SHCN Flashcards
Definition of SHCN
Any physical, developmental, mental, sensory, behavioral, cognitive or emotional impairment or limiting condition requiring medical management, health care intervention and/or use of specialized services
AAPD Policy on Transitioning Care
Specific transitioning planning should begin 14-16 years
Dentistry is most common category of unmet health needs
Guideline on management of patients with SCHN
Main recommendation is preventive strategies
Education of parents, sealants, fluoride, etc.
Lesch-Nyhan Syndrome
X-linked trait
Affects purine metabolism = high uric acid
Intellectual disability, speech articulation
Self-mutilative behavior
Hurler, Hunter Syndromes
Mucopolysaccharidosis type I (Hurler) and II (Hunter)
Build up of glycosaminoglycans
Claw hand, mental decline, heart valve problems, abnormal bones, coarse features
Macrocephaly, large tongue, wide spacing of teeth
Sturge-Weber Syndrome
Port-wine stain Seizures Bleeding issue with treatment Early eruption of teeth due to increased vascularity Hemorrhage from angiomas Some intellectual disabilities
Neurofibromatosis
Tumors of nervous system Wide inferior alveolar canal Enlarged fungiform papilla Oral neurofibromas Malpositioned teeth Small cafe au lait spots
Rubenstein-Taybi Syndrome
Heart defects Broad toes, thumbs Excess hair Intellectual disability Seizures Thin upper lip Talon cusps Crowding High caries
Treacher Collins Syndrome
Small mandible, short ramus/absence of ramus, glenoid fossa, TMJ Difficult oral intubation Normal intelligence Facial nerve may be affected Malocclusion Tooth agenesis Ectopic eruption of first molars
Pierre Robin Sequence
Small lower jaw
Cleft palate
Difficulties breathing and feeding
Glossoptosis (associated with airway obstruction)
Not candidates for oral sedation in office
Rett Syndrome
Disorder of brain and nervous system Normal development for 6-18 months Floppy arms and legs, apraxia Excessive saliva/drooling Loss of social engagement Intellectual disability Seizures Tongue thrusting, mouth breathing, open bite, gingivitis
Beckwith-Wiedemann Syndrome
Congenital growth disorder causing large body size/large organs
Ridge in forehead (premature closure of sutures)
Macroglossia,
increased risk for certain cancers especially Wilm’s tumor (kidney)
Crouzon Syndrome (craniofacial dysostosis)
Premature fusion of skull bones Hypertelorism PDA and aortic coarctation Normal intelligence Hypodontia, crowding, high palate, clefts, underbite Maxillary hypoplasia
Ellis-Van Creveld (chondroectodermal dysplasia)
Affects bone growth Polydactyly Congenital heart defects Cleft lip, palate Microdontia, abnormally shaped teeth Multiple frenula and abnormal attachments Natal teeth Congenitally missing teeth Microdontia
Fragile X
Elongated face
Large ears
High palate
Higher occurrence of malocclusion
Marfan Syndrome
Hypermobile joints High arched palate, narrow jaw Crowding of teeth Congenital heart problems Small lower jaw
Prader-Willi
Obesity is main feature Soft tooth (enamel hypoplasia) Poor oral hygiene Bruxism Thick saliva
Tuberous Sclerosis
Hypomelanotic macules Fibromas Seizures Enamel hypoplasia/pitted enamel Gingival hyperplasia (secondary to seizure meds)
Sotos Syndrome (cerebral gigantism)
Facial features Learning disability Overgrowth Hypotonia Premature eruption of teeth Second premolar hypodontia
Hemihyperplasia
Cases with hemihypertrophy not fulfilling criteria of complicated hemihypertrophies are grouped under isolated hemihypertrophy
Non-progressive
May or may not have intellectual disability
Hemi-mandibular hypertrophy: excessive unilateral growth of mandible
Unknown etiology
Oculo-Auriculo-Vertebral (Goldenhar, hemifacial microsomia)
1st and 2nd branchial arches affected Condyle, middle ear, facial nerve affected Facial asymmetry Diminished to absent parotid secretion Anomalies in tongue Clefts
Fetal Alcohol
Smooth philtrum Thin upper lip Micrognathia Hypoplastic mandible Flat midface Minor ear abnormalities Cleft palate
Cornelia de Lange
Low birth weight, hirsutism, microcephaly
Self-injury, compulsive repetition, autism-like
Thin eyebrows (unibrow)
Long eyelashes
Cleft/high palate
Delayed eruption of teeth
Apert Syndrome
Craniosynostosis Syndactyly (mitten hands) Midface hypoplasia Tooth agenesis Supernumerary teeth Ectopic eruption of maxillary first molars Cleft palate Enamel hypoplasia
Turner Syndrome
