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1
Q

Who is most likely to experience traumatic brain injury?

A

Children 0-4yo, adolescents 15-19yo, adults 75+

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2
Q

Leading causes of TBI

A

Falls, car accidents, violence, sports injuries

Shaken baby syndrome is a form of TBI

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3
Q

Symptoms of TBI

A
  • Severity can be classified by mild, moderate, or severe, determined by degree of secondary injury (i.e. hypoxia, hypotension) following the event
  • Disturbed cognition, behavior + emotional changes, motor function deficits
  • Headaches, fatigue, sleep disturbance, mood disorders, post-traumatic epilepsy, blood vessel damage, nerve damage-paralysis of facial muscles, vision disturbance, altered function of smell, loss of facial sensation, swallowing problems
  • Increased long-term risk of Alzheimer’s, Parkinson’s, dementia
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4
Q

Acute medical management of TBI

A
  • Medical imaging (skull + neck radiographs, CT scan, MRI) followed by emergency surgical + medical care
  • Degree of disability may depend on severity of injury, location, emergent care received, age + general health of individual
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5
Q

Chronic medical management of TBI

A
  • TBI rehab may focus on ability to perform tasks independently, engage w/ family + friends, participate in the community
  • Involves physical therapy, occupational therapy, speech/language therapy, physiatry (physical medicine), psychology/psychiatry, social support
  • Cognitive rehab w/ focus on communication + memory
  • Physical rehabilitation w/ focus on range-of-motion + strength exercises
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6
Q

Oral findings w/ TBI

A
  • Oral trauma related to heady injury
  • Auditory, visual or taste disturbance
  • Altered oral sensation, oral motor disorder, including swallowing problems
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7
Q

Fetal alcohol spectrum disorders

A

Describes a group of specific conditions

  • Fetal alcohol syndrome (FAS) is the most severe manifestation
  • Partial fetal alcohol syndrome
  • Neurobehavioral disorder associated w/ prenatal alcohol exposure
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8
Q

FASD Diagnosis

A
  • No medical test for FASD - diagnosis made based on
    • Abnormal facial features
    • Lower than average height, weight, or both
    • CNS problems
    • Prenatal alcohol exposure (confirmation but not required to make a diagnosis)
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9
Q

FASD epidemiology

A
  • 7.6% of pregnant women (1 in 13) report drinking in past 30 days, and 1.4% report binge drinking in past 30 days
  • Studies of school-aged children estimate 6-9 out of 100 children affected w/ FASD
  • Full range might be as high as 20-50 out of 1,000 children (2-5% of population)
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10
Q

FASD Etiology

A
  • Mother drinking during pregnancy
  • Alcohol in mother’s blood passes to baby through umbilical cord
  • There is no known safe amount of alcohol during pregnancy
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11
Q

FASD Physical features

A
  • Abnormal facial features
    • Short palpebral fissures
    • Flat nasal bridge w/ upturned nasal tip
    • Hypoplastic philtrum w/ thin upper vermillion border
    • Flat midface
    • Micrognathia
    • Cleft lip and/or palate
    • Small teeth w/ defective enamel
  • Small head size
  • Shorter than average height
  • Low body weight
  • Vision/hearing problems
  • Poor coordination
  • Sleep + sucking problems as a baby
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12
Q

FASD Mental + behavioral features

A
  • Hyperactivity + attention difficulty
  • Poor memory
  • Learning disability
  • Speech/language delay
  • Poor reasoning + judgment skills
  • Possible comorbid conditions: ADHD, mood dysregulation, aggression, anxiety
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13
Q

FASD Treatment

A
  • Occupational therapy
  • Behavioral therapy
  • Speech + language therapy
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14
Q

Factors that promote positive outcomes w/ FASD

A
  • Diagnosis before 6yo w/ early intervention
  • Loving, nurturing, stable home
  • Absence of violence
  • Involvement in special education + social services
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15
Q

FASD Dental considerations

A
  • Behavioral, hearing, vision constraints may impair ability to provide tx
  • Cooperation facilitated by:
    • Increasing familiarity + predictability of dental experience
    • Using visual prompts + simple instructions
    • Rewarding positive behavior
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16
Q

Autism spectrum disorder definition

A
  • Disorder of neural development characterized by impaired social interaction + communication, and by restrictive, repetitive behavior
  • Diagnosis based on behavioral criteria – there are no biomarkers
  • DSMV
    • ASD includes:
      • Autistic disorder
      • Asperger’s disorder
      • Childhood disintegrative disorder
      • Pervasive developmental disorder-not otherwise specified
  • Diagnosis now based on level of severity
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17
Q

