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9/7/2013 1 Pediatric Cholesteatoma Melissa M. Raines, MSN, CPNP, APNP Pediatric Nurse Practitioner Department of Otolaryngology Medical College of Wisconsin/Children’s Hospital of Wisconsin SOHN Congress 2013 Vancouver, British Columbia, Canada Disclosures I have no relevant financial relationships or commercial interests to reveal in regards to this presentation. Objectives 1. Describe the pathogenesis of cholesteatomaformation 2. Identify the impact cholesteatomacan have on a child and their family 3. Define key points in the history, physical examination and diagnostic techniques used in diagnosis confirmation 4. Discuss surgical options and medical therapies for patients with cholesteatoma(s) 5. Examine the post-operative expectations for the patient with a cholesteatoma 6. Participate in a case study discussion of a pediatric patient diagnosed with a cholesteatoma What is a Cholesteatoma? Cyst-like expansilelesion(s) of the temporal bone lined by stratified squamous epithelium that contain desquamated keratin Most frequently involve the middle ear (ME) and mastoid but may develop anywhere in the pneumatizedportions of the temporal bone Physiology of Cholesteatoma The matrix contains fully differentiated squamous epithelium resting on connective tissue Deeper layers of the epithelium of a cholesteatomamatrix show activity in the form of downgrowthsinto the underlying connective tissue There is always a layer of granulation tissue in contact with bone The layer of granulation tissue elaborates various enzymes such as collagenaseresulting in bone destruction

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  • 9/7/2013

    1

    Pediatric Cholesteatoma

    Melissa M. Raines, MSN, CPNP, APNPPediatric Nurse Practitioner

    Department of Otolaryngology

    Medical College of Wisconsin/Childrens Hospital of Wisconsin

    SOHN Congress 2013

    Vancouver, British Columbia, Canada

    Disclosures

    I have no relevant financial relationships

    or commercial interests to reveal

    in regards to this presentation.

    Objectives

    1. Describe the pathogenesis of cholesteatoma formation

    2. Identify the impact cholesteatoma can have on a child and their family

    3. Define key points in the history, physical examination and diagnostic techniques used in diagnosis confirmation

    4. Discuss surgical options and medical therapies for patients with cholesteatoma(s)

    5. Examine the post-operative expectations for the patient with a cholesteatoma

    6. Participate in a case study discussion of a pediatric patient diagnosed with a cholesteatoma

    What is a Cholesteatoma?

    Cyst-like expansile lesion(s) of the temporal

    bone lined by stratified squamous epithelium

    that contain desquamated keratin

    Most frequently involve the middle ear (ME) and mastoid but may develop anywhere in the

    pneumatized portions of the temporal bone

    Physiology of Cholesteatoma

    The matrix contains fully differentiated squamousepithelium resting on connective tissue

    Deeper layers of the epithelium of a cholesteatoma matrix show activity in the form of downgrowths into the underlying connective tissue

    There is always a layer of granulation tissue in contact with bone

    The layer of granulation tissue elaborates various enzymes such as collagenase resulting in bone destruction

  • 9/7/2013

    2

    Histology History 1st description of cholesteatoma like mass by DuVerney in

    1683

    Misnomer originated by Johannes Mueller in 1838 Described it as a layered pearly tumor of fat, which was

    distinguished from other fat tumors by the biliary fat or cholesterin that is interspersed among the sheets of polyhedral cells

    Cholesteatomas do not contain fat and not usually cholesterin

    More appropriate term suggested by Schuknecht was keratoma

    In 1889, Habermann known for 1st temporal bone report describing the pathology of cholesteatoma

    (Bluestone et al, 1978), (Golz et al, 1999), (Al Anazy, 2006)

    Anatomy Review

    www.studyblue.com www.directhearingaids.co.uk

    www.kck.usm.my

    Anatomy -Middle Ear Regions

    Name based on position relative to superior

    and inferior aspect of external auditory canal

    (EAC)

    Epitympanum

    Mesotympanum

    Hypotympanum

  • 9/7/2013

    3

    Anatomy Middle Ear Regions Epitympanum

    Lies above short process of malleus; includes the head of the malleus, body of incus and associated ligaments and mucosal folds

    Mesotympanum Includes the stapes, long process of incus, handle of malleus and

    oval and round windows

    Eustachian tube exits from anterior aspect

    2 recesses extend posteriorly that are often not visible Facial recess Sinus tympani

