CRANIOSYNOSTOSIS
INTRODUCTION
 Designates premature fusion of one or more sutures in either the cranial vault or cranial base.
 Most isolated craniosynostosis are sporadic in occurrence. Reported cases of AD or AR inheritance
 Incidence 1 per 2000 births. At least 57 different conditions.
CLASSIFICATION
1. NON SYNDROMIC CRANIOSYNOSTOSES
1) Scaphocephaly (Boat skull)  Sagittal sutural synostosis
2) Plagiocephaly (oblique skull)  Unilateral coronal synostosis
3) Brachycephaly (short skull)  Bilateral coronal synostosis
4) Trigonocephaly (triangle skull)  Metopic synostosis
5) Acrocephaly/Turricephaly (topmost/tower skull)  Multiple sutures involved.
6) Oxycephaly (sharp skull)  Multiple sutures involved.
2. CRANIOFACIAL SYNOSTOSIS SYNDROMES
1) Kleeblattschadel (cloverleaf skull)  Multiple sutures involved
2) Crouzon, Apert, Saethre-Chotzen, Carpenter  Single/Multiple sutures involved
3) Other: (Goldenhar’s, Orbitofacial cleft, etc)
BASICS
 Metopic suture separates frontal bones
 Coronal suture separates the frontal, and parietal
 Squamosal suture separates parietal, greater wing of sphenoid, squamous temporal bone and occipital
 Lambdoid suture separates the parietal and occipital bones
SUTURE GROWTH
 cranium is made up of
1. neurocranium - which includes the chondrocranium of the skull base and the membranous bone of
the calvarium
2. viscerocranium that forms the membranous bones of the face.
 The various areas of the craniomaxillofacial skeleton grow by very different methods.
 Neurocranium
a. The cranial vault sutures are skeletal joints of the syndesmosis type
b. The sagital, metopic and lambdoid are formed by narrowing of membranous gaps between bones
that are initially widely separate. They overlie areas in which brain tissue does not lie close to the
surface
c. The coronal suture is different and the parietal bone can be seen to overlap the frontal bone from
the outset – this is a flexible/sliding joint.
d. Its base is formed by endochondral ossification and the joints are of the synchondrosis type. The
cartilaginous precursors form around the pre-existing cranial nerves and blood vessels. Thus
the foramina around the great vessels occur within the endochondral bones of the skull base
e. Thus endocondral ossification in the cranium exists in the petrous and mastoid process of temporal bone, occipital, ethmoid, sphenoid and Meckel’s part of the mandible
 Viscerocranium
a. zygomatic, maxillary, and palatine
i. overlapping or sliding joints where the direction of bone growth tends to parallel the plane
of the suture.
ii. This arrangement provides for adaptive adjustments to pressure in utero and early infancy.
Growth of cranial vault
 The sutures are growth sites where cells undergo proliferation and differentiation into osteoblasts.

The interlocking peg-and-socket arrangement allows osteogenesis to occur mainly at the bottom of the
socket and point of the peg (suture front) for simultaneous jointing and growth of the bone against the suture.
 The sutures produce new bone in response to the expanding neurocranium
 The dura contains osteoprogenitor cells which is not present in the periosteum layer. In very young animals, if skull is excised but dura left behind, the dura has the ability to regenerate the cranium and its sutures.
 Timing of normal suture closure
o Posterior fontanelle closed within first 3 months
o Anterior fontanelle closed about 24-30 months
o Metopic suture
 Closure commences at nasion and proceeds superiorly and concludes at the anterior fontanelle.
 closes normally as early as 3 months and complete fusion by 9 months of age (thus CT showing
complete metopic closure at >6 months should not be the decisive factor for surgery) – J Craniofac
Surg 2001
o Other sutures close after age 25, beginning in the inner table and then outer table. Occurs first in the
inferior part of the coronal suture, than posterior part of sagittal, and then in lambdoid suture.
PATHOGENESIS
 Virchow (1851) noted that growth perpendicular to the involved suture(s) ceases, while growth parallel
to the suture proceeds or over-compensates.
 Early theories of formation of craniosynostosis:
1) Primary abnormalities of the suture(s)
 shown not to be true
2) Cranial base abnormality
 Moss suggested that cranial base was the primary locus of abnormality with the altered forces being
transmitted by tensile forces to the sutures via the dura
 Did not explain nonsyndromic craniosynostosis who have a normal cranial base
Most work has since focused on the role of the dura. Likely mechanisms:
1) Primary synostosis: Abnormalities of the dura or its attachments
 Early in life, dura initially plays an inductive role
 Later it assumes a permissive role in maintaining suture patency through various signalling factors
 Primary craniosynostosis involves failure of the signalling system that governs the growth and
differentiation at the sutural margins.