Autism prevalence

A

1 in 68 children

4 : 1

Boys > Girls

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18
Q

Autism etiology

A
  • Heterogeneous w/ multiple known causes (i.e. genetic causes such as Down syndrome + fragile X), although in most cases, the etiology is unknown.
  • Strong genetic basis, although the genetics of autism are complex and it is unclear whether ASD is explained more by rare mutations or by rare combinations of common genetic variants
  • Environmental factors – stress, pesticide exposure, prematurity, increasing parental age, and maternal autoimmune disorders may contribute to autism
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19
Q

Autism symptoms

A
  • Per diagnostic criteria
    • Must exist in the early developmental period (generally before 5yo)
    • Cause clinically significant impairment in function
    • Are not better explained by intellectual disability or developmental delay
  • May include
    • Delayed development of speech + language (some cases severe or nonverbal)
    • Difficulty w/ relationships
    • Restrictive, repetitive behaviors
    • Obsessive interests
    • Insistence on sameness + resistance to change
    • Seemingly meaningless routines
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20
Q

Early signs of autism

A
  • Does not respond to name
  • Acts as though deaf
  • Does not smile socially
  • Does not point or gesture by age 1
  • No babbling
  • Talks + uses language but w/o social intent (lacks social communication)
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21
Q

What two medications are FDA approved for use in autism?

A

Aripiprazole + Risperidone

For aggression + irritability

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22
Q

Oral findings in autism

A
  • OH may be poor due to inability to brush or accept assistance w/ brushing
  • Factitial injuries due to self-injurious behavior
  • Cariogenic rewards used in behavior therapy may contribute to higher caries risk
  • Occlusal attrition due to esophageal reflux or bruxism
  • May exhibit side effects of any medications taken
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23
Q

Advanced behavior guidance w/ autism

A
  • Physical restraint requires appropriate informed consent, but may be preferred by parents of some children
  • SED + GA may be appropriate to address dental needs in an atraumatic fashion and/or when non-pharmacologic attempts have failed
  • Must factor in behavioral + sedative daily medications when planning care under SED/anesthesia
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24
Q

Fragile X syndrome definition

A

A genetic condition w/ variable expression that causes a range of developmental problems + cognitive impairment

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25
Q

What is the most common inherited cause of intellectual disability?

A

Fragile X syndrome

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26
Q

Fragile X syndrome prevalence

A

Full mutation in males = 1 : 3,600

Females = 1 : 4,000-6,000

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27
Q

Fragile X syndrome etiology

A
  • Caused by a mutation in the FMR1 gene on the X chromosome
  • Mutation consists of repeated trinucleotide sequence (CGG)
  • The mutation is thought to be related to maternal folate deficiency
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28
Q

Symptoms of fragile X syndrome

A
  • Large or prominent ears
  • Elongated face w/ prominent forehead
  • Hyperextensible finger joints
  • Enlarged testicles
  • Many children will not show the characteristic phenotypic features
  • Deficits in cognitive skills
  • Impairments in adaptive behaviiors
  • Many children will also have autism, ADHD, anxiety
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29
Q

Fragile X syndrome medical management

A
  • Should be considered in any child who has idiopathic intellectual disability bc the physical features are not always present
  • Benefit from early intensive intervention, including speech + language therapy, occupational therapy, special education
  • May benefit from medications that provide symptomatic (not curative) therapy, including stimulants for ADHD, antidepressants for anxiety, atypical antipsychotics from autism symptoms, mood instability, aggression
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30
Q

Fragile X syndrome dental considerations

A
  • Oral findings
    • Side effects from medications
    • TMJ problems due to hyperextensibility of the joint
    • Additional physical characteristics can include:
      • Macrocephaly
      • Prominent frontal bone
      • Hypotelorism
      • Strabismus
      • Midface hypoplasia
      • Pierre Robin sequence
      • Cleft palate
      • Dental hypomineralization
      • Mesiodens
      • Abnormal tooth size
  • Tx
    • May require extensive behavior guidance due to anxiety and/or behavior issues
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31
Q

ADHD definition

A

Persistent pattern of inattention and/or hyperactivity/impulsivity w/ functioning or development (DSM V)

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32
Q

What is the most common neurobehavioral disability in children?

