cmf clinic 28 may, 2004
DESCRIPTION
CMF Clinic 28 May, 2004. Caitlyn GRANLAND DOB 05/01/1989 Mandibular hypoplasia: for repeat distraction and genioplasty Alana SUTHERLAND DOB 23/05/1990 Left anopthalmia: for left orbital/lid reconstruction Huu Y-Vi NGUYEN DOB 30/01/1987 Treacher-Collins Syndrome: mandibular DOG and LF 1 - PowerPoint PPT PresentationTRANSCRIPT
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CLP & CMF
CMF Clinic28 May, 2004
Caitlyn GRANLAND DOB 05/01/1989Mandibular hypoplasia: for repeat distraction and genioplasty
Alana SUTHERLAND DOB 23/05/1990Left anopthalmia: for left orbital/lid reconstruction
Huu Y-Vi NGUYEN DOB 30/01/1987Treacher-Collins Syndrome: mandibular DOG and LF 1
Kate SHIRLEY DOB 6/12/2003 Apert’s Syndrome: for FOA and ACVR
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CLP & CMF
Caitlyn GRANLAND DOB 05/01/1989Age 15 years
DiagnosisDiagnosis: : Mandibular hypoplasiaMandibular hypoplasia
unknown aetiologyunknown aetiology
Clinical features:Clinical features:
class II malocclusionclass II malocclusion
reduced posterior facial heightreduced posterior facial height
reduced TMJ functionreduced TMJ function
History:History:
progressive micrognathia since age 5progressive micrognathia since age 5
possibly related to traumapossibly related to trauma
coeliac disease diagnosed 1997coeliac disease diagnosed 1997
bilateral mandibular distraction osteogenesis Aug 99bilateral mandibular distraction osteogenesis Aug 99
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CLP & CMF
Caitlyn GRANLAND
Problems:Problems:recurrence of microretrognathiarecurrence of microretrognathia
mandibular relapse vs AP maxillary growthmandibular relapse vs AP maxillary growth
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CLP & CMF
Caitlyn GRANLAND
Photographs
14 Jan 2004 14 Jan 2004
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CLP & CMF
Caitlyn GRANLAND
14 Jan 200414 Jan 2004
Occlusal intraoral photographs
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CLP & CMF
Caitlyn GRANLAND
14 Jan 200414 Jan 2004
Occlusal intraoral photographs
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CLP & CMF
Caitlyn GRANLAND 14 Jan 200414 Jan 2004Dental Models
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CLP & CMF
Caitlyn GRANLANDCephalograms
1/12/03
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CLP & CMF
Caitlyn GRANLANDOPG
1.12.03
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CLP & CMF
Caitlyn GRANLAND
Progress:Progress:CMF May 02CMF May 02
defer until complete eruption of 2nd dentition and defer until complete eruption of 2nd dentition and orthodontic treatmentorthodontic treatment
Discussion:Discussion:
Timing ofTiming of mandibular DOG mandibular DOG
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CLP & CMF
Alana SUTHERLAND DOB 23/05/1990
Age 14 yearsDiagnosisDiagnosis::left anopthalmia-microorbitleft anopthalmia-microorbit
Clinical featuresClinical features::
orbitomalar hypoplasiaorbitomalar hypoplasia
left anopthalmia/microorbitleft anopthalmia/microorbit
right orbital volume 4x largerright orbital volume 4x larger
isolated soft palate cleftisolated soft palate cleft
intracerebral cystintracerebral cyst
13 paired ribs13 paired ribs
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CLP & CMF
Alana SUTHERLAND
Past HistoryPast History::
subperiorbita scleral insertionssubperiorbita scleral insertions
orbital tissue expander, orbital osteotomies Nov orbital tissue expander, orbital osteotomies Nov 19951995
removed Oct 96removed Oct 96
Problem:Problem:
short palpebral fissure (10mm horizontal, 5mm vertical) andshort palpebral fissure (10mm horizontal, 5mm vertical) and
shallow orbitshallow orbit
limited space for insertion of prosthesislimited space for insertion of prosthesis
left brow ptosisleft brow ptosis
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CLP & CMF
Alana SUTHERLAND Photographs: 23/2/04
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CLP & CMF
Alana SUTHERLAND Photographs: 23/2/04
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CLP & CMF
Alana SUTHERLANDCephalograms
10/7/02
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CLP & CMF
Alana SUTHERLAND 23/2/0423/2/04Dental Models
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CLP & CMF
Alana SUTHERLAND
10 Mar 200410 Mar 2004
Facial helical CT
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CLP & CMF
