Wednesday, January 27, 2016

Down Syndrome and sleep-disordered breathing

Department of Pediatric Dentistry
Lutheran Medical Center

Resident’s Name: Nicholas Paquin                                                                           Date: 1/27/16

Article Title: Down Syndrome and sleep-disordered breathing
Author(s): Waldman DDS, MPH, PhD; Hasan, Faysal, MD; Perlman, Steven DDS, MScD
Journal: JADA
Date: March 2009
Major Topic: Downs syndrome associated sleep disorders

Main Purpose: Explain a dentists role in treating sleep disorders in down syndrome patients
Key Points/Summary:

- Sleep apnea is common in Down syndrome, occurring in at least half of the population.

- It is a result of the patient’s anatomical facial abnormalities and decreased muscle tone in the upper airway.

- Diagnosis and treatment of sleep disordered breathing in DS patients has gone unrecognized by parents and health care providers which can lead to learning and behavioral problems. The dentists role in helping to diagnose can potentially reduce the co-morbidities associated with sleep disordered breathing.

- Sequela of OSA include pulmonary hypertension, delayed development and behavioral abnormalities, many of these are often associated with DS patients (maybe OSA is the cause and not DS)

- Sleep abnormalities in children with DS are not always cured by surgical procedures. Since these patients may not tolerate a CPAP, oral appliances are often the best treatment.

-appliances Have 2 categories, tongue-retaining devices and mandibular advancement devices
Tongue retaining devices aren’t used often because the compliance is not good, advancement appliances can be used and adjusted for patient comfort and cooperation.

-Combined reality of deinstitutionalization, increased life span and increased association with OSA leads to need to address OSA to improve the sequela listed above.

Providing Comprehensive, Quality Dental Care to Children with Autism Spectrum Disorder

 Article Title: Providing Comprehensive, Quality Dental Care to Children with Autism Spectrum Disorder
Author(s): David A. Tesini, DMD, MS, FDS RCSEd
Journal: Inside Dental Assisting
Date: 2/2014 Issue - Expires April 30th
Major Topic: Quality Dental Care to Children with Autism Spectrum Disorder
Type of Article: Informational/Educational
Main Purpose: Non- pharmacological approach for dental teams in treating ASD patients.

Key Points/Summary:

Autism and autism spectrum disorder

ASD is a sensory processing disorder, which refers to the way in which the central nervous system integrates sensations from the body and environment. 
Individuals with ASD have characteristics of persistent impairments in social communication and interaction. 
They show restrictive activities and repetitive behaviors that are usually present in the early developmental period. 
The spectrum disorder affects approximately 1 in 88 children, with males approximately four times more likely to have ASD as females. 

 In order for dental team members to understand why children with ASD act the way they do., they must start by learning to recognize the five core characteristics of autistic behavioral pattern:
• Impairment in social interaction
• Impairment in communication
• Stereotypic repetitive behavior patterning
  • Impairment in sensory processing and detection anxiety
  • Dyspraxia – defined as the inability to perform a purposeful movement.

Dental considerations
The dental team members should not perceive ASD as a disorder, but instead should understand that the challenges and different behaviors that present as ASD stems from how these children view their worlds. 

Children with ASD have aversions to consistency, texture, taste, and temperature of food, which all could affect their diets. Sweets, sometimes used in behavioral modification programs, may contribute to caries development. Tongue coordination difficulties, oral tactile sensitivities,
medication side effects, bruxism, and food pouching may contribute to dental problems.6 However, children with ASD have been reported to have lower caries experience than children without the condition.

Behavioral Guidance:
Non- pharmacologic management approaches have been contemporized and correspond to the
learning techniques used in the school environment. Verbal cueing (“tell, show, do”) and reinforcement alone may not be sufficient.

D-Termined Program of Familiarization and Sequential Tasking

D-Termined Program of Familiarization and Sequential Tasking is based in applied behavior analysis (ABA) theory and uses the “Familiarization through Repetitive Tasking” philosophy. ABA theory systematically applies the principles of behavior analysis in order to identify the variables
that improve social behavior.
Repetition and familiarization form the basis of this non-pharmalogic approach.
Three repetition factors are the keys to success in this behavioral guidance technique. 
1. “Look at me” for eye contact 
2. “Put your feet out straight and hands on your tummy” for positional modeling 
3. Verbal “1, 2, 3, 4, 5, 6...” or visual (picture stories or charts) for a counting framework

