Pediatric Dentistry - a Clinical Approach, 3ed.

CHAPTER 24. Dental Care for the Child and Adolescent with Disabilities

Gunilla Klingberg, June Nunn, Johanna Norderyd, and Pernille Endrup Jacobsen

Context

Disability is a term that evokes different images for readers, depending on their cultural background, their personal experiences of disabling conditions, and the norms of the society in which they live. In recent years there has been a move away from the fairly narrow definition imposed by the medical model, with its emphasis on an individual’s impairment “handicapping” that person. For example, a child with Down syndrome is disabled by being intellectually impaired and unable to keep up educationally with his or her peers. Advocates for people with disabilities, including parent support organizations, have promoted a more inclusive definition that places an emphasis on an individual’s ability, rather than what they cannot do. This is in part captured in the social model, in which it is acknowledged that it is society that disables an individual with an impairment, rather than the other way round. For example, a flight of stairs into a dental surgery disables a person who has quadriplegic cerebral palsy and so needs to use a wheelchair to move around.

The World Health Organization’s International Classification of Functioning Disability and Health (ICF) [1] and the children and youth version (ICF‐CY) [2] seek to stress the health status and functioning of the individual, rather than their potential disability. These classifications are based on a biopsychosocial model that aims to integrate the medical and social models referred to above. Functioning and disability are defined in relation to the outcomes between the health condition(s) of the person and the context in which he or she operates. This context is defined at three levels of functioning: at the level of a body part, at the level of the whole individual, and lastly, functioning that occurs at the level of society (Figure 24.1).

Flow diagram of the ICF theoretical, biopsychosocial model. It features the functioning and disabilities in relation to outcomes between the health conditions of a person and the context in which they operate.

Figure 24.1 The ICF theoretical, biopsychosocial model defines functioning and disabilities in relation to outcomes between the health conditions of a person and the context in which they operate. This context is defined at three levels of functioning, at the level of a body part, at the level of the whole individual, and the functioning that occurs at the level of society.

Clearly, this framework opens up the possibility of refining the definitions of, for example, which children it is considered need special care dentistry and which do not, judged by the functioning needs of the child rather than a mere diagnosis. Examples of this are given in Table 24.1 [3]. In both these examples, the need for specialist facilities such as expert knowledge about different conditions, conscious sedation, or wheelchair‐accessible dental surgeries may mean that special dental care is required.

Table 24.1 ICF structure and domains applied on example patients: a child with Autism spectrum disorder and a child with quadriplegic cerebral palsy and epilepsy.

Source: Faulks & Hennequin 2006 [3]. Reproduced with permission of Stephen Hancocks Limited.

Domain (examples)

Example 1: child with Autism spectrum disorder

Example 2: child with quadriplegic cerebral palsy and epilepsy

Body function (physiological and psychological functions of body systems)

Global mental functions (e.g., global psychosocial functions) Specific mental functions (e.g., emotional functions)

Control of voluntary movement functions Involuntary movement functions

Body structures (anatomical parts of the body)

Structure of the brain

Structure of upper extremity

Structure of lower extremity

Impairments (significant deviation or loss of body function or structure)

Impaired ability to sustain attention Impaired control of voluntary movements

Impaired involuntary movement reaction functions (e.g., posture)

 

Delayed acquisition of the mental functions of language

Impaired control of voluntary movements and functions

 

Impaired psychosocial functions

Impaired involuntary movement functions (e.g., involuntary muscle contraction)

Activity (the execution of a task or action)

Undertaking single or multiple tasks Handling stress and other psychological demands

Undertaking single or multiple tasks

Participation (involvement in a life situation)

Maintaining one’s health

Maintaining one’s health

Activity limitations (difficulty in executing activities)

Difficulty in completing a given task, e.g., simple dental instruction

Difficulty in washing oneself Difficulty in caring for teeth

Participation restrictions (problems in involvement in life situations)

Problems with seeking care and accepting dental treatment

Problems with cooperating with dental treatment due to movement disorder

Environmental factors (physical, social, and attitudinal environment)

Support given by health professionals

Attitudes of health professionals, health services

Support given by health professionals

Attitudes of health professionals, health services

Environmental facilitators

Well‐informed and understanding dental team

Well‐informed and understanding dental team

 

Appropriate adaptation of dental services (e.g., assimilation of patient’s routine)

Appropriate adaptation of dental services (e.g., wheelchair access, nonflickering lighting)

 

Additional services (e.g., use of conscious sedation)

Additional services (e.g., use of conscious sedation)

The ICF and the ICF‐CY complement the World Health Organization’s (1993) ICD‐10 codes [4], which is an international statistical classification of diseases and health‐related problems. The latter system will identify people with an intellectual disability, for example, and divide them into four categories of intellectual functioning based on intelligence quotient (IQ), with people with an IQ <70 classified as having intellectual disability and those with an IQ <50 having severe intellectual disability.

Prevalence

For the reasons outlined in the preceding section, the definitions of impairments may vary so that determining accurate prevalence figures for some disabling conditions can be difficult. In a review of disability in children conducted in 2004, the prevalence of all disabilities in children varied between 5.8% in the USA and 9.8% in Finland [5]. One finding from most prevalence studies is that there are more boys than girls affected, with respiratory disorders (e.g., asthma) being the most frequently occurring impairment. In the USA, children living in poverty or whose parents had little formal education were at greater risk of disability [6]. Adolescents with disabilities were three times more likely to visit a doctor each year and nine times more likely to have hospital visits annually compared with their nondisabled peers.

As in some other countries, Denmark has a birth cohort of children that are being followed longitudinally. Of the 9125 children born prematurely, some 673 did not survive beyond the first 28 days of life [7]. Many of these babies will have had impairments that were not compatible with life. Of the survivors, 11.2% were diagnosed with a disability. In Sweden, a survey of 10‐year‐old children concluded that 26% had health problems that significantly impacted on the child’s day‐to‐day functioning [8]. Physical disorders were the most frequent, occurring in 11.7% of the sample of children. In the UK there are approximately 0.8 million children living with a disability [9]. Some families care for two or more children with severe disability. Such families are more likely to be single parents, less likely to be in paid employment but, if they are, for them to be in semi‐skilled or unskilled jobs. They are thus families who are reliant on state aid and less likely to own their own homes. A hidden group of children are those who, because of chronic physical or mental illness, may be in health care settings for prolonged periods and thus not accessible to, or looked after by, dental services. In one study in England over a year, children aged between birth and 19 years occupied two million “bed days” because of their complex needs [10].

Survival from life‐threatening illnesses is increasing among children and adolescents; those with, for example, cystic fibrosis have improved their median survival from 14 years in 1969 to 32 years in 2000. The median life expectancy of a baby with cystic fibrosis now is approximately 40 years.

Oral health and disability

Impairments tend to occur more commonly in boys, in those from low‐income families, and in school‐age children. Their health needs, including oral and dental needs, are frequently unmet. Oral and dental diseases may be more prevalent in children and adolescents with impairments because of the potential for increased risk. Some countries have undertaken national surveys of children with disabilities, others have taken samples of children with different types of impairments and compared the data collected with those from a nondisabled population. No data exist on the outcome from implementation of policy arising out of the survey findings.

Most of these national or local studies report similar findings; while the numbers of children with impairments who are caries‐free may be higher than for non‐impaired children, the management of dental disease is different. Children with impairments are more likely to have untreated dental caries and, where it is managed, this has in the past been by extraction rather than restoration. However, as dental care is developing with improvements in both oral health and management/treatment techniques there is a chance that these differences will diminish. Preventive dental services vary in different countries. In some places prevention is provided and well organized while in others it is only accessible for a minority of patients.

Despite overt needs, there is a lack of awareness among medical professionals of the oral and dental needs of their child patients with impairments, and dentists express an unwillingness to treat because of uncertainty, as well as a lack of competence to manage such groups in the population. Families often experience severe financial hardship in obtaining necessary dental care for their children, although in Scandinavian countries comprehensive dental care is free from birth to 18 or 19 years.

