Periodontal Condition and Orofacial Changes in Patients with Thalassemia Major: A Clinical and Radiographic Overview

To assess the prevalence of periodontal disease, orofacial changes and craniofacial abnormalities in patients with thalassemia major (TM). Dental management is discussed. Study design: The sample consisted of 54 patients with TM, 31 males and 23 females aged 5.5 to 18.3 years, with the mean age (± SD) of 11.6 ± 3.2 years. The sample was divided into two subgroups according to age. A similar number of unaffected control group matched by age and sex served as a control. Clinical and radiographic examinations were carried out to assess the prevalence of changes caused by this disorder. Student’s t-test was used to compare the means between thalassemic group and the control group. The Chi-square test was employed to determine statistical differences in frequencies between the two groups. Results: Poor oral hygiene and gingivitis were observed in 61.1% and 43.0% of the thalassemic patients, respectively. The overall mean plaque score was 1.66 ± 0.51 and gingival score 1.43 ± 0.59. In all tested periodontal parameters, a higher frequency and severity were noted in the thalassemic patients compared with controls. More than half of the patients exhibited frontal bossing, saddle nose and to less extent maxillary protrusion; giving in severe cases (16.7%) a “chipmunk” like appearance. Dental discoloration and pallor oral mucosa were noted in 44.4% and 38.9%, respectively. Dental / jaw pain was reported by 40.0% and headache by 29.6% of the patients. Increased overjet was evident in 25.9% of the patients. The majority of the patients had thickened frontal bone (66.7%), and thinned inferior border of the mandible (64.6%). Widened dipolic spaces and spiky roots and were observed in one-third of the patients. The ramus length and width in the patients were significantly smaller than in controls (P < 0.001). Conclusion: TM may particularly diagnose through orofacial abnormalities. Dentists required understanding the complications and management of the disease.

periodontal status, orofacial features, thalassemia major, children

1. Weatheral JD, Clegg JB. The Thalassemia Syndrome, 3rd Ed. Oxford: Blackwell Scientific; 1981.

2. Lukens JN. The thalassemia and related disorders: quantitative disorders of hemoglobin synthesis. In Lee GR, Bithell TC, Foster J, Athens JW, Lukens JN, editors. Wintrobe’s Clinical Hematology, 9th ed. Philadelphia: Lea & Febiger, 1102–33, 1993.

3. Logothetis J, Economidou J, Constantoulakis M, Augoustaki O, Loewenson RB, Bilek M. Cephalofacial deformities in thalassemia major (Cooley’s anemia). Am J Dis Child, 121: 300–6, 1971.

4. Hes J, van der Waal I, de Man K. Bimaxillary hyperplasia: The facial expression of homozygous B-thalassemia. Oral Surg Oral Med Oral Pathol, 69: 185–90, 1990.

5. Abu Alhaija ESJ, Hattab FN. Cephalometric measurements and facial deformities in subjects with β-thalassemia major. Eur J Orthod, 24: 9–19, 2002.

6. Leonardi R, Verzi P, Caltabiano M. Epidemiological survey of the prevalence of dental caries in young thalassemia major patients. Stomatol Mediter, 10: 133–6, 1990.

7. Hattab FN, Hazza’a AM, Yassin OM, AL-Rimawi HS. Caries risk in patients with thalassemia major. Int Dent J, 51: 35–8, 2001;

8. Luglie PF, Campus G, Deiola C, Mela MG, Gallisai D. Oral condition, chemistry of saliva, and salivary levels of streptococcus mutans in thalassemic patients. Clin Oral Investig, 6: 223–6, 2002.

9. Van Dis ML, Langlais RP. The thalassemias: Oral manifestations and complications. Oral Surg Oral Med Oral Pathol, 62: 229–33, 1986.

10. Wang SC, Lin KH, Chern JPS, Lu MY, Jou ST, Lind DT et al. Severe bacterial infection in transfusion-dependent patients with thalassemia major. Clin Infect Dis, 37: 984–8, 2003.

