Late-onset Idiopathic Scoliosis Research Articles

Cytoskeletal Keratins Are Overexpressed in a Zebrafish Model of Idiopathic Scoliosis.

Idiopathic scoliosis (IS) is a three-dimensional rotation of the spine >10 degrees with an unknown etiology. Our laboratory established a late-onset IS model in zebrafish (Danio rerio) containing a deletion in kif7. A total of 25% of kif7co63/co63 zebrafish develop spinal curvatures and are otherwise developmentally normal, although the molecular mechanisms underlying the scoliosis are unknown. To define transcripts associated with scoliosis in this model, we performed bulk mRNA sequencing on 6 weeks past fertilization (wpf) kif7co63/co63 zebrafish with and without scoliosis. Additionally, we sequenced kif7co63/co63, kif7co63/+, and AB zebrafish (n = 3 per genotype). Sequencing reads were aligned to the GRCz11 genome and FPKM values were calculated. Differences between groups were calculated for each transcript by the t-test. Principal component analysis showed that transcriptomes clustered by sample age and genotype. kif7 mRNA was mildly reduced in both homozygous and heterozygous zebrafish compared to AB. Sonic hedgehog target genes were upregulated in kif7co63/co63 zebrafish over AB, but no difference was detected between scoliotic and non-scoliotic mutants. The top upregulated genes in scoliotic zebrafish were cytoskeletal keratins. Pankeratin staining of 6 wpf scoliotic and non-scoliotic kif7co63/co63 zebrafish showed increased keratin levels within the zebrafish musculature and intervertebral disc (IVD). Keratins are major components of the embryonic notochord, and aberrant keratin expression has been associated with intervertebral disc degeneration (IVDD) in both zebrafish and humans. The role of increased keratin accumulation as a molecular mechanism associated with the onset of scoliosis warrants further study.

Positive Association between TGFB1 Gene and Susceptibility to Idiopathic Scoliosis in Bulgarian Population.

Idiopathic scoliosis (IS) is a common medical condition beginning in childhood and characterized by strong evidence for a genetic susceptibility to three-dimensional spinal deformity. The primary goal of the current case-control study is to examine the association between the TGFB1 (-509C/T) functional polymorphic variant and genetic predisposition to IS in the Bulgarian population and the genotype-phenotype correlations in distinct case-control subgroups based on age at onset, family history, and gender. A total of 127 patients with primary scoliosis and 254 gender-matched control subjects were recruited. The mean Cobb angle was 53.8 ± 21.2°. Genotyping of cases and controls was performed using the TaqMan real-time amplification technique. The results were processed statistically using Pearson's Chi-squared test and Fisher's exact test with a value of p less than 0.05 as statistically significant. The polymorphic T allele and TT genotype were associated with a greater incidence of IS and can be considered as predisposing factors with a moderate effect on deformity development. The current results suggested that there was a genetic predisposition in early and late onset IS and familial, sporadic, and female cases. Nevertheless, replication studies are needed to reveal the relationship between the TGFB1 locus and certain subtypes of IS in different populations.

Management of Spinal Deformities and Evidence of Treatment Effectiveness.

