Phẫu Thuật Tạo Hình Grabb & Smith’s (Bản Cập Nhật – Kevin C. Chung)

Soft Tissue Involvement
Soft tissue involvement includes the middle third of the upper eyelid and central eyebrow. Eyelid
deficiencies are common, ranging from colobomas to complete ablepharon. A paramedian forehead soft
tissue furrow may extend to a frontal hair projection in the temporoparietal scalp (see Figure 32.8).
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Skeletal Involvement
Skeletal involvement begins at the superior orbit, lateral to the supraorbital foramen, and extends
cephalad. An encephalocele may develop through the anterior cranial fossa resulting in inferior-lateral
displacement of the orbit and hypertelorism.
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No. 11 Cleft
The no. 11 cleft is the cranial extension of the no. 3 facial cleft. 17
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Soft Tissue Involvement
This cleft originates in the medial orbit, and as such, the medial upper lid is often involved. Similar to a
no. 10 cleft, eyelid involvement may range from a coloboma to ablepharon. The cleft may extend into the
eyebrow and up to the frontal hairline with a fingerlike projection of hair extending into the medial third
of the forehead (Figure 32.9).
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FIGURE 32.9 No. 11 cleft. Patient with right-sided no. 11 cleft demonstrating involvement of the medial third of the upper lid,
canthal involvement, and orbital malposition.
(Reproduced from Losee, JE, Kirschner, RE. Rare craniofacial defects. In: Comprehensive Cleft Care. Vol 2. 2nd ed. New York, NY: Thieme Medical
Publishing; 2016
.)
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Skeletal Involvement
The bony involvement of the no. 11 cleft involves the medial third of the orbital rim and may extend
cephalad similarly to a no. 10 cleft. If the ethmoid sinus is involved, significant hypertelorism can be seen.
The cranial base and anterior cranial fossa are uninvolved.
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No. 12 Cleft
The no. 12 cleft is the cranial extension of the no. 2 facial cleft. 18
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Soft Tissue Involvement
The soft tissue cleft lies medial to the medial canthus, which is laterally displaced and extends cephalad
to the medial eyebrow with effacement of the eyebrow root. A short anterior projection of the hairline
occurs just lateral to midline.
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Skeletal Involvement
The bony component of the no. 12 cleft originates from the frontal process of the maxilla and extends
superiorly. The ethmoid sinuses are widened which contributes to hypertelorism and telecanthus. The
anterior and middle cranial fossa may be widened but encephaloceles are uncommon. The cribriform
plate and olfactory groove are unaffected.
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No. 13 Cleft
The no. 13 cleft represents the paramedian cranial extension of the no. 1 facial cleft.
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Soft Tissue Involvement
The eyelid and eyebrow are intact in this cleft; however, the medial brow may be inferiorly displaced. A
frontal encephalocele may be present, along with a paramedian V-shaped frontal hair projection (Figure
32.10).
FIGURE 32.10 No. 13 cleft. A. Skeletal involvement occurs between the nasal bone and frontal process of the maxilla and is
associated with widened ethmoids and hypertelorism. B. Patient demonstrating right-sided no. 13 cleft with preservation of the
lid and canthal structures, orbital dystopia, and frontal soft tissue and bony abnormalities.
(B. Reproduced from Losee, JE, Kirschner, RE. Rare craniofacial defects. In: Comprehensive Cleft Care. Vol 2. 2nd ed. New York, NY: Thieme Medical
Publishing; 2016
.)
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Skeletal Involvement
This cleft originates from between the nasal bone and frontal process of the maxilla where an
encephalocele may be present leading to cribriform displacement and vertical ocular dystopia. A classic
finding of the no. 13 cleft is the involvement of the cribriform plate, leading to widening of the olfactory
groove, cribriform plate, and ethmoid sinuses. Especially when bilateral, this involvement can produce
some of the most severe forms of hypertelorism encountered.
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No. 30 Cleft
The no. 30 cleft is exceedingly rare with few cases reported. 19 It presents as an extension of the no. 0 cleft
inferiorly into the mandible and cervical structures (Figure 32.11) and can range in presentation.
FIGURE 32.11 No. 30 cleft. Patient demonstrating a no. 30 cleft with bony and soft tissue deficiency.
(Reproduced from Losee, JE, Kirschner, RE. Rare craniofacial defects. In: Comprehensive Cleft Care. Vol 2. 2nd ed. New York, NY: Thieme Medical
Publishing; 2016
.)
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Soft Tissue Involvement
In microforms/mild forms, a lower lip notch may be the only sign of a no. 30 cleft. In more severe cases,
the lip and chin may be completely cleft, with the addition of a bifid tongue, ankyloglossia, or tongue
agenesis. There may be diastatic or atrophic cervical strap muscles with scarring and neck contracture.
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Skeletal Involvement
Skeletal involvement includes an alveolar cleft between the mandibular central incisors that extends into
the symphysis. The hyoid bone may be clefted, or absent, as well as the thyroid cartilage and rarely the
sternum.
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Orbital Hypertelorism
OHT is an uncommon malformation which results in an abnormally wide spacing of the orbits, eyes, and
periorbita. 20 It may be isolated, associated with a craniofacial cleft or encephalocele as detailed above, or
one of a variety of findings in any of several craniofacial syndromes. It is important to distinguish
hypertelorism from telecanthus (or pseudohypertelorism). Telecanthus is the widening of the intercanthal
distance often a result of medial canthal tendon avulsion or nasoorbitalethmoid fracture. In telecanthus,
the bony orbits are in a normal position. In contrast, the hyperteloric orbit is entirely displaced and the
bony distance between the orbits (interdacryon distance) is increased. In addition to the horizontal vector,
orbits can be displaced vertically (orbital dystopia), anteriorly or posteriorly, and rotationally. It is crucial
to identify the complete extent of orbital malposition to assist in the diagnosis and correctly identify the
most appropriate treatment modality.
