Ehlers-Danlos syndrome (EDS) and can adversely impact upon the function of the mouth and in turn potentially lessen quality of life. While many people with EDS not have any notable oral problems specifically due to EDS, this connective tissue disorder can affect the teeth and gums as well as the temporomandibular joint. In addition, the systemic complications of EDS could sometimes compromise the delivery of routine dental care. This article provides an overview of the impact of EDS upon oral health and dental care.
Not all people with hypermobility syndromes will have disorders of the mouth directly due to their systemic disorder, indeed it is probable that most oral problems of patients with symptomatic hypermobility will be similar to those of healthy persons (e.g. dental decay (caries) and gum disease (gingivitis and periodontitis)) that reflect the effects of dental plaque. The precise frequencies of oral and facial disorders due to EDS are not well described, as there are few studies of large numbers of patients with well characterized EDS. The oral and facial features of EDS vary with each type of disease and in general the greater the laxity of the skin and mucosa the more likely that patients will have orofacial features.
The haemorrhagic types of EDS are probably the most likely to give rise to gingival (gum) bleeding.
Pain and laxity of the lining of the mouth
Pain and dislocation of the jaw (temporo-mandibular; TMJ)) joint may arise with classical, hypermobile and vascular types of EDS. In view of the laxity of the joint the lower jaw (mandible) is more mobile and may dislocate from its fossa in the temporal bone (the base of skull) causing the jaw to deviate away from the side of the dislocation and the patient unable to close the mouth.
Sometimes the mandible will spontaneously relocate while some patients develop a method by which they are able to easily relocate the lower jaw into its fossa. Unfortunately sometimes this is not possible hence relocation must be undertaken by a clinician (e.g. in an Accident and Emergency clinic). Repeated dislocation can damage the bones of the joint causing pain and paradoxically limited movement. Similarly even if the joint is not repeatedly dislocating the increased movement of the joint in normal activities can cause pain of the joint(s) and surrounding areas of the face and head.
However it is important to be aware that pain in and around the TMJ may not always be a direct reflection of EDS as it might be expected that some people with EDS will have symptoms of a much more common problem termed temporomandibular joint disorder (TMD) in which there is pain of the joint and surrounding muscles with possible limitation of mouth opening. This disorder is common, is not a reflection of any structural defects of the joints or muscles but seems to reflect psychological distress of some kind. Temporomandibular disorder may be most common in young adults and possibly more frequent in women than men. The symptoms often may respond to analgesics and they often abate when the distress of the affected individual lessens.
There is no well-defined protocol of the management of joint laxity of EDS. Suggested methods to lessen the risk of subluxation or dislocation are principally based upon not opening the mouth wide while therapies for those with pain have included splints, local ultrasound, low intensity laser, exercises, acupuncture, transcutaneous electrical nerve stimulation (TENS), cognitive behavioural therapy (CBT), and use of drugs such as antidepressants.
Interventions for patients with radiological and/or arthroscopic evidence of joint damage may include arthrocentesis and balloon distraction of the joints, injection of opioids (i.e.morphine-based agents) into the joint and rarely open joint surgery. (e.g to recontour the joint surfaces and/or alter the joint structure to lessen the risk of joint displacement/dislocation). However there remain no consistent data on the efficacy of the non-surgical or surgical methods of truly lessening the risk of pain and laxity of the TMJ in EDS.
In general the gums (gingivae) and periodontal tissues (the part that links the teeth to the bones) are not specifically affected by EDS.
However type VIII EDS may cause an increased risk of gingivitis and periodontitis with resultant non-painful red bleeding gums, oral malodour (halitosis) and mobility and early loss of teeth. Periodontal disease has also been suggested to arise in classical and vascular EDS.
Jaw bone anomalies
Aside from the aforementioned possible damage to the jaw joint there is no striking evidence that EDS causes defects of the jaw bones. Multiple odontogenic keratocysts (that have the potential to cause local bony destruction of the jaws) have been described in vascular EDS, but this would seem to be very rare.
A spectrum of dental anomalies have been described in EDS, particularly in the classical and hypermobile types and include high cusps and deep fissures of premolars and molars, shortened or abnormally shaped roots with stones in the pulp of crowns, and enamel hypoplasia (underdevelopment) with microscopic evidence of various enamel and/or dentine defects. The enamel defects may predispose to easy loss of the tissue of crowns (attrition) and if these give rise to a loss of calcification of the enamel will increase the risk of caries.
