Mladenov Kiril V, Stücker Ralf
Pediatric Orthopedic Department, Altona Children's Hospital, Bleickenallee 38, D-22763 Hamburg, Germany.
Department of Orthopedics, University Medical Center Hamburg-Eppendorf, D-20246 Hamburg, Germany.
Cancers (Basel). 2024 Dec 5;16(23):4079. doi: 10.3390/cancers16234079.
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.
A retrospective single-center study on pediatric patients with spinal deformities associated with NF-1 who received surgical treatment between 2006 and 2024.
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%).
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.
1型神经纤维瘤病(NF-1)患者脊柱畸形的管理具有挑战性。本研究旨在评估采用我们的治疗方法对1型神经纤维瘤病儿童脊柱畸形进行手术治疗的效果。
对2006年至2024年间接受手术治疗的伴有NF-1的小儿脊柱畸形患者进行回顾性单中心研究。
研究组包括42例患者,手术时的平均年龄为9.8岁。25例(60%)患者采用保留生长技术治疗,17例(40%)患者采用确定性融合治疗。14例(33%)患者术前使用了头环重力牵引。在采用保留生长技术治疗的组中,最新随访时平均曲线矫正率为54.1%,胸椎生长维持在生理速率;然而,需要进行25次计划外翻修手术(主要由于机械并发症)。在采用确定性融合治疗的组中,初次手术时实现了66%的曲线矫正,在最新随访时保持不变,3例患者进行了翻修手术以增加融合块。有1例神经并发症(2%)。另1例患者发生了深部伤口感染(2%)。
1型神经纤维瘤病小儿患者可实现良好且可持续的脊柱畸形手术矫正。由于骨质营养不良性改变,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。