Developmental disorders

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Developmental disorders

• Jan Laco, M.D., Ph.D.

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Developmental disorders

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

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Developmental disorders - teeth

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

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1a. Abnormalities of size and form

• microdontia teeth lt normal
• permanent teeth
• absolute x relative
• generalized x single (upper 2. I, 3. M)
• macrodontia teeth gt normal
• absolute x relative
• generalized x single

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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

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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

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1a. Abnormalities of size and form

• taurodontism (bull tooth)
• wide pulp chamber
• molars
• enamel pearl
• ameloblasts below amelocemental junction
• mm nodule, upper M

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Developmental disorders - teeth

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

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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

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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)

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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

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Developmental disorders - teeth

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

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1c. Defects of structure

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

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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

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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

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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

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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

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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

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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

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1c. Defects of structure systemic diseases

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

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Developmental disorders - teeth

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

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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

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Developmental disorders

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

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2. Developmental disorders - jaws

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

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Developmental disorders

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

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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

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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

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3. Developmental disorders - clefts

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

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Developmental disorders

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

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4. Developmental disorders oral soft tissues

• 4a. oral mucosa
• 4b. gingiva and periodontium
• 4c. tongue

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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

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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

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4c. Developmental disorders tongue

• macroglossia
• muscle hypertrophy
• congenital haemangioma or lymphangioma
• Downs syndrome
• lingual thyroid
• inc. 10
• nodule, foramen caecum
• cleft tongue
• ankyloglossia