Temporomandibular disorders, bite force and osseous changes of the temporomandibular joints in patients with hypermobile Ehlers-Danlos syndrome compared to a healthy control group.

BACKGROUND: Ehlers-Danlos syndrome (EDS) is a hereditary disorder that affects the connective tissue and collagen structures in the body characterised by joint hypermobility, skin hyperextensibility and tissue fragility. OBJECTIVE: The aim was to investigate temporomandibular disorders (TMD), bite force, teeth in occlusal contact and osseous changes of the temporomandibular joints (TMJs) in 26 patients with hypermobile EDS (hEDS), differentiated by a genetic test, compared to 39 healthy controls. METHODS: Clinical examination according to Diagnostic Criteria for Temporomandibular Disorders (DC/TMD), radiological examinations of the TMJs by cone-beam-computed tomographic (CBCT) scans, registration of bite force and teeth in occlusal contact was performed. Statistical analyses included Fisher's Exact Test, multiple logistic and linear regression models adjusted for age, gender and Body Mass Index (BMI). RESULTS: Single symptoms and signs of TMD occurred significantly more often in hEDS (p = .002; p = .001; p = .003; p = <.0001; p = .012) and maximum mouth opening was significantly smaller in hEDS compared to controls (p = <.0001). The DC/TMD diagnosis myalgia, myofascial pain with referral, arthralgia, headache attributed to TMD, disc displacement disorders and degenerative joint disease occurred significantly more often in hEDS compared to controls (p = .000; p = .008; p = .003; p = .000; p = <.0001; p = .010, respectively). No significant differences were found in bite force and in teeth in occlusal contact between the groups (p > .05). On CBCT of the TMJs, subcortical sclerosis occurred significantly more often in hEDS compared to controls (p = .005). CONCLUSION: Symptoms and signs of TMD and osseous changes of the TMJs occurred significantly more often in hEDS. Bite force and teeth in occlusal contact were comparable to controls.

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

OBJECTIVES: The 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 METHODS: The 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). RESULTS: No 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). CONCLUSIONS: The 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.

Treatment Strategies for Patients with Regional Odontodysplasia: A Presentation of Seven New Cases and a Review of the Literature.

PURPOSE: Regional odontodysplasia (RO) is a rare dental anomaly affecting primary and/or permanent dentition, and leads to comprehensive treatment need. The purpose of this study was to present a larger consecutive sample with RO, discuss treatment strategies for patients with RO, and review the literature. MATERIALS AND METHODS: A consecutive, retrospective sample of seven children with RO (6 males, 1 female) including all patients diagnosed with RO in the eastern part of Denmark was conducted over a period of 15 years. The evaluation included gender, localisation and treatment outcome. A review of the literature and cases published within the last 15 years was conducted. RESULT: Referral age was 2-12 years (mean: 7.3 years). The gender ratio was 1:6 (female:male), and the right:left ratio was 3:4. 71% of the patients had RO in the mandible and 29% in the maxilla. 43% had RO in the permanent dentition, while both primary and permanent dentition were affected in 57%. Typically, RO affected incisors and canines. In some patients, RO also affected more distal tooth types. Treatment included early multiple extractions and subsequent combined orthodontics, surgery and prosthetics. A search on RO cases published within the last 15 years was conducted and included 44 cases. The review showed a male and maxillary preponderance. The most common treatment of RO is extraction. CONCLUSION: Treatment of RO should take place in interdisciplinary, specialised teams, and individual treatment plans should be designed. Fewer but more extensive treatment sessions under general anesthaesia may minimise the burden of care for the patients.

Oro-dental characteristics in patients with hypermobile Ehlers-Danlos Syndrome compared to a healthy control group.

BACKGROUND: Ehlers-Danlos syndrome (EDS) is a hereditary disorder that affects the connective tissue and collagen structures in the body characterised by joint hypermobility, skin hyperextensibility and tissue fragility. OBJECTIVE: The aim of the study was to investigate the oro-dental characteristics including measurements of tooth size of 26 patients with hypermobile EDS (hEDS), differentiated by a genetic test, compared to 39 healthy controls. METHODS: Interview, clinical and radiological examination on panoramic radiograph and cone-beam computed tomographic (CBCT) scan were performed. Statistical analyses included Fisher's exact test, paired t test and multiple logistic and linear models adjusted for age and gender. RESULTS: The experience of xerostomia (P = 0.039), local anaesthetic insufficiency (P < 0.001) and tooth extraction complications (P < 0.003) were significantly higher in hEDS compared to controls. The debris index was significantly higher in hEDS (P < 0.001), and the distance between the cement-enamel junction (CEJ) and the bone level on the upper left first incisor and molar and the lower right first molar was significantly larger in hEDS compared to controls (P = 0.021, P = 0.024, P = 0.021, respectively). The crown heights of the upper and lower first incisors were significantly smaller (P = 0.001, P = 0.003, P = 0.002, P < 0.001, respectively) in hEDS compared to controls. When adjusting for debris index, only the distance between CEJ and the marginal bone level on the upper left and lower right molar was associated with hEDS. CONCLUSION: The results indicate that xerostomia, resistance to local anaesthesia, tooth extraction complications, poor oral hygiene, larger distance between CEJ and marginal bone level and small crown heights can be found in patients with hEDS.