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ABPD 2021 > Weebly Study Guide > Flashcards

Weebly Study Guide Flashcards

1
Q

Achondroplasia

A

Autosomal dominant
Short stature/extremities
Normal intelligence
Frontal bossing, midface hypoplasia, macroglossia
Dental crowding, missing teeth, open bite
May have atlantoaxial instability, difficult airway

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2
Q

ADHD

A

10% prevalence 4-17 years
Boys 2-3x more likely
Inattention, hyperactivity/impulsivity
Increased caries risk (poor OH)
Increased attrition (nail biting, chewing)
Increased trauma
Meds have side effects (bruxism, tachycardia, xerostomia)
Avoid tx on drug holidays, keep appt. short

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3
Q

Adrenal insufficiency (Addison’s disease)

A

Autoimmune or after infection
Salt craving, tiredness, weight loss, nausea, vomiting, diarrhea
Hyperpigmentation of body
Hyperpigmentation of mucous membranes, tongue, lips
Pt should take regular steroid dose before dental visit
Consult w/ endocrinologist for invasive tx

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4
Q

Allergic Rhinitis (hay fever)

A

IgE mediated alleric reaction
Rhinorrhea, sneezing, nasal obstruction, itchy eyes, cough, fatigue
Dolichofacial, allergic shiners
Malocclusion, high arched palate (mouth breathing)

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5
Q

Angelman Syndrome

A

Maternal genetic issue chromosome 15
Intellectual delay, sleep issues, microcephaly, hand flapping, speech issues, neurologic issues
Happy demeanor, hyperactivity
Microcephaly, prognathic mandible, widely spaced teeth, wide mouth, drooling, protruding tongue
Increased CRA, gingival hypertrophy (anticonvulsants)

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6
Q

Apert Syndrome

A

Autosomal dominant
Craniosynostosis
Syndactyly (mitten hands), high forehead, hearing loss, intellectual disability (sometimes)
Cleft palate sometimes, hypertelorism, flat nose
Dental crowding, class III, missing or extra teeth, shovel shaped incisors
May be difficult airway

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7
Q

Asthma

A

Chronic inflammatory disorder
Wheezing, cough, shortness of breath
Dolichofacial
Increased caries risk
Enamel defects
Increased gingivitis and periodontal disease risk
Malocclusion (mouth breathing)
Need to keep anxiety low (avoid triggering attack)
Higher risk for laryngospasm/bronchospasm in sedation
Avoid narcotics (meperidine) with sedation)
N2O not contraindicated in mild/moderate asthma

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8
Q

Autism Spectrum Disorder

A

Neurotransmitter abnormalities
More likely in male
41% intellectual disability
Impaired social interaction, impaired communication, presence of restricted/obsessive interests
OH may be poor and CRA higher
Consider desensitization visits

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9
Q

Beckwith-Wiedemann

A

Abnormal chromosome 11
Large size for newborn, poor feeding, seizures, increased tumor development
Large tongue, large eyes
Macroglossia may cause malocclusion
Difficult airway from tongue obstruction

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10
Q

Behcet’s Syndrome

A

Autoimmune disorder
Multi-system disease; mucocutanous ulcers, ocular disease, arthralgia, vascular disease, GI issues, neurologic issues
Recurrent aphthous ulcers
Increased caries, periodontal disease
Treated with steroids, immunosuppressants
Eye involvement may lead to blindness

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11
Q

Beta Thalassemia

A

Abnormal synthesis of hemoglobin
Failure to thrive, chronic fatigue, pale skin
Maxillary expansion, hypertelorism, frontal bossing
Iron deposits on teeth and in salivary glands (decrease salivary flow)
Increase caries, dental delay, “hair on end” alveolar bone, mucosal pallor
Burning tongue
Need hematology consult (PT/INR and ANC status)
Treated with RBC transfusion and iron; BMT is only definitive cure

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12
Q

Cardiac arrhythmias

A

Variation in heart rhythm
Caused by cardiomyopathy, CHD, infection, chemical imbalance, fever, medications, etc.
Most common type is atrial fib
Medications can cause oral issues (ulcers, xerostomia, taste, gingival hyperplasia)
May be on anticoagulants - need INR
Minimizing stress important

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13
Q

Celiac

A

Immune mediated
More in women
Stomach pain, diarrhea, weight loss, anemia, weakness, failure to thrive
Delayed tooth eruption
Atrophic glossitis
Enamel defects (esp. MIH)
Can have recurrent aphthous ulcers

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14
Q

Cerebral Palsy

A

Abnormal brain development in prenatal or perinatal period
Various classifications - spastic, dyskinetic, ataxic, mixed
Intellectual disability, speech issues, joint/muscle issues, respiratory issues, seizures
Periodontal disease, caries, malocclusions, constricted maxilla (hypotonia of tongue)
Oral motor dysfunction (poor swallowing with tongue thrust, bruxism, erosion, trauma, gag reflex, sialorrhea)

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15
Q

CHARGE

A

Autosomal dominant or de novo
Coloboma of eye
Heart defects
Atresia of nasal choanae
Restriction of growth
Genital or urinary issues
Ear abnormalities
Dental findings: clefts, micrognathia, taurodont, hypodontia, supernumerary, delayed eruption
Be concerned with airway issues, SBE prophylaxis

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16
Q

Chediak-Higashi

A

Autosomal recessive
Defect in lysosomal trafficking
Recurrent skin infections, neutropenia, neurologic degredation
Gingival bleeding, early exfoliation of teeth, periodontitis, aphthous ulcers
Need CBC prior to dental tx (ANC and platelet count)
BMT for treatment, platelet transfusions, splenectomy

