DOWN SYNDROME cystectomy (intact mucous coating with a normal

appearance)

Clinical Scenario ● Unstable upper partial denture

A 48-year-old patient attends the dental clinic complaining that

his removable upper partial denture is unstable and “does not Radiographic Examination

work when eating”. The patient has been given several dentures ● Orthopantogram and cone beam computed tomography

in recent years but none of them has been successful. undertaken

● No radiological evidence of recurrence of the

Medical History odontogenic cyst, but there is loss of bone mineral

● Down syndrome density

● Atrial septal defect corrected in childhood ● The only available bone volume for direct insertion of

● Recurrent respiratory infections dental implants identified in positions corresponding to

● Mild hearing loss teeth #14 and #26

● Gastroesophageal reflux
● Chronic anemia
● Hyperuricemia

Medications
● Budesonide
[a] Irregular palate, with erythema of the denture bearing
● Theophylline
mucosa (clinically suggestive of candidiasis). [b] unstable upper
● Allopurinol
partial denture
● Lansoprazole
● Iron and folic acid
Structured Learning
1. What are lip fissures and what causes them?
Dental History
a. Lip fissures are a frequent finding in patients with
● Regular dental attender
Down Syndrome (>25%), especially among men,
● Previous dental treatment with local anesthesia tolerated
with a peak prevalence in the third decade, and
on numerous occasions without the need for
occur preferentially in the lower lip
pharmacological adjuncts (calculus removal, extraction,
b. Their etiology is unknown (embryological defects,
fillings, endodontics, non-surgical periodontal treatment
mandibular prognathism and lip eversion have been
and prosthetic rehabilitation)
implicated)
● Three years ago, the patient underwent excision of a
c. In most patients, the lesions:
maxillary odontogenic cyst (5x2.5 cm) under general
i. Coexist with angular cheilitis
anesthesia
ii. Are colonized by Candida albicans
● The patient brushes his teeth independently twice a day
using a fluoride toothpaste (without supervision)
2. What factors are considered important in assessing the
risks of managing this patient?
Social History
a. Social
● Patents deceased; lives with one of his sisters
i. Favorable family environment
● Independent for activities of daily life
ii. Hearing impairment
● Attends a center where he participates in cognitive
iii. Complications can arise due to other
simulation and craft workshops
comorbidities associated with Down
Syndrome (e.g. premature aging and
Oral Examination
cognitive impairment)
● Very cooperative
b. Medical
● Lip fissures
i. Respiratory dysfunction
● Fissured tongue
ii. fatigue/reduced tolerance for treatment in
● Fair oral hygiene
relation to anemia
● Microdontia
iii. Corrected atrial septal defect is not
● Dental spacing
associated with risk when delivering dental
● Missing maxillary teeth: #14, #15, #16, #17, #21, #22,
intervention
#23, #24, #25 and #26
c. Dental
● The remaining maxillary teeth have significant gingival
i. Multiple failed attempts at providing
recession with cervical exposure and grade 1-2 mobility
removable partial dentures
● Missing mandibular teeth: #32, #35, #42, and #45
ii. Oral hygiene could be improved
(possibly due to agenesis, based on the findings of
iii. Multiple missing teeth but low caries rate;
previous radiographs)
chronic periodontal disease likely cause of
● Irregular alveolar bone crest in the upper left quadrant,
tooth loss
with a considerable bone defect as sequela of the
iv. Prognosis of the remaining teeth guarded
 v. Implication for success of osseointegrated ● Lingual protrusion
dental implants ● Fissures tongue
vi. Gastroesophageal reflux-related risk of dental ● Increased incidence of gag reflex
erosion ● Dental agenesis (especially of the maxillary lateral
vii. Anemia-related oral side-effects (pale incisors)
mucosa, glossitis, oral ulceration) ● Tooth eruption delayed
● Tooth morphology abnormalities (microdontia, enamel
3. What factors determine the prognosis of the dental implants hypoplasia and hypocalcification)
in this patient? ● Low prevalence of caries
a. The available bone volume is limited ● Severe periodontal disease
b. Osteopenia ● Early tooth loss (compromised immune system and teeth
c. Susceptibility to infections (potential defects in with short conical roots lead to early loss of teeth from
neutrophil chemotaxis due to Down Syndrome) periodontal disease)
d. Suboptimal oral hygiene and a history of periodontal ● Bruxism
disease can favor the onset of peri-implantitis ● Hypoplasia of the middle third of the face, with Angle class
i. Ongoing oral hygiene/periodontal support III malocclusion and posterior cross-bite
provided due to variable compliance
e. Observed higher failure rate; in patients with Down
Syndrome, 1 in every 5 dental implants fails
4. If considering the use of dental premedication/sedation to
place dental implants, what additional factors should be
taken into account?
a. Benzodiazepines should not be prescribed for
patients with severe respiratory dysfunction or
hypotonia (musculoskeletal effect of Down
Syndrome)
b. Theophylline reverses the sedative effect of
Benzodiazepines
5. Is administering antibiotic prophylaxis before a surgical
procedure such as implant insertion justified?
Dental Management
a. The corrected atrial septal defect does not justify the
● The treatment plan will be determined by the patient’s
prescription of antibiotic prophylaxis for the
manifestations, degree of co-operation and the presence
prevention of bacterial endocarditis
of comorbidities
b. However, the immunological defects observed in
● Orthodontic therapy can be performed in selected cases
Down Syndrome may:
i. Constitute an indication for administering
Considerations for Dental Management
antibiotics prior to surgical procedure; and
ii. For maintaining them in the postoperative Risk ● Postoperative infections (immunological
Assessment
period deficiencies)
● Risk of bacterial endocarditis (congenital
heart disease
● Variable co-operation
● Speech disorders and intelligibility
● Hearing loss
● Atlantoaxial instability