Introduction:The review evaluates the up-to-date evidence for the treatment of spinal deformities, including scoliosis and hyperkyphosis in adolescents and adults.Material and Methods:The PubMed database was searched for review articles, prospective controlled trials and randomized controlled trials related to the treatment of spinal deformities. Articles on syndromic scoliosis were excluded and so were the articles on hyperkyphosis of the spine with causes other than Scheuermann’s disease and osteoporosis. Articles on conservative and surgical treatments of idiopathic scoliosis, adult scoliosis and hyperkyphosis were also included. For retrospective papers, only studies with a follow up period exceeding 10 years were included.Results:The review showed that early-onset idiopathic scoliosis has a worse outcome than late-onset idiopathic scoliosis, which is rather benign. Patients with AIS function well as adults; they have no more health problems when compared to patients without scoliosis, other than a slight increase in back pain and aesthetic concern. Conservative treatment of adolescent idiopathic scoliosis (AIS) using physiotherapeutic scoliosis-specific exercises (PSSE), specifically PSSR and rigid bracing was supported by level I evidence. Yet to date, there is no high quality evidence (RCT`s) demonstrating that surgical treatment is superior to conservative treatment for the management of AIS. For adult scoliosis, there are only a few studies on the effectiveness of PSSEs and a conclusion cannot as yet be drawn.For hyperkyphosis, there is no high-quality evidence for physiotherapy, bracing or surgery for the treatment of adolescents and adults. However, bracing has been found to reduce thoracic hyperkyphosis, ranging from 55 to 80° in adolescents. In patients over the age of 60, bracing improves the balance score, and reduces spinal deformity and pain. Surgery is indicated in adolescents and adults in the presence of progression of kyphosis, refractory pain and loss of balance.Discussion:The available evidence reviewed has suggested that different approaches are needed towards the management of different spinal deformities. Specific exercises should be prescribed in children and adolescents with a Cobb angle in excess of 15°. In progressive curves, they should be used in conjunction with bracing. Clarity regarding differences and similarities is given as to what makes PSSE and PSSR specific exercises. As AIS is relatively benign in nature, conservative treatment should be tried when the curve is at a surgical threshold, before surgery is considered. Similarly, bracing and exercises should be prescribed for patients with hyperkyphosis, particularly when the lumbar spine is afflicted. Surgery should be considered only when the symptoms cannot be managed conservatively.Conclusion:There is at present high quality evidence in support of the conservative treatment of AIS. The current evidence supports the use of PSSE, especially those using PSSR, together with bracing in the treatment of AIS. In view of the lack of medical consequences in adults with AIS, conservative treatment should be considered for curves exceeding the formerly assumed range of conservative indications.There is, however a lack of evidence in support of any treatment of choice for hyperkyphosis in adolescents and spinal deformities in adults. Yet, conservative treatment should be considered first. Yet to date, there is no high quality evidence (RCT`s) demonstrating that surgical treatment is superior to conservative treatment for the management of AIS and hyperkyphosis. Additionally, surgery needs to be considered with caution, as it is associated with a number of long-term complications.

Radiological Outcome of Cotrel-Dubousset Instrumentation in Nineteen Patients with Adolescent Idiopathic Scoliosis

Objective: Adolescent idiopathic scoliosis is a progressive type of scoliosis that may lead to permanent deformity unless prevented and treated effectively. To present radiological outcome of patients with adolescent idiopathic scoliosis treated with the Cotrel-Dubousset (CD) instrumentation in our clinic. Methods: This was a prospective follow-up study of 19 patients (8 males, 11 females; mean age 18.5 years; age range 12-43 years) who underwent CD instrumentation for late onset idiopathic scoliosis. The CD instrumentation and posterior spinal fusion was performed using the standard technique through either anterior or posterior approach. On average, 13.52 vertebrae (range, 8-16) were included in the spinal fusion. The mean postoperative follow-up duration was 18 months (2-32 months). Results: Cobb angle on frontal plane was corrected 47.74%±21.73% at thoracic region and 34.52%±15.96% at lumbar region. On sagittal plane, the percentage of correction was 28.61%±20.91% on thoracic kyphosis angle and 38.96%±29.73% lumbar lordosis angle. In general, physiological sagittal contour of spine was obtained in 49.2% of patients after CD instrumentation. The most common postoperative complications were hook dislocation (n=8), bending and dislocation of screw (n=7), broken lamina and pedicule (n=5), and infection (n=3), all of which were effectively treated. Conclusio n: CD instrumentation effectively corrects the late onset idiopathic scoliosis if it is performed after a proper preoperative planning.

Scoliosis bracing and exercise for pain management in adults-a case report.