11. Hattab FN, Qudeimat MA, AL-Rimawi HS. Dental discoloration: An overview. J Esthet Dent, 11: 291–310, 1999.

12. Hattab FN. Mesiodistal crown diameters and tooth size discrepancy of permanent dentition in patients with thalassemia major. Int J Clin Dent (In press).

13. Reuland-Bosma W, van Dijk L J. Periodontal disease in Down’s syndrome: A review. J Clin Periodontol, 13: 64–69, 1986.

14. Soskolne WA, Klinger A. The relationship between periodontal disease and diabetes: an overview. Ann Periodontol, 6: 91–98, 2001.

15. Hattab FN, Amin WM. Papillon-Lefèvre syndrome with albinism: A review of the literature and report of 2 brothers. Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 100: 709–16, 2005.

16. Silness J, Löe H. Periodontal disease in pregnancy. Part II. Correlation between oral hygiene and periodontal condition. Acta Odontol Scand, 22: 121–135, 1964.

17. Löe H, Silness J. Periodontal disease in pregnancy. Part I. prevalence and severity. Acta Odontol Scand, 21: 533–51, 1963.

18. Hattab FN, Abu Alhaija ESJ. Radiographic evaluation of mandibular third molar eruption space. Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 88: 285–91, 1999.

19. Vento S, Cainelli F, Cesario F. Infections and thalassemia. Lancet Infect Dis, 6: 226–33, 2006.

20. Modell B, Khan M, Darlison M, Westwood M, Ingram D, Penell DJ. Improved survival of thalassemia major in the UK and relation to T2 cardiovascular magnetic resonance. J Cardiovasc Magn Reson, 10: 42, 2008;

21. Kaplan RI, Werther R, Castano FA. Dental and oral findings in Cooley’s anemia: a study of fifty cases. Ann NY Acad Sci, 119: 664–666, 1964.

22. Mehdizadeh M, Mehdizadeh M, Zamani G. Orodental complications in patients with major beta-thalassemia. Dent Res J, 5: 17–20, 2008.

23. De Mattia D, Pettini PL, Sabato V, Rubini G, Laforgia A, Schettini F. Oromaxillofacial changes in thalassemia major. Minerva Pediatr, 48: 11–20, 1996.

24. Scutellari PN, Orzincolo C, Andraghetti D, Gamberini MR. Anomalies of masticatory apparatus in beta-thalassemia. The present status after transfusion and iron-chelating therapy. Radiol Med, 87: 389–96, 1994.

25. Amini F, Jafari A, Eslamian L, Sharifzadeh S. A cephalometric study on craniofacial morphology of Iranian children with beta-thalassemia major. Orthod Craniofacial Res, 10: 36–44, 2007.

26. Piras V, Tuveri F, Dessi C, Pittau R, Origa R, Basile R, et al. Relation between hypogonadism and malocclusion in beta-thalassemia major patients: Analysis of 122 subjects. Minerva Stomatol, 52: 241–6, 2003.

27. Cox GM. A study of oral pain experience in sickle cell patients. Oral Surg, 58: 39–41, 1984.

28. Olivieri NF. The β-thalassemia. N Eng J Med, 34: 99–109, 1999.

29. Poyton HG, Darvey KW. Thalassemia: Changes visible in radiographs used in dentistry. Oral Surg Oral Med Oral Pathol, 25: 564–76, 1968.

30. Wisetsin S. Cephalography in thalassemic patients. J Dent Assoc Thai, 40: 260–8, 1990.

31. Reynolds J. The roentgenological features of sickle cell disease and related hemoglobinopathies. Springfield: Charles C Thomas; 87–93, 1965.

32. Licciardello V, Bertuna G, Samperi P. Craniofacial morphology in patients with sickle cell disease: a cephalometric analysis. Eur J Orthod, 29: 238–42, 2007.

33. Terezhalmy GT, Hall ET. The asplenic patients: a consideration for antimicrobial prophylaxis. Oral Surg Oral Med Oral Pathol, 57: 114–7, 1984.

34. Galanello R. A thalassemic child becomes adult. Rev Clin Exp Hematol, 7: 4–20, 2003.