The classification of hypertelorism is based on the interdacryon distance, sometimes referred to the
interorbital distance, which is defined as the distance between the junction points of the contralateral
lacrimal bones, frontal processes of the maxilla, and frontal bones. 21 This measurement is best made
radiographically or intraoperatively; however, a rough approximation can be made clinically by
subtracting 4 to 6 mm from the caliper measurement to account for the soft tissue. In an adult, the average
interdacryon distance is 25 mm in a female and 28 mm in a male. First-degree hypertelorism is defined as an
interdacryon distance of 30 to 34 mm, second degree is defined as 34 to 40 mm, and third degree is defined as
an interdacryon distance of greater than 40 mm. 22,23 In contrast, in a growing child, the distance between
the orbits changes over time. Table 32.2 demonstrates the normal interdacryon distance at varying times
during development. In children, first-degree hypertelorism is defined as an interdacryon distance 2 to
4 mm greater than normal, second degree is 4 to 8 mm greater than normal, and third degree is more than
8 mm greater than normal (Table 32.2).
TABLE 32.2
Normative Interdacryon (Interorbital) Distance by Age
Age (years)
Interdacryon Distance (mm)
1
18.5
2
20.5
3
21
5
22
7
23 ± 4
10
25 ± 2
12
26 ± 1
Craniofrontonasal dysplasia is a unique inherited form of hypertelorism associated with the X-linked
EFNB1 gene. 24 It occurs in both inherited and sporadic forms; the typical manifestations include
unicoronal or bicoronal craniosynostosis, hypertelorism, bifid or absent nasal tip, widow’s peak, and wiry
hair. Additionally, patients may have cleft lip and/or palate and limb anomalies including syndactyly.
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Principles of Treatment
Craniofacial Clefts
Each patient with a craniofacial cleft is unique and should be treated with an individualized plan based
on the specific anomalies, functional issues, and social situation. It is helpful to break down various
treatment modalities based on the ideal age at which they are performed. For patients with frontonasal
dysplasia, frontoethmoidal encephaloceles, or any of the medial cranial clefts (nos. 11–14), early
intervention may be warranted to reduce and separate the intracranial compartment from the paranasal
sinuses. To avoid the dangerous sequelae of an ascending CNS infection such as meningitis, surgery
should be performed as soon as the patient is old enough to tolerate the procedure (>3 months).
Additionally, patients with significant ocular involvement including eyelid deficiency or displacement
may require earlier intervention if eye lubrication is insufficient to prevent corneal damage.
Soft tissue reconstruction of isolated perioral clefts (nos. 0–7) can be performed at 3 to 6 months of age,
similar to repairing a common cleft lip deformity. Similarly, palatal involvement should be considered
around 9 to 12 months of age to maximize potential for speech development. Nasal anomalies can be
treated at the time of perioral cleft repair; however, patients with frontonasal dysplasia and disorganized
accessory nasal cartilage may benefit from surgery at an older age to minimize growth restriction. Medial
orbital (nos. 3/11 and 4/10) clefts may require dacryocystorhinostomy if symptomatic.
Bony anomalies should be reconstructed in a delayed manner unless causing functional impairment. In
general, cranial bone grafts are necessary to reconstruct the orbits, facial buttresses, and nose, whereas
cancellous iliac crest bone grafts can be used to treat persistent alveolar clefts. Repair of orbital dystopia
and hypertelorism should be avoided before school age and preferably performed at orbital maturity (6–
9 years of age).
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Hypertelorism
Hypertelorism is not a diagnosis. Rather, it is a physical finding which can occur in a range of
abnormalities. When determining the correct treatment, it is crucial to consider patient’s overall diagnosis
as well as other pertinent physical findings. For example, hypertelorism secondary to Apert syndrome
should be treated in a completely different manner than that caused by a no. 0/14 cleft.
When hypertelorism occurs, treatment should focus on safely decreasing the interorbital distance to less
than 17 mm by performing a box osteotomy or a facial bipartition. Although these two operations both
reduce the interdacryon distance, they differ significantly in the degree of other facial changes they
produce. 25–28 A box osteotomy is limited to repositioning the orbits and the zygomas and can effectively
narrow the interorbital distance while preserving midfacial and occlusal relationships. It is performed by
mobilizing the orbits via a coronal incision, intracranial exposure, and orbital osteotomies in all four
walls. Conversely, a facial bipartition procedure is ideal for patients who, in addition to OHT, also have
maxillary hypoplasia, a narrow maxillary arch, and malocclusion. The bipartition requires mobilizing the
orbits laterally and superiorly and by removing a central wedge of bone from the crista galli extending
inferiorly to include the central frontal bone, ethmoids, nasal bones, and nasal septum. A central
maxillary osteotomy/ostectomy is made inferior to the piriform and extending to the alveolus to complete
the separation of the facial skeleton into two pieces. As the interorbital distance is narrowed, the lower
facial skeleton is widened. In both procedures, rigid fixation with titanium miniplates are used to secure
the bone segments, medial canthopexies are required to resuspend the soft tissue, and cantilevered bone
grafts are used to maintain nasal projection. 29
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Questions
1. The proper assessment of hypertelorism most appropriately includes measurement of which of the
following?
a. Distance between lacrimal crests
b. Distance between medial canthi
c. Distance between pupils
d. Distance between lateral canthi
e. Distance between zygomaticofrontal sutures
2. A one-year-old presents with brachycephaly, bilateral superior orbital rim retrusion with an
interorbital distance of 28 mm, and a wide nasal root. Mutation in which of the following genes is
associated with these findings:
a. FGFR2
b. EFNB1
c. IRF6
d. TWIST
e. TCOF1
3. After cleft lip, the most common craniofacial cleft is:
a. no. 2 cleft
b. no. 15 cleft
c. no. 7 cleft
d. no. 11 cleft
4. Which of the following disorders is not associated with a 
 craniofacial cleft?