A

ADHD

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33
Q

Epidemiology ADHD

A

5-8% of all children; 2.5% of adults

3 : 1 , male : female predilection

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34
Q

ADHD etiology

A
  • Genetic predisposition may be influenced by:
    • Temperament
    • VLBW
    • Smoking during pregnancy
    • Diet
    • Toxic stress
    • Trauma
    • Exposure to environmental toxins
  • May be associated w/ other conditions, syndromes, psychiatric disorders
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35
Q

ADHD symptoms

A
  • ADHD characterized by
    • Inattention: Inability/difficulty focusing on a task or sustaining a task, though the child intellectually understands the task and wants to perform it
    • Hyperactivity: Excessive, inappropriate motor activity
    • Impulsivity: Hasty actions that are made w/o forethought that may pose a high risk of harm to the individual
  • Several symptoms must be present before 12yo for a child to receive a diagnosis of ADHD, but many children are diagnosed much earlier
  • Parents may first notice symptoms when child is a toddler, but these symptoms cannot be distinguished from normal behavior until 4yo
  • Symptoms must be present for at least 6mo to be ADHD
  • Symptoms must be present in multiple settings (home, school) to be diagnosed as ADHD
  • In teens, may present as antisocial behavior, jitteriness, restlessness, impatience
  • Symptoms will lead to functional impairment, which may contribute to:
    • Poor performance in school
    • Social rejection
    • Poor occupational performance
    • Interpersonal conflict
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36
Q

Comorbidities of ADHD may include

A
  • Oppositional defiant disorder
  • Conduct disorder
  • Depression
  • Anxiety disorder
  • Speech/language disorder
  • Intellectual/learning disabilities
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37
Q

Therapy considerations with ADHD

A
  • Anticipatory guidance is critical: child + family should receive age-appropriate education regarding ADHD
  • Child should have an individualized education program (IEP) w/ the school system
  • Therapy should be monitored, re-evaluated, and periodically adjusted
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38
Q

Medications in ADHD

A
  • First line is typically a stimulant [methylphenidate, dextroamphetamine, mixed amphetamine salts, lisdexamfetamine, dexmethylphenidate]
  • Side effects of stimulant/amphetamine:
    • Tachycardia
    • Xerostomia
    • Bruxism
    • Decreased appetite
    • Stomach ache
    • Jitteriness
    • Delayed sleep onset
    • Social withdrawal
    • Motor tics
  • Symptoms of excessive dose: Child appears dull or overly restricted
  • Supplementary or alternative medication may include anti-hypertensive/alpha-2 agonist (clonidine, methyldopa, guanfacine)
    • Side effects
      • Dysphagia
      • Xerostomia
      • Sialadenitis
      • Potentiates CNS depressants (caution w/ sedative agents)
      • Cardiac arrhythmias
  • Child may take “holidays” from drug therapies
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39
Q

ADHD oral findings

A
  • Dental wear due to bruxism
  • May be at increased risk of caries
  • May be at increased risk for dental trauma due to impulsive behaviors
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40
Q

Dental considerations w/ ADHD

A
  • Update med hx, update account progress/changes in ADHD history
  • If possible, the child should take his/her regular ADHD medications on days that dental treatment is provided
  • Keep appointments short
  • Use positive reinforcement
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41
Q

Major depressive disorder (classical depression)

A

Typically a recurrent condition consisting of periods of at least 2 weeks w/ demonstrable changes in emotion, intellectual function, overall neurological function

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42
Q

Depression epidemiology

A
  • Major depressive disorder can occur at any age, occurring most frequently in the late 20s
  • Prevalence over a 12mo period is 7%, and lifetime prevalence in 16%
  • 1.5-3x more common females than males
  • Risk of recurrence is higher for children than adults
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43
Q

Etiology of depression

A
  • Classic hypothesis: Deficiency of 5HT (and other monoamine neurotransmitters) in the synaptic cleft
  • Modern hypothesis: Pathophysiology of major depressive disorder is complex, involving multiple neurotransmitter systems
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44
Q

Depression symptoms

A
  • Someone w/ major depressive disorder will exhibit at least 5 of the following symptoms over a period of at least 2 weeks:
    • Depressed mood most of the time
    • Significantly decreased interest in most activities
    • Unexplained weight loss (or failure to gain weight in children)
    • Insomnia or hypersomnia
    • Psychomotor changes
    • Fatigue
    • Feelings of worthlessness or guilt
    • Inattentiveness or indecisiveness
    • Recurrent thoughts of death and/or suicide
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45
Q

Dental considerations w/ depression

A
  • Medications can cause changes in saliva production as well as a subjective feeling of dry mouth (xerostomia)
  • Medications can cause nausea + vomiting, leading to dental erosion
  • Most nausea + vomiting will subside after two weeks on the medications
  • Comorbid conditions that occur w/ depression can have oral side effects (bulimia, substance abuse, obsessive compulsive disorder)
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46
Q

What type of hearing loss are hearing aids not effective for?

A

Sensorineural hearing loss

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47
Q

What type of hearing loss do bone anchored hearing aids help treat?

A

Conductive or mixed hearing loss

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48
Q

What type of hearing loss can cochlear implants effectively treat?

