Scoliosis-Associated Cervical Spine Pathologies

BackgroundThough several studies have reported the incidence of intraspinal neural axis abnormalities in infantile and juvenile “presumed idiopathic” scoliosis, there has been a varying prevalence ranging from 11.1 to 26.0 % based on a limited sample size. Therefore, such inconclusive findings have resulted in some questions on the MRI-associated role in the management of these patients. We aimed to investigate the prevalence and distribution of intraspinal anomalies in the infantile and juvenile patients with “presumed idiopathic” scoliosis and to explore the radiographic and clinical indicators with large sample size.MethodsA total of 504 infantile and juvenile patients diagnosed with “presumed idiopathic” scoliosis were examined for potentially-existing neural axis abnormalities by MRI. Patients were grouped into two cohorts according to the presence of neural axis abnormalities. Radiographic parameters including curve magnitude, curve pattern, location of apex, degree of thoracic kyphosis, and span of curve were recorded and compared between the two groups. The prevalence of the neural abnormalities between the infantile-age group and juvenile-age group was also compared. The student t test was used to evaluate the differences of continuous variables and the chi-square test was used to evaluate the difference of categorical variables. Fisher exact test was applied to detect the difference of the rate of intraspinal anomalies between the “infantile idiopathic scoliosis” and “juvenile idiopathic scoliosis” group.ResultsInvolving the spinal cord, 94 patients (18.7 %) were found to have a neural abnormality: Arnold-Chiari malformation alone in 43 patients, Arnold-Chiari malformation combined with syringomyelia in 18 patients, isolated syringomyelia in 13 patients, diastematomyelia in six patients, tethered cord combined with diastematomyelia in six patients, tethered cord alone in four patients, and other uncommon intraspinal abnormalities in the remaining four patients. Totally Arnold-Chiari malformation with or without syringomyelia accounted for 64.8 % (61/94) among all these abnormalities. Male gender, left thoracic curve and right lumbar curve were found to be significantly associated with the presence of neural axis abnormalities on MRI.ConclusionsThe incidence of neural axis abnormalities in the presumed IIS and JIS was 18.7 %. Thus a routine MRI evaluation appears warranted for those “presumed idiopathic” scoliosis patients if aged less than 10 years, being male or having left thoracic or right lumbar curve.

Study DesignRetrospective case series.PurposeThe purpose of this study was to examine the incidence of neural axis abnormalities and the relevant risk factors in patients with adolescent idiopathic scoliosis (AIS).Overview of LiteratureThe use of preoperative magnetic resonance imaging (MRI) to assess the whole spine in patients with idiopathic scoliosis is controversial, and indications for such MRI evaluations have not been definitively established. However, we routinely use whole-spine MRI in patients with scoliosis who are scheduled to undergo surgical correction.MethodsA total of 378 consecutive patients with presumed AIS who were admitted for spinal surgery were examined for neural axis abnormalities using MRI. To differentiate patients with normal and abnormal MRI findings, the following clinical parameters were evaluated: age, sex, menarcheal status, rotation angle (using a scoliometer), coronal balance, shoulder height difference, and low back pain. We radiographically evaluated curve type, thoracic or thoracolumbar curve direction, curve magnitude and flexibility, apical vertebral rotation, curve length, coronal balance, sagittal balance, shoulder height difference, thoracic kyphosis, and the Risser sign.ResultsNeural axis abnormalities were detected in 24 patients (6.3%). Abnormal MRI findings were significantly more common in males than in females and were associated with increased thoracic kyphosis. However, there were no significant differences in terms of the other measured parameters.ConclusionsAmong the patients with presumed AIS who received preoperative whole-spine MRI, 6.3% had neural axis abnormalities. Males and patients with increased thoracic kyphosis were at a higher risk.

