this is the seminar on craniofacial anomalies and Cranisynostosis Included details about lefort III distraction osteogenesis and cranioplasty.

1. Craniofacial Anomalies & Craniosyostois
Presenter:
Dr. Surendra Acharya
JR, OMFS
CDER, AIIMS

2. Brief development of face and skull
Introduction
Classification
Etiology
Cranisynostosis
Screening and pre op evaluation
Management
Complications
Conclusion
References

3. Embryogenesis of face and skull

4. Embryogenesis of face and skull

5. Embryology of face and cranium

6. Embryology of face and cranium

7. Embryology of face and cranium

8. Embryology of face and cranium

9. Embryology of face and cranium

10. Embryology of face and cranium

11. Growth And Development of Cranifacial
Complex: An Epigenetic Viewpoint
The functional matrix theory:
Cranial vs. cephalic.
Functional cranial component(FCC): functional matrix(FM)
+ skeletal unit(SU).
Functional matrices: Periosteal and capsular(Tenon’s
capsule).
Active and passive growth.
The classical triad of cranial growth process:
Role of suture
Role of deposition and resorption
Role of cephalic cartilage
Neural regulation of functional matrices

12. Craniofacial anomalies
Malformation:
A morphological defect of an organ, part of an organ, or larger
region resulting from an intrinsically abnormal
developmental process.
The risk of recurrence depends on the etiology of the
malformation.
Congenital anomalies:
All forms of developmental defects present at birth, whether
caused by genetic, chromosomal, or environmental factors.
Dysmorphology:
The study of congenital anomalies. The term dysmorphic
describes an individual with obvious multiple and severe
malformations.

13. Craniofacial anomalies
Sequence:
Pattern of multiple anomalies derived from a single structural
defect or mechanical factor (Mobius sequence).
Developmental field:
A group of cells or a region that responds as a coordinated
unit to embryonic interaction; defects in developmental fields
result in multiple malformations (formation of the lens
depends on interaction with the optic cup). Many
malformations are field defects.
Syndrome:
Pattern of anomalies thought to be pathologically related. The
term implies a single cause, not a field defect.

14. Anomalies:
Something that deviates from what is standard,
normal, or expected.
Craniofacial abnormalities:
Are birth defects of the face or head, affecting 2-3%
of all babies. Some, like cleft lip and palate, are
among the most common of all birth defects. Others
are very rare. Most of them affect how a person's
face or head looks.
Craniofacial anomalies

15. Craniofacial anomalies
‘‘Craniofacial anomalies (CFA) are a diverse group of
deformities in the growth of the head and facial bones.
Anomaly is a medical term meaning "irregularity" or
"different from normal." These abnormalities are congenital
(present at birth) and there are numerous variations- some are
mild and some are severe and require surgery’’.

16. Craniofacial anomalies
Congenital anomalies (CA) are a major cause of infant mortality
and childhood morbitity, affecting 2-3% of all babies.
Approximately 1% of these newborns have syndromes or multiple
anomalies; CFA are often a component part.
Syndromes that have cleft lip and/or cleft palate as one of the
features are of interest in the quest for etiologic and pathogenetic
factors, and it is estimated that 30% of cleft cases are syndromic.
Conversely, therefore, approximately 70% are non-syndromic

17. Craniofacial anomalies

18. Craniofacial anomalies
Proposed new classification system: Based on extensive
experiences in treatment of craniofacial anomalies,
classification suggested as below:
1. Cleft: centric and acentric
2. Synostosis: symmetric and asymmetric
3. Atrophy: hypoplasia
4. Hypertrophy: neoplasia
5. Unclassified

19. Classification of craniofacial
anomalies
Craniofacial anomalies can be broadly divided into three
main subgroups:
Craniofacial clefts
Craniosynostoses
Hemifacial microsomia
Deformational plagiocephaly
Treacher collins syndrome
Miscellaneous craniofacial anomalies

20. Craniofacial anatomy

21. Cephalic index

22. Cleft lip and palate
Cleft lip and/or cleft palate
Cleft lip and cleft palate are the most common congenital
craniofacial anomalies seen at birth.
Cleft lip.
An abnormality in which the lip does not completely form. The degree of the
cleft lip can vary greatly, from mild (notching of the lip) to severe (large opening
from the lip up through the nose).