    Hypotympanum Lies inferior & medial to floor of bony canal; irregular bony

    groove that is seldom involved by cholesteatoma www.kck.usm.my

    Cholesteatoma Classification

    AcquiredCongenital

    www.nejm.org , www.ganfyd.org

    Incidence

    The true incidence is not known

    Mean annual incidence of 9.2 cases per 100,000 persons of all ages (range 3.7 to 13.9) (Kemppainen, Puhakka, Laippala, Sipil & Karma, 1999)

    Approximately 5 to 15 per 100,000 in children alone (Bluestone & Klein, 2003)

    Congenital cholesteatomas account for 1 to 5 percent in most published series (Bennett, Warren, Jackson & Kaylie, 2006)

    Spilsbury, Miller, Semmens & Lannigan, 2010: 0.9% percent was seen in a retrospective series of 45,980

    children who had undergone 1 set of ventilation tubes

    Two or more ventilation tube placements increased rate of cholesteatoma to 21%

    Incidence In 172 cases of congenital cholesteatoma between 1981

    and 2000, the following observations were made: 72 percent of cases occurred in boys The average age was 5 y/o

    Nearly one-half of cases involved 2 quadrants; 82 percent of cases confined to one quadrant were in the anterosuperiorquadrant

    The ossicular chain and mastoid were involved in 43 percent and 23 percent of cases, respectively

    Cholesteatomas are bilateral in approximately 4 percent of cases

    Bilateral congenital cholesteatomas are more common in boys, whereas bilateral acquired cholesteatomas are more common in girls

    (Potsic, Korman , Samadi & Wetmore, 2002), (Lin, Daniel, James & Friedberg, 2004)

    Congenital Cholesteatoma

    Definition(s): Congenital rest of

    epithelial tissue in the ear without tympanic membrane perforation and without history of otitismedia (Derlacki & Clemis, 1965)

    Includes a normal pars flaccida and pars tensawith no history of otorrheaand no history of otologicprocedures (Levenson et al, 1989)

    Not excluded if there is a history of otitis media

  • 9/7/2013

    4

    Congenital Cholesteatoma

    White, cyst-like structure in the middle ear (ME)

    space or temporal bone

    Can occur anywhere in temporal bone

    Most common in the

    anterosuperior quadrant

    (AS) in the ME above the

    eustachian tube (ET)

    opening in the

    mesotympanum

    Congenital Cholesteatoma

    Pathogenesis not completely understood

    Most popular theory

    Presence of an epidermoid residue in the AS quadrant of the ME in the human fetus, on the

    lateral wall of the ET, near the annulus

    Failure to reabsorb the epidermoid residue normally found in embryos between the 10th and

    33rd week of gestation has been observed(Michaels, 1986), (Teed, 1936)

    Acquired Cholesteatoma

    2 types

    Primary acquired

    Secondary acquired

    Occur after birth

    Most common type typically as result of

    chronic middle ear (ME)

    disease

    Acquired Cholesteatoma

    Primary acquired

    Most common

    Forms as retraction

    pockets of the TM

    Occurs mostly in pars

    flaccida although pars

    tensa retractions can occur

    Bacteria can affect the

    keratin matrix, forming

    biofilms leading to chronic

    infection and epithelial

    proliferation

    Secondary acquired

    Form as result of either squamous epithelial

    migration from the TM or

    implantation of squamous

    epithelium into the ME

    during surgery (ie:

    Ventilation tube placement

    or tympanoplasty)

    Perforations from infection

    or trauma

    Acquired Cholesteatoma

    radiopaedia.org

    Acquired Cholesteatoma Staging Stage 1

    Confined to ME (hypo- & mesotympanum) without erosion of ossicular chain

    Stage 2 Same as 1, but in addition, there is erosion of one or more ossicles

    Stage 3 Involvement of middle ear and mastoid gas-cell system without erosion of

    ossicles

    Stage 4 Same as 3, but with erosion of one or more ossicles

    Stage 5 Extensive cholesteatoma of the middle ear, mastoid, & other portions of the

    temporal bone including one or more ossicles

    Not able to complete surgical removal (ie: medial to labyrinth) A fistula of the labyrinth may or may not be present

    Stage 6 Same as 5 but now cholesteatoma extends beyond the temporal bone

    (Bluestone & Klein, 2003)

  • 9/7/2013

    5

    Predisposing Factors

    History of recurrent acute otitis media and/or chronic middle ear effusions

    Older age at first placement of tympanostomy tubes, and increasing number of, and longer interval between, insertions