Absent or incomplete development at puberty (sparse pubic hair and small breasts) XO Webbed neck Premature eruption of permanent molars Micrognathia
Noonan Syndrome
Congenital heart issues Delayed puberty Down slanting/wide eyes Hearing loss, low set ears Mild intellectual disability sometimes Delayed eruption of teeth Atypical eruption sequence
Lowe Syndrome (Oculo-Cerebral-Renal)
Periodontal disease with severe bone loss
Defective inositol phosphate metabolism
Taurodontism
Kleinfelters Syndrome
XXY Only in males Some learning problems Taurodontism Weak muscles, sparse body hair, enlarged breasts
Down Syndrome
Trisomy 21 Microdontia Macroglossia Small chin Delayed eruption of teeth Small conical roots Oval palate Hypoplasia of midface Congenital heart disease
Cri-Du-Chat
Micrognathia, high palate, malocclusion
Dental erosion provoked by GERD/bruxism
Achondroplasia
Large head-to-body size Frontal bossing Maxillary hypoplasia Disturbed genesis of teeth Disturbed eruption of teeth Tooth shape abnormalities
ADHD
3-5% of school-age children affected (up to 7-8%)
Risk factors: age, male, chronic health problems, family dysfunction, low SES
Pre-frontal cortex and dopamine implicated
Treatment = stimulants
Prefrontal cortex and dopamine affected
Cerebral Palsy
Non-progressive disorder
Paralysis, weakness, motor function aberrations
1.5-3.6 cases/1000 children
No cure but can have near normal lives if neurologic problems are managed
Common = spastic-tightness CP (lack of control of muscle movements)
60% have intellectual disability
Dental: periodontal disease, bruxism, drooling, erosion, malocclusion, caries
No cure but many patients live near normal lives if neurological issues are controlled
Maternal Risk Factors for Congenital Heart Conditions
Rubella Diabetes Alcoholism Irradiation Drugs - thalidomide, phenytoin, warfarin
Incidence of congenital heart conditions
8-10 per 1000 births
Non-cyanotic heart conditions
Connection between systemic and pulmonary circulation
Left-to-right shunt
ASD, VSD and PDA most common
Others: coarctation of aorta, aortic stenosis, pulmonary stenosis
Cyanotic heart conditions
Right-to-left shunt
Tetralogy of Fallot
Transposition of great vessels
Tricuspid atresia
Tetralogy of Fallot
VSD
Pulmonary stenosis
Overriding aorta
Right ventricular hypertrophy
AHA Prophylaxis Recommendations for SBE
Prosthetic cardiac valve Previous infective endocarditis Cardiac transplantation that develop valvulopathy Congenital Heart Disease -unrepaired cyanotic CHD -completely repaired in first 6 months -repaired with residual defects
Conditions where SBE prophylaxis is no longer recommended
Mitral valve prolapse with regurgitation Rheumatic heart disease and other acquired valvular heart disease VSD ASD Hypertrophic cardiomyopathy
Dental procedures which endocarditis prophylaxis is recommended
Procedures involving manipulation of gingival tissues or periapical region of teeth or perforation of oral mucosa
Does NOT include
-routine anesthetic injection through non-infected tissue
-taking radiographs
-placement or removable prosth/ortho appliances
-adjustment of ortho appliances
-shedding of deciduous teeth
-bleeding from trauma to lips or oral mucosa
Asthma
Inflammatory restrictive obstructive disease
Agents used for asthma
Corticosteroid treatments (fluticasone)
Beta-2 agonists (albuterol) - cause relaxation of lung
Leukotriene receptor inhibitors
Mast cell stabilizers
Oral manifestations of asthma
Increased prevalence of caries with moderate/severe asthma (secondary to medications)
Decreased salivary flow from beta-2 agonists
Candidiasis risk with corticosteroids
Optimal asthma control is desirable before treatment
Macrolide antibiotics may elevate theophylline levels (erythromycin)
Asthma and aspirin
Aspirin and other NSAIDs should be avoided in aspirin-sensitive persons
10-28% of children with asthma may be intolerant to asthma
Types of Diabetes
Type I: insulin deficiency, autoimmune disease
Type II: relative, progressive insulin deficiency, non-autoimmune
Type III: caused by other identifiable etiology (genetic defect)
Type IV: gestational
Insulin
Beta-cells of pancreas
Promotes entry of glucose into cells
Increases with eating
Glucagon
Produced by alpha-cells of pancreas
Glucagon decreases with eating, increases at rest
Hemoglobin A1c
Measures blood glucose past 2-3 months
Percentage of glucose captured by hemoglobin
