RESUMO
OBJECTIVE: Von Recklinghausen's neurofibromatosis is a dominant autosomic genetic disease characterized by different clinical manifestations. The goal of this work was to study its orthopaedic manifestations and to show the characteristics of their management. METHOD: A retrospective study was carried out on 15 patients having a Von Recklinghausen's neurofibromatosis. For each patient, different orthopaedic manifestations and their evolution after treatment were analyzed. These manifestations were classified in spinal deformities, pseudarthrosis of long bones and tumours of the peripheral nerves. RESULTS: The spinal deformities were observed in 9 cases. A dystrophic scoliosis was observed in 6 patients with an average angle of 50° and was associated to a kyphosis in 5 patients. The treatment was surgical by posterior arthrodesis in 2 cases and circumferential arthrodesis in 2 cases. The congenital curves and pseudarthroses of leg were observed in 5 cases, localized at the lower third of the leg in all cases. An Ilizarov external fixator with segmental osseous transport was carried out in 2 patients. The duration of the external fixator was 23 months ½ with 5 interventions in each case. Four plexiform neurofibromas and 3 nodular neurofibromas were observed. A transformation into neurofibrosarcoma was found in 2 patients. In one case, a resection without functional sacrifice was carried out and in the other case the patient was dead before the resection. CONCLUSION: The orthopaedic manifestations of Von Recklinghausen's neurofibromatosis are frequent, varied and have a difficult management. The functional and sometimes vital prognoses are challenging.
Assuntos
Cifose/diagnóstico , Neurofibromatose 1/diagnóstico , Neoplasias do Sistema Nervoso Periférico/diagnóstico , Pseudoartrose/diagnóstico , Escoliose/diagnóstico , Adolescente , Adulto , Idade de Início , Transformação Celular Neoplásica/patologia , Criança , Pré-Escolar , Feminino , Humanos , Técnica de Ilizarov , Lactente , Cifose/genética , Cifose/cirurgia , Masculino , Pessoa de Meia-Idade , Neurofibroma Plexiforme/diagnóstico , Neurofibroma Plexiforme/cirurgia , Neurofibromatose 1/genética , Neurofibromatose 1/cirurgia , Neurofibrossarcoma/diagnóstico , Neurofibrossarcoma/cirurgia , Osteotomia , Neoplasias do Sistema Nervoso Periférico/genética , Neoplasias do Sistema Nervoso Periférico/cirurgia , Prognóstico , Pseudoartrose/genética , Pseudoartrose/cirurgia , Estudos Retrospectivos , Escoliose/genética , Escoliose/cirurgia , Fusão Vertebral , Tomografia Computadorizada por Raios XRESUMO
In this study, we report a rare fracture of the proximal radial metaphysis in an 11-year-old child, involving an anterior displacement of the proximal radial metaphysis instead of a displacement of the epiphysis, which preserved its normal connections with the capitellum. This fracture was successfully treated by closed reduction and wire fixation. The reduction maneuver attempted to reduce the proximal radial metaphysis under the epiphysis instead of doing the opposite. The mechanism of this injury is probably an extension strain on a forearm in pronation associated with a violent contraction of the biceps brachialis muscle. This fracture is considered as a rare example of Wilkins group II angular fracture of the proximal radial metaphysis, without proximal ulnar fracture.
