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Cleft Lip and Palate (Harelip or Cheiloschisis and Palatoschisis) cleft

What is Cleft lip and palate "Harelip” or "Lip-nose Deformities"?
Cleft lip and Palate
Cleft lip (cheiloschisis) and cleft palate (palatoschisis), which can also occur together as cleft lip and palate, are variations of a type of clefting congenital deformity caused by abnormal facial development during gestation. A cleft is a fissure or opening or a gap. It is the non-fusion of the body's natural structures that form before birth.
Approximately 1 in 700 children born have a cleft lip and/or a cleft palate. It has sometimes colloquially been equated with the phrase harelip, but this is used incorrectly, as that term refers to the pulling of the lip towards the nose.
A cleft lip or palate can be successfully treated with surgery, especially so if conducted soon after birth or in early childhood.

Clefts can also affect other parts of the face, such as the eyes, ears, nose, cheeks, and forehead. In 1976, Paul Tessier described fifteen lines of cleft. Most of these craniofacial clefts are even rarer and are frequently described as Tessier clefts using the numerical locator devised by Tessier.
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Embryology and causes of cleft !

The development of the face is coordinated by complex morphogenetic events and rapid proliferative expansion, and is thus highly susceptible to environmental and genetic factors, rationalising the high incidence of facial malformations. During the first six to eight weeks of pregnancy, the shape of the embryo's head is formed. Five primitive tissue lobes grow:


Embryology and causes of cleft



(Orange) one from the top of the head down towards the future upper lip; (Frontonasal Prominence)
(Bluish) two from the cheeks, which meet the first lobe to form the upper lip; (Maxillar Prominence)
(Greenish) and just below, two additional lobes grow from each side, which form the chin and lower lip; (Mandibular Prominence)
If these tissues fail to meet, a gap appears where the tissues should have joined (fused). This may happen in any single joining site, or simultaneously in several or all of them. The resulting birth defect reflects the locations and severity of individual fusion failures (e.g., from a small lip or palate fissure up to a completely malformed face).

The upper lip is formed earlier than the palate, from the first three lobes (Frontonasal and Maxillar Prominences). Formation of the palate is the last step in joining the five embryonic facial lobes, and involves the back portions of the lobes (Maxillar Prominences). These back portions are called palatal shelves, which grow towards each other until they fuse in the middle. This process is very vulnerable to multiple toxic substances, environmental pollutants, and nutritional imbalance.

Genetic factors contributing to cleft lip and cleft palate formation have been identified for some syndromic cases, but knowledge about genetic factors that contribute to the more common isolated cases of cleft lip/palate is still patchy.
Many clefts run in families, even though in some cases there does not seem to be an identifiable syndrome present, possibly because of the current incomplete genetic understanding of midfacial development. (e.g. if a person is born with a cleft, the chances of that person having a child with a cleft, given no other obvious factor, rises to 1 in 14).

Syndromic cases:
The Van der Woude Syndrome: is caused by a specific variation in the gene IRF6 that increases the occurrence of these deformities three folds.
Siderius X-linked mental retardation syndrome: is caused by mutations in the PHF8 gene.
Stickler's Syndrome: cleft lip and palate, joint pain, and myopia.
Loeys-Dietz syndrome: cleft palate or bifid uvula, hypertelorism, and aortic aneurysm.
Hardikar syndrome: cleft lip and palate, Hydronephrosis, Intestinal obstruction and other symptoms.
Patau Syndrome (trisomy 13): Cleft lip/palate and other symptoms and disorders.

Non-syndromic cases:
This includes in particular sequence variants in the genes IRF6, PVRL1 and MSX1.
Environmental influences may also cause orofacial clefting. In humans, fetal cleft lip had been linked to maternal hypoxia, as caused by e.g. maternal smoking, alcohol abuse or some forms of maternal hypertension treatment.
Other environmental factors that have been studied include: seasonal causes (such as pesticide exposure); maternal diet and vitamin intake; retinoids - which are members of the vitamin A family; anticonvulsant drugs; alcohol; cigarette use; nitrate compounds; organic solvents; parental exposure to lead; and illegal drugs (cocaine, crack cocaine, heroin, etc).
Current research continues to investigate the extent to which Folic acid can reduce the incidence of clefting.


