Skeletal Dentoalveolar and Soft Tissue Effects of β Thalassemia Major

Abstract

Objective: Thalassemia (TH) is a genetic disorder of hemoglobin synthesis caused by the absence or reduced synthesis of globin chains which causes different craniofacial effects. The purpose of this study is to evaluate the skeletal, dental and soft tissue cephalometric features of TH patients.
Materials and Methods: Present study consisted of lateral cephalometric images obtained from CBCT scans of 25 TH (mean age: 13,7±3,8 years) and 50 normal (mean age: 13,9±1,81 years) individuals. An Independent sample t was used to compare the cephalometric measurements.
Results: TH patients revealed a high angle Class II pattern (SnGoMe, Sum p<0,01, ANS-PNS/GoMe, ANB, p<0,001) caused by retruded mandible (SNB, SNPog, p<0,001) with retrusive upper (U1-Na°, p<0,01, U1-Na mm p<0,001) and protrusive lower incisors (L1Nb °, p<0,001, IMPA, p<0,05, L1Nb mm, p<0,01). Except the S-N length (p>0.05), all craniofacial parameters (S-Ar p<0,05, S-Go p<0,01, Ar-Go, N-Me p<0,05, Go-Me, S-Go/N-Me p<0,001) of TH patients’ found significantly smaller than that obtained from normal individuals. On the contrary ANS-PNS parameter (p<0,01) was found significantly bigger in TH patients’. In addition TH patients have protrusive upper (p<0,05) and lower lips (p<0,01) accompanied with an increased nasolabial angle (p<0,01) when compared with normal individuals.
Conclusion: A high angle Class II pattern caused by the retrusion and clockwise rotation of mandible accompanied with retrusive upper, protrusive lower incisors and protrusive upper and lower lips may be considered the most common craniofacial features of TH patients.

Keywords

• protrusive

References

1. 1. Weatherall DJ, Clegg JB. The β thalassaemia. In: The thalassaemia syndromes. Oxford: Blackwell Science. 1981: 149–56.
2. 2. Duggal MS, Bedi R, Kinsey SE, Williams SA. The dental management of children with sickle cell disease and β thalassaemia: a review. International Journal of Paediatric Dentistry. 1996; 6: 227–234.
3. 3. Mohamed N, Jackson N. Severe thalassaemia intermedia: clinical problems in the absence of hypertransfusion. Blood Reviews. 1998;12: 163–170.
4. 4. Tyler P A, Madani G, Chaudhuri R, Wilson L F, Dick E A. The radiological appearances of thalassaemia. Clinical Radiology. 2006;61: 40–52.
5. 5. Caffey J. Cooley’s anemia : a review of the roentgenographic findings in the skeleton. Am J Roentgenol Radium Ther Nucl Med.1957;78:381-91.
6. 6. Flynn DM, Fairney A, Jackson D, Clayton BE. Hormonal changes in thalassaemia major. Arch Dis Child. 1976;51:828-36.
7. 7. Saenger P, Schwartz E, Markenson AL, Graziano JH, Levine LS, New MI, Hilgartner MW. Depressed serum somatomedin activity in beta-thalassemia. J Pediatr. 1980;9:214-8.
8. 8. Logothetis J, Economidou J, Constantoulakis M, Augoustaki O, Loewenson R B, Bilek M. Cephalofacial deformities in thalassemia major (Cooley’s anemia). A correlative study among 138 cases. American Journal of Diseases of Children 1971;121: 300–306.

