Outcomes of Traditional Dual Growing Rods With Apical Control Techniques for the Treatment of Early-Onset Scoliosis: Comparison With Patients Treated With Traditional Dual Growing Rods Only With a Minimum 2-Year Follow-up After Graduation.

BACKGROUND AND OBJECTIVES: Correction with traditional dual growing rods (TDGR) might not be sufficient for severe and rigid spinal deformity. TDGR combines with apical control techniques (ACT) could theoretically improve curve correction and decrease the incidence of mechanical complications. However, long-term results for TDGR with ACT are limited. The aim of this study was to retrospectively review and compare the outcomes of patients who graduated from TDGR with or without ACT. METHODS: Patients who were treated by TDGR with or without ACT with a minimum 2-year follow-up after graduation were enrolled. According to the intervention for the apex, patients were further divided into the TDGR group, the TDGR + apical control pedicle screws group (without apical fusion), and the TDGR + hybrid technique group. Clinical outcomes, radiological parameters, pulmonary function, and complications were compared among the 3 groups. RESULTS: A total of 76 patients (51 patients in the TDGR group, 10 patients in the apical control pedicle screws group, and 15 patients in the hybrid technique group) were enrolled. Compared with TDGR, TDGR + ACT achieved better main curve correction, better control of apical vertebral translation and rotation, and lower incidence of complications and revision surgery (P < .05) while maintaining development of the spine and chest. Although the difference was not significant, patients in the TDGR + ACT group had better pulmonary function at the last follow-up (P > .05). The percentage of patients receiving final fusion in the TDGR + ACT group was significantly lower than that in the TDGR group (P < .05). CONCLUSION: Compared with TDGR, TDGR + ACT can achieve better curve correction and apical control and comparable clinical outcomes while maintaining the growth of the spine and chest. Patients may derive more benefits from treatment with TDGR + ACT, including a lower incidence of mechanical complications and revision surgery, better pulmonary function, and the avoidance of final fusion.

Safety of Traditional Growing Rods in Patients with Early-Onset Congenital Scoliosis Associated with Type-I Split Cord Malformation.

BACKGROUND: Literature regarding the application of traditional growing rod (TGR) instrumentation in patients with early-onset congenital scoliosis with type-I split cord malformation is scarce. The purpose of the present study was to assess the safety and effectiveness of TGR surgery and repeated lengthening procedures in patients with congenital scoliosis with type-I split cord malformation not treated with prophylactic osseous spur excision. METHODS: Thirteen patients with early-onset congenital scoliosis associated with type-I split cord malformation and a stable neurologic status between March 2009 and July 2020 were recruited. All patients underwent primary TGR surgery and subsequent repeated lengthening procedures without osseous spur excision by the same surgical team. Clinical information and radiographic data from the preoperative, postoperative, and latest follow-up periods were collected. RESULTS: The mean preoperative Cobb angle of the major coronal curve was 74.62° ± 25.59°, the mean early postoperative angle was 40.23° ± 17.89°, and the mean latest follow-up angle was 40.62° ± 16.60°. The scoliotic deformity correction percentage was 46.81% ± 12.26% after the initial operation and 45.08% ± 15.53% at the latest follow-up. Compared with the preoperative values, significant improvements were observed in the coronal and sagittal balance early postoperatively and at the latest follow-up (p < 0.05 for all). The average annual amounts of spinal height gained were 15.73 ± 5.95 mm at T1-S1, 8.94 ± 3.94 mm at T1-T12, and 12.02 ± 6.70 mm between the instrumented segments. The total height gained at T1-S1 and T1-T12 was 72.18 ± 28.74 mm and 37.62 ± 12.53 mm, respectively. No intraoperative neurophysiological monitoring events were observed, and no case of neurological deficit was observed postoperatively or during follow-up. CONCLUSIONS: Patients without neurologic deficit and having a stable neurologic exam who have early-onset congenital scoliosis associated with type-I split cord malformation can safely and effectively undergo TGR surgery, followed by repeated lengthening procedures, without prophylactic osseous spur excision. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

A Novel Classification of Juvenile and Adolescent Idiopathic Scoliosis for Conservative Treatment.

