Craniofacial Syndromes

The Le Fort III Osteotomy for Correction of Severe Midface

Hypoplasia
Daniel Schlieder, DDS, MD, FACS a,b,*, Michael R. Markiewicz, DDS, MPH, MD c,d,e,f,g

KEYWORDS
 Le Fort III  Midface hypoplasia  Midface distraction  Syndromic craniosynostosis  Craniofacial dysostosis

KEY POINTS
 Severe subtotal midface hypoplasia is commonly seen in patients with syndromic craniosynostosis and orofacial clefting
(eg, Crouzon syndrome).
 The subcranial Le Fort III osteotomy with or without distraction is an effective procedure that can be used to improve
exorbitism (relationship of anterior cornea to infraorbital rim), obstructive sleep apnea, facial esthetics, and occlusion.
 Computer-aided planning allows the surgeon to 3-dimensionally evaluate a patient’s anatomy and deformity, bone density,
vital landmarks, and osteotomy vector and allows for the fabrication of custom patient-specific guides during Le Fort III
midface advancement.
 Le Fort III with or without distraction is a complex procedure with associated morbidity that requires meticulous preop-
erative planning, patient counseling, and postoperative care.

Introduction: nature of the problem sleep apnea. Severe class III skeletal position results in a dental
occlusal relationship that makes it often impossible for pa-
Severe skeletal disharmony between the middle and lower face tients to properly masticate due to a minimal number of teeth
is a challenge faced by all craniofacial surgeons. Growth re- in occlusion. There also exists a psychological burden on pa-
striction of the middle face may be the result of scarring from tients who are perceived to lack a normalized facial
the numerous surgical repairs that children born with orofacial appearance.4
clefting often undergo1 or can be associated with syndromic There are various methods and procedures described for
forms of craniofacial dysostosis where skull base anomalies treating severe midface hypoplasia. A Le Fort III osteotomy
contribute to a lack of anterior and inferior growth of the with midface advancement is an effective maneuver that is
midface.2 capable of addressing all of the aforementioned anatomic
Hypoplasia of the upper two-thirds of the face brings about deformities. Today, selection criteria include those patients in
a multitude of issues for the patient.3 Hypoplastic inferior whom a simpler, less invasive surgery is unable to address one
orbital rims may contribute to relative orbital exorbitism and or more of the issues described earlier. In the authors’ expe-
lead to lagophthalmos. Posterior positioning of nasoethmoid rience an en-bloc movement at the Le Fort III level is often
and maxillary structures is often associated with obstructive unachievable due to the distance needed to advance the
skeleton in the face of hypoplastic and often scarred soft tis-
sue. As many institutions, the authors often perform the
a correction of severe midface hypoplasia in a 2-staged manner.
Department of Oral and Maxillofacial Surgery, San Antonio Military
Medical Center, San Antonio, TX
For example, often during stage 1, the surgeon performs the Le
b
Craniofacial Anomaly Team, Wilford Hall Surgery Center, Lackland Fort III midface separation with application of a skeletal
AFB TX distraction device. In stage 2 the distraction device is removed,
c
Department of Oral and Maxillofacial Surgery, School of Dental and if needed, definitive orthognathic surgery is completed at
Medicine, University at Buffalo, 3435 Main Street, 112 Squire Hall, the Le Fort I or Le Fort II level. Alternative techniques and
Buffalo, NY 14214, USA combinations of osteotomies performed simultaneously have
d
Department of Neurosurgery, Jacobs School of Medicine and also been described. This text focuses on the key steps in
Biomedical Sciences, University at Buffalo, Buffalo NY performing a Le Fort III osteotomy with application of a
e
Division of Pediatric Surgery, Department of Surgery, Jacobs School distraction device. Technique and steps for both the removable
of Medicine and Biomedical Sciences, University at Buffalo, Buffalo,
external distraction (RED) and internal distraction approaches
NY, USA
f
Craniofacial Center of Western New York, John Oishei Children’s
will be discussed. These techniques will be referred to as the
Hospital, Buffalo, NY “external distraction” approach and “internal distraction”
g
Roswell Park Comprehensive Cancer Center, Buffalo, NY approach.
* Corresponding author.
E-mail address: Dan.Schlieder@gmail.com

Atlas Oral Maxillofacial Surg Clin N Am 30 (2022) 85–99

1061-3315/22/Published by Elsevier Inc.
https://doi.org/10.1016/j.cxom.2021.11.004 oralmaxsurgeryatlas.theclinics.com

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86 Schlieder & Markiewicz

Fig. 1 Preoperative planning demonstrating the planned cuts for a Le Fort III osteotomy (A) Virtually planned osteotome guides with
custom osteotomes to aid in subcranial midface separation. The millimeter depth to complete the planned osteotomy can be precisely
measured (B).

