Title: MICROTIA
1MICROTIA
With an Emphasis on Reconstructive Options
- Viet Pham, M.D.
- Harold Pine, M.D.
- Raghu Athre, M.D.
- University of Texas Medical Branch
- Department of Otolaryngology
- Grand Rounds Presentation
- January 26, 2010
All images obtained via Google search unless
otherwise specified. All images used without
permission except for those provided by Dr. Athre.
2Outline
- Anatomy and Development
- Microtia
- Reconstructive Options
- Autogenous cartilage
- Tissue expanders
- Osseo-integrated prosthesis
- Tissue engineering
- Alloplastic implants
- Conclusion
3Anatomy and DevelopmentEmbryology
- Development begins at 5 weeks gestation
- First branchial cleft
- Dorsal end of first (mandibular) and second
(hyoid) branchial arches - Six mesenchymal proliferations (Hillocks of His)
- First arch (hillocks 1-3), second arch
(hillocks 4-6) - Tragus (1)
- Helix (2,3)
- Antihelix (4,5)
- Lobule (6)
- Starts on lower neck but ascends with mandible
development (weeks 8-12), adult location by
week 20
4Anatomy and DevelopmentMusculature
- Intrinsic musculature
- Major and minor helixes
- Tragus
- Antitragus
- Transverse
- Oblique
- Extrinsic musculature
- Anterior auricularis
- Superior auricularis
- Posterior auricularis
5Anatomy and DevelopmentBlood Supply and
Lymphatics
Superficial Temporal Artery
Posterior Auricular Artery
- Arterial
- Superficial temporal
- Posterior auricular
- Occipital
- Venous
- Superficial temporal
- Posterior auricular
- External jugular
- Retromandibular veins
- Lymphatics
- Parotid lymph nodes
- Cervical lymph nodes
Occipital Artery
6Anatomy and DevelopmentInnervation
Auriculotemporal
Posterior Auricular
- Auricle (facial nerve)
- Temporal branch ? anterior and superior
auricularis - Posterior auricular nerve ? posterior
auricularis - Sensory
- Anterior
- Auriculotemporal nerve (V3)
- Greater auricular nerve (anterior branch)
- Arnolds nerve (branch of vagus)
- Posterior
- Greater auricular nerve (C3)
- Lesser occipital nerve (C2,C3)
Temporal Branch
Greater Occipital
Lesser Occipital
Greater Auricular
7Anatomy and DevelopmentNormal External Ear
- Ear development with age
- 66 of adult size at birth
- 85 of adult size at age 3 years
- 95 of adult size at age 6 years
- Normal height 5.5-6.5cm
- Posterior vertical inclination 5-30º
- Vertical angle parallel or within 15º of nasal
dorsum - Helical rim protrudes 1.5-2.0cm from mastoid with
15-20º protrusion angle
(Bailey, 2006)
8Microtia
- Abnormal development of external ear
- Incidence of 0.76-2.35 per 10,000 births
- Males affected more than females (2.51)
- Higher occurrence in Hispanics, Asians
(Japanese), and Native Americans (Navajo, Eskimo) - Increased risk with increasing multiparity (four
or greater) - Predilection for the right ear than left
- Family history in less than 15 of cases
9MicrotiaClassification and Grading
- First classification system by Marx (1926)
- Amended by Jarhsdoerfer and Aguilar (1988)
- Further refined and reaffirmed by Aguilar (1996),
only auricular malformations - Additional classification schemes with
concomitant congenital aural atresia - Altmann (1955)
- Lapchenko (1967)
- Gil (1969)
- Jarhsdoerfer and Aguilar (1988)
10MicrotiaClassification and Grading (Marx)
Normal ear
- Grade I
- Slightly smaller auricle
- Mild deformity but can distinguish each part
- Grade II
- 1/2 2/3 of normal size
- Mild deformity but can distinguish each part
- Grade III
- Severe malformation
- Peanut ear
Grade IV (anotia)
11MicrotiaClassification and Grading (Aguilar)
Normal ear
- Grade I
- Slightly smaller auricle
- Mild deformity but can distinguish each part
- Grade II
- 1/2 2/3 of normal size
- Mild deformity but can distinguish each part
- Grade III
- Severe malformation
- Peanut ear
Grade IV (anotia)
Grade II (deformities present)
Grade III (includes anotia)
Grade I (normal ear)
12Microtia Considerations
- Limb reduction
- Renal malformation
- Holoprosencephaly
- Association with other congenital abnormalities
- Cleft palate/lip
- Cardiac defects
- Anophthalmia
- Microphthalmia
- Hearing loss (80-90 conductive hearing loss)
