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Cleft lip and cleft palate
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==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.<ref>{{cite book | vauthors = Bristow L, Bristow S |title=Making faces: Logan's cleft lip and palate story |publisher=Pulsus Group |location=Oakville, Ontaria, CA |year=2007 |pages=1β92 }}</ref> Care can be lifelong and are looked after by craniofacial cleft teams often consist of: cleft surgeons, [[Orthodontics|orthodontists]], speech and language therapists, [[Restorative dentistry|restorative dentists]], [[psychologist]]s, ENT surgeons and audio-logical physicians.<ref name="Gill_2011" />{{rp|255}} 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=== Within the first 2β3 months after birth, surgery is performed to close the cleft lip. While surgery to repair a cleft lip can be performed soon after birth, often the preferred age is at approximately 10 weeks of age, following the "[[Millard criteria|rule of 10s]]" coined by surgeons Wilhelmmesen and Musgrave in 1969 (the child is at least 10 weeks of age; weighs at least {{convert | 10 | lb | kg}}, and has at least 10g/dL hemoglobin).<ref>{{cite journal | vauthors = Lydiatt DD, Yonkers AJ, Schall DG | title = The management of the cleft lip and palate patient | journal = The Nebraska Medical Journal | volume = 74 | issue = 11 | pages = 325β8; discussion 328β9 | date = November 1989 | pmid = 2586685 }}</ref><ref>{{cite book| vauthors = Sriram Bhat M |title=SRB's Surgical Operations: Text & Atlas|date=2014|publisher=JP Medical Ltd|isbn=978-93-5025-121-8|page=414|url=https://books.google.com/books?id=ZN7fBAAAQBAJ&pg=PA414|language=en}}</ref> 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.<ref>{{cite web |url=http://calder.med.miami.edu/Ralph_Millard/biography.html |title=Biography and Personal Archive |access-date=July 1, 2007 |archive-url = https://web.archive.org/web/20070617215647/http://calder.med.miami.edu/Ralph_Millard/biography.html <!-- Bot retrieved archive --> |archive-date = June 17, 2007}} at miami.edu</ref> Often an incomplete cleft lip requires the same surgery as complete cleft. This is done for two reasons. Firstly the group of [[muscle]]s required to purse the lips run through the upper lip. 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. <gallery class="center" widths="150px" heights="150px"> File:Millardrepair1.svg|The blue lines indicate incisions. File:Millardrepair2.svg|Movement of the flaps; flap A is moved between B and C. C is rotated slightly while B is pushed down. File:Baby_girl_with_cleft_palate,_smiling.jpg | Pre-operation File:17161036 POSTOPT3.jpg| Post-operation, the lip is swollen from surgery and will look more natural within weeks. See photos in the [[#Cleft lip|section above]]. </gallery> ====Pre-surgical devices==== In some cases of a severe bilateral complete cleft, the premaxillary segment will be protruded far outside the mouth. [[Nasolabial fold|Nasoalveolar molding]] prior to surgery can improve long-term nasal symmetry where there is complete unilateral cleft lipβcleft palate, compared to correction by surgery alone, according to a retrospective [[cohort study]].<ref>{{cite journal | vauthors = Barillas I, Dec W, Warren SM, Cutting CB, Grayson BH | title = Nasoalveolar molding improves long-term nasal symmetry in complete unilateral cleft lip-cleft palate patients | journal = Plastic and Reconstructive Surgery | volume = 123 | issue = 3 | pages = 1002β1006 | date = March 2009 | pmid = 19319066 | doi = 10.1097/PRS.0b013e318199f46e | s2cid = 24514123 }}</ref> In this study, significant improvements in nasal symmetry were observed in multiple areas including measurements of the projected length of the nasal ala (lateral surface of the external nose), 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. A [[systematic review]] found in conclusion that nasoalveolar molding had a positive effect on the primary surgery of cleft lip/or palate treatment and aesthetics.