Three-dimensional assessment of pharyngeal airway in individuals with myotonic dystrophy type 1

The objectives of this study were to assess pharyngeal airway volume (PAV) in patients with myotonic dystrophy type 1 (DM1) by cone-beam computerized tomography (CBCT) and to evaluate the impact of diaphragm thickness and pulmonary function tests on PAV.

Thirty DM1 patients (10 female and 20 male; mean age 42.40 ± 12.07) were included in the study. Age and sex-matched thirty patients were participated as control group. In DM1 group pulmonary function tests (PFT) were performed. Independent t-test was used to compare PAV values of patients with DM1 and control group. The Mann–Whitney U test was used to compare the parameters according to sex ( p < 0.05). Pearson and Spearman correlation tests were used to evaluate the relationships between parameters of DM1 patients (p < 0.05). A multiple linear regression analysis was performed to explain the PAV with parameters that showed positive correlation with PAV.

Age of onset and disease duration were 22.37 ± 8.45 and 20.03 ± 12.08, respectively, in patients with DM1. PAV values of control group were significantly lower than DM1 group ( p < 0.001). Forced expiratory volume in 1 s and forced volume vital capacity values were higher in males than females in DM1 group according to sex ( p < 0.001). PAV values were greater in male patients than females of the DM1 group ( p = 0.022). Diaphragm thickness in DM1 group after inspiration and expiration were 2.60 ± 0.65 and 1.94 ± 0.40, respectively. According to the regression analysis, DTai and FVC were significantly explained the PAV.

PAV was higher in DM1 group. There was a significant positive correlation between diaphragm thickness, pulmonary functions, and PAVs of DM1 patients. The amount of the PAV was mostly influenced by DTai and FVC. It is recommended to evaluate the PAV in patients with DM1 because of impaired respiratory functions and pharyngeal muscle involvement.