Cleft Lip and Palate Review Article PDF 2020

Review Article Cleft of lip and palate: A review Tarun Vyas1, Prabhakar Gupta2, Sachin Kumar2, Rajat Gupta2, Tanu Gupta3, Harkanwal Preet Singh4 1 Department of Oral Medicine and Radiology, R.R. Dental College and Hospital, Udaipur...

Review Article Cleft of lip and palate: A review Tarun Vyas1, Prabhakar Gupta2, Sachin Kumar2, Rajat Gupta2, Tanu Gupta3, Harkanwal Preet Singh4 1 Department of Oral Medicine and Radiology, R.R. Dental College and Hospital, Udaipur, Rajasthan, Departments of 2Oral and Maxillofacial Surgery and 3Prosthodontics and Crown and Bridge, School of Dental Sciences, Sharda University, Greater Noida, Uttar Pradesh, 4Department of Oral and Maxillofacial Pathology and Microbiology, Dasmesh Institute of Research and Dental Sciences, Faridkot, Punjab, India A bstract Cleft of lip and palate are most common serial congenital anomalies to affect the orofacial region. It can occur isolated or together in various combination and/or along with other congenital deformities particularly congenital heart diseases..Patient with oro-facial cleft deformity needs to be treated at right time and at right age to achieve functional and esthetic well being. Successful management of the child born with a cleft lip and palate requires coordinated care provided by a number of different specialties including oral/ maxillofacial surgery, otolaryngology, genetics/dysmorphology, speech/language pathology, orthodontics, prosthodontics, and other. This article aims to the review the point primary care physicians in literature knowledge about cleft lip and palate. A review of literature have made to discuss introduction, epidemiology, clinical feature, etiology factor and management of cleft lip and palate. Keywords: Cleft lip, cleft palate, congentiel anomelies, genetic and environmental factor Introduction through the nasal floor, while a complete cleft implies lack of connection between the alar base and the medial labial element). These are the most severe of congenital anomalies which affect the mouth and related structures. The roof is shaped from the palate Cleft palate: The failure of fusion of the palatal shelves of and flooring from the constructions at the floor of the mouth. the maxillary processes, resulting in a cleft of the hard and/ Laterally, it’s bounded from the cheeks. A cleft is a congenital or soft palates. Clefts arises during the fourth developmental abnormal space or gap in the upper lip, alveolus, or palate. The stage. Exactly where they appears is determined by locations at colloquial term for this condition is harelip. The use of this term which fusion of various facial processes failed to occur, this in should be discouraged due to its demeaning connotation of turn is influenced by the time in embryologic life when some inferiority. The more appropriate terms are cleft lip, cleft palate or interference with development occurred. cleft lip and palate. So cleft lip and cleft palate can be defined as: Clefts of lip and palate can occur isolated or together in various Cleft lip: The failure of fusion of the frontonasal and maxillary combination and/or along with other congenital deformities processes, resulting in a cleft of varying extent through the lip, particularly congenital heart diseases. They are also associated alveolus, and nasal floor (an incomplete cleft does not extend features in over 300 recognized syndromes. In the developed Address for correspondence: Dr. Tarun Vyas, world, most scientists believe that clefts occur due to a Assistant Professor, Department of Oral Medicine and Radiology, combination of genetic and environmental factors (e.g., maternal R.R. Dental College & Hospital, Udaipur, Rajasthan, India. illness, drugs, malnutrition). In developed countries, CL/P E‑mail: [email protected] is typically identified before birth by ultrasonography. Early Received: 26‑03‑2020 Revised: 25‑04‑2020 detection allows time for parental education about the potential Accepted: 02-05-2020 Published: 30-06-2020 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to Access this article online remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is Quick Response Code: given and the new creations are licensed under the identical terms. Website: www.jfmpc.com For reprints contact: [email protected] DOI: How to cite this article: Vyas T, Gupta P, Kumar S, Gupta R, Gupta T, 10.4103/jfmpc.jfmpc_472_20 Singh HP. Cleft of lip and palate: A review. J Family Med Prim Care 2020;9:2621-5. © 2020 Journal of Family Medicine and Primary Care | Published by Wolters Kluwer ‑ Medknow 2621  Vyas, et al.: Cleft of lip and palate causes of the CL/P and procedures that the child may need after The incidence appears high among Asians (0.82 – 4.04 per live birth. Consequently, due to the widespread access to medical births) intermediate in Caucasian (0.9 – 2.69 per 1000 live birth) care in developed countries, although beliefs unsupported by and low in Africans (0.18 – 1.67 per 1000 live births). Chinese science (e.g., superstitious beliefs) do exist, scientific causal beliefs showed 1.76 per 1000 live birth, while Japanese reported 0.85 to are the most commonly endorsed (Nelson et al., 2009). 