Surgical Treatment of the Intraspinal Rib Head Dislocation in Children With Dystrophic Scoliosis Secondary to Type 1 Neurofibromatosis

Abstract

Background: 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). Methods: 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. Results: 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. Conclusions: 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 of Evidence: Level III.

Outcomes and Safety of Traditional Growing Rod Technique in the Treatment of Early-onset Dystrophic Scoliosis Secondary to Type 1 Neurofibromatosis With Intraspinal Rib Head Dislocation in Children

“…[6][7][8][9] Previously, we had reported the literature of the surgical treatment of the IRH in children with NF1-DS. 10 In that literature, we focused on the treatment outcomes of patient with NF1-DS with and without IRH resection, but this study focused on patients with NF1-EOS treated with TGR to investigate the outcomes and surgical safety.…”

“…Coronal plane parameters10: The major coronal curve. Trunk shift: the horizontal distance between the C7 plumb line and center sacral vertical line.…”

“…The remaining 14 patients underwent PSF because they were over 10 years old. Eighteen of the patients in this report were used in our previous report 10. Patients comprised 13 boys and 8 girls, with an average age of 7.1±1.5 years.…”

“…Eighteen of the patients in this report were used in our previous report. 10 Patients comprised 13 boys and 8 girls, with an average age of 7.1 ± 1.5 years. Patient characteristics are described in Table 1.…”

“…Several reports regarding this rare clinical manifestation suggest that for patients without neurological symptoms, it is safe to retain the IRH and directly correct the spinal deformity, which is not an absolute contraindication for surgery. [6][7][8][9] Gao et al 10 reported on our experiences and thought that practice of correcting spinal deformities should only be practiced in patients with mild or no spinal cord injury. However, most cases reported to date have involved adolescents or adults who were treated with posterior spinal fusion (PSF).…”