Etiology • An inherited disorder (autosomal dominant) of circumpulpal
dentin with associated alterations of root morphology; no other organs affected
Clinical Presentation • Premature tooth loss • All teeth affected • Two forms, as follows:
• More severe form (type I) characterized by “rootless teeth,” with normal-colored crowns, obliterated pulp chambers, multiple periapical radiolucencies (periapical granulomas/cysts)
dentin with associated alterations of root morphology; no other organs affected
Clinical Presentation • Premature tooth loss • All teeth affected • Two forms, as follows:
• More severe form (type I) characterized by “rootless teeth,” with normal-colored crowns, obliterated pulp chambers, multiple periapical radiolucencies (periapical granulomas/cysts)
• Less severe form (type II) characterized by amber-colored primary teeth with susceptibility to wear; permanent teeth of normal color with thistle-shaped pulp chambers; frequent pulp stones noted
Diagnosis • Combined clinical and radiographic features • Normal clinical color of permanent teeth and periapical lesions
help to distinguish from dentinogenesis imperfecta • Clinical crowns of primary teeth are amber and opalescent. • Absent root formation (type I) • Thistle-shaped pulp chambers and pulp stones (type II)
Differential Diagnosis • Chemotherapy-/radiation therapy–induced root development
alteration • Pulpal dysplasia
Treatment • Teeth usually unsalvageable (type I) • Observation • Genetic counseling
Prognosis • Guarded