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17
Q

Cleft Lip/Palate

A

Most common birth defect worldwide
CL: males; CP (only): female
Maxillary hypoplasia, supernumerary/missing teeth, enamel hypoplasia, taurodontism
Increased caries risk (abnormal salivary gland ducts)
Upredictable airway

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18
Q

Cleidocranial dysostosis

A

Autosomal dominant
Underdeveloped/missing clavicle
Other joint issues (scoliosis, loose joints, short limbs)
Brachycephaly, small upper jaw, wide nasal bridge, hypertelorism
Delayed exfoliation/eruption, impacted teeth, supernumerary teeth, malocclusion, taurodontism, enamel hypoplasia

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19
Q

Cockayne Syndrome

A

AKA progeria (accelerated aging)
Progressive neurologic dysfunction, short stature, failure to thrive, aged/wrinkled skin
Small mouth, enamel hypoplasia, high caries risk, crowding, missing teeth
Difficult airway

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20
Q

Congestive Heart Failure

A

Cardiac output is unable to meet demands
Fatigue, weakness, irritability, dyspnea, tachypnea with rales, chronic cough
Peripheral edema
Infection of mouth, petechiae, xerostomia (drugs)
Need MD consult
Minimize epinephrine
Prevent orthostatic hypotension
INR when on anticoagulant

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21
Q

Cornelia de Lange

A

Spontaneous mutation
Thick eyebrows, short nose, upturned nose,, long philtrum, thin upper lip, congenital diaphragmatic hernia
Developmental delay, short 5th finger
Seizures, heart defects, behavior issues
Small teeth, delayed eruption, dental erosion (GERD), malocclusion, missing teeth
Can have cleft palate

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22
Q

Cri du chat

A

High pitched cry
Lowbirth weight, physical and cognitive growth retardation/feeding problems
Cardiac issues
Microcephaly, short neck, hypertelorism, facial asymmetry, low set ears
Bifid uvula, malocclusions, anterior open bite, poor OH, enamel hypoplasia, chronic periodontitis, erosion
Behavior guidance/anticipatory guidance important (aggressive prevention)

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23
Q

Crohn Disease

A

Immune mediated
Slight female predilection; runs in families
Can occur any part of GI tract (oral, esophageal, intestinal)
Ulceration of mucosa; cobblestoning of mucosa
Increased risk of caries
Mucogingivitis, ulcers, deep linear ulceration
May have aphthous stomatitis

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24
Q

Crouzon Syndrome

A

Autosomal dominant
Tall forehead, proptosis (bulging eyes), hypertelorism, small airway
Most common type of craniosynostosis
Normal intelligence; some ID
High palate, clefts, hyperdontia/hypodontia, crowding, tooth morphology abnormalities

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25
Q

Cyclic Neutropenia

A

Spontaneous episodes of neutropenia recurring every 14-35 days, lasting 3-5 days
Cyclic fever, pain, malaise, ulcers, gingivitis
Aggressive removal of dental plaque/calculus needed; consider CHX during neutropenia
May need antibiotic coverage`

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26
Q

Cystic Fibrosis

A

Autosomal recessive
Chronic pulmonary disease and pancreatic insufficiency
Enamel hypoplasia and discoloration of permanent teeth (from abx)
Salivary gland involvement
Increased calculus, gingivitis
Decreased caries (may be from abx)
Increased candidiasis
Consult physician for dental tx, antibiotic prescription (to make sure it doesn’t interact with antibiotic for CF treatment)
Upright sitting position, short appointments, afternoon visits (coughing in morning)
Nitrous oxide contraindicated if emphysema

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27
Q

Diabetes

A

Type I: beta cells don’t produce insulin
Type II: body is immune to insulin
7% prevalence
Fruity odor on breath
Diminished salivary flow
Peripheral neuropathy
Gingivitis/periodontitis
Candidiasis
Delayed wound healing
Make sure HbA1c > 7; may need aggressive antibiotics in uncontrolled
Uncontrolled DM = NO ORTHO
Be ready for hypoglycemic events

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28
Q

DiGeorge Syndrome (22q11.2 deletion)

A

Cardiac anomalies
Hypoparathyroidism
Intellectual disability/Psych disorders
Asymmetric facies, small mouth, velopharyngeal insufficiency, cleft palate, wide eyes, flat nasal bridge
Microdontism, enamel aberrations, delayed eruption
Difficult airway; increased infection risk
*Most common cleft lip syndrome

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29
Q

Down Syndrome

A

Varying degrees of cognitive delay
Hypotonia, broad/short hands and fingers, cardiac defects 40%
Higher tendency towards leukemia, other infectious diseases, Alzheimer’s, hypothyroidism
Maxillary hypoplasia, flat back of head, small ears, short nose, upward slanting eyes
Increased risk atlantoaxial instability (no protective stabilization if so)
Increased periodontal disease, low level of caries, mouth breathing, malocclusion, delayed eruption, bruxism, tooth number issues, taurodontism
Airway obstruction issues due to macroglossia, large tonsils, atlantoaxial instability, hypotonia

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30
Q

Eating Disorders

A

Anorexia, bulimia, and binge eating disorder
Anorexia: hirsutism, amenorrhea, brittle nails, hypotension, muscular weakness, osteoporosis
Bulimia: normal range of weight, depression, dehydration, electrolyte imbalance, trauma to esophagus, Russell’s sign (hardened calluses over knuckles of hand)
BED: at least once a week over 3 months
All have increased dental caries risk; some dental erosion, perimolysis, enlarged salivary gland with BN
Encourage to rinse with alkaline solution after vomiting

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31
Q

Ectodermal Dysplasias

A

Over 150 syndromes with abnormalities of ectodermal structures
Most common is hypohidrotic ectodermal dysplasia
Sparse hair, thin hair, thin skin with decreased pigment, dry mucosa, small brittle nails, variable function of sweat glands, hypodontia to anodontia of teeth
Maxillary hypoplasia, pinched nose, thick lips, possible cleft
Teeth often conical/peg shaped, taurodont molars
Enamel hypoplasia possible
Prosth may be needed as early as 2 years of age with multiple replacements as child grows