a. Hemifacial microsomia
b. Treacher Collins syndrome
c. Cleft lip/palate
d. Ethmocephaly
e. Nasofrontal encephalocele
1. Answer: a. OHT is a lateral displacement of the orbits with an increase in the interorbital distance.
In patients with OHT, all of the distances in the answer choices are increased; however, OHT is
defined by the bony widening between the eyes best measured by the interdacryon distance, or the
distance between the anterior lacrimal crests. Pseudohypertelorism, or telecanthus, can be seen after
nasoorbitalethmoid fractures and results in an isolated widening of the medial canthi without lateral
displacement of the globes. In skeletally mature patients, first-degree hypertelorism is defined as an
interdacryon distance greater than 30 mm.
2. Answer: b. The patient described has bicoronal craniosynostosis and hypertelorism (defined as an
interorbital difference of greater than a standard deviation over the age-corrected mean; 18.5 mm in a
1-year-old) and nasal anomalies. These findings are typical of patients with craniofrontonasal
dysplasia, an X-linked disorder associated with mutations in the EFNB1 gene. Additionally, patients
may have cleft lip/cleft palate, curly hair, and syndactyly. FGFR2 is associated with Crouzon, Apert,
and Pfeiffer syndromes. IRF6 is the mutation associated with van der Woude syndrome, TCOF1 is
associated with Treacher Collins syndrome, and TWIST is associated with Saethre-Chotzen
syndrome. None of these syndromes is associated with the constellation of symptoms described
earlier.
3. Answer: c. The Tessier rare craniofacial cleft classification describes both bony and soft tissue
clefts. The Tessier no. 7 cleft describes a soft tissue cleft which starts at the lateral oral commissure
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and extends to varying extents toward the tragus. It is the most common of the Tessier clefts and is
associated with multiple craniofacial syndromes including Treacher Collins syndrome and
hemifacial microsomia.
4. Answer: c. Hemifacial microsomia is often associated with a no. 7 (lateral commissure) cleft.
Treacher Collins syndrome is associated with no. 6, 7, and 8 clefts. Ethmocephaly is associated with
midline craniofacial hypoplasia and no. 14 cleft. Craniofrontonasal dysplasia and nasofrontal
encephaloceles are associated with no. 14 or 0/14 clefts. The Tessier (rare craniofacial) cleft
classification is not used to describe common facial clefts such as those that occur in unilateral or
bilateral cleft lip and/or palate.
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References
Wolf, SA. A Man from Héric: The Life and Work of Paul Tessier, MD, Father of Craniofacial Surgery. Lulu Enterprises; 2012.
Tessier, P. Anatomical classification of facial, craniofacial, and latero-facial clefts. 
 J Maxillofac Surg. 1976;4:69.
Kawamoto, HK Jr. The kaleidoscopic world of rare craniofacial clefts: order out of chaos (Tessier classification). Clin Plast Surg. 1976;3(4):529-
572.
Allam, KA, Wan, DC, Kawamoto, HK, et al. The spectrum of median craniofacial dysplasia. Plast Reconstr Surg. 2011;127(2):812-821.
Gaball, CW, Yencha, MW, Kosnik, S. Frontonasal dysplasia. Otolaryngol Head Neck Surg. 2005;133(4):637-638.
Guion-Almeida, ML, Richieri-Costa, A, Saavedra, D, Cohen, MM. Frontonasal dysplasia: analysis of 21 cases and literature review. Int J Oral
Maxillofac Surg. 1996;25(2):91-97.
Jones, MC. Etiology of facial clefts: prospective evaluation of 428 patients. Cleft Palate J. 1988;25(1):16-20.
Resnick, JI, Kawamoto, HK. Rare craniofacial clefts: Tessier no. 4 clefts. Plast Reconstr Surg. 1990;85(6):843-849.
Stark, RB, Saunders, DE. The first branchial syndrome. The oral-mandibular-auricular syndrome. Plast Reconstr Surg Transpl Bull.
1962;29:229-239.
Longacre, JJ. The surgical management of the first and second branchial arch syndrome. Br J Plast Surg. 1965;18:243-253.
Converse, JM, Coccaro, PJ, Becker, M, Wood-Smith, D. On hemifacial microsomia: the first and second branchial arch syndrome. Plast
Reconstr Surg. 1973;51(3):268-279.
Grabb, WC. The first and second branchial arch syndrome. Plast Reconstr Surg. 1965;36(5):485-508.
Limaye, SR. Coloboma of the iris and choroid and retinal detachment in oculo-auricular dysplasia (Goldenhar syndrome). Eye Ear Nose
Throat Mon. 1972;51(10):384-386.
Raulo, Y, Tessier, P. Mandibulo-facial dysostosis: analysis; principles of surgery. Scand J Plast Reconstr Surg. 1981;15(3):251-256.
Roddi, R, Vaandrager, JM, van der Meulen, JC. Treacher Collins syndrome: early surgical treatment of orbitomalar malformations. J Craniofac
Surg. 1995;6(3):211-217.
Halal, F, Herrmann, J, Pallister, PD, et al. Differential diagnosis of Nager acrofacial dysostosis syndrome: report of four patients with Nager
syndrome and discussion of other related syndromes. Am J Med Genet. 1983;14(2):209-224.
Bodin, F, Salazard, B, Bardot, J, Magalon, G. Craniofacial cleft: a case of Tessier no. 3, 7 and 11 cleft. J Plast Reconstr Aesthet Surg.
