J Oral Maxillofac Surg

66:2318-2321, 2008

Effect of Midfacial Distraction on the

Obstructed Airway in Patients With
Syndromic Bilateral Coronal Synostosis
Tyler E. Nelson, DMD,* John B. Mulliken, MD,† and
Bonnie L. Padwa, DMD, MD‡
Purpose: Le Fort III osteotomy and midfacial advancement expand the nasopharynx and potentially
increase airway dimensions in patients with syndromic bilateral coronal synostosis. Distraction osteogenesis
allows greater midfacial advancement, and may offer more improvement in airway obstruction, compared
with the conventional 1-stage procedure. This study aimed to document the effect of midfacial distraction-
advancement on airway obstruction in patients with syndromic bilateral coronal synostosis.
Patients and Methods: Charts of patients with syndromic bilateral coronal synostosis who had
undergone Le Fort III distraction were reviewed. Preoperative and postoperative demographic, cepha-
lometric, polysomnographic, and subjective patient/parental reported data were reviewed. Descriptive
and nonparametric bivariate statistics were computed, to document changes in airway parameters.
Results: Of 25 patients who underwent midfacial Le Fort III distraction, only 18 had preoperative airway
obstruction. The mean age at operation for patients with airway obstruction was (⫾SD) 10.4 ⫾ 4.2 years
(range, 2.7 to 17.4 years), and the average advancement was 20.5 ⫾ 7.4 mm (range, 10 to 30 mm). Five of
6 patients with a tracheostomy were decannulated; 1 patient had persistent central apnea that prevented
decannulation. The mean respiratory distress index for the group improved from 33.4 ⫾ 37.57 (range, 1.8 to
109.2) to 12.6 ⫾ 26.32 (range, 0.00 to 72.0) (P ⬍ .05). Six of 9 patients no longer required continuous or
bilevel positive airway pressure. All patients reported decreased snoring (P ⬍ .05).
Conclusions: Midfacial distraction improves airway obstruction in patients with syndromic bilateral
coronal synostosis. Clinicians can counsel patients and families that this procedure usually permits
decannulation and discontinuation of continuous or bilevel positive airway pressure.
© 2008 American Association of Oral and Maxillofacial Surgeons
J Oral Maxillofac Surg 66:2318-2321, 2008

Syndromic bilateral coronal synostosis is usually

*Dental Student, Harvard School of Dental Medicine, Boston, MA.
†Professor of Surgery, Harvard Medical School, and Director,
Craniofacial Center, Department of Plastic and Oral Surgery, Chil-
dren’s Hospital, Boston, MA.
‡Associate Professor of Oral and Maxillofacial Surgery, Harvard
School of Dental Medicine, and Chief, Division of Oral and Maxil-
lofacial Surgery, Children’s Hospital, Boston, MA.
This study was supported in part by the Department of Oral and
Maxillofacial Surgery Education and Research Fund, Massachusetts
General Hospital, Boston, MA.
This study was presented at the Annual Meeting of the Amer-
ican Association of Oral and Maxillofacial Surgeons on October
4, 2006.
Address correspondence and reprint requests to Dr Padwa:
Department of Plastic and Oral Surgery, Children’s Hospital, 300
Longwood Avenue, Boston, MA 02115; e-mail: Bonnie.Padwa@
childrens.harvard.edu
© 2008 American Association of Oral and Maxillofacial Surgeons
0278-2391/08/6611-0020$34.00/0
doi:10.1016/j.joms.2008.06.063
associated with midfacial hypoplasia. The retruded
midface impinges on the nasopharynx, and when
the patient falls asleep, postural muscles (that nor-
mally act to hold the airway open) relax, causing
collapse and resultant obstructive sleep apnea
(OSA).1,2
Obstructive sleep apnea is manifested by snoring
and repeated hypopneas and apneas, leading to hy-
poxia and an interruption in the normal cycles of
rapid eye movement sleep.2,3 A child with OSA typi-
cally shows certain signs and symptoms: daytime fa-
tigue, malaise, irritability, sleep terrors, crying spells,
growth perturbations, enuresis, delayed puberty, ag-
gressiveness, or poor eating.4,5 The treatment goals
for a child with OSA are to open the narrowed airway
with either continuous positive airway pressure
(CPAP) or bilevel positive airway pressure (BIPAP) or
operative procedures.2,6-9 Continuous positive airway
pressure is difficult for many children with syndromic
craniosynostosis to tolerate because of the con-
stricted nasal airway. Furthermore, it is often difficult
to fit a mask comfortably because of the surgically

