Fairly common congenital disorder in which a fissure forms in the roof of the mouth. It may affect only the soft palate or extend through the hard palate, so that the nasal cavity opens into the mouth. The septum (dividing wall) between the nostrils is often absent. Cleft lip, a fissure in the lip beneath the nostril, or other abnormalities may accompany it. Cleft palate limits the ability of an infant to suck, which may lead to malnutrition, and causes speech problems in childhood. Surgical repair, usually at about 18 months of age, forms an airtight separation between nose and mouth. Speech training is still needed, and patients may have a high risk of nose, ear, and sinus infections.
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The term harelip is no longer used to describe the condition as it is considered offensive.
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 severeness of the cleft.
The complete restoration of this Cleft Child girl from above can be seen here. http://www.pbase.com/stella97king/cleft_lip
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. It 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).
The hole in the roof of the mouth caused by a cleft connects the mouth directly to the nasal cavity.
Note: the next images show the roof of the mouth. The top shows the nose, the lips are colored pink. For clarity the images depict a toothless infant.
A direct result of an open connection between the oral cavity and nasal cavity is velopharyngeal insufficiency (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.
Submucous cleft palate (SMCP) can also occur, which is an occult cleft of the soft palate with a classic clinical triad of bifid uvula, notching of the hard palate, and zona pellucida.
Rate of occurrence of CPO is similar for Caucasians, Africans, North American natives and Asians.
The upper lip is formed earlier than the palate, from the first three lobes named a to c above. Formation of the palate is the last step in joining the five embryonic facial lobes, and involves the back portions of the lobes b and c. 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. The biologic mechanisms of mutual recognition of the two cabinets, and the way they are glued together, are quite complex and obscure despite intensive scientific research.
The cause of cleft lip and cleft palate formation can be genetic in nature. A specific gene that increases threefold the occurrence of these deformities has been identified by Zucchero et al in 2004 as reported by the BBC.
Environmental influences may also cause, or interact with genetics to produce, orofacial clefting. Some 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).
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. Research continues to investigate the extent to which Folic acid can reduce the incidence of clefting.
In some cases, cleft palate is caused by syndromes which also cause other problems. Stickler's Syndrome can cause cleft lip and palate, joint pain, and myopia. Loeys-Dietz syndrome can cause cleft palate or bifid uvula, hypertelorism, and aortic aneurysm. Cleft lip/palate may be present in Patau Syndrome (trisomy 13). Many clefts run in families, even though there does not seem to be any identifiable syndrome present.
Most children with a form of clefting are monitored by a cleft palate team or craniofacial team through young adulthood. Care can be lifelong. Treatment procedures can vary between craniofacial teams. For example, some teams wait on jaw correction until the child is aged 10 to 12 (argument: growth is less influential as deciduous teeth are replaced by permanent teeth, thus saving the child from repeated corrective surgeries), while other teams correct the jaw earlier (argument: less speech therapy is needed than at a later age when speech therapy becomes harder). Within teams, treatment can differ between individual cases depending on the type and severity of the cleft.
Pioneering Surgeon Dr. Victor Collins has devised the first non skin graft procedure for the treatment of infantile unilateral incomplete and complete cleft lips. This technique involves the partial circumcision of the foreskin and then being applied to the affected area. This technique has led the way in the the modern day removal of the two most common types of cleft 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. Incomplete cleft gives the surgeon more tissue to work with, creating a more supple and natural-looking upper lip.
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. 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.
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.
| || || || || || || || || || || || || || || || || || || || || || |
|Repair cleft lip|
|Repair soft palate|
|Repair hard palate|
|Speech therapy/Pharyngeal surgery|
|Bone grafting jaw|
|Further cosmetic corrections|
Many of the top pediatric hospitals are developing their own CLP clinics in order to provide patients with comprehensive multi-disciplinary care from birth through adolescence. Allowing an entire team to care for a child throughout their cleft lip and palate treatment (which is ongoing) allows for the best outcomes in every aspect of a child's care. While the individual approach can yield significant results, current trends indicate that team based care leads to better outcomes for CLP patients. .
A complete listing of craniofacial teams is available through the Cleft Palate Foundation
Cleft may cause problems with feeding, ear disease, speech and socialization.
Due to lack of suction, an infant with a cleft may have trouble feeding. An infant with a cleft palate will have greater success feeding in a more upright position. Gravity will help prevent milk from coming through the baby's nose if he/she has cleft palate. Gravity feeding can be accomplished by using specialized equipment, such as the Haberman Feeder, or by using a combination of nipples and bottle inserts like the one shown, is commonly used with other infants. A large hole, crosscut, or slit in the nipple, a protruding nipple and pressure applied to the bottle insert by the caregiver's hand can result in controllable flow to the infant without the stigma caused by specialized equipment.
