title: [Orthopaedic manifestations of Von Recklinghausen's neurofibromatosis].
abstract: 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  The orthopaedic manifestations of Von Recklinghausen's neurofibromatosis are frequent, varied and have a difficult management. The functional and sometimes vital prognoses are challenging.
authors.affiliation: CHU Habib Bourguiba, Faculté de médecine de Sfax, Service de chirurgie orthopédique et traumatologique, 3029 Sfax, Tunisie. dr_moez_trigui@yahoo.fr
authors.forename: Moez
authors.initials: M
authors.lastname: Trigui
authors.forename: Kamel
authors.initials: K
authors.lastname: Ayadi
authors.forename: Mourad
authors.initials: M
authors.lastname: Sakka
authors.forename: Wassim
authors.initials: W
authors.lastname: Zribi
authors.forename: Faten
authors.initials: F
authors.lastname: Frikha
authors.forename: Fakher
authors.initials: F
authors.lastname: Gdoura
authors.forename: Sami
authors.initials: S
authors.lastname: Sallemi
authors.forename: Mohamed
authors.initials: M
authors.lastname: Zribi
authors.forename: Hassib
authors.initials: H
authors.lastname: Keskes
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002471:Cell Transformation, Neoplastic / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D018889:Ilizarov Technique; D007223:Infant; D007738:Kyphosis / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D008297:Male; D008875:Middle Aged; D018318:Neurofibroma, Plexiform / Q000175:diagnosis / Q000601:surgery; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D018319:Neurofibrosarcoma / Q000175:diagnosis / Q000601:surgery; D010027:Osteotomy; D010524:Peripheral Nervous System Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D011379:Prognosis; D011542:Pseudarthrosis / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D012189:Retrospective Studies; D012600:Scoliosis / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D013123:Spinal Fusion; D014057:Tomography, X-Ray Computed