MANAGEMENT OF CRANIOFACIAL ANOMALIES

1. DR AJAY SRINIVAS
DEPT OF ORTHODONTICS
PG STUDENT
MANAGEMENT OF
CRANIOFACIAL ANOMALIES

2. INTRODUCTION
The term craniofacial anomalies literally encompasses
all congenital deformities of the cranium and face.
More specifically however the term has come to
imply congenital deformities of the head that
interfere with the physical and mental well being
(Marsh and Vannier 1985)

3.  The main problem in craniofacial developmental
biology is understanding when, where and how are
genes expressed and how is differential gene
regulation associated with specific pattern of
morphogeneis.

4. HOX HOMEOBOX NETWORK
 Patterning of much of the craniofacial region is laid
down by cluster of genes, the Hox homeobox
network. These are expressed through patterning of
rhombomeres. The structures of the craniofacies are
largely derived from neural crest cells. They undergo
extensive migrations and interactions in the facial
region they give rise to almost all the skeletal and
connective tissues.Interaction of crest cells with
other cells, with matrix and growth factors at various
locations along the migratory path, or at their
destination determine the differentiation of the cells.

5.  Failure of neural crest to migrate, inadequate
migration, failure to proliferate during migration,
and premature cell death (necrosis) serve as a basis
for the many syndromes, collectively known as
neurocristopathies.

6. ETIOLOGY
 Chromosomal disorders
 Single gene disorders
 Multifactorial inheritance
 Maternal infections in pregnancy
 Maternal metabolic derangements
 Maternal use of medication
 Radiation exposure
 Disturbances of embryonic differentiation and fetal
growth

7. Cleft lip and palate
 They are one of the most common congenital
anomalies occurring in about 1.97 to 1.23 /1000 in
Indians and 2/1000 in mongoloids In 2/3rd of the
cases cleft palate is on the left than the right side
CL(P) is seen more in male and CP alone more in
females.

8.  The pathogenesis is heterogeneous and
multifactorial and is ultimately due to deficiency of
neural crest mesenchyme failing to migrate and/or
proliferate to coalesce individual embryonic
prominences and processes that combine into the
fetal orofacies.
 cleft lip results from failure of fusion of the median
nasal ;lateral nasal and the maxillary processes on
either or both sides.

9. Reasons
 Hypoplasia of the facial processes
 Altered facial geometry
 Defective ability of surface epithelia to participate
in the fusion process
 Excessive cell depth in the fusing palatal seams,
mesenchymal deficiency and post fusion rupture

10.  Thus they can be unilateral or bilateral clefting ;
complete or incomplete , of the lip and/or primary
palate till the incisive foramen.

11. Reasons
 Hypoplasia of the palatal shelves
 Failure of the palatal shelf elevation at the correct
time due to diminished intrinsic force
 increased resistance mainly by the tongue position
being high.
 an under developed mandible also prevents the
descent
 Excessive head width causing failure of normal sized
palatal shelves to meet

12. Dentofacial relationships in unoperated cases
 Unilateral cleft - nasal septum and columella is
deviated to the non cleft side of facial midline
whereas incisors deviate towards the cleft
 In UCLP and BCLP - tendency for the mandible
to be retruded and for the mandibular plane to be
steep with a relatively shorter posterior facial height
and a longer anterior facial height.

13.  Mandibular incisors- labially proclined in UCLA
while lingually inclined in CLP
 In BCLP -maxillary intercanine dimension were
much smaller than UCLP and UCLA
 In maxillary arch the non cleft segment has a
tendency to rotate forwards hence increasing the
overjet while the cleft side rotates medially hence
edge to edge bite of the canines. Teeth also tend to
roll superiorly hence an openbite on that side due to
infraocclusion .

14. Maxillo-mandibular clefting
 It is not formed between the maxillary and
mandibular bone but between the facial processes
with the same names. It is essentially a soft tissue
defect affecting skin, muscle and mucosa, is usually
called macrostomia. It may be unilateral or rarely
bilateral. Its range of malformations varies from
minor elongation of the oral angle to a wide cleft
extending towards the tragal area

15.  In the majority of cases it is associated with
preauricular appendages or fistulae that may be
found anywhere between the angle of the mouth and
the tragus occasionally also with temporoaural
and/or mandibular abnormalities.

