Child Dental Patient with WILLIAMS SYNDROME

Good to know

   the Child Dental Patient with


 

 

Williams Syndrome

 

            

 

General characteristics of Williams syndrome


  • Williams syndrome (= Williams-Beuren syndrome) affects approximately one in 10 000 people and is caused by the deletion of genes on chromosome 7q11.23 which code for elastin.
  • Williams syndrome is a multisystem disorder.
  • The phenotypic appearance of people with Williams syndrome is well characterized, but there continues to be new genetic and therapeutic discoveries.
  • The neurodevelopmental profile of patients with Williams syndrome is characterized by high sociability and a variety of retained and compromised linguistic and cognitive abilities. Also they have an hypersensitivity to sound.
  • Feeding and eating problems are common.
  • Patients with Williams syndrome have increased morbidity and mortality under sedation and anesthesia, largely as a result of cardiovascular abnormalities (mainly :  aortic stenosis, pulmonary valve stenosis, ventricular/auricular septal defects).

 

Physical features


  • Main facial features:
    • Flat midface
    • Depressed nasal bridge
    • Anterverted nostrils
    • Long philtrum
    • Thick lips
    • Wide open mouth
    • Blue eyes with stellate iris pattern

 

  • Also found:
    • Short palpebral fissures
    • Ocular hypertelorism
    • Epicanthal folds
    • Peri-orbital fullness
    • Strabismus, visual impairment, photophobia
    • Sensorineural hearing loss, hyperacusis
    • Hoarse voice

 

 

Oral features


  • SPECIFIC to the syndrome:
    • Anomalies of dental development = 90% of the patients are concerned
    • Orofacial dysfunction (sucking problems, chewing and swallowing problems, mouth-breathing…)
    • Hypersensivity mouth
    • Eruption disorders
    • Malocclusions
  • Non specific:
    • Dental caries
    • Periodontal problems

 

Anomalies of tooth number


  • 40,5% of children have agenesis of one or more permanent teeth = hypodontia (Axelsson et coll, 2003)
  • 12% have oligodontia (6 missing teeth or more)
  • Premolars are often concerned

 

Common other dental anomalies: abnormal tooth morphology


  • Size and shape anomalies found in:  
    • 12,5 % in primary dentition
    • 40,7 % in permanent dentition
  • Microdontia:
    • mesiodistal incisor crown dimensions significantly smaller
  • Anomalies of shape:
    • Tapered or screwdriver shaped incisors
    • Conical teeth
    • Taurodontism

 

 

Developmental defects of enamel


  • 9,4% of the patients present at least one defect in primary dentition
  • 18,5% of the patients in permanent dentition
  • Type of defect :
    • Hypomineralisation = qualitative defect (yellow or white spots - opacities)
    • Enamel hypoplasia = quantitative defect

 

 

Malocclusions


  • Higher prevalence of Class II and III occlusions
  • Narrow or large maxilla - Deep palate
  • Late exfoliation of primary teeth
  • Ectopic eruption
  • Crowding or spacing (missing teeth)
  • Cross-bites
  • Anterior Open-bite (25% of the patients)

 

Non specific pathologies


  • Increased carious risk:
    • Mild intellectual deficiency
    • Behaviour management difficulties
    • Some cardiac medication may contain sucrose.
  • At risk of gingivitis and periodontal diseases:
    • Poor oral hygiene practices, linked to the weak cooperation of the young patients, also associated with malocclusion and agenesis that limit the self-cleansing
    • Severe alteration of elastin components of gingival connective fibers, increasing the susceptibility of periodontal tissue to be attacked by plaque bacteria.

 

Medical and dental management


  • The medical management of children with Williams syndrome requires a multidisciplinary team familiar with the organ systems affected as well as the developmental and behavioral problems.
  • Guidelines exist for pediatricians and many tertiary centers now offer clinics for patients with Williams syndrome.
  • The website of the Williams Syndrome Association (www.williams-syndrome.org) proposes helpful information for parents as well as physicians.
  • The dentist should particularly pay attention to:
    • The congenital heart defects
    • The impaired orofacial motor function
    • The oro-facial anomalies
    • The hypersensivity to sound and of hypersensitive mouth
  • The dentist has to evaluate the benefice risk of sedation and GA for patients with Williams syndrome. If conscious sedation used, careful monitor of the vital signs and oxygen saturation during the procedure

 

Dental management


  • Dental anxiety and communication disorders are present in almost all the children with Williams syndrome.
  • One third of the children present several anomalies and/or pathologies as anomalies of number, size, shape and structure, or severe malocclusions. These malformations, associated with oral motor impairment, may cause oral dysfunction, beginning early in the infancy.
  • The limited motor skills shown in patients with WS contributed to a poor oral hygiene, requiring a comprehensive preventive approach to reduce the caries risk and activity.
  • A comprehensive radiographic examination may reveal the presence of dental anomalies, which are typical findings of this syndrome, and help planning the restorative and orthodontic rehabilitation.
  • Nevertheless, orthodontic management is often limited.
  • The use of headphones could be recommanded to minimize the sounds and noises of the dental office, which caused great discomfort to the child due to his hypersensitivity to loud sounds.

