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•Pathogenesis
•Classification
•Complications
Vishav
Yadav
1
 Keratin-producing squamous epithelium in the
middle ear, mastoid or petrous apex
 Johannes Müller (1838):German Physiologist
coined cholesteatoma i.e. layered paerly
tumpour of fat
 Schuknecht : keratoma
2
Abramson
3D epithelial and connective tissue structure usually
in form of sac , mostly confined to middle ear
spaces , with tendency of independent growth on
cost of underlying bone , with tendency of
recurrence despite complete removal .
3
 Histologically made up of :



Cystic content – anucleate keratin squames
Matrix – keratinizing squamous epithelium
Perimatrix – granulation tissue in contact with bone
(produces proteolytic enzymes)
4
 Congenital
 Acquired



Primary acquired (retraction pocket)
Secondary acquired
Tertiary acquired
5
6
The incidence of CC - 1% to 5% of all
cholesteatomas
 Congenital cholesteatoma of the middle
ear was first described by Howard House
Location (AS,PS , petrous pyramid, mastoid
and middle ear cleft , CP Angle)
7

Levenson criteria (Modified Derlacki & Clemis)
 White mass medial to normal TM
 Normal pars flaccida and tensa
 No history of otorrhea or perforations
 No prior otologic procedures
 Prior bouts of otitis media not grounds for exclusion
8
 The accepted cause of CC remains controversial
Teed’s epithelial cell rest theory
 Friedberg’s implantation theory
 Ruedi’s invagination theory
 Aimi’s epithelial migration theory
 Michael’s epidermoid formation theory

9
 Most commonly accepted and quoted theory
 Teed
 An epidermal structure : these rests as
ectodermal implants in the fusion plates between
the first and second branchial arches that appear
around 10 weeks
 It aided in middle ear and tympanic membrane
development. Initially dormant, it undergoes
rapid proliferation before resorption around 33
weeks’ gestation
 CC is thought to form if resorption is incomplete
10
 Friedberg observed viable squamous cells in the
amniotic fluid present in the middle ear of
neonates.
 Could they be a cause for congenital
cholesteatoma?
11
 Ruedi
 Inflammatory injury to an intact tympanic
membrane
 Microperforations in the basal layer that lead to
invasion of the squamous epithelium by
proliferating epithelial cones through a
macroscopically intact but microscopically injured
tympanic membrane.
 Epithelial cones fuse and expand forming a
middle ear cholesteatoma
12
 First proposed by Aimi
 He suggested that ectoderm of external canal
managed to grow / migrate in to the middle ear
cavity somehow overcoming the restraining
influence of the annulus.
13
 First proposed by Michael
 He observed nests of squamous epithelium in the
lateral wall of tympanic cavity below the level of
pars flaccida
 These nests normally involute
 Failure of this involution process can possibly
cause cholesteatoma
14
15
(1) aid the clinician in preoperative planning of
treatment
(2) Indicate prognosis
(3) facilitate exchange of information between
different clinicians
(4) evaluate the results of treatment.
16
 Petrous Pyramid
 Mastoid
 Tympanic
17
 Stage 1: Single quadrant with no ossicular or
mastoid involvement
 Stage 2: Multiple quadrants with no ossicular or
mastoid involvement
 Stage 3: Ossicular involvement but no mastoid
involvement
 Stage 4: Mastoid extension
18
 Type 1: Mesotympanum
with no incus or stapes
erosion
 Type 2: Mesotympanum
or attic with ossicular
erosion but no mastoid
extension
 Type 3: Mesotympanum
with mastoid extension
Clinical
Stage
Recurrence
Rates
TYPE 1
15
0
TYPE 2
59
34
TYPE 3
26
55
19
20

Invagination theory of Wittmaack
 Eustachian tube dysfunction
 Poor aeration of the epitympanic space
 Retraction of the pars flaccida
 Normal migratory pattern altered
 Accumulation of keratin, enlargement of sac
21
 Basal cell hyperplasia (Ruedi theory)
 Inflammatory injury to an intact tympanic
membrane
 Microperforations in the basal layer that
lead to invasion of the squamous epithelium
by proliferating epithelial cones through a
macroscopically intact but microscopically
injured tympanic membrane.
 Epithelial cones fuse and expand forming a
middle ear cholesteatoma
22
 Squmaous metaplasia (Sade theory)
 transformation of cuboidal epithelium to
squamous epithelium from chronic infection
23


