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 Table of Contents  
REVIEW ARTICLE
Year : 2020  |  Volume : 7  |  Issue : 1  |  Page : 8-16

Approach to patients with cleft lip and palate in orthodontics


Department of Orthodontics, Faculty of Dentistry, Firat University, Elazig, Turkey

Date of Submission28-Aug-2019
Date of Acceptance06-Dec-2019
Date of Web Publication20-Jan-2020

Correspondence Address:
Dr. Yasin Akbulut
Department of Orthodontics, Faculty of Dentistry, Firat University, 23119, Elazig
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jclpca.jclpca_23_19

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  Abstract 


Cleft lip and palate (CLP) are the most commonly observed congenital anomaly in the craniofacial region, which are observed in one in every one thousand children and caused by multifactorial factors. CLP occur due to the inability of embryonic protrusions, which forms the roof of the mouth with the maxillary bone, to integrate completely. Due to the scar tissue formed as a result of the operation conducted for dental problems, such as missing teeth or extra teeth, location anomalies, enamel defects, and anomalies of size, formation, and eruption, CLP can cause developmental problems, problems in speech due to difficulties in phonation, and hearing problems due to frequently experienced otitis media. CLP treatment requires a multidisciplinary approach. For the CLP treatment to succeed, it is very important for a team that consists of an orthodontic specialist, ENT specialist, audiologist, psychologist, dentist, and speech therapist to work in harmony. The treatment process of CLP starts as soon as the infant is born. The period of the first 6 months is the preoperative period. In this period, a feeding plaque implementation is conducted by an orthodontic specialist. Operations for lips are usually conducted between the 3rd and 6th months, whereas operations for palates are usually conducted between the 12th and 18th months. In mixed dentition, maxillary expansion and protraction, tooth location correction, and bone grafting are conducted. In continuous dentition, if constant orthodontic treatment is necessary, orthognathic surgical operations and prosthetic dentistry treatments are conducted. In CLP anomalies, the main aim is to educate future mothers and fathers about the factors that can be eliminated. For infants who are born with CLP, the treatment should be initiated without losing any time. The fact that the treatment of these patients is conducted at an esthetically and functionally satisfactory level will also improve the quality of life for both the patients and their families. Furthermore, in patients with CLP, esthetics, phonation, and speech, briefly, the external appearance should be emphasized with recurring operations and follow-ups and their mental states should not be ignored in addition to supporting them with psychological rehabilitation and a constant boost of morale.

Keywords: Cleft lip and palate, congenital anomaly, review


How to cite this article:
Akbulut Y. Approach to patients with cleft lip and palate in orthodontics. J Cleft Lip Palate Craniofac Anomal 2020;7:8-16

How to cite this URL:
Akbulut Y. Approach to patients with cleft lip and palate in orthodontics. J Cleft Lip Palate Craniofac Anomal [serial online] 2020 [cited 2020 Feb 24];7:8-16. Available from: http://www.jclpca.org/text.asp?2020/7/1/8/276199




  Introduction Top


What are cleft lip and palate?

Cleft lips and palates (CLP) are the most commonly observed congenital anomalies in the craniofacial region and they embryologically emerge in the organogenesis phase.[1],[2],[3],[4] CLP occur due to incomplete combination and integration of embryonic protrusions, which creates the soft and solid tissues that form the roof of the mouth. Cleft lip occurs due to the failed combination between 4th and 6th weeks of pregnancy, whereas cleft palate occurs between the 6th and 12th weeks of pregnancy.[5],[6],[7],[8] CLP can be diagnosed by ultrasonography between 16th and 20th weeks of pregnancy [Figure 1], [Figure 2], [Figure 3]. Ulgen[9] has schematized the appearance of healthy tissues [Figure 1], isolated (partial) clefts [Figure 2] and total clefts [Figure 3].
Figure 1: Healthy appearance without cleft lip and palate ([a]: Nose and lips frontally [b]: Maxillary alveolar bone from below, hard palate, soft palate and uvula)

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Figure 2: Isolated (Partial) Clefts ([a] Isolated cleft lip [b] Isolated cleft alveolar bone [c] Isolated cleft hard palate [d] Isolated cleft soft palate and uvula bifida [uvula split in half])

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Figure 3: Total Clefts ([a] A1 and A2 demonstrate unilateral total clefts formed on the left. They cover the left nostril, left upper lip, left alveolar bone, hard palate, soft palate and uvula. [b] B1 and B2 demonstrate bilateral total clefts. Bilateral clefts cover both sides of the regions above and solely remain in the middle, on premaxilla)