2.68 per 1000 live birth of orofacial clefting. In contrast, in developing countries where prenatal care is less Isolated CL comprises about 25% of all clefts, while combined advanced or less available, a CL/P is usually unexpected and CL/P accounts for about 45%. CL/P occurs more frequent families rely less on medical explanations for the cleft from and more severe in boys than in girls. Unilateral clefts are more doctors and rely more on religion and folklore to explain the common than bilateral clefts with a ratio of 4:1, and for unilateral deformity. For example, individuals in India who practice clefts, about 70% occur on the left side of the face. Cleft palate is Hinduism believe that a CL/P is the result of sins from a seen more frequently in females than in males. CL/P is frequently past life (Weatherley‑White et al., 2005). Other religious and associated with other developmental abnormalities and majority cultural beliefs regarding causation of clefts include witchcraft, of cases are presented as part of a syndrome. Syndromic clefts God’s will, and engaging in a behaviour associated with causal account for about 50% of the total cases in some reports with power (e.g., looking at a child with a facial deformity when about 300 syndromes described. Although the percentage of pregnant). cases directly linked to genetic factors is estimated to be about 40%, all clefts appear to show a familial tendency. Overall incidence of cleft lip and palate is approximately 1 in 600 to 800 live births (1.42 in 1000) and isolated cleft palate Various epidemiological studies show that, if one parent affected occurs approximately in 1 in 2000 live births. Thus, the typical with a cleft has a 3.2% chance of having a child with cleft lip and distribution of cleft types are: palate and a 6.8% chance of having a child with isolated cleft palate (Grosen et al., 2010). Presence of a cleft in one parent and 1. Cleft lip alone – 15% in one sibling is associated with a 15.8% chance that the next 2. Cleft lip and palate – 45% child will have a cleft lip or palate, and a 14.9% chance that the 3. Isolated cleft palate – 40%. next child will have a cleft palate (Christensen et al., 1996). In case where parents with one is child affected with a cleft have a The potential problems of the condition include social 4.4% chance of having another child with a cleft lip and palate handicaps such as impaired suckling and resultant failure to and a 2.5% chance of having a child with isolated cleft palate. thrive, speech impediment, deafness, malocclusion, gross facial deformity and severe psychological problems. The clefting of Embryology lip and/or palate occurs at such a strategic place in the orofacial region, at such a crucial time (before birth) that it becomes a Throughout this time, the fundamental morphology of the face complex congenital deformity. is shaped using the combination of the five fundamental facial prominences. CLP happen as a result of incomplete mix and Patient with oro‑facial cleft deformity needs to be treated at integration of rectal protrusions, which generates the delicate right time and at right age to achieve functional and aesthetic and strong tissues that form the roof of their mouth. Cleft lip well‑being. The treatment process is complex, multidisciplinary happens because of the failed mix between 4th and 6th months of pregnancy, whereas the cleft palate occurs between the 6th and and interdisciplinary approach. Successful management of the 12th months of pregnancy. To understand the reason behind child born with a cleft lip and palate requires coordinated care oral cleft a review of lip, nose, and palate embryology is essential. provided by a number of different specialties including oral/ The whole procedure occurs between the 5th to 14th days of life. maxillofacial surgery, otolaryngology, genetics/dysmorphology, speech/language pathology, orthodontics, prosthodontics, and It is that the “critical period” of embryonic confront. It’s that time other. This successful reconstruction routinely requires multiple period through which the human craniofacial morphogenesis phase of surgical intervention.