Dental and maxillofacial manifestations of Steinert's myotonic dystrophy: A retrospective case series and therapeutic investigation

Abstract

This retrospective study was performed to analyse the facial features and occlusal anomalies in 18 patients with Steinert's myotonic dystrophy (MD1). Medical and surgical management issues noted in this study may contribute to clinical decision-making. This series included 18 patients with MD1 who presented for maxillofacial consultations. For all patients, the following characteristics were assessed: sex, age, intellectual ability, oral condition, initial assessment of the occlusion and facial aspect. In total, 11 of 18 patients underwent surgery (10 achieved occlusion modification, whereas one did not). amongst patients who underwent surgery and achieved occlusion modification, six had stable class I results and four had unstable results or exhibited a slight degradation. Facial muscles play an important role in craniomaxillofacial development and facial aspects. A high prevalence of malocclusions is present in patients with MD1. Orthodontics and orthognathic surgery can improve the quality of life for affected patients. However, the long-term results of these treatments may be disappointing, and relapse can occur in patients with the most severe disease. Aspects of disease to consider while planning for surgery include oral health, risks of instability and relapse, and risks involving anaesthesia.