319. Minimally invasive surgery for neuromuscular scoliosis: a case control study of 140 consecutive patients

Progressive scoliosis eventually leads to extensive spinal fusion surgery, which carries a risk for significant bleeding. Neuromuscular scoliosis (NMS) patients have an additional inherent risk of major perioperative bleeding. The purpose of our research was to investigate the risk factors for measured (intraoperative, drain output) and hidden blood loss related to pedicle screw instrumentation in adolescents, divided into adolescent idiopathic scoliosis (AIS) and NMS patient groups. A retrospective cohort study with prospectively collected data of consecutive AIS and NMS patients undergoing segmental pedicle screw instrumentation at a tertiary level hospital between 2009 and 2021 was conducted. In total, 199 AIS (mean age 15.8 years, 143 females) and 81 NMS patients (mean age 15.2 years, 37 females) were included in the analysis. In both groups, levels fused, increased operative time, and smaller or larger size of erythrocytes were associated with perioperative blood loss (p < 0.05 for all correlations). In AIS, male sex (p < 0.001) and the number of osteotomies correlated with more drain output. In NMS, levels fused correlated with drain output, p = 0.00180. In AIS, patients’ lower preoperative MCV levels (p = 0.0391) and longer operation times, p = 0.0038, resulted into more hidden blood loss, but we did not find any significant risk factors for hidden blood loss in NMS patients.

We investigated the presence of neural axis abnormalities by magnetic resonance imaging (MRI) in patients with clinically and radiologically confirmed absolute flexible thoracic curves (King-Moe type III). Preoperative MRI scans of 84 patients (34 males, 50 females; mean age 14.1 years; range 11 to 17 years) with adolescent idiopathic scoliosis and a flexible thoracic curve (King-Moe type III) were prospectively evaluated to determine neural axis abnormalities. Clinical and radiologic indications for preoperative MRI examination were sought. All patients were treated with posterior fusion. All patients were intraoperatively monitored by means of motor evoked potentials by magnetic transcortical stimulation and somatosensory evoked potentials. The mean follow-up was 50.1 months (range 24 to 105 months). Neural axis abnormalities were detected in six patients (7.1%), including syringomyelia in five patients (5.9%) and diastometamyelia in one patient (1.2%). Of 84 patients, 51 patients (60.7%) had a relatively decreased spinal cord diameter, and an increased epidural distance. No clinical or radiologic indicators were found for preoperative MRI examination. No neurologic deficits developed during operation and in the postoperative period. The detection of neural axis abnormalities implies the necessity of intraoperative neurologic monitoring to minimize the risks for neurologic deficits. Where this is not available, preoperative MRI seems to be an essential tool in the prevention of surgery-associated neurologic risks.

Retrospective review of the Healthcare Cost and Utilization Project's Kids' Inpatient Database. The aim of this study was to investigate variations in surgical case volume in the US for spinal arthrodesis for pediatric patients with scoliosis. Approximately 38,000 spinal arthrodesis procedures are performed each year in the United States for pediatric patients with scoliosis. Anecdotally, pediatric spine surgeons report performing more scoliosis procedures during summer months and before national holidays than during other periods, but this has not been reported in the literature. A thorough understanding of seasonal variations in surgical volume may guide the allocation of health care resources. Using the Healthcare Cost and Utilization Project's Kids' Inpatient Database, we identified 32,563 patients aged <21 years with adolescent idiopathic scoliosis (54%), neuromuscular scoliosis (19%), congenital scoliosis (12%), early-onset scoliosis (12%), or scoliosis from other causes (5%) who underwent primary spinal arthrodesis from 2000 through 2016. χ goodness-of-fit tests were used to evaluate seasonal differences in the number of spinal arthrodesis procedures performed. A negative binomial distribution model was used to compare surgical volume by month. Alpha = 0.05. We found significant seasonal variation in the volume of spinal arthrodesis procedures performed, which persisted after stratifying by type of scoliosis (all, P < 0.001). For all types of scoliosis, surgical volume was highest during the summer. The 3 months with the highest surgical volumes were June, July, and December. The number of spinal arthrodesis procedures performed in the United States for pediatric scoliosis peaked significantly during the summer, with the highest volume of surgeries performed during June, July, and December. An understanding of these variations can help hospitals and providers allocate resources appropriately throughout the year according to predictable changes in surgical volume. 4.

The evaluation of brain tumor recurrence after surgery is based on the comparison between tumor regions on pre-operative and follow-up magnetic resonance imaging (MRI) scans in clinical practice. Accurate alignment of MRI scans is important in this evaluation process. However, existing methods often fail to yield accurate alignment due to substantial appearance and shape changes of tumor regions. The study aimed to improve this misalignment situation through multimodal information and compensation for shape changes. In this work, a deep learning-based deformation registration method using bilateral pyramid to create multi-scale image features was developed. Moreover, morphology operations were employed to build correspondence between the surgical resection on the follow-up and pre-operative MRI scans. Compared with baseline methods, the proposed method achieved the lowest mean absolute error of 1.82 mm on the public BraTS-Reg 2022 dataset. The results suggest that the proposed method is potentially useful for evaluating tumor recurrence after surgery. We effectively verified its ability to extract and integrate the information of the second modality, and also revealed the micro representation of tumor recurrence. This study can assist doctors in registering multiple sequence images of patients, observing lesions and surrounding areas, analyzing and processing them, and guiding doctors in their treatment plans.

