Patients with Ehlers Danlos Syndrome are frequent attenders in Maxillofacial Departments
This article gives an overview on Ehlers Danlos Syndrome (EDS) and the increasing public awareness due to increasing number of EDS patient organisations.
Joint hypermobility is the cardinal characteristic of EDS, and this letter is pointing out the patient’s journey with manifestation of symptoms in their jaw joints. In fact spontaneous dislocation of temporo-mandibular joints (TMJ) may be one of the first symptoms EDS patients present with. EDS patients are frequent attenders in accident and emergency departments with a unilaterally or bilaterally dislocated jaw joint.
In a dislocated jaw joint the condyle is displaced out of its fossa stretching the already hyperlax joint ligaments.[1,2] The latter contributes to further and repeated dislocations of the joints which can be caused by trivial movements of the mandible such as eating and yawning. More bothersome to the patient is the concurrent spasm of surrounding muscles of mastication which make the repositioning of the jaw joint with hippocratic maneuver rather challenging in an awake patient.
EDS patients with a dislocated jaw joint often spend some time in A&E departments waiting for their admission for their general anesthetic procedure with their mouth in an open lock position. After repositioning of the jaw joints inter-maxillary fixation is placed to limit the mouth opening temporarily. Unfortunately patients frequently re-dislocate their jaw shortly after inter-maxillary fixation has been removed. Surgery is often the treatment of choice in order to break the vicious circle of recurrent dislocation of the jaw joints. The surgery aims to limit the range of movement of the condyle. Alternatively the articular eminence can be removed which would prevent further dislocations.
Furthermore removal of the TMJ condyle (condylectomy) has been reported for treatment of recurrent dislocations.[2] Closer collaboration among GPs, GDPs and Rheumatologists helps to raise awareness and identify ED patients with TMJ manifestation symptoms sooner.
1. Temporomandibular joint dysfunction syndrome: a close association with systemic joint laxity (the hypermobile joint syndrome) Buckingham RB, Braun T, Harinstein DA, Oral K, Bauman D, Bartynski W, Killian PJ, Bidula LP. Oral Surg Oral Med Oral Pathol. 1991 Nov;72(5):514-9.
2. Condylectomy: treatment of recurrent unilateral dislocation of the temporomandibular joint in a patient with Ehlers-Danlos syndrome. Campbell SJ, Chegini S, Heliotis M. Br J Oral Maxillofac Surg. 2019 Jan;57(1):76-78.
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Patients with Ehlers Danlos Syndrome are frequent attenders in Maxillofacial Departments
This article gives an overview on Ehlers Danlos Syndrome (EDS) and the increasing public awareness due to increasing number of EDS patient organisations.
Joint hypermobility is the cardinal characteristic of EDS, and this letter is pointing out the patient’s journey with manifestation of symptoms in their jaw joints. In fact spontaneous dislocation of temporo-mandibular joints (TMJ) may be one of the first symptoms EDS patients present with. EDS patients are frequent attenders in accident and emergency departments with a unilaterally or bilaterally dislocated jaw joint.
In a dislocated jaw joint the condyle is displaced out of its fossa stretching the already hyperlax joint ligaments.[1,2] The latter contributes to further and repeated dislocations of the joints which can be caused by trivial movements of the mandible such as eating and yawning. More bothersome to the patient is the concurrent spasm of surrounding muscles of mastication which make the repositioning of the jaw joint with hippocratic maneuver rather challenging in an awake patient.
EDS patients with a dislocated jaw joint often spend some time in A&E departments waiting for their admission for their general anesthetic procedure with their mouth in an open lock position. After repositioning of the jaw joints inter-maxillary fixation is placed to limit the mouth opening temporarily. Unfortunately patients frequently re-dislocate their jaw shortly after inter-maxillary fixation has been removed. Surgery is often the treatment of choice in order to break the vicious circle of recurrent dislocation of the jaw joints. The surgery aims to limit the range of movement of the condyle. Alternatively the articular eminence can be removed which would prevent further dislocations.
Furthermore removal of the TMJ condyle (condylectomy) has been reported for treatment of recurrent dislocations.[2] Closer collaboration among GPs, GDPs and Rheumatologists helps to raise awareness and identify ED patients with TMJ manifestation symptoms sooner.
1. Temporomandibular joint dysfunction syndrome: a close association with systemic joint laxity (the hypermobile joint syndrome) Buckingham RB, Braun T, Harinstein DA, Oral K, Bauman D, Bartynski W, Killian PJ, Bidula LP. Oral Surg Oral Med Oral Pathol. 1991 Nov;72(5):514-9.
2. Condylectomy: treatment of recurrent unilateral dislocation of the temporomandibular joint in a patient with Ehlers-Danlos syndrome. Campbell SJ, Chegini S, Heliotis M. Br J Oral Maxillofac Surg. 2019 Jan;57(1):76-78.
Competing interests: No competing interests