oral and dental implications

8/12/2019 Oral and Dental Implications

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ORAL AND DENTAL IMPLICATIONS OF EHLERS-DANLOS SYNDROMEStephen R Porter BSc MD PhD FDS RCSEng FDS RCSEd FHEA, Institute Director and

Professor of Oral Medicine, UCL Eastman Dental Institute, London

IntroductionEhlers-Danlos Syndrome (EDS) gives rise to a spectrum of features affecting the mouththat may lessen quality of life. Additionally routine dental care has the potential to becompromised as a consequence of some of the systemic features of the disease. Thepresent chapter provides a review of the oral and dental aspects of EDS.

Orofacial manifestationsThe 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 recentlydescribed types of EDS. In general the greater the laxity of the skin and mucosa themore likely that patients will have orofacial features. Similarly the haemorrhagictypes are more likely than others to give rise to gingival (gum) bleeding.

The various potential orofacial features of EDS are detailed below:

EyesEpicanthic folds: these are folds that extend from the nasal bridge to the upper eyelidsand 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 orchange to increased distance between the eyes, hence giving the appearance of widespread eyes.

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

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

NoseThe 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 appearanceThe skin may be hyperelastic (very stretchy) and there may be 'cigarette-paper'scarring of the face and forehead (classical type). Individuals with vascular type diseasehave a distinct facial appearance of prominent eyes (see above), sharpened nose, thinlips and hollow cheeks, sometimes collectively termed 'acrogenic' facies (olderappearance). Type VIIc (?) disease may give rise to a small lower jaw (micrognathia).

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OralAbout 50% of individuals with EDS have the ability to touch the tip of the nose with theirtongue (Gorlin's sign) - this is especially likely with classical and hypermobile EDS. The oralmucosa may be thin, easily tear and give rise to mouth ulcers (classical and hypermobileEDS). Individuals with these types may also lack labial and/or lingual fraenula (the folds ofmucosa 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 andpossibly some subtypes of the arthrochalasia EDS.

A spectrum of dental anomalies have been described, particularly in classical andhypermobile including high cusps and deep fissures of premolars and molars, shortened orabnormally 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 ifthese 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 bonydestruction of the jaws) have been described in vascular EDS.

An increased liability to gum disease (gingivitis and periodontitis) have been describedin 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 careEDS has the potential to lessen oral health by virtue of increasing the risk of dentaldecay (caries) as a consequence of the dental anomalies as these can trap food anddental plaque. Caries initially gives rise to painless white and darkened areas of thecrowns, but without treatment will cause painful pulpitis ('toothache' with hot, cold andsweet foods) and later death of the tooth and painful abscess formation (periapicalperiodontitis). Additionally, patients with some types of EDS may have an increasedliability to gum disease (especially periodontitis). In ammation of the superficial gums(ginigivitis) causes swelling and bleeding, and may give rise to easy gingival bleeding, anunpleasant taste and oral malodour (halitosis). In ammation of the deeper tissues (theperiodontium) also causes bad taste and breath, but can also lead to mobility andmigration of teeth, and potentially early loss of teeth. It must also be recalled that somepatients with EDS may have gums that bleed more easily as part of their underlyingconnective tissue disorder. Prevention of tooth decay and gum disease is cardinal forall persons as this avoids the need for complex dental treatment and lessens the risk ofloss of time from education or employment that would occur in having to have dentaltreatment. Furthermore, invasive dental procedures such as dental extractions or

complex treatment of periodontal disease may be complicated by poor wound healingand possibly excess post-surgical bleeding. Thus there is a need for ALL individualswith EDS to have a diet that avoids the development of caries and maintain a highstandard of oral hygiene that will lessen the risk of caries and gum disease.

Maintaining good oral healthThe principles of sustaining good oral health are centred upon dietary restriction of sugarsand maintaining a good oral hygiene regime.

Dietary considerations

Sugars increase the risk of tooth decay as plaque bacteria thrive on these and generateacids that can attack the teeth and cause caries. The simple measures that lessen acidicdamage 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 willincrease the frequency of acidic attack upon teeth) To avoid sticky sweet foods (e.g. toffees etc) as these will not be easily dislodgedwith normal mouth action or saliva. Foods that contain sugar substitutes such assorbitol are not as harmful as those that contain sucrose, glucose or fructose, but thesugar 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 notableharm to the teeth, indeed salty snacks may actually protect the teeth bystimulating the ow of saliva.

Diet need not be boring. There is no need to entirely avoid sugars - as providedindividuals are sensible and maintain a high standard of oral hygiene (see below) their riskof caries will generally be low.

Good oral hygieneTooth brushingPlaque must be removed from the teeth, otherwise the bacteria will cause caries andgum disease. The teeth should be cleaned at least twice a day using a suitabletoothbrush and a uoride-containing toothpaste. The brush should have a small headthat will allow all accessible surfaces of the teeth to be reached. The bristles should benot be hard as this may cause loss of tooth tissue if there is any exposure of roots atthe gum margin. A variety of techniques can be used (e.g. a gentle up-and-down rollingor figure of eight action), but importantly the teeth should not be scrubbed in ahorizontal direction as this increases the risk of damage to the gums and any exposedroot surfaces. Brushing should include gentle massage of the gum margin, as this willhelp to remove any plaque trapped beneath this site. EDS is unlikely to have anysignificant implications for tooth brushing.

