Evaluation of Clinicoradiological Orofacial Structures in Children with β-thalassemia Major
Megha Pradhan, P Sudha
Keywords :
Chronological age, Demirjian's method, Dental age, β-Thalassemia major
Citation Information :
Pradhan M, Sudha P. Evaluation of Clinicoradiological Orofacial Structures in Children with β-thalassemia Major. J South Asian Assoc Pediatr Dent 2020; 3 (1):23-26.
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.
Ay ZY, Oruçoğlu A, Klnç G, et al. Does periodontal health of thalassemia major patients have an impact on the blood lipid profiles. J Pediatr Hematol Oncol 2007;29(10):694–699. DOI: 10.1097/MPH.0b013e31814d68c3.
Hoffman R, Benz EJ, Shallil SJ, et al. Hematology basic principles and practices. . 4th ed. pp. 558–559.
Van Dis ML, Langlais RP. Thalassemias: oral manifestations and complications. Oral Surg Oral Med Oral Pathol 1986;64(2):229–233. DOI: 10.1016/0030-4220(86)90055-1.
Elham SJ, Abu Alhaija ESJ, Hattab FN, et al. Cephalometric measurements and facial deformities in subjects with β-thalassemia major. Eur J of Orthod 2002;24(1):9–19. DOI: 10.1093/ejo/24.1.9.
Goldfarb A, Nitzan DW, Marmary Y. Changes in the parotid salivary glands of β-thalassemia patient due to hemosiderin deposits. Int J Oral Surg 1983;1-12(2):115–119. DOI: 10.1016/s0300-9785(83)80007-6.
Al-Wahadni AM, Taani DQ, Al-Omari MO. Dental diseases in subjects with β-thalassemia major. Community Dent Oral Epidemiol 2002;30(6):418–422. DOI: 10.1034/j.1600-0528.2002.00012.x.
Nykanen R, Espeland L, Kvaal SI, et al. Validity of the demirjian method for dental age estimation when applied to Norwegian children. Acta odontol scand 1998;56(4):238–244. DOI: 10.1080/00016359850142862.
Hazza'a AM, Al-Jamal G. Dental development in subjects with thalassemia major. J Contemp Dent Pract 2006;7(4):63–70. DOI: 10.5005/jcdp-7-4-63.
Laor E, Garfunkel A, Koyoumidijsky-Kaye E. Skeletal and dental retardation in β-thalassemia major. Hum Biol 1982;54(1):85–92.
Logothetis J, Constantoulakis M, Lowenson RB. Cephalofacial deformities in thalassemia major (Cooley's anemia). Amer J Dis Child 1971;121(4):300–306. DOI: 10.1001/archpedi.1971.02100150074007.
Mattia De, Pettini PL, Sabato V, et al. Oromaxillofacial changes in thalassemia major. Minerva Pediatr 1996;48(1-2):11–20.
Chaillet N, Demirjian A. Dental maturity in South France: a comparison between Demirjian's method and polynomial functions. J Forensic Sci 2004;49(5):1059–1066. DOI: 10.1520/JFS2004037.
Demirjian A, Goldstein H. New systems for dental maturity based on seven and four teeth. Ann Hum Biol 1976;3(5):411–421. DOI: 10.1080/03014467600001671.
Cutando A, Gil JA, López JD. Thalassemia and their dental implications. Medicina Oral 2002;7:36–45.
Eleftheriou A. About thalassemia. Thalassemia International Federation Publications; 2007;4.
Helmi N, Bashir M, Shireen A, et al. Thalassemia review: features, dental considerations and management. Electron Physician 2017;9(3):4003–4008. DOI: 10.19082/4003.
Salehi MR, Farhud DD, Tohidast TZ, et al. Prevalence of orofacial complications in Iranian patients with β – thalassemia major. Iranian J Publ Health 2007;36(2):43–46.
