Congenital external ear deformities and reconstruction

Congenital external ear
deformities and reconstruction
Dr. Jason Goodwin
Dr. David Chang
Overview
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Ear development
Congenital ear deformities
Classification systems
Evaluation
Reconstruction options
Microtia
• Abnormal development of external ear resulting
in deformed auricle
– Ranges from mild deformity to complete agenesis of
ear.
– 1:10000-20000 births
– Most often unilateral
– Right side predominance (twice left)
– 2.5:1 Male to female ratio
– Associated with congenital aural atresia
• Degree of external ear malformation mirror EAC and middle
Kelley, P. Et al. Microtia and Congenital Aural Atresia. Otolaryngol Clin N Am 40 (2007) 61–80
Demographics
• Associated with other anomalies in ~50%
– Various syndromes of H&N
• Increased incidence in Japanese, Native
American, Hispanic populations
• Causes
– Teratogens (Vit A, thalidomide), intrauterine
infections (Rubella, CMV etc.), vascular insults to
fetus
– Genetic anomalies
Kelley, P. Et al. Microtia and Congenital Aural Atresia. Otolaryngol Clin N Am 40 (2007) 61–80
Ear landmarks
Auricle embryology
• 6 Hillocks of His (mesoderm)
• 1st pharyngeal arch
– 1: Tragus
– 2: Helical crus
– 3: Helix
• 2nd pharyngeal arch
– 4: antihelix
– 5: antihelix
– 6: antitragus
Ear embryology
• Week 6: Hillocks begin to form around 1st
branchial pouch
• Starts low and anteriorly then migrates
dorsally and cephalad during months 2-3
• In final position at month 5
Evaluation
• Typically present early in life
• Focus on mandible, eyes, spine – rule out other
anomalies that may lead to a specific syndrome
– Cleft lip/palate: 4.3%
• Facial nerve function
– Some weakness found in up to 15%
– 43% of those involve multiple branches
• Hearing assessment
– Mostly conductive, but 10-15% will have some sort of
SNHL
Kelley, P. Et al. Microtia and Congenital Aural Atresia. Otolaryngol Clin N Am 40 (2007) 61–80
Brent, B. http://www.microtia.us.com/
Evaluation
• Careful counseling to family
• Protect good ear aggressively
• Additional studies
– CT T-bone
– Renal ultrasound: 4%
– Cardiovascular: 2.5%
– Genetics
Kelley, P. Et al. Microtia and Congenital Aural Atresia. Otolaryngol Clin N Am 40 (2007) 61–80
Brent, B. http://www.microtia.us.com/
Associated syndromes
• Up to 50% association with syndrome
• Often in conjunction with aural atresia
• Higher likelihood in facioauriculovertebral
syndromes
– Goldenhar
– Hemifacial microsomia
– Treacher Collins
Kelley, P. Et al. Microtia and Congenital Aural Atresia. Otolaryngol Clin N Am 40 (2007) 61–80
Name the syndrome
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A. Treacher Collins
B. Hemifacial microsomia
C. Goldenhar
D. Down
Hemifacial microsomia: congenital disorder that affects the development of the
lower half of the face, most commonly the ears, the mouth and the mandible.
Name the syndrome
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A. Treacher Collins
B. Hemifacial microsomia
C. Goldenhar
D. Down
Treacher-Collins: rare autosomal dominant congenital
disorder characterized by craniofacial deformities The
typical physical features include downward slanting
eyes, micrognathia (a small lower jaw), conductive hearing
loss, underdeveloped zygoma, drooping part of the lateral
lower eyelids, and malformed or absent ears.
Name the syndrome
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A. Treacher Collins
B. Hemifacial microsomia
C. Goldenhar
D. Down
Goldenhar: Oculo-auricular-vertebral syndrome. Characterized by incomplete
development of the ear, nose, soft palate, lip, and mandible. It is associated with
anomalous development of the first branchial arch and second branchial arch. Common
clinical manifestations include limbal dermoids, preauricular skin tags, and strabismus.
