|Year : 2019 | Volume
| Issue : 4 | Page : 200-206
Pattern of malocclusion in children living with sickle cell anemia
Orighoye Tosan Temisanren1, Babatunde Oludare Fakuade2, Fadekemi Adetutu Ajibade1, Saleh Yuguda3, Ahmed Girei3
1 Department of Child Oral Health, University of Ibadan, Ibadan, Nigeria
2 Department of Dental and Maxillofacial Surgery, Federal Teaching Hospital, Gombe, Nigeria
3 Department of Haematology and Blood Transfusion, Gombe State University, Gombe, Nigeria
|Date of Submission||01-Sep-2018|
|Date of Acceptance||22-Mar-2019|
|Date of Web Publication||29-Nov-2019|
Dr. Orighoye Tosan Temisanren
Department of Child Oral Health, University of Ibadan, Ibadan
Background: Sickle cell anemia is a hereditary disorder of the hematological system. It involves the production of abnormal hemoglobin, which is sickle in shape and has a short life span, resulting in secondary hemopoietic function by the long bones and the jaw bones. The disorder burden not only presents with systemic affectations and morbidity that is commonly observed but also has immense effects on the jaw bones presenting as malocclusion and in severe cases causing gnathopathy and psychosocial disturbance, especially with esthetics. Other dental effects include functional disturbances and diseases of the oral tissue. Objective: The aim of this study is to determine the occlusal pattern of children with sickle cell anemia. Materials and Methods: Consecutive patients attending the Sickle Cell Clinic of the department of Hematology, Federal Teaching Hospital Gombe, aged 6 to 16years. The inclusion criteria included individuals who have been diagnosed to have HbSS using electrophoresis. Sociodemographic data and oral examination were carried out, and dental and occlusal parameters were recorded in a data collection form. Data were analyzed using the SPSS software version 19. The level of statistical significance was set at P < 0.05. Results: The mean age was 10.12 ± 3.10 years. Ninety (55.6%) patients were male. The samples were grouped into two: Group I ages 6–10 years comprising 92 children and ages 11–16 years comprising 70 children in Group II. Angle's Class I molar relationship was observed to be 86 (93.5%) and 57 (81.4%), respectively, for Groups I and II. Overjet and overbite were found to be increased in 34 (37.0%) and 5 (5.4%) for Group I and 23 (32.9%) and 5 (7.1%) for Group II, respectively. Conclusion: The burden of sickle cell anemia is not limited to systemic manifestations alone. Dental presentations that could affect psychosocial, function, health of oral tissues, and speech have also been observed. Therefore, it is expedient that these individuals on presentation to the clinic are examined in a holistic manner with a view to attending to all their presenting problems at an early stage.
Keywords: Malocclusion, sickle cell anemia, teeth
|How to cite this article:|
Temisanren OT, Fakuade BO, Ajibade FA, Yuguda S, Girei A. Pattern of malocclusion in children living with sickle cell anemia. Sahel Med J 2019;22:200-6
|How to cite this URL:|
Temisanren OT, Fakuade BO, Ajibade FA, Yuguda S, Girei A. Pattern of malocclusion in children living with sickle cell anemia. Sahel Med J [serial online] 2019 [cited 2020 Feb 22];22:200-6. Available from: http://www.smjonline.org/text.asp?2019/22/4/200/272143
| Introduction|| |
Malocclusion is a state where the teeth are malpositioned and are not in good occlusal harmony when the jaws are brought together. It is a global public health problem, and sickle cell anemia individuals are frequently presenting with malocclusion., The prevalence of malocclusion in Nigeria's general population, where sickle cell disease individuals constitute about 2.0%,, is between 76.0% and 88.0%.,, Mustapha and Abubakar  in their study of northern Nigerian sample observed a sickle cell prevalence of 11.87%. Sickle cell disease is a hereditary disorder that is characterized by the presence in the individual's genotype of at least one sickle gene (HbS) making up half (50%) of their hemoglobin. When the genotype is made up of homozygotic HbSS, this is referred to as sickle cell anemia. Although the equivalent of about 25% of under-five-year-old deaths in Africa has been ascribed to sickle cell anemia, an increasing number of children are now surviving beyond 5 years with better and improved management. In the process of trying to cater for the short life of the red blood cells, compensatory mechanisms associated with hyperplasia and expansion of bone marrow take place, and these result in changes in bony structures.