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Incidence of cleft lip and palate

CLP is observed in one in every 775 thousand newborn children.[10],[11],[12],[13] According to a study conducted in Turkey, this rate is one in every one thousand children.[14] If one of the parents has CLP, the incidence rate for the infant is 5%.[11] In general, the most common topic of curiosity for parents who have children with CLP is whether their infant will be born with CLP. The parents who have an infant with CLP previously have a higher risk of having CLP in subsequent children.[7],[15],[16] If the parents do not have CLP and the first child has CLP, the chance for the second child to be born with CLP is 4%, whereas if the parents do not have CLP and the first child has isolated cleft palate (ICP), the chance for the second child to be born with ICP is 2%.[7],[15],[16],[17] The anomaly rates in studies conducted with various risk groups are presented in [Table 1] and [Table 2].[18],[19],[20],[21],[22]
Table 1: Incidence of cleft lip and palate in risk groups

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Table 2: Incidence of cleft lip and palate according to race and gender

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The incidence rate of male infants is twice of those in female infants.[11],[23] 25% of the isolated cleft lips occur in the left side.[11],[23] In addition, secondary cleft palates are observed two times more frequently in female infants compared to male infants. The incidence rate in African-derived populations is also lower compared to other populations.[24] 85% of the bilateral cleft lips also include cleft palates, as well as 70% of the unilateral cleft lips.[25],[26],[27]

Etiology of cleft lip and palate

In the etiology of CLP, although genetic and environmental factors play various roles, it is believed that multifactorial inheritance is mostly valid and more than one factor is usually emphasized as reasons.[28],[29] Various models were proposed to explain the multifactorial etiology, and according to these models, the occurrence of the anomaly is influenced by genetic factor along with environmental factors by keeping the genetic tendency toward any characteristic under control with various genes.[30],[31] No gene with genetically proven efficiency was determined up until now. In several studies, genes in the 4th and 6th chromosomes were emphasized; however, no definitive result was obtained.[32],[33] Nevertheless, significant results were obtained regarding the role of a certain region located in the p13 band, which is located in the short arm of the 2nd chromosome, on this subject.[30] As for environmental factors, the factors of CLP include deficiencies of Vitamins B and A;[34] smoking, consuming alcohol, and using drugs during pregnancy;[7],[35] radiation; viral infections; consanguineous marriage; stress; systemic diseases; anti-inflammatory steroids with cortisone content; teratogenic drugs such as phenytoin, salicylates, amino proteins, and busulfan;[36],[37] and mothers with diabetes.[35],[37] Furthermore, older mothers have a higher risk compared to younger mother.[38]

Classification of cleft lip and palate

CLP is classified in different aspects as anatomically, etiologically, and embryologically. In the anatomical classification, the incisive foramen is used as the reference. The clefts located in the frontal section of the incisive foramen, in the premaxilla, are classified as primary cleft palates, whereas the clefts located in the rear section are classified as secondary cleft palates. The classifications of Davies and Ritchie,[8] Kernahan and Stark,[39] Sadler,[34] and Veau[40] are among the frequently used classifications [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]. The anatomical points referenced in the classifications are shown in the [Figure 9].[41]
Figure 4: The classification of Davies and Ritchie

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Figure 5: The classification of Kernahan and Stark ([a] Partial cleft lip [b] Complete cleft lip [c] Partial cleft palate [d] Complete cleft palate [e] Unilateral complete cleft lip and palate [f] Bilateral complete cleft lip and palate)

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Figure 6: The classification of Kernahan and Stark

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Figure 7: The classification of Sadler ([a] Normal [b] Unilateral cleft lip [c] Unilateral cleft lip and alveolar cleft [d] Bilateral cleft lip and alveolar cleft [e] Cleft palate [f] Unilateral cleft lip and/or cleft palate)

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Figure 8: The classification of Vaeu ([a] Type 1: Soft palate and uvula cleft; [b] Type 2: Hard palate, soft palate, and uvula cleft (the cleft does not get ahead of the foramen incision); [c] Type 3: Unilateral complete lip, alveolar, hard palate, soft palate, and uvula cleft; [d] Type 4: Bilateral complete lip, alveolar, hard palate, soft palate, and uvula cleft.)

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Figure 9: Primary and Secondary palate regions

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The classification is performed on a numbered scale that is called the Y scale to facilitate an easier expression. According to this scale, the areas 1 and 4 refer to cleft lips, whereas areas 2 and 5 refer to alveolar clefts followed by areas 3 and 6 referring to hard palate clefts in the anterior of the incisive foramen and areas 7, 8, and 9 referring to hard palate, soft palate, and uvula clefts in the posterior of incisive foramen, respectively [Figure 7] and [Figure 8].

