Ehlers-Danlos syndrome (EDS) is a genetic disorder that can negatively influence the mouth’s function, resulting in a decreased quality of life. The teeth and the gums, as well as the temporomandibular joint, can be affected by this connective tissue disorder, but many people with EDS do not have any noticeable oral issues as a result of their condition. Furthermore, the systemic problems of EDS may occasionally make it difficult to provide basic dental treatment.
Tooth and Gum Issues in EDS
For the time being, researchers have not conducted comprehensive surveys of individuals with EDS in order to establish the impact of the condition on their oral and dental health. Single research, which included a survey of individuals with classical and hypermobile EDS, discovered a higher prevalence of oral issues, including discomfort, difficult tooth extractions, gum disease, and spontaneous tooth breaking in patients with EDS.
Because EDS patients tend to have readily dislocated joints, the jaw may dislocate as well. Many people have delicate, easily damaged epidermis, which means they are more likely to get mouth injuries. Other complications include bleeding or infections as a result of the longer time it takes for wounds to seal and heal.
Patients suffering from periodontal EDS are more likely to develop severe gum disease, resulting in tooth loss.
The majority of oral issues experienced by patients with EDS will likely be comparable to those experienced by healthy individuals (e.g., tooth decay (caries) and gum disease (gingivitis and periodontitis)) as a result of the impact of dental plaque on the oral mucosa. Because there have been few studies, including large numbers of individuals with well-characterized EDS, it is difficult to determine the precise frequency of oral and facial abnormalities caused by EDS in detail. Each form of EDS has its own set of oral and facial characteristics, but in general, the more skin and mucosa laxity there is in patients’ skin and mucosa, the greater the likelihood of having orofacial characteristics. According to the evidence available, the hemorrhagic forms of EDS are the most likely to cause gingival (gum) bleeding.
Pain and Laxity of the Mucosal Lining of the Mouth
The jaw (temporo-mandibular joint, or TMJ) joint may become painful and dislocated due to EDS’s classical, hypermobile, and vascular forms, among others. In light of the joint’s laxity, the lower jaw (mandible) is more mobile and is more likely to dislocate from its fossa in the temporal bone (the base of the skull), leading the jaw to deviate away from the side of displacement and the patient to be unable to close the mouth.
Sometimes the mandible will move on its own, and other times patients may devise a way by which they will be able to effortlessly move the lower jaw into the fossa, which is a natural process.
However, it is essential to remember that pain in and around the TMJ is not always a direct reflection of EDS. For example, some people with EDS may experience symptoms of a much more common problem known as temporomandibular joint disorder (TMD), which causes pain in the joint and surrounding muscles and a possible limitation of mouth opening. This disease is widespread, and it does not appear to be a result of any anatomical abnormalities in the joints or muscles but instead appears to be a result of some form of psychological anguish. According to some studies, temporomandibular disorder (TMD) is most common in young people and maybe more common in women than in males. The symptoms are frequently responsive to analgesics, and they are commonly alleviated when the anguish of the individual experiencing them subsides.
A well-defined procedure for managing joint laxity in EDS does not exist at the time of writing. Preventing subluxation or dislocation is primarily accomplished by not opening the mouth wide, whereas treatments for pain have included splints, local ultrasound, low-intensity laser, exercises, and acupuncture. Other treatments have included cognitive-behavioral therapy (CBT), transcutaneous electrical nerve stimulation (TENS), and antidepressant medication.
Generally, the gums (gingivae) and periodontal tissues (the tissues that connect the teeth to the bones) are not impacted by EDS in the same way that other tissues are. On the other hand, Type VIII EDS has been linked to an increased risk of gingivitis and periodontitis, resulting in non-painful red bleeding gums, bad breath (halitosis), tooth mobility, and early age tooth loss in children. Periodontal disease has also been linked to both classical and vascular EDS, according to some researchers.
Anomalies of the Teeth
Dental anomalies have been explained in EDS, particularly in the classical and hypermobile types. These include high cusps and deep fissures in premolar and molar teeth, short or abnormally shaped roots with stones in the pulp of crowns, enamel hypoplasia (under development) with microscopic evidence of various enamel and dentine defects, as well as enamel hypoplasia (under development) with microscopic evidence of various enamel and dentine defects. It is possible that enamel flaws would predispose a patient to easy loss of the tissue of crowns (attrition), which, in turn, will result in a reduction in enamel calcification, increasing the risk of caries.
