raise suspicion.  Previous history of middle ear disease 1. Chronic otitis media 2. Tympanic membrane perforation: Pars flaccida 3. Prior surgery
  24. 24. Examination:  Otoscopy  Microscopy  Positive fistula (pneumatic otoscopy will result in nystagmus and vertigo) response suggests erosion of the semicircular canals or cochlea  512Hz tuning fork exam - Always relate with audiometry results
  25. 25. Hearing evaluation  PTA: Conductive hearing loss  Tympanometry May suggest decreased compliance or TM perforation
  26. 26.  Preoperative imaging with computed tomographies (CTs ) of temporal bones (2mm ) section without contrast in axial and coronal planes. 1. Allows for evaluation of anatomy 2. May reveal evidence of the extent 3. Screen for asymptomatic complications Imaging
  27. 27.  Treated surgically with primary goal of total eradication of cholesteatoma to obtain a safe to and dry ear 1. Canal-wall -down procedures (CWD) 2. Canal-wall -up procedure (CWU) 3. Anterior atticotomy 4. Bondy’s procedure Treatment
  28. 28.  CWD surgery approach procedure involves: Taking down posterior canal wall to level of vertical facial nerve Exteriorizing the mastoid into external auditory canal
  29. 29.  Classic CWD operation is the modified radical mastoidectomy in which middle ear space is preserved  Radical mastoidectomy is CWD operation in which: Middle ear space is eliminated Eustachian tube is plugged  Meatoplasty should be large enough to allow good aeration of mastoid cavity and permit easy visualization to facilitate postoperative care and self cleaning
  30. 30. Indications for CWD approach:  Cholesteatoma in an only hearing ear  Significant erosion of the posterior bony canal wall  History of vertigo suggesting a labyrinthine fistula  Recurrent cholesteatoma after canal-wall -up surgery  Poor eustachian tube function  Sclerotic mastoid with limited access to epitympanum
  31. 31.  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
  32. 32. Canal-Wall -Down
  33. 33. Bondy’s operation  A type of modified radical mastoidectomy in which the mastoid cavity is exteriorized without disturbing the intact ossicular chain and pars tensa.  Indication: epitympanic cholesteatoma with intact ossicular chain, normal pars tensa, and good hearing.  The advantages of the technique are one-stage surgery with preservation of preoperative hearing levels, which is not possible with any other procedure.
  34. 34. 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 mastoidectomy with 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 - Ossicular chain reconstruction if necessary  Procedure should be adapted to extent of disease as well as skill of otologist
  35. 35.  CWU indication: -large pneumatized mastoid and well aerated middle space -Suggests good eustachian tube function  CWU contraindicated in: -Only hearing ear Patients with labyrinthine fistula -Long-standing ear disease -Poor eustachian tube function
  36. 36. 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
  37. 37. Canal-Wall -Up
  38. 38. Complications
  39. 39.  Hearing loss  Facial nerve paresis or paralysis  Labyrinthine fistula: semicircular canal erosion  Extradural or perisinus abscess  Serous or suppurative labyrinthitis Extracranial complications
  40. 40. Intracranial Complications  Potentially life-threatening  Incidence: as high as 1%  Complications 1. Periosteal abscess 2. Lateral sinus thrombosis 3. Intracranial abscess 4. Meningitis  Symptom: 1. Suppurative malodorous otorrhea 2. Chronic headache 3. Fever 4. Otalgia
  41. 41. Take Home Message  Theories /Pathogenesis of cholesteatoma remains uncertain  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  Aim of treatment: eradicate disease and provide a safe and dry ear  Awareness of serious and potentially life-threatening complications of cholesteatomas
  42. 42. Thank you so much for your kind attention
  43. 43. Reference: 1. Otology: an Overview By Prasad T Deshmukh, Ansu Sam 2. Abramson et all. Cholesteatoma pathogenesis; evidence of migration theory. Birmingham; Aesculapius 1977. p176 -86 3. Imaging of the Temporal Bone By Joel D. Swartz, Laurie A. Loevner 4. Ear, Nose and Throat Histopathology By Leslie Michael