Spinal Muscular Atrophy Scoliosis in the Era of Background Therapies-A Review of the Literature.

Spinal deformities are considered an important complication of neuromuscular disorders such as spinal muscular atrophy (SMA). SMA patients typically develop progressive early-onset scoliosis, which is associated with increased functional decline, discomfort, and respiratory dysfunction. Over the second decade of the twenty-first century, a lot has changed in terms of the therapeutic options available to people with SMA. Specifically, the use of pharmaceutical agents such as nusinersen (Spinraza), onasemnogene abeparvovec (Zolgensma), and risdiplam (Evrysdi) has dramatically changed the landscape for SMA patients. These medications significantly alter motor- and respiratory functioning, as well as the natural progression of spinal deformities. When evaluating these agents and their impact on the development of scoliosis and motor functioning, it is important to consider the timing of treatment initiation. In patients treated after they had already developed symptoms, a shift of phenotype to a less severe subtype has been observed. This results in a delay in the onset of scoliosis for the less severe SMA types and an increase in early-onset scoliosis for the severe types in patients who would typically not live to develop scoliosis. Patients who receive treatment before they develop symptoms achieve almost normal motor functioning and will likely have a significant decrease in scoliosis prevalence or at least delay its onset.

Abnormal eyes and spine development in zebrafish (Danio rerio) embryos and larvae induced by triphenyltin.

Triphenyltin (TPT) is widely used in crop pest control and ship antifouling coatings, which leads to its entry into aquatic environment and poses a threat to aquatic organisms. However, the effects of TPT on the early life stages of wild fish in natural water environments remains unclear. The aim of this study was to assess the toxic effects of TPT on the early life stages of fish under two different environments: field investigation and laboratory experiment. The occurrence of deformities in wild fish embryos and larvae in the Three Gorges Reservoir (TGR) and the developmental toxicity of TPT at different concentrations (0, 0.15, 1.5 and 15 µg Sn/L) to zebrafish embryos and larvae were observed. The results showed that TPT content was higher in wild larvae, reaching 27.21 ng Sn/g w, and the malformation of wild fish larvae mainly occurred in the eyes and spine under natural water environment. Controlled experiment exposure of zebrafish larvae to TPT also resulted in eye and spinal deformities. Gene expression analysis showed that compared with the control group, the expression levels of genes related to eye development (sox2, otx2, stra6 and rx1) and spine development (sox9a and bmp2b) were significantly up-regulated in the 15 µg Sn/L exposure group, which may be the main cause of eye and spine deformity in the early development stage of fish. In addition, the molecular docking results further elucidate that the strong hydrophobic and electrostatic interactions between TPT and protein residues are the main mechanism of TPT induced abnormal gene expression. Based on these results, it can be inferred that TPT is one of the teratogenic factors of abnormal eye and spine development in the early life stage of fish in the TGR. These findings have important implications for understanding the toxicity of TPT on fish.

Bibliometric Analysis of the Development, Current Status, and Trends in Adult Degenerative Scoliosis Research: A Systematic Review from 1998 to 2023.

Purpose: Adult degenerative scoliosis (ADS) research lacks bibliometric analysis, despite numerous studies. This study aimed to systematically analyze the development, current status, hot topics, frontier areas, and trends in ADS research. Patients and Methods: A systematic literature review was conducted in the Web of Science Core Collection database from January 1998 to June 2023. Information regarding the country, institution, author, journal, and keywords was collected for each article. Bibliometric analysis was performed using VOSviewer and Citespace software. Results: The final analysis covered 1695 publications, demonstrating a steady increase in ADS research. The United States was the most prolific and influential country with 684 publications, followed by China and Japan. The University of California System was the most productive institution with 113 publications. Shaffrey, CI (47 publications) and Lenke, LG (41 publications) were top authors. The analysis revealed seven main research clusters: "intervertebral disc", "adult spinal deformity", "lumbar fusion", "minimally invasive surgery", "navigation", "postoperative complications", and "mental retardation". Keywords with strong bursts of activity included degeneration, prevalence, imbalance, classification, lumbar spinal stenosis, and kyphosis. Conclusion: In conclusion, in recent years, ADS research has undergone rapid development. This study analyzed its hot topics, advancements, and research directions, making it the latest bibliometric analysis in this field. The findings aim to provide a new perspective and guidance for clinical practitioners and researchers.