16. CRANIOFACIAL DYSPLASIAS WITH
DYSOSTOSIS
 MEDIAN CLEFT FACE SYNDROME/ fronto-
nasal syndrome/Internasal dysplasia
 A whole spectrum of malformations may be
observed and the severity of the reported examples
can be graded in a sequence.

17.  At one end is bifidity of the nasal tip or dorsum,
sometimes associated with a median cleft lip and
with duplication of the labial frenulum. Grooves and
folds along the dorsum nasi are also occasionally
observed.
 At the other end widely separated nasal halves and
extreme orbital hypertelorism, including other
anomalies caused by frontonasoethmoidal dysplasia,
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18.  Premaxilla may be retarded in development and
bifid, The maxilla may show a keel-shaped
deformity, with the incisors rotated upward in each
half of the alveolar process. Sometimes a medial cleft
of the palate is also found and this may extend
upwards to the cribriform plate as an inverted V .

19.  NASAL APLASIA - characterized by complete
absence of one nasal half. The nasal cavity is missing
and pneumatiziation of the maxillary ethmoidal and
frontal sinuses has failed . There is no nasolacrimal
duct. The affected half of the maxilla is hypoplastic
and the palatal vault is high and acutely arched .
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20.  NASAL DUPLICATION-ranges from a
supernumerary nostril in an otherwise normal nose
to duplication of the upper face (diprosopia). The
supernumerary nostril is usually the medial one. It
may end blindly, be stenotic or open into a nasal
cavity..
 In the milder cases there may be one continuous
midline septum, while in the more severe cases
duplication of the anterior part of the septum or full
duplication may be observed

21. Treacher Collins' syndrome /Zygomatic
dysplasia / mandibulofacial dysostosis
 caused by a change in a single gene & this
Treacher Collin gene is located on chromosome 5
inherited as an autosomal dominant gene with
complete penetrance but variable expressivity

22. Features
 Malar & zygomatic hypoplasia
 Anti mongoloid slant of the palpebral fissures
 Coloboma in the outer third of the lower eyelid(75%)
 deficiency of eyelashes in the medial third of these
eyelids
 Unusual tongue shape (25% cases)

23.  Hair extending down & forward from the temporal
region on to the cheek. flattening of the cheeks body
of the mandible is frequently hypoplastic and the
chin severely retruded.

24.  Radiographs show antigonial notch in the lower
border of the mandible along with hypoplasia of
coronoid & condylar processes. Cleft palate is found
in approximately 30% of the cases.

25.  Posterior maxillary height is decreased and anterior
height is increased resulting in a steep anteroinferior
cant. open bite is related to shortening of the
mandibular rami and premature posterior teeth
contact .
 Deformed external ear,ear tags & pre-auricular
pits,absence of external auditory meatus frequently
accompanied by malformations of the middle ear

26. Miller syndrome / Postaxial acrofacial
dysostosis
 Has resemblance to that of mandibulofacial
dysostosis but there is postaxial limb deficiency.
Malar bones are hypoplastic with downslanting
palpebral fissures.Eyelids may exhibit coloboma
Cleft lip and/or cleft palate are common

27.  Pinnae tend to be cup-shaped. The external auditory
canals and middle ears are often malformed. Various
congenital heart defects have been documented

28. Nager syndrome / Preaxial acrofacial
dysostosis
 Similar to mandibulofacial dysostosis. The zygomatic
hypoplasia results in downslanting palpebral
fissures. The lower eyelids exhibit colobomas
reduced number
 External ear defects and cleft palate are common
Velopharyngeal insufficiency ‘

29.  Micrognathia is usually more marked mild mental
retardation www.indiandentalacademy.com s of
eyelashes.

30. HEMIFACIAL MICROSOMIA
 Temporo-auromandibular dysplasia / Goldenhars
syndrome
 Facial asymmetry with deviation of the chin towards
the affected side and ear anomalies are the
'hallmarks' of this entity

31.  Ear - anotia to an ill-defined mass of tissue that is
displaced anteriorly and inferiorly, to a mildly
dysmorphic ear are found in over 65%. Preauricular
tags of skin and cartilage are extremely common, and
maybe unilateral or bilateral.

32.  Both the horizontal and ascending ramus of the
mandible may have macrostomia. malformations are
most severe in the condylar region and less near the
middle sector, with flattening of the gonial angle .
 Hypoplasia of the maxilla on the affected side is
shown by obliquity of the occlusal plane entuation of
the antegonial notch.

