Yang Huiliang, Zhou Chunguang, Song Yueming
Department of Orthopedics, West China Hospital, Sichuan University, Chengdu Sichuan, 610041, P. R. China.
Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi. 2016 Sep 8;30(9):1174-1178. doi: 10.7507/1002-1892.20160239.
To review the research progress of pathogenesis mechanism of spinal deformity in neurofibromatosis type 1 (NF1).
Recent literature concerning the pathogenesis mechanism of spinal deformity in NF1 was extensively reviewed, and current developments of the correction of spinal deformity and NF1 and the pathogenesis mechanism were summarized.
The pathogenesis mechanism of spinal deformity in NF1 is not yet clearly known. Current theories include erosion and stress of neurofibromas, melatonin-related decreased contractility of paraspinal muscles, osteopenia and osteoporosis, sexual precocity and mesoderm dysplasia.
The clinical manifestations of NF1 may cause the spinal deformities in patients with NF1. The research of pathogenesis mechanism of spinal deformity in NF1 will be conducive to further understanding, diagnosis and treatment of NF1-related spinal deformity.
回顾1型神经纤维瘤病（NF1）脊柱畸形发病机制的研究进展。
广泛查阅近期有关NF1脊柱畸形发病机制的文献，总结脊柱畸形矫正及NF1的当前进展和发病机制。
NF1脊柱畸形的发病机制尚不清楚。目前的理论包括神经纤维瘤的侵蚀和应力、褪黑素相关的椎旁肌收缩力下降、骨质减少和骨质疏松、性早熟及中胚层发育异常。
NF1的临床表现可能导致NF1患者出现脊柱畸形。对NF1脊柱畸形发病机制的研究将有助于进一步了解、诊断和治疗与NF1相关的脊柱畸形。
