Orthognathic Surgery

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CHAPTER 27  Treacher Collins Syndrome: Evaluation and Treatment
age had permanent conductive hearing loss and bilateral
implanted BAHAs. Eleven studies met the criteria for data
analysis. Bilateral BAHAs were found to provide audiologic
benefit as compared with unilateral BAHAs (i.e., improved
thresholds for tones, speech in quiet and in noise, improved
localization/lateralization, and patient’s perceived subjective
benefit). Disadvantages of bilateral BAHAs included 1) lis­
tening in noise in some condition; 2) presumed additional
cost; and 3) presumed increasing adverse event risk. The
authors concluded that bilateral BAHAs provided addi­
tional objective and subjective benefit as compared with
unilateral BAHAs.
Conclusions
The current approach to the correction of malformations
associated with TCS is to stage the reconstruction to coin­
cide with facial growth patterns, visceral function (i.e.,
vision, breathing, chewing, swallowing, speech, hearing),
and psychosocial needs. Precise morphologic analysis of
each patient and the recognition of the need for a staged
reconstructive approach serve to clarify the objectives
at each interval for both the clinicians and the family.
By continuing to define a rationale for the timing,
methods, and extent of surgical, medical, and dental inter­
ventions and then objectively evaluating head and neck
function, facial aesthetics, and psychosocial outcomes, the
outlook for individuals affected by TCS will be greatly
improved.
following: 1) conventional hearing aids, which are also
known as air-conduction hearing aids (ACHAs); 2) bone-
conduction hearing aids (BCHAs); and 3) unaided hearing
and ear surgery. The study also looked at 4) unilateral versus
bilateral BAHAs. Outcomes reviewed included hearing
measures, validated measures of quality of life, adverse
events, and measures of cost-effectiveness.
There appeared to be some audiologic benefits of BAHAs
for this group of patients, including 1) improvements in
speech as compared with BCHAs; and 2) understanding
and noise as compared with ACHAs (however, ACHAs may
produce better audiologic results for other outcomes). In
addition, 3) hearing was found to be improved with the use
of BAHAs as compared with unaided hearing; and 4)
studies that compared unilateral with bilateral BAHAs sug­
gested the benefits of bilateral BAHAs in many situations.
However, prospective case series of BAHAs reported rates
of 6.1% to 19.4% for the loss of the implants over time. In
addition, the financial analysis suggests that BAHAs are
unlikely to be a cost-effective option in terms of hearing
gain and probability of needing alternative aids. Interest­
ingly, the greater the benefit from aided hearing and the
greater the difference in the proportion of people using the
hearing aid for more than 8 hours per day, the more likely
the BAHAs were a cost-effective option.
Janssen and colleagues completed a systematic review of
bilateral BAHAs for bilateral permanent conductive hearing
loss.68 This was a review of the literature from between 1977
and 2011, and it included studies in which subjects of any
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145. Tanzer RC: Total reconstruction of the
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1095

28
Hemifacial Microsomia:
Evaluation and Treatment
JEFFREY C. POSNICK, DMD, MD
• Inheritance Pattern
• Considerations during Infancy and into Early
Childhood
• Dysmorphology Associated with Hemifacial
Microsomia
• Facial Growth Potential with Hemifacial
Microsomia
• Classification of Temporomandibular
Joint–Mandibular Malformation
• Staging of Skeletal Reconstruction: Timing
and Techniques
• Consideration of First-Stage Mandibular
Reconstruction during the Mixed Dentition
• Facial Soft-Tissue Reconstruction
• External Ear Reconstruction
• External Auditory Canal and Middle-Ear
Reconstruction
• Conclusions
addition to HFM, the malformation has been called cranio­
facial microsomia, oculo–auriculo–vertebral dysplasia, first
and second branchial arch syndrome, lateral facial dysplasia,
unilateral oto–mandibular dysostosis, facio–auriculo–vertebral
sequence, and oculo–auriculo–vertebral spectrum.
Although there are no agreed-upon minimal diagnostic
criteria, the facial phenotype is characteristic when enough
manifestations are present. In most patients, microtia or
other auricular or preauricular abnormalities (i.e., preau­
ricular tags or sinus pits) may present the mildest manifesta­
tion. Involvement is not always limited to the facial
structures. Cardiac, renal, additional skeletal (vertebral),
central nervous system, and other more unusual anomalies
may also occur. Goldenhar syndrome is considered a variant
of HFM, and it is present in about 10% of HFM patients.
In addition to maxillofacial malformation, it presents with
epibulbar dermoid cysts and cervical spine involvement.
Inheritance Pattern
The occurrence of HFM has been estimated to range from
1 in 3500 to 1 in 26,550 live births. Cohen suggests that
the birth prevalence is likely to be around 1 in 5600 live
births.62,26 Morrison and colleagues stated a prevalence rate
of conditions on the oculo–auriculo–vertebral spectrum of
1 in 45,000 in Northern Ireland.26
Kelberman and colleagues performed a genome-wide
search for linkage in two families with features of hemifacial
microsomia.105 In one of these families, the data were highly
suggestive of linkage to a region of approximately 10.7 cM
on chromosome 14q32, with a maximum multipoint LOD
score of 3.00 between microsatellite markers D14S987 and
D14S65. Linkage to this region was excluded in the second
family, thereby suggesting genetic heterogeneity. With the
use of an animal model.
Poswillo demonstrated that early vascular disruption
with expanding hematoma formation in utero resulted in
the destruction of differentiating tissues in the region of the
ear and the jaw.214-218 The degree of local destruction
Hemifacial microsomia (HFM) is a craniofacial malfor­
mation that results in varying degrees of hypoplasia of
the structures within the first and second branchial
archs.34,35,38,53,62,65,66,81,84,174,227 Congenital hypoplasia is gen­
erally unilateral, although bilateral (asymmetric) involve­
ment occurs in 5% to 15% of patients. Most cases of this
condition are sporadic, but there are rare familial causes that
exhibit autosomal dominant inheritance. The term hemifa­
cial microsomia was first used by Gorlin and Pindborg
during the early 1960s, although the first recorded cases
may have been those of Canton in 1861 and Von Arlt in
1881.62 Many terms have been used for this malformation,
thus indicating the wide spectrum of anomalies observed
and emphasized by authors from various disciplines. In

