神经纤维瘤病性脊柱侧凸的诊断与治疗

The Diagnosis and Treatment of Scoliosis with Neurofibromatosis

本文通过文献复习及对７例神经纤维瘤病性脊柱侧凸病例的分析，着重讨论了本病的诊断，治疗和预后的特征。作为诊断标准，成人皮肤色素沉着直径大于１．５ｃｍ，小儿大于０．５ｃｍ，且具有６个以上者才有诊断价值。本组７例均符合标准。典型的Ｘ线表现为椎体的营养不良性改变。本组７个弯曲共累及３９个椎体，平均每个弯曲累及５．５个椎体。５例行手术治疗，术中自体植骨结合大量异体骨移植。２例行保守治疗。对有椎体营养不良者，应及早行手术治疗。由于骨的营养不良改变，植骨不易融合。尽管完美的手术和植骨其假关节的发生率相当高，故应高度重视植骨融合的质和量。

By reviewed literatures and analysed 7 cases of neurofibromatosis with scoliosis,we discussed the character of diagnosis,treatment and prognosis of this disease.The lesions that typically characterize neurofbromatosis are cafe ua lait spots,in the adult,an individual with 6 or more spots measuring 1 5 cm or gerater in diameter and a child who has spots measuring 0 5 cm or greater in diameter should be considered to have neurofbromatosis.All of 7 cases accorded with this standards.The typical radiographic deformities seen in the spine with neurofibromatosis are dystrophic bony changes.The curves in the 7 patients involved 39 vertebrae,each curve involving 5 5 vertebrae on average.5 cases had surgical treatment with abundant allogeneic bone graft.2 cases had conservative treatment.It is the dystrophic spine defomity that exhibits an unremitting progression with non operative care and must be dealt with surgically.Despite meticulous technique pseudarthrosis is common.So it is important to perform a high quality of bone graft and fusion.