[Purpose] For adult patients with late-onset idiopathic scoliosis, cosmetic concerns and pain are the main reasons for seeking treatment at a physician’s office. The purpose of this paper was to describe the mid-term effect of physical rehabilitation and part-time bracing on an adult scoliosis patient who had been suffering from chronic low back pain for fourteen years. [Subject and Methods] Case description: A 37-year-old female patient with late-onset idiopathic scoliosis presented in the office of the first author in January 2014. She reported having chronic pain (low back pain) since the age of 23 and reported daily pain at a level of 5–7 on average on a Visual Analogue Scale of 0 to 10. She received a short scoliosis-specific Schroth exercise program and was also fitted with a Gensingen brace for part-time wear. [Results] At a 16 month follow-up, the patient no longer suffered from daily low back pain (with heavy lifting only) and was fully active. Additionally, her lumbar Cobb angle and angle of trunk rotation improved. [Conclusion] Patients with late-onset idiopathic scoliosis and pain may benefit from a pattern-specific conservative treatment approach. In this population, surgical intervention should be regarded as the last resort, since there are many long-term unknowns with surgery.

Expert's comment concerning Grand Rounds case entitled "The management of high-grade spondylolisthesis and co-existent late-onset idiopathic scoliosis" (Abhishek Srivastava, Edward Bayley, Bronek M. Boszczyk).

Expert's comment concerning Grand Rounds case entitled "The management of high-grade spondylolisthesis and co-existent late-onset idiopathic scoliosis" (Abhishek Srivastava, Edward Bayley, Bronek M. Boszczyk).

Adolescent Idiopathic Scoliosis: An in Depth Analysis and Historical Review

Adolescent idiopathic Scoliosis (AIS) is a common disorder of children about which there is a vast amount of research available for review. This manuscript aims to discuss significant classic and modern literature regarding this spinal deformity. An organized review of topics including the definition, epidemiology, etiology, physiology, diagnosis, and management of adolescent idiopathic scoliosis has been included herein. In this review, it is first explained that AIS now falls under the definition of late-onset idiopathic scoliosis. Epidemiologic discussion then reveals the relatively high prevalence of this disorder in children and lists the variations seen in prevalence by gender. Etiologic review explores the multifactorial nature of AIS and describes various conditions implicated in AIS pathogenesis including abnormalities in connective tissue structure, neuro-osseous development, platelet chemistry and hormone levels. The discussion of physiology explains the three dimensional structure of the AIS deformity and reviews long term complications such as back pain and compromised self-image. The section covering diagnostic methods highlights the physical exam for AIS and details important radiographic measurements including the Cobb angle, clavicle angle, and sagittal balance. Finally, the review of management emphasizes the importance of individualized therapy for AIS and discusses techniques involved in the non-operative and operative treatment of this spinal deformity.

Study of the rib cage deformity in children with 10°-20° of Cobb angle Late Onset Idiopathic Scoliosis, using Rib-Vertebra Angles Aetiologic implications

