Syndromes Flashcards
Syndromes with Delayed Tooth Eruption
DTE: Downs Cleidocranial dysplasia **Both Downs and CCD have DTE but Downs has missing teeth and CCD has supernumerary teeth Crouzons (?) Aperts
Ectodermal dysplasia Hypothyroidism Hypoparathyroidism Hemifacial microsomia (on the affected side) Ricketts Anemia Amelogenesis imperfecta Dentin dysplasia Gardner’s Cherubism Progeria
NOT Hemifacial microsomia, Crouzon’s (?..Uop)
Syndromes with supernumerary teeth:
Gardner’s Cleidocranial dysplasia (both supernumerary and DTE) CL/CP Crouzons Aperts
Supernumerary teeth are more common in the anterior region and are usually associated with syndromes
Tooth transposition
More common in maxilla than mandible
Mx: most common is U3/U4
Md: most common is L3/L2
*Associated with other dental anomalies like peg laterals and missing teeth
Underlying genetic basis
Unilateral > bilateral
May be related to arch length discrepancy (i.e., mx canine eruption path being altered due to lack of space)
NOT associated with:
*Supernumerary teeth
Second possible answer: crowding, narrow palate (UopH)
Tooth agenesis
Third molars > L5s > U2s
More common in females
MSX 1 and PAX9
Chromosome 4
Results from disturbances during 1) Initiation stage and 2) Proliferation stage
There is NO relationship between 3rd molar agenesis and peg laterals
Treacher Collins
Class II with ANB greater than 5 Zygomatic/malar hypoplasia (but maxilla is considered normal but there is "involvement" of the maxilla) Mandibular hypoplasia Coloboma Hearing impairment Cleft
Derived from a problem with *neural crest cell migration and involves both mx and md, during the 1st trimester (for TC, 4 weeks)
TCOF1 gene
Autosomal dominant
NOT mandibular prognathism/hyperplasia
**neural crest cell problems/week 4: Treacher Collins & Hemifacial Microsomia
Hemifacial microsomia
Derived from problem with *neural crest cell migration
Mandible affected more than maxilla - can affect condyle, ramus, and/or body
Usually unilateral but can be bilateral
2nd most common congenital disease after CLCP
**neural crest cell problems/week 4: Treacher Collins & Hemifacial Microsomia
Downs
1/800 births *Greatest tendency for DTE Slanting epicanthal folds Trisomy 21 Fissured tongue Wormian bones
NOT premature eruption of teeth
Aperts
Syndactyly Mental retardation Hypoplastia of orbital rim/mx/midface Class III Early fusion of coronal suture Supernumerary teeth Cleft
Cleidocranial dysplasia
Absence of clavicles Class III Supernumerary teeth & DTE Narrow high arched palate Frontal and parietal bossing Midface hypoplasia Cleft Wormian bones
Crouzon’s
Craniofacial dysostosis
Mx/midface hypoplasia
Hypertelorism
NOT delayed tooth eruption
Syndromes with Class II or Class III
Class II:
Treacher Collins, Pierre Robin
Class III:
Crouzon’s, Aperts, Downs, Achondroplasia
Pierre Robin
Cyanosis at birth
Mandibular micrognathia
Cleft
Glossoptosis
CL/CP
1/750
CL/CP occures at 6-8 weeks
Cleft Lip more common M>F
Cleft Palate alone more common F>M
Native American > Asian > Hispanic > White > Black
Reduced upper facial height
Clefted soft palate causes: hypernasality, inability to build up intraoral pressure, snoring
Timeline:
3 months: lip repair (10 weeks old, 10 lbs, 10 Hb)
1 year: palate repair
6-10 years: ortho + secondary alveolar bone graft (when canine root is 2/3 formed, iliac > calvaria > ribs)
13-18 years: orthognathic surgery
18+: implants
Syndromes with cleft palate: Pierre Robin Aperts Crouzons Treacher Collins DiGeorge
Golson Yardstick:
To assess dental arch relationships and categorize malocclusion in 3 dimensions: transverse, AP, vertical
Ratings 1-5 with 1 being excellent prognosis, minimal or no need for ortho tx and 5 being definite requirement of orthognathic surgery
Bacterial endocarditis
Acute bacterial endocarditis: staph aureus
Osteomyelities: staph aureus
Subacute bacterial endocarditis: strep viridans
