8 - Special Needs Patients Flashcards

1
Q

What intellectual ability do patients with Trisomy 21 have?

A

Most individuals with Trisomy 21 function in the range of mild to moderate intellectual disability.

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2
Q

What are the craniofacial characteristics of Trisomy 21?

A
  1. Hypoplastic midface and maxilla
  2. Mild microcephaly
  3. Upslanting palpebral fissures
  4. Short neck
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3
Q

What health conditions are frequently seen in individuals with Trisomy 21?

A
  1. Congenital heart disease
  2. Hypotonia
  3. Compromised immune function
  4. Hearing problems
  5. Thyroid dysfunction
  6. Skeletal abnormalities - cervical spine instability
  7. Eye problems, especially cataracts
  8. Increased risk for development of leukemia
  9. Early development of senile dementia
  10. Cognitive impairment
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4
Q

What is the frequency of congenital cardiac defects in individuals with Trisomy 21?

A

40% to 50%

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5
Q

What questions should you ask in the medical history of a patient with Trisomy 21?

A
  1. History of cardiac problems
  2. Symptoms of cardiac problems
  3. Any surgeries for cardiac problems
  4. Medications for cardiac problems
  5. Restrictions on activities
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6
Q

What are the oral-facial abnormalities and dental anomalies in individuals with Trisomy 21?

A
  1. Maxillary hypoplasia - class III malocclusion, crowding of maxillary dentition
  2. Relative macroglossia due to the small size of the oral cavity
  3. Delayed dental eruption
  4. Hypodontia
  5. Microdontia and other anatomic anomalies of teeth
  6. Ectopic eruption and impaction of teeth
  7. Increased risk for periodontal disease (due to compromised immune response)
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7
Q

What anatomic variations/medical conditions of patients with Trisomy 21 place the patients at increased risk for complications associated with sedation and general anesthesia?

A
  1. Congenital heart defects
  2. Small naso-pharyngeal complex
  3. Increased incidence of airway anomalies, including laryngomalacia, tracheomalacia, bronchomalacia
  4. Increased incidence of cervical spine instability
  5. Obesity
  6. Hypotonia
  7. Obstructive apnea
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8
Q

Describe the periodontal disease in pts with Trisomy 21?

A
  1. Onset of periodontal disease is frequently seen by the late teen years.
  2. Incidence in some adult populations has been reported to be over 90%.
  3. Periodontal disease is probably due to the reduced immune response, which places them at increased susceptibility for chronic infectious processes.
  4. Early, aggressive therapy, including frequent cleanings and possible systemic antibiotic therapy, should be considered.
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9
Q

Describe the correction of malocclusion, ectopic eruption and impactions in pts with Trisomy 21?

A
  1. The class III malocclusion seen in Trisomy 21 involves midface hypoplasia, and would, in most cases, require extensive surgical procedures to correct.
  2. Patients should be monitored for dental crowding, ectopia, and impactions. These problems may be correctable with orthodontic and minor oral surgical treatment.
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10
Q

What is the comprehensive treatment plan for pts with Trisomy 21?

A
  1. Establish a dental home
  2. Dental prophylaxis and topical fluoride
  3. Encourage parent to reduce sugared beverage intake
  4. Encourage improved oral hygiene, (in this case, with parent) focusing on brushing and flossing.
  5. Three-month recall
    - -Re-evaluate caries risk
    - -Re-evaluate oral hygiene status
    - -Monitor eruption of permanent dentition
    - -Re-evaluate pt’s ability to cooperate for radiographic exam. If cooperative, take radiographs to evaluate for interproximal caries and development of permanent dentition.
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11
Q

Describe the behavioral capabilities of pts with Trisomy 21 for dental treatment?

A

There is a wide range of behaviors in children with Trisomy 21, and many of them are treatable for routine dental care with little or no modifications in care necessary.

These children are at an increased risk for complications related to sedation and general anesthesia, and careful consideration must be made for each individual as to whether or not an anesthetic procedure is warranted for routine diagnostic and dental procedures.

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12
Q

List four characteristic craniofacial features of individuals with Trisomy 21?

A
  1. Hypoplastic midface
  2. Mild microcephaly
  3. Upslanted palpebral fissures
  4. Short neck
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13
Q

What is bronchopulmonary dysplasia?

A

Bronchopulmonary dysplasia is a common complication of preterm birth. It is related to the need for positive pressure ventilation (PPV) in neonates with poorly developed pulmonary systems. PPV can result in injury to the pulmonary microvasculature and alveolar structures.

