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

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.

Evaluation of natural head position over five minutes: A comparison between an instantaneous and a five‐minute analysis with an inertial measurement unit

“…For each segment and axis, the first to the second session was compared. Shown are the mean and standard deviation (SD) of the difference between the two sessions, Pearson's correlation coefficient (r), the intraclass correlation coefficient (ICC) 17 with 95% confidence interval (95% CI), and their standard error measurement (SEM) for each axis.…”

“…The study of Billiaert et al 16 as well as Al-Yassary et al 19 demonstrated excellent accuracy of the IMU system for measuring head position. Note: Shown are the mean and standard deviation (SD) of the difference (T 1 -T 2 ) between the instantaneous (corresponding to T 1 ) and the five-minute recording (corresponding to T 2 ), Pearson's correlation (r), the intraclass correlation coefficient (ICC) 17 with 95% confidence interval (95% CI), and their standard error measurement (SEM) for each axis.…”

“…For each set of measurements, the difference between the means was calculated, with p values inferior to .05 considered statistically significant. Pearson's correlations and intraclass correlation (ICC)* 17 with 95% confidence intervals (95% CI) (based on a mean‐rating (k = 2), absolute‐agreement, 2‐way mixed‐effects model) were also calculated. ICC values less than 0.5 are indicative of poor reliability, values between 0.5 and 0.75 indicate moderate reliability, values between 0.75 and 0.9 indicate good reliability, and values greater than 0.90 indicate excellent reliability 18 .…”

Major clinical findings of ortho-surgical procedures in Class II patients: a concise systematic review

“…In the ortho-surgery treatment, the anterior open bite (AOB) is the lack of vertical contact or negative overbite between the anterior teeth of the superior and inferior arches when the posterior teeth are in occlusion [1][2][3]. The AOB is an easily recognized malocclusion and presents only aesthetic and functional problems [4,5]. Its etiology is multifactorial, including heredity, oral habits, unfavorable growth patterns, and increased lymphatic tissue along with mouth breathing and functional oral matrices.…”

Main clinical approaches to orthognathic surgery for class II open bite and genioplasty patients: a brief systematic review

“…Moreover, anterior open bite (AOB) is the lack of vertical contact or negative overbite between the anterior teeth of the superior and inferior arches when the posterior teeth are in occlusion [15,16]. The AOB is an easily recognized malocclusion and presents only aesthetic and functional problems [16].…”