[8,9] usually is vulnerable to known or suspected birth defect generating brokers, or teratogens. Ethical approval This research did not need any informed consent because we did Etiology library research. References and quotations were written based on the journal guidelines. The etiology of cleft lip and palate is complex and thought to involve genetic influences with variable interactions from Epidemiology environmental factors. The etiological factors of cleft lip and palate can be grouped as under: Overall incidence of orofacial clefting is around 1.5 per 1000 live birth (about 220,000 new cases per year) with wide variation A. Non‑genetic: this includes various environmental across geographic areas, ethnic group and nature of cleft itself. (teratogenic) risk factors which may cause CL/P. Journal of Family Medicine and Primary Care 2622 Volume 9 : Issue 6 : June 2020  Vyas, et al.: Cleft of lip and palate B. Genetic: Genetic cause includes: Clinical Features (1) Syndromic: Here cleft is associated with other malformation. Usually it is due to a single gene (monogenic or Mendelian) To date, few studies have evaluated the knowledge and experience disorder. of primary care physicians regarding the physical, dental, and (2) Non‑syndromic: Here the cleft is mostly an isolated feature behavioural/emotional needs of a child with an oral cleft.[19,20] and occurs in the vast majority of individuals having a cleft lip or palate (up to 70% cases). In this form, a cleft is neither The various clinical findings in patient with cleft lip and palate a recognized pattern of malformation nor a known cause for can be categorized under two headings: the disorder can be identified. Dental problems in cleft lip and palate Non‑genetic factors: Besides genetic factor environmental Various abnormal dental conditions includes: factors also play a very important role in etiology of CL/P. Various environmental factors includes: 1. Natal and neonatal teeth: Presence of neonatal teeth does not appear to influence primary a) Smoking: The relationship between maternal smoking and or secondary dentition in clefts. Most natal teeth among clefts are CLP is not strong, but it is significant. Several studies have located in the lateral margin of the premaxillary and maxillary consistently yielded a relative risk of about 1.3–1.5. When segments unlike in non‑cleft neonates.[21,22] maternal smoking was considered together with a positive genetic background, the combined effect was more significant. 2. Microdontia Furthermore, Beaty et al.(2002) reported that maternal smoking Small teeth (microdonts) frequently are found with CL/P. This and infant MSX1 genotypes acted together to increase the risk is usually more common in cases where lateral incisors are not for CLP by 7.16 times. missing (van der Wal, 1993; Stahl et al., 2006;Rawashdeh and Bakir, 2007). Generally peg shaped upper lateral incisors are b) Alcohol use: Heavy maternal drinking, apart from causing seen. fetal alcohol syndrome, also increases the risk of CLP. Munger et al. (1996) showed that maternal drinking increased 3. Taurodontism the risk for CLP by 1.5–4.7 times in a dose‑dependent manner Taurodontism has been reported to be associated with certain Low‑level alcohol consumption, however, did not seem to syndromes and dental developmental disorders (Cichon and increase the risk of orofacial clefts. The link between alcohol Pack, 1985). consumption and genotypes on the risk of CLP has yet to be demonstrated. 4. Ectopic eruption Clefts also contribute to the ectopic eruption of primary lateral c) Others: Environmental factor includes maternal diseases, incisors which may erupt palatally adjacent to or within the stress during pregnancy chemical exposure. Decreased blood cleft side while permanent canine on side of alveolar clefts may supply in nasomaxillary region. increased maternal and parental erupt palatally. Delayed eruption of permanent incisors may be age are also said to increase risk of cleft lip with and without seen.[22,23] palate while higher parental age has been associated with cleft palate only. Fetal exposure to retinoid drugs can results in 5. Enamel hypoplasia severe craniofacial anomalies. Enamel hypoplasia was found to occur more frequently in CL/P subjects compared with non‑cleft populations, especially Genetic factor: Various epidemiological observation have laid involving the maxillary central incisors (Vichi and Franchi, the foundation of role of genetics in etiology of cleft lip and 1995). palate. Many studies have shown that monozygotic twins (60%) have considerable higher concordance rate than dizygotic twins 6. Delayed tooth maturation and siblings (5‑10%).