Types of Cleft lip and palate:

Lip cleft can occur as a one sided (unilateral) or two sided (bilateral). It is due to the failure of fusion of the maxillary and medial nasal processes (formation of the primary palate).
Palate cleft occurs due to the failure of fusion of the lateral palatine processes, the nasal septum, and/or the median palatine processes (formation of the secondary palate).

If the cleft does not affect the palate structure of the mouth it is referred to as cleft lip.
Cleft lip is formed in the top of the lip as either a small gap or an indentation in the lip (partial or incomplete cleft) or it continues into the nose (complete cleft).
A mild form of a cleft lip is a microform cleft. A microform cleft can appear as small as a little dent in the red part of the lip or look like a scar from the lip up to the nostril. In some cases muscle tissue in the lip underneath the scar is affected and might require reconstructive surgery. It is advised to have newborn infants with a microform cleft checked with a craniofacial team as soon as possible to determine the severity of the cleft.

types of cleft lip



Cleft palate is a condition in which the two plates of the skull that form the hard palate (roof of the mouth) are not completely joined. The hole in the roof of the mouth caused by a cleft connects the mouth directly to the nasal cavity. The soft palate is in these cases cleft as well. In most cases, cleft lip is also present.
Palate cleft can occur as complete (soft and hard palate, possibly including a gap in the jaw) or incomplete (a 'hole' in the roof of the mouth, usually as a cleft soft palate). When cleft palate occurs, the uvula is usually split. Submucous cleft palate (SMCP) can also occur, which is an occult cleft of the soft palate with a classic clinical triad of bifid uvula, a furrow along the midline of the soft palate, and a notch in the posterior margin of the hard palate.

types of cleft palate



A direct result of an open connection between the oral cavity and nasal cavity is velopharyngeal inadequacy (VPI). Because of the gap, air leaks into the nasal cavity resulting in a hypernasal voice resonance and nasal emissions.

Secondary effects of VPI include speech articulation errors (e.g., distortions, substitutions, and omissions) and compensatory misarticulations (e.g., glottal stops and posterior nasal fricatives). Possible treatment options include speech therapy, prosthetics, augmentation of the posterior pharyngeal wall, lengthening of the palate, and surgical procedures.
3D US

Complaints manifestations and Diagnosis!

The spectrum of severity in visible cleft lip and palate is broad, ranging from notches of the vermilion and/or grooves in the philtrum to complete unilateral and bilateral clefts of the lip and palate.

Traditionally, the diagnosis is made at the time of birth by physical examination. Recent advances in prenatal diagnosis have allowed obstetricians to diagnose facial clefts in utero.


Plain of Management:

Cleft lip and palate is very treatable; however, the kind of treatment depends on the type and severity of the cleft. Cleft may cause problems with feeding, ear disease, speech and socialization.
The members of the Cleft Management Team at a minimum include: a plastic or facial plastic surgeon, or an Oral and maxillofacial surgeon trained in craniofacial surgery, otolaryngologist, geneticist, orthodontist, speech therapist and social worker.


