Oro-facial characteristics and the surgical correction of patients affected by beta thalassaemia: a review of the literature and report of a case


Share / Export Citation / Email / Print / Text size:

Australasian Orthodontic Journal

Australian Society of Orthodontists

Subject: Dentistry, Orthodontics & Medicine


ISSN: 2207-7472
eISSN: 2207-7480





Volume / Issue / page

Volume 38 (2022)
Volume 37 (2021)
Volume 36 (2020)
Volume 35 (2019)
Volume 34 (2018)
Volume 33 (2017)
Volume 32 (2016)
Volume 31 (2015)
Related articles

VOLUME 31 , ISSUE 1 (May 2015) > List of articles

Oro-facial characteristics and the surgical correction of patients affected by beta thalassaemia: a review of the literature and report of a case

Azita Tehranchi / Hossein Behnia / Mostafa Sadeghi Ghochani * / Farnaz Younessian

Citation Information : Australasian Orthodontic Journal. Volume 31, Issue 1, Pages 98-106, DOI: https://doi.org/10.21307/aoj-2020-145

License : (CC BY 4.0)

Published Online: 15-August-2021



Despite the fact that recent medical advances have improved the quality of life and increased the life expectancy of patients suffering from thalassaemia, no standard strategy or clinical guidelines are available for the correction of the presenting craniofacial anomalies. The aim of the present study is to review the craniofacial features of affected patients, and to discuss the orthodontic and orthognathic surgical treatment options available to manage the associated and characteristic facial deformity.

Content not available PDF Share



1. Weatherall DJ, Clegg JB. Inherited haemoglobin disorders: an increasing global health problem. Bull World Health Organ 2001;79:704-12.

2. Weatherall DJ. The definition and epidemiology of non-transfusiondependent thalassemia. Blood Rev 2012;26 Suppl 1:S3-6.

3. Weatherall DJ. Thalassemia as a global health problem: recent progress toward its control in the developing countries. Ann N Y Acad Sci 2010;1202:17-23.

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

5. Berjis N, Sonbolestan SM, Nemati S, Mokhtarinejad F, Danesh Z, Abdeyazdan Z. Otorhinolaryngologic manifestations in thalassemia major patients. Iran J Ped 2007;17:15-8.

6. Adeyemo TA, Adeyemo WL, Adediran A, Akinbami AJ, Akanmu AS. Orofacial manifestations of hematological disorders: anemia and hemostatic disorders. Indian J Dent Res 2011;22:454-61.

7. 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 2011;42:1233-40.

8. Porter J, Bowden DK, Economou M, Troncy J, Ganser A, Habr D et al. Health-related quality of life, treatment satisfaction, adherence and persistence in β-thalassemia and myelodysplastic syndrome patients with iron overload receiving deferasirox: results from the EPIC clinical trial. Anemia 2012:297641.

9. Torcharus K, Pankaew T. Health-related quality of life in Thai thalassemic children treated with iron chelation. Southeast Asian J Trop Med Public Health 2011;42:951-9.

10. Thavorncharoensap M, Torcharus K, Nuchprayoon I, Riewpaiboon A, Indaratna K, Ubol BO. Factors affecting health-related quality of life in Thai children with thalassemia. BMC Blood Disord 2010;10:1.

11. Riewpaiboon A, Nuchprayoon I, Torcharus K, Indaratna K, Thavorncharoensap M, Ubol BO. Economic burden of betathalassemia/Hb E and beta-thalassemia major in Thai children. BMC Res Notes 2010;3:29.

12. Messina G, Colombo E, Cassinerio E, Ferri F, Curti R, Altamura C et al. Psychosocial aspects and psychiatric disorders in young adult with thalassemia major. Intern Emerg Med 2008;3:339-43.

13. Başsimitçi S, Yücel-Eroğlu E, Akalar M. Effects of thalassaemia major on components of the craniofacial complex. Br J Orthod 1996;23:157-62.

14. Hattab FN. Periodontal condition and orofacial changes in patients with thalassemia major: a clinical and radiographic overview. J Clin Pediatr Dent 2012;36:301-7.

15. 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 1971;121:300-6.

