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

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

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)

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

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)

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

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

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

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

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

The Acid “See-Saw”

Prolonged / Frequent Sugar Exposure Drinks and Snacks

Choose Safer Snacks

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

Summary Guidance Tables

Amount of toothpaste “Smear” “Pea”

Summary Guidance Tables

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 ppm 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

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…..”

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

“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…….

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

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

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

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

Positioning for Oral Hygiene Joanna Douglass

Fun Toothbrushes Musical Lighting up

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

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) TePe Special Care Toothbrush Care Toothbrush Chlorhexidine and fluoride

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

Parents Central members of MDTs “ Experts by Experience”