view. The devices are placed at a vector parallel to the
tangent of the vertex of the skull (arrow).
■ Scalp Closure
■ The activation arms of the devices are passed through separate small
incisions of the scalp (TECH FIG 3).
■ The coronal incision is closed with galeal resorbable sutures and resorbable
skin sutures.
■ A percutaneous drain can be placed under the flap but is often not required.
TECH FIG 3 • The extension arms of the distraction
arms exit percutaneously away from the coronal
incision. They can exit either anterior or posterior
depending on the patient. Posterior exit was chosen
for this patient to avoid the non–hair-bearing forehead.
The extension arms can be detached in the clinic once
the activation is complete so that no percutaneous
wounds exist during the consolidation period.
PEARLS AND PITFALLS
Timing
■ Timing of PVDO should include a consideration of early
intervention to optimize defect healing balanced with the thicker
bone present at later surgery, which optimizes device stability.
■ The typical age for PVDO is 6–9 months, which allows sufficient
time for healing before the need for frontal surgery.
Technique
■ Accurate identification of the torcula and lateral venous sinuses is
essential to plan a safe osteotomy. In some syndromes like Pfeiffer
syndrome, the torcula can be inferiorly displaced and in the line of
the osteotomy.
■ A piezoelectric saw can improve safety of performing the
osteotomy due to its integrated irrigation and decreased risk of
dura violation. Thermal injury to the dura and/or bone edges
should be avoided when using high-speed drills.
■ Prior to planning the osteotomy, a straight elevator in line with the
desired vector can be used to identify the tangent to the bone (see
TECH FIG 2D). The osteotomy is then placed at this tangential
point to ensure parallel vectors.
■ The scalp incision for the exiting activation arms should be far
enough away from the osteotomy to ensure the device is fully
buried after activation and removal of the activation arms. It needs
to be posterior enough that the tenting of the skin by the activation
arms does not cause skin injury.
■ A red rubber catheter attached over the activation arm tip can be
used to pass the arms through the percutaneous scalp incisions.
Linear traction on these rubber catheters can then be used to ensure
that the vectors of all devices are parallel.
■ Some authors have recommended placing acellularized dermis or
other padding on the intracranial surface underneath the device
footplates to prevent dura tearing. This author has not observed
this complication but recognizes the importance of ensuring no
damage to the dura during the surgery. If there are protruding
screw tips touching the dura, they can be removed using a rongeur.
Postoperative
care
■ Although postoperative imaging (radiograph or CT scan) can be
valuable, in many cases, it is not necessary. Patients with shunts in
place do benefit from imaging (ultrasound or CT scan) during
large activations to ensure continued functioning of the shunt.
■ If the devices are removed separately, and not at the time of a
frontal surgery, this can be done through small sagittal incisions
over the footplates to minimize blood loss and dissection of the
healing osteotomy gap compared to reopening the coronal scar.
POSTOPERATIVE CARE
■ PCVD requires less bone dissection, less operative time, and less fluid shift
than does traditional cranial bone removal and remodeling.
■ In other types of distraction osteogenesis, the bone generated at the opening
osteotomy is from formation of a unique undifferentiated generated tissue from
the cross-sectional area of the opposing bone surfaces at the osteotomy site.
■ In PVDO, it is not clear whether distracted generate tissue forms, or whether
the defect healing is from the osteogenic potential of the underlying immature
dura. Because of this possibility, intact and healthy dura is important at the
site of the osteotomy in PVDO.
■ We typically start device activation the day after operation, but activation can
be delayed 3 to 5 days based on surgeon preference.
■ The rate of device activation ranges from 1 to 2 mm a day based on bone
thickness and patient age.
■ Patient caregivers should be educated and observed regarding appropriate and
symmetric device activation.
■ A typical length of distraction is 15 to 30 mm based on preoperative planning
and patient cranial volume needs.
■ Once the activation is complete, the percutaneous activation arms are removed
and consolidation is begun.
■ A typical consolidation period in an infant is 6 to 10 weeks before device
removal. After 10 weeks, the devices can become encased in bone,
complicating their removal.
■ The devices can be removed either as a separate operation or at the time of
subsequent frontal surgery depending on timing.
COMPLICATIONS
■ Intraoperative complications such as VAE, sinus bleeding, or CSF leakage are
shared with other cranial vault surgeries but minimized compared to traditional
remodeling procedures due to less epidural dissection and shorter operating
times.11
■ If the dura is violated during the intraoperative dissection, the surgeon needs to
decide whether the PVDO should be aborted or converted to an open
remodeling procedure.
■ PVDO can proceed in the presence of well-repaired dura tears away from the
devices and areas of anticipated distraction strain.
■ During activation of the devices, the dura is placed under strain and can tear if
care has not been taken to avoid prominence of the mounting screws or if there
is violation of the dura during the dissection.
■ Cellulitis around the activation arm sites usually responds to cleaning and oral
antibiotics, but persistent infection or signs of abscess formation require open
procedure inspection, wound irrigation, and possible removal of the devices.12
■ Exposure of the portions of the device during consolidation due to skin erosion
does not require immediate treatment in the absence of deep space infection.
■ Closure of the erosion wounds is best achieved at the time of device removal
due to improved skin laxity.
■ Device dislodgement during or after activation may require operative
treatment such as replacement of the device or stabilization of the achieved
expansion with a resorbable plate if there is instability.
REFERENCES
1. White N, Evans M, Dover MS, et al. Posterior calvarial vault expansion using
distraction osteogenesis. Childs Nerv Syst. 2009;25:231-236.
2. Derderian CA, Wink JD, McGrath JL, et al. Volumetric changes in cranial
vault expansion: comparison of fronto-orbital advancement and posterior
cranial vault distraction osteogenesis. Plast Reconstr Surg. 2015;135:1665-
1672.
3. Shimizu A, Komuro Y, Shimoji K, et al. Quantitative analysis of change in
intracranial volume after posterior cranial vault distraction. J Craniofac Surg.
2016;27:1135-1138.
4. Ahmad F, Evans M, White N, et al. Amelioration of Chiari type 1
malformation and syringomyelia following posterior calvarial distraction in
Crouzon's syndrome: a case report. Childs Nerv Syst. 2014;30:177-179.
5. Levitt MR, Niazi TN, Hopper RA, et al. Resolution of syndromic
craniosynostosis-associated Chiari malformation Type I without suboccipital
decompression after posterior cranial vault release. J Neurosurg Pediatr.
2012;9:111-115.
6. Hayward R. Venous hypertension and craniosynostosis. Childs Nerv Syst.
2005;21:880-888.