<ref>{{cite journal | vauthors = Maillard S, Retrouvey JM, Ahmed MK, Taub PJ | title = Correlation between Nasoalveolar Molding and Surgical, Aesthetic, Functional and Socioeconomic Outcomes Following Primary Repair Surgery: a Systematic Review | journal = Journal of Oral & Maxillofacial Research | volume = 8 | issue = 3 | pages = e2 | date = September 30, 2017 | pmid = 29142654 | pmc = 5676312 | doi = 10.5037/jomr.2017.8302 }}</ref> ===Cleft palate=== [[File:Repairedcleftpalate.JPG|thumb|A repaired cleft palate on a 64-year-old female]] Often a cleft palate is temporarily covered by a [[palatal obturator]] (a prosthetic device made to fit the roof of the mouth covering the gap). This device re-positions displaced alveolar segments and helps reduce the cleft lip separation. The obturator will improve speech as there's now proper airflow and improve feeding and breathing as the gap in the hard and soft palate is closed over so cannot affect it.<ref name="Gill_2011">{{Cite book| vauthors = Gill DS, Naini FB |title=Orthodontics: Principles and Practice|year=2011|pages=257}}</ref>{{rp|257}} Cleft palate can also be corrected by [[Alveolar cleft grafting|surgery]], usually performed between 6 and 12 months. Approximately 20β25% 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]].<ref>{{cite journal | vauthors = Fukuyama E, Omura S, Fujita K, Soma K, Torikai K | title = Excessive rapid palatal expansion with Latham appliance for distal repositioning of protruded premaxilla in bilateral cleft lip and alveolus | journal = The Cleft Palate-Craniofacial Journal | volume = 43 | issue = 6 | pages = 673β677 | date = November 2006 | pmid = 17105324 | doi = 10.1597/05-109 | s2cid = 34126577 }}</ref> The Latham is surgically inserted by use of pins during the child's fourth or fifth month. After it is in place, the doctor, or parents, turn a screw daily to bring the cleft together to assist with future lip 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 individual's own chin, rib or hip. At age 1β7 years the child is regularly reviewed by the cleft team.<ref name="Gill_2011" />{{rp|257}} Age 7β12 years, for the children born with alveolar clefts, they may need to have a secondary alveolar bone graft. This is where autogenous [[cancellous bone]] from a donor site (often the pelvic bone) is transplanted into the alveolar cleft region. This transplant of bone will close the osseous cleft of the alveolus, close any oro-nasal [[fistula]]e and will become integrated with the [[maxillary bone]]. It provides bone for teeth to erupt into and to allow implants to be placed as a possible future treatment option. The procedure should be carried out before the upper canine has erupted. Ideally the root of the canine should be one to two-thirds formed and that there is a space available to place the bone graft. [[Radiography|Radio-graphs]] are taken to determine the quantity of missing bone in the cleft area.<ref name="Gill_2011" />{{rp|258}} ==== Other surgeries ==== [[Orthognathic surgery]] β surgical cutting of bone to realign the upper jaw ([[osteotomy]]). The bone is cut then re-positioned and held together by wires or rigid fixation plates to ensure there's no anterior-posterior discrepancy, also to reduce scarring as it reduces growth. Single piece or multi-piece osteotomy exist. Single piece osteotomy is carried out where there is sufficient alveolar continuity achieved from a successful bone graft. Multi piece osteotomy is performed when there is a notable residual alveolar defect with a dental gap and oronasal fistula (communication between the oral and nasal cavities). The goal of both single and multi piece osteotomy is to displace the maxilla forward to obtain adequate occlusion as well to provide better support for upper lip and the nose and to close any [[fistula]]e.<ref name=":2">{{cite journal | vauthors = Kloukos D, Fudalej P, Sequeira-Byron P, Katsaros C | title = Maxillary distraction osteogenesis versus orthognathic surgery for cleft lip and palate patients | journal = The Cochrane Database of Systematic Reviews | volume = 8 | issue = 8 | pages = CD010403 | date = August 2018 | pmid = 30095853 | pmc = 6513261 | doi = 10.1002/14651858.CD010403.pub3 }}</ref> [[Distraction osteogenesis]] β bone lengthening by gradual distraction. This involves cutting bone and moving ends apart incrementally to allow new bone to form in the gap. This consists of several phases. After attachment of the distracting device and the bone cuts, there is a latency phase of 3β7 days when a [[Callus (cell biology)|callus]] forms. In the activation phase distraction of the callus induces bony ingrowth which can last up to 15 days depending on the required distraction. Once the required bone length is reached, the distraction device is left to remain in situ as it acts as a rigid skeletal fixation device until the new bone has matured (known as the consolidation period).