Ehlers-Danlos syndrome is strongly associated with several oral and mandibular (jaw) conditions, including craniofacial pain and temporomandibular joint (TMJ) syndrome, according to a recent review.
The inclusion of these symptoms may help in the diagnosis and treatment of this clinically and genetically heterogeneous disease.
The review article, “The effect of Ehlers-Danlos syndromes on TMJ function and craniofacial pain,” was published in Cranio, The Journal of Craniomandibular & Sleep Practice.
The review was written by John E. Mitakides, a practicing dentist in Ohio, member of the medical board of the Ehlers-Danlos Society, and staff of The Ehlers-Danlos Society Center for EDS Research & Clinical Care at The Greater Baltimore Medical Center.
Ehlers-Danlos syndrome (EDS) is a group of inherited disorders that affect the connective tissue, which provides structure and support throughout the body.
EDS is caused by mutations in an increasing number of genes. As of 2017, 13 disease subtypes have been recognized. All the subtypes are generally characterized by tissue fragility, joint hypermobility (joints that extend beyond the normal range of motion), and skin hyperextensibility — skin that stretches more than normal.
Collagen, the major component of connective tissue and the most frequently affected protein in EDS, is present throughout the head and neck. Therefore, defects in collagen also impair oral health, including bone, teeth, gum tissue, nerve tissue, blood vessels, as well as tendons and ligaments of the jaw and face, and the temporomandibular joints.
The temporomandibular joints connect the jawbone to the skull in front of the ear, and are essential for talking, chewing, and swallowing.
While oral and jaw problems have been observed in all types of EDS patients for many years, these manifestations are often poorly recognized by healthcare providers as symptoms of EDS.
Recently, studies have started to reveal the specific challenges these patients face with oral and jaw disorders, including craniofacial pain and TMJ syndrome, which can cause pain and dysfunction of the jaw joint and the muscles that control jaw movement.
Several studies have pointed to a link between EDS, joint hypermobility, and TMJ syndrome.
Hypermobility of the temporomandibular joints was shown to be highly frequent in 114 patients with several types of EDS compared to healthy people, and a recent review found that TMJ syndrome has been reported in 40-100% of EDS patients.
Mitakides emphasized that in the presence of TMJ syndrome, healthcare providers should perform a careful observation of the oral cavity, head, and neck tissues — including muscles used to chew — as they might show alterations indicative of EDS.
“Even practitioners highly trained in the area of TMD [TMJ syndrome] can face unexpected challenges in diagnosing and treating an EDS patient if they do not have an in-depth understanding of EDS,” Mitakides wrote.
Because temporomandibular joint hypermobility and generalized joint hypermobility may increase the likelihood of developing TMJ syndrome, preventive measures or treatment should be implemented in EDS patients, he said in the review article.
Lifestyle changes such as altering diet and chewing patterns, stress reduction techniques, and the management of physical activities, can be used to prevent TMJ injuries, according to Mitakides.
Due to the heterogeneity found in EDS patients, treatments to manage pain and TMJ syndrome-associated disorders in these patients should be focused and highly informed, he said.
“Research has confirmed a variety of oral and mandibular manifestations associated with EDS,” Mitakides wrote. “[TMJ] and craniofacial pain resulting from a malfunctioning oral and mandibular structure appear to be highly prevalent in the EDS patient population.
“The exact nature of this relationship remains unclear, however, and thus presents an opportunity for meaningful research, with the goal of providing more effective treatment,” he added.