1096 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
appeared to be related to the severity of tissue damage
caused by the hematoma. The constellation of anomalies
seen in patients with HFM suggests an origin that occurs
at about 30 to 45 days’ gestation. Naora and colleagues
described a transgenic mouse line that carried an autosomal
dominant insertion mutation that resulted in facial anoma­
lies that resemble HFM, including microtia and abnormal
occlusion.164
First and second branchial arch malformations similar to
HFM have been observed in infants who are born to women
who have been exposed to thalidomide and primidone. The
administration of isotretinoin (Accutane) may also injure
neural crest cells and prevent their normal development,
thereby resulting in hemifacial hypoplasia similar to that
observed in patients with HFM. It has been documented
that acute exposure to ethanol during gastrulation (i.e., the
transformation of the blastula into the gastrula) may induce
asymmetric facial development.
Discordance in monozygotic twins has been frequently
reported. Concordance with variable expression has also
been rarely documented in monozygotic twins. The rarity
of the reports of the concordance of the malformation in
twins supports the suggestion that the condition is sporadic
in most families. Familial instances have been observed with
variable expressivity. Overall, the empiric recurrence risk is
on the order of 2% to 3%; however, 1% to 2% of cases
suggest an autosomal dominant inheritance pattern.
Engiz and colleagues reviewed the clinical and laboratory
findings of 31 individuals with Goldenhar syndrome (15
boys and 16 girls) between the ages of 1 day and 16 years.47
The characteristic features were preauricular skin tags
(90%), microtia (52%), hemifacial microsomia (77%), and
epibulbar dermoids (39%). Vertebral anomalies were noted
in 70%, cardiac malformations were found in 39%, geni­
tourinary anomaly were noted in 23%, and various central
nervous system malformations were found in 47%.
Several classification systems for HFM have focused on
one or more fundamental anatomic features of this anomaly.
In 1991, Vento and colleagues described the OMENS clas­
sification for HFM. This system substratifies each of five
anatomic manifestations of HFM in accordance with their
dysmorphic severity on a scale from 0 to 3. The five mani­
festations each constitute one letter of the OMENS acronym:
orbital asymmetry; mandibular hypoplasia; ear deformity;
nerve dysfunction; and soft-tissue deficiency. Scoring was done
on the basis of conventional radiographs (e.g., posteroante­
rior, lateral, submental, panoramic), physical examinations,
and photographs. Although the OMENS classification does
allow for the objective cataloguing of a range of the abnor­
malities that constitute the spectrum of HFM, it falls short
in terms of providing effective communication when deter­
mining the timing and technique that are best suited
for the reconstruction of each component of the anomaly
(i.e., maxillo-mandibular, orbito–zygomatic, soft tissues,
external ear). It also is of limited value with regard to overall
patient management.276
At the present time, HFM should be regarded as a non­
specific symptom complex that is etiologically and
pathogenetically heterogeneous.* Extreme variability of
expression is the characteristic finding.
Considerations during Infancy and into
Early Childhood
At the time of birth, for a child with HFM, concerns
will center on the adequacy of the airway; swallowing
and feeding; hearing; vision; the presence of other
associated malformations; and family unit psychosocial
issues.3,16,27,28,56,57,112,125,131,165,175,193,196,197,199,207,208
The airway may be compromised as a result of 1) maxil­
lary hypoplasia with choanal (nasal) obstruction 2) man­
dibular micrognathia with a retropositioned tongue
obstructing the oropharyngeal/hypopharyngeal spaces 3)
laryngomalacia/tracheomalacia or 4) neuromotor involve­
ment.3,16,27,28,57,195 Depending on the severity of these mal­
formations, a spectrum of airway problems may be present
and necessitate treatment that ranges from special infant
positioning with an extended hospital stay to mandibular
osteotomies with advancement; on rare occasions, immedi­
ate or delayed tracheostomy may be required.
When the airway is compromised, the newborn has dif­
ficulty swallowing enough fluids and taking in enough calo­
ries (i.e., maintaining hydration and positive nitrogen
balance) while simultaneously breathing effectively (i.e.,
sufficient oxygenation). In this situation, gavage-assisted
feedings or the placement of a gastrostomy tube may be
necessary to ensure adequate nutrition without aspiration
or respiratory distress.
When a cleft (of the lip, palate, or both) or macrostomia
is present, the timing of correction of these anomalies is
generally similar to that used for the patient with non-
syndromic cleft lip and palate (Fig. 28-1).
Soon after birth, examination by a medical geneticist and
the procurement of a blood sample for chromosomal studies
(i.e., genetic analysis) will help to discern the extent of birth
defects and the risk of recurrence. Evaluation by the pedi­
atric otolaryngologist in combination with the speech and
feeding specialist will clarify the extent of breathing, swal­
lowing, and feeding difficulties. Initial audiologic testing via
auditory evoked brain responses is essential to clarify neu­
rosensory hearing function. A pediatric ophthalmologic
assessment is useful to assess extraocular muscle function,
corneal exposure difficulties, visual potential, and adnexal
region malformations.
When moderate to severe skeletal malformations are
present, a craniofacial computed tomography (CT) scan from
the top of the skull through the cervical spine with three-
dimensional reformation will be useful to document the
extent of craniofacial skeletal dysmorphology. A focused
petrous temporal bone CT scan is also required to document
external auditory canal anatomy as well as middle and inner
*References 9, 26, 36, 41, 42, 47, 50, 52, 64, 69, 75, 78, 83, 90, 104-106,
119, 164, 181, 198, 206, 214-218, 229-231, 247, 248, 255, 263, 264,
267, 276, 281, 282

1097
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
bulk of the cutaneous and subcutaneous tissue, the volume
of fat, the muscles of mastication and facial expression, the
cranial nerves, and the parotid and submandibular glands.
According to the research of Kane and colleagues, in
patients with HFM, the extent of hypoplasia of specific
muscles of mastication frequently predicts the extent of
dysplasia of the osseous origin and insertion of those
muscles.98 If the temporalis muscle is hypoplastic, a defi­
ciency of the coronoid process will be present. When the
masseter muscle is hypoplastic, the gonial region of the
mandible will also be deficient. When the lateral pterygoid
muscle is deficient, the condylar head is deficient or absent.
Preauricular–cheek region soft-tissue thickness and skin
surface area are generally related to the skeletal deficiencies.
Skin tags are small vestigial rests of epithelial tissue that are
generally found at the cleft between the first and second
arches. Skin tags are frequently associated with small carti­
laginous remnants that are found within the subcutaneous
tissue. Sinus tracts may form inclusion cysts or result in
ear anatomy. Because of the potential increased risk of radia­
tion exposure to infants, these imaging studies may be
delayed until they are necessary for surgical treatment plan­
ning. Appropriate craniofacial, maxillofacial, cleft lip and
palate, and auricular reconstructive surgeons should also be
consulted as indicated.
Dysmorphology Associated with
Hemifacial Microsomia
Facial Soft Tissues
From a clinical perspective, the soft-tissue deficiencies asso­
ciated with HFM can be considered to affect four anatomic
regions of the head and neck: 1) the external ear; 2) the
eyelid–adnexal structures; 3) the preauricular–cheek–lip
soft tissues; and 4) the temporal fossa (Figs. 28-2 and 28-
3).19,51,85,98,101,106,130,133,129,228,251 The soft tissues within each
region that may be deficient and dysmorphic include the
• Figure 28-1  A newborn with a mild form hemifacial microsomia, including macrostomia
of the left oral commissure. She is shown before and 1 year after macrostomia repair.
A, Facial views before macrostomia repair. B, Facial views after oral commissure
reconstruction.
B
©Elsevier Inc.
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1098 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
• Figure 28-2  A 9-year-old boy who was born with left-sided hemifacial microso­
mia and a unilateral cleft lip and palate. He underwent lip and palate repair during
early childhood at another institution. He is congenitally missing the left external ear
and external auditory canal. The soft tissues of the eyelids and the cheek region
are also deficient. The skeletal malformation involves the upper facial skeleton (i.e.,
the left zygomatic complex, the left orbit, and the left squamous temporal bone)
and the lower facial skeleton (i.e., the maxilla and the mandible). He has a type III
glenoid fossa mandibular malformation. A, Frontal facial and computed tomography
scan views. B, Oblique facial and computed tomography scan views.
©Elsevier Inc.
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infection if they are obstructed. Macrostomia is a clefting or
failure of fusion of the maxillary and mandibular processes
and of the first branchial arch, respectively. This results in
a cleft of the orbicularis oris muscle, the overlying skin, and
the underlying mucosa directly through the oral commis­
sure. Anophthalmia or microphthalmia may also occur. Colo­
bomata of the iris or eyelids with the absence of the eyelashes
is a frequent finding. Ptosis of the upper eyelid (i.e., as a result
of levator palpebral muscle function) with narrowing of the
vertical palpebral fissure is often seen. Deficiencies of the
lateral canthi with a decreased horizontal fissure are common.
Epibulbar dermoid cysts are found in a significant percent­
age of patients; they appear as solid yellowish or pinkish-
white ovoid masses that vary in size from that of a pinhead
to 8 to 10 mm in diameter. The cysts occur most often at
the inferotemporal quadrant of the limbus. The surface is
usually smooth, and it frequently has fine hairs. These cysts
can occur at any location on the globe or in the orbit, and
they may be movable or fixed to the dermis. Unilateral
epibulbar dermoid cysts are seen in a high percentage of
patients, and multiple lesions may occur in each eye. Occa­
sionally, vision is impaired by encroachment on the pupil­
lary axis or by lipid infiltration of the cornea.
Abnormalities of the external ear are a consistent finding
and range from anotia to a mildly dysmorphic ear. Farkas and
James were unable to demonstrate a direct relationship
between the degree of microtia and the extent of skeletal
deformity.51
The External Auditory Canal, the Middle- and
Inner-Ear Structures, and Audiologic Findings
A narrow external auditory canal is frequently found in
patients with mild external ear deformity, whereas atretic
canals are expected in more severe cases.17,18,223,280 At times,
a small external ear with normal middle-ear architecture is
seen. Audiometry will delineate the nature of the hearing
loss, which likely includes conductive and, less frequently,

1099
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
• Figure 28-3  A 14-year-old girl who was born with right-sided hemifacial micro­
somia, including microtia and an absent external auditory canal. There is soft-tissue
deficiency on the right side of the face. The skeletal malformations involve the right
zygomatic complex as well as the mandible and the maxilla. There is a Type IIB
mandibular malformation. The patient had previously undergone right ear recon­
struction and the placement of a bone-anchored hearing aid (BAHA) at another
institution. A, Frontal facial and computed tomography scan views. B, Oblique facial
and computed tomography scan views.
©Elsevier Inc.
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sensorineural loss in 15% of patients. Hypoplasia or agen­
esis of the ossicles may occur. In a comprehensive study,
Caldarelli and colleagues used air and bone conduction
audiometry and temporal bone tomography to evaluate 57
patients with hemifacial microsomia.17,18 The authors were
unable to correlate the degree of auricular (i.e., external ear)
deformity with hearing function. Focused temporal bone
CT scans offer the best documentation of the middle-ear
structures. Assessment of the unaffected and presumed
normal ear is an essential part of the evaluation.
Maxillomandibular Region
A variable degree of hypoplasia of the skeletal structures
within the first and second branchial arches will occur. As
a result, the anteroposterior, transverse, and vertical dimen­
sions of the face are diminished on the affected side, with
secondary deformities on the contralateral side. This is espe­
cially true in the maxillomandibular region (see Figs. 28-2
and 28-3).134
Cranio–Orbito–Zygomatic Region
A variable degree of hypoplasia of the zygomatic complex
is a frequent finding. The consequences of zygomatic hypo­
plasia may be seen clinically as orbital dystopia; maxillary
hypoplasia; deficiency of the glenoid fossa; and deficiency
of the squamous aspect of the temporal bone. This is best
visualized with a three-dimensional reconstruction of a cra­
niofacial CT scan. As a result of the asymmetric nature of
the deformity, identifying consistent reproducible land­
marks is difficult in the upper face. This makes a quantita­
tive assessment of the skeletal malformation difficult and
imprecise.
Facial Growth Potential
with Hemifacial Microsomia
The potential for longitudinal facial growth in a child
who is born with HFM is an important factor when

