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ORIGINAL ARTICLE

Combined maxillary and mandibular


distraction osteogenesis in patients with
hemifacial microsomia
 rgia W. T. Lau,b Mariana Marquezan,c Mo
^ nica Tirre de Souza Arau
 jo,a
Eduardo Franzotti Sant'Anna,a Geo
d
d
John W. Polley, and Alvaro A. Figueroa
Rio de Janeiro, Brazil, and Chicago, Ill

Introduction: Hemifacial microsomia is a deformity of variable expressivity with unilateral hypoplasia of the
mandible and the ear. In this study, we evaluated skeletal soft tissue changes after bimaxillary unilateral vertical
distraction. Methods: Eight patients (4 preadolescents 4 adolescents) each with a grade II mandibular deformity
underwent a LeFort I osteotomy and an ipsilateral horizontal mandibular ramus osteotomy. A semiburied distraction device was placed over the ramus, and intermaxillary xation was applied. Anteroposterior cephalometric
and frontal photographic analyses were conducted before and after distraction. Statistics were used to analyze
the preoperative and postoperative changes. Results: Cephalometrically, the nasal oor and the occlusal and
gonial plane angles decreased. The ratios of affected-unaffected ramus and gonial angle heights improved by
15% and 20%, respectively. The position of menton moved toward the midline. The photographic analysis
showed a decrease of the nasal and commissure plane angles, and the chin moved to the unaffected side.
The parallelism between the horizontal skeletal and soft tissue planes improved, with an increase in the
affected side ramus height and correction of the chin point toward the midline. Conclusions: Simultaneous
maxillary and mandibular distraction improved facial balance and symmetry. Patients in the permanent dentition
with xed orthodontic appliances and well-aligned dental arches responded well to this intervention. (Am J
Orthod Dentofacial Orthop 2015;147:566-77)

emifacial microsomia (HFM) is the best known


branchial arch syndrome1-3 and the second
most common craniofacial birth defect after

a
Associate professor, Department of Pedodontics and Orthodontics, School of
Dentistry, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil; formerly,
postdoctoral fellow, Department of Anatomy and Rush Craniofacial Center,
Rush University Medical Center, Chicago, Ill.
b
PhD student, Department of Pedodontics and Orthodontics, School of Dentistry,
Federal University of Rio de Janeiro, Rio de Janeiro, Brazil; formerly, postdoctoral
fellow, Department of Anatomy and Rush Craniofacial Center, Rush University
Medical Center, Chicago, Ill.
c
Postdoctoral fellow, Department of Pedodontics and Orthodontics, School of
Dentistry, Federal University of Rio de Janeiro; Brazilian Army dentist, Santa
Maria, Rio de Janeiro, Brazil.
d
Codirector, Craniofacial Center, Department of Plastic and Reconstructive Surgery, Rush University Medical Center, Chicago, Ill.
All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conicts of Interest, and none were reported.
Eduardo Franzotti Sant0 Anna and Georgia W.T. Lau are recipients of scholarships
from Coordenac~ao de Aperfeicoamento de Pessoal de Nvel Superior (CAPES),
and Eduardo Franzotti Sant0 Anna is recipient of grants n. E-26/171.246/2006
and n. E-26/111.647/2010 from Fundac~ao de Amparo a Pesquisa do Estado
do Rio de Janeiro (FAPERJ), Brazil.
Address correspondence to: Alvaro A. Figueroa, Craniofacial Center, Rush University Medical Center, 1725 W Harrison St, Suite 425, Professional Bldg I, Chicago,
IL 60612; e-mail, Alvaro_Figueroa@rush.edu.
Submitted, April 2014; revised and accepted, December 2014.
0889-5406/$36.00
Copyright 2015 by the American Association of Orthodontists.
http://dx.doi.org/10.1016/j.ajodo.2014.12.027