Diabetes >7%
-8% or below reflects good control
Complications of diabetes
Macrovascular disease -poor healing -accelerated atherosclerosis Microvascular disease -thickened capillary basement membrane -nephropathy -retinopathy Neuropathy Decreased resistance to infection Hypoglycemia
Hypoglycemia
Most likely problem to be encountered in dental office
Low blood sugar from excess insulin, excess exercise, stress, poor diet
Acute, rapidly progressive
Do not give oral carbohydrates if unconscious
Hyperglycemia
Deficient insulin
Diabetic ketoacidosis
Chronic, slowly progressive
Dental considerations for diabetes
Morning appointments are best (avoid peak insulin)
Prevent hypoglycemia
May need antibiotic prophylaxis
Well-controlled, stable diabetic taking insulin requires little or no modification for routine dental care including surgery
Poorly controlled or uncontrolled - defer elective treatment
Renal osteodystrophy
Muscle weakness, bone pain, fractures with minor trauma
Treatment: vitamin D
Results from chronic kidney disease
Can result in poor calcification of bones (maxilla/mandible)
Delays eruption of permanent teeth
End Stage Renal Disease
Peritoneal dialysis is required -Continuous or cycles -daily, usually at night Hemodialysis -usually kids 5 and older
Consequences for patients on dialyssi
Bleeding tendencies Impaired drug excretion Hypertension Shunt infections Hep B or C Anemia Renal osteodystrophy
Dental management of patients on dialysis
Screen for bleeding disorder prior to surgery
Medical consultation indicated
Avoid dental treatment on day of dialysis
May need SBE
Oral changes in renal failure
Brown tumor of hyperparathyroidism (scattered, multinucleate giant cells)
Ground glass appearance and loss of lamina dura
Pallor of mucosa
Xerostomia
Metallic taste
Petechiae/ecchymoses
Management of kidney transplant patients
Susceptibility to infection is primary concern
May need frequent recalls, daily antibacterial mouth rinses
Avoid NSAIDs
Consider need for supplemental corticosteroids
Most frequent documented source of sepsis in immunosuppressed cancer patients
Mouth
Prevention for cancer patients
All patients with cancer should have an oral examination before chemotherapy
Chlorhexidine rinses recommended
Dental floss is not contraindicated (but careful)
Antibiotic prophylaxis for central lines
Only indicated at time of placement
Absolute neutrophil count
Calculated from WBC count (neutrophils are 50% of WBC)
When less than 1000/mm3, defer elective treatment
When greater than 1000/mm3, no need for antibiotic prophylaxis but may be prescribed if infection is present
Platelet count
> 75000/mm3: no additional support needed (but be ready to treat prolonged bleeding)
40-75000/mm3: platelet transfusions may be considered prior to treatment
<40000/mm3: defer elective treatment; platelet transfusions necessary for emergencies
Dental Treatment and Cancer Therapy
Ideally all dental treatment occurs before cancer therapy starts
When should RCT be completed before cancer treatment?
1 week before
Pulp therapy in primary teeth is ok, but most pediatric dentists prefer to provide more definitive treatment of extraction
When should extractions be completed before cancer treatment?
7-10 days
Ortho and cancer therapy
Appliances should be removed if patient has poor oral hygiene and/or treatment protocol of HCT regimen carries risk for mucositis
Patients may start or resume ortho treatment after completion of all therapy and after at least 2 years disease-free survival
Phases of hematopoietic cell transplantation
Phase 1: pre-transplantation
-chemotherapy and/or radiation results in prolonged immunosuppression following transplant
-elective dentistry must be postponed until immunological recovery (9-12 months post HCT)
Phase 2: conditioning/neutropenic phase
-no elective treatment
-ANC is low, neutrophils almost 0
Phase 3: initial engraftment to hematopoietic reconstitution
Hemophilia A
Factor 8 Limited to males mostly Prolonged bleeding is hallmark Bleeding into joints is common X-linked recessive Long PTT time and normal PT time
Hemophilia B
Factor 9
Other similarities with hemophilia A
Von-Willibrand
Autosomal dominant
Bleeding, epistaxis, easy bruising
vWf serves as carrier protein for factor 8
Treated with desmopressin or vWf replacement
What is the most common inherited bleeding disorder
Von-Willibrand
Chromosome 12
