CSOM SQUAMOSAL TYPE ETIOLOGY, PATHOLOGY CLINICAL FEATURES

MODERATOR DR.C .RAVISHANKAR PRESENTER- DR. ANILA VISWANATH

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Definition of csom
Chronic

inflammation of the mucoperiosteal lining of the middle ear cleft

classification
Inactive

mucosal com- perforation Inactive squamosal com- retraction Active mucosal com Active squamosal com - cholesteatoma Healed com

Inactive squamosal
Negative

static middle ear pressure lead to retraction of tm Retraction pocket invagination of a part of tm into middle ear


Fixed

Retraction pocket
free can move medially or laterally

TM adherent to M E structures

 Epidermisation Advanced

type of retraction Refers to replacement of middle ear mucosa by keratinising squamous epithelium without retention of keratin debris Often remains quiescent

Active squamosal com- cholesteatoma

History

Johannes

Mller (1838) coined the term cholesteatoma

a pearly tumor of fatamong sheets of polyhedral cells

Shucknecht

coined the term keratoma.

Definition
Cholesteatoma

is a bag like cystic structure lined by keratinizing squamous epithelium containing desquamated epithelium having erosive properties and mostly seen in temporal bone.

May develop anywhere within pneumatized portions of the temporal bone

Most

frequent locations: Middle ear space Mastoid

 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 of keratinising squamous epithelium Epithelium shows maturation without dysplasia
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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 an embryonic rest of epithelial tissue in an ear without tympanic membrane perforation in a patient without a history of ear infection

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Criteria
Levenson,

1989

White mass medial to normal tympanic membrane Normal pars flaccida and pars tensa No prior history of otorrhea or perforations No prior otologic procedures Prior bouts of otitis media were not grounds for exclusion as was the case in original definition
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Congenital cholesteatoma

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Pathogenesis theories

Failure of involution of ectodermal epithelial thickening called epidermoid formation that is present during fetal development found at junction of middle ear & ET (10 -33 wks of gestation)

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 Location
Male

-petrous pyramid, mastoid and middle ear cleft

preponderance Mean age of presentation- 4.5 yrs

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Congenital Cholesteatoma
Anterosuperior > Posterosuperior quadrant quadrant

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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

Nelson et. al Congenital Cholesteatoma: Classification, Management and Outcome. Arch Oto 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 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 2002;126(4):409 14 22

Primary Acquired Cholesteatomas

Eustachian Persistent Results

tube dysfunction

negative pressure in middle ear

in poor aeration of epitympanic

space

attic or posterosuperior retraction pocket

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Normal migratory pattern of the tympanic membrane epithelium altered by retraction pocket Enhances potential accumulation of keratin primary acquired cholesteatoma

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Primary acquired
Pars Pars

tensa cholesteatoma

flaccida cholesteatoma/ attic cholesteatoma

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Pathology of pars flaccida cholesteatoma

The epithelium of the cholesteatoma sac is continuous with that of pars flaccida and the accumulation 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 minor degree of hearing loss The disease further progresses into the anterior epitympanum and posteriorly in to the mastoid antrum and air cell system
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Pars flaccida retraction

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Pathology of para tensa cholesteatoma

Retraction onto the long process of incus, incudostapedial joint and promontory Long process involved early marked conductive deafness Occasionally cholesteatoma hearer Invagination into facial recess, sinus tympani and round window niche difficulty in eradication In the region of posterior annulus there is exposed bone that can be the site of osteitis and granulation tissue
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 Pars

tensa retraction

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Primary acquired cholesteatoma

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Secondary Acquired Cholesteatomas

Repeated

infection through perforation of epithelial

Migration through perforation

Metaplasia

middle

ear mucosa

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Metaplasia theory-Sade middle ear epithelium is transformed to keratinized stratified squamous epithelium secondary to chronic or recurrent otitis media Epithelial invasion theory - Habermann Squamous epithelium migrates along perforation edge medially along undersurface of tympanic membrane Papillary ingrowth theory- Ruedis Inflammatory reaction in Prussacks space with an intact pars flaccida (likely secondary to poor ventilation) may cause break in basal membrane allowing cord of epithelial cells to start inward proliferation Implantation theory Squamous epithelium implanted in the middle ear as a result of surgery, foreign body, blast injury, 32 etc.

