Osteogenesis imperfecta (OI) is a congenital bone disorder, more commonly known as brittle bone disease. While many patients experience serious symptoms in the larger bones in their bodies, the condition can also cause problems with your teeth. Find out how OI affects the body, and learn more about the dental issues sufferers may also experience.
Types of OI
Osteogenesis imperfecta occurs when you have a problem with the connective tissue surrounding the bones. In some cases, this tissue is defective and cannot adequately support your bones. In other cases, patients' bodies cannot even produce this tissue because of a type 1 collagen deficiency. This material is normally abundant in the human body, and helps form scar tissue after an injury. Type I collagen is also one of the important building blocks in your tendons, ligaments and the dentin in your teeth.
Doctors recognize eight types of osteogenesis imperfecta. Type I, the mildest form of the disease, is the most common variety and experts estimate that around 50 to 60 percent of people with OI have this version of the condition.
Other types of OI include:
- Type II disease, which is severe and can kill unborn children
- Type III disease, which, over time, can lead to deformity
- Type VII disease, which doctors associate with cartilage associated protein
- Type VIII disease, which relates to the protein leprecan and is often lethal
Doctors break Type I OI down into two further categories – type IA and type IB. People with Type IA generally have normal teeth, while type IB sufferers experience unusual dental symptoms.
People with OI experience different levels of bone fragility. In Type I patients, the condition is sometimes so mild that people don't even notice any symptoms. For other people, the symptoms can limit physical activity. For type II patients, the problem is so severe that massive injuries can easily occur. Many people with the disease also experience changes to their eyes, where the sclera (outer coat of the eyeball) is a bluish color.
People with type IB can also suffer several unusual dental symptoms. The teeth can appear opalescent and translucent due to a condition called dentinogenesis imperfecta. With this condition, the teeth are often blue-gray or yellow-brown, and patients may also find the teeth become more fragile. People with dentinogenesis imperfecta are more likely to have chipped, cracked and damaged teeth, and the issue generally affects the baby (primary) teeth and their adult (permanent) replacements.
Dentinogenesis imperfecta doesn't just affect people with OI, and other gene mutations can result in this unusual condition. For OI patients, the teeth are also often smaller than normal, and adult teeth sometimes grow in a bell-shape.
Diagnosis generally occurs when people notice symptoms of brittle bones, but earlier signs can include problems with the baby teeth. There's no definitive test that doctors can carry out, but you'll often need a skin biopsy to see if you lack type I collagen. DNA testing can also help your doctor isolate a missing or defective gene.
Unfortunately, doctors cannot cure the disease. As such, your doctor will recommend a treatment plan that aims to cut the risk of bone injury and generally improves life quality. Dentists can also take steps to improve the symptoms of dentinogenesis imperfecta.
Your options for dental treatment will vary according to the extent of your symptoms. Amalgams and composites can strengthen teeth in some patients, particularly where the dentin is strong enough to avoid fracturing. A dentist may also bond veneers to the teeth, as these implants can mask any discoloration or opalescence. Bleaching is only effective in mild cases of discoloration.
For more severe cases, your dentist will probably recommend full coverage crowns. Dental fillings don't generally work well for these patients because the tooth structure cannot adequately support the amalgam or composite filling. Dentures and dental implants may become necessary if dentists have to extract problem teeth.
Osteogenesis imperfecta type I is a genetic condition that causes brittle bones and other symptoms, and people with this condition can also experience problems with their teeth. If you're concerned about the symptoms of this disease, talk to your dentist or doctor for more advice.