The acceptable and unacceptable treatment positions should be reviewed and practiced with the parent and patient. 
After learning the use of these repetition commands, the dental provider can now plan the five major steps of the D-Termined program. 
  • Divide the skill into small components. Coming into the operatory, sitting in the chair, putting chair back, sitting back in the chair with feet out straight and hands on tummy, counting fingers, counting a tooth, counting four upper front teeth, etc. are part of 20 steps, identified as tasks needed for a classic first visit.
  • Demonstrate the skill. Positional modeling means that when teaching a skill, such as “legs out straight,” the dental provider will actually put the legs out straight and then support that position for a 10-second count. Follow immediately after every 10 count with praise. Continue with “hands on your tummy,” meaning position the hands on the tummy and support that position with a 10-second count, followed immediately with praise for all 20 steps.
  • Drill the skill. Schedule a series of 5 to 6 repetitive visits 1 week apart. Use the task list to divide the tasks and skills to be mastered and customized for each patient.
  • Delight in the repetition. Everyone in the operatory remains happy, upbeat, smiling, and determined to keep trying.
  • Delegate the patient to your trained auxiliary. Dental assistants and dental hygienists are often the key to the success of the D-Termined program. Each weekly visit is scheduled for 30 minutes (may take more or less time). These appointments must not be dentist time intensive. A trained dental auxiliary will start each visit and move the procedure along the task/step progression. The dentist then sees the patient at the end of the visit, starting with putting the chair back down and then progressing to the point on the task list that was the defined objective for the day. The bulk of the repetitive tasking must be performed by the trained auxiliary members in order to remain financially balanced for the office.

The D-Termined program should not be dentist time intensive, but it will be staff time intensive. All staff should be trained in the technique. Support materials and an updated DVD are also available for use in the office.

Addressing health disparities through dental-medical collaborations

Department of Pediatric Dentistry
Lutheran Medical Center

Resident’s Name: Mark Dawoud, DMD                                                         Date: 01/27/16
Article Title: Addressing health disparities through dental-medical
collaborations, part II. Cross-cutting themes in the care of special populations.
Authors: Mouradian et al.
Journal: J Dent Educ 2003
Date: 2003
Major Topic: Special needs populations and dental care
Type of Article: literature review
Main Purpose: This article is a dental-medical collaboration to reduce oral health disparities.

·      Five themes are common to these populations: the importance of oral-systemic interactions and need for interprofessional collaboration in care and training; the possibility of diminished mental and motor capacities and ability to provide self-care; difficulty accessing appropriate dental care; complex social and cultural factors; and the lack of a sufficient evidence base on basic mechanisms and clinical interventions.
·       1. Oral-systemic health interactions are important for these populations.
o   Systemic conditions can directly affect oral health as seen in diabetes, HIV-AIDS, and other immune disorders. Required medical therapies can create oral disorders and disease.
o   Medications can induce xerostomia and in- crease the risk for caries; immune suppression can predispose to oral candidiasis; and cancer chemo- therapies can produce acute mucosal damage.
·       2. Diminished capacities present in many individuals can impact their oral health.
o   For these populations, community-based health promotion efforts must be emphasized: water fluoridation, provision of healthy food choices whatever the residential setting, and access to routine dental prophylaxis and treatment as indicated. The greater use of allied dental professionals should be considered in residential facilities; such settings do offer opportunities for promoting oral health if services can be provided regularly.
o   Individuals with diminished competency are unable to advocate for themselves.
·       3. These populations generally have difficulty accessing dental care.
o   Many of these individuals have Medicaid, coverage varies depending on the state.
·       4. Culturally sensitive, patient- and family- centered care is important for these patients.
o   Oral health care must be tailored to meet the circumstances and preferences of the individual patient, rather than an arbitrary standard of care. Sensitivity in oral health care includes attention to the social circumstances, personal beliefs/preferences, and other medical or behavioral/emotional concerns of patients.
·       5. Care must be backed by a solid evidence base from which to make clinical decisions.

Internal Validity: III;

Unmet Dental Needs and Barriers to Care for Children with Significant Special Health Care Needs

Department of Pediatric Dentistry
Lutheran Medical Center

Resident’s Name:             Leslie M Slowikowski                                                                        Date: 01/27/2016
Article Title: Unmet Dental Needs and Barriers to Care for Children with Significant Special Health Care Needs
Author(s): Nelson et al.
Journal: Pediatric Dentistry
Date: Jan/Feb 2011
Major Topic: Unmet health dental needs in children with SHCN
Type of Article: Scientific article
Main Purpose: Provide results to large scale survey of parents of children with SHCN
Key Points/Summary:  Children with special health care needs (CSHCN) : “those who have or are increased risk for a chronic physical, developmental, behavioral, or emotional condition and who also require health and related services of a type of amount beyond that required by children generally.”