[13,16,18] Several growth factors are of major importance during Syndromic form of cleft lip and palate: It accounts for more craniofacial development, and these factors may be overexpressed than 400 known syndromes and many of them follows classic or underexpressed when a cleft defect occurs. This aberrant Mendelian inheritance pattern.[13,16,18] In some of the syndromes expression can modify odontogenesis and cause abnormalities with cleft lip and palate genes have been identified and are listed of the dental lamina. as under [Table 1]. Other associated conditions Non‑syndromic form of cleft lip and palate: It accounts for 1. Speech difficulties 70% of CL/P cases and 50% of all CPO cases. These associated Due to the dysfunction of m. levator veli palatini muscle studies have identified many genes for clefting whose mutation phonation are affected. Retardation of consonant sound (p, b, may lead to non‑syndromic cleft lip and palate [Table 2].[13,16,18] t, d, k, g) is most common findings. Abnormal nasal resonance Journal of Family Medicine and Primary Care 2623 Volume 9 : Issue 6 : June 2020  Vyas, et al.: Cleft of lip and palate Table 1: Syndromic form of cleft lip and palate Syndromes Gene name (symbol) Location on chromosome Inheritance Waardenburg syndrome, type II A Microphtalmia associated transcription(MLTF) 3p14,1-12,3 AD Di George syndrome Di George syndrome chromosome region (CATCH 22) 22g11 AD Treacher - Collins mandibulofacialdysostosis Treacle (TCOF1) 5q32-q33,1 AD Van der woude syndrome Interferor regulatory factor - 6 (IRF 6) 1q32-q41 AD CLP-Ectodermal dysplasia syndrome Poliovirus receptor related-1(PVRL-1) 11q23,3 AD Ectrodactyly, ectodermal dysplasia orofacial P 63 3q27 AD cleft syndrome Zollinger syndrome-3 Peroxisomalmembrame protein-3 (PXMP3) 8q21,1 AD Diastrophic dysplasia Diastrophic dysplasia sulphate transporter(DTDST) 5q32-q33,1 AD Gorlin syndrome (Basal cell nevus syndrome) Patched (PTCH) 9q22,3 AD Table 2: Possible genes whose mutation may result in expertise, the team may be composed of individual in: (1) the non syndromic clefting dental specialties (orthodontics, oral surgery, pediatric dentistry, Name of gene Symbol Chromosome location and prosthodontics), (2) the medical specialties (genetics, otolaryngology, pediatrics, plastic surgery, and psychiatry), and (3) Transforming growth factor - alpha TGFA 2p13 Transforming growth factor - 133 TGF 133 14q24 allied health care fields (audiology, nursing, psychology, social Methylene tetra - MTHF3 1p36,3 work, and speech pathology). hydrofolateReductase Blood clotting factor XIII gene ET1 6p24 Surgery Treatment Endothelin - 1 gene ET1 6p24 Proto-oncogene BCL3 BCL3 19q13,2 Unlike the artistic nature of the cleft lip repair, the cleft palate Retinoic acid receptor alpha gene RARA 17(t15/17) repair is very functional in nature. A team approach has decreased MSX-1 MSX-1 4q25 the morbidity and secondary deformities caused by the cleft and mostly focuses quality of speech. Soft palate repair and difficulty in articulation are another characteristic feature in techniques may be used in isolation or combined with hard most individuals with cleft lip and palate.[20,25] palate procedures, as necessary. Most surgeons today perform either some modification of an intravelar veloplasty, vs. a two 2. Ear infection: flap palatoplasty with double opposing z‑plasty to achieve levator Due to improper function of m. tensor veli palatini muscle, muscular repositioning. Maxillary distraction is increasingly which opens the Eustachian tube, otitis media is observed in used for the correction of severe maxillary retrusion in patients these patients. In a case where infections frequently occur, with cleft lip and palate. Cleft lip and palate children benefit results that can lead to hearing loss may occur. The incidence, from team approach special treatment requirements. such a team however, increases sharply when there is associated submucous lead by the plastic surgeon should include a speech therapist and cleft palate.