Children with impairments are more likely to use publicly funded services and increasingly to access those services that can provide care under sedation or general anesthesia. In those countries where people with impairments are still in residential care in large units, there are reports of poorer oral hygiene and worse dental health.

There are risk factors that are more prevalent in children with impairments, compared with the rest of the child population. Many more children with impairments will be taking sweetened liquid oral medicines over the long term with consequences for poor dental health including dental caries and possibly erosion. The prevalence of another form of tooth wear, bruxism, is more frequently seen in children with disabilities. Periodontal health is often poorer in children who are impaired because of underlying host factors, as in Down syndrome, as well as poor manual dexterity and no or inefficient plaque removal.

However, it is aspects of oral health and function, as depicted in the earlier discussion of the World Health Organization’s ICF classification, that often concern parents and carers, namely drooling, grinding, and feeding problems, not necessarily dental caries and periodontal diseases. This should be acknowledged. However, it is important that dentists also pay attention to the dental and gingival health in these patients and encourage the parents and family to be committed to the prevention of these diseases. If not, there is a risk that, for example, dental caries is overlooked.

Consent

As for any child, parents or legal guardians are the only people entitled to give consent for dental care. In some countries this will be until the age of 16 years, in others, 18 years. In a number of countries, adolescents, if they are judged to have the maturity to give their consent, or indeed to withhold their agreement for a dental procedure, will be able to make that decision without the need for parents to give their consent. However, many young people who have a communication disorder and especially those with a learning disability may never develop the capacity to give consent, verbal or written, for dental procedures. It will be for the members of the dental team to decide if the person has the capacity to consent and, if not, to work with the legislation in their country to manage issues of consent for the adolescent near or above the usual age of consent. Capacity will vary from day to day and will depend on the nature of the procedure. Regardless of this, the patient should always receive individually tailored information about treatment and be given the opportunity to give or refuse their consent.

Neuropsychiatric disorders

Neuropsychiatric disorders constitute a substantial group of diagnoses, for example, autism spectrum disorder and attention deficit hyperactivity disorder (ADHD). It has been estimated that at least 5% of the child population may have a neuropsychiatric disorder. The diagnoses are based on a specific set of symptoms describing the main domains of problems experienced by the individual person. A person’s diagnosis may change over time, as problems and symptoms change with individual development [11].

Autism spectrum disorder

An estimated prevalence for autism spectrum disorder (ASD) has been suggested as 6 per 1000 people [12]. In the 2013 fifth edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM‐5) [13] autism spectrum disorder replaced a number of diagnoses, for example, autism and Asperger syndrome. ASD is a behavioral expression of neurobiological dysfunction. The prevalence varies, but is approximately one to three per 1000, and some increase in prevalence has been noted since the 1990s. This is probably due to more knowledge about the diagnoses leading to higher ascertainment rates. More boys than girls are affected [11]. According to DSM‐5, four criteria need to be met in order to be diagnosed with ASD:

·     persistent deficits in social communication and social interaction across context, not accounted for by general developmental delays

·     restricted, repetitive patterns of behavior, interests, or activities

·     symptoms must be present in early childhood (but may not become fully manifest until social demands exceed limited capacities)

·     symptoms together limit and impair everyday functioning.

Additional problems that may affect some individuals include hyperactivity, sleeping problems, and abnormal and strong reactions to perception (hearing, sight, physical contact). Up to 50% of people diagnosed with ASD never achieve spoken language. They also have problems with social interaction and communication including difficulties in using and understanding eye contact, facial expressions, and gestures, as well as in understanding, especially language’s deeper meaning. Children and adolescents with ASD are often engaged in a restricted range of behaviors, interests, and activities; repetitive and stereotypic behaviors are common. Comorbidity, that is having one or more diagnoses in addition to the main diagnosis, in this case autism, is frequently seen. In particular, there is an increased risk of epilepsy. ASD occur more frequently in individuals with, for example, some syndromes and in persons with intellectual disability. It is important to recognize that ASD has a very wide range in phenotype, i.e., there are large variations between individuals.

The former Asperger syndrome represents a high functioning autism spectrum disorder with a prevalence of three to five per 1000 people, and affects boys more often than girls; the disorder can range from mild to severe. People with this type of ASD usually have a normal or above normal IQ and many exhibit exceptional skills or talents in specific areas. At the same time, they have significant deficiencies regarding social skills and can be preoccupied with their special interests. Although having a rich vocabulary, the patient may sound like a “little professor” and the language development may appear normal on the surface, but there are often deficits in pragmatics and prosody.

ASD is a lifelong disability but many children can develop considerably provided early and individually tailored educational input is provided. It is important to help the child with tools for communication and in the structured training for daily living; different pedagogic concepts have developed. It is important to provide good information and education to parents and family and to establish a trusting relationship between the family and the educational system. Depending on the individual’s deficits, the need for assistance varies considerably between people with autism spectrum disorders. Some may be in need of assistance full time, while others only require help in organizing their studies or for some special activities.

Oral health considerations

As many patients with ASD are dependent on others for their daily life, risk factors may be different compared with nonaffected children. Provided there is no other underlying medical condition, the literature reports no differences in caries prevalence. This is probably due to family or caregivers being able to provide a good diet for the child, with a low intake of cariogenic items. On the other hand, there are reports of a higher prevalence of plaque and gingivitis compared with healthy children. Again the reason may relate to family/caregivers’ influence as many children and adolescents are dependent on help from others to carry out oral hygiene procedures and brushing the teeth of, for example, a teenager with ASD is often difficult.

Special considerations for dental care

People with ASD need help to understand and focus on dental treatment. Often several appointments are required just for an introduction to dentistry. It is important for the patient to meet the same dentist and preferably the same assistant or dental hygienist in order to get to know the personnel and be able to trust them. If the dental team knows beforehand that a patient has ASD it is advisable to contact the family by phone in advance. It is useful to get as much information about the patient as possible from this telephone interview: focusing on her or his strengths, what the child likes, what rewards are appropriate, does the child speak and, if not, the best way to communicate, does the child have fears of particular things like noise or strong light, etc. Often, people with ASD are overly sensitive to sounds, tastes, smells, and sights. Sometimes it is good for the parents or carers to visit the clinic beforehand without the child in order to get to know the facilities, where to park, what door to enter, who to meet, and so on.

The introduction to examination or treatment can be carried out over a number of appointments scheduled just one or a few days apart, or as several short, clearly constructed appointments sequentially on the same day. It is usually possible to carry out a good examination with mirror and probe and to perform preventive items such as toothbrushing, polishing, and applying topical fluorides, after this kind of special introduction to dental care. However, exposing radiographs or carrying out treatment such as fillings often requires sedation or general anesthesia. To reduce the need for general anesthesia, it is very important to emphasize prevention of oral/dental disease. The preventive care may be carried out by a dental hygienist or a trained dental nurse, but regular appointments with short intervals in between, in order to maintain contact and ensure a successful experience for the child, are vital.

Many children and adolescents with ASD use pictures or photographs as an aid in communication. Today, modern techniques with digital cameras and high‐quality printers have made it easy for the dentist to create this kind of individually customized help. Photos of the dental clinic and the dentist and staff the patient will meet should be included. Also, photos of an open mouth symbolizing “open your mouth,” a toothbrush, equipment for polishing, mirror, operatory lamp, and dental chair are useful (Figure 24.2). The pictures or photographs can be put in the sequence the patient will see them at the appointment in a photo album. This can be used both at home when preparing for the visit, and during the appointment as a help to remember and understand what is going to happen. This kind of aid is useful also when treating other patients, such as patients with ADHD, intellectual disability, children with another language, or even young or anxious children [14,15].

Clockwise: Photos of the entrance, a toothbrush, the operatory light, a mirror, the dental chair, and an open mouth (symbolizing “open your mouth”).

Figure 24.2 Examples of photographs that can be used as pedagogic tools in patients with neuropsychiatric disorders. Based on individual needs: pictures showing the entrance, the dental chair, the operatory light, a toothbrush, a mirror and an open mouth (symbolizing “open your mouth”) were chosen in this case. The photos can be put in sequence in a photo album.

