SHCN Flashcards
Who is most likely to experience traumatic brain injury?
Children 0-4yo, adolescents 15-19yo, adults 75+
Leading causes of TBI
Falls, car accidents, violence, sports injuries
Shaken baby syndrome is a form of TBI
Symptoms of TBI
- 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
Acute medical management of TBI
- 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
Chronic medical management of TBI
- 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
Oral findings w/ TBI
- Oral trauma related to heady injury
- Auditory, visual or taste disturbance
- Altered oral sensation, oral motor disorder, including swallowing problems
Fetal alcohol spectrum disorders
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
FASD Diagnosis
- 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)
FASD epidemiology
- 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)
FASD Etiology
- 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
FASD Physical features
- 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
FASD Mental + behavioral features
- Hyperactivity + attention difficulty
- Poor memory
- Learning disability
- Speech/language delay
- Poor reasoning + judgment skills
- Possible comorbid conditions: ADHD, mood dysregulation, aggression, anxiety
FASD Treatment
- Occupational therapy
- Behavioral therapy
- Speech + language therapy
Factors that promote positive outcomes w/ FASD
- Diagnosis before 6yo w/ early intervention
- Loving, nurturing, stable home
- Absence of violence
- Involvement in special education + social services
FASD Dental considerations
- 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
Autism spectrum disorder definition
- 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
- ASD includes:
- Diagnosis now based on level of severity
Autism prevalence
1 in 68 children
4 : 1
Boys > Girls
Autism etiology
- 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
Autism symptoms
- 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
Early signs of autism
- 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)
What two medications are FDA approved for use in autism?
Aripiprazole + Risperidone
For aggression + irritability
Oral findings in autism
- 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
Advanced behavior guidance w/ autism
- 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
Fragile X syndrome definition
A genetic condition w/ variable expression that causes a range of developmental problems + cognitive impairment
What is the most common inherited cause of intellectual disability?
Fragile X syndrome
Fragile X syndrome prevalence
Full mutation in males = 1 : 3,600
Females = 1 : 4,000-6,000
Fragile X syndrome etiology
- 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
Symptoms of fragile X syndrome
- 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
Fragile X syndrome medical management
- 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
Fragile X syndrome dental considerations
- 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
ADHD definition
Persistent pattern of inattention and/or hyperactivity/impulsivity w/ functioning or development (DSM V)
What is the most common neurobehavioral disability in children?
ADHD
Epidemiology ADHD
5-8% of all children; 2.5% of adults
3 : 1 , male : female predilection
ADHD etiology
- 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
ADHD symptoms
- 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
Comorbidities of ADHD may include
- Oppositional defiant disorder
- Conduct disorder
- Depression
- Anxiety disorder
- Speech/language disorder
- Intellectual/learning disabilities
Therapy considerations with ADHD
- 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
Medications in ADHD
- 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
- Side effects
- Child may take “holidays” from drug therapies
ADHD oral findings
- Dental wear due to bruxism
- May be at increased risk of caries
- May be at increased risk for dental trauma due to impulsive behaviors
Dental considerations w/ ADHD
- 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
Major depressive disorder (classical depression)
Typically a recurrent condition consisting of periods of at least 2 weeks w/ demonstrable changes in emotion, intellectual function, overall neurological function
Depression epidemiology
- 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
Etiology of depression
- 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
Depression symptoms
- 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
Dental considerations w/ depression
- 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)
What type of hearing loss are hearing aids not effective for?
Sensorineural hearing loss
What type of hearing loss do bone anchored hearing aids help treat?
Conductive or mixed hearing loss
What type of hearing loss can cochlear implants effectively treat?
Sensorineural hearing loss
Dental considerations w/ hearing loss + deafness
- 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
A1c
Average glucose for past 2-3mo
- Normal: <5.7%
- Prediabetes: 5.7-6.4%
- Diabetes: 6.5%+
Fasting plasma glucose
Glucose after fasting for at least 8hr
- Normal: <100mg/dL
- Prediabetes: 100-125mg/dL
- Diabetes: 126mg/dL+
Oral glucose test
Blood glucose measurement levels before and 2hr after drinking a sweet liquid with a measured amount of glucose
Diabetes: 200mg/dL +
Risk of Type 2 diabetes associated with?
- Obesity
- Female gender
- Family hx
- Physical activity
- Race/ethnicity
What does gestational diabetes increase the risk of?
- Preeclampsia
- High birth weight
- Birth related trauma
- Jaundice
- Hypoglycemia
- Birth defects
Symptoms of diabetes
- 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
Dental considerations w/ diabetes
- 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
Hypothyroidism
Hypometabolic state caused by insufficient synthesis + release of thyroid hormone
Prevalence increases w/ age
Serum testing reveals high TSH + low free T4
What is the leading preventable cause of intellectual delay?
Hypothyroidism
Symptoms of hypothyroidism
- 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
Dental considerations for hypothyroidism
- 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
Hyperthyroidism
Hypermetabolic state caused by excess synthesis + release of thyroid hormone
TSH is suppressed, free thyroxine (T4) and triiodothyronine (T3) are elevated
What accounts for 96% of pediatric hyperthyroidism cases?
Graves disease
Symptoms of hyperthyroidism
- 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
Medical management of hyperthyroidism
- Antithyroid drugs
- Radioactive iodine
- Sx (thyroidectomy)
Hyperthyroidism dental considerations
- 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
What characterizes cystic fibrosis
Chronic pulmonary disease, pancreatic insufficiency, high sweat electrolyte concentrations
How is cystic fibrosis diagnosed?
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
Cystic fibrosis etiology
- 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
Cystic fibrosis symptoms
- 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
Dental considerations for cystic fibrosis
- 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
Partial seizures
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
Generalized seizures
40% - affects all areas of brain simultaneously; categorized as convulsive (tonic-clonic) or non-convulsive (absence)
Tonic-clonic seizures
- Aura or prodromal mood change
- Post-ictal phase (10-30min): atonia + incontinence
- Most common form of generalized seizure
Absence seizure
- Onset at ages 4-10yo
- May be misdiagnosed as behavior or learning problems
Side effects of anti-convulsive medications
- Xerostomia
- Stomatitis
- Dysgeusia
- Gingival hyperplasia
- Bone marrow suppression
- Decreased platelet count
- Mood/behavior changes
- Neurologic symptoms
- Depression
- Anorexia
- Infection
What should you ask regarding med hx for a patient w/ h/o seizures?
- 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
Dental considerations w/ seizures
- 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
Seizure management in dental office
- 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
When should mitochondrial disorders be considered?
- 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
Mitochondrial disorder etiology
- 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
Mitochondria disorder symptoms
- Variable depending on the organ system involved
- Seizure disorder
- Developmental delay
- Movement disorders
- Complicated migraine
- Stroke
MItochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS)
- Classic mitochondrial encephalopathy
- Variable clinical presentation
- May present at any age:
- Childhood: Nuclear DNA abnormalities
- Childhood or adult life: Mitochondrial DNA abnormalities
how are nuclear DNA gene defects inherited?
By autosomal recessive or autosomal dominant transmission
How are mitochondrial DNA defects transmitted?
By maternal inheritance
Mitochondrial disorder medical management
Supplementation w/ vitamins, co-factors (CoQ10) to increase ATP production
Oral findings with mitochondrial disorders
- Acid erosion related to GERD
- Dysfunctional swallowing
Dental tx considerations with mitochondrial disorders
- 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
What are the most common forms of neural tube defects?
Anencephaly + spina bifida
Neural tube defects: etiology
- Folic acid deficiency - most common
- Maternal insulin-dependent diabetes
- Maternal medications for epilepsy
- Maternal obesity
Neural tube defects: classification
- 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
Cranial forms of neural tube defects
- 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
Neural tube defects: clinical symptoms
- Hydrocephalus
- Spastic quadriplegia
- Microcephaly
- Ataxia
What is the most common type of spina bifida?
Spina bifida occulta
- Most mild form
- Outer part of some vertebrae are not completely formed
- Spinal cord intact
- No long-term effects
What is the least common type of spina bifida?
Meningocele
- Meninges pushed out through cleft in vertebrae
- Meningeal sac usually covered by skin
- Nerve damage atypical
- Few, if any, long-term side effects
What is the most severe form of spina bifida?
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
Arnold-Chiari II malformation
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
Dental considerations for neural tube defects
- 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
Hydrocephalus: congenital etiology
- Accounts for ~50% of all hydrocephalus cases.
- Developmental disorders (neural tube defects such as spina bifida)
- Arnold-Chiari malformation
- Craniosynostosis
- Genetic inheritance (aqueductal stenosis)
Hydrocephalus: acquired
- Head trauma
- Meningitis
- Tumors/cysts
“Sunsetting” is seen in what condition?
Hydrocephalus
Hydrocephalus: symptoms
- Rapid increase in head circumference (in infants)
- Vomiting
- Seizures
- “Sunsetting” = downward casting of eyes
- Sleepiness
- Irritability
- Blurred vision
- Inability to balance
Dental considerations for hydrocephalus
- 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
What is the most common motor disability in childhood?
Cerebral palsy
What type of cerebral palsy comprises most of the CP cases?
Spastic
- Spastic: contractures, lack of control (>75% of patients)
- Dyskinetic: slow, writing involuntary movements (15%)
- Ataxic: tremors or uncoordinated voluntary movements (5%)
- Mixed: combination
Diplegia
2 extremities
Hemiplegia
One side of the body
Monoplegia
One limb
Symptoms + associated conditions w/ cerebral palsy
- 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%)
CP treatment
- 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
Dental considerations w/ CP
- 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
Oral findings w/ CP
- 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
Elevated serum creatine kinase may be detected before clinical signs of what condition?
Muscular dystrophy
Classification of muscular dystrophy
- 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)
Medical management of muscular dystrophy
- 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
Oral findings w/ muscular dystrophy
- 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
Dental considerations w/ muscular dystrophy
- 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
What SHCN conditions may be more susceptible to dental trauma?
ADHD, seizure disorder, developmental/intellectual disability
Considerations of patients w/ SHCN w/ dental trauma management
- 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
Replantation of avulsed permanent teeth may be contraindicated for patients w/ which medical conditions
- Severe cardiac disease
- Severe seizure disorder
- Severe immunocompromised patients
- Severe developmental disability
SHCN definition
- 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.
What are the cornerstones of patient-centered health care?
- Flexibility
- Responsiveness
- Continuity
- Comprehensiveness
- Coordination
Down syndrome: dental manifestations
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
Down syndrome: treatment considerations
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
Other facts about Down syndrome
Increased risk with maternal age, 99% non-heritable
Increased risk after having one child with Down Syndrome
Cleidocranial dysplasia: dental manifestations
Delayed exfoliation
Delayed eruption
Impacted teeth
Hyperdontia (supernumerary) Peg like teeth
Taurodontism
Cleidocranial dysplasia: treatment considerations
Runx2 gene mutation
Other facts about cleidocranial dysplasia
Underdeveloped/absent clavicles
