developmental-disorders in children’s teeth

Who shot

1. Developmental disorders
Assoc. Professor Jan Laco, MD, PhD

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

3. 1.Developmental disorders
- teeth
•1a. Abnormalities of size and form
•1b. Abnormalities of number
•1c. Defects of structure
•1d. Disorders of eruption

4. 1a. Abnormalities of size
and form
•microdontia …teeth < normal
–permanent teeth
–absolute x relative
–generalized x single (upper I2, M3)
•macrodontia …teeth > normal
–absolute x relative
–generalized x single

5. 1a. Abnormalities of size
and form
•gemination (schizodontia, twinning)
–incomplete division of tooth germ
–2 separated crowns on single root, upper I1
•fusion (synodontia)
•concrescence
–fusion after development of roots (injury)
–teeth united by cementum

6. 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 I2, bilateral, caries

7. 1a. Abnormalities of size
and form
•taurodontism (bull tooth)
–wide pulp chamber
–molars
•enamel pearl
–displaced ameloblasts below amelocemental
junction
–mm nodule, upper M

8. 1.Developmental disorders
- teeth
•1a. Abnormalities of size and form
•1b. Abnormalities of number
•1c. Defects of structure
•1d. Disorders of eruption

9. 1b. Abnormalities of number
•Isolated hypodontia – common
–hereditary
–M3 + P2 + I2
–M3 + P2: NO effect
–I2: false eruption of C to I1
•Anodontia – very rare

10. 1b. Abnormalities of number
•Anhidrotic ectodermal dysplasia
–X-linked inheritance
–hypodontia + hypotrichosis + anhidrosis
–failure of development of alveolar process
•Down´ s syndrome
–no M3 (90% patients)

11. 1b. Abnormalities of number
•Hyperdontia (additional teeth) – common
–supernumerary: conical
•incisor + molar region + midline (mesiodens)
–supplemental: normal
•excessive growth of l. dentalis
•M4
•Cleidocranial dysplasia
•Gardner´s syndrome

12. 1.Developmental disorders
- teeth
•1a. Abnormalities of size and form
•1b. Abnormalities of number
•1c. Defects of structure
•1d. Disorders of eruption

13. 1c. Defects of structure
•deciduous teeth
–neonatal jaudice  yellow
–congenital porphyria  red
–Tetracycline
•permanent teeth
–periapical infection of d. predeccesor – Turner
tooth
–systemic diseases

14. 1c. Defects of structure
– systemic diseases
•genetic
–amelogenesis imperfecta
–dentinogenesis imperfecta
–Ehlers-Danlos syndrome, Gardner´s syndrome
•infective – congenital syphilis
•metabolic – childhood infections, rickets
•drugs – tetracycline, cytotoxic chemotherapy
•fluorosis

15. 1c. Defects of structure
– systemic diseases
•Amelogenesis imperfecta
–all teeth + whole enamel x dentine normal

–Hereditary enamel hypoplasia
•AD inheritance
•defect of matrix formation – pits, grooves, hard
–Hereditary enamel hypocalcification
•AD + AR inheritance
•normal matrix poorly calcified – soft, chalky

16. 1c. Defects of structure
– systemic diseases
•Chronological hypoplasia
–transient impairment of enamel formation in case of
severe illness
–severe fever, gastroenteritis, measles in the past
–horizontál bands of pits, grooves, defects
•Molar-incisor hypomineralisation
–all M1 a I1 a I2
–enamel - hard surface, but soft beneath
–yellow brown discoloration, ↑ caries

17. 1c. Defects of structure
– systemic diseases
•Dentinogenesis imperfecta
–defect of collagen/sialoprotein, AD
–normal teeth shape
–brownish, translucent enamel  chip away
–obliteration of pulp
–dentine - few tubules + abnormal matrix + incomplete calcification
–“shell teeth“ – thin mantle of dentine covers abnormal dentine, few
odontoblasts, no roots
Dentinal dysplasia
–very short and conical roots, teeth are lost early
Regional odontodysplasia (ghost teeth (radiolucent on RTG))
–hypoplastic / hypomineralised enamel + thin dentine