2318
NELSON ET AL
advanced forehead, maxillary retrusion, and short
nose. Young patients may not cooperate with at-
tempts to provide nocturnal positive airway pressure.
Tracheotomy is necessary for some children who are
refractory to treatment, or who have had severe air-
way obstruction from birth.6-9
Le Fort III osteotomy and midfacial advancement
expand the nasopharynx and have the potential to
increase airway dimensions in patients with syn-
dromic bilateral coronal synostosis.10 Conventional
advancement and rigid internal fixation often fail to
create significant improvement because of inadequate
expansion and problems with relapse.5,8,11 The tech-
nique of distraction osteogenesis allows greater ad-
vancement of the midface, compared with single-
stage Le Fort III osteotomy.11 There is some evidence
for better correction of the narrowed airway using
distraction.12,13 The goal of this study was to docu-
ment improvement in the airways of patients with
bilateral coronal synostosis who had midfacial ad-
vancement by distraction.
Materials and Methods
The records of patients with syndromic bilateral
coronal synostosis who had Le Fort III osteotomy and
midfacial distraction were reviewed, and demo-
graphic data were recorded, ie, date of birth, gender,
ethnicity, syndrome type, molecular diagnosis, and
associated intracranial malformations. In addition,
clinical/anatomic, operative, polysomnographic, and
patient/parental reported observations were docu-
mented preoperatively and postoperatively. The mag-
nitude of sagittal midfacial advancement was mea-
sured at the maxillary incisors on preoperative and
immediate postoperative lateral cephalograms. All
data were entered into an SPSS database for analysis
(SPSS Graduate Pack, version 11.0; SPSS, Inc, Chicago,
IL). Descriptive statistics were computed along with
the McNemar test for preoperative and postoperative
nominal scale variables, and the Wilcoxon signed-rank
test for continuous data, to document changes in
airway parameters that occurred after midfacial dis-
traction.
PROCEDURE
A standard subcranial Le Fort III osteotomy was
performed, and the midface was mobilized. Distrac-
tion was performed with either semiburied or rigid
external distraction devices or a combination of
these.14 The semiburied devices (Synthes, Paoli, PA)
were placed deep in the temporalis muscle and se-
cured proximally to the temporal bone, and distally to
the lateral orbital rims below the frontozygomatic
osteotomy. The devices were placed as parallel as
possible to one another, to control advancement in
2319
the coronal and horizontal planes. The turning ele-
ment was passed percutaneously, posterior to the
coronal incision, and was used to activate the expan-
sion screw to advance the upper portion of the mid-
face. The rigid external distraction device (RED; KLS
Martin LP, Jacksonville, FL) was secured to the cra-
nium, using 3 or 4 percutaneous screws per side after
the coronal incision was closed. The device was po-
sitioned 3 to 4 cm above the superior border of the
helix on each side, with the plane of the device
parallel to the Frankfurt horizontal. Twenty-six-gauge
traction wires were connected from the external
hooks of a rigid intraoral splint or a bone-retained
plate to the activating screws located on the horizon-
tal bar of the device.
Distraction commenced between 1 and 3 days post-
operatively, at a rate of 1 mm per day for 15 to 25 days
for both semiburied and external devices. Patients were
returned to the operating room for removal of the de-
vices 4 to 6 weeks after completion of distraction.
Results
Between 2000 and 2006, 25 patients with syndromic
bilateral coronal synostosis underwent Le Fort III os-
teotomy and midfacial advancement. Seven of these
25 patients did not have preoperative airway prob-
lems, and were excluded from the analysis. Airway
obstruction in 18 patients was documented by the
presence of a tracheostomy (6 patients) and preoper-
ative polysomnography (12 patients), with a mean
preoperative respiratory disturbance index (RDI) of
(⫾SD) 33.4 ⫾ 37.57 (range, 1.8 to 109.2), indicative
of moderate obstructive sleep apnea. There were 11
males and 7 females. The mean age at time of opera-
tion was 10.4 ⫾ 4.2 years (range, 2.7 to 17.4 years).
The study group comprised patients with the most
common forms of syndromic bilateral coronal synos-
tosis: Crouzon (n ⫽ 6), Apert (n ⫽ 6), Pfeiffer (n ⫽ 4),
Muenke (n ⫽ 1), and indeterminate pansynostosis
(n ⫽ 1). A Chiari I malformation was documented in
all 6 patients with Crouzon syndrome, in 1 with Apert
syndrome, and in 1 with indeterminate pansynostosis.
The average midfacial advancement, measured at
the incisors on preoperative to immediately postop-
erative lateral cephalograms, for the group with air-
way obstruction was 20.5 ⫾ 7.4 mm (range, 10 to 30
mm), performed at a standard rate of 1 mm/day, with
a consolidation period of at least 4 weeks (mean
device duration, 64.4 ⫾ 20.2 days; range, 34 to 120
days), and a mean time to follow-up of 3.18 years
(range, 0.91 to 10.38 years).
Of 18 patients with documented airway obstruction,
6 with a tracheostomy had postoperative sleep studies
to document airways adequate for decannulation. There
were no preoperative data for these patients because of
2320 MIDFACIAL DISTRACTION IN SYNDROMIC BILATERAL CORONAL SYNOSTOSIS

