Ehlers-Danlos Syndrome (EDS) type IV. Review of the literature

Ehlers-Danlos syndrome (EDS) is a heterogeneous group of connective tissue heritable disorders. EDS type IV is a rare form that presents typical clinical signs, such as easy bruising and haematomas at sites of trauma, skin manifestations (translucent skin with visible veins), and joint hyperlaxity. To illustrate the dermatological features and describe an aggressive periodontitis, a symptom not yet reported in this EDS type, we present a case of a 23-year-old young man. This patient has been suffering from bruised skin, haematomas, and varicose veins in his legs. These lesions, typical of EDS type IV, were associated with trauma followed by slow and difficult cicatrization. Teeth loss and clinical attachment loss in all the remaining teeth, a symptom compatible with a severe destruction of the periodontal support, was reported after orthodontic treatment. The treatment is limited to control the disease and teeth loss. Considering this new clinical symptom associated with EDS type IV, we suggest that the use of orthodontic apparatus should be carefully considered in such patients.     

牙周病型Ehlers-Danlos综合征研究进展

Ehlers-Danlos综合征（Ehlers-Danlos syndromes，EDS）是一组罕见的遗传异质性结缔组织疾病，表现为广泛的不同程度的皮肤、韧带、血管和内部器官结缔组织脆性增加，主要临床特征包括皮肤脆弱、易擦伤、皮肤弹性过度和关节活动过度等。2017年，国际EDS联盟重新修订了EDS分类方法，根据临床表现、分子诊断及致病基因将其分为13型，其中造成早期牙周组织严重破坏和牙齿松动脱落的主要是牙周病型EDS。文章就牙周病型EDS的研究进展做一综述。     

Orthodontic treatment protocol of Ehlers-Danlos syndrome type VI

Ehlers-Danlos syndrome (EDS) type VI is an autosomal recessive disorder of the connective tissue characterized by joint hypermobility, muscle hypotonia, scoliosis, and ocular fragility. In this case report, an EDS type VI patient with a skeletal and dental Class III malocclusion is presented and the clinical approach to his orthodontic problem is emphasized. A 17-year-old male patient presenting some major and minor symptoms of the syndrome was referred to our orthodontic department for diagnosis and treatment. The typical clinical signs confirmed the diagnosis of EDS type VI. He was a skeletal and dental Class III malocclusion patient (both mandibular protrusion and maxillary retrusion) with a noncontributory family history. He had severe crowding in the lower and upper dental arches with retruded incisors. His first treatment plan included orthognathic surgery, but because of the risks of bleeding and poor healing, we elected to treat the patient without surgery.     

Abnormal oral mucosal light reflectance: a new clinical sign of Ehlers-Danlos syndrome

Ehlers-Danlos syndrome (EDS) is a major inherited connective tissue disorder leading to an impaired extracellular matrix structure. Although several odontostomatologic signs have been reported, their diagnostic accuracy remains to be ascertained. We tested the hypothesis that EDS is associated with an abnormal reflectance of the oral mucosa. Twelve patients with EDS-II or EDS-III and 12 age- and gender-matched controls were examined. Reflectance of the lower gingival and vestibular oral mucosa in the optical spectrum was measured using an imaging spectrophotometer. EDS patients showed significantly higher reflectance values in the 400-590 nm wavelengths (P10.51% at the 400 nm wavelength identified the EDS patients with 100% sensitivity and 100% specificity. These findings indicate that an abnormal oral mucosal reflectance is a previously unrecognized clinical marker of EDS.     

Temporomandibular joint problems and self-registration of mandibular opening capacity among adults with Ehlers-Danlos syndrome. A questionnaire study

