Enamel: Hypoplastic, Hypomineralised, Combination of both
Exist in isolation or in association with other syndromes
Variable inheritance patterns
Amelogenesis imperfecta
Enamel of all teeth affected
Tooth discolouration
Tooth sensitivity
Post-eruptive and pre-eruptive disintegration
Types of amelogenesis imperfecta
Hypoplastic
Hypomaturation
Hypocalcification
Hypoplastic amelogenesis imperfecta
Inadequate matrix formation
Pitted,grooved or thin enamel, but hard and translucent
Hypomaturation amelogenesis imperfecta
Normal enamel on eruption
Opaque, white, brown-yellow
Soft,vulnerable to attrition
Hypocalcified amelogenesis imperfecta
Normal enamel matrix quantity
Poor calcification
Enamel thickness normal at eruption
Opaque, chalky appearance
Stains and wears rapidly
Amelogenesis imperfecta - Diagnosis
Family history
Clinical observation
Radiographs
Differential diagnosis for amelogenesis imperfecta
Fluorosis
Chronological hypoplasia
Diagnostic tools for amelogenesis imperfecta
Preoperative intraoral photographs
Orthopantomograph (OPG)
Dentine defects
Dentinogenesis imperfecta
Shell teeth
Dentinal dysplasia
Regional odontodysplasia (affects more than one dental hard tissue)
Dentinogenesis imperfecta
Uncommon defect of dentine formation (in dentine sialoprotein)
Autosomal dominant trait –related to osteogenesis imperfecta
Affects both dentitions
Dentinogenesis imperfecta: Affecting teeth alone, Due to specific mutations in dentinesialoprotein (DSPP). Osteogenesis imperfecta with hereditary opalescent dentine, Previously called dentinogenesis imperfecta type I, Mutations in genes encoding collagen: COL1A1 and COL1A2
Dentinogenesis imperfecta
Teeth have normal contour at eruption, but present with a distinctive translucent, opalescent or amber-like hue
Although enamel is normal it is weakly attached to the dentine and is rapidly lost meaning the teeth show marked attrition
Radiographically teeth have short,blunt roots with partial or total obliteration of pulpchambers
Histology of dentinogenesis imperfecta
a thin layer of normaltubularmantle dentine (immediately adjacent enamel or cementum), the remainder of the dentine is abnormal with reduced number of tubules that are often wide and irregular and areas of atubular dentine
Shell teeth
Dentinogenesis imperfecta type 3
Thin shell of dentine surrounding abnormally large pulp chambers
Dentinal Dysplasia
Rare autosomaldominant disease, two forms identified: Type I (rootless teeth), Type II (coronal dentine dysplasia)
Dentinal Dysplasia Type I
Permanent teeth have normal crowns associated with roots composed of dysplasticdentine. Pulp chamber usually obliterated and roots are very short
Dentinal Dysplasia Type II
Primary teeth have an amber colour and obliterated pulp chambers similar to dentinogenesisimperfecta,Secondary teeth are of normal colour and have normal root length, but pulp chambers are reduced in size and often contain multiple pulp stones
Regional odontodysplasia (ghost teeth)
Localised disorder affecting a group of teeth
All dental tissues affected
Unknown aetiology
Maxillary teeth most often affected, abnormal teeth may fail to erupt
Thin enamel and dentine, Irregular enamel and dentine, No enamel prisms, irregular dentine tubules, clefting and interglobular dentine, Erupted teeth are susceptible to caries and wear
Morphological changes in teeth
Disturbances in tooth number: Anodontia, Hypodontia, Supernumerary teeth
Disturbances in tooth size
Disturbances in tooth shape: Fusion, Gemination,Concresence,Dilaceration