2006;59(12):1388-1390.
Ozek, C, Gundogan, H, Bilkay, U, et al. Rare craniofacial anomaly: Tessier no. 2 cleft. J Craniofac Surg. 2001;12(4):355-361.
Ladani, P, Sailer, HF, Sabnis, R. Tessier 30 symphyseal mandibular cleft: early simultaneous soft and hard tissue correction: a case report. J
Craniomaxillofac Surg. 2013;41(8):735-739.
Tan, ST, Mulliken, JB. Hypertelorism: nosologic analysis of 90 patients. Plast Reconstr Surg. 1997;99(2):317-327.
Tessier, P. Orbital hypertelorism. I. Successive surgical attempts. Material and methods: causes and mechanisms. Scand J Plast Reconstr Surg.
1972;6(2):135-155.
Costaras, M, Pruzansky, S, Broadbent, BH. Bony interorbital distance (BIOD), head size, and level of the cribriform plate relative to orbital
height: I. Normal standards for age and sex. J Craniofac Genet Dev Biol. 1982;2(1):5-18.
Costaras, M, Pruzansky, S. Bony interorbital distance (BIOD), head size, and level of the cribriform plate relative to orbital height: II. Possible
pathogenesis of orbital hypertelorism. J Craniofac Genet Dev Biol. 1982;2(1):19-34.
Wolfswinkel, EM, Weathers, WM, Correa, B, et al. Craniofrontonasal dysplasia: variability of the frontonasal suture and implications for
treatment. J Craniofac Surg. 2013;24(4):1303-1306.
Marchac, D, Sati, S, Renier, D, Deschamps-Braly, J, Marchac, A. Hypertelorism correction: what happens with growth? Evaluation of a series
of 95 surgical cases. Plast Reconstr Surg. 2012;129(3):713-727.
Kawamoto, HK, Heller, JB, Heller, MM, et al. Craniofrontonasal dysplasia: a surgical treatment algorithm. Plast Reconstr Surg.
2007;120(7):1943-1956.
McCarthy, JG, La Trenta, GS, Breitbart, AS, et al. Hypertelorism correction in the young child. Plast Reconstr Surg. 1990;86(2):214-225.
Tessier, P, Guiot, G, Derome, P. Orbital hypertelorism. II. Definite treatment of orbital hypertelorism (OR.H.) by craniofacial or by
extracranial osteotomies. Scand J Plast Reconstr Surg. 1973;7(1):39-58.
Wan, DC, Levi, B, Kawamoto, H, et al. Correction of hypertelorbitism: evaluation of relapse on long-term follow-up. J Craniofac Surg.
2012;23(1):113-117.
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C H A P T E R 3 3
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Ear Reconstruction
Akira Yamada and Arun K. Gosain
Key Points
The understanding of precise location, dimension, and proportion of the normal auricle is the
foundation for optimal ear reconstruction.
There are four principles in ear reconstruction: proper ear location, well-vascularized thin skin
envelope, anatomical 3D framework, and beautiful curves.
Team approach to address both hearing and external ear shape from infancy to adulthood will give
patients optimal integration to the society.
Timely ear molding less than 3 months of age, ideally less than 1 month of age, is a powerful option
for miscellaneous ear anomalies to obviate the need for surgery.
Current three major options for microtia reconstruction are autogenous rib reconstruction, Medpor
reconstruction, and prosthesis. Surgeons should explain to patients the pros and cons of each option.
Precise 3D framework is the essential foundation for total auricular construction.
Secondary ear reconstruction may require total replacement of 3D framework, new skin flap/fascial
flap coverage, and correction of the ear location.
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The Normal Auricle
Anatomy
The auricle is difficult to reproduce surgically because it is made up of a complexly convoluted frame of
delicate elastic cartilage surrounded by a thin skin envelope. Like spiral architecture in the inner ear, the
external ear also has a spiral architecture, connecting the basal layer to the top layer. The auricle’s rich
vascular supply comes from the superficial temporal anteriorly and posterior auricular vessels
posteriorly. The sensory supply to the auricle is mainly derived from the inferiorly coursing greater
auricular nerve (C2-C3). Upper portions of the auricle are supplied by lesser occipital (C2-C3) and
auriculotemporal nerves (V3) (tragus and crus helicis), whereas the concha region is supplied by a branch
of vagal nerve. Arnold nerve (CN 7,9,10) is an auricular branch of vagus nerve that receives contributions
from facial nerve and glossopharyngeal nerve. Arnold nerve supplies posterior inferior external auditory
canal and meatus, and inferior conchal bowl. This nerve mediates clinical phenomena, such as otalgia.
Lymphatic drainage correlates with six embryonic hillocks. The tragus, root of the helix, and superior
helix arise from first branchial arch (anterior hillocks 1–3) and drain into parotid nodes. The antihelix,
antitragus, and lobule arise from second branchial arch (posterior hillocks 4–6) and drain into cervical
nodes.
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Embryology
The auricle begins to protrude from the developing face at approximately 3 to 4 months of gestation
(Figure 33.1). The auricle arises from two branchial arches: mandibular branchial arch (first) and hyoid
branchial arch (second). First branchial arch (anterior hillocks 1–3) contributes the tragus, root of helix,
and superior helix (upper third of auricle). Second branchial arch (posterior hillocks 4–6) contributes the
rest (antihelix, antitragus, lobule) (lower two-thirds of auricle).

FIGURE 33.1 Embryology of the external ear. A. Hillock formation in an 11-mm human embryo. B. Hillock configuration in a
15-mm embryo at 6 weeks of gestation. C. Adult auricle depicting hillock derivations.
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Location and Dimensions
The auricle is located one ear length posterior to the lateral orbital rim, with superior pole level with brow
and inferior pole level with alar base. Vertical height of an adult auricle is approximately 55 to 65 mm.
