Accessibility / Report Error

A Descriptive Epidemiology Study of Oral Cleft in Sergipe, Brazil

Andrea Luiza Diego Noronha de Góis Jadson Alípio Santana de Sousa Santos Rosany Larissa Brito de Oliveira Luiz Carlos Ferreira da Silva About the authors

Abstract

Introduction

 The nonsyndromic orofacial cleft is the fourth most common birth defect, but in Brazil, data about the cleft epidemiology are not accurate.

Objective

 This study aimed to describe the epidemiologic characteristics of oral cleft cases at Specialized Society Attending Cleft Patient in Sergipe State.

Methods

 Data were obtained from patients' medical records in relation to the following characteristics: age; gender; race; origin; cleft type; additional malformations and/or complications; prenatal accomplishment; treatment applied. For diagnosis analysis, it was noted if mothers had received prenatal care and if they had ultrasonography performed and if the cleft was viewed in it.

Results

 We observed a prevalence of male gender (54%). Age between 0 and 4 years old was most prevalent (53%), and pheoderma race was observed in 47%. Transincisive foramen cleft was found in 52.3% of the individuals. The prevalence of pre- and transincisive foramen cleft was higher in men (66.3 and 55.7%), women accounted for 65.0% of postincisive foramen, and atypical facial cleft (0.3%) occurred in one case. Associated malformations and complications were present in 12% of patients. Prenatal care was reported by 48% of the mothers.

Conclusion

 In this study male gender was the most affected, and 0 to 4 years was the most frequent age group. Transincisive foramen cleft type was most frequently encountered. Prenatal care was reported by most mothers. So, this study found that early treatment is a reality in SEAFESE (Service Specializing in Cleft Care of Sergipe), and consequently the chances of successful integration of the child to society will be better.

Keywords
cleft lip; cleft palate; epidemiology


Introduction

The nonsyndromic orofacial cleft is the fourth most common birth defect, besides being the most observed craniofacial malformation.11 Cobourne MT. The complex genetics of cleft lip and palate. Eur J Orthod 2004;26:7-16 , 22 Munz SM, Edwards SP, InglehartMR. Oral health-related quality of life, and satisfaction with treatment and treatment outcomes of adolescents/young adultswith cleft lip/palate: an exploration. Int J Oral Maxillofac Surg 2011;40:790-796 Although its etiology still remains unclear, most likely both exogenous (teratogenic) and endogenous (genetic) factors contribute to this embryopathy in humans.33 Donkor P, Plange-Rhule G, Amponsah EK. A prospective survey of patients with cleft lip and palate in Kumasi. West Afr J Med 2007;26:14-16 , 44 Poletta FA, Castilla EE, Orioli IM, Lopez-Camelo JS. Regional analysis on the occurrence of oral clefts in South America. Am J Med Genet A 2007;143A:3216-3227 In addition, it is a major oral health problem in the world and its treatment requires specialized multiprofessional and integrated actions.55 Tan KB, Tan KH, Yeo GS. Cleft deformities in Singapore: a populationbased series 1993-2002. Singapore Med J 2008;49:710-714

Several studies of the epidemiologic patterns of cleft deformities show a marked variability in the frequency of this anomaly, ranging from 1 in 500 to 1 in 2,500 live births.55 Tan KB, Tan KH, Yeo GS. Cleft deformities in Singapore: a populationbased series 1993-2002. Singapore Med J 2008;49:710-714 In Brazil, data about cleft lip and/or palate epidemiology are not accurate; however, the incidence of this malformation is 0.36 in 1,000 live births. In northeastern Brazil, the incidence is 0.39 in 1,000 live births.66 Freitas e Silva DS,Mauro LDP, Oliveira LB, et al. Estudo descritivo de fissuras lábio-palatinas relacionadas a fatores individuais, sistêmicos e sociais. RGO 2008;56(4):387-391 , 77 Rodrigues K, Sena MF, Roncalli AG, Ferreira MA. Prevalence of orofacial clefts and social factors in Brazil. Braz Oral Res 2009;23: 38-42 In Sergipe, approximately 22 new cases of cleft lip and/or palate occur each year.88 Ministério da Saúde. Brazil: DATASUS. Available at: http://tabnet. datasus.gov.br/cgi/tabcgi.exe?sinasc/cnv/nvuf.def.Accessed March 22, 2012
Available at: http://tabnet. ...
The SEAFESE (Service Specializing in Cleft Care of Sergipe) is a public interest civil social organization formed by a multidisciplinary team comprised of 13 professionals in the following specialties: plastic surgery, maxillofacial surgery, nurse, speech therapy, dentistry, orthodontics, psychology, social work, and anesthesiology. All surgeries performed within SEAFESE follow a protocol created by its professionals to obtain the best results for patients with cleft (Table 1).

Table 1
Surgical treatment protocol followed by SEAFESE

According to that, the most detailed information about these birth defects in populations is essential to recognize and prevent the problem, and, consequently, to plan assistance policies in general. Thus, the aim of this study was to characterize the cases of cleft lip and palate based on the medical records of patients treated at the SEAFESE.

Methods

For the purpose of this study, a descriptive cross-sectional design was adopted, based on medical records of patients treated at the SEAFESE. It included all the medical records of patients attended at SEAFESE from October 2003 to June 2007. Data not reported on medical record were considered as “not defined.”

All medical records were analyzed, regarding (1) demographic characteristics (gender, race, age, origin); (2) cleft diagnosis and classification (cleft type, associated malformations and complications, prenatal accomplishment); and (3) treatment applied.

The variable age was divided into 6 groups with an interval of 4 years for each group according to the classification made by the Brazilian Institute of Geography and Statistics, Population Census, 2000.99 IBGE. Instituto Brasileiro de Geografia e Estatística. Regiões de Influência das Cidades. 2007. Available at: http://www.ibge.gov. br/home/geociencias/geografia/regic.shtm. Accessed May 5, 2012
Available at: http://www.ibge.gov. ...
Patients were classified according to race as leukoderma (white), pheoderma (brown), xanthoderma (yellow), and melanoderma (black); according to the origin, they were grouped based on where they live (metropolis, countryside, and other states).

Cleft classification was based on the work of Spina et al,1010 Spina V, Psillakis JM, Lapa FS, Ferreira MC. Classificação das fissuras lábio-palatinas. Sugestão de modificação. Rev Hosp Clin Fac Med Sao Paulo 1972;27:5-6 which defines four groups: preincisive foramen cleft (uni- or bilateral, median); transincisive foramen cleft (uni- or bilateral); postincisive foramen clefts; and atypical (rare) facial clefts.

For diagnosis analysis, it was noted if mothers received prenatal care and if they had ultrasonography done and if the cleft was viewed in it. Regarding treatment, only surgeries to repair cleft lip and palate (lip repair and palatoplasty, respectively) were considered in this study; additional surgeries performed in patients with cleft were included as “other surgeries.”

The collected data were tabulated and calculated in Excel for Windows 2003, and the data are presented in absolute frequencies and percentages.

This study was approved by the Committee on Ethics in Research Involving Humans, from Federal University of Sergipe (CEP-UFS/CAAE No.: 0082.0.107.000–07).

Results

A total of 350 medical records were achieved. The general analysis of the data allows tracing back the profile of participants, as shown in Table 2. In relation to gender, 54.0% of patients who comprised the sample were male; the distribution of patients by age focused predominantly between 0 and 14 years (77.4%), with a higher prevalence in the age group of 0 to 4 years. Regarding race, 165 patients (47.2%) were pheoderma and 146 (41.7%) leukoderma. Finally, most of them were from the countryside (58.0%).