33.  A depression and recession of the inferiolateral
angle of the orbit indicates involvement of the malar
bone. Orbital dystopia may be observed temporalis,
masseter and lateral pterygoid may be differentially
hypoplastic. A fused mass may be observed on CT
scans, containing elements of each of these muscles.

34.  Aplasia of the levator veli palatini, resulting in
abnormal elevation of the soft palate towards the
unaffected side parotid gland may be
absent,producing a preauricular concavity.

35.  Maxillary, temporal, and malar bones on the
involved side are reduced in size and flattened
Narrow external auditory canals are found in more
mild cases; atretic canals are seen in more severe
cases.

36.  Unilateral or bilateral cleft lip and/or cleft palate
occurs in 7-15% of patients Tooth development tends
to be delayed and missing on the affected side 35%
have velopharyngeal insufficiency.

37. CRANIOSYNOSTOSIS
 conditions in which one or more sutures close too
early causing problems with normal brain & skull
growth Occurs 1 in 2000 live births Affects males
twice as often as females .Most often occurs
sporadically .Can be inherited as: Autosomal
recessive Autosomal dominant

38. Pachycephaly
 Premature closure of the lambdoid sutures found
isolated, associated with synostosis of the sagittal
suture or as part of multiple synostoses. It causes
hypoplasia and flattening of the occiput, with slight
compensatory development of the ipsilateral anterior
cranial region.

39. Scaphocephaly/ interparietal
 Elongated narrow shape of the skull, resembling the
hull of a ship resulting from early fusion of the
interparietal sagittal suture. From front, the skull is
high and narrow ; from side, skull is elongated from
front to back with posterior occipital protrusion and
excessive bulging of the frontal bones anteriorly.

40. Trigonocephaly / interfrontal
 Premature closure of the frontal suture. The frontal
area becomes triangular. extent of skull
malformation depends on how early the synostosis
takes place; this usually occurs during intra-uterine
life. Results in a prominent ridge running down the
forehead Forehead may look pointed like a triangle
with closely placed eyes

41. Plagiocephaly
 Asymmetric malformation secondary to fusion
of one half of the coronal suture. Mainly affecting the
sphenotemporal suture . Produces flattening of
forehead & the brow on the affected side with
forehead excessively prominent on the opposite side.

42. Brachycephaly / frontoparietal
 Refers to craniofacial dysmorphism secondary to
premature bilateral coronal stenosis the skull is
shortened in the sagittal plane and compensatory
lateral development occurs in breadth or in height.

43. Binder's syndrome / Maxillo-nasal dysostosis
 Nasomaxillary deformity which mainly affects the
lower part of the nose and the premaxilla It is due to
an alteration of the inferior mesenchymal portion of
the medial strut formed by the vomer pushing the
premaxilla forward.

44.  Philtrum is poorly developed
 Premaxilla is hypoplastic with shortening of the
dental arch
 All patients have relative mandibular prognathism
with anterior crossbite

45. Crouzon syndrome
 The term refers to a typical deformation, but this
anomaly may be due to various causes. The
developmental arrest affects the Maxilla, the Orbit
and the Vault .It is an autosomal dominant
condition. Two genes known to be associated are
FGFR2 and FGFR3.

46.  Cranium- Some people have craniosynostosis at
birth in which several sutures are always involved . A
very pronounced bregmatic boss “Clown’s Hat” may
be observed. The severity of cranial malformations
does not parallel that of face

47.  Eyes – Exopthalmos, the cardinal sign is constant
eyes give the patient a ‘ toad like ’ appearance. This
appearance is due to hypoplasia of the maxilla, of the
malar bone and of the orbital roof, resulting in the
reduction in the size of the orbital cavities Divergent
strabismus or defective convergence is frequent.

48.  Face – flattened and sometimes concave. Parrot
beak appearance of nose b’coz of maxillary retrusion.
Dental malpositioning is common, sometimes with
supernumerary or abnormal ‘peg-top’ teeth. Palate is
high, arched, narrow & pointed nasal root is flat, the
dorsum and the nostrils are wide.

49.  Vision- Lack of skeletal protection may result in
exposure keratitis or even dislocation of the globe.
Respiration Constriction of airway may result in
chronic or intermittent respiratory problems.