Neonates with bronchopulmonary dysplasia are at increased risk for abnormal pulmonary function into late childhood and possibly adulthood.

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14
Q

How many weeks premature are patients with bronchopulmonary dysplasia born?

A

Bronchopulmonary dysplasia is seen most frequently in infants born prior to 30 weeks gestational age and weighing

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15
Q

What medical and developmental conditions are children with extreme low birth weight at risk for?

A

Children who are born at extremely low birth weights (

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16
Q

What oral/perioral complications are sometimes seen in patients with cerebral palsy?

A

These are all caused by hypotonia (low muscle tone) of the perioral musculature:

  1. Anterior open bite - caused by hypotonia of the tongue and perioral musculature, immature swallow pattern with characteristic tongue thrust.
  2. Constricted/narrow palate - caused by hypotonia of the tongue and perioral musculature.
  3. Persistent drooling - caused by immature/poor swallow reflex.
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17
Q

What can you do to maintain oral health in patients with cerebral palsy?

A
  1. Gingival inflammation:
    - -Stress to parent the importance of daily oral home care. Instruct the parent to help with child’s oral hygiene.
    - -Schedule recall visits at more regular intervals to assess home care, perform prophylaxis and monitor gingival inflammation.
  2. Malocclusion: conduct orthodontic evaluation in early mixed dentition - assess tooth size, shape, position, space analysis.
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18
Q

True or False: Cerebral palsy is a progressive neurologic disorder?

A

False. Cerebral palsy is sometimes termed a “static encephalopathy.” The original lesion in the brain does not progress or result in further deterioration to the central nervous system. Be aware, however, that the clinical presentation of CP, especially muscle function, can change as an individual matures and ages.

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19
Q

What is cerebral palsy?

A
  1. A group of disorders of the development of movement and posture causing activity limitations that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain.
  2. The motor disturbances of cerebral palsy are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behavior and/or a seizure disorder.
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20
Q

What is congenital adrenal hyperplasia?

A

Congenital adrenal hyperplasia is a term used for several autosomal recessive disorders, all of which exhibit defects in aldosterone synthesis, cortisol synthesis, or both.

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21
Q

What clinical findings are seen in congenital adrenal hyperplasia?

A

The resulting cortisol and/or aldosterone deficiencies can result in different clinical findings in females and males:

  1. Females - virilization, with ambiguous genitalia at birth, accelerated skeletal maturity, hirsutism, oligomenorrhea, infertility, inability to maintain sodium balance (“salt-wasting”), hypertension.
  2. Males - Often not diagnosed as a neonate bc of the normal appearance of the genitalia. May present at several months of age due to failure to thrive, vomiting, dehydration, hypotension, hyponatremia, hyperkalemia. Later in childhood, these males may exhibit precocious puberty and accelerated skeletal maturity.
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22
Q

What consideration should you have for patients who have been on long-term corticosteroid therapy?

A

Patients who have been on long-term corticosteroid therapy may be unable to cope with stressful situations (such as a surgical or dental procedure). It is advisable to consult with the patient’s physician to determine current dosages of corticosteroids, explain the planned treatment to the physician, and determine if steroid supplementation is advisable prior to and after any procedures.

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23
Q

What are the symptoms of adrenal crisis?

A
  1. Headache
  2. Weakness
  3. Nausea, vomiting
  4. Confusion
  5. Rapid heart rate
  6. Excessive sweating - sweaty face, palms of hands
  7. Low blood pressure
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24
Q

What is the treatment for adrenal crisis?

A
  1. IV or IM injection of hydrocortisone
  2. Supportive treatment of low blood pressure with IV fluids
  3. Lack of timely treatment may result in shock
25
Q

What do you do when a family member helps with the radiographic exam?

A

When a family member is asked to help with a radiographic exam, that person must be appropriately shielded to minimize radiation exposure. Placement of the film in the patient’s mouth should be done by the oral health provider, with the family member being shown carefully how to maintain that placement.

26
Q

What class of medications is used to manage the clinical manifestations of congenital adrenal hyperplasia?

A

Corticosteroids, both glucocorticoids and mineralocorticoids.

27
Q

Why is outpatient oral sedation not an appropriate management for a patient with congenital adrenal hyperplasia?

A

Children receiving oral sedative medications are generally kept NPO for a number of hours prior to the procedure. This could result in dehydration, which can be a precipitating factor for adrenal crisis.

28
Q

What is a seizure?

A

Synchronized discharged from a population of cortical neurons resulting in a clinically evident alteration of function or behavior.

29
Q

What is epilepsy?