Scoliosis is a three-dimensional spinal deformity exceeding 10 degrees. Left untreated, it can lead to comorbidities, as well as surface deformity. Brace treatment is common in smaller curves (20-40 degrees), with strong evidence in adolescent idiopathic scoliosis (AIS), but overall effectiveness and impact on quality of life is controversial. This scoping review sought to map existing reviews on all aspects of bracing in scoliosis of any aetiology, to identify future research priorities. Searches were conducted in MEDLINE and EMBASE, excluding abstracts, narrative reviews and guidelines. Included articles reported on scoliosis patients of any age and aetiology and examined the use of spinal bracing. Four independent reviewers screened articles for inclusion and completed data extraction. Data were summarised narratively in themes, looking at effectiveness of bracing in AIS, patient factors influencing outcomes of AIS brace treatment, interventions to improve bracing success in AIS, patient and family experiences with bracing in AIS and bracing in adult and neuromuscular scoliosis. AMSTAR2 was used to assess confidence in the results in the reviews. Searches yielded 59 eligible studies which were included. Whilst bracing is recommended for curves 20°-40° in AIS, it may be successful in those over 40° with good compliance. Bracing is effective in lowering rates of curve progression in AIS and therefore reducing surgery rates. There is no strong evidence that one brace type produces superior outcomes over another, compared to other treatments. Brace adherence is associated with significantly lower rates of curve progression; this is affected by appearance, comfort and psychology. Evidence shows adherence improves with sensor monitoring and psychosocial interventions. Some evidence suggests in-brace correction can be predicted by curve flexibility. More remaining growth potential and associated factors (younger age, lower Risser stage, pre-menarchial, open triradiate cartilage) can increase the risk of curve progression during bracing. Scoliosis-specific exercises may be beneficial alongside brace treatment. Long-term QoL does not appear to be affected by brace treatment. Some low-quality evidence suggests reduced QoL during bracing compared to observation. Function may be impacted by brace treatment, but pain is not increased. In degenerative spinal deformity, there may be some shorter term benefit for pain and function. Little evidence on bracing in neuromuscular scoliosis exists. There is a large research base of evidence to support bracing for AIS; however, this base is limited due to the substantial amount of low-quality research it includes. The aim of this scoping review was to identify gaps in the literature to guide future research. This comprehensive review captured the breadth of existing review evidence on all aspects of bracing in scoliosis. Evidence supports bracing as an effective treatment in scoliosis, controlling curve progression, and often increasing patient satisfaction. Compliance is key, and measures such as compliance sensors can be effective. This scoping review has summarised the existing literature; however, the evidence base is limited. Further research could explore objective measures for compliance monitoring, optimal treatment protocols around brace cessation and effects of bracing on patient quality of life.

Introduction Cervical spine pathologies are common in Down syndrome (DS) patients. Cervical pathologies may cause cord compression and neurologic deterioration if left untreated. Complication rates of 73-100% have been reported in DS patients after cervical spine surgery in historical studies. This study reports updated perioperative complications rates and long-term outcome in patients with DS undergoing cervical spine surgery. Methods Retrospective review of patients with DS who have undergone cervical spine surgery from 1998 to 2011 (≥ 24 months of follow-up) was undertaken. Series of 17 adults with preoperative diagnoses that included atlantoaxial instability, stenosis, spondylosis, or cervical spondylolisthesis were evaluated. Nine patients received recombinant human bone morphogenetic protein-2 (rhBMP-2). Neurologic and ambulatory status was evaluated at regular intervals included pre- and postoperative imaging, range of motion evaluation, strength/neurologic testing, ambulation observation, and patient and caretaker pain reporting. Results A total of 20 surgical procedures were performed in 17 patients. Average follow-up was 78.7 months (range: 25-156 months). Overall, 37 complications were observed including pneumonia, respiratory distress, reintubation, dysphagia, deep venous thrombosis, sepsis, wound infection, dehiscence, neurologic complications, loss of reduction (LOR), pseudarthrosis, and hardware failure. Postoperative pneumonia was most common (23.5%). Three patients developed pseudarthrosis (all in the rhBMP-2 group); three demonstrated LOR. Neurologic complications (N = 3) included spasticity, loss of ambulation, and postoperative weakness with myelomalacia. Two were transient. Respiratory complications in the rhBMP-2 group were the most common (N = 3). The anterior approach resulted in a higher likelihood of complications than the posterior (p = 0.032). Conclusions Current techniques may improve pseudarthrosis (p = 0.009), LOR (p = 0.043), and first attempt (p = 0.038) and overall fusion rates (p = 0.018) compared with historical studies. Complications continue to challenge most patients (82.4%). A total of 16 of 17 patients (94.1%) demonstrated stabilization or improvement in neurologic status. Apparent successful outcome in the majority appears to warrant the high complication risk associated with cervical spine surgery in DS patients. The anterior approach resulted in a higher risk of complications than posterior (p = 0.032). We report a higher than expected incidence of pseudarthrosis in DS patients receiving rhBMP-2, putting its benefit in DS patients into question.