Criteria for ● Most patients can be treated in a

Referral
conventional dental clinic
● Referral to a specialized clinic or hospital
center is determined mainly by the patient’s
cooperation and extent of comorbidities
6. What antibiotics should be avoided for this patient?
(e.g. severe heart disease)
a. The toxicity of Theophylline increases with macrolide
antibiotics and quinolones Access/ ● Prevent neck hypertension (atlantoaxial
Position
instability)
General Dental Considerations ● Access to the teeth may be impeded by
Oral Findings lingual protrusion
● Orofacial muscle hypotonia ● Minimize waiting time
● Poor labial seal/ open mouth posture (may lead to ● Consider shorter sessions
xerostomia)
● Lip fissures Communication ● Adapt in relation to cognition
● Macroglossia or pseudomacroglossia
 ● If hearing aids are worn, ensure these are During
present and switched on at the dental ● Consider the use of tongue guards,
appointments supplemented by high volume suction to
improve vision/ access to the dentition
Consent/ ● Capacity assessment is required (should
Capacity
be decision specific) After
● Discussion of risks should include those ● Orthodontic treatments, with both
related to comorbidities (e.g. predisposition removable and fixed multibracket
to infections) and the level of oral hygiene appliances, usually take longer than in the
● Dementia may occur at an early age and general population (slow activation rhythm)
have an additional impact on reducing and complications are more frequent
capacity (particularly traumatic ulcers)
● The prognosis for dental implants in these
Anesthesia/ ● Local anesthesia
Sedation patients is poorer than in the general
○ This may be challenging to
population, with an estimated failure rate of
administer in relation to patient
20% (generally, the losers occur before
cooperation, altered anatomy and
completing the prosthetic rehabilitation)
lingual protrusion
● Sedation Drug ● Consider drug interactions with
○ The nasal hood used for inhalation Prescription
medications used to treat comorbidities
sedation may not fit well due to (e.g. avoid macrolide antibiotics for patients
hypoplasia of mid-third of the face taking antihypertensive such as
○ Associated comorbidities should be verapamil/diltiazem)
assessed, including the degree of
hypotonia, associated cardiac Education/ ● Oral hygiene education
Prevention
disease and respiratory dysfunction ● Involve the relatives and care-givers
● General Anesthesia ● Increased frequency of reviews (every 3
○ Difficulties in endotracheal intubation months) to closely monitor periodontal
(hypoplasia of the middle third of the disease
face, short neck, adenoid ● Periodic calculus removal
hypertrophy, atlantoaxial subluxation) ● Dietary counseling
○ Increased risk due to cardiac
complications (due to underlying
Background Information and Guidelines
heart disease and/or anemia),
Definition
respiratory dysfunction and infections
Down syndrome is a congenital disorder of chromosomal origin
(increased susceptibility)
characterized by intellectual disability, systemic abnormalities
and a particular phenotype, The essential prevalence is 1 case
per 800 live births.
Dental Before
Treatment
● Early periodontal treatment and the use of
Aetiopathogenesis
adjuvant antimicrobial mouthwashes are
● .The mother’s age (>35 years) is considered a risk factor
effective in improving periodontal health
● In 95% of cases, the syndrome is due to presence of an
● Pulpal treatments in primary dentition are
additional copy of chromosome 21 in all cells (‘trisonomy21’)
not recommended
● In 5% of the remaining cases, the syndrome expresses
● The prognosis for orthodontic therapy is
translocation or mosaicism
determined by the patient’s degree of
collaboration, the level of oral hygiene the
presence of parafunctions and the state of
the periodontium
● Rehabilitation with fixed prosthesis can be
performed if the oral hygiene is optimal, the
dental morphology is appropriate and the
periodontal state is acceptable; otherwise,
opt for a removable prosthesis (not always Clinical Presentation
well accepted by patients) ● Apart from the classic facial characteristics (80%), multiple
● Stimulating palatal plaques combined with systems may be affected in Down syndrome
orofacial physical therapy and speech
therapy exercises i proves muscle tone and Diagnosis
orofacial abnormalities ● Suspected prenatal diagnosis (imaging techniques and
invasive tests) is confirmed with the study for foetal cell DNA
 ● In newborns, the diagnosis is suspected based on the
Endocrine ● Hypothyroidism (~15%)
phenotypic characteristics and is confirmed with karyotyping ● Increased incidence diabetes
● Decreased fertility

