Syndromic cranial synostosis

Syndromic cranial synostosis
Dr Jameel Kifayatullah
Lecturer KCD

Syndromic craniosynostosis
• Also called craniofacial dysostosis

• There are reported more than 90 syndromes
associated with craniosynostosis. Most of these are
also associated with other anomalies of the limbs, ears,
and cardiovascular system. The syndromes which are
most commonly encountered by surgeons are the :
• Apert syndrome
• Crouzon syndrome
• Pfeiffer syndrome
• Saethre-Chotzen syndrome
• Muenke syndrome

Cardinal feature
• A cardinal feature of the syndromic cranial
synostosis is:
• midface hypoplasia
• exorbitism
• forehead retrusion
• Most of these patients have bicoronal synostosis
but multiple cranial sutures in different
combinations may be involved.

SYNDROMIC CRANIAL SYNOSTOSIS
• Clover leaf skull or
Kleeblattschädel, is seen in
any of the syndromes listed
above and is characterized
by closure of all cranial
sutures except sagittal and
squamosal.
• To the left, the appearance
of the "Beaten Copper
Cranium" pattern in a CT-
scan of a patient with a
clover leaf skull deformity

• Apert syndrome
(Acrocephalosyndactyly
type I)
• caused by a mutation in
the FGFR2 gene and
occurs sporadically, but
can be inherited in an
autosomal dominant
fashion.
• 1:160,000 live births.

Syndromic synostosis
• The Apert syndrome is characterized
by:
• Bicoronal or multiple suture
synostosis
• Exorbitism
• Midface hypoplasia
• Maxillary hypoplasia with Class III
malocclusion
• Mild hypertelorism
• Complex syndactyly of the fingers
and toes
• Cleft palate (30%), high arched palate
• CNS malformations – macrocephaly,
hydrocephalus (5-10%),
developmental delay and lowered IQ

Syndromic Cranial Synostosis
• Crouzon syndrome
(Acrocephalosyndactyly
type II)
• caused by a mutation
in the FGFR2 gene and
inherited in an
autosomal dominant
fashion.
• reported in 1:25,000
live births.

Syndromic Craniosynostosis
• The Crouzon syndrome is
characterized by:
• Bicoronal (or multiple
suture) synostosis
• Midface hypoplasia
• Maxillary hypoplasia with
Class III malocclusion
• Exorbitism
• Absence of limb
anomalies is a defining
characteristic

Saethre-Chotzen syndrome (Acrocephalosyndactyly
type III)
• Caused by mutations in
the TWIST1 gene, and is
inherited in an
autosomal dominant
fashion.
• Saethre-Chotzen
syndrome is reported in
1:25,000 – 1:50,000 live
births.

• The Saethre-Chotzen syndrome
is characterized by:
• Bicoronal synostosis
• Skull asymmetry
• Maxillary hypoplasia with a
narrow palate (not typically
midface retrusion)
• Low set hairline
• Ptosis of the eyelids
• Ear anomalies
• Incomplete simple syndactyly of
the index and middle fingers
and the 3rd and 4th toes
• Typically normal intelligence

• Pfeiffer syndrome is linked
to a mutation in in both the
FGFR1 (5-10 %) and the
FGFR2 (90-95%) gene and is
inherited in an autosomal
dominant fashion with
incomplete penetrance.
• Pfeiffer syndrome is
reported in 1:100,000 live
births.

Pfeiffer syndrome is
characterized by:
• Bicoronal or multiple
suture synostosis
• Maxillary hypoplasia
• Exorbitism
• Hypertelorism
• Broad thumbs and toes
• IQ varying from normal to
severely impaired

• Muenke Syndrome
• Muenke syndrome is
linked to a mutation in
the FGFR3 gene and is
inherited as an
autosomal dominant
trait.
reported in 1:50'000
live births.

characterized by:
• Bi- or uni-coronal suture
synostosis
• Mild or no midface
hypoplasia
• Typically normal
intelligence

Posterior vault expansion by
distraction osteogenesis
Main indications
• Syndromic
craniosynostosis affecting
the posterior skull e.g.
turribrachycephaly.
Further indications
• In patients with
turribrachycephaly, where
conventional reshaping is
often associated with a
high degree of relapse

Advantages
• Allows for greater increase in
intracranial volume
• Allows for the slow expansion of
the soft tissue envelope to
accommodate the movement and
reduce the relapse potential]
• Note: considered to be the
procedure of choice in the
management of the syndromic
patient.
• Disadvantages
• Need for absolute
patient/parent compliance
with treatment (daily
activation of device)
• Higher risk of infection
• Need for secondary
procedures to adjust and
remove distractor(s)

Posterior vault expansion by distraction
osteogenesis
• Posterior cranial vault distraction is being
increasingly used for the treatment of
Syndromic synostosis. Many patients have
turribrachycephaly, and expansion of the
posterior vault allows for more complete
total vault reshaping. Conventional
reshaping in these patients is often
associated with a high degree of relapse.
Distraction gives one the ability to not
only expand posteriorly to a greater
degree, but as the brain is expanded as
well, there may be less relapse. Many
people consider it to be the procedure of
choice in the management of the
syndromic patient.
• Although no specific distraction devices
for posterior cranial vault distraction are
commercially available at the time of
writing, mandibular devices utilizing 1.3
to 2.0 mm with a 25 to 40 mm barrel
length can be used.