Implications of poor Oral Health upon general Well Being
Oral disease, regardless of any associations with EDS can lessen quality of life. Dental decay (caries) causes pain, limits the ability to eat certain foods and may ultimately cause notably painful abscesses. Gingival disease (gingivitis) may cause halitosis and lessen confidence and socializing while periodontal disease may cause the teeth to drift, alter the smile and may interfere in the ability to chew. In EDS there is the possible added impact of the physical and psychological impact of the laxity of the jaw joint. There is some evidence that EDS, for a number of reasons, can lessen nutritional intake and increase the risk of eating disorders, hence minimizing the risk of common oral disease is important as this may further add to the burden of problems of EDS.
Prevention of tooth decay and gum disease is important for all persons as this avoids pain and the other symptoms discussed above. In addition the need for complex dental treatment can be costly both in money and time (e.g. children miss school, adults and cares have to take time away from work or other activities). Invasive dental procedures such as dental extractions or complex treatment of periodontal disease may be complicated by poor wound healing and possibly excess post-surgical bleeding. Thus there is a need for all persons with with EDS to have a diet that avoids the development of caries and maintain a high standard of oral hygiene that will lessen the risk of caries and gum disease.
Advice on maintaining good dentition
Dental decay is due to plaque generating acids from sugars that cause destruction of the teeth. Hence the 3 main principles of lessening caries are 1. Clean the teeth to remove plaque 2. Lessen the intake of sugars from which dental plaque generates acids and 3. Protect the surfaces of teeth form the effects of acids by using fluoride mouthwashes and/or toothpastes.
Avoid sugary agents
Dietary sugars can be reduced by avoiding sweet sticky foods at all times, not snacking on sweets between meals and restricting sweet foods to meal times. Sweets or foods that contain non-sucrose sweeteners cause less caries than sugar, but can cause gastrointestinal upset in some people. Diet need not be boring. There is no need to entirely avoid sugars – as provided individuals are sensible and maintain a high standard of oral hygiene (see below) their risk of caries will generally be low. Snacks that contain crisps, nuts (if they are not too hard and not going to cause TMJ pain) and many other savoury agents contain low levels of sugar and also may stimulate saliva that will neutralise the action of acids.
Clean teeth well
The teeth should be cleaned at least twice a day using a suitable toothbrush and a ﬂuoride-containing toothpaste.
The brush should have a small head that and relatively soft bristles.
A variety of tooth brushing techniques can be used (e.g. a gentle up-and-down rolling or figure of eight action), but importantly the teeth should not be scrubbed in a horizontal direction as this increases the risk of damage to the gums and any exposed root surfaces.
Brushing should include gentle massage of the gum margin, as this will help to remove any plaque trapped beneath this site.
There are no specified guidelines for tooth cleaning in relation to EDS but it would seem sensible for each patient to find a method that suits themselves – i.e. avoids the need to open the mouth overly wide.
Toothbrushes only remove the plaque and debris from the upper and exposed (smooth) surfaces of teeth, hence the areas between teeth (interdental sites) require to be cleaned separately. A variety of interdental aids are available particularly floss, interdental brushes and interdental sticks. Floss needs to be used carefully to avoid traumatising the gums. Floss holders can aid ﬂossing, particularly if individuals have difficulties in reaching the posterior teeth. EDS is unlikely to have any significant implications upon interdental cleaning other that the avoidance of trauma and opening the mouth notably wide.
Fluoride in toothpastes and mouthwashes will lessen the resistance of decay of only the surface layer of enamel. Twice daily use of a ﬂuoride-containing toothpaste is recommended. Fluoride mouthwashes can also be helpful although are probably not needed if a patient is already using a ﬂuoridated toothpaste.
Antimicrobial mouthwashes may reduce the risk of gingivitis and periodontitis and may lessen oral malodour. A wide range of mouthwashes are available; these should be used on a daily basis. There is no strong evidence that alcohol-containing mouthwashes increase the risk of mouth cancer.