 Mediator factors include fibroblast growth factor receptor, TWIST, MSX2 and transforming growth
factor
 FGFR2 and TWIST mutations results in gain-of-function, leading to premature osteoblast
differentiation and cranial suture fusion.
 In most cases, fusion occurs soon after formation in utero
2) Secondary synostosis
a. Inadequate expansive forces exerted by the brain or excessive external pressure
i. As cranial growth is due to brain expansion, thus lack of brain growth in microcephaly
or reduced ICP from shunting may lead to craniosynostosis
ii. Severe constraint in utero
1. In nonsyndromic synostosis, where mutation of all known genes have been excluded, right coronal is affected twice as commonly as the left, correlating with
a laterality nias in head orientation prior to and during delivery
2. not universally accepted, some suggests it causes deformational plagiocephaly
instead.
b. Metabolic bone disorder
i. Hyperthyroidism
ii. Hypercalcemia
iii. Vit D deficiency
iv. Hurlers syndrome – mucopolysaccharidosis
FUNCTIONAL ASPECTS
1)









Raised Intracranial Pressure (> 15 mmHg)
Clinical signs: headaches, difficulty sleeping and irritability
Tessier and Marchac have both found that RICP occurs with craniosynostosis.
Among the non-syndromic children, a direct relation between the number of sutures involved and raised
ICP is documented.
Marchac found RICP in 14% of patients with single suture involvement (McC in 2%), versus 42% with
multiple sutures involved
If the coronal suture is affected, RICP is more likely than if other sutures involved.
Volume studies have shown that overall intracranial volume is not reduced in craniosynostosis. Surgery
seems to be beneficial by redistributing the volume.
Focal perfusion defects in the brain were found to be corrected after surgery.
Persistent postoperative raised ICP seen in 6-15% of patients with craniofacial synostosis.
It must be related to the multifactorial etiology of increased ICP in these patients, which includes:
1. cerebral venous congestion
2. upper airway obstruction
3. hydrocephalus.
2) Hydrocephalus
 True incidence unknown. Seems to be more common in craniosynostosis.
 Sagittal and uni- and bilateral coronal suture involvement seems to have the lowest incidence of hydrocephalus.
 When multiples sutures involved, the incidence is higher.
 Common in Crouzon’s and Apert’s.
3) Visual Abnormalities
 Optic atrophy and papilloedema are not uncommon, especially at an early age.
 Papilloedema is reflective of RICP and more common in Crouzon’s, Apert’s, acrocephaly and oxycephaly.
 Optic atrophy more common with the more severe synostoses and rare with the simple synostoses. Multiple aetiologies have been proposed: bony overgrowth compressing the nerve; stretching of the nerve; compression by carotid vessels; secondary to papilloedema from RICP.
 If orbital hypertelorism present, binocular vision may not develop and amblyopia may result. Plagiocephaly can also affect vision.
4) Neuro-psychiatric Disorders
 Range from mild behavioural disturbances to severe mental retardation, possibly secondary to cerebral
compression.
 Psychological problems can arise from the cosmetic deformity.
 Teasing and taunting frequently occurs and this can compound slow learning, poor motor skills and generally bad school performance.
 With correction of the deformity, self esteem, self confidence, behaviour, inhibition and functioning improve, but social interactions may still remain poor.
 Evidence suggests that neurocognitive impairment is independent of whether surgery is performed, over
50% of these children have learning difficulties. Rate of mental retardation is 2-3x higher.
 PRS Aug 2005 – incidence of speech, cognitive, behavioural problems in nonsyndromic craniosynostosis
– 61% right unilateral coronal, 55% bilateral coronal, 57% metopic, left coronal 52%, lambdoid 44% and
sagittal 39%
5) Mental Retardation
 Incidence of mental retardation difficult to determine as previously many of these children were condemned to an institution and allowed no normal social interaction.
 Nevertheless, the risk of mental retardation is higher than the general population.
 Mental retardation has been attributed to a number of factors:
1) Unrelieved RICP with resultant cerebral at4) Meningitis
rophy
5) Prematurity
2) Hydrocephalus
6) Family Hx of mental retardation
3) Associated intracranial anomalies
 According to Renier and Marchac: Single suture synostosis has a lower incidence of mental retardation
than if multiple sutures involved (except metopic suture involvement).
