Dentin dysplasia - Click here

Dentin dysplasia is a rare inherited developmental disorder of dentin formation, usually transmitted as an autosomal dominant trait. Both primary and permanent teeth are affected, while enamel formation is typically normal.

Two main types are recognized. Type I (Radicular dentin dysplasia) is characterized by clinically normal crowns with short or absent roots, obliterated pulp chambers, and frequent periapical radiolucencies in non-carious teeth, often leading to premature tooth loss. Type II (Coronal dentin dysplasia) presents with amber or bluish discoloration of primary teeth resembling dentinogenesis imperfecta, while permanent teeth show thistle-tube–shaped pulp chambers with pulp stones.

Management is primarily preventive and supportive, focusing on maintaining tooth vitality, preventing infection, and preserving teeth for as long as possible through careful restorative and periodontal care.