Dentinogenesis Imperfecta

What Is Dentinogenesis Imperfecta?

Dentinogenesis imperfecta is an autosomal dominant disorder that affects both primary and permanent dentition. It is an inherited condition that is characterized by an altered gene with an abnormal dentin structure and presents with a localized mesodermal dysplasia that needs an early diagnosis and treatment to prevent further damage to the teeth.

The affected teeth are gray to yellowish-brown and have broad crowns with constriction on the cervical area resulting in a tulip shape. The enamel is easily broken, leading to exposure of dentin that undergoes accelerated attrition. Radiographically, the teeth appear solid, lacking pulp chambers and root canals. Dentinogenesis imperfecta is also known as:

• Hereditary opalescent dentine.

• Dentinogenesis imperfecta without osteogenesis imperfecta.

• Osteogenesis imperfecta with opalescent teeth.

• Opalescent teeth without osteogenesis imperfecta.

• Capdepont teeth.

• Shields type dentinogenesis imperfecta.

• Brandywine type dentinogenesis imperfecta.

• Syndromic dentinogenesis imperfecta.

• Non-syndromic dentinogenesis imperfecta.

What Are the Types of Dentinogenesis Imperfecta?

Dentinogenesis Imperfecta Type 1: People with dentinogenesis imperfecta type 1 also have osteogenesis imperfecta. It affects both the primary and permanent dentitions. The tooth has significant translucency, attrition and is amber shaped with “opalescent dentin.” Radiographically, the following features are seen:

• Root has a constricted appearance.

• The Crown appears short and brittle.

• Pulp obliteration is seen.

Dentinogenesis Imperfecta Type 2: It is the most common type of dentinogenesis imperfecta and is similar to dentinogenesis imperfecta type 1, but osteogenesis imperfecta is not a feature. This type is not associated with any other hereditary disorder and predominantly affects the primary teeth when compared to permanent teeth.

Dentinogenesis Imperfecta Type 3: This type is not associated with any other hereditary disorder and is found in Maryland, Washington, which is known as Brandywine isolate. It is rarer and paradoxically characterized by too little dentin resulting in "shell teeth." Radiographically, the following features are seen:

• Enamel has normal thickness.

• Dentin appears extremely thin.

• It shows dramatically enlarged pulp.

What Causes Dentinogenesis Imperfecta?

Dentinogenesis imperfecta type 2 and 3 is caused by the mutations in the genes, where chromosome number 4 encodes a protein called dentin sialophosphoprotein (DSPP). This dentin sialophosphoprotein is important for the development of normal teeth as it provides information to the genes to produce two proteins. This protein constitutes about 50% of the non-collagenous component of the dentin matrix, and it causes obliteration of the pulp, discolored weak teeth with soft dentin as this protein is involved in the formation of the dentin, which is the middle layer of the tooth. Clinically and radiographically, the indistinguishable dental condition is sometimes seen in patients with osteogenesis imperfecta. Dentin defect associated with osteogenesis imperfecta is known as dentinogenesis imperfecta type 1, and it is caused by the mutations in COL1A1 and COL1A2 genes.

What Are the Clinical Features of Dentinogenesis Imperfecta?

1) Dentinogenesis Imperfecta Type 1 - It most commonly occurs in children, and the frequency may be one in 6000 to 8000 children. The term opalescent dentin is preferred for this type, and the teeth may present with:

• Blue-gray color.

• Amber brown.

• Opalescent.

The organic content is increased 60% above normal, while the inorganic content is less than that of normal dentin. The low density and low hardness of the dentin explains the attrition of the teeth. The enamel is susceptible to break when subjected to heavy occlusal load. Affected members show,

• Discoloration of the teeth.

• Severe attrition of the teeth.

• Obliteration of pulp chambers.

2) Dentinogenesis Imperfecta Type 2 - This type has a bulbous crown which is the typical feature, along with the cervical constriction of the teeth. Extraoral features such as sensorineural hearing loss are marked with this condition, and it is a rare feature. It occurs in people with signs of deafness or age-related hearing loss. Dentinogenesis imperfecta is a common type, and normal teeth are very rare to find. Radiographically, the teeth have bulbous crowns, roots that are narrower than normal, and pulp chambers and root canals that are smaller than normal.

3) Dentinogenesis Imperfecta Type 3 -This disorder was found in the Brandywine tri-racial isolate in southern Maryland. The deciduous and permanent teeth are susceptible to break after the eruption, and multiple pulp exposures may occur. The radiograph of the deciduous dentition shows dilated pulp chambers and root canals initially, which reduces in size over age. In the permanent teeth, the pulpal spaces are smaller, and sometimes it is completely obliterated.

How Is Dentinogenesis Imperfecta Managed?

If you start to show symptoms of dentinogenesis imperfecta, you should contact a dental practitioner for a complete assessment.

The treatment for dentinogenesis imperfecta aims to:

• Remove infection.

• Remove pain.

• Restore esthetics.

• Protect teeth from wear.

Treatment depends according to:

• Age of the patient.

• The severity of the disease.

• Chief complaint.

The treatment of patients with dentinogenesis imperfecta prevents the loss of enamel and subsequent loss of dentin through attrition. The treatment options could be:

• Amalgams restorations to fill the cavities.

• Veneers and bleaching as a treatment for discoloration of teeth.

• Crowns and bridges are used to replace teeth lost and prevent sensitivity of the teeth.

• Dental implants can be given to affordable patients.

• Dentures are advised when the majority of the tooth is lost.

The treatment is focused first on the patient's primary teeth, where restorative procedures, such as crowns or fillings, are done to strengthen the weak teeth. As the patient grows, the dentist may recommend dental implants or dentures according to the required teeth.

Cast metal crowns for the posterior teeth and jacket crowns for the anterior teeth have a high success rate. Care should be taken during the preparation of teeth as good tooth preparation helps with longevity and shear stress. Caution for partial appliances is needed as it takes support from the neighboring tooth and spreads force on the underlying tissues or palate, which exerts pressure on the teeth, and the roots are easily fractured.

It is shown that fillings are not usually permanent because of the softness of the dentin, and composite restoration in severe cases of dentinogenesis imperfecta is not durable. It is important to start the treatment early to improve the facial appearance of young patients and provide genetic counseling for families of children with dentinogenesis imperfecta.

The prognosis depends upon the age of diagnosis of the disease and the quality of treatment provided. With early diagnosis, the patient is minimized with nutritional deficits and psychosocial distress.

Conclusion:

Dentinogenesis imperfecta makes one lose their self-confidence during a smile. By analyzing the symptoms and visiting a dental professional, one can get their smiles back on track. The early diagnosis and management of dentinogenesis imperfecta helps prevent further damages to the teeth and improves the esthetics. Severe cases of dentinogenesis imperfecta require two treatment stages which are to be performed under general anesthesia, and moderate cases of dentinogenesis imperfecta require minimal treatment. So considering the oral changes seek a dentist’s help when there are visible signs and symptoms for early prevention. Follow-up is essential to interpret further complications and to treat the occlusal changes of the dentition.