3. In 4-week old 5 mesenchymal
prominence can be recognized :
1. Unpaired Frontonasal Prominence
2. Medial and Lateral Nasal
prominences
3. 2 maxillary prominences
4. 2 mandibular prominences
EMBRYOLOGY
4. EMBRYOLOGY
During the fifth week, two fast growing ridges, the
lateral and medial nasal swellings, surround the nasal
vestige.
The lateral swellings will form the alae of the nose, the
medial swellings will give rise to four areas :
(1) the middle portion of the nose
(2) the middle portion of the upper lip
(3) the middle portion of the maxilla
(4) the entire primary palate
Simultaneously the maxillary swellings will approach the
medial and lateral nasal swellings but remain separated
from them by well marked grooves.
5. During the next 2 weeks, the appearance of the face
changes considerably.
The maxillary swellings continue to grow in a medial
direction and compress the medial nasal swellings
toward the midline.
Subsequently these swellings simultaneously merge
with each other and with the maxillary swellings
laterally.
Hence the upper lip is formed by the two medial nasal
swellings and the two maxillary swellings.
The two medial swellings merge not only at the
surface but also at the deeper level.
6. The structures formed by the two merged swellings are
known together as the intermaxillary segament which is
comprised of
:
three components
(1) a labial component, which forms the philtrum of the
upper lip
(2) an upper jaw component, which carries the four incisor
teeth
(3) a palatal component, which forms the triangular primary
palate.
7. Two shelflike outgrowths from the maxillary swellings
form the secondary palate.
These palatine shelves appear in the sixth week of
development and are directed obliquely downward on
either side of the tongue.
In the seventh week, however, the palatine shelves
ascend to attain a horizontal position above the tongue
and fuse with each other, thereby forming the
secondary palate.
Anteriorly the shelves fuse with the triangular primary
palate, and the incisive foramen is formed at this
junction.
8.
9. Cleft lip : is a birth defect that results in a unilateral
or bilateral opening in the upper lip between the
mouth and nose (interruption of development before
7th week lead to lip deformity).
Cleft palate : is a birth defect characterized by
an opening in the roof of the mouth caused by a
lack of tissue development( interruption of
development .
(
before 12th week lead to palate
deformity
10. .
Development Of cleft :
1.Cleft Lip :
:
Various theories have been given for its development
1.Failure of fusion between median nasal process and
maxillary process
2.Failure of mesodermal migration between the two
layered
epithelial membrane .This leads to breakdown
and cleft formation.
3.Rupture of cyst formed at the site of fusion.
11. :
2.Cleft palate
:
Various theories have been given
1.Alteration in intrinsic palatal shelf force
2.Failure of tongue to dropdown
3.Non fusion of shelves
4.Failure of mesodermal migration
5.Rupture of cyst formed at the siteof fusion
12. Etiology
1.GENETIC FACTOR AND ASSOCIATED WITH SOME
SYNDROME LIKE PIERRE ROBIN SYNDROME.
VIT. A,B DEFICIENCY))2.NUTRITIONAL DISTURBANCES
DURING DEVELOPMENT
3.PHYSIOLOGIC ,EMOTIONAL OR TRAUMATIC STRESSES
DURING DEVELOPMENT
4. DEFECTIVE VASCULAR SUPPLY TO AREA INVOLVED
5.MECHANICAL DISTURBANCES WHERE THE SIZE OF THE
TONGUE MAY PREVENT UNION PARTS
6.VARIOUS ENVIRONMENTAL FACTOR LIKE :
INFECTION (E.G. RUBELLA)
EXPOSURE TO RADIATION
DRUGS (E.G. THALIDOMIDE , ANTI
EPILEPTIC DRUGS , HORMONAL PILLS)
6. MATERNAL CONSUMPTION OF ALCOHOL AND SMOKING
13. General Classification
1- Veau classification
2- Kernahan and Stark classification
3- Kernahan classification
4- Harkins' classification
5- Spina classification
6- Tessier's classification
14. -Tessier described a classification scheme that is
universally utilised,
in a landmark article of 1976.