50. Apert's syndrome
 Inherited in an autosomal dominant manner. The
gene involved is FGFR2 (fibroblast growth factor
receptor 2) located on chromosome 10 of those
having craniosynostosis, 4-5% have Apert’s
syndrome.

51.  Down slanting palpebral fissures, strabismus,
orbital hypertelorism.
 ears may appear low set and Otitis media is common
 Midface deficiency (maxillary hypoplasia).
 Class III malocclusion is present, with anterior open
bite and anterior and posterior crossbite
 Delayed dental eruption

52.  Palate is high arched; constricted, and has a median
furrow. Lateral palatal swellings (Hyaluronic acid)
are present, which increase in size with age. The
maxillary dental arch is Vshaped with severely
crowded teeth and bulging alveolar ridge

53. Pfeiffer syndrome
 Mostly autosomal dominant transmission
 Main features-
 craniosynostosis,broad thumbs, broad great toes,
and soft tissue
 syndactyly of the hands skull is usually
turribrachycephalic.
 Craniofacial asymmetry may be present Maxillary
hypoplasia

54.  Hypertelorism, downslanting palpebral
fissures,ocular proptosis, and strabismus are
common
 palate is highly arched, alveolar ridges are broad,
and teeth are crowded
 thumbs and great toes are broad
 Mild soft tissue syndactyly.

55. Saethre-Chotzen syndrome
 Craniosynostosisis is a facultative feature
 Brachycephaly or acrocephaly with coronal sutural
synostosis is seen, producing plagiocephaly and
facial asymmetry
 Frontal bossing, parietal bossing, and flattened
occiput with late-closing fontanels are seen.

56.  Oral anomalies include narrow or highly arched
palate, cleft palate supernumerary teeth, enamel
hypoplasia .Some degree of brachydactyly and
partial cutaneous syndactyly is present.

57. Cloverleaf anomaly ,Triphyllocephaly
 Characterized by hydrocephalus and a trilobular
skull with synostosis of the lambdoidal and coronal
and metopic sutures, with bulging of the cerebrum
through the open sagittal sutures and a widely patent
anterior fontanelle.

58. Main characteristics
 hydrocephaly
 Retrusion of orbital roof
 exorbitism
 maxillary retrusion & severe downward
displacement of ears and zygomatic arches

59.  antimongoloid slanting, nasal flattening and an
arched palate
 Macrostomia;macroglossia
 oblique facial clefting
 Iris colobomas and blindness
 Obstructed nasolacrimal ducts
 Absent external auditory canals

60. Ectodermal dysplasia
 affect series of ectodermal derivatives including the
teeth, the sweat glands and adnexa of the skin
derivatives(nails, hairs, setaceous glands).
 Hypohydrosis,hypotrichosis, hypodontia are the
main characteristics
 sex-linked recessive trait. It occurs in males

61. Main features
 Thin hair Thin and/or small nails
 Person cannot perspire and consequently suffers
from hyperpyrexia & inability to endure warm temp
 The midface is retruded due to deficient alveolar
growth.
 Jaw and facial development are normal forehead is
prominent and the nose flattened

62.  the skin is thin and dry with multiple ridges hairs are
scarce and underdeveloped.
 complete or partial absence of teeth & when present
teeth may be truncated or cone shaped.
 Palatal arch is frequently high and a cleft palate may
be present.

63.  Forehead is prominent and nose flattened
xerostomia may be present. Hypoplasia of the nasal
& pharyngeal mucous glands which leads to chronic
rhinitis &/or pharyngitis, sometimes associated with
dysphagia & hoarseness.

64. NEUROFIBROMATOSIS
 characterized by neurofibromas or other neural
tumours and by focal cutaneous hyperpigmentation
(cafeaulait spots) caused by aggregation of
melanoblasts in the basal layer of the epidermis.
derivatives from the neural crest, are primarily
affected.

65. Skeletal malformations –
 macrocranium
 interosseous cysts and perforating defects, expansion
of the middle cranial fossa
 hypoplasia of the sphenoid resulting in wide areas of
communication between the cranial cavity and the
orbit
 downward displacement of the zygoma, maxilla and
the mandible on the affected side.

