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This presentation describes cholesteatoma and its treatment

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  1. 1. Cholesteatoma Dr. T. Balasubramanian M.S. D.L.O. drtbalu
  2. 2. Definition • It is defined as a cystic bag like structure filled with desquamated squamous debris lying on fibrous matrix. • In lay terms it is defined as “skin in wrong place” • It is also known as keratoma drtbalu
  3. 3. History 1. The term cholesteatoma was coined by Johannes Muller in 1838. 2. Virchow, in 1855, considered cholesteatoma to be a tumor arising from the metaplasia of mesenchymal cells to epidermal cells, growing then as tumoral cells. 3. Politzer, in 1869, assumed that cholesteatoma was a glandular neoplasm of middle ear mucosa. 4. Bezold and Habermann, in 1889, considered cholesteatoma to be the result of migration of the external ear canal epidermis into the tympanic cavity via a marginal perforation after acute or chronic otitis. drtbalu
  4. 4. Histology • Expansile lesion of temporal bone composed of: 1. Cystic content: Desquamated keratin center 2. Matrix: Keratinizing stratified squamous epithelium 3. Perimatrix: granulation tissue that secretes multiple proteolytic enzymes capable of bone destruction. (Lie in contact with bone). drtbalu
  5. 5. Classification • Congenital • Acquired: 1. Primary 2. Secondary drtbalu
  6. 6. Congenital cholesteatoma drtbalu
  7. 7. Congenital cholesteatoma Levenson’s definition: • White mass behind the normal ear drum • Normal pars flaccida and pars tensa • No prior history of perforation / Otorrhoea • No previous otological procedures • Prior bouts of otitis media were not grounds for exclusion as was the case in original definition drtbalu
  8. 8. Theories “Congenital cholesteatoma” • Teed’s epithelial cell rest theory • Friedberg’s implantation theory • Ruedi’s invagination theory • Aimi’s epithelial migration theory • Michael’s epidermoid formation theory drtbalu
  9. 9. Teed’s theory • Proposed in 1936 • Persistence of 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. They involute at a later date. drtbalu
  10. 10. Implantation theory • Friedberg observed viable squamous cells in the amniotic fluid present in the middle ear of neonates. • Could they be a cause for congenital cholesteatoma? drtbalu
  11. 11. Invagination theory • First proposed by Ruedi • Suggested inutero infection of ear drum causing it to invaginate into the middle ear cavity. • These invaginations usually predispose to cholesteatoma formation drtbalu
  12. 12. Epithelial migration theory • First proposed by Aimi • He suggested that ectoderm of external canal managed to grow / migrate in to the middle ear cavity somehow overcoming the restraining influence of the annulus. drtbalu
  13. 13. Epidermoid formation theory • First proposed by Michael • He observed nests of squamous epithelium in the lateral wall of tympanic cavity below the level of pars flaccida • These nests normally involute • Failure of this involution process can possibly cause cholesteatoma drtbalu
  14. 14. Features of 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 drtbalu
  15. 15. Primary acquired cholesteatoma • Etiology unknown • Various theories have been proposed drtbalu
  16. 16. Theories of primary acquired cholesteatoma • Cawthrone theory • Tumarkin’s theory • Toss theory of invagination • Wendt’s metaplastic theory • Habermann’s epithelial invasion theory drtbalu
  17. 17. Cawthrone theory • Suggested by Cawthrone in 1963 • He suggested that cholesteatoma originated from congenital embryonic cell rests present in various areas of temporal bone drtbalu
  18. 18. Tumarkin’s theory • Tumarkin believed that cholesteatomas occur due to migration of squamous epithelium from the deep portion of the external canal in to the middle ear cleft through a marginal perforation / total perforation of ear drum. • Total perforation is seen in acute necrotizing otitis media drtbalu
  19. 19. Toss theory of invagination drtbalu
  20. 20. Toss theory (contd) • Toss postulated that persistent negative pressure in the attic region causes invagination of pars flaccida thus forming a retraction pocket. • This retraction pocket later becomes filled with desquamated squamous epithelial debris. These debris form a nidus for bacterial infections. drtbalu
  21. 21. Toss’s grades of retraction pockets • Grade I : The pars flaccida is retracted, but is not in contact with the neck of the malleus. • Grade II : The retracted pars flaccida is in contact with the neck of the malleus clothing it. • Grade III : Here in addition to grade II features there is minimal erosion of the outer attic wall • Grade IV : In this condition, in addition to grade III features the outer attic wall is drastically eroded. drtbalu
  22. 22. 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. drtbalu
  23. 23. Epithelial invasion theory • This theory was first proposed by Habermann • He suggested that attic perforation caused squamous epithelium to migrate into the middle ear cavity drtbalu
  24. 24. Secondary acquired cholesteatoma • Commonly caused in patients with acute necrotizing otitis media • There is associated destruction of the annulus of the ear drum • The annulus prevents epithelial migration from the external canal to the middle ear cavity. drtbalu
  25. 25. Features of acute necrotizing otitis media • Caused by exanthematous fever like measles. • Should be suspected in cases with early spontaneous perforation, aural secretions devoid of mucin, secretions with foul odor, and presence of extensive hearing loss. • Pneumococci type III have been implicated drtbalu