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cleft lip and palate.4-6 It occurs in 1:35005 to 1:56006


live births. The term HFM refers to an asymmetric
congenital condition of variable expressivity affecting
primarily aural, oral, and mandibular development.
HFM can be part of a broader and variable phenotypic
spectrum known as oculoauriculovertebral dysplasia
and always involves mandibular and ear malformations,
but its severity varies.7,8 The mandibular deciency
usually is associated with microtia, macrostomia, and
craniomaxillofacial asymmetry on the affected side.
The maxilla, temporal bone, and orbit are also
affected as a result of the primary malformation and
not secondarily affected by the mandibular hypoplasia,
as suggested by some.6 In addition to the skeletal components, neural, muscular, and soft tissues are also
affected. The mandibular deformity in HFM was classied by Pruzansky2 in 1969. In his classication, a grade
I mandibular deformity consists of a normally shaped
but small mandible. Grade II is a small and abnormally
shaped mandibular ramus. In grade III, the mandibular
deformity is characterized by absence of the mandibular
ramus including the temporomandibular joint.
The treatment of HFM is centered on the mandibular
deformity, but surgical timing is still controversial. The

Sant'Anna et al

reason for this controversy relates to what clinicians


believe is the facial growth potential in patients with
HFM. Those who support early reconstruction, before
skeletal maturity, assume that the mandibular skeletal
asymmetry will worsen with time, and that early reconstruction will prevent secondary growth deformities.5,9-11 However, studies have found that the
growth of the mandible on the affected side parallels
that of the unaffected side, with the degree of
asymmetry remaining relatively constant throughout
craniofacial development.12-14
Another concern is the psychological adjustment
problems caused by differences in facial appearance.15,16
Some clinicians consider that the surgery should be done
earlier to prevent social adjustment problems. Others,
who prefer to wait for the completion of growth,
believe that expectations of reconstructive surgery can
cause disillusionment when performed too early,
because the patient will still grow asymmetrically and
additional surgery will be required.
The critical step in achieving better facial skeletal harmony is to restore maxillary and mandibular symmetry.
Facial asymmetry is a main indication for orthognathic
surgery. Traditionally, skeletal hypoplastic malformations
are corrected with segment repositioning with autogenous bone grafts to increase the volume and size.17
Recently, in patients with HFM, distraction osteogenesis has been used for correcting mandibular asymmetry.8,18 Mandibular elongation by gradual
distraction is mainly indicated in HFM patients with
mandibular deformities grades I and II.19 Since HFM primarily affects not only the mandible but also the maxilla
and the orbit, clinicians have suggested simultaneous
maxillary and mandibular interventions to correct these
asymmetries with a single procedure.19-21 The purpose
of this study was to assess skeletal and soft tissue
changes after simultaneous maxillary and mandibular
distractions in patients with HFM.
MATERIAL AND METHODS

Eight patients with HFM grade II mandibular deformity and maxillary asymmetry with a mean age of
13 years 2 months underwent combined maxillary and
mandibular distractions.3
The surgical procedures were done under general
anesthesia with nasotracheal intubation. A complete
horizontal LeFort I osteotomy was performed. In
contrast to the original method of Ortiz Monasterio
et al,19 the pterygomaxillary junction was freed with a
curved chisel on both sides, not only on the affected
side. The unaffected maxillary side LeFort I osteotomy
was loosely secured with 1 surgical wire placed above

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the maxillary rst and second permanent molars. This


wire acted as a hinge and pivot point where the maxillomandibular complex was expected to rotate toward the
unaffected side (Fig 1).
The ascending ramus was exposed along its anterior
border behind the last molar and extended to the gonial
angle and the surrounding area. The orientation of the
osteotomy and the position of the distractor device
determined the vector of distraction. A semiburied
distraction device (Z
urich II Distraction System; KLS
Martin, Jacksonville, Fla) was positioned over the ramus
in the planned direction (usually parallel to the long axis
of the ramus) and xed with self-tapping screws to
secure the foot plates above and below the initially
incomplete horizontal ramus osteotomy. The activating
arm, from the semiburied device, was placed externally
through a small incision below the mandibular angle.
The osteotomy was completed and veried by opening
the distractor. Before closure, the device was deactivated. The incision was closed, and rigid intermaxillary
xation with surgical wires was applied. Younger patients in the mixed dentition had custom-banded
(maxillary and mandibular rst permanent molars) arch
bars with soldered hooks secured with circumdental
wires for additional support during intermaxillary wire
xation and elastic therapy. In the adolescent patients,
the orthodontic appliances were used for presurgical
alignment and intermaxillary wire xation and postoperative elastic therapy.
The extent of the required bone elongation and the
vector of the distraction were determined by the severity
of the ipsilateral mandibular deformity, the shape of the
contralateral or unaffected mandibular ramus, and the
transverse cant of the occlusal plane. After 7 days (latency period), the device was lengthened by 0.5 mm
twice a day, for an average of 22 consecutive days. In
younger patients, vertical elongation of over 15 mm
was usually not required; therefore, a single 15-mm distractor was able to correct the occlusal plane discrepancy. In the adolescent patients who required more
than 15 mm of vertical distraction, we preplanned 2
consecutive surgical procedures; after full activation of
the rst 15-mm distractor, it was removed, and another
distractor of 30 mm was placed to complete the required
vertical distraction. This is a required step, since the bone
available to xate the device above and below the
planned osteotomy over the ramus is only sufcient to
accommodate a distractor of limited length (usually
15 mm). After the initial distraction, a longer distractor
can be placed. This device is partially opened before
placement to clear the newly generated bone. Another
reason why in certain patients it is necessary to use 2
consecutive devices relates to the fact that the initial

