Csom_squamosal Type Anila

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    CSOM

    SQUAMOSAL TYPEETIOLOGY, PATHOLOGY

    CLINICAL FEATURES

    MODERATOR DR.C .RAVISHANKARPRESENTER- DR. ANILA VISWANATH

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    Definition of csom

    Chronic inflammation of themucoperiosteal lining of the middle earcleft

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    classification

    Inactive mucosal com- perforation

    Inactive squamosal com- retraction

    Active mucosal com- Active squamosal com - cholesteatoma

    Healed com

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    Inactive squamosal

    Negative static middle ear pressure lead toretraction of tm

    Retraction pocket invagination of a partof tm into middle ear

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    Retraction pocket

    Fixed free

    TM adherent to can move mediallyM E structures or laterally

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    Epidermisation

    Advanced type of retraction

    Refers to replacement of middle earmucosa by keratinising squamous

    epithelium without retention of keratindebris

    Often remains quiescent

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    Active squamosal com- cholesteatoma

    History

    Johannes Mller (1838) coined the termcholesteatoma

    a pearly tumor of fatamong sheets of

    polyhedral cells

    Shucknecht coined the term keratoma.

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    Definition

    Cholesteatoma is a bag like cysticstructure lined by keratinizing squamousepithelium containing desquamated

    epithelium having erosive properties andmostly seen in temporal bone.

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    May develop anywhere within pneumatizedportions of the temporal bone

    Most frequent locations:

    Middle ear space

    Mastoid

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    Histologically made up of :

    Cystic content anucleate keratin

    squames Matrix keratinizing squamous epithelium

    Perimatrix granulation tissue in contact

    with bone (produces proteolytic enzymes)

    Essential diagnostic feature presence ofkeratinising squamous epithelium

    Epithelium shows maturation withoutdysplasia

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    Histology

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    Histology

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    Cholesteatoma Classification

    Congenital

    Acquired

    Primary

    Secondary

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    Congenital Cholesteatoma

    Defined by Derlacki & Clemis as anembryonic rest of epithelial tissue in anear without tympanic membrane

    perforation in a patient without a history ofear infection

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    Criteria

    Levenson, 1989

    White mass medial to normal tympanicmembrane

    Normal pars flaccida and pars tensa

    No prior history of otorrhea or perforations

    No prior otologic procedures

    Prior bouts of otitis media were not groundsfor exclusion as was the case in originaldefinition

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    Congenital cholesteatoma

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    Pathogenesis theories

    Failure of involution of ectodermal epithelialthickening called epidermoid formation that is

    present during fetal development found atjunction of middle ear & ET (10 -33 wks ofgestation)

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    Location -petrous pyramid,

    mastoid and middle ear cleft

    Male preponderanceMean age of presentation- 4.5 yrs

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    Congenital Cholesteatoma

    Anterosuperior > Posterosuperiorquadrant quadrant

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    Congenital Cholesteatoma - TypeNelson

    Type 1 Confined to the middle ear and do notinvolve the ossicles

    Type 2 Involve the posterior superiorquadrants and attic, the site of the ossicular

    chain Type 3 Involve the sites of type 1 and 2 as well

    as the mastoid

    Nelson et. al Congenital Cholesteatoma:Classification, Management and Outcome. ArchOto Head Neck Surg July 2002; 128: 810:814.

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    Congenital Cholesteatoma -Stage

    Stage I Limited to one quadrant

    Stage II Involving multiple quadrants withoutossciular involvement

    Stage III Ossicular involvement withoutmastoid extension

    Stage IV Mastoid involvement (67% risk ofresidual cholesteatoma)

    Potsic WP, Korman SB, Samadi DS, et al.Congenital cholesteatoma: 20 years experienceat The Childrens Hospital of Philadelphia.Otolaryngol Head Neck Surg 2002;126(4):409

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    Primary Acquired Cholesteatomas

    Eustachian tube dysfunction

    Persistent negative pressure in middle ear

    Results in poor aeration of epitympanic

    space

    attic or posterosuperior retraction pocket

    N l i t tt f th t i

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    Normal migratory pattern of the tympanicmembrane epithelium altered by retractionpocket

    Enhances potential accumulation of keratin

    primary acquired cholesteatoma

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    Primary acquired

    Pars tensa cholesteatoma

    Pars flaccida cholesteatoma/ atticcholesteatoma

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    Pathology of pars flaccidacholesteatoma

    The epithelium of the cholesteatoma sac iscontinuous with that of pars flaccida and theaccumulation of white keratin debris is visible

    Associated with osteitis, granulation tissue and

    erosion of the outer attic wall Erosion of the ossicular heads occurs relatively

    late in the disease process hence minordegree of hearing loss

    The disease further progresses into the anteriorepitympanum and posteriorly in to the mastoidantrum and air cell system

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    Pars flaccida retraction

