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Cleft Lip & Palate From Orthodontic Point of View

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Page 1: Cleft lip and palate by almuzian

Cleft Lip & Palate

From Orthodontic Point of View

Page 2: Cleft lip and palate by almuzian

Table of ContentsDefinition.................................................................................................................................1

Incidence..................................................................................................................................1

Syndromic and non-Syndromic clefting...............................................................................2

Embryology of clefts of the lip and palate................................................................................3

Lip development.......................................................................................................................3

Abnormal lip Development...................................................................................................5

Development of the palate........................................................................................................6

Theories of palatal shelf elevation. (Ferguson 1981)................................................................6

Abnormal palate Development.............................................................................................7

Aetiology..................................................................................................................................8

Classifications...........................................................................................................................9

Descriptive method by Veau.................................................................................................9

Symbolic method using the “stripped Y” Kernahan 1971.......................................................10

LAHSHAL classification developed by Kriens 1989.............................................................10

Preventive treatment...............................................................................................................11

CSAG Report (Clinical Standards Advisory Group) by Shaw 1995.......................................11

Indices and grading used in cleft cases...................................................................................13

Index of 5-year old children (Attack et al., 1997):..................................................................13

Grading...........................................................................................................................13

GOSLON index (Great Ormond Street, London and Oslo Net) Yardstick (Mars et al., 1987)................................................................................................................................................15

This depend on..............................................................................................................15

I. Anteroposterior Assessment of labial segements....................................................15

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II. Vertical Assessment................................................................................................16

III. Transverse Assessment.......................................................................................16

Ranking of GOLSON index...................................................................................................16

1. Groups 1 and 2 have occlusions that require either straightforward orthodontic treatment or none at all...................................................................................................16

2. Group 3 require complex orthodontic treatment to correct the Class III malocclusion but a good result can be anticipated................................................................................16

3. Group 4 are at the limits of orthodontic treatment, and if facial growth is unfavorable, orthognathic surgery will be required.........................................................16

4. Cases in group 5 require orthognathic surgery........................................................16

Bergland index for secondary ABG outcomes........................................................................16

Kindelan score........................................................................................................................17

Problems Associated with Cleft Lip and Palate......................................................................18

I. General difficulties.........................................................................................................18

II. Dental disturbances in both repaired and unrepaired cleft cases.....................................18

III. Skeletal Features of unrepaired cleft lip and palate.....................................................19

IV. Skeletal Features of repaired cleft lip and palate (Shaw 1990)....................................19

Iatrogenic effect of surgery.....................................................................................................20

Ideal Cleft palate Team...........................................................................................................20

Summary of the whole Treatment...........................................................................................21

In details:................................................................................................................................24

Prenatal age............................................................................................................................24

At Birth...................................................................................................................................24

Three Months of age...............................................................................................................26

D. Dental roles:............................................................................................................27

6-12 Months of age.................................................................................................................27

A. Palatal Repair..........................................................................................................27

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B. Sometime Lip and soft palate repair undertaken at 6 months at one time...............28

C. Dental roles:............................................................................................................28

D. Pharygoplasty:........................................................................................................28

Aetiological factors pf speech problems:-.......................................................................29

1-5 Years of age......................................................................................................................29

1. Lee records at 5 years stage....................................................................................29

2. Assessment using the 5-year-old index introduced by Attack 1997........................29

3. Interceptive Orthodontic treatment.........................................................................29

4. Dentist roles............................................................................................................30

5. Speech and hearing assessment...............................................................................30

6. Primary bone grafting.............................................................................................30

7-10 years of age.....................................................................................................................30

1. Lee’s records...........................................................................................................30

2. GOSLON Yardstick (Mars 1987)...........................................................................30

3. Secondary alveolar bone grafting:...........................................................................30

The main aims secondary ABG......................................................................................32

Surgical technique of ABG.............................................................................................33

Postoperative instruction.................................................................................................34

Postoperative assessment................................................................................................34

The complications...........................................................................................................34

Influencing success.........................................................................................................35

Segmental Surgery at the same time of the secondary alveolar bone grafting, Harris 2008 36

Lesser Segment Alveolar Distraction..................................................................................36

11-15 Years of age..................................................................................................................37

Pharyngoplasty...............................................................................................................37

Orthodontics...................................................................................................................37

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18+ Years of age.....................................................................................................................38

1. Lee’s Records.........................................................................................................38

2. Orthognathic surgery..............................................................................................38

Secondary surgical correction for CLP patient.......................................................................38

Specific Problems in Cleft Patients.....................................................................................38

Treatment Planning for CLP...............................................................................................39

The Choice of Operation for CLP.......................................................................................40

Airway Considerations for CLP during surgery......................................................................42

Postoperative considerations for CLP.................................................................................43

3. Secondary plastic procedures..................................................................................44

Terminology...........................................................................................................................44

Velopharyngeal impairment............................................................................................44

Velopharyngeal insufficiency.........................................................................................44

Velopharyngeal incompetenceis.....................................................................................44

Hypernasality..................................................................................................................44

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Cleft Lip & Palate

From Orthodontic Point of View

Definition

Incomplete fusion of hard and /or soft tissue structures of the lip and

palate.

Incidence

A. Genetic risks

One affected parent, risk of the first child 2%

One affected child, risk of next child with is (4%).

One affected parent and one affected child risk of next child

with is 10%

Two affected parents, risk of first child 60%

B. Prevalence in the UK population

UCLP 40%

CP 30%

BCLP 10%

CL 10%

Submucous cleft or soft palate cleft 10%

C. CLP

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Incidence of unilateral CL(P) varies with race:

1. In Negros is around 1 in every 2000 live birth.

2. In UK 1 in every 700 live births

3. In Caucasians it is about 1 in every 750 live births (Mitchell, 2000).

4. In oriental populations is around 1 in very 600 live births.

5. Left side is more affected than the right side (2:1).

6. More common in male 3:1

D. CP:

Prevalence around 1:2000 live births.

