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1型强直性肌营养不良(斯坦纳特综合征)患者的外科正畸治疗

Surgical Orthodontic Treatment of a Patient Affected by Type 1 Myotonic Dystrophy (Steinert Syndrome).

作者信息

Cacucci Laura, Ricci Beatrice, Moretti Maria, Gasparini Giulio, Pelo Sandro, Grippaudo Cristina

机构信息

Università Cattolica del Sacro Cuore, Largo A. Gemelli 8, 00168 Rome, Italy.

出版信息

Case Rep Dent. 2017;2017:7957961. doi: 10.1155/2017/7957961. Epub 2017 May 31.

Abstract

Myotonic dystrophy, or Steinert's disease, is the most common form of muscular dystrophy that occurs in adults. This multisystemic form involves the skeletal muscles but affects also the eye, the endocrine system, the central nervous system, and the cardiac system. The weakness of the facial muscles causes a characteristic facial appearance frequently associated with malocclusions. Young people with myotonic dystrophy, who also have severe malocclusions, have bad oral functions such as chewing, breathing, and phonation. We present a case report of a 15-year-old boy with anterior open bite, upper and lower dental crowding, bilateral crossbite, and constriction of the upper jaw with a high and narrow palate. The patient's need was to improve his quality of life. Because of the severity of skeletal malocclusion, it was necessary to schedule a combined orthodontic and surgical therapy in order to achieve the highest aesthetic and functional result. Although therapy caused an improvement in patient's quality of life, the clinical management of the case was hard. The article shows a balance between costs and benefits of a therapy that challenges the nature of the main problem of the patient, and it is useful to identify the most appropriate course of treatment for similar cases.

摘要

强直性肌营养不良症,即斯坦纳特氏病,是成人中最常见的肌营养不良症形式。这种多系统疾病累及骨骼肌,但也会影响眼睛、内分泌系统、中枢神经系统和心脏系统。面部肌肉无力会导致一种特征性的面部外观,常伴有错牙合畸形。患有强直性肌营养不良症且伴有严重错牙合畸形的年轻人,其口腔功能如咀嚼、呼吸和发声都很差。我们报告一例15岁男孩的病例,该男孩存在前牙开牙合、上下牙列拥挤、双侧反牙合以及上颌缩窄伴高而窄的腭部。患者的需求是改善生活质量。由于骨骼错牙合畸形的严重程度,有必要安排正畸和外科联合治疗,以获得最佳的美学和功能效果。尽管治疗改善了患者的生活质量,但该病例的临床管理仍很困难。本文展示了一种治疗方法在成本和效益之间的平衡,这种治疗方法挑战了患者主要问题的本质,对于确定类似病例的最合适治疗方案很有帮助。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7d55/5469988/609054daacd4/CRID2017-7957961.001.jpg

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