A

Sensorineural hearing loss

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49
Q

Dental considerations w/ hearing loss + deafness

A
  • Providers can build rapport through basic sign language
  • Maintain eye contact when using sign language
  • Speak slowly but do not change your lip movements so the person can read your lips
  • Be aware when wearing a face mask that the individual will not be able to see your lips
  • People who are deaf or hard of hearing will depend more on their other senses using nonverbal communication such as eye and head movement
  • Intelligence is normal to superior
  • Be cognizant of other medical issues
  • Consider having parent, caregiver or other Sign Language Interpreter present to aid in communication w/ the patient
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50
Q

A1c

A

Average glucose for past 2-3mo

  • Normal: <5.7%
  • Prediabetes: 5.7-6.4%
  • Diabetes: 6.5%+
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51
Q

Fasting plasma glucose

A

Glucose after fasting for at least 8hr

  • Normal: <100mg/dL
  • Prediabetes: 100-125mg/dL
  • Diabetes: 126mg/dL+
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52
Q

Oral glucose test

A

Blood glucose measurement levels before and 2hr after drinking a sweet liquid with a measured amount of glucose

Diabetes: 200mg/dL +

53
Q

Risk of Type 2 diabetes associated with?

A
  • Obesity
  • Female gender
  • Family hx
  • Physical activity
  • Race/ethnicity
54
Q

What does gestational diabetes increase the risk of?

A
  • Preeclampsia
  • High birth weight
  • Birth related trauma
  • Jaundice
  • Hypoglycemia
  • Birth defects
55
Q

Symptoms of diabetes

A
  • Polyuria, polydipsia, polyphagia
  • Weight loss associated w/ Type 1 diabetes
  • Fatigue, lethargy, and symptoms of insulin resistance (acanthosis nigricans, hypertension, dyslipidemia, PCOS) when poorly controlled
56
Q

Dental considerations w/ diabetes

A
  • Candidiasis is present in a significant portion of younger children at diagnosis
  • Salivary hypofunction, xerostomia, taste alteration
  • Most studies report that children w/ Type 1 diabetes have greater plaque accumulation + poorer periodontal health than healthy children
  • Periodontal disease increases w/ age
  • Children w/ poorly controlled diabetes may have greater caries incidence while those who are well controlled often experience less decay
  • Slightly accelerated tooth eruption in Type 1 diabetes
  • Impaired wound healing w/ poor disease control
  • Palliative care for dry mouth w/ salivary substitutes
  • Ensure glycemic control for dental appointments
    • Short morning appointments
    • Eat + take usual meds before appointment
    • Minimize stress
  • In case of hypoglycemic episode
    • Stop dental tx
    • High carbohydrate beverage/gel or IM glucagon
  • Relative contraindication for oral sedation
  • Special precautions needed for GA
57
Q

Hypothyroidism

A

Hypometabolic state caused by insufficient synthesis + release of thyroid hormone

Prevalence increases w/ age

Serum testing reveals high TSH + low free T4

58
Q

What is the leading preventable cause of intellectual delay?

A

Hypothyroidism

59
Q

Symptoms of hypothyroidism

A
  • Majority have no clinical signs at birth
  • Infants + children may demonstrate lethargy + failure to thrive
  • Women may present w/ menstrual irregularities + infertility
  • Older patients may have cognitive decline as the sole manifestation
  • Untreated hypothyroidism can cause hypertension, dyslipidemia, infertility, cognitive impairment, and neuromuscular dysfunction
60
Q

Dental considerations for hypothyroidism

A
  • Oral findings
    • Macroglossia
    • Thick lips
    • Malocclusion
    • Delayed eruption + skeletal development
    • Gingival edema
    • Hyposalivation
  • Tx
    • Sensitivity to sedative medications
    • Delayed healing + susceptibility to infection
    • Slightly delayed coagulation following surgical procedures
61
Q

Hyperthyroidism

A

Hypermetabolic state caused by excess synthesis + release of thyroid hormone

TSH is suppressed, free thyroxine (T4) and triiodothyronine (T3) are elevated

62
Q

What accounts for 96% of pediatric hyperthyroidism cases?

A

Graves disease

63
Q

Symptoms of hyperthyroidism

A
  • Acceleration of growth + epiphyseal maturation
  • Ophthalmopathy caused by inflammation of the extraocular muscles, orbital fat, connective tissue results in proptosis (exophthalmos)
  • Diffuse goiter
  • Increased cardiac output, w/ 2-3x increase in prevalence of mitral valve prolapse
  • Failure to gain weight despite increase in appetite
  • Mood swings + behavioral disturbances, attention span decreases, hyperactivity, poor sleep, deteriorating school performance
64
Q

