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ITALIAN JOURNAL OF MAXILLOFACIAL SURGERY
A Journal on Maxillofacial Surgery
Official Journal of the Italian Society of Maxillofacial Surgery
Italian Journal of Maxillofacial Surgery 2010 August;21(2):101-6
Dento-skeletal and cutaneous changes after orthognathic surgery in a case of skeletal “open-bite” deformity associated to Steinert disease
Tosco P., Boffano P., Schellino E., Ramieri G.
Department of Orthodontics, University of Milan, Division of Maxillofacial Surgery, Head and Neck Department, University of Turin
Steinert disease, or myotonic dystrophy (DM), is a neuromuscular autosomal disorder associated to alterations of the cranio-facial morphology and causes important functional impairment. The aim of this article is to present the clinical features and facial treatment of a DM patient and to discuss the benefits that were obtained by surgical-orthodontic correction. A 19-year-old male, affected by DM, presented huge anterior open bite, posterior cross-bite and “ogival” palatal vault with a remarkable weakness of the masticatory and peri-oral muscles. This resulted in inability in approaching the lips, saliva leakage and impairment in drinking and eating. Maxillary multiple-segment LeFort I and mandibular bilateral sagittal split osteotomies with counter-clockwise maxillo-mandibular rotation were performed. Dento-skeletal and cutaneous facial changes were documented with conventional cephalometry and surface laser scanning. Normalisation of the dental occlusion and of lips position resulted in striking functional improvement, both at rest and during function, and appeared stable 1 year after surgery. Orthognathic surgery in patients with DM may result in an outstanding improvement of facial harmony, oral function and quality of life. However, a careful multidisciplinary approach is needed to obtain proper planning, timely surgical intervention and efficient post-operative care, due to the increased peri-operative risks.