    Cleft palate

    Craniofacial anomalies

    Turner syndrome

    Down syndrome

    Family history of chronic middle ear disease and/or cholesteatoma

    Signs and Symptoms

    Acquired Typically present with recurrent or persistent

    purulent otorrhea and hearing loss

    Also can present with tinnitus, vertigo or dysequillibrium

    Facial nerve twitching, palsy or paralysis Can result from inflammatory process or mechanical

    compression of the nerve

    Congenital Conductive hearing loss

    Impact of Cholesteatoma

    Hearing loss (HL)

    Late recognition of cholesteatoma formation still a

    problem

    Speech delay

    Psychosocial effects

    Cholesteatoma in Children vs. Adults

    More aggressive disease in children than

    adults

    Very extensive disease is found in children at the time of surgery vs. adults

    Rates of persistent and recurrent cholesteatomaare higher in children

    Persistent eustachian tube dysfunction

    Well pneumatized mastoid complex(Bluestone & Klein, 2003), (Visvanathan et al, 2012)

    Cholesteatoma in Children vs. Adults

    Children frequently unaware of signs and symptoms

    Rarely complain about HL; especially if unilateral

    Frequently no otorrhea

    Otalgia may be absent

    Unable to determine fullness of the ear, tinnitus, mild vertigo

    Most adults have history of HL that is progressive and recurrent otorrhea

  • 9/7/2013

    6

    Complications

    Accumulation of keratin may cause:

    Infection

    Otorrhea

    Bone destruction

    Hearing loss Conductive &/or SNHL

    Vertigo

    Facial nerve paralysis

    Labyrinthine fistula

    Intratemporal infection

    Intracranial complications

    Epidural and subdural abscesses

    Parenchymal brain abscesses

    Meningitis

    Thrombophlebitis of the dural venous sinuses

    Brain herniation

    Complications: Epidural Abscess

    Complications: Sigmoid Sinus

    ThrombosisPatient History

    Recurrent acute otitis media

    Chronic otitis media with effusion Cleft palate

    Craniofacial abnormalities Turner or Down syndromes

    Family history of chronic ME including cholesteatoma New onset HL in previously operated ear

    Persistent otorrhea > 2weeks Foul smelling otorrhea

    Otalgia Concern about intratemporal or intracranial complication with otalgia symptomology

    Fullness of the ear Tinnitus

    Mild vertigo Facial muscle weakness

    Physical Exam

    White mass behind intact TM

    Deep retraction pocket with or without

    granulation and skin debris

    Focal granulation on surface of TM

    Otorrhea >2 weeks despite treatment

    Hearing loss

    Differential Diagnoses

    Tympanosclerosis

    White foreign body

    Exostoses

    Prosthetic and graft material

    Inclusion cysts of the tympanic membrane

    Bulging acute otitis media(Sie, 1996)

  • 9/7/2013

    7

    Differential Diagnoses Diagnosis

    May go undetected for many years as s/s may

    be lacking

    Otoscopy/Oto-microscopy

    Detailed otologic history

    Audiogram

    CT scan of the temporal bones

    Diffusion weighted MRI

    Diagnosis

    CT Scan

    CT of the temporal bones without contrast in the

    coronal and axial views often of value

    Identifies

    Extent of disease

    Possibility of ossicular involvement

    Complications such as labyrinthine fistula

    Used to aide in decisions regarding surgical

    approach and extent of surgery

    Axial View

    http://bestpractice.bmj.com/best-

    practice/monograph/1033/resources/image/bp/4.html

    Treatment

    Surgical intervention

    Tympanoplasty

    Canal Wall-Up mastoidectomy (CWU)

    Canal Wall-Down mastoidectomy (CWD)

    Partial Ossicular Replacement Prosthesis (PORP)

    Total Ossicular Replacement Prosthesis (TORP)

    Second-Look Procedure

  • 9/7/2013

    8

    Cholesteatoma Surgery in Children -

    Goals

    1. Eradicate disease

    2. Preserve or reconstruct the anatomic

    structure

    3. Preserve or restore hearing

    4. Prevent residual and recurrent disease

    (Bluestone, 2002)