1100 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
involved side. In the study group, subsequent growth
occurred in both mandibular rami in each patient. This was
true for all patients, irrespective of the Pruzansky grade and
the side of the mandible that was affected.
Meazzini and colleagues conducted a long-term facial
growth study of children affected by HFM with Type I or
Type II mandibular malformations.136A The study group (N
= 8) was not subjected to any treatment until adulthood.
With the use of the panoramic radiograph analysis of ramus
vertical heights, data confirmed that facial proportions in
patients with HFM were maintained throughout growth
when no treatment was undertaken. The authors concluded
that HFM does not progress in the degree of facial asym­
metry or deformity when left to mature naturally.
Ongkosuwito and colleagues studied mandibular
growth in 84 consecutive patients with a confirmed diag­
nosis of unilateral HFM. The mandibular malformation for
each subject was categorized into one of four grades on the
basis of the Pruzansky/Kaban classification. The malforma­
tions were then regrouped to reflect those with a functional
glenoid fossa and condyle (Type I and Type IIA) and those
without (Type IIB and Type III). The study groups were
compared with a normal age-matched Danish control
group. For each subject, an orthopantomogram was obtained
and used to perform measurements of ramal height (i.e., the
distance measured in millimeters between the condylion
and the gonion) on each side. The data confirmed that
patients with HFM start with a shorter ramal height and
end up with a shorter ramal height, although growth is the
same in both patients with HFM (regardless of the extent
of malformation) and controls. In patients with HFM,
the ramal height is smaller on both sides, which means
that growth is characterized not simply by unilateral under­
development but by a complex three-dimensional
phenomenon.161A
According to the independent findings of Polley and
colleagues, Rune and colleagues, Meazzini and colleagues,
Ongkosuwito and colleagues, the mandibular asymmetry
associated with HFM is not progressive. In these studies, pro­
gressive canting of the maxillary plane was not routinely
observed (Figs. 28-5 through 28-7).
An alternative perspective is presented by Kaban and
colleagues.92,93,95 Their research suggests to them that, as a
result of ongoing growth after birth in patients with HFM,
there is progressive canting measured at the pyriform rims,
the occlusal plane, and the intergonial angles, especially
with the Type IIB and III mandibular malformations. The
authors recommend that mandibular reconstruction during
the mixed dentition be considered to correct the lower jaw
deformity in anticipation of normal ongoing longitudinal
growth; to prevent secondary maxillary deformities (in an
otherwise normal maxilla); and to alleviate psychosocial
issues that would otherwise occur during childhood. I
believe that Kaban and colleagues’ rationale for first-stage
mandibular reconstruction during the mixed dentition in
children with HFM are overly optimistic.
considering the timing and techniques of reconstruc­
tion.96,97,114,115,133,144-147,152-154,168,194,202-204,225,226,230-232 An addi­
tional
pivotal
consideration
for
the
maxillofacial
reconstruction of the individual with HFM is the integrity
of the glenoid fossa–condyle–ascending ramus of the man­
dible (Fig. 28-4). Some authors speculate that a reduced
mandibular growth potential secondarily leads to the
observed maxillary deficiency on the ipsilateral side with
progressive canting of the occlusal plane. Those who advo­
cate mandibular surgery during the mixed dentition often
do so with the belief that it will effectively correct the lower
jaw malformation and that normal ongoing mandibular
growth will continue with the prevention of secondary max­
illary growth deformities in an otherwise normal upper jaw.
There are objective clinical and radiographic studies in the
literature that review the issue of the progression of defor­
mity with HFM during growth.87-89,194,225,226
Rune and associates placed metallic implants in the jaws
to study the facial growth of 11 patients with HFM.225,226
They collected and studied serial roentgen stereophoto­
grammetry images from each patient. They found that, in
five patients, the cant of the occlusal plane seemed to
“slightly” increase; in the remaining six patients, the occlusal
plane asymmetry either remained the same or improved
with time. The authors also stated that the pattern of
mandibular displacement showed no correlation with the
severity of the presenting deformity. They concluded
that the data “do not support the claim that the asymmetry
of the jaws is invariably increased in time because of
growth disparity between the affected and the unaffected
sides.”225,226
Polley and associates completed a longitudinal radio­
graphic cephalometric study of the maxillofacial region in
patients with HFM.194 Twenty-six patients were included in
the study and were followed longitudinally. Five patients
(19%) had a Pruzansky grade I mandibular deformity, 14
patients (54%) had a Pruzansky grade II deformity, and 7
patient (27%) had a Pruzansky grade III deformity. The
average age at the time that the initial cephalometric records
for all patients were obtained was 3.5 years (range, 0.7 to
9.2 years), and the average age at the time that the final
cephalometric records were obtained was 16.7 years (range,
10.1 to 22.5 years). None of the study patients had under­
gone surgical or orthopedic jaw manipulation during the
time of the study, although nine patients underwent fixed
appliance orthodontic treatment. Vertical and horizontal
skeletal mandibular asymmetry was evaluated with postero­
anterior cephalometry. The authors found that the growth
of the affected side in patients with HFM parallels that of
the contralateral side. The degree of mandibular asymmetry
in the study patients remained relatively constant through­
out craniofacial development. The mandibular skeletal
deformity as measured in the study subjects did not progress
with time. The growth rate of the affected side was similar
to that of the contralateral side in each subject, irrespective
of the degree of presenting mandibular deformity on the

1101
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
• Figure 28-4  Kaban and colleagues described a classification system to define the
degree of glenoid fossa–mandibular malformation observed in patients with hemifacial
microsomia. Three-dimensional craniofacial computed tomography (CT) scans illustrate
this classification system (see text for complete description). A, CT scans of a Type I
mandibular malformation. B and C, CT scans of a Type IIA mandibular malformation.
B
Kaban Type IIA
C
Kaban Type IIA
A
Kaban Type I
Continued

1102 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
Kaban Type IIB
E
Kaban Type III
E
D
D, CT scans of a Type IIB mandibular malformation. E, CT
scans of a Type III glenoid fossa–mandibular malformation in a patient who also has
a unilateral cleft lip and palate.
• Figure 28-4, cont’d 
1. In perspective, even if a small degree of asymmetric
jaw growth occurs during childhood in the patient
with HFM, significant progressive dysfunction (i.e.,
in the areas of chewing, breathing, speech, lip control,
or swallowing) that would justify a first-stage man­
dibular procedure is not generally seen.
2. The effectiveness of a first-stage mandibular proce­
dure during the mixed dentition as a definitive
long-term maxillofacial reconstruction has not been
documented. Not only would a successful initial
lengthening and derotation of the mandible be
required, but the fabrication and then the periodic
modification of an effective removable acrylic splint
to create and maintain an ipsilateral posterior open
bite (to allow for the supra-eruption of the maxillary
posterior teeth) would be needed. The necessity of
constant clinician monitoring and splint modifica­
tion in conjunction with ongoing orthodontic treat­
ment and rigorous patient compliance would be
essential. This requires an experienced and dedicated
orthodontist, a persistent surgical coordinator, and a
zealous patient and family. All of the treating clini­
cians and the family must be in close geographic
proximity and without socioeconomic barriers to
treatment. Unfortunately, this is not a combination
of events that is readily achievable.
3. Unfortunately, the costochondral graft construction
of the neocondyle–ascending ramus required for the
treatment of HFM Type IIB and III mandibular
deformities carried out during the mixed dentition
will result in a significant percentage of poorly
growing mandibles (i.e., overgrowth or undergrowth).
At minimum, a second definitive orthognathic recon­
struction will be required (see the section to follow in
this chapter about costochondral grafting).
4. Researchers have documented that, when a distraction
osteogenesis (DO) approach is used as a first-stage
mandibular procedure for HFM during the mixed
dentition, symmetric lower jaw growth does not
occur. The resulting residual mandibular asymmetry
and deformity will, at minimum, require later
Text continued on p. 1110

1103
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
C
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Age 5
Age 9
Age 12
• Figure 28-5  A girl who was born with left-sided hemifacial microsomia is followed up longitudinally at 5, 9, and 12 years of age
without treatment intervention. There is a Type III glenoid fossa–mandibular malformation. No progression of the deformity has
occurred. A, Frontal facial views at 5, 9, and 12 years of age. B, Oblique facial views at 5, 9, and 12 years of age. C, Profile views
at 5, 9, and 12 years of age.
Continued