Presence of preexisting perforation in pars tensa
Often associated with posterosuperior marginal
perforation or large central perforation
24

Immigration & Invasion:





Habermann
medial migration along permanent perforation of TM
Implantation & invasion –foreign body, blast injury
Metaplasia – transformation of cuboidal epithelium
to squamous epithelium from chronic infection
Papillary ingrowth – intact pars flaccida,
inflammation in Prussack’s space, break in the basal
membrane, cords of epithelium migrate inward
25





iatrogenic – surgery, foreign body, blast injury
Tympanoplasty
Ossiculoplasty
Stapedotomy
Typmpanostomy tubes
26
27
 Epitympanic
cholesteatoma patterns
of spread from
Prussack’s space



Posterior epitympanum
Posterior mesotympanum
Anterior epitympanum
28




Medially :neck of malleus
laterally :Shrapnell’s membrane
Sup : lat malleal fold
Anteriorly anterior malleolar fold & anterior ligament
30
 Posterior
epitympanum –
through superior
incudal space to
mastoid antrum
31

Posterior
mesotympanum –
inferiorly through
posterior pouch of Von
Troeltsch to stapes,
round window, sinus
tympani and facial
recess
32
 Anterior epitympanum –
anterior to head of
malleus, may gain access
to supratubal recess
 To ant mesotympanum
via anterior pouch of von
Troeltsch
33
*Medially – Medial Wall Of Middle Ear
with Oval & Round Window Niches
*Laterally – Pyramid & Facial Nv
*Superiorly– Ponticulus
*Inferiorly – Subiculum
-can extend as far as 9mm into the
mastoid
-probably the most inaccessible site in
middle ear & mastoid
 Molecular models



Preneoplastic transformation events
Defective wound-healing process
Collision between host inflammatory response, normal middle
ear epithelium, and bacterial infection
35
 Hyperproliferative keratinocytes


Increased proliferation
Decreased terminal differentiation
 Expression of epithelial markers in the basal
and suprabasal layers (cytokeratins –10,13,16,
filaggrin, involucrin); confirm they arise from
pars flaccida and overlying EAC skin
 High expression of epidermal growth factor
receptor, transforming growth factor
 Upregulation of p53
36
 Chronic inflammatory response around matrix
(granulation/perimatrix)
 Infiltration of activated T-cells and
macrophages
 Production of cytokines (TGF,TNF,IL-1,IL2,FGF,PDGF)
 Causes increased migration and invasion of
cholesteatoma epithelium and fibroblasts
37
 Bacterial related antigens producing host
inflammatory response may stimulate the
migrating epithelium’s uncoordinated
proliferation
 Granulation induces invasion of keratinocytes
 Granulation – contains proteases, acid
phosphatases, bone resorption proteins,
osteoclast-activating factors, prostaglandins
38
39
Intracranial
Extradural abscess
 Subdural abscess
(empyema)
 Sigmoid sinus
thrombophlebitis
 Meningitis
 Brain abscess
 Otitic
hydrocephalus

40

Intratemporal
 Facial paralysis
 Labrynthine infections
 Labyrinthine fistula
 Petrositis
 Mastoiditis
 Other rare complications:
 Abscess:
 Bezold’s abscess
 Luc’s abscess
 Citeli’s abscess
 Distant pyaemic abscess

Extratemporal complications:
 Subperiosteal abscess
 Migrating
thrombophlebitis of IJV
 Migrating
thrombophlebitis of IJV
41
Possible routes of spread:
Extension through bone
 Infected clot within small veins
 Normal anatomical pathways
 Non anatomical bony defects
 Surgical defects
 Into brain tissue along
periarteriolar spaces of Robin
Virchow

42
 General principles:





The complications are multiple in about 1/3rd of the cases
The symptoms of intracranial spread of infection are those
of infection and those of brain tissue compression
Otalgia is never a symptom of uncomplicated
cholesteatoma
Investigation and treatment must run concurrently
The principles of treatment :
 Systemic antibiotic therapy
 Local neurosurgical attention to the complication(s)
 Treatment of the ear lesion
43

Intracranial
complication:
 G-ve organisms
 Staphylococci- beta
lactamase producing
 Obligate anaerobes
Bacteroides fragilis

Extratemporal
and temporal
complications:
 Pseudomonas
 Proteus
44

High grade fever
Headache with vomiting
Copious ear discharge
Gait changes
Visual alterations
Seizure activity
Neck stiffness

