Dental Management of a Child with Autism Spectrum Disorder and Attention-Deficit

Hyperactivity Disorder (Villador)

Introduction
● Autism spectrum disorder (ASD) and attention-deficit hyperactivity disorder
(ADHD) are common neurodevelopmental disorders that can affect children's
behavior and communication.
● Children with ASD and ADHD may have difficulty cooperating with dental
procedures, and they may be more sensitive to pain and discomfort.
● It is important to create a calm and supportive environment for these children
during dental visits, and to use positive reinforcement.
● In some cases, it may be necessary to use sedation or general anesthesia to
provide dental care.

Etiology
● Although the cause of ASD is known in some people and not known in others, genetics,
biology, and environment are all important factors. Having older parents, a difficult birth,
or infections during pregnancy are all examples of factors that might increase the risk for
having ASD. Beyond these factors, certain people are at higher risk than others. For
example, ASD is four times more common in males than females. People with certain
genetic disorders, such as fragile X syndrome, tuberous sclerosis, and Down syndrome,
are more likely to have ASD.

Dental management
● Techniques that are commonly used in patients with ASD are the same as those
that are used for non-autistic individuals: tell, show, do and immediate, frequent
positive and negative reinforcement, paired with firmness, wherever necessary

Case study
A 7-year-old boy (KH) presented to the Discipline of Paediatric Dentistry, Faculty of
Dentistry, University of Otago, New Zealand for dental recall examination. He was first
referred to the Department of Paediatric Dentistry for the assessment of carious lesions
by a Dunedin Public Hospital Paediatrician when he was five years old.

Oral Hygiene
● Lives in an area without water fluoridation
● Brushes once or twice a day without supervision using 1000 ppm fluoride
toothpaste
● Diet consists of soft food without meat and very limited fruits and vegetables

Medical History
● KH was diagnosed with Autism Spectrum Disorder (ASD) at the age of three.
● He was also diagnosed with Attention Deficit Hyperactivity Disorder (ADHD) at
the age of four.
 ● He exhibits various characteristics associated with these conditions, including
hyperactivity, impulsiveness, attention difficulties, limited social interaction skills,
sensory hypersensitivity, and repetitive behavior.
● He has speech impairments and has been receiving speech therapy since the
age of three.
● He previously took methylphenidate for ADHD and is currently taking Melatonin
(20 mg Tab in the evening) for a sleep disorder.

Dental Treatment
● First Visit/ Initial Visit
○ At that first visit background information was gathered from the parents,
and a very limited dental examination was achieved.
○ Use of a social story alongside a behavior shaping approach was
discussed for the next visit, with the aim of achieving a more thorough
oral examination, and bitewing radiographs. Use of bitewing radiographs
was shown to the parent, and a “practice film” was given to take home to
help with familiarization.

● Second visit
○ A social story was introduced and was strictly adhered to at the next
dental visit. This was supplemented with modified tell-show-do, modeling
and positive reinforcement.
○ Extra-oral examination showed no significant findings

●
○ He was in the early mixed dentition stage, with first permanent molars and
maxillary and mandibular central incisors fully erupted in the mouth.
Permanent lower lateral incisors were partially erupted, and upper primary
lateral incisors were mobile. Occlusion showed Class II molar relationship
bilaterally with 2 mm overjet and 5% overbite
●
○ Mild plaque accumulation was noted on the posterior teeth with otherwise
satisfactory oral hygiene on examination. Enamel wear was noted on the
upper primary lateral incisors and upper primary canines, and the first
permanent molars were not fissure sealed. There were stained composite
restorations on the occlusal of the upper right first primary molar, upper
and lower left second primary molars, and stainless steel crowns on the
lower left first primary molar and lower right second primary molar. All of
this treatment had been carried out under general anesthesia
○ There were carious lesions affecting the lower right first primary molar,
and upper left first primary molar.
○ Keeping in mind his previous dental experience, recent improvements in
his general behavior, and that he coped with a thorough dental
examination, radiographs and intraoral photographs for the first time, it
was planned to provide preventive treatment in the dental chair using a
social story with short appointments, and then to carry out extraction of
the lower first primary molars and restorations under GA.

● Subsequent Visits
○ Plaque disclosure and demonstration of brushing and flossing, a
full-mouth prophylaxis, fluoride application and fissure sealants on the first
permanent molars were successfully completed.
○ There was discussion about whether he may manage to have the more
invasive treatment in the same manner, however in agreement with the
parents it was decided to continue with the planned general anesthetic
care for the extractions and restorations. Thus, the patient also received a
stainless steel crown of the upper left first primary molar, occlusal
composite restorations of both upper second primary molars, and
extractions of both lower first primary molars under GA. A post GA review
appointment was arranged after three weeks, and at this visit the caries
risk assessment was updated, and prevention of caries was discussed
with the family. He was placed on 6-monthly recall visit.
● Recall
 ○ At recall appointments, again using a social story, the same staff and
dental chair, bitewings were taken with no difficulty, and full mouth
prophylaxis and fluoride application was carried out.

