- 作者：Moez Trigui1 ; dr_moez_trigui@yahoo.fr ; Kamel Ayadi1 ; Mourad Sakka1 ; Wassim Zribi1 ; Faten Frikha2 ; Fakher Gdoura1 ; Sami Sallemi1 ; Mohamed Zribi1 ; Hassib Keskes1
- 刊名：La Presse M¡§|dicale
- 出版年：2011
- 出版时间：March 2011
- 年：2011
- 卷：40
- 期：3
- 页码：e152-e162
- 全文大小：463 K
Summary
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??nd 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 ? 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.