66. Pierre Robin syndrome
 It’s a combination of problems that begins with
Micrognathia . Causing not enough room for the
tongue to lie flat in the mouth, so it rests at the back
of the mouth. Glossoptosis prevents palate from
closing resulting in Cleft palate.

67.  It is a disturbance of muscular maturation of
nervous origin which affects the masticatory mu
 Retromandibulism is caused by deficient activity of
the pterygoid muscle, which is unable to bring the
mandible forward.

68. Stickler syndrome
 It is a connective tissue disorder caused by a change
in one of the 3 genes for connective tissue.

69. Features
 Cleft palate and a small lower jaw
 Of those with stickler syndrome , 60% have pierre
robbin syndrome
 Eyes - near sightedness. increased risk of
cataracts & retinal detachment.

70. Mobius syndrome
 involves paralysis of certain facial nerves (unilateral
or bilateral). Mainly the intra-cerebral nuclear part
of the 6th & 7th nerves are affected. face is
motionless with a characteristic nasiolabial grin.

71.  Patient cannot do side to side eye movements, but
they will be able to move them up & down. Blinking
action may be difficult hypoglossia & microstomia
may be seen skeletal involvement include clubfoot,
missing or webbed.

72. Hemangioma
 A type of birth mark. Most common benign tumor of
the skin. May be present at birth (faint red mark) or
may appear in the first months after birth. Also
known as port wine stain, strawberry hemangioma,
and salmon patch.

73.  Hemangiomas are usually not present at birth or are
very faint red marks After birth, they grow rapidly-
often faster than the child’s growth. Over time, they
become smaller (involute) and lighter in colour
 Vascular malformations- These are present at birth.
Enlarge proportionately with growth of the child.
They do not involute spontaneously and may become
more apparent as the child grows.

74. Velocardiofacial syndrome
 Autosomal dominant inheritance
 Features Face -Approx 40% are microcephalic
face is long with vertical maxillary excess malar
flatness and mandibular retrusion. nose is
prominent with squared nasal root, hypoplastic alae
nasi, and narrow nasal passages

75.  Adenoids are hypoplastic Narrow palpebral fissures
with blue suborbital coloring occurs Small ear
auricles and minor thickening of the helical rims
have been seen Multiple cardiac anomalies are
present in over 80%, especially VSD.

76.  Cleft palate (35%), submucous cleft palate
(33%), and occult submucous cleft palate or velar
paresis (33%) resulting in hypernasal speech have
been found in nearly all patients Class I malocclusion
is common .The pharynx is hypotonic

77. Cleidocranial dysplasia
 autosomal dominant inheritance
 individuals are usually short
 skull is brachycephalic, with pronounced frontal and
parietal bossing.
 maxilla and zygomas are hypoplastic.

78.  skull is large and short
 Closure of the anterior fontanel and sagittal and
metopic sutures is delayed
 Secondary centers of ossification appear in the
suture lines, and many Wormian bones are formed

79.  Delayed union at the mandibular symphysisis
characteristic.
 nose is broad at the base, with the bridge depressed.
 neck appears long, and the shoulders are narrow and
droop markedly

80.  Clavicles are absent unilaterally or bilaterally
 variations in size, origin, and insertion of muscles
related to the clavicles, especially the
sternocleidomastoid,trapezius, deltoid, and
pectoralis major.

81.  palate is highly arched.
 Submucous cleft of palate and complete cleft of the
hard and soft palates is seen
 Development of the premaxilla is poor with relative
prognathism

82.  multiple supernumerary teeth
 Multiple crown and root abnormalities, crypt
formation around impacted teeth, ectopic location of
teeth, and lack of tooth eruption

83. CFA TEAM
 It is agreed worldwide that management of patients
with CFAs is best provided by a multidisciplinary
team of specialists.
 Plastic /craniofacial surgeon
 Neurosurgeon
 Pediatrician
 Orthodontist
 Pediatric dentist
 Speech & language specialist
 Otolaryngologist
 Audiologist

84.  Opthalmologist
 Genetic councellor
 Nurse team coordinator
 Social worker
 Psychiatrist

85.  The surgeon and the orthodontist plan at the very
beginning for diagnosis and treatment planning . A
detailed treatment plan should be written, including
a specific definition of what orthodontic teeth
movement is to be done prior to surgery; how the
orthodontic appliance will be used for surgical
fixation; and what orthodontic tooth movement will
be required to finish the case following surgery.

