Myelopathy Associated with Rapid Progression of a Dystrophic Neurofibromatosis-1 Curve - A Case Report and Review of Literature.

Introduction: Thoracic myelopathy in neuro fibromatosis-1 (NF-1) is most commonly due to intra-spinal neurofibromas/dumb-bell tumors/intra-canal rib head penetration (RHP) causing cord compression. However, acute thoracic myelopathy due to rapid progression of the kyphoscoliotic curve alone in NF-1 without a significant spinal cord compression occurs very rarely. This case report discusses our experience with one such patient and we also discuss intraoperative and post-operative challenges encountered with this patient and a rare complication of hemothorax postoperatively.

Case Report: A 15-year-old male presented to the clinic after being lost to follow-up for 4 years with a rapid acute deterioration of dystrophic curve and no myelopathic symptoms (Scoliosis - 65°, Kyphosis - 77°). His subsequent examination in 6 weeks showed acute development of myelopathic gait with right ankle and extensor hallucis longus weakness. He was admitted for halo gravity traction for 6 weeks and a single-stage posterior instrumentation with excision of rib heads at the apex was planned. Postoperatively, the patient developed massive left hemothorax and loss of power in both lower limbs at day 2. He subsequently regained full power and complete resolution of myelopathic symptoms at the end of 9- month follow-up with a satisfactory alignment of spine in the follow-up X-rays.

Conclusion: Acute onset of myelopathy is a rare and uncommon finding with a rapid deterioration of dystrophic curve alone without any major spinal cord compromise. Early detection of dysplastic changes with early aggressive surgical management and deformity correction is necessary with dystrophic NF-1 curves to prevent pre-operative and post-operative morbidities.