Surgical technique ventriculoperitoneal shunt. Clinical examination should be

targeted at addressing globe position, malar projection, nasal-
Preoperative Planning maxillary morphology, and position of the maxilla and
mandible in 3D planes. In addition, a detailed intraoral ex-
amination should address caries, periodontal disease, crowd-
For both the external and internal distraction approaches, the
ing, and maxilla-mandibular dental relationships. The presence
patient’s 3-dimensional (3D) anatomy and deformity should be
of any residual clefts should also be noted. In the setting of
assessed with imaging along with using clinical examination.
external or internal distraction, close coordination with an
The appropriate surgical procedure may then be identified. It is
orthodontist is crucial. Aligning and coordinating the dental
important to realize that the subcranial Le Fort III osteotomy
arches is often followed in the same manner as traditional
will only address symmetric deformities of the middle face.
orthognathic surgery.
Patients with defects of the anterior cranial vault or with
Historically, midface separation was performed in a “free-
associated hypertelorism may be better suited for a frontofa-
handed” manner. Today, computer-aided surgery allows sur-
cial procedure described elsewhere in this text (eg, monobloc
geons to plan the extent of midface movement and if desired
advancement with or without bipartition). In addition, those
fabricate custom osteotome guides (Fig. 1). Endpoints for Le
with more isolated nasomaxillary hypoplasia (eg, Binder syn-
Fort III distraction include malar position, maxillary incisor
drome), or those with differential hypoplasia of the central
position, and most importantly, the position of the external
midface (achondroplasia, Apert syndrome), may be indicated
orbit relative to the anterior cornea. The position of the
for an isolated or combination approach of Le Fort I or Le Fort II
external orbital rim in relation to the anterior cornea is often
osteotomies (with Le Fort III). Preoperative planning begins
the rate-limiting factor with advancement. This movement
with a thorough multidisciplinary workup. Subjective ques-
may result in an acceptable dental relationship; however, an
tionnaires such as the STOP-BANG5 or Epworth Sleepiness
additional Le Fort I osteotomy is often used in a separate stage
Scale6 will point toward the presence of obstructive sleep
to finalize the patient’s occlusion. In the growing patient, an
apnea. A polysomnogram provides a definitive diagnosis, and a
overcorrection of a goal overjet of 5 to 7 mm is often ideal. A
dynamic drug-induced sleep endoscopy may be useful in
bone density map may also be obtained that demonstrates the
identifying the level of obstruction.7 A preoperative ophthal-
thickness of the calvarium where external distractor pins or
mologic examination will establish the presence of lagoph-
internal distractor devices may be placed (Fig. 2).
thalmos or any corneal scaring and provides baseline visual
In patients who are dependent on continuous positive
acuity. Neurosurgical consultation is warranted for patients
airway pressure (CPAP) due to severe obstructive sleep apnea,
with syndromic forms of craniosynostosis where previous sur-
a temporary tracheostomy may be necessary, as they will be
gery has left skull defects or the presence of a

Fig. 2 A bone density map can identify areas of calvarial thinning or defects that may warrant consideration when placing RED pins.

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Craniofacial Syndromes 87

unable to wear their CPAP at night following surgery; this is due

to the risk of positive pressure air forcibly entering the surgical
incisions as well as mechanical interference from distractor
devices.
When possible, the orthodontist should apply a rigid arch
wire with lugs and banded molars. The banded molars may
provide an anchorage point to pull on to aid in mobilization
during down fracture of the midface. Surgical hooks should be
placed to aid in application of class III elastics, which can be
used to modify the vector during internal distraction. In the
setting of external distraction, the RED device wires may be
fixed into a bone-borne or a tooth-borne occlusal splint. If an
occlusal splint is to be used, then this will be fabricated by the
orthodontist before surgery.