 

Management of infectious risk


More than 80 % of the patients have
a cardiac malformation

Reduce the risk of infective endocarditis:
 Antibiotic prophylaxis before certain dental procedures
 Preoperative oral antiseptic mouth wash, such as 0,2% chlorhexidine gluconate
 Eliminate all dental infections
 Pulpotomy or pulpectomy contraindicated
 Evaluate benefice versus risk for orthodontic treatment

 

 

Dental care under general anesthesia


Risk factors:

  • Congenital heart diseases
  • Exemple : death associated with general anesthesia in a Williams syndrome patient with a aortic stenosis (Burch et al, 2008)
  • Complicated intubation linked to difficult airway:

► Craniofacial/oral malformations: micrognathia, macroglossia

► Breathing difficulties

  • Sleep apneoa
  • Drug interactions (with certain cardiac medication)

 

Recommandation for dental management and follow-up

 


Work together with other professionals
as a member of the rehabilitation team!

 
  1. Primary dentition
  2. Mixted dentition
  3. Adolescence
  4. Adult
 

"Preventive dental protocols and dietary counseling must be individually designed and implemented"

 

1. Primary dentition


Age

Objectives Management

12-24 months

Control of eruption
Early diagnosis of dental anomalies
Assessment of oral function (sucking, feeding abilities)

Parental counseling about preventive measures: tooth brushing methods, diet, use of fluorides…
Prevention of mouth hypersensitivity

30 months

Control of eruption
Early diagnosis of dental anomalies
Assessment of oral function (eating disorders..)
Introduction to the dental environment

Preventive program: oral hygiene, diet…
Prevention of mouth hypersensitivity
Topical application of fluoride, sealant (if indicated)

3 y

Control of eruption
Diagnosis of dental anomalies
Assessment of the occlusion and of oral habits
Carious risk assessment

Intraoral radiographies if indicated
Sealants if enamel defects
Conservative treatment (if needed)
Physiotherapy/orofacial therapy if indicated

4 y

Follow up

Preventive program
Physiotherapy/orofacial therapy if indicated

5 y

Follow up

Preventive program
Physiotherapy/orofacial therapy if indicated

 

2. Mixted dentition


 

Age

Objectives Management

6

Control of eruption
Diagnosis of dental anomalies
Assessment of occlusion and of oral habits, tongue position, speech articulation problems
Carious risk assessment

Preventive program
Topical application of fluoride
Panoramic Xray
Sealant of the first permanent molars
Orofacial therapy

7

Control of eruption
Diagnosis of dental anomalies
Assessment of occlusion and of oral habits

Preventive program
Topical application of fluoride
Sealant of the first permanent molars
Orofacial therapy

8

Follow up

Preventive program
Topical application of fluoride

9

Follow up
Orthodontic examination

Preventive program
Topical application of fluoride
Panoramic Xray – lateral cephalogram
Orthodontic treatment if indicated

10

Follow up

Preventive program

 

3. Adolescence


 

 

Age

Objectives Management

12

Diagnosis of sketetal malocclusion, tooth malpositions, oral soft tissue and dysfunction
Evaluation of gingival health
Eruption of permanent teeth

Preventive measures
Topical application of fluoride
Sealant of the second permanent molars
Panoramic Xray

13

Follow up (one visit every year if low carious risk)

Preventive measures
Orthodontic treatment

14

Follow up

Preventive measures
Orthodontic treatment

15 Follow up Preventive measures
Orthodontic treatment
16

Follow up

Preventive measures
Orthodontic treatment

 

4. Adult


 

 

Age

Objectives Management

16-20

Evaluation:  occlusion, periodontal diseases, eruption of wisdom teeth
Multidisciplinary evaluation if orthognatic surgery needed

Preventive program (gingivitis and periodontal diseases)
Scaling
Panoramic Xray

>20

1 visit every year

Preventive program (gingivitis and periodontal diseases)
Orthognatic surgery (if indicated)