Temporomandibular dysfunction in Ehlers-Danlos Syndrome: case report
DOI:
https://doi.org/10.22456/2177-0018.148349Keywords:
Temporomandibular Joint Dysfunction Syndrome, Ehlers-Danlos Syndrome, Case ReportsAbstract
Aim To report a case of Temporomandibular Disorder (TMD) associated with Ehlers-Danlos Syndrome (EDS), hypermobile type. Case Report: A 30-year-old White female patient presented with a chief complaint of severe chronic pain in the temporomandibular joint (TMJ), masticatory muscles, and craniocervical region, in addition to joint noises and daily episodes of dislocation and subluxation, especially when yawning. Her medical history included a previous diagnosis of hypermobile EDS, with recurrent episodes of joint instability, falls, subluxations, and dislocations involving multiple joints. The TMD diagnosis was established based on the DC/TMD protocol and imaging exams, including cone-beam computed tomography and magnetic resonance imaging of the TMJ, which revealed significant morphological and functional alterations. The therapeutic approach was interdisciplinary, involving the use of an occlusal stabilization splint, specialized physical therapy, psychological counseling, pharmacological management, and guidance for controlling harmful oral habits. Discussion: TMD may be associated with joint hypermobility, as observed in EDS, which adds complexity to both diagnosis and management. However, there is still a lack of clinical studies addressing this comorbidity. Conclusion: The patient reported significant improvement in sleep quality, reduction in orofacial and craniocervical pain, and greater functional stability of the TMJ, although symptom fluctuations persist. This case highlights the need for a comprehensive and interdisciplinary approach, considering TMD not as an isolated condition but as a possible manifestation of broader systemic disorders, such as hypermobile EDS.
Downloads
References
Carrara SV, Conti PCR, Barbosa JS. Termo do 1º Consenso em Disfunção Temporomandibular e Dor Orofacial. Dental Press J Orthod. 2010;15(3):114-20.
List T, Jensen RH. Temporomandibular disorders: Old ideas and new concepts. Cephalalgia. 2017;37(7):692-704.
Campos JADB, Carrascosa AC, Bonafé FSS, Maroco J. Epidemiology of severity of temporomandibular disorders in Brazilian women. J Oral Facial Pain Headache. 2014;28(2):147-52.
Valesan LF, Da-Cas CD, Réus JC, Denardin ACS, Garanhani RR, Bonotto D, et al. Prevalence of temporomandibular joint disorders: a systematic review and meta-analysis. Clin Oral Investig. 2021;25(2):441-53.
Ferreira CLP, Silva MAMR, Felício CM. Sinais e sintomas de desordem temporomandibular em mulheres e homens. CoDAS. 2016;28(1):17-21.
Lemos GA, Paulino MR, Forte FDS, Beltrão RTS, Batista AUD. Influence of temporomandibular disorder presence and severity on oral health-related quality of life. Rev Dor. 2015;16(1):10-4.
Paulino MR, Moreira VG, Lemos GA, Silva PLPD, Bonan PRF, Batista AUD. Prevalence of signs and symptoms of temporomandibular disorders in college preparatory students: associations with emotional factors, parafunctional habits, and impact on quality of life. Cien Saude Colet. 2018;23(1):173-86.
Malfait F, Francomano C, Byers P, Belmont J, Berglund B, Black J, et al. The 2017 international classification of the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017;175(1):8-26.
Malfait F, De Paepe A. The Ehlers-Danlos Syndrome. In: Halper J, editor. Progress in Heritable Soft Connective Tissue Diseases. Adv Exp Med Biol. 2014; 802:129-43.
Tinkle BT, Bird HA, Grahame R, Lavallee M, Levy HP, Sillence D. The lack of clinical distinction between hypermobility type of Ehlers-Danlos syndrome and the joint hypermobility syndrome. Am J Med Genet A. 2009;149A (11):2368-70.
Ferrell WR, Tennant N, Sturrock RD, Ashton L, Creed G, Brydson G, et al. Amelioration of symptoms by enhancement of proprioception in patients with joint hypermobility syndrome. Arthritis Rheum. 2004;50(10):3323-8.
Bravo JF. Síndrome de Ehlers-Danlos tipo III, llamado también Síndrome de Hiperlaxitud Articular (SHA): Epidemiología y manifestaciones clínicas. Rev Chil Reumatol. 2010;26(2):194-202.
Beighton P, De Paepe A, Steinmann B, Tsipouras P, Wenstrup RJ. Ehlers-Danlos syndromes: revised nosology, Villefranche, 1997. Am J Med Genet. 1998;77(1):31-7.