The aim of the study is to compare the rib-vertebra angles (RVAs) between children with 10 degrees - 20 degrees of Cobb angle late onset idiopathic scoliosis (LOIS) and non-scoliotic children. The RVAs of 47 children, with mean age 12.4 years, who presented LOIS with a Cobb angle 10 degrees - 20 degrees, were studied. The children were classified into three groups according to the site of the scoliotic curve: 17 children had thoracic (T), 14 children had thoracolumbar (TL) and 16 children had lumbar (L) curves. The RVAs of the scoliotic children were compared to the RVAs of 60 non-scoliotic children of a similar age group, who were studied in the past. The comparison of the right and left RVAs within each group showed that the children who had: T curves differ at the level T4, T5, T6, T7 and T8, TL curves differ at the level T3, and L curves differ at the level T7 and T12. The comparison of the ipsilateral RVA's between the scoliotic groups showed that between: T and TL curves there are no differences at any thoracic level, between T and L curves the RVAs differ at the T7 level on the right side, whereas there are no differences between the RVAs on the left side, between TL and L curves the RVAs differ at the level T5, T6, and T7 on the right and at the level T5 on the left side. Comparing the RVAs between the scoliotic and nonscoliotic children, it was apparent that the scoliotic children rib cage had lower RVAs (p<0.01) at almost all thoracic levels. It has been reported that RVAs is an expression of the resultant muscle forces, which act on each rib. It was also suggested that RVA asymmetries by weakening the spinal rotation-defending system are aetiological for idiopathic scoliosis, (Burwell et al 1992). This study shows that scoliotic children with small curves have underdeveloped thoracic cage compared to nonscoliotic counterparts. The differences are more apparent in the scoliotic children with thoracic curves. It is suggested that the differences of the RVAs between right and left side in this group are an expression of asymmetric muscle forces acting on the thoracic cage. It is concluded that asymmetric muscle forces participate in the pathogenesis of idiopathic scoliosis on the thoracic cage, which deforms early.

Differential diagnosis of back pain in adult scoliosis (non operated patients)

The relationship between back pain and scoliosis is not clear. Old long-term follow-up studies reported no greater incidence or degree of back pain in adult scoliosis than in normal population when matched for age and sex. At the end of the seventies studies were contradictories. While Fowles et al (1978) concluded that untreated scoliosis could be considered as a disabling disease in the adult particularly after the age of 30, Nachemson (1979) reported a minimal risk of disabling back pain in adult patients with lumbar curves stating that ‘whether severe low back pain occurs more often in adults who have lumbar curves than in those whose spines are straight is open to question’. Later, in 1981, Weinstein et al found that backache was more common in scoliosis patients than in the general population with no relation between back pain and the presence of osteoarthritic changes. Kostuik and Ventivoglio (1981) reported a similar incidence of low back pain (59%) in subjects with lumbar or thoracolumbar curves and normal population. However, they found a correlation between back pain and severity of the curve especially for curves of more than 45°, while patients with no pain tended to have smaller curves. Jackson et al (1983) confirmed that incidence was comparable between adult scoliosis and general population but severity was greater in scoliotic patients. They found that patients with lumbar curve had more pain. Compensatory lumbosacral curves were most painful and disabling. The controversy about how well or bad scoliosis population do it (pain and function) during adult life still continued during the nineties and while the results from the Ste-Justine Adolescent Idiopathic Scoliosis Cohort Study suggested that back pain is responsible for a considerable amount of disability and handicap in later life, the revisited sample from Weinstein et al, showed that untreated late onset idiopathic scoliosis causes little physical impairment other than back pain and cosmetic concerns, with these patients being productive and functional at high level at 50-year follow up. In agreement with the Ste-Justine’s study, Schwab F et al (2003) studied the effect of scoliosis in the self-perception of health and showed that patients in this study averaged scores (SF-36) much lower than the norm from both the general US population in all 8 categories and the US population for ages 55-64 in 7 out of 8 categories of the SF-36 questionnaire. Whether or not idiopathic scoliosis developed during growth will alter health related quality of life later and how conservative and/or surgical treatment will change its natural history is something needing further research, however, from a practical point of view, here the question is not that but how scoliosis condition affects the diagnose, prognosis and management of adult patients suffering from chronic back pain. Adult patients attending our institution - a scoliosisspecific rehabilitation centre - could be divided in two main groups: Group I: Patients attending the clinic with a clear self-conscience of belonging to the scoliosis population because they were mostly diagnosed during childhood or during adolescence – treated or not treated-; Group II: Patients belonging to the back pain population referred to us by their doctors, mostly because they showed a bad response to general rehabilitation and such a bad response was related to a non previously diagnosed scoliosis condition. Generally speaking we could identify patients with idiopathic, congenital and secondary scoliosis in Group I while most of the patients in Group II have developed de novo degenerative scoliosis or scoliosis secondary to any pelvic or lower limbs biomechanical disturbance. The main reasons for consultation in Group I are: documented curve progression, cosmesis, chronic back pain and disability, and prevention in patients that have been followed-up

