Oral and dental implications of the Ehlers-Danlos syndromes
Stephen Porter, Director and Professor of Oral Medicine, UCL Eastman Dental Institute, London
Please note: The following text cannot and should not replace advice from the patient's healthcare professional(s). Any person who experiences symptoms or feels that something may be wrong should seek individual, professional help for evaluation and/or treatment. This information is for guidance only and is not intended to provide individual medical advice.
The Ehlers-Danlos Syndromes (EDS) give rise to a spectrum of features affecting the mouth that may lessen quality of life. Additionally, routine dental care has the potential to be compromised as a consequence of some of the systemic features of the disease. This article provides a review of the oral and dental aspects of EDS.
The oral and facial features of EDS vary with each type of disease. There have been few detailed studies of the orofacial manifestations of the rare and/or recently described types of EDS. In general the greater the laxity of the skin and mucosa the more likely that someone will have orofacial features. Similarly, the haemorrhagic types are more likely than others to give rise to gingival (gum) bleeding.
The various potential orofacial features of EDS are detailed below.
Epicanthic folds: these are folds that extend from the nasal bridge to the upper eyelids and can give the appearance of a widened nasal bridge. These seem to be most common in classical and kyphoscoliotic EDS. Epicanthic folds may lessen with age or change to increased distance between the eyes, giving the appearance of wide spread eyes.
Other ocular features of EDS include puffy or prominent upper eyelids, blue sclera (classical, kyphoscoliotic and arthrochalasia types), the ability to evert the upper eyelid (Meitenier’s sign, classical type), myopia (short-sightedness, classical type) and strabismus (squint, classical type). People with vascular EDS may have large prominent ‘staring’ eyes due to a lack of subcutaneous tissue. Kyphoscoliotic EDS may give rise to down-staring palpebral fissures.
There may be a lack of ear lobes and the pinna of the ear may be firm (vascular type).
The bridge of the nose can be widened or ﬂattened (classical and kyphoscoliotic types) while in vascular EDS the nose can appear pinched or sharp.
Facial skin and appearance
The skin may be hyperelastic (very stretchy) and there may be ‘cigarette paper’ scarring of the face and forehead (classical type). Individuals with vascular EDS have a distinct facial appearance of prominent eyes (see above), sharpened nose, thin lips and hollow cheeks, sometimes collectively termed ‘acrogenic’ facies (older appearance). The lower jaw has been suggested to be of reduced size in some patients. Brown spots (termed Café-au-lait macules) may have been observed on the skin of the neck in some types of EDS.
About 50% of individuals with EDS have the ability to touch the tip of the nose with their tongue (Gorlin’s sign) – this is especially likely with classical and hypermobile EDS. The oral mucosa may be thin, easily tear and give rise to mouth ulcers (classical and hypermobile EDS). Individuals with these types may also lack labial and/or lingual frenulae (the folds of mucosa that are in the midline of the lips and beneath the tongue). Dislocation of the jaw joint (temporomandibular joint) is a possible feature of classical and hypermobile EDS and possibly some subtypes of arthrochalasia EDS.
A spectrum of dental anomalies have been described, particularly in the classical and hypermobile types, including 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. Multiple odontogenic keratocysts (that have the potential to cause local bony destruction of the jaws) have been described in vascular EDS.
An increased tendency towards gum disease (gingivitis and periodontitis) has been described in periodontal EDS, this having the potential to cause early tooth loss in adults. Periodontal disease has also been suggested to arise in classical and vascular EDS.
Implications for oral health care
EDS has the potential to lessen oral health by virtue of increasing the risk of dental decay (caries) as a consequence of the dental anomalies – these can trap food and dental plaque. Caries initially give rise to painless white and darkened areas of the crowns, but without treatment will cause painful pulpitis (‘toothache’ with hot, cold and sweet foods) and later death of the tooth and painful abscess formation (periapical periodontitis). Additionally, patients with some types of EDS may have an increased liability to gum disease (especially periodontitis). Inﬂammation of the superficial gums (gingivitis) causes swelling and bleeding, and may give rise to easy gingival bleeding, an unpleasant taste and oral malodour (halitosis). Inﬂammation of the deeper tissues (the periodontium) also causes bad breath and altered taste, but can also lead to mobility and migration of teeth, and potentially early loss of teeth. It must also be recalled that some patients with EDS may have gums that bleed more easily as part of their underlying connective tissue disorder.
