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Medical problems and oral care of patients with Down syndrome: A literature
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Article  in  Special Care in Dentistry · November 2011

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M E D I C A L P R O B L E M S O F PAT I E N T S W I T H D O W N S Y N D R O M E
ARTICLE

ABSTRACT Medical problems and oral care

When planning the dental treatment of
patients with Down syndrome (DS),
dental practitioners should always
of patients with Down syndrome:
consider their general health, in order to
achieve a holistic and interdisciplinary a literature review
approach. This article presents a litera-
ture review of the primary medical
conditions in patients with DS that may Jenny Abanto, DDS, MSc;1 Ana Lidia Ciamponi, DDS, PhD;2
affect their general health care and the Elizabeth Francischini, DDS;3 Christiana Murakami, DDS, MSc;1
appropriate clinical delivery of oral
Nathalie Pepe Medeiros de Rezende, DDS, PhD;3 Marina Gallottini, DMD, PhD4*
health care.
1Department of Orthodontics and Pediatric Dentistry, School of Dentistry; 2Assistant Professor,
KEY WORDS: medical conditions,
Department of Orthodontics and Pediatric Dentistry; 3Department of Oral Pathology, Special Care
Down syndrome, dental management
Dentistry Center, School of Dentistry; 4Chairman, Department of Oral Pathology, Special Care Dentistry
Center, School of Dentistry, University of São Paulo, São Paulo, Brazil.
*Corresponding author e-mail: mhcgmaga@usp.br/jennyaa@usp.br

Spec Care Dentist 31(6): 197-203, 2011

Introduction
Down syndrome (DS) represents the most common chromosomal abnormality associated
with intellectual impairment. It affects an estimated 1 in 800 births or 5,400 infants in
the United States each year.1
In addition to intellectual disability, motor disorders, and dismorphologies, individ-
uals with DS present with medical conditions such as cardiovascular, immunological,
hematological, respiratory, neurological, and musculoskeletal abnormalities.2 Patients
with DS also have a higher prevalence of dysfunction within certain organs, such as the
esophagus and thyroid gland.2

In spite of the high prevalence of

such comorbidities, life expectancy
among individuals with DS has been
increasing substantially over time.2
Advances in the medical field have con-
tributed to increasing numbers in the
aging population with DS who have sec-
ondary disorders, such as Alzheimer’s
disease and diabetes mellitus, conditions
which dental care providers need to be
aware2 (Table 1).
Assessment of medical conditions in
patients with DS is particularly important
for oral healthcare treatment. DS is a
common disability and patients with DS
currently have a longer life expectancy
than in the past. The purpose of this
review is to inform dental professionals
of the medical conditions that may affect
the delivery of oral health care for
patients with DS.
Cardiovascular
a bnor malities
The prevalence of congenital heart dis-
ease in patients with DS ranges from 40%
to 60% at birth and varies according to
ethnic group and geographic location.3-5
Recent findings5 suggest that gender and
ethnicity may be factors in the incidence
of congenital heart defects in patients
with DS. Atrioventricular septal defects
show the most significant gender and
ethnic differences, with twice as many
females and African descendants affected;
Hispanics are less affected than
Caucasians.5 About half of all deaths in
children with such congenital cardiac
malformations occur within the first year
of life.3 Atrioventricular septal defect is
the most prevalent cardiac alteration
(39%), followed by atrial septal defect

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should be avoided during dental treat-