○ There were no new carious lesions, and the lower first premolars erupted.
His anxiety was much less evident in the dental setting, and 18 months
later his family advised that he now appeared to look forward to the dental
visits, and they felt he could now be seen with the family in the general
dental setting, and no longer needed specialist pediatric dentistry support.

Dental Management
● Things to consider
○ The reduced cognitive functioning, sensory hypersensitivities (visual,
auditory, olfactory or gustatory stimuli) and limited motor skills in some
children with these disorders are associated with oral care difficulties such
as reluctance to use toothpaste, poor oral hygiene, gingivitis and/or
periodontal problems
○ The use of the medications (that may cause xerostomia or a binge-eating
pattern),
 ○ Selective diet, and poor oral hygiene practices are risk factors for dental
caries
○ Some studies have also shown a high prevalence of bruxism and higher
levels of dental trauma in children and adults with ASD

● The use of desensitization appointments in which the child is repeatedly exposed

to the dental environment may help in the adaptation of the child to a dental
setting
● The duration of the appointment should be short, and the same dental staff
should see the child at each appointment, with adherence to routines and as little
change to the environment as possible
● Some studies have shown that the use of visual supports including books with
color pictures, video modeling or the use of social stories was successful when
introducing dentistry to children, and this is used widely with those affected by
ASDs.
● The standard behavior management strategies such as tellshow-do, direct
observation (using a hand mirror), positive reinforcement, distraction, and voice
control may not be successful in these children.
● Early caries risk assessment and caries reduction strategies are of prime
importance in minimizing the healthcare needs of special patients.

Dental Management of Child Diagnosed with Pierre Robin Syndrome (Zapanta)

A. Background of the Disease

i. Pierre Robin syndrome, now more correctly referred to as Pierre
Robin sequence, is characterized by micrognathia, glossoptosis,
and airway obstruction. Lannelongue and Menard first described
Pierre Robin syndrome in 1891 in a report on 2 patients with
micrognathia, cleft palate, and retroglossoptosis. In 1926, Pierre
Robin published the case of an infant with the complete syndrome.
Until 1974, the triad was known as Pierre Robin syndrome;
however, the term syndrome is now reserved for those errors of
morphogenesis with the simultaneous presence of multiple
anomalies caused by a single etiology. The term sequence has
been introduced to include any condition that includes a series of
anomalies caused by a cascade of events initiated by a single
malformation.
B. Pathophysiology / Etiology
i. 2q32-3-q33.2 gene as a result of an unbalanced reciprocal
translocation with 21q32.3-q33.21
ii. Fetal malposition and interposition tongue between palatal
shelves: palatal shelf elevation and fusion
 iii. Genetically influenced metabolic growth disturbances of the maxilla
and mandible
iv. Organogenetic differences lead to variable presentation of
micrognathia and cleft palate
v. Incidence: 5.3 to 22.7 per 100,000 births with 39% infants exhibiting
no additional abnormalities, 25% have known syndromes, 36%
have one or more anomalies that are not part of a known
syndrome.
C. Signs and Symptoms
i. Very small jaw and chin (called micrognathia)
ii. Tongue that is placed further back than normal and falls back into
the throat (glossoptosis)
iii. Glossoptosis leads to loud breathing, snoring and signs of
obstructive sleep apnea (OSA), a syndrome where a child has
problems breathing while sleeping
iv. Feeding issues leading to failure to thrive
D. Medical Management
● Treatment for Pierre Robin sequence is based on what is found during the
evaluation process. It includes treating airway issues to address sleep
issues and breathing problems, and screening for other health concerns.
Treatment depends on how severe the conditions are in each child.
i. Medical supervision to prevent airway obstruction and hypoxia, cor
pulmonale, GERD, bronchopneumonia and exhaustion
ii. Prone positioning, where the baby is placed on their stomach for
sleeping
iii. Nasogastric (NG) tube for feeding
iv. Surgeries, including distraction surgery to increase the size of the
lower jaw
v. Continuous pulse oximetry and apnea monitoring
vi. Intraoral or nasopharyngeal airway placement in severe cases
vii. In severe cases, tracheostomy (making an incision in the windpipe
to create an opening for the baby to breathe) and supraglottoplasty
(a surgery on the upper airway to help a child breathe more easily)
E. Dental Management / Dental Considerations
i. Further orthodontic treatment followed by surgery is often required
ii. Surgeries, including distraction surgery to increase the size of the
lower jaw
 iii. Frequent plastic surgery procedures to correct aesthetics and
function of vermillion border, lip, philtrum and nose

F. CASE REPORT
Introduction:
According to the Clinical Consensus report on Pierre Robin sequence (PRS), Pierre
Robin is comprising of mandibular micrognathia, glossoptosis and airway obstruction,
leading to life- threatening obstructive apnea and feeding difficulties during the neonatal
period. Cleft palate is considered a common and additional feature, but not a perquisite
for diagnosis.