 Apert’s and Kleeblattschadel have the highest incidence of retardation.
CLINICAL
Deformational plagiocephaly
 Due to positional deformation – increase in incidence with SIDS campaign
 Distinguish from secondary deformation plagiocephaly due to torticollis
1) Muscular
a. Head turned away from the side of the lesion – therefore contralateral occipital flattening
2) Skeletal
3) Ocular
a. CN VI palsy – head turns contralateral to obtain binocular vision
 parallelogram-shaped head, which differs from unicoronal or unilambdoid synostosis
 best treated with positioning, frequent head turning, and helmet therapy. Keeping the child in the prone
position while awake.
7
Unilateral coronal synostosis
8
 (Gr: ‘crooked head’). Commonest in McC series: 57 cases.
Asymmetrical appearance with forehead flattened on affected side.
 Ipsilateral brow and orbit are elevated on the affected side.
 The ear on the more affected side is anterior and superior.
 Due to growth restrictions of the sphenoid bone,
 Ipsilateral orbit is narrower and taller
 Anterior skull base deviates to the affected side
 Nasal tip usually deviated to the affected side.
 On the contralateral side, persistent growth causes frontal
bossing, infero-lateral orbital dystopia and bulging of the
occipital prominence.
 Craniofacial scoliosis can occur in severe cases.
 Plagiocephaly can occur without coronal synostosis in
craniofacial microsomia and muscular torticollis. Torticollis often associated (14/57 in McC series).
 Harlequin sign is evident radiologically due to elevation
of the lesser wing of the sphenoid.
 Recently differentiated into synostotic, compensational
and deformational (Mulliken)
 Treatment is ACVR and FOA
Bilateral coronal synostosis (Brachycephaly )
 Sagittal shortening of the skull with retropositoned lower
forehead and supra-orbital bar.
 occipital region is flattened
 Reduced AP dimension and compensatory  in bitemporal
distance.
 Recessed orbital rims
 Supraorbitale is usually 2mm ventral to the corneal plane – here it lies posterior – this leads to exorbitism.
 Crouzon’s and Apert’s usually have brachycephaly, thought to be on the basis of cranial base synostosis.
 May be associated with maxillary retrusion, but underdevelopment of the midface is unusual.
 Surgery is ACVR and FOA
Sagittal sutural synostosis (Scaphocephaly)
 Transverse narrowing of the skull with A-P elongation
 Boat shaped head – various degrees depending on whether only anterior
or posterior part is fused, or the entire suture
 frontal bossing and occipital protrusion
 Commonest of the isolated sutural synostoses (60%)
 M>F 4:1
 6-10% familial
 15% have RICP
 Many options for treatment
1) Strip craniectomies
a. Variable results
b. Required prolonged helmet moulding
c. Does not shorten the AP dimension or address frontal bossing
9
d. One variation is to use spring loaded devices as a form of internal distraction
2) Pi procedure
a. Routine at PMH
b. Some reports of association with elevated ICP
c. Best to perform around 9 months of age – best compromise between risk of relapse and
skull malleability.
3) Total cranial vault remodelling
Trigonocephaly  Metopic sutural synostosis
 10-20% of craniosynostosis
 M>F 3:1
 Triangular or keel shaped forehead.
 Visible and palpable midline frontal ridge
 Leads to interorbital hypotelorism - reduction in intermedial canthal distance
and interdacryon distance (between the lacrimal crests), but true/orbital hypotelorism (distance between centroids of the globe) does not exist.
 May be associated with bi-temporal narrowing and hypotelorism.
 10% risk of raised ICP
 Up to 50% incidence of cognitive impairment – not correlated with severity of
involvement (PRS May 2005)
 Traditional treatment of choice is frontoobital advancement to widen the bitemporal narrowing (increase
the central angle – angle between both halves of the frontoorbital bar) and bone graft in the midline to increase the interdacryon distance
 McCarthy does not recommend surgery for mild trigonocephaly with no functional disturbance as risk of
surgically induced complications, including failure of frontal sinus development, outweighed the potential
benefits of surgery.