(1) Background: In the preoperative planning stage of scoliosis surgery, it is routine to use radiographs obtained with and without traction to observe the curve flexibility in order to estimate curve correction, but its association with mechanical complications is not completely understood. (2) Methods: Retrospective cohort study of all patients undergoing infantile, congenital, neuromuscular or idiopathic adolescent scoliosis correction surgery at a single institution between 2015 and 2019, with a minimum follow-up of 24 months. Associations between qualitative variables were tested with the chi-square test. The association between qualitative and quantitative variables were tested with the Mann–Whitney test, and correlations between quantitative variables was tested with Spearman’s correlation. (3) Results: A total of 330 patients, 88 males and 242 females, with a mean age of 16.98 years at surgery, were included. The mean value of preoperative main curves, its flexibility and postoperative value were 54.44 degrees, 21.73 degrees and 18.08 degrees, respectively. (4) Conclusions: Preoperative spinal X-ray examination with traction or bending films is a reasonable option for assessing scoliotic curve flexibility, and patients with neuromuscular scoliosis who are not ambulatory can be informed of the increased risks of late mechanical complications.

Objective The primary objective of this study was to analyze the prevalence of PJK in patients with neuromuscular scoliosis submitted to posterior spinal arthrodesis with instrumentation. Proximal junctional kyphosis (PJK) is a frequent phenomenon that, due to its importance, began to be studied by several authors, who laid the foundations for the radiographic definition and possible risk factors for its occurrence after long spinal arthrodesis with instrumentation. Despite the large number of PJK studies, most were related to the occurrence of adolescent idiopathic scoliosis, adult deformity and early-onset scoliosis, with few being targeted to patients with congenital and neuromuscular scoliosis. Methods In this study, data from electronic medical records of patients with neuromuscular scoliosis who underwent posterior arthrodesis with instrumentation between the years 2014 and 2016 were analyzed. Information on age, gender, pathology and radiographic measurements were extracted from this sample at the 2nd and 24th postoperative months. Results A total of 39 patients with neuromuscular scoliosis were analyzed. The sample was predominantly male (58.87%) and the mean age was 14.05 years. PJK occurred in 18 patients during the two years following surgery, with a prevalence of 46.15%. The incidence of PJK in the 2nd and 24th postoperative months was 23.1% and 30%, respectively. Conclusions A prevalence of PJK of 46.15% was found in patients with neuromuscular scoliosis treated surgically with posterior instrumentation after two years of follow-up, as compared to previous results . Level of Evidence III; Cross-sectional observational study.

Surgical outcome of Chiari I malformations--an experience sharing and literature review.