Patient Preparation and Patient Positioning

The patient is positioned supine with a horseshoe head rest. At

the surgeon’s preference, hair may be shaved along the plan-
ned coronal incision (Fig. 3). Lubricated corneal shields or
temporary tarsorrhaphy sutures are placed to protect the eyes.
If the patient does not have a tracheostomy, then intubation is
Fig. 4 A subgaleal dissection provides a relatively bloodless sur-
performed orally with an armored endotracheal tube that is
gical approach to the superior orbital rims.
then wired and stabilized to the patient’s mandibular teeth. In
cases where a final occlusion is going to be attempted in a
single-stage en-bloc movement, a submental pull-through proceeds subperiosteally (Fig. 5). Alternatively, an anteriorly
intubation will allow for establishing a final occlusion. Alter- based pericranial flap may be raised in the subperiosteal plan
natively, after osteotomies are made, oral intubation may be for later use. If the supraorbital neurovascular bundle is housed
converted to a nasal intubation to establish occlusion. A small in a bony foramen, this can be mobilized with a wedge
strip of hair is shaved in preparation for the coronal incision. osteotomy at the orbital rim. The zygomatic arches are
One percent lidocaine with 1:100,000 epinephrine is infiltrated exposed in the standard fashion by protecting the temporal
for hemostasis along the planned coronal incision, in the branch of the facial nerve within the superficial temporal
conjunctival inferior fornix of the orbits, and along the
maxillary vestibule.

Surgical ApproachdExternal Distraction Approach

Standard surgical approaches are not discussed in detail;

however, key steps are highlighted.
A coronal incision with a postauricular inferior extension is
made. The authors prefer to perform dissection in a subgaleal
plane to minimize blood loss (Fig. 4); this is continued until 1 to
2 cm superior to the orbital rims at which point dissection

Fig. 5 Approximately 2 cm superior to the supraorbital rims,

Fig. 3 The patient positioned in a horseshoe with hair shaved dissection proceeds in a subperiosteal fashion to expose the entire
over the planned coronal incision. nasofrontal junction.

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88 Schlieder & Markiewicz

Step 2
Malleable retractors are used to protect the eye. A recipro-
cating saw is used to make a 1 cm cut laterally through the
lateral orbital rims.

Step 3
A 701 fissure burr is used to direct the lateral orbital osteotomy
inferior as far as can be reached from the posterior external
approach. A spatula osteotome is then used to complete the
osteotomy inferiorly to the maxilla.

Step 4
From the orbital side, the lateral orbital osteotomy is now
directed inferiorly using a spatula osteotome or ultrasonic saw.

Step 5
Fig. 6 Complete exposure of the zygomatic arches and posterior A sagittal saw is now used to complete the nasofrontal
lateral orbits. osteotomy (Fig. 8).
This cut may be guided if using prefabricated cut guides. It
is angled in a downward vector and extends posteriorly into the
fascia. The temporalis is reflected posteriorly to provide visu- medial orbital walls approximately 1 cm.
alization and dissection of the entire posterior wall of the
lateral orbit and posterior zygoma (Fig. 6). Step 6
Dissection of the medial orbit from the coronal approach The orbit is protected with malleable retractors, and the
proceeds inferiorly down the medial orbital wall, taking care to medial wall osteotomy is now completed with a spatula
identify and gently retract the lacrimal sac such that a clear osteotome or ultrasonic cutting instrument. It is crucial to
pathway behind the posterior lacrimal crest is identified visualize the lacrimal sac directly and direct the osteotomy
(Fig. 7). The lateral orbit is stripped posteriorly and inferiorly posterior to the posterior lacrimal crest.
to expose the entire lateral orbit. A transconjunctival surgical
incision is then used to expose the orbital floor and connect to Step 7
the medial and lateral dissections. Through the transconjunctival incision the orbital floor
Some surgeons choose to perform pterygoid plate osteoto- osteotomy is completed using either a spatula osteotome or
mies through the coronal incision, whereas others will perform ultrasonic cutting instrument. This osteotomy is extended to
them in a standard orthognathic fashion. If the latter is used, a connect to the lateral and medial wall osteotomies. It should
Le Fort I incision is completed with dissection to the pterygoid be positioned 5 mm posterior to the orbital rim.
plates. Of note, exposure through a transconjunctival incision can
sometimes be avoided, and all osteotomy sites may be
Surgical Procedure accessed via the coronal incision.

