Craniosynostosis

Craniosynostosis
DR JAMEEL KIFAYATULLAH
LECTURER

CRANIOSYNOSTOSIS
• Craniosynostosis is the
premature closure of
one or more cranial
vault or cranial base
sutures.

Effects/results
• The prematurely closed suture imposes
restrictions to the growth of underlying brain,
resulting in compensatory growth and
expansion of less restricted areas.

OCCURENCE
• Craniosynostosis has been estimated to occur
in 1:2000 to 1:2500 births.

Distribution
The distribution between the different synostosis are as
follows:
• Sagittal synostosis :40 – 55 %
• Coronal synostosis: 20 – 25 %
• Metopic synostosis :5 - 15 %
• Lambdoid synostosis: 0 – 5 %
• Multiple suture synostosis 5 – 15 %, mainly as a part of a
syndrome.

Causes for premature fusion of cranial
sutures
Factors associated with nonsyndromic premature fusion of
cranial sutures are as follows:
• Environmental factors
• Multiple gestation (eg. twins, triplets)
• Large infant size
• Abnormal infant position
• Uterine abnormalities
• Head constraint
• Drugs (eg. Nitrofurantoin, Warfarin,)
• Endocrine abnormalities

Functional issues
• During the first year of life the brain will triple in
size, followed by slower growth until it reaches its
full size in the teen age years. Restrictions of this
growth may have functional consequences. The
most typical ones are listed below.
• Elevated intracranial pressure (>15-17mm Hg)
occurs in approximately 47% of patients with
multiple, and in 14% of patients with single
suture fusion

Functional issues
• Blindness: optic nerve atrophy, corneal exposure
• Hydrocephalus (mainly for Syndromic patients)
• Developmental delay
• Abnormalities of speech and hearing
• Abnormalities of the ocular axis and adnexa
• Abnormalities of the airway
• Malocclusion (Angle class III)

Functional issues
• The x-ray on the left
show the appearance of
the "Beaten Copper
Cranium" pattern found
in a patient with
elevated intracranial
pressure

Functional issues
• The appearance of the
"Beaten Copper
Cranium" pattern in a
CT-scan of a patient
with a clover leaf skull
deformity.

Patient examination
The diagnosis of craniosynostosis is made by physical examination and
radiographic analysis.
History of risk factors during pregnancy
• Multiple gestation (eg. twins, triplets)
• Large infant size
• Abnormal infant position
• Uterine abnormalities
• Head constraint
• Maternal smoking
• Caucasian maternal race
• Advanced maternal age
• High altitude
• Maternal use of nitrofurantoin
• Paternal occupation (agriculture, forestry)
• Fertility treatments
• Endocrine abnormalities
• Warfarin ingestion

Family history
• A positive family history may be found in up to:
• 2 % of patients with nonsyndromic sagittal suture
closure
• 10 % of patients with nonsyndromic coronal
suture closure
• 10 % of patients with nonsyndromic metopic
suture closure
• 50 % of patients with a syndromic
craniosynostosis

Elevated ICP
• Elevated ICP occurs in approximately 47% of patients
with multiple, and in 14% of patients with single suture
fusion.
• ICP can be recognized clinically by the finding of
papilledema on fundoscopic examination and, in later
stages, "thumb printing" or the "beaten copper"
appearance on plain radiographs of the skull.
• Headaches, irritability, developmental delays, and
sophisticated eye evaluation (visual evoked potentials,
colour analysis) may suggest increased ICP.

Respiratory problems
• Patients with significant midface retrusion
should be evaluated for the presence of sleep
apnea and airway compromise. Syndromic
patients may also have intrinsic airway
anomalies such as tracheal sleeve and
laryngomalacia.

