Assessment of orthodontic treatment needs in patients with β-thalassemia major and sickle cell disease

1. Introduction

Oral and dental management of patients with blood disorders, especially in recent years, has been one of the topics of interest to dental professionals [1]. Orthodontics is a field in dentistry that deals with diagnosing, preventing, and correcting dentofacial abnormalities. This goal is expressed in patients with altered orofacial features and severe malocclusions [2]. Many patients with blood disorders, such as coagulopathies and anemias fall in this category [3]. In life-threatening illnesses, managing the patient’s main diseases proper becomes the priority of treatment. This leads to ignoring less grave conditions, such as oral and dental problems, which may eventuate in deterioration of such conditions and consequently, demanding more complex treatment strategies [3].

Hemoglobinopathies are inherited autosomal recessive diseases that influence the production and structure of the hemoglobin molecule. The most prevalent hemoglobinopathies are sickle cell disease (SCD), alpha (α)-thalassemia, and Beta (β)-thalassemia [4]. The high rate of hemoglobinopathies in Iran has been attributed to consanguineous marriages and intermediate malaria endemicity in some provinces [5]. Thalassemia is a heterogeneous group of congenital disorders characterized by defective hemoglobin synthesis that reduces red blood cells’ survival and their precursors in the bone marrow [6]. Thalassemic patients, especially those suffering from Beta (β)-thalassemia, experience bone deformities due to bone marrow expansions, dentition protrusion and flaring, severe malocclusion, trabecular enlargement, and maxillary hyperplasia [6]. In its turn, sickle cell disease is the most frequent monogenic disorder (homozygosity for Glu6Val mutation) in the beta-globin gene, which produces hemoglobin S. Reports confirm that more than 300,000 infants are born with the disease each year, and it is estimated that this number will increase to more than 400,000 by 2050, both in low-income and high-income countries [7, 8]. Patients with sickle cell disease experience orofacial deformities, maxillary expansions, exaggerated protrusion of the midface, and craniofacial alterations. Craniofacial skeletal alterations and orthodontic conditions are prevalent manifestations of sickle cell disease and thalassemia patients. These are the outcome of extramedullary hematopoiesis and erythroid hyperplasia and lead to eating, speech, and emotional complications [9, 10]. The severity of hemoglobinopathy, patients’ age, and treatment strategy influence these abnormalities. Patients with these deformities and manifestations often have social appearance anxiety and functional disorders that affect their quality of life. Dealing with orthodontic conditions in these patients requires advanced treatment strategies, such as the use of several appliances, and surgical approaches [11].

Although, due to the mentioned reasons, these diseases are common globally, the long-term treatment needs of these patients have led to the negligence of their other disorders. In addition, the lack of comprehensive guidelines for managing orthodontic conditions in these patients makes it important to assess the orthodontic treatment needs [10]. The general dentists and specialists, including pediatric dentists and orthodontists should be cognizant of the patient’s previous medical history and the specific measures involving the clinical management of patients with hemoglobinopathies. These health providers are responsible for following the patients closely, becoming involved in the treatment process, and referring them to other clinicians when need be. To elaborate, these patients are commonly submitted to blood transfusion, which exposes them to the risk of contamination by microorganisms; therefore, essential care should be taken to provide sufficient infection control measures during the treatment process. In addition, measures should be taken to provide a stress-free environment for the patients so that their adequate body temperature and oxygenation could be maintained throughout treatment sessions [12].

Various studies evaluated the malocclusion and the need for orthodontic treatment in different population sectors, but there is a paucity of studies on patients with blood disorders. Therefore, the primary aim of the present study was to assess the types of malocclusion and orthodontic treatment needs in children suffering from transfusion-dependent sickle cell disease and beta thalassemia patients using the Orthodontic Treatment Need-Dental Health Component (DHC) Index.

2. Materials and Method

3. Results

The details of age and gender distribution of the participants are given in Table 1. In the present study, 311 patients (220 males and 91 females, mean age 12.67) suffering from transfusion-dependent sickle cell disease and beta-thalassemia who were referred for blood transfusion were examined to assess the frequency of oral habits, types of malocclusion, and evaluate the orthodontic treatment needs using IOTN-DHC. All participants were between 10 and 16 years old.