23. Cleft palate

25. Kernahan and Stark classification:
1.Primary palate includes those structures anterior to the
incisive foramen (lip, pre-maxilla, anterior septum).
2.Secondary palate includes those structures posterior to the
incisive foramen (lateral palatine shelves, soft palate, and
uvula)

26. Timing of cleft lip and palate
repair
1. Age 3 months - Repair of CL (and placement of
ventilation tubes)
2. Age 6 months – Pre surgical orthodontics, if necessary;
first speech evaluation
3. Age 9 months - Speech therapy begins
4. Age 9-12 months - Repair of CP (placement of ventilation
tubes if not done at the time of CL repair)
5. Age 1-7 years - Orthodontic treatment
6. Age 7-8 years - Alveolar bone graft
7. Older than 8 years - Orthodontic treatment continues

27. Craniosynostosis
The premature fusion of the sutures between the
growth plates in an infant's skull that prevents
normal skull expansion which can cause an
abnormally shaped skull. Premature closure of all
the sutures can cause microcephaly (an abnormally
small head), which prevents the normal growth of
the brain and results in mental retardation.

28. Craniosynostosis

29. Craniosynostosis
1. Primary:
a. Isolated (mostly sporadic)
b. Associated anomalies:
I. limb defects(mostly single gene
defect)
II. Limb + others defects (single gene
+ chromosomal syndromes)
2. Secondary:
a. CNS malformation
b. Metabolic disorder
c. Others disorder

30. Types of craniosynostosis
Primary craniosynostosis : found as an idiopathic
developmental error occurring in otherwise normal
individuals.
It also occurs as part of complex syndromes involving
other developmental aberrations; such syndromes often
show Mendelian inheritance.
It should be noted, however, that there is no familial
incidence in the large majority of cases of primary
craniosynostosis.

31. Types of craniosynostosis
2. Secondary craniosynostosis :A failure of brain growth
as in microcephaly or an encephaloclastic process
occurring during the first years of life will result in
premature fusion of the cranial sutures.
A similar process may also be seen when severe
hydrocephalus has been treated with a low-pressure
shunt. Metabolic craniosynostosis

32. Types of craniosynostosis
3. Metabolic craniosynostosis : results from premature
sutural fusion determined by obvious biochemical
disorders such as the mucopolysaccharidoses, rickets,
hypophosphatasia or hypercalcaemia.

33. Symptomatology of craniosynostosis
 Raised intracranial pressure
 Exorbitism and orbitostenosis
 Orbital hypertelorism
 Orbital hypotelorism
 Orbital dystopia
 Midface hypoplasia
 Airway restriction
 Effects on speech
 Impair normal mastication.

34. Ocular complications
Proptosis or exophthalmos
Increased inner and outer canthal distances
Increased interpupillary distance palpebral fissure size
position of the lacrimal puncta obliquity of the
palpebral fissure and asymmetry of orbits and orbital
structures.

35. Some common syndrome with
Craniosynostosis
I. Single gene disorders:
Autosomal dominant:
1. Crouzon syndrome (coronal, Sagital)
2. Apert syndrome (coronal, sagital and lamdoid
3. Pfeiffer syndrome: type 1(coronal and sagital),
type 2- all sutures
type 3- all sutures
Autosomal recesive:
1. Carpenenter syndrome- all sutures

36. Some common syndrome with
Craniosynostosis
II. Chromosomal disorders:
1. Duplication 3q syndrome- coronal
2. Deletion 13q syndrome- metopic
III. Teratogenic syndrome:
1. Valporic acid embryopathic- all
2. Fetal aminopterin syndrome- coronal and lambdoidal

37. Craniosynostosis
PATHOGENESIS:
The causes of craniosynostosis are known to be
heterogeneous:
Chromosomal
Monogenetic and teratogenic syndromes;
Nutritional deficiency
Other syndromes of unknown genesis.

38. Craniosynostosis
PATHOGENESIS:
Most cranial and facial sutures do not close until
adulthood, but a few synostose spontaneously early in
development (premaxial, maxiofacial and metopic).
The reason for this predictably age-related synostosis is
unknown, but it has been proposed to be related to the
functional environment of a particular suture and the need
for adaptive skeletal change in the craniofacial area.

39. Causes for craniosynostosis
Complex Multisuture
Synostosis
Secondary Synostosis
Hematologic disorders • Congenital hemolytic icterus
• Polycythemia vera
• Sickle cell anemia
• Thalassemia
Iatrogenic disorders • Hydrocephalus with shunt
Malformations • Encephalocele
• Holoprosencephaly
• Microcephaly
Metabolic disorders • Hyperthyroidism
• Rickets (various forms)
• Mucolipidosis III

40. Causes for craniosynostosis
Complex Multisuture Synostosis Secondary Synostosis
Mucopolysaccharidoses and
related disorders
• D-mannosidase deficiency
• ß-glucuronidase deficiency
• Hurler syndrome
• Morquio syndrome
• Mucolipidosis III
Teratogens • Aminopterin, Cyclophosphamide,
Fluconazole,Retinoidse
• Valproate

41. Ocular finding in
craniosynostosis
Vision
Proptosis (sometimes called exorbitism)
Strabismus
Corneal exposure
Restricted eye ball movement
Increased ICP
papilledema, optic atrophy, corneal exposure,
or amblyopia secondary to strabismus or anisometropia
Poor visual acquity
Dacrocystitis