Skeletal Morphogenesis and Anomalies in Gilthead Seabream: A Comprehensive Review.

The gilthead seabream, one of the most important species in Mediterranean aquaculture, with an increasing status of exploitation in terms of production volume and aquafarming technologies, has become an important research topic over the years. The accumulation of knowledge from several studies conducted during recent decades on their functional and biological characteristics has significantly improved their aquacultural aspects, namely their reproductive success, survival, and growth. Despite the remarkable progress in the aquaculture industry, hatchery conditions are still far from ideal, resulting in frequent abnormalities at the beginning of intensive culture, entailing significant economic losses. Those deformities are induced during the embryonic and post-embryonic periods of life, and their development is still poorly understood. In the present review, we created a comprehensive synthesis that covers the various aspects of skeletal morphogenesis and anomalies in the gilthead seabream, highlighting the genetic, environmental, and nutritional factors contributing to bone deformities and emphasized the potential of the gilthead seabream as a model organism for understanding bone morphogenesis in both aquaculture and translational biological research. This review article addresses the existing lack in the literature regarding gilthead seabream bone deformities, as there are currently no comprehensive reviews on this subject.

Gender-Related Factors Influence the Subjective Perception of Deformity in Patients Undergoing Surgery for Idiopathic Scoliosis.

The present study aims to depict the importance of gender-related factors in the subjective perception of spine deformity in adolescents undergoing posterior instrumented fusion for scoliosis. Patients undergoing posterior spinal instrumentation and fusion (PSF) for idiopathic adolescent scoliosis (AIS) were recruited. The following data were recorded: gender, age, parents' civil status, Tegner Activity Scale (TAS), body mass index (BMI), concomitant diseases, and history of neuropsychological disorders. Each patient underwent clinical and radiological evaluations according to the protocol used at our institution. All the patients were assessed before surgery using the following Patient-Reported Outcome Measures (PROMs): the Italian version of the revised Scoliosis Research Society-22 patient questionnaire (SRS-22R), the Quality-of-Life Profile for Spinal Deformities (QLPSDs) questionnaire, and the Spinal Appearance Questionnaire (SAQ). The present study recruited 80 patients (male: 19, female: 61). A significant correlation was observed between BMI, TAS, and subjective perception scores. A worse deformity perception was observed in female patients and patients with divorced parents. Gender-related factors impact the subjective perception of spine deformity in patients undergoing PSF for AIS. Specific assessment and correction are needed to improve postoperative outcomes in these patients.

Surgical Treatment of Spinal Deformities in Pediatric Orthopedic Patients.

Scoliosis and Scheuermann's disease are common spinal deformities that affect a substantial population, particularly adolescents, often impacting their quality of life. This comprehensive review aims to present a detailed understanding of these conditions, their diagnosis, and various treatment strategies. Through an extensive exploration of current literature, the review discusses the etiology of these spinal deformities and the use of diagnostic tools such as X-rays and MRI. It further delves into the range of treatment options available, from conservative approaches such as physiotherapy and bracing to more invasive surgical interventions. The review underscores the necessity of an individualized treatment approach, taking into account factors such as the patient's age, the severity of the curvature, and overall health. This all-encompassing perspective on scoliosis and Scheuermann's disease will aid in evidence-based decision making in their management with the goal of improving patient outcomes.

Future Data Points to Implement in Adult Spinal Deformity Assessment for Artificial Intelligence Modeling Prediction: The Importance of the Biological Dimension.

