Craniofacial Deformities in Patients With Beta-Thalassemia: Orthodontic Versus Surgical Correction - A Systematic Review

Rapid blood cell turnover and bone marrow expansion caused by beta-thalassemia (βT) result in craniofacial and dentoalveolar anomalies. This report presents a systematic review of the literature over the past 50 years on orthodontic and surgical considerations in the management of βT-affected patients. Seventeen publications encompassed 24 patients, 11 male individuals and 13 female individuals, 7 to 43 years of age. Eleven patients underwent only surgical treatment, eleven combined orthodontic-surgical treatment, and 2 orthodontic treatment. Surgical treatment primarily addressed typical maxillary overgrowth by maxillary reshaping, premaxillary segmental repositioning, or complete Le Fort I impaction and set back osteotomy. In severe maxilla-mandibular discrepancy and/or increased lower facial height, a bilateral sagittal split mandibular osteotomy is the treatment of choice. Although surgery involves risks of excessive bleeding, morbidity, and impaired nasal esthetics, little attention is given to the orthodontic modality. In conclusion, the current literature recommends early interceptive orthodontics aimed to decrease dentoskeletal deformities, severe malocclusion, and soft tissue imbalance. Treatment includes maxillo-mandibular orthopedic and functional manipulation with dentoalveolar treatment, which might either prevent orthosurgical procedures later or reduce its extent. This suggested a multidisciplinary approach comprising a hematologist, a pediatrician, a pediatric dentist, and an orthodontist, which might also significantly improve the patient's quality of life.