A- Since birth till 2 months:
- Care of breast feeding: a baby being fed using a customized bottle, the Haberman Feeder or a spoon. The upright sitting position allows gravity to help the baby swallow the milk more easily. A large hole, crosscut, or slit in the nipple, a protruding nipple and rhythmically squeezing the bottle insert can result in controllable flow to the infant without the stigma caused by specialized equipment.

method of feeding cleft lip pateint



- Care of chest infection: guard against regurgitation.
- Care of middle ear infection (otitis media): Recurrent middle ear infections - which can eventually lead to total hearing loss - is caused by the abnormal Eustachian tubes where food or secretions is stagnated.
To avoid these problems feeding and sleeping should be with elevated head. A tympanostomy tube is often inserted into the eardrum to aerate the middle ear. This is often beneficial for the hearing ability of the child.
- Pre-surgical devices for cleft lip treatment: In some cases of a severe bilateral complete cleft, the premaxillary segment will be protruded far outside the mouth. Nasoalveolar molding followed by surgery can improve long-term nasal symmetry among patients with complete unilateral cleft lip-cleft palate patients compared to surgery alone.

B- At 2 to 3 months (Lip repair +/- alveolus repair):
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" (the child is at least 10 weeks of age; weighs at least 10 pounds, and has at least 10g hemoglobin). The most common procedure to repair a cleft lip is the Millard procedure.

steps of cleft lip



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. Incomplete cleft gives the surgeon more tissue to work with, creating a more supple and natural-looking upper lip. Often an incomplete cleft lip requires the same surgery as complete cleft. This is done for two reasons:
- Firstly the group of muscles required to purse the lips run through the upper lip. In order to restore the complete group a full incision must be made.
- Secondly, to create a less obvious scar the surgeon tries to line up the scar with the natural lines in the upper lip (such as the edges of the philtrum) and tuck away stitches as far up the nose as possible.

C- At 9 up to 18 months: (Palate repair +/- Orthodontics):
- Pre-surgical palatal obturator (a prosthetic device made to fit the roof of the mouth covering the gap).
- Approximately 20-25% only require one palatal surgery to achieve a competent velopharyngeal valve capable of producing normal, non-hypernasal speech. However, combinations of surgical methods and repeated surgeries are often necessary as the child grows. - One of the new innovations of cleft lip and cleft palate repair is the Latham appliance. The Latham is surgically inserted by use of pins during the child's 4th or 5th month. After it is in place, the doctor or parents, turn a screw daily to bring the cleft together to assist with future lip and/or palate repair.

D- At 3 years: (Speech and Hearing Problems):
- Soft palate repair: Speech problems are usually treated by a speech-language pathologist. In some cases pharyngeal flap surgery or augmentation pharyngoplasty is performed to reduce the escape of nasal airflow in speech sounds requiring oral air pressure, to improve the pronunciation of those sounds, and reduce nasality in those parts of speech that are not normally nasalized.
- The speech-language pathologist may also be called on to correct incorrect speaking habits that the child developed before the cleft was corrected surgically. Speech is both receptive and expressive:
i. We hear and understand spoken language (receptive).
ii. We learn to manipulate our mouth, tongue, oral cavity, to express ourselves (expressive).
Babies with palatal clefts may have compromised hearing and therefore, if the baby cannot hear, it cannot try to mimic the sounds of speech. Thus, even before expressive language acquisition, the baby with the cleft palate is at risk for receptive language acquisition. Because the lips and palate are both used in pronunciation, individuals with cleft usually need the aid of a speech therapist.

E- At 7 years (Orthodontics):
- Alveolus repair by bone grafting: If the cleft extends into the maxillary alveolar ridge, the gap is usually corrected by filling the gap with bone tissue. The bone tissue can be acquired from the patients own chin, rib or hip.

F- At 18 years:
- Rhinoplasty
- jawbone surgery


The Psychosocial Aspects of Cleft Lip and Palate Cases:

Having a cleft lip and/or palate does not inevitably lead to a psychosocial problem. Most children who have their clefts repaired early enough are able to have a happy youth and a healthy social life. However, it is important to remember that adolescents with cleft lip and/or palate are at an elevated risk for developing psychosocial problems especially those relating to self-concept, peer relationships, and appearance. It is important for parents to be aware of the psychosocial challenges their adolescents may face and to know where to find professional help if problems arise.

