Spinal Cord Compression, a Rare Neurofibromatosis Complication

Abstract

Objective: The objective of this study is to report a case of spinal cord compression, which is a rare complication of neurofibromatosis type 1. Observation: We report the case of a 45-year-old man, which presented a syndrome of thoracic spinal cord compression at the stage of spastic paraparesis. Its installation was gradually over 6 months associated with the inaugural back pain. He had a clinical history of neurofibromatosis type 1 with “Café-au-lait” spots. There were multiple painless nodules under the skin of different size on the chest, forearms and legs. A large isolated nodule, purplish was observed on the chest. The neuro-imaging showed a para-spinal anterior mass expansion inside the spinal canal causing spinal compression at the level of the second and third thoracic vertebra. It extends into the intervertebral foramen of the third and fourth thoracic vertebra leading to a scalloping. A second large heterogeneous left intra-abdominal mass containing cyst areas and calcifications was discovered in imaging. After a spinal decompression with laminectomy of the second and third thoracic vertebra, the reduction of pain and motor recovery was gradual. The large nodule excision was performed. Histology found a plexiform neurofibroma. Excision of the left intra-abdominal mass could not be performed because the patient’s consent had not been obtained. Conclusion: The spinal cord compression is a rare complication of neurofibromatosis type 1. However, it is essential to think about it in front of any spinal cord symptoms or any atypical long term spinal pain.