Table 1. Medical conditions commonly seen in patients with DS,
the main symptoms and dental treatment implications. ment of patients with cyanotic heart
diseases and, when necessary, sedation
Medical conditions can be used to reduce anxiety.9
Estimated frequency Dental implications
commonly seen in DS
Intravenous sedation is an effective and
Immunologic safe alternative for some individuals with
90% • Periodontitis
abnormalities DS and may improve the quality and
Hematologic
12- to 40-fold
• Increased risk of leukemia
scope of the dental treatment provided.10
increased risk However, according to the literature,9,10
• Tooth wear before sedation, patients should be radio-
Esophageal 13.8% to 59% logically evaluated for vertebral
• Hypersensitivity
(atlantoaxial instability) and rib anom-
• Controlling oral hygiene because of the
Respiratory infection 40% to 60% alies and for cardiac, intestinal, and
aspiration of oral secretions
endocrine disorders. Careful intubation
Cardiovascular 40% • Risk of infective endocarditis and close monitoring of the airway are
• Risk of medullary injury during manipulation of necessary to guard against obstructions
Atlantoaxial instability 10% to 30% as a result of anatomic problems, such as
the patient’s neck
short neck and rib anomalies. Suitable
• Hypotonicity of the perioral muscles, lips, positioning and supportive care of the
Muscle hypotonia 100% and chewing muscle
patient’s head during intubation and pro-
• Protruding tongue
cedures should prevent most spinal
• Mild to moderate anxiety to phobia, or nerve injuries and possible paralysis in
Alzheimer’s disease 45%
complete lack of cooperation. these patients, since many show
Seizures 5% to 7% • Interruption of the dental appointment atlantoaxial instability and hypotonia.10
However, there may be complications
• Xerostomia, burning mouth/tongue, candidal of airway management due to the short
infection, progressive periodontitis, parotid
Diabetes 1.4% to 10.6% neck, large tongue, obstructed nasal pas-
enlargement, sialosis, and delayed wound
healing sages, and sleep apnea symptoms often
associated with DS.11,12
• Check high blood pressure Furthermore, maintaining good daily
Sleep apnea 57%
• Consider oral appliances oral hygiene is important because signifi-
cant transient bacteremias occur during
routine daily activities such as chewing
(29% to 42%), patent ductus arteriosus develop cardiac valvulopathy, the food, toothbrushing and flossing, and
(17%), ventricular septal defect (14% to American Heart Association (AHA)8 rec- using toothpicks.13 Chlorhexidine
43%), and tetralogy of Fallot (6%).4,5 ommends the use of antibiotic mouthwash or gel may be useful in con-
In patients with complete atrioven- prophylaxis (Table 2) before dental pro- trolling periodontal disease. We
tricular septal defects, symptoms usually cedures, which could cause transient encourage regular dental visits at least
occur in infancy as a result of systemic- bacteremias. According to the most every “six months” for review and pro-
pulmonary shunting, high pulmonary recent AHA guidelines,8 such procedures fessional support in order to maintain
blood flow, and an increased risk of are those which involve manipulation of adequate oral health for people with
developing pulmonary arterial hyperten- gingival tissue or the periapical region DS.14
sion.6 Patients with DS are considered to and perforation of the oral mucosa, such
be at higher risk for pulmonary hyper- as during subgingival scaling, dental
tension probably because of a diminished extractions, suture removal, rubber dam Immunological
number of alveoli, a thinner media of matrix placement, and placing of ortho-
pulmonary arterioles, and an impaired dontic bands. Antibiotic prophylaxis is a bnor malities
endothelial function.6 Early corrective no longer recommended for patients While the etiology of abnormal humeral
cardiac surgeries are necessary to prevent with other types of congenital heart and cell-mediated immunological func-
irreversible pulmonary-vascular damage.7 defects or who have had surgical repair.8 tion in patients with DS remains
For patients presenting with unre- Patients with unrepaired cyanotic unclear,15 defects have been shown in B,
paired cyanotic congenital heart defects, congenital heart disease may become T, and natural killer cell function, in
prosthetic valves, or previous episodes of cyanotic, hypoxic, and tired.9 The adult cytokine production, in phagocytic and
infective endocarditis (IE), as well as for population is also predisposed to postop- chemotactic responses, and in
recipients of cardiac transplantation who erative hemorrhage.9 Stressful situations immunoglobulin levels with reduced