3The prevalence of sickle cell anemia in Nigeria is said to be on the increase, especially among the urban educated elite and in other communities with access to effective basic health care. With a steadily growing population like Nigeria, the likelihood of an exponential growth in the population of individuals with the sickle cell disorder will be high; hence, many of them may likely seek orthodontic intervention to improve their smile, function, and quality of life, and self-esteem as a result of increasing awareness of the possibility of managing their occlusal discrepancies through the use of various orthodontic appliances. This intervention is better carried out in the younger age group during the growth phase, where the jaw bones can be manipulated in favor of desired results and better outcome. Therefore, this study is aimed at investigating the presenting malocclusion and lip seal in children living with sickle cell anemia.
| Materials and Methods|| |
The study is a descriptive study of the pattern of malocclusion in individuals with sickle cell disease seen at the Sickle Cell Clinic of the Hematology Department of the Federal Teaching Hospital, Gombe, over a 6-month period. The study population comprised consecutive sickle cell patients, aged 6–16 years, who have been diagnosed using hemoglobin electrophoresis who met the inclusion criteria.
The inclusion criteria were:
- All HbSS patients who have been diagnosed by electrophoresis
- HbSS patients with no known other systemic diseases
- HbSS patients with no form of oral habit
- HbSS patients with full dental complement for age
- All HbSS patients who have not undergone any form of orthodontic treatment
- All HbSS patients with no abnormal tooth morphology or tooth crown loss
- All HbSS patients who have consented and willing to be part of the study.
General consent for the study was sought and obtained from the Ethical Committee of the Federal Teaching Hospital, Gombe (NHREC/25/10/2013). Both verbal and written consents were obtained from patients and or guardians, and only those who positively responded were examined and included in the study.
The sampling technique adopted due to the specificity of the population was a nonprobability (convenient) sampling of 184 consecutive sickle cell patients attending the sickle cell clinic. Eligible individuals had an oral examination done with gloved hands, facemask and a disposable dental explorer, dental mirror, and a meter rule after verbal consent and assent were obtained from both parent/guardian and child, respectively. Following assessment of all individuals for the inclusion criteria, only 162 met the criteria for the study and were recruited.
The recruited sample was stratified using their ages based on the World Health Organization criteria for epidemiological research in children into two groups: Group 1 ages 6–10.9 years and Group 2 ages 11–16 years. Demographic data such as age, gender, and tribe and orthodontic data such as teeth present, Angle's molar relationship, overjet, overbite, crossbite, dental anomalies, peg-shaped laterals, diastema, and lips line and seal. The lip line and seal were determined using Jackson's classification.,,, All data were documented following orofacial examinations using a structured data collection tool comprising two sections: Section A – for the demographic information and Section B –for the orthodontic assessment. All data were entered and analyzed using International Business Machines Corporation (IBM) Statistical Package for the Social Sciences (SPSS) United States of America (USA) version 19 statistical software. Frequency distribution of variables was generated, and a measure of central tendency was calculated to summarize the numerical data. The data generated were presented as tables and charts. The level of significance was set at P < 0.05.
| Results|| |
There were 162 children examined during the study period, and their age ranged from 6 to 16 years with a mean age of 10.12 ± 3.10 years. The total number of children was 90 (55.6%) males and 72 (44.4%) females. Children in the group 6–10 years and 11 months were 92 children (56.8%) and group 11–16 years were 70 children (43.2%) [Figure 1]. Majority of the children were from the Hausa tribe (63.0%) of northern Nigeria [Figure 2].
|Figure 1: Age frequency based on the World Health Organization stratification in children|
Click here to view
|Figure 2: Demographic distribution of children in whole population by gender and tribe|
Click here to view
Age group 6–10 years
Group 1 comprised 92 children, 52 (56.5%) males and 40 (43.5%) females with a mean age of 7.72 ± 1.34 years. Assessing their molar relationship reveals that 86 (93.5%) presented with Angle's Class I, while 6 (6.5%) presented with Angle's Class II molar relationships. Assessing their overjet, it was observed that 49 (53.3%) of the children had an overjet of between 2 and 4 mm (mm), which is considered to be normal. Thirty-four (37.0%) had an overjet that is >4 mm and 9 (9.8%) presented with overjet of <2 mm. In relation to the overbite, 86 (93.5%) presented with normal overbite, which means that the maxillary central incisors overlap between one-third and half of the clinical crown of the mandibular central incisors. Out of the remaining 6 children, five [5.4%] and 1 (1.1%) had increased and decreased overbite, respectively. Eighty-eight (95.7%) children had complete overbite, whereas 3 (3.3%) and 1 (1.1%) had incomplete overbite and anterior open bite (AOB), respectively. Crossbite was observed in only two of the children; there was one anterior and posterior crossbites each. Midline diastema was also observed in 11 (12.0%) of the children, of which 7 (7.6%) children had the diastema in the maxillary arch alone, while 4 (4.3%) had it present in both the arches. Dental anomalies were also observed, and the numbers of children affected were: hypodontia – 8, retained primary teeth – 3, delayed eruption – 1, enamel staining – 1, and taurodontism – 2. Other dental anomalies observed were bilateral peg-shaped maxillary lateral incisors seen in three (3.3%) children. It might be important to note here also that one of the paired peg-shaped maxillary laterals was related to the primary dentition (teeth 52 and 62). However, the result of normal soft-tissue lip seal was observed in about 26 (28.3%) of the children. Using the Jackson's classification, 25.0% and 3.3% were found to have a lip seal of 3/1 and 2/2, respectively. The remaining 66 (71.7%) had incompetent lip seal; 70.7% of them presented with upper and lower lip lines of 2/1, while 1 (1.3%) had 2/0 [Table 1].