Step 1 Step 8
A reciprocating saw is used to separate the zygomatic arches at The pterygoid plates are now osteotomized. This may be
the point where the zygomatic arch begins to increase in width guided using a custom titanium guide and custom osteotome or
before meeting the body of the zygoma. Typically, this is at or
near the zygomatic-temporal suture.

Fig. 7 The medial orbital wall dissection with the lacrimal sac
retracted and a metal malleable retractor placed posterior to the
posterior lacrimal crest. Fig. 8 A sagittal saw is used at the nasofrontal junction.

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Craniofacial Syndromes 89

free-handed. The authors prefer to use the coronal approach

for this step, although if mobilization proves difficult, then a
standard intraoral approach with a curved osteotome may be a
useful adjunct to compete the pterygoid separation (Fig. 9A).

Step 9
The last maneuver to complete midface separation is the
nasofrontal dysjunction, which is an osteotomy through the
nasofrontal suture to the posterior maxillary spine. One of the
authors again prefer a guided osteotome to minimize the risk
of intracranial embarrassment as well as precisely guiding the
depth of the osteotome (Fig. 9B).
However, if the surgeon is choosing to free hand this ma-
neuver, then a finger is placed on the posterior maxillary crest
as a reference point to aim the osteotome toward.

Step 10 Fig. 10 Smith spreaders placed at the nasofrontal junction aid in

Midface down fracture is completed by gentle downward subcranial midface separation during down fracture. (Courtesy of
distraction with Rowe disimpaction forceps while simulta- Jeffrey James MD, DDS, FACS, Augusta, GA)
neously spreading with Smith spreaders at the nasofrontal
osteotomy, lateral orbit osteotomies, and zygomatic arch
osteotomies (Fig. 10). Step 16
Closure of the Le Fort 1 level incision proceeds with 3-0 chro-
Step 11 mic gut suture in a running fashion.
Mobility is assured.
Potential Complications
Step 12
Resuspension of the temporalis is achieved by drilling 2 or 3
holes with a 701 fissure burr in the lateral orbit above the Intraoperative
osteotomy site and securing the muscle with 3-0 vicryl sutures Severe bleeding from the maxillary artery may occur during the
(Ethicon, Johnson & Johnson, New Brunswick, NJ) at these pterygoid osteotomy, which may be controlled locally or by
sites. The temporalis fascia is closed with 3-0 vicryl as well. embolization. Intracranial injury is possible during the naso-
frontal osteotomy in cases with severe verticalization of the
Step 13 anterior cranial floor and lack of a guided osteotome in this
The coronal incision is closed with 3-0 vicryl subdermal/galeal region; this can be avoided by precise preoperative planning of
sutures and skin with staples. the path of this osteotome. Another potential complication is
incomplete midface separation, which may occur during down
Step 14 fracture, and this may result in uncontrolled fracturing of the
The RED device is placed with cranial pins secured finger tight zygomas without full mobilization of the nasomaxillary struc-
(Fig. 11). tures. This can be minimized by slow spreading with Smith
spreaders and repeat osteotomies at all sites.
Step 15
The authors prefer 2 crossbar fixation levels for a Le Fort III, Postoperative
one at the inferior orbital rim and one at the piriform rim. Pins The most immediate postoperative complication is airway
are secured to plates in these locations and fixated to the RED obstruction secondary to edema. This risk can be minimized by
with 24-gauge prestretched wires. planning in advance for a temporary tracheostomy in high-risk
patients. In otherwise lower risk patients, but where surgery

Fig. 9 Custom titanium osteotome guides fabricated to precisely guide separation of the pterygoid plates from a coronal approach (A) as
well as perform the nasofrontal disjunction (B).

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90 Schlieder & Markiewicz

Fig. 11 A patient with an RED device secured after completion of a Le Fort III osteotomy.

Fig. 12 A patient with severe midface hypoplasia as a result of congenital orofacial clefting. Results demonstrating the significant
improvement in orbital rim, midface, and occlusal relationship after a Le Fort III midface advancement from a frontal (A), lateral (B), and
birds-eye view (C). Occlusal views showing a stable occlusion in class 1 relationship from a frontal (D) and lateral (E) view.

