Progression Of Scoliosis In Patients Research Articles

Active Rheumatoid Arthritis and Scoliosis: A Mid-Term Cohort Study.

Retrospective cohort study. To elucidate the factors related to progression of scoliosis in patients with rheumatoid arthritis (RA) using longitudinal cohort data. Thirty percent of patients with RA have lumbar scoliosis. However, the effectiveness of current treatment methods in preventing the progression of scoliosis is not well-understood due to a lack of longitudinal studies. We enrolled 180 patients with RA who were followed up for over two years, all of whom underwent standing spinal X-rays. These patients were categorized based on their disease activity score-28 with erythrocyte sedimentation rate (DAS28-ESR) into two groups: those in remission (n=76) and those in non-remission (n=104). We evaluated various radiographic measures, including C7 center sacral vertical line (C7-CSVL), pelvic obliquity, major Cobb angle, and curve location. Fifty-three (29.4%) patients presented progression of scoliosis during a mean follow-up period of 4.8 years. Patients in the non-remission showed larger Cobb angle at baseline and final follow-up, compared to those in remission. The annual progression rate of the curve was also greater in the non-remission group (1.04 degree /year), than in the remission group (0.59 degree /year, P=0.001). There was no difference in the incidence of new vertebral fractures. The presence of a baseline cobb angle of 10 degree or more (OR: 3.14; 95% CI: 1.38-7.13; P=0.006), glucocorticoid use (OR: 2.88; 95% CI: 1.18-7.06; P=0.021), and non-remission at baseline (OR: 2.83; 95% CI: 1.25-6.41; P=0.012) were significant risk factors for progression of scoliosis. RA disease activity is linked to progression of scoliosis in patients with RA. Patients with RA who present with an initial scoliosis of 10 degrees or greater, require glucocorticoids for treatment and are in non-remission at baseline may be at high risk for scoliosis progression.

Scoliosis in Spinal Muscular Atrophy Type 1 in the Nusinersen Era.

The introduction of spinal muscular dystrophy (SMA)-modifying therapies, such as antisense oligonucleotide therapy, has changed the natural history of SMA. Most reports on treatment outcomes have focused on motor scores and respiratory function. The objective of this study is to document the development and progression of scoliosis in patients with SMA1 treated with nusinersen. A descriptive single-center study was conducted in patients with SMA1 who were treated with nusinersen before 6 months of age. Data were collected on patients who met criteria, including age at the first nusinersen dose, number of nusinersen doses, degree of scoliosis, respiratory parameters, feeding route, and motor scores at baseline and follow-up. The Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND) was subanalyzed using axial (AxS) and appendicular motor (ApS) scores to evaluate a possible correlation between scoliosis and axial muscle strength. From our cohort, 31 percent (11/35) of patients had a diagnosis of SMA1. Sixty-three percent (7/11) met the inclusion criteria. All patients (7/7) showed initial improvement in their CHOP-INTEND scores in correlation with improvement on the ApS. Despite this, most patients did not show improvement in the AxS. Subsequently, all patients developed scoliosis in the first year of life with Cobb angles that ranged between 18° and 60°. Furthermore, total CHOP-INTEND scores had dropped in 2 patients alongside the development of a Cobb angle of >40°. Despite the significant improvement in functional motor assessment in patients with SMA1, there is a progression of significant scoliosis despite treatment. Subsequently, lack or minimal improvement on the axial CHOP-INTEND scores may predict worsening on the total motor scores.

Progressão da escoliose neuromuscular e opção cirúrgica na encefalopatia crônica não progressiva infantil: Uma revisão de escopo

OBJECTIVE: To carry out a systematic review of the progression of scoliosis in patients with cerebral palsy. METHODS: Systematic review study with searches for journals in the PubMed database from September to December 2020 with specific descriptors associated with the Boolean operator “or” “longitudinal” or “cohort” or “control-case” and “prospective” and “ cerebral palsy” and “scoliosis” or “spine deformity” or “spinal deformity” or “neuromuscular”. Eligibility criteria for inclusion and exclusion of studies were applied, with subsequent data tabulation. RESULTS: A total of 986 articles were found, only seven articles were eligible for desired data extraction after applying eligibility criteria. CONCLUSION: Age and Gross Motor Function Classification System (GMFCS) can be clinical predictors of scoliosis progression. Scoliosis was more progressive in patients with GMFCS III, IV and V. Patients with a smaller Cobb angle during the bone growth period have a lower chance of curve progression.