BACKGROUND: The operative classification of scoliosis is well-developed but inadequate for guiding conservative treatment. The current conservative classification for juvenile and adolescent idiopathic scoliosis (JAIS) exhibits noticeable deficiencies. This study aimed to establish the Peking Union Medical College Hospital (PUMCH) classification and assess its clinical value in the conservative treatment of JAIS. METHODS: This study consisted of 2 parts. First, it involved a retrospective analysis of patients treated for JAIS in the Department of Rehabilitation Medicine, the ∗∗∗ Union Medical College Hospital, between January 2013 and June 2020. Second, it involved an ambispective cohort study that enrolled patients with JAIS in the above hospital between July and December 2020. RESULTS: A total of 989 patients with JAIS were enrolled, with 899 patients for establishing the PUMCH classification and 90 patients with JAIS for validating the PUMCH classification. The classification demonstrated an average reliability of 88.22% with a kappa coefficient of 0.862. After 1 week, the remeasured results presented a mean reproducibility of 92.78% and a kappa coefficient of 0.908. After 1-year follow-up, the Cobb angle decreased significantly from 16.61 ± 2.88° to 12.16°± 9.97° (P = 0.002) in 51 patients with PUMCH-scoliosis-specific exercise (SSE) treatment, while the Cobb angle increased significantly from 15.74 ± 2.75° to 17.64 ± 5.60° (P = 0.014) in 39 patients without PUMCH-SSE treatment. CONCLUSIONS: The PUMCH-SSE classification demonstrates good inter-observer reliability and intra-observer reproducibility. In addition, the classification may be used to guide the conservative treatment of JAIS in clinical settings.

Profound hypoxemia and hypotension during posterior spinal fusion in a spinal muscular atrophy child with severe scoliosis: a case report.

BACKGROUND: Anesthesia for spinal muscular atrophy (SMA) patients undergoing spinal deformity surgery is challenging. We report an unusual case of an SMA girl who developed severe intraoperative hypoxemia and hypotension during posterior spinal fusion related with surgical positioning. CASE PRESENTATION: A 13-yr-old girl diagnosed with SMA type 2, severe kyphoscoliosis and thoracic deformity was scheduled for elective posterior spinal fusion. She developed severe hypoxemia and profound hypotension intraoperatively in the prone position with surgical table tilted 45° to the right. Though transesophageal echocardiography (TEE) could not be performed due to limited mouth opening, her preoperative computed tomography revealed a severely distorted thoracic cavity with much reduced volume of the right side. A reasonable explanation was when the surgeons performed surgical procedure with the tilted surgical table, the pressure was directly put on the shortest diameter of the significantly deformed thoracic cavity, causing severe compression of the pulmonary artery, resulting in both hypoxemia and hypotension. The patient stabilized when the surgical table was tilted back and successfully went through the surgery in the leveled prone position. CONCLUSIONS: Spinal fusion surgery is beneficial for SMA patients in preventing scoliosis progression and improving ventilation. However, severe scoliosis and thoracic deformities put them at risk of both hemodynamic and respiratory instability during surgical positioning. When advanced monitoring like TEE is not practical intraoperatively, preoperative imaging may help with differential diagnosis, and guide the surgical positioning to minimize mechanical compression of the thoracic cavity, thereby helping the patient complete the surgery safely.

Comparative analysis of paraspinal muscle imbalance between idiopathic scoliosis and congenital scoliosis from the transcriptome aspect.

Background: Previous studies have analyzed paraspinal muscle imbalance in idiopathic scoliosis (IS) with methods including imaging, histology and electromyography. However, whether paraspinal muscle imbalance is the cause or the consequence of spinal deformities in IS remains unclear. Comparison of paraspinal muscle imbalance between IS and congenital scoliosis (CS) may shed some light on the causality of paraspinal muscle imbalance and IS. This study aimed to elucidate the generality and individuality of paraspinal muscle imbalance between IS and CS from gene expression. Methods: Five pairs of surgical-treated IS and CS patients were matched. Bilateral paraspinal muscles at the apex were collected for transcriptome sequencing. Differentially expressed genes (DEGs) between the convexity and concavity in both IS and CS were identified. Comparison of DEGs between IS and CS was conducted to discriminate IS-specific DEGs from DEGs shared by both IS and CS. Bioinformatics analysis was performed. The top 10 hub genes in the protein-protein interaction (PPI) network of IS-specific DEGs were validated by quantitative PCR (qPCR) in 10 pairs of IS and CS patients. Results: A total of 370 DEGs were identified in IS, whereas 380 DEGs were identified in CS. Comparison of DEGs between IS and CS identified 59 DEGs shared by IS and CS, along with 311 DEGs specific for IS. These IS-specific DEGs were enriched in response to external stimulus and signaling receptor binding in GO terms and calcium signaling pathway in KEGG pathways. The top 10 hub genes in the PPI network of IS-specific DEGs include BDKRB1, PRH1-TAS2R14, CNR2, NPY4R, HTR1E, CXCL3, ICAM1, ALB, ADIPOQ, and GCGR. Among these hub genes, the asymmetrical expression of PRH1-TAS2R14 and ADIPOQ in IS but not CS were validated by qPCR. Conclusions: Transcriptomic differences in bilateral paraspinal muscles between the convexity and concavity in IS share few similarities with those in CS.

