Dentinogenesis Imperfecta

What is dentinogenesis imperfecta?

Dentinogenesis imperfecta (DGI) is a tooth development disorder. It causes weak, brittle teeth that wear down or break easily. People with this condition have see-through (translucent) teeth that are blue-gray or yellow-brown in color. Dentinogenesis imperfecta can affect primary (baby) teeth or permanent (adult) teeth, and symptoms can appear at any age.

DGI interferes with dentin production. Dentin is the calcified substance beneath your tooth enamel. When your dentin doesn’t form properly, it can cause your tooth enamel to chip off or wear away. Dentinogenesis imperfecta is different from amelogenesis imperfecta, which directly affects your tooth enamel rather than your dentin.

Dentinogenesis imperfecta is a rare disorder. It affects about 1 out of every 6,000 to 8,000 people in the general population.

Types of this condition

There are three types of dentinogenesis imperfecta:

• Dentinogenesis imperfecta type 1 (I). This occurs in people who have osteogenesis imperfecta (brittle bone disease). It’s related to variations (changes) in a certain gene, usually either the COL1A1 or COL1A2 gene (both responsible for making collagen).
• Dentinogenesis imperfecta type 2 (II). This type affects baby teeth more than permanent adult teeth. People with type II typically don’t have any other inherited disorders, but they may experience age-related hearing loss.
• Dentinogenesis imperfecta type 3 (III): The rarest type of DGI, type III affects permanent teeth more often than primary teeth. Researchers have identified cases in people of Ashkenazi Jewish descent and a few families living in southern Maryland.

What are the symptoms of dentinogenesis imperfecta?

Tooth discoloration and weak, brittle teeth are the most common dentinogenesis imperfecta symptoms. People with DGI have teeth that are translucent and yellow-brown or blue-gray in color.

Other dentinogenesis imperfecta symptoms may include:

• Baby teeth that fall out later than expected
• Enamel hypoplasia (you don’t have enough tooth enamel)
• Joint hypermobility (extreme flexibility)
• Tooth agenesis (you were born missing certain teeth)
• Weakened tooth enamel

What causes dentinogenesis imperfecta?

A variation (change) in the DSPP gene causes dentinogenesis imperfecta. The DSPP gene gives instructions for making dentin (a hard substance that makes up the middle layer of each tooth). You can inherit this gene change from one of your biological parents, or it can happen randomly.

People with dentinogenesis imperfecta type I have a variation in the COL1A1 or COL1A2 genes — the same gene changes that occur in people with osteogenesis imperfecta (brittle bone disease).

How is dentinogenesis imperfecta diagnosed?

A healthcare provider will start with a physical exam. They’ll also ask you about your family history. If they suspect dentinogenesis imperfecta, they’ll take imaging tests like dental X-rays or CT scans to confirm the diagnosis.

Your healthcare provider may also recommend genetic testing. This lab test can detect the DNA changes that cause dentinogenesis imperfecta.

How is dentinogenesis imperfecta treated?

Dentinogenesis imperfecta treatment focuses on strengthening weak teeth. Dentists do this with restorative procedures like fillings, veneers or crowns. They can use tooth-colored materials to enhance the appearance of your smile.

With dentinogenesis imperfecta, you’re more likely to lose teeth as you grow older. If this happens, dentists can replace missing teeth with dental implants or dentures.

Can dentinogenesis imperfecta be prevented?

You can’t prevent dentinogenesis imperfecta because you can’t control the DNA changes that cause it. But treating it early gives you the best chance of reducing dental damage in the long run.

What’s the outlook for dentinogenesis imperfecta?

Your outlook depends on a few things, like:

• Your age at the time of your diagnosis
• How soon you begin treatment
• The type of dentinogenesis imperfecta you have

Dentinogenesis imperfecta isn’t curable. But the right dental care can strengthen your teeth and reduce your risk of further damage. The sooner you get a diagnosis and start treatment, the better your outlook will be.

How do I take care of myself?

If you have dentinogenesis imperfecta, good oral hygiene is imperative. To reduce your risk for tooth decay and damage:

• Ask your dentist about preventive fluoride treatments to strengthen your teeth.
• Brush your teeth two to three times a day using a soft-bristled toothbrush and fluoride toothpaste.
• Limit sugary, acidic foods.
• Swish with a fluoride mouth rinse once a day.
• Visit your dentist regularly for checkups and cleanings.

What can I eat or drink with dentinogenesis imperfecta?

It’s best to choose foods and drinks that are low in sugar and acidity. This means limiting things like sodas, citrus fruits and tomato-based products. It’s also a good idea to avoid foods that are hard or crunchy.

Here are some examples of foods to eat if you have dentinogenesis imperfecta:

• Baked fish
• Casseroles
• Cooked vegetables
• Oatmeal
• Pasta with cream-based sauce
• Scrambled eggs
• Smoothies

When should I see my healthcare provider?

It’s best to tell your healthcare provider as soon as you notice something isn’t quite right. Remember, DGI symptoms can appear at any age. If your baby’s first teeth look bluish, gray or brown, take them to a pediatric dentist for a consultation.

What questions should I ask my doctor?

If you received a dentinogenesis imperfecta diagnosis, here are some questions you might want to ask your healthcare provider:

• What type of DGI do I have?
• What kind of treatment do I need?
• How long will treatment take, and how long will it last?
• How often should I get my teeth cleaned?
• What are some preventive steps I can take to reduce dental wear and tear?