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Fig 1. Schematic representation of bimaxillary unilateral vertical distraction surgical plan. A, Complete
maxillary LeFort I (dashed line), unilateral horizontal ramus osteotomy; mandibular buried single-vector
distractor; intermaxillary wire xation (dotted line); single wire acting as a hinge (circle, contralateral
side); vertical and curved arrows indicate the expected direction of the maxillary and mandibular movements after distraction. B, Expected vertical bone formation between osteotomies, downward and
medial rotation of the maxilla and mandible to the contralateral side with leveling of the occlusal plane
and restoration of symmetry. (Reproduced with permission from Figueroa and Polley.21)

placement is such that true vertical elongation is not obtained. The placement of the device has mainly a vertical
component with a forward and medial vector because of
the shape of the hypoplastic ramus and the contralateral
ramus that needs to be emulated to correct as much as
possible not only the size but also the form of the ramus.
The placement of the distractor in this manner results in
an effective loss of vertical length. Semiburied distractors are rigid and true to their length, but as explained
above, the skeletal change is less than the true expression of the distractor; therefore, the activation to skeletal
change ratio is not 1:1.
The goal of bimaxillary distraction is mainly to obtain
a level occlusal plane and not perfect symmetry of the
gonial angles. The reason is that the vertical discrepancy
is usually greater at the gonial angles, and if they are leveled, the occlusal plane will be canted downward on the
affected side. The gonial angle asymmetry can be addressed secondarily with bone grafting combined with
other required procedureseg, genioplasty or soft tissue
augmentationto further improve appearance.
Activation of the mandibular distractor resulted in vertical elongation of the affected ramus and medial
displacement of both the maxillary and mandibular dentitions toward the unaffected side. This was possible
because the patients had a complete affected side horizontal ramus osteotomy and a complete LeFort I

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osteotomy. In addition, they were placed in intermaxillary


wire xation to move the maxilla, the mandible, and the
dentition as a unit. The patient's preoperative occlusion
was preserved through intermaxillary wire xation during
distraction. After distraction, a consolidation period with
wire intermaxillary xation was completed (4 weeks for
the younger patients and up to 6 weeks for the adolescent
patients). After this period, the wires were replaced with
6-oz, -in orthodontic elastics for the next 3 months.
The patients were placed on a liquid diet during the
intermaxillary xation period and a soft diet for 4 to
6 weeks after the wires were removed. Orthodontic treatment was continued in the adolescent patients for 6 to
8 weeks after the wire xation was removed. In the
younger patients, the xation appliances were removed
after 2 to 3 months of elastic therapy. The distraction device was left in place for 6 months and removed in the
operating room under general anesthesia.
All patients had anteroposterior (AP) cephalometric
radiographs and clinical frontal photographs taken before
and after distraction in a standard manner. Skeletal and
soft tissue facial asymmetries were respectively measured
with AP cephalometric and soft tissue photographic frontal analysis. Radiographs and photographs were hand
traced by the same investigator (E.F.S.). Each measurement was repeated 3 times, and the mean was recorded
for data comparison. There was no statistical difference

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Table I. Description of the cephalometric AP analysis for the vertical and horizontal measurements
Measurement
Denition
Vertical angle measurements
HL-Co0 Co
Angle between the HL and the bicondylar plane
HL-NF0 NF

