Etiology • Hereditary disorder (autosomal dominant) of dentin (1:8,000
frequency in population) • May be seen in association with osteogenesis imperfecta • Altered dentin matrix is related to the defective degradation of
dentin phosphoprotein during dentinogenesis.
Clinical Presentation • Primary and permanent dentition exhibit gray to brownish
opalescence • Normal enamel fractures easily from defective underlying
dentin • Severe tooth abrasion related to exposed dentin following
enamel loss • Radiographically, roots are slender to spiked with pronounced
frequency in population) • May be seen in association with osteogenesis imperfecta • Altered dentin matrix is related to the defective degradation of
dentin phosphoprotein during dentinogenesis.
Clinical Presentation • Primary and permanent dentition exhibit gray to brownish
opalescence • Normal enamel fractures easily from defective underlying
dentin • Severe tooth abrasion related to exposed dentin following
enamel loss • Radiographically, roots are slender to spiked with pronounced
cervical constriction and obliterative pulpal calcification • Constricted tooth cervix gives molar crowns a “tulip” profile
Diagnosis • Clinical and radiographic appearance • Family history
Differential Diagnosis • Osteogenesis imperfecta
Treatment • Functional and esthetic restorations (full crowns) • Genetic counseling