7. Thompson D, Hayward R, Jones B. Anomalous intracranial venous drainage:
implications for cranial exposure in the craniofacial patient. Plast Reconstr
Surg. 1995;95:1126.
8. McCarthy JG, Glasberg SB, Cutting CB, et al. Twenty-year experience with
early surgery for craniosynostosis. II. The craniofacial synostosis syndromes
and pansynostosis: results and unsolved problems. Plast Reconstr Surg.
1995;96(2):284-295.
9. Faberowski LW, Black S, Mickle JP. Incidence of venous air embolism during
craniectomy for craniosynostosis repair. Anesthesiology. 2000;92:20-23.
10. Phillips RJ, Mulliken JB. Venous air embolism during a craniofacial
procedure. Plast Reconstr Surg. 1988;82:155-159.
11. Taylor JA, Derderian CA, Bartlett SP, et al. Perioperative morbidity in
posterior cranial vault expansion: distraction osteogenesis versus conventional
osteotomy. Plast Reconstr Surg. 2012;129:674e-680e.
12. Greives MR, Ware BW, Tian AG, et al. Complications in posterior cranial
vault distraction. Ann Plast Surg. 2016;76:211-215.
5
CHAPTER
Total Cranial Vault
Remodeling
Akira Yamada, Kenneth E. Salyer, Derek A. Bruce, and Frank
R. Graewe
DEFINITION/ANATOMY
■ Premature fusion of the sagittal suture restricts the transverse growth of the
skull.
■ The skull is longer in anteroposterior dimension and narrower in temporal and
lateral width.
■ Frontal and occipital bossing is characteristic, the degree of which depends on
the exact location and extent of synostosis (FIG 1).
PATHOGENESIS
■ Exact cause of primary (isolated) craniosynostosis is unknown.
■ Sagittal synostosis has an approximately 2% familial or genetic predisposition.
NATURAL HISTORY
■ Isolated nonsyndromic sagittal synostosis is infrequently associated with
abnormal brain development. Intelligence is generally normal, but sagittal
synostosis may be associated with subtle psychosocial and learning issues.
PATIENT HISTORY AND PHYSICAL FINDINGS
■ Physical examination includes assessment of suture ridging in the midline,
evidence of other sutural patencies by manual palpation, overall skull and
facial configuration, and an age-appropriate neurological examination.
■ Although papilledema (signs of raised intracranial pressure) is rare in infants
with sagittal synostosis, ophthalmologic examination, including visual acuity
and the presence of diplopia, can be tested by a pediatric ophthalmologist.
IMAGING
■ Plain radiographs may be used to document closure of the sagittal suture or to
confirm the patency of other sutures.
■ Adjacent sutures may have compensatory widening due to elevated
intracranial pressure.
■ CT scan of the skull and skull base define brain and skeletal anatomy.
NONOPERATIVE MANAGEMENT
■ Surgery is routinely advised as soon as the infant is physiologically capable of
undergoing surgery.
■ Nonoperative management is not available for this condition.
SURGICAL MANAGEMENT
Preoperative Planning
■ Plain radiographs may be used to document closure of the sagittal suture or to
confirm the patency of other sutures. Adjacent sutures may have compensatory
widening due to elevated intracranial pressure.
■ CT scan of the skull and skull base define brain and skeletal anatomy.
■ 3D reconstruction of the cranium, orbit, and face is useful to use computer-
aided design planning, especially for secondary cranial vault reconstruction
(FIG 2).
■ In suspected cases, MRI may be used to rule out the risk of tonsillar
herniation, as in Arnold-Chiari malformation.
■ The cranial vault is removed in three sections. Designing a new bandeau
posterior to the coronal suture is the key point of reference for remodeling.
FIG 1 • Preoperative views: frontal (A), left profile (B),
and overhead (C).
FIG 2 • Preoperative skull shape, in comparison with the
goal of surgery (normalization of skull shape), as shown
in purple. Goals are to widen at the posterior side,
shorten in anteroposterior dimension, smooth the
forehead, and make the occipital bone round.
■ Access to the brain is planned using a bifrontal craniotomy, with expanding
and widening of the forehead using the new bandeau and shortening of the
anteroposterior dimension of the head.
Positioning
■ The authors use a supine position for total cranial vault remodeling.
■ Other centers use a modified prone position for total cranial vault remodeling.
Approach
■ If the child presents at an age of less than 3 to 5 months, there are three
options for treatment:
■ Endoscopic strip craniotomy with helmet therapy
■ Extended suture craniotomies with or without dynamic reduction of the
abnormally long anteroposterior axis with parietal and occipital extensions
■ Total cranial vault remodeling1
■ Simple strip craniotomies with no further remodeling may result in suture
refusion with persistence of an abnormally shaped head.
■ If the child presents at 1 year or older in age, neither simple strip craniotomies
nor extended suture craniotomies are appropriate:
■ Total cranial vault remodeling2 is the first and only choice for treatment.
■ In this age group, the bone is generally much less malleable than in the child
less than 1 year old, and bone remodeling techniques are adapted to the more
brittle nature and handling characteristics.
T E C H N I Q U E S
■ Exposure
■ A zigzag skin incision is marked prior to preparation (TECH FIG 1).
■ The initial incision is made with the Colorado needle and continued laterally
on both sides simultaneously.
■ The scalp is raised above the periosteal, and under the galeal, plane.
■ The scalp dissection initially proceeds anteriorly first in the same
subgaleal/supraperiosteal plane, until 2 cm above the supraorbital rim.
■ A subperiosteal dissection is then initiated to identify the supraorbital nerves
bilaterally, which are released from the bony canal using a small osteotome
bilaterally.
■ Malleable retractors protect the globe.
■ Further subgaleal/supraperiosteal skin flap dissection is performed in the
occipital region.
TECH FIG 1 • The child is placed in a supine position,
and the skin is marked for a zigzag incision.
■ Pericranial Flap Dissection
■ Four periosteal flaps are designed (TECH FIG 2A).
■ Anteriorly two periosteal flaps are based on the left and right temporal
muscle.
■ The posterior two periosteal flaps are designed based on the occipital
muscle (TEC FIG 2B).
■ A Colorado needle facilitates the design of periosteal flaps. These flaps are
raised with periosteal elevators and wet sponges.
■ Bleeding from bone is controlled using bone wax.
■ Skull exposure is finished after the completion of the pericranial flaps
(TECH FIG 2C).
TECH FIG 2 • A. Design of anterior-based pericranial
flap. B. Anterior pericranial flap is raised based on
temporal muscle. C. The view of exposed skull, prior
to total cranial vault remodeling.