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    Pathology of para tensacholesteatoma

    Retraction onto the long process of incus,incudostapedial joint and promontory

    Long process involved early markedconductive deafness

    Occasionally cholesteatoma hearer Invagination into facial recess, sinus tympani

    and round window niche difficulty ineradication

    In the region of posterior annulus there isexposed bone that can be the site of osteitisand granulation tissue

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    Pars tensa retraction

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    Primary acquired cholesteatoma

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    Secondary Acquired

    CholesteatomasRepeated infection through perforation

    Metaplasia of epithelialmiddle ear mucosa Migration through

    perforation

    Metaplasia theory-Sade

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    Metaplasia theory Sade

    middle ear epithelium is transformed to keratinizedstratified squamous epithelium secondary tochronic or recurrent otitis media

    Epithelial invasion theory - HabermannSquamous epithelium migrates along perforationedge medially along undersurface of tympanicmembrane

    Papillary ingrowth theory- RuedisInflammatory reaction in Prussacks space with anintact pars flaccida (likely secondary to poorventilation) may cause break in basal membrane

    allowing cord of epithelial cells to start inwardproliferation

    Implantation theory

    Squamous epithelium implanted in the middle earas a result of surgery, foreign body, blast injury,etc.

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    Once cholesteatoma enters middle earcleft it invades surrounding structures firstby folllowing path of least resistance &

    then by enzymatic bone destruction

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    bone destruction produced by

    cholesteatoma explained by

    Pressure theory not accepted now

    Enzymatic theory- first by Lautenslager

    Acid po4ase

    Collagenase

    Acid protease

    Pge2, IL-1a, IL-1b, TNF-a, TNF-b

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    AnatomyMiddle Ear Regions

    Epitympanum: superior to superior limit of EAC

    Mesotympanum: bound superiorly by superior

    limit of EAC and inferiorly by inferior limit ofEAC

    Hypotympanum: inferior to inferior limit of

    EAC

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    Middle Ear Regions

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    Epitympanum

    Lies above the level of the short processof the malleus

    Contents:

    Head of the malleus

    Body of the incus

    Associated ligaments and mucosal folds

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    Mesotympanum

    Contents: Stapes Long process of the incus Handle of the malleus Oval and round windows

    Eustachian tube exits from the anterior aspect Two recesses extend posteriorly that are often not visible

    directly 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

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    Hypotympanum

    Lies inferior and medial to the floor of thebony ear canal

    Irregular bony groove that is seldominvolved by cholesteatoma

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    Common Sites of CholesteatomaOrigin

    Posterior epitympanum

    Posterior mesotympanum

    Anterior epitympanum

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    Cholesteatoma Spread

    Predictable in that they are channeledalong characteristic pathways by:

    Ligaments

    Folds

    Ossicles

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    B/w 3rd & 7th month 4endothelially linedsacs evaginate from

    first branchial pouch.Mucosal folds and

    ossicular suspensoryligaments formedwhen these sacscontact each other.

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    Saccus anticus

    Smallest pouch

    Form anterior pouch of von TroltschContact with anterior most saccule from

    saccus medius -Tensor fold. Above it isanterior compartment of attic.

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    Saccus medius

    Breaks into three saccules

    Anterior sacculeatticMedial sacculesuperior incudal space.

    Sends an offshoot between lateral mallear

    and lateral incudal prussaks space. Posterior saccule pneumatise petrous

    temporal

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    Saccus superior

    Extends b/w malleus handle and tip oflong crus of incus posterior pouch of vonTroltsch and inferior incudal space

    Saccus posticus

    Form round window niche , sinus tympanioval window niche

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    Cholesteatoma Spread

    Posterior epitympanic

    cholesteatoma passing

    from Prussaks space

    through superior incudal

    space and aditus ad

    antrum

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    Reaches middle ear by descendingthrough floor of prussaks space intoposterior space of von troeltsch

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    Posterior mesotympanum

    Extend to mastoid through

    posterior tympanic isthmus

    & inf. incudal space

    sinus tympani and

    facial recess commonlyinvolved

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    Extend to mastoid viaposterior tympanic

    isthmus & inf incudalspace

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    Anterior epitympanum

    Reach middle ear

    via anterior pouch

    of von Troltsch.facial n. dysfunction

    common

    may gain access to

    supratubal recess

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    Bacteriology

    Pseudomonas aeruginosa

    Streptococcus

    Proteus

    E coli

    Anaerobes- Bacteroids,

    peptococcus, fusobacterium

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    Clinical features

    Symptoms

    Discharge-

    foulsmelling- anaerobic infection,

    Bloodstained- granulation tissue & osteitis

    scanty,

    purulent

    Tinnitus

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    Hearing loss

    perforation of tympanic membrane- 10 40dB

    - ossicular interruption with intact drum 54 dB

    - ossicular interruption with perforation 38 dB

    - closure of oval window 60 dB Sometimes , patient hears better in presence of

    cholesteatoma- cholesteatoma hearer

    Ear acheDizziness- labyrinth involved

    Facial deviation- facial canal eroded

    Signs

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    Signs

    Foul smelling discharge in eac

    Attic perforation/ marginal perforation

    Attic retraction

    Cholesteatoma flakes- fishy odour

    Granulation tissue

    Aural polyp

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    Pars tensa retraction - sade

