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Embryology
In facial morphogenesis, neural crest cells migrate into the facial region, where they form the
skeletal and connective tissue and all dental tissues except the enamel. Vascular endothelium
and muscle are of mesodermal origin (Cohen, 2000).
The upper lip is derived from medial nasal and maxillary processes. Failure of merging
between the medial nasal and maxillary processes at the fifth week of embryonic
development, on one or both sides, results in CL.
As mentioned in the previous section, a cleft develops when embryonic parts called processes
(which are programmed to grow, move, and join with each other to form an individual part of
the embryo) do not reach each other in time and an open space (cleft) between them persists.
In the normal situation, the processes grow into an open space by means of cellular migration
and multiplication, touch each other, and fuse together.
In general, any factor that could prevent the processes from reaching each other by slowing
down migration, multiplication, or both of neural crest cells by stopping tissue growth and
development for a time or by killing some cells that are already in that location would cause a
persistence of a cleft. Also, the epithelium that covers the mesenchyme may not undergo
programmed cell death, so that fusion of processes cannot take place (Cohen, 2000).
CL usually occurs at the junction between the central and lateral parts of the upper lip on
either side. The cleft may affect only the upper lip, or it may extend more deeply into the
maxilla and the primary palate. (Cleft of the primary palate includes CL and cleft of the
alveolus.) If the fusion of palatal shelves is impaired also, the CL is accompanied by CP,
forming the CLP abnormality.
CP is a partial or total lack of fusion of palatal shelves. It can occur in a number of ways:
• Defective growth of palatal shelves
• Failure of the shelves to attain a horizontal position
• Lack of contact between shelves
• Rupture after fusion of shelves
The secondary palate develops from the right and left palatal processes. Fusion of palatal
shelves begins at the 8th week of the fetal period and continues usually until the 12th week.
One hypothesis is that a threshold exists beyond which delayed movement of palatal shelves
does not allow closure to take place, and this results in a CP.

Embryology
• Cleft lip/palate occurs during embryonic period which ends at about the 8th week
• 3-4 weeks
• structures that will be the face can be seen
o anterior neuropore
o eye bulges
o stomodeum
• 5-6 weeks
• face becomes apparent
• 1st/mandibular branchial arch
o anterior 2/3 of the tongue
• 2nd/hyoid branchial arch
o structures of the mid- and lower face
• 3rd branchial arch
o base of the tongue
• nasal pits can be seen on either side of the face

Embryology
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Neural Crest Theory

