Wednesday, January 17, 2018

The Neuropathology, Medical Management, and Dental Implications of Autism

Resident’s Name: Michael Hatton                                                                                    Date: 1/17/2018

Article Title: The Neuropathology, medical management, and dental implications of autism
Author(s): Arthur Friedlander, John Yagiela, Victoria Paterno, Michael Mahler
Journal: JADA
Date: Nov 2006
Major Topic: Special care for special patients
Type of Article: Systematic literature review
Main Purpose: Evidence based presentation of autism, its clinical manifestations, dental and medical treatment, and dental implications
Key Points: There is insufficient dental literature on autism and its management in the dental setting.

Autism is a neurodevelopmental disorder in which social interaction, language, behavior, and cognitive functions are impaired severely.

Autistic individuals:
-Fail to respond to normal interactive activities; such as seeking attention of the caregiver, fails to cuddle, no direct eye contact, not raising arms to be picked up, not pointing to objects, no responding to smiles or mother’s voice
-As they get older, young child may not participate in group play or share in other children’s activities and appear to in their own world.
-Verbal and nonverbal communication is delayed or absent (50% do speak)
-Become obsessed with lining things up, and sensory features like buttons on electrical appliances; and immutable rules like timetables, numbers, tables.
-Stress or excitement or stimuli like noise may trigger repetitive actions
-Cognitive impairment is evident in ~70% of people with autism (Severe in ~40%)
-Behavior symptoms associated with autism include temper tantrums, impulsivity, agitation, anger, aggression, self-injury
-Psychological illnesses such as anxiety disorders, mood disorders, ADHD, OCD, schizophrenia

MRI studies have shown abnormal structure, function, or both of the limbic system; this system responsible for emotional and social behaviors, and deciphering facial expressions of other people and understanding their thoughts, feelings, and intentions. Structural imaging also shows overgrowth of brain during first 2 years of life, possibly during prenatal period as well

Medications cannot effect ability to socialize or communicate, but can help control associated symptoms such as hyperactivity, repetitive behaviors, aggression.

Dental management
-Visual and auditory hypersensitivity
-Generally dislike being touched
-Parental presence may calm patients
-Tell-show-do and short, clear commands, positive/negative reinforcement useful
- Look for erosion of teeth due to regurgitation
-Look for signs of bruxism
-Surgical procedures need to consider hematopoietic system (for patients taking carbamazepine, methylphenidate, risperidone, valproate)
-May need CBC including platelets

-Psychiatrist usually monitors 2x/year (ask for records)

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

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

Resident’s Name: Suhyun Rue                                                                              Date: 01/17/2018

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

Osteogenesis Imperfecta: Clinical Diagnosis, Nomenclature and Severity Assessment

Department of Pediatric Dentistry

Lutheran Medical Center

Resident’s Name: Wayne Dobbins                                                                        Date: 01/17/2018

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
Present 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

·         In 2004 expanded 
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 (DI) 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)      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

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

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
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) 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.      Patients with OI type 4 with DI have 5X higher risk for basilar impression

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:

Characteristics of Mesiodens and Its Related Complication

Department of Pediatric Dentistry
Lutheran Medical Center
Resident: Albert Yamoah, DDS                                                                                                     Date: 01/16/2018
Article Title: Characteristics of Mesiodens and Its Related Complication
Author(s): Nam, et al.
Journal: Pediatric Dentistry
Date: Nov /Dec 2015
Major Topic: Mesiodens, Supernumerary Tooth, Tooth Eruption, Complication
Type of Article: Retrospective Cohort Study
Main Purpose: To evaluate the effect of mesiodens on adjacent permanent anterior teeth according to mesiodens characteristics
Key Points:
·   Most supernumerary teeth appear in the anterior region of the maxilla and are most often categorized as mesiodens
·   Approximately 25% of mesiodens erupt normally
o    The remainder do not erupt spontaneously and remain impacted
·    Prolonged retention of the primary maxillary incisors
o    May indicate the presence of mesiodens
o    Warrants screening
·    Traditional techniques used for screening:
o    Periapical
o    Panoramic
o    Bitewing
o    Cephalometric radiography
·    Recently, cone beam computed tomography (CBCT) has been used as an effective screening tool for mesiodens
·    Several types of complications can result without early detection and proper treatment of mesiodens. Mesiodens can cause:
o    Ectopic eruption
o    Displacement or rotation of the central incisors
o    Root resorption
o    Eruption into the nasal cavity
o    Cystic degeneration (i.e. degeneration with formation of cysts)
o    Diastema
o    Crowding
·    The severity of complications resulting from mesiodens is affected by:
o    Number and type of mesiodens
o    Depth and angle of impaction
·   107 children aged 6-11 years old were selected
·   Clinical data and demographic (age and gender) were collected from medical records
·   CBCT images were used to evaluate characteristics of mesiodens including:
o    Number
o    Morphology
o    Eruption path of mesiodens
o    Root development stage of adjacent permanent maxillary central incisors (APMCIs)
·   Study calculated correlation between these factors and complication rate
·   Morphology of mesiodens was classified into
o    Conical (small peg-shaped mesiodens)
o    Tuberculate (more than one cusp or tubercle)
o    Supplemental (resembles normal tooth)
o    Molariform (resembles molar)
o    Atypical types (does not belong to any type)
·   Eruption path of mesiodens
o    Normal
o    Inverted (position of mesiodens crown reversed)
o    Rotated (mesiodens vertical axis parallel to medial palatal suture)
o    Horizontal (mesiodens vertical axis transverse)
o    Angulated (eruption path between horizontal and rotated)

·    Degree of root development of APMCIs evaluated according to Nolla’s developmental stages

·   Positions of mesiodens were divided into 6 groups
o    Groups based on relationship crown of mesiodens and axes of adjacent central incisors on sagittal plane

·   2 categories of complications
o    Eruption disturbance (comparison of central incisor with opposite permanent maxillary CI or nearby LI
o    Displacement of APMCIs (diastema, rotation, and labiolingual movement of APMCIs)

·   Correlation and complication rates calculated

·   The final sample was 107 children
o    80 males and 27 females (2.96:1)
o    Average age 7.3 years +/- 1.4 years
·   The total number of mesiodens was 153
o    58.9% had 1 mesiodens
o    39.3% had 2 mesiodens
o    1.9% had 3 mesiodens
·   58.7% of patients with 1 mesiodens, 67% with 2 mesiodens, and 100% with 3 mesiodens showed clinical complications
·   The primary morphologic type was conical (96.1%)
·   The most common direction of eruption was inverted (56.2%)
·   Most mesiodens (80.4%) were located on the palatal side of the APMCIs
·   The root formation for APMCIs was:
o    Nolla stage 7 in 40.5% of mesiodens
o    Nolla stage 8 in 32%

·   During the eruption period of adjacent permanent incisors is when mesiodens complications were observed
·   Rate of complications increased when mesiodens were located either close to the eruption path or on the labial side of the adjacent permanent maxillary central incisors
·   Rate of complications decreased when mesiodens were located on the palatal side of the cervical area of adjacent permanent incisors  
·   Within the limitations of the present study, the positional relationship between mesiodens and APMCIs had the strongest influence on complications associated with mesiodens
Assessment of Article: 
·    Level of Evidence/Comments: Level III Retrospective Study.