Dentin dysplasia (DD) is a rare, inherited condition. It affects the formation of the dentin, which is the mineralized portion of the tooth underneath the enamel and cementum. The condition is characterized by abnormal dentin and root development, resulting in atypical tooth formation.
This condition is extremely rare, affecting 1 person in every 100,000, according to the Journal of Oral and Maxillofacial Radiology. Considered an autosomal dominant trait, it can occur in baby or permanent teeth as either type I or II. Type I, known as radicular dysplasia, refers to the roots of teeth. Type II, known as coronal dysplasia, refers to the crown of the teeth, which is the part visible above the gumline.
Type I Dentin Dysplasia
In type I DD, the teeth typically appear normal (or occasionally with a brownish hue), but the teeth have poor or nonexistent root development. The roots appear on X-rays with shortened, pointed ends. Problems in the roots can be present even with the absence of cavities.
Eruption of the teeth can be delayed with this condition. The abnormal growth of the dentin also leads to reduced pulp space in permanent teeth and virtually a complete absence in baby teeth. This results in early tooth loss, with trauma being the most prevalent cause.
According to Contemporary Clinical Dentistry, treatment for this type of dentinal dysplasia can be challenging. Maintaining dental health through good oral hygiene and select dental procedures is most effective. Very often an endodontist — a dentist who treats the roots and the pulps of the teeth — can perform procedures that extend the life of the tooth.
Type II Dentin Dysplasia
In type II, the baby teeth are translucent and show varying colors, according to the National Organization for Rare Disorders. The pulp chambers are obscure or eliminated. In the adult tooth, the crowns of the teeth appear normal, but the pulp chamber appears flame-shaped and can exhibit pulp stones, which are abnormal calcifications. The improperly formed layer of dentin below the enamel may cause premature wear or loss of teeth, requiring tooth replacement options like implants or dentures.
Treatment is dictated by the individual's specific needs. However, because the permanent teeth are not dramatically affected by this type of dysplasia, regular dental care is usually sufficient.
A rare condition, dentin dysplasia causes abnormal tooth development that can result in tooth loss. Regular dental visits starting at an early age can lead to early diagnosis, helping your dentist provide a targeted treatment plan for a healthy smile.