Dental Manifestations of Ehlers-Danlos Syndromes: A Systematic Review

Ehlers-Danlos syndromes (EDS) are a group of inherited connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and variable tissue fragility. However, there are limited published data on the dental manifestations of EDS. This review systematically assessed the spectrum of published dental anomalies in various types of EDS. Twentyfour individual case reports/series and 3 longer case-control studies, reporting on a total of 84 individuals with a clinical diagnosis of EDS, were included in the data analysis. The main dental features listed in classical EDS were pulp calcification and localized root hypoplasia. Common dental abnormalities observed in vascular EDS were pulp shape modifications (52.2%), exceeding root length (34.8%), and molar root fusion (47.8%). Dentinogenesis imperfecta is a consistent finding in osteogenesis imperfecta/EDS overlap syndrome. Data on dental manifestations in other types of EDS are both rare and generally inconclusive.

[1]  T. Porntaveetus,et al.  A novel mutation in COL1A2 leads to osteogenesis imperfecta/Ehlers-Danlos overlap syndrome with brachydactyly , 2019, Genes & diseases.

[2]  G. Dahllöf,et al.  Dentinogenesis imperfecta type II in Swedish children and adolescents , 2018, Orphanet Journal of Rare Diseases.

[3]  R. Pfundt,et al.  Biallelic B3GALT6 mutations cause spondylodysplastic Ehlers‐Danlos syndrome , 2018, Human molecular genetics.

[4]  C. Susin,et al.  Manifestations of systemic diseases and conditions that affect the periodontal attachment apparatus: Case definitions and diagnostic considerations , 2018, Journal of periodontology.

[5]  R. Havale,et al.  Ehler Danlos Syndrome: Oral, Ocular Manifestations and Prophylactic Measures – A Rare Case Report , 2018 .

[6]  J. Zschocke,et al.  Periodontal manifestations of Ehlers–Danlos syndromes: A systematic review , 2017, Journal of clinical periodontology.

[7]  E. Hakki,et al.  Dental Findings and Mutational Analysis of a Case with Ehlers-Danlos Syndrome , 2017 .

[8]  L. Bertassoni.  Dentin on the nanoscale: Hierarchical organization, mechanical behavior and bioinspired engineering. , 2017, Dental materials : official publication of the Academy of Dental Materials.

[9]  J. Belmont,et al.  The 2017 international classification of the Ehlers–Danlos syndromes , 2017, American journal of medical genetics. Part C, Seminars in medical genetics.

[10]  B. Tinkle,et al.  Oral and mandibular manifestations in the Ehlers–Danlos syndromes , 2017, American journal of medical genetics. Part C, Seminars in medical genetics.

[11]  M. Deshpande,et al.  Unusual case of globe perforation: the brittle cornea without systemic manifestations , 2016, BMJ Case Reports.

[12]  A. Janas,et al.  Dental problems in a patient with the classic type of Ehlers-Danlos syndrome--a case report. , 2015, Developmental period medicine.

[13]  D. Bowers,et al.  ImageJ analysis of dentin tubule distribution in human teeth. , 2015, Tissue & cell.

[14]  D. Awal,et al.  The role of the dental practitioner in diagnosing connective tissue and other systemic disorders: a case report of a patient diagnosed with type III Ehlers–Danlos syndrome , 2015 .

[15]  G. Aren,et al.  Familial gigantiform cementoma with Ehlers - Danlos syndrome: A report of 2 cases , 2015, The journal of advanced prosthodontics.

[16]  B. Erfanian,et al.  Dental Considerations in Ehlers-Danlos Syndrome: A Case Report , 2015 .

[17]  Jinxiang Han,et al.  Heterozygous mutation of c.3521C>T in COL1A1 may cause mild osteogenesis imperfecta/Ehlers-Danlos syndrome in a Chinese family. , 2015, Intractable & rare diseases research.

[18]  Pragyan Das,et al.  A rare case of Ehler-Danlos syndrome with literature review , 2015 .