Alana SUTHERLAND
10 Mar 200410 Mar 2004
3D CT Reconstruction
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CLP & CMF
Alana SUTHERLAND
Progress:Progress:
Managing well at school - no teasingManaging well at school - no teasing
aiming to be nurse or teacheraiming to be nurse or teacher
Discussion:Discussion:
Eyelid/orbital reconstructionEyelid/orbital reconstruction
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CLP & CMF
Huu Y-Vi NGUYEN DOB 30/01/1987 Age 17 years
DiagnosisDiagnosis::Treacher Collins SyndromeTreacher Collins Syndrome
Clinical featuresClinical features::
Downward slanting eyesDownward slanting eyes
Bilateral ear atresiaBilateral ear atresia
Marked hypoplasia of zygomaMarked hypoplasia of zygoma
Absent arches, right mandibular condyleAbsent arches, right mandibular condyle
Wide cleft palateWide cleft palate
Narrow nasopharynxNarrow nasopharynx
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CLP & CMF
Treacher Collins SyndromeMandibulofacial dysostosisMandibulofacial dysostosis
Autosomal dominant, 50% sporadic
chromosome 5q3 - treacle gene
1 in 10,000 live births
Features: symmetrical involvement
* down-slanting eyes, lower eyelid coloboma
* underdevelopment or absence of zygoma and orbital floor
* micrognathic and steep mandibular plane angle
* underdeveloped, malformed and/or prominent ears
* normal IQ
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CLP & CMF
Huu Y-Vi NGUYEN DOB 30/01/1987
Past HistoryPast History::
Feeding difficulty - NG feed until age 5Feeding difficulty - NG feed until age 5
cleft palate repair x3cleft palate repair x3
tracheostomy required post optracheostomy required post op
orbitomalar augment with bone graft 1992orbitomalar augment with bone graft 1992
conductive hearing loss - hearing aidconductive hearing loss - hearing aid
velopharyngeal incompetencevelopharyngeal incompetence
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CLP & CMF
Huu Y-Vi NGUYEN Photographs
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CLP & CMF
Huu Y-Vi NGUYEN Dental Models
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CLP & CMF
Huu Y-Vi NGUYEN DOB 30/01/1987Cephalograms
19.04.04
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CLP & CMF
Huu Y-Vi NGUYEN DOB 30/01/1987OPG
19.04.04
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CLP & CMF
Huu Y-Vi NGUYEN DOB 30/01/1987
Problems:Problems:
short posterior facial heightshort posterior facial height
steep occlusal planesteep occlusal plane
retrusive chinretrusive chin
ProgressProgress::
Coping well at school, achieving academicallyCoping well at school, achieving academically
Parents ambivalent about surgeryParents ambivalent about surgery
Discussion:Discussion:
?mandibula DOG and Le Fort I osteotomy?mandibula DOG and Le Fort I osteotomy
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CLP & CMF
Kate SHIRLEY DOB 6/12/2003 Age 6 months
DiagnosisDiagnosis::Aperts syndromeAperts syndrome
Clinical featuresClinical features::
turribrachychephalicturribrachychephalic
wide anterior fontanellewide anterior fontanelle
shallow orbitsshallow orbits
complex Type II syndactylycomplex Type II syndactyly
low broad nasal rootlow broad nasal root
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CLP & CMF
Apert’s SyndromeAcrocephalosyndactyly Type 1
Eugene Apert, 1868–1940, French paediatrician
Autosomal dominant, 90% sporadic
chromosome 10q - FGFR2 gene
1 in 160,000 live births
Features:
* craniosynososis
* syndactyly of hands and feet
* midface hypoplasia
* mental retardation
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CLP & CMF
Kate SHIRLEY
History:
Normal antenatal ultrasound
Anomalies noted at birth
Progress:
Release of syndactyly both hands
first stage 18.05.04
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CLP & CMF
Kate SHIRLEY
Plan:
Second stage syndactyly release next month
Fronto-orbital advancement and anterior cranial vault remodelling at 9 months
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CLP & CMF
Kate SHIRLEY Photographs
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CLP & CMF
Kate SHIRLEY Photographs
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CLP & CMF
Kate SHIRLEY
24 Mar 200424 Mar 2004
3D CT Reconstruction
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CLP & CMF
Kate SHIRLEY
24 Mar 200424 Mar 2004
3D CT Reconstruction