- Sensorineural hearing loss in 10-15
- Can delay additional testing to 6-7 months if
nonmicrotic ear passes newborn hearing screen - Middle ear abnormalities but no direct relation
- Associated facial nerve dysfunction
13Microtia Reconstruction
- Reports of first attempts in 1500s
- Folded mastoid flap by Dieffenbach in mid-1800s
(for traumatic defect) - Autogenous cartilage by Pierce in 1930s
- Tanzer subcutaneous placement of autologous
cartilage graft framework (1959) - Modified by Brent in 1970s
- Cronin silicone rubber implant (Silastic)
(1966) - Williams polyethylene implants (Medpor) (1997)
14Microtia Reconstruction
- Reconstruction typically delayed until 6 years of
age - Especially for unilateral cases
- Sufficient autogenous costal cartilage
- Psychological factors (i.e. manage postoperative
care, school) - Congenital aural atresia drill-out no earlier
than 7 years - Autogenous cartilage is considered gold standard
- Brent technique
- Nagata technique
15Microtia ReconstructionBrent Technique
- Adapted from Tanzers six-stage procedure (1959),
four-stage procedure (1971) - Prefer to perform by 6 years of age
- Approximately 60-70 of cases done between 6-10
years - Four-stage procedure (1974)
- Develop auricular framework
- Lobule transposition
- Auricular framework elevation
- Tragus formation
- Any otologic surgery follows after reconstruction
16Brent TechniqueStage I Fabricate Auricular Frame
- Template of normal ear (if unilateral) or
parents ear (if bilateral) on X-ray film - Decrease size of template by a few millimeters to
accommodate for skin cover thickness - Harvest costal cartilage from ribs 6-8
contralateral to microtic ear - Base formed by synchondrosis of ribs 6 and 7
- Helical rim formed by floating rib 8
- Pieces attached with clear Nylon suture
(Bailey, 2006)
17Brent TechniqueStage I Fabricate Auricular Frame
- Place framework in subcutaneous pocket at
posterior and inferior borders of vestige - Extra cartilage banked with frame work or in
chest incision - Two suction drains beneath and near framework
- Left for five days
- Avoid complications related to pressure and
bolster dressings
(Walton, 2002)
18Brent TechniqueStage II Transpose Lobule
- Performed several months after Stage I completed
- Inferiorly based rotational flap to receive
framework
(Bailey, 2006)
(Walton, 2002)
(Shen, 2004)
19Brent TechniqueStage III Elevate Framework
- Incision a few millimeters from helical rim
- Dissect over the posterior aspect of the capsule
until desired amount of projection achieved - Ear position stabilized with banked cartilage
posteriorly beneath frame in fascial pocket - Retroauricular scalp advanced, followed by
split-thickness skin graft
(Walton, 2002)
(Shen, 2004)
20Brent TechniqueStage IV Construct Tragus
- Composite skin/cartilage graft from anterolateral
aspect of contralateral (i.e. normal) conchal
vault - J-shaped incision along posterior tragal margin
- Composite graft inserted into incision to project
neotragus and cavitate retrotragal hollow
(Bailey, 2006)
21Brent TechniqueStage IV Construct Tragus
- Shadow of neotragus imitates external auditory
canal - Conchal bowl deepened with subcutaneous tissue
excavation - Adjust for frontal symmetry as needed
(Walton, 2002)
22Brent TechniquePreoperative and Postoperative
(Walton, 2002)
23Brent TechniquePreoperative and Postoperative
(Thorne, 2001)
24Brent TechniqueProcedural Modifications
- Laser hair removal of scalp flaps prior to
reconstruction - Create tragus with cartilage framework in Stage I
- Floating cartilage creates helix
- Second strut arches around to form antitragus,
intertragic notch, and tragus - Tip of strut affixed to helical crus of main
frame with horizontal mattress suture using clear
nylon
Horizontal Mattress Nylon Suture
(Walton, 2002)
Second Strut
25Brent TechniqueCritique
- High number of stages
- Operative morbidity
- Increased cost
- Aesthetic result of tragal reconstruction
- Lack of definition to conchal bowl, intertragic
notch, and antitragal contour - Hyperpigmentation of skin grafts to conchal bowl
