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 Congenital disorder involves defects in or damage to a developing fetus. It may be the result of genetic abnormalities, the intrauterine environment, errors of morphogenesis, or a chromosomal abnormality. The outcome of the disorder will further depend on complex interactions between the pre-natal deficit and the post-natal environment. Congenital caused by abnormal facial development during gestation Gestation is the carrying of an embryo or fetus inside a female viviparous animal. Mammals during pregnancy can have one or more gestations at the same time . The time interval of a gestation plus 2 weeks is called gestation period, and the length of time plus 2 weeks that the offspring have spent developing in the uterus is called gestational age. A cleft is a fissure or opening—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 considered deeply offensive, and is used incorrectly, as that term refers to the pulling of the lip towards the nose.

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 more rare and are frequently described as Tessier clefts using the numerical locator devised by Tessier.^[1]

A cleft lip or palate can be successfully treated with surgery Surgery is a medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, to help improve bodily function or appearance, and sometimes for religious reasons. An act of performing surgery may be called a surgical procedure, operation, or simply, especially so if conducted soon after birth Birth is the act or process of bearing or bringing forth offspring. The offspring is brought forth from the mother. Different forms of birth are oviparity, vivipary and ovovivipary or in early childhood.

Cleft lip and 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). 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 The medial nasal prominence is an embryological structure that forms the upper lip and nose (formation of the primary palate).

A mild form of a cleft lip is a microform cleft.^{[citation needed]} 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.^{[citation needed]} In some cases muscle tissue in the lip In human anatomy, the orbicularis oris muscle is the sphincter muscle around the mouth underneath the scar is affected and might require reconstructive surgery.^{[citation needed]} 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.^{[citation needed]}

Cleft palate

Cleft palate is a condition in which the two plates of the skull In humans, the adult skull is normally made up of 22 bones. Except for the mandible, all of the bones of the skull are joined together by sutures, synarthrodial joints formed by bony ossification, with Sharpey's fibres permitting some flexibility that form the hard palate The hard palate is a thin horizontal bony plate of the skull, located in the roof of the mouth. It spans the arch formed by the upper teeth (roof of the mouth) are not completely joined. The soft palate The soft palate is the soft tissue constituting the back of the roof of the mouth. The soft palate is distinguished from the hard palate at the front of the mouth in that it does not contain bone is in these cases cleft as well. In most cases, cleft lip is also present. Cleft palate occurs in about one in 700 live births worldwide.^[2]

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 The uvula is the conic projection from the posterior edge of the middle of the soft palate, composed of connective tissue containing a number of racemose glands, and some muscular fibers (musculus uvulae). It is frequently confused with the epiglottis and the tonsils 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 secondary palate is an anatomical structure that divides the nasal cavity from the oral cavity in many vertebrates).

The hole in the roof of the mouth caused by a cleft connects the mouth directly to the nasal cavity The nasal cavity conditions the air to be received by the other areas of the respiratory tract. Owing to the large surface area provided by the conchae, the air passing through the nasal cavity is warmed or cooled to within 1 degree of body temperature. In addition, the air is humidified, and dust and other particulate matter is removed by.