Authors – Hagberg C, Korpe L, Berglund B Objectives – To study maximal mandibular opening capacity and the prevalence of temporomandibular joint (TMJ) problems reported among a larger group of adults with Ehlers‐Danlos syndrome (EDS). Furthermore, to compare proportions of disorders with those in a cohort of randomized population‐ based controls. Design – A questionnaire study with self‐registration of maximal mandibular opening capacity. Setting and Sample Population – One hundred and fourteen persons with EDS and 114 controls in a randomized population‐based cohort. Experimental variables – Self‐registered maximal mandibular opening capacity values that were calculated from the markings of maximal interincisal distance on spatulas and the assessments of overbite with the aid of photos. Questions concerning EDS, TMJ problems and other related questions. Outcome Measure – Mean values of maximal mandibular opening capacity compared between groups. Proportions of affirmative answers about TMJ problems compared between persons with EDS and controls who did not have the syndrome. Results – The EDS persons who reported problems with poor mouth opening capacity when biting into thick pieces of food had a lower mean maximal mandibular opening value compared with the other EDS persons (p < 0.05). The proportions of affirmative answers concerning mobile joints during mouth opening, present TMJ problems, poor mouth opening capacity when biting into thick food, clicking, crepitations and permanent locking were greater compared with the controls (p < 0.05). Conclusion – These data corroborate the reports in literature that persons with EDS are naturally predisposed to TMJ problems. The self‐registration of maximal mandibular opening capacity was a useful diagnostic tool to provide an objective clinical measure of movement capacity of the TMJ. The clinical measure was in line with the affirmative answers on having problems with poor mouth opening capacity among the EDS persons.     

Treatment of periodontal disease in a patient with Ehlers-Danlos syndrome. A case report and literature review

BACKGROUND: Ehlers-Danlos syndrome (EDS) designates a heterogeneous group of connective tissue disorders characterized by skin elasticity, tissue fragility, and chronic joint pain. Dental findings have been reported with some types of EDS. This case report describes the periodontal findings in a patient with a previously undiagnosed EDS type VIII. METHODS: Diagnostic aids utilized included microbial testing, histological examination, gingival crevicular fluid (GCF) analysis for the levels of C-telopeptide pyridinoline cross-links (ICTP), and genetic counseling. Periodontal treatment consisted of mechanical debridement and adjunctive antibiotic therapy. RESULTS: Genetic counseling and clinical presentation confirmed the diagnosis of EDS type VIII. Periodontal treatment led to marked clinical improvements and GCF levels of the bone resorptive marker ICTP were significantly reduced. The patient and her siblings are currently pursuing appropriate medical care and genetic counseling. CONCLUSION: Periodontal involvement may lead to the diagnosis of an underlying systemic condition. Identification of suspected etiological factors of periodontal disease may prove critical for the general well-being of some patients.     

Ehlers⁃Danlos综合征的口腔颌面部表现

Ehlers-Danlos综合征是一组罕见的临床和遗传异质性的结缔组织疾病,表现为广泛的不同程度的皮肤、韧带、血管和内部器官结缔组织脆性增加,主要的临床特征包括皮肤脆弱,易擦伤,皮肤延展性过度和关节活动过度.尽管目前有关Ehlers-Danlos综合征口腔颌面部表现的临床数据并不多,但文献检索发现几乎Ehlers-Da...     

Ligneous periodontitis and Ehlers-Danlos syndrome

BACKGROUND: Generalized membranous gingival enlargement due to an accumulation of fibrin deposits associated with severe alveolar bone loss (ligneous periodontitis) is a rare condition, and plasminogen deficiency seems to play a central role in its pathogenesis. However, this condition has not been described in association with syndromes. This article reports a case of ligneous periodontitis in a boy with the classic type of Ehlers-Danlos syndrome (EDS). METHODS: A 12-year-old white male presented with generalized gingival overgrowth and severe alveolar bone loss. A physical examination revealed clinical signs of EDS (velvety skin with mild hyperextensibility, marked hypermobility of the limb joints, atrophic scars on his knees, and easy bruising), which is associated with a positive family history for joint hypermobility. A biopsy of gingival tissues was submitted for routine histology, hematoxylin and eosin (H&E), and direct immunofluorescence (antifibrinogen). An evaluation of plasminogen activity was also performed. RESULTS: Histopathology revealed chronic periodontitis with fibrinoid material deposition, and direct immunofluorescence proved to be positive for fibrin. Functional plasminogen was reduced. A conclusive diagnosis of ligneous periodontitis due to plasminogen deficiency associated with the classic type of EDS was rendered. CONCLUSIONS: Ehlers-Danlos syndrome can be associated with ligneous periodontitis. In the present case, the histologic examination represented an important tool in the differential diagnosis, because it ruled out EDS type VIII as the associated systemic factor to periodontal breakdown.     