- Effacement of postauricular sulcus after
elevating framework results in decreased
projection - Caused by contraction of skin grafts
- Minimize with thicker skin grafts (prefer
full-thickness) or advancing postauricular skin
to depth of sulcus and grafting only posterior ear
26Microtia ReconstructionNagata Technique
- Two-stage technique introduced in 1993
- Technical refinements dependent on type of
microtia - Lobular
- Small concha
- Conchal
- Anotia
- Low hairline
- Reconstruction begins at age 10 years and at
least 60cm chest circumference
27Nagata TechniqueStage I
- Roughly encompasses first three Brent stages
- Rib cartilage framework with tragal component
housed in subcutaneous pocket - Lobule transposition
- Framework assembled with fine-gauge wire sutures
- Three floors correspond to three different
elevations of frame - Base cymba, cavum conchae
- Second level crus helicis, fossa triangularis,
scapha - Top level helix, antihelix, tragus, antitragus
(Walton, 2002)
28Nagata TechniqueStage I
- Ipsilateral costal cartilage of ribs 6-9
- Base 6th and 7th costal cartilages
- Helix and crus helicis 8th costal cartilage
- Superior and inferior crus and antihelix 9th
costal cartilage - Remaining structures carved from residual
cartilage - Most, if not all, perichondrium left intact
(Walton, 2002)
29Nagata TechniqueStage I
- Anterior lobule/tragal incision with removal of
2mm circular portion of skin - W-shaped posterior lobule incision
- Increases surface area to cover cartilage
construct - Allows lobule transposition
- Flaps undermined and reapproximated to form cup
of intertragal notch
2mm circular portion of skin
Anterior incision
Posterior W incision
Not elevated and left intact to augment blood
supply to flaps
Cup of intertragal notch
(Walton, 2002)
30Nagata TechniqueStage I
- Cartilage framework inserted and positioned
- Posterior flap advanced to anterior flap
- Inverted dog-ear formed from 2mm circular skin
defect creates incisura intertragica - Reassembling flaps in Z-plasty fashion transposes
lobule - Bolsters secured with mattress sutures and left
in place for 2 weeks
Excessive skin inverted to form pseudoacoustic
meatus
(Walton, 2002)
31Nagata TechniqueStage II
- Six months after Stage I
- Crescent-shaped piece of costal cartilage
- Harvested from fifth rib
- Wedged into position to elevate framework
- Serves as posterior conchal wall
(Walton, 2002)
32Nagata TechniqueStage II
Occipital scalp harvest site
- Temporoparietal fascial flap
- Raised through new scalp incision
- Tunneled subcutaneously to cover
- Posterior aspect of cartilage graft
- Reconstructed auricle
- Mastoid surface
- Retroauricular skin advanced followed by
split-thickness skin graft - Ultra-delicate
- Freehand harvest from occipital scalp
(Walton, 2002)
Temporoparietal fascial flap origin
33Nagata TechniquePreoperative and Postoperative
Stage I Completed
(Walton, 2002)
34Nagata TechniquePreoperative and Postoperative
Stage II Completed
(Walton, 2002)
35Nagata TechniqueCritique
- Provides a deeper and more natural conchal bowl
(no need to excavate subcutaneous tissue) - High rate of peri-lobular flap necrosis (14)
from vascular compromise - More cartilage harvested
- Significant anterior chest wall deformity
- Thicker ears
- High extrusion rate of cartilage framework from
wire sutures (8) - Frontal symmetry not addressed, would require
third stage
36Autogenous Cartilage GraftRisks and Benefits
- Advantages
- Native tissue
- Less infection
- Potential for 5 growth with time
- Disadvantages
- Cartilage may warp or resorb with time
- Framework extrusion with skin flap necrosis
- Cartilage donor effects
- Pneumothorax
- Atelectasis
- Chest wall deformity/scarring
- Low hairline
- Aesthetics
- Thicker skin with less contour
- Inflammation/infection
37Other Reconstructive Options
- Single-stage reconstruction
- Tissue expanders
- Osseo-integrated prosthesis
- Tissue engineering
- Alloplastic implant
- Silastic
- Medpor
38Other Reconstructive OptionsSingle-Stage
Reconstruction
- Used predominantly for partial defects (i.e.