Oro-facial clefts can manifest as:
Unilateral or bilateral
Complete, incomplete, or microform (e.g., sub-
mucous cleft palate)
Clefting of the lip with or without the palate, or of the
palate in isolation
Atypical cranio-facial clefts
15.
16. complete : cleft involves the entire primary and secondary palates. It
extends from the uvula all the way into the alveolar ridge. It
involves both the primary palate and secondary palate.
Types
-Unilateral
-Bilateral
17. incomplete: cleft starts at the back of the palate with the uvula and
extends forward. It may or may not reach the incisive foramen. In
simpler terms, it only involves the secondary palate as it does not
extend all the way forward to include the alveolar ridge.
Bifid Uvula: The least severe of the
incomplete clefts in appearance, a bifid
uvula is the most common palatal cleft. It is
also referred to as a “cleft uvula.” A bifid
uvula appears as a splitting or forking of
the uvula. It may be very subtle, evidenced
only by a small notch, or the uvula may
appear as two distinct entities. A bifid uvula
in and of itself is not problematic. This
occurs in about 2 percent of the population.
However, usually a bifid uvula is indicative
of a submucosal cleft.
18. Submucosal Cleft: A submucosal cleft is a cleft that is
under the mucosa that lines the roof of the mouth --
hence the term “sub.” Because a submucosal cleft is
under the mucosa, the only physical indicator of its
presence may be a bifid uvula. Even though not seen
from the surface, the muscles of the palate are not
joined at the midline in a submucosal cleft. This creates
an inability to move the palate for some speech sounds.
Hence, a submucosal cleft is usually diagnosed when a
child has abnormal speech development and a bifid
uvula is present.
Soft Palate Cleft: A cleft of the soft palate runs from
the tip of the uvula and stops before or at the junction
of the soft and hard palate. Not only is it more obvious
in its appearance than a submucosal cleft, it creates
the same speech problems as a submucosal cleft. The
more severe (longer) soft palate clefts are detected at
birth due to feeding difficulties. The cleft of the palate
makes it difficult for the infant to create a tight oral seal
around the nipple. As a result, the infant may not be
able to suckle. A partial or shorter soft palate cleft may
not show symptoms at birth or may reveal itself as
nasal reflux of liquids or foods.
19. Soft and Hard Palate Cleft: A cleft that
involves both the hard and soft palate will
include the entire soft palate and any part of
the hard palate up to the incisive foramen.
The most severe form involves the entire
secondary palate, seen as a gap in the palate
from the tip of the uvula to the incisive
foramen. This is the most overt of the
incomplete palate clefts. Similar to the
isolated soft palate clefts, the combined soft
and hard palate cleft is usually detected at
birth because of feeding problems. Speech
development will be impaired.
20.
21. complete: cleft extends all the way from the lip to the nose, The orbicularis oris
muscle is not in continuity, Instead of encircling the mouth, the muscle inserts into
the base of the nose on both sides of the cleft. This disruption creates difficulty
speaking, eating and drinking. The nose is distorted with a cleft lip deformity. The
nostril is widened and the floor of the nostril is missing
Unilateral: only affects one side of the
upper lip, Two thirds of the Cupid’s bow,
one philtral column and the philtral dimple
are preserved on the noncleft/normal sid
Bilateral: A bilateral complete cleft lip affects both the right and
left sides of the lip. There are no philtral columns, no philtral
dimple, and there is no orbicularis muscle in the central
segment. On both sides, the cleft extends from the vermillion to
the nostril. The lack of continuity in the muscle makes clear
speech difficult for these individuals. Feeding can be more
difficult.
There is nasal distortion with a bilateral complete cleft lip. Both
alar rims are widened, the nasal sills are missing and the nose
takes on a flattened appearance.
22. Incomplete: can take on a variety of appearances. There may be just a small
gap or cleft in the vermillion or it may slightly extend into the skin above the lip
or extend almost to the nostril. As with a complete cleft lip deformity, the nose
has some distortion. The distortion is usually to a lesser degree. The nostril
may be widened, but the floor of the nostril is intact. Additionally, there is a
bridge of tissue at the base of the nostril on the cleft side. This is known as a
Simonart’s band
Unilateral: has clefting of the lip on one side
only. There is a normal philtral column,
Cupid's bow and philtral dimple on the side
without a cleft. What makes it different from
a complete cleft lip is that some orbicularis
muscle fibers may cross the cleft
Bilateral: A bilateral cleft lip has a gap on both sides of the upper
lip. Just like with a bilateral complete cleft lip, the philtral columns
are affected. To what degree depends on how far up the cleft
extends from the vermillion. There may be some orbicularis muscle
intact. The nostril sills are intact. The philtral dimple is intact.
