Craniofacial Morphology and Upper Airway Dimensions in Patients with Hypermobile Ehlers-Danlos Syndrome Compared to Healthy Controls.

Abstract

ABSTRACTObjectivesThe aims of the present case-control study were to compare craniofacial morphology, airway minimum cross-sectional area and airway volume between patients with hypermobile Ehlers-Danlos syndrome and healthy controls. Material and MethodsThe sample comprised 18 hypermobile Ehlers-Danlos syndrome (hEDS) patients (16 females, 2 males, mean age 34.1 [SD 10.35] years), clinically diagnosed and genetically tested in order to exclude other types of EDS, and 16 controls (14 females, 2 males, mean age 37.9 [SD 10.87] years) with neutral occlusion and normal craniofacial morphology. Craniofacial morphology was assessed on lateral cephalograms. Minimum cross-sectional area and upper airway volume were assessed on cone-beam computed tomography and analysed by standard and well-validated methods. Differences were tested by logistic regression analysis adjusted for age, gender and body mass index (BMI).ResultsNo significant differences in craniofacial morphology were found between hEDS patients and controls. Airway minimum cross-sectional area (P = 0.019) and airway volume (P = 0.044) were significantly smaller in hEDS patients compared to controls. When adjusted for age, gender and BMI no significant differences were found. However, minimum cross-sectional area was almost significant (P = 0.077). ConclusionsThe craniofacial morphology and airway dimensions of hypermobile Ehlers-Danlos syndrome patients were comparable to controls, with a tendency towards a smaller minimum cross-sectional area in the hypermobile Ehlers-Danlos syndrome group. The results may prove valuable for understanding the effect of hypermobile Ehlers-Danlos syndrome on craniofacial morphology and the upper airways.