Hakim AJ, Grahame R. A simple questionnaire to detect hypermobility: an adjunct to the assessment of patients with diffuse musculoskeletal pain. Int J Clin Pract. 2003;57(3):163-6.
Moraes DA, Cavalcante AC, Brasil AS, Oliveira RR, Assumpção RM, Kowalski SC. Translation into Brazilian Portuguese and validation of the five-part questionnaire for identifying hypermobility. Rev Bras Reumatol. 2011;51(1):54-60.
Miller SMC, Lamari MM, Lamari NM. Síndrome de Ehlers-Danlos - tipo hipermobilidade: estratégias de inclusão. Arq Ciênc Saúde. 2015;22(1):21-7.
Mitakides J, Tinkle BT. Oral and mandibular manifestations in the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017;175(1):220-5.
Conti PCR, Costa YM, Gonçalves DA Svensson P. Orofacial pain and temporomandibular disorders: the impact on oral health and quality of life. Braz Oral Res. 2012;26(Suppl 1):120-3.
Hakim AJ. Joint Hypermobility Syndrome (JHS) and Ehlers-Danlos Syndrome Hypermobility Type (EDS-HM). A Brief Guide for Medical Professionals on Presentation, Diagnosis, and Treatment. Hypermobility Syndromes Association; 2013 [cited 2023 Aug 16]. Available from: http://hypermobility.org/help-advice/hypermobility-syndromes/jhseds-hm-clinicians-guide/
Oelerich O, Daume L, Yekkalam N, Hanisch M, Menne MC. Temporomandibular disorders among Ehlers-Danlos syndromes: a narrative review. J Int Med Res. 2024;52(4):03000605241242582.
Demeslay J, Cosnay M, Gunst D, et al. Prevalence and quality of temporomandibular disorders, chronic pain and psychological distress in patients with classical and hypermobile Ehlers-Danlos syndrome: an exploratory study. Orphanet J Rare Dis. 2023; 18:294.
Willich L, Bohner L, Köppe J, Jackowski J, Hanisch M, Oelerich O. Prevalence and quality of temporomandibular disorders, chronic pain and psychological distress in patients with classical and hypermobile Ehlers-Danlos syndrome: an exploratory study. Orphanet J Rare Dis. 2023; 18:294.
Bech K, Fogh FM, Lauridsen EF, Sonnesen L. Temporomandibular disorders, bite force and osseous changes of the temporomandibular joint in hypermobile Ehlers-Danlos syndrome. J Oral Rehabil. 2022;49(9):872-83.
Sipilä K, Pezold M, Wänman A, Dittmar J, Äyräväinen L, Maixner W, et al. Risk factors for temporomandibular disorders and orofacial pain among women with hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorder: a multicenter cross-sectional study. J Oral Rehabil. 2024;51(1):5-13.
Malfait F, Francomano C, Byers P, Belmont J, Berglund B, Black J, et al. The 2017 international classification of the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017;175(1):8-26.
Castori M, Morlino S, Celletti C, Celli M, Morrone A, Giannini E, et al. Management of pain and fatigue in the joint hypermobility syndrome (aka Ehlers-Danlos syndrome, hypermobility type): principles and proposal for a multidisciplinary approach. Am J Med Genet A. 2012;158A (8):2055-65.
Song JZ, Luong D, Feldman ECH, et al. Psychological interventions for individuals with Ehlers-Danlos syndrome and hypermobility spectrum disorder: a scoping review. Orphanet J Rare Dis. 2023; 18:254.
Schubart JR, Schaefer E, Janicki P, Adhikary SD, Schilling A, Hakim AJ, et al. Resistance to local anesthesia in people with the Ehlers-Danlos syndromes presenting for dental surgery. J Dent Anesth Pain Med. 2019;19(5):261-70.
Wloch K, Simpson M, Gowrie Mohan S. Local anaesthetic resistance in a patient with Ehlers-Danlos syndrome undergoing caesarean section with continuous spinal anesthesia. Anaesth Rep. 2020;8(1):56-8.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2026 Marina Sartório, Giordana Dal Más, Daniela Disconzi Seitenfus Rhem, Karen Dantur Batista Chaves
This work is licensed under a Creative Commons Attribution 4.0 International License.
All articles published in this journal adhere to Creative Commons License Attributions 4.0 (CC BY 4.0), and its use must be ruled by its licensed means. Authors are allowed to agree to additional and separate contracts for the non-exclusive distribution of the article published in this journal, such as publishing in an institutional repository, as long as the article has a recognition of authorship and first publication in this journal.
