Dental Care for Persons with OI

Osteogenesis imperfecta (OI) is always associated with bone fragility. In addition, OI may affect the growth of the jaws and may or may not affect the teeth. This summary is intended to provide an overall view of the subject and may not pertain to a given individual. Adults with OI and parents of children with OI are encouraged to discuss their questions and concerns with their own private practitioners.

About half of the people who have OI have teeth that appear normal, and their major concerns are routine care. However, the other half has a defect in the teeth called dentinogenesis imperfecta (DI), sometimes referred to as opalescent teeth. These teeth may be misshapen, may chip or break easily, and will require special care.

Oral cavity problems related to osteogenesis imperfecta may include the following:

- A skeletal Class III malocclusion. The bite is affected because of the size and/or position of the upper and lower jaws and, therefore, the teeth do not match.

- An open bite. There is a vertical gap between some of the upper and lower teeth.

- Impacted teeth. The first or second permanent molars do not erupt, or they erupt out of the usual location.

- Dental development. Tooth development may be delayed or advanced in some individuals affected by OI.

- Gum disease (periodontitis) is not considered to increase because of OI.

Major Parts of the Teeth

The teeth are made up of four distinct parts.

- Enamel is the outside part of the crown. It is the hardest substance in the body and the point of contact for chewing.

- Dentin is the substance under the enamel forming the rest of the crown and surrounding the pulp chamber and almost all of the root structure. It is similar to bone.

- The Pulp Chamber is the inner hollow part of the tooth containing blood vessels and nerves.

- The Dentinoenamel Junction (DEJ) is the term for where the enamel and dentin are attached to each other.

Dentinogenesis Imperfecta (DI)
Dentinogenesis imperfecta can be part of osteogenesis imperfecta (DI type I) or it can be a separate inherited autosomal dominant trait (DI type II) without OI. DI occurring with OI seems to run true in families but can vary in severity from one member to another. If someone has OI and DI, all of their teeth may not be affected to the same degree. DI has a variable affect on the color, shape, and wear of both primary and permanent teeth.

Teeth affected by DI have essentially normal enamel, but the DEJ and the dentin are not normal. The enamel tends to crack away from the dentin, which wears away more quickly than enamel. The dentin makes the teeth look darker or opalescent and grows to fill in the pulp chamber, causing a loss of feeling in the tooth. Affected teeth will have an increased incidence of fracture, wear and decay.

Dentinogenesis imperfecta may be diagnosed with the first baby tooth. If the tooth does not appear white, or if it is gray, blue or brown, DI should be suspected. Children should be taken to a dentist (if possible a specialist in pediatric dentistry) when the first teeth are erupting. This may happen as early as 6 months to 1 year of age. Radiographs, or X-rays, can be useful but may be difficult to obtain until the child is older. Sometimes there are changes visible on the X-rays that are not obvious just by looking at the teeth. Crowns appear bulbous and roots may be shorter and more slender than standard. Primary teeth are usually more affected than the permanent teeth. Special care will be needed even with the baby teeth. If the teeth are wearing down too quickly, stainless steel crowns can be placed on them.

General Care for a Person With OI and Without DI

A dentist should see a child with OI by 2 to 3 years of age at the latest. Baby teeth require care. They are important for chewing, speaking, holding space for the permanent teeth to grow in, and growth of the jaws. There appears to be minimal risk of jaw fracture from routine dental care and dental extractions. No particular precautions are needed other than those that would be taken anyway, such as support of a very thin lower jaw when an extraction procedure is being done. Excessive callus formation has not been reported to be a problem when a tooth is extracted.

Good care involves brushing and flossing the teeth of young children, then teaching them how to do it themselves and checking them as they grow older. Soft toothbrushes are good for everybody and easier on gums, since gums also need brushing. Mechanical toothbrushes tend to be more effective than brushing by hand. Use of fluoride toothpaste is recommended. Children should use a small dab of toothpaste, or a children's toothpaste, and be taught to spit it out well after brushing so they do not swallow excessive amounts. Before going to bed, children should spit out the toothpaste after brushing, but not rinse their mouths. This will leave more fluoride in the mouth to work overnight. Parents should talk to their child's dentist about the fluoride content of their drinking water and ask if supplemental fluoride is needed from a pill, a non-alcohol fluoride rinse, or a fluoride gel. Sealants placed on the biting surface of the permanent molars in children may reduce the chance of developing cavities in the grooves of the teeth.