Unusual persistent pain






45
 Etiology :Bone
erosion
 Sigmoid perisinus
abscess
46
47
 Clinical features:



Site and size
Duration and rate
Incidental finding


Headache with malaise
Intermittent relief from pain during episodes of aural
discharge
 Diagnosis :


Operative findings
CT scan
48
49
 Management :


Surgical exploration
????Don’t remove the granulation tissue attached to the dura
Appropriate antibiotics
50
 Spread of infection



Loculated infection
Thrombophlebitis
Obliteration of subdural space
 Commonly associated
organisms:


Strp. milleri
H. influenzae
51
52
 Clinical features:






Severe headache
Fever
Drowsiness
Focal neurological deficit
Seizure
Paralysis
 The course is much more rapid than brain abscess
 Papilledema is uncommon, as are the cranial nerve palsy
 Seizures and FND differentiates from meningitis
53
 Diagnosis



CT scan
MRI
LP : helpful but risky
 ↑pressure
 Normal sugar content
 Cultures are sterile
 Marked pleocytosis
54
 Management :




Removal of subdural fluid
Massive antibiotics
Treatment of ear disease
Antiepileptics
55
 Lateral sinus :
Sigmoid sinus
 Transverse sinus
Usually preceded by the development of extradural
perisinus abscess



Spread of infection







Torcula herophili (confluence of sinuses)
Superior sagittal sinus
Superior and inferior petrosal sinus
Cavernous sinus
Brain abscess
Internal jugular vein
Subclavian vein
56
57
58
 Commonly associated organisms:



Beta hemolytic streptococci
Staphylococus aureus
Now a days : mixed flora :
 Venezio et al in 1982 grew Proteus mirabilis,
staphylococci, streptococcus pneumoniae and
Bacteroides oralis
59
 Clinical features:
Classical picture before
antibiotics:






Spread along the IJV:


Severe pyrexial wasting illness
Develop over several weeks
Picket fence fever
Headache and neck pain
Emaciation with anaemia





Perivenous inflamation
Perivenous edema
Suppuration of the
lymph node
Paralysis of the lower
cranial nerves
↑ICP
Hydrocephalus
Superior extension


Superior sagittal sinus
Cavernous sinus
60

Septic embolisation (sinus thrombophlebitis)






Lung fields
Large joints
Subcutaneous tissue
Other viscera
Pleuroperitoneal cavity
Mastoid emissary vein: suboccipital abscess
Griesinger’s sign:
oedema of the postauricular soft tissues overlying the
mastoid process as a result of thrombosis of the
mastoid emissary vein
61
 Investigations :








Hemogram
Blood cultures
Lumbar puncture :
 Low WBC count
 Normal CSF pressure
Queckstedt or Tobey-Ayer test
CT scan:
 An empty triangle at the level of the sigmoid sinus, clot
surrounded by a high-intensity rim of contrast-enhanced
dura: delta sign
Angiography
Venography
MRI
62
•The jugular
vein on the
side of the
suspected
thrombosis is
compressed
• A rise in
spinal fluid
pressure
should occur
•Its absence
indicates
presence of
thrombosis.
63
64
65
 Treatment :
 Administration of
antibiotics
 Exposure of the sinus:
incision and removal of
contents

Perisinus cells need to
be cleared: chances of
delayed sinus
thrombosis if not
cleared

???? Anticoagulants :


If in spite of the
treatment the infection
is progressively
involving the cortical
veins
Role of IJV ligation:

if the thrombus is
getting dislodged then
the need to ligate IJV
66



The commonest intracranial complication
Childhood otogenic meningitis is seen most often as a
complication of acute middle ear infection
In adults, it is now more commonly a complication of
chronic disease
67
Pathways of spread:
Bone erosion
Suppurative labrynthitis
Rupture of established brain abscess

Organisms responsible
for acute infection:


Hemophilus influenzae
type B
Streptococcus
pneumoniae type III

Organisms responsible
in chronic infection:




G-ve enteric organisms
Proteus
Pseudomonas
Anaerobes such as
Bacteroides species
68
 Clinical features:






Headache and neck stiffness
Malaise
Pyrexia mental hyperactivity
Exaggerated Tendon reflexes
Photophobia
Vomiting
69

Diagnisis :