Age group 11–16 years
Group 2 children were made up of 38 (54.3%) male and 32 (45.7%) female with a mean age of 13.27 ± 1.54 years. On analyzing their occlusal variables, 57 (81.4%), 11 (15.7%), 1 (1.4%), and 1 (1.4%) presented with Angle's Class I, Angle's Class II, Angle's Class III, and asymmetrical molar relationship respectively. In relation to overjet, it was observed to be increased in 23 (32.9%) and reduced in 5 (7.1%). Overbite was found to be increased in 5 (7.1%) and decreased in 2 (2.9%) and also observed to be incomplete in 3 (4.3%) of children. A child presented with anterior open bite (AOB). Crossbites were recorded in 4 (5.7%) children, 1 (1.4%) of which was located in the anterior segment of the jaws and 3 (4.3%) in the posterior segment. Seven (10%) children presented with midline diastema. Three (4.3%) of this midline diastema were present in the maxillary jaw, while 4 (5.7%) were observed to be present in the mandible only. A child each presented with ankyloglossia and amelogenesis imperfecta, while another two had peg-shaped laterals. Twenty-four (43.3%) of the children presented with competent lips, of which 23 (32.9%) had a lip line of 3/1 and 1 (1.4%) had a lip line of 2/2. The remaining 46 (65.7%) children who are incompetently presented with a lip line of 43 (61.4%) 2/1 and 1 (1.4%) each for lip lines 2/0, 1/2, and 1/1, respectively [Table 1].
Analyzing all the variables for both groups against gender, there was no gender predilection observed in the population [Table 2].
| Discussion|| |
A few studies ,,,,,, have documented the occlusal presentation of sickle cell individual. Only few of these studies were carried out in this environment with a growing burden of HbSS with an associated malocclusion, which has been reported to be affected by race and ethnicity. Furthermore, studies found in the literature were not stratified based on the age of participants rather they were either a combination of both children and adult age groups in their analytic samples or a concentration of adults' samples only. Many skeletal base occlusal anomalies could be managed by manipulating facial jaw growth in favor of its resolution in children through the use of myofunctional appliances, as they are in their active growing phase. Therefore, this is the first study locally to the best of our knowledge that is limiting its sample population to children only, and this was carefully thought about because of the area of interceptive orthodontics. Interceptive orthodontics allows for a quick and better resolution of incipient malocclusion in the growing child, where growth potential is a key and added advantage to its management in children. This is usually not the case when it comes to orthodontic management of malocclusion in adults. For these reasons, Okafor et al. advocated early and regular orthodontic assessment for HbSS individuals to alleviate or prevent these malocclusions. Nigeria has been quoted to have the largest concentration of sickle cell anemia individuals worldwide, with the prevalence of between 2% and 2.9% in the southern region ,,, and about 11.8% in the northern part. The actual prevalence could be more than cited as these studies are only hospital-based samples.
