CHEILO – GNATO PALATOSCHISIS
• Clefts are the congenital defects that
may cause severe functional and aesthetic
problems.
• The prevalence has increasing tendecy.
While at the beginning of this century it
was about 1 in 1600, in 1940 it was 1:770
and in 1990 1: 500.
• The prevalence varies between racial
groups. The clefts are very rare among the
Negroes and the most frequent are in
chinese, japanese and american indian
population.
•
•
•
1.
2.
3.
4.
5.
6.
There is often a history of clefts within a family
the mode of inheritance is not simple
Many enviromental insults to the embryo
infectious diseases
corticosteroids
mechanical and chemical insults
alkohol
drugs
X-rays
can produce clefts in animals but whether
these are of any significance in human
population is uncertain.
• Many classifications of clefts have been
proposed but none is entirely satisfactory.
Nowadays the classification based on
embryological principles is used.
DEVELOPMENT OF THE FACE
Development of the face starts
from ventral part of the head
process of an embryo and
the first branchial arch.
The face develops mainly
between the 4th and 8th
weeks.
By the end of the embryonic
period ( eight weeks ) the
face has an unquestionably
human appearance.
DEVELOPMENT OF THE FACE
The first branchial arch develops two elevations called the
“ maxillary prominence” and “mandibular
prominence”.
In the 4th week the five facial primordia are around the
stomodeum ( primitive mouth ):
• The large “frontonasal prominence” constitutes the
cranial boundary of the stomodeum.
• The paired “maxillary prominences” of the firsth
branchial arch form the lateral boundaries and
• the paired “mandibular prominences” of the same arch
constitute the caudal boundary of the stomodeum.
Early formation of the face about 24 days after
conception
Bilateral oval-shaped thickenings of the surface ectoderm,
called “nasal placodes”, develop on each side of the
caudal part of frontonasal elevation
Horseshoe-shaped “medial and lateral prominences” develop
at the margins of the nasal placodes. As a result, the nasal
placodes lie in depressions called “nasal pits”.
The maxillary prominences grow rapidly and soon approach each
other and the medial nasal prominences. During the 6th and 7th
weeks the medial nasal prominences merge with each other
and the maxillary prominences.
• Diagrammatic representation of the
structures at the beginning of the 5th
week when fusion is just beginning.
• Relationship at the beginning of the 6th
week, when the fusion is well – advanced.
• Schematic representation of the
contribution of the embryonic facial
processes to the structures of the adult
face
DEVELOPMENT OF THE FACE
As the medial nasal prominences
merge with each other, they form
an “intermaxillary segment” of
the maxilla. This segment give rise
to:
• the middle portion of the upper lip
called the philtrum
• the premaxillary part of the maxilla
and its associated gingiva
• the primary palate
The lateral parts of the upper lip, most
of the maxilla and the secondary
palate form from the maxillary
prominences.
These prominences merge laterally
with the mandibular prominences
DEVELOPMENT OF THE FACE
The mandibular prominences
merge with each other in the fourth
week and the groove between
them disappears before the end of
the fifth week.
The mandibular prominences give rise
to:
• the mandible
• lower lip and
• the inferior part of the face
The frontonasal prominence forms
the forehead and the dorsum and
apex of the nose.
The sides of the nose are derived
from the lateral nasal
prominences.
DEVELOPMENT OF THE PALATE
The palate develops from
the::
• the primary palate and
• the secondary palate
Although palatogenesis
begins toward the end
of the 5 week, fusion of
the palate´s parts is not
complete until the 12
week.
DEVELOPMENT OF THE PALATE
The primary palate – or
median palatine
process, develops at the
end of the fifth week
from the innermost part
of the intermaxillary
segment of the maxilla.
It forms a wedge-shaped
mass of mesoderm
between the maxillary
prominences of the
developing maxilla.
DEVELOPMENT OF THE PALATE
The secondary palate –
develops from two internal
projections from the
maxillary prominences,
called the lateral palatine
processes.
These shelflike structures
inicially project inferomedially
on each side of the tongue.
As the jaws develop, the
tongue moves inferiorly and
the lateral palatine processes
gradually grow toward each
other and fuse. They also
fuse with the primary palate
and nasal septum.
The fusion of the palatal processes begins anteriorly during the
9th week and ends posteriorly in the region of the uvula by
the 12th week. The palatine raphe indicates the line of fusion
of the lateral palatine processes. The posterior portion of the
lateral palatine processes do not become ossified. They extend
beyond the nasal septum and form the soft palate and uvula.
CLASSIFICATION OF THE CLEFTS :
The embryological classification divides clefts in four groups:
1. Clefts of the primary palate: may involve only the lip or the lip
and alveolar process as far back as the incisive foramen.
2. Clefts involving both the primary and secondary palate.
3. Clefts of the secondary palate: may involve the soft palate
only or the soft palate and hard palate as forwards as the
incisive foramen.