A

Two or more seizures not precipitated by a known cause (i.e., idiopathic seizure activity).

30
Q

What percent of seizure disorders are idiopathic?

A

70-75%

31
Q

What are non-idiopathic seizure etiologies?

A
  1. Fevers, particularly in children under 2 years old
  2. Inborn errors of metabolism
  3. Congenital brain malformation
  4. Acquired cortical defect (neoplasm, infection, trauma)
  5. Neurodegenerative disease
  6. Neurocutaneous disorders
  7. Electrolyte imbalances
  8. Toxins/drugs
32
Q

What are the classifications of seizures?

A
  1. Generalized – abnormal electrical activity involves both cerebral hemispheres, involves loss of consciousness.
  2. Partial – seizure activity is limited to a discrete segment of the cerebral cortex, no loss of consciousness, may have altered consciousness.

Status epilepticus – prolonged, non-self –limiting seizure activity, can be life threatening.

33
Q

What medical history information needs to be obtained from the parental report or from medical consult for patients with seizure disorders?

A
  1. Type of seizures
  2. Frequency of seizures
  3. Date of last seizure
  4. Anti-epileptic medications being taken
  5. Precipitating factors (if known)
34
Q

What kind of seizure is at higher risk for dental trauma and oral soft tissue trauma?

A

Patients who experience seizures that involve loss of consciousness and/or loss of motor control are at higher risk for dental trauma and oral soft tissue trauma than the general population.

35
Q

What common oral side effects are associated with use of anti-epileptic drug Dilantin (phenytoin)?

A

Gingival hypertrophy – Dilantin (phenytoin) causes gingival hypertrophy in approximately 50% of pts who take it. Gingival hyperplasia is not a reported side-effect of other anti-epileptic drugs.

36
Q

What are the side effects of valproic acid?

A

Valproic acid can cause decreased platelet count. Although thrombocytopenia is not usually severe, planning for significant elective surgery should include laboratory evaluation of bleeding parameters.

37
Q

What is the emergency management for a seizure in the dental office?

A
  1. Remove all instruments from the patient’s mouth and away from the dental chair.
  2. Place the dental chair in a supine position as close to the floor as possible.
  3. Place patient on his side, if possible.
  4. Do not attempt to restrain the patient.
  5. Time the seizure.
  6. Call 911 if the seizure lasts longer than three minutes or if the patient becomes cyanotic at any time.
  7. Administer oxygen at a rate of 6-8 L/minute.
  8. After seizure, try to evaluate patient’s level of consciousness.
  9. Do not allow the pt to leave the office if his/her level of awareness has not returned to baseline (this may be difficult to determine in a pt who has significant cognitive impairment).
  10. Do a brief oral exam to determine if any oral injuries were sustained during seizure.
  11. Depending on pt’s post-ictal status, either discharge to home with responsible adult or transfer to emergency room for further monitoring and evaluation.
38
Q

What are the diagnostic criteria for Asperger’s syndrome?

A
  1. Impairment in social interaction. Must include at least two of the following:
    - -Impairment of nonverbal behaviors such as facial expression, body postures, eye-to-eye contact.
    - -Failure to develop age-appropriate peer relationships.
    - -Lack of seeking to share enjoyment, interests or achievements with others.
    - -Lack of social or emotional reciprocity.
  2. Restricted repetitive and stereotyped patterns of behavior, interests and activities. Must include at least one of the following:
    - -Preoccupation with one or more restricted pattern of interest; abnormal in intensity or focus.
    - -Inflexible adherence to specific routines or rituals.
    - -Stereotyped and repetitive motor mannerisms.
    - -Persistent preoccupation with parts of objects.
  3. Clinically significant impairment in social, occupational, or other important areas of functioning.
  4. No clinically significant delay in language.
  5. No clinically significant delay in cognitive development or in the development of age-appropriate self-help skills, adaptive behavior and curiosity about the environment.
  6. Criteria are not met for another specific pervasive developmental disorder or schizophrenia.
39
Q

Children with autistic spectrum disorders can experience heightened reactions to what sensory inputs?

A
  1. Light, touch and auditory stimuli.

It can be imagined that a routine visit to a dental office could be perceived by the child with autism as a virtual bombardment of the senses.

40
Q

How can the parent help in managing the behavior of a child with autism?

A
  1. A parent can be very helpful in providing information concerning which type of stimuli might be most disturbing to the child.
  2. The parent may also be able to inform the dentist about distraction techniques that have worked in the past to help the child through stressful situations. These may include listening to music (for some other type of auditory input) through headphones or using a hand-held game system.
41
Q

What behavioral considerations should be considered for a pt with autism?