    Stage 1-mild retraction not touching longprocess of incus

    Stage 2- retracted drum touching long

    process of incus

    Stage 3- retracted drum touchingpromontory

    Stage 4- drum plastered to promontory

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    Pars flaccida retraction- tos

    Stage 1-Mild retraction not touching neckof malleus

    Stage 2-Attic retraction touching neck of

    malleus

    Stage 3-limited outer attic wall erosion

    Stage 4- severe attic wall erosion

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    Complications of CSOM

    Causes

    Pathway of spread

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    Classification

    Intra temporal

    Mastoiditis

    Petrositis

    Facial Paralysis

    Labyrinthitis

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    Intra Cranial

    Extradural abscess

    Subdural abscess

    Meningitis

    Brain abscess

    Lateral sinus thrombophlebitisOtitic Hydrocephalus

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    Acute Mastoiditis Infection involve bony walls of mastoid

    Beta hemolytic streptococcus

    CL/F Pain behind ear, fever, ear discharge

    Signs:

    Mastoid tenderness

    Sagging of posterosuperior meatal wall

    Swelling over mastoid- Ironed out mastoid

    Ab i R l i id i f i

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    Abscess in Relation to mastoid infection

    Post auricular abscess commonest

    Zygomatic abscess

    Bezold abscess

    Meatal / lucs abscess

    Citellis abscess

    Petrositis

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    Petrositis

    Infection of petrous part of temporal bone

    2 groups of air cell tracts-Posterosup & anteroinf

    Attic ,around semicircular hypotympanum

    Canal to apex around ET

    around cochlea

    to apex

    6th N & trigeminal ganglion closely related

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    Gradenigo syndrome

    Triad of LR palsy, deep seated retro orbitalpain, persistent ear discharge

    Fever

    headache

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    Facial paralysis

    destruction of bony canal of facial nerve

    by cholesteatoma or from penetratinggranulation tissue

    genu or horizontal portion

    Labyrinthitis

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    Labyrinthitis

    Diffuse serous Labyrinthitis

    Diffuse suppurative Labyrinthitis

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    Diffuse serous LabyrinthitisDiffuse intralabyrinthine inflammation

    without pus formation

    VertigoNausea

    nystagmus

    High frequency SN hearing loss

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    Diffuse suppurative Labyrinthitis

    Diffuse infection of labyrinth withpermanent loss of vestibular & cochlearfunctions

    Severe vertigo nausea vomitingspontaneous nystagmus

    Loss of hearing

    Extradural abscess

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    Extradural abscess

    b/w bone & dura

    Cl/f:Persistent headache

    Severe pain in ear

    Malaise

    Low grade fever

    persistent purulent ear discharge

    disappearance of headache with free flowof pus from ear

    S

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    Subdural abscess

    b/w dura & arachnoid

    Meningeal irritation- headache fever malaiseneck rigidity

    Cortical venous thrombophlebitis- aphasia

    hemiplegia ,hemianopia

    Raised ICT-papilloedema, ptosis

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    Meningitis

    Inflammation of leptomeninges

    With bacterial invasion of CSF insubarachnoid space

    Fever, headache, neck rigidity, nausea,vomiting

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    Otogenic brain abscess

    Stage of invasion pass unnoticed ,headache malaise

    Stage of localisation pus localised bycapsule

    stage of enlargement-oedema around

    abscess, ICT

    Stage of termination

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    Lateralsinus thrombophlebitis

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    Lateralsinus thrombophlebitis

    Inflammation of inner wall of lateralsinus

    with thrombus formationStage of perisinus abscess

    Endophlebitis & mural thrombus formation

    Obliteration of sinus lumen & intrasinusabscess

    Extension of thrombus

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    Picket fence fever Headache

    Greisingers sign- oedema over posterior part ofmastoid- thrombosis of mastoid emissary vein

    Pappiloedema

    Tobey ayer test -rise in csf pressure on healthyside no effect on d/s side on compression of

    jugular veinCrowe Back test- engorgement of retinal veins on

    healthy side on pressure on jugular vein

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    Otitic hydrocephalusRaised ICT with normal csf finding

    Lateral sinus thrombosis obstructs venous

    return, if extending to superior sagittalsinus impede absorption of villi to absorbCSF

    headache diplopia blurring of visionpapilloeedema nystagmus CSF pr. >300mmwater

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