42. Proptosis

43. Evaluation
1. Assessment of craniofacial shape.
2. Movement or lack of movement of the calvarial bones during
infancy.
3. The presence or absence of sutural ridging.
4. Can be confirmed by an X-ray of the skull or head CT
examination.
5. In some instances, particularly with sagittal or coronal
involvement, plain X- rays may suffice. If radiographic
interpretation is equivocal, CT imaging is mandatory.
6. 25-26 weeks ultrasound detects syndrome

44. Craniosynostosis syndrome

45. Syndromic craniosynostosis
key points
Syndromic craniosynostosis is rare, occurring in
1:30,000 to 1:100,000 live births.
Fibroblast growth factor receptor and tumor growth
factor-b receptor mutations have been reported to be
associated with many forms of syndromic
craniosynostosis.

46. Syndromic craniosynostosis
key points
Intracranial hypertension, developmental delays, and
strabismus are more frequent in syndromic forms of
craniosynostosis than isolated synostosis.
Distraction osteogenesis is a useful adjunct in syndromic
synostosis to increase intracranial volume and is helpful
with fronto-orbital and midface advancements.

47. Syndromic craniosynostosis
Addressing decompression by increasing
intracranial volume and decreasing intracranial
pressure before 1 year of age is a common goal
through an interdisciplinary team approach.

48. Syndromic craniosynostosis
More liable to have ventricular expansion, hydrocephalus,
expanded subarachnoid space, and cerebellar tonsillar
herniation compared with patients with sporadic single
suture synostoses.
Increased intracranial pressure (ICP) is more likely to
occur in patients with syndromic craniosynostosis and
multisuture synostosis.

49. Secondary Craniosynostosis
Causes:
1.congenital
2.metabolic
3.iatrogenic
4.infectious
Whatever the cause, the resultant underdeveloped brain
fails to drive normal calvarial bone growth, cranial sutures
fuse prematurely, and the head often remains
microcephalic.

50. Secondary Craniosynostosis
Blood disorders like thalassemia, sickle cell anemia,
polycythemia vera can also cause cranial sutures to fuse
prematurely.
Iatrogenic craniofacial anomalies can occur in patients receiving
ventricular shunts in infancy and early childhood.
In children with patent cranial sutures over shunting of CSF leads
to shunt-induced craniosynostosis and frequently microcephaly.
Trauma and neoplasms rare causes of acquired craniofacial
deformities.

51. Crouzon syndrome
It is also called craniofacial dysostosis, has autosomal dominant
transmission, but up to 50% of cases occur sporadically,
representing fresh mutations.
Clinical features
1. Cranium : exihibit calvarial deformity, the brachycephalic
deformities predominate, but many cases of the syndrome show no
obvious calvarial deformity, even when there are marked
radiological abnormalities.

52. 2. Face: Midface hypolasia with relative mandibular prognathism,
drooping lower lip and short upper lip are typical features. The
nasal bridge is often flattened, and the tip of the nose may appear
beak-like. There is deviation of the nasal septum in 35% and
obstruction of the nasopharynx in 30% of cases.
3. Oral findings : A narrow high-arched palate, crowding of the
dental arches and an anterior open bite. Ectopic eruption of the
maxillary first molar teeth occurs in about half of the patients, and
35% are obligate mouth breathers. exhibit a cleft palate(3%) and
10% have a bifid uvula.
Crouzon syndrome

53. 4. Eyes: Proptosis secondary to the shallow orbits. Divergent
strabismus, nystagmus and hypertelorism are frequently found.
Exposure conjunctivitis (50%), keratitis (10%), poor vision (45%) and
optic atrophy (25%); rarely there is luxation of the globes.
5. Ears: More than 50% of patients have a conductive hearing loss
associated with malformed auditory ossicles, and some 15% patients
have atresia of the auditory canals.
6. Other anomalies : Stiffness of the joints, especially the elbows.
Cervical spine anomalies occur in 30% of patients, and 85% exhibit
calcification of the stylohyoid ligament
Crouzon syndrome

54. Crouzon syndrome

55. Genetics:
It has a clear autosomal dominant mode of inheritance,
with about 67% of cases being familial.
44 Variability of expression characterizes Crouzon
syndrome.
Most of the mutations associated with Crouzon
syndrome are located in FGFR2.
Crouzon syndrome

56. Crouzon syndrome

57. It is characterized by craniosynostosis of coronal suture, midface
hypoplasia and syndactyly of extremities.
Its transmissions is believed to be autosomal dominant, but most
cases are sporadic and caused by high neonatal mortality and
reduced fitness of affected individuals.
Blank estimated a frequency of 1 in 160,000 population, but Cohen
believed the prevalence may be higher.
Prenatal diagnosis is possible.
Apert syndrome

58. Apert syndrome
1. Face : The facial dysplasia is severe, especially in older patients.
maxilla is grossly hypoplastic, while the nose and mandible are
relatively prominent. Facial asymmetry is sometimes present, and can
be very pronounced.
2. Oral findings : high arched palate, constricted and may have a median
furrow. The soft palate is cleft with bifid uvula. The maxillary dental
arch V-shaped, with severe dental crowding and bulging alveolar
ridges. A skeletal class III malocclusion and an anterior open bite.
Retarded dental eruption is common.
All these deformities, together with mental impairment, frequently
combine to impair speech.