1104 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
E
Age 9
Kaban III
Orth
r ognathic Surg
r ery; J.C. Posnick
Orth
r ognathic Surg
r ery; J.C. Posnick
D
Age 9
Kaban III
D and E, Facial and computed tomography scan views at 9 years of age confirmed the
extent of skeletal malformations involving the zygomatic–orbital complex, the maxilla, and the mandible.
• Figure 28-5, cont’d

1105
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
• Figure 28-6  A girl who was born with right-sided hemifacial microsomia is followed up longitudinally at 5 and
12 years of age without treatment intervention. There is a Type IIB–mandibular malformation. No progression of the
deformity is demonstrated. A, Frontal facial views in repose at 5 and 12 years of age. B, Profile views at 5 and 12
years of age.
Orth
r ognathic Surg
r ery; J.C. Posnick
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r ery; J.C. Posnick
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Orth
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r ery; J.C. Posnick
A
Age 5
Age 12
B
Age 5
Age 12
Continued

1106 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
D
C
Age 5
Age 12
Age 5
Age 12
E
Age 5
Age 12
C, D, and E, Computed tomography scan views at 5 and 12 years of age that confirm
no progression of the skeletal malformation.
• Figure 28-6, cont’d

1107
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
Orth
r ognathic Surg
r ery; J.C. Posnick
Orth
r ognathic Surg
r ery; J.C. Posnick
A
B
6 months
Kaban III
6 months
Kaban III
• Figure 28-7  A child who was born with hemifacial microsomia of the right side. The extent of the soft-tissue
and skeletal deficiencies of the structures within the first and second branchial arches is shown. There is a Type
III glenoid fossa–mandibular malformation. The only treatment intervention was the excision of displaced redundant
auricular tissue in the right cheek region. No progression of the skeletal malformation occurred between 6 months
and 10 years of age. A, Frontal facial and computed tomography (CT) scan views at 6 months of age. B, Oblique
facial and CT scan views at 6 months of age.
Continued

1108 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
C
D
6 months
Kaban III
6 months
5 years
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r ognathic Surg
r ery; J.C. Posnick
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r ery; J.C. Posnick
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r ognathic Surg
r ery; J.C. Posnick
C, Right profile and CT scan views at 6 months of age. D, Profile views at 6 months and
5 years of age. The excision of right cheek redundant soft tissue was carried out at 1 year of age with facial nerve
preservation.
• Figure 28-7, cont’d

1109
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
G
Age 4 years
Age 7 years
Age 10 years
E
Age 4 years
Age 7 years
Age 10 years
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F
Age 4 years
Age 7 years
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E, Frontal views at 4 years, 7 years, and 10 years of age without treatment intervention. F, Oblique facial
views at 4 years, 7 years, and 10 years of age without treatment intervention. G, Profile views at 4 years, 7 years, and 10 years
of age without treatment intervention.
• Figure 28-7, cont’d 
Continued

1110 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
I
Age 6 months
Age 10 years
H
Age 6 months
Age 10 years
H and I, Left and right CT scan profile views at 6 months and
10 years of age.
• Figure 28-7, cont’d 
definitive orthognathic reconstruction. In addition,
the completion of a mandibular ramus–body osteot­
omy and DO during the mixed dentition is docu­
mented to result in the destruction of ipsilateral
developing permanent molars in a significant per­
centage of children who have undergone such an
operation.
Classification of Temporomandibular
Joint–Mandibular Malformation
The extent of temporomandibular joint (TMJ)–mandibular
deficiency is an important factor when considering the
timing of and techniques for reconstruction.108,118,119,249
Imprecise definitions of the degrees of TMJ–mandibular
anomalies have resulted in miscommunication among clini­
cians and in the literature. In an article published during
the late 1960s, Pruzansky classified the presenting
NOTE: In this author’s view, the benefits of mixed-
dentition first-stage mandibular procedures rarely
outweigh the downsides. The argument that first-stage
mandibular surgery during the mixed dentition is
necessary to allow the child to develop normal self-
esteem is well intended but not realistic. Multiple doctor
visits and procedures give a child a “sick kid” mentality
and draw more attention to his or her differences.199 The
unfortunate reality is that, for a majority of HFM patients
even with well-done mixed dentition first stage
mandibular reconstruction, the residual external ear, soft
tissue, and other skeletal malformations will continue to
draw attention to the child’s facial disproportion. The
overall burden of care for the patient, the family, and the
health care system is minimized when definitive single-
stage maxillomandibular reconstruction is carried out at
the time of early skeletal maturity (i.e., between the ages
of 13 and 15) in conjunction with orthodontic treatment.

1111
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
and all or part of the ramus on the ipsilateral side are absent.
The disc, the TMJ capsule, and the glenoid fossa are also
not developed. Mandibular dysmorphology is severe and
includes ipsilateral retrognathia and decreased posterior
(ramus) facial vertical height. The masticatory muscles are
severely hypoplastic, and the lateral pterygoid remnant is
not attached to the mandibular structures. There is signifi­
cant hypoplasia of the maxilla, with obvious canting. Occa­
sionally, a tracheostomy is required shortly after birth to
manage the airway compromise that results from a combi­
nation of factors, including (but generally not limited to)
the degree of mandibular hypoplasia. By definition, with a
Type III glenoid fossa–mandibular malformation, the even­
tual construction of both a neo–glenoid fossa and a neocon­
dyle ascending ramus will be required to achieve successful
maxillofacial reconstruction.
Staging of Skeletal Reconstruction:
Timing and Techniques
The facial rehabilitation of the patient with HFM must
address unique and specific components of the malforma­
tion that may include the following: 1) the zygomatic and
orbital region; 2) the maxillomandibular regions; 3) the
facial soft tissues; 4) the external ear; 5) the auditory canal;
and 6) the middle-ear structures.*
Some clinicians have speculated that there is a reduced
facial growth potential in patients with HFM which then
leads to a progression of the primary malformation and
secondary deformities of the maxillofacial skeleton. This
assumption has led some to advocate early mandibular
reconstruction to prevent progression. This author agrees
with Rune and colleagues,225,226 Polley and colleagues,194
Meazzini et al.136A and Ongkosuwito and colleagues161A that
the observed facial asymmetry in patients with HFM is not
progressive with ongoing growth (see the section earlier
in this chapter about facial growth potential with HFM)
(Figs. 28-5 through 28-7).203,209-212,233,234
Zygomatic and Orbital Reconstruction
The benefits of thoughtfully timed and meticulously exe­
cuted orbito–zygomatic reconstruction for the patient with
HFM are often underappreciated.209-213 When upper face
(i.e., orbito–zygomatic) reconstruction is needed, it is
carried out through a coronal scalp incision. Correction of
the upper face skeletal deformity may require the intracra­
nial disassembly and reconstruction of the dysmorphic ipsi­
lateral anterior cranial vault; the squamous portion of the
temporal bone; and the lateral orbit regions with complete
construction of the zygomatic complex, including the
glenoid fossa. The use of full- and split-thickness cranial
bone grafts is required (Fig. 28-8).
mandibular anomalies of HFM into three grades (Type I
through Type III) in accordance with the extent of hypo­
plasia of the mandibular condyle and ramus.220 Kaban and
colleagues refined the Pruzansky classification to further
clarify the degree of glenoid fossa–condyle–ascending ramus
malformation observed in patients with HFM.94,157 The
Kaban classification provides an excellent starting point for
defining the reconstruction required in each individual
patient (see Fig. 28-4).
A Type I mandible has only a minimal degree of hypo­
plasia of the glenoid fossa, the condyle, and the ascending
ramus (see Fig. 28-4, A). All of the skeletal components
are present, all of the masticatory muscles are present, and
function is within normal limits. There is a degree of asym­
metric mandibular retrognathia, with a shift of the chin off
of the facial midline. A limited degree of maxillary cant can
usually be documented. Note: With Type I malformations,
the condyle and the glenoid fossa are fully intact. There
is no need to construct a new condyle or a new glenoid
fossa.
A Type IIA mandible has a moderate degree of glenoid
fossa and condyle–ascending ramus hypoplasia (see Fig.
28-4, B and C). By definition, the extent of hypoplasia
results in the TMJ complex being located anteriorly and
medially as compared with the normal side; however, joint
function remains satisfactory. The masticatory muscles have
a variable degree of hypoplasia. The mandible is retrogna­
thic, the chin is shifted to the ipsilateral side, and an anterior
open bite is frequently seen. A moderate maxillary cant is
usually present. With a Type IIA malformation, despite
significant hypoplasia, a decision to retain the TMJ complex
(i.e., the condyle and the glenoid fossa) as part of the maxil­
lomandibular reconstruction has been made.
The Type IIB mandible involves severe hypoplasia of the
condyle–ascending ramus (see Fig. 28-4, D). The glenoid
fossa has an anterior and medial location, but its placement
is adequate for the construction of a neocondyle. The hypo­
plastic condyle, if it is present at all, is rudimentary and not
substantial enough to seat into the glenoid fossa. There is
not a consistent “posterior stop” to the mandible against the
glenoid fossa. The mandible functions with rotation but not
with a condylar component in the glenoid fossa, and there
is little or no translational jaw movement. Vertical mouth
opening is present (albeit reduced), with a shift of the chin
to the ipsilateral side. The extent of mandibular dysmor­
phology, asymmetric retrognathia, and anterior open bite
is marked. Variable but definite deficiencies of the mastica­
tory muscles are present. There is usually significant hypo­
plasia of the ipsilateral maxilla, with obvious canting. By
definition, with a Type IIB mandibular deficiency and
deformity, the condylar remnant is too far displaced and
rudimentary to be useful. Construction of a neocondyle–
ascending ramus into the current acceptable glenoid fossa is
required.
The Type III mandible is free-floating on the ipsilateral
side, with no “posterior stop” of the lower jaw against the
skull base on the affected side (see Fig. 28-4, E). The condyle
*References 70, 76, 77, 92-95, 99, 109, 135, 137, 142, 145, 151, 157,
166-168, 170, 172, 236, 243, 253, 256, 266, 270-275, 277, 287

