aural microtia and atresia - thomas jefferson university
TRANSCRIPT
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Katie McKee-Cole
June 15, 2016
CME Code: MUNQOX
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Microtia Introduction
Classification schemes
Clinical evaluation
Physiologic/Psychological Impact
Reconstruction
Atresia Introduction
Classification schemes
Reconstruction
Conclusion
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Incidence of microtia 1-3 in 10,000
58% right sided, 32% left sided, 9% bilateral
63% male, 37% female
Genetics
w/ positive family history
AD 9%, AR 90%, X 1%
Familial non-syndromal high grade microtia
Predominantly AD w/ variable penetrance
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Ischemia
In utero obliteration of stapedial a. or hemorrhage into
local tissues
Chromosomal aberrations
Goldenhar, Treacher-Collins, 18q deletion, others
Teratogens
Thalidomide, isotretinoin, vincristine, colchicine,
cadmium
Maternal infections
Unknown
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1900 1950 2000
Marx Rogers
Tanzer
Weerda
Nagata
Aguilar
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Grade 1
Pinna malformed and
smaller than normal but
with good definition and
minor anomalies
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Grade 2
Helix may or may not
be fully developed
Triangular fossa,
scapha, antihelix have
much less definition
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Grade 3
Pinna essentially
absent except for
vertical remnant of
unorganized elastic
cartilage superiorly with
relatively well-formed
lobule
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Anotia
Total absence of the
pinna
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Brent Classical microtia
Larger lobule with amorphous cartilage remnant
Usually w/ EAC absence
Atypical microtia More recognizable
portions of concha, antihelix, tragus and antitragus
Upper portion absent
EAC may or may not be present
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Nagata
Lobular remnant type
deformity
Corresponds to classic
type microtia
Conchal remnant type
deformity
Corresponds to atypical
type microtia
small and large type
depending on conchal
development
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Degree of associated facial hypoplasia
Auricular dystopia Medial, inferior and
anterior displacement of auricle or microtic vestige due to underlying facial skeleton deficiency
May also have a dystopic EAC
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Timing: ideally within a few weeks after birth
H/o exposure to teratogens sought
Examination:
Anomalous ear, normal ear
Other possible associated anomalies
Hearing status
OAE and/or ABR within first 2-3 months
Bone conduction usually but not always normal w/ CAA
CHL usually maximal due to lack of EAC/ossicle fixation
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Renal US
Xray: C, T, L spine to r/o malformations
CT T-bone Possibility of reconstruction
Presence of cholesteatoma
Some obtain at 1 yr, others delay until 5-7 years
3D CT scans in those w/ craniofacial microsomia
Follow in office in 6-12 month intervals
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Functional Hearing in b/l cases
Difficulty wearing glasses/hearing aids
Psychological Age < 5 yrs: usually little
psychological impact
Age > 5 yrs: begins to notice difference between ears
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Goals to emphasize with parents
1st: Maximizing hearing
Speech development usually normal in u/l microtia/atresia
Frequent otologic evaluations to r/o problems in normal ear
Early use of BAHA softband especially for b/l, but also u/l
Hearing may improve with recon, perfect hearing unlikely
2nd: Cosmesis
An auricle can be created that looks much better than the
vestige but it may not be completely normal
Reconstruction requires several stages
Revisions may be necessary
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Observation
Molding
Prosthetic replacement of external ear
Reconstruction with prosthetic framework
Autologous framework with local tissue/flap
coverage
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Some grade 1
malformations
Instituted very shortly
after birth
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Newer osseointegrated
anchoring systems
Surgical placement of a
titanium anchor
Magnet system and
prosthesis attached
Bone conduction HA
can be built in
Expensive
Precludes future recon
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First reported in 1960’s
Then abandoned due
to erosion/exposure
Newer medpor
framework has seen
better results
Long-term follow-up
needed
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First reported in 1930’s, expanded in 1940’s
Came to forefront with Brent in 1980’s
Further refinements by Nagata recently
3 main elements among all methods
Construction and placement of cartilage framework
Lobule rotation, conchal excavation and tragus
formation
Elevation of the pinna
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B/l vs u/l microtia w/ or w/o atresia
Recommended age varies widely
Early
Psychological impact minimized, hearing function improved
at earlier age
Later
Better cosmetic outcome, patient compliance
Microtia repair prior to atresia to preserve blood
supply
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Template for positioning
of reconstructed auricle
Template for cartilage
framework
Low hairline
Assessment of costal
cartilage volume
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3 levels or complexes of
reconstruction
Proportions and relative
position
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Constructed from costal
cartilage fragments from
6th-8th ribs
Opinions on ipsilateral
vs contralateral differ
Alterations with conchal
remnant microtia
Cartilage banked for
projection
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Cartilage framework constructed
Vary by technique Nagata, Firmin, Bauer:
rotation of the lobule and tragal construction at 1st
Brent: delay lobule transposition and tragal reconstruction till 2nd
Amorphous cartilage excised, framework can be spliced into usable portions
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3-4 months after 1st
Nagata and Firmin:
