脊髓压迫,一种罕见的神经纤维瘤病并发症

H. Ouiminga, S. Zabsonré, A. Kelani, S. Ouattara, A. Dravé, R. Kabore, Désiré Harouna Sankara, M. Gaye
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摘要

目的:本研究的目的是报告1型神经纤维瘤病罕见的并发症脊髓压迫。观察:我们报告的情况下,45岁的男子,其中提出了综合征的胸脊髓压迫在痉挛性截瘫阶段。它的安装逐渐超过6个月与就职背部疼痛有关。他有1型神经纤维瘤病的临床病史,伴有“咖啡-黑糖”斑点。胸部、前臂、腿部皮肤下有大小不等的多发无痛结节。胸部见一大孤立结节,呈紫色。神经影像学显示椎管内的前棘旁肿物扩张,导致第二和第三胸椎水平的脊柱受压。它延伸到第三和第四胸椎的椎间孔,形成扇贝。在影像学上发现第二个大的不均匀左侧腹内肿块,包含囊肿区和钙化。在第二和第三胸椎椎板切除术脊柱减压后,疼痛减轻和运动恢复是逐渐的。行大结节切除术。组织学发现丛状神经纤维瘤。由于未征得患者同意,不能切除左侧腹内肿块。结论:脊髓受压是1型神经纤维瘤病的罕见并发症。然而,在任何脊髓症状或任何非典型的长期脊髓疼痛之前,必须考虑它。
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Spinal Cord Compression, a Rare Neurofibromatosis Complication
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 “Cafe-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.
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