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Authors: Dr. Shah Aakash M, Dr. Shah Purvesh M, Dr. Shah Romil B

Abstract

The most severe expression of the congenital clefts of the lip and palate (CLP) is the bilateral cleft lip and palate (BCLP), which runs through the lip, the hard, as well as the soft palate. Congenital clefts of the lip and palate (CLP) constantly raise high research interest not only because the treatment of these defects still presents a major clinical problem, but also because they interfere with two of the most important human means of communication: facial expression and speech.

Keywords: Cleft lip and palate, Surgery, Growth

Introduction

Cleft lip and palate (CLP) is the most common craniofacial anomaly. CLP is categorized into different groups. The classification system varies depending on the purpose. [1] Extra-oral clefts (one-sided or bilateral) involve the upper lip and often the base of the nose. The extra-oral cleft can be combined with a cleft in the alveolar process with or without involving the palate. The severity of the condition and difficulty in treatment of the same type of cleft varies. The inherited growth pattern is a factor that may affect the final treatment outcome. The bilateral cleft lip and palate (BCLP) is a cleft that involves the base of the nose, the upper lip, and the alveolar process through the hard and soft palate (Fig. 1A, 1B). The isolated cleft palate (iCP) is not affecting the facial appearance since it only involves a secondary palate. It can either be a total or a partial cleft. The total iCP is a palatal cleft with an origin directly behind the foramen incisivum region. It extends all the way back through the hard and soft palate. A partial cleft palate sometimes partly involves the hard palate, and extends all through the soft palate. At times, only the soft palate is affected by the cleft.

CLP related research has been pushing forward for decades. However, there is still little known about etiology, the life situations for persons born with a cleft and so on.

Etiology

BCLP or iCP could be either a feature in syndromes or isolated ones, so-called non-syndromic cleft. Causes of non-syndromic CLP still remain unclear. 2 Many studies have suggested a relation between some maternal habits during the first tri-semester of pregnancy and the development of a cleft. There is strong evidence that maternal smoking is linked with an increased risk of having CLP. 3, 4 Regarding nutrition, multivitamin supplements are not confirmed as being a protective factor. 5, 6 Some animal studies have shown that a nutrient deficiency of folic acid and Zinc increased the risk of having a cleft.7-9 Exposures to some chemical substances; for example, agricutural chemicals, retinoids (Vitamin A derivatives), corticosteriods, some anticonvulsants drugs e.g. diazepam, carbamazipine, phenobarbital, or a virus infection also increases the risk for clefts and other congenital malformations.10-13

Genetic factors are also important. In non-syndromic CLP, some growth factors and metabolic enzymes such as TGFA, TGFß3 and MTHFR have been intensively studied. 14-17 Hereditary patterns have been described for families with syndromes e.g. the van der Woude syndrome.

The gene IRF6, TBX22 and PVRL1, which causally associates with syndromes, also links to an occurrence of a non- syndromic CLP. 18-20 It leads to a hypothesis that any gene which relates to any syndromic CLP could possibly induce the risks of having non-syndromic CLP. 14 Spontaneous mutations are likely to keep the incidence values for rare syndromes, with clefts as a feature, at a rather stable level throughout decades. The research in gene-disease association is still a wide challenging field for future research.

Epidemiology of CLP

From the available data, cleft lip with or without cleft palate was reported at high rates in some parts of Latin America, China, and Japan. It was found in low rates in Israel and Southern Europe. Regarding iCP, the rates were high in Canada, some Scandinavian countries, but low in Latin America. South Africa had low rates of both types of cleft.2 However, the number of iCP might be underestimated since the disease is not externally visible. The global data is hardly comparable due to several reasons. The differences in collecting methods, sample sources, inclusion/exclusion criteria, and others, restrict the comparability.21

Cleft related problems: Feeding, ear infections and speech development

A person born with a CLP encounters problems from birth. Breathing problems are common in a combination of an iCP and an underdeveloped mandible, the Pierre Robin sequence. 22 A connection between oral and nasal cavity reduces the sucking ability. Feeding problems or general weakness may disturb normal growth and development. Instructions and exercises of feeding position and using a special feeding bottle might be needed. 23 When the feeding problems are severe, a special feeder such as a naso-gastric tube and syringe may be advised. Recurrent ear infections are common and associated with pain, loss of hearing and delayed speech development.24

The cleft involving the palate mostly affects speech to various degrees. The Velopharyngeal function and the length of the soft palate are important factors that relate to speech problems. 25 Often there are problems in constructing a soft palate long enough to prevent the air flow passing from the pharyngeal area up to the nose, thereby affecting the speech. A small partial palatal cleft is easier to treat than a very extensive cleft. Sometimes the speech is affected, but most often young adults develop a normal speech. However, speech training and additional surgical corrections may be needed in order to normalize the speech. 26

Despite being complete or partial, the BCLP is considered to be the most challenging type of cleft, in order to receive a good result from both an esthetical point of view and in terms of developing a normal speech.