• Positioning
• With the patient in a
prone position with the
head resting on a
horseshoe head rest,
exposure of the
posterior half of the
skull is made via
a coronal incision.

• Outline of osteotomy
lines
• After exposure of the
posterior cranial vault, a
craniotomy of the
occiput and the parietal
regions is outlined.

• Burr holes are placed by the
neurosurgeon along the
planned osteotomy lines and
an epidural dissection
between these points is made.
• The osteotomies are then
completed using a craniotome.
• Note: The bone flap is not
removed during this
procedure. Leaving the dura
attached to the endocranial
surface of the bone flap
maintains the vascular supply
to the segment, and allows the
brain to be expanded
concurrently.

• Barrel-stave" osteotomies
with outfracture are
performed in the inferior
occipital segment to avoid
any step-off between the
distracted segment and
the cranial base. The
newly positioned barrel-
staves may be left to float
or are stabilized with a
long resorbable plate.

• In case a posteroinferior
trajectory is chosen, an
osseous wedge is
removed from the
junction of the
craniotomy segment
and the posterior vault
to ensure unhindered
travel of the distracted
piece.

• Placement of the distractors
• Two distractors, one on each side, are
adapted to the underlying bone and
placed in a parasaggital colinear
position, oriented with a trajectory
either directly posteriorly or posterior
inferior. As it is a single vector device
one must be certain that the position
fits the vector chosen. The distractor
is then attached with at least five 4
mm titanium screws per footplate.
• The distractor is activated to ensure
proper function and free travel of the
bone flap. The distractors are then
returned to neutral position.

• Fixation of the
distractor
• This 3D-CT scan shows
the distractor in place

• The activation arm of
the distractor is brought
out through a stab
incision either in the
posterior surface of the
scalp or the anterior
scalp flap.

• After a latency period of
3-5 days, distraction
commences at the rate of
approximately 1 mm/day.
It is common practice to
instruct a relative of the
patient how to perform
the distraction. Periodic
(usually weekly) review in
the office is carried out
until the desired position
is reached (15-30 mm).

• Removal of internal distractor
• Following the completion of distraction, the
device is left in place for 2-3 months to allow
for consolidation of the regenerate bone and
is then removed.

Bilateral orbital advancement
• The standard treatment
is bilateral supraorbital
bar advancement and
reshaping within the
first year of life.

• For this procedure
the coronal approach is
used.

• Craniotomy
• After the exposure of the
forehead and the orbits via a
coronal approach, a bifrontal
craniotomy beginning 1 cm
superior to the superior orbital
rim and extending to behind the
coronal sutures is outlined.
• Burr holes are first placed at the
vertex, avoiding the sagittal sinus,
and nasal frontal region as well as
temporally. An epidural dissection
• The neurosurgeon then
completes the osteotomies using
a craniotome.

• Osteotomy
• After the bone flap is
removed, the dura is
freed from the anterior
and middle fossae in
the epidural plane.
• The dura is protected
with neurosurgical
cottonoids.

• Supraorbital bar
osteotomy
• Malleable retractors are
used intracranially to
retract and protect the
dura and intraorbitally
to protect the orbital
contents when
performing the
osteotomies.

• The supraorbital bar is
then osteotomized.
• A vertical osteotomy near
the pterion (1) is followed
by a horizontal osteotomy
to the lateral orbital rim
(2). An oblique osteotomy
is then made through the
orbital rim (3) and a
transverse osteotomy is
completed at the nasal
frontal junction (4).

• Alternatively, a tongue-
in-groove a) or a step
osteotomy b) can be
used in the temporal
region as shown.

• Orbital roof osteotomy
• With the brain carefully
retracted, a right angle saw
is then turned intracranially
and the orbital roof is
osteotomized beginning at
pterion laterally and ending
at the nasal frontal
osteotomy medially, joining
the osteotomy made extra
cranially across the nasal
frontal region.

• Mobilization of the
supraorbital bar
• An osteotome is then
inserted at the pterion
and the lateral orbital
wall osteotomy
completed on both
sides, releasing the
supraorbital bar.

• Positioning of the
supraorbital bar
• The supraorbital bar is then
advanced 10-15 mm.
• In infants a maximal
advancement is usually
chosen.
• In adults who have finished
craniofacial growth,
advancement is done so
that the superior orbital rim
is approximately 12 mm
anterior to the cornea

• Fixation of the
supraorbital bar
• The advanced bar is
then stabilized. A wire
or suture is placed
between the stable
lateral orbital rim and
the supraorbital bar.

• Pearl: To improve the
stability of the
advanced segment, a
cranial bone graft may
also be wedged and
placed in the orbital
roof between the stable
posterior segment and
the advanced anterior
segment.

• Grafting of temporal
bone gap
• Bone graft harvested
from the frontal bone flap
is then inserted in the
temporal gap and held in
position with a resorbable
plate (infants and
children) or metallic plate
(adults).