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Craniofacial Syndromes 91

Fig. 13 Computer-aided virtual planning offers the ability for the surgeon to assess the 3D anatomy (A) and to import STL files of various
external rigid devices such as the RED (B) and a variety of internal distractors (C, D) to select the ideal device for the patient. The final
movement and vector can be visualized (E). Distractor position can be simulated and positioning guides can be virtually fabricated (F).
Bony thickness can be assessed for optimal screw placement and vector (G). A bony thickness heat map is helpful when planning distractor
foot-plate placement (H). The final movement and occlusion can be assessed as is seen her in a virtual frontal view (I).

was difficult, leaving them intubated overnight until airway nonchew, mush diet for comfort. Showering and washing their
edema has subsided is prudent. Another later complication of hair using baby shampoo and water is encouraged. RED pins are
RED hardware failure can occur during the active phase of wrapped in xeroform gauze for the first 3 days after surgery
distraction necessitating immediate return to the operating and then kept clean by removing dried heme with a 50:50
room to resecure it. hydrogen peroxide and sterile saline solution. RED removal
usually requires a return to the operating room for hardware
Immediate Postoperative Care removal as well as pin site scar revision. If a second stage
orthognathic surgery is planned to finalize the occlusion, it may
also be performed during this encounter (Fig. 12).
When an uneventful surgery is accomplished, the patient may
be extubated at the end of the case in the safety of the
operating room, with all equipment necessary to rapidly Surgical techniquedinternal distraction
remove the RED cross bars available if necessary. They are then approach
observed in the intensive care unit for at least one night after
surgery with subsequent step down and discharge dependent Preoperative Planning
on the patient’s recovery course.
Preoperative planning is similar to that of the external
Rehabilitation and Recovery distraction approach using an RED device. Computer-aided
preoperative planning is advantageous when using internal
When a midface distraction has been planned, an active distractors in that the anatomic anomaly can be assessed in the
distraction period followed by a consolidation period takes 3D plane (Fig. 13A), STL files of external and internal dis-
place. Typically, a 2-week distraction period will be followed tractors can be imported (Fig. 13BeD), the vector of move-
by a 6-week consolidation period while bone heals, after which ment can be simulated (Fig. 13E), as well as simulation of the
the RED may be removed. The rate of distraction is determined device footprint (Fig. 13F); this is crucial to choosing either
by the age of the patient and ranges from 0.5 to 2 mm per day. external or internal devices or the specific type of internal
The patient should be seen on a biweekly basis during active device. Bony thickness can be assessed to precisely plan screw
distraction such that the vector of distraction may be adjusted placement for internal devices (Fig. 13G, H). The final occlu-
in a real-time basis. During this time the patient is on a sion and movement can then be simulated (Fig. 13I). Syndromic

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92 Schlieder & Markiewicz

Fig. 14 The patients imaging should be compared with frontal (A), worms-eye (B), and side profile (C) views, as well as intraoral views of
the patient’s occlusion (DeF). This should be compared side by side when performing the computer planning session. Patients who have
undergone previous anterior cranial vault remodeling should be assessed for full-thickness defects of the cranial vault as well as the need
for volume augmentation. Patients who have had several surgeries prior often have significant deformities and deficiencies of the temporal
and parietal areas as well as the forehead (G, H).

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Craniofacial Syndromes 93

Fig. 15 During the preoperative planning session, the areas of deformity should be identified and can also be compared with age-
standardized norms. Critically evaluating these areas will allow the surgeon to add volume to those areas that are area deficient, and more
importantly, provide coverage for full-thickness cranial defects (AeC).

Fig. 16 The patient is positioned. The dysmorphology of the forehead, temporal, and parietal areas can be assessed from a side (A) and
birds-eye (B) view.