<ref name=":2" /> ===Speech=== [[Velopharyngeal insufficiency]] (VPI) can occur as a result of an unrepaired or repaired cleft lip and palate. VPI is the inability of the soft palate to close tightly against the back of the throat during speech, resulting in incomplete velopharyngeal closure. In turn, this results in speech abnormalities. Velopharyngeal closure is necessary during speech because it forms a seal between the nose and mouth, allowing the production of normal speech sounds. VPI can cause [[hypernasality]] (excessive nasal resonance), [[hyponasality]] (reduced nasal resonance), or a mixed nasal resonance, which is when hypernasality and hyponasality occur simultaneously.<ref>{{cite journal | vauthors = Nasser M, Fedorowicz Z, Newton JT, Nouri M | title = Interventions for the management of submucous cleft palate | journal = The Cochrane Database of Systematic Reviews | issue = 1 | pages = CD006703 | date = January 2008 | pmid = 18254111 | doi = 10.1002/14651858.CD006703.pub2 | veditors = Nasser M }}</ref> In addition, CLP may cause abnormal positioning of individual teeth, which can in turn affect the patient's ability to make certain sounds when speaking such as the "f" or "v" sound and can also result in a lisp. The changes in speech may also be a manifestation on CLP's effects on the patient's occlusion.<ref name=":5" /><ref name=":6" /> ===Hearing=== Children with cleft palate have a very high risk of developing a middle ear infection, specifically [[otitis media]]. This is due to the immature development of the different bones and muscles in the ear. Otitis media is caused by the obstruction of the [[Eustachian tube]], negative middle ear pressure and fluid build-up in the normally air-filled space of the middle ear.<ref>{{Cite book| vauthors = Steele D, Adam GP, Di M, Halladay C, Pan I, Coppersmith N, Balk EM, Trikalinos TA |url=http://www.ncbi.nlm.nih.gov/books/NBK447521/|title=Tympanostomy Tubes in Children With Otitis Media|date=2017|publisher=Agency for Healthcare Research and Quality (US)|series=AHRQ Comparative Effectiveness Reviews|location=Rockville (MD)|pmid=28817250}}</ref> This is associated with hearing impairment or loss. The insertion of a ventilation tube into the eardrum is a surgical treatment option commonly used to improve hearing in children with otitis media.<ref>{{cite journal | vauthors = Browning GG, Rovers MM, Williamson I, Lous J, Burton MJ | title = Grommets (ventilation tubes) for hearing loss associated with otitis media with effusion in children | journal = The Cochrane Database of Systematic Reviews | issue = 10 | pages = CD001801 | date = October 2010 | pmid = 20927726 | doi = 10.1002/14651858.CD001801.pub3 | s2cid = 43568574 }}</ref> In addition, breast milk has been proven to decrease the incidence of otitis media in infants with clefts.<ref name=":5" /> === Feeding === There are different options on how to feed a baby with cleft lip or cleft palate which include: [[Breastfeeding|breast-feeding]], bottle feeding, spoon feeding and syringe feeding. Although breast-feeding is challenging, it improves weight-gain compared to spoon-feeding.<ref name=":3">{{Cite web|url=https://abm.memberclicks.net/assets/DOCUMENTS/PROTOCOLS/17-cleft-lip-cleft-palate-protocol-english.pdf|title='ABM clinical protocol #18: guidelines for breastfeeding infants with cleft lip, cleft palate, or cleft lip and palate, revised 2013.', Academy of Breastfeeding Medicine|vauthors=Reilly S, Reid J, Skeat J, Cahir P, Mei C, Bunik M|date=2013}}</ref> Nasal regurgitation is common due to the open space between the oral cavity and the nasal cavity. Bottle feeding can help (with squeezable bottles being easier to use than rigid bottles). In addition, maxillary plates can be added to aid in feeding. Whatever feeding method is established, it is important to keep the baby's weight gain and hydration monitored. Infants with cleft lip or palate may require supplemental feeds for adequate growth and nutrition. Breast feeding position as suggested by specialists can also improve success rate.<ref>{{cite journal | vauthors = Matsunaka E, Ueki S, Makimoto K | title = Impact of breastfeeding or bottle-feeding on surgical wound dehiscence after cleft lip repair in infants: a systematic review protocol | language = en-US | journal = JBI Database of Systematic Reviews and Implementation Reports | volume = 13 | issue = 10 | pages = 3β11 | date = October 2015 | pmid = 26571277 | doi = 10.11124/jbisrir-2015-2336 | s2cid = 45396841 }}</ref> ==== Breast-feeding ==== Babies with cleft lip are more likely to breastfeed successfully than those with cleft palate and cleft lip and palate. Larger clefts of the soft or hard palate may not be able to generate suction as the oral cavity cannot be separated from the nasal cavity when feeding which leads to fatigue, prolonged feeding time, impaired growth and nutrition. Changes in swallowing mechanics may result in coughing, choking, gagging and nasal regurgitation. Even after cleft repair, the problem may still persist as significant motor learning of swallowing and sucking was absent for many months before repair.<ref>{{cite journal | vauthors = Bessell A, Hooper L, Shaw WC, Reilly S, Reid J, Glenny AM | title = Feeding interventions for growth and development in infants with cleft lip, cleft palate or cleft lip and palate | journal = The Cochrane Database of Systematic Reviews | volume = 2011 | issue = 2 | pages = CD003315 | date = February 2011 | pmid = 21328261 | doi = 10.1002/14651858.cd003315 | publisher = John Wiley & Sons, Ltd | hdl-access = free | hdl = 10072/172084 }}</ref> These difficulties in feeding may result in secondary problems such as poor weight gain, excessive energy expenditure during feeding, lengthy feeding times, discomfort during feeding, and stressful feeding interactions between the infant and the mother. A potential source of discomfort for the baby during or after feeding is bloating or frequent "spit up" which is due to the excessive air intake through the nose and mouth in the open cleft.<ref name=":5" /> Babies with cleft lip and or palate should be evaluated individually taking into account the size and location of the cleft and the mother's previous experience with breastfeeding.<ref name=":3" /> Another option is feeding breast milk via bottle or syringe. Since babies with clip lip and cleft palate generate less suction when breastfeeding, their nutrition, hydration and weight gain may be affected. This may result in the need for supplemental feeds. Modifying the position of holding the baby may increase the effectiveness and efficiency of breastfeeding. ==== Alternative Feeding Methods ==== Preoperative feeding β using a squeezable bottle instead of a rigid bottle can allow a higher volume of food intake and less effort to extract food. Using a syringe is practical, easy to perform and allows greater administered volume of food. It also means there will be weight gain and less time spent feeding.<ref name=":4">{{cite journal | vauthors = Duarte GA, Ramos RB, Cardoso MC | title = Feeding methods for children with cleft lip and/or palate: a systematic review | journal = Brazilian Journal of Otorhinolaryngology | volume = 82 | issue = 5 | pages = 602β609 | date = September 1, 2016 | pmid = 26997574 | pmc = 9444722 | doi = 10.1016/j.bjorl.2015.10.020 | doi-access = free }}</ref> Post-operative feeding (isolated lip repair, or lip repair associated or not with [[palatoplasty]]) β post [[palatoplasty]], some studies believe that inappropriate negative pressure on the suture line may affect results. Babies can be fed by a [[nasogastric tube]] instead. Studies suggest babies required less analgesics and shorter hospital stay with nasogastric feeding post-surgery. With bottle-feeding, there was higher feeding rejection and pain and required more frequent and prolonged feeding times.<ref name=":4" /> ===Treatment schedule=== Each person's treatment schedule is individualized. 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, it is an ongoing therapy, for example speech therapy. In most cases of cleft lip and palate that involve the alveolar bone, patients will need a treatment plan including the prevention of cavities, orthodontics, alveolar bone grafting, and possibly jaw surgery.