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Cholesteatoma

Case Study. 16 year old female with left-sided otorrhea for two months. History of Present Illness. . History of Present Illness. Otorrhea is yellow and foul-smellingOtorrhea has not resolved with otic dropsOccasional dizzinessNo feverOtalgia has subsidedIntermittent unilateral tinnitusHearing

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Cholesteatoma

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    1. Cholesteatoma Vanessa Rothholtz, M.D. Department of Otolaryngology - Head and Neck Surgery University of California - Irvine September 27, 2007

    2. Case Study 16 year old female with left-sided otorrhea for two months

    3. History of Present Illness

    4. History of Present Illness Otorrhea is yellow and foul-smelling Otorrhea has not resolved with otic drops Occasional dizziness No fever Otalgia has subsided Intermittent unilateral tinnitus Hearing loss

    5. PMH / PSH / FH / Meds / Allergies Frequent ear infections as a child PE tubes x 2 No family history of hearing loss or ear problems No medications NKDA

    6. Physical Exam Weber localizes to left Rinne AC > BC AD BC > AC AS Facial nerve intact No nystagmus Otherwise - within normal limits

    7. Otoscopic Exam - Right Ear

    8. Otoscopic Exam - Left Ear

    9. Otoscopic Exam

    10. Otoscopic Exam

    11. Otoscopic Exam

    12. Otoscopic Exam

    13. Work-Up

    14. Work-Up Audiogram CT MRI

    15. Audiogram

    16. Computed Tomography CT is not essential for preoperative evaluation Should be obtained for: Revision cases due to altered landmarks from previous surgery Chronic suppurative otitis media Suspected congenital abnormalities Cases of cholesteatoma in which sensorineural hearing loss, vestibular symptoms, or other complication evidence exists

    17. Computed Tomography Erosion of scutum Destruction of ossicular chain Erosion of the labyrinthe (fistula) Low tegmen / tegmen defect Facial nerve dehiscence Petrous Apex Involvement

    18. Computed Tomography

    19. Computed Tomography

    20. Computed Tomography

    21. Magnetic Resonance Imaging Determine between neoplasm, encephalocele Determine between recurrence or persistent cholesteatoma vs. scar tissue / granulation tissue Dural involvement or invasion Subdural or epidural abscess Facial nerve involvement Tegmen defect / brain herniation Sigmoid sinus thrombosis

    22. Magnetic Resonance Dubrelle F et. al. Diffusion weighted MR imaging sequence in the detection of postoperative recurrent cholesteatoma. Radiology Feb 2006; 238 (2): 604-610. a. Coronal T2 b. Coronal delayed T1 c/ d. both coronal diffusion weighted. C has b factor of 0 d has b factor of 800 sec/ mm2. Granulation tissue on right. Recurrent cholesteatoma on left.a. Coronal T2 b. Coronal delayed T1 c/ d. both coronal diffusion weighted. C has b factor of 0 d has b factor of 800 sec/ mm2. Granulation tissue on right. Recurrent cholesteatoma on left.

    23. Differential Diagnosis

    24. Differential Diagnosis V Jugular bulb anomalies (high riding bulb, dehiscent jugular bulb, and jugular bulb diverticulum), aberrant internal carotid artery (ICA), hemangioma, persistent stapedial artery I Otitis Media, otitis externa, malignant otitis externa, tuberculous otitis T Tympanosclerosis A Granulomatous Diseases (a.k.a., Wegener’s granulomatosis)s M Osteoradionecrosis I Retained PE tube, Foreign Body N Cholesteatoma, paraganglioma / glomus tympanicum tumor, schwannoma, adenoma, endolymphatic sac tumor, cholesterol granuloma, polyps, adenocarcinoma, squamous cell carcinoma, adenoid cystic carcinoma C Cholesteatoma, encephalocele

    25. Congenital or Acquired Cholesteatoma?

    26. Congenital Cholesteatoma Criteria White mass medial to normal tympanic membrane Normal pars flaccida and pars tensa No prior history of otorrhea or perforations No prior otologic procedures Mean Age = 5 years (4.5 and 5.6) 1,2 Male : Female = 3:1