55% Associated with syndromes such as Down, Treacher-

Collin, Pierre-Robin sequence.

CP the incidence is higher in females overall (3:2).

E. Gender distribution:

CLP has greater incidence in males 3:1

CP the incidence is higher in females overall (3:2).

There is a male predominance of submucus clefts.

There is equal gender incidence of isolated soft palate clefts and

cleft lip alone

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Syndromic and non-Syndromic clefting

15% of cleft children have additional malformations especially BCLP and

CP to have additional malformations (400 syndrome) , example:

1. Van der Woude

1:28000

Autosomal dominant.

Lower lip pits

+/or CL/P or CPO (2% of cleft cases).

Hypodontia.

No other anomalies.

2. Pierre Robin sequence. Triad of cleft palate, micrognathia,

macroglossia

3. Treacher Collins

Embryology of clefts of the lip and palate

Lip development

Facial development begins at 4-6 weeks

5 facial prominences. Frontonasal process ‘’FNP’’ (unpaired), paired

maxillary process and paired mandibular process

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By four weeks of development the two mandibular processes are the

first to unite and give rise

to the lower lip, lower

portion of the cheeks and

other mandibular

structures.

By five weeks of

development, medial and

lateral nasal processes form

within the enlarged

frontonasal process to

surround an early

ectodermal thickening, the

nasal placode.

The nasal placode gives

rise to highly specialized

olfactory receptor cells and nerve fibre bundles innervating the future

nasal cavity.

As the medial and lateral nasal processes enlarge, the nasal placodes

sink into the nasal pits, which demarcate the nostrils.

Medial growth of the maxillary processes dominates subsequent

development of the face, resulting first in contact and then fusion with the

lateral nasal processes (6 weeks IU) to form:

1. Nasolacrimal duct

2. Cheek

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3. Alar base of the future nose.

Further growth towards the midline pushes the lateral nasal processes

superiorly and allows fusion of the maxillary processes with the medial

nasal processes inferiorly, merging them together in the midline to form:

1. Central portion of the nose;

2. Upper lip philtrum;

3. Primary palate.

Thus, the upper lip is formed from the maxillary processes laterally

and the medial nasal processes in the midline (Jiang et al, 2006).

Posteriorly, from the medial sides of the maxillary process, the

secondary palate is formed via growth, elevation and subsequent fusion

between the paired palatine processes. These processes also fuse with the

nasal septum superiorly and the primary palate anteriorly, ultimately

separating the oral and nasal cavities. The essential features of the human

face have formed by eight weeks of development.

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Abnormal lip Development

Defective fusion at any of the sites highlighted in the above figures may

result in a facial cleft.

1. Cleft mandible

2. Lateral facial cleft

3. Oblique facial cleft

4. Cleft Lip (Unilateral or Bilateral)

5. Median cleft

Development of the palate

1° palate is made up of the medial nasal process. It contains the first

four teeth and contributes the philtrum of the upper lip.

2° palate apparent at 6 weeks as inferiorly lying outgrowths from the

maxillary process, lying lateral to the tongue.

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At 8 weeks shelf elevation begins.

Theories of palatal shelf elevation. (Ferguson 1981)

Extrinsic

1. Straightening of the cranial base.

2. Lifting of the head relative to the body.

3. Increased height of the oro-nasal cavity.

4. Tongue movement downward.

5. Increased mandibular prominence.

Intrinsic

1. Increase in the osmotic pressure of the palatal shelves

2. Cellular reorganisation (increased density of epithelial/mesenchymal cells

on the palatal side of the shelf causing rotation),

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3. Contraction (muscle/non-muscle)

4. Vascular erectile force.

Following elevation, at 9 weeks, further growth brings the medial edge of

each shelf into close contact. At this stage, mesenchyme from each shelf

is still separated by an epithelial seam of medial edge epithelium.

Three mechanisms have been proposed to explain medial edge epithelium

breakdown:

1. Apoptosis (programmed cell death)

2. Epithelial to mesenchymal transformation, and

3. Migration of epithelium to the oral and nasal compartments.

Regardless of the mechanism, breakdown of the epithelial seam results in

mesenchymal continuity and palatal fusion. As well as fusion between

secondary palatal shelves, an important step during palatogenesis is

fusion of the primary palate to the secondary palate.

Abnormal palate Development

Clefts form when there is failure of process growth or fusion, this is due

to:

1. Primary defects leading to cleft palate include:

Failure of shelf elevation

Failure of shelf growth

Failure of shelf fusion

Breakdown of shelf fusion

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2. Secondary defects leading to cleft palate include:

Growth disturbances in craniofacial structures

Mechanical obstruction of palatal elevation.

Aetiology

In normal development, fusions of the embryological processes that

comprise the upper lip appear around 6 W.I.U life while fusion to form

the secondary palate occur around 8 W.I.U life. Any disruption affecting

the timing at which the fusion occurs will increase the incidence of cleft.

The etiological factors are:

A. Genetic: A gene coding for TGF has been implicated. These

include: homebox gene, SHH, MSX1, MSX2, BMP-2, FGF-8

B. Environmental

IU position

Social deprivation

Smoking

Alcohol

Trauma.

Radiation.

Maternal hypoxia

Drugs like Steroids, Anticonvulsant drugs

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Infection like CMV, Rubella

Endocrine like Diabetes

Deficiency of nutritional supplements such as deficiency in folic acids

Bixler (1981) divided clefts into 3 aetiological domains

C. Syndromic . Represent 70% of clefting

D. Familial or hereditary .

E. Sporadically or Isolated or non-Familial . The patient is the first

person in a family with the defect. Most commonly:

IU position

Social deprivation

Smoking

Alcohol

Trauma.