Medical management of hyperthyroidism

A
  • Antithyroid drugs
  • Radioactive iodine
  • Sx (thyroidectomy)
65
Q

Hyperthyroidism dental considerations

A
  • Oral findings
    • Increased risk of caries, periodontal conditions
    • Enlargement of extraglandular thyroid tissue (lateral posterior tongue)
    • Maxillary/mandibular osteoporosis
    • Accelerated dental eruption
    • Burning mouth syndrome
  • Tx
    • May have increased bleeding time due to elevated BP/HR + anti-thyroid drugs that may cause hypoprothombinemia
    • Analgesics containing acetylsalicylic acid (ASA) are contraindicated due to interference w/ protein binding of T4 + T3
    • Avoid use of epinephrine or pressor amines in poorly controlled patients
66
Q

What characterizes cystic fibrosis

A

Chronic pulmonary disease, pancreatic insufficiency, high sweat electrolyte concentrations

67
Q

How is cystic fibrosis diagnosed?

A

Genetic testing

Newborn screening: Immunoreactive trysinogen - if positive, repeat in 2wks, if positive again, sweat chloride test

Sweat chloride test is gold standard but not always definitive

68
Q

Cystic fibrosis etiology

A
  • Abnormal cystic fibrosis transmembrane conductance regulator (CFTR) protein
  • Abnormal ion transport in sweat glands
    • Decreased secretion of chloride, increased absorption of sodium
  • Exocrine glands produce thick sticky secretions leading to mucus formation
  • Lungs, GI, reproductive epithelium most affected
69
Q

Cystic fibrosis symptoms

A
  • Salty tasting skin
  • Difficulty breathing, recurrent wheezing, cough; recurrent pneumonia; nasal polyps sinusitis
  • Pancreatic insufficiency, maldigestion (difficulty digesting fat), abdominal pain, excess gas
  • Failure to gain weight; abnormal bowel movements
  • Clubbing of fingers + toes related to inadequate oxygenation
  • Male infertility
70
Q

Dental considerations for cystic fibrosis

A
  • Oral findings
    • Increased enamel defects
    • Decreased caries – may be related to oral abx and/or elevated salivary pH
    • Increased calculus
    • Gingivitis
    • Mouthbreathing/malocclusion
    • Increased risk for candidiasis
  • Tx
    • Consult w/ physician to assess patient ability to tolerate tx in routine setting
    • Short appointments – later in the day b/c of mucous accumulations at night + increased coughing in the morning
    • More upright positioning
    • Consult regarding any needed abx due to chronic antibiotic use
71
Q

Partial seizures

A

40% - affects one area of the brain; categorized as simple or complex.

  • Most common site of origin: Within frontal or temporal lobes
  • Patient may experience aura (taste, smell)
  • Seizure presentation varies - lip smacking, abnormal behavior, localized numbness or tingling, hallucinations, visual disturbances, overwhelming sense of fear
  • Impairment of consciousness
  • Amnesia, confusion, sleepiness accompany seizure
72
Q

Generalized seizures

A

40% - affects all areas of brain simultaneously; categorized as convulsive (tonic-clonic) or non-convulsive (absence)

73
Q

Tonic-clonic seizures

A
  • Aura or prodromal mood change
  • Post-ictal phase (10-30min): atonia + incontinence
  • Most common form of generalized seizure
74
Q

Absence seizure

A
  • Onset at ages 4-10yo
  • May be misdiagnosed as behavior or learning problems
75
Q

Side effects of anti-convulsive medications

A
  • Xerostomia
    • Stomatitis
    • Dysgeusia
  • Gingival hyperplasia
  • Bone marrow suppression
  • Decreased platelet count
  • Mood/behavior changes
  • Neurologic symptoms
  • Depression
  • Anorexia
  • Infection
76
Q

What should you ask regarding med hx for a patient w/ h/o seizures?

A
  • Type of seizures
  • Frequency - date of last seizure
  • Control of seizures - date of last hospitalization for seizure
  • H/o injuries from previous seizures
  • Duration
  • Triggers
  • Medications + compliance
  • Diet
77
Q

Dental considerations w/ seizures

A
  • Consult neurologist
  • Verify patient has taken medications + eaten before dental appt
  • Schedule patient at time of day when seizures are less likely to occur, based on hx
  • Schedule when well-rested
  • Consider anxiety management
  • Dental light may trigger seizures - consider dark glasses
  • Be prepared to manage a seizure
  • Use rubber dam for restorative tx to minimize oral debris
  • Recognized increased risk for dental trauma
78
Q

Seizure management in dental office

A
  • Note time seizure began
  • Stay calm
  • Move onlookers away
  • Clear area from loose instruments
  • Position patient to prevent injury
    • Chair supine + at lowest position
    • Roll patient on side if possible to prevent aspiration
    • Do not place anything in the mouth
  • Post-ictal airway support
    • Maintain patent airway
    • Place rolled towel under shoulders
    • Suction airway
    • Record vital signs
    • Provide supplemental O2 as needed
  • Activate EMS in cases of continued seizures (status epilepticus) for longer than 5min or if patient becomes cyanotic
79
Q

When should mitochondrial disorders be considered?