Social and psychological impact

The family situation is obviously different from that of a family with a healthy infant. When a newborn infant is diagnosed as having a cleft, several different emotions may possibly come into existence. Shock, anxiety, guilt and/or disappointment are common feelings. 27 Inappropriate family adaptation could psychologically affect children with CLP. Parents sometimes turn to protective behaviors.28

Living in a city or rural area can make a difference on how other people address a person who has been treated for a CLP. Parents, family, friends, teachers and the school system are factors in a society that may have influence on a person’s self- esteem. Social interactions are other factors related to a person’s appearance. 29 Compared to children without craniofacial conditions, children with CLP received less positive responses and avoided longer conversations with their friends. 30 They showed problems, reported by parents or teachers, with depression, anxiety, unhappiness and behavioral problems. 31 There is individual variation in adjusting to living with a visible deviation. Some manage to cope but others become psychologically distressed.33 A similar situation of living with a visible deviation appears with persons with CLP. They could, to some extent, have difficulties regarding the severity of psychological problems.34 Some studies have suggested that persons with treated CLP showed no evidence of severe psychological problems. However, it is obvious that even in the completely treated cases of CLP, the scars remain visible.

Conclusion:

In conclusion, habilitation of patients with BCLP proves to be a very controversial area and demanding process, as seen both from a treatment as well as from a scientific point of view. Although no consensus has been reached on the efficacy and the effectiveness of the different treatment modalities applied, it is evident how tremendously important it becomes to offer centralized treatment to these suffering patients, based on team work of closely cooperating medical and dental specialties.

References:
  1. Shprintzen, R. J. (2002). Terminology and classification of facial clefting. In M. P. Mooney & M. I. Siegel (Eds.), Understanding craniofacial anomalies: The etiopathogenesis of craniosynostoses and facial clefting (pp. 17-28): New York : Wiley-Liss, cop.
  2. Mossey, P., Little, J., Munger, R. G., Dixon, M. J., & Shaw, W. C. (2009). Cleft lip and palate. Lancet, 374(9703), 1773-1785.
  3. Wyszynski, D. F., Duffy, D. L., & Beaty, T. H. (1997). Maternal cigarette smoking and oral clefts: a meta-analysis. Cleft Palate Craniofac J, 34(3), 206-210.
  4. Little, J., Cardy, A., & Munger, R. G. (2004). Tobacco smoking and oral clefts: a metaanalysis. Bull World Health Organ, 82(3), 213-218.
  5. Loffredo, L. C., Souza, J. M., Freitas, J. A., & Mossey, P. A. (2001). Oral clefts and vitamin supplementation. Cleft Palate Craniofac J, 38(1), 76-83.
  6. Goh, Y. I., Bollano, E., Einarson, T. R., & Koren, G. (2006). Prenatal multivitamin supplementation and rates of congenital anomalies: a meta-analysis. J Obstet Gynaecol Can, 28(8), 680-689.
  7. Warkany, J., & Petering, H. G. (1972). Congenital malformations of the central nervous system in rats produced by maternal zinc deficiency. Teratology, 5(3), 319-334.
  8. Bienengraber, V., Malek, F. A., Moritz, K. U., Fanghanel, J., Gundlach, K. K., & Weingartner, J. (2001). Is it possible to prevent cleft palate by prenatal administration of folic acid? An experimental study. Cleft Palate Craniofac J, 38(4), 393-398.
  9. Malek, F. A., Moritz, K. U., Fanghanel, J., & Bienengraber, V. (2004). Reduction of procarbazine-induced cleft palates by prenatal folic acid supplementation in rats. Pathol Res Pract, 200(1), 33-40.
  10. Dolovich, L. R., Addis, A., Vaillancourt, J. M., Power, J. D., Koren, G., & Einarson, T. R. (1998). Benzodiazepine use in pregnancy and major malformations or oral cleft: meta-analysis of cohort and case-control studies. BMJ, 317(7162), 839-843.

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