Microtia classification system
• Multiple classification systems exist
– Some incorporate EAC , middle ear, or other
syndromic features into scoring system
• Most common is one that only takes external
appearance into account (Weerda)
• Practically the helix/antihelix, conchal bowl,
tragus, and lobule are the structures the
reconstructive surgeon wishes to repair and
should be considered in staging
Bartel-Friedrich et al.: Classification and diagnosis of ear malformations Current Topics in Otorhinolaryngology - Head and
Neck Surgery 2007, Vol. 6, ISSN 1865-1011
Microtia classification system
• Type I: Mild deformity, major structures
present to some degree, no tissue needed
• Type II: major structures present, but tissue
deficit to degree additional tissue needed
• Type III: Few recognizable landmarks, lobule
usually present to some degree
• Type IV: anotia
Bartel-Friedrich et al.: Classification and diagnosis of ear malformations Current Topics in Otorhinolaryngology - Head and
Neck Surgery 2007, Vol. 6, ISSN 1865-1011
Type I
Bartel-Friedrich et al.: Classification and diagnosis of ear malformations Current Topics in Otorhinolaryngology - Head and
Neck Surgery 2007, Vol. 6, ISSN 1865-1011
Type II
Bartel-Friedrich et al.: Classification and diagnosis of ear malformations Current Topics in Otorhinolaryngology - Head and
Neck Surgery 2007, Vol. 6, ISSN 1865-1011
Type III
Bartel-Friedrich et al.: Classification and diagnosis of ear malformations Current Topics in Otorhinolaryngology - Head and
Neck Surgery 2007, Vol. 6, ISSN 1865-1011
Type IV (Anotia)
Bartel-Friedrich et al.: Classification and diagnosis of ear malformations Current Topics in Otorhinolaryngology - Head and
Neck Surgery 2007, Vol. 6, ISSN 1865-1011
Reconstructive options
• No surgery
• Prosthetic
• Surgery
– Autogenous material
– Synthetic implant
History of surgery
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Scattered reports of repair 2000 yrs ago with local flaps
1575 – first published article with metal ear prosthesis
First attempts at repairing entire auricle in late 1800s
Gillies first to use rib cartilage (1920)
Tanzer in mid 60s gained prominence with larger series
of rib graft patients
• Tanzer’s pupil Burt Brent expanded and modified the
Tanzer technique and is now arguably the world’s
preeminent expert on microtia reconstruction
Surgery
• Timing of repair
• Minimum of 6 years old
– Ear is 85-90% of adult size at this age
– Need time for sufficient rib cartilage to grow
• Ribs Not 85% adult size at this age
• Rib cartilage retains growth potential of source
– Psychologic issues start around this age
• Any surgical correction of the external ear needs
to consider the effect of surgery on possible EAC
or middle ear reconstruction
Brent Reconstruction Technique
• Modification of Tanzer technique
• Four stage technique
– 1:
– 2:
– 3:
– 4:
Rib harvest of construction of framework
Lobule interposition
Elevation of ear
Tragus construction
• Minimum of two months between first and
second and then three months between
subsequent stages
Brent, B. http://www.microtia.us.com/
Pre-op planning
From Brent, B.: Total auricular construction with sculpted
costal cartilage. In B. Brent (Ed.), The Artistry of
Reconstructive Surgery, St. Louis, 1987, The C.V. Mosby Co.,
pp. 113-127
First Brent Stage
• Harvest of rib cartilage
• Taken from contralateral side
• Fused section of ribs 6 and 7
Brent, B. http://www.microtia.us.com/
First stage
• Framework construction
Brent, B. http://www.microtia.us.com/
First stage
• Incision made along
area of planned ear
• Unusable cartilage
removed
• Undermine widely
• Close with drains
Brent, B. http://www.microtia.us.com/
Second Brent stage
• Lobule transposition
Brent, B. http://www.microtia.us.com/
Third Stage
• Elevating the ear
• Incise along the
posterior ear and raise
it
• Cover post-auricular
areas with FTSG,
typically taken from
abdomen
Brent, B. http://www.microtia.us.com/
Fourth stage
• Tragus reconstruction
• Composite graft taken
from opposite conchal
bowl
Brent, B. http://www.microtia.us.com/
Nagata Reconstruction
• Multiple variations of Brent technique, Nagata
being most popular
• Two-stage technique
– Stage I: fabrication of auricular framework from
ipsilateral costal cartilage, tragus reconstruction,
and lobule transposition
– Stage II: framework elevation
http://www.nagata-microtia.com/Medical_care/
Nagata first stage
http://www.nagata-microtia.com/Medical_care/
Nagata first stage
http://www.nagata-microtia.com/Medical_care/
Alloplastic material
• In past implants have been made from: wire
mesh, steel wire, acrylic, silastic, teflon
• All of these had unacceptably high extrusion
rates
• Medpor started in early 1990s
• Typical Medpor surgery is a two stage surgery
Medpor reconstruction
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No rib harvest
Earlier age of repair
Excellent contour and shape
Shorter learning curve?
• Does not grow with patient
• Implant extrusion
• Need for TP flap
Prosthetic ear
• Osseo-integrated anchoring device
• Indication:
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Failed autogenous reconstruction
Severe soft- tissue/skeletal hypoplasia
Low or unfavorable hairline
Acquired total or subtotal auricular defect, usually in
adults – cartilage may be unfavorable
• Prosthesis changes every 2 to 3 years
– $2000-4000
• Meticulous hygiene at skin/implant interface
• Preclude future autogenous reconstruction
Prosthetic ear
Prosthesis
Advantages
• Avoids chest surgery
• May look very realistic
• May be of value for an older
patient who does not want
surgery or is at risk for a
surgical procedure
Disadvantage
• Does not grow with patient
• Still requires surgery (unless
adhesive-type is used)
• Screws are drilled on side of
head and a bar is placed to
hold the prosthetic ear
• Color mismatch if skin tans
• The patient will never feel like
the ear is part of his/her body,
although this may not be the
case in a poorly reconstructed
ear or an ear affected by
trauma
Complications
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Pneumothorax from rib harvest
Infection
Hematoma
Skin loss
Implant extrusion (Medpor)