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Most cases of congenital spinal deformities were sporadic and without strong evidence of heritability. The etiology of congenital spinal deformities is still elusive and assumed to be multi-factorial. The current study seeks to elucidate the effect of maternal vitamin A deficiency and the production of congenital spinal deformities in the offsping. Thirty two female rats were randomized into two groups: control group, which was fed a normal diet; vitamin A deficient group, which were given vitamin A-deficient diet from at least 2 weeks before mating till delivery. Three random neonatal rats from each group were killed the next day of parturition. Female rats were fed an AIN-93G diet sufficient in vitamin A to feed the rest of neonates for two weeks until euthanasia. Serum levels of vitamin A were assessed in the adult and filial rats. Anteroposterior (AP) spine radiographs were obtained at week 2 after delivery to evaluate the presence of the skeletal abnormalities especially of spinal deformities. Liver and vertebral body expression of retinaldehyde dehydrogenase (RALDHs) and RARs mRNA was assessed by reverse transcription-real time PCR. VAD neonates displayed many skeletal malformations in the cervical, thoracic, the pelvic and sacral and limbs regions. The incidence of congenital scoliosis was 13.79% (8/58) in the filial rats of vitamin A deficiency group and 0% in the control group. Furthermore, vitamin A deficiency negatively regulate the liver and verterbral body mRNA levels of RALDH1, RALDH2, RALDH3, RAR-α, RAR-β and RAR-γ. Vitamin A deficiency in pregnancy may induce congenital spinal deformities in the postnatal rats. The decreases of RALDHs and RARs mRNA expression induced by vitamin A deprivation suggest that vertebral birth defects may be caused by a defect in RA signaling pathway during somitogenesis. [ABSTRACT FROM AUTHOR]

Background: Vertebrae, ribs, and spinal cord are anatomically adjacent structures, and their close relationships are clinically important for planning better corrective surgical approach. The objective is to identify the radiographic characteristics in surgical patients with congenital scoliosis (CS) and coexisting split cord malformation (SCM).Methods: A total of 266 patients with CS and SCM underwent surgical treatment at our hospital between May 2000 and December 2015 was retrospectively identified. The demographic distribution and radiographic data were collected to investigate the characteristics of spine curve, vertebral, rib, and intraspinal anomalies. According to Pang's classification, all patients were divided into two groups: type I group is defined as two hemicords, each within a separate dural tube separated by a bony or cartilaginous medial spur, while type II group is defined as two hemicords within a single dural tube separated by a nonrigid fibrous septum.Results: There were 104 patients (39.1%) in Type I group and 162 patients (60.9%) in Type II group. SCM was most commonly found in the lower thoracic and lumbar regions. The mean length of the septum in Type I SCM was significantly shorter than Type II SCM (2.7 vs. 5.2 segments). Patients in Type I group had a higher proportion of kyphotic deformity (22.1%). The vertebral deformities were simple in only 16.5% and multiple in 83.5% of 266 cases. Patients in Type I group presented higher prevalence of multiple (90.4%) and extensive (5.1 segments) malformation of vertebrae. In addition, hypertrophic lamina and bulbous spinous processes were more frequent in Type I group (29.7%), even developing into the "volcano-shape" deformities. Rib anomalies occurred in 62.8% of all patients and 46.1% of them were complex anomalies. The overall prevalence of other intraspinal anomalies was 42.9%. The most common coexisting intraspinal anomalies was syringomyelia (30.5%).Conclusion: The current study, with the largest cohort to date, demonstrated that patients with CS and coexisting SCM presented high prevalence of multiple vertebral deformities, rib and intraspinal anomalies. The length of the split segment in Type I SCM was shorter than that in Type II SCM. Compared with Type II SCM, patients with Type I SCM presented with higher incidence of kyphotic deformity, more extensive and complicated vertebral anomalies, and more complex rib anomalies. [ABSTRACT FROM AUTHOR]