Problems experienced in cleft lip and palate

Dental problems

The incidence of anomalies is higher in children with CLP compared to healthy children, and the anomalies are directly proportional to the intensity of the cleft.[42] Number anomalies are frequently observed in teeth.[43],[44] As number anomalies, absent teeth and supernumerary teeth are observed. The teeth with the most frequent absence are maxillary canine or lateral teeth.[45],[46] As a supernumerary, mesiodens or extra incisor tooth may be observed in the cleft line[47] and these usually remain embedded.[46] In addition to number anomalies, dental anomalies of size, formation and eruption time,[42],[48] location anomalies, conic-shaped laterals, eruption disorders, and enamel defects in the teeth neighboring the cleft line are frequently observed.[49],[50],[51],[52] These anomalies were reported as risk factors of cavities in several studies.[13],[49],[53],[54]

Developmental problems

In patients with CLP, maxilla demonstrates development deficiencies at all three ways of the space. Due to the scar on the lip region, the development toward the anterior is limited while the palatoplasty practiced on the palatal region causes collapses at the transversal direction and maxillary development deficiencies in the vertical direction. The development deficiency occurring in the maxilla causes Class III malocclusion at the sagittal direction, anterior open bite in the vertical direction, and narrow maxillary at the transversal direction.[24]

Speech and hearing problems

In patients with CLP, in addition to the cranial base and facial morphology, phonation is also affected. Due to the dysfunction of m. levator veli palatini muscle, the phonations of several sounds are distorted during speech and hypernasal phonation occurs. The patients have difficulties in the phonation of letters s, f, p, t, and k.[55],[56],[57],[58],[59],[60]

Because of the dysfunction of m. tensor veli palatini muscle, which opens the  Eustachian tube More Details, otitis media is observed in these patients. In a case where infections frequently occur, results that can lead to hearing loss may occur.[44],[50]

Treatment disciplines of cleft lip and palate

CLP treatment requires a multidisciplinary approach. For the success of CLP treatment, it is very important to work with a crew that consists of an orthodontic specialist, who can lead the development and provide the modifications, a plastic surgeon for surgical procedures, an ear–nose–throat specialist and audiologist to eliminate hearing and speech problems, a pediatrician to follow the general health status, a child psychologist for the psychological preparation of the child at the time of starting to go to school, a child dentist to provide the control of dental cavities, a speech therapist for the ideal speaking ability, and a prosthodontist for the final esthetical results.[61],[62],[63] The treatment process of CLP starts as soon as the infant is born. In general, the treatment is divided into four periods.

The period of the first 6 months (preoperative orthopedics)

In patients with CLP, because the oral cavity and the nasal cavity are not separated from each other and a connection is formed between them, difficulties in feeding occur. Therefore, the most vital action to take is to ensure that the infant is fed in a healthy way and prevent further problems, mainly aspiration dangers, due to the clefts during feeding. For this, a dental plaque is implemented to ensure that the cleft is closed as much as possible before the surgical operation by creating a vacuum effect between the tongue of the infant for comfortable feeding and directing the cleft surfaces to develop toward each other through preventing the tongue from entering into the clefts.[64],[65] Before the operation, it is very important for the infant to feed to meet the required conditions. In addition, this plaque also prevents mother's milk or infant food from being aspirated through the clefts, seeping into the middle ear through the eustachian tube and, therefore, prevents otitis media. Infants with CLP have a risk of otitis media and therefore hearing loss.[44],[50] In individuals with unilateral CLP, the smaller segment relocates through the medial and the fact that the segments are far away from their ideal positions leads to difficulties during the surgical restoration. In bilateral clefts, the fact that the both of the small segments are displaced from the medial both on the right and the left and the burst of premaxilla toward the anterior cause difficulties in ensuring the arch form. Therefore, nasoalveolar molding (reshaping) is implemented in these cases. Nasoalveolar molding should start in the 0–2 weeks old period.[66] Furthermore, precautions should be taken against heart and skeletal system problems, which are usually accompanied by CLP in infants.[29]

In infants, lip operations are generally conducted between the 3 and 6 months. Palate operations are generally conducted between 12 and 18 months.[67],[68],[69] This surgery should be conducted before the 18th month because the infant starts speaking in this period. Besides speaking, these infants also have trouble in reading when they start going to school. In fact, despite the fact that the rate of difficulty in reading observed in healthy children is the same with the 9% rate of difficulty in reading observed in children with CLP, this rate is 33% in children with ICP.[70] When the infant becomes 2.5-year-old, the infant is reevaluated in terms of the quality of sound and speech.

