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RARE CRANIOFACIAL CLEFTS :
A Review
Dr. Pritham N. ShettyMDS
Prof. Y.S.Rai MS FRCS
Dr. Niranjan KumarMS MCh
Dr. GopalkrishnanMDS FDSRCS
Dr. Dinesh KadamMS MCh
Departments of Maxillofacial & Plastic Surgery
Craniofacial Surgery & Research Centre
S.D.M. College of Dental Sciences & Hospital
Dharwad, Karnataka S D MDHARWADDEC 2003
MANGALORE
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KULUMUS in 1732
EtiopathogenesisFailure of mesodermal structures to fuse
Vascular disruption
Aberrant tissue bandsRupture of the amnion
RARE CRANIOFACIAL CLEFTS
S D MDHARWAD
DEC 2003MANGALORE
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No. of cases: 27 out of 1350 clefts
Incidence: 2% (9.534 for every1000 clefts)
Male : female:- 15:12
Duration of the study : 20012003
RARE CRANIOFACIAL CLEFTS
S D MDHARWAD
DEC 2003MANGALORE
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CLASSIFICATION
American Association of Cleft palate
rehabilitation
Boo-Chai
Karfik
Van der meulen
Tessier
RARE CRANIOFACIAL CLEFTS
S D MDHARWAD
DEC 2003MANGALORE
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Tessier classification
RARE CRANIOFACIAL CLEFTS
S D MDHARWAD
DEC 2003MANGALORE
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Tessier classification
RARE CRANIOFACIAL CLEFTS
S D MDHARWAD
DEC 2003MANGALORE
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RARE CRANIOFACIAL CLEFTS
10
21
9
1
4
Tessier 0
Tessier 3Tessier 4
Tessier 5
Tessier 6
Tessier 7
S D MDHARWAD
DEC 2003MANGALORE
Distribution
n = 27
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Tessier no 0
Midline deformity of the upper lip & nose
Commonly associated with no 14 cleft
C.T. scan of the brain
Tissue
RARE CRANIOFACIAL CLEFTS
Deficiency
Excess
S D MDHARWAD
DEC 2003MANGALORE
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RARE CRANIOFACIAL CLEFTS
S D MDHARWAD
DEC 2003MANGALORE
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RARE CRANIOFACIAL CLEFTS
S D MDHARWAD
DEC 2003MANGALORE
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Tessier no3(oblique or oro-naso-ocular cleft)
Morion
Cleft passes through the mouth,
nose, maxillary sinus and orbit
Nasolacrimal drainage systemPreservation of vision
Canthopexy
Skeletal reconstruction
RARE CRANIOFACIAL CLEFTS
S D MDHARWAD
DEC 2003MANGALORE
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RARE CRANIOFACIAL CLEFTS
S D MDHARWAD
DEC 2003MANGALORE
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RARE CRANIOFACIAL CLEFTS
S D MDHARWAD
DEC 2003MANGALORE
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Tessier no 4(oro-ocular cleft)
Boo-Chai
Cleft starts lateral to the
cupids bow and end at the
lower eyelid medial to the
punctum
,
RARE CRANIOFACIAL CLEFTS
Osseous component is less extensive
Loss of vision
MicrophthalmosS D M
DHARWADDEC 2003
MANGALORE
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RARE CRANIOFACIAL CLEFTS
S D MDHARWAD
DEC 2003MANGALORE
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Tessier no 5(oculofacial cleft II)
Cleft starts medial to the oral
commissure, traverses the cheekto enter the lower eyelid in its
middle third
Very rareUsually associated with no. 9 cranial cleft
Early correction to prevent blindness
Orbital dystopia
RARE CRANIOFACIAL CLEFTS
S D MDHARWAD
DEC 2003MANGALORE
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Tessier no 6(zygomaticomaxillary cleft)
Cleft presents as a vertical furrow
from the commissure to the lateralaspect of the inferior eyelid
RARE CRANIOFACIAL CLEFTS
Incomplete form of treacher-collins
syndromeNo alveolar cleftLower lid ectropionColobomasRarely hearing deficit
S D MDHARWAD
DEC 2003MANGALORE
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Tessier no 7(Hemifacial microsomia ,first and
second branchial arch syndrome)
Cleft runs from the oralcommissure to the pre auricular
region, never crosses the anterior
border of masseter
Most common
Males are more commonly
affected
RARE CRANIOFACIAL CLEFTS
S D MDHARWAD
DEC 2003MANGALORE
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Associated anomalies:
Fifth or seventh cranial nerve weakness
Accessory auricular tags
Coloboma
Facial asymmetry
Epibulbar dermoid
Parotid gland or duct may be absent
Fascial skeletal discrepancy
RARE CRANIOFACIAL CLEFTS
S D MDHARWAD
DEC 2003MANGALORE
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RARE CRANIOFACIAL CLEFTS
S D MDHARWAD
DEC 2003MANGALORE
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Tessier no 11Cleft transverses the medial third
of the upper eye lid ,the eye brow
and progress towards the frontal
hairline
RARE CRANIOFACIAL CLEFTS
Cranial extension of no 3 cleft
orbital hypertelorism
pneumatisation of ethmoid air cell
S D MDHARWAD
DEC 2003MANGALORE
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Hemifacial microsomiaGoldenhar syndrome
Treacher - collins syndrome
RARE CRANIOFACIAL CLEFTS
S D MDHARWAD
DEC 2003MANGALORE
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RARE CRANIOFACIAL CLEFTS
S D MDHARWAD
DEC 2003MANGALORE
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CONCLUSIONNeeds immediate attention
Multiple surgeries
Other anomalies
The greatest obstacles in the reconstruction
of rare craniofacial clefts depends upon the
extent of soft tissue hypoplasia and our
limited ability to normalize them
RARE CRANIOFACIAL CLEFTS
S D MDHARWAD
DEC 2003MANGALORE
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Thank YouS D MDEC 2003
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