 Surgery often improves the keel shape but undercorrects the interorbital hypotelorism
 In general, surgical correction of metopic synostosis was associated with the best esthetic results of all the
isolated craniosynostoses
 Minimal access (endoscopic assisted) surgery has been described (Clin Plas Surg Jul 2005)
 To minimise blood loss, length of stay, large scalp incisions
 Require post operative moulding with helmet
Lambdoid synostosis (Posterior plagiocephaly)
 Rare
 Need to distinguish from deformational plagiocephaly
 Contralateral occiput protrudes
 Frontal bossing is not a typical feature, but when it occurs it is contralateral
 Ipsilateral ear and mastoid pulled posteriorly
 Treatment is posterior cranial vault remodelling
Multiple suture synostosis(Turricephaly)
Turricephaly
 Untreated brachycephaly (Apert’s and Crouzon’s) may go on to turricephaly (towering head)
 Characterised by excessive skull height and a vertical forehead
10
Oxycephaly
 Pointed head. Usually due to fusion of multiple sutures.
 The forehead is retroverted and tilted back in continuity with the nasal dorsum. Forehead usually reduced in horizontal dimension.
Kleeblattschadel
 Cloverleaf skull. Usually results from pan-sutural synostosis although can
occur without synostosis.
 Bitemporal and vertex bulging.
 Usually requires early aggressive treatment to prevent cerebral compromise.
Non syndromic Pansynostosis
 Non syndromic synostosis of 3 or more cranial sutures.
 Incidence of RICP is high when so many sutures are fused (McC: 5/7  1 VPS).
 May require an early radical vertex craniectomy (McC: 4 months) to stabilise the patient and relieve the
RICP (which presumably is not controlled by VPS).
 Cranial vault remodelling procedure: FOA + calvarioplasty.
Clinical assessment
 History
o Pregnancy problems
o Family history
o Drugs, smoking
o Illness (viruses)
 Examination
o Head circumference
o Head shape
o Globe exposure
o Facial symmetry
 Forehead
 Brow
 Orbits
 Intercanthal distance
 Nasal tip
 Chin point
 Neck - torticollis
o Other systems
 Hands, feet, spine, chest
Investigations
1. Imaging
a. xrays
i. not sensitive
11
2.
3.
4.
5.
b. US
i. Better than xray but need a specialist sonographer
c. CT
i. Gold standard
d. MRI
i. helps to delineate the pattern of cortical gyri and sulci underneath the fused suture – whether this
is an indicator of RICP is debated
ii. Hindbrain herniation has been observed in as many as 70% of Apert and Crouzon patients caused by early closure of the lambdoid sutures.
Blood tests
a. Order thyroid, calcium and parathyroid studies when associated features suggest these diagnoses
Neurodevelopmental analysis
a. pre and post op measurements useful
b. Bayley Scales of Infant Development II most commonly used
ICP measurement
 No reliable indirect indicators exist for intracranial hypertension.
 Papilledema is rarely seen in children with craniosynostosis, even in the presence of intracranial hypertension. Among patients with verified intracranial hypertension, only a small proportion (16-25%) have
papilledema.
 On the other hand, a late presentation of craniosynostosis can lead to irrecoverable optic atrophy and
visual failure due to sustained intracranial hypertension.
 A copper-beaten appearance of the skull xray does not correlate well with the level of ICP.
 Previous studies have demonstrated the relative value of transcranial Doppler. The practicalities of performing such an examination are so complex that they prevent the routine use of Doppler in very young
patients.
 Recent work has indicated that the tympanic membrane could be used for ICP measurement.
 The current techniques of measuring ICP are invasive, employing subdural or intracerebral
transducers and having a small but appreciable complication rate.
Cervical spine anomalies
 Common in craniosynostosis:
 71% of children with Apert’s, usually C5-6 level.
 1/3 of children with Crouzon’s and Pfeiffer’s, usually high.
 These can have implications with airway management especially during anaesthesia
HISTORY OF TREATMENT OPTIONS
 Previously much debate about timing and many surgical options.
 Recent trends have been towards early surgery (< 1-1.5 years) with the aim of getting the child to look as
good as possible as early as possible to spare the child social and psychological trauma.
 PMH – 3-6 months for sagittal (tend to get worse plus head shape can be moulded by sleeping supine) and
6-9 months for the rest.
 The goals of surgery are:
1) to decompress the intracranial space to reduce ICP, to prevent visual problems and to permit normal
mental development
2) to achieve satisfactory craniofacial form
3) (to optimise growth)(my addition)
Tessier’s Principles
1. Adequate exposure through inconspicuous incisions.
12
2. Eyes can be transposed forward without impairing vision.
3. Large segments of the craniofacial skeleton can be extensively removed, osteotomised and translocated
without altering bone viability despite devascularising the bone.
