2 - Congenital Defects - Abo

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CONGENITAL

DEFECTS
Dr. Abo Al-Mawaheb Ebrahim
Congenital defects Acquired defects

 Detected at birth  Occur after birth


due to trauma,
infection, or tumor
resection
 Confined to the line of
fusion between palatine  Occur anywhere in
and pre- maxillary
the palate
processes (Y- shaped
fusion of the
processes)
 It may occur as
 Once it started, it
continues along the confined area in the
suture midline, with
complete fusion of
 Normally the oral and nasal cavity are
separated by a complete partition (the
palate) which prevent communication
except at the most posterior end through a
well coordinated velo-pharyngeal sphincter.
 The palate is formed
due to fusion of
palatine process and
the median nasal
process
 The fusion starts at
the future position of
the incisive papilla
and continues
anteriorly and
posteriorly till
complete fusion
occurs (Y- shaped
Palatal Development

 It begins at 5 th week intra-uterine (W.I.U)


 completed by the end of 12 th W.I.U.
 Develop from two primordial structures:
1. primary palate (median nasal process )
Develops at the end of the 5 th W.I.U.
2. Secondary palate
Begins at 7 th W.I.U
primary palate
 Develops at the end
of the 5th W.I.U.
• Develops from the 2
median nasal
processes
 It gives rise to
1. PREMAXELLA [part of the hard palate
anterior to the incisive foramen]
2. The upper lip
3. The anterior part of the maxillary
alveolar process and the incisor teeth
A- Median nasal process
B- lateral nasal process
C- maxillary process
Secondary palate
 Develops from the
2 lateral palatine
processes
posterior to the
incisive foramen
 Fusion begins at
9th W.I.U and
completed by the
12th W.I.U.
 Gives rise to the hard and soft palate
The palate
 The incisive
foramen
remains the
embryologic
border between
1ry and 2ry
palate
• From this meeting point (the incisive
foramen) union of median and lateral
nasal processes progresses in 2
routes in the form of Y-shaped
The palate
 Anteriorly
To form the Premaxilla,
upper alveolar ridge and
lip
 Posteriorly:
To form hard and soft
palate
 Lack of fusion
between the
embryologic
processes leads to
what is called clefts
Congenital Cleft Lip And Palate