Predictive Physical Manifestations for Progression of Scoliosis in Marfan Syndrome.

A retrospective study of the prospective cohort. To demonstrate the accurate distribution of the severity of scoliosis in patients with Marfan syndrome, and to identify the predictive physical features for progression of scoliosis in Marfan syndrome. To date, no study has unveiled the risk factors for the progression of scoliosis in Marfan syndrome. We retrospectively obtained data from a prospective cohort of the Marfan syndrome clinic at our institute. We enrolled patients whose whole spine radiographs in the standing position were evaluated at the age of 15 or above, from January 2014 to March 2020. The collected variables were physical manifestations defined as in the systemic score of the revised Ghent nosology. We classified the degree of scoliosis into four categories: "not apparent," "mild" (10° ≤ Cobb < 25°), "moderate" (25° ≤ Cobb < 40°), and "severe" (40° ≤ Cobb or surgery conducted). To identify the risk factors for progression of scoliosis in Marfan syndrome, we conducted univariate and multivariate association analyses between severe scoliosis and each physical manifestation. We identified 131 eligible patients (61 men and 70 women) with a mean age of 31.2 years. Scoliosis with a Cobb angle of ≥10° was identified in 116 patients (88.5%). Moderate scoliosis was identified in 33 patients (25.2%) and severe scoliosis in 53 patients (40.5%). The prevalence of each physical manifestation was equivalent to that reported in previous studies. Multivariate logistic regression analysis revealed that female sex (odds ratio, 3.27) and positive wrist sign (4.45) were predictive factors for progression of scoliosis into severe state in patients with Marfan syndrome. The present study demonstrated the accurate distribution of the severity of scoliosis and identified the predictive factors for progression of scoliosis in patients with Marfan syndrome.Level of Evidence: 3.

Impact of growth hormone treatment on scoliosis development and progression: analysis of 1128 patients with idiopathic short stature.

The purpose of this study was to evaluate the impact of recombinant human growth hormone (rhGH) on the development and progression of scoliosis in patients with idiopathic short stature (ISS). Patients with ISS who underwent rhGH treatment from 1997 to 2017 and were followed up for scoliosis screening with serial radiographic examination were included. For assessing scoliosis development, patients who did not have scoliosis at the time of rhGH treatment were included and followed up to determine whether de novo scoliosis developed during the treatment. For evaluating scoliosis progression, patients who already had scoliosis were analyzed. Univariate and multivariate Cox regression analyses of demographic and radiographic variables were performed to determine the related factors in the development and progression of scoliosis. For assessing scoliosis development, 1093 patients were included. The average duration of rhGH treatment was about 2 years. De novo scoliosis developed in 32 patients (3.7%). The analysis revealed that sex (p=0.016) and chronological age (p=0.048) were statistically significant factors associated with scoliosis development. However, no relationship was observed between scoliosis development and rhGH treatment types or duration. Among 67 patients who already had scoliosis at the time of rhGH treatment, 11 (16.4%) showed scoliosis progression. However, the rhGH types and duration also did not affect scoliosis progression. De novo scoliosis developed in 3.7% and scoliosis progressed in 16.4% of the patients during rhGH treatment. However, scoliosis development or progression was not affected by the types or duration of rhGH treatment in patients with ISS.

The Spine in Patients With Osteogenesis Imperfecta

Osteogenesis imperfecta is a genetic disorder of type I collagen. Although multiple genotypes and phenotypes are associated with osteogenesis imperfecta, approximately 90% of the mutations are in the COL1A1 and COL1A2 genes. Osteogenesis imperfecta is characterized by bone fragility. Patients typically have multiple fractures or limb deformity; however, the spine can also be affected. Spinal manifestations include scoliosis, kyphosis, craniocervical junction abnormalities, and lumbosacral pathology. The incidence of lumbosacral spondylolysis and spondylolisthesis is higher in patients with osteogenesis imperfecta than in the general population. Use of diphosphonates has been found to decrease the rate of progression of scoliosis in patients with osteogenesis imperfecta. A lateral cervical radiograph is recommended in patients with this condition before age 6 years for surveillance of craniocervical junction abnormalities, such as basilar impression. Intraoperative and anesthetic considerations in patients with osteogenesis imperfecta include challenges related to fracture risk, airway management, pulmonary function, and blood loss.

