Dentinogenesis imperfecta with osteogenesis imperfecta : a case report

Abstract EN

Dentinogenesis imperfecta (DI) is an autosomal dominant disorder of the dentine tissue that forms the majority of the hard structure in teeth.

DI may appear as a solitary dentine inherited disorder (DI-2) or in association with OI (DI-1).

DI affects both primary and permanent dentitions and the teeth appear dusky blue to brownish.

Osteogenesis imperfecta (OI) is an inherited autosomal dominant disorder of collagen type 1 with many clinical varieties depending on its severity.

Type 1 collagen is present in bone and other body tissues such as dentine in teeth.

This case report describes a 5-year-old male patient with clinical and radiological features of DI-1, the patient had multiple bone fractures over the years, the patient had multiple carious teeth affecting the primary molars, and presented largely with grayish teeth.

Additionally the etiology, clinical features, differential diagnosis, and treatment options have been explored.