From our study, the prevalence of malocclusion in the 6–10-year-old group was found to be 40.2%, with a peak prevalence age of 7 years. The majority of respondents (93.5%) examined presented with normal anteroposterior relationship (Angle's Class 1 molar relationship). This is consistent with findings from a previous Nigerian study among HbSS participants, where Angle's Class 1 molar relationship was recorded in 88.5% of the patients examined. There was, however in this current study, abnormal, abnormal molar relationship occurrence of Angle's Class II relationship compared to Angle's Class III which was not recorded at all and this is similar to the findings by daCosta et al. in their study though they recorded a few Angle's Class III molar relationships. The prevailing abnormal molar relationship (Angle's Class II) observed in the current study is much lower than the 20.0% that was reported by Helaly and Abuaffan  in their study of 212 sickle cell disease children, aged 3–14 years. The disparity could be attributed to the difference in the age range of participants in both studies. Findings in Nigerian studies of nonsickle cell anemia patients also showed an increase in Angle's Class 1 anterioposterior relationship over Angle's Class II and Class III.,
Other malocclusion traits observed in these individuals such as overjet and overbite had their assessment values consistent with the findings of Helaly and Abuaffan  and daCosta et al. in a combined children and adult age groups of HbSS patients. The overjet findings from the current study were in contrast with what was observed by Oredugba et al. in 1–18-year-old sickle cell anemia individuals, where majority of the participants (67.0%) presented with reduced overjet. Proclined incisors in sickle cell anemia individuals have been related to maxillary gnathopathy, and it is said to be common among them. Furthermore, the number of HbSS individuals with increased overjet was also observed to be greater in the age group 6–10 years than the age group 11–16 years. With regard to crossbites, only two individuals presented with this feature, and the distribution was found to be one each for the anterior and posterior segments and these represent 2.2% of the population in Group I. Malocclusion in the form of midline diastema was found to be more prevalent in the maxillary arch in this age group. Midline diastema value observed in both arches in this age group was comparable with the observation of Alves e Luna et al. in their study, but much less than what was found in another study by Okafor et al.
As regards soft-tissue assessment with respect to the lips, almost three-quarters of the sample were found to present with noncompetent lips seal. Of these, the majority of the individuals have a Jackson's lip score of 2/1, which is mild noncompetent lips. Only one of the patients had a score of 2/0, reflecting poor development (short) of the upper lip. Although only a little above a third were observed to have increased overjet which could serve as an obstruction to lip competence, the high noncompetence could be attributed to possible affectation of soft-tissue development by the disorder. Therefore, an investigation of the relationship of the growth and development of the lips and the HbSS disorder would be necessary in future studies. This finding is contrary to the findings of Oredugba et al. who observed only 17.0% noncompetence lips in their study. Although, the Oredugba study had a population of both children and adults.
As regards the 11–16 years age group, the prevalence of malocclusion was found to be 44.3%. The peak of prevalence of malocclusion was at age 12 years. Angles Class 1 molar relationship was observed in 81.4% and the prevalent abnormal molar relationship for this group was Angle's Class II, and the value observed in this study was consistent with the value reported by Helaly and Abuaffan  in their study (22.9%) but lower than what Alves e Luna et al. observed (37.1%) in 36 adolescents aged 12–18 years. The difference might be due to race and sample size. About one-third (32.9%) presented with an increased overjet, and this was consistent with Alves e Luna et al. findings among a similar age group of HbSS individuals, where they recorded 28.6% of the participants in their study presenting with increased overjet. However, the increased overjet value from this study was marginally lower than that observed by Helaly and Abuaffan. It was noted that a very high percentage of the participants in this study presented with normal overbite. This is contrary to the findings of Alves e Luna et al., where just a little above half of the participants were found to have normal overbite. About 10.0% of participants in this study recorded abnormal overbite, of which 7.1% were found to have increased overbite. This observation is contrary to what was reported by Helaly and Abuaffan 2016 and Alves e Luna et al., where one-fifth of their samples, presented with abnormal overbite and non had an increased overbite. No literature was found to have assessed overbite using the parameter of complete or incomplete. In this study, majority of the participants presented with complete overbite with only 4.3% presenting with an incomplete overbite.
Unlike in studies , conducted outside Nigeria, where 15%–18% of participants were reported to present with AOB, only 1.4% of the participants in this current study had AOB. Furthermore, the finding of this present study was lower than what was reported by daCosta et al. (7.6%) in a similar Nigerian study among sickle cell individuals, aged 10–45 years, and of different ethnic background. The tendency toward open bite in sickle cell anemia patients can be attributed to the steepness of the mandibular plane; hence, an increased Frankfort mandibular plane (FMA) angle observed in them. Findings in nonsickle cell anemia patients in Nigeria have shown an AOB prevalence of between 2.8% and 4.1%.,
The soft-tissue lip assessment for competence was observed to be 30.9%, which is in contrast to the findings among 239 HbSS individuals of age 1–18 years by Oredugba and Savage  where 83.0% had a competent lip seal and daCosta et al. where 60.6% had a competent lip seal. This means that a high number of the participants in the current study presented with varied degree of lips incompetence according to the Jackson lips evaluation with no gender predilection [Table 2]. The occurrence of lip incompetence in sickle cell anemia can be attributed to maxillary hyperplasia, which results in an increased overjet; hence, difficulty in achieving an anterior oral seal.
| Conclusion|| |
The population of individuals with dental presentations of HbSS is growing with the prevalence of malocclusion being 40.2% and 44.3%, respectively, among 6–10 and 11–16 years.