Communication with a patient with autism should be very clear and objective as he or she understands you literally and will have a great deal of difficulty reading nonverbal cues (body language). Tell the patient what you want and avoid “small talk.” This will help the patient to focus on the dental treatment. See further under section “Attention deficit hyperactivity disorder.”.

Attention deficit hyperactivity disorder

Attention deficit hyperactivity disorder (ADHD) is a common disorder affecting 3–7% of children and adolescents and thus something that all dental health care personnel working with young patients are likely to meet. Knowledge about the condition is therefore important. ADHD is caused by neurobiological dysfunction. There is evidence of a genetic background affecting the frontal lobe and dopamine and norepinephrine neurotransmitters, although the etiology is not fully understood. The main problems in ADHD are displayed as inattention, hyperactivity, and impulsivity. The diagnosis can be either of a combined type (most common) where the individual exhibits symptoms in all domains, or inattentive, or hyperactive–impulsive type, depending on the main grouping of impairments. The prevalence varies considerably between different studies, mainly due to differences in definitions applied, but also due to differences in ascertaining study populations. Boys are diagnosed more often than girls, who are supposed to be underdiagnosed. The reason for this is likely to be that girls present with fewer observable impairments such as hyperactivity. Many of the problems in ADHD are persistent as the individual grows older, and many of the patients have difficulties as adults. These include higher frequencies of alcohol or drug abuse and psychiatric disorders.

Therapy for ADHD includes the use of psychoeducative strategies with educational programs for parents and teachers. Medication, mainly with methylphenidate (inhibiting dopamine and noradrenaline transport) or atomoxetine (increasing noradrenaline levels in the brain), has been shown to be effective in many children.

Oral health considerations

It is likely that children with attention disorders of various types have difficulties adjusting their activity level to situational demands and have more problems complying with dental care. There are reports of more behavioral management problems during dental care in patients with ADHD, and probably a risk of more dental anxiety. Many patients with ADHD behave and function at a lower age level in the dental setting. With respect to oral health, dental caries studies have reported somewhat contradictory results but, on balance, there would appear to be an increased risk. Children with ADHD have also been reported to have poorer oral health behavior in terms of higher frequency of sugary food and beverage intakes and a lower frequency of toothbrushing [16]. Furthermore, side effects of the medication for ADHD include risk of xerostomia, altered or decreased feelings of hunger, and nausea/vomiting, which increase the risk of oral health problems.

Special considerations for dental care

Prevention of oral health problems should be the main target together with promoting a positive attitude and acceptance of dental care. Many patients with ADHD have problems in concentrating and focusing on the dental treatment. In order to help the patients, disturbing visual and auditory noise should be reduced. For example, there should be no radio on, the door to the treatment room should be closed to reduce disturbance, and visual distractions such as toys or books should be kept to a minimum in the room. Communication should focus on clear information to the child about what will happen during treatment, who will be involved, how long the procedure will last, and what to do afterwards. Direct and objective guidance during the treatment can help the patient to focus; for example, directions like “Sit in the chair” rather than “Would you like to sit in the chair?” [16]. As for many other children with neuropsychiatric disorders, pictures or photos can be used as pedagogic tools to provide a “travel plan” of the appointment. Pictures can also be used as tools and aids for toothbrushing at home. Praising is essential as in all dental care for children, and should come directly when the child is cooperating instead of summing up at the end of the appointment.

Tourette syndrome

Tourette syndrome is defined by multiple involuntary motor and vocal tics, present for more than a year. The prevalence is approximately 1% and boys are affected three to four times more often than girls. The motor tics may be complicated, involving the entire body, such as kicking and stamping. The verbal tics include making sounds like grunting, throat clearing, shouting and barking, and may also be expressed as coprolalia (involuntary use of obscene words or sentences). Associated conditions can include ADHD, anxiety, and obsessive compulsive disorder. Therapy includes, apart from psychoeducative strategies, cognitive behavior therapy and in the case of obsessive compulsive disorder, medication often with selective serotonin reuptake inhibitors.

Oral health considerations

Children and adolescents with Tourette syndrome have more difficulties complying with dental care. This probably leads to an increased risk of both dental behavior management problems and also in the long term, dental anxiety. The oral health considerations are similar to those of patients with ADHD.

Special considerations for dental care

See under section “Attention deficit hyperactivity disorder.”

Other disorders

Intellectual disability

Intellectual disability is, according to DSM‐5, “characterized by significant limitations both in intellectual functioning and in adaptive behavior, which covers many everyday social and practical skills. The disability originates before age 18” [13]. The term mental retardation was earlier used in North America, while the term learning disability has been used in the UK and most of Europe. Previously, the definition of intellectual disability was solely based on psychometric measures where an IQ <70 served as classification for intellectual disability, and an IQ <50 denoted severe intellectual disability. The IQ test is still a major tool for measuring intellectual functioning and is described in the DSM‐5. The new definition, however, includes both mental functioning and functioning skills in the individual’s environment; this is why a person with a below‐average IQ, i.e. <70, may not be considered to have an intellectual disability unless exhibiting deficits in two or more adaptive behaviors. Intellectual disability affects approximately 2.5–3% of the population, and 0.6% have a severe disability. Intellectual disability can be caused by genetic or congenital factors or acquired at an early age. There is a wide range in how the individual is affected. Some individuals with a mild learning disability may need very little support and go to regular schools, while those with a profound learning disability will need full‐time support in all types of activities. Comorbidity is common and includes, for example, physical, behavioral, and sensory impairments, syndromes, congenital heart defects, and epilepsy. Examples of conditions where learning disability are frequent are Down syndrome, fragile X syndrome, and 22q11 deletion syndrome.

Oral health considerations

Children with intellectual disability generally have the same prevalence of dental caries and periodontal problems as other children. However, there are reports of more oral/dental disease in older people, maybe due to difficulties in carrying out treatment, which result in untreated disease that progresses. As with patients with other conditions, many patients with intellectual disability are dependent on help from others to carry out toothbrushing; poor manual dexterity or lack of motivation often leads to poor oral hygiene if the person has to carry out this task for themselves. However, the use of powered brushes for those who have the motivation but who may lack dexterity can impact positively on gingival health [17].

Special considerations for dental care

Children and adolescents with intellectual disability accept dental treatment, but it has to be tailored to the individual person’s capacities and needs. The level of co‐operation depends on the individual’s degree of understanding of the treatment as well as on age. Local analgesia is, for example, abstract and involves aspects that can be fear‐provoking in a patient with intellectual disability as in any other patient. In some cases a strong negative reaction can be expected after the injection, mainly due to the feeling of numbness. The patient may hurt herself or himself by biting because of the lack of sensation in the anesthetized part. It is still important that pain‐free treatment is provided in these patients and the use of periodontal ligament injections or local analgesia without vasoconstrictors can be helpful. As when treating patients with neuropsychiatric disorders, introduction and appropriate communication are important. Introduction using tell–show–do (see Chapter 6) is often effective and can be accompanied by the use of photos or pictures as pedagogic tools. Competence regarding communication varies considerably and the dentist should be prepared to use body and even sign language. Children and adolescents with intellectual disability need time to feel comfortable in the dental setting, and effort has to be invested in these patients. These patients also benefit from meeting the same dental team during well‐structured dental visits.

22q11 deletion syndrome

The 22q11 deletion syndrome is one of the most common multiple anomaly syndromes with an incidence of approximately one per 4000 newborns. The syndrome has previously been described as DiGeorge syndrome or velocardiofacial syndrome. It has an autosomal dominant inheritance, but for 80–90% of the patients the deletion occurs de novo. The clinical appearance varies considerably and many patients may show a complex medical picture. Characteristic features that may be present include congenital heart defects, velopharyngeal insufficiency with or without cleft palate, frequent infections due to thymic hypoplasia or aplasia, feeding difficulties, hypocalcemia/hypoparathyroidism, intellectual disability and behavioral abnormalities.