Table 1. PATIENT SUMMARY

Twenty-five patients
● 7 without history of airway obstruction
● 18 with history of airway obstruction
● 6 with longstanding tracheostomy could not tolerate
capped preoperative polysomnography
● 2 with significant central apnea on polysomnography
were excluded from analysis
● 10 with complete preoperative and postoperative
polysomnography were used for data analysis
Nelson et al. Midfacial Distraction in Syndromic Bilateral Coro-
nal Synostosis. J Oral Maxillofac Surg 2008.
their inability to tolerate capped observation. Five of 6
patients with a tracheostomy were decannulated post-
operatively. The 1 patient who required a tracheostomy
postoperatively had significant central apnea (central
apnea index, 13.9) and a Chiari malformation. There
were 12 patients who had preoperative and postopera-
tive polysomnography. Two of these had a Chiari mal-
formation and severe central apnea (central apnea in-
dex, 12.24 and 30.0, respectively), and were excluded
from analysis (Table 1). The remaining 10 patients
(Table 2) showed improvements in RDI (P ⬍ .05), min-
imum oxygen saturation, and highest CO2.
Nine of 12 patients without a tracheostomy who had
OSA were managed preoperatively with CPAP/BIPAP,
and no longer required positive airway pressure after
midfacial distraction (P ⬍ .05). Three patients continued
to require postoperative CPAP/BIPAP: 1 had significant
postoperative central apnea, 1 had advancement of only
10 mm and residual OSA syndrome, and another had
multiple sites of residual obstruction, including choanal
atresia and laryngomalacia.
Subjectively, all 18 patients reported decreased snor-
ing (P ⬍ .05) and symptoms of daytime sleepiness (P ⬍
.05). Two patients noted improved mood after the op-
eration, with noticeably reduced irritability.
Discussion
This retrospective study documented objective and
subjective improvements in airway obstruction after
midfacial distraction in patients with syndromic bilat-
eral coronal synostosis. Eight of 10 patients with air-
way obstruction and no central apnea showed a
greater than 50% improvement in RDI to values of less
than 10, indicating operative success. In addition, 5 of
6 patients with a tracheostomy were decannulated,
and 6 of 9 patients no longer required CPAP/BIPAP.
Our study also documented decreased snoring and
daytime sequelae of sleep-disordered breathing (eg,
daytime sleepiness and irritability).4,5 Although this
study would have been strengthened with the inclusion
of a standardized assessment of daytime sleepiness, such
as the Epworth Sleep Scale, the retrospective study de-
sign precluded its use. Nevertheless, patients reported
improvement in all areas, indicating that midfacial ad-
vancement affects not only absolute indicators of ob-
structive sleep apnea, but also symptoms.
This study confirmed the limited body of evi-
dence that Le Fort III midfacial distraction opens
the airways of patients with syndromic bilateral
coronal synostosis. Several studies showed that tra-
ditional 1-stage Le Fort III osteotomy and advance-
ment were unpredictable and ineffective in treating
obstructive sleep apnea.5,8,12 This was attributed to
insufficient advancement, poor stability, and re-

