There is no cure for<unk> . Maintaining a healthy lifestyle by exercising and avoiding smoking can help prevent fractures. Treatment may include care of broken bones, pain medication, physical therapy, mobility aids such as braces or wheelchairs, and surgery. Judging the success or failure of treatment can be difficult in<unk>  patients, as decreased bone fracture rates may just be coincidental. While these rates are often used in medical studies to judge treatment efficacy, a Norwegian study of fifteen people with<unk>  emphasized that they feel doctors should consider the whole patient and not just fracture rates. Acute bone fracture care Bone fractures are treated in individuals with<unk>  in much the same way as they are treated in the general population—OI bone heals at the same rate as non-OI bone. A greater emphasis is placed on using lightweight materials to immobilize the fracture, as in moderate or severe types of<unk> , using heavy casts, such as hip spica casts, can cause fractures at the bones at the boundaries of the cast, as well as generalized osteopenia. The lightweight cast or splint is then replaced with a removable orthosis after a few weeks and once evidence of union is seen on X-ray. In order to prevent a nonunion or malunion, all fractures should be immobilized, even if the fracture seems trivial (microfracture), as people with<unk>  are at greater risk of nonunion. Bone infections secondary to fractures are treated as and when they occur with the appropriate antibiotics and antiseptics, as in the general population. Medications Bisphosphonates Pamidronate In 1998, an initial observational trial demonstrated the effectiveness of intravenous pamidronate, a bisphosphonate which had previously been used in adults to treat osteoporosis. In severe<unk> , this trial showed that pamidronate reduced bone pain, prevented new vertebral fractures, reshaped previously fractured vertebral bodies, and reduced the number of long-bone fractures. Although oral bisphosphonates are more convenient and cheaper, they are not absorbed as well, and intravenous bisphosphonates are generally more effective, although this is under study. Some studies have found oral and intravenous bisphosphonates, such as oral alendronate and intravenous pamidronate, equivalent. In a 2013 double-blind trial of children with mild<unk> , oral risedronate increased bone mineral densities, and reduced nonvertebral fractures. However, it did not decrease new vertebral fractures. A Cochrane review in 2016 concluded that though bisphosphonates seem to improve bone mineral density, it is uncertain whether this leads either to a reduction in bone fractures or improvement in the quality of life of individuals with<unk> . Even in trials with as many as 125 children, no causal link has been found between bisphosphonates and decreased fracture rates; placebo controlled trials were also unable to prove that they brought about increased strength, motor control or lower pain levels. Bisphosphonates are not as effective at increasing the bone mineral density of adults. Nutritional supplements<unk>  is a genetic disorder and is not caused by insufficient intake of any vitamin or mineral; supplementation cannot cure<unk> . With that said, people with<unk>  tend to be severely deficient in vitamin D at much higher rates than the general population, and the cause of this is not well understood. The severity of the deficiency and the likelihood of its occurrence is thought to be related to severity of<unk> . Vitamin D supplementation may be recommended, at least until levels of 25(OH)D3 in blood return to normal. Below normal levels of vitamin D are also concerning as they may decrease the benefit of bisphosphanates. Surgery A surgery of any type inherently carries more risks when done on a patient who has (especially moderate to severe)<unk> . Skeletal deformities and dentinogenesis<unk>  may hinder access to the airway. Use of, and weaning off of, mechanical ventilation is also more challenging to carry out on patients with<unk> . During the procedure itself or the healing process, defective<unk>  collagen may lead to bleeding diatheses. The safety of anesthesia is also of more concern among patients with<unk> , with anesthetic complications 5.6x more likely to occur when the patient has<unk>  type III. A unique concern of anesthesia in<unk>  is perioperative fracture—fractures sustained due to patient transfer and airway access techniques that, while routine when a patient's bones are strong, may cause injury with brittle<unk>  bones. As an example, due to a 1972 report of a humerus fracture from a sphygmomanometer cuff sustained in an<unk>  patient during surgery, blood pressure monitoring protocols are often modified for patients with<unk> , with neonatal size cuffs