superior helix or lobule) - Total ear reconstruction usually entails
- Skin flaps
- Fascial flaps
- External stents
- Results not comparable to multiple-staged
procedures - Two-flap, single-stage procedure has been
converted to a three-stage one (Park, 1997 and
2000)
39Other Reconstructive OptionsTissue Expanders
- Hata and Umeda (2000)
- Reconstruct auricle in single stage without skin
graft - Good skin texture and match
- Skin innervation preserved
- Park (2000)
- First Stage
- Expand skin and fascial layers
- Cartilage framework sandwiched between both
layers - Skin graft over fascial layer
- Second Stage
- Remove cartilage and shape structures from
framework - May need skin graft to posterior surface of frame
(Yang, 2009)
40Other Reconstructive OptionsTissue Expanders
- Pain
- Not well tolerated in young children
- Insertion of expander is a procedure number of
stages not truly reduced - Fibrous capsule formation around expander may
marginalize contouring results
(Yang, 2009)
41Other Reconstructive OptionsOsseo-Integrated
Prosthesis
- At least 3mm of bone to secure implant
- Indications for use
- Severe soft-tissue or skeletal hypoplasia
- Poor local tissue
- Cancer
- Radiation
- Failed autogenous reconstruction
- High operative risk factors
- Prosthesis changes
- Approximately 2-5 years with ultraviolet
degradation - Multiple prosthesis for seasonal skin tones
(Tollefson, 2006)
42Other Reconstructive OptionsOsseo-Integrated
Prosthesis
- Advantages
- Single, less involved procedure
- Best cosmetic outcome
- No resorption
- Minimal infection
- Inflammation around anchoring pins
- Mechanical trauma to pins
- Tissue overgrowth
- Disadvantages
- Repeat procedures (2,000-7000 per prosthesis)
- Mr. Potato Head social stigma (i.e. if ear
falls off) - Precludes future autogenous reconstruction
43Other Reconstructive OptionsTissue Engineering
- Potential to offer autogenous cartilage without
morbidity from rib harvest - Prefabricated framework
- Taut pressure from overlying skin can deform
frame - Firm alloplastic frames can withstand pressure
but extrude - Difficult to match size and shape of normal ear
- Requires replication of numerous human
chondrocytes to create adequate framework - Cao et al (1997)
- Bovine chondrocytes grown in vitro transplanted
onto synthetic ear-shaped biodegradable scaffold - Scaffold implanted into immunocompetent mouse
- New cartilage formation with human ear shape
after 12 weeks
44Other Reconstructive OptionsTissue Engineering
- Human-sized auricle with hydrogel scaffold in
pigs (Kamil, 2004) - Grow chondrocytes on perforated pure gold ear
mold (Kamil, 2004) - Harvest chondrocytes from microtic human ears in
mouse model (Kamil, 2004)
45Other Reconstructive OptionsSilastic Alloplastic
Implant
- Silicone implant investigated by Cronin (1966)
and Ohmori (1978) - Promising results initially
- Disappointing long-term outcomes
- Implant extrusion/resorption secondary to skin
flap erosion/necrosis - Minor trauma or abrasions can cause implant
failure - May occur years after operation
- Salvage can be done with local skin and
fascial flaps - Use has since been abandoned
46Other Reconstructive OptionsMedpor Alloplastic
Implant
- Prefabricated porous polyethylene auricular
implant - Excellent biocompatibility, stability, tissue
integration, and resistance to infection - Minimal tissue reaction
- Pore size 150µm allows soft-tissue ingrowth
improves stability - Can be bent when heated to 82-100ºC, carved to
shape, or affixed to other pieces with cautery - Lobule transposition can be
performed after three months - Bone-anchored hearing aid can be placed
concurrently (Romo, 2006 and 2009)
47Other Reconstructive OptionsMedpor Alloplastic
Implant
