Orthodontic Consideration in Patients with Beta-Thalassemia Major: Case Report and Literature Review

Objective: Beta Thalassemia (βT) patients present a unique facial appearance and specific craniofacial, jaw and dental patterns. Although this anomaly often requires orthodontic management, βT patients have received scant attention in the orthodontic and dental literature over the past 50 years. The aim of this article is to review the characteristic craniofacial and dental manifestation pattern of βT patients and to emphasize their preferred orthodontic management protocol by presenting a βT orthodontic treated patient. Case report: A 10 year old patient presented with a complaint of severe esthetic and functional disorders due to her diagnosis of βT. We initiated orthodontic treatment including a combined orthopedic and functional treatment modality to improve facial appearance. Results: Maxillary restraint and increased mandibular size during treatment along with an increase in the vertical dimension were achieved. The patient presented with Angle class I molar relationship, with reduction of the excessive overjet and deep overbite. Conclusion: Orthodontic treatment comprised of maxillary orthopedic treatment directed especially toward premaxilla with light forces, and mandibular modification by functional appliance along with fixed orthodontic treatment is recommended in βT patients.

beta thalassemia, orthodontic, children

1. Nathan D, Orkin S, Look A, Ginsburg D. Nathan and Oski’s Hematology of Infancy and Childhood. Chapter 20: The Thalassemias. 2009.

2. Wintrobe, M. M. Clinical hematology. Philadelphia: Lea & Febiger,; 1981.

3. Schwartz E, Benz E, Forget B. Thalassemia syndromes. In: Hoffman R, Benz E, Shattil S, editors. Hematology Basic Principles and Practice. New York, NY: Churchill Livingstone; 1995.

4. Baker DH. Roentgen Manifestations Cooley’s Anemia. Ann N Y Acad Sci;119:641-661. 1964.

5. Asbell MB. Orthodontic Aspects of Cooley’s Anemia. Ann N Y Acad Sci;119:662-663. 1964.

6. Kaplan RI, Werther R, Castano FA. Dental and Oral Findings in Cooley’s Anemia: A Study of Fifty Cases. Ann N Y Acad Sci;119:664-666. 1964.

7. Pusaksrikit S, Isarangkura P, P: H. Occlusion of the teeth in thalassemic patients. Birth Defects; 23(5A):429-433. 1988.

8. Hattab FN. Periodontal condition and orofacial changes in patients with thalassemia major: a clinical and radiographic overview. J Clin Pediatr Dent;36:301-307. 2012.

9. Toman HA, Nasir A, Hassan R, Hassan R. Skeletal, dentoalveolar, and soft tissue cephalometric measurements of Malay transfusion-dependent thalassaemia patients. Eur J Orthod; 33:700-704. 2011

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

11. Abu Alhaija ES, Hattab FN, al-Omari MA. Cephalometric measurements and facial deformities in subjects with beta-thalassaemia major. Eur J Orthod;24:9-19. 2002.

12. Bassimitci S, Yucel-Eroglu E, Akalar M. Effects of thalassaemia major on components of the craniofacial complex. Br J Orthod ;23:157-162. 1996.

13. Drew SJ, Sachs SA. Management of the thalassemia-induced skeletal facial deformity: case reports and review of the literature. J Oral Maxillofac Surg;55:1331-1339. 1997.

14. Silling G, Moss SJ. Cooley’s anemia--orthodontic and surgical treatment. Am J Orthod;74:444-449. 1978.

15. Gotte P, Consolo U, Faccioni F, Bertoldi C. Associated orthodontic, surgical and hematological management of Cooley s anemia. Report of a case. Minerva Stomatol;50:47-54. 2001.

16. Bjork A. The face in profile: An anthropological X ray investigation on Swedish children and conscripts Berlingska Boktryckeriet. Lund; 1949.

17. Jurkiewicz MJ, Pearson HA, Furlow LT, Jr. Reconstruction of the maxilla in thalassemia. Ann N Y Acad Sci;165:437-442. 1969.

18. Schudy FF. The control of vertical overbite in clinical orthodontics. Angle Orthod;38:19-39. 1968.

19. Moyers R. Handbook of orthodontics for students and general practitioner. Chicago: Year book medical publishers incorporated; 1973.

20. Logothetis J, Economidou J, Constantoulakis M, Augoustaki O, Loewenson RB, Bilek M. Cephalofacial deformities in thalassemia major (Cooley’s anemia). A correlative study among 138 cases. Am J Dis Child;121:300- 306. 1971.

21. Jurkiewicz MJ, Pearson HA, Furlow LT, Jr. Surgical reconstruction of the maxilla in severe thalassemia. Plast Reconstr Surg;39:459-464. 1967.

22. Solow B. The dentoalveolar compensatory mechanism: background and clinical implications. Br J Orthod;7:145-161. 1980.

23. Adelman AB. Cooley’s anemia from an orthodontic viewpoint. N Y State Dent J;31:405-408. 1965.

24. Amini F, Borzabadi-Farahani A, Mashayekhi Z, Pousti M, Amirtouri M. Soft-tissue profile characteristics in children with beta thalassaemia major. Acta Odontol Scand;71:1071-1076. 2013.

25. Adjrad L, Amara A, Rouabhi F, Girot R, Labie D, Benabadji M. [Treatment of homozygote beta thalassemia in Algiers. A 5-year follow-up of 66 patients]. Presse Med;18:1010-1013. 1989.

26. Cutando Soriano A, Gil Montoya JA, Lopez-Gonzalez Garrido Jde D. Thalassemias and their dental implications. Med Oral;7:36-40, 41-35. 2002.

27. Toman HA, Hassan R, Hassan R, Nasir A. Craniofacial deformities in transfusion- dependent thalassemia patients in Malaysia: prevalence and effect of treatment. Southeast Asian J Trop Med Public Health;42:1233-1240. 2011.

28. Johnston FE, Krogman WM. Patterns of Growth in Children with Thalassemia Major. Ann N Y Acad Sci;119:667-679. 1964.

29. Caffey J. Cooley’s anemia: a review of the roentgenographic findings in the skeleton: Hickey lecture, 1957. Am J Roentgenol Radium Ther Nucl Med;78:381-391. 1957.

30. Laor E, Garfunkel A, Koyoumdjisky-Kaye E. Skeletal and dental retardation in beta-thalassemia major. Hum Biol;54:85-92. 1982.

31. George A, Bhaduri A, Sen S, Choudhry VP. Physical growth parameters in thalassemic children. Indian J Pediatr;64:861-871. 1997.

32. Logothetis J, Loewenson RB, Augoustaki O, Economidou J, Constantoulakis M. Body growth in Cooley’s anemia (homozygous beta-thalassemia) with a correlative study as to other aspects of the illness in 138 cases. Pediatrics; 50:92-99. 1972.

33. Hattab FN. Patterns of physical growth and dental development in Jordanian children and adolescents with thalassemia major. J Oral Sci;55:71-77. 2013.

34. Poyton HG, Davey KW. Thalassemia. Changes visible in radiographs used in dentistry. Oral Surg Oral Med Oral Pathol;25:564-576. 1968.

35. Graber T, Vanarsdall R. Orthodontics, Current Principles and Techniques The Mosby Co; 2005.

36. Showkatbakhsh R, Behnia H, Jamilian A, Heydarpour M, Fetrati A. Lefort 1 osteotomy in a beta-thalassemia major patient--a case report. Int J Orthod Milwaukee;24:15-18. 2013.