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which is due to premature fusion of cranial sutures and resultant
compensatory growth according to properties of Virchow law.
3. What is the most common craniofacial cleft.
Tessier cleft 7 resulting in ipsilateral microtia and macrostomia.
4. Describe the characteristic findings in syndromic
craniosynostosis.
See Section Syndromic Synostosis and Figure 16-3.
Recommended Readings
Czerwinski M, Hopper RA, Gruss J, Fearon JA. Major morbidity and mortality
rates in craniofacial surgery: an analysis of 8101 major procedures. Plast
Reconstr Surg. 2010;126(1):181‐186.
Czerwinski M, Kolar JC, Fearon JA. Complex craniosynostosis. Plast Reconstr
Surg. 2011;128(4):955‐961.
Fearon JA, Ruotolo RA, Kolar JC. Single sutural craniosynostoses: surgical
outcomes and long-term growth. Plast Reconstr Surg. 2009;123(2):635‐642.
Kaban LB, Padwa BL, Mulliken JB. Surgical correction of mandibular
hypoplasia in hemifacial microsomia: the case for treatment in early childhood.
J Oral Maxillofac Surg. 1998;56(5):628‐638. doi:10.1016/s0278-
2391(98)90465-7
Oh AK, Wong J, Ohta E, Rogers GF, Deutsch CK, Mulliken JB. Facial
asymmetry in unilateral coronal synostosis: long-term results after fronto-orbital
advancement. Plast Reconstr Surg. 2008;121(2):545‐562.
Smartt JM Jr, Reid RR, Singh DJ, Bartlett SP. True lambdoid craniosynostosis:
long-term results of surgical and conservative therapy. Plast Reconstr Surg.
2007;120(4):993‐1003.
Tessier P, Kawamoto H, Posnick J, Raulo Y, Tulasne JF, Wolfe SA. Taking
calvarial grafts, either split in situ or splitting of the parietal bone flap ex vivo—
tools and techniques: V. A 9650-case experience in craniofacial and
maxillofacial surgery. Plast Reconstr Surg. 2005;116(5 Suppl):54S‐71S;
discussion 92S-94S.
Warren SM, Proctor MR, Bartlett SP, et al. Parameters of care for
craniosynostosis: craniofacial and neurologic surgery perspectives. Plast
Reconstr Surg. 2012;129(3):731‐737.
*
Denotes common in-service examination topics.
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17
Reconstruction of Congenital
Ear Deformities
Chien-Wei Wang
EXTERNAL EAR EMBRYOLOGY AND
ANATOMY
Embryology of the External Ear
Auricle
Hillocks of His: six condensations of mesoderm that
develop into the auricle (Fig. 17-1)
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Figure 17-1 The auricle is formed
from six auricular hillocks, three
each from branchial arches I and II.
Surface anatomy of the auricle
(bottom right).
(From Johnson J. Bailey’s Head
and Neck Surgery. 5th ed. Wolters
Kluwer; 2014. Figure 146.1.)
Contribution from both 1st (mandibular) and 2nd
(hyoid) brachial arches
*1-3 anterior (1st brachial arch): tragus,
root of the helix, and helix.
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*4-6 posterior (2nd branchial arch):
antitragus, antihelix, and lobule. Lobule is
the last to develop.
Development time frame: begins at 6-8 weeks and is
fully formed by 4 months.
The auricle forms in the lower neck and migrates
cranially during mandibular development. Arrested
growth results in low-set ears.
External auditory canal (EAC)
Canalization of the meatal plug: ectodermal cells (1st
brachial cleft) degenerate at 28 weeks to form the EAC
Failure to canalize leads to congenital aural atresia
or stenosis.
Sigmoid shaped and ~2.5 cm in length and 1 cm in
diameter
Outer one-third: fibrocartilaginous
Inner two-thirds: bony
Tympanic membrane (ear drum)
Development: thin membrane formed from invagination
and meeting of the 1st brachial cleft (groove) with the
1st branchial pouch
Three layers: outer epithelial layer (ectoderm), middle
fibrous layer (mesoderm), and inner mucosal layer
(endoderm)
Two parts: pars flaccida (small, triangular, flaccid) and
pars tensa (large, oval-shaped, tense)
Separates external ear from middle ear
Surface Topographic Landmarks (Fig. 17-1)
Anatomy
Blood supply
Arterial supply from branches of external carotid artery
(Fig. 17-2A)
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Figure 17-2 A. Arterial anatomy of
the posterior ear. (The right ear is
shown.) The posterior auricular
artery anastomoses with the
superficial temporal artery via the
superior auricular artery. (From
Hanasono M. Postauricular flap for
ear reconstruction. In: Chung KC,
Disa JJ, Gosain A, eds. Operative
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Techniques in Plastic Surgery.
Wolters Kluwer; 2020:1133-1137.
Figure 3.30.1B.) B. The
auriculotemporal nerve innervates
the superior aspect of the external
ear, the lesser occipital nerve is
responsible for the mid posterior
external ear, and the great auricular
nerve innervates the inferior aspect
of the ear. (From McNabb JW,
O’Connor F. A Practical Guide to
Joint & Soft Tissue Injection.
Wolters Kluwer; 2022. Figure
7.12A.)