- Less extrusion rates compared to silicone with
use of temporoparietal fascial (TPF) flap to
cover framework - Lower 2/3 inserted in to skin pocket
- Upper portion covered with TPF and skin graft
- Flap must be water-tight
- Soft tissue flaps to treat early implant exposure
from flap ischemia - If exposure is less than 1cm
- If tissue integration is seen
- Good short-term results (2-year) but
long-term results still lacking
TPF Flap
Medpor Implant
(Tollefson, 2006)
48Other Reconstructive OptionsMedpor Alloplastic
Implant
- Dr. John Reinischs Five Main Advantages
- Good projection and definition
- Decreased reconstruction time
- No scarring from costal cartilage harvest
- Can perform in younger children (3-4 years)
- Shorter learning curve than autogenous cartilage
graft
49Other Reconstructive OptionsMedpor Alloplastic
Implant
- Advantages
- Improved cosmetic result compared to autogenous
cartilage graft - No resorption
- Low extrusion rate
- Disadvantages
- Not native tissue
- Infection risk with any cut
- Sensitive to trauma and direct contact
TPF Flap
Full-thickness skin graft over top 1/3 of Medpor
Y-shaped incision
Small vacuum drain
(Romo, 2009)
50Medpor Alloplastic ImplantFirst Stage
Reconstruction
- Scalp incisions
- Made to avoid junctions over arteries
- Perpendicular to direction of hair growth
Subcutaneous pedicle for blood supply to skin
over future conchal bowl
Template marked for implant position
Superficial temporal arteries identified with
Doppler
Long and wide TPF flap is needed to cover the
entire implant
(Reinish, 1998)
51Medpor Alloplastic ImplantFirst Stage
Reconstruction
Skin exposure flaps over fascia reapproximated to
protect tissue
Lobule mobilized and opened to fit over implant
rim
Postauricular skin harvested from contralateral
ear
(Reinish, 1998)
52Medpor Alloplastic ImplantFirst Stage
Reconstruction
Abdominal skin graft to cover harvest site
Antibiotic-covered bolster to cover the graft site
(Reinish, 1998)
53Medpor Alloplastic ImplantFirst Stage
Reconstruction
- Medpor pieces soaked in betadine
- Placed inside 60mL syringe
- Betadine suctioned against finger to drive
betadine into pores
(Reinish, 1998)
54Medpor Alloplastic ImplantFirst Stage
Reconstruction
Medpor pieces affixed with electrocautery or
non-absorbable suture
TPF flap lifted with enough to cover the entire
implant
(Reinish, 1998)
55Medpor Alloplastic ImplantFirst Stage
Reconstruction
TPF flap draped over implant
Two drains, one placed right under implant
- TPF flap must be watertight
- Skin flap will press against TPF and implant if
truly tight
(Reinish, 1998)
56Medpor Alloplastic ImplantFirst Stage
Reconstruction
Contralateral postauricular skin flap placed
anteriorly
Trim flaps as needed to keep abdominal flap
behind helical rim
Abdominal skin flap placed posteriorly
(Reinish, 1998)
57Medpor Alloplastic ImplantSecond Stage
Reconstruction
Excess tissue removed
Anteriorly based flap made near tragus
(Reinish, 1998)
58Medpor Alloplastic ImplantSecond Stage
Reconstruction
Part of parotid gland removed to make room for
conchal bowl
Anterior flap sutured over itself to make tragus
(Reinish, 1998)
59Medpor Alloplastic ImplantPreoperative
(Courtesy of Dr. Athre)
60Medpor Alloplastic ImplantIntraoperative
(Courtesy of Dr. Athre)
61Medpor Alloplastic ImplantPostoperative
(Courtesy of Dr. Athre)
62Conclusion
- Microtia can be associated with other congenital
abnormalities and adverse psychosocial effects - Surgical intervention can be delayed until the
child is older - Reconstruction with autogenous cartilage graft is
the gold standard - Alloplastic implants provide improved cosmetic
results with less staged procedures
(http//www.smbc-comics.com)
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