Abstract
OBJETIVO: Fazer uma revisão dos pacientes portadores de Osteogênese Imperfeita avaliando o tratamento cirúrgico das fraturas e deformidades nos membros inferiores para determinar a eficiência da técnica utilizando as hastes fixas (não-extensíveis). CASUÍSTICA E MÉTODO: Foram revisados os prontuários, radiografias pré-operatórias e pós-operatórias de todos os pacientes portadores de Osteogênese Imperfeita que foram tratados no Alfred I duPont Institute (EUA) entre 1965 e 1999. RESULTADOS: Quatorze pacientes (cinco meninos e nove meninas) foram submetidos às hastes fixas nos membros inferiores com um total de 37 procedimentos realizados. CONCLUSÃO: O procedimento de fixação intramedular com hastes não extensíveis mostrou ser um método de baixa morbidade, capaz de manter e até mesmo de melhorar o status de deambulador destes pacientes.
Highlights
Osteogenesis imperfecta, according to Melebranche, in 1684, Ekman, in 1788, Lobstein, in 1834, Vrolick, in 1845, and Porak-Durante, in 1905 apud Correa et al[1] was described as a syndrome caused by a change on the connective tissue involving type-I collagen, which is the organic component of the bone
The classification by Sillence[3], published in 1979, has been employed for grouping these patients. The orthopaedic treatment, both for fractures and for deformities correction, which consists of corrective osteotomy and stabilization of those bones, using several kinds of implant materials for osteosynthesis has been used by a number of authors.[4,5,6,7,8,9]
Osteosynthesis can be made with fixed or non-extensible nails, which may present complications due to bone growth, such as nail protrusion through cortical, with recurrence of deformity, and extensible nails with different fixation techniques on the epiphyseal region of the long bones, targeting nail stretching as the bone grows, avoiding complications and reducing the required time between procedures
Summary
Osteogenesis imperfecta, according to Melebranche, in 1684, Ekman, in 1788, Lobstein, in 1834, Vrolick, in 1845, and Porak-Durante, in 1905 apud Correa et al[1] was described as a syndrome caused by a change on the connective tissue involving type-I collagen, which is the organic component of the bone. Surgical treatment of deformities and fractures on lower limbs with osteogenesis imperfecta. This procedure using fixed (non-extensible) nails allows long bones growth to “create” bone segments without nail protection, which occasionally presents fractures or deformities requiring new surgeries.
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