Table 2. POLYSOMNOGRAPHY RESULTS FOR 10 PATIENTS

Variable Preoperative Postoperative P Value

Clinical variables
CPAP/BIPAP 9 (37.5) 3 (12.5) .014
Tracheostomy 6 (26.1) 1 (4.3) *
Polysomnographic variables
Time between operation and sleep study (y) 2.0 ⫾ 1.22 (0.17 - 12.4) 3.18 ⫾ 3.19 (0.41 - 6.5) .26
Respiratory disturbance index 33.4 ⫾ 37.6 (1.8 - 109.2) 12.6 ⫾ 26.3 (0 - 72.0) .031
Apneic index 12.1 ⫾ 9.4 (1.8 - 20.4) 3.0 ⫾ 4.2 (0 - 5.9) *
Minimum O2 saturation (mm Hg) 82.8 ⫾ 9.4 (68 - 92) 89.3 ⫾ 4.5 (82 - 96) 1.00
Maximum CO2 saturation (mm Hg) 47.6 ⫾ 5.7 (41.0 - 56.0) 46.0 ⫾ 1.0 (45.0 - 47.0) .50
Central apneic index 2.22 ⫾ 1.8 (0.20 - 4.7) 0.75 ⫾ 1.5 (0 - 3.0) .625
Subjective variables
Snoring 20.0 (80.0) 1 (4.0) .01
Daytime sleepiness 5 (21.7) 0 (0.0) .025
Irritability 2 (8.7) 0 (0.0) .16
NOTE. Categorical data are listed as numbers (percentages). Continuous data are listed as means ⫾ SD (ranges). P values were
calculated using nonparametric tests.
*For some variables, small sample sizes within groups prevented statistical comparisons.
Nelson et al. Midfacial Distraction in Syndromic Bilateral Coronal Synostosis. J Oral Maxillofac Surg 2008.
NELSON ET AL
lapse.8,11 The high rate of relapse associated with
the standard procedure may be attributable to re-
sorption of bone grafts and the force exhibited by
soft tissue recoil after advancement.11,12 In contrast,
Le Fort III midfacial distraction permits greater forward
movement, obviates the need for bone grafting, and
minimizes relapse because of the gradual stretch of the
soft tissue envelope.5,11,12
Our findings are consistent with those of Fearon
(2005), who found significant improvement in RDI
and minimum oxygen saturation after midfacial dis-
traction in 12 patients. He decannulated 3 out of 4
patients.13 Likewise, Mathijssen et al (2006) reported
that 2 of 7 patients with airway obstruction improved,
and 4/7 patients were decannulated, after midfacial
distraction.15 Other investigators provided anecdotal
evidence for decreased airway obstruction, as evi-
denced by decannulation, higher oxygen saturation,
and decreased BIPAP dependence.12,16
Although the airway improved in most of our pa-
tients, there was a subset, as in previous reports, who
continued to have airway problems after midfacial
distraction. This may be ascribed to incomplete ad-
vancement, central apnea, or airway obstruction be-
low the level of the nasopharynx. One patient in this
study had inadequate movement (only a 10-mm ad-
vancement) and residual OSA syndrome requiring
CPAP. Two other patients who continued to have
disordered breathing after distraction (one who could
not be decannulated, and one with a continued CPAP
requirement) had a Chiari I malformation and signifi-
cant central sleep apnea. Herniation of the cerebellar
tonsils through the foramen magnum causes neuronal
impingement of the central respiratory centers, result-
ing in apnea.17 Although not a common feature of
syndromic craniosynostosis, mandibular retrognathia
could also compromise the airway.3 One young girl
with Crouzon syndrome in our study had residual
minor airway obstruction after midfacial distraction
that did not require intervention. She had a small
lower jaw, with a retropositioned tongue. Cartilagi-
nous abnormalities were also documented in patients
with Apert and Pfeiffer syndromes: complete or par-
tial cartilage sleeves, absence of tracheal rings, bron-
chial abnormalities, and tracheomalacia secondary to
longstanding tracheostomies.6,8 One patient in our
study had choanal atresia, whereas another had both
choanal atresia and laryngomalacia, necessitating
postoperative CPAP.
Although midfacial distraction is the anatomic correc-
tion for most airway problems in syndromic bilateral
coronal synostosis, our ability to counsel patients de-
pends on our capacity to identify other sites of obstruc-
tion. Before considering midfacial distraction, these
anomalies should be documented with pharyngoscopy
and a pulmonary examination. Although not a routine
2321
practice for patients with syndromic craniosynostosis,
the use of dynamic magnetic resonance imaging or dy-
namic computed tomography may prove useful in iden-
tifying the precise level of airway obstruction.18
This retrospective case series has inherent limita-
tions, because syndromic bilateral coronal synostosis
is a relatively rare condition. The number of patients
was insufficient to allow for rigorous statistical analy-
sis. Nevertheless, our findings indicate that midfacial
distraction improves airway obstruction in patients
with bilateral coronal synostosis who have an ade-
quate amount of advancement, and in those who do
not have significant central apnea or other anomalies
of the lower airway. A comprehensive workup will
allow for more accurate predictions regarding opera-
tive success in treating airway obstruction in this
patient population.
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