Angle between the HL and the nasal oor plane

HL-J0 J

Angle between the HL and the maxillary jugal points

HL-OCP

Angle between the HL and the occlusal plane

HL-Go0 Go

Angle between the HL and the gonial plane

Landmark
Condylion (Co), external lateral marginal portion of the
condylar head
Nasal oor (NF), most inferior point on inside surface of the
bony nasal cavity
Jugal process (J), bilateral points on the jugal process of the
maxilla at a crossing with the tuberosity of the maxilla
Occlusal plane (OCP), horizontal plane passing through the
molar and the incisors
Gonion (Go), most lateral and inferior point of the
mandibular angle

Horizontal angle measurements


VL-isf
Angle between the VL and the superior midline
VL-Me

Angle between the VL and the mental line

Tns-ANS

Angle between the VL and the nasal septum

Incision superior frontale (isf), midpoint between the


maxillary central incisor at the level of the incisal edges
Menton (Me), point on the inferior border of the symphysis
directly inferior to the mental protuberance
Anterior nasal spine (ANS), tip of the ANS below the nasal
cavity and above the hard palate
Top of the nasal septum (Tns), the highest point on the
superior aspect of the nasal septum

Fig 2. Cephalometric analysis of vertical and horizontal changes. A, Horizontal planes relative to the HL
and VL references used for analysis: 1, HL-Co0 Co bicondylar plane; 2, HL HL-NF0 NF nasal oor plane; 3,
J0 J maxillary jugal plane; 4, HL-occlusal plane; 5, HL-Go0 Go gonial plane. B, Vertical planes used for
analysis: 1, VL-isf superior midline; 2, VL-Me mental line; 3, Tns-ANS nasal septum deviation to the VL.

between the 3 measurements as determined by the Dahlberg22 double determination method.


The measurements from the AP cephalograms are
given in Table I. Two reference lines were traced
(Fig 2) and used to make vertical and horizontal measurements: a horizontal line (HL), the line connecting
the right and left latero-orbitale points, and a vertical
line (VL), the line perpendicular to the HL through the
center of crista galli (most constricted point of the projection of the perpendicular lamina of the ethmoid).

The gonial height ratio (HL-Go0 /HL-Go) and the


ramus height ratio (CoGo0 /CoGo) were calculated from
the AP cephalograms to compare the affected vs the unaffected sides. HL-Go0 /HL-Go is the ratio between the
linear perpendicular distance from the horizontal reference line to the affected Go0 point, and the linear
perpendicular distance from the horizontal reference
line to the unaffected Go point. Co0 Go0 /CoGo is the ratio
between the linear perpendicular distance from the
affected Co0 point to the affected Go0 point, and the linear

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Fig 3. Cephalometric analysis of the ratio of affected vs


unaffected gonial height (HL-Go0 /HL-Go) and ramus
height (Co0 -Go0 /Co-Go).

perpendicular distance from the unaffected Co point to


the unaffected Go point (Fig 3).
In the photographic facial analysis, we used 3 bilateral soft tissue landmarks identied on the frontal photographs: bilateral endocanthion (inner commissure of
the eye ssure) and the center point of a line connecting
the right and left endocanthions. Two reference lines
were traced on the frontal photographs (Fig 4): an HL,
the line connecting the right endocanthion to the left
endocanthion points; and a VL, the line perpendicular
to the HL through the midline of the distance between
the right and left endocanthion points. The measurements obtained from the frontal photographs are shown
in Table II.
Statistical analysis

Paired t tests were used to examine the difference


between the preoperative (T1) and postoperative
(T2, 6 months after surgery) measurements using the
Statistical Package for Social Sciences software
(version15.0; SPSS, Chicago, Ill). The power of the paired
t test was calculated for each variable considering a sample size of 8 and an a of 0.05, using the free software
power and sample size calculator (version 3.1.2; Statistical Solutions, Boston, Mass).
RESULTS

The results of the AP cephalometric analysis demonstrated vertical improvement in all patients as seen by statistically signicant decreases relative to the HL of the
nasal oor angle (P 5 0.004), the maxillary jugal plane

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Fig 4. Planes used in the photographic analysis to evaluate vertical and horizontal facial changes relative to the
HL and VL reference lines: 1, HL-sbal0 sbal nasal base
plane; 2, HL-ch0 ch labial commissure plane; 3, VL-Pog
vertical line chin point.