    Tympanoplasty

    Tympanoplasty

    Surgical reconstruction of the

    TM/ossicle transformer mechanism

    ie: If a perforation is present, it will be repaired

    with a connective tissue graft but unlike with a

    myringoplasty, the middle ear is explored

    Ossicles can be repositioned (ossiculoplasty) to

    restore ossicular chain continuity

    Trans-canal or post-auricular

    Tympanoplasty

    (Trans-Canal & Post-Auricular) Pros

    Trans-canal

    Shorter surgery

    Less discomfort

    Patient perception

    Post-Auricular

    Better exposure

    Better graft harvest

    Anterior exposure

    Better handedness

    Limited disruption of canal skin vasculature

    Faster healing

    Tympanoplasty

    (Trans-Canal & Post-Auricular) Cons

    Trans-canal

    Limited exposure

    Difficult handedness

    Smaller grafts

    Disruption of vascular supply to canal skin

    Canal needs to heal

    Post-Auricular

    Longer surgery

    More discomfort

    Patient perception

    Tympanoplasty

    www.pedsent.com & www.med.unc.edu

    Mastoidectomy

    Mastoidectomy

    Removal of mastoid bone

    Involves the surgical exposure and removal of

    mastoid air cells

    http://www.ghorayeb.com/mastoidectomy.html

  • 9/7/2013

    9

    emedicine.medscape.com

    Tympanomastoidectomy with

    Tympanoplasty

    Tympanomastoidectomy with tympanoplasty

    Tympanoplasty performed in combination with

    mastoidectomy

    Canal Wall-Up procedure (aka: Closed cavity procedure)

    Canal Wall-Down procedure (aka: Open cavity procedure)

    Canal Wall-Up Mastoidectomy

    Posterior canal wall remains intact Preserves middle ear structures

    Hope that ME function may improve with age

    Higher rate of reoccurrence and need for second surgery

    Hearing aids fit better

    Maintenance free Fewer activity restrictions

    More natural appearance Better hearing results

    Canal Wall-Up Mastoidectomy

    Canal Wall-Down Mastoidectomy

    Every effort made to avoid CWD procedure

    Hearing may be worse (typically worse)

    Open mastoid cavity

    Enlarged canal visible to the naked eye About 2x the size

    More difficult to clean in children Would require general anesthesia for cleaning and

    debridement

    Swimming Common activity for children; More susceptible to

    infection with water exposure

    Canal Wall Down Mastoidectomy

    Pros

    Greatly reduces the risk of recurrence

    Good option for those with co-morbidities that are at high risk undergoing anesthesia

    Good option for those who have poor-follow-up

  • 9/7/2013

    10

    Canal Wall Down Mastoidectomy

    www.susmedicos.com

    Canal Wall-Up vs. Canal Wall-Down

    Literature Controversy with CWU vs. CWD

    Osborn, Papsin and James, 2012 Retrospective review 1996-2010 420 patients (435 total ears)

    Extent of cholesteatoma graded according to Mills Classification

    Pre & post air conduction hearing thresholds assessed

    CWU preferred Preserved canal in 89.5% of cases Financial and emotional costs of 2nd look offset by avoiding open

    cavity management

    Better hearing results

    CWD Lower rates of reccurence and revision

    Canal Wall-Up vs. Canal Wall-Down

    Literature Wilson, Hoggan & Shelton, 2013

    Case series with chart review

    All cases treated with Intact Canal Wall (ICW) mastoidectomy over 9 years with at least 2 years of follow-up data

    145 patients with 156 affected ears (median age 5.3 years; range 2.1-14.8 years)

    35% had residual cholesteatoma at 2nd stage

    13 patients (8%) had recurrence with 6 needing CWD mastoidectomy

    ICW mastoidectomy w/tympanoplasty continues to be successful treatment

    Substantial recurrence rate at 2nd stage justifying need for staging Recurrence rate using ICW is low; excellent disease control and

    functional results for majority of patients

    Ossicular Reconstruction

    Partial Ossicular Replacement Prosthesis (PORP)

    Used when the stapes superstructure is Intact

    Stapes superstructure is bridged with a synthetic

    biocompatible prosthesis to

    the tympanic membrane,

    graft or malleus

    PORP is positioned on the stapes head

    Total Ossicular Replacement Prosthesis (TORP)

    Used only when the stapes footplate is available

    Used when an intact spesfootplate is bridged with a

    synthetic biocompatible

    prosthesis to the tympanic

    membrane, graft or malleus

    TORP is placed on the stapes footplate

    (Batti & Bluestone, 2002)

    PORP & TORP

    www.getfitosc.com

  • 9/7/2013

    11

    Prosthesis Middle Ear Prosthesis

    www.entusa.com

    Second-Look Procedure

    Detects residual cholesteatoma or recurrent disease as the middle ear and mastoid are not directly visible Recommended at 6 months post-op in children 12 months post-op in adults Difference secondary to children experiencing more

    aggressive disease

    If disease is found at second-look, it is removed and the child is recommended to go to the OR again in 6 months Repeated until residual disease is not present