Traction Radiography Performed under General Anesthetic: A New Technique for Assessing Idiopathic Scoliosis Curves

A prospective review of 24 patients with late-onset idiopathic scoliosis. OBJECTIVES.: To compare curve flexibility measured using supine bending radiography and traction radiography; to examine the correlation of each technique with postoperative correction; and to determine the influence of each technique on the decision to perform concomitant anterior release surgery with posterior instrumentation. Assessment of curve flexibility is important in decision making before surgical correction of scoliosis. Supine bending radiographs are presently the gold standard technique by which flexibility is assessed, but their reliability has been questioned. No literature has shown a conclusively superior role for traction radiography in assessing idiopathic scoliosis curves. Each patient had erect anteroposterior radiographs and supine bending radiographs. On the day of surgery, traction radiography was performed under general anesthetic. The correction obtained in the Cobb angle between the bending and traction radiographs was compared. The influence of the traction radiography on the decision for anterior release surgery and its correlation with postoperative result was examined. Traction radiography demonstrated significantly greater curve flexibility than supine bending radiographs (P < 0.001). Eleven of 13 patients planned for anterior release surgery and posterior instrumentation avoided anterior release after review of the traction radiography. No significant difference was demonstrated between the traction radiography and postoperative correction (P = 0.13). Traction radiography is superior to supine bending radiography in assessing curve mobility before surgery. This method benefits patients by allowing them to avoid anterior release surgery and helps predict postoperative correction.

The Natural History of Untreated Adolescent Idiopathic Scoliosis

Orthopedics| May 01 2003 The Natural History of Untreated Adolescent Idiopathic Scoliosis AAP Grand Rounds (2003) 9 (5): 57–58. https://doi.org/10.1542/gr.9-5-57 Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Peer Review Share Icon Share Twitter LinkedIn Tools Icon Tools Get Permissions Cite Icon Cite Search Site Citation The Natural History of Untreated Adolescent Idiopathic Scoliosis. AAP Grand Rounds May 2003; 9 (5): 57–58. https://doi.org/10.1542/gr.9-5-57 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search nav search search input Search input auto suggest search filter All PublicationsAll JournalsAAP Grand RoundsPediatricsHospital PediatricsPediatrics In ReviewNeoReviewsAAP NewsAll AAP Sites Search Advanced Search Topics: adolescent idiopathic scoliosis, idiopathic scoliosis Source: Weinstein SL, Dolan LA, Spratt KF, et al. Health and function of patients with untreated idiopathic scoliosis. JAMA. 2003;289:559–567. The authors from the Universities of Iowa and Southern California report a 50-year prospective study of a cohort of untreated patients with late-onset idiopathic scoliosis (LIS), defined as a structural lateral spinal curvature occurring during puberty in otherwise healthy adolescents. The diagnosis of LIS was made at the University of Iowa between 1932 and 1948. One hundred seventeen of 314 eligible patients comprised the study group. Their mean age was 66 years. Follow-up studies of this cohort were reported at 30 and 40 years. For this study, 62 age- and sex-matched controls were included. Outcome measures were mortality, pulmonary symptoms, general function, depression, and body image. Data from the 30- and 40-year follow-up studies were used for longitudinal comparisons. Radiographic and physical examinations were performed.... You do not currently have access to this content.

Health and function of patients with untreated idiopathic scoliosis: a 50-year natural history study.