Oral health considerations

There are reports of a high prevalence of dental caries in patients with 22q11 deletion syndrome. This may be explained by a low saliva secretion rate, altered properties of saliva, and high levels of cariogenic bacteria. In addition, problems related to frequent infections (foremost in the preschool age group) and feeding difficulties increase the risk of more frequent meals and possibly also the intake of food and beverages containing sucrose. Dental aberrations, primarily enamel hypoplasia and hypomineralization, hypodontia and late eruption of teeth, may occur [18].

Special considerations for dental care

Dental care for patients with 22q11 deletion syndrome should focus on prevention. Many patients have difficulties related to neuropsychiatric problems and mild learning disability and the treatment should be modified according to individual needs. However, most patients accept dental treatment if this is preceded by introduction and sufficient time is allocated for the appointment. Several patients have problems keeping appointments, and there are often frequent cancellations because of illness and infections in young children or the numerous medical appointments. In cases of congenital heart defects, endocarditis prophylaxis should be considered according to national guidelines.

Fragile X syndrome

Fragile X syndrome is caused by an unstable DNA fragment on the X chromosome. Approximately one in 4000 boys and girls is affected, but only 20–30% of the girls present with symptoms as they have a second X chromosome that usually functions well. Common features include intellectual disability, autism spectrum disorders, attention deficit disorders, speech disturbances, cardiac defects, low muscle tone, and connective tissue problems. Many children and adolescents with fragile X are very shy and stressed in novel situations.

Oral health considerations

Apart from problems related to autism and learning disability it is common to find feeding difficulties. There are also reports of higher frequencies of malocclusions.

Special considerations for dental care

The dental care should focus on prevention. The treatment should be modified with regard to the individual patient’s problems related to any autism or intellectual disability.

Down syndrome

Down syndrome is the commonest chromosomal syndrome and cause of diagnosed intellectual disability. There are three different types of Down syndrome: trisomy 21, mosaicism, and translocation. Trisomy 21 occurs when there is an extra chromosome 21 and this form accounts for the majority of cases. In mosaicism (2–5%), the person has a mix of some cells containing 46 and some 47 chromosomes. The person affected will have some of the usual characteristics of the syndrome, depending on the number of cells involved and which they are. In the translocation type of Down syndrome, part of chromosome 21 becomes detached and is attached to another chromosome. Thus, genetically they have 47 chromosomes. The prevalence of this condition is in the range of one in 800–1000 live births, and even higher in mothers older than 40 years. Approximately 25% of all babies born with Down syndrome are born to mothers over 35 years of age.

Specific comorbid features associated with Down syndrome are intellectual disability, neuropsychiatric disorders and behavioral problems (20–40%), congenital cardiac anomalies, thyroid problems, seizures, hearing and visual impairments, early onset dementia (Alzheimer‐like), frequent infections, hepatitis B carriage, especially in countries where people with disabilities may still live in residential care with suboptimal standards of hygiene, and leukemias.

The general features of Down syndrome are short stature, tendency to obesity, muscle hypotonia, atlantoaxial instability, mid‐face hypoplasia, slanting palpebral fissures, prominent epicanthic folds, and simian palmar creases [19].

Specific intraoral features seen in Down syndrome are a mouth‐open posture, sometimes accompanied by drooling, a protrusive tongue often with deep fissuring (Figure 24.3), which can be a challenge for oral hygiene, a relative prognathism, hypodontia, microdontia, aggressive periodontal disease, delayed eruption in both dentitions, and delayed exfoliation of primary teeth.

Photo of the fissured tongue of a boy with Down syndrome.

Figure 24.3 Large, fissured tongue of a boy with Down syndrome.

Oral health considerations

Unlike unaffected children of a similar age, many young people with Down syndrome may not ever be able to consent to more invasive dental procedures although they may have capacity to consent for less invasive preventive care, such as fissure sealants and topical fluoride therapy. Increasingly, experts are becoming more aware of a premature disintegrative disorder that manifests in adolescence and may be the precursor to dementia, especially of the Alzheimer type. Young people who are affected suddenly start to lose skills, become introverted and appear depressed.

Significant sensory problems, as a barrier to oral care, do not become obvious until adulthood although hearing may be impaired early on. Visual disturbances are seen initially as a consequence of cataract formation, but later, other visual disturbances impair the person’s ability to read instructions and to visualize toothbrushing.

Although in the past, caries prevalence has been reported as low in a child with Down syndrome, this may be a false picture since, by comparison with nonaffected peers, there may be fewer teeth erupted for dental caries to affect. Enamel hypoplasia and spacing may make children more or less prone to dental caries, respectively. However, use of sweetened medicines for seizure control or infection management may predispose to dental caries as will a dry mouth induced by some medications, especially those drugs used to manage mood swings. The literature supports the theory that secretion rates, as well as quality of saliva, may be reduced in young people with Down syndrome. Because of a compromised immune system, in particular, impaired phagocytosis together with altered cell‐mediated but especially humoral immune responses, children with Down syndrome may develop a severe form of periodontal disease, similar to localized aggressive periodontitis in that it mainly affects the lower incisor area [20]. Regular reviews and professional tooth cleaning of young people with Down syndrome have been shown to be effective in slowing the progression of destructive periodontal disease.

Special considerations for dental care

Despite the assumed picture of amiable, lovable children, a proportion of children with Down syndrome will have a significant degree of intellectual impairment together with behavioral problems. These may mean that dental treatment needs to be provided under sedation, or possibly general anesthesia. For any dental care, consideration needs to be given to the use of antibiotic prophylaxis, where there is a significant cardiac condition requiring this, according to national guidelines.

Where dental care needs to be delivered with the aid of sedation or general anesthesia, care in handling is required where atlantoaxial instability is known or suspected. Sedation is preferable to general anesthesia, where difficulties in intubation because of a retrusive maxilla and propensity to develop chest infections make this a more hazardous procedure.

In many countries, orofacial regulation therapy is employed to train and stimulate the tongue and lips. This is carried out in collaboration with a speech‐language pathologist (see section “Oral motor function”).

Cerebral palsy

Cerebral palsy is the commonest cause of significant childhood disability. It is characterized as a nonprogressive disorder of the brain stem that results in varying impairment of motor activities. The defect occurs as a consequence of some form of insult (trauma, infection) prenatally, perinatally, or postnatally. Cerebral palsy presents as four main types: spastic (muscle stiffness), athetoid (slow, writhing movements), ataxic (rarer type affecting balance, coordination), and mixed. Frequently there is comorbidity in the form of intellectual impairment and/or epilepsy [21]. A consensus conference in 2004 questioned the traditional classification of cerebral palsy and has proposed a new classification which excludes those children who have no motor defect and places more emphasis on the accompanying nonmotor, neurodevelopmental impairments of performance and behavior, consistent with the ICF classification outlined above. The prevalence of cerebral palsy is of the range 1.5–2.5 per 1000 live births and in Sweden, as in other countries, has been declining [22].

Oral health considerations

Children with impaired mobility, or epilepsy with frequent seizures, might be expected to have an increased prevalence of trauma and the literature would support this theory. The altered muscle control produces two other oral effects in these children. One is an increase in malocclusions and the second is the higher prevalence of drooling (Figure 24.4). Most studies involve too few children to indicate the type of malocclusion, but in those that have been reported, an increased overjet and a tendency to an Angle Class II malocclusion is seen.

Photo of a human dentition with a vacuum‐moulded splint in place to prevent further self-mutilation in a child with cerebral palsy.

Figure 24.4 Drooling in a child with cerebral palsy.

Drooling occurs for a number of reasons. Children with cerebral palsy often have poor head control and, together with an inadequate lip seal, gravity results in pooling of saliva at the front of the mouth and drooling or sialorrhea. The dysfunctional second stage of swallowing also adds to the problem. Most of the resting saliva in the mouth comes from the submandibular glands. The management of drooling includes behavioral, pharmacological, and oral motor therapy. In special cases where this is not sufficient, surgical treatment to reroute submandibular ducts to the oropharynx is carried out. This irreversible treatment may increase the risk for dental caries.