Morphological analysis of isolated hemivertebra: radiographic manifestations related to the severity of congenital scoliosis.

PURPOSE: The natural history of congenital scoliosis (CS) caused by hemivertebra varies greatly. This study aimed to explore the association between the morphology of hemivertebra and the severity of CS, since the diagnosis of the hemivertebra. METHODS: Patients with isolated (single fully segmented) hemivertebra were enrolled. The degree and progression of deformity were compared by three morphological parameters of hemivertebra, comprising whether the width of hemivertebra extends across the central vertical line of lower adjacent vertebra (midline); the lateral height ratio (LHR, lateral height of hemivertebra× 2/(lateral height of HV-1 plus HV + 1) with the cut-point being 0.9; and the sagittal position of hemivertebra that was divided into the lateral and posterolateral group. RESULTS: In total, 156 patients (mean age 9.7 ± 6.2 years, 81 males) were enrolled. The number of thoracic, thoracolumbar (T12/13-L1), and lumbar hemivertebrae were 63, 41, and 52, respectively. Hemivertebrae across the midline had larger scoliosis and kyphosis (58.3 ± 20.6° vs. 42.8 ± 15.0°, P <  0.001; 45.1 ± 32.5° vs. 29.5 ± 25.7°, P = 0.013, respectively). Hemivertebrae with LHR ≥0.9 was associated with larger scoliosis (55.7 ± 20.6° vs. 41.4 ± 13.3°, P <  0.001). Larger scoliosis and kyphosis were observed in posterolateral hemivertebrae (54.4 ± 21.0° vs. 44.4 ± 15.6°, P = 0.026; 51.4 ± 31.5° vs. 20.6 ± 17.1°, P <  0.001, respectively). Co-occurrence of more than one of the three positive parameters above indicated higher annual progression (5.0 ± 2.2° vs. 3.3 ± 1.3°, P <  0.001). CONCLUSION: Three positive parameters, width across the midline, LHR ≥0.9, and posterolateral position were associated with a more severe deformity in patients with isolated hemivertebra. Hemivertebrae with more than one positive parameter may cause progressive deformity, and thus need prompt surgery. LEVEL OF EVIDENCE: Prognostic, level IV.

Effects of ferric derisomaltose on postoperative anaemia in adult spinal deformity surgery: a study protocol for a randomised controlled trial.

INTRODUCTION: Postoperative anaemia is prevalent in adult spinal deformity (ASD) surgery in association with unfavourable outcomes. Ferric derisomaltose, a novel iron supplement, offers a promising solution in rapidly treating postoperative anaemia. However, the clinical evidence of its effect on patients receiving spinal surgery remains inadequate. This randomised controlled trial aims to evaluate the safety and efficacy of ferric derisomaltose on postoperative anaemia in ASD patients. METHODS AND ANALYSIS: This single-centre, phase 4, randomised controlled trial will be conducted at Department of Orthopaedics at Peking Union Medical College Hospital and aims to recruit adult patients who received ASD surgery with postoperative anaemia. Eligible participants will be randomly assigned to receive ferric derisomaltose infusion or oral ferrous succinate. The primary outcome is the change in haemoglobin concentrations from postoperative days 1-14. Secondary outcomes include changes in iron parameters, reticulocyte parameters, postoperative complications, allogeneic red blood cell infusion rates, length of hospital stay, functional assessment and quality-of-life evaluation. ETHICS AND DISSEMINATION: This study has been approved by the Research Ethics Committee of Peking Union Medical College Hospital and registered at ClinicalTrials.gov. Informed consent will be obtained from all participants prior to enrolment and the study will be conducted in accordance with the principles of the Declaration of Helsinki. The results of this study are expected to be disseminated through peer-reviewed journals and academic conferences. TRIAL REGISTRATION NUMBER: NCT05714007.

Dual Growing Rods and the Apical Control Technique for Treating Congenital Early-Onset Scoliosis: Lessons Learned.