angle (P 5 0.008), the occlusal plane angle (P 5 0.003),


and the gonial plane angle (P 5 0.035). Of the horizontal
changes, only the menton to the VL measurement
(P 5 0.004) was statistically signicant (Table III).
The ratio of the affected-unaffected gonial height
(HL-Go0 /HL-Go) signicantly improved by 20% from
65.85% to 86.61% (P 5 0.027). Ramus height
(Co0 Go0 /CoGo) also signicantly improved by almost
15% from 78.12% to 93.06% (P 5 0.042) (Table III).
The ndings demonstrated parallelism among the horizontal planes, vertical elongation of the affected side
ramus height, and improvement of the midline deviation
measurements. These were favorable changes toward
restoring symmetry.
The facial photographic analysis (Table IV) demonstrated signicant changes, with the nasal plane improving
by 3.00 (P 5 0.014), and the chin position changing toward the unaffected side by 4.83 (P 5 0.000), thus
improving the midline symmetry, but without reaching
full correction. Although not statistically signicant, the
labial commissure plane improved by 2.3 .
The power of the t test (ie, the probability of correctly
rejecting the null hypothesis) is included in Tables III and
IV. Most variables with statistically signicant differences between T1 and T2 (6 of 9) had an over 90% power
for the t tests.

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Table II. Description of photographic facial analysis for the vertical and horizontal measurements
Measurement
Vertical angle measurements
HL-sbal0 sbal (nasal base angle plane)
HL-ch0 ch (labial commissure angle plane)
Horizontal angle measurement
VL-Pog (chin point)

Denition

Landmark

Angle between the HL and the nasal base plane


Angle between the HL and the labial commissure
plane, and 1 horizontal angle measurement

Subalare (sbal), lower limit of each alar base


Cheilion (ch) point at each labial commissure

Angle between the VL and the chin point

Pogonion (Pog), most anterior midpoint of


the chin

Table III. Vertical and horizontal angular measurements and ratios from anteroposterior cephalometric radiographs

Measurement
Bicondylar plane (HL-Co0 -Co) ( )
Nasal oor plane (HL-NF0 NF) ( )
Maxillary jugal plane (HL-J0 J) ( )
Occlusal plane (HL-OCP) ( )
Gonial plane (HL-Go0 Go) ( )
Superior midline (VL-isf) ( )
Mental line (VL-Me) ( )
Nasal septum deviation (Tns-ANS) ( )
Gonial height (HL-Go0 /HL-Go) (%)
Ramus height (Co0 -Go0 /Co-Go) (%)

T1 mean 6 SD
3.42 6 2.07
14.28 6 6.36
11.85 6 6.76
12.71 6 5.85
12.42 6 7.18
5.71 6 4.95
7.00 6 7.58
14.71 6 6.36
78.12 6 14.45
65.85 6 16.01

T2 mean 6 SD
2.14 6 1.46
8.00 6 6.55
3.00 6 4.12
4.42 6 4.07
4.00 6 5.13
3.85 6 5.08
3.4 6 7.05
11.14 6 6.36
93.06 6 9.32
86.61 6 12.35

Difference
T1  T2
mean 6 SD
1.28 6 1.70
6.28 6 3.72
8.85 6 6.03
8.28 6 4.46
8.42 6 8.24
1.85 6 3.18
3.6 6 2.50
3.57 6 4.03
14.93 6 13.59
20.76 6 21.37

95% CI of the difference


P value
0.093
0.004y
0.008y
0.003y
0.035*
0.174
0.033*
0.058
0.027*
0.042*

Lower
0.29
2.83
2.28
4.15
0.80
1.08
0.48
0.16
2.36
0.99

Upper
2.86
9.73
3.27
12.41
16.05
4.80
6.71
7.30
27.50
40.53

Power of
paired t
test (%)
41.2
97.7
94.2
98.8
69.3
25.0
93.5
55.5
76.0
64.4

0
Affected side.
*P \ 0.05; yP \ 0.01.

Table IV. Vertical and horizontal angular measurements from frontal photographs
Difference
T1T2
Measurement
T1 mean 6 SD T2 mean 6 SD mean 6 SD P value
Nasal base angle plane (HL-sbal0 sbal) ( )
7.50 6 5.91
4.25 6 5.25 3.00 6 2.00 0.014*
Labial commissure angle plane (HL-ch0 ch) ( ) 9.75 6 4.99
6.75 6 6.89 2.33 6 3.14 0.128
8.75 6 6.23
3.75 6 6.23 4.83 6 0.98 0.000y
Vertical line-chin point angle (VL-pog) ( )