Starting when the child is 7 years old, an orthodontist should check the child's bite for evidence of an open bite or Class III malocclusion.

General Care for a Person With OI and With DI

Children with OI and DI need the same basic care as discussed above, but they also need to have their teeth carefully monitored for excessive wear. Brushing and cleaning will not make teeth affected by DI white, and bleaching is not recommended because the discoloration is not in the enamel. Regular care is needed so the teeth will last as long as possible and to prevent abscesses and pain. If the teeth are wearing excessively, caps, also called crowns, will probably need to be placed on at least some of the teeth. This will serve to keep the teeth in place and encourage proper development of the jaw. More specialized treatment, including veneers, may be more appropriate for permanent teeth.

The condition of the enamel on adult or permanent teeth will need to be monitored. If the enamel cracks away and the underlying dentin starts to wear, some type of coverage such as crowns or an overlying denture is needed. If there is not enough of the tooth above the gum to allow placement of a crown, some additional gum surgery or root canal treatment may be necessary.

Treating Malocclusions with Orthodontia or Orthognathic Surgery


A malocclusion can be defined as an abnormal relationship between the upper and lower teeth and/or their alignment, which creates problems with how the teeth come together. This may be due to the relationship of the upper and lower jaws to each other, the alignment of the teeth, or both. This type of problem includes crooked teeth, "underbite," "overbite" and "open bite." Treatment is usually provided by an orthodontist. The particular treatment plan depends on the specific problem(s) with the bite and the teeth. If the malocclusion is caused by skeletal discrepancies, then orthognathic surgery may be required along with orthodontia.

Although there are no published studies regarding orthodontia for persons with OI, it seems to be safe to treat persons with OI if DI is not present. If DI is present, the orthodontist will have to decide if the enamel can withstand gluing brackets to the teeth for the braces and removing the brackets later.

Unfortunately, it is difficult to determine how strong the enamel is until it is tried. If there is concern about the enamel cracking off and treatment is still desired, placing bands on all the teeth to hold the brackets may work. Although bands are considered an "old fashioned" method, the technique still works. It may be necessary to seek out an older orthodontist who learned to install braces before the current practice of gluing bands directly to teeth was discovered. The orthodontist will need to minimize forces on the teeth as well as movement of teeth over long distances. Caps, or crowns, may be effective in correcting rotations or mildly malpositioned teeth.

In some children with OI the upper jaw, or maxilla, does not grow as much as the lower jaw, or mandible. Sometimes the way that both jaws grow makes it difficult, if not impossible, to bring the teeth together properly, even after orthodontic braces. If the malocclusion is due to a problem with the growth of one or both jaws, then a combination of orthodontic braces and orthognathic surgery may be used to align the teeth. Some period of orthodontic braces is also usually needed after the jaw surgery. There are a few published reports about these surgeries indicating good post-operative healing of the jaws. The same concerns that one would have with any surgery in a patient with OI, such as potential bleeding problems and reaction to general anesthesia, still apply.

Treating Impacted Teeth

The dentist needs to consider if the impacted teeth should be left alone or extracted, or if an attempt should be made to move them into a functional position in the mouth. To move a tooth, a coordinated effort is needed between the oral surgeon and the orthodontist to surgically uncover the impacted tooth and glue an attachment onto the tooth so that light force from the braces can be used to bring the tooth into the proper position. The orthodontist may also use braces prior to surgery to be sure there is space to bring the impacted tooth into the proper position.

Other Treatments

Dental Implants are used to replace missing teeth. Theoretically it is possible to do this successfully for a person with OI and there is anecdotal evidence that this has been accomplished. However, there are no controlled studies on the use of dental implants in patients with OI. There is also a question of how the use of bisphosphonates, medicines that are increasingly being used experimentally to treat people with OI, might affect the long-term retention of the implant. (See the sections Wound Healing in Bone, and Bisphosphonates, Teeth and Implants below for more information.)

Dental implants are somewhat like screws. In order to function, there must be enough bone in the jaw for the implant to be securely placed. After healing, a "post" is placed in the implant and an artificial tooth is attached. Good, strong healing around the implant is critical.

The healing mechanism for dental implants is similar to that of normal wound healing with one major difference. Instead of the bone returning to its normal basal level of turnover, or remodeling, where old bone is removed and new bone is added, the implant healing mechanism appears to involve a long-term retention process. Substantially elevated bone remodeling appears to be necessary as long as the implant is in place. The reason for this is not clear, but it may serve as a way of preventing fatigue damage due to high mechanical stress concentrations that are always found around surgical screws.

