Syndromes

Question 1

Sturge Weber syndrome

Answer 1

Seizures
Port wine stain birthmark
vascular malformations
Dental findings 40%
Vascular hyperplasia on bucal mucosa and lips
Early eruption of teeth from increased vascularity
Hemorragia from angiomas**

Question 2

Major problem for sturgeon Weber

Answer 2

Hemorrhage

Angiomas

Question 3

Rubinstein taybi is associated with

Answer 3

Heart defects possibly requiring surgery

Question 4

Dental findings for rubenstein- taybi

Answer 4

Talon cusp**

Question 5

Treacher Collins what is important

Answer 5

Small mandible and short ramus or complete absence of ramus
Normal intelligence
Small mandible-malocclusion
Tooth a genesis (60%)
Ectopic eruption of first perm molars* because small mandible

Question 6

Pierre robin sequence

Answer 6

Spectrum of anormalities affecting head and face
Glossoptosis**
Tongue appears big due to small mandible
Can lead to cleft palate
Difficulties in breathing and feeding**

Question 7

Beck with-wiedemann syndrome

Answer 7

Ridge in the forehead- metopic ridge 
Macroglossia**
Wilhms tumor 
Big kids 
Large tongue protrudes

Question 8

Crouzon syndrome

Answer 8

Premature fusion of bones
PDA* and coarctation
Normal intelligence

Question 9

Crouzon syndrome dental findings

Answer 9

Hypodontia

Question 10

Ellis can syndrome (chondroectodermal dysplasia)

Answer 10

Multiple frenae and abonar al attachments**
Polydactyl
Congenital heart defects- ASD*

Question 11

Marfan syndrome

Answer 11

Connective tissue
Affects skeletal system
Hyper mobile joints
Scoliosis

Question 12

Dental for marfan

Answer 12

High arched palate
Crowding
Narrow jaws

Question 13

Prader-Willi syndrome

Answer 13

Present from brith
Morbid obesity -intense craving for food
Almond shaped eyes

Question 14

Dental findings for prader William

Answer 14

Soft tooth enamel

Thick sticky saliva

Question 15

Goldenhar syndrome

Answer 15

Congenital abscence of facial structures 
1st and 2nd branchial arches
Facial asymmetry 
Normal intelligence 
Oral- absent parotid secretion*

Question 16

Fetal alcohol syndrome

Answer 16

Smooth Philtrum thin upper lip
Flat mid face 
Cleft palate often 
Mild micrognathia, hypo plastic syndrome
Minor ear abnormalities

Question 17

Apert syndrome

Answer 17

Mitten hands

Question 18

Apert dental findings

Answer 18

Bifid uvula
Missing canines
Supernumerary teeth
Ectopic eruption of max 1st molars

Question 19

Turner syndrome

Answer 19

Incomplete development at puberty

Premature eruption of perm molars**

Question 20

Kleinfelter syndrome

Answer 20

Affects male
Infertility
Taurodontism**

Question 21

Down syndrome

Answer 21

Microdontia
Macro glossia
Congenital heart disease-ASD

Question 22

Main feature of Lesch nyhan syndrome

Answer 22

Build up of uric acid
Intellectual disability
Self mutilating behavior** begins with eruption of teeth
Rapid loss of tissue

Question 23

Non cyanotic conditions

Answer 23

ASD
VSD
PDA

Question 24

Cyanotic conditions

Answer 24

Tetralogy of fallot (prove)
Transposition of vessels
Tricuspid atresia

Question 25

Needs sBE prophylaxis

Answer 25

Prosthetic cardiac valve
Previous infective endocarditis
Cardiac transplant who develop cardiac valvulopathy
Congenital heart disease-unrepaired cyanotic CHD (tetralogy of fallot) including those with palliative shunts
Completely repaired CHD with prosthetic material or device during first 6 months
Repaired CHD with residual defects