4. Special clefts of the face: this group is not based on
embryology but on the topography. It involve: medial cleft of the
upper lip, medial cleft of the nose, aplasia of the ala nasi,
unilateral or bilateral oblique cleft of the face, transverse cleft of
the face, cleft of the lower lip, cleft of the mandible.
These clefts are very rare.
MECHANISMS OF CLEFT
FORMATION :
• A cleft is formed because the epitelium or the
mesoderm failed to unite the various processes.
• A cleft also may be caused by inadequate
mesoderm, or mesoderm that may have had
insufficient migrating potential.
• It is thought that clefts of the primary palate result
from failure of mesenchymal consolidation
whereas clefts of the secondary palate may arise
if the palatal processes fail to come into contact.
CLEFT OF THE PRIMARY PALATE
• The deformity varies from
notching of the lip to a
complete lip cleft with alveolar
involvement.
• It may be unilateral or
bilateral.
• The most striking clinical
feature of the unilateral cleft is
the distortion that occurs due
to the major segment of the
alveolus moving away from
the cleft side. Thus, the major
segment is on the noncleft
side, and the minor segment
on the cleft side. The lip is
distorted, and the nose is
always flattened on the cleft
side.
CLEFT OF THE PRIMARY PALATE
• The alveolar cleft is in the
lateral incisor area so the
anomalies of this tooth are
often seen : it may be absent
or poorly developed and/or
malpositioned, or there may
be dichotomy of the lateral
incisor with one small pegshaped tooth on either side
of the cleft line. The central
incisor on the side of the cleft
is often rotated and
hypoplastic. Normally the
arch form is good.
CLEFT OF THE PRIMARY PALATE
• In the cases of bilateral
cleft there is abnormal
position of the primary
palate, or premaxilla,
which is rotated and
everted out of the oral
cavity. Thus the two
major segments form the
lateral aspect of the
mouth, and the minor
segment protrudes
extraorally in the midline.
CLEFT OF THE PRIMARY AND
SECONDARY PALATE
• These may be unilateral ( if
primary palate involved on one
side only) or bilateral ( if
primary palate involved on both
sides ).
• In cases of unilateral cleft there
if a major segmet that consist
of the lip, premaxilla, frontal
and one lateral part of alveolar
process, one half of the hard
palate which is connected with
womer and nasal septum. The
minor segment comprise of the
rest of lip, alveolar process and
hard palate. There may be
wide gap between the
segments or they may
collapse.
CLEFT OF THE PRIMARY AND
SECONDARY PALATE
• In the cases of bilateral cleft
the premaxilla with womer
and nasal septum form an
independent segment which
protrudes extraorally in the
midline or wedges inside
the oral cavity.
These cases present the
greatest problems: surgical,
dental, orthodontic and
speech.
CLEFT OF THE SECONDARY
PALATE
• These vary from a
submucous cleft of the
soft palate to a
complete cleft of the
soft and hard palate as
far forward as the
incisive canal.
• The submucosus cleft
means that there is
diastasis of the soft
palate muscles and
space between them is
filed by membrane.
TREATMENT OF CLEFTS
:
• In order to improve the function and appearance of the various
parts of the face and oral cavity many different specialists
have to work in close collaboration.
• A great deal of consideration must be given to the parents who
may be under severe psychlogical stress. The mother in
particular is often under considerable stress.
• Parents must be povided with adequate information about the
future treatment and management of their child because almost
all of them have the same concerns and questions. Before
surgery it is helpful to warn parents about how the child will look
immediately after surgery.
• In addition it is helpful for the parents and the child to attend a
clinic where there are other children with similar defects and
problems.
PRESURGICAL MANAGEMENT
• careful management of the neonate is important for presurgical
preparation.
• Because feeding is a major problem, it is essential to establish
a satisfactory feeding technique as soon after birth as possible.
It must be remembered that these babies take longer to feed
than normal childs. The mother should be encouraged to
breast feed the child whenever possible.
• sometimes the baby may not be able to generate adequate
physiological reflexes to stimulate mother milk to flow. Bottle
feeding may be difficult too because of inability to generate
adequate suction. To overcome this problem, a long nipple or
nipple with a flange is used. It has the effect of filling the cleft
and directing the milk into the pharynx. A diagonal cross cut in
the nipple prevents milk flowing unless the nipple is squeezed.
Throughout bottle feeding the child should be in a semiupright
position to prevent choking.
PRESURGICAL MANAGEMENT
In order to cover the roof of the mouth and fill the space a
feeding plate can be made. It fills the defect so that food and
liquids do not enter the nasal cavity. At first an impression of
the palate is taken using a specially constructed impression
tray. After making a study cast the area
of the defect is filled
and hard acrylic plate
is fabricated. The plate
should be checked
immediately after fitting
and regurarly afterward
at approximately 4-week
intervals.
