References

Kliegman RM, Stanton B, St Geme J, 19th edn. Philadelphia, PA, USA: Saunders; 2011
Gotte P, Consolo U, Faccioni F, Bertoldi C Associated orthodontic, surgical and hematological management of Cooley s anemia. Report of a case. Minerva Stomatol. 2001; 50:47-54
Hattab FNSharjah, UAE: Bentham Science Publishers; 2021
Drew SJ, Sachs SA Management of the thalassemia-induced skeletal facial deformity: case reports and review of the literature. J Oral Maxillofac Surg. 1997; 55:1331-1339 https://doi.org/10.1016/s0278-2391(97)90197-x
Başsimitçi S, Yücel-Eroğlu E, Akalar M Effects of thalassaemia major on components of the craniofacial complex. Br J Orthod. 1996; 23:157-162 https://doi.org/10.1179/bjo.23.2.157
Einy S, Hazan-Molina H, Ben-Barak A, Aizenbud D Orthodontic consideration in patients with beta-thalassemia major: case report and literature review. J Clin Pediatr Dent. 2016; 40:241-246 https://doi.org/10.17796/1053-4628-40.3.241
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Surgical orthodontics in a patient with β-thalassaemia major. A case report

From Volume 17, Issue 4, October 2024 | Pages 164-170

Authors

Maziar Farhadi

School of Dentistry, Azad Tehran University of Medical Sciences, Tehran, Iran.

Articles by Maziar Farhadi

Rosana Farjaminejad

School of Health and Psychological Sciences, Department of Health Services Research and Management, University of London.

Articles by Rosana Farjaminejad

Samira Farjaminejad

School of Health and Psychological Sciences, Department of Health Services Research and Management, University of London.

Articles by Samira Farjaminejad

Anand Marya

Deputy-Dean of Dentistry (Research) and Program Director of Orthodontics, Faculty of Dentistry, University of Puthisastra, Phnom Penh, Cambodia

Articles by Anand Marya

Abdolreza Jamilian

Orthodontic Department

Articles by Abdolreza Jamilian

Email Abdolreza Jamilian

Abstract

Treating patients with prognathic maxillae is a multifaceted process, especially when dealing with thalassaemia complications. This case study addresses the issue of severe maxillary protrusion in a woman living with β-thalassaemia major who had presented with pronounced maxillary protrusion, lip incompetence and an everted upper lip. Following initial alignment and levelling using the 0.018inch standard edgewise system, the patient underwent a Le Fort I osteotomy procedure. Segmental osteotomy was ruled out owing to the heightened risk of excessive bleeding. The outcomes demonstrated successful management of the thalassaemic patient through presurgical orthodontics, LeFort I osteotomy, and subsequent post-surgical orthodontics and periodontics. The patient exhibited improved facial aesthetics after an active treatment period of 4 years.

CPD/Clinical Relevance:

Orthodontic treatment for patients with β-thalassaemia major and skeletal Class II malocclusion with prognathic maxillae requires a multidisciplinary approach, including Le Fort I osteotomy and careful management of elevated bleeding risks

Article

Thalassaemia is a family of hereditary genetic conditions that are common worldwide. They can be categorized into two primary divisions: α- and β-thalassaemia.1 β-thalassaemia, alternatively referred to as Cooly's anaemia, is an inherited blood disorder marked by an abnormality in skull formation, resulting in a distinctive appearance in the affected individual.2

Clinically, β-thalassaemias can be categorized as thalassaemia trait, minima, minor, intermediate and major, indicating the level of anaemia present. This disorder primarily affects regions around the Mediterranean Sea, a significant portion of Central Africa, the Middle East, the Indian subcontinent, and southeast Asia, encompassing Indonesia, with occurrence rates ranging from 5% to 20%.1 These haematological challenges contribute significantly to orthodontic complications, because the disorder is associated with extensive craniofacial changes as a result of bone marrow hyperplasia and altered bone metabolism, complicating standard orthodontic treatments.3 Thalassaemia may be linked to skeletal facial deformities.4

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