[20,26] orthodontist having ready access to pediatric, ENT and dental treatment facilities. Esenlik et al. reviewed the literature on 3. Feeding problems: nasoalveolar molding (NAM) with an eye to both benefits and A child with a cleft palate can have difficulty sucking through a limitations. A review of the literature suggests that NAM Cleft lip regular nipple due to the gap in the roof of the mouth. An infant’s and palate children benefit from team approach special treatment ability to suck is related to two factors: the ability of the external requirements. such a team lead by the plastic surgeon should lips to perform the necessary sucking movements and the ability include a speech therapist and orthodontist having ready access of the palate to allow the necessary build‑up of pressure inside to pediatric, ENT and dental treatment facilities does not alter the mouth so that foodstuff can be propelled into the mouth. skeletal facial growth when compared with the samples that did Most babies require a personalized or special nipple to properly not receive PSIO (Presurgical infant orthopedics). Nevertheless, feed. It may take a couple of days for the baby and parents to the published studies on NAM show evidence of benefits to adjust to using the nipple before going home. Most babies learn the patient, caregivers, the surgeon, and society. These benefits to feed normally with a cleft palate nipple. include documented reduction in severity of the cleft deformity prior to surgery and as a consequence improved surgical Treatment of Cleft Lip And Palate outcomes, reduced burden of care on the caregivers, reduction in the need for revision surgery and consequent reduced overall This correction involves surgically producing a face that does not cost of care to the patient and society. Robotic cleft surgery attract attention, a vocal apparatus that permits intelligible speech is a new and exciting field that holds numerous advantages to and a dentition that allows optimal function and aesthetics. The both patients and surgeons. Previous research in allied health cleft palate team concept has evolved from that need. Because specialities has paved the way to the feasibility studies of robotic optimal care is best achieved by multiple types of clinical cleft surgery. Finally, the use of surgical robots at present Journal of Family Medicine and Primary Care 2624 Volume 9 : Issue 6 : June 2020  Vyas, et al.: Cleft of lip and palate introduces economic challenges to implementation because of races: A review. Cleft Palate J 1987;24:216‑25. increased operative time and high capital and operating costs and 12. Berkowitz S. cleft lip and palate diagnosis and management. it is hoped that over time, costs will reduce and performance will 2nd ed. Springer; 2005. increase as more systems are developed in the future. 13. Lakhanpal M, Gupta N, Rao N, Vashishth S. Genetics of cleft lip and palate‑ is it still patchy. JSM Dent 2014;2:1‑4. Summary and Conclusion 14. Kohli S, Kohli V. A comprehensive review of genetic basis of cleft lip and palate. J Oral Maxillofac Pathol 2012;16:64‑72. The perfect thing to do for CLP is certainly to prevent its 15. Marwah N. Textbook of Pediatric Dentistry. 3rd ed. Jaypee: occurrence in the first place. The primary aim in CLP is to educate 2014. parents and future mothers and fathers. Cleft lip and palate are 16. Bille C, Skytthe A, Vach W, Knudsen LB, Andersen AM, both birth defects that affect different structure and function Murray JC, et al. Parent’s age and risk of oral clefts. Epidemiology 2005;16:311‑6. such as speech difficulty, aesthetic, eating, nutrition etc. Patients 17. Sousa A, Devare S, Ghanshani J. Psychological issues in cleft with oro‑facial cleft deformity needs to be treated at right time lip and palate. J Indian Assoc Pediatr Surg 2009;14:55‑58. and at right age to achieve functional and aesthetic well‑being. 18. Lorente C, Miller S. Vitamin A induction of cleft palate. Cleft The mental status of patients with CLP should be considered Palate J 1978;15:378‑85. and supported by psychological rehabilitation and their morale 19. Grow JL, Lehman JA. A local perspective on the initial should always be bolstered. Extensive dental treatment may be management of children with cleft lip and palate by primary requires but it should not be made more extensive or complex care physicians. Cleft Palate Craniofac J 2002;39:535‑40. than is necessary to achieve a reasonable standard of dental 20. Mitchell JC, Robert Wood RJ. Management of cleft lip and perfection. The multidisciplinary approach towards this problem palate in primary care. J Pediatr Health Care 2000;14:13‑9. led to a steady improvement in its end results. 21. Kadam M, Kadam D, Bhandary S, Hukkeri R. Natal and neonatal teeth among cleft lip and palate infants. Natt J Financial support and sponsorship Maxillofac Surg 2013;4:73‑6. Nil. 22. Al Jamal GA, Hazza’a AM, Rawashdeh MA. Prevalence of dental Anomalies in a population of cleft lip and palate patients. Cleft Palate Craniofac J 2010;47:413‑20. Conflicts of interest 23. Qureshi WA, Beiraghi S, Salazar VL. 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