A national survey conducted by the CDC in 2001 in general painted a positive picture to access of health care but the service most commonly reported as needed nationally, but not received, was preventative dental care (more then 8% nationally).  Approximately 78% of CSHCN needed dental care (second only to prescription medicines) as a specific service in the past year.  A more recent study in 2005 also showed the same trend in access to dental care. 

Large scale survey of parents of CSHCN to determine their child’s: oral health status; access to dental care, perceived barriers (environmental/system and nonenviromental /family) and oral health quality of life, accounting for each child’s medical diagnosis and severity of diagnosis in rural and urban Massachusetts. 

Methods: 72-item survey was sent to 3,760 families with CSHCN, 1,128 were complete. 
·      More then 90% has seen a dentistry with in the past year
·      66% were seen by a pediatric dentist
·      21% needed intense behavioral interventions
·      Most families had high education levels, private dental insurance, and above average incomes, 20% of CSHCN had an unmet dental need.  It was determined in this study that middle income families were under represented and low income families had average representation. 
·      Children with craniofacial anomalies had twice as many unmet needs and children with cystic fibrosis had fewer unmet needs.
·      Children with Cerebral palsy, autism, developmental delay, and Down syndrome had more aversions to dental treatment, more treatment complications posed by their medial conditions and more difficulty finding a dentist willing to treat.
·      Children with cystic fibrosis, metabolic disorder, o hemophilia encountered fewer parries to care.

Conclusions of the study:
1.     Children with more medically complex SHCN have more dental concerns than the general CSHCN population.
2.     Despite higher education levels, higher incomes, and private insurances 20% of the CSHCN still had unmet dental needs.
3.     Environmental barriers were greatest for families that were more geographically isolated  and for those with linguistic or economic barriers.

Evidence class: IV

Tuesday, January 26, 2016

Management of a pediatric patient with epidermolysis bullosa receiving comprehensive dental treatment under general anesthesia

Resident’s Name: Amir Yavari                                                                               Date: 01/28/2016

Article Title:  
Management of a pediatric patient with epidermolysis bullosa receiving comprehensive dental treatment under general anesthesia
Yoon, RK, Ohkawa S
Journal: Pediatric Dentistry
Date: 2012
Major Topic:
Clinical considerations and a GA approach for a 3 year old pediatric dental patient with epidermolysis bullosa
Type of Article:
Case Report
Key Points/Summary:
EB is an atypical genetic disorder characterized by blister formation and extreme fragility of the skin and mucous membranes. Vesicuobullous lesions occur in response to trauma or heat. The disease is usually detected at birth or early childhood due to the appearance of bullae and erosion of the skin.

Case Description: 3 y/o female presents for comprehensive dental treatment under GA.  Medical history is significant for EB and penicillin allergy.  Blister and peeling of the skin was found throughout the body.

Anesthesia considerations:
- Induction mask à Lubricated with antibiotic ointment and lightly held to the face
- Blood pressure cuff à Over a layer of Vigilon
- Pulse ox à With no adhesive (non-sticky adhesive Mepitac was used.)
- Nasal ET tube à Placed with fiber optic bronchoscope
- Eyes àLubricated, closed, and draped
- After throat pack placement, the patient was draped and extra padding and wrapping were provided.
- All perioral tissues and commissures were protected with lubricant.
- No sutures à Hemostasis was obtained with pressure

Children with EB are in a chronically hyper metabolic state due to repeated injury and healing of the skin.  Frequent trauma, poor nutrition, and chronic blood loss often lead to chronic anemia.  Infection is also common, as the patients have poor cellular and humoral immunity. In infants, blister of the oral mucosa can be detected on the tongue, lips, gingival tissue, and hard palate due to friction from sucking.
Treatment is largely supportive, including prevention and protection from further trauma and palliative care.  Management of infection is critical for these patients, as inadequate barrier to microbial penetration and decreased immunity are common problems with EB.