Early mixed dentition

In patients with CLP, the problems that emerge in this period frequently include the problems occurring due to the limiting impact of the scar tissue, which develops following the surgery, on the development of maxilla. The arch form in the maxilla especially demonstrates narrowing in premaxilla and this results in a crossbite. Maxillary central incisor teeth erupt in crossbite and with rotation. Because the lateral and canine teeth are neighbors with the cleft line, there is no healthy bone line for them to erupt. Due to these factors, there is a need for enlargement at the transversal direction in the maxilla.[71],[72],[73] The locations of central and lateral teeth are corrected.[74],[75] Moreover, to ensure the eruption of laterals and canines, a secondary alveolar bone graft is conducted between 7 and 10 years old.[76] Ideally, the secondary bone graft should be conducted at an early period to enable the eruption of the teeth in the cleft region.[77],[78],[79] If there is also a maxillary development deficiency, the initiation of facemask is also implemented in this period.[74],[80],[81] In individuals with CLP, use of facemask along with rapid maxillary enlargement is an efficient technique used in maxillary protraction.[73],[82],[83]

Permanent dentition

Following the eruptions of canine and premolars, almost all patients with CLP requires orthodontic treatment. Frequently, crossbite develops in the cleft side and missing teeth are observed. The treatment of the patient is continued by opening/closing the gaps formed due to this deficiency.

Late adolescence period

Following the completion of the growth and development, orthognathic surgery and restorative treatments of the patient are planned. The maxilla is generally moved lower and brought forward while set-back surgery is practiced for the mandible. When necessary, late grafting or tertiary bone graft is implemented.[76],[84],[85],[86],[87]

It is very important to ensure the psychological rehabilitation of these patients. In addition, the fact that the treatments of these patients are conducted at an esthetically and functionally satisfactory level improves the quality of life of these patients.[88]

There is a strong connection between an individual's passion for a healthy personality, a strong personality structure and living within society harmoniously, and the level of self-acceptance.[89] The concept of self-acceptance is explained as the acceptance of self with all positive and negative aspects without emotions of inability, guilt, bragging, or appreciation.[90],[91],[92] Self-acceptance is an emotion that starts in childhood and peaks at adolescence. Individuals who know and accept themselves are aware that they are not perfect and know their flaws and limits. They neither try to reach others nor feel guilty by giving way to the feeling of inferiority.[93],[94] Because the completion of ideal esthetic and phonetic treatment of individuals with CLP occurs after the formation of the sense of self, the problems experienced in esthetic appearance and speech in this period bring along various problems.[89],[95],[96],[97] In previous studies, it was reported that individuals with CLP had problems such as not having self-confidence and being isolated from society in addition to the problems they experience in speech and learning.[98],[99],[100],[101] Moreover, even families with infants with CLP were reported to be disconnected from the society and more withdrawn compared to other families.[101]


  Conclusion Top


The perfect thing to do for CLP is certainly to prevent its occurrence in the first place. The primary aim in CLP is to educate parents and future mothers and fathers. By setting superstition, superstitious beliefs, and fatalism aside, awareness should be raised on factors that can be eliminated. Especially in consanguineous marriages, the health of future mothers should be followed and it should be known that no drug should be taken after one's own heart during pregnancy or suspicion of pregnancy and even doctors should inform patients while prescribing to raise the awareness of society on this subject. Considering the fact that not everyone studies for a university degree, it is believed that this awareness should be moved to the high school education level and awareness should be raised with additional consultation lessons or conferences.

The most important point in children who are born with CLP is to overcome the fear and sadness of parents as soon as possible and ensure that they are involved in the treatment process. CLP is a treatable deformity. The success of CLP treatment depends on the initiation of the treatment by the family without being late, the following of the directives in the treatment process and the loyal continuation of the treatment until the end, which is teamwork. The pictures of previous patients with CLP, conducted procedures and the progress achieved following these procedures can be shown to parents to motivate them.

Furthermore, the esthetic appearance, phonation, and speech of the patients with CLP, the external appearance of the patients, should be emphasized by recurring operations and follow-ups, and their mental status should not be ignored. The mental status of patients with CLP should be considered and supported by psychological rehabilitation and their morale should always be bolstered.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
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