patients who have undergone previous anterior cranial vault muscles. The temporalis should only be reflected to the extent
remodeling often have a grossly dysmorphic forehead. When a needed and to where the zygomatic arches can be exposed and
forehead plasty is planned using a custom patient-specific to facilitate placement of the internal distractors (Fig. 17).
implant, this may be planned at the same time or at a time Once exposure is obtained, the custom cranial implant may be
prior or after the subcranial midface advancement (Fig. 14). tested on the back table for positioning using an selective laser
The implant provides 2 primary purposes: (1) to provide melting (SLM) model (Fig. 18A) and then inserted and fixated to
coverage of any full-thickness cranial defects and (2) to restore the patient (Fig. 18B). Alternatively, the implant may be
and add volume to deficient areas of the upper third of the placed just before closure after completing the Le Fort III
face and anterior cranial vault (Fig. 15). osteotomy and applying distractors. The preinsertion and
postinsertion view of the implant can be seen in Fig. 19,
Patient Preparation and Patient Positioning respectively. SLMs are invaluable in the operating room for
highlighting preplanned osteotomies and comparing this with
Patient preparation and positioning is similar to that of the the live patient (Fig. 20A). In addition, a palatal splint fabri-
previously described external approach (Fig. 16). cated by the orthodontic team before surgery is brought to the
operating room to be used during down fracture of the midface
(Fig. 20B).
Surgical ApproachdInternal Distraction Approach
Steps 2 to 11
A similar approach is used as previously described for external Steps 2 through 7 as previously described for the external
distraction use. However, unlike external distractor use, more distraction approach are similar. An ultrasonic cutting instru-
lateral exposure of the cranial vault is needed. The authors ment is again helpful when making cuts around the orbits and
prefer to apply internal distractors underneath the temporalis

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94 Schlieder & Markiewicz

Fig. 17 Once exposed, the deformity can be appreciated from a birds-eye (A), lateral (B), and frontal (C) views.

Fig. 18 The implant is then tried on the SLM model on the back table (A) and then applied to the patient in situ (B). The custom implant
is then fixated using several prefabricated drill holes.

Fig. 19 The effect of the implant and resolution of the volume-related defects can be appreciated before (A) and after the coronal skin
flap is temporarily repositioned (B).

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Craniofacial Syndromes 95

Fig. 20 An SLM is invaluable for aiding in visualization of the preplanned osteotomies of the preplanned osteotomies (A). An occlusal
splint is fabricated for planned use during the down fracture (B). This will protect the palate during midface manipulation.

Fig. 21 Similar to the external approach, all osteotomy cuts are similar, including the nasofrontal osteotomy cut (A). The vector can be
confirmed once the guide is removed (B).

periorbita. The cut at the nasal-frontal junction be performed (Fig. 24); this negates the need for suture removal. No increase
as previously described with the use of the guide as to avoid in inflammation or scaring has been seen when using this suture
the cribriform plate (Fig. 21). If being performed free hand, in the scalp when compared with nonabsorbable sutures or
the cut should be directed inferiorly and posteriorly. Steps 8 staples.
through 11 are similar for the external and internal distractor
approach as well.
Potential Complications
Step 12
Before closure, the internal distractor positioning guides are Intraoperative
compared on the SLM and the patient for replication of position Intraoperative complications are similar to that of the external
and vector when comparing with the preoperative plan distractor approach. An additional complication is failure of
(Fig. 22A, B). The same is then done for the distractors the hardware at either the footplate or the distractor arm/
(Fig. 22C). Once the distractors are placed, symmetry of the distractor interface. Fracture of hardware is usually due to
distractors is then compared before closure (Fig. 23). The resistance from incomplete osteotomies. If this occurs, the
distractors are tested, and the midface is advanced 5 to mobility of the midface and separation at all osteotomy sites
10 mm, noting passive spreading at all osteotomy sites, should be confirmed. Fracture of the distractor arm itself or
bilaterally. around the interface between the distractor arm and foot
plate is often due to excessive pressure by the soft tissues on
Step 13 the distractor arm at the posterior stab incision. In the event of
Closure then takes place in a similar manor to that described distractor arm fracture, a passive vector of the distractor arm
for the external device. As an alternative to drilling holes in as it exits the skin should be confirmed.
the lateral orbits, the custom forehead implant may be used as
an anchor to suspend the temporalis muscles to. When a Postoperative
pericranial flap is raised, it is meticulously resuspended along Postoperative complications are similar to that of the external
with the temporalis muscles and galea aponeurotica. Next, a approach. Failure of hardware during this period is likely due
stab incision is made in the posterior aspect of the coronal flap, to the reasons stated in the previous section. Hardware failure
and the distractor arms are passively facilitated through the in this setting would necessitate immediate return to the
stab incision posteriorly. After deep closure, one of the authors operating room. In the case where one or both sides of the
prefers to close skin with 4-0 vicryl rapid suture (Ethicon, distractors do not turn or when turning does not advance the
Johnson & Johnson, New Brunswick, NJ) in a running fashion midface, imaging in the form of a computed tomography (CT)
with 3D reconstructions should be obtained to ensure all

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96 Schlieder & Markiewicz

Fig. 22 The distractor positioning guide can be applied to the SLM model (A) and then compared once applied to the patient (B). The
distractors are then be double checked on the model (C).