A
  • When the following are noted, especially in combination:
    • Encephalopathy
    • Neuropathy
    • Cardiac conduction defects/cardiomyopathy
    • Hearing deficits
    • Short stature
    • Disorders of extraocular muscles
    • Diabetes
    • Renal tubular disease
    • Visual loss
    • Lactic acidosis
    • Susceptibility to malignant hyperthermia
80
Q

Mitochondrial disorder etiology

A
  • Mutations in nuclear DNA or mitochondrial DNA cause changes in mitochondria that lead to energy-deficient state
  • May affect a single organ or multiple organ systems
81
Q

Mitochondria disorder symptoms

A
  • Variable depending on the organ system involved
  • Seizure disorder
  • Developmental delay
  • Movement disorders
  • Complicated migraine
  • Stroke
82
Q

MItochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS)

A
  • Classic mitochondrial encephalopathy
  • Variable clinical presentation
  • May present at any age:
    • Childhood: Nuclear DNA abnormalities
    • Childhood or adult life: Mitochondrial DNA abnormalities
83
Q

how are nuclear DNA gene defects inherited?

A

By autosomal recessive or autosomal dominant transmission

84
Q

How are mitochondrial DNA defects transmitted?

A

By maternal inheritance

85
Q

Mitochondrial disorder medical management

A

Supplementation w/ vitamins, co-factors (CoQ10) to increase ATP production

86
Q

Oral findings with mitochondrial disorders

A
  • Acid erosion related to GERD
  • Dysfunctional swallowing
87
Q

Dental tx considerations with mitochondrial disorders

A
  • Consult w/ MD RE: patient’s specific form of disorder
  • Consider medical manifestations of disorder + manage dental care as indicated by medical condition
  • Early institution of preventive oral care - consider increased frequency of preventive visits
  • Schedule appointments when patient is well-rested
  • Avoidance of physiologic stress (heat, cold, lack of sleep, lack of food)
  • If GA, malignant hyperthermia precautions should be used
88
Q

What are the most common forms of neural tube defects?

A

Anencephaly + spina bifida

89
Q

Neural tube defects: etiology

A
  • Folic acid deficiency - most common
  • Maternal insulin-dependent diabetes
  • Maternal medications for epilepsy
  • Maternal obesity
90
Q

Neural tube defects: classification

A
  • Open neural tube defects
    • Involve entire CNS
    • Neural tissue is exposed w/ associated cerebrospinal fluid leakage
  • Closed neural tube defects
    • Localized + confined to the spine
    • Neural tissue is not exposed and the defect is fully epithelialized, although the skin covering the defect may not be dysplastic
91
Q

Cranial forms of neural tube defects

A
  • Anencephaly
    • Cephalic end of neural tube fails to close
    • Results in absence of forebrain + cerebrum
    • Patient dies within days of birth, if not stillborn
  • Encephalocele (meningocele or meningomylocele)
    • Failure of neural tube to close completely
    • Results in groove down middle of upper part of skull
    • Accompanied by other craniofacial abnormalities
92
Q

Neural tube defects: clinical symptoms

A
  • Hydrocephalus
  • Spastic quadriplegia
  • Microcephaly
  • Ataxia
93
Q

What is the most common type of spina bifida?

A

Spina bifida occulta

  • Most mild form
  • Outer part of some vertebrae are not completely formed
  • Spinal cord intact
  • No long-term effects
94
Q

What is the least common type of spina bifida?

A

Meningocele

  • Meninges pushed out through cleft in vertebrae
  • Meningeal sac usually covered by skin
  • Nerve damage atypical
  • Few, if any, long-term side effects
95
Q

What is the most severe form of spina bifida?

A

Myelomeningocele

  • Meninges protrudes as a sac through a cleft in vertebrae
  • Sac contains CSF + nerve tissue
  • Clinical symptoms:
    • Paralysis
    • Incontinence (bladder + bowel)
    • Hydrocephalus
  • Arnold-Chiari II malformation
96
Q

Arnold-Chiari II malformation

A

Myelomeningocele

  • Portion of cerebellum protrudes through foramen magnum into spinal cord.
  • Patients may only present with symptoms in adolescence or adulthood.
  • Headache in back of head.
  • Dizziness/double vision
  • Feeling of discomfort and/or choking on reclining
  • Medical management of A-CII: Surgery to enlarge foramen magnum, relieve pressure on cerebellum
97
Q

