Dental care for children with Down syndrome: a guide for the dental practice team

Medical considerations

Down syndrome is the most easily recognized chromosomal condition (Figure 2). Children with Down syndrome can present with complex medical histories. Medical conditions diagnosed are not in themselves different to medical conditions in other patients; however, certain medical conditions may be over-represented in children with Down syndrome.^4,8 Such conditions are outlined in Table 1. Ensuring time is available to gather a thorough medical history is an important first step in the dental consultation for a patient with Down syndrome. A personal child health record (PCHR) is given to parents/carers in many countries at a child's birth for health professionals to record information every time the child is seen in a medical setting. In the UK, the PCHR (‘red book’) has an additional insert for children with Down syndrome. The PCHR can be useful for the dental team to discuss the child's medical and developmental history, and to draw attention to the section on dental health.

Dental history and examination

Parents and carers are usually keen to provide information that can make the dental visit easier both for the patient and the dental team. Families may offer information from a hospital passport that they use in partnership with medical teams to highlight extremely important information or specific requirements their child may have. Providing an opportunity for the parent or carer to share information about the child's specific likes, dislikes or anything that the parent or child is particularly worried about regarding the dental visit will help the dental team understand any patient behavioural or sensory issues. Information gathered regarding experience of local anaesthesia, sedation and general anaesthesia, as well as behaviour management techniques that have enabled dental examination or treatment will influence the expectations of the dental appointment. This information will help the dental team decide whether a thorough intra-oral examination, including any appropriate radiographs, is likely to be successful, or whether a staged approach is more appropriate. Common oral and facial features are summarized in Table 2.

Preventive dental care

Patients diagnosed with Down syndrome should be considered at increased risk of gingivitis and periodontal disease.¹¹ Increased risk may be related to individual characteristics, skills and host immune factors, such as reduced activity of neutrophils and T lymphocytes, as well as increased production of inflammatory mediators and proteolytic enzymes.¹⁰ Any intellectual disability (ID) or decreased manual dexterity is important to establish. Dental professionals should be aware that their Down syndrome patients with ID may not understand the importance of tooth brushing or forget to brush, and limited dexterity may make it difficult to brush teeth physically.¹² It is important to be sure that the patient's family or caregiver is supported with appropriate information⁵ including information about toothbrushes, aids and non-foaming toothpastes that can be useful for providing oral hygiene for patients who struggle to understand what is being done, and may be uncooperative during tooth brushing. Some parents and carers may find brushing with adapted toothbrushes (eg Dr Barman, Dentaco, Norway; Collis Curve, Collis Curve, USA; Figures 3 and 4) easier when providing oral care. Independence with tooth-brushing skills should be supported bearing in mind that the age at which a child with Down syndrome should be expected to take care of his/her own teeth may be later than the usual recommendation of 7–8 years of age. Caries risk status should be established and regularly reviewed, so that the correct concentration of fluoride toothpaste for the patient's age and risk is advised, or prescribed as appropriate.¹³

Dental development and anomalies

The dental team should be alert to the likelihood of abnormal dental development and quality of enamel in children with Down syndrome (Figures 5 and 6). Delayed dental development, hypodontia, microdontia and ectopic eruption of teeth with associated problems of impaction or resorption occur frequently in children presenting with Down syndrome. Clinical vigilance and appropriate timing of radiographic examination for planning and liaising with specialist paediatric dentistry, orthodontic and surgical colleagues may be required for any appropriate interceptive treatment. Co-operation for clinical and radiographic examination may be challenging, particularly if the child has not been a regular dental attender and had the opportunity to become accustomed to the dental environment and team. Prior to radiographic examination, the risk–benefit ratio of radiographic exposure and anticipation of diagnostic gain should be considered. Increasing the duration of appointments can be helpful to allow demonstration, which may enable radiographic examination, potentially yielding important information for treatment planning. (Figure 5).

Caries

Systematic reviews have found either fewer dental caries in children with Down syndrome^14,15 or no difference in caries experience between children with and without Down syndrome.¹⁶ Reduced caries prevelance may be assocated with delayed eruption, bruxism (decreased fissure depth), microdont teeth, diastemas, spaced dentitions, salivary composition and buffering capacity.