Radiation.

Maternal hypoxia

Drugs like Steroids, Anticonvulsant drugs

Infection like CMV, Rubella

Endocrine like Diabetes

Deficiency of vitamin supplements such as deficiency in folic acids

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Classifications

Descriptive method by Veau

This is most commonly used nowadays.

A. Cleft lip

Notched lip

Incomplete cleft lip

Complete cleft lip

Unilateral or bilateral

B. Cleft alveolus (primary palate)

C. Cleft palate

Cleft uvula

Soft palate only

Submucous cleft

Complete

Incomplete

D. Combinations

Symbolic method using the “stripped Y” Kernahan 1971

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LAHSHAL classification developed by Kriens 1989

L lip

A alveolus

H hard palate

S soft palate

UPPER CASE FOR COMPLETE CLEFT

Lower case for incomplete cleft

It starts from right side to left side

modified by the Royal College of Surgeons, omitting one ‘H’

Preventive treatment

Hartridge et al 1999 in a review investigating the role of pre-conceptional

folic acid supplementation concluded that 0.4 mgs of folic acid from pre-

conception to the 12 week of pregnancy (4mgs for mothers with cleft

children) although not proved conclusively can have significant

protective effects.

CSAG Report (Clinical Standards Advisory Group) by Shaw 1995

First Mars 1987 show that UK is the worst.

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Professionals in the field of cleft work expressed concern regarding the

quality of treatment outcome for patients with cleft lip and palate in the

UK.

This was based principally upon the outcome of two studies:

A. The GOSLON Yardstick (Great Ormond Street, London and Oslo)

was developed as a clinical tool that categorised dental arch

relationships into five discrete categories. Using this yardstick,

comparison between UK and Norwegian cleft centres demonstrated

significant shortcomings in outcomes associated with the UK

centre.

B. Eurocleft - a European, multicentre clinical audit of treatment

outcome for complete unilateral CLP. It found the two UK centres

that participated to be weakest on almost every aspect of care

In 1995 the Department of Health in the UK charged the Clinical

Standards Advisory Group to investigate the quality of care within the

UK.

All children in the UK with a unilateral complete cleft lip and palate aged

5 or 12 years of age in 1996-1997 were examined. Their speech, hearing,

facial appearance, dental malocclusion, dental health, quality of bone

graft and skeletal base relationships were examined. (457 children non

syndromic with UCLP)

Cleft care was provided in 57 centres.

The study found that the average result in all these areas was poor.

Children from the UK centres were more likely to suffer mid-face

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retrusion (70% of pt 12 years has class III) and poor dental relationships

than three of the European centres.

Fewer than 60% of children in the UK had a successful bone graft in

comparison with 97% from one of the other European centres. It was

therefore clear that some patients were not receiving optimal care in the

UK.

The CSAG1 report 1998 made several recommendations, including:

I. Centres should be limited to 8-15 in the UK.

England and Wales, 10 centres

Northern Ireland operates as a single centre

Scotland operates as one single centre known as CLEFTSiS

II. Each centre should provide a full range of cleft care.

III. Nationwide database.

IV. Results should be regularly audited.

V. Training should be provided for specialists in cleft care in high

volume centres only.

VI. Each clinical team consists of specialist orthodontists, surgeons,

speech and language therapists, specialist nurses, geneticists,

paediatricians, ENT specialists, anaesthetists and psychologists. In

addition they have support staff responsible for data collection, audit

documentation and photography.

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Indices and grading used in cleft cases

Index of 5-year old children (Attack et al., 1997):

Index for dental relationships of 5 year old patients born with

unilateral cleft lip and palate.

It divided the cases into 5 categories to be able to compare

treatment outcomes earlier and before surgical procedures and

orthodontic treatment.

The categories used in the grading are:

1. Overjet

2. Inclination of ULS

3. Presence of Crossbite

4. Presence of Open bite

5. Maxillary arch shape and palatal vault anatomy

Grading

1. Grade I

Positive overjet

Average inclined or retroclined incisors

No crossbites

No openbites

Good maxillary arch shape and palatal vault anatomy.

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Prognosis: Excellent outcomes

2. Grade 2

Positive overjet

Average inclined or proclined upper incisors

Unilateral crossbite/crossbite tendency

Open bite tendency around cleft site.

Prognosis: Good outcomes

3. Grade 3

Edge-to-edge bite average inclined or proclined incisors;

OR

Reverse overjet with retroclined incisors

Unilateral crossbite

Open bite tendency around cleft site

Prognosis: Fair outcomes

4. Grade 4

Reverse overjet

Average inclined or proclined incisors

Unilateral crossbite, bilateral crossbite tendency

Open bite tendency around cleft site .

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Prognosis: Poor outcomes

5. Grade 5

Reverse overjet

proclined incisors

Bilateral crossbite

Open bite

Very Poor maxillary arch form and palatal vault anatomy

Prognosis: Very poor outcomes

GOSLON index (Great Ormond Street, London and Oslo Net)

Yardstick (Mars et al., 1987)

It was developed by Mars and used to compare UK results with the

rest of Eu, which actually instigate the CSAG

It is a record of 10 year old patients

It measures the severity of malocclusion, the difficulty of

correcting it and the outcomes of the child with a unilateral cleft lip and

palate of children in the early permanent dentition

This depend on

I. Anteroposterior Assessment of labial segements

The overjet is examined first. If there is a reverse overjet of 3-5

mm, this indicates that the case might belong to group 3.

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However, if there is already dentoalveolar compensation a higher

category should be considered.

The anteroposterior relationships of the buccal segments are not of

importance in determining the grouping of a case.