■ Planning the New Bandeau and Removing the
Forehead
■ The new bandeau is marked with a pencil, using a malleable retractor,
posterior to the coronal suture where the bone has the best contour and width
for a more ideal forehead (TECH FIG 3A,B):
■ The initial bur hole is made in the midline, posterior to the new bandeau.
■ The second bur hole is made in the midline, anterior to the new bandeau
(TECH FIG 3C).
■ Bone dust is collected for later use.
■ The dura is entirely freed from overlying bone that will be removed.
■ A craniotome is used to cut the bandeau.
■ The bandeau is removed, and hemostasis is obtained.
■ The forehead is marked with a pencil and cut, after the dura is completely
freed from the overlying forehead.
■ The forehead is freed and removed.
■ Hemostasis is achieved.
TECH FIG 3 • A. The new bandeau is designed,
posterior to coronal suture. B. Diagram to show the
location of harvesting new bandeau. C. Two bur holes
are placed in the midline, anterior and posterior to the
marked new bandeau.
■ New Bandeau and Forehead Bone Remodeling
■ On the back table, multiple partial-thickness cuts are made on the inner
surface of the new bandeau, using a reciprocating saw.
■ A Tessier bone bender is then used to change the arc of the bandeau for
correcting the contracted, narrow forehead (TECH FIG 4A).
■ A preoperative forehead pattern is used to assess the shape of the new
bandeau.
■ The removed forehead is narrow and contracted in comparison to the new
bandeau (TECH FIG 4B):
■ Multiple radial cuts are made to allow for expansion.
■ A Tessier bone bender is then used to change the arc of the bandeau to match
the planned curve of the new bandeau (TECH FIG 4C–E).
TECH FIG 4 • A. Tessier bone bender is used to
change the arc of bandeau. B. The original forehead
is narrow, and contracted, compared with the
expanded bandeau. The comparison between (C) a
preremodeled bandeau and (D) a postremodeled
bandeau. E. The view of expanded forehead and new
bandeau.
■ Removing and Remodeling the Occipital Bone
■ The occipital bone is dissected from the underlying dura.
■ A bur hole is made in the posterior midline of the occipital bone just above
the lateral sinus.
■ The occipital bone is cut with a craniotome and removed.
■ Hemostasis is achieved with bipolar cautery.
■ On the back table, multiple cuts are made in the bone and then remodeled.
■ A Tessier bone bender is used to change the arc of the bone, to expand it in
a transverse dimension, changing the radius of the curve.
■ Expanding the Skull Base
■ Attention is turned anteriorly, where the old bandeau is removed.
■ The skull base is then widened by creating bone flaps.
■ A bone flap is created on both sides in the temporoparietal region.
■ The bone flaps are posteriorly based and expanded laterally to match the
expanded forehead (TECH FIG 5).
TECH FIG 5 • The skull base is widened by creating
bone flaps. A. A bone flap is created on both sides in
the temporoparietal region. B. The bone flaps are
posteriorly based and expanded laterally to match the
expanded forehead.
■ Replacing the New Bandeau, Remodeled
Forehead, and Occipital Bone
■ The new bandeau and forehead are secured in place with multiple 2-0 PDS
sutures (TECH FIG 6A).
■ The skin is draped to assess the forehead shape.
■ Posteriorly, the new remodeled occipital bone has been shortened and
widened and is fixed in place (TECH FIG 6B), creating parietal eminences.
■ Multiple 2-0 PDS sutures are used to secure the occipital bone (TECH FIG
6C,D).
TECH FIG 6 • A. The new bandeau and remodeled
forehead are placed back in the remodeled skull. B.
Posteriorly, the new remodeled occipital bone has
been shortened and widened and is fixed in place. C.
The view of the remodeled total cranial vault. D.
Schematic presentation of totally remodeled skull.
■ Closure
■ Pericranial flaps are secured over the bone with 4-0 Vicryl.
■ After copious irrigation of the wound with saline, bone dust covered by
Surgicel is used to fill the bony gaps.
■ The skin incision is closed in two layers.
PEARLS AND PITFALLS
Hemostasis
■ The scalp flap is dissected with a micropoint needle to reduce blood
loss. Using a low setting minimizes damage to tissues (both
coagulation and cutting less than 10).
Fixation
materials
■ Although older children may need resorbable plates and screws, 2-0
PDS and occasional wire fixation reduces surgical duration and
provides adequate stability of the skull.
Team
approach
■ When the neurosurgeon and craniofacial surgeon work together
during the perioperative planning and care, outcomes are improved.
POSTOPERATIVE CARE
■ Pediatric intensive care unit (PICU) for first night
■ Head of the bed to 30 degrees; parents may hold the child when cleared by
PICU staff
■ O2: FiO2 40% via face shield for O2 saturations less than 93%
■ Labs: complete blood count (CBC)/basic metabolic panel (BMP) at 6 PM,
midnight, and 6 AM unless patient needs change
■ Hemoglobin greater than 6.5 no concerns (evaluate clinically), less than 6.5
evaluate clinically, consider a repeat CBC
■ Decadron is used only if airway issues are raised by anesthesia or the PICU
team: up to four doses in 24 hours.
■ Incision care: Cleanse with ½ strength peroxide twice daily, and then apply a
thin ribbon of bacitracin ointment twice daily.
■ Medications:
■ Antibiotics: Ancef 30 mg/kg/dose IV; give two doses postop 8 hours apart
starting 8 hours from the last intraoperative dose.
■ Intravenous fluids: D5LR at maintenance rate, may saline lock when
tolerating adequate PO per oral intake.
■ Acetaminophen: 12.5 mg/kg IV dose q6h.
■ Ketorolac/ibuprofen combo panel: ketorolac 0.25 mg/kg IV q6h exact “or”
ibuprofen 10 mg/kg PO q6h (ibuprofen up to 400 mg max. dose). Try to
stagger acetaminophen and ketorolac/ibuprofen every 3 hours.
■ Ativan: 0.05 mg/kg IV up to a maximum of 0.5 mg per dose q6h prn for
pain/anxiety, if no dexmedetomidine being used
■ Precedex: 0.1-0.5 mcg/kg/h IV infusion, titrate to an appropriate level of
consciousness, DC at 0600 hours on POD#1. Generally started at 0.5 and
titrated the dose down. Keep the child arousable and not heavily sedated. The
dose only goes above 0.5 if the child is significantly fussy.