Management Reproduction ● Women with Down syndrome are

● Physical therapy to combat hypotonia fertile and may become pregnant
● Nearly all males with Down syndrome
● Early stimulation programs (including speech therapy) are infertile due to impairment of
● Treatment of comorbidities spermatogenesis

● Special education and occupational therapy Neuropsychiatric ● Intellectual disability (100%)

disorders ● Average prevalence of dementia 50%
(7-50%); risk increase when the person
Prognosis is over the age of 35
● Median age of death has increased from 25 years in the ● Tonic-clonic seizures
● Psychiatric disorders:
1980s to 55 years, with many living into their 60s and 70s ○ Obsessive-compulsive disorder
● Mortality by infections diseases, especially pneumonia, is ○ Autism
○ Attention deficit hyperactivity
12-fold greater than in general population disorder
○ Tpurette syndrome
○ Depressive disorder
A World/Transcultural View
● There is racial disparity in the mortality of patients with Down Skeletal ● Short stature (85%)
● Increased joint flexibility (80%)
Syndrome; this may be related to healthcare access, for ● Spine
example early referral to cardiology to allow timely surgical ○ Atlantaoaxial instability (14%),
with excessive mobility of the
intervention atlas (CI) and axis (C2); may lead
● Social acceptance of the phenotypic appearance is variable; to subluxation of the cervical
spine/ spinal cord compression
parents surveyed in sub- Saharan Africa demonstrated ○ Pelvic dysplasia (70%)
favorable attitudes towards plastic surgery for their children ● Skull
○ Brachycephaly (80%),
with Down Syndrome, although they admitted their lack of microcephaly, sloping forehead
knowledge about the procedure ○ Large fontanelles with late
closure, patent metopic suture
● Even after several decades, the use of orofacial stimulation ○ Absence of frontal and sphenoid
therapy has not become widespread and is applied on a sinuses
○ Hypoplasia of maxillary sinuses,
regular basis only in a number of South American and hypoplastic midface with relative
northern European countries prognathia
● Nose
○ Hypoplastic nasal bone and flat
Most common systemic conditions in Down Syndrome nasal bridge are typical
characteristics
Systems Conditions ● Hands
○ Short and broad hands
Cardiac ● Congenital heart defects are common ○ Clindactylyl of the fifth fingers
(40-50%) (45%)
● Endocardial cushion defect (43%) ○ ‘Simian’ single flexion crease
● Ventriculoseptal defect (32%) (20-40%)
● Secundum atrial spetal defect (10%) ● Feet
● Tetralogy of Fallot (6%) ○ Wide gap between 1st and 2nd
● Isolated patent ductus arteriosus (4%) toes

● The risk of leukemia is 1-1.5%, much

Hematological higher than the general population Muscles ● Hypotonia
(10-15 times increased risk)
● 65% of newborns have transient Eyes ● Up slanting palpebral fissures, bilateral
myelodysplasia epicanthal folds
● Brushfield spots (35-90%)
Immunological ● Abnormal IgA levels ● Refractory error (35-76%)
● Abnormal T-cell function ● Strabismus (25-57%)
● Dysfunctional and short-lived ● Nystagmus (18-22%)
neutrophils ● Cataract (5% of newborns)