 Definition
 Lack of fusion of the
embryological processes
which normally unite during
the inter-uterine growth to
form the lip and palate
 The child can have cleft lip,
cleft palate or both
Congenital Cleft palate
 Definition
 Lack of continuity of the
roof of the mouth through
the whole or part of its
length in the form of a
fissure extending
Anteroposteriorly
• The degree of cleft can
vary greatly from bifid
uvula to severe cleft
involving both the soft and
hard palates
Congenital Cleft lip
 Definition
 It is an abnormality in
which the lip does not
completely form during the
fetal development
 The degree of cleft vary
from notching of the lip to
severe opening from the lip
up to the nose
 It may be unilateral or
bilateral
Incidence of the Cleft
 The general accepted
incidence rate is 1-700
of infants were born
 Unilateral cleft is more
common than bilateral
clefts of the lip
 Left-sided clefts
compromise 70% of
unilateral clefts
 Incidence of clefts is
twice in males than in
females
Etiology of Clefts
 The specific cause of many birth
defects is unknown
1. The abnormal position of the embryo
2. Pressure of the amniotic fluid
3. Failure of the tongue to drop
4. Persistence of epithelium at the
junction of the two palatal shelves
5. multiple genes inherited from both
parents and environmental factors
Factors affecting induction of
cleft palate
I. Hereditary or genetic factors
II. Environmental factors such as :
1. Endocrine factors
 Hormonal disturbance or cortisone
therapy influence cleft formation
2. Chemical irritation
 Mothers exposed to chemical irritation As
hypoxia and hypervitaminosis ( vit. A )
3. Radiation and x-ray
4. Nutritional insufficiency
 Dietary deficiency as (vitamin A &
riboflavin)
5. Infection and disease
 Infectious disease of the mother and
German measles
6. Stress and Anxiety
 Disturb fetal circulation with first
trimester of pregnancy
Classification of clefts
 Clefts can be classified into
three broad classification
1. Cleft lip without a cleft palate
2. Cleft palate without a cleft lip
3. Cleft lip and cleft palate
together
 Cleft lip with or without cleft
palate is generally more
common in boys
 cleft palate occurring alone
more common in girls
Veau's classification
 Veau's classification system
depends on the extent of the
cleft
A. Group I : cleft of the soft palate
only
B. Group II : cleft involving the
hard palate and soft palate
C. Group III : cleft involving the
soft palate to the alveolus
involving the lip
D. Group IV : complete bilateral
clefts
Disabilities associated with
cleft lip and palate
1. Improper feeding and mastication
 Babies cannot perform sucking
 Masticatory function is impaired due to
missing teeth and malocclusion causing
malnutrition and debilitation
2. swallowing
 Fluids and food will regurgitate to the nasal
cavity
3. Esthetics
 It causes a distorted facial appearance
4. General health
 Deteriorated due to inefficient feeding and
mouth breathing
4. psychological trauma
5. speech
 Children with clefts suffer from incompetent lips
and\or inadequate velo-pharyngeal closure
 The air stream is necessary for production of
sound will escape through the nose rather than
through the oral cavity
 Vowels and Nasal consonants are the only sounds
that are not affected by cleft palates
 speech problem is more complicated in congenital
defects than in acquired defects as speech is a
learned process
Sequence of treatment
I- Presurgical phase
 Assess the severity of the case
and counselling
 Feeding devices
II- Surgical treatment
 Lip repair
 Palatal repair
III- Orthodontic treatment
IV-Definitive prosthetic treatment
I-Pre-surgical phase
 It Starts at birth and continue up to 3
months
 it includes
1. Assess the severity of the case and
counselling
2. Reassure the parents
3. Feeding
 nutrition is necessary for growth
 It helps infant's preparation for the
1st surgery
 According to the TYPE and SEVERITY of the
cleft, a variety of feeding devices are
available
I- Feeding device
 Infants with cleft lip only can
feed normally
 Infants with cleft palate the
feeding problem is more
significant and should use
feeding device
 it includes
1. A soft nipple
2. Cross-cut nipples
3. Longer nipples
4. The squeezable bottle
5. Feeding Appliance
I- Feeding Appliance
 It is a custom made
plate covering the
palatal defect
 It is in the form of
acrylic plate attached
to the neck of the
feeding bottle
 Or it may be designed  It helps in
with a wire handle to
feeding of cleft
allow the mother to
push the plate against
palate infants
the cleft in order to during the pre-
obliterate it during palatal surgery
breast feeding period
Function Of Feeding Plate
1. It obturates the cleft and restores the
separation
2. It creates a rigid platform towards which the
baby can press the nipple and extract the
milk
3. It facilitates feeding and reduces nasal
regurgitation
4. It reduces the incidence of choking
5. It helps to position the tongue in correct
position
6. It prevents the tongue from entering the
defect
7. It helps in speech development
8. It reduces the passage of food into the naso-
pharynx hence reducing the incidence of otitis
media and naso- pharynhgeal infections
Fabrication Of Feeding Plate
1. Primary impression
2. A special tray
3. The final impression
4. Master cast
5. The wax pattern
6. Flashing, de-waxing
and feeding plate was
fabricated with heat
cured clear acrylic
7. 10 inch silk suture
was passed through
and tied to the
feeding plate
Hold the feeding(plate) appliance

1. By parent's finger
during feeding
2. Attached to the
feeding bottle
3. Hold by stuck
sheets (Plastic
sheet / Steristrips)
A. Lip repair
 It is the treatment of
choice for congenital
defects
 Timing of surgery
 depends on THE
RULE OF TEN

