Developments in Congenital Scoliosis and Related Research from 1992 to 2021: A Thirty-Year Bibliometric Analysis.

BACKGROUND: Many studies, mainly original articles and reviews, have been reported on congenital scoliosis (CS), but there is a lack of bibliometric analyses. This study aimed to systematically analyze the developments and focuses in CS and related research fields. METHODS: Data were retrieved from the Web of Science Core Collection database, and the top 100 most-cited studies were analyzed emphatically. The Web of Science Results Analysis and Citation Report was used to analyze different aspects of the literature. CiteSpace was used to analyze the cooperation network, reference co-citation, burst keywords, and burst citations. RESULTS: The final analysis included 749 studies. CS and related research has been rapidly expanding. Several journals have published relevant studies and most-cited studies on this topic. Cooperation was noted among authors, institutions, and countries/regions in multiple instances. Surgical techniques (hemivertebra resection/posterior vertebral column resection/vertical expandable prosthetic titanium rib/double approach/grade 4 osteotomy) was one of the most common research focuses. In addition, research on genetics and molecular biology related to CS has become an emerging trend as a result of advances in basic science. CONCLUSIONS: Over time, research on CS and in related fields has gained greater attention and has been expanding continuously, showing a trend toward globalization. We recommend that researchers focus on the progress of surgical techniques, advances in molecular biology and genetics, and characteristics of CS. The top clusters, most-cited articles, and references with the strongest burst citations should be studied further.

Analysis of risk factors and treatment outcome in patients presenting with neglected congenital spinal deformity and neurological deficit.

PURPOSE: Congenital spinal deformities (CSD) are uncommon; and usually present during early childhood. Rarely, patients have been reported to present with neglected CSD in association with myelopathy. The current study reports the largest series of patients with neglected CSD and major neuro-deficit; and discusses their long-term outcome. METHODS: We retrospectively analysed patients with CSD who presented with myelopathy and underwent surgical management between January 2008 and January 2018. Only patients who had complete clinico-radiological records and completed minimum 2-year follow-up were included. Neurology was graded according to American Spinal Injury Association Impairment, Nurick and modified Japanese Orthopaedic Association scores. Radiological details like deformity type, location, magnitude, and underlying anomaly were recorded. A comparative analysis of parameters between pre-operative and final follow-up periods was performed. RESULTS: Thirty three (age = 21.1 ± 11.9 years) patients with CSD and myelopathy were included. Mean duration since myelopathy was 5.2 ± 6.2 months. Thirty (91%) patients presented with kyphosis or kyphoscoliosis; and 18 had upper thoracic-level lesion (12 and 3 with mid- or low-thoracic and lumbar lesions). 16 (48.5%) and 12 (36.4%) presented with types 1 and 3 anomalies, respectively. Mean pre-operative mJOA and Nurick grades were 8.8 ± 2.4 and 3.4 ± 0.7. Based on ASIA score, 2, 5, 21 and five patients presented with grades A, C, D and E, respectively. All patients underwent surgeries from posterior-only approach. Mean follow-up was 4.7 ± 2.6 years. In kyphoscoliosis group, coronal and sagittal Cobb improved by 23.8° and 25.9°, respectively. Mean deformity improved by 19.6° and 15.6° in scoliosis and kyphosis groups, respectively. Neurological status improved in 5, remained stable in 23 and deteriorated in five patients. Mean mJOA and Nurick grades at final follow-up was 8.3 ± 3.1and 3.4 ± 0.9. CONCLUSION: Major neurological deficit is a rare complication of neglected CSD. In our series, such a presentation was observed in patients with kyphotic or kyphoscoliotic deformities, type 1 or 3 vertebral anomalies and proximal thoracic vertebral lesions. Surgery can be valuable in these patients, as it not only stabilises deformity, but also provides the best chance of preventing neuro-deterioration.

Melatonin protected against the detrimental effects of microRNA-363 in a rat model of vitamin A-associated congenital spinal deformities: Involvement of Notch signaling.

Congenital spinal deformities are a result of defective somitogenesis and are associated with vitamin A deficiency (VAD). However, the molecular mechanisms of VAD-associated congenital spinal deformities remain largely unknown. Increasing number of studies suggested that microRNAs and melatonin played important roles in the development of congenital spinal deformities. In this study, we showed that the whole-embryo expression of miR-363 was upregulated in VAD rats. Furthermore, we demonstrated that miR-363 inhibited the proliferation and neuronal differentiation of primary cultured NSCs, accompanied by downregulation of Notch1. To this end, melatonin suppressed miR-363 expression and rescued the effects of miR-363 on NSC proliferation and neuronal differentiation together with restoration of Notch signaling. The present study provided new insights into the mechanism of VAD-associated spinal deformities and the therapeutic effect of melatonin that may lead to novel understanding of the molecular mechanisms of congenital spinal deformities.

Evaluation of renal function in congenital spinal deformities patients with urinary malformations / 西安交通大学学报(医学版)

Objective To evaluate renal function in congenital spinal deformities patients with urinary malformations.Methods We prospectively enrolled patients diagnosed with congenital spinal deformities combined with urinary malformation as well as age- and weight-matched healthy individuals and divided them into three groups:renal malformations group,urinary tract malformations group,and healthy control group.Serum creatinine,blood urea,blood uric acid,serum cystatin C,and estimate glomerular filtration rate(eGFR)level were used to evaluate general renal function.Urinary microalbumin(mALB),urinary alpha-1-microglobulin(α1-MG), beta-2-microglobulin(β2-MG),and N-acetyl-beta-D-glucosaminidase(NAG)level were determined to evaluate early renal function.Results We enrolled totally 16 patients with renal malformations,14 patients with urinary tract malformations group,and 20 healthy individuals as controls.The concentration of serum creatinine,blood urea, blood uric acid,serum cystatin C,and the value of eGFR in the three groups were within normal reference values, with no significant difference(P＞0.05).There were significant differences in the urinary levels of mALB,α1-MG and NAG in the three groups(P＜0.05),but not for the concentration of β2-MG(P＞0.05).Urinary levels of mALB and NAG were significantly higher in renal malformations group than in urinary tract malformations group (P＜0.05),but not for the concentration of α1-MG(P＞ 0.05).Conclusion Early renal function impairment occurs in congenital spinal deformities children with urinary malformation.Moreover,it appears more severe in patients with renal malformations than in those with urinary tract malformations.