86.  he efficacy of orthodontic and orthopedic treatment
in case of craniofacial anomalies depend on the type
of deformity, taking mainly into consideration the
growth potential.

87. Presurgical orthodontic treatment
 The main objective of this stage is to arrange the
teeth so that they will approximately fit when the
arches are surgically moved.
 Continuous arch wire technique
 Segmented arch technique

88.  Continuous arch wire technique
 Used for total maxillary surgical procedures.
 progressively the size of the arch wires is increased to
achieve final stability in the postsurgical occlusion.
 If .018 slot is used, the minimum size of arch wire
for a total maxillary surgical splint is .016x.022
without palatal splinting and .016x.016 if acrylic or
metal palatal splinting

89.  Segmented arch technique-
 used in preparation for a segmented surgical
procedure.
 orthodontic treatment time is shortened because
alignment of each segment is done without being
concerned about the relationship of the segments to
each other.

90.  Disadvantage- when surgical suspension wires are
used inadequate fixation will allow the crowns of the
segments to be buccally torqued, causing posterior
buccal overjet and open bite

91.  Post surgical orthodontic treatment - Involves
various final adjustments in the occlusal
relationships and the final tooth alignment. This
final phase usually lasts form 3 to 4 month.

92. Transverse Maxillary Deficiency
3 main factors should be considered
 amount of arch length discrepancy
 In moderate to minimal space deficiency, RME
will increase arch circumference sufficiently to
permit alignment of the crowded anteriors without
the necessity of extraction of premolars

93. Arch morphology
 Cases in which a transverse deficiency exists will
exhibit a narrow, tapering arch form. The
discrepancy will be most pronounced in the canine
region.
 If nonextraction orthodontic therapy is decided
lateral maxillary osteotomies and rapid maxillary
expansion is the treatment of choice to achieve
proper arch morphology

94.  Cases which do not exhibit severe constriction in the
anterior region, a two-piece maxillary procedure
with a midline osteotomy and resultant diastema
may be done consideration to wound healing after
creation of an interincisal space should be done.
 When excessive the gingiva may detach and
interproximal bone may be exposed with a possibility
of devascularization and osteonecrosis of the
underlying bone

95. vertical dimension
 In cases exhibiting an anterior open-bite with a
severely accentuated maxillary curve of spee ;
orthodontic treatment by extrusion of incisors
and/or intrusion of posterior teeth may compromise
the postsurgical stability. Segmentalized orthodontic
therapy with a three-piece or four-piece maxillary
surgical procedure is indicated

96. True Unilateral Transverse Maxillary Deficiency
 should be treated by maxillary segmental surgery
with the osteotomy mesial to the most anterior tooth
in palatal cross-bite. Orthodontic management of
such patients will depend upon the necessity of
extra In some cases the apparent maxillary
deficiency may be due to the ectopic eruption of one
or two posterior teeth in one quadrant and be treated
by orthodontic means ctions for alignment of
crowded anterior teeth.

97. Transverse Maxillary Excess
 Seen mostly in cases with skeletal class II The aim of
presurgical orthodontics in these cases is to position
the malaligned teeth over their bases so that the
maxilla can be surgically positioned into satisfactory
overbite-overjet relationship.

98.  Many technical modifications of the Le Fort I
osteotomy are feasible to facilitate simultaneous
anteroposterior, vertical, or horizontal movements of
the anterior and posterior segments of the maxilla

99. Hemifacial Microsomia
 Harvold advocates the use of activators to guide
eruption of teeth and prevent midline shift until the
time of surgery. This approach may have a stimulator
effect on muscle development and serves to prevent
canting of the occlusal plane. conventional
orthodontic tooth movement is of little value.

100.  In a cephalometric study by Bachmayer, Ross and
Munro (AJO 1986) on maxillary growth following Le
Fort III osteotomy in children with Crouzon-Apert
Pfeiffer (CAP) syndromes it was found that the
maxillary growth after surgery is negligible. Vertical
maxillary growth following surgery is identical to
that in unoperated CAP and normal children,
amounting to 1.3 mm/yr.