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32
Q

Ehlers-Danlos

A

Defect in collagen
Flexible joints, stretchy skin that bruises easily
Hypermobility type, Vascular type, Kyphosis type, Arthrochalasia type, Cardiac Type, or Periodontal Type
Slender and asymmetric face, hypertelorism, short nose, low ears, high palate
Periodontal disease, TMJ issues, thin mucosa, enamel hypoplasia, pulp stones and malformed roots
Avoid fluoroquinolones
Be prepared for hemorrhage and test coagulation prior to procedures
Ortho relapse common

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33
Q

Epidermolysis Bullosa

A

Simplex, Junctional, Dystrophic, and Kindler
Issues with keratin causing blistering in response to mechanical trauma or heat
Risk for infection due to open wounds, contracture/mitten deformities, malnutrition and dehydration
Bullae, scarring, dry skin and lips with hair loss and blindness occasionally
Scarring may lead to limited mouth opening
Enamel hypoplasia and hypomineralization
Periodontal disease, oral blisters
Use lubrication with dental instruments and minimally stretch or touch tissues
Avoid blocks with LA
Difficult intubation; but most dental treatment needs to be done under GA if invasive

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34
Q

Epilepsy

A

Disruption in neuron activity in the brain
Acute symptomatic seizure: occurs with systemic or brain insult
Unprovoked seizure; status epilepticus: seizures prolonged or immediately recurrent without return to consciousness
Partial: most common type; affects only part of brain, variable presentation
Generalized: affects all of brain; absence, atonic, myoclonic, tonic-clonic
Increased risk of dental caries due to medication-related xerostomia
Medication-induced gingival hypertrophy
Trauma risk
Verify pt has medications with them, avoid xylocaine and meperidine, avoid seizure triggers (light, stress, etc.)

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35
Q

End Stage Renal Disease (ESRD)

A

Chronic, irreversible progressive disease characterized by destruction of 50-75% of nephrons
Requires long-term dialysis or kidney transplant
Caused by congenital anomalies, glomerulonephritis, DM, HTN
Fatigue, malaise, loss of appetite, failure to thrive, HTN, edema, frequent urination
Xerostomia, metallic taste, anemia, petechiae/ecchymosis, enamel hypoplasia, rootless teeth, gingivitis, parotitis
May require Abx prophylaxis
Defer tx if not controlled (ANC < 1000/mm3, PLT < 75k/mm3); if well-controlled no contraindications for dental tx
If transplantation is planned, follow normal transplantation guidelines
Avoid dental tx day before dialysis
Systemic fluoride not recommended due to fluoride retention
Avoid meds metabolized by kidneys (NSAIDs, some Abx)

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36
Q

Fetal Alcohol Spectrum Disorder

A

Growth deficits, joint and bone anomalies, intellectual disability, seizures
Can have cardiac defects and renal defects
Underdeveloped/absent philtrum, thin vermillion border, small head, hypoplastic mandible, hypertelorism, short nose, flat midface, cleft lip/palate
Malformed, missing, misaligned teeth
Delayed dental development
May need SBE, may need to avoid drugs processed by renal system, need good behavior management

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37
Q

Fragile X

A

Most common cause of inherited intellectual disability
Seizures, mitral valve prolapse, flat feet, HTN, hearing issues, autism/ADD
High palate, elongated face, macrocephaly, strabismus, large ears
Malocclusion, supernumeraries
May need sedation for tx due to behavior
No N2O if active otitis media

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38
Q

Gardner Syndrome

A

Mutation in chromosome 5 tumor suppressor gene
Multiple polyposis of large intestines with high malignant potential
Osteomas, sebaceous cysts, fibromas, lipomas, other lesions
Increased prevalence of thyroid carcinoma
Compound odontomas, hypodontia or hyperdontia, multiple odontomas (esp. in mandible and outer cortex of skull)
Increased density of bone slows ortho movements
Need early screening for cancers; NSAIDS may reduce number of polyps formed

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39
Q

GERD

A

Various causes (weak sphincter, hernia, obesity, pulmonary disorders, epilepsy, CP, Down syndrome, prematurity)
Vomiting, heartburn, poor weight gain, feeding refusal, nocturnal cough/pain, sore throat
Dental erosion with enamel loss, dry mouth (medications), impaired taste
Increased risk of caries
Consider ortho extrusion if needed and full coverage restorations

40
Q

Hallermann-Streiff Syndrome

A

Hypotrichosis, hypoplastic ribs/clavicles, small nares, congenital cataract, narrow upper airway
Brachycephaly with frontal bossing, bird-like face, glossoptosis, microphthalmia
Hypodontia, delayed exfoliation/eruption, natal teeth/neonatal teeth, enamel hypoplasia, class II malocclusion
Difficult airway due to micrognathia; can have sleep apnea

41
Q

Hemophilia

A

A: factor VIII; B: factor IX
Most common in males (x-linked recessive)
Spontaneous bleeding, easy bruising, hematuria
Oral bleeding, ulcerations, ecchymosis, poor OH with cavities and gingival disease
Caution with IAN or lingual infiltration (hematoma risk)
Endo treatment preferred over extraction
Use rubber dam to protect soft tissues
Consult with hematologist; be ready for hemostasis
May need desmopressin (DDAVP), Amicar, factor replacement, etc.
Avoid NSAIDs and aspirin