    27. Congenital Cholesteatoma - Type Nelson Type 1 – Confined to the middle ear and do not involve the ossicles Type 2 – Involve the posterior superior quadrants and attic, the site of the ossicular chain Type 3 – Involve the sites of type 1 and 2 as well as the mastoid

    28. Congenital Cholesteatoma - Type Nelson Type 1 – Controlled by extended tympanotomy. No second-look re-operation. Type 2 – Extended tympanotomy. Possibly atticotomy and canal wall up tympano-mastoidectomy with or without opening of the facial recess. Require second look. Possible ossicular reconstruction. Type 3 – Similar to type 2, but occasionally need a canal wall down tympanomastoidectomy

    29. Congenital Cholesteatoma -Stage Stage I – Limited to one quadrant Stage II – Involving multiple quadrants without ossciular involvement Stage III – Ossicular involvement without mastoid extension Stage IV – Mastoid involvement (67% risk of residual cholesteatoma)

    30. In which quadrant can a pediatric cholesteatoma most often be found?

    31. Anterosuperior > Posteriosuperior > Attic > Posterioinferior > Anterior Inferior > Mastoid

    32. Acquired Cholesteatoma Primary acquired – Attic retraction pocket cholesteatoma Secondary acquired – Occurs secondary to epithelial migration into the middle ear at the site of tympanic membrane perforation

    33. Anatomy - Tympanic Membrane Epitympanum Mesotympanum Hypotympanum Prussak’s Space The area between the pars flaccida laterally and the malleus neck and the lower portion of the head medially.

    34. Anatomy - Tympanic Membrane Epitympanic Space

    35. Anatomy - Facial Recess Lateral to facial nerve Bounded by the fossa incudis superiorly Bounded by the chorda tympani nerve laterally Sinus tympani Lies between the facial nerve and the medial wall of the mesotympanum

    36. Anatomic Locations Intradural / Extradural Middle ear cleft Mastoid Petrous Apex External auditory canal Cerebellopontine Angle

    37. Congenital Cholesteatoma Pathogenesis - Theory

    38. Congenital Cholesteatoma Pathogenesis - Theory “Acquired” inclusion theory - Tos

    39. Primary Acquired Cholesteatoma Pathogenesis - Theory Invagination of the tympanic membrane (retraction pocket cholesteatoma) Basal cell hyperplasia or Papillary ingrowth Epithelial invasion / ingrowth through a perforation (migration theory) Squamous metaplasia of the middle ear epithelium

    40. Primary Acquired Cholesteatoma Pathogenesis - Theory Implantation theory Squamous epithelium implanted in the middle ear as a result of disruption of the anatomy Papillary ingrowth theory Inflammatory reaction in Prussak’s space with an intact pars flaccida May cause break in basal membrane allowing cord of epithelial cells to start inward proliferation

    41. Primary Acquired Cholesteatoma Pathogenesis - Theory Epithelial invasion theory Squamous epithelium migrates along perforation edge medially and undersurface of tympanic membrane destroying the columnar epithelium Metaplasia theory Desquamated epithelium is transformed to keratinized stratified squamous epithelium secondary to chronic or recurrent otitis media

    42. Secondary Acquired Cholesteatoma Pathogenesis - Theory Result of the migration of tympanic membrane epidermis into the middle ear at the site of a marginal perforation The result of the implantation of viable keratinocytes into the middle ear cleft

    43. Predilection for Cholesteatoma Formation Children aged less than 5 years Goode T-tubes Frequent reinsertions Duration of placement exceeding 12 months Ears with history of frequent postoperative otorrhea

    44. Pathology Cystic content - Desquamated keratin center Matrix - Keratinizing stratified squamous epithelium Perimatrix - Granulation tissue - secretes proteolytic enzymes capable of bone destruction Hyperkeratosis

    45. Pathology

    46. Pathology

    47. Pathology

    48. Treatment Create a “dry and safe” ear

    49. Treatment Non-surgical Treat the Infection – Floxin Otic Drops Decrease the inflammation – Topical steroids Debridement of the external canal