 10 pounds in weight.
 10 weeks old
 Hemoglobin count of 10.
A. Lip repair
 The 1st surgery for the lip is
performed at AROUND 3 MONTHS in
order to:
1. Facilitate feeding
2. Improve appearance.
Lip repair without excessive
tension will help to:
1. Establish favorable contour in
the premaxillary area
2. Narrow the palatal cleft (if
present)
II- Palatal Repair

1. To enhance development of speech


2. Proper swallowing and breathing
3. Decrease dento-alveolar deformities
4. Maintain proper maxillary growth
 Timing of surgery
 It varies from 12 months to 4 years,
depending on the width of the cleft
 surgery on the palatal shelves should
not be performed too early to allow
Palatal shelves continue to grow to
III- Orthodontic Treatment
 Principles Of Orthodontic Treatment
1. Expansion of maxilla
 Done by using a palatal
expansion device done
at the age of 7 years
 To correct segment
position and cross-bite

2. Monitoring eruption of
teeth
3. Decision to leave the
missing lateral space
opened or not
 Indications Expansion of maxilla
1. Preoperatively for
cases having
complete unilateral
or bilateral
collapsed clefts
2. To align lateral
segments of the
palate
3. Diagnostic aid to
assess the need for
speech progress
Expansion device of maxilla
 It consists of two parts
1. Palatal part
 composed of 2 separate
lateral sections united
by expansion devices
covering the hard palate
2. Pharyngeal part
(speech aid)
 To achieve improvement
in speech and
deglutition
Bilateral Cleft Palate Appliance:
activation of the appliance causes the
anterior portion of the appliance to be
drawn posteriorly reducing the
prognathic segment
IV- Definitive Prosthetic Treatment
 The initial Prosthodontic care is to
fabricate a well-fitting interim R.P.D.
I. Rehabilitation of patient undergo
surgical treatment
 Indications :
1. Failure of surgery to close the defect
2. Movement of soft palate is inactive
3. When a transitional prosthesis is
required
4. Incompetent palatopharyngeal
closure
II. Rehabilitation of Un-operated Patient
 Indications :
1. Wide soft palate defect
2. Wide hard palate
defect
3. Debilitated patient
4. Cases requiring
expansion prosthesis
5. Partial or complete
paralysis of soft
palatal
6. When surgery is to be
delayed to allow for
proper growth
III.Prosthetic Rehabilitation For
Adolescents And Adults
1. Fixed partial dentures (FPD)
2. Removable partial dentures
(RPD)
3. Complete denture (CD)
4. Maxillary overdenture (Max.
OD)
5. Osseointegrated implants
(Imp.)
6. Speech aid appliance
1. Fixed partial dentures (FPD)
 Most prosthodontists prefer to restore
all anterior fixed units (at 25 Y)

2. Removable partial dentures (RPD)


 The same designs of normal
patients EXCEPT for patients
with Velopharyngeal
deficiencies, where the R.P.D
must support
 R.P.D must support
1. Palatal lift prosthesis
2. Obturator prosthesis
 Prosthodontist must
consider the long lever
arm created by the
extension specially in
Kennedy Class I Or II
Cases which need
adequate indirect
retention
3. Complete denture (CD)
The problems encountered due to:

1. The reduced size of


the cleft maxilla
2. Excessive inter-arch
space
3. Lack of bony palate
4. Poor alveolar ridge
development and
shallow depth of
the palate
5. Scarring from lip
closure
4. Maxillary overdenture (Max. OD)

 May be supported by

1. The remaining
teeth
2. Combination of
remaining teeth
and implants
3. Implants alone.
5. Osseointegrated implants
(Imp.)
 patient with an alveolar
cleft is usually missing
the permanent lateral
incisor on the side of the
cleft.
 An implant- supported
restoration to replace
the missing lateral
incisor
 Advantages of implant- supported
restoration
1. Abutment tooth
preparation is not
required with the
decreased possibility of
damage to the dental pulp
2. Increased loading of the
abutment teeth is avoided
3. The implant in the
alveolar cleft may
transfer functional
forces to the graft
which could decrease
resorption of the graft

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