[19]  J. Cohen-Lévy,et al.  Painful hyperlaxity (Ehlers-Danlos syndrome) , 2015 .

[20]  Yong Wang,et al.  Type VII collagen is enriched in the enamel organic matrix associated with the dentin-enamel junction of mature human teeth. , 2014, Bone.

[21]  Y. Yilmaz,et al.  Dental Treatments under the General Anesthesia in a Child with Keratitis, Ichthyosis, and Deafness Syndrome , 2013, Case reports in dentistry.

[22]  X. Jeunemaître,et al.  Oral phenotype and scoring of vascular Ehlers–Danlos syndrome: a case–control study , 2012, BMJ Open.

[23]  E. Björck,et al.  Women with Ehlers-Danlos syndrome experience low oral health-related quality of life. , 2012, Journal of orofacial pain.

[24]  Shiu-yin Cho.  Ehlers-Danlos Syndrome (Classic Type): Report of a Case Presenting with an Unusual Dental Anomaly , 2011, Primary dental care : journal of the Faculty of General Dental Practitioners.

[25]  D. Moher,et al.  Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. , 2010, International journal of surgery.

[26]  K. Lahiri,et al.  REVERSE-NAMASKAR: A NEW SIGN IN EHLERS-DANLOS SYNDROME: A FAMILY PEDIGREE STUDY OF FOUR GENERATIONS , 2010, Indian journal of dermatology.

[27]  G. Klingberg,et al.  Aspects on dental hard tissues in primary teeth from patients with Ehlers-Danlos syndrome. , 2009, International journal of paediatric dentistry.

[28]  Sevgi Şener,et al.  Calcifications of the pulp chamber: prevalence and implicated factors , 2009, Clinical Oral Investigations.

[29]  L. Brocklebank,et al.  External apical root resorption: two case reports. , 2008, International endodontic journal.

[30]  N P Chandler,et al.  Pulp stones: a review. , 2008, International endodontic journal.

[31]  C. Cardoso,et al.  Odontogenic keratocyst and multiple supernumerary teeth in a patient with Ehlers-Danlos syndrome--a case report and review of the literature. , 2008, Quintessence international.

[32]  A. Vral,et al.  Abnormal dentin structure in two novel gene mutations [COL1A1, Arg134Cys] and [ADAMTS2, Trp795-to-ter] causing rare type I collagen disorders. , 2007, Archives of oral biology.

[33]  K. Sayınsu,et al.  Orthodontic treatment protocol of Ehlers-Danlos syndrome type VI. , 2009, The Angle orthodontist.

[34]  J. Chies,et al.  Ehlers-Danlos Syndrome (EDS) type IV. Review of the literature , 2007, Clinical Oral Investigations.

[35]  L. Carrasco,et al.  Ehlers-Danlos syndrome: classifications, oral manifestations, and dental considerations. , 2006, Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics.

[36]  Farouk Bassam Rihani,et al.  Multiple developmental dental anomalies and hypermobility type Ehlers-Danlos syndrome. , 2006, The Journal of clinical pediatric dentistry.

[37]  M. Young,et al.  Targeted Disruption of Two Small Leucine-rich Proteoglycans, Biglycan and Decorin, Excerpts Divergent Effects on Enamel and Dentin Formation , 2005, Calcified Tissue International.

[38]  A. De Paepe,et al.  Oral health in prevalent types of Ehlers-Danlos syndromes. , 2005, Journal of oral pathology & medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology.

[39]  I. Springer,et al.  Detection of Mature Collagen in Human Dental Enamel , 2005, Calcified Tissue International.

[40]  A. De Paepe,et al.  The natural history, including orofacial features of three patients with Ehlers–Danlos syndrome, dermatosparaxis type (EDS type VIIC) , 2004, American journal of medical genetics. Part A.

[41]  B. Berglund,et al.  Ehlers-Danlos Syndrome (EDS) focusing on oral symptoms: a questionnaire study. , 2004, Orthodontics & craniofacial research.