A cleft lip and/or palate may impact an individual’s self-esteem, social skills, and behavior. Experts conclude that this is probably due to the associated stigma of visible deformities and speech abnormalities, if present. Children with clefts tend to report feelings of anger, sadness, fear, and alienation from their peers. Yet these children were similar to their peers in regard to "how well they liked themselves."

The relationship between parental attitudes and a child’s self-concept is crucial during the preschool years. Strong parent support networks may help to prevent the development of negative self-concept in children with cleft palate. Experts suggest that parents discuss with their children ways to handle negative social situations related to their cleft lip and or cleft palate.

As children reach adolescence, the period of time between age 13 and 19, the dynamics of the parent-child relationship change as peer groups are now the focus of attention. Adolescents are susceptible to additional problems because they cannot hide their facial differences from their peers. Adolescent boys typically deal with issues relating to withdrawal, attention, thought, and internalizing problems and may possibly develop anxiousness-depression and aggressive behaviors. Adolescent girls are more likely to develop problems relating to self-concept and appearance.
Individuals with cleft lip and or cleft palate often deal with threats to their quality of life for multiple reasons including: unsuccessful social relationships, deviance in social appearance, and multiple surgeries. Psychosocial functioning of individuals with cleft lip and or cleft palate often improves after surgery, but does not last due to unrealistic expectations of surgery.

In some countries, cleft lip or palate deformities are considered reasons to perform abortion beyond the legal fetal age limit. Islamic beliefs and some human rights activists contend this practice is a form of murder or crime.


How Much Do Cleft lip and/or palate repair Typically Cost?

Although price is a factor when deciding on surgery, the most important factor is that you feel you are getting a good quality, natural-looking result from the procedure.

As Lip and /or palate repair is a staged procedures. The costs are variable according to the deformity and the used technique. Usually the cost is ranging from 1500 to 2500 U.S. dollars per each stage. When dealing with costs, you also need to take into consideration the fact that the procedure may need to be repeated. Most medical insurance providers cover the procedure.


FAQ from patients when considering undergoing lip or palate repair?

Q - What is the type of anesthesia?
A - General anesthesia.

Q - What are the symptoms that may occur in the first five days after the procedures?
A - There may be edema or swelling of the face.

Q - What are the guarantees that the success of the process?
A - There is no doubt that the followers of the medical instructions and close follow up visits are the main factors for the success of any surgical procedure.

Q - When can we get the final results?
A - The final results is achieved after the 18 years.

Q - Is the Repaired Lip and palate seems naturally?
A - To variable degree.

Q - What is ideal age for Lip repair? Palate repair? Nose job?
A - It could be done at any age. However ideally, lip repair will be done before 3 months, palate repair will be done before 18 months and Rhinoplasty should be done after 18 years.

Q - If you are not Egyptian, What is the minimum period for follow up?
A - One week per each stage.

Q - What are the contraindications of lip repair procedures?
A - There are no absolute contraindications but the parents should inform surgeons about any allergies, serious medical conditions, and medications they may be taking.

Q - Where will the procedures be performed?
A - In a hospital without stay.

Q - How much does lip or palate repair procedures cost and what elements factor into that cost like hospital fees, anesthesia, and so on?
A - Ranging from 1500 to 2000 U.S. dollars per stage. Medical insurance covers these procedures.

Q - What is the surgeon’s level of experience in performing cleft Lip and palate repair procedures?
A - Consultant or Doctoral in Plastic surgery.

Q - What percentage of patients experience complications with cleft Lip and palate repair procedures?
A - 10 - 15%


Summary of Cleft Lip and Palate Repair Procedures:

The time of the procedure:
from one to two hours.
Type of anesthesia:General anesthesia.
Location:hospital without postoperative hospital stay.
Expected Side effects:Temporary swelling and pain.
Recovery time:Within 2 to 3 weeks.
Cost:according to procedure ranging from 1500 to 2000 U.S. dollars per stage.
Results: Permanent.