Case Report:
● N.A. was 7 years, 2 months old, when first referred from the cleft lip and palate
forum (a multidisciplinary group at Soroka Medical Center, Beer Sheva), to the
Pediatric Dentistry Unit, Barzilai Medical University Center, Ashkelon, Israel. He
was the oldest son, with 2 younger brothers, from a family of Bedouin. The
reason for his referral was a need for dental treatment of severe carious lesions
and dental crowding.
● He was born in the 29th gestation week, weighing 1.180 Kg and diagnosed as
suffering from Pierre Robin Sequence. During bodily systems screening, the
ultrasound examination revealed a small and backwards mandible.

Dental History
● N.A. had never visited a dental clinic till the age of 7 years. Occasionally, he brushes his
teeth with a toothpaste. He eats many sweets and snacks and his drinks are usually
sweet carbonated drinks.
Clinical Examination
● Extra oral:
○ Facial asymmetry, especially in the nose area, was observed due to a different
size of the nostrils (Figure 1). Submandibular lymph nodes were not detected.
● Intra oral:
○ Soft tissue: a small residual fistula at the site of the soft palate cleft repair, which
does not interfere with function. Otherwise, the soft tissues were normal with mild
gingivitis.
○ Dentition: Early mixed crowded dentition, with skeletal Angle class II and X-bite of
the canines; carious lesions and poor oral hygiene. No hypodontia was found.
○ Special Findings: small retrognathic mandible, retruded maxilla and retruded
upper incisors, lower incisors too upright, enamel hypoplasia of tooth 21 (Figure
2); ectopic eruption of the left maxillary first permanent molar and resorption of
the distal part of the second primary left molar (Figure 3). Cephalometric view
showed retrognathic mandible and maxilla (SNA=72.42, SNB=67.33) proclined
upper centrals I/ to /I=141.28) and Cl II skeletal problem (WITS=7.15) (Figures
4-5).
Management of dental patients with special health needs
● In the guidelines of the AAPD for patients with special needs, dental care
requires special knowledge, awareness, and special attention. Although N.A. is in
a favorable health condition, he is defined as suffering from a chronic condition
that puts him in the category of children with special needs. According to the
guidelines, care of the group of SCHN (Special Care Health Needs) also includes
children with isolated oro-facial complications such as Amelogenesis imperfecta,
Dentinogenesis imperfecta and cleft lip and palate.
● These children are exposed to increased risk of oral diseases; therefore, it is
crucial to establish a preventive dental home. Dental services are among the best
health services required for children with special needs.
● The presented case describes a relatively restricted oro-facial anomaly but it
required involvement of multidisciplinary attention. N.A had coordinated visits and
consultations at the cleft lip and palate department, pediatrics, ENT, speech
therapy, orthodontics, plastic surgery and maxillofacial surgery. There were also
psychological aspects in the context of aesthetics, appearance, and language
development.
● Another consideration in this case is the economic consideration. N.A. comes
from a lower socioeconomic status and his parents found it extremely difficult to
meet the expenses of the treatment.
● Nevertheless, by establishing trust and dedication, he has finished the necessary
dental treatment and meticulous supervision and was ready for orthodontic
treatment.

Dental Management under the General Anesthesia in a child with

Keratitis,Ichthyosis,and DeafnessSyndrome(Zano)

Introduction
● Keratitis-ichthyosis-deafness (KID) syndrome is congenital ectodermal disorder
without a clear mode of inheritance and is characterized by erythrokeratoderma,
sensorineural hearing loss, and vascularizing keratitis
● KID syndrome is usually associated with less severe keratoderma and a milder
hearing problem, but the eye involvement (keratitis) may eventually lead to
impaired vision.
● The well-demarcated, erythematous, hyperkeratotic plaques with verrucous
surface are distributed over face and extremities alternating with smoother areas
● The second one is presence of sensorineural hearing loss. The third component
is ophthalmologic defects, which can progress total blindness .
Case study
In this case report, dental and oral symptoms of a three year and five months old
boy with KID syndrome, suffering severe Early Childhood Caries (s-ECC) and
dental treatments done under General Anesthesia (GA) were reported. He was
admitted to our dental clinic for treatment of his extensive carious and infectious
teeth.