Dental considerations for neural tube defects

A
  • Latex allergy precautions
  • Consultation w/ patient’s MD regarding nature of defect + past med hx
  • Patient may have increased anxiety due to other medical interventions
  • Some patients w/ neural tube defects have mental disability
  • Some adolescent patients w/ spina bifida develop anxiety
  • Office access for wheelchair
  • Consider tx in wheelchair
  • Antibiotic prophylaxis for VA or VV shunt for hydrocephalus
  • Patients on long-term abx may develop resistance – consult MD
  • Chiari II malformation – consider tx w/ patient in more upright position
98
Q

Hydrocephalus: congenital etiology

A
  • Accounts for ~50% of all hydrocephalus cases.
    • Developmental disorders (neural tube defects such as spina bifida)
    • Arnold-Chiari malformation
    • Craniosynostosis
    • Genetic inheritance (aqueductal stenosis)
99
Q

Hydrocephalus: acquired

A
  • Head trauma
  • Meningitis
  • Tumors/cysts
100
Q

“Sunsetting” is seen in what condition?

A

Hydrocephalus

101
Q

Hydrocephalus: symptoms

A
  • Rapid increase in head circumference (in infants)
  • Vomiting
  • Seizures
  • “Sunsetting” = downward casting of eyes
  • Sleepiness
  • Irritability
  • Blurred vision
  • Inability to balance
102
Q

Dental considerations for hydrocephalus

A
  • Consult MD regarding patient’s condition
    • Type of hydrocephalus, type of shunt, need for IE prophylaxis
  • Patient may be apprehensive of dental treatment due to frequent medical interventions
  • Strong emphasis on preventive oral care to minimize the need for restorative tx
  • Awareness that any symptom indicative of shunt malfunction or infection (soreness, redness, headache, return of hydrocephalus symptoms) warrant immediate medical attention
103
Q

What is the most common motor disability in childhood?

A

Cerebral palsy

104
Q

What type of cerebral palsy comprises most of the CP cases?

A

Spastic

  • Spastic: contractures, lack of control (>75% of patients)
  • Dyskinetic: slow, writing involuntary movements (15%)
  • Ataxic: tremors or uncoordinated voluntary movements (5%)
  • Mixed: combination
105
Q

Diplegia

A

2 extremities

106
Q

Hemiplegia

A

One side of the body

107
Q

Monoplegia

A

One limb

108
Q

Symptoms + associated conditions w/ cerebral palsy

A
  • Pain (50-75%)
  • Bladder control problems (30-60%)
  • Intellectual disability (50%)
  • Speech + language impairment (40-50%)
  • Vision impairment (30-50%)
  • Epilepsy (25-40%)
  • Behavior disorder (25%)
  • Drooling (20%)
  • Hearing impairment (10-20%)
  • G-tube dependence (7%)
  • Autism (6%)
109
Q

CP treatment

A
  • Spasticity
    • Botox
    • Oral anti-spastic drugs (benzos, dantrolene, baclofen)
    • Intrathecal baclofen (via pump)
    • Sx is now rarely performed to tx spasticity
  • Orthopedic interventions
    • Serial casting to stretch shortened muscles + improve range of motion
    • Muscle-tendon sx to release restricted joint motion (rarer today)
  • Physical therapy
  • Feeding + nutrition
    • G-tube to improve nutritional status, growth, reduce aspiration + provide route for medication
  • Drooling
    • Anticholinergic agents (benzhexol HCl, scopolamine, glycopyrrolate)
    • Botox into salivary glands
  • Osteopenia
    • IV bisphosphonate may be used in some children w/ severe reduction of bone material density
110
Q

Dental considerations w/ CP

A
  • May require assistance in moving to + staying in dental chair
  • Do not force limbs into unnatural positions
  • Consider treating in wheelchair
  • Support head + consider mouth prop
  • Keep back elevated to minimize swallowing difficulties
  • Caution w/ irrigation or ultrasonic scaling due to possibility of aspiration
  • Rubber dam for restorative procedures
  • May require GA for extensive dental work
  • If h/o IV bisphosphonate, use caution, get appropriate consent + consider oral surgery consult prior to EXT
111
Q

Oral findings w/ CP

A
  • Poor OH
  • Increased risk for dental caries + periodontal disease
  • Enamel hypoplasia
  • Erosion from GERD
  • Delayed eruption of permanent teeth
  • Gingival hyperplasia in patients prescribed phenytoin for epilepsy
  • Increased incidence of malocclusion (Class I Div I)
  • Increased risk for oral trauma (prominent incisors, incompetent lips, difficulty in ambulation, seizures)
  • Bruxism
  • Sialorrhea
  • Oral hypersensitivity, dysphagia, hyper/hypoactive gag reflex, tongue thrust, mouth breathing
112
Q

Elevated serum creatine kinase may be detected before clinical signs of what condition?