    Alternative To Second-Look Procedure

    Diffusion Weighted MRI Imaging (DWI)

    More recently introduced in cholesteatoma

    diagnosis

    Non-invasive

    Prevents unnecessary second-look surgery in patients without cholesteatoma

    Alternative To Second-Look Procedure

    How it works: Provide information about diffusion of water molecules in

    biological tissue

    Greater the diffusion of water molecules, the less signal enhancement appears in the image; lower the diffusion of water molecules, the greater signal enhancement appears

    Not exactly known why cholesteatoma shows a high level of signal enhancement on DWI

    DWI shows a cholesteatoma as high signal intensity; granulation and other tissue visualized at a low signal intensity

    Resultant image is bright at cholesteatoma and darker at surrounding tissues

    Diffusion Weighted MRI

  • 9/7/2013

    12

    Diffusion Weighted MRI Study

    Turkish Study (Evlice, Tarkan, Kiroglu, Bicakci, Ozdemir, Tuncer & Cekic, 2012)

    58 chronic otitis media patients with suspected cholesteatoma evaluated 2 weeks pre-operatively (41 with no previous surgery & 17 with previous surgery scheduled for second-look)

    Mean age 22 y/o (Range 9-67 y/o)

    All underwent Echo-planar diffusion-weighted MRI pre-operatively

    Operative findings and pathology results compared with MRI results

    Diffusion Weighted MRI Study

    Results Cholesteatoma found in 63% of patients with no

    history of surgical intervention and 58% found in second-look group

    Diffusion-weighted MRI accurate in 90% of group 1 and 76% of group 2 patients

    Conclusion Echo-planar diffusion-weighted MRI is a valuable

    imaging technique for detection of primary of recurrent acquired cholesteatoma

    Can be considered instead of second-look procedure

    Surgical Risks & Complications

    Tympanoplasty with or without Mastoidectomy

    Infection

    Hearing loss

    Tinnitus

    Dizziness

    Taste disturbance and mouth dryness

    General anesthesia complications

    Mastoidectomy

    Facial paralysis

    Hematoma

    Spinal fluid leak

    Dural injury

    Intracranial complications

    General anesthesia complications

    http://www.youtube.com/watch?v=xCQBSNF5DY

    Post-Operative Course

    Bulky dressing covering the ear for 24 hours Numbness of the ear

    Resolves over weeks to several months

    Sharp, sudden stabbing pains are common May take weeks to resolve

    Protrusion of the ear

    Pain with eating/chewing Taste disturbance (metallic taste)

    May take up to 6 months to resolve

    Blood tinged otorrhea www.otomed.com

    Post-operative Course

    Packing/sponge plug/wick may extrude Inform family not to not replace

    Hearing noises such as swishing, gurgling, your heartbeat is normal and slowly subsides

    Decreased hearing Swelling Hemotympanum Packing(All normal; takes several weeks to resolve)

    Dysequillibrium with quick movements Common for several days to 1 week post-op

  • 9/7/2013

    13

    Care & Cleaning of the Ear

    Strict water precautions until 1st post-op appointment

    If post-auricular incision present, may clean gently with soap and water after 1st post-op visit

    No water in canal up to 2-3 months Place cotton ball with Vaseline in outer part of canal

    No commercial ear plugs recommended May put pressure on packing

    Sleep with head elevated 30 degrees for 1 week; try not to lye on ear that was surgically repaired Decreases swelling

    Pad eyeglasses if you wear them

    Consider use of ice pack for 15-20 minutes at a time

    Care & Cleaning

    Clean post-auricular incision with H2O2 on a cotton swab morning and night

    Do not clean external auditory canal

    May use antibiotic ointment until 1st post-op visit Place dry cotton ball in outer canal to absorb

    drainage; change at least 2x daily and as needed

    Cover ear in frigid weather if post-auricular incision Post-op numbness Sensitivity

    Activity Restrictions

    No work or school for 1 week

    Light activity during the 1st week

    No strenuous activity, exertion, sports or lifting over 20 lbs for 1 month

    No playing musical wind instruments for 6 weeks

    No vigorous nose blowing or popping of the ears for 6 weeks

    Keep mouth open with sneezing Helps avoid transmission of pressure to the ear

    Use stool softener if constipated Avoids straining

    No airline flights for 1 month

    Medications

    Prescription pain medication Shouldnt be necessary after 7-10 days

    No aspirin, aspirin containing products or anti-inflammatory agents until 1st post-op visit