*Posterior auricular artery: dominant blood
supply to both the anterior (through
perforating branches) and posterior surfaces
of the ear
Superficial temporal artery: supplies the anterior
surface of the ear and forms numerous
interconnections with the posterior auricular artery,
allowing ear replantation based solely on either
arterial network
Occipital artery: supplies the posterior surface of
the ear (minor contributor) and the retroauricular
skin
Venous drainage: follows the feeding arteries and
empties into the retromandibular vein (external jugular
system)
Sensory innervation (Fig. 17-2B)
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Great auricular nerve (C2, C3)
Innervates: lower half of both anterior and
posterior surfaces of the ear
Landmarks
Erb point (6.5 cm below the tragus at the
posterior border of the SCM)
Runs posterior and parallel to the external
jugular vein
Auriculotemporal nerve (V3)
Innervates the upper half of the anterior surface of
the ear and the anterior portion of the EAC
Landmarks: ascends with the superficial temporal
vessels
Lesser occipital nerve (C2)
Innervates: upper half of the posterior surface of
the ear
Landmarks: Erb point and ascends along the
posterior border of sternocleidomastoid muscle
Auricular branch of the vagus nerve (X, Arnold nerve)
Innervates: the concha and the posterior portion of
the EAC
*A ring block will not provide adequate
anesthesia to the concha; direct local
infiltration is required
External auditory canal receives sensory innervation
from cranial nerves V, VII, IX, and X
Lymphatic drainage
Parallels embryologic development
The tragus, root of helix, and superior helix (1st
branchial arch) → parotid nodes
The antitragus, antihelix, and lobule (2nd branchial
arch) → cervical nodes
Vestigial musculature
Intrinsic muscles: helicis major and minor, tragicus,
antitragicus, and the transverse and oblique auricular
muscles
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Extrinsic muscles: anterior, posterior, and superior
auricular muscles
Clinical Measurements of the Normal Ear
Growth
Approximately 85% of ear growth is achieved by the
age of 3
Ear width reaches full size by the age of 10, while ear
height continues to grow into adulthood
*Near-adult size is routinely considered between
the ages of 6 and 7
Location
The ear is located roughly 6 cm, or a single ear-length,
posterior to the lateral orbital rim.
The superior aspect of the helix is at the level of the
lateral brow.
The inferior aspect of the lobule is at the level of the
nasal ala.
On frontal view, the helical rim is slightly lateral to the
antihelical fold.
Measurements
Normal auricular angles
The long axis of the ear is inclined posteriorly 20°.
Auriculocephalic angle: the angle formed
between the midpoint of the lateral helix and the
mastoid bone (normally between 20° and 30°).
Conchoscaphal angle: the angle formed between
the scapha and the concha (normally <90°).
Conchomastoid angle: the angle formed
between the concha and the mastoid (normally
about 90°).
Normal auricular projections
Upper third: 10-12 mm
Middle third: 16-18 mm
Lower third: 20-22 mm
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RECONSTRUCTION OF
CONGENITAL EAR DEFORMITIES
Microtia
Epidemiology
Incidence: 1 in 6000 births.
Prevalence: higher in Hispanics, Asians, and Native
Americans.
Male to female ratio is 2:1.
*Right/left/bilateral ratio of 6:3:1.
Etiology: poorly understood
Most cases are isolated and sporadic.
Proposed causes: ischemia (eg, acute vascular
obstruction), drugs (eg, thalidomide, isotretinoin,
retinoic acid), and infection (eg, rubella).
Commonly associated syndromes: hemifacial
microsomia, Goldenhar syndrome, and TreacherCollins syndrome.
Based on embryologic development, the inner ear is
often spared, but defects of the external auditory canal
and middle ear are common.
Hearing loss is predominantly due to atresia or
stenosis of the external auditory canal but can
result from the absence or structural abnormalities
of the ossicular chain.
Conductive component (80%) is more prevalent
than a sensorineural component (20%).
Classification
Many classification systems have been proposed, but
they are rarely clinically useful.
Marx classification: based on severity of the microtia
deformity.
Nagata classification: most commonly used; based on
vestigial structures present
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Lobular type: microtic ears have a remnant ear
and lobule but lack a concha, acoustic meatus,
and tragus (correspond to grade III).
Conchal type: microtic ears possess some
degree of a lobule, concha, acoustic meatus, and
tragus (correspond to grade II).
Small conchal type: microtic ears contain the
remnant ear and lobule with a small indentation for
a concha.
Anotia: absence of auricular tissue (correspond to
grade IV).
Timing of repair
*General rule of thumb: delayed until ear reaches
near-adult size for optimal result
Usually around age of 6-7
Wait until the patient (not the parents) requests
surgery
Self-awareness of the ear deformity
Ability to participate postoperative care and
comply with restrictions
Different techniques have different age requirements
for optimal reconstructive outcomes
Brent technique: performed at the age of 6 (ear
maturity)
Nagata technique: performed at the age of 10
when patient’s chest circumference is at least 60
cm at the level of xiphoid process (need of
additional cartilage for tragal reconstruction)
If there is presence of conductive hearing loss,
coordination of operative care with an otolaryngologist
is paramount
Unilateral microtia with normal contralateral
hearing: bone-anchored hearing aids (BAHAs) and
middle ear reconstruction are not routinely
required.
*If contralateral conductive hearing loss is
present: BAHA placement should be deferred
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