Oral health in prevalent types of Ehlers–Danlos syndromes

BACKGROUND: The Ehlers-Danlos syndromes (EDS) comprise a heterogenous group of heritable disorders of connective tissue, characterized by joint hypermobility, skin hyperextensibility and tissue fragility. Most EDS types are caused by mutations in genes encoding different types of collagen or enzymes, essential for normal processing of collagen. METHODS: Oral health was assessed in 31 subjects with EDS (16 with hypermobility EDS, nine with classical EDS and six with vascular EDS), including signs and symptoms of temporomandibular disorders (TMD), alterations of dental hard tissues, oral mucosa and periodontium, and was compared with matched controls. RESULTS: All EDS subjects were symptomatic for TMD and reported recurrent temporomandibular joint (TMJ) dislocations. Abnormal pulp shape (13%) and pulp calcification (78%) were observed in subjects affected with classical EDS. Caries experience was higher in EDS compared with controls and was related to poor oral hygiene, influenced by increased mucosal fragility and restraint of (wrist) joint mobility. The overall periodontal status in EDS was poor, with 62% of EDS subjects presenting high periodontal treatment needs (community periodontal index for treatment need, CPITN = II). CONCLUSION: Oral health may be severely compromised in EDS as a result of specific alterations of collagen in orofacial structures. When considering dental treatment in EDS, a number of tissue responses (mucosa, periodontium, pulp) and precautions (TMJ dislocation) should be anticipated.     

Ehlers-Danlos Syndrome (EDS) focusing on oral symptoms: a questionnaire study

OBJECTIVES: To study the prevalence of oral problems reported among a large group of adults with Ehlers-Danlos Syndrome (EDS). Furthermore, to compare proportions of disorders and general psychological well-being with those in a cohort of randomized population-based controls. DESIGN: A questionnaire study. SETTING AND SAMPLE POPULATION: A total of 265 persons with EDS (response rate 77%) and 750 controls (response rate 63%) in a population-based cohort. Sixty persons were excluded from the EDS group (support members, children, undiagnosed EDS) leaving a final study group of 144 adults. The final control group consisted of 331 persons since many had sent back unanswered questionnaires. EXPERIMENTAL VARIABLES: Questions concerning general and oral symptoms, VAS scales for masticatory muscle pain ratings and General Health Questionnaire (GHQ12) questions were included. OUTCOME MEASURE: Proportions of affirmative answers in EDS group and controls were compared. Mean values for ratings on VAS scales. Proportions of GHQ12 scores with a threshold score of four or more. RESULTS: The proportions of affirmative answers for persons with EDS concerning general health problems, oral problems and masticatory muscle symptoms were significantly higher compared with controls. In the EDS group those who had daily pain in the masticatory muscles had significantly higher mean values on the VAS scales compared with those who had pain a few days per month. A GHQ12 score of four or more was significantly more common in the EDS group than in the controls, suggesting that a decline in psychological well-being was also more common in this group. CONCLUSION: It is important that dental practitioners should be aware of the oral problems associated with EDS and the impact the disease has on quality of life.     

Aspects on dental hard tissues in primary teeth from patients with Ehlers–Danlos syndrome

Background.  Ehlers–Danlos syndrome (EDS) is a rare hereditary condition affecting connective tissues and dental hard tissues.
Hypotheses.  Primary enamel and dentine from EDS patients were expected to differ from those of healthy subjects regarding morphology and chemical composition.
Design.  Forty-seven exfoliated primary teeth from 25 patients with EDS were investigated. Morphology was studied using a polarized light microscope, scanning electron microscope, and X-ray microanalysis. Comparisons were made with 36 primary teeth from 36 healthy patients.
Results.  Morphological analysis of enamel in EDS teeth showed a high frequency of postnatally hypomineralized enamel and postnatally located incremental lines, whereas dentine was normal in all patients. Chemical analysis could not reveal any differences between EDS and control patients except for lower content of C and a higher Ca/P ratio in the enamel in the EDS teeth, indicating porous enamel. Regarding dentine, EDS teeth had a lower content of C, and a higher content of Ca, P, and O. Ratios for Ca/C and Ca/O were also higher compared with controls.
Conclusions.  There are several aberrations of booth enamel and dentine in primary teeth from patients with EDS. These could explain the occurrence of both more dental caries and tooth fractures in patients with EDS.     