II. Vertical Assessment

It helps in modification of the provisional category in borderline cases.

Deep bite is favorable and AOB is unfavorable

III. Transverse Assessment

It indicate a modification of the provisional category in borderline cases

Ranking of GOLSON index

1. Groups 1 and 2 have occlusions that require either straightforward

orthodontic treatment or none at all.

2. Group 3 require complex orthodontic treatment to correct the Class III

malocclusion but a good result can be anticipated.

3. Group 4 are at the limits of orthodontic treatment, and if facial growth is

unfavorable, orthognathic surgery will be required.

4. Cases in group 5 require orthognathic surgery.

Bergland index for secondary ABG outcomes

Take periapical x-ray and assess the bone formation at interseptal area

around the ERUPTED canine to assess bone formation Bergland (1986).

1. Grade I: inter-alveolar bone at normal height

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2. Grade II: inter-alveolar bone ¾ of normal height

3. Grade III: inter-alveolar bone less than ¼-¾ of normal height

4. Grade IV: no bone at inter-alveolar area. Failed outcomes.

Kindelan score

After 4-6 months of ABG, take anterior occlusal radiograph and

assess the success using Kindelan score 1997.

It has an advantages that it can be applied even before the eruption

of the canine.

The degree of bony fill in the cleft area was assessed using a 4-

point scale:

1. Grade 1 > 75% bony fill;

2. Grade 2 50-75% bony fill;

3. Grade 3 < 50% bony fill;

4. Grade 4 no complete bony bridge.

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Problems Associated with Cleft Lip and Palate

I. General difficulties

1. Feeding

2. Speech

3. Hearing (which in turn can effect speech development) and middle

ear infections

4. Psychological problems

II. Dental disturbances in both repaired and unrepaired cleft cases

1. Hypodontia , 28% of UCLP and 60% BLCP

2. Supernumeraries

3. Delayed eruption of teeth on cleft side

4. Increased incidence of impacted upper first molar in cleft side (4times

than non-clefts individuals) (Bjerklin et al., 1993)

5. Dilacerations

6. Hypoplasia

7. Microdontia

The above due to:

Distortions of the development of the dental lamina which produce

tooth germs. In the patient with a cleft this process is disturbed and result

in dental problems.s

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Msx1 genes mutation.

III. Skeletal Features of unrepaired cleft lip and palate

It is called Embryological defects

1. Cleft Lip only, maxillary arch development is generally normal.

2. Clefts in to the alveolus (incomplete) only with or without lip,

increased incidence of cross bites (19%)

3. Complete bilateral, Premaxilla is anteriorly displaced beyond the tip of

the nasal septum. The lateral segments may have collapsed medially

producing bilateral crossbites.

4. Complete unilateral, Major segment is rotated outward so the incisor

area appears prominent, the lesser (lateral) segment is more variable and

may be rotated outwards producing a wide cleft or there may be inward

displacement and segment overlap.

5. Isolated clefts of the palate, Excessive inter-tuberosity width may be

observed causing a scissor bite bilaterally

6. Mandibular growth reduced genetically

7. Increase MMP angle, Possibly due to

Disrupted nasal respiration leading to oral respiration and a mouth open

posture, allowing buccal segments to over-erupt.

IV. Skeletal Features of repaired cleft lip and palate (Shaw 1990)

Embryological defects + Iatrogenic effect of surgery

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Iatrogenic effect of surgery

Lip repair has minimal effect on facial growth.

1. AP disturbances. Palatal scar tissue around the tuberosity region hinders

maxillary translation.

2. Transverse disturbances. Scar tissue in the palate leads to a tendency for

buccal cross bites in the 1and 2 dentitions.

3. Vertical disturbances.

An in LFH is often found. Possibly due to disrupted nasal respiration,

oral respiration and a mouth open posture, allowing buccal segments to

over-erupt.

Ideal Cleft palate Team

1. Cleft nurse

2. Plastic surgeon

3. Orthodontist

4. Maxillofacial surgeon

5. ENT surgeon

6. Speech therapist

7. Audiologist

8. Pediatrician

9. Psychologist

10.Geneticist

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Summary of the whole Treatment In red are the roles of the orthodontist and GDP

Prenatal Ultrasound assessment, 70% of the cases are

detected on ultrasound scan at 16-18 weeks

At birth Parent counseling

Feeding

Pre-surgical orthopaedic appliance

6 months Primary surgical lip repair

Primary alveolar bone grafting (old regime)

Nasal repair

Tympanoplasty or grommet,

1 year Palate repair

Preventive dentistry/advice

2-7 years Revision of lip repair

Pharyngoplasty

Tympanoplasty or grommet,

Lee’s records

5 years Index Assessment

Interceptive orthodontic to:

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Correct X bite

Align the maxillary dentition (usually using

fixed appliances) in the growing child if the

appearance causes the child distress or the

irregular teeth are traumatizing soft tissues

Cleft orthodontists can be asked to provide

obturators to assist with speech prior to closure of

any residual fistulae at the time of alveolar bone

grafting

8-10 years Lee’s records

GOSOLN Index Assessment

Maxillary expansion prior to bone grafting,

extract supernumerary teeth and retained primary

teeth.

Bone grafting

OHI and optimal oral health

11-15 Lee’s records

Definitive alignment of the maxillary and

mandibular teeth using fixed appliances

Reverse facial mask

16-more Lee’s records

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Orthognathic surgery

Decompensation and alignment for

orthognathic surgery using fixed appliances

For patients with velo-pharyngeal

dysfunction, the poorly functioning soft palate is

raised with a palatal lift appliance and the velo-

pharyngeal space obturators to reduce hypernasal

speech, which assists the Speech and Language

Therapist in cases that are otherwise untreatable

by language therapy alone with/without surgery.