Previous long-term studies of idiopathic scoliosis have included patients with other etiologies, leading to the erroneous conclusion that all types of idiopathic scoliosis inevitably end in disability. Late-onset idiopathic scoliosis (LIS) is a distinct entity with a unique natural history. To present the outcomes related to health and function in untreated patients with LIS. Prospective natural history study performed at a midwestern university with outpatient evaluation of patients who presented between 1932 and 1948. At 50-year follow-up, which began in 1992, 117 untreated patients were compared with 62 age- and sex-matched volunteers. The patients' mean age was 66 years (range, 54-80 years). Mortality, back pain, pulmonary symptoms, general function, depression, and body image. The estimated probability of survival was approximately 0.55 (95% confidence interval [CI], 0.47-0.63) compared with 0.57 expected for the general population. There was no significant difference in the demographic characteristics of the 2 groups. Twenty-two (22%) of 98 patients complained of shortness of breath during everyday activities compared with 8 (15%) of 53 controls. An increased risk of shortness of breath was also associated with the combination of a Cobb angle greater than 80 degrees and a thoracic apex (adjusted odds ratio, 9.75; 95% CI, 1.15-82.98). Sixty-six (61%) of 109 patients reported chronic back pain compared with 22 (35%) of 62 controls (P =.003). However, of those with pain, 48 (68%) of 71 patients and 12 (71%) of 17 controls reported only little or moderate back pain. Untreated adults with LIS are productive and functional at a high level at 50-year follow-up. Untreated LIS causes little physical impairment other than back pain and cosmetic concerns.

Late-onset idiopathic scoliosis in children six to fourteen years old. A cross-sectional prevalence study.

We performed a point-prevalence survey of 15,799 children, six to fourteen years old, who formed part of a prospective longitudinal study. Our purpose was to detect the prevalence of scoliosis and to investigate associated factors. On the basis of the initial screening, 934 children (5.9 per cent) were referred for additional clinical and radiographic examinations; 896 children returned for this second evaluation. A lateral spinal curve with a Cobb angle of more than 5 degrees was seen in 431 children (2.7 per cent of the 15,799 children). Only seventy-six children (0.5 per cent) had a curve that met our definition of idiopathic scoliosis (a curve of more than 10 degrees with concordant apical rotation). The point-prevalence rate was higher in girls, and it increased with age. The rate was 0.1 per cent (four of 5246) in the age-group of six to eight years, 0.3 per cent (sixteen of 5831) in the age-group of nine to eleven years, and 1.2 per cent (fifty-six of 4722) in the age-group of twelve to fourteen years old. With allowance for the fact that different definitions of idiopathic scoliosis have been used in earlier studies, our results suggest that the natural history of idiopathic scoliosis may be becoming more benign spontaneously.

Scoliosis in children and adolescents

Scoliosis is a curvature in the frontal plane of the spine (Fig. 1). The associated spinal rotation causes a hump on the child’s back or a prominence of one hip. The deformity of the trunk, which is threedimensional, develops usually from unknown cause and almost exclusively in children (idiopathic infantile, juvenile and adolescent scoliosis). A child may develop a scoliosis because of a congenital defect in the spine (congenital scoliosis) or from disorder of the nervous system, muscles, ligaments or bones (secondary scoliosis). In Britain, we now separate early onset from late onset idiopathic scoliosis by the child’s fifth birthday. This is mainly because young scoliotics are more vulnerable to abnormal lung development and eventual cardiorespiratory failure.3

Surgical treatment of late-onset idiopathic thoracic scoliosis. The Leeds procedure.

Late-onset idiopathic scoliosis is associated with a rib hump in the thoracic region, and surgery is indicated when this deformity becomes unacceptable. Fifty patients with this deformity were treated by the Leeds procedure, which consists of segmental wiring to a kyphotically-contoured square-ended Harrington rod; this procedure not only derotates the spine but restores the natural thoracic kyphosis, thus avoiding subsequent buckling. All patients were followed up for a minimum of two years. Forty-two of these, who had a pre-operative Cobb angle of less than 60 degrees, were treated by one-stage instrumentation and fusion, while the remaining eight with greater curves underwent preliminary anterior multiple discectomy to provide flexibility with shortening. Postoperative loss of correction was not observed and there were no neurological complications.