Excessive daytime sleepiness in temporomandibular disorder patients

ABSTRACT

Objective: To investigate the relationship between excessive daytime sleepiness (EDS) and associated factors in temporomandibular disorder (TMD) patients.

Methods: Medical records of 350 TMD patients were collected. The EDS status was measured by Epworth Sleepiness Scale (ESS). TMD patients were classified according to ESS scores into TMD patients with EDS (ESS ≥10) and without EDS (ESS <10). The relationship between EDS status and associated factors, such as demographic, TMD symptom severity, and psychological status was analyzed using chi-square and t-test. The level of statistical significance was set at 0.05.

Results: Approximately 28.57% of TMD patients presented with EDS. These patients possessed a significantly higher level of TMD symptom severity, stress, anxiety, and depression, compared to TMD patients without EDS.

Discussion: TMD patients with EDS distinctively suffer both physically and psychologically. This warrants further investigation for the causes and effects as well as underlying mechanisms of EDS in TMD.     

Ehlers-Danlos syndrome: classifications, oral manifestations, and dental considerations

Ehlers-Danlos syndrome (EDS) is the name given for 6 types of connective tissue disorders. While the prevalence of this disease is small, it is seen on every continent and affects both sexes and all races. The various types of EDS are reviewed with reference given to both the older Berlin nosology and the newer Villefranche nosology. Phenotypes of EDS vary depending upon which type of collagen is altered, leading the practitioner to the diagnosis before biochemical confirmation is obtained. In this regard, because collagen is present throughout the head and neck, oral and maxillofacial manifestations of the disease are discussed and are readily noticeable to the astute dentist. Specialists in several fields of dentistry are made aware of the complications EDS can pose on treatment, healing, and follow-up care.     

Problems in the orofacial region associated with Ehlers-Danlos and Marfan syndromes: a case series

Ehlers-Danlos syndrome (EDS) and Marfan syndrome (MFS) are characterised by hypermobility of joints and cardiovascular morbidity, and typical orofacial signs and symptoms are associated with both. Basic knowledge of these should prevent late-stage diagnosis and enable adequate management. This case series comprises all EDS and MFS patients who consulted the Department of Oral and Maxillofacial Surgery at University Hospitals Leuven between 2005 and 2017. Thirty patients had EDS or MFS, and in seven the diagnosis was made based on temporomandibular dysfunction or craniofacial dysmorphism. Non-facial symptoms led to diagnosis in the remaining patients. Twenty-five interventions were followed by two infections and one haemorrhage. One patient developed trismus. No major complications were reported. The complications we observed did not seem to differ from those often seen in patients who did not have EDS or MFS, possibly as a result of our strictly applied precautions and postoperative instructions. However, our study of relevant publications shows that patients with EDS and MFS are a vulnerable group.     

Ehlers Danlos syndrome type I with novel dental features

The clinical, radiographic and histologic findings are described in two cases of Ehlers Danlos Syndrome Type I with novel dental features. Defective dentinogenesis principally affecting the mandibular incisors result in aplasia or hypoplasia of root development predisposing to localized periodontal disease. A striking radiographic appearance with a bulbous enlargement of the roots together with pulp stones is seen in other teeth. 'Giant channels' and vascular inclusions resembling 'intermediate cementum' are prominent within this area. No evidence of Type III procollagen or collagen was detected with indirect immunofluorescence. It is suggested that an inherited collagen abnormality in a component common to dentin, skin, ligament and tendon probably explains both EDS I and the dentin dysplasia.     

High temperature reactivity between titanium and refractory oxides in dental casting procedure. Fundamental study on refractoriness of investments and ceramo-metal bonding by analytical SEM and oxide single crystals

The reactivity of various refractory oxides (SiO2, Al2O3, MgO, CaO, ZrO2) with molten titanium was investigated by energy dispersive X-ray spectroscopy (EDS), using single crystals of oxide as specimen. The region of the reaction boundary between titanium and single crystal oxide was observed by electron microscopy (SEM). Elemental analysis across the Ti/oxide boundary was done by EDS and the reaction layer thickness was quantitatively determined. Of these refractory oxides, SiO2 is the most reactive with titanium followed by Al2O3. The 250 microns thick Si-rich region and 100 microns thick Al-rich region were formed respectively in Ti around the remnant oxides. The diffusion of Si as a result of reaction is greater than that of Al. MgO, CaO, ZrO2 show very little reaction with Ti and the reaction layer thickness was below the spatial resolution of EDS (approximately 1 micron). The thickness and composition of the reaction layer and reactivity were determined. These results were in good agreement with those obtained by ESCA.     