Limitation of study
One of the limitations of this study was the absence of a cephalometric machine in the center of recruitment of participants. This investigation could not be requested for the assessment skeletal pattern. This would have helped to analyze the level of gnathopathy of these children.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Alves e Luna AC, Godoy F, de Menezes VA. Malocclusion and treatment need in children and adolescents with sickle cell disease. Angle Orthod 2014;84:467-72.
Mello SM, Araujo RP, Alves C. Oral considerations in the management of sickle cell disease: A case report. Oral Health Dent Manag 2012;11:125-8.
Oredugba FA, Savage KO. Anthropometric finding in Nigerian children with sickle cell disease. Pediatr Dent 2002;24:321-5.
World Health Organization. Sickle cell Anaemia – Report by the Secretariat: Fifty Ninth World Health Assembly Provisional Agenda Item 11.4. World Health Organization; 2006.
Ajayi EO. Prevalence of malocclusion among school children in Benin city, Nigeria. J Med Biomed Res 2008;7:58-65.
Onyeaso CO. Prevalence of malocclusion among adolescents in Ibadan, Nigeria. Am J Orthod Dentofacial Orthop 2004;126:604-7.
Dacosta OO. The prevalence of malocclusion among a population of Northern Nigeria school children. West Afr J Med 1999;18:91-6.
Mustapha OT, Abubakar FH. Study of the prevalence of sickle cell disease in Kano metropolis and its suburbs in Northern Nigeria. Niger J Basic Appl Sci 2001;10:219-25.
George IO, Opara PI. Sickle cell anaemia: A survey of associated morbidities in Nigerian children. Afr J Haematol Oncol 2011;2:187-10.
Costa CP, Carvalho HL, Souza Sde F, Thomaz EB. Is sickle cell anemia a risk factor for severe dental malocclusion? Braz Oral Res 2015;29. pii: S1806-83242015000100219.
Helaly M, Abuaffan AH. Association between sickle cell disease and malocclusion among a sample of Sudanese children. Indian J Dent Educ 2016;9:81-7.
Costa CP, de Carvalho HL, Thomaz EB, Sousa Sde F. Craniofacial bone abnormalities and malocclusion in individuals with sickle cell anemia: A critical review of the literature. Rev Bras Hematol Hemoter 2012;34:60-3.
Yata R, Motegi E, Ueda K, Torikai T, Harazaki M, Isshiki Y, et al
. A lip seal study of Japanese children with malocclusion. Bull Tokyo Dent Coll 2001;42:73-8.
Jackson D. Lip position and incisor relationship. Br Dent J 1962;112:147-55.
Okafor LA, Nonnoo DC, Ojehanon PI, Aikhionbare O. Oral and dental complications of sickle cell disease in Nigerians. Angiology 1986;37:672-5.
Hardy DK, Cubas YP, Orellana MF. Prevalence of angle class III malocclusion: A systematic review and meta-analysis. Open J Epidemiol 2012;2:75-82.
Grosse SD, Odame I, Atrash HK, Amendah DD, Piel FB, Williams TN, et al.
Sickle cell disease in Africa: A neglected cause of early childhood mortality. Am J Prev Med 2011;41:S398-405.
Adegoke SA, Kuteyi EA. Psychosocial burden of sickle cell disease on the family, Nigeria. Afr J Prim Health Care Fam Med 2012;4:6-11.
Odunvbun ME, Okolo AA, Rahimy CM. Newborn screening for sickle cell disease in a Nigerian hospital. Public Health 2008;122:1111-6.
daCosta OO, Kehinde MO, Ibidapo MO. Occlusal features of sickle cell anaemia patients in Lagos, Nigeria. Niger Postgrad Med J 2005;12:121-4.
Aikins EA, Onyeaso CO. Prevalence of malocclusion and occlusal traits among adolescents and young adults in Rivers state, Nigeria. Trop Dent J 2015;37:5-12.
Brown DL, Sebes JI. Sickle cell gnathopathy: Radiologic assessment. Oral Surg Oral Med Oral Pathol 1986;61:653-6.
Bandeen TC. Effects of Sickle Cell Disease on Growth of the Craniofacial Complexes. Thesis. The University of Tennessee; 2005.
Ize-Iyamu IN, Isiekwe MC. Prevalence and factors associated with anterior open bite in 2 to 5 year old children in Benin city, Nigeria. Afr Health Sci 2012;12:446-51.
[Figure 1], [Figure 2]
[Table 1], [Table 2]