Adult spinal deformity (ASD) surgery is still associated with high surgical risks. Machine learning algorithms applied to multicenter databases have been created to predict outcomes and complications, optimize patient selection, and improve overall results. However, the multiple data points currently used to create these models allow for 70% of accuracy in prediction. We need to find new variables that can capture the spectrum of probability that is escaping from our control. These proposed variables are based on patients' biological dimensions, such as frailty, sarcopenia, muscle and bone (tissue) sampling, serological assessment of cellular senescence, and circulating biomarkers that can measure epigenetics, inflammaging, and -omics. Many of these variables are proven to be modifiable and could be improved with proper nutrition, toxin avoidance, endurance exercise, and even surgery. The purpose of this manuscript is to describe the different future data points that can be implemented in ASD assessment to improve modeling prediction, allow monitoring their response to prerehabilitation programs, and improve patient counseling.

Risk factors for postoperative coronal imbalance after surgical correction of adult spinal deformities: a systematic review with pooled analysis.

OBJECTIVE: The aim of this study was to identify preoperative risk factors for postoperative coronal imbalance (CIB) in patients undergoing surgical correction for adult spinal deformity (ASD). METHODS: A systematic search of articles about risk factors for postoperative CIB was conducted on electronic databases (PubMed, EMBASE, and Cochrane Library) according to the PRISMA guidelines. The methodological quality of the included articles was assessed using the Institute of Health Economics Quality Appraisal Checklist for Case Series Studies. Data regarding the patient population (demographics, diagnosis, and follow-up), pre- and postoperative radiographic and clinical data, surgical procedure details, and complications were extracted and summarized. Meta-analyses were performed when possible. RESULTS: Nine studies comprising 838 patients were included. The pooled incidence of postoperative CIB was 26%; preoperative sagittal vertical axis (SVA) and type C imbalance (trunk shifted toward the convexity) were found to be the only primary risk factors for iatrogenic CIB (p < 0.001 and p = 0.012, respectively). CONCLUSIONS: Baseline evaluation of a patient's global alignment is of outmost importance when planning surgery for ASD. Type C coronal imbalance (trunk shifted toward the convexity of the main curve) and increased preoperative SVA put the patient at risk of postoperative CIB. Intraoperatively, care should be taken to properly correct the lumbosacral fractional curve.

A novel Hinge-Link correction system for vertebral column resection: a pilot study in a porcine model.

PURPOSE: To introduce a novel Hinge-Link (HL) correction system for vertebral column resection (VCR) in the most severe scoliosis and surgically assess the device in a porcine scoliosis model. METHODS: The HL was introduced and described how it works on a reproduced three-dimensional spine model of severe scoliosis. A right thoracic scoliosis was surgically created in five 4-month-old pigs. Two weeks later, the VCR was performed to correct the deformity using the HL to test its feasibility and neurological safety in the pig scoliosis model. RESULTS: The surgically created right thoracic scoliosis averaged 32° ± 9.3 prior to the VCR. All animals tolerated the VCR procedure awakening neurologically intact and ambulated for 24 h. The HL takes advantage of the ability to provide three-dimensional correction of the deformity and excellent control of the spine segments to prevent damage to the spinal cord in the VCR procedure. The mean correction of the scoliosis was 94% correcting to 1.8° ± 0.8. At sacrifice, the pathological anatomy of the neural axis demonstrated no vertebral subluxation and no dural impingement in any animal. CONCLUSION: The HL was feasible and neurologically safe for the VCR procedure in the pig scoliosis model. It can provide significant correction of the spinal deformity with appropriate mild shortening at the resected levels and overall lengthening without neurologic deficits. The rigid control of the spine segments provided by this device should allow for improved correction with decreased neurologic deficits and potentially shorter surgical time.

Risk Factors in Administering Spinal Anesthesia: A Comprehensive Review.