Infections ● Increased risk (x12) of developing Ears ● Small ears with overfolded helices
infectious diseases, including: ● Hearing loss (75%)
○ Respiratory (pneumonia) ● Otitis media
○ Gastrointestinal ● Increased risk of retinoblastoma
○ Mucosal
○ Dermal Skin ● Psoriasis
○ Oral infections (periodontal ● Eczema
disease, candidal infection, acute ● Palmoplantar hyperkeratosis
necrotizing ulcerative gingivitis) ● Seborrheic dermatitis

Gastrointestinal ● Gastro-oesophageal reflux disease Others ● Obstructive sleep apnoea

(GORD) ● Hypotonia
● Vomiting ● Premature ageing
● Duodenal atresia or stenosis. ● Obesity
Associated with annular pancreas in ● Fine, short hair
2.5% of cases
● Oesophageal atresia
● Hirschsprung disease (blockage of
colon)
● Imperforate anus
● Coelia disease (5-16- folf increase
compared to general population)
 Dental Care for Children with Special Needs, p. 123

NUTRITIONAL CONSIDERATIONS FOR OPTIMAL ● Many medications, such as diuretics (e.g. furosemide)
used to treat cardiac conditions,
ORAL HEALTH IN CSHCN
○ Have the potential for causing xerostomia
● Many other medications alter the patient’s ability to taste, so
Introduction
helping them develop and maintain a healthy balanced diet
● Children with special healthcare needs are at increased risk
is essential.
for experiencing nutritional complications that can pose
● Long-term effects of medications
great challenges to maintaining a healthy body and mouth
○ Best example of the dramatic lifetime effects of
● The sources of difficulties are numerous
medication treatment: chemotherapy treatment for
○ Child’s physiological condition itself
pediatric cancer
○ Medications and/or diet prescribed to treat the health
● Chemotherapy has several immediate & long-term effects
condition
on the oral cavity
○ Behavioral and psychosocial approaches toward
○ Mucositis, xerostomia, stomatitis, bleeding, infection,
feeding in general
and ulcerative lesions
● Children with disabilities and chronic conditions
○ Long-term dental malformations: tooth agenesis or
○ May have decreased ability to consume adequate
malformation, root malformation, malocclusion, and
nutrients
salivary malfunction
■ Oral motor dysfunction
■ Cleft lip/palate
○ Inability to utilize adequate calories
■ Gastroesophageal reflux
○ Increased caloric requirement
■ Hyperthyroidism
● Many CSHCNs take medications in order to manage their
medical conditions, which can negatively affect the child’s
dentition & periodontium
● CSHCNs are often on a selective, limited diet, either
○ Prescribed by the care provider; or
■ E.g. ketogenic diet for seizure disorder
○ Dictated by the child
■ E.g. sensory processing dysfunction
● As a dental provider, treatment may bring additional
challenges to maintaining oral health
○ Thus, as key members of the medical team, dental ● For a patient that has been treated with chemotherapeutic
providers are in a prime position to help the child medicaments, long-term effects may last a lifetime
maintain her optimal dentition and periodontium ● Oral medications are often delivered to children in liquid
suspension
Medications and Their Effects on the Dentition ○ Due to young age and inability to swallow tablets
● Medications are common in the lives of CSHCN ○ Oral liquid medications are typically sweetened with
○ They are necessary but pose multiple threats to their sucrose in order to decrease the bitter taste of the
oral health medication and increase compliance in taking them
● Both the medication itself and the vehicle by which the as directed
medicine is delivered may have potential negative side ■ Contain varying amounts of sucrose, up to 4
effects g/5 mL — it is important to explain to the
● Many CSHCNs receive medications for more than patient and caregiver that exposure to the
comorbidity, resulting in a complex drug regimen and sugar-sweetened medication, which is often
multiple potential effects several times per day, increases the child’s risk
● CSHCNs with systemic conditions and behavioral for the development of caries
diagnoses are typically treated with established classes of ● Children also receive medications via the inhalation
medications delivery route
● Many medications have negative effects on: ○ Inhaled corticosteroids can cause:
○ Gingiva ■ Stomatitis
○ Salivary gland function ■ Alteration of the oral flora resulting in
○ Taste receptors candidiasis and xerostomia
○ Oral microbiology Limited Diets
● When patients experience such changes in their oral cavity, ● A CSHCN is often on a selective, limited diet that makes
their nutritional status may be affected obtaining proper nutrition difficult
○ They may avoid certain types of food due to: ● Elimination diets
■ Difficulty chewing or swallowing; or ○ Reduce exposure to key food classes in an attempt
■ Because the food doesn't taste good to manage the symptoms of some systemic diseases