Lumbar puncture
 Rise in fluid pressure above the normal 100-150 mm Hg
 Gross appearance of fluid: cloudy and then turbid
 On histological inspection PMN cells ranging from 0.1-10x109 /l
 Biochemical tests:





Bacteriological examination : gram staining and then culture
D/d :





Protein content may rise from normal of 150-400mg/l to a raised level of 2-3g/l
Chloride content may fall from the normal 120mmol/l to 80mmol/l
Fall in glucose level from the normal value of 1.7-3.0 mmol/l to zero
Rupture of brain abscess
Rupture of subdural abscess
CT scan
MRI
PCR
70
 Treatment :

Medical :





Repeated LP to lower the raised pressure
Large doses of systemic antibiotics
Systemic antibiotics must continue for 10 days after
apparent clinical recovery
Dexamethasone??
Surgical :

Certainly deterioration or failure of response over 48hrs
implies loculated infection in the mastoid, needing surgical
drainage
71
72
 Failure of an adequate response to antimicrobial
therapy may be a result of :



Organism resistant to chosen antibiotics
Persistent leakage of infected material into the CSF
Persistence of a previously unidentified other
complication/Leakage into the CSF from an unrecognized
brain abscess.
73

It is a focal suppurative process within the brain
parenchyma serrounded by a region of encephalitis.



Bimodal age of distribution with peaks in the paediatric age
group and in the 4th decade of life.
Almost always develop in the temporal lobe or the
cerebellum
Temporal lobe abscess is twice as common as
cerebellum
74

Various routes of spread:




Through an osteitic tegmen tympani , with middle fossa
extradural abscess
Local pachymeningitis followed by thrombophlebitis
Extension of infection along periarteriolar Virchow-Robin
spaces
Cerebellar abscess are frequently preceded by lateral sinus
thrombophlebitis
75
76

Bacteriology :polymicrobial (also extradural)



Anaerobes
Streptococcus & staphylococcus
G- organism:
 Escherichia coli
 Proteus
 Klebsiella
 Pseudomonas
77

Clinical presentation:




Very toxic and drowsy
Brain abscess is associated with the triad of:
 Headache
 High grade fever
 Focal neurological deficits
Cerebellar abscess:
 Dizziness
 Ataxia
 Nystagmus
 Vomiting
Temporal lobe lesion:
 Seizure
 Visual field defects
 Nominal aphasia
78
79
 Diagnosis :

Imaging :


Computed tomography
 Hypodense area by an area of edema, ring sign
MRI: superior to CT scan except for delineation of temporal
bone is poor.
80
81
82
 Other d/d:





Meningitis
Subdural abscess
Lateral sinus thrombophlebitis
Otitic hydrocephalus
Other masses such as brain tumour.
83
 Management :


High-dose antimicrobial medication
Patient should be first stabilized neurologically:



? Aspiration with high dose of antibiotics
?Total excision
?parenteral antibiotics
84
 Syndrome
(pseudotumour cerebrii) associated
with otitis media with





Increased intracranial pressure
Normal CSF findings
Spontaneous recovery
No abscess
No associated ventricular dilation: benign raised
intracranial tension.
85
 Pathophysiology:

Symonds: decreased absorption of CSF secondary to
blockage of arachnoid villi


Sahs and Joynt: secondary to brain edema
Weed and Flexner: disruption in venous circulation as a
cause .
86
87

Symptoms :






Headache
Drowsiness
Vomiting
Blurring of vision
Diplopia
Signs:



Papilledema
6th cranial nerve palsy
Optic disc atrophy
88
 Diagnosis :



↑CSF pressure
Normal CSF biochemistry
MRI is the modality of choice
 Management:



Lowering of the elevated intracranial pressure
Medical therapy:
 Corticosteroids
 Mannitol
 Diuretics
 Acetazolamide
Eradication of ear disease
89





In CSOM ostietis and subsequent bone erosion likely expose the
nerve to infection
This results in inflammation and ultimately facial nerve
compression
The tympanic segment is the most common area of involvement
HRCT of the temporal bone
Management :
 Intravenous antibiotics
 Prompt surgical intervention:
 Any attached granulation to the nerve should not be
removed
 Healthy bone should be removed from the nerve on
either side of the diseased segment.
 Concomitant corticosteroids
90

It has two clinically distinguishable subtypes:
 Localised, circumscribed, or serous labyrinthitis without total
or permanent loss of function
 Diffuse, purulent or suppurative labyrinthitis with permanent
total destruction of the sensory elements within the labyrinth
91