A

Muscular dystrophy

113
Q

Classification of muscular dystrophy

A
  • Duchenne (DMD/Becker (BMD)
    • Most common forms – BMD is milder form
    • X-linked recessive, affects mostly boys
    • Intellectual disability common
  • Other forms: Limb-Girdle (LGMD), congenital (CMD), and myopathies, distal (DD), oculopharyngeal (OPMD), Emery-Dreifuss (EDMD), facioscapulohumeral (FSH)
114
Q

Medical management of muscular dystrophy

A
  • Clinical course
    • Most DMD patients confined to wheelchair after 12yo, with life expectancy into teens + 20’s
  • Disease management
    • No cure/effective tx
    • Physical, respiratory, speech therapy
    • Orthopedic appliances, corrective orthopedic sx, assisted ventilation, tracheostomy
    • Pharmacology: immunosuppressants, abx for respiratory infections, benzos, calcium antagonists, antiarrhythmias
115
Q

Oral findings w/ muscular dystrophy

A
  • Malocclusion
    • Small maximum opening, anterior open bite, excessive interocclusal distance, posterior crossbite, broad/shallow dental arches, open bite
  • Poor OH
  • Tongue thrusting, mouth breathing
  • Weakness of muscles of mastication
  • Delayed eruption of permanent dentition
  • Hypoplasia, microdontia, agenesis common
116
Q

Dental considerations w/ muscular dystrophy

A
  • Consult w/ MD
  • May require assistance w/ ambulation + chair positioning
  • Diligent suctioning of fluids
  • Caution w/ use of vasoconstrictors for patients w/ cardiomyopathy
  • Caregivers may need to perform OH
  • Severely affected individuals may be better treated under GA
  • Caution w/ neuromuscular blocking agents (e.g. succinylcholine) during GA
117
Q

What SHCN conditions may be more susceptible to dental trauma?

A

ADHD, seizure disorder, developmental/intellectual disability

118
Q

Considerations of patients w/ SHCN w/ dental trauma management

A
  • Use existing trauma guidelines
  • Consider patient’s condition + ability to tolerate immediate and future tx needs
    • Discuss implications w/ parent/caregiver
    • Consider consult w/ patient’s MD
    • Patient’s health condition may impact tx outcome (e.g. patients w/ diabetes or immunocompromised status may have delayed or impaired healing response)
  • Replantation of avulsed permanent teeth may be contraindicated for patients w/ certain medical conditions
    • Severe cardiac disease
    • Severe seizure disorder
    • Severe immunocompromised patients
    • Severe developmental disability
  • Protective stabilization (w/ informed consent) may be indicated for emergency assessment + management
  • If unable to record radiographs, consider clinical photographs of the injury
119
Q

Replantation of avulsed permanent teeth may be contraindicated for patients w/ which medical conditions

A
  • Severe cardiac disease
  • Severe seizure disorder
  • Severe immunocompromised patients
  • Severe developmental disability
120
Q

SHCN definition

A
  • Any physical, developmental, mental, sensory, behavioral, cognitive or emotional impairment or limiting condition that requires medical management, health care intervention, and/or use of specialized services or programs.
  • This condition may be congenital, developmental, or acquired through disease, trauma or environmental cause and may impose limitations in performing daily self-maintenance activities or substantial limitations in major life activity.
121
Q

What are the cornerstones of patient-centered health care?

A
  • Flexibility
  • Responsiveness
  • Continuity
  • Comprehensiveness
  • Coordination
122
Q

Down syndrome: dental manifestations

A

Delayed eruption

Missing and supernumerary teeth -

Malformed Teeth (taurodontism) -

Microdontia

Increased risk of perio (Down syndrome : neutrophil chemotaxis defect)

Low levels of caries

Open Bite

Class III tendency

Appearance of Macroglossia

Fissured tongue

123
Q

Down syndrome: treatment considerations

A

Cardiac defects (40%)(SBE)-
Atrioventricular septal defect

Leukemia (15x) (AML)

Atlantoaxial instability (contraindication in restraint)

3 copies of chromosome 21 (47 total)

Risk of airway obstruction due to large tonsils/adenoids

Possible reduced risk to infection

124
Q

Other facts about Down syndrome

A

Increased risk with maternal age, 99% non-heritable

Increased risk after having one child with Down Syndrome

125
Q

Cleidocranial dysplasia: dental manifestations

A

Delayed exfoliation

Delayed eruption

Impacted teeth

Hyperdontia (supernumerary) Peg like teeth

Taurodontism

126
Q

Cleidocranial dysplasia: treatment considerations

A

Runx2 gene mutation

127
Q

Other facts about cleidocranial dysplasia

A

Underdeveloped/absent clavicles

128
Q
A

* (22 decks)

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