Background: Thalassemia is one of the most common genetic disorders worldwide. Orofacial manifestations are the result of bony changes occurring due to ineffective erythropoiesis, with the formation of a bone-expanding erythroid mass. The orofacial manifestations such as saddle nose, prominent malar bones, and proclined anteriors lead to characteristic “rodent facies.” Growth impairment is observed in most of the patients even under regular transfusions along with delayed dental development.
Aims and objectives: To assess the orofacial manifestations clinically, compare the difference between chronological and dental age of β-thalassemia major subjects with that of normal using the Demirjian\'s method radiographically, and also to find out any association between the pretransfusion hemoglobin and dental development.
Materials and methods: Forty β-thalassemia major-diagnosed subjects aged 3–16 years on regular blood transfusion were examined clinically for assessment of their orofacial features. Demographic data were collected including the pretransfusion hemoglobin level for at least 6 months. The “DMFT” and “dmft” indices were also assessed. Dental development was assessed along with their age- and sex-matched normal controls using the Demirjian\'s method for age estimation. Results were compiled and subjected to the statistical analysis.
Results: Majority of the thalassemic patients in the study were in their first decade. Saddle nose was the commonest orofacial manifestations observed while the characteristic feature “rodent facies” was seen only in 9 (22.5%) subjects. The “DMFT” and “dmft” indices were high in thalassemic subjects than normal. Out of the 40 β-thalassemia major subjects, 37 demonstrated delay in development of their dentition by 7 months than the controls, which was significant statistically. No association could be found between pretransfusion hemoglobin and the delay observed.
Conclusion: Decline in the occurrence of the “rodent facies” indicative of severe facial bone deformation could indicate the benefits of early diagnosis and regular transfusion but growth impairment in the form of dental developmental delay seen postulates a multifactorial etiology. Awareness must be given to β-thalassemia major subjects and their parents about oral health to achieve a better quality of life.
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