Ehlers-Danlos syndrome (classic type): report of a case presenting with an unusual dental anomaly

Ehlers-Danlos syndrome (EDS) comprises a group of generalised connective tissue disorders. Deficiency or alteration of the collagen present in the tissues results in some classic signs such as skin hyper extensibility, joint hypermobility, and vascular fragility. Multiple supernumerary teeth, congenitally missing teeth, and odontogenic keratocysts have been reported in some patients with EDS. To the author's knowledge, transposition of permanent canines has not previously been reported in any case of EDS. This case report presents the dental findings of a sporadic case of classic-type EDS in a 14-year-old Chinese male who had transposition of a permanent maxillary canine as well as the presence of two supernumerary premolars in the mandible.     

How to become involved in EDS.

This article presents ways in which dental students may become involved in EDS. It is important that EDS has the support of dental students as it is a Committee which works in their interest.     

Corrosion of orthodontic brackets: qualitative and quantitative surface analysis

ObjectivesTo determine and compare surface characteristics and presence of corrosion in new and used brackets with optical light microscopy (OLM) and scanning electron microscopy (SEM), and with elemental chemical analysis with energy-dispersive X-ray spectroscopy (EDS).Materials and MethodsOLM and SEM were used to analyze 24 new and 24 used conventional premolar brackets. EDS analysis was performed in six used brackets and four new brackets with corrosion-suspected spots.ResultsOLM and SEM images showed wear/abfraction signs, striations, pits/crevices, and adherent material. Used brackets showed more deterioration than new brackets. SEM images disclosed more morphological features than OLM images. EDS analysis revealed a significantly higher phosphorus (P = .001) and sodium (P < .005) weight fraction and significantly lower amounts of chromium (P < .001) in used brackets. The iron, chromium, and nickel weight fractions did not differ significantly between the clean and corrosion-suspected spots. Of the corrosion-suspected spots analyzed by combined SEM and EDS, 44.14% and 6.90% remained corrosion-suspected on used and new brackets, respectively.ConclusionsUsed brackets showed more signs of corrosion than new ones. Combined assessment of SEM and EDS indicates that the bracket surface is affected during orthodontic treatment as a result of corrosion.     

Retention of maxillary implant overdenture bars of different designs

STATEMENT OF PROBLEM: The specific degree of retention for overdenture attachments is unknown in relation to design, location, and alignment to supporting dental implants. PURPOSE: The purpose of this study was to evaluate the initial retention characteristics of 5 implant maxillary overdenture designs under in vitro dislodging forces. MATERIAL AND METHODS: A simulated edentulous maxilla was fabricated with 4 screw-type 3.75 x 13-mm implants anteriorly. Five overdenture designs with the following attachments were evaluated: 4 plastic Hader clips with an EDS bar; 2 plastic anterior Hader clips with an identical EDS bar; 2 Hader clips with 2 posterior ERA attachments; 3 Zaag attachments on a bar; and 4 Zaag attachments with no bar. Overdentures were fabricated with full palatal coverage. Each design was subjected to 10 consecutive retention pulls on a universal testing machine. Data were subjected to analysis of variance and t tests to determine differences. RESULTS: The highest average value after 10 pulls was 19.8 lb for the combination ERA and Hader clip design. The lowest retentive values were recorded for the 2 and 4 Hader clip designs (5.08 +/- 0.89 lb and 5.06 +/- 0.67 lb, respectively). Retention decreased over the course of consecutive pulls for all designs, especially for the most retentive designs. The smallest retention decrease occurred with the least retentive designs. CONCLUSION: The results of this in vitro study suggest that the precise selection and placement of attachments may affect the clinical success of maxillary implant-retained overdentures.