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Cleft lip and cleft palate
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==Signs and symptoms== === Cleft lip === 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 [[Frontonasal process#Nasal processes|medial nasal processes]] (formation of the primary palate). <gallery class="center" widths="150px" heights="150px"> File:CleftLip1.svg|Unilateral incomplete File:Cleftlip2.svg|Unilateral complete File:CleftLip3.png|Bilateral complete </gallery> A mild form of a cleft lip is a microform cleft.<ref>{{cite journal | vauthors = Kim EK, Khang SK, Lee TJ, Kim TG | title = Clinical features of the microform cleft lip and the ultrastructural characteristics of the orbicularis oris muscle | journal = The Cleft Palate-Craniofacial Journal | volume = 47 | issue = 3 | pages = 297โ302 | date = May 2010 | pmid = 19860522 | doi = 10.1597/08-270.1 | s2cid = 71448247 }}{{dead link|date=September 2017 |bot=Level C |fix-attempted=yes }}</ref> 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.<ref>{{cite journal | vauthors = Yuzuriha S, Mulliken JB | title = Minor-form, microform, and mini-microform cleft lip: anatomical features, operative techniques, and revisions | journal = Plastic and Reconstructive Surgery | volume = 122 | issue = 5 | pages = 1485โ1493 | date = November 2008 | pmid = 18971733 | doi = 10.1097/PRS.0b013e31818820bc | s2cid = 8551875 }}</ref> In some cases [[Orbicularis oris muscle|muscle tissue in the lip]] underneath the scar is affected and might require reconstructive surgery.<ref>{{cite journal | vauthors = Tosun Z, Hoลnuter M, Sentรผrk S, Savaci N | title = Reconstruction of microform cleft lip | journal = Scandinavian Journal of Plastic and Reconstructive Surgery and Hand Surgery | volume = 37 | issue = 4 | pages = 232โ235 | year = 2003 | pmid = 14582757 | doi = 10.1080/02844310310016412 | s2cid = 40850672 }}</ref> 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.<ref>{{cite journal | vauthors = Tollefson TT, Humphrey CD, Larrabee WF, Adelson RT, Karimi K, Kriet JD | title = The spectrum of isolated congenital nasal deformities resembling the cleft lip nasal morphology | journal = Archives of Facial Plastic Surgery | volume = 13 | issue = 3 | pages = 152โ160 | year = 2011 | pmid = 21576661 | doi = 10.1001/archfacial.2011.26 | doi-access = free }}</ref> <gallery class="center" widths="150" heights="150"> File:13900470 3PREOPERATION0.jpg|Six-month-old girl before going into surgery to have her unilateral complete cleft lip repaired File:10-month-old girl showing scar from facial reconstruction surgery for cleft lip.jpg|The same girl, one month after the surgery File:8-year-old girl showing scar from infantile facial reconstruction surgery.jpg|The same girl, age eight, the scar almost gone </gallery> ===Cleft palate=== Cleft palate is a condition in which the two plates of the [[human skull|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 [[Palatine uvula|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 [[nasal cavity|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. <gallery class="center" widths="150px" heights="150px"> File:Cleftpalate3.png|Incomplete cleft palate File:Cleftpalate1.png|Unilateral complete lip and palate File:Cleftpalate2.png|Bilateral complete lip and palate </gallery> A result of an open connection between the [[oral cavity|mouth]] and inside the nose is called [[velopharyngeal insufficiency]] (VPI). Because of the gap, air leaks into the nasal cavity resulting in a [[Hypernasal speech|hypernasal]] voice [[resonance]] and nasal emissions while talking.<ref name="Sloan">{{cite journal | vauthors = Sloan GM | title = Posterior pharyngeal flap and sphincter pharyngoplasty: the state of the art | journal = The Cleft Palate-Craniofacial Journal | volume = 37 | issue = 2 | pages = 112โ122 | date = March 2000 | pmid = 10749049 | doi = 10.1597/1545-1569(2000)037<0112:PPFASP>2.3.CO;2 }}</ref> Secondary effects of VPI include speech [[Manner of articulation|articulation]] errors (e.g., [[distortions]], substitutions, and omissions) and compensatory misarticulations and mispronunciations (e.g., [[glottal stop]]s and posterior nasal [[fricative]]s).