Dental Care for the Patient with Down Syndrome
This study consists of a clinical and literature review of the special dental considerations unique to individuals with Down syndrome. The author is both a dentist and a parent of a child with Down syndrome. Physical and orofacial characteristics of Down syndrome are discussed including the teeth, gingiva, tongue, palate, and occlusion. Incidences of dental decay and periodontal disease are discussed and how best to treat these diseases in persons with Down syndrome. Most if not all persons with Down syndrome have some type of occlusal disharmony. Techniques to help prevent occlusal problems are discussed, including orthodontic therapy. Medical problems associated with Down syndrome that can affect dental treatment are discussed. Also, social and emotional factors involved in dental treatment are covered, including techniques to help children with Down syndrome become co-operative dental patients. Information on how to choose the right dentist for your child and how to communicate effectively with the dental staff is given. Finally, information on proper home care and prevention of dental disease is covered, including information on the most recent dental products.
Pilcher ES. Dental Care for the Patient with Down Syndrome. Down Syndrome Research and Practice. 1998;5(3);111-116.
* From a paper presented at the 6th World Congress on Down Syndrome, Madrid, Spain,
Approximately one out of every 800-1,100 births results in an extra chromosome of
the twenty first group called Trisomy 21, or Down syndrome. Affecting over 250,000
people in the U.S. alone, this population has progressed tremendously over recent
years to be able to function in the mainstream of society. Inclusive school, work
and community settings are now becoming the norm for persons with Down syndrome.
This has resulted in a higher level of functioning for most of these individuals
with resulting increases in self-esteem and self-image. The demand for dental care
in persons with Down syndrome is increasing with this inclusive trend. Most dental
treatment for persons with Down syndrome can take place in a general dental office
with relatively minor adaptations. In undergraduate dental training there is usually
little or no exposure to treating patients with disabilities, and general practitioners
may be hesitant to treat these patients with confidence. This paper will attempt
to summarise the unique characteristics associated with Down syndrome that influence
the dental care and treatment of this population.
Systemic factors influencing dental care
Although 40 to 50% of babies with Down syndrome are born with some type of cardiac
abnormality, most receive surgical correction within the first few years of life.
There is however, an abnormally large percentage who develop mitral valve prolapse
(MVP) by adulthood. The incidence of MVP in the normal population is between 5-15%.
Approximately 50% of adults with Down syndrome have mitral valve prolapse requiring
subacute bacterial endoconditis (SBE) prophylaxis for dental treatment (Barnett, Friedman, &
Kastner, 1988). One third of these adults with MVP do not have ausculitory
findings, requiring diagnosis of the MVP by echocardiogram. Patients with Down syndrome,
or their caregivers may not be aware of the need for diagnostic echocardiology in
A compromised immune system with a corresponding decrease in number of T cells is
characteristic of most individuals with Down syndrome This contributes to a higher
rate of infections and is also a contributing factor in the extremely high incidence
of periodontal disease. Children with Down syndrome often have chronic upper respiratory
infections (URIs). These contribute to mouth breathing with its associated effects
of xerostomia (dry mouth) and fissuring of the tongue and lips. There is also a
greater incidence of apthous ulcers, oral candida infections and ANUG.
A reduced degree of muscle tone (hypotonia) is generally found in Down syndrome.
This affects the musculature of the head and oral cavity as well as the large skeletal
muscles. The reduced muscle tone in the lips and cheeks contribute to an imbalance
of forces on the teeth with the force of the tongue being a greater influence. This
contributes to the open bite often seen in Down syndrome. Additionally, reduced
muscle tone causes less efficient chewing and natural cleansing of the teeth. More
food may remain on the teeth after eating due to this inefficient chewing. Associated
with the low muscle tone seen in Down syndrome is a ligamentous laxity seen throughout
the body. This causes hyperflexibility of the joints and it is theorised that the
ligaments around teeth may be influenced as well (Southern
Assoc. of Institutional Dentists, pg.4, 1994). A condition related to ligamentous
laxity is that of Atlanto Axial Instability. The diagnosis and significance of this
condition is controversial but is described as an increase in mobility between the
C1 and C2 cervical vertebrae and may be seen in 10-20% of individuals with Down
syndrome. If a patient has this instability, careful positioning in the dental chair
is required to avoid any potential harm to the spinal cord.
Persons with Down syndrome vary widely as to their degree of intellectual impairment.
Most have IQs in the mild to moderate range and are able to be treated in a normal
setting. There is often a relatively severe delay in language development. The patient
with Down syndrome will probably understand more than their apparent level of verbal
skills. The assistance of the patient's family or caregiver will be necessary in
conveying to the dentist and staff what level of communication should be used with
the patient. It may take a little extra appointment time to explain procedures to
the patient with Down syndrome, but once a level of trust is achieved they are likely
to be very co-operative patients.
Down syndrome is frequently seen in conjunction with other medical problems. There
is a higher incidence of epilepsy, diabetes, leukaemia, hypothyroidism and other
conditions. Alzheimer's disease and Down syndrome appear to have a strong connection
to one another. The importance of a thorough medical history including a work-up
by a physician cannot be over emphasised.