Background: The X-ray films of the patients with Marfan syndrome scoliosis (MSS) look like those with adolescent idiopathic scoliosis (AIS). In literature, there are many reports on the correction results of AIS, while there are a few studies focused on the difference of the correction results between MSS and AIS. This study aims to analyze whether there are differences of posterior correction surgery in MSS and AIS. Methods: All the patients included underwent posterior correction surgery. The radiographic data, operation duration, estimated blood loss, transfusion, fusion levels, and correction rate were retrospectively reviewed and analyzed between the two groups. Results: Group MSS included 42 patients, 11 male and 31 female, with an average age of 15.2 years old. Group AIS included 168 patients (ratio, 1:4), 34 male and 134 female, with an average age of 14.5 years old. Twenty-three patients in group MSS and 94 patients in group AIS were followed up regularly, with an average time of 18.4 and 18.5 months, respectively. The mean coronal Cobb angle of the major curve before operation and at final follow-up, the correction rate, fusion level, operation duration, estimated blood loss during operation, and transfusion between the two groups were 60.4 and 57.5°, 14.6 and 15.2°, 76.4 and 74.1 %, 11.5 and 11.0 vertebrae, 4.6 and 4.0 h, 845 and 698 ml, and 1151 and 894 ml, respectively. The age, gender ratio, curve type, and coronal Cobb angle of the major curve were all matched (all P > 0.05). Group MSS had a longer operation duration and more estimated blood loss compared with those of group AIS (both P < 0.05), while there was no significant difference in terms of fusion level, transfusion, coronal Cobb angle of the major curve at final follow-up, and the correction rate (all P > 0.05). Conclusions: When performing posterior correction for scoliosis, the surgeons should be aware that the patients with Marfan syndrome scoliosis had more estimated blood loss and longer operation duration than AIS patients, while the correction rate was similar. [ABSTRACT FROM AUTHOR]

Background: The patients with extremely severe spinal deformity are commonly considered high-risk candidates for surgical treatment because of their underlying lung disease. Currently, little has been reported about the postoperative pulmonary complication events in this population. This retrospective study sought to evaluate preoperative pulmonary function tests in the surgical treatment of extremely severe scoliosis. Methods: Preoperative forced vital capacity (FVC), FVC ratio, forced expiratory volume at the end of the first second (FEV1), FEV1 ratio, peak expiratory flow (PEF), and PEF ratio were performed and evaluated on 60 patients with extremely severe scoliosis (coronary main Cobb angle ≥100°). Results: Among the 60 patients, 11 (18.3%), 13 (21.7%), and 22 (36.7%) had severe, moderate, and mild pulmonary dysfunction, respectively. Compared with the moderate and mild scoliosis groups, significant differences were observed in Cobb, FVC, FVC ratio, FEV1, FEV1 ratio, and PEF ratio in the extremely severe scoliosis group. Various postoperative pulmonary complications occurred in nine cases (15%). Patients with severe or moderate dysfunction as measured by the FVC ratio had a higher incidence of postoperative pulmonary complications. A transthoracic procedure was not related to postoperative pulmonary complications, but thoracoplasty significantly increased the incidence of postoperative pulmonary complications (P < 0.001, OR = 20, 95% CI = 3.45–115.97). Discussion: Pulmonary function was impaired in extremely severe scoliosis. Patients with severe restrictive pulmonary dysfunction had a higher incidence of postoperative pulmonary complications. Thoracoplasty was an important risk factor in the prediction of postoperative pulmonary complications. [ABSTRACT FROM AUTHOR]

Background: In idiopathic scoliosis patients, after anterior spinal fusion and instrumentation, the discs (interspace angle) between the lowest instrumented vertebra (LIV) and the next caudal vertebra became more wedged. We reviewed these patients and analyzed the changes of the angle. Methods: By reviewing the medical records and roentgenograms of adolescent idiopathic scoliosis patients underwent anterior spinal fusion and instrumentation, Cobb angle of the curve, correction rate, coronal balance, LIV rotation, interspace angle were measured and analyzed. Results: There were total 30 patients included. The mean coronal Cobb angle of the main curve (thoracolumbar/lumbar curve) before and after surgery were 48.9° and 11.7°, respectively, with an average correction rate of 76.1%. The average rotation of LIV before surgery was 2.1 degree, and was improved to 1.2 degree after surgery. The interspace angle before surgery, on convex side-bending films, after surgery, at final follow up were 3.2°, -2.3°, 1.8° and 4.9°, respectively. The difference between the interspace angle after surgery and that preoperatively was not significant (P = 0.261), while the interspace angle at final follow-up became larger than that after surgery, and the difference was significant(P = 0.012). The interspace angle after surgery was correlated with that on convex side-bending films (r = 0.418, P = 0.022), and the interspace angle at final follow-up was correlated with that after surgery (r = 0.625, P = 0.000). There was significant correlation between the loss of the interspace angle and the loss of coronal Cobb angle of the main curve during follow-up (r = 0.483, P = 0.007). Conclusion: The interspace angle could be improved after anterior correction and instrumentation surgery, but it became larger during follow-up. The loss of the interspace angle was correlated with the loss of coronal Cobb angle of the main curve during follow-up. [ABSTRACT FROM AUTHOR]