Journal d'hygiène bucco-dentaire et de santé

Abstrait

Modified le fort I osteotomy and genioplasty for management of severe facial deformity in ?-Thalassemia major patient

Khamis Mohammed Al Hasani

β-Thalassemia major is an autosomal recessive form of hemoglobinopathy that is characterized by complete lack of production of the β chains resulting in multiple complications including; severe anemia, failure to thrive, and skeletal abnormalities. β-Thalassemia major induced facial deformity is a rare but known skeletal complication, which is very challenging to treat from a surgical point of view. We report here a case of β-thalassemia major in a 33-year-old female patient, presenting with gross skeletal dentofacial deformity that is contributing to her psychosocial issues. The facial deformity was corrected surgically by excision of the enlarged maxilla, modified Le Fort I osteotomy and advancement genioplasty. This paper highlights the pre-operative preparation, surgical management, encountered complications, and the treatment outcome with a 24-month follow-up.