The primary skeletal abnormality affecting the orofacial structures in Down syndrome
is an underdevelopment or hypoplasia of the midfacial region. The bridge of the
nose, bones of the midface and maxilla are relatively smaller in size. In many instances
this causes a prognathic Class III occlusal relationship which contributes to an
open bite (Vittek,
Winik, Winik, Sioris, Tarangelo et al., 1994). Absence or reduction in size
of the frontal and maxillary sinuses is common.
The incidence of mouth breathing is very high due to a small nasal airway. The tongue
may protrude and appear to be too large. True macroglossia is rare, rather a relative
macroglossia is found where the tongue is of normal size but the oral cavity is
decreased in size due to the underdevelopment of the mid-face. Upon examination
the palate in a person with Down syndrome appears to be narrow with a high vault.
In actuality the vault is of normal height but the sides of the hard palate are
abnormally thick. This creates less space in the oral cavity for the tongue affecting
both speech and mastication. Speech pathologists can be of help in teaching correct
tongue positioning and increasing the tone of the orofacial musculature. In extreme
cases, surgical reduction of the tongue may be indicated (Margar-Bacal, Witzel,
& Munro, 1987;
Starmans & Bloem, 1991).
With age, both the tongue and the lips in people with Down syndrome tend to develop
cracks and fissures. This is a result of chronic mouth breathing. Fissuring of the
tongue can become severe and be a contributing factor in halitosis. Patients should
be instructed to brush their tongue when they brush their teeth. Another result
of chronic mouth breathing may be a decrease in saliva with a dry mouth. This reduces
the natural cleansing that occurs in the oral cavity and may contribute to the development
of caries. Irritation at the corners of the mouth (angular cheilitis) may also be
a result of mouth breathing.
The eruption of teeth in persons with Down syndrome is usually delayed and may occur
in an unusual order (Mussig,
Hickel, & Zschiesche, 1990;
Fisher-Brandeis, 1989). The delay in eruption may be as long as two
to three years. Although independence in feeding and the introduction of adult food
is encouraged in the toddler with Down syndrome, the diet may need to be altered
because not enough teeth have erupted to safely chew some foods. There is an extremely
high rate of missing teeth in both the primary and permanent dentitions. Other irregularities
such as microdontia and malformed teeth may be seen. However in an oral cavity with
undersized bone structure, microdontia and missing teeth may be more of a blessing
than a problem. Severe crowding can occur in persons with Down syndrome who have
developed all of their permanent dentition (Ondarza,
Jara, Bertonati, & Blanco, 1995). In these cases, selective extractions
under the supervision of an orthodontist may be of benefit.
The roots of the teeth in patients with Down syndrome tend to be small and conical.
This is an important factor when considering orthodontic tooth movement and also
contributes to early tooth loss in periodontal disease.
Historically the incidence of dental decay in persons with Down syndrome has been
reported to be extremely low. Recent studies however have shown that while the incidence
is lower, it is not as rare as once thought and it certainly should not be taken
for granted that "these patients won't get decay" (Barnett,
Press, Friedman, & Sonnenburg, 1986). Older studies of caries in persons
with Down syndrome used institutionalised populations whose diets were controlled.
These groups may not have had the exposure to cariogenic foods at the rate of today's
children with Down syndrome who are growing up at home. The incidence is lower however,
and it is theorised that this may be due to delayed eruption of the teeth, increased
spacing between teeth or possible differences in the chemical content of the saliva
Lopatin, & Tanaka, 1995).
Today, children with Down syndrome should be educated in proper oral hygiene, and
receive the benefits of both systemic and topical fluoride. Occlusal sealants are
also recommended. Decay in the primary dentition should be promptly treated. With
the delayed emergence of the permanent teeth and the high number of missing teeth,
it is critical to maintain the primary dentition as long as possible.
On the opposite end of the spectrum from caries is the high rate of periodontal
disease seen in Down syndrome (Shapira,
Stabholtz, Schurr, Sela, & Mann, 1991). Early, severe periodontal disease
is often seen with onset in the mid to late teen years. Some studies report an incidence
of periodontal disease to be between 90 and 96% in adults with Down syndrome. This
is thought to be related to a lowered host response due to the compromised immune
system in Down syndrome (Sohoel,
Johannessen, Kristofferson, & Nilsen, 1995;
Sohoel, Johannessen, Kristofferson, Haugstvedt, & Nilsen, 1992;
Shaw & Saxby,
1986). The amount of plaque and calculus seen on the teeth is not proportionate
to the severity of the disease (Yavuzyilmaz,
Ersoy, Sanal, Tezcan, & Ercal, 1993). The teeth most affected are the
mandibular incisors and maxillary molars. Good oral hygiene and semi-annual prophylaxis
appointments may not be enough to prevent the progression of periodontal disease
in these patients. Early, aggressive treatment is needed. These patients may need
to be seen as often as every three months for scaling and root planing and may also
benefit from the use of chlorhexedine mouth rinse and possibly systemic antibiotic
Shapira, Shur, Friedman, Guberman, et al., 1991).