• If metallic or resorbable
plate fixation is
unavailable, self-
retaining osteotomies
may be designed and
utilized in conjunction
with wire or suture
fixation.

• Replacement and fixation of the
frontal bone flap
• The frontal bone flap is then
replaced in the desired position.
• If the frontal bone flap is
irregular, it can be rotated, bent
and/or reshaped to get the best
possible fit and contour before
replacement using:
• A bone bending forceps
(children).
• Partial osteotomies and plate
fixation (adults).

• The bone flap is then
affixed to the
supraorbital bar with
resorbable plates or
resorbable sutures
(infants), or titanium
plates (adults).

• Grafting of coronal bone
gap
• The coronal bone gap
created from the
advancement and
harvesting of bone is then
filled with particulate bone
shavings harvested with a
manual hand-held burr-hole
instrument or commercial
harvester (eg, Safe scraper)
or the posterior skull.

• This picture
demonstrates the large
volume of particulate
bone that can be
harvested from the
inner surface of a bone
flap using a hand-held
burr-hole instruments.

Le Fort II osteotomy
• Main indications
• Patient with nasomaxillary
deficiency without significant
exorbitism.
• The majority of syndromic
synostosis patients with
maxillary deficiency will
benefit from advancement of
the maxilla at either a le Fort I
or a le Fort III level. However, a
few patients who have
nasomaxillary deficiency may
require a le Fort II osteotomy.

• Le Fort II osteotomy
Advantage
• Single stage procedure to
advance complete naso-
maxillary complex resulting
in total midface
advancement
Disadvantage
• More complex surgical
procedure requiring
additional skills and carrying
more risks for complications

Introduction
• The majority of synostosis patients with
maxillary deficiency will benefit from
advancement of the maxilla at either a le Fort
I or a le Fort III level. However, a few patients
who have nasomaxillary deficiency may
require a le Fort II osteotomy.

Approach
• The midface is accessed by
a coronal
approach combined
with maxillary buccal sulcus
incisions.
• Note: As an alternative
some surgeons use a
combination of paranasal
and oral incisions and avoid
the coronal approach.
•

• Comments on the coronal approach
• The coronal approach is used with
the following considerations:
• Only the nasal bridge requires
exposure, so lateral dissection of the
coronal flap can be minimized (as
illustrated)
• The dissection proceeds into the
orbit behind the posterior lacrimal
crest inferiorly to the orbital floor
taking great care not to damage the
naso-lacrimal ducts which are
displaced anteriorly with the flap.
• The dissection in the orbital floor
should then communicate with the
subperiosteal dissection inferiorly
from the oral approach.

• Comments on the buccal
sulcus approach
• The buccal sulcus
approach is used with the
following considerations:
• The intraoral dissection
must communicate with
the inferior aspect of the
coronal approach in the
nasomaxillary region

• Osteotomies
• An osteotomy cut is made
across the nasal bridge above
the lacrimal crests and angling
slightly downwards. This cut
should extend to a few mm
behind the posterior lacrimal
crest.
• Care has to be taken that this
cut is below the level of the
cribriform plate in the anterior
cranial fossa. This is best
evaluated preoperatively by
coronal CT.

• Orbital osteotomies
• The nasal osteotomy is
extended inferiorly behind the
posterior lacrimal crest down
towards the orbital floor. The
inferior aspect of this
osteotomy should connect
with the superior aspect of the
osteotomy to be made from
the oral approach and will be
just medial to the infraorbital
nerve. The periorbita is
retracted during these
osteotomies with flat
malleable retractors to protect
the orbital contents.

• Maxillary osteotomy
• From the transoral approach a vertical
osteotomy is made through the infraorbital
rim medial to the infraorbital nerve,
connecting with the previous osteotomy from
above in the orbital floor.
• Pearl: The distance between this vertical cut
and the pyriform rim of the nose is narrow
and produces a weak area that may fracture
during mobilization. It is advisable to make
the vertical cut as lateral as possible.
• It is usually possible to do this solely with a
combination of the coronal and transoral
approaches. If difficulty is encountered in the
region of the orbital floor, the approaches can
be supplemented by a transconjunctival
incision.

• Inferior to the
infraorbital nerve, the
osteotomy is extended
posteriorly to the
pterygomaxillary
junction as in a Le Fort I
osteotomy.
• All osteotomies are
checked for completion
with fine osteotomes.

• Pterygomaxillary
dysjunction
• The maxilla is separated
from the pterygoid
plates with a curved
osteotome from the
transoral approach.

• Nasal septum osteotomy
• The final osteotomy is made
through the nasal septum
from the nasal bridge
towards the posterior nasal
spine. To accomplish this, a
1 cm wide gently curved
osteotome with the
curvature pointing inferiorly
is introduced through the
nasal bridge osteotomy.

• The surgeon directs the
osteotome towards the
back of the nasal septum
and places a finger
behind the soft palate at
the posterior nasal spine.
The osteotome must be
directed towards the
posterior nasal spine.
• This completes the Le
Fort II osteotomies.