In a bilateral incomplete cleft lip deformity, the nose is affected,
although not to the same degree as with a bilateral complete cleft
lip deformity. In those individuals with a bilateral cleft lip deformity,
one side may be an incomplete deformity and the other side a
complete deformity.
23. Mixed Bilateral Incomplete and Complete
Cleft Lip
In those who have a bilateral cleft lip, one side
may be an incomplete cleft lip and the other
side a complete cleft lip deformity. An
incomplete cleft lip may be barely imperceptible.
A microform cleft lip
(also known as a "forme fruste") is the mildest version
of a cleft lip, and is categorized as an incomplete cleft.
A microform cleft lip does not have the obvious cleft
appearance of the other clefts. It may simply appear
as a vertical scar from the lip to the nose. There may
be a notch in the vermillion border and the nose may
be affected. While a microform cleft may not be
obvious, there may still be a problem with functionality.
There is some disruption of the continuity of the
orbicularis oris muscle. This may cause eating and
drinking issues as well as speech issues.
The nose is usually normal with a microform cleft
deformity
24.
25. Diagnosis of cleft lip and palate during pregnancy (antenatal
diagnosis)
•cleft lip and cleft palate are being diagnosed by ultrasound before the baby is
born. An ultrasound is a test that uses sound waves to create pictures of the
developing fetus. When analyzing the pictures, a doctor may detect an
abnormality in the facial structures.
A vertical hypo-echoic region through the fetal upper lip usually represents the
defect in cleft lip
Cleft lip may be detected with ultrasound beginning around the 18th-20 week of
pregnancy. As the fetus continues developing, it may be easier to accurately
diagnose a cleft lip. Cleft palate that occurs on its own is more difficult to
diagnose using ultrasound, since it can be difficult to see inside the fetus' mouth.
Occasionally a cleft is not picked up on the scan because the face is not visible
on the scan. Cleft palate without cleft lip is difficult to detect by antenatal scans
26.
27. Advantages of Prenatal
Diagnosis
Time for parental education
Time for parental psychological preparation
Opportunity to investigate other associated
anomalies
Gives parents the choice of continuing the
pregnancy
Opportunity for fetal
28. Diagnosis of cleft lip and palate after birth
Most cases of cleft lip and cleft palate are immediately apparent at birth
and don't require special tests for diagnosis.
Diagnosis may be made at birth using complete physical examination by
the physician. Since many of the associated syndromes show defects in
the genes and chromosomes, a chromosomal analysis is suggested for
the baby.
Diagnosis of associated problems like feeding problems, hearing loss,
ear infections, speech defects and teething problems is also important in
treatment of cleft lip and/or palate
29. Cleft lip and palate team
Pediatrician :provide medical care and refer to plastic
surgeon
plastic surgeon : initial lip repair and palatal surgery ,
pharyngoplasty and nose surgery
Maxillofacial surgery :bone grafting and orthopedic
surgery in later stage.
30. Neurosurgery :for any craniofacial syndrome
Pedodontic: the key member to asses patient and
his parents , monitor growth ,guide occlusion and
facial growth
Orthodontic: definitive orthodontic treatment once full
permanent arch erupt
Speech pathologist: monitor speech development to
normal ,test for adequate palate and pharyngeal
closure
31. Audiologist :test hearing in infant and
childhood
Psychologist: play important role when child
and family under stress
32. Problems Associated With Cleft Lip
and Palate
Feeding
Dental problems
Nasal Deformity and Esthetic Problems
Ear Problems
Speech Difficulties
33. Feeding Difficulties
Cleft lip= makes it more difficult for an infant to suck
on a nipple
Cleft Palate= may cause formula or breast milk to be
accidently taken up into the nasal cavity
Inability to create negative pressure inside oral cavity
Frequent regurgitations
Upper respiratory tract infections
34. Management of feeding problems :
i) For cleft lip patient
1- Breast feeding: mother can close cleft by the areola or
her hand to provide a seal.