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 The nasal cavity conditions the air to be received by the other areas of the respiratory tract. Owing to the large surface area provided by the conchae, the air passing through the nasal cavity is warmed or cooled to within 1 degree of body temperature. In addition, the air is humidified, and dust and other particulate matter is removed by is velopharyngeal inadequacy (VPI). Because of the gap, air leaks into the nasal cavity resulting in a hypernasal voice The human voice consists of sound made by a human being using the vocal folds for talking, singing, laughing, crying, screaming, etc. Human voice is specifically that part of human sound production in which the vocal folds (vocal cords) are the primary sound source. Generally speaking, the mechanism for generating the human voice can be subdivided resonance In physics, resonance is the tendency of a system to oscillate with larger amplitude at some frequencies than at others. These are known as the system's resonant frequencies (or resonance frequencies). At these frequencies, even small periodic driving forces can produce large amplitude oscillations and nasal emissions.^[3] Secondary effects of VPI include speech articulation In linguistics , manner of articulation describes how the tongue, lips, jaw, and other speech organs are involved in making a sound make contact. Often the concept is only used for the production of consonants. For any place of articulation, there may be several manners, and therefore several homorganic consonants errors (e.g., distortions A distortion is the alteration of the original shape of an object, image, sound, waveform or other form of information or representation. Distortion is usually unwanted. In some fields, distortion is desirable, such as electric guitar (where distortion is often induced purposely with the amplifier or an electronic effect to achieve an aggressive, substitutions, and omissions) and compensatory misarticulations (e.g., glottal stops The glottal stop, or more fully, the voiceless glottal plosive, is a type of consonantal sound used in many spoken languages. In English the feature is represented for example by the hyphen in uh-oh! and by the apostrophe or ʻokina in Hawaiʻi among those attempting an authentic pronunciation of that name and posterior nasal fricatives Fricatives are consonants produced by forcing air through a narrow channel made by placing two articulators close together. These may be the lower lip against the upper teeth, in the case of [f]; the back of the tongue against the soft palate, in the case of German [x], the final consonant of Bach; or the side of the tongue against the molars, in).^[4]. Possible treatment options include speech therapy Human communication includes speech , language (phonology, morphology, syntax, semantics, pragmatics), both receptive and expressive language (including reading and writing), and non-verbal communication such as facial expression, posture and gesture. Swallowing problems managed under speech therapy are problems in the oral and pharyngeal stages, prosthetics, augmentation of the posterior pharyngeal wall, lengthening of the palate, and surgical procedures Pharyngeal flap surgery is a procedure to correct the airflow during speech. The procedure is common among people with cleft palate and some types of dysarthria.^[3]

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.^[5]

Sub-clinical manifestations

The spectrum of severity in visible CL/P 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 ^[6]. At the mild end of the spectrum, studies have described minimal or microform expressions of the CL/P phenotype, typically involving subtle defects of the lip, alveolar arch, and/or inferior nasal region to varying degrees ^[7]. Histological evidence indicates that these defects extend to the muscle fibers of the superior orbicularis oris (OO) muscle ^[8]. Martin et al. ^[9] found histological evidence of defects in the OO muscle in two 18-week fetuses with no obvious visible clefting, suggesting that the CL/P phenotype might also include occult subepithelial clefts, i.e., OO defects in the absence of any overt signs of CL/P. Subsequently, Martin et al. ^[10] used ultrasonography to visualize subepithelial OO defects in a small sample of non-cleft relatives of individuals with CL/P and healthy controls. Critically, they observed a significant increase in the frequency of OO defects in the non-cleft relatives.

Prevalence among ethnic groups

Prevalence In epidemiology, the prevalence of a disease in a statistical population is defined as the total number of cases of the disease in the population at a given time, or the total number of cases in the population, divided by the number of individuals in the population. It is used as an estimate of how common a condition is within a population over a rates reported for live births for Cleft lip with or without Cleft Palate (CL +/- P) and Cleft Palate alone (CPO) varies within different ethnic An ethnic group is a group of people whose members identify with each other, through a common heritage, consisting of a common language, a common culture (often including a shared religion) and a tradition of common ancestry (corresponding to a history of endogamy) groups.

