Abstract
We report a rare case of severe dystrophic cervical kyphosis associated with neurofibromatosis type 1 (NF-1) and highlight the importance of intraoperative neuromonitoring. A 4-year-old girl with café-au-lait spots was diagnosed with NF-1. At age 5 years, she presented with posterior neck protrusion without neurological deficits. Radiographs revealed progressive cervical kyphosis measuring 87° from C2 to C6. One year later, she developed upper limb weakness and gait disturbance. Magnetic resonance imaging demonstrated severe spinal canal stenosis at the C4/C5 level. Posterior instrumentation from the occiput to T2 was performed under intraoperative motor-evoked potential (MEP) monitoring. A significant decrease in MEP signals was observed after final fixation. Removal of lateral mass screws at C3 and C4 and decompression from C2 to C5 resulted in recovery of MEP signals. Anterior spinal fusion from C3 to C7 using an autologous fibular graft was subsequently performed. Postoperatively, cervical kyphosis improved from 87° to 26°, and the patient showed complete neurological recovery at 6 months. Solid bony fusion was confirmed at 8 months. Selumetinib therapy was initiated for unresectable plexiform neurofibromas, resulting in tumor reduction. This case highlights the surgical challenges of dystrophic cervical kyphosis in NF-1 and underscores the critical role of intraoperative neuromonitoring in preventing irreversible neurological injury.