<ref>{{cite journal | vauthors = Scalzone A, Flores-Mir C, Carozza D, d'Apuzzo F, Grassia V, Perillo L | title = Secondary alveolar bone grafting using autologous versus alloplastic material in the treatment of cleft lip and palate patients: systematic review and meta-analysis | journal = Progress in Orthodontics | volume = 20 | issue = 1 | pages = 6 | date = February 2019 | pmid = 30740615 | pmc = 6369233 | doi = 10.1186/s40510-018-0252-y | doi-access = free }}</ref> {| class="wikitable" width="90%" style="margin: 1em auto;" |- | Age | {{center|0m}} | {{center|3m}} | {{center|6m}} | {{center|9m}} | {{center|1y}} | {{center|2y}} | {{center|3y}} | {{center|4y}} | {{center|5y}} | {{center|6y}} | {{center|7y}} | {{center|8y}} | {{center|9y}} | {{center|10y}} | {{center|11y}} | {{center|12y}} | {{center|13y}} | {{center|14y}} | {{center|15y}} | {{center|16y}} | {{center|17y}} | {{center|18y}} |- | [[Palatal obturator]] | bgColor="#009900" | | bgColor="#009900" | | bgColor="#009900" | | bgColor="#009900" | | bgColor="#009900" | | | | | | | | | | | | | | | | | | |- | Repair cleft lip | | bgColor="#ff0000" | | | | | | | | | | | | | | | | | | | | | |- | Repair [[soft palate]] | | | | bgColor="#ff6666" | | bgColor="#ff6666" | | | | | | | | | | | | | | | | | | |- | Repair [[hard palate]] | | | | bgColor="#9900cc" | | bgColor="#9900cc" | | | | | | | | | | | | | | | | | | |- | [[Tympanostomy tube]] | | | bgColor="#ff0099" | | bgColor="#ff0099" | | bgColor="#ff0099" | | | | | | | | | | | | | | | | | | |- | [[Speech therapy]]/[[Augmentation pharyngoplasty|pharyngoplasty]] | | | | | | | bgColor="#666699" | | bgColor="#666699" | | bgColor="#666699" | | bgColor="#666699" | | | | | | | | | | | | | |- | [[Alveolar cleft grafting]] | | | | | | | | | | | | | bgColor="#ff3300" | | bgColor="#ff3300" | | bgColor="#ff3300" | | | | | | | | |- | [[Orthodontics]] | | | | | | | | | | | bgColor="#0000cc" | | | bgColor="#0000cc" | | bgColor="#0000cc" | | bgColor="#0000cc" | | bgColor="#0000cc" | | bgColor="#0000cc" | | bgColor="#0000cc" | | bgColor="#0000cc" | | bgColor="#0000cc" | | bgColor="#0000cc" | | bgColor="#0000cc" | |- | [[Orthognathic surgery]] | | | | | | | | | | | | | | | | | | | bgColor="#ffff00" | | bgColor="#ffff00" | | bgColor="#ffff00" | | bgColor="#ffff00" | |} ===Cleft team=== People with CLP present with a multiplicity of problems. Therefore, effective management of CLP involves a wide range of specialists. The current model for delivery of this care is the multidisciplinary cleft team. This is a group of individuals from different specialist backgrounds who work closely together to provide patients with comprehensive care from birth through adolescence. This system of delivery of care enables the individuals within the team to function in an interdisciplinary way, so that all aspects of care for CLP patients can be provided in the best way possible.<ref>{{Cite journal| vauthors = Hodgkinson PD, Brown S, Duncan D, Grant C, McNaughton AM, Thomas P |date=February 2005|journal=Fetal and Maternal Medicine Review|language=en|volume=16|issue=1|pages=1β27|doi=10.1017/S0965539505001452|issn=1469-5065|title=Management of Children with Cleft Lip and Palate: A Review Describing the Application of Multidisciplinary Team Working in This Condition Based Upon the Experiences of a Regional Cleft Lip and Palate Centre in the United Kingdom|citeseerx=10.1.1.483.9042|s2cid=36404355 }}</ref> ===Outcomes assessment=== Measuring the outcomes of CLP treatment has been laden with difficulty due to the complexity and longitudinal nature of cleft care, which spans birth through young adulthood. Prior attempts to study the effectiveness of specific interventions or overall treatment protocols have been hindered by a lack of data standards for outcomes assessment in cleft care.{{citation needed|date=May 2022}} The International Consortium for Health Outcome Measurement (ICHOM) has proposed the Standard Set of Outcome Measures for Cleft Lip and Palate.<ref>{{Cite web | url=https://www.ichom.org/medical-conditions/cleft-lip-palate/ | title=ICHOM | Cleft Lip & Palate Standard Set | Measuring Outcomes}}</ref><ref name= AlloriICHOM2017 >{{cite journal | vauthors = Allori AC, Kelley T, Meara JG, Albert A, Bonanthaya K, Chapman K, Cunningham M, Daskalogiannakis J, de Gier H, Heggie AA, Hernandez C, Jackson O, Jones Y, Kangesu L, Koudstaal MJ, Kuchhal R, Lohmander A, Long RE, Magee L, Monson L, Rose E, Sitzman TJ, Taylor JA, Thorburn G, van Eeden S, Williams C, Wirthlin JO, Wong KW | title = A Standard Set of Outcome Measures for the Comprehensive Appraisal of Cleft Care | journal = The Cleft Palate-Craniofacial Journal | volume = 54 | issue = 5 | pages = 540β554 | date = September 2017 | pmid = 27223626 | doi = 10.1597/15-292 | s2cid = 43371901 }}</ref> The ICHOM Standard Set includes measures for many of the important outcome domains in cleft care (hearing, breathing, eating/drinking, speech, oral health, appearance and psychosocial well-being). It includes clinician-reported, patient-reported, and family-reported outcome measures.
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