Width is approximately 50% to 55% of its length 1 (Figure 33.2). Lateral protrusion of the helix is 1 to 2 cm
from the scalp. The long axis tilts posteriorly 15° to 20°. Projection from mastoid to helix: superior 10 to
12 mm; middle 16 to 18 mm; and inferior 20 to 22 mm. The normal auricle is located immediately behind
the face mask. Surgeons should avoid placing the new auricle inside the face mask: This is especially
important in hemifacial microsomia because the vestige lobule tends to be located anteriorly and
inferiorly and may be located inside the face mask. An anthropological study shows that the auricle is
normally located approximately 20 mm behind the sideburn. 2 There is a trapezium-shape nonhair-
bearing skin space, between the sideburn and the auricle in normal anatomy (Figure 33.3). Avoiding
placing ear framework in this preauricular trapezium is important to prevent anterior inclination of the
auricle.
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FIGURE 33.2 The normal location and dimensions of the auricle, in relation to the surrounding facial anatomy. FH, Frankfurt
horizontal line.
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FIGURE 33.3 Nagata-type microtia construction: A. Preoperative view of an 11-year-old girl with lobule-type microtia. B.
Harvested ipsilateral six to nine rib cartilage. Note the color of cartilage is white, meaning the perichondrium is completely left
intact at the donor site for regeneration. C. 3D Framework with 52 mm type A2 template. D. Appearance at second-stage
surgery. Temporoparietal fascia flap is raised, and the cartilage block is fabricated according to thermos-elastic template. E.
The final view of the patient 1 year after surgery.
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Miscellaneous Ear Anomalies
Cryptotia
Cryptotia is a congenital ear deformity in which upper pole of ear cartilage is buried underneath the
scalp. The superior auriculocephalic sulcus is absent but can be demonstrated when you pull up the
helical pole. Various surgical corrections are reported from Japan, due to the high prevalence of cryptotia,
as frequently as 1:400. Nonsurgical ear-molding treatment may be applied if the child is in early neonate
stage. The goal of surgical treatment is to create the retroauricular sulcus by skin grafts, Z-plasty, V-Y
advancement, or rotation flap. 3 Common cartilage deformity associated with cryptotia is helix-scapha
adhesion, which may be addressed by cartilage remodeling techniques.
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Stahl Ear
Stahl ear, a rare congenital auricular deformity, is characterized by the third crus extending toward the
helical rim. Stahl ear is classified into three types:
Type 1: Obtuse-angled bifurcation of antihelix; looks as though superior crus is missing
Type 2: Trifurcation of antihelix
Type 3: Broad superior crus and broad third crus (protruded scaphoid fossa)
Ear molding may work well if ear molding is started in early infancy. Surgical treatment is broadly
categorized into two types: cartilage/skin excision and cartilage alteration. Type 1 Stahl ear needs special
attention, to reconstruct missing superior crus, by using excised third crus or rib cartilage graft or creating
superior crus by sutures or cartilage cutting.
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Constricted Ear
Constricted ear is a concept proposed by Tanzer in 1975, 4 and his classification is shown in Table 33.1. In
constricted ear, helix and scapha fossa are hooded, and crura of antihelix is flattened in various degrees.
One gains an impression that the rim of helix has been tightened. Constricted ear is often referred as cup
or lop ear. Tanzer classified constricted ear into three groups based on the severity of defect/deformities.
Tanzer group 1: Mild deformities of helix, often called lop ear. Defect involves helical cartilage with
minimum skin defect. Musgrave technique is a useful method to expand the helix. Through either
anterior or posterior skin incision, multiple cuts were made to the curled cartilage, fan upward and
backward, fixed to the curved strut made of concha cartilage graft. The skin is then redraped across
the reconstructed framework. For milder constricted ear (group 1,2A), focusing surgical correction to
construct helical curve is the reasonable option while keeping the original elastic cartilage
framework, avoiding hard rib cartilage framework. When superior crus is deficient, partial helix plus
superior crus frame from rib cartilage 5 can normalize the deformity.
Tanzer group 2B: Has both skin and cartilage defect in the upper one-third of the auricle. The loss of
folding may involve antihelical crura, and hooding is more pronounced. The height of the ear is
sharply reduced. Park 6 proposed versatile solution for group 2B constricted ear. For helical skin
defect, Park modified the Grotting flap (postauricular flap), creating both skin flap and fascia flap
with the same pedicle. For helical cartilage defect, eight rib cartilage is harvested, the helix is
fabricated, and the entire length of helix is constructed.
Tanzer group 3: Most severe cupping and failure of migration. Brent recommends to treat severe
constricted ear as if it is a form of microtia, when the construction is severe enough to produce a
height difference of 1.5 cm. Nagata recommends treating severe constricted ear as a concha-type
microtia, to replace the defective framework with a full rib cartilage framework. 7
TABLE 33.1
Tanzer Classification of Constricted Ear
Group
Description
I
Involvement of helix only
II
Involvement of helix and scapha
IIA
No supplemental skin needed at margin of auricle
IIB
Supplemental skin needed at margin of auricle
III
Extreme cupping deformity; often associated with incomplete migration, forward title, stenosis of external auditory canal, and
deafness
(From Tanzer, RC. The constricted ear (cup and lop) ear. Plast Reconstr Surg. 1975;55:406.)
Based on the degree of defects, for both skin defects and cartilage defects, the authors’ recommendations
for treatment are summarized in Figure 33.4.
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FIGURE 33.4 Recommended strategy for constricted ear. A. Skin augmentation strategy based of the tissue defect severity. B.
Cartilage modification strategy based on the severity of defect.