Table 2
Distribution of oral cleft cases by gender, age group and race

Table 3 shows patient distribution according to cleft type and gender. The most frequent type of cleft was the transincisive foramen, with 183 cases (52.3%), and this cleft was more frequent in males, with 102 cases (55.7%). The second most frequent type of cleft was the preincisive foramen, with 89 cases (25.4); this cleft was also more frequent in males, with 59 cases (66.3%). The third most frequent type of cleft was the postincisive foramen, with 77 cases (22.0%), and this cleft occurred more often in females with 50 cases (65.0%). Finally, the atypical (rare) facial cleft was less frequent, with only 1 case (0.3%), a male.

Table 3
Distribution of patients according to cleft type and gender

Among the 350 medical reports analyzed, only 41 (12.0%) showed data regarding malformations and additional complications in the patients, without specifying. Prenatal care was reported by almost half the sample (168; 48.0%), and only 26 mothers (7.0%) reported on prenatal care. In 156 records (45.0%), this information was missing. According to information provided by patients to fill the medical record, only in 7 cases (4.2%) of 168 referred to prenatal care did the ultrasonography reveal the cleft; in 107 cases (63.7%), nothing was detected, and in 54 (32.1%) there was no information (not defined).

Associated malformations and complications occurred in 41 individuals, more frequently in men (26 subjects; 63.4%). The associated malformations and complications can be seen in Table 4.

Table 4
Associated malformations and complications

Since 2003, when the service was available, 173 surgeries were performed in patients with cleft (Table 5): 77 cheiloplasty, 79 palatoplasty, and 17 other surgeries (fistuloplasty, rhinoplasty, otoplasty). The highest rate was recorded in 2004 (69 surgeries) and the lowest in 2007 (3 surgeries). This decrease in the number of surgeries was due to a change in SEAFESE head office.

Table 5
Distribution of patients according to the treatment over the years

Table 6 shows the distribution of patients by age at initial assessment in SEAFESE and treatment accomplished. Demand for service was greater in the first 4 years of life; consequently, due to surgical protocol adopted by the institution, lip repair was the most performed surgery.

Table 6
Patient's age at initial assessment in SEAFESE and treatment accomplished

Discussion

This study showed a demand for an early access to the service, especially during the first 4 years of life (Table 1); the literature is in consensus that the primary surgery for cleft lip and/or palate repair is usually performed in all children before 2 years of age.1111 Kramer FJ, Gruber R, Fialka F, Sinikovic B, Hahn W, Schliephake H. Quality of life in school-age childrenwith orofacial clefts and their families. J Craniofac Surg 2009;20:2061-2066 The high concentration of patients attending in this service within this age group suggests that people are getting more conscious about the services directed to cleft treatment. Moreover, the early access to specialist services allows the structuring of a safer and easier treatment plan with a more favorable prognosis, as well as consequent and significant aesthetic and functional gains that will resonate on the quality of life, reflecting on the family.

The demographic profile of the population studied is an individual male (54.0%), pheoderma (47.2%), between 0 and 14 years (77.4%), who lives in the countryside (58.0%). In other studies similar results were observed, where the majority of subjects with oral clefts were males,1212 Ajike SO, Adebola RA, Efunkoya A, Adeoye J, Akitoye O, Veror N. Epidemiology of adult cleft patients in North-western Nigeria: our experience. Ann Afr Med 2013;12:11-15

13 Martelli DR, Machado RA, Swerts MS, Rodrigues LA, Aquino SN, Martelli Júnior H. Non syndromic cleft lip and palate: relationship between sex and clinical extension. Braz J Otorhinolaryngol 2012;78:116-120
- 1414 Costa CH, Diniz LV, Lacerda RH, Forte FD, Sampaio FC. Prevalence of dental anomalies in patients with cleft lip and palate, Paraiba, Brazil: clinic and radiographic study. Acta Odontol Latinoam 2012;25:181-185 lived in metropolitan areas, and were brown.1515 Baptista EVP. Malformações congênitas associadas à fissura labial e/ou palatal em pacientes atendidos em um serviço de referência para tratamento de defeitos da face: um estudo de série de casos [dissertation]. Recife, Brazil: Instituto Materno Infantil Prof. Fernando Figueira; 2007:67

