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Developmental disorders Jan Laco, M.D., Ph.D.. Developmental disorders 1. Teeth 2. Jaws 3. Clefts 4. Oral soft tissue.

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Presentation on theme: "Developmental disorders Jan Laco, M.D., Ph.D.. Developmental disorders 1. Teeth 2. Jaws 3. Clefts 4. Oral soft tissue."— Presentation transcript:

1 Developmental disorders Jan Laco, M.D., Ph.D.

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 2. I, 3. M) 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 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 2. I, bilateral, caries

7 1a. Abnormalities of size and form taurodontism (bull tooth) –wide pulp chamber –molars enamel pearl –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 –3. M + 2. P + 2. I –3. M + 2. P: NO effect –2. I: false eruption of C to 1. I 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 3. M (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 4. M 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 defect of matrix formation – pits, grooves, hard –Hereditary enamel hypocalcification normal matrix poorly calcified – soft, chalky

16 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

17 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

18 1c. Defects of structure – systemic diseases Congenital syphilis - Hutchinson –later fetal infection –dental follicle infection by T. pallidum –permanent teeth upper 1. I (Hutchinson´s incisors) – barrel-shaped fissure on incisal edge 1. M (Moon´s molar) – pitted occlusal surface

19 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

20 1c. Defects of structure – systemic diseases Fluorosis (mottled enamel) – fluoride in drinking water –paper-white patches brown –permanent teeth –hydroxyapatite calcium fluoroapatite –matrix normal

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

22 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

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

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

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

26 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

27 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

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

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

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

31 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 sebaceous glands –80% adults –soft, creamy spots, papules –symetrical, buccal location

32 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

33 4c. Developmental disorders – tongue macroglossia –muscle hypertrophy –congenital haemangioma or lymphangioma –Down´s syndrome –lingual thyroid inc. 10% nodule, foramen caecum cleft tongue ankyloglossia


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