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ORAL AND DENTAL IMPLICATIONS OF EHLERS-DANLOS SYNDROME

Stephen R Porter BSc MD PhD FDS RCSEng FDS RCSEd FHEA, Institute Director and
Professor of Oral Medicine, UCL Eastman Dental Institute, London

Introduction
Ehlers-Danlos Syndrome (EDS) gives 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. The
present chapter provides a review of the oral and dental aspects of EDS.

Orofacial manifestations
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 patients 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:

Eyes
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 kyphoscoliosis EDS. Epicanthic folds may lessen with age or
change to increased distance between the eyes, hence giving the appearance of wide
spread eyes.

Other ocular features of EDS include puffy or prominent upper eyelids, blue sclera
(classical, kyphoscoliotic and arthrochalasic types), the ability to evert the upper
eyelid (Meitenier's sign, classical type ), myopia (short-sightedness, classical Type)
and strabismus (squint, classical Type ). Patients with vascular type EDS may have
large prominent "staring" eyes due to a lack of subcutaneous tissue. Kyphoscoliotic
type EDS may give rise to down-staring palpebral fissures.

Ears
There may be a lack of ear lobes and the pinna of the ear may be firm (vascular type).

Nose
The bridge of the nose can be widened or attened (classical and kyphoscoliotic types)
while in vascular type 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 type disease
have a distinct facial appearance of prominent eyes (see above), sharpened nose, thin
lips and hollow cheeks, sometimes collectively termed 'acrogenic' facies (older
appearance). Type VIIc (?) disease may give rise to a small lower jaw (micrognathia).

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Oral
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 fraenula (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, hypermobile EDS and
possibly some subtypes of the arthrochalasia EDS.

A spectrum of dental anomalies have been described, particularly in classical and
hypermobile 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 calicification 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 liability to gum disease (gingivitis and periodontitis) have been described
in Type VIII disease, 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 as these can trap food and
dental plaque. Caries initially gives 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). Inammation of the superficial gums
(ginigivitis) causes swelling and bleeding, and may give rise to easy gingival bleeding, an
unpleasant taste and oral malodour (halitosis). Inammation of the deeper tissues (the
periodontium) also causes bad taste and breath, 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.

Dietary considerations
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
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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 etc) 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 sugars 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 ow of saliva.

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.

Good oral hygiene
Tooth brushing
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 uoride-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.

Interdental cleaning
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 oss should be icked 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
ossing, 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.

Fluorides
Fluoride hardens the surface enamel of teeth and lessens the risk of caries. Children
living in a geographic region where the uoride 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
uorides are thus of benefit and are recommended for all individuals with EDS. Twice daily
use of a uoride-containing toothpaste is thus recommended. Fluoride mouthwashes can
also be helpful although are probably not required if a patient is already using a
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uoridated 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
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 the EDS support group 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
Dental extractions
There are two concerns with regards 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 inammation (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).

Post-extraction bleeding
Patients with haemorrhagic types of EDS may be liable 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
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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 (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 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
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
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
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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.

Oral 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, although 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 ulcer(s) should be
referred to an appropriate specialist (usually a specialist in Oral
Medicine).

Temporomandibular joint disease
Recurrent dislocation of the temporomandibular joint may, very rarely, warrant surgical
treatment. This always requires consultation with an Oral and Maxillofacial surgeon.

Conclusion
Ehlers-Danlos Syndrome 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.


The views expressed are those of the author(s) and should not be construed to represent the
opinions or policy of the Ehlers-Danlos Support UK or its Trustees.


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