42
Q

HIV/AIDS

A

Target CD4 lymphocytes, leading to increased risk for opportunistic infection
Early childhood usually from maternal transmission; may see arthralgia, fever, headache, lymphadenopathy, recurrent bacterial infections, candidiasis
Gingivitis (can have NUG), fungal infections, viral infections (EBV), bacterial infections, neoplasms (Kaposi’s sarcoma)
If neutrophil <500 cell/mL; CD4 cell counts <100/mm3 defer treatment and consider antibiotic prophylaxis
May process drugs differently due to antiretroviral therapy (OCS risk)

43
Q

Hypoplastic Left Heart Syndrome

A

Unknown etiology; an be part of syndrome (Holt-Oram, Rubinstein-Taybi)
Underdeveloped left ventricle of heart resulting in inability to support cardiac output needed
Pounding heart, weakened pulse, cyanosis, failure to thrive, fatigue
Need for SBE
Cardiac meds may cause xerostomia, gingival hyperplasia
Several heart surgeries required

44
Q

Hyperthyroidism (Grave’s disease, Plummer’s disease)

A

Increased metabolism, arrhythmias, intolerance to heat, increased appetite, weight loss
Goiter = enlarged thyroid; Graves = exophthalmos
Increased caries/perio susceptibility, early exfoliation/eruption, osteoporosis, burning mouth syndrome
Consult with endocrinologist, ensure thyroid collar with radiographs, caution with hemostasis (avoid NSAIDS), avoid epinephrine in untreated or poorly controlled
Thyroid storm: 911, ice, hydrocortisone 100-300mg, IV glucose, CPR

45
Q

Hypothyroidism

A

Can be congenital (cretinism) or acquired (iodine deficiency), or autoimmune (Hashimoto’s)
Slow metabolic rate, weight gain, lethargy, intolerance to cold, slow HR, delayed wound healing
If not treated in 6 months, short stature, intellectual disability, low muscle tone, hearing loss, respiratory distress
Coarse facial features, hair loss, large head with small jaws
Macroglossia, delayed dental eruption, enamel hypoplasia, salivary gland enlargement, malocclusion, impacted 2nd molars

46
Q

Idiopathic thrombocytopenic purpura (ITP) and Thrombotic Thrombocytopenic Purpura (TTP)

A

Platelet count of <150mil/L
ITP is primary immune, TTP is congenital
More in women
Petechiae/ecchymosis
ITP: increased risk of bleeding from mucous membranes, hematuria
TTP: thrombocytopenia, hemolytic anemia
Nose bleeds, easy bruising, excessive bleeding after surgery
Don’t use NSAIDs
Perform elective dental treatment only w/ platelet count above 50k; be ready for hemostasis
Do not block for LA

47
Q

Juvenile Arthritis

A

Autoimmune disorder
Persistent fever for at least 2 weeks and arthritis for at least 6 weeks in child less than 16
Joint swelling, joint pain, persistent loss of function and stiffness; usually affects knees, hands and feet
Worse in morning
Untreated TMJ may cause growth disturbances
Increased risk for dental caries
May have difficulty opening or holding toothbrush for OH
Steroid use may lead to adrenal cortical insufficiency (consider supplemental steroids)

48
Q

Kabuki Syndrome

A

“Make up” appearance of face
Developmental delay, possible seizures, spina bifida, short fifth fingers, heart abnormalities
Microcephaly, CLP, long palpebral fissure, strabismus, arched eyebrows, prominent ears, downturning corners of mouth
Missing teeth, misaligned or misshaped teeth (microdontia), class III malocclusion
Cardiac consult recommended
Difficult airway
Aggressive caries plan

49
Q

Kawasaki Disease

A

Vascular - conjunctivitis, rash, adenopathy, strawberry tongue, swelling hands/feet
Fever more than 5 days
Vasculitis can lead to coronary artery disease
Self-limiting - lasts about 12 days without treatment
MSIC mimics Kawasaki after COVID infection
Treated in hospital with IVIG (reduces cardiovascular complications)

50
Q

Klinefelter Syndrome (XXY)

A

Less testosterone, slow puberty, infertility, gynecomastia
Increased endocrine and autoimmune disease risk
Reduced muscle tone, weaker bones, low energy, ADHD, learning difficulties
Mandibular prognathism, larger teeth, taurodont molars, missing teeth
High caries rate
Pulp exposures due to taurodontism
May be difficult to manage behavior

51
Q

Langerhans Cell Histiocytosis

A

Neoplastic disease of myeloid cells
Classic triad: punched out radiolucency in membranous bones, exophthalmos, diabetes insipidus
Radiolucent lesions with ill-defined borders
Can have pain and swelling of the face and lymph nodes
Gingival ulcerations/lesions are associated with adjacent bone involvement
Floating teeth may occur
(premature exfoliation)
Treated with chemotherapy, bisphosphonates, surgery, radiation, etc. - may need to consult with heme/onc

52
Q

Lesch-Nyhan

A

Self-mutiliation
Issue with purine metabolism
Almost exclusively in males (X-inked recessive)
Kidney stones, renal failure, gout
Intellectual disability, CP, dystonia, chorea
Microcephaly, distorted facies, atrophic/scarring of lips from self-chewing
Enamel hypoplasia
May need protective stabilization (relaxed when restrained)
May need extraction of teeth for self-injury prevention
Poor life expectancy

53
Q

Leukemia

A

Most common cancer in children
Higher incidence in pt with Down Syndrome (esp. AML)
ALL: most common in kids
AML: difficult to treat
CLL: most common in adults
CML: three phases, can have Philadelphia chromosome
Gingival bleeding, gingival hypertrophy, ulcers, opportunistic infections, oral pain, enlarged lymph nodes
After therapy: mucositis, microdontia, agenesis of teeth, morphology issues, root disturbances, xerostomia, GVH disease
Try to complete dental tx prior to cancer therapy
ANC > 1000/mm3