■ Zofran: 0.15 mg/kg up to a maximum of 4 mg IV q8h prn. May add
Phenergan as needed 12.5 mg/kg IV
OUTCOMES
■ Three-dimensional head shape is improved. The elongated A-P dimension of
the skull is shortened. The pointed forehead and occiput become more rounded.
The width of the head is increased (FIG 3).
COMPLICATIONS
■ Perioperative airway complications
■ Hypovolemic shock
■ Dural tear, postoperative cerebral spinal fluid (CSF) leak
■ Postoperative bleeding (epidural, subdural hematoma)
■ Wound infection
■ In the most severe case, any of the above can lead to death.
■ Irregular head shape
■ Incomplete bone healing (bone defect), especially for older children
■ Recurrent deformities
FIG 3 • Six months postoperative views: frontal (A),
profile (B), and overhead (C).
REFERENCES
1. Heller JB, Heller MM, Knoll B, et al. Intracranial volume and cephalic index
outcomes for total calvarial reconstruction among nonsyndromic sagittal
synostosis patients. Plast Reconstr Surg. 2008;121(1):187-195.
2. Salyer KE. Personal contributions to craniofacial surgery. Scand J Plast
Reconstr Surg Hand Surg Suppl. 1995;27:19-47.
6
CHAPTER
Spring-Assisted Surgery for
Sagittal Synostosis
Lisa R. David and Claire Sanger Dillingham
DEFINITION
■ Craniosynostosis is the premature fusion of one or more cranial sutures (FIG
1).
■ Sagittal synostosis is the premature fusion of the sagittal suture, which leads to
a long narrow-shaped head with frontal and occipital bossing due to the
restriction in lateral growth as predicted by Virchow law.1
■ It is the most common form of craniosynostosis accounting for 55% of cases
and is called scaphocephaly.2
ANATOMY
■ The skull is made up of bones that abut at sutures that allow growth of the
underlying brain.
■ The primary sutures in an infant skull are the sagittal, coronal, metopic,
lambdoid, and squamosal.
■ The sagittal suture is a dense, fibrous connective tissue joint that lies between
the two parietal bones of the skull.
■ The bregma is the intersection between the sagittal and coronal sutures often
referred to as the anterior fontanelle at birth.
■ The posterior portion of the sagittal suture ends at the intersection of the
lambdoid sutures.
■ The point between these two is the vertex of the skull and is often the highest
point on the skull.
PATHOGENESIS
■ Most craniosynostosis cases are not associated with a syndrome, although over
100 syndromes have been connected to this condition.3
■ Single-suture sagittal synostosis is the most prevalent type of craniosynostosis
with an incidence of 1 in 5000 births.4
■ There is a higher observed incidence in boys compared to girls with a 4:1
ratio, respectively.5
■ Genetic mutations in the cell surface receptor fibroblast growth factor receptor
(FGFR) 1 to 3 have been linked to the development of nonsyndromic
craniosynostosis and syndromic cases such as Apert, Pfeiffer, Jackson-Weiss,
Crouzon, and Muenke.6
■ Mutations identified in the targets TWIST1, bone morphogenetic proteins
(BMPs), and RUNX2 have been linked to craniosynostosis.
FIG 1 • A. Cranial sutures. B. Most common types of
craniosynostosis and their associated shape change.
(© WFBHS Plastic Surgery Collection.)
■ Transforming growth factor β (TGFβ) mutations have been involved in suture
fusion but have not yet been linked to a syndrome.
■ Most craniosynostosis syndromes have an autosomal dominant inheritance.
NATURAL HISTORY
■ The time period of the most rapid brain growth in healthy humans is from the
third trimester of pregnancy to the first year of life.
■ The average cranial capacity at birth is 350 cm4 and triples by 9 months of
age.
■ In the absence of pathology, such as craniofacial malformations or
malnutrition, the head circumference relates directly to the brain size, protein
and DNA content, and the number of neurons.
■ Most cranial sutures remain open until several years of age with the exception
being the metopic suture that can close as early as 1 year.
■ Most commonly, the sagittal suture begins to close from the posterior to
anterior direction in the late second decade of life with complete closure seen
at 35 years of age.
■ Sagittal synostosis can be detected as early as the 16th week of gestation by
magnetic resonance imaging (MRI) and sonography.7
■ Sagittal synostosis is most commonly diagnosed in the first year of life.
■ Patients with sagittal synostosis are typically referred to the craniofacial
surgeon after the pediatrician observes one or more of the following: the
narrow and elongated head shape or frontal and occipital bossing, or the
parents have expressed concern regarding the skull shape.
■ The incidence of elevated intracranial pressure (ICP) is low in patients with
single-suture fusion; however, it is possible and should be considered.
■ Neurocognitive deficits are linked to craniosynostosis especially with multiple
suture involvement.
■ Treatment aims to restore the calvarial shape, reduce ICP if needed, enhance
development, and improve the appearance.
PATIENT HISTORY AND PHYSICAL FINDINGS
■ A thorough history and physical exam can provide information that leads to a
diagnosis of associated conditions and avoids errors in failure to address
significant concerns prior to surgery.
■ Perinatal history
■ Gestational age (this shape can be seen in prematurity without synostosis)
■ Age of parents
■ History of previous maternal miscarriages
■ Multiple births such as twins or triplets
■ Any perinatal distress
■ C-section vs natural vaginal delivery or assisted delivery with a device
■ Time spent in the ICU, intubated, or immobilized
■ Feeding history and if any intolerance is noted
■ Family history
■ Family members with any craniofacial anomalies or syndromes
■ Siblings of an affected child have double the estimated risk than the general
population.
■ Physical examination
■ The cephalic circumference and index are measured and documented as they
will be followed postoperatively.
■ Documentation should include intraocular distance, any signs of exorbitism,
airway compromise, midface hypoplasia, or limb anomalies.
■ Evaluation for cleft lip or palate
■ An ophthalmology evaluation documenting the presence of increased ICP
leading to optic nerve swelling, which results in papilledema
■ Consultation with a geneticist is warranted.
IMAGING
■ A computed tomographic (CT) scan provides valuable information as well as
documentation of the condition (FIG 2).
■ It may also reveal additional pathologies that would have otherwise been
hidden.
■ An MRI is not part of the standard imaging assessment in single-suture
craniosynostosis but may be indicated if there is evidence of increased ICP.
■ The presence of agenesis of the corpus callosum or ventriculomegaly should
prompt further evaluation.
■ A portable laser 3D scanning device is utilized at our institution and, if
available, provides the team and family an objective analysis of cephalometric
measurements, including cranial index (CI), 3D shape, and cranial volume
calculated using Delta Scan Comparison software.