• Positioning of the
nasomaxillary complex
• Mandibular-maxillary fixation
is performed to position the
nasomaxillary complex to the
desired relationship with the
mandible. A prefabricated
surgical splint/wafer may be
used to facilitate this.
• The maxillomandibular
complex is now rotated
around the condylar hinge
until the planned position has
been attained.

• The nasomaxillary complex is
then mobilized with Rowe's
disimpaction forceps taking
great care to observe that all
the osteotomy sites are
opening.
• Full mobilization is necessary
to allow passive repositioning
of the nasomaxillary complex.
• Mobilization can be
supplemented with the use of
Tessier mobilizers inserted
behind the maxillary
tuberosities from the transoral
approach

• Fixation
• The nasomaxillary complex is
stabilized with two miniplates across
the nasal bridge, one on each side.
This is supplemented by two
additional miniplates placed across
the zygomaticomaxillary buttresses.
• After completion of osteosynthesis
on both sides, the MMF is removed
and the resulting occlusion is checked
against the pre-planned position.
• The splint may be fixed to the
maxillary teeth with a few thin wires
and left in place during the healing
phase to allow for neuromuscular
adaption and position control.

• Grafting of bone gaps
• Bone grafting is carried out with
cortico-cancellous bone blocks. The
principal area for grafting is the nasal
bridge, but grafts are also sometimes
required within the orbit and on the
lateral maxilla.
• The graft over the nasal bridge can be
contoured with a burr to provide the
correct shape of the nasofrontal
angle.
• Pearl: It is sometimes necessary to
reposition the medial canthal
ligaments in order to reduce the
intercanthal distance. This should be
performed before the bone grafting
procedure.

Le Fort III - Conventional
• Syndromic craniosynostosis
affecting the midface.
• Further indications
• The supraorbital rim and
forehead are in a
satisfactory position and it
is only the midface that
requires advancement
• The craniofacial growth is
complete (although some
proponents perform surgery
during the period of
growth)

• Advantage
maxillary zygomatic
complex resulting in total
midface advancement
• Disadvantage
procedure requiring
additional skills and
carrying more risks for
complications

• Cardinal features of the syndromic
cranial synostosis are:
• exorbitism
• Most of these patients have bicoronal
synostosis but multiple cranial
sutures in different combinations
may also be involved.
• Most children therefore undergo
frontal/superior orbital rim
advancement in infancy with repeat
advancement as needed.
• For the midface deformity, a Le Fort
III or monoblock osteotomy is
required.

• The "standard" Le Fort III osteotomy is performed when
the superior orbital rim and forehead are in a
satisfactory position and it is only the midface that
requires advancement.
• A monoblock osteotomy done transcranially is utilized
when the forehead, orbits, and midface all require a
similar magnitude of advancement.
• The Le Fort III osteotomy using conventional fixation
and bone grafts is usually performed after craniofacial
growth is complete, although some proponents do it
during the period of growth.
• More often the Le Fort III osteotomy is done via
distraction osteogenesis in the younger patients in order
to overcorrect the deformity, reduce complications,
obviate the need for bone grafts, and hopefully reduce
the total number of operations the child might need.
• The conventional monoblock osteotomy can also be
done as a single stage or via distraction in children up to
age 6-8 years. Single stage advancements are not often
done after this age due to the excessive risk of
complications (infections, CSF leak, etc)

• Preparation of occlusal
splint
• The patient is prepared
for surgery with
presurgical orthodontia
and fabrication of an
occlusal splint to establish
a Class I relationship or, if
overcorrection is desired,
the desired occlusal
relationship.

• Positioning and
intubation
• The patient is placed in
a supine position on a
well-padded headrest.
• Nasal intubation is
required as the patient
will be placed in
temporary MMF during
the procedure.

• Approach
• The osteotomy is approached
through a coronal incision with or
without an upper buccal sulcus
incision. Extensive dissection of
the orbit (deep orbit, anterior
and posterior to medial canthus
which remains attached),
zygoma, midface, and nose is
required.
• Some surgeons use a lower lid
incision as well, but if this can be
avoided fewer complications of
lid malposition will result.
Similarly the osteotomies can
usually be done without a buccal
sulcus incision.

Osteotomy
• The osteotomy begins
with a vertical cut (1)
using a saw at the
junction of the
zygomatic arch and
zygoma tic eminence.

• A second cut (2) is
made at the junction of
the inferior and lateral
orbital rims.

• A transverse osteotomy
(3) below the level of
the cribriform plate is
then completed.

• An osteotome is used to
perform osteotomies
across the orbital floor
anterior to the inferior
orbital fissure,
connecting to the
medial wall
osteotomies.

• The osteotome is inserted
through the transverse
nasal osteotomy in the
midline to complete an
osteotomy through the
nasal septum.
• It is safest to put a finger
in the mouth at the
posterior palate to assure
the osteotome does not
go too deep or penetrate
the mucosa.

• The final osteotomy through
the pterygomaxillary junction
is completed with an
osteotome from the
infratemporal fossa or
transorally if an upper buccal
sulcus incision has been made.
• To assure that the osteotome
does not damage vital
structures, one usually guides
the direction of the osteotome
with one hand while a
palpating finger is placed
medially at the posterior edge
of the palate.