54
Q

Leukocyte Adhesion Deficiency

A

Impaired wound healing and frequent soft-tissue infections
Three types, different issues with leukocyte adhesion
Severe gingivitis and rapidly progressing juvenile periodontitis
Rapid bone loss around teeth
Complete loss of permanent teeth by late adolescence
Oral ulcerations that are slow healing and scarring
May. need additional antibiotic therapy for dental tx
Rigorous OH including CHX rinses is essential

55
Q

Li-Fraumeni Syndrome

A

Cancer predisposition syndrome
Early onset of cancers (breast, skin, etc.)
Soft tissue sarcomas, bone sarcomas, and brain tumors
Most common cancer for kids with LFS to have is soft tissue sarcomas and bone sarcomas
Careful with radiation to reduce risk of secondary radiation induced malignancies

56
Q

Lymphoma

A

Uncommon before 5
Hodgkins more common in adolescents; Non-Hodgkin more common in children
Lymphadenopathy, splenomegaly, hepatomegaly, fever, night sweats, weight loss
Swelling is nonpainful; bone loss can mimic periodontal disease
Can have trouble swallowing
Complete dental tx prior to medical tx

57
Q

Malignant Bone Tumor

A

Most common site of metastasis is lungs
Osteosarcoma: most common bone cancer in children, rapid growth, pain, poorly defined bone destruction lesion with mixed radiographic appearance (sunray); in maxilla usually is in posterior region, in mandible usually is in body and angle of ramus
Ewing Sarcoma: several small round blue cell tumors, arises equally in bones of extremities and other parts of body; in mouth, mandible more common
Try and do dental treatment prior to cancer treatment
The earlier treatment occurs with less metastasis, the better prognosis

58
Q

Marfan Syndrome

A

Chromosome 15, autosomal dominant
Vision issues, cardiovascular abnormalities
Tall with long, thin arms and legs, spider-like fingers, funnel chest or pigeon breast, flat feet, hypotonia
Asthma, sleep apnea, sudden lung collapse
Flexible joints, scoliosis
Dolichocephalic face, deep palate, small lower jaw, TMJ issues
Gingivitis and periodontitis, flat molars, crowded teeth, abnormal pulp chambers
SBE often needed
Avoid fluoroquinolones

59
Q

McCune-Albright

A

3 hallmark signs: Endocrine dysfunction, Cafe-au-lait spots, Polyostotic fibrous dysplasia
90% polyostotic lesions occur in bones of skull and jaw -> multilocular radiolucent lesions, asymmetrical
Often “ground glass” radiographically
High caries risk, can have oral pigmentation, oligodontia, taurodontism
Treated with bisphosphonates

60
Q

Multisystem Inflammatory Syndrome in Children (MIS-C)

A

Infection with COVID within 2-6 weeks prior to symptom onset
Inflammation of organs/tissues
Kawasaki-like and toxic-shock-like symptoms
Conjunctivitis, rashes, adenopathy, strawberry tongue, swollen hands or feet or red
Refer to hospital if MIS-C is suspected
Defer elective dental care; may need to get platelet values and use steroid therapy
Treatment is supportive care, autoinflammatory care (IVIG)
Can result in long-term cardiac compromise, kidney issues, liver injury

61
Q

Moebius Syndrome

A

Affects CN6 and CN7; other cranial nerves may also be affected
Inability to suck at birth
Limitations with eye movement and facial expression, difficulty speaking
Autism in 30%
May have lip paralysis, hypoplastic mandible, hypoplastic tongue, CLP, deformity of tongue/ear/jaw
Open bite, crowding, missing teeth, CLP
Increased caries risk (less food clearance due to lack of tongue movement)
Risk for aspiration

62
Q

Mucopolysaccharidoses

A

Includes Hurler (I), Hunger (II), and Sanfilippo (III) and others
Group of lysosomal storage disorders
Multi-organ involvement, short stature, skeletal, joint, airway and cardiac abnormalities
Intellectual disabilities
Claw-like hands, joint stiffness
Coarse facial features, large head with enlarged lips and mouth and tongue
Hyperplasia of alveolar ridge and gingival hypertrophy
Malocclusions
Enamel hypoplasia, taurodontism, impacted teeth; large root chambers and short roots (easy for abscess formation)
OCS and protective stabilization contraindicated due to high BMI, limited neck movement, and macroglossia/small airway

63
Q

Muscular Dystrophy

A

Several types; Duchenne most common in boys
Progressive muscle weakness
Can lead to inability to walk, breathing problems, scoliosis, decreased heart efficiency, swallowing problems
Can have malocclusion, delayed eruption, missing teeth
May receive treatment in outpatient setting if mild
Consult with PCP to determine stability and involvement of other body systems
Risk for malignant hyperthermia with GA
Contraindicated for OCS (aspiration risk)
May require aid with OH

64
Q

Neural Tube Defects

A

Birth defects of brain, spine, spinal cord due to complications in 1st month of pregnancy
Risks: poorly controlled diabetes, obesity, not enough folic acid, anti-seizure meds
Spina bifida is most common, most mild form
Myelomeningocele is most severe form
Others: anencephaly, encephaloceles, hydrancephaly
Commonly associated with CLP
Increased caries risk due to poor diet, poor OH and chronic syrup-based medications
Ensure comfortable positioning to avoid pressure wounds
Screen for latex allergy and other food cross-reactivities

65
Q

Neurofibromatosis

A

Includes NF1, NF2 and Schwannomatosis
NF1: birth/infancy onset; pigmentation, neurofibromas on skin/brain/other, cafe-au-lait spots, increased risk for cancers, HTN, seizures, ADHD, pheochromocytoma, nodules in eyes can lead to vision loss
NF2: teens/early 20s onset; benign tumors on auditory nerve, cataracts, hearing loss, balance issues
Oral findings: neurofibromas, gingival hyperplasia, impacted, SN or missing teeth, macroglossia
Increased risk of dental caries
Minimal issues delivering care to mildly affected pt
SBE may be needed if cardiac issues persist