Numerous advantages, including a quick start and consistent anesthesia, are provided by spinal anesthesia, a method often utilized in contemporary medicine for various surgical operations. However, it has some hazards, just like any medical procedure. With an emphasis on identifying and assessing the risk factors associated with administering spinal anesthesia, the review analyzes published literature and clinical investigations carried out in the field of anesthesia. Various key factors, including technique-related procedural and patient-related aspects, can influence the effectiveness of spinal anesthesia. Among these factors are age, sex, body mass index, concurrent conditions (such as cardiovascular disease, diabetes, and respiratory problems), pre-existing neurological issues, allergies, and a history of adverse responses to anesthesia drugs. Additionally, the chance of problems might be increased by physical abnormalities or malformations in the spinal canal and vertebral column. The safety and effectiveness of spinal anesthesia depend significantly on procedural factors, such as the type and dosage of anesthesia agents administered and the patient's position and alignment maintained during the entire surgical procedure and the injection rate. Increased risks can also be caused by inadequate monitoring and a slow response to unfavorable circumstances. Risk factors related to the technique include the expertise and competency of the anesthesiologist or medical professional carrying out the procedure. Inadequate post-procedure monitoring, inadvertent dural puncture, and improper needle placement might lead to complications during or after the spinal anesthesia administration. This review emphasizes the need for a complete preoperative assessment, suitable patient selection, and rigorous procedural planning to reduce the likelihood of problems during the administration of spinal anesthesia. It also emphasizes the significance of ongoing monitoring and timely management of adverse events to guarantee patient safety and the best results. Healthcare professionals may put preventative measures in place and follow best practices to limit possible consequences efficiently by recognizing the risk factors associated with spinal anesthesia. This review helps encourage safer anesthesia practices and improve patient care as medical knowledge and technology advance. However, further study and evidence-based recommendations are required to enhance patient outcomes and risk assessment.

Assessing the influence of modifiable patient-related factors on complication rates after adult spinal deformity surgery.

AIMS: Postoperative complication rates remain relatively high after adult spinal deformity (ASD) surgery. The extent to which modifiable patient-related factors influence complication rates in patients with ASD has not been effectively evaluated. The aim of this retrospective cohort study was to evaluate the association between modifiable patient-related factors and complications after corrective surgery for ASD. METHODS: ASD patients with two-year data were included. Complications were categorized as follows: any complication, major, medical, surgical, major mechanical, major radiological, and reoperation. Modifiable risk factors included smoking, obesity, osteoporosis, alcohol use, depression, psychiatric diagnosis, and hypertension. Patients were stratified by the degree of baseline deformity (low degree of deformity (LowDef)/high degree of deformity (HighDef): below or above 20°) and age (Older/Younger: above or below 65 years). Complication rates were compared for modifiable risk factors in each age/deformity group, using multivariable logistic regression analysis to adjust for confounders. RESULTS: A total of 480 ASD patients met the inclusion criteria. By two years, complication rates were 72% ≥ one complication, 28% major, 21% medical, 27% surgical, 11% major radiological, 8% major mechanical, and 22% required reoperation. Younger LowDef patients with osteoporosis were more likely to suffer either a major mechanical (odds ratio (OR) 5.9 (95% confidence interval (CI) 1.1 to 36.9); p = 0.048) or radiological complication (OR 7.0 (95% CI 1.9 to 25.9); p = 0.003). Younger HighDef patients were much more likely to develop complications if obese, especially major mechanical complications (OR 2.8 (95% CI 1.1 to 8.6); p = 0.044). Older HighDef patients developed more complications when diagnosed with depression, including major radiological complications (OR 3.5 (95% CI 1.1 to 10.6); p = 0.033). Overall, a diagnosis of depression proved to be a risk factor for the development of major radiological complications (OR 2.4 (95% CI 1.3 to 4.5); p = 0.005). CONCLUSION: Certain modifiable patient-related factors, especially osteoporosis, obesity, and mental health status, are associated with an increased risk of complications after surgery for spinal deformity. Surgeons should look for these conditions when assessing a patient for surgery, and optimize them to the fullest extent possible before proceeding to surgical correction so as to minimize the prospect of postoperative morbidity.Cite this article: Bone Joint J 2022;104-B(11):1249-1255.

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.

Pelvic incidence significance relative to spinopelvic risk factors for total hip arthroplasty instability.