As the result of osteitis of the labyrinth capsule or of extension
along preformed pathways
Potential pathway include:
 oval window
 Round window membrane
 Cholesteatomatous fistula
92
 Clinical features:


Symptoms




Vestibular symptoms precede cochlear symptoms by hours
to days when the site of invasion is semicircular canal
Profound vertigo
Nausea
Vomiting
Signs:




Spontaneous nystagmus towards the unaffected ear.
Some degree of ataxia with pastpointing
High frequency sensorineural hearing loss
Distortion of hearing.
93
 Treatment :

Parenteral antibiotics
94





Usually following the serous labyrinthitis
Involvement of labyrinth secondary to generalized meningitis
Symptoms: same as serous labyrinthitis including the lack of
fever
Key differences:
 Symptoms are more rapid and intense
 The onset of severe vestibular symptoms is accompanied
by a complete loss of cochleovestibular response
 A caloric response is conspicuously absent from the disease
ear.
d/d: cerebellar abscess
95
 Treatment:







Close and continuous monitoring for symptoms of intracranial extension
Bed rest with minimal head movements
Antiemetics
Antibiotics
If meningeal signs are noted: LP
Any signs of intracranial spread: labyrinthectomy
Premature surgical trauma can promote dissemination of
infection: mastoid exploration to be deferred until acute
symptoms subside.
96
Bone erosion by cholesteatoma
 Most common site: dome of the lateral semicircular canal.
 Symptoms :



Episodic vertigo
Signs:
Fistula test
Negative test does not rule out fistula
 Tullio’s phenomenon


Site of fistula:





The dome of the LSCC: deviation of eyes towards normal ear.
LSCC fistula anterior to the ampulla: deviation towards the affected ear.
Erosion of the vestibule: rotatory horizontal deviation towards the diseased
ear.
SSCC: rotatory movements towards the normal ear
PSCC: vertical deviation of the eyes.
97
 Investigations:

HRCT temporal bone
 Treatment:

Surgery :
 Managed according to the site, size and status of hearing
 The rate of total hearing loss is 8% to 56%
98


It is the infected petrosal cells with inadequate drainage causing
bone changes of coalescence in the cell walls and resulting in
symptoms referable to the petrosa.
Symptoms:
 Gradneigo’s syndrome:
 1.Deep-boring pain: depending upon the site of involvement:
 Posterior group of cells:



Anterior petrositis:




occipital, parietal, or temporal
Discharge is from the mastoid
Frontal or behind the eye
Drainage is from the tympanum
2.Aural drainage
3,Diplopia : 6th cranial nerve palsy occurs when the apex is
involved
99


Investigations:
 HRCT temporal bone
 Gallium 67 scan
 Technetium 99m scan
Management :
 Parenteral antibiotics
 Surgical drainage
100

Can occur with:


Long standing perforation
Cholesteatoma
Once any type of mastoiditis causes continuous purulent
drainage for 8 or more weeks, the likelihood of complete
resolution with antibiotics significantly decreases.
 Hallmark triad:





Otalgia
Postauricular pain
Fever
Signs:




Postauricular tenderness
Protrusion of pinna
Postauricular erythema
Induration over the mastoid: impending subperiosteal abscess.
101
 Investigaton :

HRCT temporal bone
 Treatment :


Parenteral antibiotics
Mastoidectomy:


Significant bony destruction
Poor response to up to 2 weeks of conservative
management.
102




Direct destruction of cortical bone /
hematogenous spread through small vascular
channels.
Well pneumatized mastoids are more
susceptible
The most common site of cortex breakdown is
through the thin trabecular bone Macewen’s
triangle
Signs and symptoms:




Auricle is displaced forward and outward
Fluctuant mass can be palpated behind the
ear
Bezold’s abscess
Luc’s abscess
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 Investigations: HRCT temporal bone
 Management:


Simple mastoidectomy
Antibiotics
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 Benzold abscess:






Breach in the bony plate forming the inner surface of
mastoid tip.
Erosion of inner or outer cortex of the mastoid if
periphlebitis or phlebitis propagate the infection.
Pus track below the sternocleidomastoid or even the
layer of cervical fascia.
d/d : inflamed lymph nodes
Investigation : CT scan
Treatment :



Complete excision of mastoid pathology
Drainage of the abscess
Removal of associated granulation tissue.
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