文章：

小儿脊柱畸形外科治疗新进展：基于42例1型神经纤维瘤病患者的单中心系列经验

Recent Developments in Surgical Treatment of Spinal Deformity in Pediatric Patients: Experience from a Single-Center Series of 42 Neurofibromatosis Type 1 Patients

原文发布日期：5 December 2024

DOI: 10.3390/cancers16234079

类型: Article

开放获取: 是

英文摘要：

Background: The management of spinal deformities in patients with NF-1 is challenging. The study aimed to assess the outcomes of the surgical treatment of spine deformities in children with neurofibromatosis type 1 with our treatment approach. Methods: A retrospective single-center study on pediatric patients with spinal deformities associated with NF-1 who received surgical treatment between 2006 and 2024. Results: The study group comprised 42 patients with a mean age at surgery of 9.8 years. Twenty-five patients (60%) were treated by means of growth-preserving techniques and 17 patients (40%) by means of definitive fusion. Preoperative halo-gravity traction was used in 14 (33%) cases. In the group treated with a growth-preserving technique, a 54.1% mean curve correction was observed at the latest follow-up, and growth of the thoracic spine was maintained at a physiological rate; however, 25 unplanned revision surgeries (mostly due to mechanical complications) were necessary. In the group treated by definitive fusion, a 66% curve correction was achieved at initial surgery, which remained unchanged at latest follow-up, and revision surgery was performed in three cases for augmentation of the fusion mass. There was one neurological complication (2%). Another patient developed a deep wound infection (2%). Conclusions: Good and sustainable surgical correction of spinal deformities can be achieved in pediatric patients with NF-1. Due to the bony dystrophic changes, surgical treatment is challenging and the complication rate is higher than in spinal deformities of other etiologies.

摘要翻译： 

背景：神经纤维瘤病1型（NF-1）患者脊柱畸形的治疗具有挑战性。本研究旨在评估采用本中心治疗方案对1型神经纤维瘤病儿童脊柱畸形进行手术治疗的效果。方法：对2006年至2024年间接受手术治疗的NF-1相关脊柱畸形患儿进行回顾性单中心研究。结果：研究组共纳入42例患者，平均手术年龄为9.8岁。其中25例（60%）采用保留生长技术治疗，17例（40%）采用终极融合术治疗。14例（33%）患者术前使用了头环重力牵引。在保留生长技术治疗组中，末次随访时观察到平均54.1%的侧弯矫正率，胸椎生长维持在生理速率；但需要进行25次非计划翻修手术（主要因机械并发症）。在终极融合术治疗组中，初次手术实现66%的侧弯矫正率，末次随访时矫正效果保持稳定，其中3例因需增强融合节段进行了翻修手术。出现1例神经并发症（2%），另1例发生深部伤口感染（2%）。结论：NF-1儿童患者可获得良好且持久的脊柱畸形手术矫正效果。但由于骨性营养不良性改变，手术治疗具有挑战性，并发症发生率高于其他病因的脊柱畸形。

原文链接：

Recent Developments in Surgical Treatment of Spinal Deformity in Pediatric Patients: Experience from a Single-Center Series of 42 Neurofibromatosis Type 1 Patients