1112 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
• Figure 28-8  Intraoperative views of a 7-year-old child
demonstrate the technique of zygomatic–orbital reconstruc­
tion as described in this chapter. When working through a
coronal scalp incision, the zygomatic and orbital structures
are exposed. A full-thickness cranial bone graft is harvested,
and a zygomatic complex is crafted from this. The crafted
zygoma is inset and stabilized with microplates and screws.
The orbital defects are reconstructed as indicated with
cranial bone graft, and the orbital rim is reshaped with a
rotary drill. A lateral canthopexy is completed through the
coronal incision and fixed to the new frontozygomatic suture
region. A, View of the dissected rudimentary zygomatic
remnant. A template of the proposed zygomatic complex
had been made. B, The template is taken to the temporo­
parietal region of the skull, and its outline is marked out for
craniotomy. Through a craniotomy, a full-thickness cranial
bone graft is harvested for zygomatic complex reconstruc­
tion. C, The full-thickness cranial bone graft is fixed in place
with microscrews. The lateral canthus is identified through
the coronal incision, and a 28-gauge stainless steel wire is
passed through it. The lateral canthus is pexied to the new
frontozygomatic region. From Posnick JC, Goldstein JA,
Waitzman AA: Surgical correction of the Treacher-Collins
malar deficiency: quantitative CT scan analysis of long-term
results, Plast Reconstr Surg 92:12-22, 1993.
C
A
B

1113
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
This author believes that the reconstruction of the
cranial vault, malar, and orbital deficiencies before the age
of 7 years is not indicated unless functional disability
warrants it.213 After the patient reaches this age, cranio–
orbitozygomatic skeletal development is nearly mature.
When indicated, an adult-sized anterior cranial vault, orbit,
and zygomatic complex may be constructed and matched
with the contralateral normal side with little concern about
how future growth will alter the initial results achieved.
Donor site skull reconstruction is also simpler after the age
of 7 years than it is in younger children, because the pres­
ence of bicortical cranial bone will facilitate the splitting of
the inner and outer tables for efficient reconstruction. Arti­
ficial bone material may also be used to reconstruct the graft
donor site of the posterior skull (see Chapter 27).
Other surgeons have recommended variations in the
methods and timing of the reconstruction of the upper face
skeletal anomalies associated with HFM.31 Posnick and col­
leagues confirmed that the construction of the complete
zygomatic complex with full-thickness autogenous cranial
bone maintains volume and shape better than onlay grafts,
which have been universally disappointing. Over time,
onlay autogenous bone grafts that have been placed in the
craniofacial region (e.g., supraorbital ridge, zygoma, ante­
rior maxilla, angle of mandible, chin) from all tried donor
sites (e.g., skull, hip, rib) have demonstrated significant and
unpredictable resorption. Furthermore, no commensurate
growth (i.e., volume expansion) of the graft with the under­
lying bone (e.g., the zygoma) should be expected. Some
feel that, even if the onlay graft partially resorbs, at least
something has been gained; however, this philosophy should
be abandoned, because surface irregularities result in sec­
ondary deformities that further detract from the baseline
malformation.
Maxillomandibular Reconstruction
An essential consideration when choosing the timing and
techniques for maxillomandibular reconstruction in a
patient with HFM is an understanding of the presenting
TMJ–mandibular anatomy.2,6,7,20,25,30-32,43,46,59,96,97,180,209-212,226
The classification of the TMJ–mandibular malformation,
which was described earlier in this chapter, is clinically
relevant and facilitates communication.94,157 In the patient
with HFM with either a Type I or Type IIA malformation,
the basic maxillomandibular skeletal asymmetry and dys­
morphology that requires reconstruction include the fol­
lowing: 1) degrees of altered facial height (i.e., decreased
posterior facial height on the ipsilateral side); 2) diminished
horizontal projection (i.e., deficiency more prominent on
the ipsilateral side); and 3) decreased facial width (i.e. defi­
ciency on the ipsilateral side). These malformations fre­
quently result in canting (i.e., roll orientation) of the
pyriform apertures, the maxilla, and the gonial angles; the
shifting of the maxillary, mandibular, and chin midlines off
of the facial midline (i.e. yaw orientation); clockwise
rotation of the occlusal plane (i.e., pitch orientation); and
an asymmetric class II malocclusion often with anterior
open-bite malocclusion. According to the classification
described, a patient who presents with a Type IIB malfor­
mation requires the construction of a neocondyle. For the
individual with a Type III malformation in addition to this,
there is a need for the construction of a neo–glenoid fossa.
The most favorable long-term function (i.e., speech,
swallowing, chewing, breathing) and enhanced facial aes­
thetics are generally achieved in patients who undergo
definitive reconstructive surgery of the maxilla, mandible,
and chin regions at any time after jaw maturity (i.e., at 13
to 15 years of age for the HFM patient); this should be
carried out in combination with effective orthodontic treat­
ment after the permanent teeth have erupted and before the
patient graduates from high school. As with other dentofa­
cial deformities, extractions may be required to orthodonti­
cally relieve dental root crowding and to normalize the
inclination of the anterior teeth in preparation for the surgi­
cal repositioning of the jaws.
Type I and IIA mandibular malformations are best
reconstructed after all of the permanent teeth have erupted
and orthodontic goals have been reached (see Fig. 28-4, A,
B, and C). Surgical objectives can be met by making use of
sagittal split ramus osteotomies of the mandible in combi­
nation with Le Fort I osteotomy (often in segments) and
osseous genioplasty. This combination represents standard
techniques, and it does not require bone grafts. The success
of reconstruction is dependent on approximating mirror-
image symmetry and Euclidian proportions of the skeleton
through orthognathic procedures.
For Type IIB mandibular malformations, costochondral
graft reconstruction of the deficient condyle ascending
ramus at the time of skeletal maturity remains this surgeon’s
preferred approach in most cases (see Fig. 28-4, D), despite
its limitations (see the section to follow concerning condylar
reconstruction with the use of costochondral graft). A sagit­
tal split ramus osteotomy is completed on the contralateral
side to derotate the distal mandible. This is combined with
a Le Fort I osteotomy (often in segments) and an osseous
genioplasty.
A ramus osteotomy of the contralateral side of the man­
dible is completed first to control repositioning of the distal
mandible. The distal mandible is then secured to the maxilla
via intermaxillary fixation through a custom designed acrylic
splint to create the preferred reorientation of the lower jaw
(see Chapter 14). It may be necessary to resect the ipsilateral
coronoid process before repositioning the distal mandible.
The contralateral ramus osteotomy is then rigidly fixed with
bicortical screws. The ipsilateral proximal mandible is recon­
structed with the autogenous costochondral graft. Harvest­
ing the rib graft from the contralateral chest wall provides
the best contour for mandibular reconstruction. The fixation
of the rib graft to the native distal mandible is accomplished
with a titanium miniplate and 2.0-mm or 2.3-mm screws.
The fixation plate extends from the graft forward along the