DIFFERENTIAL DIAGNOSIS
■ Single-suture craniosynostosis
■ Multiple suture craniosynostosis
■ Scaphocephalic head shape with no synostosis seen with significant
prematurity
■ Positional plagiocephaly
■ Secondary craniosynostosis due to shunted hydrocephalus, lack of brain push,
or trauma
FIG 2 • 3D CT scan showing fusion of the sagittal
suture. (© WFBHS Plastic Surgery Collection.)
NONOPERATIVE MANAGEMENT
■ Nonoperative treatment of craniosynostosis is not recommended.
■ If there is a more serious life-threatening diagnosis, surgery for
craniosynostosis should be delayed or undertaken with complete risks and
benefits discussed with the family.
SURGICAL MANAGEMENT
■ Operative intervention for craniosynostosis is widely accepted and is the
standard of care for these conditions.
■ The indication for surgical intervention for sagittal suture synostosis is full
fusion of the suture or partial fusion with skull dysmorphology or increased
ICP.
■ The ideal age for spring-assisted surgical correction is between 3 and 7 months
of age.
■ This provides enough bone stock to handle the strength of the spring and a
strong enough spring force to expand the skull bones.
■ This age range also allows for native bone regeneration and correction of
further skull growth.
■ Risks to the patient include a scar on the scalp, bleeding, infection, spring
dislodgement, cranial asymmetry, and need for further surgery.
Preoperative Planning
■ Preoperative evaluation should include a 3D CT scan of the skull to confirm
the diagnosis of isolated sagittal craniosynostosis and rule out additional
intracranial anomalies such as a Chiari malformation.
■ The CT also aids in operative planning for the selection of the spring strength
and placement.
Positioning
■ The airway is secured, and ophthalmic ointment is placed in the eyes and
secured with tape to keep the eyes closed and protected.
FIG 3 • Sphinx positioning with appropriate padding. (©
WFBHS Plastic Surgery Collection.)
■ For the open and endoscopic approach, the patient is positioned prone on a
beanbag in the sphinx position (FIG 3).
■ Padding around the ears and shoulders is used to prevent pressure points.
Approach
■ Standard ASA monitors should be used with inhalation induction followed by
endotracheal intubation and placement of mechanical ventilation.
■ Intravenous (IV) access with two lines is advised.
■ IV fluid, muscle relaxants, and narcotic analgesics are administered during the
procedure.
■ IV antibiotics are administered.
■ End-tidal CO2 is decreased to approximately 30 mm Hg to decrease ICP.
■ The surgical field is prepared with Betadine scrub and paint.
T E C H N I Q U E S
■ Open Technique
Exposure
■ A marker is used to draw an S-shaped longitudinal incision from the anterior
to the posterior fontanelle landmarks (TECH FIG 1A).
■ The marked incision site is infiltrated with bupivacaine 0.25% with 1:200
000 epinephrine (not to exceed 1 cc/kg) for improved intraoperative
hemostasis.
■ The incision is made and the sagittal suture exposed with minimal stripping
of the periosteum to diminish bleeding (TECH FIG 1B).
■ A 1-cm-wide strip craniectomy of the calvarium is performed, which should
include the sagittal suture (TECH FIG 1C).
■ Hemostasis of the bone edges is obtained as well as of the underlying dura.
Spring Placement
■ The springs are selected based on the patient’s age, bone thickness, and type
of scaphocephaly.
■ Typical range is 6 to 8 Newtons for the 3 to 6 months’ age group (TECH
FIG 2A,B).
■ If significant frontal bossing is present, the child will need a stronger
spring anteriorly.
■ If significant elongation is present, the child will need a possible third
spring.
■ With postcoronal banding, the child will need a strong anterior spring.
■ With significant posterior banding, the child will need a stronger posterior
spring.
■ The anterior spring is placed with the footplate anterior and behind the
coronal sutures and the posterior spring with the footplate posterior and in
front of the lambdoid sutures.
TECH FIG 1 • A. S-shaped incision used for the open
technique. B. Sagittal suture is exposed with minimal
periosteal stripping. C. A 1-cm strip craniectomy
removing the sagittal suture is performed. (© WFBHS
Plastic Surgery Collection.)
TECH FIG 2 • A,B. Springs are selected and placed in
the calvarial defect. C. Springs are secured at point of
overlap using a Vicryl suture. (© WFBHS Plastic
Surgery Collection.)
■ An absorbable suture is placed around the two springs where they overlap
in the midline to prevent rotation or shifting (TECH FIG 2C).
■ Custom bending can be done to reduce the prominence of the spring but
should be done minimally.
■ Every adjustment can change the force of the spring and so should be
limited.
■ The scalp is closed in two layers with absorbable, buried sutures.
■ A drain is not necessary. The blood loss is negligible and transfusions have
not been needed.
Spring Removal
■ Once the skull defect has reossified, the spring can be removed. This is
typically 4 to 5 months after the first procedure.
■ This is an outpatient procedure.
■ The patient is positioned as in the first case using a beanbag and in the
sphinx position.
■ Local anesthetic is injected over each of the footplates and in the midline
incision where the springs overlap (TECH FIG 3A).
■ Small incisions are made over each of the footplates as well as where the
springs intersect in the midline (TECH FIG 3B).
■ The springs are cut and the segments removed through the footplate
incisions (TECH FIG 3C, D).
■ Incisions are closed with buried and interrupted absorbable sutures (TECH
FIG 3E).
■ If there were no intraoperative concerns, the patient can be discharged from
the recovery room.
■ Spring removal takes an average of 20 minutes of surgical time and an hour
of anesthetic time.
TECH FIG 3 • A. Local anesthetic is injected over the
four footplates and previous incision for spring
removal. B. Small incisions are made over the
footplates. C,D The footplates are rotated out and the
spring brought through the small individual incisions.
E. The incisions are closed with absorbable sutures.
(© WFBHS Plastic Surgery Collection.)
■ Endoscopic-Assisted Technique
Incisions and Endoscope Insertion
■ The patient is placed in the sphinx position with the head on a beanbag,
similar to the open approach (TECH FIG 4A).
■ The marked incision site is infiltrated with bupivacaine 0.25% with 1:200
000 epinephrine (not to exceed 1 cc/kg) before incision for improved
intraoperative hemostasis.
■ A 3- to 4-cm W-shaped incision is made midway between the anterior and
posterior fontanelle positions. Typically, it is placed where the head starts to
curve to allow good access anteriorly and posteriorly (TECH FIG 4B).