BACKGROUND: Congenital early-onset scoliosis (CEOS) is characterized by a spectrum of vertebral anomalies, including formation failures and segmentation failures at the apex segment, which makes CEOS different from other etiologies of early-onset scoliosis. To date, studies on patients who have graduated from CEOS treatment using traditional dual growing rods (TDGR) have been scarce, and the preliminary results of TDGR with or without the apical control technique (ACT) have varied. We therefore compared the final outcomes of patients with CEOS who graduated from TDGR with or without the ACT. METHODS: A retrospective study of patients with CEOS who had graduated from TDGR treatment performed from 2007 to 2020 was conducted. Graduation included final fusion or observation after reaching skeletal maturity. Patients were divided into the ACT-TDGR group (apical vertebrectomy and/or hemivertebrectomy with short fusion and TDGR) and the TDGR-only group. Demographic characteristics, radiographic data, patient-reported clinical outcomes, pulmonary function, and complications were analyzed. RESULTS: A total of 41 patients with CEOS were enrolled: 13 in the ACT-TDGR group and 28 in the TDGR-only group. The lengthening intervals were longer in the ACT-TDGR group (mean [and standard deviation], 1.26 ± 0.66 years) than in the TDGR-only group (0.80 ± 0.27 years). The preoperative main curve was larger in the ACT-TDGR group (80.53° ± 19.50°) than in the TDGR-only group (64.11° ± 17.50°). The residual curve was comparable between groups (26.31° ± 12.82° in the ACT-TDGR group compared with 27.76° ± 15.0° in the TDGR group) at the latest follow-up. The changes in apical vertebral rotation and thoracic rotation were significantly larger in the ACT-TDGR group. Patients had comparable T1-12 and T1-S1 heights, pulmonary function, and 22-item Scoliosis Research Society (SRS-22) scores at the latest follow-up. The mean number of mechanical-related complications per patient was lower in the ACT-TDGR group (0.77 ± 0.73) than in the TDGR-only group (1.54 ± 1.43). Seventeen patients underwent final fusion. CONCLUSIONS: In this small-scale study, we observed that both ACT-TDGR and TDGR-only could correct the deformity while allowing for spinal growth in patients with CEOS. ACT-TDGR yielded better correction in severe cases and did not have a deleterious effect on spinal height. A large number of cases will be needed to validate the clinical value of the ACT. LEVEL OF EVIDENCE: Therapeutic Level III . See Instructions for Authors for a complete description of levels of evidence.

Surgical treatment of atlantoaxial dysplasia and scoliosis in spondyloepiphyseal dysplasia congenita: A case report.

BACKGROUND: Spondyloepiphyseal dysplasia congenita (SEDC) is a rare autosomal dominant hereditary disease caused by COL2A1 mutations. SEDC primarily involves the skeletal system, with typical clinical manifestations, including short stature, hip dysplasia, and spinal deformity. Due to the low incidence of SEDC, there are only a few case reports regarding the surgical treatment of SEDC complicated with spinal deformities. CASE SUMMARY: We report a case of a 16-year-old male patient with SEDC. He presented with typical short stature, atlantoaxial dysplasia, scoliosis, and hip dysplasia. Cervical magnetic resonance imaging showed spinal canal stenosis at the atlas level and cervical spinal cord compression with myelopathy. The scoliosis was a right thoracic curve with a Cobb angle of 65°. He underwent atlantoaxial reduction, decompression, and internal fixation from C1-C2 to relieve cervical myelopathy. Three months after cervical surgery, posterior correction surgery for scoliosis was performed from T3 to L4. Scoliosis was corrected from 66° to 8° and remained stable at 2-year follow-up. CONCLUSION: This is the first case report of a patient with SEDC who successfully underwent surgery for atlantoaxial dysplasia and scoliosis. The study provides an important reference for the surgical treatment of SEDC complicated with spinal deformities.

Unilateral interlaminar fenestration on the convex side provides a reliable access for intrathecal administration of nusinersen in spinal muscular atrophy: a retrospective study.

BACKGROUND: As the first gene therapy for spinal muscular atrophy (SMA), nusinersen is supposed to be administrated via intrathecal injection regularly for a lifetime. However, for SMA patients with severe spinal deformities, bony fusion following posterior spinal instrumentation sets great obstacles for the application of nusinersen. Therefore, efforts have been devoted to the exploration of appropriate approach for nusinersen administration. This study aims to evaluate the safety and reliability of unilateral interlaminar fenestration on the convex side during spinal fusion surgery for intrathecal nusinersen injection in SMA. RESULTS: SMA patients receiving posterior spinal fusion and interlaminar fenestration in Peking Union Medical College Hospital from January 2020 to October 2021 were retrospectively analyzed. 13 patients were included. Of the 13 patients, 10 were classified into SMA type II and 3 into SMA type III. Distal fusion to pelvis was undertaken in 11 patients; while L5 was selected as the lowest instrumented vertebra in the other 2 patients. All patients received interlaminar fenestration on the convex side only with an area of about 15 mm × 20 mm. Fenestration at L2-L3 level was performed in 6 patients; while L3-L4 level was selected for windowing in the remaining 7 patients. 9 of the 13 patients received lumbar puncture and intrathecal nusinersen administration during the 1-year follow-up, with an accumulative total of 50 times. All injections were performed successfully under ultrasound guidance, with no one transferred to radiographic assistance. No severe complications occurred after injection. CONCLUSIONS: In SMA with severe scoliosis planning to receive posterior spinal fusion, unilateral lumbar interlaminar fenestration on the convex side provides a feasible and reliable access for intrathecal nusinersen administration after surgery.