3 developmental disorders

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Developmental disorders

Jan Laco, M.D., Ph.D.




1. Teeth

2. Jaws

3. Clefts

4. Oral soft tissue



1. Developmental disorders

- teeth

1a. Abnormalities of size and form

1b. A bnormalities of num ber

1c

. Defec

ts of struc

ture

1d. Disorders of eruption



1a. A bnormalities of size

and form

microdontia «teeth < normal

± permanent teeth

± a bsolute x relative

± generalized x single (upper 2. I, 3. M)

macrodontia «teeth > normal ± a bsolute x relative

± generalized x single




and form

gemination (schizodontia, twinning)

± incomplete division of tooth germ

± 2 separated crowns on single root

fusion (synodontia)

concrescence

± fusion after development of roots (injury)

± teeth united by cementum




and form

dilaceration

± angulation at crown-root junction (injury)

± difficult extraction

talon cusp

± lingual or palatal aspect of permanent teeth

dens invaginatus (dens in dente) ± invagination of enamel into pulp cham ber

± upper 2. I, bilateral, caries




and form

taurodontism ( bull tooth)

± wide pulp cham ber

± molars

enamel pearl

± amelo blasts below amelocemental junction

± mm nodule, upper M




- teeth

1a. A bnormalities of size and form

1b. Abnormalities of number

1c. Defects of structure





Isolated hypodontia ± common

± hereditary

± 3. M + 2. P + 2. I

± 3. M + 2. P: NO effect

± 2. I: false eruption of C to 1. I

Anodontia ± very rare




Anhidrotic ectodermal dysplasia

± X-linked inheritance

± hypodontia + hypotrichosis + anhidrosis

± failure of development of alveolar process

Down´ s syndrome

± no 3. M (90% patients)



1b. A bnormalities of num ber Hyperdontia (additional teeth) ± common

± supernumerary: conical

incisor + molar region + midline (mesiodens) ± supplemental : normal

excessive growth of l. dentalis

4. M

Cleidocranial dysplasia

Gardner´s syndrome




- teeth







1c. Defects of structure deciduous teeth

± neonatal jaudicep yellow

± congenital porphyriap red ± Tetracycline

permanent teeth

± periapical infection of d. predeccesor ± Turnertooth

± systemic diseases




± systemic diseases g enetic

± amelogenesis imperfecta

± dentinogenesis imperfecta ± Ehlers-Danlos syndrome, Gardner´s syndrome

infective ± congenital syphilis

metabolic ± childhood infections, rickets dru g s ± tetracycline, cytotoxic chemotherapy

fluorosis





Amelogenesis imperfecta

± all teeth + whole enamel x dentine normal

± H ereditary enamel hypoplasia

defect of matrix formation ± pits, grooves, hard

± H ereditary enamel hypocalcification normal matrix poorly calcified ± soft, chalky





Dentinogenesis imperfecta

± defect of collagen, AD

± normal teeth shape

± brownish, translucent enamelp chip away

± o bliteration of pulp

± dentine - few tu bules + a bnormal matrix + incomplete

calcification

Dentinal dysplasia

Regional odontodysplasia, Shell teeth





Ehlers - Danlos syndrome (floppy joints)

± collagen disorder

± hypermo bile joints + loose skin

± small teeth + short roots + pulp stones

Gardner´s syndrome

± multiple osteomas (jaws) + skin tumors +colonic polypspmalignant change

± impacted teeth, supernumerary teeth





Congenital syphilis - Hutchinson

± later fetal infection

± dental follicle infection by T . pallidum

± permanent teeth

upper 1. I ( H utchinson´s incisors) ± barrel-shaped

fissure onincisal edge

1. M ( Moon´s molar ) ± pitted occlusal surface





Tetracycline (TTC)

± calcifying tissues

± yellow fluorescence (UV light)

± during teeth development ± fetus x infancy

± dentine

± bright yellowp brownp grey

± permanent

± NOT TTC under 12 years





Fluorosis (mottled enamel)

± o fluoride in drinking water

± paper-white patchesp brown

± permanent teeth

± hydroxyapatitep calcium fluoroapatite

± matrix normal




- teeth








deciduous teeth: 6th month ± 2nd year

± at birth: natal teeth

± 1st month: neonatal teeth (lower 1. I)

permanent teeth: 6th year ± 25th year

± loss of space, retention of a dec. predecessor

± cretenism, rickets




1. Teeth

2. Jaws

3. Clefts

4. Oral soft tissue




- jaws

micrognathia x macrognathia

hereditary prognathism

craniofacial anomalies

cheru bism

Gardner´s syndrome

osteogenesis imperfecta




1. Teeth

2. Jaws

3. Clefts

4. Oral soft tissue




- clefts

NOT clefts non-fusion !!!

? ethiology - genetic component (40%)

cleft lip - o males, inc. 1/1000

cleft palate - o females, inc. 1/2000

lip : lip + palate : palate «22% : 58% : 20%

+ Down´s syndrome




- clefts

cleft lip ± cheiloschisis

± lateral: unilateral (left side) x bilateral

± medial

cleft alveolar ridge ± gnathoschisis

hard palate cleft ± palatoschisis

soft palate cleft - uranoschisis

uvula cleft - staphyloschisis




- clefts

complications

± malformation or a bsence of teeth in defect

± + other congenital defects

± feeding disorder

± breathing disorder

± speech defect ± cosmetic defect




1. Teeth

2. Jaws

3. Clefts

4. Oral soft tissue




± oral soft tissues

4a. oral mucosa

4 b. gingiva and periodontium

4c. tongue



4a. Developmental disorders

± oral mucosa white sponge nevus ± AD inheritance

± white, soft, thickened area of oral mucosa

± Mi: acantosis + hyperkeratosis + intracellular oedema

Fordyce´s granules

± heterotopic se baceous glands ± 80% adults

± soft, creamy spots, papules

± symetrical, buccal location



4 b. Developmental disorders

± gingiva and periodontium hereditary gingival fibromatosis

± AD inheritance

± grossly enlarged gingivae - preceding of teeth

eruption

± Mi: collagen bundles

± gingivectomy after pu

berty

Ehlers-Danlos syndrome

3 developmental disorders

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