■ E.g. ketogenic diet for seizure disorder
● These limited diets are either prescribed by the child’s care
provider or may be implemented by the caregiver without
professional recommendation
● 4 of the most common elimination diets include:
○ Ketogenic diet
○ Gluten-free and/or casein-free diet
○ Food additives exclusion diet
○ Oligoantigenic diet
I. KETOGENIC DIET
● A non-pharmacological treatment for seizure disorders
● It is often used in either combination with anticonvulsants or
when anticonvulsants are not successful
● Has been demonstrated to be effective in treating several
types of seizure disorders, including
○ Intractable epilepsy
○ Tuberous sclerosis complex
○ Rett syndrome
○ Lennox-Gastaut syndrome
○ Lissencephaly
○ Myoclonic-astatic epilepsy
○ Mitochondrial disorders
● A potential treatment option for other neurological disorders
such as
○ Brain tumors
○ Migraines
○ Autism Spectrum Disorder (ASD)
● While successful, its restrictive nature gives rise to
nutritional challenges, as well as difficulty with compliance
and long-term maintenance for the patient and the
caregivers
● Classic ketogenic diet uses a ratio of 4:1 of total energy
from fat to combined protein and carbohydrate
○ Been thought as unpalatable, so several modified
diets including the modified Atkins diet, medium
chain triglyceride diet, and the low glycemic index
diet have been demonstrated to also be effective in
treatment of seizures
● These diets still limit specific types of carbohydrates but
have a lower ratio of fat to combined protein and
carbohydrate
○ As a result, they are often better tolerated by the
patients
● It is theorized that the ketogenic diets generate large
quantities of ketone bodies, effectively inducing a metabolic
state of ketosis
○ Ketone bodies interact with multiple molecular
targets in the brain, influencing brain
hyperexcitability & hypersynchrony
● All ketogenic diets have several possible adverse effects
○ Vitamin deficiency
○ Growth inhibition
○ Gastroesophageal reflux
○ Constipation
○ Kidney stones
○ Cardiovascular complications
● Patients following a ketogenic diet require careful meal
planning and vitamin and mineral supplement
Dental Care for Children with Special Needs, p. 123
● A child following a ketogenic diet requires individualized
dental care
● the child’s dentition and gingival health should be evaluated
for signs of vitamin deficiencies such as:
○ Enamel defects and vitamin D deficiency; or
○ Bleeding gingiva and vitamin C deficiency
● The provider should also evaluate the tooth structure for
wear facets characteristic of gastroesophageal reflux
● It is critical that dental providers take care not to upset the
patient’s state of ketosis
○ Ex: provider should not prescribe liquid suspension
pediatric medications that are high in sucrose
■ It is essential to consult with the patient’s
physician or nutritionist that is overseeing the
ketogenic regimen
○ Avoid use of dental prophylaxis paste and fluoride
varnish that may contain sweeteners
● One advantage of the ketogenic diet is that it is low in
carbohydrates = not cariogenic in nature
○ The child following it should be at a low caries risk in
regard to her food intake
II. GLUTEN-FREE and/or CASEIN-FREE DIET
● Food allergies are estimated to be present in 5% of the
adult and 8% of the pediatric populations worldwide
● Celiac disease
○ An autoimmune disorder in which gluten, a mixture
of proteins isolated from wheat, barley, oats, or rye
causes an immune response in the small intestine
○ Is estimated to be present in 1% of people worldwide
● Casein
○ A protein commonly found in cow’s milk and dairy
products
● Gluten-free and/or Casein-free diets (GFCF) are often
prescribed to treat people with food sensitivities or allergies
to gluten and casein
● GFCF diets are being studied and employed in an attempt
to lessen negative behaviors associated with
neurodevelopmental diagnosis, including:
○ ASD
○ ADHD
○ Bipolar disorder
○ Schizophrenia
○ Psychosis
● The link between each diagnosis and the gastrointestinal
problems associated with gluten and casein are still being
evaluated
● While these diets are not strongly endorsed for treatment of
negative behaviors, caregivers often implement diets in an
attempt to help the child
● The provider who has a patient following one or both of
these diets should consider:
○ Frequent nutritional monitoring to ensure the patient
is receiving adequate fiber, vitamins, and minerals
 Dental Care for Children with Special Needs, p. 123