• Disimpaction forceps
are then introduced and
the midface is down-
and out-fractured,
mobilizing it completely.

• Positioning with MMF
• The patient is then put
in MMF with the
occlusal splint.

• Fixation
• Once the desired
vertical dimension is
established, fixation
with titanium plates
and screws (1.5 or 2.0
mm) at the zygoma and
nasal frontal region
secures the
advancement.

• Grafting of bony gaps
• The bony gaps are then
filled with bone grafts
wedged into position
and secured to the
plate. Inlay bone grafts
may also be placed
along the orbital floor.

• CANTHOPEXY
• Most patients with
have canthal
malposition so a lateral
canthopexy in an
overcorrected superior
position is completed
prior to closure.

• If the advancement is
thought to be secure,
the MMF may be
removed, but most
surgeons prefer a
period of 3-6 weeks of
MMF.
•

Le Fort III - Distraction osteogenesis
• Syndromic craniosynostosis affecting
the anterior craniofacial skeleton.
• The forehead, superior orbital rims
and midface all require a similar
magnitude of advancement
• To overcorrect
• To reduce complications
• To obviate the need for bone grafts
• To reduce the total number of
operations the child might need

• Advantages
intracranial volume
reduce the relapse potential
• Disadvantages
• Need for absolute patient/parent
compliance with treatment (daily
• Need for secondary procedures
to adjust and remove distractor(s)

• Cardinal features of the Syndromic cranial synostosis
are:
• exorbitism
• Most of these patients have bicoronal synostosis but
multiple cranial sutures in different combinations
may also be involved.
• Most children therefore undergo frontal/superior
orbital rim advancement in infancy with repeat
advancement as needed.
• For the midface deformity, a Le Fort III or
monoblock osteotomy is required.
• The "standard" Le Fort III osteotomy is performed
when the superior orbital rim and forehead are in a
satisfactory position and it is only the midface that

• A monoblock
osteotomy done
transcranially is utilized
when the forehead
orbits and midface all
require a similar
magnitude of
advancement.

Le Fort III - Distraction osteogesis
• The Le Fort III
osteotomy using
conventional fixation
and bone grafts is
usually performed after
craniofacial growth is
complete, although
some proponents do it
growth.

• More often the Le Fort III
osteotomy is done via
in the younger patients in
order to overcorrect the
deformity, reduce
complications, obviate
the need for bone grafts,
and hopefully therefore
reduces the total number
of operations the child
might need.

• The conventional
monoblock osteotomy
can also be done as a
single stage.

• …or via distraction in
children up to age 6-8
years.
• Single stage
advancements are not
often done after this
age due to the excessive
risk of complications
(infections, CSF leak,
etc.).

• Choice of distraction device
• The decision to use either an
internal buried device or an
external halo should already have
been made after careful
discussion with the family.
• External halo distractors are
cumbersome, may be more easily
dislodged, may leave scars at the
temporal attachment, and may
penetrate the skull if there is a
direct force on the device.
However, they allow for multiple
vector distraction, and do not
require a large second operation
to remove.

• Internal distractors are
less cumbersome with
less chance for
dislodgement and injury
and are well suited to an
active lifestyle. However,
they are only single
vector devices, require
second operations to
remove, and have a
greater risk of infection.

• Positioning
For distraction, the
patient can be
intubated orally…

Nasal intubation
• …or nasally.

• Approach
required.
• Some surgons use a lower lid
sulcus incision.

• Osteotomies
• The osteotomy begins
with a vertical cut (1)
using a saw at the
junction of the
zygomatic arch and
zygomatic eminence.

• A second cut (2) is
made at the junction of
the inferior and lateral
orbital rims.

• A transverse osteotomy
(3) below the level of
the cribriform plate is
then completed.

perform osteotomies
orbital fissure,
connecting to the
medial wall
osteotomies.

• The osteotome is inserted
through the transverse
nasal osteotomy in the
midline to complete an
osteotomy through the
nasal septum.
• It is safest to put a finger
in the mouth at the
posterior palate to assure
the osteotome does not
go too deep or penetrate
the mucosa.

• The final osteotomy through
the pterygomaxillary junction
is completed with an
osteotome from the
infratemporal fossa or
transorally if an upper buccal
sulcus incision has been made.
does not damage vital
structures, one usually guides
the direction of the osteotome
with one hand while a
medially at the posterior edge
of the palate.

• Once the Le Fort III
segment is completely
mobilized it is returned
to its original position,
sometimes loosely held
here with resorbable
sutures. The distractor
is then applied.

Fixation to the midface
• If one is using a halo device, fixation to the
midface is gained through either:
• occlusal splint with outriggers
• direct screws to the maxilla either at:
- the pyriform aperture
- the zygomatic region
- both pyriform and zygomatic regions
• Direct screws to the maxilla require the
addition of an upper buccal sulcus incision.
• If attached to the teeth or midface, a heavy
bent rod exits the mouth and wraps around
in front of the lip and will be attached to the
halo. If the pyriform plates are used, the pins
exits transcutaneously near the alar rim on
the cheek or lip skin, or just inside the
nostril.
• Some surgeons also place an additional set of
pins in the zygoma which exit
transcutaneously.