4. Discussion

The current study represents the first epidemiologic, and orthodontic investigation carried out on an Iranian population of patients with transfusion-dependent sickle cell disease and beta-thalassemia. Regarding oral habits, Laganà et al. [17] studied the orthodontic treatment needs of a 7-to 15-year-old Albanian population. They reported that more than 80% of the total sample showed oral habits, whereas approximately 60% of our study population had such habits. In a study, Gupta et al. [18] reported Class I, II, and III malocclusions in 44%, 55%, and 1% of the transfusion-dependent thalassemia patients, respectively, which was not in consistence with the healthy children malocclusion ratios. This result could be due to the fact that hemoglobinopathies are associated with extramedullary hematopoiesis, and bone marrow hyperplasia, leading to maxillary prominence and higher prevalence of Class II malocclusion [18, 19]. In addition, there is evidence reported in many studies about the generalized growth retardation resulting in mandibular retrusion in these patients [20, 21, 22]. However, based on the studies conducted on normal population, more than half of the healthy children presented with a class I malocclusion, which is higher than the outcomes of other epidemiologic surveys [17, 23]. In Iran, the prevalence of malocclusion in different provinces was investigated in several studies. In the study conducted on 502 students in Isfahan province, the prevalence of Class I, II, and III malocclusions were 41.8%, 27.5%, and 7.8%, respectively [24]. Arabiun et al. [25], in another study conducted in Shiraz province, reported that the prevalence of Class I, II, and III malocclusions were 12.78%, 9.94%, and 0.97%, respectively. One of our previous studies was also another investigation into the epidemiology of malocclusions in Iranian BMT and SCD children [9] which showed a slight discordance with this study. This difference could be justified by the family history of severe skeletal mandibular prognathism which runs through the majority of our Class III patients [9]. In another study on thalassemic patients, Treatment Priority Index scores showed definite malocclusion, handicapping malocclusion, and severely handicapping malocclusion to be significantly high in beta thalassemia subjects compared to the control population [18]. The results obtained from the current study were in line with those of Gupta et al.’s [18], indicating higher prevalence of Class II malocclusion among BTM and SCD patients, which can be attributed to a couple of reasons, such as the higher percentage of thumb sucking habit and/or maxillary prominence due to bone marrow hyperplasia in these patients and also possibly lower occurrence of genetic background of Class III malocclusion within our randomly selected sample. Previous studies showed that increased overjet is one of the most significant contributing factors to tooth traumatic injuries. Treating an abnormal overjet not only minimizes the risk of traumatic dental injuries, but also is beneficial from an aesthetic point of view [26, 27]. The objective need for orthodontic treatment (Grade 4, and 5) was registered in 41% of healthy children in the Laganà study [17]. Danaei et al. [28] studied an Iranian adolescent population through Dental Aesthetic Index, and the prevalence of participants with intense-to-disabling and disabling malocclusions was determined at 21% and 8.4%, respectively, which is significantly higher than our normal group. This discrepancy can be attributed to the differences in the ethnicity of the study populations and age groups. These findings on normal children are in line with one of our previous studies about the prevalence of malocclusion on normal Iranian children using IOTN in which an objective treatment need was recorded in 17.71% of participants [26]. The majority of orthodontic need evaluation studies have been conducted on normal population and there are few studies on thalassemic patients proper. In a British population of 12- to 15-year-old schoolchildren, about 35% of subjects were registered to have a definite orthodontic treatment need [29], while other studies on French [30], Turkish [31], and Iraqi [32] children reported an objective treatment need (grades 4, and 5), respectively, in 21%, 16.7% and 30.1% of the populations. These findings could be explained considering the economic and social status of the studied populations. European epidemiological surveys on normal adolescents reported a lower prevalence of grade 4 and 5 IOTN-DHC, which could be attributed to preventive dental management policies [30, 33]. However, Jeelani et al. [33] reported grades 4 and 5 of IOTN were significantly more prevalent in thalassemic children compared to the control group; however, there were no SCD patients included in their study. In our study, this gap was bridged through which the DHC of the IOTN was used to evaluate the orthodontic treatment need, where the higher percentage of grade 4 and 5 in BTM and SCD was revealed compared to normal patients which was in line with the results obtained by Jeelani et al. [33]. These results can be used as source data for the prevalence of different classes of malocclusion in different age groups of BTM and SCD patients to determine the optimal timing for orthodontic treatment. These findings might suggest that preventive and early-stage orthodontic evaluation could be integrated into developing a dental-health-monitoring program in Iran aimed at increasing dental health in both patients and normal children with a more emphasis on those inflicted children who have diseases which threaten their dental health. Adopting a panoramic point of view, regular blood transfusion and monitoring of hemoglobin levels in these patients is a crucial measure to increase the life expectancy and decrease the associated complications, which otherwise might be reversed in poorly-equipped environment with the lack of proper monitoring programs.

5. Conclusions

Class II malocclusion frequency was higher in sickle cell anemic and beta thalassemic patients. Oral habits were observed in 61.88% of the study population. IOTN-DHC scores showed treatment need grades to be significantly higher in the above-mentioned patients compared to the control population (p < 0.01) which implies a real need for these populations to be provided with preventive orthodontic treatment. Public subsidized orthodontic treatment should be provided to BTM and SCD patients as well as healthy children registered in grades 4 and 5 of DHC.

Availability of Data and Materials

Not applicable.

Author contributions

SK—designed and performed the study, collected the records, performed the measurements, and analyzed the results. SLA—analyzed the results, provided the statistical analysis of the data. MRY—performed the measurements for the different variables in SPSS and wrote the manuscript. All authors read and approved the final manuscript.

Ethics approval and consent to participate

Informed consent was obtained from patients or their caregivers, and the Medical Ethics Committee of Islamic Azad University, which complied with the Helsinki Declaration, approved this research.

Acknowledgment

Not applicable.

Funding

This research received no external funding.

Conflict of interest

The authors declare no conflict of interest.