○ Individuals that have celiac disease and exposure to DSM-5 (Diagnostic and Statistical Manual of Mental
gluten often present enamel developmental Disorders) feeding and eating disorders.
defects & aphthous ulcers ● The DSM-5 includes three feeding disorder diagnoses:
○ pica
III. OTHER ELIMINATION DIETS ○ rumination disorder
● There are many other elimination diets, but perhaps ○ avoidant/restrictive food intake disorder
two of the most common ones are the
○ Food additives exclusion diet I. PICA
○ Oligoantigenic diet ● Defined as the persistent eating of nonnutritive
● The food additives exclusion diet (Feingold diet) was substances for a period of at least 1 month
proposed to eliminate food additives, such as artificial ● The nonfood substances most often described as
flavors, colors, fragrances, sweeteners, and ingested by children with pica include soil, chalk,
preservatives as well as naturally occurring salicylates plaster, clay, paint, paper, cloth, sand, hair, plastic,
● In the oligoantigenic diet, there is typically an initial coal, insects, wood, pebbles, and animal feces
elimination phase with the exclusion of most foods ● The exact cause of pica is unknown, but in some cases
except a limited list of hypoallergenic foods. it may present in people experiencing nutritional
● In both diets, if the person has decreased symptoms, deficiencies (e.g., iron, zinc, and calcium deficiencies).
then foods may be slowly added back in order to ● In such cases, when the deficiency is corrected, the
determine which foods are responsible for the adverse behavior typically resolves.
physical or behavioral reactions. ● Also associated with children who have developmental
● These diets have been used to improve physical disability and neurodevelopmental diagnoses and
response in people with unknown allergies and to thought to be correlated with an inability to use
improve behavior in those with neurodevelopmental appropriate discriminatory behavior.
diagnoses ○ It has been theorized that such disorders are
● Prophylaxis paste, fluoride treatment, and home oral often an attempt to seek comfort and
hygiene products may all be on the exclusion lists for self-soothe due to emotional deprivation
patients following a strict diet ● Ingesting fibrous materials may result in fibers
○ These items should be avoided unless they becoming trapped in the gingiva and may cause
are cleared by the patient’s physician or gingivitis or a periodontal abscess
nutritionist ○ Ingesting hard, abrasive substances may
● The pediatric dental care provider must be aware of cause excessive dental wear and fracture, as
specific requirements related to medically well as potentially causing many other
recommended diets, understand that ingredients health-related issues
found in common dental products may affect the diet, ● If a patient with a diagnosis of pica is seen in a dental
and be prepared to consult with the other members practice, it is best to have minimal loose objects
of the healthcare team where potential concerns exist available for her to pick up and ingest (e.g., gauze,
cups of prophylaxis paste) and to ensure that someone
Feeding Disorders on the dental team stays with her throughout the
● Feeding difficulties may be associated with medical appointment to both supervise and engage with the
conditions, oropharyngeal dysfunction, behavioral child.
diagnoses, as well as environmental and caregiver
factors II. RUMINATION DISORDER
● Physical challenges with feeding such as ● Is a feeding disorder defined as the repeated
gastroesophageal reflux and/or oropharyngeal regurgitation and re-chewing of swallowed or
dysphagia are seen in the vast majority of children with partially digested food
moderate-to-severe developmental disability who have ● The regurgitation occurs daily and must be
feeding difficulties differentiated from a child’s potential for other
● Within the population of CSHCN, several groups of gastroesophageal diagnoses (e.g., gastroesophageal
children are prone to clinically significant feeding reflux) or an eating disorder (e.g., bulimia)
challenges: ● Typically develops prior to age one, but it can present
○ Medical conditions such as premature birth initially at any age.
○ Craniofacial anomalies (e.g., cleft palate) ● It has been proposed that rumination disorder develops
○ Genetic syndromes (e.g., Russell-Silver as a result of a child’s need for stimulation or as the
syndrome) result of stress and anxiety in an attempt to
○ Neurological impairments (e.g., cerebral self-soothe.
palsy, sensorineural deafness, severe vision ○ In infants, the behavior tends to resolve
loss) spontaneously over time.
● When a child’s feeding difficulty actually hinders her ○ In older children, particularly those with
growth and/or emotional development, she may be developmental disability, it may develop into a
classified as having a feeding disorder under the