4. Autogenous bone grafting is critical for filling defects, contour and stability.
5. Rigid skeletal fixation must be attained.
6. Multidisciplinary team and experienced surgeon are prerequisates.
RECENT ADVANCES IN CRANIOFACIAL SURGERY
 These include
1. the work of Tessier (the founding father of craniofacial surgery)
2. miniplate and screw fixation
3. radiographic improvement (3D CT, MRI)
4. distraction osteogenesis
5. the use of microvascular techniques
6. the application of TE to craniofacial surgery
7. an improved understanding of alloplastic materials.
TIMING
Advantages of Early Surgery (< 18 months)
1. craniofacial function or prevent deterioration in function
 Neurological
 Opthalmological – exorbitism, strabismus, amblyopia
 psycho-social development
2. craniofacial form (cosmesis): both of the upper face (vault, forehead, orbit) and of the lower face (nose,
maxilla, zygoma).
3. growth normalisation
 less deformation of cranium and face
 Most brain growth occurs in the first year of life.
 A delay in surgery beyond the first 9 to 12 months of life leads to progressive deformity of the cranial
base, resulting in abnormal facial growth and asymmetry of the maxilla and mandible.
4. Easy malleability of bone
5. rapid re-ossification of large defects
Disadvantages of Early Surgery
1. Possible ed operative stress.
2. Bones may be more fragile.
3. Possible deliterious effect on subsequent craniofacial growth and development.
 Optimal age controversial. Early surgery indicated for multiple suture involvement and for sagittal suture
synostosis (strip or extended vertex craniectomy < 3 mths). Severe deformities require early surgery.
When single sutures are involved, there is a trend towards later surgery (6-12 mths) as the risk of RICP is
low. Some delay in selected cases (eg Apert’s) to  the need for secondary revisional operations.
 When mid face surgery is also required, it has been shown that a staged approach (FOA and later Le Fort
III maxillary osteotomy) is safer than monobloc advancement of forehead and midface. Others favour a
single stage operation.
 Timing still a controversial issue especially wrt to the midface. If early surgery done, later revision is often necessary, but if it is not done the patient has to suffer the psychological and other effects of the deformity for longer.
13
SURGICAL OPTIONS
Early Surgery
1. Strip Craniectomies
2. Fronto-orbital advancement
3. Cranial vault remodelling
4. Barrel stave osteotomy
5. Monobloc advancement (forehead and either
orbits or orbits and midface)
Late Surgery
1. Le Fort III advancement
2. Le Fort I and II osteotomies
3. Onlay grafts
4. Zygomatic osteomies
5. Rhinoplasty, genioplasty
6. Monobloc midface and frontal advancement
(frontal and midface)
EARLY SURGERY - OPTIONS
1. Strip Craniectomies
 Originally reported late 1800s. Popular by the 1920s. Now mostly out of favour.
 Linear craniectomy along the course of the stenosed suture adequately decompresses the brain, but fails to
give satisfactory craniofacial form (except isolated sagittal synostosis) and recurrent bony re-closure of the
craniectomy site is common.
 Traditionally done before the age of 3 months.
 In isolated sagittal synostosis, either sagittal (patient < 3 months), or para-sagittal (patient > 3 months)
strip craniectomies may still be indicated. Vertex craniectomies seem to be even better at preventing recurrence.
 In bilateral coronal synostosis, bilateral coronal strip craniectomies have been replaced by FOA. McC
states that if mild, bilateral coronal synostosis with minimal, if any, evidence of exorbitism is present, an
extended coronal-spheno-zygomatic strip craniectomy may be indicated.
 For plagiocephaly, trigonocephaly, or the craniofacial synostosis syndromes, simple strip craniectomies
have been abandoned.
2. Fronto-Orbital Advancement (FOA)
 Recommended by Tessier (1971, 1979).
 Indications:
1) Bilateral coronal synostosis and a moderate degree of exorbitism
2) Craniofacial synostosis syndromes (Crouzon’s, Apert’s, etc)
 Osteotomy lines are extended along the lateral orbital wall to accomplish wide bony resection in the region
of the spheno-zygomatic suture.
 Easier to do in the infant than the adult. McC recommends doing it at 6 months of age.
 Bicoronal incision and flap turned down. Frontal bone removed. Osteotomies across the nasofrontal junction, across the roof of the orbit and along the lateral orbital wall. Tongue and groove arrangement extends into the temporal fossa to allow advancement without the need for bone grafts. The supra-orbital
bar, thus freed, is advanced as required to  the orbital and cranial volumes. The frontal bone flap, or
parieto-occipital bone graft, is wired to the supra-orbital bar after appropriate remodelling. Closure of the
scalp may be difficult after significant advancement, but is facilitated by parallel incisions in the galea.