18. 1c. Defects of structure
– systemic diseases
•Ehlers - Danlos syndrome (floppy joints)
–collagen disorder
–hypermobile joints + loose skin
–small teeth + short roots + pulp stones
•Gardner´s syndrome
–multiple osteomas (jaws) + skin tumors +
colonic polyps  malignant change
–impacted teeth, supernumerary teeth

19. 1c. Defects of structure
– systemic diseases
•Congenital syphilis – Hutchinson (1858)
–later fetal infection
–dental follicle infection by T. pallidum
–chronic inflammation  fibrosis of tooth sac 
compression of developing tooth  distortion of
ameloblasts
–permanent teeth
•upper I1 (Hutchinson´s incisors) – barrel-shaped,
fissure on incisal edge
•M1 (Moon´s molars) – dome-shaped,
pitted occlusal surface (“mulbery molar“)

20. 1c. Defects of structure
– systemic diseases
•Tetracycline (TTC)
–taken up calcifying tissues
–yellow fluorescence (UV light)
–during teeth development – fetus x infancy
–dentine
–bright yellow  brown  grey
–permanent teeth
–Avoid TTC under 12 years

21. 1c. Defects of structure
– systemic diseases
•Fluorosis (mottled enamel)
 fluoride in drinking water, India
–during dental development
–paper-white patches  brown
–permanent teeth
–hydroxyapatite  calcium fluoroapatite
–patches of incomplete calcification with high organic
and water content
–enamel matrix usualy normal

22. 1.Developmental disorders
- teeth
•1a. Abnormalities of size and form
•1b. Abnormalities of number
•1c. Defects of structure
•1d. Disorders of eruption

23. 1d. Disorders of eruption
•deciduous teeth: 6
th
– 30
th
month
–at birth: natal teeth
–1st month: neonatal teeth (lower I1)
•permanent teeth: 6
th
– 13
th
y (M3: 18
th
– 30
th
y)
–loss of space, retention of a dec. predecessor
–cretenism, rickets

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

25. 2. Developmental disorders
- jaws
•micrognathia x macrognathia
•hereditary prognathism
•craniofacial anomalies
•cherubism
•Gardner´s syndrome
•osteogenesis imperfecta

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

27. 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%
•+ Down´s syndrome (1 in 200 pts.)
•van der Woude syndrome (AD, IRF6 gene)
–clefts + pits on lower lips + hypodontia

28. 3. Developmental disorders
- clefts
•cleft lip – cheiloschisis
–lateral: unilateral (left side) x bilateral
–medial
•alveolar ridge cleft – gnathoschisis
•hard palate cleft – palatoschisis
•soft palate cleft - uranoschisis
•uvula cleft - staphyloschisis

29. 3. Developmental disorders
- clefts
•complications
–malformation or absence of teeth in defect
–+ other congenital defects
–feeding disorder
–breathing disorder
–middle ear infections
–speech defect
–cosmetic defect

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

31. 4. Developmental disorders
– oral soft tissues
•4a. oral mucosa
•4b. gingiva and periodontium
•4c. tongue

32. 4a. Developmental disorders
– oral mucosa
•white sponge nevus – AD inheritance
–white, soft, thickened area of oral mucosa
–Mi: acantosis + hyperkeratosis + intracellular edema
•Fordyce´s granules
–heterotopic sebaceous glands
–80% adults
–soft yellowish spots or papules
–symmetrical, buccal location

33. 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

34. 4c. Developmental disorders
– tongue
•macroglossia
–muscle hypertrophy
–congenital hemangioma or lymphangioma
–Down´s syndrome
–lingual thyroid
•incidence 10%
•nodule, foramen caecum
•cleft tongue
•ankyloglossia

35. 4c. Tongue
Lingua plicata
•cerebelli-, cerebriformis, fissurata, gyrata, scrotalis
•fissured dorsal aspect of tongue
•children x adults
•asymptomatic x pain during eating, hygiene
•+ geographical tongue
•+ Cowden syndrome
•+ Melkersson-Rosenthal syndrome

36. 4c. Jazyk
Lingua geographica
•geographical tongue, glossitis migrans
•+ lingua plicata; ± psoriasis
•asymptomatic x pain during eating
•irregular smooth red area  healing (hours / days) 
another red area appears
•centre – epithelium thinning; periphery – hyperplasia +
keratinization; mild inflammation