Electropalatography is a relatively new

technique where patients are provided with an

upper removable orthodontic appliance

incorporating numerous electrodes. When attached

to a PC, the patient can visualize tongue to hard

palate contact on various sounds and the Speech

and Language Therapist can direct therapy

sessions using this technique. Indeed portable EPG

hardware is now available such that the patient can

practice tongue positioning at home.

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In details:

Prenatal age

70% of the cases are detected on ultrasound scan at 16-18 weeks when

looking for it.

Cleft Lip and Palate Association (CLAPA) provide support for the

parents this stage.

Nurse to provide home visit for support

Psychologist to provide support

At Birth

1. Parent counseling

Parents are usually in a shock after birth, therefore a counseling is

important to reassure them and facilitate the development of a bond

between the mother and the child. The parent reaction could be

depression, social avoidance, rejection and feelings of guilt

2. Feeding:

Orthodontist should give counselling and advice on feeding.

Acrylic plates are no longer used nowadays.

In isolated CL the nipple will fill the gap so using large teats bottle

is enough.

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Soft feeding bottles with modified long teats which help to direct

the flow of the milk into the mouth are helpful. (Haberman feeder or

bottle or soft Plas bottle)

Fortified milk

Some babies are fed by nasogastric tube. One of the most common

reasons for a cleft baby being fed this way is due to Pierre Robin

sequence.

3. Airway

Many of these babies have severe airway problems and due to the

smallness of the lower jaw, the tongue remains in a very posterior

position, making oral feeding impossible for weeks or even months.

4. Pre-surgical orthopaedics:

A. NASOALVEOLAR MOULDING: NAM inserted in the nostrile

NAM uses an intraoral molding plate to gradually approximate the

displaced alveolar segments, which results in approximation of the cleft

lip segments as well as the medial and lateral aspects of the nose.

Once the underlying alveolar segments are approximated, the

overlying lip and nasal soft tissues attain a degree of laxity that permits

reshaping and alignment with the introduction of the nasal stent.

In addition, owing to the plasticity of the alar cartilage during

infancy, the achieved changes can become permanent

It is indicated in:

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1. Infant with complete unilateral cleft lip and palate (UCLP) and the

associated nasal deformity

2. Infants born with complete bilateral cleft lip and palate (BCLP)

often present with the premaxillary segment positioned outside the

oral cavity, a wide nasal tip and alar base, and a severely deficient

columella.

if nasal moulding is required then it should be add after dentolaveolatr

moulding

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Potential Complications and Clinical Pearls of NAM1. Soft Tissue Complications

Mucosal irritatiom

Nasal irritation

Cheek irritation

2. Hard Tissue Complications

severe reduction in the width of an alveolar segment in the labiopalatal dimension which leads to an ectopic or premature eruption of the underlying primary teeth.

Sometimes a neonatal tooth is present on the cleft edge of the alveolar process. These “teeth” are ectopically located in a soft tissue sack that covers the bone on the alveolar gap and

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often must be surgically removed prior to the initiation of NAM therapy.

3. Compliance

Various restraints can be employed to prevent the child from removing the NAM ppliance from its mouth. (a) Mittens can be seen covering the hands; (

A. NASODENTAL MOULDING:

a. McNeil type plate

Pioneered by McNeil.

It is usually carried out immediately after birth.

The types and their aims are:

In unilateral clefts reduces displacement of the greater segment

and maintain the position of the lesser segment.

In bilateral clefts to move the lateral segments outwards while the

prolabium is moved palatally and rotated downwards. Reduction of

premaxillary protrusion in bilateral clefts. Treatment comprises an intra

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oral appliance carrying an active component (screws) to separate the

lateral segments. Elastic strapping across the prolabium (upper lip) is

used to restrain the premaxillary growth.

b. lip taping

c. a pin-retained Latham

appliance (Latham lip

strapping)

B. OBTURATORS

Passive obturating plates that assist feeding

Active obturating plates

Recent evidences by Shaw et al in 1992 showed that:

Low outcome if presurgical orthopaedic is used

Dutch-cleft study by Anderson suggest that these devices offer

no benefit to outcome either in terms of the surgery or feeding

during this period.

SIX Months of age

A. Lip Repair

Tennison technique or Randall technique (grives Fuller lip)

Millard technique (gives best scar)

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B. Nose Repair: alar cartilages may be repositioned at this time to increase

symmetry and improve the appearance

C. Alveolar Repair, primary alveolar bone grafting not recommended.

However it depend on the use of vomerian flap to close the cleft

D. Dental roles:

Continued advise on feeding

E. ENT: tympanoplasty, aspiration and grommets

A cleft involving the posterior part of the palate and the soft palate will

also involve the tensor palatini muscles, which act on the Eustachian

tube.

This predispose to problems in the middle ear ventilation (glue ear).

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Therefore, it is important that the cleft patient’s ears should be examined

at the time of lip surgery to ensure adequate middle ear drainage.

About 98% of the cleft patients will have otitis media (Grant et al., 1988)

and will need tympanoplasty, aspiration (ventilation tubes inserted

through the tympanic membrane under general anaesthetic) and

grommets

6-12 Months of age

A. Palatal Repair

Soft palate repair

1. Z-plasty

2. Intra-velar veolplasty: radical dissection and reorientation

Hard Palate repair

1. V-Y closure technique

2. Von Langenbeck technique

3. Delair technique

4. Primary tongue flab technique

Some claim that it is better to delay the closure to 5-6 years to avoid scar

occurrence and subsequent growth retardation. But in this case the defect

should be closed with obturators; otherwise the speech would be

dramatically influenced.