• Fixation to the skull
• Following closure of the
wound of the coronal
incision, the halo device is
affixed to the skull
temporally by a series of
pins, usually 5 on each side.
It is positioned so that the
transverse bar is at the level
of the brow and the vertical
bar is in the midline. It
should be stable enough to
lift the patients head off the
table without shifting.

• Wires then attach the
screw arms of the
distractor to the
midface anchoring
component(s), and
should not be over-
tightened.

and out-fractured,

• Distraction is usually begun at postoperative
day 5 at a rate of 1 mm/day. As the midface
is brought forward the device may be
adjusted to change the occlusal cant vector
or pull more to the right or the left to
optimize the resulting occlusion.
• Children: In children in the middle years,
over-distraction to Class II relationship is
advised to accommodate future mandibular
growth.
• Adults: In adults, distraction is usually
performed to attain Class I relationship and is
aided by orthodontic elastics to guide the
maxilla into the proper occlusion with the
mandible (docking the occlusion).

• Removal of halo device
• Following the completion of
distraction, the halo device is
left in place for 1-3 months to
allow for consolidation of the
regenerated bone and is then
removed. If an occlusal device
is used this may be removed in
an office setting without
anesthesia. General
anaesthesia is usually
indicated when pins and plates
in the maxilla must be
removed.

• Fixation of the distractor
• If one has chosen an internal
distraction device, it is adapted to
the zygoma and the stable
temporal bone and affixed here
with screws (1.5-2.0 titanium).
The device is tested to be certain
that it is functional. As it is a
single vector device, one must be
certain that the desired position
fits the vector chosen. Generally
in the younger patients this is a
straight AP vector to avoid
vertical elongation of the orbit.
Although, an anterior open bite
may develop, this can be closed
later in life or may self-correct

out through the coronal
incision or through a
separate stab wound.
(See photo to the left).

• Distraction
• Distraction is usually begun at
postoperative day 5 at a rate
of 1 mm/day. In children in the
middle years, over distraction
to Class II occlusal relationship
is advised to accommodate
mandibular growth. In adults,
distraction is usually to attain a
Class I occlusal relationship.
•

• Following the completion of distraction, the device is
left in place for 1-3 months to allow for consolidation
of the regenerated bone, and is then removed. This
may require a complete re-opening of the coronal
incision and removing the device from both the
temporal region and the advanced midface. Some
devices do not rigidly affix to the midface but rather
are adapted without fixation to the posterior edge of
the zygoma and may be removed with less extensive
dissection.

have canthal malposition
so a lateral canthopexy in
an overcorrected superior
prior to closure. The
canthus is attached to the
stable lateral orbital rim
which will not be moved
by distraction.

Le Fort III Monoblock - Conventional
• Syndromic craniosynostosis affecting
the anterior craniofacial skeleton.
• The forehead, superior orbital rims
• The child is younger than 6 years old
(a high infection rate caused by
contamination from developing
sinuses is observed in children older
than 6 years)

• Advantage
maxillary zygomatic and
frontal bone complex resulting
in total midface advancement
• Disadvantages
procedure requiring additional
skills and carrying more risks
for complications
• Possible intracranial injury

• Cardinal features of the Syndromic cranial
synostosis are:
• exorbitism
• Most of these patients have bicoronal
synostosis but multiple cranial sutures in
different combinations may also be
involved.
• For the midface deformity, a Le Fort III or
monoblock osteotomy is required.
• A monoblock osteotomy done transcranially
is utilized when the forehead, orbits, and
midface all require a similar magnitude of
advancement.

• The "standard" Le Fort
III osteotomy is
performed when the
superior orbital rim and
forehead are in a
satisfactory position
and only the midface

• The Le Fort III
osteotomy using
and bone grafts is
complete, although
growth.

deformity, reduce
and hopefully reduces the
total number of
operations the child
might need.

monoblock osteotomy
single stage or…

• …via distraction in
years. Single stage
etc.).

• Preparation of occlusal
splint
• The patient is prepared
for surgery with
presurgical orthodontia
and fabrication of an
occlusal splint to establish
a Class I relationship or, if
overcorrection is desired,
the desired occlusal
relationship.

• Intubation
• Nasal intubation is
required as the patient
will be placed in
temporary MMF during
the procedure.

• Approach
required.
sulcus incision.

• Craniotomy
• After the exposure of the
forehead and the orbits, a
bifrontal craniotomy beginning 1
cm above the superior orbital rim
and extending to behind the
coronal sutures is outlined.
• The neurosurgeon then
completes the osteotomies using
a craniotome.

and middle fossae in
the epidural plane.
with neurosurgical
cottonoids.

contents when
performing the
osteotomies.

• Vertical osteotomies are
made just behind the
lateral orbital rims and
down through the
lateral orbital wall with
a saw.

