• Moez Trigui, Kamel Ayadi, Mourad Sakka, Wassim Zribi, Faten Frikha, Fakher Gdoura, Sami Sallemi, Mohamed Zribi, and Hassib Keskes.
• CHU Habib Bourguiba, Faculté de médecine de Sfax, Service de chirurgie orthopédique et traumatologique, 3029 Sfax, Tunisie. dr_moez_trigui@yahoo.fr
• Presse Med. 2011 Mar 1; 40 (3): e152-62.

ObjectiveVon 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.MethodA 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.ResultsThe 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.ConclusionThe orthopaedic manifestations of Von Recklinghausen's neurofibromatosis are frequent, varied and have a difficult management. The functional and sometimes vital prognoses are challenging.Copyright © 2010 Elsevier Masson SAS. All rights reserved.

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