Abramson et al (1977) -A three dimensional epidermal and connective tissue structure, usually in the form of a sac. Frequently conforming to the architecture of various spaces of the middle ear, attic, mastoid. Capacity for progressive and independent growth at the expense of underlying bone, and a tendency to recur after removal
Pathology Cholesteatoma first forms when keratinizing squamous epithelium from the external canal traverses the plane of the tympanic membrane. Once this plane is breached, the cholesteatoma sheds squamous debris into its center and grows passively to occupy the middle ear cleft Cholesteatoma has two components—the acellular keratin debris , which forms the contents of the sac, and the matrix , which forms the sac itself.
ORIGIN OF CHOLESTEOTOMA Presence of congenital cell rests Invagination theory ( Whittmaack’s ) Epithelial invasion theory ( Habermann’s ) Basal cell hyperplasia theory ( Reudi’s ) Metaplasia theory (Sade’s)
Etiological classification- cholesteatoma 7
Acquired Cholesteatoma Theories Histopathology Bone destruction in cholesteatoma Signs and S ymptoms Grades of retraction
Theories of cholesteatoma Primary acquired cholesteatoma Secondary acquired cholesteatoma Invagination theory ( Whittmaack’s ) Epithelial invasion theory ( Habermann’s ) Basal cell hyperplasia theory ( Reudi’s ) Metaplasia theory (Sade’s)
Invagination ( Whittmaack’s)
Grades of Retraction- Sade
Basal cell hyperplasia (Reudi’s)
Invasion ( Habermann’s)
Metaplasia ( Sade’s)
Theories of genesis of cholesteatoma acquired
Bone erosion Bone erosion is responsible for the invasive nature of cholesteatoma Erosion of scutum occurs early as the cholesteatoma expands into the attic. Erosion of the ossicles (the lenticular process of the incus, superstructure of the stapes, body of incus and manubrium) - causes conductive hearing loss.
Causes of Bone destruction Erosion of the bony septations of the mastoid appears as coalescence on CT this finding, plus scutum,erosion , is the radiologic hallmark of cholesteatoma. Pressure induced bone resorption- i.e osteoclastic- induced bone resorption Enzymatic dissolution
Congenital Cholesteatoma Definition History Theories Histopathology Presentation and growth Diagnostic criteria Various locations
Definition Congenital cholesteatomas comprise of squamous epithelium retained in middle ear space, during embryologic migration of squamous cells
Embryonic Cell Rest Theory (Teeds Theory) Squamous epithelial cell rests are frequently found in the mucosa of the foetal mastoid. Normally disappears at around 33 weeks of gestation. Congenital cholesteatoma results from the failure of these cells to involute
Histopathology Congenital cholesteatoma is a stratified squamous epithelium lined cyst filled with keratin debris Indistinguishable from the acquired variety Keratin cyst surrounded by epithelial cells Squamous epithelium surrounding keratin can erode into the ossicular chain No connection between congenital cholesteatoma and TM
Clinical Features Male:Female = 3:1 Rarely diagnosed in infancy Mean age of presentation : 4-5 years 82% patients are asymptomatic (incidental finding) Painless, white, globular pearl-like structure, Most common in antero-superior quadrant of TM.
There is no connection to the tympanic membrane, which is normal in appearance and intact Conductive hearing loss: Anteriorly, affect the eustachian tube function , causing middle ear effusion Posteriorly, cause impairment of ossicular chain mobility Vertigo and sensory neural hearing loss if labyrinth is involved
Revised Criteria (by Levenson, 1989) White mass medial to an intact TM Normal pars tensa and flaccida No prior otological operations
The Middle Ear The Mastoid The Petrous Apex Cerebello-Pontine Angle Sites Involved
CHRONIC OTITIS MEDIA: AAD (SQUAMOSAL)
Contents Introduction to Chronic Otitis Media Chronic Otitis Media - Squamosal type Cholesteatoma Acquired cholesteatoma Congenital cholesteatoma Management of Chronic Otitis Media - Squamosal
Definition Chronic suppurative otitis media : Chronic suppurative otitis media (CSOM) is defined as chronic inflammation of the middle ear cleft and characterised by permanent perforation and discharge.
