Estefan Martin M, Camino-Willhuber Gaston, Bosio Santiago T, Puigdevall Miguel, Maenza Ruben A
Instituto de Ortopedia y Traumatología "Carlos E. Ottolenghi", Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.
The Centre for Spinal Studies and Surgery (CSSS)- Queen's Medical Centre, Nottingham University Hospitals-NHS, Nottingham, United Kingdom.
Spine Deform. 2022 Mar;10(2):285-294. doi: 10.1007/s43390-021-00422-3. Epub 2021 Oct 27.
The management of scoliosis and kyphoscoliosis in patients with Type 1 Neurofibromatosis (NF-1) among spinal surgeons is still challenging due to the severity of the deformity especially in dystrophic deformity types. This rapid and progressive condition is likely to be associated with dislocated rib heads into the spinal canal, hence representing a real dilemma on the decision making between its resection versus not resection during the corrective surgery, especially in patients with normal neurological status. The objective of this publication is to discuss the management options in this patient population through a literature review.
A comprehensive systematic literature search was performed for relevant studies using PubMed, Web of Science, and Scopus databases. Previous publications depicting neurologically intact patients with NF-1 and rib dislocation into the canal were reviewed. Articles reporting individual cases or case series/cohorts with patient-discriminated findings were included.
The data collection retrieved a total of 55 neurologically intact patients with NF-1 dystrophic scoliosis and rib penetration into the canal who underwent spinal surgery. Among them, 37 patients underwent surgery without head rib resection and 18 patients with rib excision. No patient presented postoperative neurological deficit except for one case of late postoperative neurological deterioration reported in a patient within situ fusion in which the surgeons ignored the presence of previous spinal cord compression.
Corrective surgery for patients with NF-1 and rib penetration into the canal in neurologically intact patients can be safely performed without the resection of the dislocated rib heads without a higher risk of neurological compromise.
1型神经纤维瘤病（NF-1）患者脊柱侧凸和脊柱后凸侧凸的管理，对于脊柱外科医生来说仍然具有挑战性，因为畸形严重，尤其是在营养不良性畸形类型中。这种快速进展的情况可能与肋骨头部脱位进入椎管有关，因此在矫正手术中，对于是否切除脱位肋骨头部的决策构成了真正的困境，尤其是对于神经状态正常的患者。本出版物的目的是通过文献综述来讨论该患者群体的管理选择。
使用PubMed、科学网和Scopus数据库对相关研究进行了全面的系统文献检索。回顾了先前描述神经功能正常的NF-1患者且肋骨脱位进入椎管的出版物。纳入报告了具有患者特异性发现的个案或病例系列/队列的文章。
数据收集共检索到55例神经功能正常的NF-1营养不良性脊柱侧凸且肋骨穿入椎管并接受脊柱手术的患者。其中，37例患者接受了无头肋骨切除的手术，18例患者进行了肋骨切除。除了1例原位融合患者术后出现迟发性神经功能恶化（外科医生忽略了先前存在的脊髓压迫）外，没有患者出现术后神经功能缺损。
对于神经功能正常的NF-1患者且肋骨穿入椎管的情况，在不切除脱位肋骨头部的情况下可以安全地进行矫正手术，且不会有更高的神经功能受损风险。