and machine settings being used even in adults; further, the least deformed of the patient's limbs is preferred to receive the cuff. Rodding Metal rods can be surgically inserted in the long bones to improve strength, a procedure developed by Harold A. Sofield when he was Chief of Staff at Chicago's Shriners Hospitals for Children, a hospital that offers orthopedic care and surgery to children regardless of their family's ability to pay. Large numbers of children with<unk>  came to Shriners, and Sofield experimented with various methods to strengthen their bones. In 1959, with Edward A. , Sofield wrote a seminal article describing a three-part surgery that seemed radical at the time: precisely breaking the bones ("fragmentation"), putting the resulting bone fragments in a straight line ("realignment"), then placing metal rods into the intramedullary canals of the long bones to stabilize and strengthen them ("rod fixation"). His treatment proved useful for increasing the mobility of people with<unk> , and it has been adopted throughout the world—it became standard surgical treatment for severe<unk>  by 1979, in which year David Sillence found that ≈ of the patients he surveyed with<unk>  type III had undergone at least one rodding surgery. Rodding surgery is often done with the hope that it will offer a path to ambulation, walking, to patients with moderate or severe<unk> . A 2020 review in The Journal of Bone and Joint Surgery (JB&JS) found it remains broadly popular: ≈ of people with<unk>  types III and IV (severe<unk>  have undergone some form of rodding surgery in their lives, at a mean age of 4 and 7 years respectively; one possible explanation for a tendency towards earlier intervention in type III is that one half of affected children could not walk at all without the surgery, as their limbs were more bowed, so surgery was sought sooner. In those with type III<unk>  who had undergone rodding surgery, 79.5% had the femurs and tibias of both legs rodded. The most common form of rods used are intramedullary (IM) rods, some of which, such as the Fassier–Duval IM rod, are telescoping, meaning that they are designed to grow as the child grows, in an attempt to avoid the necessity of revision surgeries. Telescoping IM rods are widely used, and the common Fassier–Duval IM rod is designed to be used to rod the femur, tibia, and humerus. The surgery involves breaking the long bones in between one and three (or more) places, then fixing the rod alongside the bone to keep it straight. While telescoping IM rods are intended to grow along with both the femur and tibia in developing children; surgeons have a preference to use non-telescoping IM rods, such as Rush rods, in the tibia, which grows less comparatively—the JB&JS review found that while 69.7% of femurs were treated with telescoping IM rods, only 36.9% of tibiae were. While the review in the JB&JS was able to correlate receiving rodding surgery with greater mobility across all types of<unk> , in patients with type IV, the surgery did not decrease the incidence of broken bones as compared to non-rodded patients—while type IV patients with rodded tibiae experienced 0.93 tibia fractures per year, patients with natural tibiae experienced only 0.81. However, in patients with type III, rodding surgery decreased the average number of tibia fractures per year from 0.84 to 0.57. Spinal Spinal fusion can be performed either as a preventative measure or to correct existing scoliosis, although the inherent fragility of<unk>  bone makes this operation more complex in<unk>  patients than it does with patients who have adolescent idiopathic scoliosis, but normal bone density. Despite the risks, however, three Nemours–duPont orthopedic surgeons who specialize in surgical intervention for<unk>  recommend operating if the curve is greater than 50° after a child is past peak height velocity, as the spine's curve can continue to worsen even into adulthood. Due to the risk involved, the same surgeons recommend that surgery for basilar impressions and basilar invaginations should only be carried out if the pressure being exerted on the spinal cord and brain stem is causing actual neurological symptoms. Once basilar invagination has become symptomatic, only surgery can halt or reverse the progression of neurological deficits. Physical therapy Physical therapy is generally recommended, however individualized protocols are required due to the variability of<unk> . Physical therapy is used to strengthen muscles, improve motility, improve flexibility, and help with weight maintenance, although it must be done in a gentle
Osteogenesis imperfecta
Lhermitte's sign
Dentinogenesis imperfecta
Cleidocranial dysostosis
Gerodermia osteodysplastica
Kyrle disease
Rhizomelic chondrodysplasia punctata
Acrodysostosis