101.  Olow-Norderam and Thilander (AJO 1989) studied
the influence of orthodontic treatment on Binder's
syndrome . Although the orthodontic treatment led
to acceptable dental conditions in some patients, no
influence on craniofacial growth could be
demonstrated

102.  Graysun et al ( AJO 1983) in a study on unilateral
craniofacial microsomia said that the lateral ceph
analysis of patients with unilateral craniofacial
microsomia confirmed the clinical impression of an
increased gonial angle and decreased ramal height
and body length on the affected side.

103.  The ramal height on the unaffected side was also
decreased. The mandibular plane angle was greater
than normal on both affected and unaffected sides.
They conclude that the unaffected side too is
characterized by abnormalities in the skeletal
anatomy.

104.  Schudy ( JCO 1986) described the surgical
correction of Crouzon's and Apert's syndromes by
Dr. Paul Tessier. The orthodontic treatment involves
no special procedures and is performed in the usual
manner. Good arch forms were established for the
prospect of good future occlusion before the surgery
was performed. After the surgery was done brackets
remained on for a further 24 months to improve the
occlusion

105.  Skeletal Mandibular Deficiency. 3 types of
dentoalveolar problems that require orthodontic
treatment often accompany it – Malalignment of the
teeth ie: crowding or protrusion.
 Most of these are dental compensation for the
skeletal deficiency crossbite tendency appears as the
mandible is advanced.

106.  Deep bite, with an accentuated curve of Spee due
either to elongation of the mandibular incisors or
due to vertical under development of the premolar
segment of the arch.

107. Distraction ostegenesis.
 specially effective in cases of unilateral mandibular
deficiency involves the deliberate fracturing of the
bone side and holding it in close but not exact
approximation by means of a complex system of
extra oral positioners.

108. Principle
 osteogenesis takes place in the intervening space. As
soon the bone formation is complete the set up is
adjusted so that the bone segments move a bit away
from each other. The bone segments are held in that
place till new bone is formed and so forth, till the
bone achieves the required length.

109.  General principles of treatment
 Orthodontic intrusion of teeth must be done prior to
surgery.
 Extrusion of teeth can be done following surgery.
tooth movement in the transverse or crossbite plane
of space can be deferred until after surgery

110.  Tooth movement that occurs immediately after
surgery, while the patient is in IMF but before bone
healing occurs should also be considered.
Orthodontic tooth movement takes place to maintain
the dental relationship.
 The mandibular dentition slips forward on the
mandible (2mm) increasing the prominence of the
lower incisors. The maxillary dentition is retracted,
decreasing the prominence of the maxillary incisors.

111.  Moving teeth laterally for crossbite correction
introduces interferences along the line of the cusps
and leads to some lengthening of the posterior
vertical dimension and a downward positioning of
the mandible.
 desirable - skeletal deep bite
 undesirable - steep mandibular plane angle

112.  Orthodontic Procedures To Be Avoided Prior To
Surgery For Mandibular Deficiency.
 use of Class II intermaxillary elastics to reduce
overjet
 produces forward positioning of the lower incisors.
 will cause vertical extrusion of the anterior maxillary
segment, tending to extrude teeth.

113.  Mandibular excess
 characterized by a prominent lower third of the face.
orthodontic treatment modalities-
 Chin-cap therapy Activator appliances Fully
banded orthodontic appliances.

114.  Chin-cap therapy the pressure against the chin
would be transmitted to the growing areas of the
mandible and the growth would be impeded or at
least directed more favorably

115. Two approaches
 impede mandibular growth by applying heavy
pressure in the vicinity of the growing condyle of the
mandible.
 The force is applied upward and backward, opposite
to the vector of downward and forward mandibular
growth. redirect the growth of the mandible.
 It is based on the principle that when the mandible is
rotated downward it rotates backward.

116.  Activator appliances: effective in the treatment of
class I I I malocclusion using a class III activator
causing a downward and backward displacement of
the mandible. It may be trimmed to allow posterior
teeth to erupt so that the vertical dimension is
maintained

117.  Fully banded orthodontic appliances can only be
carried out satisfactorily without surgery only when
the problem is minor, because it is very difficult to
position mandibular teeth so as to camouflage the
mandibular prominence.

118. CONCLUSION
 Although craniofacial anomalies have been
reported and depicted from ancient times; a team
approach to diagnosis ; management and treatment
of dysmorphic patients is a recent event.
understanding of normal and pathogenesis helps us
diagnose and treat to the best of our capabilities

119. REFERENCES