1114 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
inferior border of the body of the mandible (fig. 28-15). It
is generally necessary to recontour (i.e., with a bur on a
rotary drill) the outer cortex of the distal mandible before
onlaying the graft. Graft placement and fixation is often
carried out through an extraoral Risdon neck incision. The
avoidance of intraoral incisions on the ipsilateral mandibular
ramus region during this procedure may minimize the inci­
dence of infection. The effective seating of the neocondyle
in the glenoid fossa is a critical step for successful reconstruc­
tion. In some patients, the extent of associated soft-tissue
and skeletal deficiency will favor use of a vascularized fibula
composite flap over the use of a costochondral graft.
The Type III glenoid fossa–mandibular malformation
requires the surgical construction of the congenitally
missing parts (see Fig. 28-4, E). The mandibular recon­
struction is generally carried out as previously described for
the Type IIB deformity. When the glenoid fossa requires
construction (i.e., with a Type III malformation), so will
the zygomatic complex. The glenoid fossa–zygoma and
orbital reconstruction are best carried out as a separate
operation when the patient is at least 7 years old (see the
previous section about orbito–zygomatic reconstruction)
and before mandibular reconstruction. With regard to the
mandibular reconstruction, the deficiency of both the
condyle-ascending ramus and the associated soft tissues
may benefit from a vascularized fibula composite flap
rather than a costochondral graft.
Consideration of First-Stage
Mandibular Reconstruction
during the Mixed Dentition
Personal Perspective Concerning
First-Stage Mandibular Reconstruction
during Childhood
The maxillofacial malformations in patients with HFM may
be severe enough that, for airway or psychosocial reasons,
the clinician feels compelled to recommend first-stage man­
dibular reconstruction during the mixed detention (i.e.,
between the ages of 7 and 11 years). Reported options for
first-stage mandibular reconstruction in patients with HFM
generally include 1) a ramus/body osteotomy followed by
DO carried out over time for Type I and Type IIA malforma­
tions or 2) costochondral grafting with the immediate con­
struction of a neocondyle ascending ramus for Type IIB and
Type III malformations. In either case, derotation of the
mandible to position the chin in the facial midline with the
creation of an ipsilateral posterior open bite is usually con­
sidered the objective. The reconstructive option selected
(i.e., costochondral graft versus DO) should be based on the
presenting malformation and then on the following: 1) the
three-dimensional morphologic results believed to be achiev­
able; 2) the anticipated ongoing growth potential after
surgery; 3) the differences in perioperative morbidity among
the techniques; and 4) the overall burden of treatment to the
patient, the family, the clinicians, and the health care
system.209-212
Interestingly, most published reports of first-stage man­
dibular reconstruction in patients with HFM do not fully
clarify the details of the presenting dysmorphology of the
native glenoid fossa and condylar components before
surgery (Figure 28-9). As stated, for DO to be effective, a
functional glenoid fossa and an adequate condyle must be
present (i.e., in the presence of Type I or Type IIA malfor­
mation). Unfortunately, a high incidence of bony and
fibrous ankylosis has been reported in conjunction with
mandibular DO in HFM.49 In addition, the consistent
occurance of “undergrowth” after mandibular DO recon­
struction carried out during the mixed dentition was con­
firmed by Meazzini and colleagues.143 Those authors
conducted a comparison study of long-term follow up until
the completion of facial growth of two homogenous samples
of children with HFM. The experimental group was treated
with mandibular DO during the deciduous or early mixed
dentition in an attempt to correct the mandibular defor­
mity. The control group was not subjected to any treatment
until adulthood. The experimental group included children
(n = 14) who underwent mandibular ramus osteotomies
with DO (mean age, 5.9 years) with a mean follow up of
11.2 years. With the use of quantitative measurements on
serial panorex radiographs, the DO group was compared
with the control group (n = 8). The study results document
that facial proportions in patients with HFM are main­
tained throughout growth when no treatment is under­
taken. Unfortunately, after ramus osteotomies with DO, the
mandibular disproportions returned to their original level
of asymmetry during growth. The authors concluded the
following: 1) HFM does not progress with regard to the
degree of facial asymmetry or deformity when the patient
is left to mature naturally and 2) early intervention with
mandibular ramus osteotomies and DO does not effectively
reduce long-term facial asymmetry in the patient with
HFM.
Ongoing problems when choosing the costochondral
graft option to be carried out during the mixed dentition
for the Type IIB or III malformation include overgrowth,
undergrowth, ankylosis, and resorption of the mandible
(Fig. 28-10). The overgrowth concerns are diminished but
not eliminated when the costochondral graft procedure is
carried out in a child who is in the late mixed dentition
stage (i.e., 9 to 11 years old) and in whom only a minimum
amount of cartilage (i.e., ≈2 mm) is left at the articulating
surface of the graft (see section to follow concerning con­
dylar reconstruction with costochondral graft).
If a first-stage mandibular procedure is carried out during
the mixed dentition for the Type IIB or III malformation,
then the need for definitive mandibular reconstruction in
conjunction with a Le Fort I maxillary osteotomy and an
osseous genioplasty at the time of skeletal maturity should
be anticipated.3,16,28,27 The idea of carrying out a mixed
dentition mandibular reconstruction to avoid definitive
Text continued on p. 1121

1115
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
• Figure 28-9  A Hispanic girl who was born with a mild form of left-sided hemifacial microsomia. The mandibular malformation was Type I. At
another institution, she underwent a mandibular distraction procedure when she was 4 years old with an external device; the goals were to “dero­
tate” the mandible, to shift the chin to the midline, and to create a posterior open bite. An acrylic splint was then used in an attempt to hypererupt
the maxillary molars over time. The patient is shown at the time of procedure and then 7 years later after her referral to this surgeon. Unfortunately,
the procedure carried out did not improve the occlusion or correct the facial asymmetry. During the process, the left mandibular permanent molars
(i.e., the first, second, and third molars) were injured, and secondary deformities of the mandible also resulted. A, The child is shown when she
was 4 years old, during the mandibular distraction. B, She is also shown at 7 years of age note the distortion and deformity of the mandible and
the facial scarring. C, Residual malocclusion as a result of the external distraction. D, Panorex radiograph taken when the child was 11 years old
(i.e., 7 years after the distraction) that illustrates the injuries to the first, second, and third permanent molars on the distracted side.
D
Distraction osteogenesis
injured permanent molars
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1116 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
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ry
ry
ry
ry
ry
ry
ry
ry
ry
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ry
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ry
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ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C Pos
os
os
os
osnicick
A
Newborn
Newborn
B
• Figure 28-10  A newborn with left-sided hemifa­
cial microsomia. She had a good airway as well as
swallowing and chewing abilities. She was treated
at another institution until she was referred to this
surgeon when she was 15 years old. A, B, and C,
Facial and computed tomography (CT) scan views
taken during infancy that indicate microtia with an
absent external auditory canal, hypoplasia and cleft­
ing through the zygomatic arch, and mandibular
malformation (Type IIB) with a shortened posterior
facial height and a rudimentary condyle.

1117
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
©Elsevier Inc.
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©Elsevier Inc.
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©Elsevier Inc.
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ry; J.C.
C.
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C.
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osnicick
E
5 years of age
3 years of age
D
D, Frontal and CT scan views taken when the patient was
3 years old indicate no progression of the malformation. E, The same child when she
was 5 years old just before undergoing a mandibular procedure at another institution.
At surgery the mandible was derotated to shift the chin to the midline with the creation
of a posterior open bite on the ipsilateral side. A costochondral graft was harvested
and used to reconstruct the left condyle ascending ramus. An acrylic splint was then
worn for several years in an attempt to “grow” the left maxilla and relieve the posterior
open bite. The external ear was later reconstructed, but with suboptimal results.
• Figure 28-10, cont’d 
Continued

1118 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
Orth
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r ery; J.C. Posnick
Orth
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r ery; J.C. Posnick
F
15 years of age
15 years of age
G
F, G, H, and I, Facial and CT scan views of the patient when she was 15 years old, at the time of referral to this surgeon
(i.e., 10 years after costochondral grafting) and without other interventions. There has been extensive overgrowth of the costochondral graft previ­
ously placed on the left side of the mandible. This has negatively altered (distorted) the growth of the whole mandible and the maxilla, and it has
also resulted in limited mandibular mobility. There is gross distortion of the entire face, with negative effects on the airway, speech articulation,
chewing ability, lip closure/posture, and self-esteem.
• Figure 28-10, cont’d

1119
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
Orth
r ognathic Surg
r ery; J.C. Posnick
Orth
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r ery; J.C. Posnick
H
I
• Figure 28-10, cont’d 
Continued

1120 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
J
3 years
15 years
3 years
15 years
K
L
3 years
15 years
3 years
15 years
M
J, K, L, and M, Comparable CT scan
views are shown when the patient was 3 years old (i.e., before
rib grafting) and at the age of 15 years (i.e., 11 years after rib
grafting).
• Figure 28-10, cont’d

