Assessment of telescoping nail in the management of long bones fractures and deformities in patients with osteogenesis imperfecta

Abstract

Background: Osteogenesis imperfecta (OI) is a genetic disorder characterized by increased bone fragility and multiple fractures. Approximately 90% are caused by mutation in COL1A1 or COL1A2 genes, the disease is transmitted either autosomal dominantly or recessively or appears as a spontaneous mutation. Protein product of these two genes is type I collagen, the major structural protein in the bone and skin, which is affected in quality, quantity or both. The most distinct feature of this disease is repeated fractures and deformities which are very challenging to treat. Making the bone harder by bisphosphonate treatment and by Intramedullary splinting of long bones is the standard of care in most pediatric orthopedic centers. The problem of treating young child with a fixed length intramedullary rod, is overgrowth of the bone beyond the edge of the nail, creating a segment of bone which is unsupported and very liable to fractures and deformities. The need for a device that elongates with bone growth (telescoping intramedullary nail) was necessary to overcome this problem. There were different types of telescoping Intramedullary nails (baily-dubow, Sheffield, interlocking telescoping nail that has been used for the treatment of Osteogenesis imperfecta.