Physical examination
Physical examination
Physical examination should include evluation of:
• suture ridging
• sutural patency by manual palpation
• skull and facial configuration
• fullness and patency of both the anterior and posterior fontanels
• the presence of exophthalmos or orbital dystopia
• eyelid ptosis and strabismus
• occlusal relationships and dental development
• the presence of diplopia and papilledema
• the intracranial pressure

Radiographic assessment
• Although plain film radiographs
(anteroposterior and lateral skull) may be
useful, the gold standard radiographic method
is 2D- and 3D-CT-scans

Sagittal synostosis (Scaphocephaly)
• Premature closure of the sagittal
suture leads to scaphocephaly
which has the following
characteristics:
• Elongated skull in the sagittal plane
• Frontal and occipital prominence
• Bi-parietal and bi-temporal
narrowing
• Palpable sagittal ridge
•
Variations in the deformity may
exist, likely depending upon
whether the closure started
posteriorly or anteriorly and at
what age. Consequently the occiput
may be more affected than the
frontal region, or vice versa.

Sagittal synostosis
Posterior reshaping
• Main indications
Limited posterior skull deformity.
• Further indication
• When both the anterior and the posterior
skull need reshaping, this can be done as a
single stage or two separate stages
• Advantage
• Single stage restoration of cranial form
and increase cranial volume
• Disadvantage
• Risk of intracranial injury and significant
blood loss

Sagittal synostosis
• Anterior reshaping with widening
Main indications
• Anterior skull deformity with
narrowing.
• When both the anterior and the
posterior skull need reshaping, this
can be done as a single stage or two
separate stages
• Advantage
• Single stage restoration of cranial
form and increase cranial volume
• Disadvantages
• Risk of intracranial and ocular injury
• Risk of nerve injury (ptosis,
strabismus)

Early surgical treatment
• Sagittal craniosynostosis detected
early. This is a minimally invasive
treatment of sagittal synostosis.
• Note: If the diagnosis is made
within the first weeks or first
months of life, less invasive
methods of treatment are gaining
popularity. These include
endoscopically assisted strip
craniectomies with molding
helmets, and spring assisted
craniectomies. These are best
performed by 6-12 weeks of age
for the endoscopic approach and
3-6 months of age when springs
are utilized.
•

Early Surgical treatment
• Advantage
• Minimally invasive procedure with less morbidity
and shorter hospitalization
• Disadvantage
• May not correct the deformity as well as an open
approach

Total vault reshaping
• Significant skull deformities, with or
without increased intracranial
pressure.
• When both the anterior and the
posterior skull need reshaping, this
can be done as a single stage or two
separate stages.
• Advantage
• Single stage correction of a combined
anterior posterior skull deformity
• Disadvantages
• Intracranial injury, blood loss
• Patient positioning makes it difficult
to address the most anterior and most
posterior aspects simultaneously

Total cranial vault reshaping in sagittal
synostosis
• Premature closure of the sagittal suture results in
scaphocephaly (dolichocephaly) or a boat shaped
head. There may be a great deal of variability in
the head shape, depending upon whether the
closure started posteriorly or anteriorly and at
what age. Consequently the occiput may be more
affected than the frontal region, or vice versa.
Commonly both are affected.
• The general goal of surgery is to shorten the skull
in a sagittal plane and widen it in a coronal plane.

Total Vault Reshaping
• When the occipital region is significantly affected
and the anterior is not, posterior vault reshaping
alone may be all that is required.
• Similarly if the deformity is mostly frontal,
correction may be performed here only.
• Most often both the anterior and the posterior
skull need reshaping, and this can be done as a
single stage or two separate stages.

Total Vault Reshaping
• Single stage total calvarial reshaping is usually
confined to younger patients with less severe
deformities.
• When the deformity is more severe and one
has to access the orbits anteriorly or the base
of the occiput posteriorly, staged procedures
are preferred.

Positioning and approach
• With the patient in the
sphinx or "sniffing"
position anterior and
posterior exposure of
the total calvarial vault
is made via a coronal
incision.

Total Cranial Vault Reshaping
• Osteotomies
• After dissecting as far posteriorly
as possible and into the superior
orbits anteriorly, the skull is
systematically disassembled
through a series of craniotomies.
• Barrel-stave or similar
osteotomies are performed
laterally and the bone flaps are
bent shaped and replaced to give
shortening in a sagittal plane and
widening in the coronal plane.
The supraorbital bandeau might
also be widened.