 Marchac modified the technique slightly. Instead of doing a tongue in groove in the temporal region, he
performed an osseous z-plasty by cutting a V in the bone behind the lateral orbital rim which came to lie in
front of the lateral orbital margin after advancement. Marchac also uses the floating forehead method, attaching it only to the supra-orbital bar. He only does this between 2 and 4 months, not after.
 Studies (3D CT) have shown significant es in intracranial volume and marked cosmetic improvement
after FOA.
14
 For plagiocephaly, FOA can be done unilaterally (osteotomies must extend across the midline to avoid a
central depression), although in most cases, plagiocephaly is treated by conventional bilateral FOA. This
technique has been abandoned by McC, but is advoacated by Marchac.
 The supra-orbital arch is normally flat on the affected side in plagiocephaly. This is treated by either bending the bone or onlay bone grafts. Over-correction should be done.
3. Cranial Vault Remodeling
 For turricephaly, addition cranial remodeling may be required. FOA is done and, in addition, a radical
calvariectomy is performed bilateral leaving a central strip of bone over the sagittal sinus. A segment of
the strip is resected allowing advancement of the anterior part to meet the previously advanced frontal
bone segment. The L and R pieces of the radical calvariectomy are re-shaped and replaced.
 This results in sagittal advancement and vertical reduction.
4. Barrel Stave Osteotomy (Persing et al, 1987)
 An alternative to cranial vault remodeling, also for turricephaly.
 360o circumferential removal of the whole cranial vault. From the excised piece, bone is removed from 4
quadrants, leaving 4 “spokes”. Frontal bone (brow and orbital roof) is advanced to  the AP dimension.
The dura is plicated. The pieces are removed, remodeled and replaced with dural suspension sutures.
McC now likes this method.
5. Monobloc
a) Craniofacial Advancement (Muhlbauer, Anderl and Marchac, 1983)
 Indicated for infants with severe craniofacial synostoses syndromes with respiratory distress, exorbitism,
midface retrusion and corneal exposure.
 Monobloc or simultaneous advancement of forehead, orbits and midface.
 A potentially dangerous procedure recommended only for infants with combined respiratory and ocular
threats. Risk of infection is high - direct communication of nasal cavity and sinuses with anterior cranial
fossa.
 McC rather recommends trachy for respiratory obstruction and conventional FOA.
b) Monobloc advancement of orbits and midface (Ortiz-Monasterio et al):
 Osteotomy lines as for combined FOA and Le Fort III, except fronto-nasal and Z-F are spared of osteotomies.
 Allows simultaneous hypertelorism correction, but does not provide much expansion of intra-orbital volume.
 Does not correct anterior open bite.
 Major procedure with blood loss.
 Infection risk as the nasal fossae and anterior cranial cavities communicate.
15
Anterior (tongue in groove) CVR and Monobloc advancement
FIXATION METHODS
 Wires are generally advocated as miniplates are known for their passive interosseous transmission through
bone and possibly  growth.
 If Marchac uses miniplates, he removes them a few months later.
 Absorbable plates has revolutionised the surgery
 Studies have documented that the rigidity offered by absorbable plating devices is equivalent to that of titanium devices.
 The only drawback to this system is that absorbable screws must be tapped
 Hydroxyapatite may be used to fill defects
LATE SURGERY
 After 12-18 months.
Le Fort III Advancement Osteotomy
 First reported by Gillies ad Harrison (1950).
 Modified by Tessier (1967) who performed the osteotomy in the classic Le Fort III site.
Method (as described by McC):
 Trachy best, but can be done using a nasal tube of rigid fixation used and the tube is left in situ (ICU) for
24 hours.
 Approached via 3 incision: bicoronal, conjunctival or sub-ciliary, buccal sulcus.
 Can avoid the eyelid incisions, but this makes the procedure more difficult.
 Scalp flap raised. Periorbita freed from the 4 walls. Root of the nose exposed. MCL left intact, but lacrimal sac freed.
 Osteotomy starts at the nasal-frontal angle and proceeds horizontally backwards on to the upper part of the
medial orbital wall. From here, the osteotomy is directed down the medial orbital wall, behind the lacri-
16
mal sac and groove to the floor of the orbit. A transverse cut is made across the orbital floor from the medial wall to the inferior orbital fissure.