A
  1. Understanding what type of stimuli might disturb the child
  2. Distraction techniques
  3. Desensitization techniques
42
Q

What is desensitization?

A

Desensitization is a technique in which a pt is taught to replace fearful or maladaptive responses with non-fearful responses.

43
Q

What are oral side effects of psychotropic medications?

A
  1. Xerostomia

2. Sialorrhea

44
Q

How is the oral hygiene in pts with autism spectrum disorders?

A

Poor compliance with oral hygiene is not a consistent finding in children with autism spectrum disorders. Some children are very compliant, to the point of being obsessive brushers. Others have oral textural and taste sensitivities that interfere with adequate hygiene.

45
Q

On what basis are any of the autistic spectrum disorders diagnosed?

A

The diagnosis of any autistic spectrum disorder is based on behavior. There are no imaging, blood or genetic analyses that are used to make a diagnosis of an autistic spectrum disorder (although these tests may be used to rule out other disorders).

46
Q

Do individuals who are diagnosed with Asperger’s syndrome have cognitive impairment?

A

Children diagnosed with Asperger’s syndrome do not display any significant delays in either language or cognitive development.

47
Q

Are there any specific oral findings associated with autism?

A

No, there are no specific oral findings associated with autism.

48
Q

What are the factors that might put a child with autism at high risk for dental caries?

A
  1. Resistant behaviors that make it difficult to maintain oral hygiene on a daily basis, as well as in the dental setting.
  2. Candy being used as a frequent reward for acceptable behaviors.
  3. Oral sensitivities, including hypersensitivity to taste and texture.
  4. Psychotropic medications that may cause xerostomia.
49
Q

What gene is responsible for sickle cell anemia?

A

Beta globin gene (sickle hemoglobin, HbS) is responsible.

50
Q

What are the consequences of sickled red blood cells?

A
  1. Increased adhesion to vascular endothelium.
  2. Intravascular aggregation of cells.
  3. Inflammation of microvasculature.
  4. Vasoconstriction (responsible for the clinical symptoms of sickle cell crisis).
51
Q

What are the sequelae of vaso-occlusive sickle cell crises?

A
  1. Tissue anoxia
  2. Infarcts
  3. Necrosis
  4. Pain
52
Q

What factors may precipitate a sickle cell crisis?

A
  1. Dehydration
  2. Hypoxia
  3. Infection
  4. Stress
  5. Menstruation
53
Q

What questions should you ask in the medical history for sickle cell anemia?

A
  1. Vaso-occlusive crises - frequency, duration, hospitalizations, date of last crisis
  2. Damage to any organ systems
  3. History of transfusions and any related complications
  4. Current medication regimens
  5. Current and past infections
  6. Psychosocial issues
54
Q

What are the intra-oral findings associated with sickle cell anemia?

A
  1. Enlarged maxilla secondary to increased bone marrow activity
  2. Increase in maxillary incisor proclination and interdental spacing
  3. Mandibular incisor retrusion can be seen secondary to large overjet and lip pressure
  4. Pallor of oral soft tissues
  5. Gingival enlargement
  6. Glossitis
55
Q

What are the radiographic findings associated with sickle cell anemia?

A
  1. Coarse trabecular (“stepladder”) pattern in bone
  2. Mandibular radiopaque lesions
  3. Thin border of mandible
  4. Granular appearance of cranial bones
  5. Pulp stones
56
Q

What oral/systemic complications can occur in sickle cell anemia?

A
  1. Mandibular osteomyelitis
  2. Mandibular nerve neuropathies
  3. Pulpal necrosis in teeth without caries or history of trauma
  4. Facial or dental pain secondary to vaso-occlusive crisis
  5. Osteoporosis or osteopenia (patient may be taking bisphosphonates)
57
Q

What is the purpose of the three-month recall schedule following dental treatment under general anesthesia?

A
  1. Re-evaluate oral hygiene status; discuss with parents whether they feel that the techniques taught to brush the pt’s dentition have been effective.
  2. Review dietary recommendations; determine whether the parents have been able to make changes.
  3. Evaluate status of restorations placed in operating room.
58
Q

What ethnic predilection is there for sickle cell anemia?

A
  1. In the US, sickle cell anemia is seen most frequently in African-Americans. It may also be present in individuals of Mediterranean and Middle-Eastern descent.
59
Q

What is the underlying cause for the maxillary enlargement that is present in many individuals with sickle cell anemia?

A

The maxillary enlargement is secondary to heightened bone marrow activity and an increase in the marrow space in the maxilla.