<ref>{{cite journal |author=Hill JS |title=Velopharyngeal insufficiency: An update on diagnostic and surgical techniques |journal=Current Opinion in Otolaryngology & Head and Neck Surgery |volume=9 |issue=6 |pages=365โ8 |year=2001 |doi=10.1097/00020840-200112000-00005 |s2cid=76256148 }}</ref> Possible treatment options include [[speech therapy]], prosthetics, augmentation of the posterior pharyngeal wall, lengthening of the palate, and [[Pharyngeal flap surgery|surgical procedures]].<ref name="Sloan" /> 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.<ref name="Kaplan">{{cite journal | vauthors = Kaplan EN | title = The occult submucous cleft palate | journal = The Cleft Palate Journal | volume = 12 | pages = 356โ368 | date = October 1975 | pmid = 1058746 }}</ref> The diagnosis of submucous cleft palate often occurs late in children as a result of the nature of the cleft.<ref>{{cite journal | vauthors = Hanny KH, de Vries IA, Haverkamp SJ, Oomen KP, Penris WM, Eijkemans MJ, Kon M, Mink van der Molen AB, Breugem CC | title = Late detection of cleft palate | journal = European Journal of Pediatrics | volume = 175 | issue = 1 | pages = 71โ80 | date = January 2016 | pmid = 26231683 | pmc = 4709386 | doi = 10.1007/s00431-015-2590-9 }}</ref> While the muscles of the soft palate are not joined, the mucosal membranes covering the roof of the mouth appear relatively normal and intact.<ref>{{cite web |title=Cleft Lip and Palate |url=https://www.asha.org/PRPSpecificTopic.aspx?folderid=8589942918§ion=Overview |website=American-Speech-Language-Hearing Association |access-date=May 9, 2019}}</ref> === Teeth === Tooth development can be delayed with increasing severity of CLP. Some of the dental problems affect the [[Deciduous teeth|primary teeth]], but most of the problems arise after the permanent teeth erupt. 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 [[Tooth enamel|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.<ref>{{Citation|chapter=Index|date=August 23, 2013|pages=355โ370|publisher=John Wiley & Sons, Ltd,.|isbn=978-1-118-78504-1|doi=10.1002/9781118785041.index|title=Orthodontics: Principles and Practice|doi-access=free}}</ref> In addition, abnormal positioning of individual teeth may affect occlusion, which can create an open bite or cross bite. This in turn can then affect the patient's speech.<ref name=":5">{{Cite book| vauthors = Goel S |title=Feeding in Cleft Lip And Cleft Palata Infants|publisher=LAP LAMBERT Academic Publishing|year=2015|isbn=978-3-659-38212-3|location=Saarbrรผcken, Germany|pages=20โ22}}</ref><ref name=":6">{{Cite book| vauthors = Peterson-Falzone SJ, Trost-Cardamone JE, Karnell MP, Hardin-Jones MA |title=The clinician's guide to treating cleft palate speech|isbn=978-0-323-33934-6|edition=Second|location=St. Louis, Missouri | publisher = Elsevier |oclc=936145822|date=September 21, 2016}}</ref> ===Complications=== [[File:Cleft Nurser Mead Johnson.jpg|thumb|A baby being fed using a customized bottle. The upright sitting position allows [[Earth's gravity|gravity]] to help the baby swallow the milk more easily.]] Cleft may cause problems with feeding, ear disease, speech, socialization, and cognition. 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, as gravity will help prevent milk from coming through the baby's nose. 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 tube]]s 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.<ref>{{cite journal | vauthors = Roberts RM, Mathias JL, Wheaton P | title = Cognitive functioning in children and adults with nonsyndromal cleft lip and/or palate: a meta-analysis | journal = Journal of Pediatric Psychology | volume = 37 | issue = 7 | pages = 786โ797 | date = August 2012 | pmid = 22451260 | doi = 10.1093/jpepsy/jss052 | doi-access = free }}</ref> ===Psychosocial issues=== There is research dedicated to the [[psychosocial development]] of individuals with cleft palate. A cleft palate/lip may impact an individual's [[self-esteem]], [[social skills]] and [[behavior]]. Self-concept may be adversely affected by the presence of a cleft lip or cleft palate, particularly among girls.<ref>{{cite journal | vauthors = Leonard BJ, Brust JD, Abrahams G, Sielaff B | title = Self-concept of children and adolescents with cleft lip and/or palate | journal = The Cleft Palate-Craniofacial Journal | volume = 28 | issue = 4 | pages = 347โ353 | date = October 1991 | pmid = 1742302 | doi = 10.1597/1545-1569(1991)028<0347:SCOCAA>2.3.CO;2 }}</ref> Negative outcomes can also be associated with the long durations of hospitalization. 