95% CI of the difference


Lower
0.90
0.96
3.80

Upper
5.09
5.62
5.86

Power of
paired t
test (%)
94.9
40.0
100.0

Affected side.
*P \ 0.05; yP \ 0.01.

DISCUSSION

Reconstruction of an asymmetric mandible associated with a soft tissue deciency is one of the most challenging problems in patients with HFM. Numerous
surgical procedures have been advocated to correct
facial asymmetry in these patients, including costochondral grafts, mandibular osteotomies combined with bone
grafts, and maxillary osteotomies, done at an early age
or in late adolescence.6,9,18 The results can be
unpredictable because of undesirable resorption of the
graft, leading to decreases in volume and strength of
the reconstructed area. Furthermore, these procedures
can cause signicant morbidity at the donor site.
Conventional orthognathic surgery, such as maxillary
impaction on the unaffected side, is usually performed

with mandibular osteotomies.17 A large movement has


a high risk for relapse because of the soft tissue deciency of HFM. Recently, a complete fossa, condyle,
and ramus reconstruction with a prosthetic replacement
has been reported in adolescent patients with HFM with
severe mandibular deformities.23
Distraction osteogenesis has provided an alternative
in the treatment of craniomaxillofacial deformities.18
Its success is related to the fact that it uses the body0 s
own healing mechanisms to produce new bone and
elongate the soft tissues. This diminishes the need for
autografts, thus decreasing morbidity. Mandibular elongation by gradual distraction can be done at any age.19
However, acute changes in mandibular shape result in
postoperative alterations in dental occlusion, such

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Fig 5. A and D, Presurgical photographs of a 6-year-old boy with left HFM; B and E, after distraction;
and C and F, follow-up treatment frontal facial and occlusion photographs at age 16 years. Note the
transitional dentition stage (D and E) and no orthodontic appliances during distraction. A year after
distraction the asymmetry was improved and the inferior midline was overcorrected, but at the 10year follow-up the cant of the occlusal plane and the chin point had moved back toward to the affected
side.

as open bite on the affected side, crossbite on the


contralateral side, and on occasion anterior crossbite.24,25 These consequences of mandibular unilateral
distraction require orthodontic treatment over a long
period of time. Postoperative orthodontic management
can be difcult in young patients because of
challenging mechanics and limited cooperation levels.
In this study, combined maxillary and mandibular
distractions were performed to correct the bimaxillary
deformity in patients with HFM as initially suggested
by Molina et al.19 A complete LeFort I was done simultaneously with a complete horizontal ramus osteotomy
on the affected side, placement of a semiburied distraction device with a vertical vector, and wire intermaxillary
xation.19,21 External distractors were not used for our
patients, since there are some inherent problems with
them. These include facial scars and the tendency for
the external xation pins to loosen. Loose pins prevent
the required long-term retention needed to ensure
consolidation of the new regenerate and stability of
the newly elongated ramus. Early removal of the external
distractor permits muscle and soft tissue forces to act on
the newly created bone and may lead to relapse. This
problem is reduced by the ability to keep internal distractors in place for a prolonged period. This approach

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protects the newly created bone and allows the soft tissues to adjust to the new length. Although consolidation
of the regenerate is usually advanced after 6 to 8 weeks,
it continues with additional remodeling.26 In this sample, the devices were left longer (6 months) to accommodate the patients0 school schedules.
The simultaneous distractions of the maxilla and the
mandible are designed to correct the vertical and horizontal occlusal and chin asymmetries. However, these
fail to correct, if present, an orbitozygomatic deformity.14,27 In our patients, all horizontal planes
improved, but of the midline structures only the mental
deviation from the vertical was statistically signicant.
This is explained by the fact that the structures closer
to the osteotomynasal septum and maxillary
incisorsdid not change as much because they were
closer to the center of rotation of the maxillomandibular complex. The maxilla pivoted around the
surgical wire hinge, located on the unaffected side of
the LeFort I osteotomy, thus limiting the lateral
displacement of the hinge wire side of the maxilla
toward the unaffected side. However, vertical
lengthening of the maxilla and the mandible on the
affected side resulted in a signicant rotation of the
chin toward the facial midline or the unaffected side.