Prevention of tooth decay and gum disease is cardinal for all persons as this avoids the need for complex dental treatment and lessens the risk of loss of time from education or employment that would occur in having to have dental treatment. Furthermore, 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 individuals 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.
Maintaining good oral health
The principles of sustaining good oral health are centred upon dietary restriction of sugars and maintaining a good oral hygiene regime.
Sugars increase the risk of tooth decay as plaque bacteria thrive on these and generate acids that can attack the teeth and cause caries. The simple measures that lessen acidic damage to the teeth are:
- To avoid excess sugary foods
- Eat sweet foods only at meal times (sugary drinks or snacks between meals will increase the frequency of acidic attack upon teeth)
- To avoid sticky sweet foods (e.g. toffees) as these will not be easily dislodged with normal mouth action or saliva. Foods that contain sugar substitutes such as sorbitol are not as harmful as those that contain sucrose, glucose or fructose, but the sugar substitutes can cause gastrointestinal upset in some individuals – so take care!
Hard fruits and vegetables do not greatly remove plaque from the teeth, but they do contain less sugar than sweets and snacks and thus are an alternative to the latter. Similarly, savoury snacks that do not contain sugars, e.g. peanuts, cause no notable harm to the teeth, indeed salty snacks may actually protect the teeth by stimulating the ﬂow of saliva.
Diet need not be boring. There is no need to entirely avoid sugars – provided individuals are sensible and maintain a high standard of oral hygiene (see below) their risk of caries will generally be low.
Good oral hygiene
Plaque must be removed from the teeth, otherwise the bacteria will cause caries and gum disease. 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 will allow all accessible surfaces of the teeth to be reached. The bristles should be not be hard as this may cause loss of tooth tissue if there is any exposure of roots at the gum margin. A variety of 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. EDS is unlikely to have any significant implications for tooth brushing.
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, but the ﬂoss should be ﬂicked below the gum margin to remove any plaque that always accumulates at this site. Brushes and sticks must be used carefully to avoid damaging the gums – they should never be forced between the teeth, indeed sticks are best used when there are obvious spaces between the teeth. 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.
Fluoride hardens the surface enamel of teeth and lessens the risk of caries. Children living in a geographic region where the ﬂuoride content of water is naturally or artificially at a level of one part per million will have enamel that has increased strength and greater resistance to dental decay. Fluoride in toothpastes and mouthwashes will lessen the resistance of decay of only the surface layer of enamel. Without doubt ﬂuorides are thus of benefit and are recommended for all individuals with EDS. Twice daily use of a ﬂuoride-containing toothpaste is thus recommended. Fluoride mouthwashes can also be helpful although are probably not required if a patient is already using a ﬂuoridated toothpaste. Fluoride tablets are of no significant benefit to adults (as the teeth have already formed) although may be advantageous to children living in regions where the water is not fluoridated.
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. Mouthwashes based upon chlorhexidine cause superficial staining of the teeth, although this may be lessened by using them immediately following tooth cleaning and the stain can be removed by professional cleaning by a dentist, hygienist or therapist. There is no strong evidence that alcohol-containing mouthwashes increase the risk of mouth cancer.
Regular attendance at a dentist
Dentists have an important role in the identification and treatment of 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. It is advisable for all patients to attend a dentist on a six-monthly basis. Although there have been publicised concerns that not all people have ready access to an NHS dentist it is probable that this will improve as a consequence of recent initiatives by the NHS. Members of EDS UK who encounter difficulties in obtaining dental care should contact the author for guidance as to how this can be resolved.