• A vertical osteotomy
using a saw at the
junction of the
zygomatic arch and
zygomatic body is
completed.

retracted, a right angle
saw is turned
intracranially and the
orbital roof is
osteotomized beginning
at the lateral wall
osteotomy and ending
at the cribriform plate.

• An osteotome is
inserted from
medial orbit is
osteotomized behind to
the canthus and on to
the medial floor.

perform osteotomies
orbital fissure,
connecting to the
medial wall
osteotomies.

• Separation of pterygomaxillary
junction
• The osteotomy through the
pterygomaxillary junction is
completed with a curved
osteotome from the
infratemporal fossa or transorally
if an upper buccal sulcus incision
has been made.
does not hit vital structures, one
usually guides the direction of the
osteotome with one hand while a
medially at the posterior edge of
the palate.

• Cut of the nasal septum
from intracranially and goes
from the cribriform plate
through the posterior septum
to the level of the palate.
• It is safest to place a finger in
the mouth at the posterior
palate to assure the
osteotome does not go too
deep or penetrate the mucosa.

• DISIMPACTION
and out-fractured,

• Positioning of midface
with MMF
• The patient is then
placed in MMF using
the occlusal splint and
stainless steel wire
loops.

• Fixation of the midface
• Once the desired
vertical dimension is
established, fixation
with titanium plates
and screws (1.5 or 2.0
mm) at the zygoma and
temporal region secures
the advancement.

• If the monoblock procedure is
being performed in a child
above the age of 6-8 years as a
single stage, there is a high risk
of CSF leak and/or infection.
Therefore the anterior cranial
base in the midline is usually
filled with a galea frontalis flap
and tissue sealant (eg,
Fibrinogen etc.).
• This may require cutting a
small notch out of the frontal
bone to prevent constriction
of the flap.

• Grafting of bony gaps
• The bony gaps are then
filled with bone grafts
wedged into position
and secured to the
plate. Inlay bone grafts
may also be placed
along the orbital floor.

• Fixation of frontal bone
flap
• The frontal bone flap is
then replaced and
affixed to the
supraorbital rim with
titanium plates and
screws.

• Grafting of coronal bone
gap
• The coronal bone gap
created from the
advancement is then filled
with particulate bone
shavings harvested with a
manual hand-held burr-hole
instrument or commercial
harvester (eg. Safe scraper)
or the posterior skull.

• Positioning of midface
with MMF
have canthal
canthopexy in an
prior to closure.

Removal of MMF
If the advancement is
thought to be secure,
the MMF may be
removed, but most
surgeons prefer a
period of 3-6 weeks of
MMF.

Le Fort III Monoblock - Distraction osteogenesis
• Syndromic craniosynostosis affecting the
anterior craniofacial skeleton.
• When the forehead, superior orbital rims
• To overcorrect
• To reduce complications
• To obviate the need for bone grafts
• To reduce the total number of
operations the child might need

Le Fort III Monoblock - Distraction
osteogenesis
• Advantages
intracranial volume
reduce the relapse potential
• Disadvantages
• Need for absolute patient/parent
compliance with treatment (daily
• Need for secondary procedures
to adjust and remove distractor(s)

• The "standard" Le Fort
III osteotomy is
performed when the
superior orbital rim and
forehead are in a
satisfactory position
and only the midface

deformity, reduce
reduce the total number
might need.

osteogenesis
• The Le Fort III
osteotomy using
and bone grafts is
complete, although
growth.

osteogenesis
deformity, reduce
reduce the total number
might need.

monoblock osteotomy
single stage or…

• …via distraction in
years. Single stage
etc.).

• Choice of distraction device
• The decision to use either an internal
buried device or an external halo
should already have been made after
careful discussion with the family.
• External halo distractors are
cumbersome and may be more easily
dislodged, may leave scars at the
temporal attachment and may
penetrate the skull if there is direct
force on the device. However, they
allow for multiple vector distraction,
and do not require a large second
operation to remove.

• Internal distractors are
less cumbersome with
less chance for
dislodgement and injury
and are well suited to an
active lifestyle. However,
they are only single
vector devices, require
second operations to
remove and have a
greater risk of infection.

osteogenesis
• Positioning

osteogenesis
incision. Extensive dissection of the
orbit (deep orbit, anterior and
posterior to medial canthus which
remains attached), zygoma, midface,
and nose is required.
avoided fewer complications of lid
malposition will result. Similarly the
osteotomies can usually be done
without a buccal sulcus incision.

osteogenesis
• Craniotomy
• After the exposure of the forehead
and the orbits, a bifrontal craniotomy
beginning 1 cm superior to the
superior orbital rim and extending to
behind the coronal sutures is
outlined.
• The neurosurgeon then completes
the osteotomies using a craniotome.

and middle fossae in an
epidural plane.
with neurosurgical
cottonoids.

contents when
performing the
osteotomies.

• Vertical osteotomies are
made just behind the
lateral orbital rims and
down through the
lateral orbital wall.