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Table 2. Antibiotic prophylaxis regimen for dental procedures.
To be
Situation Drug
administered
Normal Orally Amoxicillin
Cephalexin or
Allergic to
Clindamycin or
penicillin or Orally
Azithromycin or
ampicillin
Clarithromycin
Intramuscularly Ampicillin or
Unable to take
or Cefazolin or
oral medication
intravenously Ceftriaxone
Allergic to
penicillin or Intramuscularly Cefazolin or
ampicillin and or Ceftriaxone or
unable to take intravenously Clindamycin
oral medication
levels of lymphocytes.15,16 Impaired T-cell
function is associated with low CD4
numbers. Autoimmune disease occurs
with antithyroid, antigliadin, and anticar-
diolipin antibodies, contributing to
worsening immunoglobulin function
with age.17
Several studies18-20 demonstrate that
hepatitis B virus (HBV) infections are
more prevalent among individuals with
DS (40%). Institutionalization and a pre-
disposition for infection are two factors
that may explain elevated levels of
chronic HBV infections in patients with
DS, which are seen as the presence of
HBV surface antigens (HbsAg) in their
serum.18 Therefore, HBV vaccination is
highly advisable for patients with DS
because of the significant risk of becom-
ing a chronic HBV carrier once
infected.19 It is particularly important in
preschool children who constitute the
most susceptible group and where the
response to immunization is more effec-
tive.20 However, hepatitis B vaccination
in children remains low in several coun-
tries19; therefore it is important to
reinforce the idea that vaccination
against hepatitis B in preschool children
with DS is effective despite their existing
immune function abnormalities.
Due to the high incidence of
immunological alterations previously
Single dose 30–60 minutes
before procedure
Adults Children
2g 50 mg/kg of body weight
2g 50 mg/kg of body weight
600 mg 20 mg/kg of body weight
500 mg 15 mg/kg of body weight
2g 50 mg/kg of body weight
1g 50 mg/kg of body weight
1g 50 mg/kg of body weight
600 mg 20 mg/kg of body weight
described in patients with DS, a careful
medical history should be taken and ver-
ification of leukocytes and platelet levels
in the blood should be done before initi-
ating any invasive dental procedures.
Hematological
a bnor malities
Newborns and children with DS may
present with many hematological prob-
lems, particularly an increased risk of
leukemia.21 For individuals with DS
between the ages of 5 and 30 years, the
risk increases approximately 12-fold for
developing acute lymphoblastic
leukemia; that risk rises to approxi-
mately 40-fold in children younger than
5 years.21 There is also a unique predis-
position to transient neonatal leukemia
(10%), known as transient abnormal
myelopoiesis.22 The etiological role of
the additional chromosome 21 in the
development of leukemia is supported
by the observation that leukemic cells of
young children with trisomy 21
mosaicism selectively involve the tri-
somic cells. Several genes on
chromosome 21 have been found to be
disrupted in leukemia.23
Leukemia is characterized by a high
incidence of oral complications at the
time of diagnosis and during treatment.24
Such oral complications can be divided
into three groups.24,25 Primary lesions are
caused by infiltration of malignant cells
into oral structures such as the gingiva
and bone. Secondary lesions are the
result of the myelophthisic character of
the disease and include symptoms of
anemia, increased tendency to bleed, and
increased susceptibility to infection.
Tertiary lesions are induced by the
patients’ antileukemia treatment. The
most common symptom detected is
mucosal pallor. Hemorrhage of the gin-
giva, petechiae, and ecchymosis of the
oral mucosa are due to thrombocytope-
nia, platelet defects, capillary fragility,
and abnormal coagulation factors.24
There is no indication that oral hygiene
should be discontinued during the treat-
ment of patients with leukemia. In
patients who are capable of maintaining
oral hygiene, this procedure seems to be
an appropriate way of avoiding the devel-
opment of oral problems without
increasing the risk of infection.25
Esophageal dysfunctions
Gastric dysfunctions like gastroe-
sophageal reflux and vomiting are
frequent findings in individuals with DS
(13.8% to 59%).26,27 The symptoms of
these dysfunctions are generally not
readily evident due to patients’ difficulty
in expressing themselves, which may
hinder the clinical assessment of the
problem and delay diagnosis.27
One study28 showed that tooth wear,
mainly dental erosion, was significantly
more frequent among persons with DS
(67%) than in the general population of
children of a similar age (34%). Most of
the children with DS showed severe to
very severe wear associated with gastric
reflux and vomiting.28 Given the poten-
tial consequences of high levels of tooth
wear associated with an acidic oral envi-
ronment, dentists need to develop
educational programs aimed at increas-
ing awareness of this problem among
caregivers and health professionals.
Since the symptoms of esophageal dys-
function are not always evident in
patients with DS, when dentists detect