Assessment of Article:  Level of Evidence/Comments:  Level III

Osteogenesis Imperfecta: Clinical Diagnosis, Nomenclature and Severity Assessment

Department of Pediatric Dentistry

Lutheran Medical Center


Resident’s Name: John Diune                                                                        Date: 01/27/2016

Article Title: Osteogenesis Imperfecta: Clinical Diagnosis, Nomenclature and Severity Assessment
Author(s): F.S. Van Dijk and D.O. Sillence
Journal: American Journal of Medical Genetics
Date: 2014
Major Topic: Special patients: Osteogenesis Imperfecta
Type of Article: Review article
Main Purpose: Present new nosology and classification for types of Osteogenesis Imperfecta
Key Points/Summary:
Osteogenesis imperfect (OI) is the collective term for a heterogeneous group of connective tissue syndromes characterized primarily by liability to fractures throughout life (p1470)
Present nosology and classification is based on publication in 1979 by Sillence et al “Genetic Heterogeneity in Osteogenesis Imperfecta” where OI patients were classified into 4 syndromes by primary clinical characteristics and pattern of inheritance.
i)                    Dominantly inherited OI with blue sclerae
ii)                   Letha perinatal OI with radiographically crumpled femora and beaded ribs
iii)                 Progressively deforming OI
iv)                 Dominantly inherited OI with normal sclera
·         Numbers OI types I-IV were inserted into the table following a meeting with a Dr. Victor McKusick, who wanted to be able to input these syndromes into a computerized database, and not originally numbered as such
·         In 2004 expanded to OI types V-VII
·         Causes confusion
New OI nomenclature and pre- and postnatal severity assessment was developed to emphasize importance of phenotyping in order to diagnose, classify, and assess severity of OI in order to provide understanding of the disorder to the patient and their families as well as allow physicians to assess effect of therapy.

1)      Non-deforming OI with Blue Sclerae – OI Type I
a.       Increased bone fragility; usually associated with low bone mass
b.      Distinctly blue-gray sclerae
c.       Susceptibility to conductive hearing loss commencing in adolescence and young adult life
d.      Uncommon to have deformity of long bones or spine
e.      When associated with Dentinogenesis Imperfecta becomes moderately severe
                                                               i.      Patients with OI type 1 and DI more likely to have fractures at birth (25% vs 6%)
                                                             ii.      As well as having higher fracture rate, more severe short stature, more skeletal deformity
2)      Common Variable OI – OI Type 4
a.       Recurrent fractures
b.      Osteoporosis
c.       Variable degrees of deformity of long bones and spine
d.      Normal sclera (may be bluish at birth but fades during childhood)
e.      Posterior fossa compression syndromes due to basilar impression (displacement of vertebral elements into the foramen magnum due to softening of bones at skull base
                                                               i.      Again, patients with OI type 4 with DI have 5X higher risk for basilar impression
3)      Progressively Deforming OI – OI Type 3
a.       Usually have newborn or infant presentation with bone fragility and multiple fractures leading to deformity of skeleton
b.      Sclera become progressively less blue with age
c.       Poor longitudinal growth
d.      Progressive kyphoscoliosis develops during childhood into adolescence
e.      At birth radiographs show generalized osteopenia and multiple fractures
f.        Ribs are thin and osteopenic, progressively crowded as platyspondyly increases
g.       In past 2/3rds of patients died by end of 2nd decade usually due to chest wall deformity, pulmonary hypertension, and cardio-respiratory failure
                                                               i.      Bisphosphonate treatment with cyclic IV Pamidronate commending in infancy can result in patients surviving to adulthood
4)      Perinatally Lethal OI Syndromes – OI type 2
a.       Skeletal, joint, and extraskeletal features of fetuses and children extremely severe
b.      Fetuses detected at 18-20wks have short crumpled long bones (accordion-like), bowing or angulation deformities of long bones deficiency of ossification of facial and skull bones
c.       rib fractures with each month in utero starting at 18-20wks
d.      No chance of normal childhood life experience, Pamidronate tx not indicated
e.      Pain relieve with analgesics or subcutaneous morphine to improve comfort and breathing, newborns are in constant pain
                                                               i.      Chest is small and respiratory excursion depressed due to pain from multiple rib fractures
f.        1/5th still born; 90% die by 4 wks of age
5)      OI  With Calcification in Interosseous Membranes – OI Type 5
a.       Progressive calcification of the inter-osseous membranes in forearms and legs
                                                               i.      Leads to restriction of pronation and supination, eventual dislocation of radial heads
b.      Sclerae are white
c.       DI and Wormian bones are not present
Assessment of Article:  Level of Evidence/Comments: