Department of Orthopaedics, Beijing Children's Hospital, National Center for Children's Health, Capital Medical University, Beijing, PR China.
Faculty of Medicine, Dalhousie University, Halifax, NS, Canada.
J Pediatr Orthop. 2022 Mar 1;42(3):e242-e249. doi: 10.1097/BPO.0000000000002048.
The purpose of this study was to explore the surgical treatment of intraspinal rib head dislocation (IRH) in children with dystrophic scoliosis secondary to type 1 neurofibromatosis (NF1-DS).
From 2006 to 2019, 32 of 128 patients with NF1-DS were found to have IRH and enrolled in this study. There were 19 boys and 13 girls with an average age of 8.8±2.6 years. Patients were divided into 2 groups: group A (n=25) without IRH resection and group B (n=7) with IRH resection. The intraspinal rib proportion (IRP), apical vertebra rotation, apical vertebral translation, main thoracic curve Cobb angle, trunk shift and thoracic kyphosis, lumbar lordosis, and sagittal vertebral axis were measured before and after the operation. Spinal injury was graded based on the American Spinal Injury Association (ASIA) Impairment Scale.
The study group had a total of 42 IRH. The mean follow-up duration was 46.1±28.7 months. The preoperative IRP in both groups was similar (35.5±14.3% vs. 31.2±15.3%, P=0.522). The postoperative IRP was lower in group B (18.5±11.2% vs. 0%, P=0.002). The IRP in group A decreased from preoperative (31.2±15.3%) to postoperative (18.5±11.2%) (P<0.05). There was no significant difference in the apical vertebra rotation, apical vertebral translation, main thoracic curve Cobb angle, trunk shift, thoracic kyphosis, lumbar lordosis, and sagittal vertebral axis between the 2 groups before surgery and after surgery. Four patients with nerve injury caused by the IRH had full neurological recovery postoperatively. All patients were ASIA grade E at the last follow-up.
The surgical treatment of IRH in children with NF1-DS should be determined on the basis of the presence of preoperative neurological symptoms. This study supports the practice of correcting spinal deformities only in patients with mild or no spinal cord injury. If there are obvious neurological symptoms, IRH resection is necessary to relieve spinal cord compression to recover nerve function.
Level III.
本研究旨在探讨 1 型神经纤维瘤病（NF1）继发营养不良性脊柱侧凸（NF1-DS）患儿椎管内肋骨头脱位（IRH）的手术治疗方法。
2006 年至 2019 年，128 例 NF1-DS 患者中发现 32 例存在 IRH 并纳入本研究。其中男 19 例，女 13 例，平均年龄 8.8±2.6 岁。患者分为 2 组：A 组（n=25）未行 IRH 切除，B 组（n=7）行 IRH 切除。测量术前和术后椎管内肋骨比例（IRP）、顶椎旋转、顶椎平移、主胸弯 Cobb 角、躯干偏移、胸腰椎后凸、腰椎前凸和矢状位脊柱中轴线。根据美国脊柱损伤协会（ASIA）损伤分级标准对脊柱损伤进行分级。
研究组共发现 42 个 IRH。平均随访时间为 46.1±28.7 个月。两组术前 IRP 相似（35.5±14.3%比 31.2±15.3%，P=0.522）。B 组术后 IRP 较低（18.5±11.2%比 0%，P=0.002）。A 组的 IRP 从术前（31.2±15.3%）降至术后（18.5±11.2%）（P<0.05）。两组术前和术后顶椎旋转、顶椎平移、主胸弯 Cobb 角、躯干偏移、胸腰椎后凸、腰椎前凸和矢状位脊柱中轴线均无统计学差异。4 例因 IRH 导致神经损伤的患者术后神经功能完全恢复。末次随访时所有患者 ASIA 分级均为 E 级。
NF1-DS 患儿的 IRH 手术治疗应根据术前是否存在神经症状来决定。本研究支持仅对轻度或无脊髓损伤的患者进行脊柱畸形矫正的做法。如果存在明显的神经症状，需要切除 IRH 以减轻脊髓压迫，恢复神经功能。
III 级。