Tooth wear is commoner in children with cerebral palsy. This may result from factors such as gastroesophageal reflux disease (GERD/GORD) that is seen in these children and which often comes under control after a gastrostomy procedure (e.g., via a percutaneous endoscopic gastrostomy, PEG) to aid feeding. Another dental feature of children who are PEG‐fed is that, once all oral feeding ceases, there is a tendency to accumulate greater quantities of calculus (Figure 24.5). Dentists may be the first people to notice the signs of reflux, from evidence of wear to, usually, palatal surfaces of maxillary teeth and occlusal/buccal surfaces of lower posterior teeth, although the child may have had symptoms from the GERD/GORD for many years. Such symptoms may lead to self‐inflicted injury, because of the inability to communicate the pain and distress of reflux. The other main cause of tooth wear seen in children and young people with cerebral palsy is bruxism. In addition, patients with habits of biting non‐nutritive objects (pica) such as toys, wooden sticks etc., may also present with tooth wear.

Photo of a drooling in a child with cerebral palsy.

Figure 24.5 A vacuum‐moulded splint in place to prevent further self‐mutilation in a child with cerebral palsy (note calculus as a consequence of nil‐by‐mouth PEG feeding).

Enamel hypoplasia as well as hypomineralization occur more frequently in children with cerebral palsy and are caused by a systemic upset where amelogenesis is disrupted. Dental development may be delayed in children with cerebral palsy but the prevalence of dental caries is usually higher than for their normal peers. This may be related to the increased prevalence of enamel defects, poor oral hygiene, and often hyperplastic gingival tissue. In addition, some studies have shown that these children have higher counts of mutans streptococcus and lactobacillus as well as diminished salivary flow, buffering, and lower pH levels as compared with non‐impaired groups. In those children with cerebral palsy and drooling that has been managed by salivary gland removal, or salivary duct rerouting, a rampant form of dental caries may develop, particularly in the lower incisor teeth.

Special considerations for dental care

As with all children with impairments for whom the risk of developing dental disease and/or its treatment may pose significant hazards for their general health and well‐being, prevention of oral and dental disease is a priority (see section “Preventive dental care”). Some children with severe mobility impairments may have difficulty accessing dental care, which in turn results in neglect and more advanced disease on presentation.

The unpredictable movement disorders may mean that parents/carers have difficulty initiating and maintaining good oral hygiene and need practical demonstrations and support from the dental team. Appropriate seating to maintain comfort and security for disabled children can be achieved by maintaining children in their own wheelchair for those who need this form of aid, on a special ramp, or using the support of cushions in a normal dental chair.

Such movement disorders may necessitate the use of conscious sedation to control these involuntary movements during dental treatment. This is especially true in a child who has a normal intellect and for whom the desire to cooperate is confounded by movement that is worse the greater degree of control they attempt to exert. In these instances, the use of nitrous oxide sedation is beneficial. A prop (Figure 24.6a,b) may be helpful to make dental treatment more comfortable for the child and safer for the dentist or hygienist.

Photo of two props with and without an occlusal “guard.”; Photo of a prop being used hold open a mouth along with a toothbrush.

Figure 24.6 (a) A prop, with and without an occlusal “guard”, and (b) in use.

Good assistance to aspirate away secretions and dental debris is necessary during dental treatment since many children with cerebral palsy have dysphagia. This is especially important in children who are PEG‐fed and accumulate large deposits of calculus that need to be removed frequently. Dysphagia in combination with poor oral hygiene and calculus also involve an increased risk of pneumonia, so good preventive dental care is essential.

For children with self‐inflicted injury, referral to a specialist is necessary. Management usually consists of identifying the cause and treating it; for some the trigger may be teething, in others it may be undiagnosed pain. In the short term the fitting of a vacuum‐formed splint may break the habit and allow the lesion to heal (Figure 24.5). For other children, a longer‐term solution is to correct their malocclusion. Only as a last resort are teeth extracted.

Spina bifida

Spina bifida is one of the commonest birth defects. The incidence and severity of the condition varies. In Sweden, as in many parts of Europe, the incidence is one per 2000 live births while in Celtic areas (Wales and Ireland) it may be as high as three to four per 1000 live births.

The cause of spina bifida is unknown but may be due to both genetic and environmental causes; supplementation of the diets of women of childbearing age with folic acid preconception can reduce the risk by up to 70%. Affected families are at greater risk of having a second affected child.

The impairment in spina bifida is an incomplete spinal cord and a defect, predominantly in the lumbar or sacral region, in the overlying vertebrae. It occurs as a result of failure of the vertebrae to fuse, usually within the first month of embryonic development. There are three main manifestations: spina bifida occulta, which is a defect of the spinal cord but with no external signs, apart from occasional dimpling and/or a collection of hairs over the affected part. There are usually no symptoms. In meningocele, the vertebrae are defective and part of the meninges protrudes through the opening but without any spinal cord involvement. Symptoms are usually not evident early in life.

The most severe form of spina bifida is a myelomeningocele or spina bifida cystica. In this form, the spinal cord and covering meninges protrude through the defective vertebrae. Because the nerve supply to this region is affected, there is accompanying paralysis at and below this level. Consequently there will be paralysis of the legs, a paraplegia, and loss of bladder and bowel control. In some children with spina bifida there is an accompanying malformation of the cerebellum, which is pushed out through the base of the skull. This produces a hydrocephalus as fluid builds up in the ventricles, because the exit channels are blocked by the displaced cerebellum. Occasionally, hydrocephalus spontaneously arrests. For most, fluid needs to be drained from the ventricles with a shunt, usually into the peritoneum (ventriculo‐peritoneal shunt). In older patients, the shunt may be directed to the right atrium (ventriculoatrial shunt).

Oral considerations

There is very little reported in the literature with respect to oral health in spina bifida. The oral health will be affected by consideration of the degree of physical impairment and use of liquid oral antibiotics for persistent urinary tract infections.

Special considerations for dental care

A significant, potential problem for the delivery of dental care is the increased likelihood of an allergy to latex seen in children with spina bifida [23]. This occurs because of the frequent catheterizations as a result of the neuropathic bladder and with the placement of shunts to manage any hydrocephalus. In addition, patients who have a ventriculoatrial shunt will usually require antibiotic prophylaxis prior to invasive dental care. However, most children will now be fitted with a ventriculo‐peritoneal shunt that will not require antibiotic cover. The majority of patients with spina bifida will use a wheelchair and so will require to be treated in a unit that has a modification to accept a wheelchair or to employ some means of manually handling the patient from wheelchair to dental chair with a slide.

Because of the potential to develop latex sensitivity or true allergy, it is advisable to restrict the latex load as much as possible. Such patients should therefore be managed in a unit that is latex safe, and first thing on a Monday morning when the latex load in the atmosphere is at a minimum. Prevention is a key to the management of these children so that they do not require dental treatment.

Muscular dystrophies

Muscular dystrophies are a group of genetic disorders caused by mutation in the gene for the dystrophin protein, that results in progressive muscle weakness, principally affecting voluntary muscles but smooth muscles are also affected, for example in the gut. Muscle fibers are gradually replaced by fat and fibrous tissue. There are more than 30 conditions under the term muscular dystrophies, but nine major categories. The three most prevalent groups are Duchenne muscular dystrophy, which is an X‐linked condition clinically manifest predominantly in boys (the most common muscular dystrophy with an incidence of one per 3500 newborn boys), Becker (which is a milder version of Duchenne muscular dystrophy), and a group of conditions based on the muscle groups affected, for example, fascioscapulohumeral.

The general presentation of the condition is in muscle weakness, difficulty walking, and reliance, by early adolescence, on the use of a wheelchair. Children tend to fall easily and have an awkward gait. As time goes on they develop muscle contractures, scoliosis, and deterioration in respiratory muscles in the Duchenne type. These patients will need medication, ventilatory support, or even assisted ventilation. With modern treatment and medication, the lifespan of these patients has increased and now there are individuals surviving into their 40s. Cardiac myopathies occur in up to 50% after the age of 12 years.

Oral considerations

Dentally, such children present with wide‐spaced arches because of the relative hypotonia so that teeth are not in the usual soft tissue balance. Shedding of primary teeth may be delayed in widely spaced arches [24].