Attend a dentist regularly
Dentists manage common dental disease. In addition they will be able to arrange referral to appropriate specialists if a patient has complex disease or possible oral manifestations of EDS that warrants further investigation or treatment. Advice about the availability of dentists can be obtained from NHS Direct. Dentists with a limited knowledge of EDS and its implications upon oral health and dental care should refer the patient to an appropriate specialist for advice.
Consideration for different dental problems
When teeth are extracted, bacteria from the gums pass into the bloodstream. In patients with cardiac valve abnormalities there is a risk that the bacteria will attach to the valve(s) and cause inﬂammation (endocarditis) advised that all patients with valvular defects required antibiotics before dental extractions to The National Institute for Clinical Excellence (NICE) previously advised the risk of endocarditis following dental extractions was low and that antibiotics (antibiotic prophylaxis) are not indicated. They have recently changed the guidance such that they advise that antibiotic prophylaxis may not be routinely required. Thus It is likely that the decision as to whether prophylaxis will be required/prescribed is likely to be based on a case by case basis following discuss by the dentist with a patient’s doctor or cardiologist.
Post-surgical bleeding and healing
Patients with haemorrhagic types of EDS may be liable to excess post-extraction bleeding. However, the dentist will usually place a haemostatic agent into the socket, carefully suture the gum and possibly provide a mouthrinse that prevents the clot from breaking down (tranexamic acid).
There is little evidence that extraction sites do not heal well in EDS– if healing seems to be abnormal (e.g. sustained pain, swelling, bad taste) the patient should be referred to a specialist in Oral and Maxillofacial surgery who will clean the area and possibly provide local or systemic antibiotics.
Local anaesthetic effectiveness
There have been occasional reports that the efficacy of local anaesthetics may be reduced in EDS. If this arises patients are likely to be referred to a specialist in Oral and Maxillofacial surgery who will be able to ensure that a suitable technique or agent is used to ensure effective anaesthesia.
Gum Disease (gingivitis and periodontitis)
Good oral hygiene reduces the risk of periodontal disease. Additionally, individuals with periodontal disease (regardless of their medical status) should be treated by a specialist in periodontology who will be able to provide professional cleaning of the teeth and gums and when indicated surgery to improve the gum status.
As some patients with EDS are more liable than others to develop mouth ulcers due to trauma from a loose denture it is essential that dentures are well fitting and regularly reviewed by a dentist.
Root canal treatment– in EDS root canal treatment (endodontics) may be complicated by the presence of pulp stones and/or the root having an unusual shape. In such instances endodontic therapy may be best undertaken by an appropriate specialist (an endodontist).
There are no detailed reports of the use of dental implants in patients with EDS but few adverse side effects would be anticipated. As the placement of an implant is a surgical procedure the same considerations of antibiotic prophylaxis and post-surgical bleeding as for dental should be given.
Orthodontic treatment for patients with EDS may have to be modified as in some patients the teeth migrate faster than would be expected. After the teeth have been positioned correctly there may be a need for patients to wear an appliance for many months to ensure that the teeth remain in position. Some patients with EDS may develop mouth ulcers due to the trauma of any orthodontic appliance. This can be lessened by use of protective wax over the brace and possibly an occlusive paste placed over any sites of ulceration.
Mouth ulcers– some patients with EDS are prone ulcers as a result of trauma from teeth or dentures. These can be lessened by ensuring there are no rough/sharp teeth or dental restorations and that dentures are well fitting. If ulcers arise a protective occlusive paste can be placed over the area of likely trauma. However any mouth ulcer that does not heal for more than 2 weeks and has no likely local cause should be investigated by a specialist.
Ehlers-Danlos syndrome can adversely impact upon the health of the mouth and the resultant disease lessen quality of life. Nevertheless the majority of persons with EDS will probably only be liable to the common disorders of the teeth and gums – these being unrelated to their connective tissue disease. The provision of oral health care can occasionally be compromised by EDS but the risks of significant complications are low – provided there is good communication between the patient and the attending dentist and that the dentist seeks advice from appropriate specialists when needed.
Professor Stephen R Porter BSc MD PhD FDS RCSEng FDS RCSEd FHEA.
Institute Director and Professor of Oral Medicine, UCL Eastman Dental Institute, 256 Grays Inn Road, London WC1X 8LD
(Last review August 2020)
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