儿童神经纤维瘤病Ⅰ型致营养不良性脊柱侧凸的CT表现

Dystrophic scoliosis caused by neurofibromatosis typeⅠin children:CT findings

目的：总结分析神经纤维瘤病I型致营养不良性脊柱侧凸的CT表现。方法：回顾性分析经临床及病理学证实的13例神经纤维瘤病I型致营养不良性脊柱侧凸患儿的CT资料。结果：13例均表现为脊柱节段短弧形侧凸成角，脊柱侧弯Cobb角为26．6°～91．2°，椎体呈不同程度的楔形变及扇贝样改变。8例合并椎管扩大、椎间孔扩大及椎弓、椎板变薄，硬膜囊扩张；11例病变累及肋骨，肋骨表现为不规则变形；12例可见椎旁软组织增厚或包块影。结论：神经纤维瘤病I型致营养不良性脊柱侧凸的CT表现具特征性，有利于监测脊柱侧弯的进展，为临床矫形手术提供更多信息。

Objective：To analyze and summarize the CT findings of dystrophic scoliosis due to neurofibromatosis type I. Methods：Thirteen children with dystrophic scoliosis associated with neurofibromatosis type I ,which was confirmed by clinical and pathological results,were collected. Their CT data were retrospectively reviewed and analyzed. Results： All 13 cases showed a short segment of angel formation and scoliosis,with Cobb＇s angle from 26.6~91.2 degrees. Varying degrees of vertebral wedging and scallop like changes were seen in the vertebral body. Expanded spinal and intervertebral foramens, thinner vertebral arches and vertebral laminas,and enlarged thecal sacs. Eleven cases had evolvement of ribs with irregular deformation. Twelve cases had thickening of para vertebral soft tissue or formation of a mass. Conclusion：Dystropic scoliosis related to neurofibromatosis type I has characteristic CT findings,which is useful for monitoring the progress of the scolio sis, providing more information for the orthopedic surgery and clinical decision.