AIMS: Pelvic incidence (PI) is a position-independent spinopelvic parameter traditionally used by spinal surgeons to determine spinal alignment. Its relevance to the arthroplasty surgeon in assessing patient risk for total hip arthroplasty (THA) instability preoperatively is unclear. This study was undertaken to investigate the significance of PI relative to other spinopelvic parameter risk factors for instability to help guide its clinical application. METHODS: Retrospective analysis was performed of a multicentre THA database of 9,414 patients with preoperative imaging (dynamic spinopelvic radiographs and pelvic CT scans). Several spinopelvic parameter measurements were made by engineers using advanced software including sacral slope (SS), standing anterior pelvic plane tilt (APPT), spinopelvic tilt (SPT), lumbar lordosis (LL), and PI. Lumbar flexion (LF) was determined by change in LL between standing and flexed-seated lateral radiographs. Abnormal pelvic mobility was defined as ∆SPT ≥ 20° between standing and flexed-forward positions. Sagittal spinal deformity (SSD) was defined as PI-LL mismatch > 10°. RESULTS: PI showed a positive correlation with parameters of SS, SPT, and LL (r-value range 0.468 to 0.661). Patients with a higher PI value showed higher degrees of standing LL, likely as a compensatory measure to maintain sagittal spine balance. There was a positive correlation between LL and LF such that patients with less standing LL had decreased LF (r = 0.49). Similarly, there was a positive correlation between increased SSD and decreased LF (r = 0.54). PI in isolation did not show any significant correlation with lumbar (r = 0.04) or pelvic mobility (r = 0.02). The majority of patients (range 89.4% to 94.2%) had normal lumbar and pelvic mobility regardless of the PI value. CONCLUSION: The PI value alone is not indicative of either spinal or pelvic mobility, and thus in isolation may not be a risk factor for THA instability. Patients with SSD had higher rates of spinopelvic stiffness, which may be the mechanism by which PI relates to THA instability risk, but further clinical studies are required. Cite this article: Bone Joint J 2022;104-B(3):352-358.

Correlation analysis of the PI-LL mismatch according to the pelvic incidence from a database of 468 asymptomatic volunteers.

PURPOSE: Previous studies on adults with degenerative scoliosis (ADS) have been fixed the threshold of PI-LL mismatch less than 10° for achieving good clinical outcomes. Recent studies discussed that PI-LL mismatch should consider individual pelvic incidence (PI) and should be set first in a normal population. The purpose of this study is to assess the variability of PI-LL mismatch according to PI in an asymptomatic population. METHODS: Full-body low dose stereoradiographic evaluation was done in a multi-ethnic cohort of 468 asymptomatic adult volunteers. Patients were clustered in three groups depending on individual PI values: PI < 45°, 45° < PI < 60° and PI > 60°. 3D measurements were performed using a commercially available 2D/3D modeling software to establish a correlation of PI with other spinopelvic parameters. ANOVA and Tukey's HSD for post-hoc analysis were used to determine the differences between the three groups. RESULTS: In our asymptomatic population, the mean value of PI-LL mismatch is - 5.4° ± 10.7°. Clusterization of the population reveals significant differences in the distribution of L1S1 lordosis, pelvic tilt and PI-LL with positive linear correlation according to PI values. As an interestingly result, PI-LL mismatch is equal to 0° when PI is around 64°. CONCLUSIONS: The present study demonstrated that PI-LL mismatch is negative in an asymptomatic population (- 5.4° ± 10.7°) and the value should be customized to each patient to be able to restore the appropriate lordosis in ADS. The PI-LL mismatch is given by the formula PI-LL = - 28.5 + 0.44 × PI.

Subjective perception of spinal deformity after selective versus non-selective fusion of Lenke 1C curves.

