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

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...

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Main Authors: Laura Cacucci, Beatrice Ricci, Maria Moretti, Giulio Gasparini, Sandro Pelo, Cristina Grippaudo
Format: Article
Language:English
Published: Wiley 2017-01-01
Series:Case Reports in Dentistry
Online Access:http://dx.doi.org/10.1155/2017/7957961
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author Laura Cacucci
Beatrice Ricci
Maria Moretti
Giulio Gasparini
Sandro Pelo
Cristina Grippaudo
author_facet Laura Cacucci
Beatrice Ricci
Maria Moretti
Giulio Gasparini
Sandro Pelo
Cristina Grippaudo
author_sort Laura Cacucci
collection DOAJ
description 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.
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institution Kabale University
issn 2090-6447
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language English
publishDate 2017-01-01
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series Case Reports in Dentistry
spelling doaj-art-d07bbfd2b45944e998e9ed49b32421282025-02-03T05:44:17ZengWileyCase Reports in Dentistry2090-64472090-64552017-01-01201710.1155/2017/79579617957961Surgical Orthodontic Treatment of a Patient Affected by Type 1 Myotonic Dystrophy (Steinert Syndrome)Laura Cacucci0Beatrice Ricci1Maria Moretti2Giulio Gasparini3Sandro Pelo4Cristina Grippaudo5Università Cattolica del Sacro Cuore, Largo A. Gemelli 8, 00168 Rome, ItalyUniversità Cattolica del Sacro Cuore, Largo A. Gemelli 8, 00168 Rome, ItalyUniversità Cattolica del Sacro Cuore, Largo A. Gemelli 8, 00168 Rome, ItalyUniversità Cattolica del Sacro Cuore, Largo A. Gemelli 8, 00168 Rome, ItalyUniversità Cattolica del Sacro Cuore, Largo A. Gemelli 8, 00168 Rome, ItalyUniversità Cattolica del Sacro Cuore, Largo A. Gemelli 8, 00168 Rome, ItalyMyotonic 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.http://dx.doi.org/10.1155/2017/7957961
spellingShingle Laura Cacucci
Beatrice Ricci
Maria Moretti
Giulio Gasparini
Sandro Pelo
Cristina Grippaudo
Surgical Orthodontic Treatment of a Patient Affected by Type 1 Myotonic Dystrophy (Steinert Syndrome)
Case Reports in Dentistry
title Surgical Orthodontic Treatment of a Patient Affected by Type 1 Myotonic Dystrophy (Steinert Syndrome)
title_full Surgical Orthodontic Treatment of a Patient Affected by Type 1 Myotonic Dystrophy (Steinert Syndrome)
title_fullStr Surgical Orthodontic Treatment of a Patient Affected by Type 1 Myotonic Dystrophy (Steinert Syndrome)
title_full_unstemmed Surgical Orthodontic Treatment of a Patient Affected by Type 1 Myotonic Dystrophy (Steinert Syndrome)
title_short Surgical Orthodontic Treatment of a Patient Affected by Type 1 Myotonic Dystrophy (Steinert Syndrome)
title_sort surgical orthodontic treatment of a patient affected by type 1 myotonic dystrophy steinert syndrome
url http://dx.doi.org/10.1155/2017/7957961
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AT mariamoretti surgicalorthodontictreatmentofapatientaffectedbytype1myotonicdystrophysteinertsyndrome
AT giuliogasparini surgicalorthodontictreatmentofapatientaffectedbytype1myotonicdystrophysteinertsyndrome
AT sandropelo surgicalorthodontictreatmentofapatientaffectedbytype1myotonicdystrophysteinertsyndrome
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