PRESURGICAL MANAGEMENT
In some cases a presurgical orthopaedic treatment is necessary
to make surgery easier. In the infant with a unilateral cleft of lip
and palate the greater and lesser segments may be malrelated:
they may be widely separated or over-lapping or somewhere in
between. Some surgeons consider that the surgical repair is
easier to do if the segments are aligned before surgery.
Presurgical orthopaedic
treatment is performed by
fitting either a hard or a soft
acrylic plate. The plate is
designed to mold the gum
pads into right position and
is usually supported by a
headcap – alveolar molding
PRESURGICAL MANAGEMENT
• In bilateral cases the
premaxilla is often far
forwards of the lateral
segments. In theese patients
the headcap may be used to
apply distal traction to the
protruding premaxilla. Soft
acrylic plates are trimmed on
the palatal surfaces to allow
the segments to aline as
growth takes place.
SURGICAL MANAGEMENT
•
Surgery usually begins at approximately 3 month of age and
involves repair to the lip. The alveolar defects may also be
repared at this time.
• Clefts of the hard and soft palate are usually repared at about
18 months of age.
• Surgical treatment is performed:
1. to improve appearance
2. to facilitate eating and drinking without nasal spillage
3. to reduce the likelihood of recurrent middle ear infections,
which may lead to deafness and reduction in speech quality
Some surgeons prefer to operate at an earlier age (6-12 months)
before the child develops adaptive speech habits. Others
repair the soft palate at 18 months but delay the hard palate
repair until further maxillary growth has taken place.
SURGICAL MANAGEMENT
The correction of the cleft defect is not simply.
There is a total absence of tissue in the cleft
region.
Closure of the defect must be made on both
the oral and nasal sides with the minimum of
tension.
Even with the best surgical techniques
available, the scar tissue that result from the
repair may adversely affect the anteroposterior
and transverse development of the palate and
whole maxilla
SURGICAL MANAGEMENT
• the upper arch is often narrow with the result
that some crowding and a crossbite ( uni- or
bilateral ) may be present.
• The anteroposterior deficiency of the maxilla
gives rise to a Class III skeletal pattern and
often to a Class III incisor relationship.
• The skeletal malrelationships become more
severe as the child grows.
• For adolescents with severe discrepancies in
jaw sizes in the anteroposterior plane, major
facial surgery can significantly improve the
aesthetics and function
SURGICAL MANAGEMENT
Repairs of the lip and palate are the two major surgical
procedures. However, as the child grows other
problems must be solved:
• Often small or even large oronasal fistula develops.
• The nose is frequently distorted and needs surgical
revision.
• Scar tissue in the lip gives it an uneven appearance.
So the child often has to undergo secondary
operations to the nose, lip or palate. This must be
taken into account in planning the dental
management.
ORTHODONTIC TREATMENT
• The objectives of treatment are the same as
those of surgery : that appearance, speech
and function should be as good as the
circumstances allow.
• No orthodontic treatment is advised until the
permanent incisor erupt.
• The extent of a malocclusion is usually less
apparent in the primary dentition and the
level of cooperation is fairly limited.
• The child should be seen at regular intervals for
routine dental checks and treatment.
ORTHODONTIC TREATMENT
Discrepanties in arch alignment and relationships are
beginning to worsen by the mixed dentition phase.
Anterior teeth are frequently malformed, hypoplastic,
erupt in ectopic positions or they may even be
missing.
It is difficult to be sure if these problems have been
caused by the cleft defect only or also by the surgical
repair.
At this stage should be carried out :
• Correction of lingually placed permanent incisors
• Supernumerary or malposed teeth may have to be
removed surgically.
Lateral expansion of the narrow upper arch should not
be undertaken at this stage as it will not be stable.
ORTHODONTIC TREATMENT
At early permanent dentition stage a full
diagnosis and treatment plan must be
formulated.
Treatment varies according to the severity of
the case.
1. Where there are reasonable arches with
minimal anteroposterior and transversal
discrepanties , orhtodontic treatment, ideally
followed by bridgework to replace missing or
malformed teeth, is indicated.
ORTHODONTIC TREATMENT
2. Where the discrepancy in arch relationship is
moderate or the patient is not suitable for complex
appliance treatment, it is often best to remove poorly
formed and malpositioned teeth and to fit a metal
denture to give a reasonable appearance without
damaging the soft tissues.
3. In severe cases where orthodontic and prosthetic
techniques alone will not give a satisfactory
aesthetic result, surgery to improve the patient´s
profile may be indicated. This will usually involve the
maxilla and sometimes a combined
maxillary/mandibular operation. Surgery may be
preceded or followed by the orthodontic treatment
and finaly restorative procedures
Speech therapy :
Cleft palate patients have also speech
problems, in part because the soft palate
tends to be rather short and lacking in
mobility, so that during the speech there is a
nasal escape of air. Many also have hearing
defects due to middle ear infections.
Speech therapy is an important part of the total
treatment.