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Fig 6. A, Predistraction; B, postdistraction; and C, 10-year follow-up AP cephalometric radiographs of


the patient shown in Figure 5. Note the initial cant of the nasal, occlusal, and gonial planes and the deviation of the chin point to the ipsilateral side (A). After distraction (B), the cant of the 3 planes was
improved, and the chin point was centered. However, at 10-year follow-up (C), the 3 planes had worsened and the chin point had deviated to the affected side.

Fig 7. A, Presurgical frontal facial photographs of a 14-year-old boy with left HFM; B, during distraction;
and C, posttreatment frontal facial photographs at age 18 years 6 months. Note the improvement of
facial symmetry.

Combined unilateral vertical maxillary and mandibular distractions corrected the cant of the occlusal plane
and the chin deviation. The desired complete correction
of the facial asymmetry, especially in the gonial region,
was not fully accomplished, even though the changes
were statistically signicant (P 5 0.035). This was not
surprising, since it is geometrically impossible to produce
mandibular symmetry with a unilateral mandibular

distraction in a bone with a multidimensional deformity.


It is likely that these patients will require additional surgical interventions to further improve their residual
asymmetry.28
Facial asymmetry in patients with HFM results from
shortness of the skeleton and hypoplastic soft tissues.
The frontal photographic analyses showed that the nasal
plane reached a better position related to the horizontal

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Fig 8. Occlusion photographs of the patient shown in Figure 7: A-C, presurgical; D-F, during distraction; and G-I, after treatment. Note the orthodontic appliances during distraction after initial alignment,
intermaxillary xation using the orthodontic appliances, and excellent facial symmetry and occlusion
after distraction and orthodontic treatment.

line (P 5 0.014), the labial commissure leveled, the chin


position moved to the unaffected side (P 5 0.000), and
the mandibular border contour improved but did not
achieve full correction. The lack of soft tissues and
severely hypoplastic muscles of mastication make
correction of HFM facial asymmetry a difcult challenge.29 The distraction process did not augment the
lateral bulk of the gonial angle and the overlying muscles
of mastication. Although the central aspect of the face
was closer to the midline, the appearance of the face
was attened on the affected side, thus compromising
overall facial symmetry. To minimize the residual facial
asymmetry, a series of secondary surgical interventions
is usually necessary. Microvascular or free dermis-fat
grafts can produce excellent results to restore the decient soft tissue facial volume.30,31
There is much debate on the timing of surgery in
patients with HFM, for either growing or nongrowing
patients. The introduction of distraction has added to
the confusion. The debate results from different understandings of how a child with HFM grows. The authors

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of 1 study recommended early intervention to prevent


the progression of the mandibular and maxillary deformities in growing children.9 It is based on the premise
that if the deformity is left untreated, it will worsen
over time. The second approach delays intervention until
the completion of growth. It is based on the premise that
the deformity, if left untreated, remains relatively stable
over time.12,13,32 The severity of HFM varies widely, and
the functional impairments (airway, mastication, and
speech) should dictate the timing of the surgical
intervention. Patients with respiratory distress, and
feeding and speech issues are candidates for early
interventions. However, the parents need to be fully
aware that additional corrective skeletal and soft tissue
procedures will become necessary during late
adolescence to fully address the facial asymmetry and
functional impairments.
To date, there is no evidence supporting the effectiveness of early mandibular osteodistraction in patients
with HFM.14 In our study, 4 patients had surgery in the
early mixed dentition to minimize the deformity. Surgery

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575

Fig 9. A, Predistraction; B, postdistraction; and C, 4.5-year follow-up AP cephalometric radiographs of


the patient shown in Figure 7. Note the initial cant of the nasal, occlusal, and gonial planes and the deviation of the chin point to the ipsilateral side (A). After distraction (B), the cant of the 3 planes was
improved, and the chin point was centered. Note the increase in ramus height, stability of all planes,
and chin position after distraction and at 4.5-year follow-up (C).

