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首页> 外文期刊>Neurosurgery >Surgical management of spinal cord compression from plexiform neurofibromas in patients with neurofibromatosis 1.
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Surgical management of spinal cord compression from plexiform neurofibromas in patients with neurofibromatosis 1.

机译：神经纤维瘤病患者从丛状神经纤维瘤压迫脊髓的手术管理1。

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OBJECTIVE: Plexiform neurofibromas with sizable intraspinal extensions and resultant spinal cord compromise pose challenging management problems, because these lesions may involve multiple nerves and engulf adjacent vascular and visceral structures. In this report, we review our experience with the surgical treatment of these lesions. METHODS: Patients were identified by a detailed review of hospital medical records and the database of our multidisciplinary neurofibromatosis clinic. Ten patients had large plexiform neurofibromas that extended intraspinally, producing a combination of myelopathy and radiculopathy. Two patients exhibited single-level intraspinal growth, and eight showed multilevel involvement. Four patients showed bilateral plexiform neurofibromatous growth intraspinally, with "hourglass" compression of the spinal cord. Operative approaches and outcomes were reviewed in detail. RESULTS: Gross total resection of the symptomatic intraspinal tumor component was achieved for nine patients. The management of the extraspinal component was individualized, depending on the pattern and extent of involvement of the surrounding structures. Nine patients experienced complete recovery of neurological function postoperatively; the remaining patient demonstrated significant functional improvement. With a median follow-up period of 4 years, only one patient has developed recurrent intraspinal compression, in this case from tumor involvement by the same plexiform lesion at a lower spinal level. Two patients treated early in the series using standard laminectomy approaches developed significant kyphotic deformities, necessitating subsequent fusion. Based on these initial results, osteoplastic laminotomy techniques were used in the last five cases, allowing anatomic reconstruction of the involved levels; none of these latter patients has developed significant kyphosis, with a median follow-up period of 3 years. CONCLUSION: Radical resection of intraspinal tumor components in patients with neurofibromatosis 1 and large plexiform neurofibromas can help to preserve excellent neurological function. Technical factors in the management of these lesions are presented.
机译：目的：椎体神经纤维瘤伴有较大的椎管内延伸并导致脊髓受损，提出了具有挑战性的管理问题，因为这些病变可能涉及多条神经并吞噬相邻的血管和内脏结构。在本报告中，我们回顾了这些病变的外科治疗经验。方法：通过详细审查医院病历和我们多学科神经纤维瘤病诊所的数据库来识别患者。十名患者有大型丛状神经纤维瘤，其在脊髓内延伸，产生脊髓病和神经根病的组合。 2名患者表现出单水平椎管内生长，8名表现出多水平受累。四名患者显示脊髓内双侧丛状神经纤维瘤生长，脊髓“沙漏”压迫。手术方法和结果进行了详细的审查。结果：9例患者的症状性椎管内肿瘤成分全部切除。脊柱外组件的管理是个性化的，具体取决于周围结构的参与方式和程度。 9例患者术后神经功能完全恢复;其余患者表现出明显的功能改善。中位随访期为4年，只有一名患者发生了反复的椎管内压迫，在这种情况下，是由于较低脊髓水平的同一丛状病变累及肿瘤。在该系列的早期使用标准椎板切除术治疗的两名患者出现了严重的后凸畸形，因此需要进行后续融合。根据这些初步结果，在最近五例病例中采用了整骨技术的开胸手术，从而可以对所涉及的水平进行解剖重建。后面这些患者均未出现明显的后凸畸形，中位随访期为3年。结论：神经纤维瘤病1和大型丛状神经纤维瘤患者根治性切除椎管内肿瘤成分可有助于保持良好的神经功能。介绍了处理这些病变的技术因素。

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