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Single or multiple fractires may be evident.They are typically transaverse in nature in nature and are more common in the lower extrmity. the fermor in particular. Several fractures at different stages of healing may be visulized. Micromelia and bowing deformities are common as a sequela of fractures in unero or in early childhood.5,8 Other finding a severe kyphoscoliosis in approximately 40% of affected individuals. This deformity is the result of a combianation of ligamentions laxity, osteoporosis, compession fractures, and posttr- aumatic damage to the end plates. Early onset of degenerative koint diease is common as a result of ligamentous laxity and joint incongruity produced by fracture deformity. Skill radiographs may show muliple wormian bones, enlaged frontal and madtoid sinuses, and platybasia with or without basilar invagination.5,8 Currently, there is no adequate thetapy aimed at treating osteogenesis imperfecta. Athough must be directed at mainraining the patient's bone density and providing relief from pain. Growth hormone adminisered to patients with modrate forms of osteogensis imperfecta has shown some benefit in increasing bone mineral density.10 Biphosphonates are currently being evaluated as a treatment option and appear to imptove pain.10,11 Intramedullary rodding of bones, especially of the fenurs and the tibia, helps to reduce the risk of fracture and has been shown to improve and maintain the patient's ability to ambulate.9,10 Surgery is also indicated in patirnts with progresive spinal deformity or basilar impression.10 Swimming may be bemeficialpatients with osteogensis imperfecta because it offers a supprted environment that should reduce the risk for fracture. The high risk for fracture with even the most routine daily activities and the unavilability of adequate treatment options suggest the need for a highly restricive lifestyle. Shea-Landry and Cole6 suggest that one of the roles of a patient of a child with osteogensis imperfacta is to help them resist participation in sports and prevent them from taking physical risks. Patients with the mild type IV forms are usually ambulatory1 and may be more physically active. Few examples of physically active patiens with osteogenesis imperfecta tarda exist in the liter- ature. Case reports include individuals with anvulsion fracture bilaterally at the insertion of the triceps tendon, patellar tendon ruptures after skiing injuries, a rupturen patellar tendon during squash, and an Achilles tendon repture in a soccer player.3,4 Each of these individuals returned to athletic partipation after thier injuries healed. Bones sofening disorders, including osteogensis imperfecta, must be considered in any patient with tendinous ruptures or avulsions in atypical locations, especiooy if the injury is bilateral. The diagnosis of ostogensis imperfecta is an important one for several reasons. Early disgnosis make theparents and child aware of the risks and nanifestation of fracture,a child may be seen with multiple at different stages of healing,which is one of the warning signs of child zbuse. Osteogensis Imperfecta can be further differtiated from nonaccidental injury (NIA), as 80% of fractures related to NIA occur befor the age of 18 months and are rare after the age of 2 years. child abuse or NIA. Alliability in child abuse cases, they may be vulnerable to potenial civil suits as result of the emotional trauma inflicted on the child and parients of a wrongful accusation. a detailed history. thorough examination, and appropriate radiographs will assist in defining the classic manifestions of this condition (ie, diffuse osteoporosis). |