ObjectivesThis study aims to define the chronological, angular, and topographic classes in presumed idiopathic scoliosis and to investigate the frequency of generalized joint hypermobility, pain, neurological deficit, ankle deformity, Risser grade, and magnetic resonance imaging (MRI) findings in these patients.Patients and methodsThis cross-sectional, descriptive study included a total of 36 patients (11 males, 25 females; mean age 13.4±4.6 years; range, 6 to 24 years) with idiopathic scoliosis between January 2015 and January 2019. Data including age, sex, complaint of pain, generalized joint hypermobility (based on Beighton score), neurological deficit, ankle deformity, and definition of scoliosis were recorded. Chronological, angular, and topographic classification were carried out. The Risser grade and MRI findings were noted.ResultsOf all patients, 30 (83.3%) were idiopathic, five (13.9%) were neuromuscular, and one (2.8%) was congenital scoliosis based on MRI findings. Of 13 (36.1%) spine MRI scans, six (46.2%) were intraspinal anomalies, four were syringomyelia (30.8%), one was Chiari type 1 malformation (7.7%), and one was hemivertebrae with diastematomyelia (7.7%). The highest rates of classes according to chronological, angular, and topographical classifications of idiopathic scoliosis were adolescent (17/30, 56.7%), low angular (24/30, 80.0%), and lumbar scoliosis (15/30, 50.0%), respectively. Ten patients (33.3%) complained of pain, while 23 patients (76.7%) had no neurological deficit and seven (23.3%) had hypoesthesia. Seventeen patients (56.7%) had generalized joint hypermobility.ConclusionIdiopathic scoliosis with non-severe spinal deformity may present with intraspinal neural axis abnormalities, even when it is neurologically intact. Based on our study results, it seems to be useful to consider whole spine MRI for the evaluation of thoracic and lumbar scoliosis.

Documents indicated that the average prevalence of intraspinal neural axis abnormalities (INAAs) in presumed idiopathic scoliosis (PIS) patients was about 17.7%. However, paucity study focuses on the incidence of INAAs in severe spinal deformity (SSDs). In this study, we investigate the incidence of intraspinal neural axis abnormalities (IINAAs) and the clinical relevance in SSD at a single center. All the patients with SSDs admitted for spinal surgery were evaluated from 2003 to 2014. patients who present with coronal Cobb over 90° (and/or the sagittal Cobb≥90°); patients with whole spine magnetic resonance imaging (MRI) done preoperatively; and patients with documented clinical findings preoperatively. ankylosing spondylitis, adult onset scoliosis, scoliosis secondary to bone destruction, and spinal dysraphism. 101 patients fulfilled the criteria were included. 43 patients were detected with INAAs (42.6%, 43/101). The most common INAAs was syrinx (S) (16/43, 37.2%). Of which, 43.7% (7/16), 37.5% (6/16), and 18.7% (3/16) were spindle, slit, and swelling types, respectively. Most of them were located in thoracic (6/16, 37.5%) and cervical (5/16, 31.3%) region. MRI revealed Chiari malformation with syringomyelia (C+S) in ten patients (10/43, 23.2%), Chiari malformation (C) in 6 patients (6/43, 13.9%) and others in 11 patients (11/43, 25.6%). As to the etiology, most patients with INAAs were PIS (34/43, 79.1%). On clinical examination, 16 of 101 patients (16/101, 15.8%) had abnormal neurologic signs. 15 of 16 patients (15/16, 93.7%) with abnormal neurologic signs had INAAs on MRI. On the other hand, 28 of 43 patients (28/43, 65.1%) with INAAs on MRI presented neurologically intact. 28 of 85 patients (28/85, 32.9%) with neurologically intact were detected with INAAs on MRI. The incidence of INAAs in SSDs was 42.6%. 65.1% of them present intact neurologic status. The most common neural anomaly was syrinx. Preoperative whole spine MRI must be beneficial for SSDs even in the absence of neurological findings. These slides can be retrieved under Electronic Supplementary Material.

Combined intraoperative monitoring (IOM) of transcranial electric motor-evoked potentials (tce-MEPs) and somatosensory-evoked potentials (SSEPs) is safe and effective for spinal cord monitoring during scoliosis surgery. However, the literature data regarding the reliability of spinal cord monitoring in patients with neuromuscular scoliosis are conflicting and need to be confirmed. We reviewed IOM records of 40 consecutive patients with neuromuscular scoliosis related to central nervous system (CNS) (29 pts) or peripheral nervous system (PNS) (11 patients) diseases, who underwent posterior fusion with instrumentation surgery for spinal deformity. Multimodalitary IOM with SSEPs and tce-MEPs was performed. Spinal cord monitoring using at least one modality was attempted in 38/40 (95 %) patients. No false-negative results were present in either group, but a relatively high incidence of false-positive cases (4/29, 13.8 %) was noted in the CNS group. Two patients in the CNS group and one patient in the PNS group presented transient postoperative motor deficits (true positive), related to surgical manoeuvres in two cases and to malposition in the other one. Multimodalitary IOM is safe and effective to detect impending spinal cord and peripheral nerves dysfunction in neuromuscular scoliosis surgery. However, the interpretation of neurophysiological data may be challenging in such patients, and the rate of false-positive results is high when pre-operatory motor deficits are severe.