Developmental disorders

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

Developmental disorders
1. Teeth
2. Jaws 3. Clefts 4. Oral soft tissue

1. Developmental disorders - teeth

1a. Abnormalities of size and form
1b. Abnormalities of number 1c. Defects of structure 1d. Disorders of eruption

1a. Abnormalities of size and form

microdontia teeth < normal
permanent teeth absolute x relative generalized x single (upper 2. I, 3. M)

macrodontia teeth > normal

absolute x relative generalized x single

1a. Abnormalities of size 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

1a. Abnormalities of size 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 chamber upper 2. I, bilateral, caries

1a. Abnormalities of size and form

taurodontism (bull tooth)
wide pulp chamber molars

enamel pearl
ameloblasts below amelocemental junction mm nodule, upper M

1. Developmental disorders - teeth

1a. Abnormalities of size and form
1b. Abnormalities of number 1c. Defects of structure 1d. Disorders of eruption

1b. Abnormalities of number

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

1b. Abnormalities of number

Anhidrotic ectodermal dysplasia
X-linked inheritance hypodontia + hypotrichosis + anhidrosis failure of development of alveolar process

Down s syndrome
no 3. M (90% patients)

1b. Abnormalities of number

Hyperdontia (additional teeth) common
supernumerary: conical incisor + molar region + midline (mesiodens) supplemental: normal excessive growth of l. dentalis 4. M

Cleidocranial dysplasia Gardners syndrome

1. Developmental disorders - teeth

1a. Abnormalities of size and form
1b. Abnormalities of number 1c. Defects of structure 1d. Disorders of eruption

1c. Defects of structure

deciduous teeth neonatal jaudice yellow congenital porphyria red Tetracycline
permanent teeth periapical infection of d. predeccesor Turner tooth systemic diseases

1c. Defects of structure systemic diseases

genetic
amelogenesis imperfecta dentinogenesis imperfecta Ehlers-Danlos syndrome, Gardners syndrome

infective congenital syphilis metabolic childhood infections, rickets drugs tetracycline, cytotoxic chemotherapy fluorosis

1c. Defects of structure systemic diseases

Amelogenesis imperfecta
all teeth + whole enamel x dentine normal

Hereditary enamel hypoplasia

defect of matrix formation pits, grooves, hard

Hereditary enamel hypocalcification

normal matrix poorly calcified soft, chalky

1c. Defects of structure systemic diseases

Dentinogenesis imperfecta
defect of collagen, AD normal teeth shape

brownish, translucent enamel chip away obliteration of pulp dentine - few tubules + abnormal matrix + incomplete calcification Dentinal dysplasia Regional odontodysplasia, Shell teeth

1c. Defects of structure systemic diseases

Ehlers - Danlos syndrome (floppy joints)
collagen disorder hypermobile joints + loose skin small teeth + short roots + pulp stones

Gardners syndrome
multiple osteomas (jaws) + skin tumors + colonic polyps malignant change impacted teeth, supernumerary teeth

1c. Defects of structure systemic diseases

Congenital syphilis - Hutchinson
later fetal infection dental follicle infection by T. pallidum permanent teeth
upper 1. I (Hutchinsons incisors) barrel-shaped fissure on incisal edge 1. M (Moons molar) pitted occlusal surface

1c. Defects of structure systemic diseases

Tetracycline (TTC)
calcifying tissues yellow fluorescence (UV light) during teeth development fetus x infancy dentine bright yellow brown grey permanent NOT TTC under 12 years

1c. Defects of structure systemic diseases

Fluorosis (mottled enamel)
fluoride in drinking water paper-white patches brown permanent teeth hydroxyapatite calcium fluoroapatite matrix normal

1. Developmental disorders - teeth

1a. Abnormalities of size and form
1b. Abnormalities of number 1c. Defects of structure 1d. Disorders of eruption

1d. Disorders of eruption

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

Developmental disorders
1. Teeth
2. Jaws 3. Clefts 4. Oral soft tissue

2. Developmental disorders - jaws

micrognathia x macrognathia hereditary prognathism craniofacial anomalies cherubism Gardners syndrome osteogenesis imperfecta

Developmental disorders
1. Teeth
2. Jaws 3. Clefts 4. Oral soft tissue

3. Developmental disorders - clefts

NOT clefts non-fusion !!! ? ethiology - genetic component (40%) cleft lip - males, inc. 1/1000 cleft palate - females, inc. 1/2000 lip : lip + palate : palate 22% : 58% : 20% + Downs syndrome

3. Developmental disorders - 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

3. Developmental disorders - clefts

complications
malformation or absence of teeth in defect + other congenital defects feeding disorder breathing disorder speech defect cosmetic defect

Developmental disorders
1. Teeth
2. Jaws 3. Clefts 4. Oral soft tissue

4. Developmental disorders oral soft tissues

4a. oral mucosa
4b. 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
Fordyces granules heterotopic sebaceous glands 80% adults soft, creamy spots, papules symetrical, buccal location

4b. Developmental disorders gingiva and periodontium

hereditary gingival fibromatosis AD inheritance grossly enlarged gingivae - preceding of teeth eruption Mi: collagen bundles gingivectomy after puberty
Ehlers-Danlos syndrome

4c. Developmental disorders tongue

macroglossia
muscle hypertrophy congenital haemangioma or lymphangioma Downs syndrome lingual thyroid inc. 10% nodule, foramen caecum

cleft tongue ankyloglossia