The data showed the prevalence of transincisive foramen cleft. These results are similar to a study conducted in Recife, where a predominance of the transincisive foramen cleft (49%) was observed, followed by postforamen (27%) and preforamen (24%) clefts.1515 Baptista EVP. Malformações congênitas associadas à fissura labial e/ou palatal em pacientes atendidos em um serviço de referência para tratamento de defeitos da face: um estudo de série de casos [dissertation]. Recife, Brazil: Instituto Materno Infantil Prof. Fernando Figueira; 2007:67 Freitas et al observed in São Paulo that the most common types of cleft lip and/or palate were trans- and postforamen (31.7%), followed by preforamen (28.4%) and rare fissure (3.8%).1616 Freitas JA, Dalben G da S, Santamaria M Jr, Freitas PZ. Current data on the characterization of oral clefts in Brazil. Braz Oral Res 2004;18:128-133 In other countries a similar prevalence was observed. In Germany, Kramer et al found that the transforamen (42.4%) cleft was most often observed, followed by preforamen (28.8%) and postforamen (28.8%) clefts.1111 Kramer FJ, Gruber R, Fialka F, Sinikovic B, Hahn W, Schliephake H. Quality of life in school-age childrenwith orofacial clefts and their families. J Craniofac Surg 2009;20:2061-2066 Sagheri et al, also in Germany, found that the transforamen (45.9%) cleft was observed most often, followed by postforamen (41%) and preforamen (9.8%) clefts and Pierre-Robin syndrome (3.3%).1717 Sagheri D, Ravens-Sieberer U, Braumann B, von Mackensen S. An Evaluation of Health-Related Quality of Life (HRQoL) in a group of 4-7 year-old children with cleft lip and palate. J Orofac Orthop 2009;70:274-284

The cleft lip and/or palate may be present as isolated deformities (or nonsyndromic clefts) or within the phenotype of a syndrome, called syndromic clefts.1515 Baptista EVP. Malformações congênitas associadas à fissura labial e/ou palatal em pacientes atendidos em um serviço de referência para tratamento de defeitos da face: um estudo de série de casos [dissertation]. Recife, Brazil: Instituto Materno Infantil Prof. Fernando Figueira; 2007:67 Several studies suggest that 30 to 40% of cases occur as a pattern of multiple malformations and are classified as a known syndrome related to chromosomal alterations, Mendelian disorder, or exposure to a known teratogen.1818 Dixon MJ, Marazita ML, Beaty TH, Murray JC. Cleft lip and palate: understanding genetic and environmental influences. Nat Rev Genet 2011;12:167-178 In this research, associated malformations and complications occurred in 41 individuals, and the data found in this study agree with the findings of Baptista,1515 Baptista EVP. Malformações congênitas associadas à fissura labial e/ou palatal em pacientes atendidos em um serviço de referência para tratamento de defeitos da face: um estudo de série de casos [dissertation]. Recife, Brazil: Instituto Materno Infantil Prof. Fernando Figueira; 2007:67 Milerad et al,1919 Milerad J, Larson O, PhD D, Hagberg C, Ideberg M. Associated malformations in infants with cleft lip and palate: a prospective, population-based study. Pediatrics 1997;100(2 Pt 1):180-186 Ellis,2020 Ellis E. Cirurgia Oral e Maxilofacial Contemporânea. 4th ed. Rio de Janeiro, Brazil: Elsevier; 2005 and Nunes et al.2121 Nunes LMN, Queluz DP, Pereira AC. Prevalência de fissuras labiopalatais no município de Campos dos Goytacazes-RJ, 1999-2004. Rev Bras Epidemiol 2007;10(1):109-116

