From the Guidelines
Dentinogenesis imperfecta is a rare genetic disorder characterized by dysplasia of dental hard tissues, causing teeth to appear translucent, discolored, and weak, often associated with genetic conditions or severe vitamin deficiencies, as noted in the study by 1.
Definition and Characteristics
Dentinogenesis imperfecta is a hereditary dental disorder that affects the development of dentin, the layer of tooth structure beneath the enamel. This condition causes teeth to appear translucent, discolored (often opalescent blue-gray or yellow-brown), and weak. People with dentinogenesis imperfecta typically have teeth that wear down easily, are prone to breakage, and may be lost prematurely.
Causes and Types
The condition affects both primary (baby) and permanent teeth and is caused by genetic mutations that interfere with proper dentin formation. There are three types of dentinogenesis imperfecta, with Type I occurring alongside osteogenesis imperfecta (brittle bone disease), while Types II and III occur independently, as mentioned in the context of dental anomalies of structure by 1.
Treatment and Management
Treatment typically involves dental restorations such as crowns to protect the teeth, and in severe cases, full dentures may be necessary. Early dental intervention is crucial to preserve tooth structure and function. The condition occurs in approximately 1 in 6,000 to 8,000 people and is inherited in an autosomal dominant pattern, meaning a child has a 50% chance of inheriting the condition if one parent has it.
Diagnostic Considerations
In the context of diagnostic imaging for dental anomalies, including dentinogenesis imperfecta, orthopantomography is the indicated 2D radiological imaging technique, especially after the age of 6, as recommended by 1 and further discussed in the guidelines for dental diagnostic imaging by 1.
From the Research
Definition and Characteristics
- Dentinogenesis imperfecta (DI) is a hereditary condition that affects the development of dentine in both primary and permanent dentitions 2, 3.
- It is characterized by abnormal dentine structure, tooth discoloration, and susceptibility to wear 3, 4.
- Three types of DI have been described in the literature, with varying presentations and severity of the disease 2.
Clinical Presentation
- The teeth of individuals with DI are often discolored and show structural defects such as bulbous crowns and small pulp chambers radiographically 3.
- The underlying defect of mineralization can result in shearing of the overlying enamel, leaving exposed weakened dentine that is prone to wear 3.
- Patients with DI may also experience early tooth loss, delayed eruption of permanent teeth, and mobile teeth 5.
Diagnosis and Treatment
- Diagnosis of DI is based on family history, pedigree construction, and detailed clinical examination, with genetic diagnosis potentially becoming useful in the future 3.
- Treatment of DI focuses on protecting affected dentin, reducing sensitivity, and improving esthetics, with options including routine restorative materials, full coverage crowns, bonding of veneers, and dental implants 3, 4, 6.
- Teeth bleaching may also be considered as a treatment option for patients with DI, offering a simple, conservative, and economical solution to improve esthetics 6.