Bilateral coronal synostosis (Brachycephaly)
• Premature closure of both coronal
sutures leads to brachycephaly
characteristics:
• Shortening of the head in the
anterior-posterior dimension.
• Transverse widening of the head
• Vertical elongation of the head
• Shortening of the anterior cranial
base
• Retrusive orbital rims
• Protuberance of the superior frontal
and squamous temporal bones
• Occiput usually flattened

Bilateral coronal synostosis
• Bilateral orbital advancement
Main indications
• Cranial vault deformity, increased
intracranial pressure, or eye
protection.
• Further indications
• Appearance abnormalities (these
abnormalities will typically
worsen as the child grows older)
• Increased intracranial pressure
caused by restricted growth of
the brain
• Eye protection
•

Bilateral orbital advancement for
Cranial Synostosis
• Advantage
• Single stage restoration of cranial form and
increase cranial volume
• Disadvantages
• Risk of nerve injury (ptosis, strabismus)

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

Bilateral Orbital Advancement
• Approach
• For this procedure
the coronal approach is
used.

• Osteotomy
• 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
between these points is made.
• 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.

• Positioning and Fixation
• 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)

Bilateral orbital advncement
• 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)
from the frontal bone flap
or the posterior skull.

Positioning and Fixation
• 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 instrument.

Aftercare following transcranial and Le Fort III
procedures in infants and children
• Intraoperatively
• Most surgeons favors placement of a bulb suction drain under the scalp
for 3-5 days. Resorbable skin sutures are often used.
Postoperatively
• A circumferential head dressing is utilized for 48 hours. The neurosurgeon
may request placement of a lumbar drain if significant dural tears have
occurred during surgery. Patients should spend at least 1-2 days in an
intensive care unit for neurological monitoring.
• Postoperative positioning
• Keeping the patient’s head in an upright position postoperatively may
significantly improve periorbital edema and pain. Some surgeons use
injectable corticosteroids during surgery to reduce periorbital swelling.

• Nose-blowing
• To prevent orbital emphysema, nose-blowing
should be avoided for at least 10 days.

Medication
• The following perioperative medications are controversial. There is little evidence
to make strong recommendations for postoperative care.
• Avoidance of aspirin or nonsteroidal antiinflammatory drugs (NSAIDs) for 7 days.
• Analgesia as necessary.
• Antibiotics (many surgeons use perioperative antibiotics. There is no clear
advantage of any one antibiotic, and the recommended duration of treatment is
debatable.).
• Nasal decongestant may be helpful for symptomatic improvement in some
patients.
• Steroids may help with postoperative edema. Some surgeons have noted increased
complications with perioperative steroids.
• Ophthalmic ointment should follow local and approved hospital protocol. This is
not generally required in case of periorbital edema. Some surgeons prefer it. Some
ointments have been found to cause significant conjunctival irritation.
• Regular perioral and oral wound care has to include disinfectant mouth rinse, lip
care if intraoral incision has been used.

Aftercare
• Ophthalmological examination
• Postoperative examination by an ophthalmologist may be requested,
although severe periorbital edema may prevent useful assessment. The
following signs and symptoms are usually evaluated:
• Vision
• Extraocular motion
• Diplopia
• Globe position
• Lid position

Aftercare
• Postoperative imaging
Postoperative imaging has to be performed
within the first days after surgery to verify
accuracy of surgery. 3-D imaging (CT, cone
beam) is recommended.

Aftercare
Wound care
Remove sutures from skin after approximately 7-
10 days if nonresorbable sutures have been used.
Apply ice packs for the first 12 postoperative
hours as able although infants and young children
do not tolerate this well (may be effective in a
short term to minimize edema).
Avoid sun exposure and tanning to skin incisions
for several months.

Aftercare
• Soft diet can be taken as tolerated until there
has been adequate healing of any maxillary
vestibular incision. In children and infants age
appropriate diets are then prescribed.
• Patients in MMF will remain on a liquid diet
until such time the MMF is released.

