Cholesteatoma

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
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Otoscopic Exam - Left Ear
9
Otoscopic Exam
10
Otoscopic Exam
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Otoscopic Exam
12
Otoscopic Exam
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Work-Up
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Work-Up

• Audiogram
• CT
• MRI

15
Audiogram
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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
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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.

23
Differential Diagnosis
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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., Wegeners
  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

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Congenital or Acquired Cholesteatoma?
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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 31

1. Levenson MJ et al. Congenital cholesteatomas
in children an embryologic correlation.
Laryngoscope. 1988 98949-955. 2. Nelson et. al
Congenital Cholesteatoma Classification,
Management and Outcome. Arch Oto Head Neck Surg
July 2002 128 810814.
27
Congenital Cholesteatoma - TypeNelson

• 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 - TypeNelson

• 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)

Potsic WP, Korman SB, Samadi DS, et al.
Congenital cholesteatoma 20 years experience at
The Childrens Hospital of Philadelphia.
Otolaryngol Head Neck Surg 2002126(4)40914.
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
• Prussaks 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 CholesteatomaPathogenesis - Theory

• Epithelial rest theory
• Teed-Michaels
• Inflammatory injury to an intact tympanic
  membrane results in microperforations in the
  basal layer
• Leads to invasion of the squamous epithelium by
  proliferating epithelial cones
• Acquired inclusion theory
• Tos
• Keratinized squamous epithelium may be implanted
  or included into the tympanic cavity during a
  pathological event affecting the tympanic
  membrane and middle ear in childhood

38
Congenital CholesteatomaPathogenesis - Theory

• Acquired inclusion theory
• - Tos

Epidermal rest theory - Teed-Michael
Semaan MT and Megerian CA. The pathophysiology
of cholesteatoma. Otolaryngol Clin N Am 39 (2006)
11431159
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 Prussaks 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

Golz A, Goldenberg D, Netzer A, et al.
Cholesteatomas associated with ventilation tube
insertion. Arch Otolaryngol Head Neck Surg
1999125(7)7547.
44
Pathology

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

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Pathology
46
Pathology
47
Pathology
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Treatment

• Create a dry and safe ear

Bennet M, Warren F and Haynes D. Indications and
technique in mastoidectomy. Otolaryngol Clin N Am
39 (2006) 10951113
49
TreatmentNon-surgical

• Treat the Infection Floxin Otic Drops
• Decrease the inflammation Topical steroids
• Debridement of the external canal

50
TreatmentSurgical

• Atticotomy
• Radical Mastoidectomy
• Bondy Modified Radical (Canal wall down)
  mastoidectomy
• Tympanoplasty and canal wall up mastoidectomy

51
General Mastoid Landmarks
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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

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Facial Nerve Landmarks
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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

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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

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Canal Wall Up Mastoidectomy

• Near-complete removal of mastoid air cell system
• Superior and posterior canal walls remain intact
• Facial recess approach

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Canal Wall Up or Canal Wall Down Mastoidectomy?
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CWU vs. CWDConsiderations

• Background of the surgeon
• Anatomy of the patients 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

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Advantages / Disadvantages of CWU Procedure

• Disadvantages
• Technically more difficult
• Staged operation often necessary
• Recurrent disease possible
• Residual disease harder to detect

• Advantages
• Rapid healing time
• Easier long-term care
• Hearing aids easier to fit
• No water precautions

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

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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
• CWU CWD - 81 vs. 47
• Predictors of cholesteatoma recidivism and poor
  hearing
• Extent of disease and stapes superstructure

Shirazi MA et. al. Surgical treatment of
pediatric cholesteatomas. Laryngoscope,
11616031607, 2006
66
Surgical Intervention?
67
Surgical Intervention?
68
Surgical Intervention?
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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

Smith JA and Danner CJ. Complications of chronic
otitis media and cholesteatoma. Otolaryngol Clin
N Am 39 (2006) 12371255
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