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B. Sometime Lip and soft palate repair undertaken at 6 months at one

time

C. Dental roles:

Continued advise on feeding,

Diet analysis

OHI

Use of fluoride

Restorative care

D. Pharygoplasty:

In proportion of cases the repaired palate does not completely seal off the

nasopharynx during speech and nasal escape of air may occur, resulting

hypernasality.

Nasopharyngoplasty is undertaken at the same time as the primary palatal

repair is performed.

However, it is preferable to carry this procedure at the age of 4-5 years.

Spreistersbach et al.25 quoted 50% of children with repaired cleft palate

develop normal speech spontaneously; 25% required speech and language

therapy and 25% required further palatal surgery.

Aetiological factors pf speech problems:-

1. Velopharyngeal insufficiency,

2. Hearing problems

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3. Dental and occlusal anomalies.

4. Developmental learning disability.

5. Psychosocial impact.

1-5 Years of age

1. Lee records at 5 years stage

2. Assessment using the 5-year-old index introduced by Attack 1997

3. Interceptive Orthodontic treatment

Elimination of anterior crossbites

Identify potential problems such as supernumaries.

If second premolars are missing plan early loss of maxillary E’s to allow

spontaneous closure by the molars.

In deep bite case consider a bite plane to allow posterior tooth eruption.

Plan loss of deciduous teeth around the cleft early to improve quality of

mucosa prior to grafting

4. Dentist roles

Diet analysis

OHI

Use of fluoride

Restorative care

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5. Speech and hearing assessment. Consideration for pharygoplasty and

grommets.

6. Primary bone grafting is carried out within the first 2 years of life and is

less popular than secondary bone grafting. Primary bone grafting is

considered unfavorable and usually results in crossbite, malocclusion and

mal-union of the maxilla.

7-10 years of age

1. Lee’s records

2. GOSLON Yardstick (Mars 1987).

3. Secondary alveolar bone grafting:

Types of ABG

1. Primary bone grafting (at the time of lip repair at age of 3 months)

2. Early secondary bone grafting (between the ages of 2 and 5 years)

3. Secondary alveolar bone grafting (ABG) for patients with orofacial clefts

is usually carried out between the ages of 9 and 11 years;

4. Tertiary bone grafting in late adolescence (Rosenstein et al., 1982; eppley

and sadove, 2000)

Alveolar bone grafting Introduced by Axhausen (1952).

Technique popularised by Boyne and Sands (1972, 1976).

Pre graft records: Occlusal radiograph, study models and photos

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Orthodontic preparation for graft at approximately 8-11 years

before the eruption of the maxillary canine (Bergland 1986), ideally when

the canine root is ¼ to ½ formed. One exception is, if the lateral incisor

tooth is present, then earlier grafting may be considered.

At this age, anteroposterior and transverse maxillary growth is

practically complete apart from the alveolar development of the erupting

permanent teeth. Hence grafting at this time does not affect mid face

growth but provides the all-important bone support for the erupting

canine.

The viability of the result depend in the presence of unerupted teeth

otherwise the bone will resorbe again.

Orthodontist might extract deciduous and supernumerary teeth to

provide sufficient attached gingiva.

Treat caries and pathology

The orthodontist is often required to expand the maxillary arch

prior to alveolar bone grafting, usually with a fixed expander such as a

tri- or quad-helix. This expansion help in:

1. Maximizes the size of the bony defect and creates access for the

surgeon to place the graft during surgery.

2. improves the maxillary arch form

Then the expander should be replaced with a stabilising

transpalatal arch with finger horizontal palatal extensions prior to surgery

to facilitate surgical access. Bilateral cleft cases require a stabilising arch

wire to secure the pre-maxilla, at least 19*25 SS.

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Transpalatal arches should remain for up to 3 months after surgery

for stabilisation.

Care should be taken when aligning the incisors, as often the bone

covering the roots of the teeth is very thin. Often the aim then is to accept

the mesio-distal tip and rotations present in the upper incisors. Therefore

when placing the brackets it is wise to accept the inclination of these teeth

rather than try to upright them and moves the roots of the teeth out of the

bone and into the cleft space. After the bone graft the brackets can be

replaced and the roots moved into the correct position.

The main aims secondary ABG

1. Improve bony support for the alar base.

2. Improve nasal symmetry.

3. Eliminate any mucosal recesses liable to cause food

retention.

4. Elimination of oro-antral communication.

5. Aims to stabilize maxillary segments

6. Allow spontaneous eruption of teeth into the cleft area

7. Enable orthodontic tooth movement through the cleft site,

8. Facilitate any prosthetic restoration via implant

Surgical technique of ABG

1. Incision

2. After closure of the nasal surface

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3. Cancelous bone is harvested from donor sites, The best source of bone for

grafting for the alveolar cleft defect is the iliac crest, The rib, the cranium,

tibia and the mandible or artificial bone graft have also been used.

4. Cortical bone is not preferred because of the reduce vascularity and high

risk of necrosis.

5. Additional bone is placed under the ala and the nose on the cleft side to

provide nasal symmetry.

6. The covering flaps are then closed.

7. A protective palatal splint or orthodontic arch wire is sometimes used for

further stabilization,

8. Modified flaps may be needed to close residual palatal fistulae defects.

9. Success rate when graft placed prior to eruption of canine 90%. But drop

to 72% after or during eruption of canine.

10. Success of UCLP=BCLP if adequate stabilisation of premaxilla.

11.Then it is usually possible to proceed with orthodontic movement of teeth

in the grafted

Postoperative instruction

1. Preoperative intravenous antibiotics should be administered and then

postoperative prophylactic antibiotics given orally for 5 days.

2. Maintain scrupulous oral hygiene.

3. The patient is given a semi- solid diet by mouth and chlorhexidine

gluconate mouthwashes.

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4. Adequate analgesia for both oral and donor sites.