    50. Treatment Surgical Atticotomy Radical Mastoidectomy Bondy Modified Radical (Canal wall down) mastoidectomy Tympanoplasty and canal wall up mastoidectomy

    51. General Mastoid Landmarks

    52. General Mastoid Landmarks Anterior - Spine of Henle Approximates the location of the underlying mastoid antrum. Superiorly - Linea temporalis The inferior border of the temporalis muscle Approximates the lowest level of the tegmen or floor of the middle fossa Inferiorly - Mastoid tip

    53. Facial Nerve Landmarks

    54. Facial Nerve Landmarks Horizontal semicircular canal Short process of the incus (1 to 2 mm deep to the plane) Posterior bony external auditory canal Care should be taken during the dissection of the posterior-superior mesotympnaum to avoid injury to the horizontal course of the facial nerve and the stapes or the stapes footplate

    55. Atticotomy Elevation of tympanomeatal flap Removal of scutum to limits of the cholesteatoma Aditus obliteration Reconstruction of the middle ear space Reconstruction of lateral attic wall with bone or cartilage is optional

    56. Radical Mastoidectomy Canal wall down mastoidectomy with exteriorization of the middle ear without attempt to restore its function Eustachian tube is occluded, malleus and incus are removed

    57. Modified Radical (Canal wall down) Mastoidectomy Disease limited to the epitympanum is exteriorized by removing portions of the adjacent superior and / or posterior canal wall Obliteration of mastoid air cells Aggressive saucerization of the cortical edges of the mastoid Complete removal of superior and posterior canal walls Meatoplasty

    58. Modified Radical Mastoidectomy (Canal wall down) - Indications Preoperative Disease in an only hearing ear Patients in poor health Patients with poor follow-up Intra-operative Unreconstructible posterior external auditory canal defect Labyrinthe fistula Obstructing low-lying middle fossa dura limiting the epitympanic access

    59. Modified Radical Mastoidectomy (Canal wall down) - Indications Involvement of the sinus tympani Involvement of the medial canal wall Ostitis or irremovable cholesteatomas in the hypotympanum Large defects of the canal wall Recurrent cholesteatoma after CWU surgery Poor Eustachian tube function (loose) Sclerotic mastoid with limited access to epitympanum

    60. Canal Wall Up Mastoidectomy Near-complete removal of mastoid air cell system Superior and posterior canal walls remain intact Facial recess approach

    61. Canal Wall Up or Canal Wall Down Mastoidectomy?

    62. CWU vs. CWD Considerations Background of the surgeon Anatomy of the patient’s temporal bone CWD – low tegmen or anterior sigmoid sinus Recurrent / Residual disease CWU – recurrent pars flaccida retraction with cholesteatoma formation into the attic State of the contralateral ear Mastoid pneumatization- Obliteration of mucosal surfaces Staged Procedure Second look Ossciular chain reconstruction after obliteration Extent of extension into the sinus tympani

    63. Advantages / Disadvantages of CWU Procedure 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

    64. Advantages / Disadvantages of CWD Procedure 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 Shallow middle ear space makes OCR difficult Dry ear precautions Difficulty to fit hearing aid

    65. CWU vs. CWD in Pediatric Cholesteatoma 86 Acquired 20 Congenital = 16 years old Cholesteatoma recurrence CWU vs CWD - 8% vs. 6% Hearing (pure-tone average <25 dB) CWU > CWD - 81% vs. 47% Predictors of cholesteatoma recidivism and poor hearing Extent of disease and stapes superstructure

    66. Surgical Intervention?

    67. Surgical Intervention?

    68. Surgical Intervention?

    69. Complications Dural tear - CSF leak Fistula of the horizontal semicircular canal (vertigo) – Up to 10% Facial nerve injury Injury to the sigmoid sinus / jugular bulb Otitic Hydrocephalus Hearing loss 30% have conductive loss pre-operatively Postoperatively, an additional 30% have worsening or onset of hearing loss due to extent of disease Infection – Meningitis, Abscess, lateral sinus thrombosis – Up to 1%

    70. Prognosis Residual or recurrent cholesteatoma over 5 years – 15 to 40% Reported to be up to 67% in the pediatric population Close follow - up Regular examinations needed - 6 months

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