16. Pusaksrikit S, Hathirat P, Isarangkura P. Cephalometric radiography in thalassemic patients. Birth Defects Orig Artic Ser 1987;23:421-7.

17. Toman HA, Nasir A, Hassan R, Hassan R. Skeletal, dentoalveolar, and soft tissue cephalometric measurements of Malay transfusiondependent thalassaemia patients. Eur J Orthod 2011;33:700-4.

18. Poyton HG, Davey KW. Thalassemia. Changes visible in radiographs used in dentistry. Oral Surg Oral Med Oral Pathol 1968;25:564-76.

19. Weel F, Jackson IT, Crookendale WA, McMichan J. A case of thalassaemia major with gross dental and jaw deformities. Br J Oral Maxillofac Surg 1987;25:348-52.

20. Wonke B. Bone disease in beta-thalassaemia major. Br J Haematol 1998;103:897-901.

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

22. Kaplan RI, Werther R, Castano FA. Dental and oral findings in Cooley’s anemia: a study of fifty cases. Ann N Y Acad Sci 1964;119:664-6.

23. Hazza’a AM, Al-Jamal G. Radiographic features of the jaws and teeth in thalassaemia major. Dentomaxillofac Radiol 2006;35:283-8.

24. Mak PH, Ooi RG. Submental intubation in a patient with betathalassaemia major undergoing elective maxillary and mandibular osteotomies. Br J Anaesth 2002;88:288-91.

25. Goldfarb A, Nitzan DW, Marmary Y. Changes in the parotid salivary gland of beta-thalassemia patients due to hemosiderin deposits. Int J Oral Surg 1983;12:115-9.

26. Gaziev J, Sodani P, Lucarelli G. Hematopoietic stem cell transplantation in thalassemia. Bone Marrow Transplant 2008;42:S41.

27. Jirarattanasopa V, Hooncharoen P, Mekaewkunchorn A, Torcharus K. Effect of different transfusion regimens on craniofacial appearance and dentition in severe thalassemic children. Southeast Asian J Trop Med Public Health 2009;40:1371-6.

28. Karagiorga-Lagana M, Voskaki E, Sbyrakis S, Karaklis A. Blood transfusions for the prevention of bone deformities in thalassemia intermedia. Birth Defects Orig Artic Ser 1987;23:435-9.

29. Cazzola M, De Stefano P, Ponchio L, Locatelli F, Beguin Y, Dessi C et al. Relationship between transfusion regimen and suppression of erythropoiesis in beta-thalassaemia major. Br J Haematol 1995;89:473-8.

30. Piomelli S, Danoff SJ, Becker MH, Lipera MJ, Travis SF. Prevention of bone malformations and cardiomegaly in Cooley’s anemia by early hypertransfusion regimen. Ann N Y Acad Sci 1969;165:427-36.

31. Cadili A, de Gara C. Complications of splenectomy. Am J Med 2008;121:371-5.

32. Adelman AB. Cooley’s anemia from an orthodontic viewpoint. N Y State Dent J 1965;31:405-8.

33. Jurkiewicz MJ, Pearson HA, Furlow LT Jr. Reconstruction of the maxilla in thalassemia. Ann N Y Acad Sci 1969;165:437-42.

34. Mortazavi SH, Khojasteh A. Superior repositioning of the maxilla in thalassemia-induced facial deformity: report of 3 cases and a review of the literature. J Oral Maxillofac Surg 2007;65:1023-31.

35. Drew SJ, Sachs SA. Management of the thalassemia-induced skeletal facial deformity: case reports and review of the literature. J Oral Maxillofac Surg. 1997;55:1331-9.

36. Amini F, Borzabadi-Farahani A, Mashayekhi Z, Pousti M, Amirtouri M. Soft-tissue profile characteristics in children with beta thalassaemia major. Acta Odontol Scand 2013;71:1071-6.

37. Park N, Lazow S, Berger J. β-thalassemia: medical and surgical considerations in managing facial deformities: case report and review of the literature. J Oral Maxillofac Surg 2012;70:e284-9.

38. Hattab FN, Yassin OM. Dental arch dimensions in subjects with beta-thalassemia major. J Contemp Dent Pract 2011;12:429-33.