Craniofacial syndromes classification Treatment Cliedocranial dysplasia Apert syndrome Fetal alcohol syndrome achondroplasia

2.  Anomalies of the craniofacial complex are
of major concern, not only for the oral
health but also for the mental health and
social well being.
 The anomalies are very vast and varied.
 For most of the cases it is very difficult or
even impossible to describe a single cause.
 Yet some thought must be given to
etiologic factor as it determines the stability
of the results after treatment.

3.  The aggregate of signs and symptoms
associated with any morbid process and
constituting together the picture of the
disease and related to each other
anatomically, biochemically or
physiologically.

4.  Richard and goodman classified the
syndromes as
1. Chromosomal abnormality
2. Gene abnormality
3. Environmental Factor
 Embryologic Classification
1. Single germ layer involved
2. All the three germ layer involved

6.  Achondroplasia
 Apert sydrome
 Crouzen Syndrome
 Cleidocranial Dysplasia
 Treacher Collins Syndrome
 Marfan Syndrome
 Hemifacial Microsomia

7.  Etiology :
1. autosomal dominant
2. about 90 percent of the cases show a
gene mutation..
3. Old parental age is a contributing
factor
4. Frequency is about 1/15000

9.  Short limb dwarfism
 Waddling gait
 Prominent buttocks
 Short metacarpals, phalanges
 Protuberant abdomen

10.  Treatment of Class 3 malocclusion and
midface deficiency is done by Lefort 3
osteotomy for the advancement of
midface. Pre Treatment

11.  Autosomal Dominant Disorder
 Incidence is 1/25000
 Bilateral Symmetrical abnormalities

12.  Cranium.. Flat perieto occipital bone
 Midface:
1. Macrostomia
2. Auricular defects
3. External auditory canal defects
4. Nasal deformity
5. Malocclusion
6. Preauricular sinuses

14.  Mandible
1. Mandibular defects
2. Microganathia
3. Open bite
 Other Features
1. Fish like facial apperance
2. Deafness frequent
3. Mental retardation infrequent

16.  Zygomatic and orbital reconstruction
 Maxilo mandibular reconstruction
 Nasal reconstruction
 Soft tissue reconstruction
 External ear reconstruction
 External auditory canal and middle ear
repair

17.  Also called bird face deformity due to
short mandible
 Primarily caused by the arrest of
mandibular development.
 May be associated with other syndromes
or isolated it may be
 This in turn prevents the normal descent
of the tongue between the palatal
shelves thus producing cleft palate.

18.  Microganathia
 Cleft palate
 Glossoptosis
 U shaped cleft palate
 Deafness
 Mental retardation
 Congenital heart defects
 Respiratory difficuilty

20.  Infants born to alcoholic mothers exhibit
a specific pattern of defects including
1. Prenatal growth deficiency
2. Postnatal growth deficiency
3. Mental retardation
4. Other abnormalities
 Exposure to high level of Alcohol during
early fetal development produces FETAL
ALCOHOL SYNDROME.

22.  Etiology:
1. Autosomal dominant
2. Most common sporadic
3. 1/160000
4. FGFR2 gene mutation lead to increase
in the number of precursor cells that
entered into the osteogenic pathway
resulting in increased bone formation
and premature ossification of calvaria
during fetal development.

23.  Cranial:
1. Short anterior posterior diameter with
high full forehead and flat occipit
2. Irregular craniosynostosis especially of
coronal suture
3. Frontals may be large and late in
closure.

24.  Flat faces
 Shallow orbits
 Hypertelorism
 Small nose
 Down slanting of palpebral fissres
 Supraorbital horizontal groove
 Maxillary hypoplasia
 V shaped dental arches
 Narrow palate

27.  Dental:
1. Crowding
2. Delayed eruption
3. Ectopic eruption
4. Supernumerary may be present
5. Class 3 malocclusion is usually seen in
these patients

29.  Surgery to:
1. Increase intracranial space
2. Increase the orbital volume
3. Enhance the morphology of forehead and
upper orbits
 It requirs
1. Bircoronal suture release with
decompression
2. Cranial vault and orbital osteotomies
3. Usually done at 9 to 11 months