    Blood thinners may be started 1 week after

    Oral antibiotics prescribed for 1 week

    Otic drops Typically not started until 1st post-op visit

    Typically will need for multiple weeks

    Post-Operative Follow-Up

    Post-op visit #1 scheduled for 7-10 days following surgery

    Recommended to call for: Increased pain, post-auricular erythema or swelling Incision drainage Opening of incision Malodorous, discolored post-auricular drainage Fever over 100.5 Developed weakness of facial muscles on surgical side

    Developing/increasing vertigo Severe nausea or vomiting

    Future Follow-Up

    Every 3 months for the 1st year post-op

    Every 6 months for the 2nd & 3rd years post-op

    After the 3rd year; annually for 5-7 more years

    (Bluestone & Rosenfeld, 2002)

  • 9/7/2013

    14

    Future Follow-up

    Pediatric 29 year study

    Retrospective study 1982-2011

    81 ears; 73 studied

    Acquired only; congenital excluded; 18 years or younger

    Follow-up every 6 months for the first 5 years then annually

    Recidivism of cholesteatoma occurred in 7 ears

    Mean detection time was 10.4 years (range 1.9-17.2 years)

    2 were residual disease and 5 were recurrent

    Recommend: Follow-up as long as possible or at least until adulthood

    (Kuo, Shiao, Liao, Ho & Lien, 2012)

    Prevention

    No methods currently for congenital

    Primary acquired Pathogenesis not clearly understood; assumed that

    ETD is required for formation

    No way to correct ET function directly

    Provide secondary ventilation to the ME with ventilation tube placement

    Secondary acquired are often iatrogenic Surgeon takes all steps to prevent implantation of

    squamous epithelium into ME

    Case Study

    History 2 y/o little boy with history of recurrent acute

    otitis media

    Underwent bilateral myringotomy & ventilation tube (BM&T) placement just prior to new patient appointment w/us at outside facility

    At time of surgery, right-sided middle ear abnormality noted; ventilation tubes were placed

    CT scan of the temporal bones obtained and he was referred to CHW

    Case Study

    What are we concerned about based on this

    information?

    Congenital cholesteatoma

    What are your next steps?

    Physical exam

    Audiogram

    Review CT scan of the temporal bones

    Case Study

    Physical exam

    Otoscopy & Micro-otoscopy

    Ears well formed externally with normal canals

    Right TM showed whitish discoloration anteriorly

    but no obvious cholesteatoma formation;

    Ventilation tube was in place and patent

    Left tympanic membrane was clear with a tube that was in place and patent

    Case Study Audiogram 6/22/2011

  • 9/7/2013

    15

    Case Study Case Study

    Right middle ear exploration with ventilation tube removal, atticotomy and cholesteatoma removal, tympanoplasty with fascia

    Findings: Extensive congenital cholesteatoma throughout the ME

    space extending into the eustachian tube into the attic

    Did not extend posteriorly into the mastoid Involved the malleolus, incus, hypotympanum, eustachian

    tube, attic and part of the stapes; incus and malleoluswere removed along with substantial portion of the TM

    Middle ear mucosa diseased at grade 2 D/Cd on Ciprodex, Lortab

    Case Study

    Post-Op Visit (2 weeks later) Subjective:

    No concerns 1st week post-op Right otalgia was playing outside without head coverage

    No otorrhea Using Ciprodex as prescribed

    Now only using Tylenol at bedtime for pain

    Objective/Physical Exam: Right post-auricular incision well approximated, C/D/I Canal with small amount of gel foam which was removed under microscopy

    TM with small amount of gelfoam left alone; could not completely visualize TM but healing

    Plan: Continue Ciprodex BID

    Strict water precautions f/u 1 week

    Case Study

    Post-Op Visit #2 Subjective:

    Patient without complaint

    Objective/Physical exam: Right post-auricular incision healing well

    External auditory canal clear without gelfoam

    TM healing well

    Plan: Follow-up 2 months

    Plan for second-look procedure in 9-12 months

    Case Study

    Post-Op Visit #3 Subjective:

    No parental concerns

    Objective/Physical exam:

    Micro-otoscopy

    Right post-auricular site healed

    Right TM with post-surgical changes; no visible

    cholesteatoma

    Audiogram obtained

    Case Study

    Post-Op Visit #3 Post-Op Audiogram

    3/14/2012

    Pre-Op Audiogram 6/22/2011

  • 9/7/2013

    16

    Case Study

    Post-Op Visit #3

    Plan:

    See at second-look procedure scheduled in 4

    months

    Case Study

    Second-Look Procedure

    Right middle ear exploration with tympanoplasty

    and PORP ossicular reconstruction

    Discharged with Ciprodex, Cefdinir & Lortab

    Follow-up in 1 week

    Case Study

    Post-Op Visit #1 for Second-Look

    Procedure Subjective:

    Doing well, no otorrhea, no otalgia, afebrile, good oral intake, sleeping well

    Objective: Post-auricular incision well approximated; C/D/I Few superior sutures removed Some gelfoam in canal; left alone TM healing well

    Plan: Ciprodex BID for 14 days Strict water precautions f/u 2 weeks

    Case Study

    Post-Op Visit #2 for Second-Look

    Procedure Subjective:

    No concerns, no otorrhea, no otalgia, afebrile, good oral intake, sleeping well

    Using Ciprodex as recommended

    Objective: Post-auricular incision well healed Significant gelfoam; partial removal as it was adhered

    to TM

    Plan: Continue Ciprodex BID for 1 month with return at that

    time

    Case Study

    Post-Op Visit #3 for Second-Look

    Procedure Subjective:

    No concerns, no otorrhea, no otalgia, afebrile, good oral intake, sleeping well

    Stopped using drops early

    Objective: Post-auricular incision well healed

    Cerumen and dry gelfoam removed

    TM clear with post-surgical changes

    Audiogram obtained

    Case Study

    Pre-op Audiogram 6/22/2011

    (1st Audio)

    Post-op 2nd Look Procedure 9/18/2012

  • 9/7/2013

    17

    Case Study

    Post-Op Visit #3 for Second-Look

    Procedure Plan

    D/C water precautions

    Follow-up in 6 months

    Case Study

    Post-Op Visit #4 for Second-Look

    Procedure

    Subjective:

    No parental concerns

    Objective:

    Small less than 5% TM perforation on the right;

    cartilage healing well

    Audiogram Normal bilaterally!!!

    Plan:

    Follow-up in 4 months

    Audiogram 2/21/2013

    Questions??? Thank You!

    Dr. David Friedland

    Dr. Steven Harvey

    Dr. Joseph Kerschner

    Roxanne Link, APNP

    Kristina Keppel, APNP

    References

    Al Anazy, F.H. (2006). Iatrogenic cholesteatoma in children with otitis media with effusion in a

    training program. International Journal of Pediatric Otorhinolaryngology (70), 1683-1686.

    Batti, J.S., & Bluestone, C.D. Ossiculoplasty. In: Surgical atlas of pediatric otolaryngology. Bluestone, C.D., & Rosenfeld, R.M. (Eds), BC Decker, Inc 2002. p.75-89.

    Bennett, M., Warren, F., Jackson, G.C., & Kaylie, D. (2006). Congenital cholesteatoma: Theories, facts, and 53 patients. Otolaryngologic Clinics of North America 39(6),1081.

    Bluestone, C.D. Mastoidectomy & Cholesteatoma. In: Surgical atlas of pediatric otolaryngology. Bluestone, C.D., & Rosenfeld, R.M. (Eds), BC Decker, Inc 2002. p.91-122.

    Bluestone, C.D., Cantekin, E.I., Beery, Q.C., & Stool, S.E. (1978). Function of the Eustachian tube related to surgical management of acquired aural cholesteatoma in children.

    Laryngoscope (88), 1155-1164.

    Bluestone, C.D., & Klein, J.O. Intratemporal complications and sequelae of otitis media. In:

    Pediatric otolaryngology, 4th ed, Bluestone, C.D., Stool, S.E., Alper, C.M., Arjmand, E.M.,

    Casselbrant, M.L., Dohar, J.E., & Yellon, R.F. (Eds), Saunders, Philadelphia 2003. p.687-753.

    References

    Department of Otolaryngology and Communication Sciences-Division of Otology, Neuro-Otology & Skull Base Surgery. (n.d.). A guide to cholesteatoma [Brochure].

    Department of Otolaryngology and Communication Sciences-Division of Otology,

    Neuro-Otology & Skull Base Surgery. (2011). Risks and complications of surgery:

    Tympanoplasty with or without mastoidectomy [Fact Sheet].

    Department of Otolaryngology and Communication Sciences-Division of Otology, Neuro-Otology & Skull Base Surgery. (2011). Post-operative instructions for ear surgery

    [Fact Sheet].