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Ear Molding in Miscellaneous Ear Anomalies
Matsuo, who first reported ear molding treatment for congenital ear deformities, states that when the ear
deformities are not hypoplastic, nonsurgical correction is easy and reliable. 8 Stahl ear responds well to
the nonsurgical correction only during the neonatal period, whereas protruding ears and cryptotia
respond until approximately 6 months of age (Matsuo). It is widely believed that the early initiation of
molding is more effective because maternal estrogen in the neonate keeps ear cartilage soft and elastic.
Most agree that if ear molding is started after 3 months of age, the response tends to be poor. Helix-
antihelix adhesion responds poorly to the ear molding treatment and may not be the indication of the ear
molding. Skin irritation is probably the most frequent complication, possibly due to tape or adhesive.
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Microtia
Epidemiology and Pathophysiology
Microtia (small ear) is a congenital condition with unknown cause. Prevalence of microtia varies
significantly among ethnic groups (0.83–17.4 per 10,000 births) and is higher in Asian countries for
unknown reason. 80% to 90% of microtia is unilateral, and 10% to 20% is bilateral. There are more than 18
different microtia-associated syndromes with single-gene or chromosomal aberrations; however, there is
no causal genetic mutation confirmed to date. A relatively common syndrome associated with microtia is
hemifacial microsomia and Treacher-Collins syndrome. Isolated microtia rarely run in families. Treacher-
Collins syndrome, inherited in an autosomal dominant fashion, often presents with bilateral microtia.
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Classification
Many attempts have been made to classify microtia based on embryogenic development and severity of
the deformities. Nagata’s classification is based on surgical correction of the deformity 9–11 :
Anotia: Absence of auricular tissue
Lobule type: Vestige ear with lobule, without concha, acoustic meatus, and tragus
Concha type: Vestige ear with lobule, concha, acoustic meatus, and tragus
Small concha type: Vestige lobule with small indentation of concha (need lobule-type construction)
Atypical microtia: Cases do not fall into previous categories
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Patient Assessment and Workup
About 20% to 60% of children with microtia have associated anomalies or an identifiable syndrome;
therefore, individuals with microtia should be examined for other dysmorphic features. Microtia is a
common feature of craniofacial microsomia, mandibular dysostoses (eg, Treacher-Collins and Nager
syndromes), and Townes-Brocks syndrome, and these conditions should be considered among the
differential diagnosis when evaluating an individual with microtia. If there is family history of syndrome,
genetic counseling may be necessary.
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Physical Examination
Identify the type of microtia, size, dimension, and type of normal auricle, if unilateral. Normal side may
not be normal and may have subtle ear deformity such as helix-antihelix adhesion. Evaluate the
symmetry of face, facial animation (partial facial paralysis is frequent finding), and dental occlusion.
Hemifacial microsomia is often associated with difficult airway for intubation.
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Diagnostic Studies
Audiologic testing to determine conductive versus sensorineural defect
Temporal bone imaging
High-resolution CT scan for evaluating middle ear ossicles to assess the possibilities of future
otologic surgery
MRI to determine the course of facial nerve, often displaced, especially in the absence of
pneumatized mastoid
Rule out the presence of cholesteatoma (squamous epithelium trapped in the middle ear), present in
4% to 7% of atresia
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Atresia and Middle Ear Anomalies
In bilateral microtia, early and conscientious use of bone-conductive hearing aid is imperative for hearing
and speech development. Most of hearing deficits in children with bilateral microtia are managed with
hearing aids. Treatments of microtia ideally involve reconstruction of the external ear and the restoration
of normal hearing. 12 Hearing impairment in microtia is related to abnormal auditory canal, tympanic
membrane, and middle ear. The problem is conduction. Typically, microtia patients have a hearing
threshold of 40 to 60 dB on the affected side. By comparison, normal function allows us to hear sounds
between 0 and 20 dB. Regarding middle ear surgery for hearing restoration, most surgeons presently feel
that potential gains from middle ear surgery in unilateral microtia are outweighed by the potential risks
and complications for the surgery, and this surgery should be reserved for bilateral cases. Careful
selection of the atresia surgery candidate is important to achieve optimal outcome and more importantly
to avoid unnecessary surgery and its complications. Jahrsdoerfer criteria are a widely accepted guideline
to select atresia surgery candidates. 13 The bone-anchored hearing aid (BAHA; Cochlear, Mölnlycke,
Sweden; and Ponto; Oticon, Kongeballen, Denmark) has been used since 1977, which does not need
functioning middle ear or patent canal. In microtia patients, BAHA was initially started to use for
bilateral microtia with bilateral conducting hearing loss: Unilateral BAHA is usually placed because a
single aid will stimulate both cochlea simultaneously. The drawback of BAHA is the interface between
titanium and skin: It may cause skin irritation or infection. BAHA has a retention rate of over 95% on
long-term follow-up, with a soft-tissue reaction rate of 30%. 14
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Syndromes Associated With Microtia
Craniofacial Microsomia
Microtia associated with craniofacial microsomia needs attention for specific characteristics. 15
Dystopic vestige: To achieve symmetry of the vertical dimension of the auricles, dystopic vestige
must be transposed backward, and upward, either as a surgery before 3D framework placement, or
simultaneous transposition 16 with 3D framework placement. Mistakes are often made to use vestige
lobule in the face mask, without transposing it.
Low hairline: Low hairline is a part of abnormal hairline most often seen in hemifacial microsomia
and Treacher-Collins syndrome. Abnormally low hairline is often associated with missing sideburns,
which can confuse surgeons on where to place the new auricle. Avoiding hairline may cause
abnormal location of the new auricle too inferiorly.
Soft-tissue defect: As a part of manifestation of hemifacial microsomia, microtia is often associated
with relatively large depression of soft tissue around the auricular site. Fat grafting may help to
improve it.