66
Q

Noonan Syndrome

A

Short stature, cardiac abnormalities, delayed puberty
Most have normal intelligence
Wide-set eyes, short neck, webbed neck, low-set ears, low hairline, macrocephaly
High palate, malocclusion, micrognathia
Be aware of potential bleeding disorders
May need SBE for cardiac conditions

67
Q

Oculo-Auriculo-Vertebral Spectrum

A

Goldenhar is most severe form
Defects of 1st and 2nd branchial arches
Symptoms commonly unilateral
Pre-auricular skin tags, CNS anomalies, hearing issues, colobomas, cardiovascular malformations, autism, renal malformations
Hemifacial microsomia in 60% of cases
Cleft palate more common than cleft lip
Can have small, misshaped, or missing ramus, condyle and TMJ
Supernumerary teeth, enamel and dentin malformations, gingival hypertrophy, class II malocclusion
Can have difficult airway
Intellectual disability can impact OH

68
Q

Orofacial-Digital Syndrome

A

Syndactyly/brachydactyly/polydactyly/clinodactyly
Polycystic kidney disease (type I), bifid hallux (type II), neurological problems, heart defects
Median cleft lip/cleft palate/cleft tongue, micrognathia, high palate, tumors of tongue, wide nose, hypertelorism, alopecia
Missing mandibular lateral incisors, supernumerary teeth, enamel dysplasia, malocclusion
Beware of drugs metabolized by kidney
If otitis media, defer N2O

69
Q

Osteogenesis Imperfecta

A

Defective collagen causing loose joints and hypermobility
Commonly mistaken for child abuse
Bowing of long bones, scoliosis, multiple fractures without cause
Can have hearing loss, respiratory problems
19 types; type 1 most common, type 2 lethal in-utero, type 3/4 associated with DI
Blue sclera, triangular face, wormian bones in skull on radiographs, prognathic mandible
Occurs with dentinogenesis imperfecta; primary teeth more severely affected
Consider full coverage restorations for DI
Extractions may be contraindicated if bisphosphonates used
Protective stabilization contraindicated
RCT contraindicated if bisphosphonate

70
Q

Papillon-Lefevre

A

Dry scaly patches on palms of hands and soles of feet at birth
Nail dystrophy
Sparse hair
Gingivitis and periodontitis
Deciduous teeth exfoliate by 4-5; permanent teeth lost by age 17
Radiographs show teeth floating in air
Early extractions/bone grafts with prosth treatment
No contraindication to N2O, OCS or GA or protective stabilization
Systemic antibiotics may slow periodontitis; traditional periodontitis therapy may not make a difference

71
Q

Peutz-Jeghers Syndrome

A

Gastrointestinal polyposis, mucocutaneous pigmentation, cancer predisposition
Small, dark macules present on the lips, inside and around the mouth, near eyes and nostrils that appear during childhood and fade with age
Polyps in GI system can cause obstructions, chronic bleeding, abdominal pain and can have malignant transformation
Caution using N2O due to potential bowel obstruction

72
Q

PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis)

A

Autoinflammatory disease
Onset by 5, resolves by 10 usually (can go into adulthood)
Predictable 3-6 day episodes of fever, 1 or more of other 3 symptoms every 21-28 days like clockwork
-minor aphthae
-pharyngitis
-cervical adenitis
Palliative care for aphthous ulcers; can consider use of systemic or topical steroids
Postpone elective dental care when pt has PFAPA episode

73
Q

Pfeiffer Syndrome

A

Clinodactyly - broad thumbs and big toes that bend away from other digits
Hypertelorism, finger and toe abnormalities, hydrocephalus, underdeveloped midface
Often normal intelligence
Craniosynostosis, proptosis, small nose, high forehead, OSA
Dental crowding, natal teeth sometimes
Difficult intubation and airway

74
Q

Pierre Robin Sequence

A

Micrognathia, cleft palate, glossoptosis
Comorbidities include GERD, Stickler syndrome, trisomies, CHARGE, Moebius, velocardiofacial syndrome, Treacher-Collins among others
Middle ear infections common
Can also have respiratory or cardiac problems
80% have cleft palate
Poor development of mandible
Severe class II malocclusion, crowding, natal teeth, hypodontia, erosion from GERD
Difficult to intubate
Treatment includes distraction osteogenesis, positive airway pressure, tracheostomy, tongue-lip adhesion, obturators, ear tubes, speech therapy

75
Q

Prader-Willi

A

Paternally derived chromosome 15 (Angelman is maternal)
Initial failure to thrive reversing to significant weight gain and obesity
Hypothalamic hypogonadism leads to infertility, growth hormone insufficiency and intellectual disability with behavioral issues
Cardio-respiratory problems due to severe obesity
Prominent forehead, strabismus, narrow face with small mandible, downturned mouth with thin upper lip
Abundant and thick saliva, increase in gingivitis and candidiasis
Enamel defects and erosion from GERD and bruxism
High caries risk
Crowding
Behavioral problems are common

76
Q

Proteus Syndrome

A

Extremely rare (100-200 people ever reported in literature)
Excessive growth of a part or portion of the body that is usually asymmetric and unilateral
Neurologic abnormalities including ID, seizures, vision loss, hearing loss
Cerebriform connective tissue nevus, linear epidermal nevus, asymmetric overrowth of limbs, skull, lipomas, vascular malformations
Dolichocephalic with down-slanting palpebral fissures, macrocephaly
Increased risk for salivary gland tumors
Impacted teeth, malocclusion, ectopic eruption, asymmetric enamel hypoplasia, missing teeth, asymmetric macroglossia
May need antibiotic prophylaxis due to multiple orthopedic procedures (?)
IAN/anesthesia landmarks may be different due to overgrowth