[42]  O. Baba,et al.  Post-translational modifications of sibling proteins and their roles in osteogenesis and dentinogenesis. , 2004, Critical reviews in oral biology and medicine : an official publication of the American Association of Oral Biologists.

[43]  A. De Paepe,et al.  Unusual oral findings in dermatosparaxis (Ehlers-Danlos syndrome type VIIC). , 2003, Journal of oral pathology & medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology.

[44]  M Goldberg,et al.  Proteoglycans in dentinogenesis. , 2001, Critical reviews in oral biology and medicine : an official publication of the American Association of Oral Biologists.

[45]  S. Weiner,et al.  A transmission electron microscope study using vitrified ice sections of predentin: structural changes in the dentin collagenous matrix prior to mineralization. , 2000, Journal of structural biology.

[46]  M. Wright,et al.  Clinical phenotypes and molecular characterisation of three patients with Ehlers-Danlos syndrome type VII , 2000, Journal of medical genetics.

[47]  S. Reichert,et al.  Early-onset periodontitis in a patient with Ehlers-Danlos syndrome type III. , 1999, Quintessence international.

[48]  P. Sudha,et al.  Dentin dysplasia--a case report. , 1995, Journal of the Indian Society of Pedodontics and Preventive Dentistry.

[49]  M. Frydman,et al.  Multiple supernumerary teeth (MSNT) and Ehlers-Danlos syndrome (EDS): a case report. , 1994, Journal of oral pathology & medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology.

[50]  P. Speight,et al.  Ehlers Danlos syndrome type I with novel dental features. , 1992, Journal of oral pathology & medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology.

[51]  S. Bhatia,et al.  Sporadic Ehlers-Danlos syndrome with neurologic, cardiac and dental involvement. , 1990, The Journal of the Association of Physicians of India.

[52]  H. Kohno,et al.  Oral manifestations of Ehlers-Danlos syndrome type VII: histological examination of a primary tooth. , 1990, Pediatric dentistry.

[53]  K. Fridrich,et al.  Dental implications in Ehlers-Danlos syndrome. A case report. , 1990, Oral surgery, oral medicine, and oral pathology.

[54]  M. Tsuzaki,et al.  Bovine dental pulp collagens: characterization of types III and V collagen. , 1990, Archives of oral biology.

[55]  A. Leung,et al.  Oral manifestations of Ehlers-Danlos syndrome. , 1989, Journal of the American Dental Association.

[56]  R. Welbury.  Ehlers-Danlos syndrome: historical review, report of two cases in one family and treatment needs. , 1989, ASDC journal of dentistry for children.

[57]  P. Ostertag,et al.  Oral manifestations of Ehlers-Danlos syndrome: report of case. , 1989, Journal of the American Dental Association.

[58]  A. Linde.  Session II: Cells and Extracellular Matrices of the Dental Pulp — C.T. Hanks, Chairman , 1985 .

[59]  L. Norton.  Orthodontic tooth movement response in Ehlers-Danlos syndrome: report of case. , 1984, Journal of the American Dental Association.

[60]  W. Majid,et al.  Ehlers-Danlos syndrome: a case report. , 1981, JPMA. The Journal of the Pakistan Medical Association.

[61]  M. Das,et al.  Ehlers-Danlos Syndrome , 2022, Neurocutaneous Disorders.

[62]  C. L. Hughes.  Odontectomy in treatment of Ehlers-Danlos syndrome: report of case. , 1970, Journal of oral surgery.

[63]  J. Lipman,et al.  The Ehlers-Danlos syndrome. A case requiring oral surgery. , 1969, Oral surgery, oral medicine, and oral pathology.

[64]  S. Hyams,et al.  Blue sclerae and keratoglobus. Ocular signs of a systemic connective tissue disorder. , 1969, The British journal of ophthalmology.

[65]  Nils-Edv. Sellisetii.  Odontologische Befunde Bei Einer Patientin Mit Ehlers—Danlos Syndrom , 1965 .