 The lateral rim of the orbit is osteotomised in the region of the Z-F suture. The osteotomy is carried
backwards in the orbit and then down to meet the inferior orbital fissure at the site of the previous osteotomy. The zygomatic arch is sectioned. The pterygo-maxillary fissure is sectioned (posterior to the maxillary buttress). The posterior nasal septum is divided.
 Rowe disimpaction forceps is used to loosen the anterior segment which is then advanced anteriorly and
tilted/rotated to face downwards.
 Bone graft is placed in the defects at the naso-frontal junction, lateral orbital wall and pterygo-maxillary
fissure. Wires or plates are applied at the Z-F, the naso-frontal region, the arch and the buttress and IMF is
applied.
Modification:
 Tessier modifies the procedure by doing an osseous z-plasty in the region of the Z-F. Instead of cutting
straight across, he cuts a bony spur, ^-shaped, which he then advances forward of the remaining lateral orbital margin.
Timing:
 Traditionally, Le Fort III was reserved for the adolescent and adult patient.
 McC advocates doing it prior to starting school so that the child can look normal.
Additional procedures
 Additional procedures can be done a the same time: onlay rib grafts to the supra-orbital (brow) region,
genioplasty, rhinoplasty, etc.
 Combined with Le Fort I osteotomy: Indicated in patients in whom the deformity is restricted to the upper midface and who have a normal dental occlusion. The Le Fort I osteotomy is made superior to the
dental roots but below the infra-orbital nerve. It extends from the piriform fossa, round the buttress to the
pterygo-maxillary fossa.
 Combined with FOA is a major procedure that may be indicated in selected cases.
Le Fort II Osteotomy
 Indicated in the patient with midface hypoplasia and adequate zygomatic projection.
 Osteotomy lines: Across the naso-frontal area, backwards, behind the lacrimal groove, down the medial
oribital wall, across the inferior orbital rim medially, medial to infra-orbital foramen and back to pterygomaxillary fissure.
Other Procedures
 Jaw surgery may indicated in adolescent years: Le Fort I or II.
 Microgenia is often a problem requiring genioplasty.
 Zygomatic advancement or onlay for improved cheek contour.
 Rhinoplasty
 Revisional surgery: FOA, monoblock advancement
MANAGEMENT RECOMMENDATION
 McC proposes early surgery (PRS, Aug 1995) - see timing, below.
 The current New York University protocol as outlined by him is as follows:
Neonatal Period (Mnemonic: AFIE, FOA, Le Fort, Jaw)
1) Airway - Tracheostomy - indicated if severe resp distress unresponsive to conservative Rx.
2) Feeding - Gastrostomy - indicated if severe feeding problems unresponsive to conservative Rx.
17
3) Investigate for RICP - U/S and/or CT scan to evaluate whether RICP  VPS if indicated. Calvariectomy for severe RICP/hydrocephalus (as in pansynostosis).
4) Eye care if corneal exposure secondary to exorbitism:
 eye lubricants,
 mist cups,
 eyelid taping,
 tarsorrhaphy
Age 6 to 9 months
1) Fronto-orbital advancement and/or cranial vault remodeling.
 In the syndromic patients this may be required to be more extensive in the parieto-occipital region. Parents should be forewarned of the possible need for secondary surgery, especially in the Apert’s and
Pfeiffer’s patients.
 Marchac does his FOA at an earlier age: 2.5 months.
Age 4 years
1) Le fort III midface advancement indicated if:
a) respiratory problems, or,
b) aesthetic/cosmetic problems.
Adolescence
1) Jaw surgery (if indicated for midface hypoplasia):
a) Le Fort I or Le Fort III advancement,
b) Maxillo-mandibular osteotomies,
c) Genioplasty.
 McC reviews his 20 year experience (1973-1992) of 363 patients. Of these, 180 had surgery before the
age of 18 months. 104 had isolated craniofacial synostosis. 76 had syndromic or pansynostosis.
 Prior to 1975, strip craniectomies were done; after 1975, fronto-orbital advancement.
 In the unilateral coronal synostosis group (the plagiocephalies), he went through a phase of doing unilateral fronto-orbital advancement. This he found inferior to bilateral fronto-orbital advancement, which he
now uses on all his patients.
 To evaluate his results, he categorised his patients according to the method proposed by Whitaker, et al:
I. No refinements or revisions advisable or necessary .
II. Soft tissue or lesser bone contouring revision advised. Can be done with < 2 day hospitalisation
(often as OPD).