59. Apert syndrome
3. Skeletal system: syndactyly of digits two, three and four is
always found; in addition, digits one and five may be joined to
digits two and four respectively.
The interphalangeal joints of the fingers are stiff, while
fingernails of the mid-digital hand mass may be continuous or
partly continuous.
In the feet, toes two, three and four are joined by soft-tissue
syndactyly; toes one and five may either be joined by soft-tissue
syndactyly to the second and fourth toes respectively.

60. Apert syndrome
The upper extremities are shortened, and there may be aplasia or
ankylosis of several joints, especially the elbow, shoulder and hip.
Progressive synostosis of the bones of the hands, feet and cervical
spine have been reported
4. Eyes: Hypertelorism is common, and there is usually some degree of
proptosis. All degrees of orbitostenosis are seen, but it is not generally
as severe as in Crouzon syndrome.
The palpebral fissures may show an antimongoloid slant, congenital
glaucoma, abnormal postiotion, origin ans insertion of extra occular
muscles.

61. Apert syndrome

62. Apert syndrome
Fronto orbital advancement

63. Apert syndrome
Facial bipartition

64. Apert syndrome
Middle third advancement A:Le Fort III osteotomy line; B:
external distractor device applied in a patients after a Le Fort III
procedure. C: internal distractor device applied in a patient after
Le Fort III osteotomies.

65. Apert syndrome

66. Apert syndrome

67. Pfeiffer’s syndrome
In 1964, Rudolf Arthur Pfeiffer
It is similar to Apert syndrome but also is
characterized by very shallow orbits (already part of
Apert) and short broad thumbs and toes. Corneal
exposure is an important problem in these children.
Types: Pfeiffer’s syndrome types 2 and 3 are more
common than 1

69. Pfeiffer’s syndrome
It is similar to Apert syndrome but also is
characterized by very shallow orbits (already part of
Apert) and short broad thumbs and toes. Corneal
exposure is an important problem in these children.
Types: Pfeiffer’s syndrome types 2 and 3 are more
common than 1

70. Pfeiffer’s syndrome
Type 1: exhibit a spectrum of craniofacial involvement
ranging from moderate to severe midfacial hypoplasia.
Skeletal features encompass broad and medially deviated
thumbs and great toes; variable degrees of brachydactyly
can occur as well.
Others include hearing loss or hydrocephalus. Intellect is
usually normal

71. Type 2: Craniofacial involvement in Pfeiffer’s syndrome
type 2 is more severe that in type 1, with cloverleaf skull
and extreme proptosis (often to the point of inability to close
eyelids laryngotracheal abnormalities, hydrocephalus,
seizures, and increased risk for early death.Ankylosis of
elbow and knee.
Type 3: is almost identical to type 2; however, the skull
shape is turribrachycephalic.
Pfeiffer’s syndrome

72. • Craniosynostosis with
turribrachycephaly (short
and tall head)
• Ocular hypertelorism
(wide spread eyes)
• Midface defficiency .
• Hypoplstic maxilla
• Prominent lower jaw
• Dental abnormalities

73. Broad Thumbs

74. Broad great toes

75. Pfeiffer syndrome
1. Cranium: Turricephaly is the commonest deformity,
being associated with premature fusion of the coronal
sutures. Other sutures may be involved, and cases with
trigonocephaly and cloverleaf skull. Intelligence is
usually normal, but mental retardation does occur, being
most severe in those cases associated with clover-leaf
skull.

76. Pfeiffer syndrome
2. Face: Maxillary hypoplasia with relative mandibular
prognathism is common, and the ears are frequently
low-set. Facial asymmetry, orbital hypertelorism,
antimongoloid palpebral fissures, proptosis and
strabismus have all been reported.
Oral findings include a high-arched palate, dental
malocclusion and, rarely, a bifid uvula.

77. Pfeiffer syndrome
3. Hands and feet : The thumbs and great toes are
broad, with varus deformity. In some patients the great
toes may be shortened.
Cutaneous syndactyly is usually present, involving
digits two and three, and at times three and four, of
both hands and feet. Clinodactyly and symphalangism
of both hands and feet have been reported. Other
skeletal anomalies described include fused cervical
vertebrae,

78. Pfeiffer syndrome
4. Other anomalies : Other features occasionally seen are
pyloric stenosis, bicuspid aortic valve, hypoplasia of
the gallbladder, single umbilical artery, umbilical
hernia, preauricular tags, choanal atresia and hearing
loss.

