Satyarthee Guru Dutta, Mankotia Dipanker Singh
Department of Neurosurgery, All Institute of Medical Sciences, New Delhi, India.
J Pediatr Neurosci. 2016 Jul-Sep;11(3):237-240. doi: 10.4103/1817-1745.193370.
Morquio's syndrome is associated with systemic skeletal hypoplasia leading to generalized skeletal deformation. The hypoplasia of odontoid process is frequent association, which is responsible for atlantoaxial dislocation causing compressive myelopathy. However, development of sub-axial cervical kyphotic deformity unassociated with odontoid hypoplasia is extremely rare, and coexistence of dorsal kyphotic deformity is not reported in the western literature till date and represents first case. Current case is 16-year-old boy, who presented with severe kyphotic deformity of cervical spine with spastic quadriparesis. Interestingly, he also had additional asymptomatic kyphotic deformity of dorsal spine; however, odontoid proves hypoplasia was not observed. He was only symptomatic for cervical compression, accordingly surgery was planned. The patient was planned for correction of cervical kyphotic deformity under general anesthesia, underwent fourth cervical corpectomy with resection of posterior longitudinal ligament and fusion with autologous bone graft derived from right fibula, which was refashioned approximating to the width of the corpectomy size after harvesting and fixed between C3 and C5 vertebral bodies and further secured with anterior cervical plating. He tolerated surgical procedure well with improvement in power with significant reduction in spasticity. Postoperative X-ray, cervical spine revealed complete correction of kyphotic deformity cervical spine. At follow-up 6 months following surgery, he is doing well. Successful surgical correction of symptomatic cervical kyphotic deformity can be achieved utilizing anterior cervical corpectomy, autologous fibular bone graft, and anterior cervical plating.
黏多糖贮积症Ⅳ型与全身性骨骼发育不全相关,可导致全身骨骼变形。齿状突发育不全是常见的伴发情况,可导致寰枢椎脱位,引起压迫性脊髓病。然而,颈椎下颈椎后凸畸形的发生与齿状突发育不全无关,极为罕见,西方文献至今未报道过伴有胸椎后凸畸形的病例,本病例为首例。本例患者为一名16岁男孩,表现为颈椎严重后凸畸形伴痉挛性四肢瘫。有趣的是,他还伴有胸椎无症状性后凸畸形;然而,未观察到齿状突发育不全。他仅表现为颈椎受压症状,因此计划进行手术。患者计划在全身麻醉下矫正颈椎后凸畸形,接受了第四颈椎椎体次全切除术,切除后纵韧带,并采用取自右腓骨的自体骨移植进行融合,腓骨在获取后重新塑形,使其宽度接近椎体次全切除的尺寸,固定于C3和C5椎体之间,并进一步用颈椎前路钢板固定。他对手术耐受良好,肌力有所改善,痉挛明显减轻。术后颈椎X线显示颈椎后凸畸形完全矫正。术后6个月随访时,他恢复良好。利用颈椎前路椎体次全切除术、自体腓骨移植和颈椎前路钢板固定,可成功地对有症状的颈椎后凸畸形进行手术矫正。