long-standing habitual behavior as the result
of anxiety or other psychiatric symptoms
● Infants and children with rumination disorder tend to
experience weight loss, malnutrition,
susceptibility, and even death
● Orally, their dentition may present with erosive wear
from the frequent stomach acid exposure, and they
may have red, inflamed mucosal and gingival tissues
III. AVOIDANT / RESTRICTIVE FOOD
DISORDER
● The diagnosis of avoidant/restrictive food intake
disorder (ARFID) describes a feeding disturbance that
causes persistent failure to meet appropriate
nutritional and/or energy needs
● It must be associated with at least one of the following:
○ significant weight loss or limited growth
○ significant nutritional deficiency
○ dependence on enteral feeding or oral
nutritional supplements
○ marked interference with psychosocial
functioning
● Food avoidance based on the sensory characteristics
of food is often termed food selectivity
● Children that present to the dental office with a known
diagnosis of ARFID, sensory processing dysfunction,
and/or oral sensory sensitivity will often also refuse
dental materials, home oral hygiene products, and
even instruments (including the toothbrush) being
placed within their mouths
● Prior to working with this child, it is important to have a
conversation with the child and caregiver about the
child’s ability to tolerate the stimulation of a toothbrush
as well as the various flavors or textures of the
prophylaxis paste
● If a child is able to use specific toothpaste at home,
suggesting that the child bring it to future dental
appointments may be helpful in lowering the child’s
anxiety and improve her ability to tolerate the
experience
DENTAL TREATMENT FOR CHILDREN WITH FEEDING
DISORDERS
● The dental provider will ideally spend time at each
dental visit discussing the child’s dietary habits
● The child’s weight should also be recorded and
monitored at each appointment
○ Because of these conversations
measurements, the pediatric dental provider
may be the first clinician to recognize a
patient’s feeding difficulty
● Anomalies in the results of an oral exam such as poor
gingival health, severe dental attrition, and wear
patterns suggestive of acid erosion should be
addressed and followed closely
● As feeding difficulties are quite prevalent in CSHCNs, it
is important to address feeding habits regularly with
each child and her caregiver
Dental Care for Children with Special Needs, p. 123
● When feeding disorder is suspected, it is important to
work with a pediatrician to obtain resources for
assessment and treatment
infection
Management of Nutritional Challenges
● A child’s ability to feed is a by-product of her
physiologic, motor, and organizational abilities, as well
as her environment and caregiver attributes
● CSHCNs are at risk for developing feeding disorders
INTAKE ● Disruption around feeding can be Nutritional
Considerations for Optimal Oral Health in CSHCN 132
detrimental to the development of certain children,
especially when such disruptions consistently interfere
with the nutritional intake
● Treatment for a child with feeding disorder will likely be
a multidisciplinary approach
○ May include pediatrician, feeding specialist,
OT, behavioral therapist, gastroenterologist,
social worker, SLP, development pedia, and
nutritionist.
● Dietary management of oral motor and behavior
feeding disorders may involve frequent feeding with
calorie-dense formulas and cereals.
○ Should be supported by the dental provider
regardless of its cariogenic nature, as the
highest priority is for the child to grown and
develop appropriately
● Feeding modifications to consider:
○ If the child’s behavior modi!cation training
involves treats, the dental provider should
suggest substituting highly cariogenic rewards
(e.g., candies) with foods that are less likely to
contribute to decay (e.g., sugar-free
alternatives)
○ This child will benefit from stressing the
importance of a good home oral hygiene
program as well as more frequent recall
examinations (every 3–4 months).
○ A child that is nutritionally deficient may also
have frequent feedings with calorie-dense
formulas and cereals putting the child at high
caries risk.
○ This child may also benefit from stressing the
importance of a good home oral hygiene
program as well as more frequent recall
examinations (every 3–4 months)
○ Consider adaptive positioning of the child and
and utensils while feeding. These same positions
may facilitate home hygiene
○ Children that avoid flavors or textures of foods
may also be averse to toothpaste. Consider
recommending brushing the teeth with just
water or, if tolerated, dipping the toothbrush in
a fluoride-containing mouthrinse and brushing
○ When children exhibit oral signs of
gastroesophageal reflux, the dental provider
should discuss the option of prescription
medication with the medical team