77
Q

Rett Syndrome

A

Almost exclusively in girls
Normal development until 6-18 months of life, followed by regression in speech and developmental milestones
Causes problems in brain function
4 criteria: partial/complete loss of purposeful hand skills; partial/complete loss of acquired spoken language, gait abnormality, stereotypic hand movements
Comorbidities include prolonged QT, arrhythmias, seizures, ID
Associated with bruxism, mouth breathing, digit sucking, high risk for dental trauma, self-injury, difficulty with mastication
Beware of diminished response to pain when diagnosing dental conditions
More frequent recalls

78
Q

Rubinstein-Taybi

A

Short stature, broad thumbs and big toes, intellectual disability, hyperflexible joints, bent pinky finger
Increased risk for malignant and benign tumors
Microcephaly, convex nasal ridge, low-set ears, small oral opening, bifid uvula, cleft palate
Dental crowding, malocclusion, enamel erosion due to GERD
Talon cusps
Enamel hypoplasia, screwdriver permanent incisors
Advanced behavior guidance techniques usually needed

79
Q

Sanfilippo Syndrome (MPS Type III)

A

Lysosomal enzyme deficiency
Initial signs start at 2-6 years and marked by progressively declined function with mild dementia first, followed by loss of motor function
Developmental delay, uncontrollable hyperactivity and aggressive behavior
Mild coarse facial features with frontal bossing, broad and thick eyebrows, depressed nasal bridge
Decreased mobility of TMJ
Higher prevalence of caries, gingival hyperplasia and gingivitis
May have excessive pharyngeal tissues, making intubation difficult

80
Q

Sickle Cell Disease

A

Autosomal recessive
Defect in hemoglobin (HbS) leading to sickled RBCs
Anemia, repeated infections, jaundice, pulmonary hypertension
Sickle cell crisis: acute pain/joint pain from occluded vessels leading to organ damage
Malocclusion with class II tendency
Petechiae, hematomas, mucosal pallor, delayed tooth eruption, spontaneous gingival bleeding, increased periodontitis risk
Radiographic expansion of bone marrow and loss of trabeculation
Diastemas due to expansion of bone
Prophylaxis similar to AHA guidelines
Paresthesia risk with >50% N2O
If Hg is <7g/dL or <20% Hct, may need transfusion
Dental treatment should be conservative and “stress free”
Often treated with hydroxyurea but BMT is only cure

81
Q

Systemic Lupus Erythematosus

A

Autoimmune disease varying from full-blown severity to chronic low-grade symptoms
In children most common presentation is persistent fever, malaise, failure to thrive
Anemia, leukopenia, arthritis or arthralgia, nephritis, abdominal complaints can occur
Classic malar “butterfly” rash only in 1/3 of patients
Can have naso-oral ulcers
-ulcers can be aphthous, honeycomb, erythematous palatal ulcers, or lupus cheilitis on lips
Sjogren’s syndrome, burning mouth and xerostomia potential secondary complications
Need blood lab values prior to invasive treatment
Oral ulcers may indicate a flare is imminent
Consult rheumatologist to determine need for antibiotic prophylaxis due to increased risk for infection

82
Q

Soft Tissue Sarcomas

A

Arise from primitive mesenchymal tissue; 10% in head and neck
Rhabdomyosarcoma: most common soft tissue sarcoma, tumor of striated muscle; presentation ranges from small cutaneous nodule on face to extensive fast-growing facial swelling
Ewing Sarcoma (soft tissue): mainly occur on trunk and extremities but some in head and neck
Can see radiographic displacement of teeth
Need to time treatment with regard to cancer treatment modalities (consult with Hem/onc)

83
Q

Sotos Syndrome

A

Prenatal and postnatal overgrowth
Learning disabilities
Delay in early developmental milestones
Large hands and feet
Broad, prominent forehead and jaw with sparse frontotemporal hair, dolichocephaly, hypertelorism, malar flushing
High arched palate, dental crowding, deep bite, missing teeth, natal teeth
Be aware of potential heart and kidney issues
May also have scoliosis, eye issues, HTN

84
Q

Stickler Syndrome

A

Progressive connective tissue disorder with eyes, ears, skeleton and hypermobile joints
Intelligence usually normal
Early onset degenerative arthritis
May have mitral valve prolapse
Pierre-Robin Sequence
Flat face, micrognathia, glossoptosis, split uvula, prominent eyes, low set ears
Malocclusion, clefts, enamel hypoplasia
Difficult airway/intubation

85
Q

Sturge-Weber

A

Unilateral excessive blood vessel growth on brain causing calcification and atrophy of cerebral cortex nerves
Headaches, seizures, paralysis
Port-wine stain
Intellectual disabilities in half, ADD, mood disorders, poor social skills
Hypothyroidism
Can have ipsilateral gingival hemangiomas intraorally that blanch under pressure
Early eruption of teeth from increased vascularity
Gingival hyperplasia from anticonvulsants
Can cause unilateral macroglossia and maxillary bone hypertrophy
Hemostasis can be challenging
GA with oral intubation, avoid deep sedation due to difficulties in airway maintenance
Stress can rupture hemangiomas

86
Q

Sympathetic Nervous System Tumor

A

Neuroblastoma more than 97% of sympathetic nervous system cancers
Neuroblastoma arises in immature nerve cells of SNS
Most common cancer of infants <1 year
Most common location is adrenal glands
Following therapy (similar to other cancers): tooth development/root shunting, enamel hypoplasia, high caries, mucositis, xerostomia, bleeding, changes in taste
Consult with hem/onc

87
Q

Tetralogy of Fallot

A

VSD, pulmonary stenosis, overriding aorta, and right ventricular hypertrophy
Most common cyanotic congenital heart defect
“Tet” spells in unrepaired ToF (paroxysmal life-threatening episodes of cyanosis, rapid/deep breathing, syncope)
Enamel defects/hypoplasia
Stomatitis, cyanotic mucous membranes and tongue
Delayed tooth eruption
Need consult with cardiology (SBE, anticoagulant therapy, etc.)