Postoperative assessment

1. General assessment After 6 weeks to check infection

2. After 4-6 months Kindealn score

3. Rarely, one year after ABG, Bergland index, Chelsea Index

(Witherow et al., 2002) comprises the Chelsea grade (position of bone)

and Chelsea scale (quality of bony bridge); whereas, the index proposed

by Long et al. (1995) assesses the percentage of bone covering the roots

of the teeth adjacent to the graft site.

The complications

1. Morbidity of donor area

2. Granuloma formation.

3. Failure

A unilateral alveolar defect.

Anterior oronasal fistula.

Alar-base asymmetry

4. Around 15% of the canines will require exposure.

5. External root resorption

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Influencing success

1. Dental development – best results when carried out before canine

eruption (Bergland et al, 1986; Lee et al, 1995; Kalaaji et al, 1996;

Enemark et al, 1997)

2. Donor site: Iliac crest best (LaRossa et al, 1995) although not statistically

significant in CSAG study (Williams and Sandy, 2003). Chin is the good

as well but morbidity is high and the bone might be insufficient.

3. Pre-operative health of graft site

4. Post-operative care

5. Socioeconomic status and ethnic group

6. Surgical procedure + more experience = better results

7. Extraction of teeth at surgery (not statistically significant in CSAG

(Williams and Sandy, 2003)

8. Surgeon specialty (OMFS better than Plastic surgeon) not statistically

significant (Williams and Sandy, 2003)

9. Bone volume – weigh alveolar bone (Kamakura et al, 2003)

10.Complete closure of fistulae

Segmental Surgery at the same time of the secondary alveolar bone

grafting, Harris 2008

Segmental surgery is now rarely required at the time of alveolar bone

grafting, as orthodontic preparation or distraction osteogenesis will

usually align the segments. It should be avoided because:

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a) Fixation is problematic

b) Bone grafts do not unite with mobile segments.

However, the local dentoalveolar relationship may be improved by

combining the alveolar bone graft with an osteotomy to the lesser

segment or premaxilla. The lesser segment osteotomy is carried out at the

LeFort I level.

The most common indications are:

1. Vertical deficiency of the lesser segment.

2. The fistula is too large to close for bone grafting

3. Orthodontic expansion of the arch has not been possible as the lesser

segment may be trapped palatally.

4. Distraction osteogenesis is not available.

Lesser Segment Alveolar Distraction

Segmental alveolar distraction may overcome the technical

difficulties of dividing and fixing small osteotomy segments.

By slowly moving the lesser segment at one millimetre per day

towards the cleft, the size of the alveolar and dental gap is reduced.

This decreases the size of both the graft and the flaps raised to

close the fistula. It may even eliminate the need for an autogenous bone

graft.

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Segmental distraction is only possible in young patients with erupted

teeth on which brackets and tubes can be applied to fit a rigid wire to

guide the distraction forward and around the arch form.

11-15 Years of age

Pharyngoplasty

Pharygoplasty may be undertaken at 11-15 years to improve velo-

pharyngeal competence, if not already undertaken at an earlier age.

VPI may become a greater problem in the adolescent as lymphoid

tissue shrinks effectively increasing the distance the scarred soft palate

needs to breach to create a seal.

Orthodontics

Conventional orthodontic treatment if the malocclusion is simple with or

without EOA

18+ Years of age

1. Lee’s Records

2. Orthognathic surgery

Secondary surgical correction for CLP patient

Specific Problems in Cleft Patients

1. Sever skeletal problem in all direction with malar hypoplasia.

2. Anterior open bites are common

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3. Posterior cross bites are common

4. Dental development may also be delayed in both arches but is most

evident in the cleft segment and may compromise the presurgical

orthodontics.

5. The repaired alveolar cleft is a potential site for fracture at the time of the

down-fracture.

6. If the maxillary alveolus has not been reconstructed, alignment of the

alveolus can be incorporated into the orthognathic procedure. However it

complicates the planning of the surgery and increases the potential

morbidity. Segmental osteotomies are less stable than one-piece

maxillary osteotomies.

7. Previous surgery produces scarring of the labial and buccal vestibule, the

palate and behind the maxillary tuberosities. This presents problems with

the surgical incisions, mobilisation and postoperative closure of the

surgical wound.

8. A pharyngeal flap may make advancement of the maxilla difficult and

will need to be divided. The patient has to be informed well in advance

about the possibility of VPI and speech problem that might developed

after the surgery.

Treatment Planning for CLP

The basic facial and orthognathic evaluation is the same as the non-cleft

case with important refinements.

1. Lip-incisor relationship. As in the non-cleft case, the lip to maxillary

incisor relationship is extremely important. The major surgical moves are

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predominantly in the maxilla and with a tight, previously scarred upper

lip, small skeletal moves have a pronounced effect on the incisor

exposure.

2. Asymmetries. Both dental and skeletal asymmetries are dominant

features, often with compensatory asymmetries in the mandible. This

should be considered

3. Pharyngeal obstruction can be caused by hypertrophied adenoidal tissue

or pharyngeal flaps. Nasal airway obstruction may arise from a deviated

nasal septum narrowing of the nares, hypertrophied turbinates, nasal

polyps and posterior choanal constriction from sub-periosteal bone and

asymmetrical vomer flaps. The management of these problems is an

essential part of the orthognathic procedure. Paradoxically the adenoid

mass may contribute to velopharyngeal function and its removal may

precipitate velopharyngeal inadequacy.

4. Preoperative speech assessment and counselling.

5. However, infection, bone and soft tissue necrosis, delayed healing, loss of

teeth and relapse all occur with greater frequency due to multiple

previous surgeries.

The Choice of Operation for CLP

Maxillary Hypoplasia

1. LeFort I osteotomy either one piece or two pieces maxilla for transverse

maxillary widening.