■ A lighted retractor is used to aid in visualization of the subgaleal plane to
dissect the entire distance of the sagittal suture (TECH FIG 4C).
■ A pediatric craniotome is used to make a single bur hole just under the
incision.
■ Kerrison rongeurs are used to enlarge the bur hole.
■ The 30-degree rigid endoscope is then inserted under the bone and directed
toward the anterior fontanelle (TECH FIG 4D).
■ The suction tip is used to keep the field clear and dissect the dura free from
the overlying bone.
■ The dura should be protected throughout with an insulated brain retractor,
and care should be taken not to disrupt the dural attachment to the fontanelle
to avoid injury to the sagittal sinus.
■ Hemostasis should be achieved with the monopolar Bovie, Surgiflo, or
liquid thrombin (TECH FIG 4E).
■ A 1-cm strip of calvarium with the sagittal suture is removed by the pediatric
neurosurgeon using curved Mayo scissors or bone cutters (TECH FIG 4F).
Spring Placement
■ The bone is inspected and springs are chosen based on the bone thickness,
patient age, and head shape (TECH FIG 5A).
■ Custom bending can be done to reduce the prominence of the spring but
should be done minimally. Every adjustment can change the force of the
spring and should be limited.
■ The springs are secured where they cross with a small drill hole on each side
in the bone using 4-0 Vicryl suture (TECH FIG 5B).
■ The scalp is closed in two layers with absorbable buried sutures (TECH FIG
5C).
■ A drain is not necessary. The blood loss is negligible, and transfusions have
not been needed.
■ A protective head wrap is placed for 24 hours (TECH FIG 5D).
Spring Removal
■ Once the skull defect has reossified, the spring can be removed similar to the
open procedure.
■ This is an outpatient procedure.
■ The patient is positioned as in the open procedure.
■ If there were no intraoperative concerns, the patient can be discharged from
the recovery room.
TECH FIG 4 • A. Patient is positioned prone in the
sphinx position with planned incision marked. B. W-
shaped incision is incised with a no. 15 blade. C. The
lighted retractor is used to visualize and give exposure
of the sagittal suture. D. The endoscope is used to
assist in freeing the dura from the overlying suture. E.
Hemostasis is assured with use of a hemostatic agent.
F. The suture is removed using a combination of bone
scissors and rongeurs. (© WFBHS Plastic Surgery
Collection.)
TECH FIG 5 • A. Springs are inserted through the W
incision. B. Springs are secured to cranial bone with
4-0 Vicryl suture at overlap site. C. Scalp is closed in
layers with absorbable suture. D. Protective head
wrap is applied for 24 hours. (© WFBHS Plastic
Surgery Collection.)
PEARLS AND PITFALLS
Hemostasis
■ Given the child’s size and blood volume, careful attention to blood
loss is critical. Good hemostasis must be attended for all steps of the
procedure. This is optimized by giving the local anesthetic with
epinephrine 7 min to work.
Spring
choice
■ Spring choice is critical and clearly a part of the learning curve. If the
spring is too strong, it will pull through the bone; if it is not strong
enough, there will be undercorrection. Normal force range is 6–8 N.
Spring
placement
■ The springs must be placed behind the coronal sutures and in front of
the lambdoid sutures to get adequate expansion. The springs must be
secured to the bone where they overlap to prevent spring
dislodgement and rotation.
Operative
approach
■ It is often best to start with the open approach and then change to the
endoscopic approach once the surgeon is experienced enough to
ensure adequate dissection, placement, and securing of the springs.
Helmet
therapy
■ While the springs are in place, a soft helmet is used only for
protection from accidental trauma. Helmets are not needed for
shaping and not required once springs are removed.
POSTOPERATIVE CARE
■ The patient is admitted to the general pediatric unit in our institution for
overnight monitoring. The appropriate location for admission will depend on
the skill set and familiarity each pediatric unit has with patients after this type
of surgical procedure.
■ The morning after surgery:
■ A hemoglobin level is obtained.
■ Anteroposterior (AP) and lateral skull film are obtained to assess spring
placement.
■ The child is fitted with a noncompressive helmet for protection until
reossification occurs (FIG 4).
■ The child is seen by the craniofacial team at 10 days; 4, 8, and 12 weeks; and
then at time of spring removal. After spring removal, the patient is seen at 10
days, 3 and 6 months, and then yearly.
■ At the follow-up appointments, the cranial defect is assessed for reossification
and skull shape is assessed.
■ The 3D laser scan is used to document three-dimensional changes in cranial
shape and volume.
OUTCOMES
■ Over 16 years of clinical outcomes in more than 200 patients with sagittal
synostosis treated with spring-assisted surgery have shown successful
correction of scaphocephaly with spring-assisted surgery.
■ The mean initial bony gap immediately after placement is 1.5 cm.
■ A final gap of 6.3 anteriorly and 6.4 cm posteriorly is the mean measure on
follow-up skull films and can be expected.8
■ Cephalometric analysis demonstrates a mean pretreatment CI of 69 and
postoperative treatment of 75.4. The mean CI at 1 year is 74.8, at 3 years is
74.5, and 5 years is 76.6.
■ The Delta Scan Comparison software can be used to calculate the cranial
volume changes of each patient from the laser Doppler scans.
■ As a result of frontal bossing seen with scaphocephaly patients, the ratios of
the anterior cranial volume to the total cranial volume are expected to be higher
preoperatively when compared to healthy controls. After treatment, the ratio
decreases to a normal value.
■ The mean age at spring placement is approximately 5 months with a mean
duration of placement of 4 to 5 months and spring removal at a mean of 8
months of age.
COMPLICATIONS
■ Scar revision of the scalp may be needed once cranial growth is complete. This
is less likely with the endoscopic approach.
■ Local skin infection may require debridement but is usually treated with
antibiotic ointment and oral antibiotics.
■ Prominence of the spring may be concerning for parents but will dissipate in
time as the expansion continues.
■ Spring dislodgement will require surgical replacement.
■ No additional surgery has been required for persistent calvarial defects or
recurrence of cranial deformity.
■ One child has had additional surgery for undercorrection of the deformity.
FIG 4 • Laser scan in use (A) and resultant scans (B, C)
used for postoperative shape monitoring. (© WFBHS
Plastic Surgery Collection.)
REFERENCES
1. Virchow R. Ueber den cretinismus, namentlich in Franken, und über
pathologische schädelformen. Verh Phys Med Ges Würzburg. 1851;2:230-271.
2. Warren SM, Proctor MR, Bartlett SP, et al. Parameters of care for
craniosynostosis: craniofacial and neurologic surgery perspectives. Plast
Reconstr Surg. 2012; 129:731-737.