    Derlacki, E.L., & Clemis, J.D. (1965). Congenital cholesteatoma of the middle ear and mastoid. Annals of Otology, Rhinology & Laryngology (74),706-727.

    Evlice, A., Tarkan, O., Kiroglu, M., Bicakci, K., Ozdemir, S., Tuncer, U. & Cekic, E. (2012). Detection of recurrent and primary acquired cholesteatoma with echo-planar diffusion-

    weighted magnetic resonance imaging. The Journal of Laryngology & Otology (126),

    670-676.

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    References

    Golz, A., Goldenberg, D., Netzer, A. (1999). Cholesteatomas associated with ventilation tube insertion. Archives of Otolaryngology Head & Neck Surgery (125), 754-757.

    Isaacson, G. (2007). Diagnosis of Pediatric Cholesteatoma. Pediatrics (120), 603-608.

    Kemppainen, H.O., Puhakka, H.J., Laippala, P.J., Sipil, M.M., Manninen, M.P., & Karma, P.H. (1999). Epidemiology and aetiology of middle ear cholesteatoma. Acta Oto-laryngologica119(5),568.

    Kuo, C.L, Shiao, A, Liao, W., Ho, C., & Lien, C. (2012). How long is long enough to follow up children after cholesteatoma surgery?: A 29-year study. The Laryngoscope (122), 2568-2573.

    Levenson, M.J., Michaels, L., & Parisier, S.C. (1989). Congenital cholesteatomas of the middle ear in children: Origin and management. Otolaryngology Clinics of North America (22), 941-954.

    Lin, V., Daniel, S., James, A., & Friedberg, J. (2004). Bilateral cholesteatomas: The hospital for sick children experience. American Journal of Otolaryngology 33(3),145.

    Meyer, T.A., Strunk, C.L. Jr., & Lambert, P.R. Cholesteatoma. In: Head & neck surgery otolaryngology, 4th ed, Bailey, Byron, J., Johnson, Jonas, T. & Newlands, S.D. (Eds), Lippincott Williams & Wilkins 2006. P. 2082-2091.

    Michaels, L. (1986). An epidermoid formation in the developing middle ear: Possible source of cholesteatoma. American Journal of Otolaryngology 15(3), 169-74.

    References

    Nevoux, J., Lenoir, M., Roger, G., Denoyelle, F., Ducou Le Pointe, H., & Garabedian, E.N. (2010). Childhood cholesteatoma. European Annals of Otorhinolaryngology, Head and Neck Diseases (127), 143-150.

    Osborn, A.J., Papsin, B.C., & James, A.L. (2012). Clinical indications for canal wall-down mastoidecotmy in a pediatric population. Americal Academy of Ootlaryngology-Head & Neck Surgery 147(2), 316-322.

    Peciado, D.A. (2012). Biology of cholesteatoma: Special considerations in pediatric patients. International Journal of Pediatric Otorhinolaryngology (76), 319-321.

    Potsic W.P., Korman, S.B., Samadi, D.S., & Wetmore, R.F. (2002). Congenital cholesteatoma: 20 years' experience at the Children's Hospital of Philadelphia. Otolaryngology- Head and Neck Surgery 126(4),409.

    Rizer, F.M., & Luxford, W.M. (1988). The management of congenital cholesteatoma: Surgical results of 42 cases. Laryngoscope (98), 254-256.

    Sie, K.C. (1996). Cholesteatoma in children. Pediatric Clinics of North America 43(6),1245.

    Spilsbury, K., Miller, I., Semmens, J.B., & Lannigan, F.J. (2010). Factors associated with developing cholesteatoma: a study of 45,980 children with middle ear disease. Laryngoscope 120(3),625.

    References

    Teed, R.W. (1936). Cholesteatoma verum tympani: Its relationship to the first epibranchial

    placode. Archives of Otolaryngology-Head & Neck Surgery (24), 455-74.

    Visvanathan, V., Kubba, H., & Morrissey, M.S.C. (2012). Choleswteatoma surgery in children: 10 year retrospective review. The Journal of Laryngology & Otology (126)450-453.

    Wilson, K.F., Hoggan, R.N., & Shelton, C. (2013). Tympanoplasty with intact canal wall mastoidectomy for cholesteatoma: Long-term surgical outcomes. Otolaryngology Head &

    Neck Surgery 149(2), 292-295.

    Contact information

    Melissa Raines, MSN, CPNP, APNP

    Phone: 414-266-4957

    E-mail: [email protected]

    Physician Referral and Consultation: (800) 266-0366