9. 9. Kaplan RI, Werther R, Castano FA. Dental and oral findings in Cooley’s anemia: a study of fifty cases. Annals New York Academy of Science. 1964;119:664-66.
10. 10. Sun NCJ. Hematolgy: An Atlas and Diagnostic Guide, W.B. Saunders Company , Philadelphia,pp:58-61.
11. 11. Cannell H. the development of oral and facial signs in β thalassemia major. Br J Orthod. 1988;164:50-51.
12. 12. Vichinsky E P. Changing patterns of thalassemia worldwide. Annals of the New York Academy of Sciences. 2005;1054: 18–24.
13. 13. Ismail A, Campbell M J, Ibrahim H M, Jones G L. Health related quality of life in Malaysian children with thalassaemia. Health and Quality of Life Outcomes. 2006;4: 39.
14. 14. Malaysian Health Technology Assessment Unit. Management of thalassaemia. Report. MOH/PAK/77.03 (TR) Malaysia Ministry of Health, Kuala Lumpur 2003.
15. 15. Farhud D, Sadighi H. Investigation of prevalence of beta thalassemia in Iranian provinces. Iranian J Pub Health. 1997;26:3-6.
16. 16. Çavdar A O, Arcasoy A. The Incidence of β-Thalassemia and Abnormal Hemoglobins in Turkey. Acta Haematol 1971;45:312-8.
17. 17. Toman HA, Nasir A, Hassan R, Hassan R. Skeletal, dentoalveolar, and soft tissue cephalometric measurements of Malay transfusion-dependent thalassaemia patients. Eur J Orthod. 2011;33:700-4.
18. 18. MR Salehi, DD Farhud , TZ Tohidast , M Sahebjamee , H Khademi. Prevalence of Orofacial Complications in Iranian Patients with β Thalassemia Major. Iranian J Publ Health. 2007;36:43-6.
19. 19. M Takriti, Dashash. Craniofacial parameters of Syrian children with b-thalassemia major. J Invest Clin Dent. 2011;2:1-9.
20. 20. Abu Alhaija ES, Hattab FN, Al-Omari MA. Cephalometric measurements and facial deformities in subjects with beta-thalassaemia major. Eur J Orthod. 2002;24:9-19.
21. 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. 22. Bassimitci S, Yucel-Eroglu E, Akalar M. Effects of thalassaemia major on components of the craniofacial complex. Br J Orthod. 1996;23: 157–62.
23. 23. Pusaksrikit S, Isarangkura P, Hathirat P. Occlusion of the teeth in thalassemic patients. Birth Defects Original Articles Series. 1987;23: 429–433.
24. 24. Hes J, van der Waal I, de Man K. Bimaxillary hyperplasia: the facial expression of homozygous beta-thalassemia. Oral Surgery, Oral Medicine, Oral Pathology. 1990;69: 185-90.
25. 25. Pullinger A, Thurston M, Turley P. Condylar adaptation to unilateral posterior crossbite in adults. J Dent Res. 1985;64: 269.
26. 26. International Commission on Radiation Protection. Recommendations of the nternational Commission on Radiation Protection, ICRP Publication 26. Ann ICRP. 1977;1:3.
27. 27. Schlueter BA. Cone Beam Computed Tomography Threedimensional Reconstruction for Evaluation of the Mandibular Condyle [thesis]. St Louis, Mo: Saint Louis University; 2007.
28. 28. Ludlow JB, Ivanovic M. Comparative dosimetry of dental CBCT devices and 64-slice CT for oral and maxillofacial radiology. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2008;106:106–14.
29. 29. White SC. Cone-beam imaging in dentistry. Health Phys. 2008;95:628–37.
30. 30. Solow B, Siersbaek-Nielsen S, Greve E. Airway adequacy, head posture, and craniofacial morphology. Am J Orthod 1984;86:214-23.
31. 31. Pereira FC, Motonaga SM, Faria PM, Matsumoto MA, Trawitzki LY, Lima SA, et al. Myofunctional and cephalometric evaluation of mouth breathers. Rev Bras Otorrinolaringolol 2001;67:43-9.
32. 32. Valera FC, Travitzki LV, Mattar SE, Matsumoto MA, Elias AM, Anselmo-Lima WT. Muscular, functional and orthodontic changes in preschool children with enlarged adenoids and tonsils. Int J Pediatr Otorhinolaryngol 2003;67:761-70.
33. 33. Johnston FE, Krogman WM. Patterns of growth in children with thalassemia major. Annals New York Academy of Sciences. 1964;119:667-69.
34. 34. Laor E, Garfunkel A, Koyoumdjisky E. Skeletal and dental retardations in β thalassemia major. Human Biyology. 1982;54:85-92.
35. 35. Bakor SF, Enlow DH, Pontes P, De Biase NG. Craniofacial growth variations in nasal-breathing, oral-breathing, and tracheotomized children. Am J Orthod Dentofacial Orthop. 2011;140:486-92.
36. 36. Solow B. The dentoalveolar compensatory mechanism: Background and clinical implications. Br J Orthod. 1980;7:145–161.
37. 37. Sarver D M. Esthetic orthodontic and orthognathic surgery. Mosby, St Louis; 1998.