• Face development requires appropriate formation of neural crest cells around the
anterior neuropore
• Neural crest cells composed of ectoderm
o Migrate at different rates
o Give rise to a variety of connective and nervous tissue of the skull, branchial
arches, the face
• Cells which form frontonasal process arise from the forebrain fold
• Move quickly into the area which will become the nose and adjacent structures
• Cells which form the right and left maxillary processes and the mandible have farther
to travel and take longer to reach the branchial arches
Mesenchymal tissue
• will become connective tissue
• formed by migrating cells
• provides most of the bone and soft tissue that will make up the face
• these cells travel to their destinations
• begin to differentiate according to DNA components
Primary Palate
• Formed during the 6th week
• Includes
• prolabium (lip tissue)
• premaxilla
• 4 maxillary incisors
• Clefts of the lip occur in disturbances in the primary palate
• clefts of lip may be due to premature cessation of epithelial fusion
• epithelial wall is too short as it comes into the nasal cavity
• frontal nasal process and one or both of the lateral maxillary processes fail to unite to
complete the upper lip
Secondary Palate
• 7-8 weeks
• Fusion of primary and secondary palates separates the oral and nasal cavities
• Tongue occupies the oral space and pushes up into nasal cavity
• Palatal shelves are in a vertical plane on either side of the tongue
• Tongue drops and shelves elevate to a horizontal position above the tongue and below
the nasal septum
• Elevation occurs from back to front
• Shelves grow and move toward midline
• Shelves meet and fusion begins and proceeds from front to back
• Nasal septum grows downward and fuses with the palate
• Hard and soft palate development complete by 10th week
• Final closure of uvula may occur as late as the 12th week
• Any disturbance or interruption from the 7th to 12th week may interrupt
elevation/fusion of palates
• Early disturbance: greater defect
• Late disturbance: less severe defect
Embryology
• Defects or delays in neural crest formation or migration may lead to clefts in the
primary palate
• Timing factor in palatal shelf movement
• elevation occurs late; shelves may be too far apart for fusion to occur
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cleft palate
Cleft palate occurs when the roof of the mouth does not completely close, leaving an opening
that can extend into the nasal cavity. The cleft may involve either side of the palate. It can
extend from the front of the mouth (hard palate) to the throat (soft palate). Often the cleft will
also include the lip. Cleft palate is not as noticeable as cleft lip because it is inside the mouth.
It may be the only abnormality in the child, or it may be associated with cleft lip or other
syndromes. In many cases, other family members have also had a cleft palate at birth.
cleft lip
Cleft lip is an abnormality in which the lip does not completely form during fetal
development. The degree of the cleft lip can vary greatly, from mild (notching of the lip) to
severe (large opening from the lip up through the nose). As a parent, it may be stressful to
adjust to the obvious abnormality of the face, as it can be very noticeable.
Causes embriology:
- Failure match between nasomedial dan nasolateral
- Failure of migration & mesodermal
Causes factors :
1. Labioschisis : parent’s age, medicine, infection, cigarette, congenital  during
pregnant
2. Palatoschisis : Vit A; corticosteroid- during pregnant, phenytoin
1) environmental insults (i.e. maternal diseases, chemotherapy, radiation, alcohol, excess
retinoic acid and anticonvulsant medications)
2) genetic factors  There is increased risk for congenital malformations because of
maternal age at the time of pregnancy.
Etiology
1. Genetic factors
• mutant genes
o responsible for clefts associated with some rare syndromes
o autosomal dominant
o only one parent must carry the trait
o autosomal recessive
o both parents must carry the trait
• chromosomal aberrations
o congenital malformation of genetic material
o trisomy 13 results in cleft lip w/ or w/o cleft palate in 60-70% of the cases
2. Environmental teratogens
• agents that act upon the developing fetus to create malformations
• maybe susceptible genotypes
• Infections, diseases, radiation, environmental chemicals, maternal metabolic and
endocrine imbalances, drugs, nutritional deficiences and excesses
• Valium and Dilantin during pregnancy
3. Multifactorial inheritance
• many genes contribute to clefting
• individually these are minor
• interactions of genotypes with negative environmental factors may cause abnormality

Diagnosed
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- ultrasound in the second trimester of pregnancy, amniocentesis and molecular genetic

techniques can be used to detect common congenital malformations, including cleft
lip, early.
Complication :
- children with cleft lip and palate may have difficulty hearing or speaking clearly
- abnormal midface development, velopharyngeal incompetence, speech defects, and
abnormal Eustachian tube function.
- Cleft patients have an incompetent velopharyngeal valve, which results in nasal
regurgitation while swallowing, as well as "typical cleft palate speech": Nasal escape
of air (heard as snorting or grunting), a hypernasal quality, and difficulty with
consonants
- the cleft palate patients had a "functional" obstruction of the tube at the
nasopharyngeal end--as opposed to the "mechanical" obstruction found in serous otitis
patients.
- feeding difficultiesFeeding difficulties occur more with cleft palate abnormalities.
The infant may be unable to suck properly because the roof of the mouth is not
formed completely.
- ear infections and hearing lossEar infections are often due to a dysfunction of the
tube that connects the middle ear and the throat. Recurrent infections can then lead to
hearing loss.
- speech and language delayDue to the opening of the roof of the mouth and the lip,
muscle function may be decreased, which can lead to a delay in speech or abnormal
speech. Referral to a speech therapist should be discussed with your child's physician.
- dental problems As a result of the abnormalities, teeth may not erupt normally and
orthodontic treatment is usually required.