Tooth surface loss (TSL)

A study by Bell et al found 67% of children with Down syndrome showed pathological TSL compared to 34% of controls (Figure 7). More children with Down syndrome showed severe wear and more showed multifactorial aetiology than the control group.¹⁷ TSL can result from a combination of erosion from intrinsic acid due gastro-oesophageal reflux disease (GERD), extrinsic acid from soft drinks and attrition due to bruxism. Bruxism can be problematic particularly when occurring in patients who also have teeth with defective enamel that wears more rapidly than normal enamel. Increased prevalence of the dental enamel defects of hypomineralization and hypoplasia have been described in Down syndrome.¹⁸ Dental professionals should be aware of possible increased caries risk in teeth with dental enamel defects.¹⁹

Operative dental procedures

Success for restorative, surgical and orthodontic procedures is optimized when dental professionals take time and effort to build a good relationship based on continuity and consistency, patience and reassurance, and good communication skills.¹²

Treatment planning

The primary care dental team has an important role in highlighting the importance of regular dental check-ups, providing advice on the best ways to prevent oral disease and initiating a positive attitude toward dentistry. Appropriate support for the family of a child with Down syndrome includes carefully explaining the child's oral health needs, discussing options for proposed treatment and obtaining valid consent for dental care. The consent process should involve comprehensible explanation of the child's oral health needs and any proposed treatment options, and requires the clinician to find out specific patient values and attitudes (or in the case of a child who is unable to consent for themselves, the values and attitudes of the person with appropriate parental responsibility), when developing an agreed care plan.²⁰

Use of acclimatization techniques and behaviour management approaches will vary depending on patient age, family preferences and degree of any intellectual disability. Techniques to reduce anxiety eg using introductory visits in advance of treatment, longer appointments, easy-read resources, social stories and videos as well as provision of choice and control i.e. ‘reasonable adjustment’¹² will enhance the likelihood of co-operation for clinical, radiographic and periodontal assessment.

Local referral protocols to community or hospital dental services should support the primary care dental team when procedural difficulty or patient modifying factors, such as complex medical or behavioural problems, require the competencies of a clinician with enhanced experience, or the skillset of a specialist or consultant in paediatric dentistry.²¹ Specialist referral may be indicated for children with Down syndrome when conscious inhalation sedation with nitrous oxide and oxygen (IS) is deemed an appropriate pharmacological behaviour management technique, but the equipment or training required to deliver IS is not available within the dental practice team. A shared-care approach, where a child with Down syndrome continues preventive and routine care with the dental practice team ensures that families continue to be supported and motivated to accept oral health advice and change unhealthy behaviours, if they exist, while awaiting treatment on an advanced or specialist care pathway.

Age appropriate assessment of periodontal health using the simplified BPE (basic periodontal examination) along with clinical and radiographic surveillance of the developing dentition will alert dental professionals to the need to liaise with specialist colleagues regarding retention of primary teeth or planning extractions for guidance of eruption in cases of hypodontia (Figure 5).

If invasive dental treatment is proposed for a child with Down syndrome who has been diagnosed with congenital heart disease (CHD), dental teams working in the UK should consult the Scottish Dental Clinical Effectiveness Programme (SDCEP) document,²³ which supports the implementation of recommendations in the NICE guideline on prophylaxis against infective endocarditis.²⁴ The SDCEP document outlines patient groups with CHD or previous cardiac surgery who require special consideration and discussion regarding prescription of antibiotic prophylaxis. These include patients with any type of cyanotic congenital heart disease and patients who have had repair of congenital heart disease with a prosthetic material. The details of diagnoses of CHD are usually set out in clinic letters that are forwarded to families. Clinic letters, if shared with the dental team, can be used to discuss the medical history and to commence assessment of the attitudes of families regarding antibiotic prophylaxis and the need to communicate further with paediatric and cardiology teams for advice. If multiple extractions are planned, and antibiotic prophylaxis is indicated, general anaesthesia (GA) may be the most appropriate treatment modality to consider. When an advanced care pathway procedure, such as GA, is required communication between the responsible dental team and medical teams is important to ensure safe standards of care are in place to recognize increased risks that may be associated with the presence of cardiac anomalies, cervical vertebral abnormalities (atlanto-axial instability), increased susceptibility to gastro-oesophageal reflux and infection, as well as higher incidence of obstructive airway problems.²⁵

Conclusion

Children with Down syndrome can present with multiple medical diagnoses and learning disabilities. In the presence of active oral disease, assessment and triage may indicate need for referral to specialist care pathways. However, a culture of early and regular dental visits is likely to increase successful attendance in primary care. A guide to assist the dental and wider healthcare teams with timing of dental checks and surveillance is presented in Table 3. Working with families and carers to tailor preparation and delivery of dental care can prove successful in reducing procedural stress and facilitate good oral health practices.