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the presence of tooth wear, they should
refer the patient to a gastroenterologist
for evaluation, thus minimizing dental
destruction and dentinal hypersensitivity
which can improve the patient’s general
health.
Respirator y
a bnor malities
Individuals with DS are generally more
susceptible to respiratory tract infec-
tions due to abnormal airway anatomy,
hypotonia, aspiration of oral secretions
and bacteria, and immunological alter-
ations.26,27 They may show quantitative
and qualitative abnormalities of innate
and adaptive immunity, which suggests
a diminished ability to clear viral and
bacterial organisms.29 Among the most
common respiratory infections in per-
sons with DS are pneumonia,
rhinopharyngitis, and bronchitis,15 asso-
ciated with Staphylococcus, Streptococcus
pneumoniae, Pseudomonas aeruginosa,
and Candida albicans.27 Upper airway
narrowing may also be present in per-
sons with DS, and may cause a range of
conditions including midface hypopla-
sia, macroglossia, narrowing of the
nasopharynx, tonsillar and adenoidal
enlargement, lingual tonsils, choanal
stenosis, shortening of the palate, and
glossoptosis.16 In addition, subglottic
stenosis, laryngomalacia, tracheomala-
cia, and congenital malformations of the
larynx, trachea, and bronchi may fur-
ther reduce airway diameter.16
Maintaining an adequate level of
daily oral hygiene care is an important
strategy for preventing pneumonia in
patients with DS.28 In patients with
acute pneumonia, the authors recom-
mend that dental treatment should be
temporarily interrupted. If dental emer-
gency procedures are necessary, the
patient can be treated if he/she is
already being medicated for pneumo-
nia. During the dental treatment of
patients with DS who have bronchitis
or asthma, bronchodilating inhalers
are recommended to be readily avail-
able in case a crisis should occur
during treatment.
200 S p e c C a re D e n t i s t 3 1 ( 6 ) 2 0 1 1
Musculosk eletal and orofacial function leading to signifi-
cantly improved speech development,
a bnor malities swallowing, and masticatory function.
Atlantoaxial instability is one of the most
common characteristics in individuals
with DS; it occurs due to a congenital Neurological and
laxity of the transverse ligaments of the
atlas and atlanto-occipital joints. This
behavioral alterations
problem is fairly frequent (10% to 30%)30
Individuals with DS have a higher rate of
Alzheimer’s-type changes in the brain
and should be evaluated during an
autopsy if the cause of death is unclear.16
with an increased risk of stroke16; the
latter has been associated with the pres-
The symptoms and signs of atlantoaxial
ence of antiphospholipid antibodies.33
instability are usually chronic, resulting
Forty-five percent of people with DS over
from compression of the cord and/or
the age of 40 years will develop
nerve roots. Lethal respiratory arrest may
Alzheimer’s disease.16 Cerebrovascular
occur from acute compression of the
hemorrhage may also be related to an
upper cervical cord.16
increased production of amyloid protein
It has been recommended14,30 that
associated with the chromosome 21 APP
patients with DS have a cervical radi-
gene, the so-called cerebral amyloid
ograph made at the age of 3 years, and
angiopathy.15 Alzheimer’s-type changes
that this should be repeated before the
develop at an earlier age in persons with
beginning and at the end of puberty to
DS, and it has been associated with pre-
evaluate the presence of atlantoaxial
mature aging.34 Histological evaluation of
instability. Injury can occur with hyper-
older brains among the general popula-
extension or radical flexion of the neck
tion may show neurofibrillary tangles,
or upper spine, and can lead to irre-
senile plaques, and granulovacuolar
versible damage to the spinal cord.16 It is
degeneration.16,34 The development of
prudent to investigate the individual’s
dementia may be associated with prob-