  26. 26. Anatomic considerations • Cholesteatomas in attic start from the Prussacks space • This space lie between pars flaccida and handle of the malleus drtbalu
  27. 27. Cholesteatoma spread • Is determined by: Ligaments Mucosal folds Ossicles drtbalu
  28. 28. Common sites of cholesteatoma • Posterior epitympanum • Posterior mesotympanum • Anterior epitympanum drtbalu
  29. 29. Cholesteatoma spread Posterior epitympanic cholesteatoma passes through superior incudal space to involve aditus and antrum drtbalu
  30. 30. Cholesteatoma spread Posterior mesotympanic cholesteatoma invades the sinus tympani and facial recess drtbalu
  31. 31. Cholesteatoma Anterior epitympanic cholesteatoma can involve the geniculate ganglion of facial nerve drtbalu
  32. 32. Evaluation • History: Detailed otologic history including Hearing loss • Otorrhea • Otalgia • Nasal obstruction • Tinnitus • Vertigo • Previous history of middle ear disease: CSOM TM perforation Previous surgery drtbalu
  33. 33. Evaluation • Head and neck examination • Otologic examination Otomicroscopy is essential in evaluating the extent of disease – Clean ear thoroughly of Otorrhea and debris with cotton-tipped applicators or suction – Culture wet, infected ears and treat with topical and/or oral antibiotics – Pneumatic otoscopy should be performed in every patient with cholesteatoma Positive fistula test (pneumatic otoscopy will result in nystagmus and vertigo) response suggests erosion of the semicircular canals or cochlea drtbalu
  34. 34. Evaluation • Hearing evaluation to access conductive hearing loss Pure tone audiometry Speech reception thresholds Word recognition • 512 Hz tuning fork examination • Tympanometry drtbalu
  35. 35. Conductive hearing loss • The degree of conductive hearing loss may vary greatly: Moderate hearing loss of more than 40 dB may indicate ossicular discontinuity due to erosion of long process of incus / head of stapes Mild conductive deafness may be present with extensive disease if cholesteatoma sac transmits sound directly to stapes or footplate drtbalu
  36. 36. Role of imaging CT scan of temporal bones 2mm sections in both axial and coronal planes will help: In evaluating the anatomy Revealing the extent of the disease Asymptomatic complications may become revealed. drtbalu
  37. 37. Indications for CT • Revision cases where the landmarks may be distorted • In patients with suspected congenital anamolies • Cholesteatoma with s/n loss and accompanying vestibular symptoms drtbalu
  38. 38. Management Cholesteatoma is a surgical problem Goals of surgery include: To make the ear safe by eradicating the cholesteatoma and infection To conserve residual hearing Improvement of hearing when possible To provide acceptable cosmetic appearance To reconstruct the ear in a manner that reduces the chances of recurrence drtbalu
  39. 39. Surgeries • Grommet insertion (to manage early retraction pockets) • Intact canal wall mastoidectomy • Canal wall down mastoidectomy • Transcanal anterior atticotomy drtbalu
  40. 40. Canal wall down procedure • Most commonly used • Good access • Middle ear space reduced • Large cavity (cavity problems) drtbalu
  41. 41. Canal wall down procedure • Posterior canal wall is reduced up to the level of vertical portion of facial nerve • Middle ear, attic, antrum,and mastoid are exteriorized and made into a single cavity • Attic is obliterated • Large meatoplasty is performed drtbalu
  42. 42. Indications of canal wall down • Cholesteatoma in the only hearing ear • Erosion of posterior canal wall • H/o vertigo suggesting labyrinthine fistula • Recurrent cholesteatoma after canal wall up surgery • Poor Eustachean tube function • Sclerosed mastoid with poor access to attic region drtbalu
  43. 43. Canal wall down surgery drtbalu
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  47. 47. Canal wall up surgery • More conservative procedure • Posterior canal wall is retained • Middle ear space is maintained • Normal physiology is maintained • Usually is performed as a staged procedure drtbalu
  48. 48. Indications of canal wall up procedure • In patients with well pneumatized mastoid • Patients with good Eustachean tube function • In patient’s with limited disease (confined to the attic) drtbalu
  49. 49. Contraindications of CWU • Patients with poor Eustachean tube function • Patient with labyrinthine involvement • Long standing ear disease • Patients with intra cranial complications drtbalu
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  51. 51. Advantages of CWU • Rapid healing time • No cavity problems • Hearing aids can be used without problems drtbalu
  52. 52. Transcanal anterior atticotomy Procedure involves: – Elevation of tympanomeatal flap via endaural incision with removal of scutum to limits of the cholesteatoma – Aditus obliteration with muscle, fascia, cartilage or bone prior to reconstruction of the middle ear space – Reconstruction of lateral attic wall with bone or cartilage is optional • May lead to retraction disease and possible recurrence in patients with poor Eustachian tube function drtbalu
  53. 53. Complications of cholesteatoma • Infection • Otorrhea • Bone destruction • Hearing loss • Facial nerve paresis or paralysis • Labyrinthine fistula • Intracranial complications drtbalu
  54. 54. Conclusions • Pathogenesis of cholesteatoma remains 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 and provide 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-threatening complications of cholesteatomas drtbalu