Treatment for cleft lip and cleft palate:

Specific treatment will be determined by your child's physician based on:
• your child's age, overall health, and medical history
• specific qualities of your child's abnormality
• your child's tolerance for specific medications, procedures, or therapies
• involvement of other body parts or systems
• your opinion or preference
Surgery is usually performed during the first 3 to 6 months (usually when the baby is 10 to 12
pounds to repair cleft lip and between 9 and 14 months to repair the cleft palate. Both types
of surgery are performed in the hospital under general anesthesia.
Cleft lip treatment
Within the first 2-3 months after birth, surgery is performed to close the cleft lip.
While surgery to repair a cleft lip can be performed soon after birth, the often
preferred age is at approximately 10 weeks of age, following the "rule of 10s" coined
by surgeons Wilhelmmesen and Musgrave in 1969 (the child is at least 10 weeks of
age; weighs at least 10 pounds, and has at least 10g hemoglobin). If the cleft is
bilateral and extensive, two surgeries may be required to close the cleft, one side first,
and the second side a few weeks later.
Cleft palate treatment
Often a cleft palate is temporarily closed using a palatal obturator. The obturator is a
prosthetic device made to fit the roof of the mouth covering the gap.Cleft palate can
also be corrected by surgery, usually performed between 6 and 12 months.
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Speech and hearing treatments

Prevention
Pregnant women and women who are likely to become pregnant can do the following to help
prevent oral-facial clefts in their unborn children:
• Consume 400 micrograms of folic acid daily by taking a multivitamin or eating foods
containing folic acid, such as:
o Fruits and orange juice
o Green leafy vegetables
o Dried beans and peas
o Pasta, rice, bread, flour, and cereals
• Do not smoke or drink alcohol during pregnancy.
• Take medications during pregnancy only as directed by your doctor.
• Get early and regular prenatal care.
• If you are thinking about having a child and have risk factors for oral-facial cleft:
o Seek medical advice on additional ways to prevent the disorder.
o Consider genetic counseling.

Types of Clefts: Cleft Lip

• involves soft tissue
• extends through the vermilion border
• incomplete cleft-only a notch in lip
• complete cleft-lip and to the nose
• unilateral or bilateral
• usually a left unilateral
• may extend through alveolus
• may occur w/ or w/o a cleft palate
• cleft lip may adversely affect nose structure
• short columnella

UNILATERAL COMPLETE CLEFT OF THE LIP

Types of Cleft: Cleft Palate
Normal palate anatomy:
• hard palate partitions the nose and mouth
• hard palate is anterior 2/3 of the roof of mouth
• hard palate is maxillary & palatine bones
• alveolar ridge forms anterior and lateral borders
• posterior 1/3 is soft palate
• hard palate is stationery, soft palate moves
Cleft Palate
• Complete cleft of lip and palate
• extends from uvula through lip
• unilateral clefts: nasal septum attached to larger palatal segments
• bilateral clefts: nasal septum not attached to either segment
• with uni- or bilateral clefts can view the inferior edge of septum through the mouth

COMPLETE BILATERAL CLEFT UVULA THROUGH THE NOSE

Isolated Cleft
• without cleft lip
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• varies in severity
• may be any of the following:
• all of hard palate and soft palate
• only the soft palate, or a portion of it
• any degree between these two extremes
• bifid uvula: the simplest defect
Bifid Uvula--Submucous Cleft???
Submucous Cleft
• palate appears to be structurally intact
• bony and muscular deficits exist
• triad of symptoms:
• a bony notch in the hard palate
• bluish line at the midline of the soft palate
• a bifid uvula
• bony notch located where the posterior nasal spine would normally appear
• may not be visible but may be palpated
• submucous cleft of hard palate IS NOT usually clinically significant
• muscular deficit of soft palate IS usually significant
• levator muscles are usually inserted into the hard palate
• the normal levator sling does not exist
• shortens the range of motion of the soft palate
• clinical result: velopharyngeal incompetence
• Kaplan study

SUBMUCOUS CLEFT
Variations in Severity
• Cleft Length
• Complete cleft is usually regarded as more severe
• Incomplete cleft is usually easier to repair
• Repair of cleft of soft palate only is usually better than a complete cleft of the lip and
palate
• Cleft Width
• wide cleft is more difficult to manage than a narrow cleft; ex: "horseshoe shaped"
Other Cleft-Related Deformities
• Microforms of clefts of the palate/lip
• Congenital hairline lip scars
• Dental anomalies including congenital missing lateral incisors
• Skeletal deformities
• Facial dysmorphia; asymmetries