Inactive squamosal (Retraction)- Retraction of the pars flaccida or pars tensa (usually posterio -superior) which has the potential to become active with retained debris Active squamosal- Retraction of the pars flaccida or tensa that has retained squamous epithelial debris and is associated with inflammation and the production of pus, often from the adjacent mucosa
CSOM- ATTICO ANTRAL TYPE (SQUAMOUS TYPE) Unsafe or Dangerous type Involves posterosuperior part of middle ear cleft( attic, antrum, posterior tymapanum and mastoid) Associated with an attic or marginal perforation Associated with cholesteotoma due to its bone eroding properties
Squamosal - Inactive Negative static middle ear pressure can result in retraction of the tympanic membrane. A retraction pocket consists of invagination into the middle ear space of part of the tympanic membrane
Etiology and etiopathogenesis of COM Squamosal Presence of congenital cell rests Invagination theory ( Whittmaack’s ) Epithelial invasion theory ( Habermann’s ) Basal cell hyperplasia theory ( Reudi’s ) Metaplasia theory (Sade’s)
Epidemiology of COM Squamosal Incidence is higher in developing countries - - Poor socio-economic standards - Poor nutrition - Lack of health education - Overcrowding in home Affects both sexes & all age groups No sex difference Annual incidence : 9.2/100000
PATHOLOGY Cholesteotoma Osteitis and Granulation tissue- involves outer attic wall and posterosuperior margin of tympanic ring Ossicular necrosis Cholesterol granuloma- it is a mass of granulation tissue with foreign body giant cells surrounding cholesterol crystals, occurs secondary to long standing retention of secretions
Bacteriology The infecting organism depends on whether the initial ear infection is acute or chronic In acute: Haemophilus influenzae Streptococcus pneumoniae In chronic: Gram negative or anaerobic pathogens are seen Pseudomonas aeruginosa, Proteus species, Streptococcus milleri and bacteroids
Signs Foul smelling discharge in EAC Attic or marginal perforation Retraction pocket in attic Cholesteatoma flakes Granulation tissue Aural polyp
Grave Clinical Features
Examination Otomicroscopy/otoendoscopy Tuning fork test Tests for Facial nerve weakness Fistula test Test for Eustachian tube patency Management of cholesteatoma
Microscopy To confirm diagnosis Site of granulation tissue Status of ossicles To confirm site & margins of perforation Examination of attic and retraction pocket Exact site of origin & posterior limit of cholesteatoma A ids in suction clearance
Rigid Endoscopy Ideal - 0 degree Depth of retraction pocket Save image (medical record, teaching purposes) To visualize anterior recess
Investigations Swab for Culture sensitivity Haemogram Viral markers Pure tone audiogram X ray mastoid CT-scan / MRI
X-Ray Mastoid (Schuller’s view ) Pneumatisation pattern Bone destruction Cavity in mastoid Status of key area of mastoid –attic, aditus, antrum
CT Scan CT scan :- Imaging modality of choice for preoperative planning Allows for evaluation of anatomy Location of the lesion Size of the lesion Screen for asymptomatic complication Coronal cuts are preferred (1.5mm)
Appearance :- Soft tissue hypodense expansile lesion, round to oval in shape, well defined margins Erosion of - Scutum Ossicles Mastoid air cells Erosion of the bony septations of the mastoid and scutum erosion is the radiological hallmark of Cholesteatoma ( Cholesteatoma ,Theime Pub.)
MRI Define the borders of large lesions Depict the relationship of the lesion to intracranial structures Evaluating complications Defines the integrity of the dura Differentiate cholesteatomas from other temporal-bone lesions
Treatment Surgical management Reconstructive surgery
Treatment modality depends on – Extent of disease Complications Hearing status ET function Mastoid pneumatisation General medical condition
TREATMENT SURGERY- Total eradication of cholesteatoma to obtain a safe and dry ear Restoration or maintaining the functional capacity of hearing Maintain a normal anatomic appearance of the ear if possible
Surgeries - Canal Wall Down Radical mastoidectomy Modified Radical Mastoidectomy - Canal Wall Up (combined approach)
Canal Wall Down Posterior canal wall lowered down so that the mastoid cavity and EAC comes to lie in the same plane. Diseased area fully exteriorised
RADICAL MASTOIDECTOMY is a canal wall down mastoidectomy performed to eradicate disease from middle ear cleft in which mastoid cavity, tympanum and EAC are converted into a common cavity exteriorised through the EAC, wherein the structures of tympanic cavity (remnants of the incus and malleus, and the drum remnant) are removed.
EAR RM Mastoid cavity ME ME EAC MAS mastoid cavity ME EAC
MRM is a surgical procedure where the disease process is eradicated from the middle ear cleft; followed by converting the mastoid cavity, middle ear and EAC into a single, smooth, self-cleansing cavity exteriorised through EAC.
EAR MRM Mastoid cavity ME ME EAC MAS mastoid cavity ME EAC
Canal Wall Up and combined approach Combined Approach Tympanoplasty (CAT) consists of cortical mastoidectomy with an intact but thin bony ear canal wall and a posterior tympanotomy.