79. Genetics:
Inheritance is autosomal dominant with complete
penetrance and variable expressivity. Mutations causing
Pfeiffer’s syndrome have been found on FGFR1 and
FGFR2. A single common missense mutation,
Pro250Arg, has been associated in five unrelated
families with a relatively mild form of Pfeiffer’s
syndrome
Pfeiffer’s syndrome

80. Carpenter’s syndrome
It is also known as acrocephalopolysyndactyly type II, is a
variation of Apert syndrome characterized by preaxial
polydactyly on the side of the thumb or big toe, syndactyly,
brachycephaly, synostosis, obesity, hypogonadism, mental
retardation, shallow orbits, proptosis, and laterally placed
intercanthi.
GENETICS
Inheritance is autosomal recessive and the molecular basis is
unknown to date. In all cases, examination of the parents has
been normal.

81. Klipel Feil Syndrome

82. Fetal alcohol syndrome

83. Hallermann-Streiff Syndrome

84. Amniotic band syndrome

85. A condition in which the tissues on one side of the face are
underdeveloped, affecting primarily the ear (aural), mouth
(oral), and jaw (mandibular) areas. Sometimes, both sides
of the face can be affected and may involve the skull, as
well as the face.
Hemifacial microsomia

86. Hemifacial microsomia is also known as Goldenhar
syndrome, brachial arch syndrome, facio-auriculo-vertebral
syndrome, oculo-auriculo-vertebral spectrum, or lateral
facial dysplasia. Defect of first and second brachial arch
Hemifacial microsomia

87. Deformational (or positional) plagiocephaly. A misshapen
(asymmetrical) shape of the head (cranium) from repeated
pressure to the same area of the head. Plagiocephaly literally
means "oblique head" (from the Greek "plagio" for oblique
and "cephale" for head).

89. History and clinical examination
Routine investigations
Cross matching and blood arrangement
Anesthetic consideration
X- ray skull, AP, Lateral, Oblique, Vertex
NCCT face and CECT or MRI face and neck
3 D virtual planning
Pre operative evaluation and
preperation

90. Anesthetic challenges
Nasal fiberoptic bronchoscopy was contraindicated and regional
nerve block was not a feasible option because of obstructed
breathing pattern.
Oral fiberoptic bronchoscopy-guided intubation with good airway
anesthesia and sedation was successfully managed in the next
attempt.

91. Anesthetic challenges
These patients often have nasal airway obstruction due to mid-face
hypoplasia and a high arched palate. incidence of C2,3 spinal
fusion. Orbit manipulation during these procedures can cause
marked bradycardia.
Anesthetic concerns in caring for these complicated patients are
airway abnormalities, adequate vascular access for hemorrhage and
monitoring and also heat loss. Preoperative sedation to a patient
with a compromised airway should be individualized and used
cautiously.

92. Anesthetic challenges
Airway management difficulty should be predicted and planned
for in advance to avoid urgent problems.
Options should include availability of oral airways, oversized
masks, fiberoptic bronchoscope, a variety of laryngoscope blades
and lighted stylets.
Control of the airway may involve awake, fiberoptic intubation.
Inhalation induction of anesthesia is preferred over intravenous.
Paralysis with neuromuscular agents should be avoided until
controlled mask ventilation is assured.

93. Anesthetic challenges
Blood loss during these procedures can be excessive with 1 to 2 blood
volumes often required. Adequate vascular access with heated
intravenous solutions are mandatory.
The use of erythropoietin injections and additional iron supplements
started 3 weeks prior to elective craniofacial surgery has been shown
to modify transfusion requirements.
An arterial line is required to monitor the hemodynamic and
laboratory values in these patients.

94. Anesthetic challenges
Use of central venous catheters to monitor volume status and
precordial doppler to detect venous air emboli should be
individualized to the procedure and patient.
Postoperative care must continue in a closely monitored
environment (intensive care unit) to continually access
hemodynamic and respiratory parameters.
Pediatric patients with craniofacial abnormalities continue to
provide the anesthesiologist with some of the most challenging
cases that we see today.
These difficult cases need careful planning and cooperation from the
many professionals involved in the care of these patients.

95. Treatment of the
craniosynostoses
The aims of early craniofacial surgery:
1. To allow the brain to expand normally
2. To provide a normal shape to the forehead and skull
3. To provide eye protection by reducing the exorbitism .
4. To prevent or minimize the problem of impaired facial
growth in facial stenosis.

