Comparison of the Pharyngeal Airway Volume Between Patients With Ectodermal Dysplasia and Unaffected Controls: A Cone-Beam Computed Tomography Study

Purpose: Ectodermal dysplasias (EDs) are a diverse set of hereditary conditions in which 2 or more ectodermal structures develop abnormally. The purpose of this study was to use cone-beam computed tomography (CBCT) scans to measure the pharyngeal airway volume and minimum cross-sectional area (MC) among patients with ED and compare them with healthy controls. Patients and Methods: The pretreatment CBCT scans of 9 individuals with ED and 61 controls were analyzed. Lateral cephalograms were created from the CBCT volumes and then traced and compared between the 2 groups. Airway volumes were evaluated by Dolphin 3D software (Dolphin Imaging, Chats-worth, CA) to compare the total pharyngeal volume, nasopharyngeal volume, oropharyngeal volume, hypopharyngeal volume, and MC. The mean airway volumes of the 2 groups were compared, and the odds ratio and relative risk of having an MC of less than 100 mm(2) in the ED group were calculated. Results: Intraclass correlation analysis showed excellent inter-rater reliability. All cephalometric features of controls were within the norms of patients with ideal skeletal-dental relationships. Patients with ED displayed significantly lower values for SNA (angle between sella-nasion and nasion-A point) (P = .018), ANB (angle between nasion-A point and nasion-B point) (P = .002), L1-MP (angle between long axis of mandibular incisor and gonion-menton plane) (P = .013), and L1-NB (distance between mandibular incisor and perpendicular line between nasion and B point) (P < .001). Although the ED group exhibited slightly smaller airway volumes for all subregions, the differences were not statistically significant for total pharyngeal volume, nasopharyngeal volume, oropharyngeal volume, hypopharyngeal volume, or MC (P > .05). The odds of having an MC of less than 100 mm(2) were almost 3 times greater among ED patients, and the relative risk of an MC of less than 100 mm(2) among ED patients was double that of controls. Conclusions: Although the craniofacial structures of individuals with ED are characterized by maxillary and midface deficiency, the airway volumes among affected individuals may not be significantly different from those of unaffected controls. However, patients with ED displayed a higher risk of having an MC of less than 100 mm(2), which may be of clinical significance and warrants further investigations with larger samples. (C) 2020 American Association of Oral and Maxillofacial Surgeons