• A vertical osteotomy
using a saw at the
junction of the
zygomatic arch and
zygomatic body is
completed.

retracted, a right angle
saw is turned
orbital roof is
osteotomized beginning
at the lateral wall
osteotomy and ending
at the cribriform plate.

osteogenesis
• An osteotome is
inserted from
medial orbit is
osteotomized behind
the canthus and on to
the medial floor.

osteogenesis
perform osteotomies
orbital fissure,
connecting to the
medial wall
osteotomies.

osteogenesis
• Separation of pterygomaxillary
junction
• The osteotomy through the
pterygomaxillary junction is
completed with a curved osteotome
from the infratemporal fossa or
transorally if an upper buccal sulcus
incision has been made.
• To assure that the osteotome does
not hit vital structures, one usually
guides the direction of the
osteotome with one hand while a
palpating finger is placed medially at
the posterior edge of the palate.

• Cut of the nasal septum
from intracranially and goes
from the cribriform plate
through the posterior septum
to the level of the palate.
• It is safest to place a finger in
the mouth at the posterior
palate to assure the
osteotome does not go too
deep or penetrate the mucosa.

Disimpaction
• Disimpaction forceps are then
introduced and the monoblock
segment is down and out fractured
• In monoblock distraction, new bone
forms in the cranial base and at the
osteotomy sites as the face is brought
slowly forward. Unlike a single stage
monoblock procedure, where one
leaves a large connection between
the anterior cranial base and the
sinuses which requires a galea flap
and sealants, no special treatment of
this area is required.

Distraction (Halo device)
• Fixation to the midface
• If one is using a halo device, fixation to the
midface is gained through either:
• occlusal splint with outriggers
• direct screws to the maxilla either at:
- the pyriform aperture
- the zygomatic region
- both pyriform and zygomatic regions
• Direct screws to the maxilla require the
addition of an upper buccal sulcus
incision.
• If attached to the teeth or zygoma, a
heavy bent rod exits the mouth and wraps
around in front of the lip and will be
attached the halo at this point. If the
pyriform plates are used the pins exits
transcutaneously near the alar rim, on the
cheek or lip skin, or just outside the
nostril.

• A second point of
fixation is made at the
superior orbit. Fixation
screws with pins that
exit the skin are
applied.

osteogenesis
• Replacement of frontal
bone flap
replaced and affixed to
the monoblock segment
with titanium plates.

osteogenesis
• Fixation of distraction device
to the skull
• Following closure of the
wound of the coronal incision,
the halo device is affixed to
the skull temporally by a series
of pins, usually 5 on each side.
It is positioned so that the
transverse bar is at the level of
the brow and the vertical bar
is in the midline. It should be
stable enough to lift the
patients head off the table
without shifting.

osteogenesis
• DISTRACTION
• Wires then attach the
screw arms of the
distractor to the
midface anchoring
component(s). They
should not be over
tightened.

osteogenesis
• Distraction
• Distraction is usually begun at
postoperative day 5 at a rate of 1
mm/day. As the midface is brought
forward the device may be adjusted
to change the occlusal cant vector or
pull more to the right or the left to
optimize the resulting occlusion.
• Children
In children in the middle years, over
distraction to Class II relationship is
advised to accommodate future
mandibular growth.
• Adults
In adults, distraction is usually
performed to attain Class I
relationship and is aided by
orthodontic elastics to guide the
maxilla into the proper occlusion with
the mandible (docking the occlusion).

osteogenesis
• Removal of distraction device
•
Following the completion of distraction, the halo
device is left in place for 1-3 months to allow for
consolidation of the regenerated bone and is then
removed. If an occlusal device is used this may be
removed in an office setting without anesthesia.
General anaesthesia is usually indicated when pins and
plates in the maxilla must be removed. The pins in the
superior orbit are usually just removed percutaneously
and the plates left behind.

osteogenesis
• Fixation of the distractor
• If one has chosen an internal
distraction device, it is adapted to the
zygoma and the stable temporal
bone and affixed here with screws
(1.5-2.0 titanium). The device is
tested to be certain that it is
functional. As it is a single vector
device, one must be certain that the
desired position fits the vector
chosen. Generally in the younger
patients this is a straight AP vector to
avoid vertical elongation of the orbit.
Although, an anterior open bite may
develop this can be closed later in life
or may self-correct.

osteogenesis
out through the coronal
incision or through a
separate stab wound.

osteogenesis
• Replacement of frontal
bone flap
replaced and affixed to
the monoblock segment
with titanium plates.
(See illustration to the
left).

osteogenesis
• Distraction
• Distraction is usually begun
at postoperative day 5 at a
rate of 1 mm/day. In
children in the middle years,
over distraction to Class II
occlusal relationship is
advised to accommodate
mandibular growth. In
adults, distraction is usually
to attain a Class I occlusal
relationship.

osteogenesis
• Following the completion of distraction, the device is
left in place for 1-3 months to allow for consolidation
of the regenerate bone and is then removed. This may
require a complete re-opening of the coronal incision
and removing the device from both the temporal
region and the advanced midface. Some devices do not
rigidly affix to the midface but rather are adapted
without fixation to the posterior edge of the zygoma
and may be removed with less extensive dissection.

Canthopexy
have canthal
canthopexy in an
prior to closure.

Syndromic cranial synostosis

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