Individuals with cleft also face many middle ear infections which can eventually lead to total hearing loss. The Eustachian tubes and external ear canals may be angled or tortuous, leading to food or other contamination of a part of the body that is normally self cleaning.
Because the lips and palate are both used in pronunciation, individuals with cleft usually need the aid of a speech therapist. Bonding with the infant, socializing with family and community may be interrupted by the unexpected appearance, unusual speech and the surgical interventions necessary. Support for the parents as well as for the child can be pivotal.(see Psychosocial issues)
A cleft palate may impact an individual’s self-esteem, social skills, and behavior. There is a large amount of research dedicated to the psychosocial development of individuals with cleft palate. Self-concept may be adversely affected by the presence of a cleft palate. Research has shown that during the early preschool years (ages 3-5), children with cleft palate tend to have a self-concept that is similar to their peers without a cleft. However, as they grow older and their social interactions with other children increase, children with clefts tend to report more dissatisfaction with peer relationships and higher levels of social anxiety. Experts conclude that this is probably due to the associated stigma of visible deformities and speech abnormalities, if present. Children who are judged as attractive tend to be perceived as more intelligent, exhibit more positive social behaviors, and are treated more positively than children with cleft palate. 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. It has been reported that elevated stress levels in mothers correlated with reduced social skills in their children. Strong parent support networks may help to prevent the development of negative self-concept in children with cleft palate . In the later preschool and early elementary years, the development of social skills is no longer only impacted by parental attitudes but is beginning to be shaped by their peers. A cleft palate may affect the behavior of preschoolers. Experts suggest that parents discuss with their children ways to handle negative social situations related to their cleft palate. A child who is entering school should learn the proper (and age-appropriate) terms related to the cleft. The ability to confidently explain the condition to others may limit feelings of awkwardness and embarrassment and reduce negative social experiences.
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. An adolescent with cleft palate will deal with the typical challenges faced by most of their peers including issues related to self esteem, dating, and social acceptance. Adolescents, however, view appearance as the most important characteristic above intelligence and humor. This being the case, 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 palate often deal with threats to their quality of life for multiple reasons including: unsuccessful social relationships, deviance in social appearance, and multiple surgeries. Individuals with cleft palate often have lower QOL scores than their peers . Psychosocial functioning of individuals with cleft palate often improves after surgery, but does not last due to unrealistic expectations of surgery .
|Tutankhamen||Egyptian Pharaoh who may have had a slightly cleft palate according to diagnostic imaging|
|Doc Holliday||Dentist, gambler and gunfighter of the American Old West frontier|
|Tad Lincoln||Fourth and youngest son of President Abraham Lincoln|
|Victor Collins||Well know surgeon who developed the pioneering technique for harelip removal.|
|Carmit Bachar||American dancer and singer|
|Jürgen Habermas||German philosopher and sociologist|
|Wendy Harmer||Australian comedian|
|Richard Hawley||English guitarist, singer, songwriter and producer|
|Michael Helm||Canadian novelist|
|Jesse Jackson||American politician, professional civil rights activist and Baptist minister|
|Stacy Keach||American actor and narrator|
|Tim Lott||English novelist|
|Rita MacNeil||Canadian country and folk singer|
|Cheech Marin||American actor and comedian|
|Jesse Merz||American actor and director|
|Ljubo Milicevic||Australian football (soccer) player|
|Nikki Payne||Canadian comedian and actress|
|Geoff Plant||Canadian lawyer and politician, Attorney-General of British Columbia|
|Lee Raymond||American business executive - former CEO and Chairman of ExxonMobil|
|Louis Wain||English artist, best known for his drawings featuring anthropomorphised large-eyed cats|
|Tom Brokaw||American television journalist and author.|
|Barney Frank||American Congressmen.|
The popular belief that Joaquin Phoenix has a repaired cleft lip is mistaken. The mark on his lip is a microform, an almost-cleft that healed itself in utero. If the tissues join up just enough to create correct bone and muscle tissues, no corrective surgery is required, as was the case with Phoenix.
Difficulty with nursing is the most common problem associated with clefts, but aspiration pneumonia, regurgitation, and malnutrition are often seen with cleft palate and is a common cause of death. Providing nutrition through a feeding tube is often necessary, but corrective surgery in dogs can be done by the age of twelve weeks. For cleft palate, there is a high rate of surgical failure resulting in repeated surgeries. Surgical techniques for cleft palate in dogs include prosthesis, mucosal flaps, and microvascular free flaps. Affected animals should not be bred due to the hereditary nature of this condition.