Data about prenatal care outlined in this study showed accompanying of almost half the analyzed sample and only 7.0% stated no kind of accompanying. This prevalence is lower than that found in other countries. In France the prenatal diagnosis of cleft occurs in 62.8% of cases,2222 Guyot A, Soupre V, VazquezMP, et al. [Prenatal diagnosis of cleft lip with or without cleft palate: retrospective study and review]. J Gynecol Obstet Biol Reprod (Paris) 2013;42:151-158 in the United States the prevalence is 16%,2323 Matthews MS, Cohen M, Viglione M, Brown AS. Prenatal counseling for cleft lip and palate. Plast Reconstr Surg 1998;101:1-5 and in England the prevalence is 30%.2424 Davalbhakta A, Hall PN. The impact of antenatal diagnosis on the effectiveness and timing of counselling for cleft lip and palate. Br J Plast Surg 2000;53:298-301 Most parents who have a prenatal diagnosis felt that the diagnosis prepared them psychologically for the birth of the child with cleft.2424 Davalbhakta A, Hall PN. The impact of antenatal diagnosis on the effectiveness and timing of counselling for cleft lip and palate. Br J Plast Surg 2000;53:298-301

Such data suggest that the primary health care policy adopted by the government, at least in terms of awareness, has been characterized by reforming the potential of primary care in favor of better health outcomes, which caused significant changes in health practice, even though 156 (45.0%) medical records do not have report data about prenatal care.

Although the increased demand for care in SEAFESE is by children between 0 and 4 years, with a high rate of newborns who have not been subjected to any type of surgery, there was a high number of patients who reported at first visit having already had previous surgery to correct cleft lip and/or palate in another institution. Besides, it was found that in the 0- to 4-year group, 44 surgeries had already occurred, followed by 40 surgeries in the 15- to 19-year group. These data confirm that the demand for treatment of cleft patients is taking place at the right time, which is in the first months/years of life.

Conclusions

In conclusion, the few epidemiologic studies on cleft in Brazil highlight the importance and need for studies that address determinants of developmental defects, such as oral cleft. In this study, male gender was the most affected, and the age group 0 to 4 years was the most frequent. Transincisive foramen cleft type was most frequently found. Prenatal care was reported by most mothers. So, this study found that early treatment is a reality in SEAFESE; therefore the chances of successful integration of the child to society will be better.