Ficarra G. Thalassemia diagnosed through facial distortion. Int J Maxillofacial surg 1987;16(2):23–25. DOI: 10.1016/S0901-5027(87)80136-4.
Cutando Soriano A, Gil Montoya JA, López-González Garrido Jde D. Thalassemias and their dental implications. Med Oral 2002;7(1):36–40, 41–45.
Madhok S, Madhok S. Dental considerations in thalassemic patients. J Dent Med Sci 2014;13(6):57–62. DOI: 10.9790/0853-13645762.
Pusaksrikit S, Isarangkura P, Hathirat P. Cephalometric radiography in thalassemia patients. Birth Defects 1988;23(5A):421.
Siamopoulou-Mavridou H, Lapatsanis P. Flow rate and chemistry of parotid saliva related to dental caries and gingivitis in patients with thalassemia major. Int J Paediatr 1992;2(2):93–97. DOI: 10.1111/j.1365-263x.1992.tb00016.x.
Ventura B. Cooley's anemia. its dental aspects. Clin Odont 1955;10:342–350.
Anand M, Garg S, Saraf BG, et al. A hospital based study of caries experience in beta –thalassemic major children. Acta Scientific Dental Sciences 2019;3(5):45–48.
Mehdizah M, Mehdizah M, Zamani G. Orodental complications in patients with major β-thalassemia. Dent Res J 2008;5(1):17–20.
Luglie PF, Campus G, Deiola C, et al. Oral condition, chemistry of saliva, and salivary levels of streptococcus mutans in thalassemic patients. Clin Oral Invest 2002;6(4):223–226. DOI: 10.1007/s00784-002-0179-y.
Hattab FN, Hazza'a AM, YassinOM, et al. Caries risk in patients with thalassemia major. Int Dent J 2001;51(1):35–38. DOI: 10.1002/j.1875-595x.2001.tb00815.x.
Kalpan RI, Werther R, Castano FA. Dental and oral findings in Cooley's anemia: A study of fifty cases. Ann NY Acad Sci 1964;119:664–666.
Prasad H, Kala N. Accuracy of two dental age estimation methods in the Indian population - a meta-analysis of published studies. J Forensic Odontostomatol 2019;3(37):2–11.
Hegde RJ, Sood PB. Dental maturity as an indicator of chronological age. Radiographic evaluation of dental age in 6 to 13 years children of Belgaum using Demirjian methods. J Indian Soc Pedo Prev Dent 2002;20(4):132–138.
Prabhakar AR, Panda AK, Raju OS. Applicability of Demirjian's methods of age assessment in children of Davangere. J Indian Soc Pedo Prev Dent 2002;20(2):54–62.
Rodda CP, Reid ED, Johnson S, et al. Short stature in homozygous beta-thalassemia is due to disproportionate truncal shortening. Clin Endocrinol (Oxf) 1995;42(6):587–592. DOI: 10.1111/j.1365-2265.1995.tb02684.x.
Ortolani M, Wolman IJ. Some clinical features of Cooley's anemia patients as related to transfusion schedules. Ann NY Acad Sci 1969;163(1 Second Confer):407–414. DOI: 10.1111/j.1749-6632.1969.tb27811.x.
Kattamis CA, Liakopoulu T, Kattamis A. Growth and development with thalassemia major. Acta Paediatr Scand 1990;366(suppl):111–117. DOI: 10.1111/j.1651-2227.1990.tb11611.x.
Low LCK. Growth, puberty and endocrine function in beta-thalassemia major. J Pediatr Endocrinol Metab 1997;10(2):175–184. DOI: 10.1515/jpem.1997.10.2.175.
Saraya AK, Kumar R, Kailash S, et al. Vitamin B 12 and folic acid deficiency in β-heterozygous thalassemia. Ind J Med Res 1984;79:783–788.
Fuchs GJ, Tienboon P, Linpisarn S, et al. Nutritional factors and thalassemia major. Arch Dis Child 1996;74(3):224–227. DOI: 10.1136/adc.74.3.224.