Radiographic analysis of the progression of congenital scoliosis with rib anomalies during the growth period

Retrospective study. To evaluate progression of various types of congenital scoliosis (CS) with rib anomalies (RA) during the various stages of the growth period, and to assess severity of progression in order to make a strategic planning of expansion thoracoplasty. VEPTR is approved for the treatment of patients with TIS in more than 30 countries. However, there is no consensus on the surgical indications, or the age or time when VEPTR surgery should be performed. Furthermore, there is no study related to natural history of congenital scoliosis with rib anomalies except two reports that are not sufficient to indicate risk factors of progression during the growth period. Based on a survey of CS and RA via questionnaires, 70 patients (32 males and 38 females with an average age of 2.6 years at the first visit.) matched the inclusion criteria: CS with RA, no procedures that could influence natural history during follow-up periods, repeated plain X-ray check-ups with at least two years interval during growth periods. Average follow-up (F/U) time was 5.4 years (2–14). Plain X-ray images of 70 patients were divided into three age groups: infantile (0–5,6), juvenile (5–10,11), and adolescent (11,12-). Each X-ray image was evaluated in terms of laterality, range and type of RA, severity of scoliosis, type of CS, thoracic height ratio, SAL, and associated anomalies. 54 of the 70 patients had unilateral rib anomalies. Rib anomalies included rib fusion in 52, mixed type (fusion and defect) in 8, rib proximity in 6, and rib defect in 4. Vertebral anomalies included formation failure in 1, segmentation failure in 16 and mixed type in 53. The magnitude of scoliosis was 46.9° at the first visit and 65.7° at the final F/U. Scoliosis progressed at the rate of 4.6°/year in 70, 3.6°/year in bilateral RA involvement and 4.9°/year in unilateral. Scoliosis progressed with the rate of 4.6°/year in 70, 3.6°/year in bilateral RA involvement and 4.9°/year in unilateral. Scoliosis progressed most severely during infantile period with the rate of 5.0°/year, followed by adolescent of 3.8°/year and juvenile of 2.3°/year. Patients with rib defect or unilateral unsegmented bar showed higher progression rates (10.7°/year and 7.0°/year) during infantile period. According to the relationship between SAL and scoliosis, four grades in severity of progression (most severe, severe, moderate, mild) were set up with the cut-off value of 70%, 85% of SAL and 45°, 85° of scoliosis for making the strategic planning of ET. Those grades were significantly related with types and location of RA and types of vertebral anomalies. Progression of scoliosis was analysed in 70 patients with CS & RA. Congenital scoliosis with rib anomalies progressed most rapidly during the early infantile period (7.8°/year), followed by the late infantile period (5.0°/year), and the adolescent period (3.8°/year). Progression of scoliosis in patients with CS and RA occurred in early phase of growth periods and significantly related with types and location of RA as well as type of vertebral anomalies. The results of this study surely suggest the timing of ET for the patients with CS and RA.

The Impact of Intrathecal Baclofen on the Natural History of Scoliosis in Cerebral Palsy

Intrathecal baclofen (ITB) is an effective treatment of spasticity in patients with cerebral palsy. However, several recent reports have raised concerns that the treatment may be associated with a rapid progression of scoliosis. The objective of this study was to further examine the effect of ITB treatment on the progression of scoliosis in patients with cerebral palsy. Spastic cerebral palsy patients who were ITB candidates were followed radiographically. Baseline Cobb angles of the primary curve were measured during the period of ITB pump insertion and at the most recent follow-up visit. Each patient was matched with a control patient by the diagnosis of cerebral palsy, age, sex, topographic involvement, and initial Cobb angle. The mean rate of change in Cobb angle was compared between ITB and control patients using paired t test. A multiple linear regression model was used to examine the difference, controlling for age, sex, topographic involvement, and initial Cobb angle. Fifty ITB patients and 50 controls were included in the analysis. There was no statistically significant difference between the mean change in Cobb angle in ITB patients (6.6 degrees per year) compared with the matched control patients (5.0 degrees per year, P = 0.39). The results from the multiple regression analysis also failed to show a statistically significant difference (0.92 degrees per year difference between ITB patients and controls, P = 0.56). The progression of scoliosis in cerebral palsy patients with ITB treatment is not significantly different from those without ITB treatment. The findings suggest that patients receiving ITB experience a natural progression of scoliosis similar to the natural history reported in the literature. Level III.