Jaw Bone Anomalies
Aside from the possibility of harm to the jaw joint described above, there is no compelling evidence that EDS leads to abnormalities in the jawbones or other structural problems. Multiple odontogenic keratocysts (which can cause local bone destruction of the jaws) have been reported in patients with vascular EDS.
Having poor oral health has negative consequences for one’s overall health.
Negative Consequences of Poor Oral Health
Dental decay (caries) is a painful condition that limits one’s ability to eat certain foods and may eventually result in excruciatingly painful abscesses. Gingival disease (gingivitis) may create bad breath and make people feel self-conscious when they socialize, but periodontal disease can cause teeth to drift, change the smile, and interfere with the ability to chew food. In the case of EDS, there is the possibility of an additional negative impact from the physical and psychological effects of the laxity of the jaw joint. There is some evidence that EDS might lower nutritional intake and raise the risk of developing eating disorders for various reasons. As a result, reducing the risk of common oral illness is essential since it may add to the burden of issues associated with EDS and its complications.
Everyone needs to practice good oral hygiene to avoid tooth decay and gum disease since this will help to prevent discomfort and the other symptoms listed above. Furthermore, the necessity for extensive dental treatment can be expensive financially and in terms of time (e.g., children miss school, adults, and cares have to take time away from work or other activities). As a result, it is essential for all people who have EDS to consume a diet that prevents the formation of caries and to maintain a high quality of dental hygiene that will reduce the chance of developing caries and gum disease.
Advice on How to Keep Teeth in Good Condition
Dental decay is caused by plaque, which produces acids from carbohydrates that eat away at the teeth and cause them to decay. As a result, the three most important concepts for reducing caries are as follows:
- Clean the teeth to eliminate plaque
- Reduce the consumption of sweets, which contribute to the formation of dental plaque, and
- Use fluoride mouthwashes and toothpaste to protect the surfaces of teeth from the effects of acids.
Avoid Sugary Agents
The consumption of sweet sticky foods should be avoided at all times. Snacking on sweets between meals should also be avoided, and sweet foods should be consumed only at mealtimes. Candy and meals containing sweeteners other than sugar are less cariogenic than sugar, although they might induce gastrointestinal discomfort in certain persons. Dieting does not have to be tedious. Although sugars should not be avoided altogether, wise persons who practice good dental hygiene will have a lower chance of developing caries in most cases. Crisps, nuts (as long as they are not too hard and do not induce TMJ discomfort), and a variety of other flavourful agents provide modest quantities of sugar and may also promote saliva production, which can help to neutralize the effects of acids.
Brush Teeth Thoroughly
Using fluoride-containing toothpaste and an appropriate toothbrush, teeth should be cleaned at least twice a day to keep them healthy. It will be possible to use a variety of tooth brushing techniques (for example, a gentle up-and-down rolling motion or a figure-of-eight motion), but it is essential to remember that the teeth should never be scrubbed in a horizontal direction because this increases the risk of damaging the gums and any exposed root surfaces.
Brushing should involve a moderate massage of the gum margin, as this will aid in the removal of any plaque that may have become trapped under this location over the day. Because toothbrushes only remove plaque and debris from the top and exposed (smooth) surfaces of teeth, it is necessary to clean the regions between teeth (interdental sites) in addition to the surfaces of the teeth themselves. Many different interdental products, like floss, interdental brushes, and interdental sticks, are available to help you clean between your teeth. Care must be used when using floss to prevent traumatizing the gums. In some cases, floss holders can make flossing more convenient, particularly for people who have difficulty reaching the rear teeth. EDS is unlikely to have any substantial consequences for interdental cleaning other than avoiding trauma and opening the mouth to a significantly greater extent.
Fluoride in toothpaste and mouthwashes will reduce the resistance to decay of only the surface layer of enamel, but the fluoride in drinking water will not. It is suggested that you use fluoride-containing toothpaste twice daily. Fluoride mouthwashes can also be beneficial. However, they are unlikely to be required if a patient is already using fluoridated toothpaste in the first place.