status before providing a general anes-
lems with swallowing, frequent choking,
thetic for dental treatment due to the fact
and aspiration pneumonia.34 Epileptic
that this procedure may increase the sus-
seizures may also occur. Although
ceptibility for atlantoaxial instability.12
epilepsy has been found35 to increase the
The dentist and the anesthesiologist need
rate of sudden and unexpected death in
to be aware of this risk during manipula-
individuals who are intellectually
tion of the patient’s neck.30 An alternate
impaired, its significance in persons with
option would be to place the patient’s
DS may vary with age. It appears that
arms extended around the head in order
seizures occur in 5% to 7% of individuals
to avoid abrupt head movements if
with DS, compared to 20% to 50% of
restraint is needed.
children with other forms of mental
Muscle hypotonia is a marked char-
impairment. A higher rate of seizures is
acteristic of patients with DS. Moreover,
seen in adults who have DS with demen-
the generalized muscular hypotonia may
tia when compared to persons with
hinder mastication as well as the devel-
Alzheimer’s disease in the general popu-
opment of speech and of the dentition.31
lation.14,16
Infants with DS often present with a typi-
In the general population, memory
cal orofacial disorder, the features of
and orientation problems are commonly
which include hypotonicity of the perio-
the first signs of developing Alzheimer’s
ral muscles, lips, and chewing muscles,
dementia. In persons with DS, prefrontal
and a protruding tongue, later followed
lobe symptoms may be more common,
by active tongue protrusion, as well as
including indifference, uncooperative-
problems with sucking, drooling, etc.30-32
ness, apathy, depression, and socially
Various clinical studies31,32 have con-
deficient communication or impaired
firmed the positive effect of the
adaptive functioning in general. Only in
stimulating plate on facial appearance
more advanced stages of the disease are
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memory problems seen.36 It is important,
however, to emphasize that not only is
cognitive decline important in the diag-
nosis of Alzheimer’s dementia, but that
behavioral, mood, daily functioning
changes, and emotional symptoms influ-
ence oral health care.
People with DS exhibit a wide range
of behavior in the dental setting, includ-
ing cooperation, compliance, mild to
moderate anxiety, phobia, and complete
lack of cooperation.14 For many patients,
it is possible to carry out simple restora-
tive treatment and preventive measures
using behavioral management techniques
such as tell-show-do, positive reinforce-
ment, modeling, distraction, and both
verbal and nonverbal communication.14
The authors also recommended keeping
appointments short and focusing on a
specific treatment for each consultation.
The symptoms of Alzheimer’s, short-
term memory loss and a progressive loss
of daily living skills, affect the ability to
cope and cooperate with dental treat-
ment and daily oral hygiene. Thus, it is
important that adults with DS have a
program of continuing oral care to mini-
mize the need for invasive dental
treatment and to ensure support for oral
hygiene from their caregivers.14 In such
cases, the use of electric toothbrushes for
oral hygiene and the professional appli-
cation of fluoride and chlorhexidine gels
may be beneficial.12,36
Thyroid gland
dysfunction
DS has been associated with a variety of
autoimmune conditions, most frequently
those affecting the thyroid gland.
Therefore, the American Academy of
Pediatrics30 recommends routine thyroid
screening early in life.
Hypothyroidism occurs in up to 30%
of people with DS.14 Knowledge is still
scarce regarding the etiology of this con-
dition. Adults with DS are more likely to
develop autoimmune thyroiditis associ-
ated with mild hypothyroidism. The risk
of developing hypothyroid autoimmune
thyroiditis increases with age, although
