Unilateral cleft lip and palate pdf
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Cleft lip and cleft palate
Cleft lip and cleft palate , also known as orofacial cleft , is a group of conditions that includes cleft lip, cleft palate, and both together.
Cleft lip and palate are the result of tissues of the face not joining properly during development. A cleft lip or palate can be successfully treated with surgery. Cleft lip and palate occurs in about 1 to 2 per births in the developed world. 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 condition. It is due to the failure of fusion of the maxillary prominence and medial nasal processes formation of the primary palate.
A mild form of a cleft lip is a microform cleft. 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 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.
It occurs due to the failure of fusion of the lateral palatine processes, the nasal septum, 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 inside of the nose.
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 result of an open connection between the mouth and inside the nose is called velopharyngeal insufficiency VPI. Because of the gap, air leaks into the nasal cavity resulting in a hypernasal voice resonance and nasal emissions while talking.
Submucous cleft palate can also occur, which is a cleft of the soft palate with a split uvula , a furrow along the midline of the soft palate, and a notch in the back margin of the hard palate. Tooth development can be delayed with increasing severity of CLP.
Some of the dental problems affect the primary teeth , but most of the problems arise after the permanent teeth erupts. Problems may include fused teeth, missing teeth, and extra teeth erupting behind normal teeth. Missing teeth or extra teeth are both normal occurrences. Typically, the lateral incisors are missing. The enamel outermost layer of the tooth is commonly found to be hypomineralized and hypoplastic, making the teeth more likely to decay. As CLP can make oral hygiene more difficult, there is an increased rate of cavities.
This in turn can then affect the patient's speech. 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 feeding can be accomplished by using specialized equipment, such as the Haberman Feeder. Another equipment commonly used for gravity feeding is a customized bottle with a combination of nipples and bottle inserts.
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 may eventually lead to 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.
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. Tentative evidence has found that those with clefts perform less well at language.
There is research dedicated to the psychosocial development of individuals with cleft palate. Self-concept may be adversely affected by the presence of a cleft lip or cleft palate, particularly among girls.
Psychological issues could extend not just to the individual with CLP but also to their families, particularly their mothers, that experience varying levels of depression and anxiety.
Research has shown that during the early preschool years ages 3—5 , children with cleft lip 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 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 possible speech impediments. 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 or cleft palate. 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. A cleft lip 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 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. 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 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.
Adolescent boys typically deal with issues relating to withdrawal, attention, thought, and internalizing problems, and may possibly develop anxiousness-depression and aggressive behaviors.
Individuals with cleft lip 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. Most clefts are polygenic and multifactorial in origin with many genetic and environmental factors contributing.
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:. 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. 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. 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. Orofacial clefts may be associated with a syndrome syndromic or may not be associated with a syndrome nonsyndromic.
Syndromic clefts are part of syndromes that are caused by a variety of factors such as environment and genetics or an unknown cause. Nonsyndromic clefts, which are not as common as syndromic clefts, also have a genetic cause. Genetic factors contributing to cleft lip and cleft palate formation have been identified for some syndromic cases.
Many clefts run in families, even though in some cases there does not seem to be an identifiable syndrome present. Environmental influences may also cause, or interact with genetics to produce, orofacial clefts. An example of the link between environmental factors and genetics comes from a research on mutations in the gene PHF8.
The research found that PHF8 encodes for a histone lysine demethylase,  and is involved in epigenetic regulation. Cleft lip and other congenital abnormalities have also been linked to maternal hypoxia caused by maternal smoking ,  with the estimated attributable fraction of orofacial clefts due to smoking in early pregnancy being 6.
Orofacial clefts occur very early in pregnancy and so smoking cessation right after recognition of pregnancy is unlikely to reduce the exposure during the critical time period.
Maternal alcohol abuse has also been linked to cleft lip and palate due to the effects on the cranial neural crest cells. The degree of the effect, however, is unknown and requires further research  and some forms of maternal hypertension treatment has been linked to cleft lip and palate.