The highest prevalence rates Rates is a Portuguese parish and town located in the municipality of Póvoa de Varzim. In the census of 2001, it had a population of 2,539 inhabitants and a total area of 13.88 square kilometres for (CL +/- P) are reported for Native Americans Native Americans in the United States are the indigenous peoples from North America now encompassed by the continental United States, including parts of Alaska and the island state of Hawaii. They comprise a large number of distinct tribes, states, and ethnic groups, many of which survive as intact political communities. The terminology used to and Asians Asian people or Asiatic people is a demonym for people from Asia. However, the use of the term varies by country and person, often referring to people from a particular region or subregion of Asia. Though it may be based on residence, it is also often considered an ethnicity or just race. Africans The African continent is home to many different ethnic groups and people of wide-ranging phenotypical traits, both indigenous and foreign to the continent. Many of these populations have diverse origins, with differing cultural, linguistic and social traits and mores. Distinctions within Africa's geography, such as the varying climates across the have the lowest prevalence rates.^[11]

• Native Americans: 3.74/1000
• Japanese The Japanese people are the predominant ethnic group of Japan. Worldwide, approximately 130 million people are of Japanese descent; of these, approximately 127 million are residents of Japan. People of Japanese ancestry who live in other countries are referred to as nikkeijin (日系人?). The term "Japanese people" may also be used in: 0.82/1000 to 3.36/1000
• Chinese Predominantly Mahayana Buddhism and Taoism. Small Christian, Muslim, Xiantian and other religious minorities. Background of Confucianism and Chinese folk religion: 1.45/1000 to 4.04/1000
• Caucasians The term Caucasian race has been used to denote the general physical type of some or all of the indigenous populations of Europe, North Africa, the Horn of Africa, West Asia, Central Asia, and South Asia. Historically, the term has been used to describe the entire population of these regions, without regard necessarily to skin tone. In common use,: 1.43/1000 to 1.86/1000
• Latin Americans Latin America is a region of the Americas where Romance languages (i.e., those derived from Latin) – particularly Spanish, Portuguese, and variably French – are primarily spoken. Latin America has an area of approximately 21,069,501 km² (7,880,000 sq mi), almost 3.9% of the Earth's surface or 14.1% of its land surface area. As of 2008, its: 1.04/1000
• Africans: 0.18/1000 to 1.67/1000

Rate of occurrence of CPO is similar for Caucasians, Africans, North American natives, Japanese and Chinese. The trait is dominant.

Prevalence of "cleft uvula The uvula is the conic projection from the posterior edge of the middle of the soft palate, composed of connective tissue containing a number of racemose glands, and some muscular fibers (musculus uvulae)" has varied from .02% to 18.8% with the highest numbers found among Chippewa The Ojibwe or Chippewa (also Chippeway) are among the largest groups of Native Americans-First Nations north of Mexico. They are the third-largest in the United States, surpassed only by Cherokee and Navajo. They are equally divided between the United States and Canada. Because they were formerly located mainly around Sault Ste. Marie, at the and Navajo The Navajo, or Diné, of the Southwestern United States are the second largest Native American tribe of Northern America. In the 2000 U.S. census, 298,197 people claimed to be fully or partly of Navajo ancestry. The Navajo Nation constitutes an independent governmental body which manages the Navajo Indian reservation in the Four Corners area of and the lowest generally in Africans.^{[citation needed]}

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:

a) one from the top of the head down towards the future upper lip; (Frontonasal Prominence)

b-c) two from the cheeks, which meet the first lobe to form the upper lip; (Maxillar Prominence)

d-e) 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 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.^[12] 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.^[13]

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.