Vascular anomalies: The course of STA is often abnormal. To avoid injury to STA, preoperative
Doppler ultrasound tracing of the STA course is advisable.
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Treacher-Collins Syndrome
Because a patient with Treacher-Collins syndrome has a relatively smaller face, the size of a new ear has
to balance with the smaller face. When you assess microtia in Treacher-Collins syndrome, two specific
findings are important not to miss:
Low hairline: To eliminate hair on the new auricle, either temporoparietal fascia (TPF) flap or
random-pattern fascia flap 17 is necessary to eliminate hair over the framework.
Coronal scar: When the patient already has a coronal scar, surgeons should be aware that STA is
severed. That means TPF is not available for either low hairline case to cover the anterior surface or
for Nagata-type ear elevation to cover the posterior surface. Occipital fascia flap may be indicated.
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Surgical Management
History of Microtia Reconstruction
Sushuruta (6 BC) was probably the earliest surgeon who performed auricular construction, repairing the
earlobe with cheek flap. Tagliacozzi described repair of ear deformities with retroauricular flaps. In 1845,
Dieffenbach reported the repair of the middle third of the ear with an advancement flap. This technique
may have application today. Early surgical attention focused mainly on traumatic deformities. However,
by the end of the 19th century, surgeons began to address congenital defects, in particular prominent
ears. The concept of microtia repair began in 1920, when Gillies buried carved costal cartilage under
masts skin and elevated it with cervical flap. In 1937, Gillies also used maternal ear cartilage for more
than 30 microtic ears; these were found to have progressively resorbed. Peer diced autogenous rib
cartilage and placed it in a vitallium ear mold beneath the abdomen in 1948. After 5 months, he retrieved
the banked mold, opened it, and harvested the ear framework for microtia repair. Steffensen’s 1955
attempt to use preserved cartilage resulted in progressive resorption of the grafted cartilage. Tanzer,
whose excellent results established autogenous construction with rib cartilage, 18 is considered to be the
father of modern auricular construction. Surgeons from around the world visited Dartmouth-Hitchcock
Medical Center, and autogenous construction spread across the globe. In the United States, Brent
succeeded Tanzer in early 1970s, and Brent’s artistry has influenced reconstructive surgeons for more
than 40 years until today. Nagata’s two-stage method emerged in the 1990s and has gradually gained
popularity, becoming the strongest alternative to Brent’s four-stage method. Autogenous cartilage
remains the most reliable material that produces results with the least complications.
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Timing of Surgery
The timing of auricular construction is governed by both psychological and physical considerations.
From a psychological perspective, it would be ideal to begin construction before the child enters school,
but autogenous construction should be postponed until rib growth provides substantial cartilage to
permit a quality framework fabrication. Brent begins ear construction at age 6 years, when normal ear has
grown to within 6 to 7 mm of its full vertical height and the amount of cartilage is enough for Brent-type
framework. Nagata begins auricular construction at the age of 10 years, and chest circumference grows
over 60 cm, at the xiphoid level. These two different timings could be explained by the difference of the
amount of cartilage required: Nagata-type 3D framework needs larger amount of cartilage from ribs six
to nine. In other words, surgeons are less likely to able to fabricate Nagata-type framework at the age
6 years.
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Autogenous Construction
The two main techniques described for autogenous auricular construction using a rib cartilage are the
Brent and Nagata techniques (see Figure 33.3).
Brent technique: Four-stage reconstruction beginning at 6 years of age 19 :
Creation and placement of a rib cartilage auricular framework
Rotation of the malpositioned ear lobule into the correct position
Elevation of the reconstructed auricle and creation of a retroauricular sulcus
Deepening of the concha and creation of the tragus
Autogenous rib cartilage framework: Rib cartilage framework has evolved from Tanzer-type in the
1950s to anatomically most complete Nagata-type 3D framework architecture (Figure 33.5).
20
Harvesting rib cartilage method: Brent harvested rib cartilage with perichondrium attached to it.
Because the perichondrium is the scaffold of cartilage matrix regeneration, after harvesting rib
cartilage, chest depression deformities are the usual consequences. Nagata harvests rib cartilage
without perichondrium. 32 By leaving 100% perichondrium to the donor site, and bringing back diced
leftover cartilage to the donor site, by creating a perichondrium pocket, Nagata stated that chest
deformities are less likely to occur. 22,23
FIGURE 33.5 Architecture of 3D framework.
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Synthetic Framework
Although silicone breast implants are used, silicone ear framework is discontinued today. Silicone
framework, introduced by Cronin in 1966, extrudes, causes infection, and loses definition in the long
term. Porous polyethylene implant framework, newer synthetic material, was first introduced by
Reinisch in 1991. 24 The advantage of porous polyethylene implant over autogenous construction is that it
can be applied to younger children whose costal cartilage are less mature and not ready for autogenous
reconstruction. The disadvantage of porous polyethylene framework includes use of TPF flap, long-term
risk of alloplastic implant exposure or loss, and compromise of any future autogenous options. Early
attempts were associated with a 42% incidence of framework extrusion leading to modifications of the
original technique and coverage of the framework using a TPF flap. This drastically reduced the
complication rate and is the technique of choice in his opinion. Long-term data to prove sustainability of
porous polyethylene implant are not available.