2- Bottle feeding : a broad based nipple on a regular bottle
to provide the seal.
ii) For cleft palate :
Depending on severity of the cleft a variety of feeding
devices are available :
a) Soft premature nipples: conforms better to the palatal
defect and thus improves sucking.
b) Cross cut nipples: allow for easier flow of formula, and
thus decreasing strain on the child.
c) Long nipples: can be positioned posterior to the defect.
35. Dental Problems
Local Dental Problems:
Congenitally Missing teeth, Hypodontia, Hyperdontia,
Oligodontia
Presence of natal and neonatal teeth
Anamalies of tooth morphology like microdontia, macrodontia
etc
Fused teeth
Enamel Hypoplasia
Poor periodontal support, early loss of teeth
Gemination, Dilacerations
Orthodontics Problems:
Class III tendency
Anterior and Posterior Cross bite
Spacing and crowding
36. Nasal Deformity and Esthetic
Problems
Facial Disfigurements
Poor nasal shape
Scar marks of surgeries
Poor lip function during
speech
Poor dental alignment and
smile
37. Ear Problems
Middle ear disease - 22% to 88%
Conductive hearing loss and chronic suppurative otitis media
may result
Repeated tympanostomy tube placement
38. Cause's of ear problem
Mechanical
Infection
Dynamic
39. Speech Problems:
Hearing loss hampers proper development of speech
Velopharyngeal Insufficiency (VPI)
Abnormal air
Poor pronunciation of Bilabial, Labiodental,
Linguoalveolar sounds
40. General Management Protocol for the
Cleft Patient
Immediately after the birth –
pediatric consultation,
feeding instructions,
evaluation by geneticist
diagnosis of life expectancy of a child
41. At 10 to 12 weeks - surgical repair of the lip
Cleft Lip Repair Goals
Approximate cleft edges
Maintain Cupid's bow and philtrum
Align vermillion border
Create an intact nasal floor
Reconstituting the circumferential
integrity of the orbicularis oris muscle
42. From age 1 year to 18 months –
team evaluation and surgical repair of cleft palate
advantages for early closure of palatal defects:-
(1) better palatal and pharyngeal muscle development
(2) ease of feeding
(3) better development of phonation skills
(4) better auditory tube function
(6) improved psychologic state for parents and baby.
43. If the cleft is very wide, it is better to delay closure.
- Delayed palatal surgery will limit the inhibition of the
maxillary growth and minimizing the need for surgery to
widen the maxilla.
But, will lead to development of compensatory speech
and swallowing pattern, which are not easily corrected.
During this period we use
Non-Surgical methods
Dental Obturator. a plastic/acrylic, removable roof of the
mouth, which aids in speech, eating, and proper air flow.
For high-risk patients or those that refuse surgery.
Advantage- High rate of closure
Disadvantage- Need to wear a prosthesis, and need to
modify prosthesis as child grows.
Three months after palate repair- team
evaluation for speech and language assessment.
44. Three to six years –
team evaluation
behavioral intervention as
needed.
Speech therapy
treatment for middle year
infection,
fistula repair,
psychological evaluation.
45. At seven years - Orthodontic treatment phase I.
Nine to eleven years - alveolar bone grafting to
Provide bone for the eruption and/or orthodontic
repositioning of teeth
Closure of oro-nasal fistulas
Stabilization of the pre-maxilla in bilateral cases
Twelve years or later - Full orthodontic treatment phase II.
Fifteen to eighteen years - at the end of orthodontic
treatment, placement of implants, fixed bridge, etc. for
missing teeth.
46.
47. Eighteen to twenty-one years –
when most of growth is completed,
Surgical advancement of maxilla, if
required.
Final nose and lip revision -
rhinoplasty, 16-18 years.
48. In conclusion:
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49.
50. Langman Essential medical embryology for
T.W.salder
Conlemporary oral and maxillofacial
surgery
Hand book of Pediatric Dentistry By
Richard P widmer