Syndromic cases: The Van der Woude Syndrome is caused by a specific variation in the gene IRF6 that increases the occurrence of these deformities threefold.^[14][15] Another syndrome, Siderius X-linked mental retardation X-Linked mental retardation refers to forms of mental retardation which are specifically associated with the X chromosome, is caused by mutations in the PHF8 gene (OMIM The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and—when possible—links them to the relevant genes in the human genome and provides references for further research and tools for genomic analysis of a catalogued gene 300263); in addition to cleft lip and/or palate, symptoms include facial dysmorphism and mild mental retardation.^[16] 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 Myopia , is a refractive defect of the eye in which collimated light produces image focus in front of the retina when accommodation is relaxed.^{[citation needed]} Loeys-Dietz syndrome Loeys-Dietz syndrome is a recently-discovered autosomal dominant genetic syndrome which has many features similar to Marfan syndrome, but which is caused by mutations in the genes encoding transforming growth factor beta receptor 1 or 2 (TGFBR2) can cause cleft palate or bifid uvula, hypertelorism, and aortic aneurysm An aortic aneurysm is a general term for any swelling of the aorta, usually representing an underlying weakness in the wall of the aorta at that location. While the stretched vessel may occasionally cause discomfort, a greater concern is the risk of rupture, which causes severe pain; massive internal hemorrhage; and, without prompt treatment,.^{[citation needed]} Cleft lip/palate may be present in many different chromosome disorders including Patau Syndrome Patau syndrome, also known as trisomy 13 and trisomy D, is a chromosomal abnormality, a syndrome in which a patient has an additional chromosome 13 due to a nondisjunction of chromosomes during meiosis. Some are caused by Robertsonian translocations. The extra chromosome 13 disrupts the normal course of development, causing heart and kidney (trisomy 13).

Non-syndromic cases: Many genes associated with syndromic cases of cleft lip/palate (see above) have been identified to contribute to the incidence of isolated cases of cleft lip/palate. This includes in particular sequence variants in the genes IRF6, PVRL1 and MSX1.^[17] The understanding of the genetic complexities involved in the morphogenesis Morphogenesis , is the biological process that causes an organism to develop its shape. It is one of three fundamental aspects of developmental biology along with the control of cell growth and cellular differentiation. The process controls the organized spatial distribution of cells during the embryonic development of an organism. Morphogenetic of the midface, including molecular and cellular processes, has been greatly aided by research on animal models, including of the genes BMP4, SHH, SHOX2, FGF10 and MSX1.^[17]

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.^{[citation needed]} Many clefts run in families, even though in some cases there does not seem to be an identifiable syndrome present,^{[citation needed]} possibly because of the current incomplete genetic understanding of midfacial development.

Environmental influences may also cause, or interact with genetics to produce, orofacial clefting. An example for how environmental factors might be linked to genetics comes from research on mutations in the gene PHF8 that cause cleft lip/palate (see above). It was found that PHF8 encodes for a histone In biology, histones are strongly alkaline proteins found in eukaryotic cell nuclei, which package and order the DNA into structural units called nucleosomes. They are the chief protein components of chromatin, act as spools around which DNA winds, and play a role in gene regulation. Without histones, the unwound DNA in chromosomes would be very lysine demethylase,^[18] and is involved in epigenetic regulation In biology, the term epigenetics refers to changes in phenotype or gene expression caused by mechanisms other than changes in the underlying DNA sequence, hence the name epi- (Greek: επί- over, above) -genetics. These changes may remain through cell divisions for the remainder of the cell's life and may also last for multiple generations. The catalytic activity of PHF8 depends on molecular oxygen Oxygen (pronounced /ˈɒksɨdʒɨn/, OK-si-jin, from the Greek roots ὀξύς (acid, literally "sharp", from the taste of acids) and -γενής (-genēs) (producer, literally begetter), is the element with atomic number 8 and represented by the symbol O. It is a member of the chalcogen group on the periodic table, and is a highly,^[18] a fact considered important with respect to reports on increased incidence of cleft lip/palate in mice that have been exposed to hypoxia Hypoxia is a pathological condition in which the body as a whole or a region of the body (tissue hypoxia) is deprived of adequate oxygen supply. Variations in arterial oxygen concentrations can be part of the normal physiology, for example, during strenuous physical exercise. A mismatch between oxygen supply and its demand at the cellular level early during pregnancy Pregnancy is the carrying of one or more offspring, known as a fetus or embryo, inside the womb of a female. In a pregnancy, there can be multiple gestations, as in the case of twins or triplets. Human pregnancy is the most studied of all mammalian pregnancies. Childbirth usually occurs about 38 weeks after conception; i.e., approximately 40 weeks.^[19] In humans, fetal A fetus is a developing mammal or other viviparous vertebrate after the embryonic stage and before birth cleft lip and other congenital abnormalities have also been linked to maternal hypoxia, as caused by e.g. maternal smoking,^[20] maternal alcohol abuse Alcohol abuse, as described in the DSM-IV, is a psychiatric diagnosis describing the recurring use of alcoholic beverages despite negative consequences. It is differentiated from alcohol dependence by the lack of symptoms such as tolerance and withdrawal. Alcohol abuse is sometimes referred to by the less specific term alcoholism. However, many or some forms of maternal hypertension Hypertension or high blood pressure is a chronic medical condition in which the blood pressure in the arteries is elevated. It is the opposite of hypotension. It is classified as either primary (essential) or secondary. About 90-95% of cases are termed "primary hypertension", which refers to high blood pressure for which no medical cause treatment.^[21] 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 The anticonvulsants are a diverse group of pharmaceuticals used in the treatment of epileptic seizures. Anticonvulsants are also increasingly being used in the treatment of bipolar disorder, since many seem to act as mood stabilizers. The goal of an anticonvulsant is to suppress the rapid and excessive firing of neurons that start a seizure 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.