There is only one case report in the literature referring to implants in an OI-affected individual. Normal implant-bone healing seemed to occur, but a greater than 50% implant failure rate within 3 years of surgery was reported. There is not enough data in the literature to fully understand long-term success rates.

Veneers are cosmetic coverings typically placed on the outer surface of the anterior teeth. Anterior teeth are seen when a person smiles. If veneers are glued to the enamel, the enamel may break off from the dentine, in which case the veneer would be lost. The enamel can be removed, and the veneers glued to the dentine, with varying degrees of success. Veneers are typically not made to withstand biting forces.

Caps and Bridges have a variety of uses. Caps, also called crowns, are made of metal or ceramic and cover the entire tooth after the enamel is removed. If teeth are wearing excessively, crowns usually provide the best treatment. Preformed stainless steel crowns are typically used for baby teeth, while cast metal or ceramic crowns are used for adult teeth. If there is not enough tooth left above the gum to place a crown, the individual may need gum surgery to make the part of the tooth showing above the gum larger. The surgeon may place a post down into the root of the tooth to act as a reinforcing rod, and then rebuild part of the tooth above the gum for the crown to sit on. In teeth not affected with DI, root canal treatment may be needed if the nerves and blood vessels inside the tooth are infected from a cavity or if the post needs to go down the center of the root(s).

In teeth with DI, the inside where the nerves and blood vessels are normally located may already be filled with dentine. This makes placing a post in the center of the root and/or root canal treatment difficult, if not impossible. Small reinforcing pins may be placed in the dentine away from the center of the root to help make the new crown of the tooth stronger.

A bridge is at least one artificial tooth attached to one or more crowns. A bridge is sometimes called a fixed partial denture.

Complete Dentures are used when there are no teeth remaining in one or both jaws. How well the denture fits depends on how much bone remains after the teeth are lost. There are no studies that compare bone loss under dentures in persons with OI to persons without OI. The bone loss that occurs when teeth are lost is a resorption of the bone, not a fracture process, so it is not known if bone loss would be more rapid in people with OI.

Removable Partial Dentures are used when some teeth remain in one or both jaws. A denture, typically made with a metal framework for strength and retention, is constructed to replace missing teeth.

Wound Healing in Bone

A dynamic physiological process is needed for bone healing. The two principal physiological processes required for any adaptive or reparative responses in bone are bone modeling and bone remodeling.

Bone modeling involves a change in shape or size of bone. It is the process that is visible during growth and wound repair. It is an "uncoupled" process, which means that bone resorption and formation are independent of each other. In other words, prior resorption or loss of bone is not necessary to form new bone.

Bone remodeling refers to the turnover of existing bone. This is a normal replacement process that takes place to renew damaged bone or to provide metabolic calcium for the body's use. Remodeling is a "coupled" process in which resorption, or loss of existing bone, must precede formation of the new bone in its place.

The healing of any bone wound, be it traumatic or surgical, is accomplished through a coordinated sequence of bone modeling and remodeling. The initial healing events are bone modeling-based and take place during the first 18 weeks following trauma or surgery. During this phase, "anabolic" modeling, or the formation of woven bone callus, occurs. Compaction of the callus follows. This involves formation of lamellar bone on the woven bone matrix to make it more dense and create composite bone, which is composed of a mix of woven and lamellar bone. Subsequent to regaining strength, the compacted callus may be "catabolically modeled" or removed.

The second set of healing events is bone remodeling-based. This phase takes place from about 18 to 52 weeks following the trauma or surgery, and involves remodeling of bone that was devitalized at the wound site in order to revitalize it. Adjacent bone is remodeled to enable it to adapt to the new environment in the region of the wound. Over time, the rate of bone turnover is diminished back to the basal or normal level.

Bisphosphonates, Teeth and Implants

The class of drugs known as bisphosphonates is being used experimentally in an increasing number of children and adults for the treatment of OI. These drugs work by reducing the remodeling rate. In general, these drugs do not appear to affect bone modeling, although there are reports in the literature of larger callus size in some fracture healing studies. Rates for remodeling in bone surrounding teeth are typically higher than in other bones of the body.

Bisphosphonates reduce this remodeling as well, and it is not clear what impact this will have long term. In the short term, reduction of the remodeling rate produces bone with a greater density, although it is not clear if this results in greater strength. It is even more unclear what effect bisphosphonates have on young children whose new teeth are erupting as they grow. Similarly, the effect of bisphosphonates on the necessary remodeling surrounding dental implants is not understood.

