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

Abstract

The Ehlers-Danlos syndrome (EDS) is a clinically and genetically heterogeneous group of heritable connective tissue disorders characterised by joint hypermobility, skin hyperextensibility and tissue fragility. 1 The estimated prevalence of Ehlers-Danlos syndrome is 1/5000, and the hypermobile EDS subtype (hEDS) is the most frequent. 2 EDS is caused by mutations in genes involved in collagen structure and/or biosynthesis, but the underlying pathophysiological mechanism is still not fully understood. 1 The clinical and genetic heterogeneity of the EDS has long been recognised, and currently, EDS is classified into thirteen subtypes according to the 2017 Abstract 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.

Surgical-orthodontic treatment in patients with Ehlers–Danlos syndrome: a report of two familial cases

“…Previous studies reported an increased prevalence of xerostomia and/or dry mouth sensation in patients with hypermobile-type EDS joint hypermobility. This observation may reflect the frequent multifactorial dysfunction frequently associated with EDS and indirectly increase the risk of caries [9,19].…”

“…Regarding the periodontal parameters, the authors found the plaque index to be significantly higher compared with the control group (mean, 0.396 vs. 0.059, p < 0.001). They also determined a greater attachment loss in the upper left and lower right upper molars and concluded that hypermobile-type EDS is associated with a potential predisposition to periodontal disease [9].…”

“…Some authors also report a lower coronary height in the central maxillary and mandibular incisors in patients with hypermobile EDS. This observation could be a consequence of the structural abnormalities of the connective tissue characteristic of this pathology or reflect greater attrition or associated dentine dysplasia [9]. Structural anomalies are also common.…”

“…The Gorlin sign (ability to touch one's nose with the tip of the tongue) is also more common than in the general population (50% vs. 15%) [11]. However, these clinical signs, although evocative, are not pathognomonic of this syndrome, and prospective studies would be necessary to determine their possible intrinsic diagnostic value [9,12]. The presence of palatal petechial lesions is frequently observed in the context of EDS and other collagen pathologies [13].…”

“…Regarding dental extractions, postoperative complications are more frequent in patients with hypermobile-type EDS than in unaffected individuals. Reported complications include delayed mucosal healing, postoperative hemorrhagic events, and prolonged high-intensity pain [9].…”