developmental disorders jan laco, m.d., ph.d.. developmental disorders 1. teeth 2. jaws 3. clefts 4....
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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• Gardner´s 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, Gardner´s 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
• Gardner´s 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 (Hutchinson´s incisors) – barrel-shaped
fissure on incisal edge
• 1. M (Moon´s 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
• Gardner´s 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%
• + Down´s 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
• Fordyce´s 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– Down´s syndrome– lingual thyroid
• inc. 10% • nodule, foramen caecum
• cleft tongue• ankyloglossia