Locating a Dentist

There is no national list of dentists who treat patients with OI. Schools of dentistry or the dental department at major medical centers may be helpful in locating dentists who are familiar with OI and DI. Referrals from others in the OI community may also be helpful. The American Academy of Pediatric Dentistry is a good source of pediatric dentists, although any particular member of this group may or may not see OI patients. Contact the Academy at:

American Academy of Pediatric Dentistry
211 East Chicago Avenue, #700
Chicago, IL 60611-2663
Telephone: (312) 337-2169
Fax: (312) 337-6329

Dental/orthodontic insurance, or medical insurance that covers dental diagnosis and treatment as a part of having OI, may cover some of the costs of dental care for people with OI. Some states also have financial assistance programs that may provide assistance.

The references included with this summary are an introduction to the professional literature about dental care for persons who have OI. Readers are encouraged to share this information with their dentists, orthodontists and other dental care providers.

This fact sheet was prepared by James K. Hartsfield, Jr., D.M.D., Ph.D. and Lawrence P. Garetto, Ph.D. from Indiana University Schools of Dentistry and Medicine.

References:
Bell R.B., White R.P. Jr. "Osteogenesis imperfecta and orthognathic surgery: case report with long-term follow-up." Int J Adult Orthodon Orthognath Surg 15 (2000): 171-178.

Byers P.H. "Osteogenesis Imperfecta," In: Connective Tissue and its Heritable Disorders. Wiley-Liss, Inc., pages 317-350.

Feigal R.J., King K.J. "Dental Care for Patients with Osteogenesis Imperfecta," In: Managing Osteogenesis Imperfecta, A Medical Manual, edited by P. Wacaster and published by the Osteogenesis Imperfecta Foundation, Inc. (1996): 109-117.

Gibbard P.D. "The management of children and adolescents suffering from amelogenesis imperfecta and dentinogenesis imperfecta." Int J Orthod 12 (1974): 15-25.

Hartsfield J.K. Jr. "Summary of Dental Concerns and Care for Persons with Dentinogenesis Imperfecta and Osteogenesis Imperfecta." Breakthrough, The National Newsletter of the Osteogenesis Imperfecta Foundation, Inc. (Spring/Summer 1992): 4-5.

Jorgenson R.J. "Dentinogenesis Imperfecta and other Dental Concerns," In: Living with Osteogenesis Imperfecta A Guidebook for Families, edited by HC Glauser and published by the Osteogenesis Imperfecta Foundation, Inc. (1994): 49-54.

Lewis M.K., Stoker N.G. "Surgical management of the patient with osteogenesis imperfecta." J Oral Maxillofac Surg 45 (1987): 430-437.

Lund A.M., Jensen B.L., Nielsen L.A., Skovby F. "Dental manifestations of osteogenesis imperfecta and abnormalities of collagen I metabolism." J Craniofac Genet Dev Biol 18 (1988): 30-37.

O'Connell A.C., Marini J.C. "Evaluation of oral problems in an osteogenesis imperfecta population." Oral Surg Oral Med Oral Pathol Oral Radiol Endod 87 (1999): 189-196.

Ormiston I.W., Tideman H. "Orthognathic surgery in osteogenesis imperfecta: a case report with management considerations." J Craniomaxillofac Surg 23 (1995): 261-265.

Rodrigo C. "Anesthesia for maxillary and mandibular osteotomies in osteogenesis imperfecta." Anesth Prog 42 (1995): 17-20.

This information is brought to you by the
NIH Osteoporosis and Related Bone Diseases~National Resource Center (ORBD~NRC)
and the Osteogenesis Imperfecta Foundation

National Institutes of Health
Osteoporosis and Related Bone Diseases
National Resource Center
1232 22nd St., NW
Washington, DC 20037-1292
Tel: 800/624-BONE or 202/223-0344
Fax: 202/293-2356, TYY: 202/466-4315
http://www.osteo.org
E-mail: orbdnrc@nof.org

The National Resource Center is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases with contributions from the National Institute of Child Health and Human Development, National Institute of Dental and Craniofacial Research, National Institute of Environmental Health Sciences, NIH Office of Research on Women's Health, Office of Women's Health, PHS, and the National Institute on Aging. The Resource Center is operated by the National Osteoporosis Foundation, in collaboration with the Paget Foundation and the Osteogenesis Imperfecta Foundation.