Diagnosis

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.^[22]

Treatment

Cleft lip and palate is very treatable; however, the kind of treatment depends on the type and severity of the cleft.

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.

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. The most common procedure to repair a cleft lip is the Millard procedure pioneered by Ralph Millard. Millard performed the first procedure at a Mobile Army Surgical Hospital (MASH) unit in Korea.^[23]

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.

Pre-surgical devices for cleft lip treatment

In some cases of a severe bi-lateral 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, according to a retrospective cohort study.^[24] Significant improvements in nasal symmetry were observed in the measurements of the projected length of the nasal ala, position of the superoinferior alar groove, position of the mediolateral nasal dome, and nasal bridge deviation. "The nasal ala projection length demonstrated an average ratio of 93.0 percent in the surgery-alone group and 96.5 percent in the nasoalveolar molding group" this study concluded.

Cleft palate treatment

Often a cleft palate is temporarily closed, the cleft isn't closed, but it is covered by a palatal obturator (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.

Speech and hearing treatments

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.

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.

Sample treatment schedule

Note that each individual patient's schedule is treated on a case-by-case basis and can vary per hospital. The table below shows a common sample treatment schedule. The colored squares indicate the average timeframe in which the indicated procedure occurs. In some cases this is usually one procedure (for example lip repair) in other cases this is an ongoing therapy (for example speech therapy).

Craniofacial team

A craniofacial team is routinely used to treat this condition. The majority of hospitals still use craniofacial teams; yet others are making a shift towards dedicated cleft lip and palate programs. While craniofacial teams are widely knowledgeable about all aspects of craniofacial conditions, dedicated cleft lip and palate teams are able to dedicate many of their efforts to being on the cutting edge of new advances in cleft lip and palate care.

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. .^[25]

The members of the craniofacial team at a minimum include a plastic or facial plastic surgeon, or an Oral and maxillofacial surgeon trained in craniofacial surgery, otolaryngologist, geneticist, orthodontist, and social worker.

A complete listing of craniofacial teams in the United States is available through the Cleft Palate Foundation

Complications

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 rhythmically squeezing the bottle insert 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.

Speech is both receptive and expressive. We hear and understand spoken language (receptive) We learn to manipulate our mouth, tongue, oral cavity, to express ourselves (expressive).

Hearing is related to learning to speak. 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.

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.

Psychosocial issues

Having a cleft palate/lip 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 palate/lip 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 palate/lip 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 lip and or cleft palate. Research has shown that during the early preschool years (ages 3–5), children with cleft lip and or 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 lip and or cleft palate.^[26] 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.^[27] Strong parent support networks may help to prevent the development of negative self-concept in children with cleft palate^{[citation needed]}. 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 lip and or 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 lip and or 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.^[28]

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 lip and or cleft palate will deal with the typical challenges faced by most of their peers including issues related to self esteem, dating, and social acceptance.^[29][30][31] Adolescents, however, view appearance as the most important characteristic above intelligence and humor.^[32] 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.^[31] 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. Individuals with cleft lip and or cleft palate often have lower QOL scores than their peers^{[citation needed]}. 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^{[citation needed]}.