Aftercare
• Clinical follow-up
• Clinical follow-up depends on the complexity of the
surgery, and whether the patient has any postoperative
problems. Most patients are discharged at postoperative
day 3-5 and seen again in 2-3 weeks.
• In patients undergoing monoblock or Le Fort III distraction,
distraction typically begins at day five at 1 mm/day and is
assessed weekly with plane radiographs and clinical
examination until the desired position is reached. After
advancement a period of consolidation of 1-3 months is
recommended before the retractors are removed.

Aftercare
Clinical Follow up
• In patients undergoing conventional advancement with
intermaxillary fixation, MMF is kept in place for 4-6 weeks.
Routine oral hygiene is prescribed. Patients with arch bars
and/or intraoral incisions and/or wounds must be
instructed in appropriate oral hygiene procedures. The
presence of the arch bars or elastics makes this a more
difficult procedure. A soft toothbrush (dipped in warm
water to make it softer) should be used to clean the
surfaces of the teeth and arch bars. Elastics are removed
for oral hygiene procedures. Chlorhexidine oral rinses
should be prescribed and used at least 3 times a day to
help sanitize the mouth.

After care
Clinical Follow up
• For larger debris, a 1:1 mixture of hydrogen
peroxide/chlorhexidine can be used. The
bubbling action of the hydrogen peroxide helps
remove debris. A Waterpik® is a very useful tool
to help remove debris from the wires. If a
Waterpik is used, care should be taken not to
direct the jet stream directly over intraoral
incisions as this may lead to wound dehiscence.

Aftercare
• Follow-up
• The patient needs to be examined and reassessed
regularly and often. Additionally, ophthalmological,
ENT, and neurological/neurosurgical examination may
be necessary. If any clinical signs for meningitis or
mental disturbances develop, professional help has to
be sought. Due to the young age of many patients,
routing CT-scans are performed only if clinically
indicated to avoid excessive radiation exposure.

Unilateral coronal synostosis (Anterior
plagiocephaly)
• Premature closure of the coronal
suture leads to anterior
plagiocephaly which has the
following characteristics:
Ipsilateral
• Retruded forehead
• Retruded supraorbital rim
• Elevated brow
• Widened palpebral fissure
• Radix deviates to affected side
Contralateral
• forehead bossing

Unilateral coronal synostosis
• The standard treatment is either bilateral or
unilateral orbital advancement, and bone
reshaping within the first year of life.
• The unilateral advancement is used less
frequently and mainly when the deformity is
less severe and does not extend across the
midline.

Unilateral Coronal synostosis
• Premature closure of the coronal
suture leads to anterior
plagiocephaly(oblique head)
characteristics:
Ipsilateral
• Retruded forehead
• Retruded supraorbital rim
• Elevated brow
• Widened palpebral fissure
• Radix deviates to affected side
Contralateral
• forehead bossing

Unilateral coronal synostosis
• Unilateral orbital advancement
protection.
abnormalities will typically worsen as
the child grows older)
caused by restricted growth of the
brain
• Eye protection
• Advantage
• Disadvantages
strabismus)

Unilateral orbital advancement for
unilateral coronal synostosis
• The standard treatment is
either bilateral or
unilateral orbital rim
advancement, and bone
reshaping within the first
year of life. The unilateral
advancement is used less
frequently and mainly
when the deformity is less
severe and not extending
across the midline.

Unilateral orbital advancement
• Approach
used.

Unilateral Orbital advancement
• Craniotomy
• After the exposure of the forehead
and the orbit via a coronal approach,
a craniotomy beginning 1 cm superior
to the superior orbital rim at the
midline and extending to behind the
coronal suture is outlined.
vertex, avoiding the sagittal sinus,
a craniotome.

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

UNILATERAL ORBITAL ADVANCEMENT
osteotomy
dura and intraorbitally to
protect the orbital
contents when
performing the
osteotomies.

osteotomized.
the pterion (1) on the
affected side 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 vertical
osteotomy is made medially
near the midline (4).