96. Timing of surgery for patients with syndromic
craniosynostosis depends on the presenting symptoms,
such as the degree of exorbitism and signs and symptoms
of elevated ICP.
There is no clear consensus on the ideal operative
window for syndromic craniosynostosis; however,
delaying surgery beyond 1 year results in a higher
likelihood of elevated ICP, cognitive deficits, and
behavioral problems.

97. Timing of surgery
Less then 6 month or less tha 12 months for primary
surgery isolated nonsyndromic craniosynostosis.
Craniofacial clinic visit: at presentation; during any
preoperative planning; and postoperatively at 2 weeks, 3
months, 12 months, and annually until the patient reached
the age of 6 years.

98. Timing of surgery for Pfeiffer
syndrome
 Mostly early craniosynostosis released to be done for increased ICP.
 Hydrocephalus need to be treated by putting a stent.
 Lefort III distraction when patient is 5-7 years of age.
 Orthognathic surgery is indicated after skeletal maturity.
 There is no review for optimum timing for surgery.
 The potential for brain compression, loss of vision through exposure
keratoconjunctivitis, significant airway compromise, and
psychosocial maladjustment may dictate the timing of surgery

99. Apert syndrome
Step 1 — from birth to age 2
Step 2 — growth period
Step 3 — adult age.
Monobloc advancement or Le Fort III osteotomy and facial
bipartition are performed at 6-7 years, midface Le Fort III
advancement at 9-12 years.

100. 1. Prone position and
2. The neck in neutral position.
3. A zig-zag bicoronal
4. Incision was created, and extensive scalp flaps were
developed in the subgaleal plane.
5. Barrel-stave osteotomies were then created in the frontal,
parietal, temporal, and occipital squamous bones, which were
then outfractured
6. Tension, and a Jackson-Pratt drain was left in the subgaleal
space at closure.
Operative technique

101. It can be used to advance the mandible, midface, orbits, and skull
including lower level and higher level Le Fort osteotomies, PCD, or
fronto-facial (Monobloc) advancements using either external or internal
distractors. Cranial distraction has been used to increase microcephalic
skulls or provide added volume in the case of shunt-induced
craniosynostosis.
Typically an open bicoronal approach is used because eventual anterior
cranial vault reconstruction (ACVR) with FOA and frontal
cranioplasties are usually required months after distraction
Distraction osteogenesis

102. It can be used to advance the face alone when coupled with
Le Fort I, II, and III osteotomies or to advance the face and
frontal skull as in Monobloc advancement, which can be
used as early as 4 years of age if deemed necessary because
of neurosurgical, ophthalmologic, or airway issues or later
in the teenage years to improve exorbitism, midface
hypoplasia, and skeletal occlusal relationships that are
poor.
Distraction osteogenesis

103. Mid face advancements can be done between 4 years of
age and adulthood to address skeletal hypoplasia and
functional problems related to sleep-disordered breathing,
OSA, malocclusion, and feeding.

104. A 6 year old with syndromic facies;
no exorbitism; but mild midface
hypoplasia, anterior open bite, and
anterior crossbite with relative
normocephaly (cephalic index
85%). 3D CT images reveal
pansynostosis, lateral
plain film shows thumb-printing
(nonspecific for increased ICP),
and sagittal CT shows Chiari
malformation (asymptomatic).
Genetic testing was negative for
common fibroblast growth factor
receptor and TWIST
mutations.

105. Distraction osteogenesis
External arm of distractor removal after 3-5 weeks
and distractor removal after 2-3 months at the time
of ACVR

106. Frontal, lateral, vertex, and occipital views with three-dimensional computed tomography demonstrating
nonsynostotic positional brachycephaly, although mild metopic ridging is evident without significant
trigonocephaly.

107. A 2 year old syndromic patient with hydrocephalus s/p shunt who developed secondary multisuture
craniosynostosis with resultant severe brachyturricephaly and underwent PCD. (A–H) Postshunt and
predistraction photographs and 3D CT. (I) Intraoperative photograph demonstrating posterior
craniotomies and distractor placement. (J) Immediately postoperative 3D CT scan. (K, M) Right, left,
vertex 3D CT scan after consolidation phase. (L, N, O) Four weeks postdistractor removal, lateral and
vertex photographs. s/p, status post.

108. Axial CT scan. (A) Predistraction and (B) postdistraction demonstrating
improved intracranial dimensions and improvement in appearance of
ventricles and gyri/sulci.

109. A 5 year old with benign intracranial hypertension, increased ICP, and pansynostosis but
relative normocephaly who underwent VP shunt but had persistent ICP elevation. (A) Right
lateral photograph after VP shunt. (B) Right lateral 3D CT scan depicting pansynostosis
before shunt. (C) Right lateral 3D CT scan after completion of PCD consolidation. (D) At
time of distractor removal noted improvement in intracranial volume but severe occipital
shelf deformity. (E) One week postoperative after removal of distractors and posterior cranial
vault reconstruction with autologous bone graft repositioning to improve occipital shape. VP,
ventriculoperitoneal.