Antimicrobial mouthwashes have been shown to lower the risk of gingivitis and periodontitis and reduce bad breath. Mouthwashes are available in various flavors and strengths, and they should be used regularly. There is no conclusive evidence that using mouthwashes containing alcohol increases the risk of developing oral cancer.
Visit the Dentist Regularly
Dentists are trained to treat common dental diseases. A patient with a complicated condition or probable oral symptoms of EDS, for example, will be referred to an appropriate specialist, and they will be able to arrange for further research or treatment on their initiative. NHS Direct is a good source of information on the availability of dentists near you. Dentists with a poor understanding of EDS and its implications for oral health and dental care should send the patient to an appropriate expert for further evaluation and treatment.
Consideration for Different Dental Issues
When teeth are removed, bacteria from the gums enter the bloodstream and cause an infection. The bacteria may adhere to the valve(s) in individuals who have cardiac valve defects, resulting in inflammation (endocarditis) of the valve(s)—recommended that all patients with valvular abnormalities require antibiotics before tooth extractions to prevent bacterial infection. Before this, the National Institute for Clinical Excellence (NICE) said that the risk of endocarditis following tooth extractions was minimal and that antibiotics (antibiotic prophylaxis) were not necessary. They have lately revised their recommendations to state that antibiotic prophylaxis may no longer be necessary regularly. The decision on whether precaution will be essential or recommended will most likely be made on a case-by-case basis after the dentist consults with the patient’s doctor or cardiologist.
Post-surgical Bleeding and Healing
Patients with hemorrhagic forms of EDS may experience excessive post-extraction bleeding, which should be avoided. But the dentist will generally inject a hemostatic substance into the socket, gently stitch the gum, and perhaps provide a mouthrinse to prevent the clot from dissolving further in the mouth (tranexamic acid).
There is minimal data to suggest that extraction sites heal poorly in EDS patients. Any signs of aberrant healing (such as persistent pain, swelling, or poor taste) should be reported to a specialist in oral and maxillofacial surgery, who will clean the region and provide local or systemic antibiotics if necessary.
The Efficacy of Local Anesthetics
On occasion, it has been reported that the efficacy of local anesthetics may be diminished in patients with EDS. Whenever this situation happens, patients will almost certainly be directed to an oral and maxillofacial surgery specialist who will guarantee that the most appropriate method or agent is utilized to ensure successful anesthesia.
Gum Disease (Gingivitis and Periodontitis)
Periodontal disease is less likely to occur if you maintain good dental hygiene. Moreover, individuals suffering from periodontal disease (regardless of their medical condition) should seek treatment from a periodontologist who will provide professional cleaning of the teeth and gums, and when necessary, surgical intervention to improve the gum status of the affected teeth.
Because certain patients with EDS are more susceptible than others to developing mouth ulcers due to stress caused by a loose denture, dentures must be properly fitted and checked frequently by a dentist.
The presence of pulp stones or the root’s unique form may make root canal therapy (endodontics) more difficult in the EDS setting. Endodontic problems may be best handled by a qualified professional in certain situations (an endodontist).
Although there are no thorough data on the use of dental implants in individuals with EDS, it is predicted that there would be minimal adverse side effects. Given that implant implantation is a surgical operation, the same attention should be provided to antibiotic prophylaxis and post-surgical bleeding as in dental surgery.
Orthodontic therapy for people with EDS may need to be adjusted in some cases since the teeth move more quickly than would be expected in some patients. Patients may need to wear an appliance for several months after their teeth have been appropriately positioned to guarantee that the teeth remain in the correct place. The stress of any orthodontic device may cause mouth ulcers in certain patients with EDS, and this is especially true for children with EDS. This can be mitigated by applying protective wax to the brace and, if necessary, applying an occlusive paste to any ulcerated areas.
Mouth ulcers – Some individuals with EDS are more prone to developing ulcers in their mouths due to damage from their teeth or their dentures. These can be minimized by ensuring that no rough or sharp teeth or dental restorations are present and that dentures are well fitted and secure. A protective occlusive paste can be applied to the region where the damage is most likely to occur if ulcers develop. In contrast, a professional should evaluate any mouth ulcer that does not heal after more than 2 weeks, and that does not appear to have a local origin.