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Prosthesis
Osseointegrated auricular prosthetic reconstruction is complementary to other approaches and provides
a reasonable alternative to poor autogenous options and poor synthetic framework outcomes. The
disadvantages of prosthesis include intermittent soft-tissue problems, long-term maintenance, prosthetic
remakes every 2 to 5 years, ongoing cost, compromise of future autogenous options, and need for a
compliant patient. 9
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Secondary Total Auricular Reconstruction
The cause of unsatisfactory ear reconstruction can be divided into three main categories: inappropriate
skin envelope, inappropriate ear framework, and inappropriate ear location. Most unsatisfactory cases
have all three components. Therefore, radical total redo of everything (skin envelope, 3D framework, and
ear location) may be the solution for the patients/family who are willing to go through the long and
challenging surgery. Assessment of the patients is important to know what kind of surgical option would
be optimal for the patients. TPF flap is the workhorse for secondary ear reconstruction. 25 The surgeon
must assess the presence of STA from the base (near the caudal end of the auricle location) up to the
parietal area (10 cm above the upper helix portion), by Doppler ultrasound. If TPF is not available,
pedicle-occipital facial flap, or free vascularized fascia flap transfer are the option for the new skin
envelope. 26
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Complications of Microtia Construction
Complication of both Brent technique and Nagata technique is rare in experienced hands. Most common
complication from surgery is that the construct does not look like a normal auricle.
Framework problem: Surgeons must understand the basic aesthetic and proportion of the normal
auricle to create appropriate 3D framework. Surgeons should receive intense hands-on laboratory
training before starting clinical cases.
Skin envelope problem: There has been scarce discussion on what is the optimal thickness of the
skin flap for ear reconstruction. Too thick skin flap will make ear definition poor; on the other hand,
too thin skin flap may die: Surgeons have to walk on a fine line between the two. Nagata stated that
proper skin thickness for ear reconstruction is 2 mm.
Ear position problem: Various types of ear malposition are possible. Surrounding pathological
anatomy influences surgeon’s judgment. To avoid misjudgment of ear positioning, careful
assessment of anatomy around the microtia site is important: overall facial symmetry, distance
between lateral canthus to the vestige, location of vestige lobule, presence of low hairline. Low
hairline is easy to miss and that may cause low-set ear. To avoid hair in the reconstructed ear, ear
position may be compromised by some surgeons: Ear may be located too low or inclined forward.
Ear positioning in secondary reconstruction is challenging because of inherent and acquired
abnormal anatomy around the ear site.
Poor definition: Poor definition is one of the most frequent unfavorable outcomes of total ear
reconstruction. To create ear definition, skin flaps have to be balanced perfectly with 3D framework,
have to have adequate thickness of skin flap—not too thick, not too thin—and must have enough
space between cartilage to set in the skin flap. For example, if both helix and antihelix are too wide,
there is minimum space between two structures, thus skin flap will not go into that space.
Atelectasis: Postoperative pain from rib cartilage harvest may aggravate atelectasis. This can be
improved with infusion pumps of local anesthetics.
Skin flap necrosis: In the Nagata technique, lobule split technique requires a delicate, meticulous
split of the lobule right in the middle. If the lobule was not split in the middle, uneven split may
cause partial skin necrosis. To make split lobule more mobile, the split must be performed down to
the fascia level. Incomplete, shallow split may cause insufficient rotation and may cause tension to
the skin flap that may lead to skin flap necrosis. Relatively large subcutaneous pedicle for posterior
skin flap must be preserved to prevent skin flap necrosis. 27
Infection: Immediate postoperative infection after total ear reconstruction is rare (<0.5%). Brent
stated that if the patient is free of infection at 10 days after surgery, infection is less likely to happen
afterward. If you see any sign of infection, such as redness, pain, fever, and fluctuation or discharge
from the wound, careful monitoring is mandatory, and incision and drainage may be necessary. If
wires are used for framework, the wires should be removed, otherwise infection may persist.
Prevention: For concha-type microtia, it is important to understand and recognize the middle ear
pathology (otitis media/cholesteatoma) preoperatively.
Hematoma: Condition is rare (0.3%), but devastating. Early recognition is vital. Treatment is
immediate draining.
Cartilage resorption: Cartilage gets nutritional supply from the surrounding environment not via
vessels but through diffusion. Therefore, cartilage resorption is believed to be caused by insufficient
blood supply to the tissue surrounding the cartilage. Traditional ear elevation is performed by
cutting posterior vascular supply and simply placing the skin graft behind the auricle. Nagata
believes that placing TPF flap, covering the entire posterior surface of the auricle, will augment
vascular supply to the cartilage framework and prevent the cartilage resorption.
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Hypertrophic scar: Oblique donor site skin incision is more likely to cause hypertrophic scar.
Transverse skin incision parallel to skin wrinkle is better to prevent hypertrophic scar.
Framework extrusion: Exposure of framework is more common in synthetic framework than
autogenous rib cartilage framework. Conservative treatment with frequent dressing change may heal
the wound for autogenous framework, but less likely to heal for synthetic framework. Because of
scar tissue surrounding the exposure, attempting primary closure is difficult, and well-perfused flap
coverage may be the only solution. If the local skin flap is not available, fascial flap such as TPF, deep
temporal fascia flap, or occipital flap may be necessary.
Wire extrusion: Brent uses nylon sutures for framework fixation to avoid wire extrusion. Nagata and
Firmin use fine wires (38G) for fixation. Nagata believes that rigid fixation is optimal for cartilage
framework fixation because cartilage pieces, unlike bone pieces, are less likely to fuse together,
simply getting fibrous union. Once the patient/family notice the wire exposure, it is important to
remove it immediately, otherwise it may cause resorption of cartilage or infection.
Thinning of temporal hair from fascia flap harvest: The Nagata technique routinely uses TPF flap
for ear elevation to cover the entire posterior aspect of the new auricle. 28 TPF may also be used in
low hairline cases or Medpor framework to cover anterior surface of the framework. TPF is located
immediately below the hair follicles. Therefore, aggressive dissection or too much heat from
coagulation hemostasis may knock out hair follicles located just above the TPF layer sharp flap
dissection with scalpel usually does not cause alopecia.
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