3. McCarthy JG, Warren SM, Bernstein J, et al. Parameters of care for
craniosynostosis. Cleft Palate Craniofac J. 2012; 49:1S-24S.
4. Kolar JC. An epidemiological study of nonsyndromal craniosynostoses. J
Craniofac Surg. 2011;1:47-49.
5. Persing JA. MOC-PS(SM) CME article: management considerations in the
treatment of craniosynostosis. Plast Reconstr Surg. 2008; 121:1-11.
6. Wilkie AO, Byren JC, Hurst JA, et al. Prevalence and complications of single-
gene and chromosomal disorders in craniosynostosis. Pediatrics.
2010;126:e391-e400.
7. Ketwaroo PD, Robson CD, Estroff JA. Prenatal imaging of craniosynostosis
syndromes. Semin Ultrasound CT MR. 2015;36(6):453-464.
8. David LR, Plikaitis CM, Couture D, et al. Outcome analysis of our first 75
spring-assisted surgeries for scaphocephaly. J Craniofac Surg. 2010;21(1):3-9.
7
CHAPTER
Transparietal/Transtemporal
Distraction for Sagittal
Craniosynostosis
Kristen Klement and Arlen Denny
DEFINITION
■ Sagittal craniosynostosis
■ Premature fusion of sagittal suture. The sagittal suture normally fuses at an
average of 26 years of age.
■ Results in scaphocephaly. Scaphocephaly is an abnormal head shape with an
increased anteroposterior dimension and narrowed bitemporal distance. This
can also be associated with frontal bossing and occipital bulging.
■ Distraction1
■ Repositions cranial vault gradually
■ Encourages intervening bony regeneration and decreasing bone grafts
■ Mitigates extradural dead space
■ Decreases blood loss
■ Shortens operative time and hospital stay
■ Promotes soft tissue acclimation
■ Minimizes relapse
■ Distraction is the ideal procedure in older infants and children.
• Allows for better bone quality for mounting the distraction device
• Decreases morbidity
ANATOMY
■ Cranial vault is a series of bones with junctions that constitute the cranial
sutures (FIG 1).
■ The sutures are syndesmoses or a joint between cranial bones.
■ The major cranial sutures are metopic, sagittal, coronal, lambdoid, and
squamosal.
■ Skull growth is normally perpendicular to sutures.
■ Premature fusion results in compensatory growth parallel to the affected
suture.
■ Premature fusion of the sagittal suture results in scaphocephaly.
PATIENT HISTORY AND PHYSICAL FINDINGS
■ History
■ Developmental delays and learning disabilities
■ Behavioral issues
■ Signs of elevated intracranial pressure:
• Chronic headaches
• Papilledema
• Color visual impairment progressing to optic nerve atrophy
■ Examination
■ Expanded anteroposterior dimension due to compensatory growth at the
active sutures
■ Narrowed bitemporal distance
■ Sagittal ridge
■ Frontal bossing due to fusion of the anterior sagittal suture and compensatory
growth by coronal and metopic sutures
■ Occipital bulge due to fusion of the posterior sagittal suture and
compensatory growth by lambdoid sutures
FIG 1 • Anatomy of normal skull bones and suture (left)
and premature fusion of sagittal suture with resultant
scaphocephaly (right).
FIG 2 • CT scan with 3D reconstruction of the head
demonstrating premature fusion of the sagittal suture
with scaphocephaly with expanded anteroposterior
dimension, narrowed bitemporal distance, sagittal ridge,
frontal bossing, and occipital bullet.
IMAGING
■ Computed tomography scanning of the head with 3D reconstruction (FIG 2)
shows the classic findings in premature fusion of the sagittal suture:
■ Expanded anteroposterior dimensions
■ Narrowed bitemporal dimension
■ Sagittal ridge at the site of the absent suture
■ Frontal bossing
■ Occipital bulge
SURGICAL MANAGEMENT
Preoperative Planning
■ Review CT scan preoperatively.
■ Plan osteotomy patterns.
■ Identify skull defects or sinus pericranium which may require changes in the
planning.
Positioning
■ Prone positioning with horseshoe headrest (FIG 3)
FIG 3 • Prone positioning with horseshoe headrest.
T E C H N I Q U E S
■ Asymmetric Transverse Distraction Osteogenesis
of the Cranial Vault
Video p2-c007
■ Coronal incision is designed from above the helical rim, including back-cut
in the hair-bearing scalp to improve access of the posterior skull.
■ Elevate posterior scalp flap below the pericranium.
■ Design craniotomy pattern on the skull with methylene blue (TECH FIG
1A,B).
■ A neurosurgeon performs craniotomies posterior to the coronal, anterior to
the lambdoid, and parasagittal using bur holes and a craniotome.
■ Corticotomies along the base of parietal bone using bur
■ Creates hinge to allow for lateral expansion
■ Occipital craniotomy along lambdoid suture using craniotome and
corticotomy along the base using bur
■ These craniotomies and corticotomies can be added to the basic pattern to
shorten the anteroposterior skull by hinging of occipital bone forward.
■ Restriction plate at anterior sagittal and coronal osteotomy junction
■ Restricts lateral expansion anteriorly
■ Results in asymmetric expansion with the greatest distance in the posterior
skull
■ Avoids visible step in temporal area
■ Mount internal distractor to base plates at posterior sagittal osteotomy site
across the parietal bone (TECH FIG 1C):
■ Mounting position of distractor device determines the area of the skull with
maximum distraction distance due to the anterior hinge plate.
■ Externalize drive rod through skin stab incision.
00:00 / 00:00
■ Activate device to full distance of device under direct visualization to test
maximum loading force (TECH FIG 1D,E).
■ Reset device to 0 mm.
■ Close the scalp.
■ 2-0 Vicryl stitch grabbing the periosteum and galea
■ Skin closure with staples
■ Apply dressing with antibiotic ointment and nonadherent gauze (TECH FIG
1F).
TECH FIG 1 • A,B. Intraoperative markings of the skull
with complete osteotomies made at the coronal,
sagittal, and lambdoid sutures and incomplete
corticotomy at lateral parietal bone and inferior
occipital bone to allow for hinging. C. Distractor
mounted at posterior sagittal osteotomy. D. Distractor
at full activation. Note the anterior hinge plate resulting
in asymmetric distraction with full activation. E.
Distractor at full activation of the lateral skull.
Complete osteotomies are made at the lambdoid
suture and incomplete corticotomy at lateral parietal
bone and inferior occipital bone to allow for hinging. F.