Controversy

In some countries, cleft lip or palate deformities are considered reasons (either generally tolerated or officially sanctioned) to perform abortion beyond the legal fetal age limit, even though the fetus is not in jeopardy of life or limb. Some human rights activists contend this practice of "cosmetic murder" amounts to eugenics. British clergywoman Joanna Jepson, who suffered from a congenital jaw deformity herself (not a cleft lip or palate as is sometimes reported), has started legal action to stop the practice in the United Kingdom^[33][34] (although in the United Kingdom, such an abortion would not be permitted under the 1967 Abortion Act, because a cleft lip and palate is not considered a serious handicap).

The Japanese anime Ghost Stories arose some controversy when people complained of an episode featuring a Kuchisake-onna (a ghost who is said to have a Glasgow smile through her face) because her scar resembled cleft lip.^[35]

Famous people born with a cleft

Cleft lip and palate in animals

Cleft lips and palates are occasionally seen in cattle and dogs, and rarely in sheep, cats, horses, pandas and ferrets. Most commonly, the defect involves the lip, rhinarium, and premaxilla. Clefts of the hard and soft palate are sometimes seen with a cleft lip. The cause is usually hereditary. Brachycephalic dogs such as Boxers and Boston Terriers are most commonly affected.^[47] An inherited disorder with incomplete penetrance has also been suggested in Shih tzus, Swiss Sheepdogs, Bulldogs, and Pointers.^[48] In horses, it is a rare condition usually involving the caudal soft palate.^[49] In Charolais cattle, clefts are seen in combination with arthrogryposis, which is inherited as an autosomal recessive trait. It is also inherited as an autosomal recessive trait in Texel sheep. Other contributing factors may include maternal nutritional deficiencies, exposure in utero to viral infections, trauma, drugs, or chemicals, or ingestion of toxins by the mother, such as certain lupines by cattle during the second or third month of gestation.^[50] The use of corticosteroids during pregnancy in dogs and the ingestion of Veratrum californicum by pregnant sheep have also been associated with cleft formation.^[51]

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.^[47] For cleft palate, there is a high rate of surgical failure resulting in repeated surgeries.^[52] Surgical techniques for cleft palate in dogs include prosthesis, mucosal flaps, and microvascular free flaps.^[53] Affected animals should not be bred due to the hereditary nature of this condition.

See also

Associated genes

• GAD1,^[54] one of the glutamate decarboxylases

Movies showing on this topic

• Smile Pinki

Treatment and aids

• Oral and maxillofacial surgery
• Haberman Feeder
• Palatal obturator
• Vomer flap surgery

Syndromes

• Hearing loss with craniofacial syndromes
• Popliteal pterygium syndrome
• Van der Woude syndrome

General support discussion boards

• Face Forward - The UK's Number One Discussion Boards , For Both Patients And Parents
• cleftAdvocate's Family-to-Family Connection Listserv

