Choksey Kevin R, Modi Jayprakash V
Department of Orthopaedics, B.J. Medical College, Ahmedabad. India.
J Orthop Case Rep. 2015 Jul-Sep;5(3):60-2. doi: 10.13107/jocr.2250-0685.310.
Dystrophic neurofibromatosis type I, involving upper cervical spines, is rare, which can cause serious complications. Myelopathy develops due to compression of the cord posteriorly. Surgical correction has its inherent risks and difficulties because of poor bone quality, difficult anterior approach because of bizarre deformities, and the necessary manipulation, which might cause more cord damage and ischemia. Anterior decompression with alignment correction was an early popular choice. But without posterior shortening, the technique proved unsatisfactory in restoring normal alignment. It tended to expose the graft bone at increased risk of insufficient union or extruding. So a combination of anterior decompression and posterior correction was generally recommended. This report describes surgical technique applicable to cases of severe cervical kyphosis using only anterior approach.
A 13-year-old boy, a case of neurofibromatosis type 1 presented with neck pain, and bilateral upper limb radiculopathy for 2 months with affected daily living, with intact neurology. Imaging demonstrated 46° kyphosis (C3-C5), marked dystrophic changes of the C4 and C5, extreme dorsal angulation indenting the cord. Anterior surgery was planned for the patient with neuromonitoring. Through left anterior approach, total C3, C4 and C5 corpectomy was accomplished, keeping the PLL intact. The defect was filled with a fibular cortical graft which was stabilized with 48 mm cervical screw plate, showing full correction of kyphosis postoperatively. At the follow-up 6 months postoperatively, lateral plain radiograph showed stability of the cervical spine fusion without correction loss. The patient had full relief from tingling and numbness with no neurological deficit and could resume his daily duties. Patient is asymptomatic at 2 years follow up.
Only anterior decompression and fusion are effective in satisfactorily correcting cervical kyphosis in cases of neurofibromatosis without neurologic compromise and avoids the risk of damage to vital neurovascular structures because of pedicle or lateral mass screw fixation.
累及上颈椎的I型营养不良性神经纤维瘤病较为罕见，可导致严重并发症。脊髓病是由于脊髓后方受压所致。由于骨质质量差、畸形怪异导致前路手术困难以及必要的操作可能会导致更多脊髓损伤和缺血，手术矫正存在其固有的风险和困难。前路减压并矫正畸形是早期常用的选择。但如果没有后路缩短，该技术在恢复正常对线方面并不理想。它往往会使移植骨暴露，增加骨不连或骨块脱出的风险。因此，一般建议采用前路减压和后路矫正相结合的方法。本报告描述了仅采用前路手术治疗严重颈椎后凸畸形病例的手术技术。
一名13岁男孩，患有I型神经纤维瘤病，出现颈部疼痛和双侧上肢神经根病2个月，日常生活受到影响，神经功能正常。影像学检查显示颈椎后凸46°（C3 - C5），C4和C5有明显的营养不良性改变，极度的背侧成角压迫脊髓。计划对该患者进行前路手术并进行神经监测。通过左前路入路，完成了C3、C4和C5椎体次全切除，保留后纵韧带完整。缺损处用腓骨皮质骨移植填充，并用48mm颈椎螺钉钢板固定，术后后凸畸形得到完全矫正。术后6个月随访时，颈椎侧位X线片显示颈椎融合稳定，无矫正丢失。患者的刺痛和麻木感完全缓解，无神经功能缺损，能够恢复日常活动。随访2年时患者无症状。
对于无神经功能损害的神经纤维瘤病病例，仅前路减压和融合术就能有效满意地矫正颈椎后凸畸形，并且避免了因椎弓根或侧块螺钉固定而损伤重要神经血管结构的风险。