110. A 13 year old with autosomal-dominant FGFR craniosynostosis. (A) Left lateral preoperative
photograph demonstrating severe brachycephaly, midface hypoplasia, and exorbitism. (B) Preoperative
left lateral 3D CT showing pansynostosis. (C) Two weeks postoperative with rigid external distractor in
place for Monobloc distraction. (D, E) Left lateral photograph and 3D CT 2.5 years postoperative
demonstrating improved midface and decreased proptosis despite some deficient cranial bone in region
of frontal cranial distraction.

111. A 2-month-old girl with brachyturricephaly and bicoronal synostosis, with midface
hypoplasia.

112. A 9 month old with partial bicoronal synostosis, midface hypoplasia, and exorbitsim with underlying
spontaneous FGFR2 mutation. (A, C, E) Preoperative frontal, left lateral, and vertex photographs at 9
months. (B, D, F). Frontal, left lateral, and vertex 3D CT after ICP pressure monitor performed.
Results revealed a mild elevation of ICP. (G) Left lateral intraoperative photograph at time of PCD
showing left internal posterior cranial distractor. (H) Left lateral skull radiograph after latency phase
before beginning active distraction. (I) Left lateral 3D CT midway through active distraction when
patient developed CSF leak, external ventricular drain placed, and endoscopic third ventriculostomy
performed shortly thereafter. (J) Left lateral skull radiograph at conclusion of 30-mm active
distraction. (K, L) One year after distractors removal and ACVR.

113. Risks of distraction osteogenesis:
Second surgery
Infection
Soft tissues laceration around the distracters
Dural tear
CSF leak
Steps off deformity
Non union
Distraction osteogenesis

114. Sagital
synostosis(scaphocephaly)
Golf tee deformity- frontal bossing with biparietal narrowing.
Early surgery gives best results. Surgical techniques are:
Pi squeeze technique
Zenker’s solution
Strip craniotomy
Conceptual modification of the pi technique is the T technique
Reverse pi and Y technique
Radical cranial reconstructive surgery for older child

115. Sagital
synostosis(scaphocephaly)

116. Sagital
synostosis(scaphocephaly)

117. Sagital
synostosis(scaphocephaly)

118. Sagital
synostosis(scaphocephaly)

119. Sagital
synostosis(scaphocephaly)

120. Sagital
synostosis(scaphocephaly)

121. Sagital
synostosis(scaphocephaly)

122. Sagital
synostosis(scaphocephaly)

123. Sagital
synostosis(scaphocephaly)

124. Sagital
synostosis(scaphocephaly)

125. Lamdoidal synostosis
▣Timing of surgery 6-12 months
▣Surgical techniques:
 Radial fan cuts
 Transposition technique
 Tiara technique

126. Lamdoidal synostosis

127. Lamdoidal synostosis

128. Lamdoidal synostosis

129. Oxycephaly

130. Oxycephaly
Characterized by pointed skull
Classifications:
Type I congenital form with limb anomalies
Type II delayed oxycephaly
Type III false oxycephaly
Features: absence of facial anomalies, normal ethmoidal
complex, major claverial anomalies, absence of temporal
overgrowth.
Increased ICP, mentall retardation, exorbitism, visual loss,
hydrocephalus, epilepsy,

131. Oxycephaly

132. Oxycephaly
Surgical technique:
linear craniotomies and fragmentation of vault
Total craniotomies
Craniofacial technique
Shaving, skin incision, periosteum and temporal
muscle, frontal osteotomies, supraorbital ridge
bandeu, z plasty,

133. Plagiocephaly

134. Plagiocephaly

135. Plagiocephaly

136. Metopic synostosis and
trigonocephaly

137. Metopic synostosis and
trigonocephaly

138. Orbital hypertelorism

139. Orbital hypertelorism

140. Orbital hypertelorism

141. Orbital hypertelorism

142. Orbital hypertelorism

143. Orbital hypertelorism

144. Management total facial deficiency
▣Lefort III osteotomy
▣Distraction osteogenesis
▣Monobloc
▣Facial bipartiton
▣Fronto-orbital advancement

145. Lefort III osteotomy
Gillies and Harrison performed the first successful Le
Fort III.
Twenty years later, Tessier revisited, refined, and
eventually popularized the operation.