Current research continues to investigate the extent to which folic acid can reduce the incidence of clefting. 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 with ultrasonography. Clefts can also affect other parts of the face, such as the eyes, ears, nose, cheeks, and forehead. In , 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.
Cleft lip and cleft palate is an "umbrella term" for a collection of orofacial clefts. It includes clefting of the upper lip, the maxillary alveolus dental arch , and the hard or soft palate , in various combinations.
Proposed anatomic combinations include: . Cleft lip with or without palate is classified as the most common congenital birth defect. It has been noted that the prevalence of orofacial clefts varies by race. The highest number of cases have been recorded among Asians and Native Americans, followed by Europeans, Hispanics and African-Americans.
The critical period for cleft development ranges from the 4th to the 12th week of intrauterine life. Clefts of the primary palate develop between the 4th and 7th weeks of intrauterine life, while clefts of the secondary palate develop between the 8th and 12th embryonic weeks. Accurate evaluation of craniofacial malformations is usually possible with the ultrasound scan performed during pregnancy. This is however not a routine procedure according to the American Institute of Ultrasound in Medicine.
Prenatal diagnosis enables appropriate and timely education and discussion with parents by the cleft team.
Cleft Lip and Palate
Click here to view a larger image. The lip forms between the fourth and seventh weeks of pregnancy. As a baby develops during pregnancy, body tissue and special cells from each side of the head grow toward the center of the face and join together to make the face. This joining of tissue forms the facial features, like the lips and mouth. A cleft lip happens if the tissue that makes up the lip does not join completely before birth. This results in an opening in the upper lip.
Cleft Lip and Palate
Table of contents
Orofacial clefts comprise a range of congenital deformities and are the most common head and neck congenital malformation. Clefting has significant psychological and socio- economic effects on patient quality of life and require a multidisciplinary team approach for management. The complex interplay between genetic and environmental factors play a significant role in the incidence and cause of clefting. In this review, the embryology, classification, epidemiology, and etiology of cleft lip are discussed. The primary goals of surgical repair are to restore normal function, speech development, and facial esthetics. Different techniques are employed based on surgeon expertise and the unique patient presentations.
Skip to content. A left unilateral complete cleft lip is repaired with the Fisher technique, which employs anatomical subunit principles to guide repair. This tutorial for medical professionals was developed to supplement learning of a common surgical technique and is not intended to replace formal surgical training. This slideshow is primarily intended for use on tablets or larger screens. Some detail might be lost on mobile screens. Founded in as part of our internationally recognized Facial Reconstruction Center, our Cleft Lip and Palate Program is one of the largest in the nation. In this video, clinicians and patient families talk about the diagnosis and treatment of cleft lip and palate, the most common congenital facial anomaly.
Cleft lip or palate is one of the most common types of craniomaxillofacial birth anomalies. Midface deficiency is a common feature of cleft lip and palate patients due to scar tissue of the lip and palate closure. Cleft lip and palate patients should be carefully evaluated by the craniofacial team in order to detect potentially serious deformities. Craniofacial team is involved with diagnosis of facial morphology, feeding problems, guidance of the growth and development of the face, occlusion, dentition, hearing and speech problems, and psychosocial issues and jaw discrepancy of the patients with cleft lip and palate or craniofacial syndromes. Treatment for cleft children requires a multidisciplinary approach including facial surgery in the first months of life, preventive and interceptive treatment in primary dentition, speech therapy, orthodontics in the mixed dentition phase, oromaxillofacial surgery, and implant and prosthetics in adults. Treatment plan from orthodontic perspective can be divided into the following stages based on the dentition stages: 1 presurgical orthopedics, 2 primary dentition, 3 mixed dentition, and 4 permanent dentition. Cleft lip or palate is one of the most common types of cranio-maxillofacial birth anomalies.
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