Fig. 23 After being applied the distractors are checked for symmetry (A) and position (B). Movement of the midface on turning the
distractors is then confirmed bilaterally.

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Craniofacial Syndromes 97

Fig. 24 Temporalis muscles and pericranium are closed bilaterally followed by skin closure. A stab incision is carefully placed so the
distractor arms passively exit the skin.

Fig. 25 A birds-eye view of the immediate postoperative CT confirms osteotomy cuts (A). All cuts using a variety of views should be
assessed for patency. The same view is shown of the postoperative CT just within a few days of completion of distraction (B).

Fig. 26 A lateral view of the immediate postoperative CT confirms osteotomy cuts (A). Again, all cuts using a variety of views should be
assessed for patency. The same view is shown of the postoperative CT just within a few days of completion of distraction (B).

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98 Schlieder & Markiewicz

Fig. 27 A lateral view of the patient is seen just after removal of distractors (A). The same patient’s occlusion is shown just after
removal of distractors (BeD).

planned osteotomies have been completed and all sites are active distraction phase. For internal distractors, the authors
being distracted adequately. To monitor osteotomies and prefer a period of at least 2.5 to 3 months of consolidation
movement, the authors obtain an immediate postoperative CT before hardware removal.
and then a CT at the completion of distraction. In the case of
any untoward movement or cessation of movement, another
CT is then obtained. Figs. 25 and 26 compare immediate Clinical results in the literature
postoperative and postdistraction imaging. Fig. 27 displays the
same patient just after distractor removal. The Le Fort III osteotomy has routinely proved to have a high
level of success. A 2019 retrospective study of 15 patients with
Immediate Postoperative Care a 10-year follow-up performed by Gibson and colleagues8
demonstrated that Le Fort III distraction produced an average
advancement of 14.86 mm at A-point and 10.54 mm at orbitale
Immediate postoperative care is similar to that of the external
with excellent 10-year stability.
approach, except that the patient does not have an RED device
Adenotonsillar hypertrophy is an important cause of
attached. The internal distractor arm sites are prone to
obstructive sleep apnea in the general pediatric population.
infection. Vaseline gauze can be wrapped around the sites for
However, in patients with syndromic forms of craniosynostosis,
the first 3 days. After this, similar to other distractor sites,
midface hypoplasia is commonly thought to be the major cause
wound care and hygiene is critical. Wound care includes
of upper airway obstruction.9 A 2010 retrospective review10 of
cleaning the distractor arm incision and arms with a mixture of
14 patients with syndromic forms of craniosynostosis and
half peroxide and half water twice to three times daily. Sur-
moderate to severe obstructive sleep apnea showed 55% of the
gical site infections at the distractor arm site incisions can
patients treated with midface advancement had improvement
usually be managed with local wound care in the form of cot-
in their sleep apnea. Kim and colleagues11 showed the poste-
ton swab cleaning with a mixture of half peroxide and half
rior pharyngeal airway space undergoes a mean increase of 33%
normal saline. Antibiotics with staphylococcal coverage may be
after Le Fort III advancement.
indicated when local wound care alone does not resolve the
One study to date has investigated the changes to orbital
infection. The need for surgical intervention is uncommon.
and globe volume following Le Fort III midface advancement.
Smektala and colleagues12 demonstrated an average of 30%
Rehabilitation and Recovery increase in orbital volume following Le Fort III advancement.
These findings have been corroborated by similar improve-
The distraction phase may begin after a 3- to 5-day latency ments following monobloc distraction.13e15
phase after surgery. Alternatives, especially in the younger
patient, include starting the distraction phase the day of or the
day after surgery. However, no longer than this period should Summary
take place to avoid preemptive fusion of the osteotomy sites.
Once distraction is complete, the arms can be removed. After The Le Fort III osteotomy is a defining surgery for the correc-
this the stab incision usually will heal, leaving the hardware tion of severe midface hypoplasia. It may be used in combi-
underneath to allow for bony healing and consolidation. Dis- nation with other osteotomies and may be performed with
tractor removal can take place after a delay of 2 to 3 times the adjunctive distraction osteogenesis. It is capable of improving