2. High LeFort I level osteotomy.

3. The modified LeFort II and Kufner LeFort III osteotomy

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4. SARPE

5. Rhinoplasty may be necessary.

6. Mismanagement of the soft tissues during closure of the labial vestibular

incision may cause shortening and thinning of the upper lip. The V-Y

closure of a maxillary vestibule incision may increase the vermilion show

in patients with a thin upper lip.

7. Maxillary advancement widens the alar base, increases the projection and

elevation of the nasal tip and the width of the nares. Various surgical

manoeuvres can be used to prevent these unwelcome side effects. These

include an alar base cinch suture, recontouring the bony piriform aperture

either by trimming and/or asymmetric bone grafting and alar base

resections.

Mid Face Distraction Osteogenesis

Indications:

With gross maxillary hypoplasia and a severe degree of scarring, the

degree of advancement may be beyond the expected limits of stability of

a conventional osteotomy. Distraction of the maxilla is preferable to a

surgical compromise such as a mandibular setback.

If the deformity is complex particularly in the upper mid face then a

higher level osteotomy with distraction often gives a better result than a

modified LeFort I with masking onlay bone grafts or modified LeFort II

and LeFort III osteotomies that are difficult to perform and can give

unsightly steps particularly over the radix of the nose.

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There is usually a need for pre-surgical orthodontics.

Due to risk of worsening VPI and due to previous surgical scarring, the

Large jaw discrepancies of 10mms and above may also require a

mandibular setback

a modified maxillary Le fort 1 advancement or Converse Wake Procedure

(that move the maxilla without influencing the position of the palate) is

used with careful attention paid to the mobilisation of the maxilla.

Use Horseshoe osteotomy as alternative

Use Distraction osteogenesis as alternative

Severe maxillary restriction may require Surgically assisted RME and 2

or 3 piece Le fort 1 osteotomies

Any expansion gained should be permanently retained. (Proffit and White

1990)

Mandibular setback (BSSO, VSO)

Mandibular set back indicated in case of:

1. Mandibular prognathisism

2. When there is a maxillary surgical limitations such as severe palatal

scarring, borderline velopharyngeal insufficiency or a tight inferiorly

based pharyngoplasty flap.

3. During maxillary advancement and inferior positioning, the anterior

maxilla is differentially positioned more inferiorly. This will produce a

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posterior open bite deformity unless a mandibular ramus procedure is

undertaken simultaneously. Differential down grafting of the anterior

maxilla also results in a counter clockwise rotation of the mandible which

may make the chin retrogenic. This can be corrected by a simultaneous

augmentation genioplasty.

Airway Considerations for CLP during surgery

1. The surgeon can do the following whilst the maxilla is down fractured

Contouring of the inner aspects of the nose

Asymmetries in the piriform region

The mucosa of the nostril floor can be repaired

Septoplasty may be indicated

Partial or complete inferior turbinectomies

Antral and nasal polyps can be removed

2. Pharyngeal flaps raise additional concerns for the anaesthetist and

surgeon which may make intubation difficult and restrict the nasal

airway, so submental intubation might be indicated

Postoperative considerations for CLP

1. Speech therapy: The soft palate mechanism in non-cleft patients has

considerable reserve capacity and can adapt to an increase in length. The

repaired cleft soft palate does not have this capacity to adapt especially

after major advances. The patient with borderline velopharyngeal

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incompetence preoperatively is likely to develop worsening of their

speech postoperatively.

2. Relapse: As a prophylactic measure, extraoral elastic traction using a face

mask can be used in patients who are considered particularly at risk of

relapse either due to scarring or who have had large surgical moves

anteriorly and inferiorly.

3. Stability: The factors that increase stability include:

High quality orthodontic preparation.

Avoiding segmental procedures

Overcorrection where possible.

Compromise position must be planned and if necessary with

incorporatation of a mandibular setback.

Alveolar bone grafting.

Bone grafting for inferior repositioning of the maxilla.

Internal rigid fixation for all moves.

3. Secondary plastic procedures

Such as nose and lip revision. These are best undertaken after growth,

since growth can detrimentally affect earlier revisions.

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Terminology

Velopharyngeal impairment is a generic term indicating that the patient

is unable to induce sufficient contact between the velum and the posterior

and lateral pharyngeal walls

Velopharyngeal insufficiency is a form of velopharyngeal impairment

caused by a soft palate whose functional length is insufficient.

Velopharyngeal incompetenceis a form of velopharyngeal impairment

caused by neuromuscular impairment .

Hypernasality is a resonance phenomenon that occurs when sound is

inappropriately generated in the nasal cavity.

Maxillary and Nasal Impression Using an appropriately sized infant impression tray loaded with a heavy

body Polysiloxane impression material

The clinician who performs the impression must remain constantly

attendant to the infant’s airway. A small dental instrument (e.g. intraoral

mirror handle) is used to prevent the tongue from occluding the airway

during the impression procedure.

Good intraoral illumination facilitating direct visualization of the tongue

position and the posterior aspect of the pharynx is essential to prevent

airway obstruction by excess impression material or the tongue.

A clinical assistant should be present to mix the impression material, load

the impression tray, and then help where needed.

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The surgeon or a physician, qualified to take appropriate action in case of

an airway emergency, holds the swaddled infant upside down during the

maxillary impression

After the impression material is set, the impression tray is removed and

the oral cavity is examined to ensure that there is no debris from the

impression remaining in the mouth.

An impression of the nose including the left and right medial canthi is

performed with a light bodied material (Memosil 2, vinyl Polysiloxane,

Heraeus Kulzer, Germany)

Recently, a computer-aided system for NAM appliance fabrication and

adjustment has been described, and appears promising

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