Considerations for different oral problems
There are two concerns with regard to dental extractions of individuals with EDS – risk of endocarditis and excessive post-extraction bleeding.
Risk of endocarditis
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) that can affect cardiac function as well as give rise to systemic disease. It was previously advised that all patients with valvular defects required antibiotics before dental extractions to prevent possible endocarditis; however the National Institute for Clinical Excellence (NICE) has now concluded that the risk of endocarditis following dental extractions in the vast majority of patients with known cardiac valve disease is low and that antibiotics (antibiotic prophylaxis) are not indicated. Nevertheless not all cardiologists agree with this recommendation. It would thus seem sensible for a dentist to contact a patient’s cardiologist to determine if he/she wishes antibiotics to be prescribed for any planned dental extractions. If the dentist does not wish to prescribe antibiotics the specialist, if wishing them to be provided, will instead prescribe these and be medicolegally responsible for any adverse consequences (which is very unlikely).
Patients with haemorrhagic types of EDS may be prone to excess post-extraction bleeding. However, in the vast majority of instances this will not arise as the dentist will 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 have been occasional reports that the efficacy of local anaesthetics may be reduced in EDS. If this arises (which is rare) patients should be referred to a specialist in Oral and Maxillofacial surgery who will be able to ensure that a suitable technique or agent is used that provides effective anaesthesia.
There is little evidence that the healing of extraction sockets is greatly compromised in patients with 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 antibiotics.
Gum disease (gingivitis and periodontitis)
As discussed above some types of EDS increase the risk of periodontitis. In addition people with EDS, like any other individual, are at risk of some periodontal disease that can ultimately lead to bad breath, gum bleeding, tooth mobility or tooth loss. Good oral hygiene will reduce the risk of periodontal disease. Additionally, individuals with periodontal disease 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. It was previously recommended that deep cleaning of the gums (scaling) required antibiotic prophylaxis, but as with dental extractions (see above) this may no longer be the case.
Dental restorations (fillings, crowns and bridges (sometimes termed fixed prosthodontics))
There are no specific concerns for fixed prosthodontics for patients with any type of EDS. Large fillings or crowns that are below the gum margin are unlikely to cause any significant bleeding, and if this does arise it will probably stop with local pressure.
Dentures (removable prosthodontics)
There are no specific concerns for removable prosthodontics for patients with any type of EDS. However, 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.
Endodontics (root canal treatment)
Root canal treatment is required when a tooth dies (usually as a consequence of dental decay) or when an abscess forms at the base of the root. Endondontic therapy requires the root canal to be cleaned and filled (usually) with gutta percha. In EDS, 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). Antibiotic prophylaxis is generally not warranted for endodontic treatment.
Dental implants are titanium screws that are placed within bone. Crowns, bridges and dentures can then be attached to the implants. 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 extractions apply.
Orthodontics is not contra-indicated for patients with EDS; however, treatment 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.
Patients with EDS may develop mouth ulcers as a result of trauma from teeth or dentures. These are best avoided by ensuring that dentures are well-fitting. If ulcers arise a protective occlusive paste can be provided. It must be emphasised that any patient, regardless of their EDS type, who has persistent or recurrent mouth ulcers should be referred to an appropriate specialist (usually a specialist in Oral Medicine).
Temporomandibular joint disease
Recurrent dislocation of the temporomandibular joint (TMJ) may, very rarely, warrant surgical treatment. This always requires consultation with an Oral and Maxillofacial surgeon.
The Ehlers-Danlos syndromes can have a significant impact upon oral health and mouth function; however the majority of patients will probably only be liable to the common disorders of the teeth and gums. Dentistry is unlikely to be greatly compromised by EDS and similarly patients are unlikely to have significant complications as a consequence of routine oral health care. Certainly, patients who have complex oral needs must be managed by appropriate clinicians such as specialists in Special Care Dentistry, Oral Medicine and Oral and Maxillofacial Surgery.
Peer reviewed by: Dr Hanadi Kazkaz, Consultant Rheumatologist, University College London Hospital
Date of last review: 01/04/2016