Complications of Cholesteatoma Dr Supreet Singh Nayyar AFMC

Overview �Cholesteatoma –A Historical perspective �Cholesteatoma �Origin of cholesteatoma �Pathophysiology of cholesteatoma �Intracranial complications

Bewildering History � 1683 - Durverney , first described abscess of bone originating form

History contd. . � 1855 – Virchow classified cholesteatoma among squamous cell carcinoma and

�Haberman & Bezold (1889)-proved that cholesteatoma arises from skin of EAC under influence of

Cholesteatoma is a three dimensional epidermal and connective tissue structure, usually in the form

Epithelial invagination 7 www. nayyar. ENT. com 18/07/2012

Structure of cholesteatoma Perimatrix Matrix Keratin (dry or active bacterial infection 8 www. nayyar.

Pathophysiology of cholesteatoma �Pressure induced bone resorption �Enzymatic disollution of bone by cytokine mediated

Pathology of complications of cholesteatoma Direct erosion of the bone Round window , oval

Relative incidence of Complications in mucosal and squamosal COM* Extra cranial complications Intracranial complications

Intracranial complications � Meningitis Temporal lobe abscess � Brain abscess � � 1 2

Although it is thought that majority of intracranial complications are due to squamous disease

Extradural abscess �Anatomically Dura is a very tough structure � when the disease reaches

�Large abscesses compress squamous part of temporal bone causing ostietic erosion and comes out

Clinical features �Extradural abscess has no specific features �Headache �Deep seated boring pain �Malaise

Treatment �Broad spectrum Antibiotics �Surgical evacuation of pus by removing underlying osteitic bone 1

Posterior cranial fossa 1 9 www. nayyar. ENT. com Perisinus abscess Lateral Sinus Thrombophlebi

Lateral sinus Thrombophlebitis � 50% of all the cases have concomitant Conditions like cerebellar

Clinical Features �Classical presentation “Picket fence fever” �Fever- sweating- symptom free period- again fever

contd… �Greisengers sign: pitting edema in post aural region due to thrombosis of mastoid

Investigations �Complete blood count �Falling Hb values �Polymorphonuclear leucocytosis �HRCT temporal � bone �CECT

Management Medical a) IV Antibiotics � b) Anticoagulants not recommended routinely �coagulation favorable to

�Surgical �Undertaken early to expose and treat infected lesion Timing of Mastoidectomy depends on

Brain abscess �Commonest intra cranial complication �Cerebellar abscess �Temporal lobe abscess �Cerebellar abscess is

Temporal lobe abscess Extra Dural abscess Localized encephalitis Septic thrombosis of pial veins (ASOM)

Cerebellar abscess � Mastoiditis Peri sinus abscess Cerebellar Abscess labyrinthitis 2 8 www. nayyar.

Clinical features �Increased intracranial tension �Focal signs �Systemic disturbances 2 9 www. nayyar. ENT.

�Increased intracranial tension Vomiting Drowsiness Confusion lethargy Papillodema - long standing abscess 3 0

�Focal signs Temporal lobe –visual field defect (homonymous hemianopia) -Aphasia (nominal aphasia) if dominant

�Focal signs – Cerebellar abscess – Truncal and limb ataxia - Cerebellar signs -Rhombergs

Systemic features �Fever. - In cases of Temporal lobe abscess the temperature may remain

�CT Scan �MRI �EEG �Arteriography �Pus culture and sensitivity from offending ear 3 4

Treatment �Emergency in case pt deteriorating fast due to raised ICT- inj hydrocortisone 2

D/D �Circumscribed serous meningitis may mimic clinical and radiological features of Cerebellar Abscess �Formed

Meningitis �Infection reaching pia arachnoid by routes already described , commonly �Serous meningitis �Purulent

Clinical features �Headache �Neck Stiffness �Fever –initial rigors , fever settles down to continuous

Investigations �Hematological investigations �CSF examination �MRI / CECT 3 9 www. nayyar. ENT. com

Treatment �Parentral antibiotic therapy- penicillin still drug of choice, 2 -4 megaunits 6 hourly

Subdural abscess 4 1 Collection of pus between Dura & Arachnoid Manifests as leptomeningitis,

Clinical presentation Clinical features Headache, fever, drowsiness Focal neurological symptoms a) irritative epilepsy b)

� Investigations : CT scan MRI � Management. Evacuation of abscess by burr hole

Otitic hydrocephalus �Benign intra cranial hypertension �Frequently affects children and adolescents �Obscure etiology; possibly