If the child requires ventilatory support, this can affect facial growth. The muscle effects are also seen in a reduction in bite force and hand grip, the latter potentially making mouth cleaning a problem. There is also a decrease in opening capacity occurring mostly in older teenagers.

Special considerations for dental care

Preventive dental care is the number one priority for these patients. As heart muscle may be affected, patients pose an anesthetic risk because of abnormal reactions to smooth muscle relaxants, made more complex by the effects on involuntary respiratory muscles. Children are sensitive to succinylcholine and volatile anesthetic gases and these should be avoided. Such patients can be at risk of sudden cardiac death because of conduction disturbances and may have pacemakers fitted electively. Local analgesia must be used with caution as tolerance is lower than for the general population.

Children with muscular dystrophy will develop malocclusions but the soft tissue balance is such that maintaining tooth movements, without permanent, fixed retention, is impractical. Children will, in the Duchenne and Becker types of muscular dystrophy, present with severe, skeletal open bites, and posterior cross bites. The latter occurs as a consequence of the relative lack of tone of the masseter muscles and the increased bulk of the hypotonic tongue. The treatment of malocclusions and the reduced opening capacity should be carried out by specialist teams as there are restrictions regarding training of muscles in some diagnoses. Referral to specialist pediatric dentists will ensure that such multiprofessional care is provided.

With decreasing muscle function, plaque control becomes more difficult and, especially associated with the open‐mouth posture, leads to gingivitis. Macroglossia due to hypotonia and tongue thrusting are also features.

Breathing difficulties, ventilatory support systems, and difficulty in stabilizing the head and neck for patients in advanced stages of Duchenne muscular dystrophy require special dental care for the individual. All treatment has to be carried out with patients in their wheelchairs and there is a risk of aspiration of, for example, water.

Cleft lip and palate

This is a congenital anomaly that occurs in one in 800 live births worldwide although there is wide cultural variation: 0.9–1.9 per 100 live births [25].

Children may have a cleft of the lip alone, palate, or lip and palate. In addition, the defects may be bilateral. Approximately 70% of cases are isolated clefts of the lip or palate. Isolated clefts especially are associated with syndromes, in up to 20% of cases.

The etiology is unclear but is thought to be multifactorial with environment playing a big part. Coexisting morbidity in the mother, such as diabetes, folate deficiency, anticonvulsant drugs, and alcohol, have all been implicated as causative factors. A genetic trait consisting of variations in the DNA of susceptible families, called single nucleotide polymorphisms, associated with the IRF6 gene, increases the risk of cleft three‐fold.

The timing of surgical correction varies from country to country but usually approximates to the “rule of tens”—that is, 10 weeks in age, 10 kg in weight, and a hemoglobin of 10. The first procedure is usually to repair the lip. Thereafter, before speech is developed the palate is repaired, both primary and secondary. Children with velopharyngeal insufficiency may require further surgery to prevent nasal escape causing hypernasal speech.

Some centers provide palatal plates in the neonate to encourage the segments to approximate to aid the surgical correction, as well as a subsidiary effect in supporting feeding. Most infants with clefts are fed with the aid of special bottles, for example, Rosti or Haberman, that reduce the effort required by the child to feed. Such bottles make it easier to achieve an oral seal and avoid the build‐up of negative pressure.

Oral considerations

Children with a cleft may have delayed dental development as well as disorders of eruption and missing teeth and/or enamel hypoplasia. Teeth adjacent to the cleft may fail to erupt, especially canine teeth. In such children, bone grafting between 9 and 10 years of age, carried out prior to tooth eruption, encourages the eruption of the canine.

Special considerations for dental care

With a cleft of the palate, the surgical repair often results in a narrowed arch. Orthodontic treatment consists of arch expansion prior to alignment and so caries prevention is paramount in these children who frequently undergo extensive orthodontic treatment. Ectopic eruption of first permanent molars is seen much more commonly, in up to 10% of children, who have clefts [26]. This is associated with crowding, as a result of the maxillary arch constriction.

Poor self‐esteem may demotivate children so that oral hygiene is poor. Frequent ear infections, because of the intimate relationship between the muscles in the oropharynx that operate the Eustachian tubes and which are defective in a child with a palatal cleft, are treated with antibiotics. In the young child these factors may lead to an increased prevalence of dental caries.

Preventive dental care

Preventing dental disease is important for everyone but especially so for children with disabilities, whose general health may be put at risk from the presence of untreated oral/dental disease and/or for whom the treatment of such disease may put the child at risk. Which particular preventive strategy is adopted as optimal, both clinically and cost‐effectively, will depend on the level of provision: at the individual or community level and on the personnel available to deliver a particular program.

Diet

Children with disabilities may not achieve developmental milestones at the same time as their normal peers and may retain infant feeding practices for longer. These can be potentially damaging, for example, the prolonged use of bottle feeding or even breastfeeding after 12 months of age, given the higher lactose content of human milk.

Frequent infections in early childhood, which are commoner in children with disabilities, are often managed by the use of liquid oral medicines, some of which will still be sweetened with sucrose. For example, the child with a cleft palate may have middle ear infections and have regular courses of antibiotics. In addition, depending on medicine formulation, the risk for dental erosion may be greater. Children with renal disease may take some of their medication in an effervescent form that contributes to tooth wear because of its low pH and high titratable acidity. Discussion with the medical team caring for the child will find ways of delivering necessary medication in a safer way, for example, if liquid, by syringe via the retromolar area or in tablet rather than liquid form. Alternatively, sugars‐free medication may be available to prescribe.

Failure to thrive is a feature of some impairments and, after a period of parenteral feeding, such children may progress to being fed directly through a PEG. This may resolve reflux, a common finding in some children with cerebral palsy, which also contributes to dental erosion. Children who are PEG‐fed exclusively develop greater amounts of calculus than usual.

Children who have difficulties in feeding because of dysphagia or other aspects of disordered oromotor function may have food liquidized since they cannot tolerate food of normal texture, especially lumps. Sometimes, liquidized food is high in sugars and/or is retained around the oral cavity for prolonged periods, leading to dental caries. Other children, for whom calorie‐dense food supplements are required, may be more vulnerable to dental caries because of the high burden of sugars contained in such products. However, the dental team have to be realistic and it is more important that the child survives and thrives, not that they have perfect teeth! Ways need to be found of working around these challenges.

Fluorides

The appropriate use of fluorides is important in the prevention of dental caries in patients with disabilities, and many of the strategies used for the child population in general are relevant [27]. However, owing to the disability or medical condition the patients are often regarded as high risk for dental caries. As many patients with disabilities have difficulties rinsing with fluorides, sucking on fluoride tablets, or chewing fluoride chewing gums, a preventive home care regime where the teeth are brushed twice a day using age‐appropriate, fluoride toothpaste for at‐risk children needs to be combined with regular chair‐side prevention. High fluoride toothpaste may be recommended based on individual assessments, taking the risk for fluorosis into consideration. This should include professional cleaning and topical application of fluoride, carried out at intervals appropriate for the individual child’s risk situation. These preventive strategies must be emphasized in the treatment plan for patients with disabilities. See also Chapter 11.

Pit and fissure sealants

Published evidence suggests that fluoride varnishes are more effective in caries prevention if susceptible tooth sites also have sealants applied. Conventional pit and fissure sealants can be difficult to apply in some children with disabilities; these children often object to the aspiration of water to wash away the etchant gel and/or to the taste of the etchant. In addition, some children object to the noise of the whole procedure. An alternative is to apply a fluoride‐rich glass ionomer cement, which is moisture tolerant and does not require pre‐etching. If the child is treated under general anesthesia, pits and fissures should be sealed as a preventive measure to reduce the risk of dental caries.

Oral hygiene aids

Children’s teeth should be cleaned on eruption. Initially, this might be with a piece of gauze wrapped around a finger. The use of a smear of toothpaste is advocated. The fluoride content will depend on the child’s risk for caries and level of impairment, which may be difficult to determine at this stage of development, approximately 6 months of age (see section “Fluorides”).

