CMF Clinic 28 May, 2004

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

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

CLP & CMF

Caitlyn GRANLAND

Problems:Problems:recurrence of microretrognathiarecurrence of microretrognathia

mandibular relapse vs AP maxillary growthmandibular relapse vs AP maxillary growth

CLP & CMF

Caitlyn GRANLAND

Photographs

14 Jan 2004 14 Jan 2004

CLP & CMF

Caitlyn GRANLAND

14 Jan 200414 Jan 2004

Occlusal intraoral photographs

CLP & CMF

Caitlyn GRANLAND 14 Jan 200414 Jan 2004Dental Models

CLP & CMF

Caitlyn GRANLANDCephalograms

1/12/03

CLP & CMF

Caitlyn GRANLANDOPG

1.12.03

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

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

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

CLP & CMF

Alana SUTHERLAND Photographs: 23/2/04

CLP & CMF

Alana SUTHERLANDCephalograms

10/7/02

CLP & CMF

Alana SUTHERLAND 23/2/0423/2/04Dental Models

CLP & CMF

Alana SUTHERLAND

10 Mar 200410 Mar 2004

Facial helical CT

CLP & CMF

Alana SUTHERLAND

10 Mar 200410 Mar 2004

3D CT Reconstruction

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


Eyelid/orbital reconstructionEyelid/orbital reconstruction

CLP & CMF

Huu Y-Vi NGUYEN DOB 30/01/1987 Age 17 years

DiagnosisDiagnosis::Treacher Collins SyndromeTreacher Collins Syndrome


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

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

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

CLP & CMF

Huu Y-Vi NGUYEN Photographs

CLP & CMF

Huu Y-Vi NGUYEN Dental Models

CLP & CMF

Huu Y-Vi NGUYEN DOB 30/01/1987Cephalograms

19.04.04

CLP & CMF

Huu Y-Vi NGUYEN DOB 30/01/1987OPG

19.04.04

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


?mandibula DOG and Le Fort I osteotomy?mandibula DOG and Le Fort I osteotomy

CLP & CMF

Kate SHIRLEY DOB 6/12/2003 Age 6 months

DiagnosisDiagnosis::Aperts syndromeAperts syndrome


turribrachychephalicturribrachychephalic

wide anterior fontanellewide anterior fontanelle

shallow orbitsshallow orbits

complex Type II syndactylycomplex Type II syndactyly

low broad nasal rootlow broad nasal root

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

CLP & CMF

Kate SHIRLEY

History:

Normal antenatal ultrasound

Anomalies noted at birth

Progress:

Release of syndactyly both hands

first stage 18.05.04

CLP & CMF

Kate SHIRLEY

Plan:

Second stage syndactyly release next month

Fronto-orbital advancement and anterior cranial vault remodelling at 9 months

CLP & CMF

Kate SHIRLEY Photographs

CLP & CMF

Kate SHIRLEY

24 Mar 200424 Mar 2004

3D CT Reconstruction

CMF Clinic 28 May, 2004

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