Organizations

• List of Cleft lip & palate organizations

References

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2. ^ "Statistics by country for cleft palate". WrongDiagnosis.com. http://www.wrongdiagnosis.com/c/cleft_palate/stats-country.htm. Retrieved 2007-04-24.
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4. ^ Hill, J.S. (2001). Velopharyngeal insufficiency: An update on diagnostic and surgical techniques. Current Opinion in Otolaryngology and Head and Neck Surgery, 9, 365-368.
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11. ^ See "Who is affected by cleft lip and cleft palate". http://www.webmd.com/hw-popup/who-is-affected-by-cleft-lip-and-cleft-palate. Retrieved 2008-06-20.
12. ^ Dudas et al. (2007): Palatal fusion – Where do the midline cells go? A review on cleft palate, a major human birth defect. Acta Histochemica, Volume 109, Issue 1, 1 March 2007, Pages 1-14
13. ^ Dudas M, Li WY, Kim J, Yang A, Kaartinen V (2007). "Palatal fusion - where do the midline cells go? A review on cleft palate, a major human birth defect". Acta Histochem. 109 (1): 1–14. doi:10.1016/j.acthis.2006.05.009. PMID 16962647.
14. ^ Zucchero, T.M. et al. 2004 Interferon Regulatory Factor 6 (IRF6) Gene Variants and the Risk of Isolated Cleft Lip or Palate New England Journal of Medicine 351:769-780 [1]
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18. ^ ^{a b} Loenarz, C.; Ge W., Coleman M. L., Rose N. R., Cooper C. D. O., Klose R. J., Ratcliffe P. J., Schofield, C. J. (2009). "PHF8, a gene associated with cleft lip/palate and mental retardation, encodes for an N{varepsilon}-dimethyl lysine demethylase". Hum. Mol. Genet.. doi:10.1093/hmg/ddp480. PMID 19843542.
19. ^ Millicovsky, G.; Johnston, M.C. (1981). "Hyperoxia and hypoxia in pregnancy: simple experimental manipulation alters the incidence of cleft lip and palate in CL/Fr mice". Proc. Natl. Acad. Sci. U. S. A. 78 (9): 5722–5723. doi:10.1073/pnas.78.9.5722. PMID 6946511.
20. ^ Shi, M.; Wehby, G.L. and Murray, J.C. (2008). "Review on genetic variants and maternal smoking in the etiology of oral clefts and other birth defects". Birth Defects Res., Part C 84 (1): 16–29. doi:10.1002/bdrc.20117. PMID 18383123.
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22. ^ PMID 18848093
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24. ^ PMID 19319066
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26. ^ Tobiasen, J.M. (1984) Psychosocial correlated of congenital facial clefts: a conceptualization and model. Cleft Palate Journal, 21, 131-139.
27. ^ Pope AW, Ward J (1997). "Self-perceived facial appearance and psychosocial adjustment in preadolescents with craniofacial anomalies". Cleft Palate Craniofac. J. 34 (5): 396–401. doi:10.1597/1545-1569(1997)034<0396:SPFAAP>2.3.CO;2. PMID 9345606.
28. ^ "Cleft Palate Foundation". http://cleftline.org. Retrieved 2007-07-01.
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30. ^ Endriga MC, Kapp-Simon KA (1999). "Psychological issues in craniofacial care: state of the art". Cleft Palate Craniofac. J. 36 (1): 3–11. doi:10.1597/1545-1569(1999)036<0001:PIICCS>2.3.CO;2. PMID 10067755.
31. ^ ^{a b} Pope, A.W. & Snyder, H.T. (2004). Psychosocial adjustment in children and adolescents with a craniofacial anomaly: Age and sex patterns. The Cleft Palate-Craniofacial Journal, 42, 4.
32. ^ Prokohorov, A.V., Perry, C.L., Kelder, S.H., & Klepp, K.I. (1993). Lifestyle values of adolescents: Results from the Minnesota Heart Health Youth Program. Adolescence, 28.