Treatment �Repeated Lumbar puncture at 48 hr intervals �Medical –diuretics / Acetazolamide �Long standing

Conclusion � Intracranial complications of COM have drastically reduced however mortality from complication remains

References �Scott- Browns ; Otolaryngology , Head & Neck 4 7 Surgery; Seventh Edition

www. nayyar. ENT. com 4 8 www. nayyar. ENT. com 18/07/2012

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Complications of Cholesteatoma Dr. Supreet Singh Nayyar, AFMC For more presentations, visit www. nayyar. ENT. com 18/07/2012

Overview �Cholesteatoma –A Historical perspective �Cholesteatoma �Origin of cholesteatoma �Pathophysiology of cholesteatoma �Intracranial complications of cholesteatoma �Conclusion 2 www. nayyar. ENT. com 18/07/2012

Bewildering History � 1683 - Durverney , first described abscess of bone originating form external auditory canal , described it as. scales � 1892 -Cruveilhier described it as avascular tumor arising from subarachnoid space � 1838 - Muller coined the term cholesteatoma as he demostrated presence of chloestrin and fat in the lesion. (despite this misnomer its still used today. ) 3 www. nayyar. ENT. com 18/07/2012

History contd. . � 1855 – Virchow classified cholesteatoma among squamous cell carcinoma and atheromas, postulated that cholesteatoma arise from mesenchymal cells � 1873 - Von Troeltch was first to consider the epidermal origin of the disease, epidermal debri originating in EAC lead to pressure on TM – pressure induced necrosis of bone � 1889 - Gruber , Wendt & Rokitanski considered that middle ear mucosa undergoes metaplasia in response to chronic inflammation 4 www. nayyar. ENT. com 18/07/2012

�Haberman & Bezold (1889)-proved that cholesteatoma arises from skin of EAC under influence of chronic middle ear inflammation 5 www. nayyar. ENT. com 18/07/2012

Cholesteatoma is a three dimensional epidermal and connective tissue structure, usually in the form of a sac and frequently conforming to the architecture of the various spaces of the middle ear, attic, and mastoid. This structure has the capacity for progressive and independent growth at the expense of underlying bone, displacing or replacing the middle ear mucosa, and has a tendency to recur after removal - Abramson ) (Cholesteatoma – First International Conference, Birmingham, 1977 Birmingh 6 www. nayyar. ENT. com 18/07/2012

Epithelial invagination 7 www. nayyar. ENT. com 18/07/2012

Structure of cholesteatoma Perimatrix Matrix Keratin (dry or active bacterial infection 8 www. nayyar. ENT. com 18/07/2012

Pathophysiology of cholesteatoma �Pressure induced bone resorption �Enzymatic disollution of bone by cytokine mediated inflammation -Enzyme – matrix metalloproteinases (MMP) - MMP 2, MMP 9 cytokines IL-1 a. IL-1 b, TNF a, TGF a, EGF. 9 www. nayyar. ENT. com 18/07/2012

Pathology of complications of cholesteatoma Direct erosion of the bone Round window , oval window Natural communications Progressive thrombophlebitis of small venules Along periarteriolar spaces of Virchow. Robin 1 0 www. nayyar. ENT. com Routes of spread of the disease Vascular channels Most common pathway by which disease extends beyond middle ear cleft Abnorma l preforme d pathway s Congenital: -aberrant arachnoid granulations -Meningo encephaloceles Acquired -temporal bone # involving otic capsule -Surgically created defects 18/07/2012

Relative incidence of Complications in mucosal and squamosal COM* Extra cranial complications Intracranial complications % Postauricular abscess 75 Meningitis Facial palsy 6 Brain abscess 52 Bezolds abscess 2 Subdural abscess 20 Extra Dural abscess 10 Lateral sinus thrombosis 20 Petrous apicitis 1 1 % 0. 2 12 * Scott- Brown’s Otolaryngology , Head and Neck Surgery : seventh edition. pg 3435 www. nayyar. ENT. com 18/07/2012

Intracranial complications � Meningitis Temporal lobe abscess � Brain abscess � � 1 2 Cerebellar abscess Lateral sinus thrombophlebitis Extradural abscess Subdural abscess Otitic hydrocephalus www. nayyar. ENT. com 18/07/2012

Although it is thought that majority of intracranial complications are due to squamous disease as compared to mucosal disease it is not the case. 1 3 Squamous(%) Mucosal(%) Intracranial 59 41 Extra cranial 41 59 overall 58 42 *Scott- Browns : Otolaryngology, Head & Neck Surgery ; Seventh edition pg 3436 www. nayyar. ENT. com 18/07/2012

Extradural abscess �Anatomically Dura is a very tough structure � when the disease reaches Dura Pachymeningitis results �Dura lightly attached Lateral to arcuate Eminence - large abscess Medial to its attachment - small abscess 1 4 www. nayyar. ENT. com 18/07/2012

�Large abscesses compress squamous part of temporal bone causing ostietic erosion and comes out as sub periosteal abscess – Potts puffy tumour Extra Dural abscess more common in posterior cranial fossa compared to middle fossa 1 5 www. nayyar. ENT. com 18/07/2012

Clinical features �Extradural abscess has no specific features �Headache �Deep seated boring pain �Malaise �Tenderness on tapping temporal region �Communication with EAC pus discharge –relief following discharge 1 6 www. nayyar. ENT. com 18/07/2012

Investigations �Routine hematological profile �Pus culture �CT scan 1 7 www. nayyar. ENT. com 18/07/2012

Treatment �Broad spectrum Antibiotics �Surgical evacuation of pus by removing underlying osteitic bone 1 8 www. nayyar. ENT. com 18/07/2012

Posterior cranial fossa 1 9 www. nayyar. ENT. com Perisinus abscess Lateral Sinus Thrombophlebi tis 18/07/2012

Lateral sinus Thrombophlebitis � 50% of all the cases have concomitant Conditions like cerebellar abscess or meningitis Peri sinus abscess Acute mastoiditis Lateral sinus thrombosis Internal jugular vein(common) Cavernous sinus (rare) Superior petrosal sinus(rare) Pyaemia Pyaemic abscess lung Subdural abscess Brain abscess 2 0 www. nayyar. ENT. com 18/07/2012

Clinical Features �Classical presentation “Picket fence fever” �Fever- sweating- symptom free period- again fever �Chills �Rigors (temp reaching up to 40 degree Celsius) �Vomiting �Dehydration �Tenderness along IJV �*However in present day and age classical 2 1 presentation is seldom seen due to advent of broad spectrum antibiotics www. nayyar. ENT. com 18/07/2012

contd… �Greisengers sign: pitting edema in post aural region due to thrombosis of mastoid emissary vein �Rise in CSF pressure as demonstrated by �Queckenstedts test � Tobey Ayer test. � Lillie Crowe test 2 2 www. nayyar. ENT. com 18/07/2012

Investigations �Complete blood count �Falling Hb values �Polymorphonuclear leucocytosis �HRCT temporal � bone �CECT – filling defect 2 3 www. nayyar. ENT. com 18/07/2012

Management Medical a) IV Antibiotics � b) Anticoagulants not recommended routinely �coagulation favorable to prevent bacterimia �thrombosis generally not too much extensive as anticipated 2 4 www. nayyar. ENT. com 18/07/2012

�Surgical �Undertaken early to expose and treat infected lesion Timing of Mastoidectomy depends on the response to medical treatment Internal jugular vein ligation-Doubtful - thrombosis already spread beyond elective site - vein difficult to expose amidst inflamed surrounding tissues 2 5 www. nayyar. ENT. com 18/07/2012

Brain abscess �Commonest intra cranial complication �Cerebellar abscess �Temporal lobe abscess �Cerebellar abscess is nearly always otogenic �Majority of brain abscess are associated with chronic otitis media although acute otitis media also accounts for significant number 2 6 www. nayyar. ENT. com 18/07/2012

Temporal lobe abscess Extra Dural abscess Localized encephalitis Septic thrombosis of pial veins (ASOM) Sub cortical white matter Perivascular Liquifactive necrosis 2 7 www. nayyar. ENT. com 18/07/2012

Cerebellar abscess � Mastoiditis Peri sinus abscess Cerebellar Abscess labyrinthitis 2 8 www. nayyar. ENT. com 18/07/2012

Clinical features �Increased intracranial tension �Focal signs �Systemic disturbances 2 9 www. nayyar. ENT. com 18/07/2012

�Increased intracranial tension Vomiting Drowsiness Confusion lethargy Papillodema - long standing abscess 3 0 www. nayyar. ENT. com 18/07/2012

�Focal signs Temporal lobe –visual field defect (homonymous hemianopia) -Aphasia (nominal aphasia) if dominant hemisphere is involved - Seizures 3 1 www. nayyar. ENT. com 18/07/2012

�Focal signs – Cerebellar abscess – Truncal and limb ataxia - Cerebellar signs -Rhombergs test positive 3 2 www. nayyar. ENT. com 18/07/2012

Systemic features �Fever. - In cases of Temporal lobe abscess the temperature may remain sub normal - High fever incase abscess raptures into ventricular system �Loss of appetite �malaise 33 www. nayyar. ENT. com 18/07/2012

�CT Scan �MRI �EEG �Arteriography �Pus culture and sensitivity from offending ear 3 4 www. nayyar. ENT. com 18/07/2012

Treatment �Emergency in case pt deteriorating fast due to raised ICT- inj hydrocortisone 2 -4 gms - 20% Mannitol �Antibiotics- essential to cross BBB �Definitive – Neurosurgical 3 5 CWD mastoidectomy (COM)/cortical mastoidectomy (AOM) www. nayyar. ENT. com 18/07/2012

D/D �Circumscribed serous meningitis may mimic clinical and radiological features of Cerebellar Abscess �Formed due to localized meningitis and cyst formation cyst in subarachnoid region 3 6 www. nayyar. ENT. com 18/07/2012

Meningitis �Infection reaching pia arachnoid by routes already described , commonly �Serous meningitis �Purulent meningitis 3 7 www. nayyar. ENT. com 18/07/2012

Clinical features �Headache �Neck Stiffness �Fever –initial rigors , fever settles down to continuous fever of 38 - 38. 5 degree Celsius �Positive Kernigs sign 3 8 www. nayyar. ENT. com 18/07/2012

Investigations �Hematological investigations �CSF examination �MRI / CECT 3 9 www. nayyar. ENT. com 18/07/2012

Treatment �Parentral antibiotic therapy- penicillin still drug of choice, 2 -4 megaunits 6 hourly �Intrathecal penicillin 10, 000 units � if initial CSF tap is turbid �When pt is stabilized can be taken up for Tympano-Mastoid exploration. 4 0 www. nayyar. ENT. com 18/07/2012

Subdural abscess 4 1 Collection of pus between Dura & Arachnoid Manifests as leptomeningitis, effusion or abscess Rate of spread determines the clinical & pathological pattern. Associated with other complications www. nayyar. ENT. com 18/07/2012

Clinical presentation Clinical features Headache, fever, drowsiness Focal neurological symptoms a) irritative epilepsy b) hemi paresis Papilloedema and cranial nerve palsies are uncommon 4 2 www. nayyar. ENT. com 18/07/2012

� Investigations : CT scan MRI � Management. Evacuation of abscess by burr hole / craniotomy � IV Antibiotics � Treatment of ear � Antiepileptic medication after recovery � 4 3 www. nayyar. ENT. com 18/07/2012

Otitic hydrocephalus �Benign intra cranial hypertension �Frequently affects children and adolescents �Obscure etiology; possibly sequale to bilateral sinus thrombosis �C/F – Intermittant headache Uni/bilateral papillodema CT scan to rule out other more serious complication 4 4 www. nayyar. ENT. com 18/07/2012

Treatment �Repeated Lumbar puncture at 48 hr intervals �Medical –diuretics / Acetazolamide �Long standing cases – surgical ventriculoperitonial shunt/subtemporal decompression 4 5 www. nayyar. ENT. com 18/07/2012

Conclusion � Intracranial complications of COM have drastically reduced however mortality from complication remains significantly high even today (8%). � The incidence of complications are nearly as common in mucosal disease as in squamous disease. � MRI remains gold standard for most of the intracranial complications � Broad spectrum antibiotic cover is to be started immediately as soon as diagnosis is established followed by specific antibiotic cover according to the culture and sensitivity � Minimum Ear surgery for a complicated COM AAD remains CWD mastoidectomy 4 6 www. nayyar. ENT. com 18/07/2012

References �Scott- Browns ; Otolaryngology , Head & Neck 4 7 Surgery; Seventh Edition �Scott –Browns ; Diseases of Ear Nose and throat; Fourth Edition �Mawson’s; Diseases of the Ear ; Fifth Edition �Logan Turners Diseases of the Nose Throat & Ear ; Tenth Edition �Otolaryngologic Clinics of North America Volume 39, Issue 6, (December 2006) �Various internet searches using Google , Google images www. nayyar. ENT. com 18/07/2012