Posted by: shirish June 3, 2005
help mr. baniya's kids
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here is the surgery part: Surgery Fractures in patients with osteogenesis imperfecta Type IA usually are treated in the same way as similar fractures in healthy children. 21 The main difference is that the period of immobilization is shortened. It is important to avoid malunions, particularly in the proximal femur because recurrent fractures, requiring realignment osteotomy and intramedullary rodding, may follow. Progressive scoliosis in adolescence may require spinal fusion with instrumentation. 37 Adults with osteogenesis imperfecta Type IA may have many fractures develop after a period of inactivity or after pregnancies and menopause. 74 Oral bisphosphonates may be needed to improve bone mass. Some children may have recurring fractures as a result of the severity of their osteopenia. Such children also may need to be considered for bisphosphonate treatment although to date, the use of this drug has been confined to patients with the severe forms of the disease. In the prebisphosphonate era, corrective osteotomies and intramedullary roddings often were done to prevent progressive deformities and recurrent fractures in children with severe forms of osteogenesis imperfecta. 10,21 Such surgery often was done in children who were confined to wheelchairs. The surgical technique of fragmentation and rodding has been modified over several decades. 4,85?87 Currently, corrective osteotomies are done through small incisions to preserve the blood supply to the bone and to maximize healing. 22,47,85 Expanding intramedullary rods are used whenever possible. Although many technical problems have been reported, some centers have reported a low rate of complications. 42,44,52,66,85 Randomized trials are needed to determine the indications for and the type of surgery in patients receiving bisphosphonates for severe forms of osteogenesis imperfecta. Current results of bisphosphonate treatment indicate that walking and running can be expected in many children who previously would have died within the first year of life or have been confined to a wheelchair. 3,9,33,46,65 Increased activity levels also have been observed in moderately affected children receiving bisphosphonates. In these children, malunions of subtrochanteric fractures of the femur are common and consequently there is a continuing need to realign the femur and internally support it with a rod. In the prebisphosphonate era, expanding rods were used in preference to nonexpanding rods to stop the distal femur from bowing anteromedially with growth beyond the end of the rod. 66 It is unclear whether it still is necessary to use expanding rods because the new bone formed during growth will have been exposed to bisphosphonates and may no longer bow anteromedially. Intramedullary rodding is used much less frequently in other bones. It may be needed in the tibia, forearm, or humerus. Progressive spinal deformities and basilar impression are two major problems in children with moderate and severe forms of osteogenesis imperfecta. 2,37,38,51,73 Surgical treatment often is difficult or impossible because of the severity of the deformities and the fragility of the spine. 29,37,41,56 It is to be hoped that the early commencement of bisphosphonate treatment will prevent these serious deformities.
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