Orbital advancement
• Osteotomy
in-groove or step
osteotomy can be used
in the temporal region
as shown.

Unilateral Orbital Advancement
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 region medially,
joining the osteotomy
made extra-cranially.

supraorbital bar
wall osteotomy
completed, releasing
the bar.

• Reshaping of the supraorbital
bar
• The bandeau will typically
need to be reshaped by
making a closing wedge
ostectomy either in the middle
segment of the superior
orbital rim or at the junction
of the superior orbital rim and
the temporal bone, or both.
The sites of the osteotomies
are chosen in order to get
symmetric reshaping of the
supraorbital bar.

• These are stabilized
with sutures, wires, or
resorbable plates
(preferably), which can
be placed intracranially
or extra-cranially. In
adults extracranial
titanium fixation is an
alternative.

• Positioning of the supraorbital
bar
advanced 10-15 mm, hinging at
the midline. In infants an
overcorrected advancement is
usually made, as there will be
some recurrence of the deformity
with growth.
craniofacial growth, advancement
is done so that the superior
orbital rim is approximately 12
mm anterior to the cornea, and
equal or symmetric with the
uninvolved opposite side.
• The key is to try to straighten the bandeau
so that both sides are equal and
symmetric, employing osteotomies and
fixation wherever required to make this
possible.

• Fixation of the supraorbital
bar
• The advanced bar is then
stabilized with a wire or
suture placed between the
stable lateral orbital rim and
the bandeau.
• The bar is further stabilized
with a resorbable plate,
wire or suture at the
midline and in the temporal
region.

stability of the
advanced segment, a
cranial bone graft may
also be wedged and
placed in the orbital
segment.

bone gap
is then inserted in the
temporal gap and held in
position with a resorbable
plate (infants and
children) or metallic plate
(adults).

• Self retaining
osteotomies
plate fixation is
unavailable, self-
may be designed and
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 before replacement
using:
• A bone bending forceps
(children).
• Partial osteotomies and plate
fixation (adults).

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

• Grafting of coronal bone
gap
• The coronal bone gap
created from the
advancement and
or the posterior skull.

• This picture
bone that can be
harvested from the
burr-hole instrument

Main indications
protection.
Further indications
abnormalities will typically
worsen as the child grows older)
caused by restricted growth of
the brain
• Eye protection
• Advantage
• Disadvantages
• Risk of intracranial and ocular
injury
strabismus)
•

either bilateral or unilateral
supraorbital bar
advancement and reshaping
within the first year of life.
The bilateral advancement
is used more frequently and
is the procedure of choice
when the deformity is more
severe, when it extends
across the midline, and
there is contralateral frontal
bossing.

Approach
used.

• Craniotomy
• After the exposure of the forehead
and the orbits, a bifrontal
craniotomy is outlined. It starts 1
cm above the superior orbital rim
and extends to behind the coronal
suture on the affected side and to
just behind the lateral orbital rim
on the contralateral side (insert)
vertex avoiding the sagittal sinus
• The neurosurgeon then completes
the osteotomies using a
craniotome.

the epidural plane.
with neurosurgical
cottonoids.

osteotomy
protect the orbital
contents when
performing the
osteotomies.

osteotomized.
• A vertical osteotomy near the
pterion (1) on the affected
side 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).

Osteotomy
in-groove or step
osteotomy can be used
in the temporal region
of the affected side as
shown.

BILATERAL ORBITAL ADVANCEMENT
• OSTEOTOMY
• On the non-affected
side, the osteotomy
typically begins just
behind the lateral
orbital rim as a vertical
osteotomy and is made
similarly through the
rim.

• With the brain carefully retracted,
a right angle saw is then turned
intracranially and the orbital roof
is osteotomized beginning at
pterion laterally on the affected
side and ending at the nasal
frontal region medially, joining
cranially across the nasal frontal
region. On the non-affected side
the orbital roof osteotomy begins
anterior to the pterion and ends
medially as above.