References

  • 1
    Cobourne MT. The complex genetics of cleft lip and palate. Eur J Orthod 2004;26:7-16
  • 2
    Munz SM, Edwards SP, InglehartMR. Oral health-related quality of life, and satisfaction with treatment and treatment outcomes of adolescents/young adultswith cleft lip/palate: an exploration. Int J Oral Maxillofac Surg 2011;40:790-796
  • 3
    Donkor P, Plange-Rhule G, Amponsah EK. A prospective survey of patients with cleft lip and palate in Kumasi. West Afr J Med 2007;26:14-16
  • 4
    Poletta FA, Castilla EE, Orioli IM, Lopez-Camelo JS. Regional analysis on the occurrence of oral clefts in South America. Am J Med Genet A 2007;143A:3216-3227
  • 5
    Tan KB, Tan KH, Yeo GS. Cleft deformities in Singapore: a populationbased series 1993-2002. Singapore Med J 2008;49:710-714
  • 6
    Freitas e Silva DS,Mauro LDP, Oliveira LB, et al. Estudo descritivo de fissuras lábio-palatinas relacionadas a fatores individuais, sistêmicos e sociais. RGO 2008;56(4):387-391
  • 7
    Rodrigues K, Sena MF, Roncalli AG, Ferreira MA. Prevalence of orofacial clefts and social factors in Brazil. Braz Oral Res 2009;23: 38-42
  • 8
    Ministério da Saúde. Brazil: DATASUS. Available at: http://tabnet. datasus.gov.br/cgi/tabcgi.exe?sinasc/cnv/nvuf.def.Accessed March 22, 2012
    » Available at: http://tabnet. datasus.gov.br/cgi/tabcgi.exe?sinasc/cnv/nvuf.def
  • 9
    IBGE. Instituto Brasileiro de Geografia e Estatística. Regiões de Influência das Cidades. 2007. Available at: http://www.ibge.gov. br/home/geociencias/geografia/regic.shtm. Accessed May 5, 2012
    » Available at: http://www.ibge.gov. br/home/geociencias/geografia/regic.shtm
  • 10
    Spina V, Psillakis JM, Lapa FS, Ferreira MC. Classificação das fissuras lábio-palatinas. Sugestão de modificação. Rev Hosp Clin Fac Med Sao Paulo 1972;27:5-6
  • 11
    Kramer FJ, Gruber R, Fialka F, Sinikovic B, Hahn W, Schliephake H. Quality of life in school-age childrenwith orofacial clefts and their families. J Craniofac Surg 2009;20:2061-2066
  • 12
    Ajike SO, Adebola RA, Efunkoya A, Adeoye J, Akitoye O, Veror N. Epidemiology of adult cleft patients in North-western Nigeria: our experience. Ann Afr Med 2013;12:11-15
  • 13
    Martelli DR, Machado RA, Swerts MS, Rodrigues LA, Aquino SN, Martelli Júnior H. Non syndromic cleft lip and palate: relationship between sex and clinical extension. Braz J Otorhinolaryngol 2012;78:116-120
  • 14
    Costa CH, Diniz LV, Lacerda RH, Forte FD, Sampaio FC. Prevalence of dental anomalies in patients with cleft lip and palate, Paraiba, Brazil: clinic and radiographic study. Acta Odontol Latinoam 2012;25:181-185
  • 15
    Baptista EVP. Malformações congênitas associadas à fissura labial e/ou palatal em pacientes atendidos em um serviço de referência para tratamento de defeitos da face: um estudo de série de casos [dissertation]. Recife, Brazil: Instituto Materno Infantil Prof. Fernando Figueira; 2007:67
  • 16
    Freitas JA, Dalben G da S, Santamaria M Jr, Freitas PZ. Current data on the characterization of oral clefts in Brazil. Braz Oral Res 2004;18:128-133
  • 17
    Sagheri D, Ravens-Sieberer U, Braumann B, von Mackensen S. An Evaluation of Health-Related Quality of Life (HRQoL) in a group of 4-7 year-old children with cleft lip and palate. J Orofac Orthop 2009;70:274-284
  • 18
    Dixon MJ, Marazita ML, Beaty TH, Murray JC. Cleft lip and palate: understanding genetic and environmental influences. Nat Rev Genet 2011;12:167-178
  • 19
    Milerad J, Larson O, PhD D, Hagberg C, Ideberg M. Associated malformations in infants with cleft lip and palate: a prospective, population-based study. Pediatrics 1997;100(2 Pt 1):180-186
  • 20
    Ellis E. Cirurgia Oral e Maxilofacial Contemporânea. 4th ed. Rio de Janeiro, Brazil: Elsevier; 2005
  • 21
    Nunes LMN, Queluz DP, Pereira AC. Prevalência de fissuras labiopalatais no município de Campos dos Goytacazes-RJ, 1999-2004. Rev Bras Epidemiol 2007;10(1):109-116
  • 22
    Guyot A, Soupre V, VazquezMP, et al. [Prenatal diagnosis of cleft lip with or without cleft palate: retrospective study and review]. J Gynecol Obstet Biol Reprod (Paris) 2013;42:151-158
  • 23
    Matthews MS, Cohen M, Viglione M, Brown AS. Prenatal counseling for cleft lip and palate. Plast Reconstr Surg 1998;101:1-5
  • 24
    Davalbhakta A, Hall PN. The impact of antenatal diagnosis on the effectiveness and timing of counselling for cleft lip and palate. Br J Plast Surg 2000;53:298-301

Publication Dates

  • Publication in this collection
    2013

History

  • Received
    22 Oct 2012
  • Accepted
    06 June 2013
Fundação Otorrinolaringologia R. Teodoro Sampaio, 483, 05405-000 São Paulo/SP Brasil, Tel.: (55 11) 3068-9855, Fax: (55 11) 3079-6769 - São Paulo - SP - Brazil
E-mail: iaorl@iaorl.org