Postoperative dressing placement with externalized
distractor rod.
PEARLS AND PITFALLS
Anesthesia
■ Recommend systolic blood pressure less than 90 to minimize bleedi
Osteotomies
■ Complete just posterior to the coronal and parasagittal sutures and
anterior to the lambdoid sutures
Corticotomies
■ Parietal bone skull base and inferior occipital bone to allow for hing
(FIG 4)
FIG 4 • Dynamics of transparietal posterior vault
distraction.
Technique
■ Limit dissection between the cranium and dura to osteotomy lines to
maximize dura-bone flap vascular connections.
■ Check device by fully expanding to test under maximum force load
before closing.
■ Anterior hinge plate results in planned asymmetric expansion.
POSTOPERATIVE CARE
■ Intensive care unit until stable hemodynamically, followed by another 1 or 2
days on nonintensive floor
■ Transfusion with whole blood for hematocrit less than 27
■ Dressing optional
■ Cleanse staple line and distractor site arms with half strength hydrogen
peroxide, and coat with antibiotic ointment.
■ Distractor protocol
■ Latency period of 5 days
■ Activation at 0.5 mm/d
■ Full distraction within 4 weeks at 20 mm full device activation
■ Rods removed in the clinic at full activation to prevent external trauma and
inadvertent turning
■ Computed tomography scan at 15 weeks of consolidation to confirm bone
regeneration (FIG 5)
■ Distractor removal at 16 weeks of consolidation, if bone regeneration is
adequate
OUTCOMES
■ Decreased intracranial pressures using intracranial pressure monitoring
■ Increased intracranial volumes including both the ventricular and
nonventricular spaces3
■ Ventricular volume expansion equivalent to 10% of total expansion
■ Nonventricular volume increased an average of 6% from preoperatively
■ Head shapes normalize postoperatively.
■ Resolution of symptoms:
■ Parents report patient progress developmentally including speech, walking,
and potty training
■ Improvement in vision detail and color demonstrated at ophthalmologic
examinations
FIG 5 • CT scan at full distraction after consolidation.
COMPLICATIONS
■ Our broad experience using this technique on children from age 1 to 16 has
demonstrated minimal complications, and those that occur are minor4.
■ None of the patients required redistraction or advancement.
■ No patient has developed recurrent symptoms after up to 10-year follow-up.
■ Premature consolidation
■ Poor parent compliance with device turning despite adequate parental
teaching
■ Bony defects requiring grafting
REFERENCES
1. Lao WW, Denny AD. Internal distraction osteogenesis to correct symptomatic
cephalocranial disproportion. Plast Reconstr Surg. 2010;126:1677-1688.
2. Praveen K, Narayanan V, Muthusekhar MR, Baig MF. Hypotensive
anaesthesia and blood loss in orthognathic surgery: a clinical study. Br J Oral
Maxillofac Surg. 2001;39(2):138-140.
3. Deschamps-Braly J, Hettinger P, El Amm C, Denny AD. Volumetric analysis
of cranial vault distraction for cephalocranial disproportion. Pediatr
Neurosurg. 2011;47(6):396-405.
4. Klement KA, Black J, Denny AD. Versatility of distraction osteogenesis for
the craniofacial skeleton. J Craniofac Surg. 2016;27(3): 565-570.
8
CHAPTER
Monobloc Frontofacial
Advancement
S. Anthony Wolfe and Nicole C. Cabbad
DEFINITION AND ANATOMY
■ The Le Fort III–type osteotomy was first described by Gillies and modified by
Tessier. (Tessier called it Le Fort III–type because the pterygoid plates were not
fractured and it was subcranial.)
■ This procedure represents the highest level of maxillofacial osteotomy
performed without a craniotomy. It separates the face from the skull with
osteotomies across the orbital floor, lateral orbital walls, zygomatic arches,
and nasofrontal area and with a pterygomaxillary disjunction (FIG 1A–C).
■ The orbital osteotomies go deep inside the orbit, behind the lacrimal system,
and instead of going through the posterior hard palate—as originally
described by Gillies—Tessier performed a disjunction between the maxillary
tuberosities and the pterygoid plates (see FIG 1B).
■ In Tessier’s patient, interpositional bone grafts were placed into all surgical
gaps, with maxillary advancement of 20 mm. Traction to a head frame was
maintained for months, and intermaxillary fixation for almost a year.
■ Tessier then developed the simultaneous fronto-orbital advancement—referred
to as the BO-Fort procedure—a combined anterior movement of the forehead
with a Le Fort III–type advancement (FIG 2).
■ The first true monobloc frontofacial advancement was performed in Mexico
City and published in 1978,1 and Tessier presented his modification of the
procedure in 1995, which was designed to provide stable anterior movement of
the entire facial skeleton.
■ It leaves the effective orbit attached to the midface, differentiating it from the
Le Fort III–type and BO-Fort that Tessier had previously developed.
■ The monobloc was eventually associated with a facial bipartition.
■ Monobloc frontofacial advancement with a facial bipartition includes
removal of the interorbital naso-orbital-ethmoid maxillary bone for
overcorrection of hypertelorism as seen in patients with Apert syndrome.
■ The new, curved frontal bandeau made it possible to correct all of the
morphological alterations of Apert patients (flat face, retrusive forehead,
mild to moderate orbital hypertelorism, scaphymaxillism) in one operation.
■ The monobloc advancement is a procedure that can make craniofacial
dysostosis patients (Crouzon, Pfeiffer) look normal (FIGS 3 and 4).
PATIENT HISTORY AND PHYSICAL FINDINGS
■ Patients with a retrusive forehead and midface, proptosis, and class 3
malocclusion (common in craniofacial dysostosis patients such as Crouzon or
Apert) benefit from the monobloc advancement.
■ Orbital expansion corrects the proptosis; a bone graft is used to augment the
nose with onlay grafts to the maxilla.2,3
IMAGING
■ Although skull x-rays and clinical exam were sufficient for Dr. Tessier in
1958, we now rely on CT scans with 3D reconstructions.
■ Virtual surgical planning (VSP) is widely available.
■ VSP can help the less experienced surgeon determine vectors of movement.
FIG 1 • Francine Gourdin’s drawings of Dr. Tessier’s first
Le Fort III procedure. A. Osteotomy lines. B. Anterior
movement of the midface. C. Bone grafts in position to
stabilize the advancement. (From Wolfe SA. A Man
from Héríc: The Life and Work of Paul Tessier, MD,
Father of Craniofacial Surgery. Miami, FL: Lulu Press;
2011, with permission.)
