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Orthopaedic treatment in OI.
J.E.H. Pruijs Wilhelmina Children's Hospital, Department of Pediatric Orthopedics, Utrecht, The Netherlands. |
Children with osteogenesis imperfecta suffer from osteoporosis in various degrees of severity due to a defect in the genes that code for collagen I. As a result bones are vulnerable for internal and external force causing fractures, bowing of the long bones, spinal deformities and many other abnormalities. Orthopaedic treatment focuses on surgical and non-surgical stabilisation of the long bones and spine. Indications for orthopaedic treatment are pain and discomfort but orthopaedic treatment can also be indicated for improvement of function of the patient. A multidisciplinary approach is highly recommendable for the optimal timing of surgical intervention since pulmonary, haematological and neurological complications may compromise postoperative rehabilitation. Treatment of scoliosis and basilar invagination is possible since the development of new techniques in spinal surgery in the eighties. For treatment of the long bones, intramedullary nailing introduced by Sofield and Millar already forty years ago, has been a major step ahead in the treatment of children with OI. Bailey and Dubow tried to improve this treatment by introducing extendable rods in 1981. Although much discussion on which of these two methods is superior still continues, the method of intramedullary nailing has proven to be superior to other methods of fracture treatment. Therefore it is amazing to see that even in our country fractures in OI are still treated with plates and screws. Apparently information needs to be spread more widely. Hopefully the development of patient organisations will have substantial part in this process. An intense interaction between patient organisations and caretakers is necessary to create maximum knowledge on both sides. The degree of osteoporosis is a main determinant in the final outcome of treatment of children with OI. Already existing osteoporosis can become worse by immobilisation for fracture treatment causing an increased danger for new fractures. Therefore early weightbearing after surgery is essential and even preventive nailing in case of excessive bowing or frequent fracturing may be necessary. Increased osteoporosis and fracture frequency has also been reported in periods of rapid growth like puberty and the indications for surgical and non-surgical treatment of osteoporosis and its sequels should be even more precise in these periods. Various non-surgical treatments to improve bone quality are currently under investigation. Effects of bisphosphonates seem promising. Bone marrow transplants can be an alternative but this treatment has a higher morbidity. Gene therapy may be the final answer, but is not yet available. References: • Bailey RW and Dubow HI. Studies of longitudinal bone growth resul-ting in an extensible nail. Surgical Forum 1963; 14: 455-8 • Engelbert RHH, Helders PJM, Keessen W, Pruijs JEH, Gooskens RHJM. Intramedullary rodding in type III osteogenesis imperfecta. Effects on motor neuron development in 10 children. Acta Orthop Scand 1995;66(4):361-4. • Hanscom DA, Bloom BA. The spine in Osteogenesis Imperfecta. Orthop Clin North Am 1988;19:449-458. • Parfitt AM. The two faces of growth: benefits and risks to bone integrity. Osteoporos Int 1994;4:382-98. • Sofield HA and Miller EA. Fragmentation, realignment and intrame-dullary rod fixation of deformities of the long bones in children. J Bone Joint Surg [Am] 1959; 41A: 1371-91 Reference: Proceedings of the 7th International Conference on Osteogenesis Imperfecta. Montreal, Canada, 1999. |