PURPOSE: To assess the self-image perception and the Quality-of-Life (QoL) in female adolescents, with Lenke 1C scoliosis curves, treated with selective versus non-selective posterior spinal instrumentation and fusion (PSF). METHODS: Patients undergoing PSF for idiopathic adolescent scoliosis (AIS) were recruited and divided into two groups: patients managed with selective thoracic fusion (STF) were included in Group A, whereas patients treated with non-selective fusion (N-STF) in Group B. Each patient completed the Italian version of the Scoliosis Research Society-22R questionnaire (SRS-22R), the Quality-of-Life Profile for Spinal Deformities questionnaire (QLPSD) and the Spinal Appearance Questionnaire (SAQ), before surgery and at 24-month follow-up. RESULTS: One hundred and fifty seven female patients (mean age 16.38) were included in this study. 80 patients underwent STF, while 77 patients received N-STF. At 24-month follow-up, patients managed with N-STF showed better SRS-22R self-image mean score (p = .012), SRS-22R satisfaction mean score (p = .033), QLPSD body image mean score (p = .005), but worse SRS-22 function mean score (p = .006) and QLPSD back flexibility mean score (p = .007), compared with patients who underwent STF. In terms of self-image perception, patients undergoing STF showed significantly worse SAQ total mean score (p = .002), SAQ appearance mean score (p = .001) and SAQ expectation (p = .001). We found a significant correlation between SAQ appearance mean score and SRS-22R self-image (R = - 0.721), SRS-22 mental health (R = - 0.8), QLPSD psychosocial functioning (R = 0.7) and QLPSD back flexibility (R = 0.8). CONCLUSION: Although the STF of Lenke 1C curves provides better functional outcomes, in the present study, female patients receiving STF revealed a worse perceived body image, compared with patients treated with N-STF, at 24-month follow-up. Particular attention should be addressed to the preoperative patient's mental health and body image perception, when choosing between STF and N-STF.

Evaluation of Brace Treatment Using the Soft Brace Spinaposture: A Four-Years Follow-Up.

The braces of today are constructed to correct the frontal plane deformity of idiopathic adolescent scoliosis (AIS). The Spinaposture brace© (Spinaposture Aps, Copenhagen, Denmark) is a soft-fabric brace for AIS and is designed to enhance rotational axial stability by inducing a sagittal plane kyphotic correction. This prospective observational study evaluated the brace in fifteen patients with AIS. The initial average CA was 16.8° (SD: 2.8). They were followed prospectively every 3 to 6 months during their brace usage until skeletal maturity of 25 months and at long-term follow-up of 44 months. In- and out-of-brace radiographs were performed in six subjects at inclusion. This resulted in an immediate in-brace correction of 25.3 percent in CA (14.3°â†’10.8°) and induced a kyphotic effect of 14.9 percent (40.8°â†’47.9°). The average in-brace improvement at first follow-up was 4.5° in CA, and the CA at skeletal maturity was 11° (SD: 7.4°) and long-term 12.0° (SD: 6.8°). In conclusion, the Spinaposture brace© had an immediate in-brace deformity correction and a thoracic kyphotic effect. At skeletal maturity, the deformities improved more than expected when compared to that of the natural history/observation and similar to that of other soft braces. No long-term deformity progression was seen. To substantiate these findings, stronger designed studies with additional subjects are needed.

Current concepts in the treatment of neuromuscular scoliosis: clinical assessment, treatment options, and surgical outcomes.

The development of spinal deformity in children with underlying neurodisability can affect their ability to function and impact on their quality of life, as well as compromise provision of nursing care. Patients with neuromuscular spinal deformity are among the most challenging due to the number and complexity of medical comorbidities that increase the risk for severe intraoperative or postoperative complications. A multidisciplinary approach is mandatory at every stage to ensure that all nonoperative measures have been applied, and that the treatment goals have been clearly defined and agreed with the family. This will involve input from multiple specialities, including allied healthcare professionals, such as physiotherapists and wheelchair services. Surgery should be considered when there is significant impact on the patients' quality of life, which is usually due to poor sitting balance, back or costo-pelvic pain, respiratory complications, or problems with self-care and feeding. Meticulous preoperative assessment is required, along with careful consideration of the nature of the deformity and the problems that it is causing. Surgery can achieve good curve correction and results in high levels of satisfaction from the patients and their caregivers. Modern modular posterior instrumentation systems allow an effective deformity correction. However, the risks of surgery remain high, and involvement of the family at all stages of decision-making is required in order to balance the risks and anticipated gains of the procedure, and to select those patients who can mostly benefit from spinal correction.