hypothyroidism may occur in the
Abanto et al.
absence of autoimmune thyroid, indicat-
ing a heterogenic cause for this
Dia betes mellitus
An increased incidence of diabetes melli-
disease.37,38 The patient with hypothy-
tus has been reported in patients with DS
roidism may present with coarse facial
who are between the ages of 24 and
features (thick lips, puffy eyelids, sad
34 years (1.4% to 10.6%).41 The phospho-
expression), dry hair, and dry and cold
fructokinase gene (PKF) located in the
skin.39 Also, the heart rate, myocardial
21q22.3 region is related to diabetes melli-
contractility, blood pressure, cardiac
tus and obesity in persons with DS.40,41 In
output, and the rate of respiration are
these individuals, a variety of alterations
decreased in patients with hypothy-
in lipid metabolism also may occur.40 Oral
roidism.39
manifestations of diabetes include xeros-
Thus, we emphasize that dentists
tomia, burning mouth/tongue, candidal
need to be able to identify signs and
infection, progressive periodontitis, oral
symptoms of this disorder and make sure
neuropathies, parotid enlargement, sialo-
that the patient receives proper medical
sis, and delayed wound healing.12,43
care before any dental procedures are
Dental management is aimed at imple-
done. Well-controlled, medically super-
menting a preventive protocol,
vised patients on thyroid replacement
symptomatic relief of any oral manifesta-
therapy and patients with mild to moder-
tions of the disease, and immediate
ate symptoms of hypothyroidism may
provision of primary care to treat dental
safely undergo routine dental care under
pain and established dental infections.
local anesthesia.39 However, patients with
Oral infections may increase insulin resist-
hypothyroidism are hyperactive to central
ance, impair the glycemic control of blood
nervous system depressants (opioid anal-
sugars and lead to a more uncontrolled
gesics, anxiolytic agents), which therefore
diabetic state.12,43 It is thus imperative to
should be administered judiciously.39
discuss any oral findings with the physi-
In patients with severe hypothy-
cian responsible for the patient’s care.42
roidism, central nervous system
Patients with well-controlled diabetes can
depressants, sedatives, or narcotic anal-
be treated in the same way as healthy
gesics must be avoided or prescribed in a
patients.43
lower dosage, because major respiratory
depression may occur.37 For this reason,
therapy with these types of drugs should
be administered cautiously and initiated
Conclusions
The oral treatment of patients with DS
at reduced dosages in individuals with
should always be based on concepts of
uncontrolled hypothyroidism.
general health. Even though individuals
Subsequent doses should be titrated
with DS are intellectually impaired and
based on individual response rather than
can present as medically complex, the
a fixed dosing schedule.37 Opioid anal-
gesics are primarily employed for pain
majority of general dental clinicians
should be able to treat them in their pri-
relief, and in general are combined with
vate practices. For this reason, dentists
nonopioids for maximum pain relief in
should be aware and knowledgeable
dental situations. Antianxiety drugs are
about identifying the patient’s medical
used in clinical dentistry primarily for
problems, to control or treat these under-
premedication of nervous and apprehen-
lying conditions, or to refer the
sive patients. The most widely used are
individuals to physicians. Ultimately, the
benzodiazepines, which affect the central
goal is to manage patients with DS so that
nervous system and are respiratory
comprehensive and regular dental care is
depressants. In normal doses, the benzo-
provided for this underserved population.
diazepines have little effect on
respiration in healthy people. The den-
tist’s primary concern in using an
antianxiety agent in individuals with
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