Feeding Tubes
● When oral feeding is considered unsafe, the child may
be fed through a feeding tube and or undergo surgery
● Enteral tube feeding can be used as a supplemental or
total replacement method for oral eating
○ Nasogastric (NG) tubes
○ Orogastric (OG) tubes
○ Duodenal tubes
○ Gastronomy (G) tubes
○ Jejunostomy (J) tubes
(Gastronomy tube)
● NG, OG, and duodenal tubes have the advantage of
nonsurgical placement and are usually prescribed for
short-term use
● G and J tubes require surgical placement
○ They bypass the mouth and nose, so they
may result in decreased sensitivity for a child
with aversions to contact of her face and head
● Nutrient-rich formula is provided through the tubes or
either continuously or in boluses
● If the child is able to safely eat orally, an oral feeding
plan will be maintained in conjunction with tube feeding
● Minimize breaks in oral feeding and stimulation
● Depending on the reason for feeding dysfunction the
goal may be to transition to oral eating exclusively or to
be nutritionally maintained via tube feeding long term
● A child that receives the bulk of her nutrition via tube
feeding may still have frequent oral “social tastings,”
during which the child is exposed to foods she finds
palatable
○ May include components of the family meals
○ Food that may have minimal nutritional value
and highly cariogenic
■ Ice cream, pudding, crackers
○ Tasting provides oral stimulation to various
food tastes and textures, as well
socialization with family at mealtime
○ Child may or may not swallow the food
presented
○ If the ultimate goal of the treatment is to
transition to oral eating, oral exposure to food
(e.g., nutrient-dense formula and other foods)
will increase over time in volume and
frequency
○ Caries risk may increase depending on
tasting frequency and whether or not the child
swallows the food or holds it in her mouth
Dental Care for Children with Special Needs, p. 123
● Children with feeding dysfunction due to
gastroesophageal reflux may undergo Nissen
fundoplication
(Nissen fundoplication)
○ A surgical procedure in which the gastric
fundus is wrapped around the esophagus to
tighten the lower esophageal sphincter in
order to prevent the reflux of gastric contents
○ Nissen procedure is often combined with
G-tube replacement
● A child treated for feeding disorder is a challenge to the
dental team, as it is prioritized for the child to gain
weight, it is important to encourage the family to follow
the feeding treatment plan even if it includes frequent
oral feedings or tastings with cariogenic food
○ Essential to educate them about the
cariogenic nature of the child’s nutritional
source
○ Caregivers should be counseled to brush,
rinse, or swab the teeth of the child
○ If a child obtains most/all of her nutrition
through a feeding tube, she will be prone to
develop gingivitis as well as calculus on all
surfaces of the teeth
○ Clinician may recommend frequent dental
prophylaxis to limit the amount of calculus that
accumulates between appointments
● Before proceeding with any in-office treatment, the
patient’s ability to safely tolerate dental scaling and
prophylaxis should be determined
● A medical consult with the child’s care providers may
be indicated
● Caution should be exercised for any child that has
been hospitalized for aspiration pneumonia or is
unable to cough and clear her airway
● During scaling or ultrasonic cleaning, special care
should be made to minimize the chance for aspiration.
as ○ The patient should be positioned in an upright
position during the procedure
○ If the child is receiving protective stabilization
(passively or active), it is important to pay
attention to the feeding tube’s position so as
not to disturb or remove it
● It is very important though to discuss with the child and
her caregiver the child’s increased risk for the
development of dental caries and the need for
excellent home oral hygiene and possibly more
frequent recall exams (e.g., every 3–4 months)
 Dental Care for Children with Special Needs, p. 123

Overweight/Obesity
● Overweight and obese children may still be nutritionally
deficient due to the limited nutrients contained in their
selected foods
● Important to monitor the oral cavity for poor gingival
health and dental caries
● Weight and height measurements and BMI should be
calculated for all children at their examination
appointments
● As many of the calorie-dense foods are also often
cariogenic, dietary counseling may address both
issues
○ Overweight/obesity adds a significant
challenge for sedation
○ It is prudent to treat a child that is overweight
or obese under general anesthesia in a
hospital setting instead of employing in-office
sedation
Conclusions
● CSHCNs are at increased risk for experiencing
nutritional challenges due to their complex medical
conditions and associated treatments
● Signs of a child’s medical or behavioral conditions and
associated treatments are often evident in the dentition
and/or periodontium
● The dental professional is in a key position to
recognize that a child is displaying signs for nutritional
deficiencies and/or feeding difficulties and should
recommend further evaluation when indicated
● CSHCNs who have nutritional complications due to
their medical and/or behavior diagnoses, medication
and dietary treatments, and altered feeding experience
require careful, individualized treatment plans in order
to best meet their specific needs