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uso personal exclusivamente. No se permiten otros usos sin autorización. Copyright ©2025. Elsevier Inc. Todos los derechos reservados.
Craniofacial Syndromes 99

orbital exorbitism, obstructive sleep apnea, dental occlusal 7. Certal V, Pratas R, Guimara ~es L, et al. Awake examination versus
relationship, and facial esthetics. Often a 2-stage approach is DISE for surgical decision making in patients with OSA: a systematic
necessary to first advance the midface at the Le Fort III level review. Laryngoscope 2015;126(3):768e74. https:
and secondarily finalize the occlusion at the Le Fort I or Le Fort //doi.org/10.1002/lary.25722.
8. Gibson T, Grayson B, McCarthy J, et al. Maxillomandibular and
II level.
occlusal relationships in preadolescent patients with syndromic
craniosynostosis treated by Le Fort III distraction osteogenesis: 10-
References year surgical and phenotypic stability. Am J Orthod Dentofacial
Orthop 2019;156(6):779e90.
1. Figueroa A, Polley J. Management of the severe cleft and syn- 9. Lo L, Chen Y. Airway obstruction in severe syndromic craniosy-
dromic midface hypoplasia. Orthod Craniofac Res 2007;10(3): nostosis. Ann Plast Surg 1999;43(3):258e64.
167e79. https://doi.org/10.1111/j.1601-6343.2007.00392.x. 10. Bannink N, Nout E, Wolvius E, et al. Obstructive sleep apnea in
2. Goldstein J, Paliga J, Wink J, et al. Earlier evidence of spheno- children with syndromic craniosynostosis: long-term respiratory
occipital synchondrosis fusion correlates with severity of midface outcome of midface advancement. Int J Oral Maxillofac Surg 2010;
hypoplasia in patients with syndromic craniosynostosis. Plast 39(2):115e21.
Reconstr Surg 2014;134(3):504e10. 11. Kim S, Min K, Jeong W, et al. Three-dimensional analysis of airway
3. Engel M, Berger M, Hoffmann J, et al. Midface correction in pa- change after Le Fort III midface advancement with distraction. Ann
tients with Crouzon syndrome is Le Fort III distraction osteogenesis Plast Surg 2018;80(4):359e63.
with a rigid external distraction device the gold standard? J Cra- 12. Smektala T, Nysjö J, Thor A, et al. Three-dimensional eyeball and
niomaxillofac Surg 2019;47(3):420e30. https://doi.org/10.1016/j orbit volume modification after LeFort III midface distraction. J
.jcms.2018.11.028. Craniofac Surg 2015;26(5):1652e5.
4. Pinckston M, Dalton L, Farrar S, et al. The psychosocial adjustment 13. Imai K, Fujimoto T, Takahashi M, et al. Preoperative and post-
of children born with a cleft lip and/or palate: cross-sectional and operative orbital volume in patients with Crouzon and Apert syn-
longitudinal analyses. Cleft Palate Craniofac J 2020;57(11): drome. J Craniofac Surg 2013;24(1):191e4.
1280e90. https://doi.org/10.1177/1055665620921669. 14. Bender C, Veneman W, Veenland J, et al. Orbital aspects following
5. Kawada T. Screening ability of STOP-bang questionnaire for monobloc advancement in syndromic craniosynostosis. J Cranio-
obstructive sleep apnea. Anesth Analg 2018;128(3):e48e9. https: maxillofac Surg 2013;41(7):e146e53.
//doi.org/10.1213/00000539-900000000-96340. 15. Nout E, van Bezooijen J, Koudstaal M, et al. Orbital change
6. Johns M. A New method for measuring daytime sleepiness: the following Le Fort III advancement in syndromic craniosynostosis:
Epworth sleepiness Scale. Sleep 1991;14(6):540e5. https: quantitative evaluation of orbital volume, infra-orbital rim and
//doi.org/10.1093/sleep/14.6.540. globe position. J Craniomaxillofac Surg 2012;40(3):223e8.

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