Obviously, good home care is essential in the management of periodontal disease
of this type. This may be difficult to achieve with the intellectual impairment
and decreased manual dexterity seen in Down syndrome. Flossing may be very hard
for these patients and instruction in the use of a floss holder may be helpful.
New mechanical tooth brushing and flossing aids on the market may also be of help.
It is important to be sure that the patient's family or caregiver is educated in
proper home care as well. Parents need to realise the importance of proper daily
home care because the child with Down syndrome may be resistant to tooth brushing.
Additionally, the age at which a child with Down syndrome can be expected to take
care of his/her own teeth may be much later than that of normal children.
Treatment objectives for any population with developmental disabilities should be
the same as that of normal patients. Treatment plans may need to be adapted as necessary
due to each individual's condition, but the overall goal should be to provide as
comprehensive treatment as possible. Areas of dental care such as cosmetic dentistry,
orthodontics, prosthodontics, and reconstructive oral surgery should not be ruled
out simply because the patient has Down syndrome. With the numbers of persons with
Down syndrome working and living out in the community, there may be many who desire
and can handle some of the more extensive dental treatment options available today.
Good behaviour in the dental office is learned. In a population with delayed learning,
this can be a challenge for the dentist and staff. Dental treatment for children
with Down syndrome may not be sought out at an early age. There may be more pressing
medical problems, financial considerations or parents may want to wait until the
child seems mature enough to handle a visit to the dentist. Unfortunately this makes
it more difficult to teach proper home care and to develop a relationship with the
child that will result in co-operative behaviour during dental treatment.
Determining the level of communication is very important in developing a co-operative
relationship with your patient with Down syndrome. The level of receptive vs. expressive
language may not be the same. The patient's family or caregiver will be able to
guide the dental staff as to what level of communication is appropriate. It is important
that the dentist communicate directly with the patient whenever possible in order
to build a level of trust. It may be advantageous to have a parent in the operatory
during some early childhood visits. Finding out what motivates the child with Down
syndrome is also important. Something as simple as receiving a pair of gloves and
a mask at the end of the appointment may be all it takes to ensure co-operation.
With more difficult patients requiring more extensive treatment, premedication and/or
restraints may be necessary. However, most patients with Down syndrome can handle
routine dental care with just a little more time and attention given during the
Scheduling appointments early in the day is beneficial as both patient and operator
are more rested. First appointments should be for orientation only, and subsequent
appointments may require a little more time than what is usually allowed. The patient's
medical history should be obtained prior to the first appointment. This allows for
medical consultation if necessary before any treatment begins.
Treating the older patient with Down syndrome may present a different set of problems.
There appears to be a high incidence of early onset Alzheimer's disease in persons
with Down syndrome. Studies vary widely, but the incidence has been reported to
be anywhere from 30-90%. The average age of onset of clinical symptoms is between
40-50 years (Sigal
& Levine, 1993;
Vicari & Caltigirone, 1990;
Devenny, Hill, Patxot, Silverman, & Wisniewski, 1992). These
patients will require a great deal of understanding and their level of co-operation
may decrease as the disease progresses.
It is important that the dental health provider be aware of the incidence of sleep
apnea in the Down syndrome population. It has been reported that the incidence of
upper airway obstruction may be as high as 31% in children with Down syndrome (Stebbens,
Dennis, Samuels, Croft, & Southhall. 1991). The decreased airway size
combined with lowered muscle tone predisposes these patients to obstructive sleep
apnea. Left untreated, obstructive sleep apnea can further increase developmental
delay and lead to pulmonary hypotension and congestive heart failure. Symptoms of
obstructive sleep apnea include snoring, restless sleep and unusual sleeping positions.
If a patient's family or caregiver reports these symptoms, then referral to a sleep
disorders clinic is indicated. Treatment ranges from occlusal repositioning appliances,
positive airway pressure and/or surgical correction. Adenotonsillectomy in children
with Down syndrome and sleep apnea has been helpful in some cases (Bloch,
Witztum, Wieser, Schmid, & Russi, 1990;
Phillips & Rogers, 1988;
Dental care for the patient with Down syndrome can be achieved in the general practitioner's
office in most instances with minor adaptations. Although this population has some
unique dental care needs, few patients require special facilities in order to receive
dental treatment. Adequate dental health care for persons with developmental disabilities
is a major unmet health need. It is hoped that the information contained in this
review will encourage general practitioners to be willing to provide comprehensive
dental care to their patients with Down syndrome.
Elizabeth S. Pilcher, Associate Professor, Department of Prosthodontics, College
of Dental Medicine, Medical University of South Carolina, 171 Ashley Avenue, Charleston,
South Carolina 29425, U.S.A. (Tel: 803-792-2341, Fax: 803-792-159, E-mail: firstname.lastname@example.org)
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