Osteogenesis imperfecta (OI) is a heterogeneous group of conditions affecting bone mass and fragility. It is a highly variable disease that is usually secondary to an abnormality in type I collagen synthesis or extracellular secretion. However, some OI patients with normal type I collagen apparently have mutations affecting other bone proteins. The hallmark sign of OI ("brittle bone disease") is an increased incidence of bone fracture, usually resulting from minimal if any trauma. In addition to a variable decrease in bone mass, there is abnormal tissue organization and aberrant morphology (size and shape) of bones. There are associated craniofacial and dental manifestations that may include dentinogenesis imperfecta (DI), a hypoplastic maxilla, and hypodontia, among others. Variable expression of dentin developmental defects has been documented, with approximately one-fourth to three-fourths of the cases showing some manifestation of DI, depending to some degree on the type of OI. To control the elevated rate of bone remodeling, some OI patients are treated with bisphosphonates, drugs that inhibit osteoclastic resorption. Bisphosphonate treatment may introduce additional problems, which have been observed in other patients, such as decreased rates of tooth movement, problems maintaining dental implant integration, or osteonecrosis following dental extractions. A review of the literature reveals that there is very little published about OI patients having orthodontic treatment. There are no reports for OI patients being treated with bisphosphonates and orthodontics, nor are there controlled studies of the outcomes of orthognathic surgery and/or orthodontic treatment in these patients. This article will present two case reports of OI patients with different manifestations of the disease that were treated with comprehensive orthodontic therapy with and without orthognathic surgery. Diagnosis, treatment planning, and clinical procedures will be reviewed to alert orthodontists about the variability of the OI disease process and the special needs of affected patients. Clinical recommendations must be tempered by the particular characteristics and condition of the affected individual. Unique collagen or other bone protein mutation(s) may influence the expression of the OI and the response to treatment as well.
|Number of pages||18|
|Journal||Seminars in Orthodontics|
|State||Published - Dec 2006|
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