1. Rubenstein Taybi Syndrome Oral and Dental Features Carol Mason Consultant in Paediatric Dentistry Great Ormond Street Hospital for Children NHS Foundation Trust

2. Overview Oral and Dental FeaturesOral and Dental Features ProblemsProblems ManagementManagement Prevention of Dental DiseasePrevention of Dental Disease Practical Tips for Oral HygienePractical Tips for Oral Hygiene Hospital PassportHospital Passport

3. Oral and Dental Features a/b:high arched, narrow palate wide alveolar ridges c:extra cusps c:extra cusps d:extra tooth (mesiodens) d:extra tooth (mesiodens) e:extra tooth e:extra tooth Oro-Dental Features as Useful Diagnostic Tool in Rubinstein–Taybi Syndrome A. Bloch-Zupan et al Am J of Med Gen Part A 143A:570–573 (2007)

4. Oral and Dental Features Oro-Dental Features as Useful Diagnostic Tool in Rubinstein–Taybi Syndrome A. Bloch-Zupan et al Am J of Med Gen Part A 143A:570–573 (2007) f: screw-driver incisors, opacities g-i: talon cusps Also – crowding, wear

5. Problems Difficulties with oral hygieneDifficulties with oral hygiene –Crowded teeth –Shape of teeth –Manual dexterity –Learning disability Increased risk of cariesIncreased risk of caries –As above (+ dietary influences)

6. Problems High, narrow palatefood trappingHigh, narrow palatefood trapping difficult denture difficult denture Extra cusps / teethExtra cusps / teeth Crowdingdifficult hygieneCrowdingdifficult hygiene WearsensitivityWearsensitivity

7. Management Talon Cusps:Talon Cusps: –Sealing –Sequential grinding –Reduction (with or without pulp exposure)

8. Management Crowding:Crowding: –Extractions and “driftodontics” –Simple orthodontic treatment –Complex orthodontic treatment

9. Dental Caries (Decay) For dental caries to develop, need Susceptible tooth surfaceSusceptible tooth surface Fermentable carbohydrateFermentable carbohydrate BacteriaBacteria TimeTime

10. Sugars: Obvious / Hidden Oxyfresh.com Healtholia.com Eluxemagazine.com

11. The Acid “See-Saw” www.oralanswers.com

12. Prolonged / Frequent Sugar Exposure Drinks and Snacks

13. Choose Safer Snacks

14. Prevention of Dental Disease Evidence-based toolkitEvidence-based toolkit AVOID DENTAL DISEASE

15. Summary Guidance Tables

17. Amount of toothpaste “Smear” “Pea”

18. Summary Guidance Tables

23. Main Messages Breast feeding best nutrition for babiesBreast feeding best nutrition for babies Brush 2x day (last thing at night + one other)Brush 2x day (last thing at night + one other) Parents/carers brush or superviseParents/carers brush or supervise Fluoride toothpaste:Fluoride toothpaste: –0-3 yrs smear of no less than 1000ppm F –3-6 yrs pea-sized 1000-1500ppm F Spit don’t rinseSpit don’t rinse Frequency + amount of sugary foods and drinks Frequency + amount of sugary foods and drinks Sugar free medicinesSugar free medicines Do not smokeDo not smoke Alcohol, Fruit and Veg Alcohol, Fruit and Veg

24. Oral Hygiene Difficulties Common Complaints “Toothbrush is difficult to hold….”“Toothbrush is difficult to hold….” “He / She doesn’t like the taste…”“He / She doesn’t like the taste…” “I only have a short time….”“I only have a short time….” “He / she clamps down on the brush…..”“He / she clamps down on the brush…..”

25. “Toothbrush is difficult to hold….” Modified handles Electric toothbrush

26. “He / She doesn’t like the taste…” Also useful in -Oral aversion / nil by mouth -Unsafe swallow -Autism (add flavour/colour) -Sore mouth / ulcers Try…….

27. “I only have a short time….” Dr Barman’s Super Brush Electric toothbrush

28. “He / she clamps down on the brush…..” Two–side / Two-brush technique Mouth “props”

29. “He / she clamps down on the brush…..” Two–side / Two-brush technique Mouth “props”

30. “He / she clamps down on the brush…..” Thimbles

31. Positioning for Oral Hygiene Joanna Douglass

32. Fun Toothbrushes Musical Lighting up

33. Hi-Tec Oral Hygiene Many Apps available Electric brushes with timers and with timers and smiley faces smiley faces

34. Oral Hygiene Aids Soft post surgical brush (TePe)Soft post surgical brush (TePe) Collis-Curve toothbrushCollis-Curve toothbrush Chemical adjuncts (chlorhexidine / triclosan)Chemical adjuncts (chlorhexidine / triclosan) www.colliscurve.co.uk TePe Special Care Toothbrush Care Toothbrush Chlorhexidine and fluoride

35. Oral Hygiene Aids Cleaning between teeth / crowded teeth Aspirating tooth-brush – fits onto suction machine – fits onto suction machine

45. Parents Central members of MDTs “ Experts by Experience”