88
Q

Transposition of great arteries

A

Idiopathic etiology
2nd most common cyanotic congenital heart defect
50% also present with VSD
Signs of CHF (tachypnea, dyspnea, pounding heart, cyanosis, weak pulse)
Cyanotic oral tissues
MD consult to determine SBE and cardiac tolerance to invasive procedures

89
Q

Treacher Collins

A

Bilateral 1st and 2nd branchial arch development abnormalities
Hypertelorism, down-slanting palpebral fissures, depressed cheekbones
Mandibular retrognathia
Large mouth
Coloboma, sparse eyelashes
Cleft in zygoma, palate, lip
Dental anomalies - tooth agenesis, enamel deformities, widely spaced teeth
Class II malocclusion, micrognathia
High airway risk (sedation and N2O contraindicated)

90
Q

Tuberculosis

A

Chronic granulomatous disease of lungs caused by various strains of mycobacteria
Chronic cough lasting 3 weeks or longer, coughing up blood, weight loss/failure to thrive, fever for 2 weeks
Oral lesions may include ulcers, nodules, tuberculomas, periapical granulomas, osteomyelitis
Ulcers most common in mouth - usually painless
Defer treatment when active/contagious TB is present

91
Q

Turner Syndrome

A

XO
Broad chest, low hairline, obesity, webbed neck
Thyroid disorders, heart conditions common
Higher incidence of diabetes, osteoporosis
Most normal intelligence
Malocclusion, crowding of teeth due to small jaw and narrow high palate
Early eruption of permanent teeth
Thinner enamel
Shallow roots
Need to assess cardiac risk for SBE and assess for obstructed airway

92
Q

Ulcerative Colitis

A

Immune-mediated inflammatory disease involving granulomatous inflammation and ulcers of large intestine (IBS)
Persistent diarrhea with loose, urgent bowel movements, blood in stool, abdominal pain, abdominal mass
Fever, fatigue, anemia, appetite and weight loss, growth failure
Similar to Crohn Disease (limited to the large intestine and colon where Crohn’s is not)
Increased risk of caries and oral infections
Glossitis, cheilitis, cobblestoning appearance of oral mucosa
Recurrent aphthous stomatitis
Avoid NSAIDs and aspirin (can cause flare ups)
Palliative care for oral ulcers

93
Q

VATER/VACTERL Association

A

Non-random cluster of congenital malformations (at least three of the following):
-Vertebral defects
-Anorectal anomalies (anal atresia)
-Cardiac defects
-Tracheo-esophageal fistula
-Renal anomalies
-Limb abnormalities
Variable dysmorphic facial features but no classical signs
Can have bilirubin discolored teeth, gingival overgrowth, calculus
MD consult to determine stability prior to dental tx including need for SBE

94
Q

Vitamin C Deficiency (Scurvy)

A

Risk factors: babies on only cow’s milk, low SES, smokers, GI tract disorders, type 1 DM, patients on hemodialysis, pt with malabsorption, and pt with psychiatric disease resulting in poor nutrition
Corkscrew hairs, perifollicular hemorrhage and gingival bleeding
Initial presentation after 8-12 weeks of deficiency
4 H: Hemorrhage, Hyperkeratosis, Hypochondriasis, Hematologic abnormalities
Poor wound healing, bone loss, stomatitis

95
Q

Vitamin D Resistant Rickets

A

X linked mutation in phosphate reglating gene that disrupts vitamin D metabolism, resulting in low vitamin D levels
Short stature, slow growth, bowing of lower extremities, joint/bone pain, bone deformities and fractures
Lab values include hypophosphatemia, normal calcium, normal to high PTH, normal to high alkaline phosphatase, normal or slightly reduced vitamin D
Craniosynostosis, dolichocephaly and frontal bossing
Thin, hypomineralized enamel and dentin with enlarged pulp chambers and pulp horns
-spontaneous or attrition-caused pulp necrosis + abscess
-periapical radiolucencies
Early loss of teeth
Aggressive preventive dentistry is necessary

96
Q

Williams Syndrome

A

Cardiac abnormalities
Joint problems, soft and loose skin, low muscle tone
Short stature, overly friendly and social, DD/learning disability
Sensitive hearing, difficulty with visual
Phobias common
Small upturned nose, broad forehead, wide mouth, full lips, small chin, puffy eyes
Small, widely spaced teeth, missing teeth, taurodontism, pulp stones, enamel hypoplasia
Caution when administering LA as sudden death has occurred
Consider headphones/earplugs due to sensitive hearing
Avoid vitamin D and calcium supplements
May have malignant hyperthermia with GA
SBE may be needed for cardiac anomalies

97
Q

Wolff-Parkinson White

A

Atypical electrical activity of heart
Shortened PR interval and widened QRS complex
Majority are asymptomatic but can have symptomatic arrhythmias with lightheadedness, dizziness, syncope, chest pain, sudden cardiac arrest
No evidence of specific dental manifestations but some antiarrhythmics can cause oral manifestations
Verapamil, enalapril and diltiazem can cause gingival hyperplasia
Need to keep the patient relaxed and use good behavior management techniques to avoid increasing heart rate
Not considered SBE requirement, but may want to consult with cardiology
Avoid use of some electrical stimulating equipment (piezo-surgery, radiofrequency scalpel, etc.)

ABPD 2021 (14 decks)

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