1121
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
Osteodistraction Reconstruction of the
Mandible during Childhood
Since 1992, DO has been used as a technique in an attempt
to correct mandibular morphology in young patients with
HFM.* In 2002, a literature overview was published by
Mommaerts and colleagues to address the long-term
results of early DO among patients with HFM.148 It was
concluded that no convincing evidence supported the ben­
efits of DO over other techniques for the treatment of
HFM, and DO was not found to offer advantages with
regard to the limiting of complications. In 2009, Nagy and
colleagues published a critical and exhaustive literature
review in search of evidence of long-term stability after early
distraction osteogenesis of the mandible in patients with
HFM.163 Eighty-nine relevant articles were reviewed. Only
13 studies were found to have sufficient scientific merit to
be included. The authors concluded the following:
1. The majority of the studies reported unstable results
after DO with regard to facial symmetry that espe­
cially affected ramus height.
2. Clinicians frequently hypothesized that the volume
of the soft tissues of the affected side would increase
after DO in patients with HFM. Interestingly, the
only published study that used a volumetric evalua­
tion showed no postsurgical improvement of the
soft-tissue deficiency on the affected side after DO
reconstruction.
3. Even after accepting the shortcomings of certain
study designs and evaluation methods, none of the
published studies demonstrated the convincing long-
term stability of the mandibular dimensions after DO
mandibular elongation.
4. Relapse in regeneration after DO was seen in the
majority of the studies that used reliable evaluation
methods.
5. Repeated DO was reported to be necessary when
pursuing attempts to maintain facial symmetry during
growth in the patient with HFM. Therefore, any
gains in mandibular dimensions after early DO were
not stable, whether as a result of relapse or growth
impairment.
6. In published reports, most children with HFM were
still rated as “unattractive” after surgery and showed
no “improvement in self-esteem.” Children with
HFM have an elevated risk for childhood psychoso­
cial difficulties.
7. If early DO is the treatment that is selected to be
carried out during childhood for HFM, the patient
maxillomandibular orthognathic reconstruction during
adulthood is generally unrealistic.
The rationale for postponing the correction of the den­
tofacial deformity until the time of “early” skeletal maturity
is the same as that for the correction of the jaw deformity
associated with a repaired cleft palate and most other routine
dentofacial deformities (e.g., long face growth pattern,
primary mandibular deficiency, maxillary deficiency in com­
bination with relative mandibular excess). This rationale
includes the consideration of the following: 1) avoiding
injury to the developing permanent dentition and the infe­
rior alveolar nerves 2) avoiding soft-tissue scarring 3) avoid­
ing the loss of ramus marrow space that will complicate the
success of necessary redo ramus osteotomies 4) minimizing
negative psychosocial memories of earlier jaw surgery that
may prevent the patient and family from later pursuing
definitive reconstruction and 5) avoiding the iatrogenic
three-dimensional deformation of the mandible that limits
long-term success. In addition, any orthodontics carried out
during the mixed dentition will not be reflective of the
permanent dentition; a final course of braces would also be
required.
Patients with HFM who have a significant degree of
vertical, horizontal, and transverse maxillary deformity will
also require Le Fort I maxillary osteotomy in segments. No
lesser approach will be sufficient. The surgical objectives of
the upper jaw surgery may include the following:
1. Cant correction of the maxilla with the asymmetric
intrusion of the anterior maxilla to establish a more
normal upper lip–to–tooth relationship in repose and
during smiling as well as to improve lip competence
(i.e., roll orientation)
2. Correction of the maxillary dental midline to the facial
midline with control of the yaw orientation to achieve
an improved cheek region appearance and a better
angle of mandibular symmetry
3. Either extrusion or intrusion of the posterior maxilla
on each side, depending on the extent of counterclock­
wise rotation required to achieve adequate horizontal
projection of the mandible and chin (i.e., pitch
orientation)
4. Widening of the maxilla (i.e., segmentation) to correct
crossbites and to add facial fullness
5. Horizontal advancement to improve midface projec­
tion and to open the upper airway
6. Bone grafting of the zygomatic buttress on the ipsilat­
eral side to improve facial symmetry
7. Provision of access for intranasal procedures (e.g., sep­
toplasty, inferior turbinate reduction, recontouring of
the pyriform rims and the nasal floor).
Safe and effective first-stage Le Fort I osteotomies carried
out during the mixed dentition are not practical as a result
of the location of the developing teeth and the expected
inhibition of any postoperative horizontal maxillary growth.
*References 1, 4-6, 21, 23, 34, 39, 55, 58, 63, 68, 71, 72, 74, 79, 80,
82, 87, 100, 109-111, 113-116, 132, 136, 138-140, 144, 146-148, 150,
171, 173, 179, 182, 200, 202, 221, 222, 225, 232, 237, 241, 242, 246,
250, 252, 254, 257, 262, 265, 278, 279, 283, 284, 286

1122 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
*References 2, 22, 37, 44, 45, 48, 60, 73, 118, 120, 126-128, 156, 158,
159, 160-162, 169, 177, 178, 183-192, 194, 195, 201, 209-211, 219,
224, 235, 285
and family must accept that the procedure will have
to be repeated later during the patient’s life.
Costochondral Graft Reconstruction of the
Mandible during Childhood
There are several basic theories regarding the mechanism of
how mandibular growth occurs:
1. One theory of mandibular growth involves chondro­
blastic proliferation (i.e., cartilage formation with sub­
sequent osseous replacement). Linear growth is
primarily thought to be the result of condylar carti­
lage proliferation pushing the mandible forward and
downward.
2. A second theory is the functional matrix theory, which
explains mandibular growth primarily as a result of
facial soft-tissue growth with subsequent projection
(or pulling) of the mandible downward and forward
in response to the forces of function. The condyle
(and the condylar cartilage) is thought to merely sec­
ondarily react to the stimulating functional matrix
forces that draw the mandible downward and forward.
This is the theory behind the use of a Herbst appli­
ance to “grow” a small mandible.
3. A third line of thinking is that both theories have
merit. This explanation holds that a condylar growth
center (i.e., chondroblastic proliferation) is present
but that it is at least somewhat influenced by the
physiologic function (i.e., functional matrix) of the
mandible.
When the condyle and the components of the ascending
ramus are hypoplastic or absent in patients with conditions
such as HFM, surgeons have selected varied donor sources
to replace the missing parts.* Autogenous replacements for
the condyle have included 1) the metatarsal bones 2) the
proximal head of the fibula 3) the costochondral junction
of the rib (CCJ/rib) and 4) the sternoclavicular joint.
In general, the CCJ/rib bone is an expendable donor site
and often an ideal morphologic contour match, with ade­
quate strength at the mandibular recipient site. For these
reasons, the CCJ/rib bone has been commonly used as a
substitute for the “condylar growth center” and for the
replacement of components of the ascending ramus. Unfor­
tunately, in the skeletally immature patient, clinical observa­
tion after transplantation confirms unpredictable growth of
the graft as compared with what would be considered
“normal” growth or growth that is equivalent to the contra­
lateral “normal” side of the mandible.
Clinical experience indicates that the CCJ/rib graft
that is placed in the child or young teenager is prone to
excessive growth. Although some would argue that over­
growth of the mandible and the need for additional
surgical correction is a relatively minor consequence, expe­
rienced clinicians have found the secondary distortions in
the maxillomandibular complex difficult to correct in a
satisfactory way as compared with what could be achieved
by single-stage reconstruction at the time of early skeletal
maturity (Fig. 28-11). Unless pressing functional issues
occur during childhood (e.g., obstructive sleep apnea), the
advantages of a single-stage approach at jaw growth matu­
rity to achieve favorable long-term function and aesthetic
enhancement are difficult to ignore (Figs. 28-12 through
28-16).
Scientists began to focus on the biodynamics and growth
of the transplanted CCJ/rib during the early 1990s.
Researchers have addressed questions about differences in
the biologic nature of the cartilage of the CCJ/rib; the
epiphyseal growth plate; the mandibular condyle; and the
sternoclavicular joint. It is now clear that the cartilage asso­
ciated with the CCJ/rib and the epiphyseal plate of a long
bone are derived from the primary cartilaginous template
of the skeleton. These cartilages are relatively resistant to
extrinsic competitive growth-related factors. Alternatively,
the mandibular condyle and sternoclavicular cartilages arise
from their own blastemas in association with the perios­
teum and then form as secondary cartilages.
Peltomaki and colleagues used a rat model to analyze the
dynamic histology that occurred with the growth of the
CCJ/rib.183-192 The observed histologic findings are depen­
dent on the amount of cartilage that remains on the autog­
enous CCJ at the time of transplant. One of the initial
findings of these authors was that “costochondral grafts do
not adapt to the new environment, but rather preserve their
inherent growth potential.” In subsequent studies, Pelto­
maki and colleagues continued to explore the idea of
whether the intrinsic growth of the CCJ/rib may also be
influenced by the interaction of hormonal factors and
masticatory movements.183-192 It is now clear that variations
in mandibular growth after the use of a CCJ/rib graft in
both humans and experimental animal model simulations
likely vary in accordance with the amount of cartilage used
in the graft.
Peltomaki notes that, in the CCJ/rib the distance from
the bone cartilage junction to the germinative zone is
approximately 0.4 mm to 0.5 mm. It is evident that the
germinative cells will invariably be included in a CCJ/rib
graft. It is also evident that, in clinical practice, considerable
variation in the amount of germinative cells maintained will
occur. The reported clinical differences in mandibular
growth after the replacement of the condylar process with
a CCJ/rib graft is likely the result of variations in the
amount of cartilage included in each graft. Peltomaki
believes that the number of germinative cells has a direct
impact on rib elongation (i.e., overgrowth). Clinicians
should be cognitive of the fact that the amount of cartilage
included in the graft is of significance with respect to the
Text continued on p. 1149