Progression of Scoliosis in Patients With Spastic Quadriplegia After the Insertion of an Intrathecal Baclofen Pump

Medical and radiographic review of 19 consecutive patients with spastic quadriplegia before and after intrathecal baclofen pump insertion with special attention paid to progression of scoliosis. Many orthopedic surgeons who treat spastic quadriplegic patients have noticed a trend of marked scoliosis progression after the administration of intrathecal baclofen (ITB) via subcutaneous pump and catheter. The purpose of this study is to quantify scoliosis progression in this patient population before and after baclofen administration and compare this to published natural history data. The authors had noted rapid progression of scoliosis in spastic quadriplegic patients after intrathecal baclofen pump insertion. This had been noted at other centers, but no significant statistical analysis had been done comparing prepump to postpump scoliosis progression in these patients. To document the magnitude and rate of scoliosis progressions after the placement of an ITB pump, the charts and radiographs of 19 consecutive nonambulatory patients with spastic quadriplegia and an ITB pump were reviewed. To document the rate of scoliosis progression, each patient had at least 2 pre and 2 postpump insertion spinal radiographs made. All radiographs were made with the patients in the supine position without orthoses. A board-certified orthopedic surgeon reviewed these radiographs. Skeletal maturity was assessed using Risser grading. Catheter tip location and rate of baclofen administration were recorded. For 19 patients with complete radiographic data, average Cobb angles were 10.2 degrees before pump insertion and 25 degrees at an average of 20.9 months after pump insertion (P < 0.0001). These 19 patients had a mean rate of change in their Cobb angles of 1.825%/year before pump insertion and 10.95 degrees /year at an average of 23.9 months after pump insertion (P = 0.024). These results represent a 6-fold increase in the curve progression rate after pump insertion. There was no association between catheter tip location or rate of Baclofen infusion on curve progression. In published data, the rate of progression of scoliosis in skeletally immature nonambulatory patients with cerebral palsy was 4.5 degrees /year. In this study, the average rate of progression of the scoliosis for the immature was 9.02 degrees /year. For the skeletally mature bed-ridden patients, the worst-case natural history progression was 4.4 degrees /year. The comparable rate of change in skeletally mature (Risser 5) nonambulatory patients (n = 6) in this study was 28.4 degrees /year. This study demonstrates a significant increase in the rate of scoliotic curve progression after ITB pump placement when compared with published natural history data. The evidence of the beneficial effects of ITB on spasticity has been confirmed, and as larger, prospective randomized studies are conducted, the authors think that support for continued use of this treatment will increase. However, early bracing and spinal fusion may be warranted to prevent significant increases in spinal deformity if scoliosis is anticipated to progress more than 10 degrees /yr for patients with spastic quadriplegia and ITB pump. The authors are now performing spinal fusions for curves that exceed 40 degrees to 50 degrees in the presence of an ITB pump as recommended by previous reviews of scoliosis and accompanying quadriplegia.

The Risk of Progression of Scoliosis in Cerebral Palsy Patients After Intrathecal Baclofen Therapy

Retrospective radiographic and medical chart review with matched control group. To identify the effect of intrathecal baclofen on the incidence of scoliosis, rate of curve progression, and pelvic obliquity compared with a matched cohort. Although intrathecal baclofen therapy (ITB) has been shown to be effective in decreasing spasticity, case reports have described some children receiving ITB in whom progressive scoliosis was noted; other authors have described no effect on the spinal column. A controlled study has not been performed. All patients with spastic CP treated with ITB between 1997 and 2003 at a single institution were reviewed. A total of 107 patients undergoing ITB for a minimum of 2 years were identified, of which 26 patients subsequently developed or had progression of scoliosis. Twenty-five age, gender, and gross motor function classification system (GMFCS) score-matched quadriplegic CP patients with scoliosis who did not receive ITB constituted the control group used to compare the rate of curve progression and pelvic obliquity. The average curve progression for the baclofen group after pump implantation was 16.3 degrees per year; and for the control group was 16.1 degrees per year. Both groups' curves progressed over time during growth (P = 0.001), but baclofen did not have an independent effect on curve progression (P = 0.181). Average pelvic obliquity for the 2 groups increased over time (P = 0.001), but there was no difference between the groups (P = 0.536). Twelve of 57 patients (21%) developed scoliosis after pump implantation during a mean of 3.6 years of follow-up. Thirty of 92 matched control patients (32%) not treated with ITB within the same time interval had scoliosis by maturity. This study demonstrates that ITB has no significant effect on curve progression, pelvic obliquity, or the incidence of scoliosis when compared with an age, gender, and GMFCS score-matched control group of patients with spastic CP without ITB.