Elevation of
reconstructed ear
Brent:
Rotation of the lobule,
conchal excavation
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Brent:
construction of tragus
elevation of the reconstructed ear
Cryptotia repair is similar to the 4th stage
Post-op scar formation can obliterate part of the sulcus and decrease projection
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Ideal procedure yet to
be achieved
Altered vascular
anatomy
Transposition of microtic
vestige
Framework construction
modified due to
hypoplastic base
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Infection
Cartilage/soft tissue loss
Chest wall deformity
Pneumothorax
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Failure of development
of EAC and middle ear
Generally have normal
cochlear function
Epidemiology
1/10K-20K live births
3-4 u/l : 1 b/l
Right > left
Male > female
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Degree depends on
point of interruption of
development
Group 1
Normal or stenotic
canal with hypoplastic
middle ear and mild
malformation of the
ossicles
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Group 2
Fistulous tract or
complete atresia of the
canal with a bony
atretic plate and some
degree of malformation
of the middle ear
structures
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Group 3
Complete ear canal
atresia with
nonpneumatized
mastoid and middle ear
Atresia commonly
coexists with microtia,
can occur alone
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Restoration and stability
of hearing
Maintenance of a
patent, skin-lined EAC
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Softband bone
conduction aid
BAHA
Alterations in technique
Additional resources for
hearing loss
Preferential seating,
FM system,
individualized
education program,
speech therapy
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Anatomic considerations
Malleus-incus complex
(MIC) large & directly
lateral to the stapes
Low lying tegmen
Facial nerve obstructing
oval window
Facial nerve turns
anterolateral obstructing
attic/middle ear
TMJ lateral to middle ear
Anomaly of the inner ear
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Unilateral
Early repair
Late repair
Bilateral
Hearing status
Favorable anatomy
Microtia implant
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Schuknecht methods
Transmastoid
Jahrsdoerfer anterior
approach
Standard in atresia
surgery today
Anesthesia concerns
Facial nerve monitoring
Avoid nitrous oxide
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Temporalis fascia graft
obtained
Mastoid periosteal
incisions
Identification of
landmarks
Drill atretic bone
Enter middle ear in
epitympanum
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Establish status of
ossicles
Fascia grafting
Skin grafting
Meatoplasty
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Periodic debridement
May swim after 1 month
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Facial nerve injury
Sensorineural hearing loss
Need for revision
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Newer alloplasts
Tissue engineering
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1. Aguilar EA III. Auricular reconstruction of congenital microtia (grade III). Laryngoscope
1996;106(suppl 82):1-26.
2. Breugem, C., et al. International Trends in the Treatment of Microtia. J Craniofac Surg, 2011.
22(4): p1367-1369.
3. Luquetti., D.V., et al. Microtia-Anotia: A Global Review of Prevalence Rates. Birth Defects
Research (Part A), 2011. 91: p. 813-822.
4. Sabbagh, W. Early experience in microtia reconstruction: The first 100 cases. J Plast, Recon
& Aesthetic Surg, 2011. 64: p. 452-458.
5. Tanzer, R.C. Total Reconstruction of the Auricle: The Evolution of a Plan of Treatment.
Plastic and Reconstructive Surgery, 1971. 47(6): p. 523-533.
6. Brent, B. The Correction of Microtia with Autogenous Cartilage Grafts: I. The Classic
Deformity. Plastic and Reconstructive Surgery. 66(1): p. 1-12.
7. Nagata, S. A New Method of Total Reconstruction of the Auricle for Microtia. Plastic and
Reconstructive Surgery, 1993. 92(2): p. 187-201..
8. Romo, T. and Reitzen, S.D. Aesthetic Microtia Reconstruction with Medpor. Facial Plast
Surg, 2008. 24: p. 120-128..
9. Shieh, S., et al. Tissue engineering auricular reconstruction: in vitro and in vivo studies.
Biomaterials, 2004. 25: p. 1545-1557.
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10. Weerda H. Classification of congenital deformities of the auricle. Facial Plast Surg 1988;5:385
11. .Zim SA. Microtia reconstruction: an update. Curr Opin Otolaryngol Head Neck Surg 2003, 11:275–281.
12. Williams JD, et al. Porous High-Density Polyethylene Implants in Auricular Reconstruction. Arch Otolaryngol Head Neck
Surg/Vol 123, June 1997.
13. Cronin TD. Use of Silastic Frame for Total and Subtotal Reconstruction of the External Ear: Preliminary Report. Plastic &
Reconstructive Surgery, Vol. 37, No. 5. 1966.
14. Cronin TD, et al. Follow-up Study of Silastic Frame for Reconstruction of External Ear. Plastic & Reconstructive Surgery, Vol.
42, No. 6. 1968.
15. Lynch JB, et al. Our Experiences with Silastic Ear Implants. Plastic & Reconstructive Surgery. Vol 49. No. 3. 1972.
16. Romo T, et al. Reconstruction of Congenital Microtia-Atresia: Outcomes with the Medpor/Bone-Anchored Hearing Aid-
Approach. Annals of Plastic Surgery, Vol. 62, No. 4. 2009.
17. Scharer SA, et al. Retrospective Analysis of the Farrior Technique for Otoplasty. Arch Facial Plast Surg. 9(3):167-173. 2007.
18. Head & Neck Surgery – Otolaryngology/[edited by] Byron J. Bailey [et al.]. 4th ed. 2006.
19. Local Flaps in Facial Reconstruction, 2nd ed. Shan R. Baker, M.D. 2007.
20. Facial plastic and reconstructive surgery / [edited by] Ira D. Papel [et al.]. 2nd ed. 2002.
21. Current Diagnosis & Treatment in Otolaryngology – Head & Neck Surgery, 3e. 2011.
22. Brent, B. (1998-2011). Microtia-Atresia. http://www.microtia.us.com.
23. Nagata, S. Nagata Microtia and Reconstructive Plastic Surgery Clinic. http://nagata-microtia.com.
24. Naumann A. Otoplasty – techniques, characteristics and risks. 2008. http://www.egms.de/static/en/journals/cto/2008-
6/cto000038.shtml
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Advisor: Dr. O’Reilly
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