33. ^ "CNN.com - Priest challenges late abortion - Dec. 1, 2003". 2003-12-01. http://www.cnn.com/2003/WORLD/europe/12/01/uk.curate.abortion/index.html. Retrieved 2007-07-01.
34. ^ "CPS examines late abortion case". BBC News. 2004-09-22. http://news.bbc.co.uk/1/hi/england/hereford/worcs/3680162.stm. Retrieved 2007-07-01.
35. ^ [2]
36. ^ "Doc Holliday: A Family Portrait", Karen Holliday Tanner, University of Omaha Press, 1998, ISBN 0-8061-3036-9.
37. ^ "King Tut Not Murdered Violently, CT Scans Show". http://news.nationalgeographic.com/news/2005/03/0308_050308_kingtutmurder_2.html. Retrieved 2007-07-01.
38. ^ "HistoryBuff.com -- Tad Lincoln: The Not-so-Famous Son of A Most-Famous President". http://www.historybuff.com/library/reftad.html. Retrieved 2007-07-01.
39. ^ "Carmit Bachar, smile ambassador". http://www.buddytv.com/articles/pussycat-dolls-present-the-search-for-the-next-doll/pussycat-dolls-carmit-bachar-i-12393.aspx. Retrieved 2007-10-13.
40. ^ Beverley Lyons, October 16, 2006. Carmite Doing Her Bit For Charity. The Daily Record
41. ^ "Jurgen Habermas". http://www.nyu.edu/classes/stephens/Habermas%20page.htm. Retrieved 2008-12-18.
42. ^ "Chat To Ljubo...LIVE". 28 May 2009. http://au.fourfourtwo.com/news/104299,chat-to-ljubolive.aspx. Retrieved 23 December 2009.
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46. ^ Whiteside, Kelly (4 Nov 2006). "Schmitt is face of West Va. toughness". http://www.usatoday.com/sports/college/football/bigeast/2006-11-01-wvu-schmitt_x.htm. Retrieved 2010-04-30.
47. ^ ^{a b} Ettinger, Stephen J.;Feldman, Edward C. (1995). Textbook of Veterinary Internal Medicine (4th ed.). W.B. Saunders Company. ISBN 0-7216-6795-3.
48. ^ Garcia, J.F. Rodriguez (2006). "Surgery of the Soft and Hard Palate". Proceedings of the North American Veterinary Conference. http://www.ivis.org/proceedings/navc/2006/SAE/516.asp?LA=1. Retrieved 2007-04-28.
49. ^ Semevolos, Stacy A.; Ducharme, Norm (1998). "Surgical Repair of Congenital Cleft Palate in Horses: Eight Cases (1979–1997)" (PDF). Proceedings of the American Association of Equine Practitioners. http://www.ivis.org/proceedings/AAEP/1998/Semevolo.pdf. Retrieved 2007-04-28.
50. ^ "Mouth". The Merck Veterinary Manual. 2006. http://www.merckvetmanual.com/mvm/index.jsp?cfile=htm/bc/20202.htm. Retrieved 2007-04-28.
51. ^ Beasley, V. (1999). "Teratogenic Agents". Veterinary Toxicology. http://www.ivis.org/advances/Beasley/cpt8a/chapter_frm.asp?LA=1&table=1. Retrieved 2007-04-28.
52. ^ Lee J, Kim Y, Kim M, Lee J, Choi J, Yeom D, Park J, Hong S (2006). "Application of a temporary palatal prosthesis in a puppy suffering from cleft palate". J. Vet. Sci. 7 (1): 93–5. PMID 16434860.
53. ^ Griffiths L, Sullivan M (2001). "Bilateral overlapping mucosal single-pedicle flaps for correction of soft palate defects". Journal of the American Animal Hospital Association 37 (2): 183–6. PMID 11300527.
54. ^ Kanno, K.; Suzuki, Y.; Yamada, A.; Aoki, Y.; Kure, S.; Matsubara, Y. : Association between nonsyndromic cleft lip with or without cleft palate and the glutamic acid decarboxylase 67 gene in the Japanese population. Am. J. Med. Genet. 127A: 11-16, 2004. PMID 15103710

External links

• Cleft lip and palate - Institute of Reconstructive Plastic Surgery @ NYU Langone Medical Center
• Cleft lip and palate - Children's Hospital Boston
• Cleft lip and palate and before/after patient photos - University of Missouri Children's Hospital

Categories: Congenital disorders of digestive system | Facial features | Dog health | Oral and maxillofacial surgery

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