146. Lefort III osteotomy
Indications:
Cosmetics:-
Ophthalmic:-
Respiratory :-
Dental:-

147. Surgical Technique

148. • Infiltration of LA with Adr (1:200000)
• Oro-endotracheal intubation is preferred)
• Hypotensive anesthesia
• Intraoperative steroid and antifibrinolytic
injection.
Step 1: Anesthesia and Preparation:

149. Step 2: Tarsorapphy

150. Step 3: Surgical approach
1) Bicoronal
2) Subcilliary /subtarsal
3) Maxillary vestibular
approach.

151. Surgical Technique
Subcranial Lefort III osteotomy
1. Zygomatic osteotomy
2. Lateral orbital wall
osteotomy
3. Frontozygomatic sutures
4. Orbital floor
5. Nasofrontal suture
6. Medial orbital wall
7. Vomer(nasal septal)
osteotomy
8. Pterygoid dysjunction
9. Disimpaction by forceps
10. Resuspention and forced
duction test
11. Distractor placement

152. Step 4: Zygomatic osteotomy

154. Step 5 : Lateral orbital wall osteotomy

155. Step 6: Nasofrontal osteotomy

156. Step 7: Medial orbital wall osteotomy

157. Step 8: Nasal Septal Osteotomy

158. Step 9: Pterygoid Dysjucntion

159. Step 10: Maxillary Dysimpaction

162. General guidelines
For sub-cranial Le Fort III external distractor placement are as follows:
(1) Adjust the transverse width of the device, allowing the lateral
arm to extend wider than the scalp or hair by 2 cm bilaterally.
(2) The halo should be aligned parallel to the Frankfort horizontal plane
with the vertical bar component approximately 3 cm anterior to the
upper lip.
(3) Hand-turn the cranial screws sequentially bilaterally until bone is
Contacted on a minimum of four screws per side.
(4) Place the two horizontal bars at the desired vector of anterior
distraction corresponding to the distraction plates and/or intraoral
appliance.
Closure and Final Distractor Placement

164. • DO was initiated after 1 week.
• The rate of distraction was 1 mm per day in 2 daily activations.
• The duration of DO depended on the desired advancement.
• During the distraction period, vector modifications took place
when necessary in patients treated with an external distractor.
• In all patients a consolidation period of three months after
distraction was respected
Lefort 3 Distraction protocol

165. Hemorrhage result primarily from the pterygomaxillary
dysjunction and down-fracture
Atypical fracture that extends to the skull base, orbit and
pterygoid plate
Unexpected skull fracture.
Lethal subarachnoid hemorrhage after Le Fort III osteotomy
followed by fracture of base of skull
Intra-operative Complication

166. CSF leak
Orbital hemoatoma
Chemosis
Minor complications include
Cutting the infra-orbital nerve
Fracturing of zygoma during mobilization
Partial exposure of the nasal bone
Intra-operative Complication

167. Major complications include respiratory distress requiring
tracheotomy,
Subgaleal hematoma
Cerebro-spinal fluid leakage and fistula
Visual loss after retro-orbital hemorrhage
Strabismus
Partial anosmia,
Post operative complication related to
Lefort III osteotomy

168.  Ptosis
 Esthetic
 Unavoidable
 most important shortcoming is the tendency of medial
canthi to drift away from nose.
 Widening of intercanthal distance
Post operative Complications
late complication related to Lefort III
osteotomy

169.  Pin loosening of halo frame (20%)
 Pin infection (7%)
 Intracranial Migration of pin
Complication related to rigid external
distraction device

170. Risks of early craniotomy
▣Blood loss
▣Intravascular air
▣Cardiovascular effects
▣Infection
▣Pressure necrosis

171. Genetic counseling

172. Genetic counseling

173. Genetic counseling

174. Take home message
It can be said that diagnostic and therapeutic planning in patients with
craniosynostosis emphasizes the need to integrate various specialties.
It is necessary to frame the patient’s clinical picture upon his arrival.
Very important are also a multi-disciplinary plan and clinical
programmed check-ups in accordance to a rational therapeutic clinical
widely agreed upon and verified in its efficiency. As noted in this
paper establishing an integrated and tailored surgery timing,
scheduling, combining and
coordinating actions to be taken at different stages of the patient’s age
reduces the number of general anesthesia thus simplifying therapy for
both patients and their families.

175. References
 Craniofacial Anomalies: Growth and Development From a Surgical Perspectives
By James Tait Goodrich, Craig D. Hall.
 Maria T. et al. Treatment timing and multidisciplinary approach in Apert
syndrome.
 Anand R. et al. Advances in the Treatment of Syndromic Midface Hypoplasia Using
Monobloc and Facial Bipartition Distraction. Semin Plast Surg. 2014;28:179–183.
 Distraction osteogensis of facial skeleton:-William H Bell
 Distraction of Craniofacial Skelton :- Mc-carthy
 Orthognathic surgery: Principle & Practice:- J.C Posnick
 Atlas of oral and Maxillofaical surgery :- D. Kademani
 Atlas of oral and maxillofacial surgery clinics of north America, Volume 16
september 2008
 Various article

176. Thank you!!!
Any questions?