Medical History
Signs and Symptoms
● His skin was dry with well-demarcated lesions, and there were plaques of mild
hyperkeratosis on the knees and elbows. Eyebrows and eyelashes were
completely absent.
● The hair was short, dry, and sparse. He had a cochlear implant because of
sensorineural hearing loss.
● Ability to speak was less than his peers.
● However, he was uncooperative, but not mentally retarded.
● The patient's physical appearance had the typical findings of KID syndrome
except for the diagnosis of “pigeon chest (pectus carinatum),” although he had no
respiratory problems due to pigeon chest

Medical Management
General Anesthesia.
● Before each treatment session, he was referred to a cardiologist, a general
pediatrician, and a radiologist in order to clear his medical status for general
anesthesia.
● General anesthesia was induced by 2% sevoflurane using a face mask,
according to a standard protocol, after an injection of the neuromuscular blocker
● The anesthesia was maintained by intravenously administered propofol. Atropine
and neostigmine were administered to reverse the vecuronium-induced muscle
relaxation when the dental procedures were completed.

Dental History and Examination

● He had not received any dental treatment before. The oral mucosa was
unremarkable.
● Intraoral examination of the child’s tongue, lip mucosa, buccal mucosa, hard and
soft palate, and sublingual region revealed no pathological findings.
 ● Hypoplasia, diffuse or limited opacities, fluorosis, and developmental
abnormalities such as dentinogenesis imperfecta and amelogenesis imperfecta
were not seen on the enamels or enamel residues.
● However, teeth 81, 82, 83, 84, 85, 72, 73, and 74 were vital; but with extensive
caries. Teeth 51, 53, 52, 54, 55, 61, 63, 64, and 65 were non vital because of
severe caries.
● Two pediatric dentists diagnosed s-ECC (severe early child caries) by
considering family history and dental findings.
● From ages 3 to 5, one or more cavitated, missing (due to caries), or filled smooth
surfaces in primary maxillary anterior teeth or a decayed, missing, or filled score
of ≥4 (age 3), ≥5 (age 4), or ≥6 (age 5) surfaces also constitutes s-ECC

Dental Treatment
● The carious lesions of teeth 81, 82, 83, 72, and 73 were removed using round
steel burs and the cavities were then prepared using self-etch dentin bonding
 agent, followed by incremental compomer resin restoration. Sof-Lex discs were
used for contouring and polishing of the restored teeth.
● The pulp of the three teeth (84, 85, and 74) was removed by a spoon excavator,
and bleeding was arrested with gentle pressure from a sterile cotton wool pledget
(CWP) moistened with saline. A 20% ferric sulphate solution was applied to pulp
stumps for 15 seconds via a CWP. After CWP was removed, zinc oxide-eugenol
cement was directly placed over pulp stumps, and then the tooth was restored
with high viscosity glass-ionomer cement.
● The prefabricated stainless steel crowns (SSC) (3M/ESPE, St. Paul, MN, USA)
for pulpotomised 84, 85, and 74 teeth were cemented using luting resin-modified
glass-ionomer cement.
● The root canals of teeth 51, 53, 61, and 63 were filled with a calcium hydroxide +
iodoform mixture paste. Then, mixture paste was removed from root canal for a
distance of 2 to 3 mm. The coronal part was reconstructed by a strip crown plus
compomer. The local anesthetic agent, articaine with epinephrine, was injected
before tooth extraction.
● The chronically infected six teeth (52, 54, 55, 64, 65, and 75) were extracted and
their alveolar sockets were sutured. After completion of the dental treatments, the
patient was transferred to the recovery room, where he recovered uneventfully
from the general anesthesia.
● All dental procedures were completed without any problems, and the entire
operation took about 90 minutes. After an oral examination, he was discharged
from the hospital the next day. The restorations were evaluated in terms of color,
aesthetics, phonetics, and parent’s general satisfaction. Their scores for each
evaluation criterion at each follow-up visit were ranged from excellent to good.
Dental Management
● Based on the psychiatric consultation, the child has delirium secondary to his
medical condition, so specific dental considerations were used including:
○ short visits,
○ appointment time near to the child’s medication time,
○ the child needs to take his medication before the dental treatment,
○ delay dental treatment if the child does not sleep well or sick.
● Anxiety management includes visual distractions (cartoon videos), using
Tell-Show-Do, short breaks time during treatment, and using simple instructions
with repetition during dental treatment.
● The parent signed the consent form that described the dental procedures and the
treatment plan.