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cholesteatoma ธิดา เหล่าไพบูลย์ อาจารย์ที่ปรึ กษา อ.สุ ธี ไกรตระกูล 24/05/60 1 cholesteatoma • Aural cholesteatomas are epidermal inclusion cysts of the middle ear or mastoid • They contain the desquamated debris (principally keratin) from their keratinizing, squamous epithelial lining. 24/05/60 2 Misnomer ? • "cholesteatoma" coined by the German physiologist Johannes Müller in 1838, is a misnomer • because this entity does not contain cholesterol • the white-yellow keratin flakes found within cholesteatomas grossly resemble cholesterol crystals 24/05/60 3 Congenital cholesteatomas PATHOGENESIS • originate : keratinizing epithelium [epidermoid cysts ] within the middle ear cleft, – "skin in the wrong place “ – pearl-like masses behind an intact membrane 24/05/60 4 Congenital cholesteatomas • Most involve the anterior superior quadrant of the middle ear • DDX : myringosclerosis by pneumatic otoscopy 24/05/60 5 Congenital cholesteatomas • Potsic et al. staging system: – stage I : one quadrant – stage II : multiple quadrants without ossicular involvement – stage III, ossicular involvement without mastoid extension – stage IV, mastoid involvement – correlation between stage and risk of residual disease; stage IV carries a 67% risk of residual cholesteatoma 24/05/60 6 MANAGEMENT Congenital cholesteatomas • prompt surgical excision • high-resolution CT scanning of the temporal bone : – extension into the attic and mastoid – Children with extensive disease : second-stage 6 to 12 months after the initial surgery • look for regrowth of microscopic residual disease • reconstruct the ossicular chain. – long-term follow-up : recurrent disease. 24/05/60 7 acquired cholesteatoma 24/05/60 8 The pathogenesis of acquired cholesteatoma There are four basic theories : (1) invagination of the tympanic membrane (retraction pocket cholesteatoma) (2) basal cell hyperplasia (3) epithelial ingrowth through a perforation (the migration theory) (4)squamous metaplasia of middle ear epithelium 24/05/60 9 Invagination of the tympanic membrane • The invagination : attic cholesteatomas • eustachian tube dysfunction (or OME) with resultant negative middle ear pressure • repeated inflammation • The pars flaccida, being less fibrous and less resistant to displacement 24/05/60 10 invagination of the tympanic membrane • retraction pocket deepens • Bacteria can infect the keratin matrix biofilm • primary acquired cholesteatoma results • in the posterosuperior quadrant 24/05/60 11 2. The epithelial invasion Theory • perforation in the tympanic membrane • keratinizing squamous epithelium migrates into the middle ear from • "contact inhibition." • concept of increased migration of the epithelium in those with cholesteatoma 24/05/60 12 2. The epithelial invasion Theory • Cholesteatomas originating after temporal bone fractures • allow ingrowth of keratinizing epithelium by contact guidance • Dimethylbenzanthracene (DMBA) – chemical carcinogen – advancing of the keratinizing squamous epithelium into or under the mucosal layer middle ear cavity and eustachian tube 24/05/60 13 3.Basal Cell Hyperplasia Theory • epithelial cells (prickle cells) of the pars flaccida • invade – subepithelial tissue – lamina propria – basement membrane 24/05/60 14 3.Basal Cell Hyperplasia Theory • formation of microcholesteatomas • perforate secondarily through the tympanic membrane • an attic cholesteatoma. 24/05/60 15 3.Basal Cell Hyperplasia Theory • Substance in cholesteatoma matrix – cytokeratin (CK) 10 ,13 16 – fibronectin and tenascin – Action • Hyperproliferation • Migration • altered differentiation of keratinocytes 24/05/60 16 4. Squamous Metaplasia Theory • inflammation • simple squamous or cuboidal epithelium of the middle ear cleft • metaplastic transformation into keratinizing epithelium. • and contact with the tympanic membrane. 24/05/60 17 4. Squamous Metaplasia Theory • infection and inflammation, • lysis of the tympanic membrane and perforation • typical appearance of an attic cholesteatoma. 24/05/60 18 Diagnosis acquired cholesteatoma • otoscopic examination – typical attic retraction : posterosuperior portion of tympanic membrane • surgical exploration 24/05/60 19 Diagnosis acquired cholesteatoma • High-resolution CT scanning temporal bone – is useful for operative planning – recommended for all revision mastoid operations • MRI temporal bone 24/05/60 20 symptoms of acquire cholesteatoma • Asymptomatic • slowly progressive conductive hearing loss • chronic otitis with purulent otorrhea : most 24/05/60 21 acquired cholesteatoma • malodorous otorrhea : anaerobic bacteria • misdiagnosed : external otitis careful follow-up canal débridement of a patient with otorrhea 24/05/60 22 Middle ear atelectasis • longstanding eustachian tube dysfunction • Negative pressure in middle ear • atelectasis develops • tympanic membrane becomes retracted onto the promontory and the ossicles of the middle ear • tympanic membrane is not adherent to the medial wall of the middle ear • mucosal lining of the middle ear is intact. 24/05/60 23 DDX acquired cholesteatoma • adhesive otitis media – middle ear space is totally obliterated – tympanic membrane is adherent to the ossicles and promontory – mucosal surfaces are not present – erosion of the long process of the incus and the stapes suprastructure 24/05/60 24 four stages of tympanic membrane retraction stage I, retracted tympanic membrane stage II, retraction with contact onto the incus 24/05/60 25 four stages of tympanic membrane retraction stage III, middle ear atelectasis stage IV, adhesive otitis media 24/05/60 26 Management middle ear atelectasis • ventilating tubes • Myringoplasty – hearing loss – ossicular erosion 24/05/60 27 secondary acquired cholesteatoma • keratinizing epithelium has migrated through a perforation into the middle ear 24/05/60 28 secondary acquired cholesteatoma "aural polyp." • An infected cholesteatoma • granulation tissue at the junction between an eroding cholesteatoma and bone. • The presence of an aural polyp in a chronically infected ear should be considered to be a cholesteatoma until proven otherwise. • cholesteatoma cannot be seen otoscopically but will be discovered during tympanomastoid surgery. 24/05/60 29 secondary acquired cholesteatoma • Cholesteatomas are prone to – recurrent infection – erode the bone of the ossicles and the otic capsule. – exhibit typical growth patterns into the temporal bone. • The pars flaccida may invaginate into – Lateral most portion of the epitympanum (Prussak's space) – recesses of the epitympanum posteriorly, lateral to the body of the incus, inferiorly into the middle ear by way of the pouch of von Tröltsch – anteriorly into the protympanum 24/05/60 30 Prussak space 24/05/60 31 • Prussak’s space connected to Posterior Pouch von Troeltsch which opens up into Mesotympanum 24/05/60 32 The Prussak’s space connects • anterior pouch von Troeltsch and the mesotympanum (Anterior Tympanic Space) • Posterior Pouch Von Troeltsch connections to the Posterior 24/05/60Tympanic Space. 33 Posterior mesotympanic cholesteatoma • retraction of the posterior portion of pars tensa • invades – sinus tympani – facial recess – Extension to mastoid occurs medial to ossicle heads 24/05/60 34 Anterior epitympanic cholesteatoma • Invagination of epitympanum anterior to malleus head and neck creates a cholesteatoma sac • threatens horizontal facial nerve and geniculate ganglion. • Forward extension into supratubal recess is common 24/05/60 35 BACTERIOLOGY OF INFECTED CHOLESTEATOMAS 24/05/60 36 Complications • bone erosion – Ossicles : most incus – otic capsule : most lateral semicircular canal – facial nerve canal – tegmen tympani – tegmen mastoideum 24/05/60 37 Complications – Sensorineural hearing loss : • secondary suppurative labyrinthitis • cochlear hair cell loss adjacent to cholesteatoma 24/05/60 38 Extratemporal (extracranial) complications Subperiosteal abscess • Cholesteatoma can block the aditus ad antrum • preventing communication of the infected contents of the mastoid with the middle ear space and the eustachian tube • This obstruction increases the possibility of infectious decompression through the mastoid cortex. 24/05/60 39 Subperiosteal abscess • systemic symptoms – fever and malaise • local signs – protruding auricle that is laterally and inferiorly displaced – fluctuant, erythematous, tender area behind the ear. • CT scan can demonstrate abscess – cortical defect in the mastoid 24/05/60 40 Subperiosteal abscess • abscess is drained • mastoidectomy is performed and the cholesteatoma matrix is removed in the standard manner. 24/05/60 41 Bezold's abscess • cervical abscess deep to the sternocleidomastoid • mastoid cortex is violated at its tip, as opposed to its lateral cortex • Partway : – direct extension – mastoid vein phlebitis • more common AOM with mastoiditis • complication of COM with cholesteatoma 24/05/60 42 Bezold's abscess • A contrasted CT scan of the neck and mastoid : – rim-enhancing abscess with surrounding inflammation – bony dehiscence in the tip of the mastoid, • Management : – drainage of the cervical abscess through a transcervical approach – combined with a cortical mastoidectomy 24/05/60 43 Labyrinthine fistulae • most common complications of chronic otitis with associated cholesteatoma • The risk of significant sensorineural hearing loss as a result of surgical manipulation makes the open labyrinth – pressure from the cholesteatoma 24/05/60 44 Labyrinthine fistulae • horizontal semicircular canal is the most common • Erosion of the bone of the otic capsule – activated mediators from the matrix 24/05/60 45 Diagnosis Labyrinthine fistulae • subjective vertigo : 62% to 64% of patients • positive fistula test on examination : 32% to 50% of patients • sensorineural hearing loss is found in most of these patients (68%), • this classic picture is not sensitive in the preoperative identification of a fistula 24/05/60 46 Labyrinthine fistulae • the ability to detect fistulae accurately on preoperative CT has been reported as 57% to 60% • in current reports CT scans are no more sensitive than history and physical examination in detecting labyrinthine fistulae. • The definitive diagnosis : intraoperatively 24/05/60 fistula (arrow) of lateral semicircular canal from cholesteatoma 47 Management Labyrinthine fistulae A tympanomastoidectomy • the most appropriate approach to the fistula is to perform a canal wall down mastoidectomy • remove the bulk of the cholesteatoma, and leave the fistula covered with the matrix exteriorizing it into the cavity • then re-operation 9-12 months after first operation 24/05/60 48 Labyrinthine fistulae • In small fistula (<2mm) 2 , cholesteatoma matrix gently removed from fistula +repaired with soft tissue graft (eg, fascia, Intraoperativepicture perichondrium), bone of a horizontal canal fistula pate 24/05/60 49 Facial paralysis • usually occurs with a dehiscent fallopian canal within its tympanic segment, • allowing direct contact of inflammatory mediators • dehiscent nerve, • bony erosion. slowly progressive facial paralysis Diagnosis 24/05/60 50 Facial paralysis • The diagnosis facial paralysis – COM, or cholesteatoma – CT scan is not required, it can be useful in therapeutic planning and patient counseling – cholesteatoma involves the fallopian canal ass. • labyrinth • tegmen 24/05/60 51 Facial paralysis • Coronal CT scan demonstrating involvement of tympanic facial nerve (arrow) from cholesteatoma (arrow heads) causing lateral semicircular canal fistula 24/05/60 52 Management • mastoidectomy is performed to remove the cholesteatoma or granulation tissue that is contacting the facial nerve. • The nerve is approached on both sides of the involvement • diamond burr is used to expose the epineurium on either side of the diseased segment. • Once the proximal and distal segments of the fallopian canal have been opened • blunt dissection is used to remove the disease from the epineurium. 24/05/60 53 24/05/60 54 24/05/60 55 Congenital cholesteatoma of the temporal bone ? • divided into four anatomic groups: 1. middle ear DDX congenital auditory canal cholesteatomas 2. perigeniculate area 3. petrous apex 4. cerebellopontine angle. 24/05/60 56 Perigeniculate and petrous apex cholesteatomas • present – insidious or rapidly progressive facial nerve paralysis – Sensorineural hearing loss from labyrinthine or internal auditory canal erosion is common – conductive hearing loss also may result from cholesteatoma extension into the middle ear or blockage of the eustachian tube. 24/05/60 57 Perigeniculate and petrous apex cholesteatomas – Facial twitching may occur in the presence of congenital cholesteatomas and with facial nerve neuromas. – Vestibular dysfunction may complete the symptom complex. – the cholesteatoma may erode into the middle or posterior fossa and expand markedly before producing symptoms 24/05/60 58 Perigeniculate and petrous apex cholesteatomas • left cerebellopontine cistern congenital epidermoid cyst • A, Axial computed tomography (CT). Displacement of the pons DDX :arachnoid cyst • B, Axial magnetic resonance imaging T1-weighted, no contrast. – This slightly higher signal on T1-weighted images is helpful in separating epidermoid cysts from arachnoid cysts.. 24/05/60 59 Perigeniculate and petrous apex cholesteatomas • MRI will provide further definition – congenital cholesteatomas • almost always slightly higher signal T1-weighted images • moderately high signal on T2-weighted images – bone marrow fat • high signal on T1-weighted images • fade dramatically on T2-weighted images. – Typical effusions • low signal on T1-weighted MRI • high signal on T2-weighted MRI. – Giant cholesterol cysts :show capsular enhancement. 24/05/60 60 Surgical management of a congenital cholesteatoma • complete removal of the cholesteatoma matrix • The isolated middle ear cholesteatoma – removed transtympanically – Routine middle ear reconstructive techniques may be used if ossicles are eroded or removed or if the tympanic membrane is sacrificed. 24/05/60 61 Perigeniculate and petrous apex cholesteatomas • congenital cholesteatomas of the perigeniculate area or petrous apex – Transmastoid – middle cranial fossa approach – the transsphenoidal approach – combination of these procedures 24/05/60 62 Cholesteatoma vs. Temporal bone fracture There are four pathogenic mechanisms for posttraumatic cholesteatoma formation: (1) epithelial entrapment in the fracture line (2) ingrowth of epithelium through the unhealed fracture line or a rent in the tympanic membrane (3) traumatic implantation of tympanic membrane skin into the middle ear (4) trapping of epithelium medial to a stenosis of the EAC. 24/05/60 63 posttraumatic cholesteatoma formation • The typical location : epitympanum and the antrum. The fracture line along the posterior superior canal wall and scutum expands and then closes • Traumatic implantation of tympanic membrane skin will result in cholesteatoma formation within the mesotympanum. • Blast injuries can result in the displacement of keratinizing stratified squamous epithelium into the mastoid air cells, the mesotympanum, the epitympanum, and even intracranially 24/05/60 64 Management posttraumatic cholesteatoma formation • careful follow-up – Débridement – stenting when narrowing progresses : ear mold – canal is adequately dilated : Oto-Wicks (Meditronic Xomed, Jacksonville, Fl) 24/05/60 65 Management posttraumatic cholesteatoma formation • canalplasty – Complete stenosis – dilation is not possible 24/05/60 66 24/05/60 67