Once teeth erupt, a parent can be guided on the use of a suitable tooth‐cleaning aid. This may be with a finger brush or baby’s toothbrush (Figure 24.7). The former gives the parents more confidence as they not only worry less about the child clamping down on their fingers as they brush, but they feel they are less likely to damage soft tissues as with a conventional brush.

Photo of a finger brush that can be used for gentle cleaning of the child’s teeth and/or for oral motor stimulation.

Figure 24.7 A finger brush that can be used for gentle cleaning of the child’s teeth and/or for oral motor stimulation.

As the child develops more teeth, a powered brush may be helpful although many children object to the sensation and noise of such brushes. An alternative is the Superbrush® that has three sets of bristles arranged so that the occlusal as well as buccal and lingual surfaces can be brushed simultaneously [17]. A Collis Curve® performs a similar task (Figure 24.8). Sometimes a prop can be helpful to maintain the mouth open so that the parent or carer can brush one side effectively (Figure 24.9).

Photo of a Superbrush® on the left and two Collis Curve® brushes on the right.

Figure 24.8 A Superbrush® on the left and two Collis Curve® brushes on the right.

Photo of a prop holding open a mouth to facilitate brushing.

Figure 24.9 A prop to facilitate brushing.

For children with significant dysphagia in whom aspiration may be a risk, special aspirating mouth cleaners are available, which clear oral secretions as well as tooth‐cleaning products.

Chlorhexidine

Chlorhexidine‐containing products (chlorhexidine gluconate) have been advocated for use with people with disabilities when mouth cleaning is challenging because of poor or non‐cooperation by the child. This can be either swabbed around the mouth on gauze, touchettes, or sponge sticks (Figure 24.10), or as a rinse or spray [28]. They are also available as gels that can substitute for toothpaste and as varnishes for professional application. Some chlorhexidine products have side‐effects, most notably for the patient as tooth staining and alteration in taste. Some patients experience swelling of the parotid glands. In children with very sore mouths, alcohol‐free solutions with 0.12% or 0.06% chlorhexidine can be used. If these are not available, 2% chlorhexidine can be diluted 1:1 with water. Where a patient is really very uncooperative, chlorhexidine can be used in a spray form (conventional or mint flavor), or applied as a gel on a finger directly or with a finger brush. The use of chlorhexidine products should be based on risk assessment and part of a treatment strategy. There is the potential for patients to develop resistance towards chlorhexidine. The agents are usually used for certain periods of medication or when the child has an infection. For children with progressive terminal conditions, chemical plaque control with use of chlorhexidine is an important substitute for traditional oral hygiene.

Photo of a “touchette” or sponge stick cleaning a mouth.

Figure 24.10 A “touchette” or sponge stick for cleaning mouths.

Dental visits

It is crucial that children with disabilities are made known to the dental team at the earliest possible opportunity. Ideally a country should have a reporting mechanism in place so that all health care professionals involved with children who have an impairment are notified, with the consent of the family, about the child. By such means, appropriate support and early interventions can be designed around the child and the family.

Behavior management

As in any other patient, the dental treatment for children and adolescents with disabilities should be based on a thorough examination and treatment planning. The dental treatment itself is essentially the same, but it has to be carried out in a way that is modified, depending on the patient’s specific diagnosis and individual needs. Treating children and adolescents with disabilities involves a triangle of people. First, the young patient, second the parents and family, and third the dental health care personnel. In many cases the patient may also be accompanied by assistants. Having a disability or a chronic disease implies going through numerous visits to different health care facilities every year, which is why it is important that the dental visits are well organized in advance. This preparation includes learning more about the patient’s diagnosis and its implications for oral health and dental treatment, preferably before the visit. This signals interest and knowledge, which patients, as well as parents, express as important in order to have confidence in the dentist and the dental treatment [29–31]. Today there are many reliable sources of knowledge about even rare diagnoses to be found on the Internet, e.g., OMIM which can be accessed via PubMed. In addition, the dentist should plan for continuity of care by means of assuring that the patient will meet the same dentist at every dental appointment.

The ability to cope with dental treatment varies in children and adolescents with disabilities. The main reason for this lies in the degree of disability, and is related to factors such as cognitive and behavioral factors. Physical disability, body posture, and medication will also affect the treatment. Apart from this, it is important to acknowledge that patients with disabilities may, in addition, have behavioral management problems related to dental fear and anxiety. The basis for the patient’s willingness to undergo treatment relies on having established a good relationship with the dental team, an enhanced feeling of control in the patient, and treatment carried out in a way that minimizes pain. For some patients, sedation with nitrous oxide–oxygen or benzodiazepines can be helpful, and should follow the normal guidelines. This is covered in Chapters 6 and 9. One difference, however, when treating patients with a disability is the greater need to customize the experience for them, which requires time. It is therefore wise to allocate some extra time for the appointments. Some patients may still be difficult to examine or treat, or have a medical condition that requires specialist treatment. In such cases, referral to a specialized pediatric dentistry clinic is advocated. It is important that patients with disabilities are ensured good oral health and dental treatment and that the disability or medical condition does not constitute a barrier to this. For some patients, general anesthesia is required to fulfill these needs. Thus, the indications for treatment under general anesthesia may be wider than for nondisabled patients. This is usually specialist care, but it should be pointed out that there is need for these facilities [32].

Body posture

High‐quality treatment and good management of the patient are facilitated if the patient is able to relax in the dental chair. It is easier for the dentist to perform good examination or treatment when the patient is seated in the dental chair. For patients using wheelchairs it is usually possible to move the patient to the dental chair with a lift or special equipment for sliding the patient over to the dental chair. Most dental chairs are constructed to fit a full‐length body, and they are often uncomfortable for young patients to lie on. In these situations it is also difficult for the dentist to work in a good ergonomic position. For many young patients this can be improved by using cushions for the child to sit on in the chair. In a hypotonic patient, specially designed cushions (Figure 24.11) can be used to further support the body posture in the chair. In the case of spasticities, such cushions are used to flex knees and hip joint and to incline the head to a chin to chest position, which can reduce spastic problems and help the patient to relax [32].

Photo of body cushions giving support to a child during treatment.

Figure 24.11 Body cushions to give support to a child during treatment.

Oral motor function

The oral cavity is more than teeth, something that is very obvious when meeting children and adolescents with disabilities. Often the patient has problems related to communication, nutrition (eating, drinking, sucking, swallowing), perception or breathing. The etiology of these problems can be a combination of deviations in anatomy, sensation, oral motor function, and also the underlying medical condition and environmental aspects play a part. The handling of oral motor problems mainly concerns multiprofessional teams, including specialist pediatric dentists, in collaboration with a speech‐language pathologist, but it is essential that the general dentist is familiar with the management of these issues [32].

Feeding disorders (difficulties in eating, drinking, sucking, swallowing) are often seen in, for example, infants born preterm, in patients with clefts, neurologic or neuromuscular disease, or congenital heart defects. For some reason, sucking and breastfeeding are unsuccessful and many children need artificial nutrition through a nasogastric tube or a gastrostomy. If the child is not fed by mouth, this will interfere with the development of oral sensory and motor skills. The oral cavity often becomes hypersensitive, making it difficult to touch the mouth or to brush the teeth. A vicious circle can be established where the child does not tolerate food or liquid in the mouth, and will thus not exercise or use the oral sensory systems or motor functions, leading to difficulties in terminating tube feeding. There are different strategies and techniques to break this pattern, all within the competence of the speech‐language pathologist. However, for the dentist it is important to know that while doing this, offering small portions of foods and drinks with different tastes (including sweetened products) usually on frequent occasions throughout the day is often recommended and has proved to be effective. In these cases the dentist is advised not to interfere with these recommendations even though they are potentially damaging dentally. Instead, the dentist should provide extra help with check‐ups and chair‐side prevention. Once the feeding problems are resolved there will be time for more conventional dietary counseling from the dentist. Liaison with the dietician is helpful in these situations.