BILATERAL ORBITAL OSTEOTOMY
• With the brain carefully retracted,
a right angle saw is then turned
intracranially and the orbital roof
is osteotomized beginning at
pterion laterally on the affected
side and ending at the nasal
frontal region medially, joining
cranially across the nasal frontal
region. On the non-affected side
the orbital roof osteotomy begins
anterior to the pterion and ends
medially as above.

bandeau
wall osteotomy
completed on both
sides, releasing the
bandeau.

• 4. Reshaping of the bandeau
• The bandeau will typically
need to be reshaped on the
affected side by making a
closing wedge ostectomy
either in the middle segment
of the superior orbital rim or
at the junction of the superior
orbital rim and the temporal
bone, or both. The sites of the
osteotomies are chosen in
order to get symmetric
reshaping of the bandeau.

• These are stabilized
with sutures, wires, or
resorbable plates
(preferably), which can
be placed intracranially
or extra cranially. In
adults titanium fixation
is an alternative.

• Positioning of the supraorbital
bar
advanced 10-15 mm on the side
of the fusion, hinging at the point
just behind the orbital rim on the
uninvolved side. In infants an
overcorrected advancement is
usually made, as there will be
some recurrence of the deformity
with growth.
craniofacial growth, advancement
is done so that the superior
orbital rim is approximately 12
mm anterior to the cornea, and
equal or symmetric with the
uninvolved opposite side.
• The key is to try to straighten the bandeau
so that both sides are equal and
symmetric, employing osteotomies and
fixation wherever required to make this
possible.

• Fixation of the
supraorbital bar
• The advanced bar is
or suture is placed
between the stable
the bandeau.

stability of the
advanced segment, a
also be wedged and
segment on the affected
side.

bone gap
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).

plate fixation is
unavailable, self-
may be designed and
with wire or suture
fixation.

• Replacement and fixation of the
frontal bone flap
• The frontal bone flap is then typically
split down the midline and reshaped
with a bone bending forceps
(children) or with a partial
osteotomies and plate fixation
(adults).
• Usually the right and left flaps are
rotated, bent contoured and
switched to get the optimal
symmetry.
• The bone flaps are 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
from the frontal bone flap or
the posterior skull.

• This picture
bone that can be
harvested from the
burr-hole instrument.

Metopic synostosis (Trigonocephaly)
• Premature closure of the
metopic suture leads to
trigonocephaly which has the
following characteristics:
• Keel-shaped forehead (best
observed from a coronal view)
• Bi-temporal narrowing
• Hypotelorism
• Palpable midline frontal ridge

Bilateral orbital advancement and
expansion(metopic synostosis)
• Cranial vault deformity,
increased intracranial
pressure, or eye protection.
Advantage
• Single stage restoration of
cranial form and increase
cranial volume
• Disadvantages
• Risk of intracranial and
ocular injury
strabismus)
•

expansion
• In metopic synostosis or
trigonocephaly there is a
prominent keel in the midline
with retrusion of the lateral
orbit and bitemporal
narrowing.
completed within the first year
of life and involves not only
bilateral advancement of the
lateral orbit but transverse
expansion of the supraorbital
bar as well. The frontal bone
needs reshaping to match the
reconstructed bandeau.

expansion
used.

expansion(Metopic synostosis)
• 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.
vertex avoiding the sagittal sinus
a craniotome.

the epidural plane.
with neurosurgical
cottonoids.

• Orbital osteotomy
protect the orbital
contents when
performing the
osteotomies.

• The supraorbital bar is
then osteotomized.
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).

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
cranially across the nasal
frontal region.

supraorbital bar
wall osteotomy
completed on both sides,
releasing the supraorbital
bar.

• Reshaping
• The bar is cut in the midline
and a cranial bone graft
taken from the frontal bone
flap is inserted between the
two hemi-orbital segments.
This bone graft varies from
10-20 mm depending on
the severity of the
deformity. It is held in place
with a resorbable plate.

• The construct is
frequently not stable
enough and often a
larger bone graft is
placed on the
endocranial surface
spanning the central
bone graft and affixed
with wires or sutures.