Interdental cleaningToothbrushes 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 cleanedseparately. A variety of interdental aids are available particularly floss, interdentalbrushes and interdental sticks. Floss needs to be used carefully to avoid traumatising thegums, but the oss should be icked below the gum margin to remove any plaque thatalways accumulates at this site. Brushes and sticks must be used carefully to avoiddamaging 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 isunlikely to have any significant implications upon interdental cleaning other that theavoidance of trauma.

FluoridesFluoride hardens the surface enamel of teeth and lessens the risk of caries. Childrenliving in a geographic region where the uoride content of water is naturally or artificiallyat a level of one part per million will have enamel that has increased strength andgreater 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 dailyuse of a uoride-containing toothpaste is thus recommended. Fluoride mouthwashes canalso 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 theteeth have already formed) although may be advantageous to children living in regionswhere the water is not fluoridated.

Antimicrobial mouthwashesAntimicrobial mouthwashes may reduce the risk of gingivitis and periodontitis and maylessen oral malodour. A wide range of mouthwashes are available; these should beused on a daily basis. Mouthwashes based upon chlorhexidine cause superficial staining of

the teeth, a lthough this may be lessened by using them immediately following toothcleaning and the stain can be removed by professional cleaning by a dentist, hygienist ortherapist. There is no strong evidence that alcohol-containing mouthwashes increasethe risk of mouth cancer.

Regular attendance at a dentistDentists have an important role in the identification and treatment of common dentaldisease. In addition they will be able to arrange referral to appropriate specialists if apatient has complex disease or possible oral manifestations of EDS that warrantsfurther investigation or treatment. It is advisable for all patients to attend a dentist ona six-monthly basis. Although there have been publicised concerns that not allpeople have ready access to an NHS dentist it is probable that this will improve as aconsequence of recent initiatives by the NHS. Members of the EDS support group whoencounter difficulties in obtaining dental care should contact the author for guidance asto how this can be resolved.

Considerations for different oral problemsDental extractionsThere are two concerns with regards to dental extractions of individuals with EDS;risk of endocarditis and excessive post- extraction bleeding.

Risk of endocarditisWhen teeth are extracted, bacteria from the gums pass into the bloodstream. Inpatients with cardiac valve abnormalities there is a risk that the bacteria will attachto the valve(s) and cause in ammation (endocarditis) that can affect cardiac function aswell as give rise to systemic disease. It was previously advised that all patients withvalvular defects required antibiotics before dental extractions to prevent possibleendocarditis; however the National Institute for Clinical Excellence (NICE) has nowconcluded that the risk of endocarditis following dental extractions in the vast majority ofpatients with known cardiac valve disease is low and that antibiotics (antibioticprophylaxis) are not indicated. Nevertheless not all cardiologists agree with this

recommendation. It would thus seem sensible for a dentist to contact a patient'scardiologist to determine if he/she wishes antibiotics to be prescribed for any planneddental extractions. If the dentist does not wish to prescribe antibiotics the specialist, ifwishing them to be provided, will instead prescribe these and be medicolegallyresponsible for any adverse consequences (which is very unlikely).

Post-extraction bleedingPatients with haemorrhagic types of EDS may be liable to excess post-extractionbleeding. However, in the vast majority of instances this will not arise as the dentist willplace 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 bereduced in EDS. If this arises (which is rare) patients should be referred to a specialist in


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patients with EDS may develop mouth ulcers due to the trauma of any orthodonticappliance. This can be lessened by use of protective wax over the brace and possibly anocclusive paste placed over any sites of ulceration.

Oral ulcerationPatients with EDS may develop mouth ulcers as a result of trauma fromteeth or dentures. These are best avoided by ensuring that dentures arewell fitting, although if ulcers arise a protective occlusive paste can be

provided. It must be emphasised that any patient, regardless of theirEDS type, who has persistent or recurrent mouth ulcer(s) should bereferred to an appropriate specialist (usually a specialist in OralMedicine).

Temporomandibular joint diseaseRecurrent dislocation of the temporomandibular joint may, very rarely, warrant surgicaltreatment. This always requires consultation with an Oral and Maxillofacial surgeon.

ConclusionEhlers-Danlos Syndrome can have a significant impact upon oral health and mouthfunction; however the majority of patients will probably only be liable to the commondisorders of the teeth and gums. Dentistry is unlikely to be greatly compromised by EDSand similarly patients are unlikely to have significant complications as a consequence ofroutine oral health care. Certainly, patients who have complex oral needs must bemanaged 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 theopinions or policy of the Ehlers-Danlos Support UK or its Trustees.

MEDICAL ADVISORY PANEL

Prof P Beighton OMB MD PhD FRCP FRCPCH, Prof H A Bird MD FRCP, Prof R Grahame CBE MD FRCP FACP,Dr D Merrild MD MPH, Dr N Burrows MD FRCP, Mr A P Barabas MD FRCS, Mr A I Attwood MB BS FRCS (Edin),

Prof F M Pope MD FRCP, Dr A Hakim MA FRCP, Dr John Sills MA MB BChir FRCP FRCPCH,Ms Jane Simmonds, MCSP BPE MA, PGDipManTher, PGCHE, BPE, Angela Hunter, MSc Dip Cst Reg MRCSLT

Registered Charity Number 1014641 Ehlers-Danlos Support UK

oral and dental implications

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