In this episode, we explore Dentinogenesis Imperfecta (DGI), a hereditary disorder of tooth development characterized by discolored, "opalescent" teeth that are prone to severe attrition, fracture, and premature loss due to abnormal dentin structure. We examine the genetic underpinnings of the disease, distinguishing between syndromic forms associated with Osteogenesis Imperfecta (linked to COL1A1 or COL1A2 mutations) and isolated forms caused by mutations in the ** DSPP gene**, while highlighting how classification systems have evolved from the traditional Shields classification to modern proposals that view isolated DGI types as a continuum of severity. Finally, we discuss the necessity of early, multidisciplinary management—ranging from stainless steel crowns in childhood to complex prosthodontics in adulthood—to address clinical challenges like pulp obliteration and to mitigate the significant psychosocial impact the condition has on patient quality of life.