Many children and adolescents with hypersensory problems will not tolerate toothbrushing or the dentist checking the teeth. The acceptance of objects in the mouth has to be built up step by step; again an area of multiprofessional collaboration. What the dentist can suggest is introducing cleaning of the mouth from an early age, either with the help of gauze wrapped around the index finger or by use of a special finger toothbrush (see Figure 24.7). In older children an electric toothbrush can be helpful as the vibrations help to stimulate and give rise to tactile sensations in the oral cavity.

In order to train and exercise the oral motor complex, new methods are constantly being developed and evaluated. For example, there are massage techniques where the sensorimotor stimulation can affect muscle tone and improve motility. One particular method has been developed by Argentinian neurologist Rodolfo Castillo Morales and includes working on body posture, use of massage, and use of palatal plates or other oral devices. Poor lip closure combined with hypotonic muscles often results in drooling. To improve the function there are exercise programs and the patient can also train with an oral screen device (Figure 24.12a,b). There are individually fabricated palatal plates (Figure 24.13) to be used for specific training stimulation of, foremost, the tongue. Some results from studies in patients with Down syndrome indicate that training with palatal plates has a positive effect on oral muscle function and speech development [33]. Palatal plates can also be used to train children in overcoming articulatory problems. For this purpose the plate is individually designed with a fixed or mobile object inserted into the plate in the region where the child is supposed to put the tongue, in order to produce the correct sound. The use of different appliances should be based on multiprofessional assessments and evaluations. While the training is usually supervised by a speech‐language pathologist, the appliances require the taking of impressions for casts and this, as well as the regular supervision of the plate or oral screen, has to be managed by the dentist.

Photo of an oral screen.

Photo of a patient training with an oral screen by straining lip muscles and pulling the screen out with a light, balanced force.

Figure 24.12 (a) An oral screen; (b) a patient training with an oral screen by straining lip muscles and pulling the screen out with a light, balanced force.

Photo of two palatal plates used for oral motor training in children.

Figure 24.13 Palatal plates used for oral motor training in children.

References

1.     1. World Health Organization. International Classification of Functioning, Disability and Health (ICF). Geneva, Switzerland: World Health Organization, 2001.

2.     2. World Health Organization. International Classification of Functioning Disability and Health: children and Youth version: ICF‐CY. Geneva, Switzerland: World Health Organization, 2007.

3.     3. Faulks D, Hennequin M. Defining the population requiring special care dentistry using the International Classification of Functioning, Disability and Health—a personal view. J Disabil Oral Health2006;7:143–52.

4.     4. World Health Organization. International statistical classification of diseases and related health problems, 10th revision. http://www.who.int/classifications/apps/icd/icd10online/. (Accessed August, 2016).

5.     5. Merrick J, Carmeli E. A review on the prevalence of disabilities in children. The Internet Journal of Pediatrics and Neonatology 2003;3(1).

6.     6. Altarac M, Saroha E. Lifetime prevalence of learning disability among US children. Pediatrics 2007;119 Suppl 1: S77–83.

7.     7. Olsen J, Melbye M, Olsen SF, Sørensen TI, Aaby P, Andersen AM, et al. The Danish National Birth Cohort—its background, structure and aim. Scand J Public Health 2001;29:300–7.

8.     8. Kornfält R, Köhler L. Physical health of ten‐year‐old children. An epidemiological study of school children and a follow‐up of previous health care. Acta Paediatr Scand 1978;67:481–9.

9.     9. Office for Disability Issues. UK Government. Disability prevalence estimates 2011/12. https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/321594/disability‐prevalence.pdf. (Accessed August, 2016).

10. 10. Henderson J, Goldacre MJ, Fairweather JM, Marcovitch H. Conditions accounting for substantial time spent in hospital in children aged 1–14 years. Arch Dis Child 1992;67:83–6.

11. 11. Gillberg C, Coleman M. The biology of the autistic syndromes, 3rd edn. London: MacKeith Press, 2000.

12. 12. Fombonne E. Epidemiology of autistic disorder and other pervasive developmental disorders. J Clin Psychiatry 2005;66 (Suppl 10):3–8.

13. 13. American Psychiatric Association. DSM‐5 Development. http://www.dsm5.org/Pages/Default.aspx. (Accessed August, 2016).

14. 14. Bäckman B, Pilebro C. Visual pedagogy in dentistry for children with autism. ASDC J Dent Child 1999;66:325–31.

15. 15. Pilebro C, Bäckman B. Teaching oral hygiene to children with autism. Int J Paediatr Dent 2005;15:1–9.

16. 16. Blomqvist M. Oral health and behavior in children with Attention Deficit Hyperactivity Disorder. Thesis. Stockholm: Karolinska Institutet, 2007.

17. 17. Doğan MC, Alaçam A, Aşici N, Odabaş M, Seydaoğlu G. Clinical evaluation of the plaque‐removing ability of three different toothbrushes in a mentally disabled group. Acta Odontol Scand2004;62:350–4.

18. 18. Klingberg G, Lingström P, Óskarsdóttir S, Friman V, Bohman E, Carlén A. Caries‐related saliva properties in individuals with 22q11 deletion syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod2007;103:497–504.

19. 19. Fiske J, Shafik HH. Down’s syndrome and oral care. Dent Update 2001;28:148–56.

20. 20. Morgan J. Why is periodontal disease more prevalent and more severe in people with Down syndrome? Spec Care 2007;27:196–201.

21. 21. Rosenbaum P, Paneth N, Leviton A, Goldstein M, Bax M, Damiano D, et al. A report: the definition and classification of cerebral palsy, April 2006. Dev Med Child Neurol Suppl 2007; 109:8–14.

22. 22. Hagberg B, Hagberg G, Beckung E, Uvebrant P. Changing panorama of cerebral palsy in Sweden. VIII. Prevalence and origin in the birth year period 1991–94. Acta Paediatr 2001;90:271–7.

23. 23. Bernardini R, Novembre E, Lombardi E, Mezzetti P, Cianferoni A, Danti DA, et al. Risk factors for latex allergy in patients with spina bifida and latex sensitization. Clin Exp Allergy 1999;29:681–6.

24. 24. Ghafari J, Clark RE, Shofer FS, Berman PH. Dental and occlusal characteristics of children with neuromuscular disease. Am J Orthod Dentofacial Orthop 1988;93:126–32.

25. 25. Calzolari E, Pierini A, Astolfi G, Bianchi F, Neville AJ, Rivieri F. Associated anomalies in multi‐malformed infants with cleft lip and palate: an epidemiologic study of nearly 6 million births in 23 EUROCAT registries. Am J Med Genet A 2007;143:528–37.

26. 26. Bjerklin K, Kürol J, Paulin G. Ectopic eruption of the maxillary first permanent molars in children with cleft lip and/or palate. Eur J Orthod 1993;15:535–40.

27. 27. Marinho VC, Higgins JP, Sheiham A, Logan S. Combinations of topical fluoride (toothpastes, mouthrinses, gels, varnishes) versus single topical fluoride for preventing dental caries in children and adolescents. Cochrane Database Syst Rev 2004;(1):CD002781.

28. 28. Stiefel DJ, Truelove EL, Chin MM, Zhu XC, Leroux BG. Chlorhexidine swabbing applications under various conditions of use in preventive oral care for persons with disabilities. Spec Care Dentist1995;15:159–65.

29. 29. Hallberg U, Klingberg G. Giving low priority to oral health care. Voices from people with disabilities in a grounded theory study. Acta Odontol Scand 2007;65:265–70.

30. 30. Klingberg G, Hallberg U. Oral health–not a priority issue a grounded theory analysis of barriers for young patients with disabilities to receive oral health care on the same premise as others. Eur J Oral Sci2012;120:232–8.

31. 31. Trulsson U, Klingberg G. Living with a child with a severe orofacial handicap: experiences from the perspectives of parents. Eur J Oral Sci 2003;111:19–25.

32. 32. Nunn J, ed. Disability and oral care. London: FDI World Dental Press, 2000.

33. 33. Bäckman B, Grevér‐Sjölander AC, Bengtsson K, Persson J, Johansson I. Children with Down syndrome: oral development and morphology after use of palatal plates between 6 and 48 months of age. Int J Paediatr Dent 2007;17:19–28.