 Once

cholesteatoma enters middle ear cleft it invades surrounding structures first by 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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Anatomy
Middle

Ear Regions

Epitympanum: superior to superior limit of EAC Mesotympanum: bound superiorly by superior limit of EAC and inferiorly by inferior limit of EAC 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 process of 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 38

Hypotympanum
Lies

inferior and medial to the floor of the bony ear canal Irregular bony groove that is seldom involved by cholesteatoma

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Common Sites of Cholesteatoma Origin

Posterior

epitympanum Posterior mesotympanum Anterior epitympanum

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Cholesteatoma Spread
Predictable

in that they are channeled along characteristic pathways by:

Ligaments Folds Ossicles

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3rd & 7th month 4 endothelially lined sacs evaginate from first branchial pouch. Mucosal folds and ossicular suspensory ligaments formed when these sacs contact each other.
B/w
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Saccus anticus Smallest pouch Form anterior pouch of von Troltsch Contact with anterior most saccule from saccus medius -Tensor fold. Above it is anterior compartment of attic.

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Saccus medius Breaks into three saccules Anterior saccule attic Medial saccule superior 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 of long crus of incus posterior pouch of von Troltsch and inferior incudal space Saccus posticus Form round window niche , sinus tympani oval 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 descending through floor of prussaks space into posterior 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 commonly involved

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Extend to mastoid via posterior tympanic isthmus & inf incudal space

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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

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 Tinnitus

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 ache Dizziness- labyrinth involved Facial deviation- facial canal eroded
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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 long process of incus Stage 2- retracted drum touching long process of incus Stage 3- retracted drum touching promontory Stage 4- drum plastered to promontory

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Pars flaccida retraction- tos

Stage

1-Mild retraction not touching neck of 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 thrombophlebitis Otitic 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
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Abscess in Relation to mastoid infection Post auricular abscess commonest Zygomatic abscess Bezold abscess Meatal / lucs abscess Citellis abscess

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Petrositis Infection of petrous part of temporal bone 2 groups of air cell tractsPosterosup & anteroinf Attic ,around semicircular Canal to apex hypotympanum 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 orbital pain, persistent ear discharge Fever headache

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Facial paralysis destruction of bony canal of facial nerve by cholesteatoma or from penetrating granulation tissue genu or horizontal portion

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Labyrinthitis

Diffuse serous Labyrinthitis

Diffuse suppurative Labyrinthitis

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Diffuse serous Labyrinthitis Diffuse intralabyrinthine inflammation without pus formation Vertigo Nausea nystagmus High frequency SN hearing loss

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Diffuse suppurative Labyrinthitis Diffuse infection of labyrinth with permanent loss of vestibular & cochlear functions Severe vertigo nausea vomiting spontaneous nystagmus Loss of hearing

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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 flow of pus from ear
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Subdural abscess b/w dura & arachnoid Meningeal irritation- headache fever malaise neck rigidity Cortical venous thrombophlebitis- aphasia hemiplegia ,hemianopia Raised ICT-papilloedema, ptosis

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 Meningitis

Inflammation

of leptomeninges With bacterial invasion of CSF in subarachnoid 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 by capsule stage of enlargement-oedema around abscess, ICT Stage of termination
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Lateralsinus thrombophlebitis Inflammation of inner wall of lateralsinus with thrombus formation Stage of perisinus abscess Endophlebitis & mural thrombus formation Obliteration of sinus lumen & intrasinus abscess Extension of thrombus

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Picket fence fever Headache Greisingers sign- oedema over posterior part of mastoid- thrombosis of mastoid emissary vein Pappiloedema Tobey ayer test -rise in csf pressure on healthy side no effect on d/s side on compression of jugular vein Crowe Back test- engorgement of retinal veins on healthy side on pressure on jugular vein
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Otitic hydrocephalus Raised ICT with normal csf finding Lateral sinus thrombosis obstructs venous return, if extending to superior sagittal sinus impede absorption of villi to absorb CSF headache diplopia blurring of vision papilloeedema nystagmus CSF pr. >300mm water
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