Bilateral Orbital advancement
• Closing wedge
osteotomies are then
made at the lateral
orbit endo cranially and
the bar is reshaped. The
new shape is
maintained with
resorbable plates
placed intra- or extra-
cranially.

Bilateral Orbital advancement
• Positioning
• The reshaped supraorbital bar
is then replaced in the now
transversely expanded and
laterally advanced position.
• Frequently there is no bony
gap temporally but if the orbit
needs more lateral
advancement, a cranial bone
graft is placed in the temporal
gap and stabilized with a
resorbable plate (insert).

• Fixation
• The advanced bar is
or suture is placed
between the stable
the bar.

stability of the
advanced segment, a
also be wedged and
segment.

• Reshaping
• The frontal bone flap is
then cut in two in the
midline. The segments
are forcefully bent with
bone bending forceps
to make them malleable
and shapeable.

• Replacement and fixation
• The two frontal bone pieces
are then replaced with an
inter-positional bone graft
harvested from the posterior
edge of each flap. The bone
flaps are affixed to the
resorbable sutures or
resorbable plates which are
also used to span the bone
graft and hold it in position
between the right and left
frontal bone segments.

• Alternatively, the two
bone flaps may be
joined in the midline
without an interposed
graft if they match well
to the supraorbital bar.

• Grafting of coronal bone gap
• The coronal bone gap created
from the advancement and
from the frontal bone flap or
the posterior skull.

• This picture
bone that can be
harvested from the
burr-hole instrument

Unilateral lambdoid synostosis (Plagiocephaly)
• Premature closure of the
lambdoid suture leads to
posterior plagiocephaly
characteristics:
• Ipsilateral occipital flatness
• Ipsilateral mastoid bulge
• Posterior/inferior
displacement of the ear
ipsilateral to the suture
• Variable ipsilateral frontal
bone retrusion

Unilateral Lambdoid synostosis
• Posterior vault reshaping
• Cranial vault deformity and increased
intracranial pressure.
abnormalities will typically worsen as
the child grows older)
• Increased intracranial pressure caused
by restricted growth of the brain
• Advantage
• Disadvantage
• Risk of intracranial injury and
significant blood loss

Posterior vault reshaping
• In lambdoid synostosis
there is ipsilateral occipital
flattening with a
compensatory mastoid
bulge, and compensatory
vault expansion on the
opposite side.
• The goal of surgery is to
release the fused suture,
and make the cranial base
symmetric. The mastoid
bulge cannot be corrected
due to its low position.

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

• OSTEOTOMIES
• After exposure of the posterior
cranial vault, a craniotomy of the
occiput and the parietal regions is
outlined.
• Burr holes are first placed at
periphery of the flap. An epidural
dissection between these points
is made.
a craniotome.

• Reshaping of the basal
occipital bone
and middle fossae in the
epidural plane.
with neurosurgical
cottonoids.

used to retract and protect
the dura when performing
the osteotomies.
• On the involved side barrel-
stave osteotomies with out-
fracturing are made. The
newly positioned barrel-
staves may be left to float
or are stabilized with a long
resorbable plate.

Posterior Vault Reshaping
• Alternatively to barrel-
stave osteotomies, an
occipital segment can
be cut and advanced
posteriorly. The
segment is secured with
resorbable plates.

• Reshaping of the occipital flap
• This typically involves splitting
the bone longitudinally and
bending and shaping the two
segments, placing the right
piece on the left side and vice
versa. However the segments
can also be replaced in their
original position and rotated.
• The principle is to make the
involved side more rounded
and full, slightly overcorrected
but generally symmetric with
the opposite side.

• Fixation
• The segments are
secured with resorbable
plates and screws,
wires, or sutures.

Craniosynostosis

Recommended

Recommended

More Related Content

What's hot

What's hot (20)

Similar to Craniosynostosis

Similar to Craniosynostosis (20)

More from Jamil Kifayatullah

More from Jamil Kifayatullah (20)

Recently uploaded

Recently uploaded (20)

Craniosynostosis