1123
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
Orth
r ognathic Surg
r ery; J.C. Posnick
A
A
Roll Orientation Correction
• Figure 28-11  The 15-year-old girl who was born with hemifacial microsomia on the left side of the face and who was shown in Fig. 28-10 is
shown at the time of referral to this surgeon. It has now been 10 years since she underwent left mandibular reconstruction with a costochondral
graft. The mandibular opening was limited to approximately 23 mm but without bony ankylosis. The physical findings were the result of both the
original malformation and the surgical intervention earlier during her life. She then underwent a combined orthodontic and surgical approach with
this surgeon. A, Facial and computed tomography scan views taken when the patient was 15 years old indicate the extent of deformity and asym­
metry. Articulated dental cast indicates analytic model planning to derotate the maxillomandibular complex (i.e., roll orientation correction).
Continued

1124 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
Orth
r ognathic Surg
r ery; J.C. Posnick
Orth
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r ery; J.C. Posnick
Orth
r ognathic Surg
r ery; J.C. Posnick
Orth
r ognathic Surg
r ery; J.C. Posnick
B
C
B, Facial views in repose before and after reconstruction. C, Frontal views with smile before and after
reconstruction.
• Figure 28-11, cont’d

1125
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
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r ery; J.C. Posnick
Orth
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r ery; J.C. Posnick
Orth
r ognathic Surg
r ery; J.C. Posnick
Orth
r ognathic Surg
r ery; J.C. Posnick
D
E
D, Right oblique facial views before and after reconstruction. E, Right profile views before and after
reconstruction.
• Figure 28-11, cont’d 
Continued

1126 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
Orth
r ognathic Surg
r ery; J.C. Posnick
Orth
r ognathic Surg
r ery; J.C. Posnick
Orth
r ognathic Surg
r ery; J.C. Posnick
Orth
r ognathic Surg
r ery; J.C. Posnick
F
G
F, Left oblique facial views before and after reconstruction. G, Left profile views before and after
reconstruction.
• Figure 28-11, cont’d

1127
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
• Figure 28-11, cont’d 
I
H
H, Occlusal views before and after reconstruction. I, Lateral cephalometric radiographs before and after
reconstruction.

1128 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
©Elsevier Inc.
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Orthththththththth
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Surg
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Orthththththth
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C.
C Po
Po
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5 years
10 years
1 year
14 years
©Elsevier Inc.
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C.
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Or
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Orththththththththththththththth
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snicicicicicicicicicick
• Figure 28-12  A child who was born with right-sided hemifacial microsomia (Type IIA mandible) was
followed up longitudinally without skeletal interventions until jaw maturity. No progression of the mal­
formation occurred with growth. The patient had nasal obstruction and forced mouth breathing, and
she developed a long face growth pattern. There is clockwise rotation of the maxillomandibular
complex with a retrognathic profile (i.e., pitch orientation). The chin is also vertically long and retrusive.
There is severe canting of the maxillomandibular complex (i.e., roll orientation). The patient was referred
to this surgeon and agreed to further orthodontic treatment and orthognathic surgery. Her procedures
included maxillary Le Fort I osteotomy (vertical intrusion, counterclockwise rotation, and correction of
cant); bilateral sagittal split ramus osteotomies (correction of asymmetry, counterclockwise rotation,
and horizontal advancement); osseous genioplasty (vertical reduction and horizontal advancement);
and septoplasty, inferior turbinate reduction, and nasal floor recontouring. A, Frontal views from 6
months to 14 years of age.

1129
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
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Orth
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Orth
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B
C
B, Frontal views in repose before and after reconstruction. C, Frontal views with smile before and after
reconstruction.
• Figure 28-12, cont’d 
Continued
NOTE: The suboptimal external ear reconstruction performed by another surgeon.

1130 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
Orth
r ognathic Surg
r ery; J.C. Posnick
Orth
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Orth
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D
E
D, Profile views before and after reconstruction. E, Worm’s-eye views before and after reconstruction.
• Figure 28-12, cont’d

1131
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
F
Roll Orientation Correction
G
Asymmetry correction
F, Occlusal views before and after reconstruction. G and H, Articulated dental casts indicate analytic
model planning.
• Figure 28-12, cont’d 
Continued

1132 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
I
H
Maxillary counter-
clockwise rotation
Mandibular counter-
clockwise rotation
I, Lateral cephalometric radiographs before and after reconstruction. C,D, From Posnick JC: Hemi­
facial microsomia: evaluation and staging of reconstruction, J Oral Maxillofac Surg 56:646, 1998.
• Figure 28-12, cont’d

1133
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
Orth
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Orth
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Orth
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r ery; J.C. Posnick
Orth
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u g
r ery; J.C. Posnick
A
B
• Figure 28-13  A teenage girl who was born with a mild form of left-sided hemifacial microsomia. She presented to this surgeon as
a teenager with a lifelong history of difficulty breathing through the nose, a long lower anterior facial height (i.e., gummy smile and lip
incompetence), facial asymmetry, and retrusive jaws in profile. Her external ears were also malformed. She agreed to a combined ortho­
dontic and surgical approach. The patient’s procedures included maxillary Le Fort I osteotomy in segments (horizontal advancement,
vertical intrusion, cant correction, and transverse widening); bilateral sagittal split ramus osteotomies (correction of asymmetry and hori­
zontal advancement); osseous genioplasty (vertical shortening and horizontal advancement); and septoplasty, inferior turbinate reduction,
and recontouring of the nasal floor. A, Frontal views in repose before and after reconstruction. B, Frontal views with smile before and
after reconstruction.
Continued

1134 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
Orth
r ognathic Surg
r ery; J.C. Posnick
Orth
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u g
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Orth
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r ery; J.C. Posnick
Orth
r ognathic Sur
u g
r ery; J.C. Posnick
C
D
C, Oblique facial views before and after reconstruction. D, Profile views before and after reconstruction.
• Figure 28-13, cont’d

F
Arch expansion
G
Roll Orientation Correction
E
E, Occlusal views with orthodontics in progress and after treatment. F, Articulated dental casts that
indicate analytic model planning. G, Lateral cephalometric radiographs before and 1 year after reconstruction.
  NOTE: The planned correction of pitch, yaw and roll orientation to enhance facial aesthetics.
• Figure 28-13, cont’d

1136 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
C
©Elsevier Inc.
Or
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Or
Or
Or
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©Elsevier Inc.
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• Figure 28-14  A girl who was born with a severe form of
hemifacial microsomia (right side) that included extensive
involvement of the upper facial skeleton (including micro-
ophthalmia, lower facial skeleton issues (Type IIA mandibular
malformation), and the external ear. She had undergone
multiple intracranial cranio–orbital procedures, with limited
success. She was referred to this surgeon as a teenager,
and she underwent two additional reconstructive proce­
dures. The first included cranio–orbito–zygomatic recon­
struction through a coronal scalp incision (i.e., an intracranial
approach) that required osteotomies of the cranial vault, the
orbit, and the zygoma; this included autogenous cranial
bone grafting. The second required orthognathic surgery,
including a Le Fort I osteotomy (cant correction and horizon­
tal advancement), bilateral sagittal split ramus osteotomies
(asymmetry correction and horizontal advancement), and an
osseous genioplasty (horizontal advancement) in combina­
tion with orthodontic treatment. The patient is shown before
and after the two described procedures. A, Frontal views in
repose before and after reconstruction. B, Worm’s-eye view
before surgery and frontal view with smile after reconstruc­
tion. C, Right and left profile views after reconstruction.

1137
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
• Figure 28-14, cont’d 
E
F
Roll Orientation Correction
D
D, Occlusal views before and after reconstruction. E, Articulated dental casts that indicate model planning.
F, Lateral cephalometric radiographs before and after reconstruction.
Continued

1138 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
G
Donor site
H
G, Intraoperative views of upper orbits before and after redo cranio-orbit zygomatic reconstruction. H, Intraoperative
views of cranial vault before and after redo cranio-orbito-zygomatic reconstruction. A,B,D, From Posnick JC: Hemifacial microsomia: evaluation
and staging of reconstruction, J Oral Maxillofac Surg 56:648, 1998.
• Figure 28-14, cont’d

1139
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
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Age 11
Age 13
Age 14
• Figure 28-15  A boy who was born with hemifacial microsomia involving the right side of face. There was also a Type IIB mandibular malforma­
tion. The patient was followed longitudinally without treatment intervention until he approached skeletal maturity. Facial views demonstrate no
progression of the malformation. After the patient was in the adult dentition and his orthodontic decompensation was complete, he underwent
single-stage reconstruction that included Le Fort I osteotomy in segments (cant correction, horizontal advancement arch expansion, and vertical
adjustment); left-sided sagittal split ramus osteotomy; right mandibular reconstruction with a costochondral graft (horizontal advancement and
asymmetry correction); osseous genioplasty (asymmetry correction and horizontal advancement); and septoplasty, inferior turbinate reduction, and
bilateral otoplasty (i.e., ear set-back). A, Frontal facial views in repose from the ages of 11 to 14 without treatment intervention indicate no progres­
sion of deformity. B, Illustrations of the presenting skeletal deformities and of the reconstruction that was carried out. Part B modified from an original
illustration by Bill Winn.
Continued