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MINERVA ORTOPEDICA E TRAUMATOLOGICA
A Journal on Orthopedics and Traumatology
BONE AND JOINT DEFORMITIES - SCIENTIFIC PAPERS 100° CONGRESS OF THE PIEMONTESE-LIGURIAN-LOMBARD SOCIETY OF ORTHOPAEDICS AND TRAUMATOLOGY (S.P.L.L.O.T.) - Pavia, June 20-21, 2003
Minerva Ortopedica e Traumatologica 2003 June;54(3):174-9
Surgical treatment of long-bone deformities in osteogenesis imperfecta. Technical notes
Brunelli P.C., Pianelli A.
Divisione di Ortopedia Pediatrica, Spedali Civili, Brescia
Osteogenesis imperfecta is caused by an altered synthesis of type I collagen, the most important structural protein of the bone, leading to bone fragility, fractures and deformities. Although pharmacological therapy has made considerable progress in recent years, in severe forms of the disease surgical treatment remains the sole means to correct deformities, prevent fractures, and improve motor performance. The surgical technique involves multiple corrective osteotomy and synthesis using various types of fixation nails. Numbering among the ones we used are the classic, the more recent elastic and the telescopic nail. These operations are highly complex and complicated by a range of problems including adequate approach to so deformed and fragile a bone structure, wide exposure, and difficulty in planning osteotomy. The lesser known telescopic nails are characterized by certain peculiarities in surgical technique. Here again difficulties abound, the main concerns being migration, difficulty in consolidation, deformation or rupture of the synthesis medium and problems specific to the telescopic nail, including blocked expansion or loss of anchorage points. Preoperative multidisciplinary assessment is valuable to avoid general complications, while from an orthopedic standpoint the integration of surgical and rehabilitation programs is of fundamental importance. Despite the technical difficulties involved, the quality of the functional results achieved with surgical therapy have made this method universally accepted in the treatment of osteogenesis imperfecta.