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.<ref>{{cite journal | vauthors = Al-Namankany A, Alhubaishi A | title = Effects of cleft lip and palate on children's psychological health: A systematic review | journal = Journal of Taibah University Medical Sciences | volume = 13 | issue = 4 | pages = 311โ318 | date = August 2018 | pmid = 31435341 | pmc = 6694901 | doi = 10.1016/j.jtumed.2018.04.007 }}</ref><ref>{{cite journal | vauthors = Hunt O, Burden D, Hepper P, Johnston C | title = The psychosocial effects of cleft lip and palate: a systematic review | journal = European Journal of Orthodontics | volume = 27 | issue = 3 | pages = 274โ285 | date = June 2005 | pmid = 15947228 | doi = 10.1093/ejo/cji004 | doi-access = free }}</ref> Research has shown that during the early preschool years (ages 3โ5), 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 impediment]]s. 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.<ref>{{cite journal | vauthors = Tobiasen JM | title = Psychosocial correlates of congenital facial clefts: a conceptualization and model | journal = The Cleft Palate Journal | volume = 21 | issue = 3 | pages = 131โ139 | date = July 1984 | pmid = 6592056 }}</ref> Children with clefts tend to report feelings of anger, sadness, fear, and alienation from their peers, but 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.<ref>{{cite journal | vauthors = Pope AW, Ward J | title = Self-perceived facial appearance and psychosocial adjustment in preadolescents with craniofacial anomalies | journal = The Cleft Palate-Craniofacial Journal | volume = 34 | issue = 5 | pages = 396โ401 | date = September 1997 | pmid = 9345606 | doi = 10.1597/1545-1569(1997)034<0396:SPFAAP>2.3.CO;2 }}</ref> Strong parent support networks may help to prevent the development of negative self-concept in children with cleft palate.<ref>{{harvnb|Bristow|Bristow|2007|pp=82โ92}}</ref> 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 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.<ref>{{cite web|title=Cleft Palate Foundation|url=http://cleftline.org|url-status=live|archive-url=https://web.archive.org/web/20070701095458/http://www.cleftline.org/|archive-date=July 1, 2007|access-date=July 1, 2007}}</ref> 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.<ref>{{cite journal | vauthors = Snyder HT, Bilboul MJ, Pope AW | title = Psychosocial adjustment in adolescents with craniofacial anomalies: a comparison of parent and self-reports | journal = The Cleft Palate-Craniofacial Journal | volume = 42 | issue = 5 | pages = 548โ555 | date = September 2005 | pmid = 16149838 | doi = 10.1597/04-078R.1 | s2cid = 37357550 | citeseerx = 10.1.1.624.1274 }}</ref><ref>{{cite journal | vauthors = Endriga MC, Kapp-Simon KA | title = Psychological issues in craniofacial care: state of the art | journal = The Cleft Palate-Craniofacial Journal | volume = 36 | issue = 1 | pages = 3โ11 | date = January 1999 | pmid = 10067755 | doi = 10.1597/1545-1569(1999)036<0001:PIICCS>2.3.CO;2 }}</ref><ref name="PopeSynder">{{cite journal | vauthors = Pope AW, Snyder HT | title = Psychosocial adjustment in children and adolescents with a craniofacial anomaly: age and sex patterns | journal = The Cleft Palate-Craniofacial Journal | volume = 42 | issue = 4 | pages = 349โ354 | date = July 2005 | pmid = 16001914 | doi = 10.1597/04-043R.1 | s2cid = 31313562 }}</ref> Adolescents, however, view appearance as the most important characteristic, above intelligence and humor.<ref>{{cite journal | vauthors = Prokhorov AV, Perry CL, Kelder SH, Klepp KI | title = Lifestyle values of adolescents: results from Minnesota Heart Health Youth Program | journal = Adolescence | volume = 28 | issue = 111 | pages = 637โ647 | year = 1993 | pmid = 8237549 }}</ref> 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 [[Internalization (psychology)|internalizing]] problems, and may possibly develop anxiousness-depression and aggressive behaviors.<ref name="PopeSynder" /> Adolescent girls are more likely to develop problems relating to self-concept and appearance. 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.
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