III.Major osteotomies or bone graft advised, but this secondary procedure not as extensive as the
primary surgery: Orbital repositioning, Onlay bone graft, Le Fort (I, II, III) advancement.
IV.Major cranio-facial procedure as big, or bigger, than the original procedure advised.
Isolated cranio-facial
Syndromic or pan craniosynostosis
facial synostosis
n
104
76
87,5%
73.7%
Satisfactory outcome
(Whitaker I & II)
13%
36.8%
Secondary surgery
0
5
Tertiary surgery
0
65%
Le Fort III osteotomy and
(others too young still)
advancement
<5%
11.3%
Peri-operative Cx
7.7%
44.7%
Cx at follow up:
3,8%
42.1%
 hydrocephalus
18


VPS
Seizures
1%
2,9%
22.4%
11.8%
In the isolated cranio-facial synostosis group, the plagiocephalies give the most problems:
1. vertical orbital dystopia, strabismus, amblyopia,
2. nasal tip deviation,
3. residual cranifacial asymmetry
COMPLICATIONS OF CRANIOFACIAL SURGERY
 High potential for Cx in craniofacial surgery.
 Improved anaesthesia and ICU has lowered the Cx rate and allowed more complex surgery.
 Cx can be divided into 3 categories:
1) potentially life threatening (+9%)
2) other serious Cx (+12%)
3) minor Cx
 Overall Cx rate: 14.3-16.5%. ed with surgeon’s experience,  op time, hypotensive anaesthesia, fewer
incisions, AB therapy, rigid fixation, avoidance of trachy.
I) Death - Mortality rates vary at about 1% (0.6-1.6%). Intracranial approach associated with a higher mortality rate. Causes of death: blood loss and hypovolaemia, cerebral oedema, respiratory obstruction, meningitis and other infections, sagittal sinus thrombosis, intracranial haematoma, etc.
II) Operative Complications
1) Bleeding: Can be considerable. Often an ooze that arises from dura, bone or arises from a tear in the
sinus and is difficult to control. Post-op drainage should be by gravity and not suction.
2) Dural tears: These, in association with the cerebral oedema that occurs with surgery, can result in herniation of brain and thus all tears should be repaired or patched.
3) Bradycardia: Secondary to oculo-cardiac reflex.
4) Anaesthetic related Cx
III) Post-operative Complications
EARLY
1) Bleeding:
Outside the cranium: external, subgaleal.
Inside the cranium: extradural, subdural, subarachnoid, i/cranial.
2) Infection: wound, bone, intracranial. The most frequent Cx (4.4-6.5%). More of a problem in adults
than children. Worse with intracranial procedures, large dead space, if communication with nasal cavity or sinuses. Pseudomonas most common. To  the incidence of infection: AB, wash, flaps to close
off dead space and communications with nasal cavity and sinuses.
3) Cerebral oedema, RICP, hydrocephalus
4) Seizures: some recommend prophylactic anticonvulsants
5) CSF leak: requires dural patch or shunt if it persists.
6) SIADH
7) Ocular problems: blindness, diploplia, corneal ulceration, III n palsy, lacrimal obstruction.
8) Respiratory problems
LATE
1) Developmental delay
2) Persistent neurological defects (Marchac’s Apert’s: (n=53) 72% had IQ < 80)
3) Cosmetic - poor craniofacial form d/t displacement, resorption, poor growth, failure to develop a
frontal sinus.
4) Psychological problems
5) Obstructive sleep apnoea (Rx: Le Fort III osteotomy and midface advancement; T&A)
19
OUTCOME
 Paucity of long term studies.
 Need to look at craniofacial form, mental and social function, occlusal status, etc.
 Growth of the advanced segment must be examined.
 Crouzon’s, Apert’s and the other syndromes remain the most difficult to treat.
 In general, Apert’s and Pfeiffer’s do worse than Crouzon’s both aesthetically and wrt Cx and RICP.
 FOA does not help the midface and patients often need later Le Fort advancement.
 According to McC, there are 3 main unsolved problems that occur, especially in the syndromic group:
1) Cranial vault maldevelopment resulting in anterior turricephaly
2) Failure to prevent midface hypoplasia ( aesthetic problems, obstr sleep apnoea)
3) ed frontal sinus development ( flat brow and forehead). According to Marchac, not a problem.
 Of the future, McC states that greater emphasis will be placed on the occiput in the hope of preventing the
turricephaly deformity.