AMELOGENESISIMPERFECTAPresented by:HARON, Sittie Aainaa M.
AMELOGENESIS IMPERFECTA(AI)ALSO CALLED: ENAMEL DYSPLASIADEFINITION:• disorder of tooth development, abnormal enamelformation.•AI defects include abnormalities that are classified ashypoplastic (defect in amount of enamel), hypomaturation(defect in final growth and maturation of enamelcrystallites), and hypocalcified (defect in initial crystalliteformation followed by defective growth)• It is due to the malfunction of the proteins in theenamel: ameloblastin, enamelin, tuftelin and amelogenin.
SIGNS AND SYMPTOMSThis condition causes teeth to be unusually small, discolored,pitted or grooved, and prone to rapid wear and breakage • The enamel of the tooth is soft and thin. • Teeth appear yellow and are easily damaged. • Both deciduous teeth and permanent teeth are affected. • brown or white chalky discoloration of the teeth
DENTAL CORRELATIONAs a dentist, we are expected to diagnose and treat thiscondition: • Full crowns will improve the appearance of the teeth and protect them from damage. compensate for the soft enamel stainless steel crowns are used in children porcelain for adults • teeth may have to be extracted and implants or dentures are required.
CLASSIFICATION:MULTIFACTORIAL- six genes are known to cause Amelogenesis Imperfecta • AMELX, ENAM, MMP20, KLK4, FAM83H, and WDR72MENDELIAN - the different types of AI are classified according to their mode of inheritance. • Autosomal Dominant Inheritance • Autosomal Recessive Inheritance • X-linked Recessive Inheritance
Type Clinical Appearance Enamel Thickness Radiographic Inheritance Appearance Hypoplastic Crowns size varies from small Varies from thin and Enamel has normal to Autosomal (Type I) to normal, small teeth may smooth to normal slightly reduced contrast/ dominant, recessive, lack proxmial contacts, color thickness with thin or X-linked varies from normal to opaque grooves, furrows white – yellow brown and/or pitsHypomaturation Varies from creamy opaque to Normal thickness Enamel has contrast Autosomal (Type II) marked yellow/brown, surface with enamel that similar to or > than dominant, recessive, of teeth soft and rough, dental often chips and dentin, unerupted crowns or X-linked sensitivity and open bite abrades easily have normal morphology common Hypocalcified Opaque white to yellow- Normal thickness Enamel has contrast Autosomal (Type III) brown, soft rough enamel with enamel that similar to or < dentin, dominant, recessive surface, dental sensitivity and often chips and unerupted crowns have open bite common, heavy abrades easily normal morphology calculus formation commonHypomaturation/ White/Yellow- Brown mottled, Reduced, Enamel contrast normal Autosomal Hypoplasia/ teeth can appear small and hypomineralized to slightly > dentin, large dominant Taurodontism lack proximal contact areas and pits pulp chambers (Type IV)