Craniofacial anomalies

Craniofacial anomalies
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Craniofacial Anomalies

Malformation

Disruption

Deformation

Cleft Lip ,Cleft Palate and Other
Orofacial Clefts


Mechanism of palatal shelf elevation
*Intrinsic Force within the shelf
*Accumulation of Glycosaminoglycans
*EGF and TGF stimulate production of Hyluronan
*Increase in vascularity
*Contraction of elastic fibres or muscle fibres.
*Unequal division in the palatal and the oral epithelium
*Neurotransmitters like Serotonin
*Increase in MMP-3
*Increase in Vimentin expression
*Master controlling gene is FSP-1,ssh,

Midpalatal suture



Zone Of Intense Proliferation


A-P Direction Of Collagen Fibres


Transversely oriented collagen fibres


Network Of Collagen Fibres In centre Of Suture

s


Sharpey fib


B
S
T


TRANSITIONAL SPACE

SINUOUS AND IMBRICATING NATURE OF SUTURE

NARROWED ZONE OF CELLULAR PROLIFERATION

S


LIGHT MICROSCOPIC VIEW


ELECTRON MICROSCOPIC VIEW

Genetics
Candidate gene

Gene locus

Name of gene

TGF alfa

2p13

TGF beta

19q13.1-q13.3

MSX1
RAR alfa

4p16.1
17q12

DLX2

2q32

BCL3

19q13.1
2q32

transforming growth
factor alfa
transforming growth
factor beta
homeobox gene
retinoic acid
receptor alfa
distal-less
homeobox 2
B cell leukemia
unknown gene but
different from DLX
possible genes are
FN1,IHH,IGFBP2
unknown gene
unknown gene

4q25-q31.1
6p23

Teratogens
• Ethyl alcohol
• Retinoids
• Hype rthermia
• Smoking
• Diphenylhydantoin
• Trimethadoine
• Aminopte rin

Site of action of teratogens in electron transport system

3-acetly-pyridine
6-nicotinic acid
analogues

hypoxia

boric acid
CO
FMN

1/2O2
+2H

acetoactate
oxaloacetate

NAD+

NADH
Dhase

CoQ.cty.b

cyt.c

cytochrome
oxidase

ketoglutarate
phenytoin

ATP

H2O

*Role of Retinoic Acid
Retinoc acid can inhibit the proliferation
of and induce the apoptosis of EMS cells.

*Role of Folic Acid Deficiency
A high dose of 6 mg of folic acid should be
recommended for the reduction of orofacial
clefts during early pregnancy.

Group I
These are defined as
clefts of the lip only.


Group II
clefts of the palate only
i.e. secondary palatal
clefts.


Group III
clefts of the lip, alveolus
and palate i.e. complete
cleft lip and palate


Group IV
These are defined as clefts of the lip and alveolus


Kernahan’s Striped ‘Y’ Classification


Unilateral cleft lip


Deviation of nasal septum

Superior
constrictor

Palatopharyngeus m.

CRANIOFACIAL CLEFTS

Primary cleft

Secondary cleft

Lateronasomaxillary cleft

Primary Cleft

Maxillomandibular
Cleft

Medionasomaxillary Cleft

Tessiers Classification


Two leading theories proposed for
formation of rare clefts
Classic Theory By Dursy and His:

States that failure of fusion of the facial processes
true clefts

The Mesodermal penetration theory By Johnston
in 1965:
Recognises the importance of neural creast cells


Tessier Classification
Cleft 0:*Represents delay in the closure of the anterior
fontanel through the frontal bone,crista galli,
midline of the nose,columella,lip,and maxilla
and may actually involve the tongue,lower lip and
the mandible.
*It may give rise to frontonasal,frontoethmoidal,
or frontal encephaloceles,duplication of the nasal
septum and midline cleft of the lip.
*Its cranial extension is cleft 14

Cleft 1
*The cleft courses through the frontal bone and
the olfactory groove of the cribiform plate,
between the nasal and the frontal process of the
maxilla and through the maxilla between the
central and lateral incisor.
*It can cause telorbitism with a deep
invagination of the dura in the area of the
cribiform plate defect,nasoorbital encephalocele,
notching of the nostrils and occasionally a cleft
lip.
*Cranial extension is cleft 13

Cleft 2
*Cleft 2 is similar to cleft 1,but is slightly more
lateral.
*Its cranial extension is cleft 12


Cleft 2


Cleft 3

*It runs through the lacrimal bone,the frontal
process of the maxilla and between the lateral
incisor and the canine.
*There is absence of the inferomedial wall of the
orbit and lack of pneumatisation of the
maxillary antrum.
*Medial canthus is displaced inferiorly.There is
coloboma of the lower eyelid,the conjuctiva and
the nasal mucosa being seperated by a thin band
of fibrous tissue.
*Northbound continuation is cleft 11.

Cleft 3


Cleft 4
*Similar to cleft 3 except that the clefting
between the nose and the cheek is more
laterally,sparing the nostrils.
*The lip cresting take place lateral to the
philtral crest


Cleft 4


Cleft 5
*It runs through the orbital floor lateral to
the infraorbital nerve and the maxillary sinus
ending behind the canine in the premolar
region.
*The soft tissue deformity involves the
coloboma of lateral third of lower lip,ending
as the cleft of the lip.

Cleft 6
*Seperates the maxilla from the malar bone.
*The corresponding soft tissue deformity
consists of coloboma of the lower lid and a
furrow of skin from the coloboma to the
angle of the mandible


Cleft 6


Cleft 7
*Courses between the malar and the temporal
bone.
* The zygomatic arch is usually absent.
*The condyle,coronoid and the ramus suffer
varying degrees of deformity.
*The temporal muscle is either absent or
atrophic forming a contineous
temporomassetric muscle.
*There may be cleft of the scalp or abnormal
hair growth.
*There may be a degrees of ear malformations

Cleft 8
*It is a frontozygomatic cleft extending to the
greater wing of the sphenoid.
*In the soft tissue there may be either a true
cleft or notching of the lower eyelid.


Cleft 9
*It is an upper orbital cleft of the superolateral
orbital ridge angle with a corresponding
coloboma of the upper lid.


Cleft 6,7,8 bilateral


Cleft 10

*It is an upper central orbital cleft of the
frontal bone,supraorbital ridge and the
orbital rod,which is lateral to the supraorbital
neurovascular bundle,causing an encephalocele.
*It should be associated with a coloboma of the
medial of the upper lid.


Cleft 11
*It is an upper medial orbital cleft through the
frontal bone,frontal sinus and lateral mass of
the ethmoid.
*The soft tissue deformity is coloboma of the
medial third of the upper eyelid.


Craniofacial anomalies

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