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.

Evaluation of parallel endplate osteotomy for severe rigid spinal deformities: a retrospective analysis of 36 cases with a minimum 2-year follow-up.

BACKGROUND: To report on the technique and results of parallel endplate osteotomy (PEO) for severe rigid spinal deformity. METHODS: We retrospectively reviewed the clinical data of 36 patients with severe rigid spinal deformities who underwent PEO between July 2016 and December 2018 and who were followed up for at least 24 months. RESULTS: Following PEO, the kyphosis and scoliosis correction rates reached 77.4 ± 14.0% and 72.2 ± 18.2%, respectively. The median intraoperative estimated blood loss was 1500 mL and the median operative time was 6.8 h. The SF-36 scores of physical function, role-physical, bodily pain, general health, vitality, social function, role-emotional and mental health changed from 62 ± 28, 51 ± 26, 49 ± 29, 35 ± 30, 53 ± 28, 45 ± 30, 32 ± 34 and 54 ± 18 at baseline to 81 ± 16, 66 ± 41, 72 ± 40, 64 ± 44, 75 ± 25, 71 ± 46, 66 ± 34 and 76 ± 28 at 12 months postoperatively, 82 ± 32, 67 ± 42, 81 ± 30, 71 ± 41, 80 ± 30, 74 ± 36, 68 ± 35 and 85 ± 33 at 18 months postoperatively, and 86 ± 21, 83 ± 33, 88 ± 26, 79 ± 39, 86 ± 36, 86 ± 48, 80 ± 47 and 91 ± 39 at 24 months postoperatively, respectively. CONCLUSIONS: PEO is an effective technique for successful correction of spinal deformities. At the two-year follow-up visit, all patients achieved better clinical results based on the SF-36 scores.

The role of femoral obliquity angle and T1 pelvic angle in predicting quality of life after spinal surgery in adult spinal deformities.

BACKGROUND: Adult spinal deformities (ASD) represent a growing clinical condition related to chronic pain, disability and reduction in quality of life (QoL). A strong correlation among spinal alignment, spinopelvic parameters and QoL after spinal fusion surgery in ASD patients was thoroughly investigated over the last decade, However, only few studies focused on the relationship between lumbo-pelvic-femoral parameters - such as Femoral Obliquity Angle (FOA), T1 Pelvic Angle (TPA) and QoL. METHODS: Radiological and clinical data from 43 patients surgically treated with thoracolumbar posterior spinal fusion for ASD between 2015 and 2018 were retrospectively analyzed. The primary outcomes were the correlation between preoperative spino-pelvic-femoral parameters and postoperative clinical, functional outcomes and QoL. Secondary outcomes were: changes in sagittal radiographic parameters spino-pelvic-femoral, clinical and functional outcomes and the rate of complications after surgery. RESULTS: Using Spearman's rank correlation coefficients, spinopelvic femoral parameters (FOA, TPA, pre and post-operative) are directly statistically correlated to the quality of life (ODI, SRS-22, pre and post-operative; > 0,6 strong correlation, p <  0.05). Stratifying the patients according pre preoperative FOA value (High FOA ≥ 10 and Normal/Low FOA <  10), those belonging to the first group showed worse clinical (VAS: 5.2 +/- 1.4 vs 2.9 +/- 0.8) and functional outcomes (ODI: 35.6+/- 6.8 vs 23.2 +/- 6.5) after 2 years of follow-up and a greater number of mechanical complications (57.9% vs 8.3% p <  0.0021). CONCLUSION: Based on our results, preoperative FOA and TPA could be important prognostic parameters for predicting disability and quality of life after spinal surgery in ASD patients and early indicators of possible spinal sagittal malalignment. FOA and TPA, like other and better known spinopelvic parameters, should always be considered when planning corrective surgery in ASD patients.