Fig 10. Occlusal photographs of a 16-year-old boy with HFM: A and C, before orthodontic alignment; B
and D, after orthodontic alignment. Note the asymmetry of the mandibular arch before orthodontic treatment with the decreased distance of the affected side to the midline (arrows). The arch was well aligned
after treatment (D), with improved symmetry. Congruent and symmetrical arches allow for better outcomes with bimaxillary unilateral vertical distraction.

was performed with full awareness by the parents that as


the child reaches skeletal maturity, more surgical procedures would be required.14,33 Although we observed

partial improvements in facial asymmetry, control of


the occlusion was challenging because the children
were in the transitional dentition stage, and also

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Sant'Anna et al

576

because of their lack of compliance with the elastics and


the limitations of the appliances designed for
transitional dentitions (Figs 5 and 6).
On the other hand, the most successful cases treated
in this small series were the adolescent patients with full
permanent dentition, with xed orthodontic appliances
placed to align and level the arches before the bimaxillary distraction. No older patient had a secondary ipsilateral open bite or a contralateral crossbite. Postoperative
occlusal relationships were close to ideal after the bimaxillary distraction procedure, and the patients
required only routine orthodontics to complete treatment during a 6- to 12-month postoperative period;
they have remained stable after the surgical and orthodontic treatments (Figs 7-9).
The younger patients in our sample will require additional interventions such as additional distraction,
double-jaw orthognathic surgery, genioplasty, and soft
tissue augmentation.
Case selection for a determined surgical procedure is
critical. From our experience, we suggest that patients
with clinically signicant occlusal plane discrepancies,
with congruent maxillary and mandibular arch forms
when adequate occlusal interdigitation can be achieved,
with minor AP maxillomandibular discrepancies, and
without excessive maxillary gingival exposure on the unaffected side are appropriate for bimaxillary distraction.
However, patients with excessive maxillary gingival show
on the affected side can be managed as described here,
but later, the vertical maxillary excess can be corrected
by maxillary impaction. Maxillary impaction is the
most stable procedure in orthognathic surgery.34 Patients with mandibular dentoalveolar arch asymmetry
with a skewed arch should be rst aligned with orthodontic treatment before proceeding with bimaxillary
distraction (Fig 10). In patients in the permanent dentition with well-aligned arches, xed orthodontic appliances are used for intermaxillary xation, comfort
(avoiding arch bars), postoperative elastic therapy, and
nishing orthodontic occlusal details.
As with many clinical distraction studies, our small
sample size makes it difcult to draw denitive conclusions. Series from other authors had similar35 or even
smaller sample sizes8,11,17,31,36-38 to study the effects
of distraction in HFM. The relatively rare incidence of
HFM and the number of patients who t the criteria
for bimaxillary distraction result in limited experience
in the various treatment centers. The challenge for
obtaining enough randomized subjects motivated the
development of the National Dental Practice-Based
Research Network.39 The aim of this initiative is to build
an investigative union of practicing dentists and academic scientists collaborating to rene dental care.

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Therefore, intercenter collaborative studies are necessary


to eventually develop a consensus for an effective protocol to treat patients with HFM.
In this study, skeletal and soft tissue changes after bimaxillary distraction osteogenesis were measured with
AP cephalometric radiographs and frontal facial photographs. Technological advances in 3-dimensional photogrammetry and radiologic scanning techniques will allow
improved evaluation of outcomes.8,40 The use of 3dimensional virtual surgical planning and CAD/CAM
generated splints and surgical guides will assist in
selecting the best surgical interventions for a particular
condition and can assist surgeons with accurate
surgical execution, thus improving outcomes.41-45
CONCLUSIONS

Simultaneous maxillary and mandibular distraction


improved facial balance and symmetry in all patients
with HFM. Patients in the permanent dentition with
xed appliance orthodontic treatment and wellaligned dental arches responded better to this approach
than did younger patients. The severity of the skeletal
and soft tissue deciencies may dictate the need for
additional surgery such as orthognathic surgery, genioplasty, and soft tissue enhancement procedures to
obtain improved facial balance and symmetry.
ACKNOWLEDGMENT

We thank Daniel Ranjbar, Lawrence, Kansas, for his


expert orthodontic support in the treatment of the
patient shown in Figures 4, 7, and 8.
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