Correlation of Scoliotic Curvature With Z-Score Bone Mineral Density and Body Mass Index in Patients With Osteogenesis Imperfecta

A cross-sectional radiographic and clinical study of patients with osteogenesis imperfecta (OI). To determine demographic, anthropometric, and radiographic parameters that correlate with development of scoliosis in patients with OI. Despite the relatively high prevalence of scoliosis in patients with OI and its clinical significance, little is known about demographic and anthropometric parameters that correlate with the development of scoliosis. Clinical records and spinal radiographs of 19 OI patients were reviewed. There were 5 male and 14 female patients with an average age of 14.2 years (range, 4-20 years). Seven patients were Sillence Type I and 12 patients were Type III. The Cobb angle of scoliosis was analyzed for correlations with age, body mass index (BMI), physical capability, leg-length discrepancy, Z-score bone mineral density (BMD) in the lumbar spine, thoracic kyphosis angle, and lumbar lordosis angle by using Pearson's correlation method. A probability of less than 0.05 was considered statistically significant. The average Cobb angle of scoliosis was 25.2 degrees (range, 5 degrees-108 degrees) including 6 patients with an angle of > or = 30 degrees. Five of these 6 patients were Sillence Type III. Statistical analysis showed a significant positive correlation between the extent of scoliosis and BMI, as well as leg-length discrepancy. There was an inverse correlation between the extent of scoliotic curvature and the Z-score BMD as well as the thoracic kyphosis angle. The correlation of scoliosis with the Z-score BMD and BMI supports the pathology of scoliosis based on vertebral fragility. Proper management of BMD and BMI may have therapeutic value in delaying the progression of scoliosis in patients with osteogenesis imperfecta.

Potential Complications of Posterior Spine Fusion and Instrumentation in Patients With Cerebral Palsy Treated With Intrathecal Baclofen Infusion

The authors evaluated a case series of 5 patients with cerebral palsy and a previously placed subcutaneous reservoir for continuous intrathecal baclofen infusion to control spasticity who underwent spinal arthrodesis with posterior instrumentation for progressive neuromuscular scoliosis deformities. To present our preliminary experience and complications with posterior spine fusion in patients with cerebral palsy and continuous intrathecal baclofen infusion and provide a literature review. Numerous studies have reported complications associated with intrathecal baclofen infusion, many of these catheter-related. Few reports address complications associated with the intrathecal baclofen infusion, including progressive spinal deformities and complications following spinal arthrodesis. Five patients with spastic quadriplegia cerebral palsy had spine fusions for progressive neuromuscular scoliosis. The mean age at surgery was 14.5 years. The mean preoperative major curve was 73 degrees and mean pelvic obliquity was 19 degrees . The patients' medical records were reviewed for complications following reinsertion of the intrathecal catheter following posterior spine instrumentation and arthrodesis, and for progression of the neuromuscular scoliosis, before and after intrathecal baclofen infusion was initiated. Two patients presented with low-pressure headaches in the postoperative period, resulting from cerebrospinal fluid leak following subarachnoid catheter reinsertion and posterior instrumentation. Four patients documented progression of the major scoliosis curve after intrathecal baclofen infusion was begun. A mean progression of 44 degrees occurred over a mean period of 11 months before the spinal arthrodesis. Low pressure headaches resulting from a cerebrospinal fluid leak following catheter reinsertion may occur in the postoperative period. Preoperative concerns with the baclofen pump reservoir placed subcutaneously and pressure sores were not seen with careful prone positioning on a 4-poster frame. Progression of scoliosis in patients with cerebral palsy requiring spinal arthrodesis was demonstrated in 4 of the patients after continuous intrathecal baclofen was started. The progression of the spinal deformity as a consequence of growth, natural history, or the intrathecal baclofen infusion is unknown at the present time.