Matthew B. Dobbs

Effects of Bracing in Adolescents with Idiopathic Scoliosis

BACKGROUND The role of bracing in patients with adolescent idiopathic scoliosis who are at risk for curve progression and eventual surgery is controversial. METHODS We conducted a multicenter study that included patients with typical indications for bracing due to their age, skeletal immaturity, and degree of scoliosis. Both a randomized cohort and a preference cohort were enrolled. Of 242 patients included in the analysis, 116 were randomly assigned to bracing or observation, and 126 chose between bracing and observation. Patients in the bracing group were instructed to wear the brace at least 18 hours per day. The primary outcomes were curve progression to 50 degrees or more (treatment failure) and skeletal maturity without this degree of curve progression (treatment success). RESULTS The trial was stopped early owing to the efficacy of bracing. In an analysis that included both the randomized and preference cohorts, the rate of treatment success was 72% after bracing, as compared with 48% after observation (propensity-score-adjusted odds ratio for treatment success, 1.93; 95% confidence interval [CI], 1.08 to 3.46). In the intention-to-treat analysis, the rate of treatment success was 75% among patients randomly assigned to bracing, as compared with 42% among those randomly assigned to observation (odds ratio, 4.11; 95% CI, 1.85 to 9.16). There was a significant positive association between hours of brace wear and rate of treatment success (P<0.001). CONCLUSIONS Bracing significantly decreased the progression of high-risk curves to the threshold for surgery in patients with adolescent idiopathic scoliosis. The benefit increased with longer hours of brace wear. (Funded by the National Institute of Arthritis and Musculoskeletal and Skin Diseases and others; BRAIST ClinicalTrials.gov number, NCT00448448.).

Factors Predictive of Outcome After Use of the Ponseti Method for the Treatment of Idiopathic Clubfeet

BACKGROUND The nonoperative technique for the treatment of idiopathic congenital talipes equinovarus (clubfoot) described by Ponseti is a popular method, but it requires two to four years of orthotic management. The purpose of this study was to examine the patient characteristics and demographic factors related to the family that are predictive of recurrent foot deformities in patients treated with this method. METHODS The cases of fifty-one consecutive infants with eighty-six idiopathic clubfeet treated with use of the Ponseti method were examined retrospectively. The patient characteristics at the time of presentation, such as the severity of the initial clubfoot deformity, previous treatment, and the age at the initiation of treatment, were examined with use of univariate logistic regression analysis modeling recurrence. Demographic data on the family, including annual income, highest level of education attained by the parents, and marital status, as well as parental reports of compliance with the use of the prescribed orthosis, were studied in relation to the risk of recurrence. RESULTS The parents of twenty-one patients did not comply with the use of orthotics. Noncompliance was the factor most related to the risk of recurrence, with an odds ratio of 183 (p < 0.00001). Parental educational level (high-school education or less) also was a significant risk factor for recurrence (odds ratio = 10.7, p < 0.03). With the numbers available, no significant relationship was found between gender, race, parental marital status, source of medical insurance, or parental income and the risk of recurrence of the clubfoot deformity. In addition, the severity of the deformity, the age of the patient at the initiation of treatment, and previous treatment were not found to have a significant effect on the risk of recurrence. CONCLUSION Noncompliance and the educational level of the parents (high-school education or less) are significant risk factors for the recurrence of clubfoot deformity after correction with the Ponseti method. The identification of patients who are at risk for recurrence may allow intervention to improve the compliance of the parents with regard to the use of orthotics, and, as a result, improve outcome. LEVEL OF EVIDENCE Prognostic study, Level II-1 (retrospective study). See Instructions to Authors for a complete description of levels of evidence.

Slipped capital femoral epiphysis.

Slipped capital femoral epiphysis is a well known disorder of the hip in adolescents that is characterized by displacement of the capital femoral epiphysis from the metaphysis through the physis. The term slipped capital femoral epiphysis is a misnomer because the epiphysis is held in the acetabulum by the ligamentum teres, and thus it is actually the metaphysis that moves upward and outward while the epiphysis remains in the acetabulum. In most patients, there is an apparent varus relationship between the head and the neck, but occasionally the slip is into a valgus position, with the epiphysis displaced superiorly in relation to the neck106,109. In the vast majority of cases, the etiology is unknown. Although the condition may be associated with a known endocrine disorder71,77,129, with renal failure osteodystrophy74, or with previous radiation therapy75,77, this Instructional Course Lecture deals only with idiopathic slipped capital femoral epiphysis. Multiple theories have been proposed for the etiology of idiopathic slipped capital femoral epiphysis, and it is likely a result of both biomechanical and biochemical factors128. The combination of these factors results in a weakened physis with subsequent failure. Mechanical factors95 associated with the disorder are obesity62,72, increased femoral retroversion36,37,95, and increased physeal obliquity83. The vast majority of children with a slipped capital femoral epiphysis are obese, which increases the shear stress across the physis. Obesity is also associated with femoral retroversion, with anteversion averaging 10.6 degrees in adolescents with normal weight but only 0.40 degree in obese adolescents36. This femoral retroversion increases the stress across the physis95. Children with a slipped capital femoral epiphysis also have a more …

Long-term follow-up of patients with clubfeet treated with extensive soft-tissue release.

BACKGROUND Although long-term follow-up studies have shown favorable results, in terms of foot function, after treatment of idiopathic clubfoot with serial manipulations and casts, we know of no long-term follow-up studies of patients in whom clubfoot was treated with an extensive surgical soft-tissue release. METHODS Forty-five patients (seventy-three feet) in whom idiopathic clubfoot was treated with either a posterior release and plantar fasciotomy (eight patients) or an extensive combined posterior, medial, and lateral release (thirty-seven patients) were followed for a mean of thirty years. Patients were evaluated with detailed examination of the lower extremities, a radiographic evaluation that included grading of osteoarthritis, and three independent quality-of-life questionnaires, including the Short Form-36 Medical Outcomes Study. RESULTS At the time of follow-up, the majority of patients in both treatment groups had significant limitation of foot function, which was consistent across the three independent quality-of-life questionnaires. No significant difference between groups was noted with regard to the results of the quality-of-life measures, the range of motion of the ankle or the position of the heel, or the radiographic findings. Six patients who had been treated with only one surgical procedure had better ranges of motion of the ankle and subtalar joints (p < 0.004) than those who had had multiple surgical procedures. CONCLUSIONS Many patients with clubfoot treated with an extensive soft-tissue release have poor long-term foot function. We found a correlation between the extent of the soft-tissue release and the degree of functional impairment. Repeated soft-tissue releases can result in a stiff, painful, and arthritic foot and significantly impaired quality of life.

Selective Posterior Thoracic Fusions for Adolescent Idiopathic Scoliosis: Comparison of Hooks versus Pedicle Screws

Study Design. A retrospective review of adolescent idiopathic scoliosis (AIS) patients with major thoracic-compensatory lumbar C modifier curves treated with a selective posterior fusion using an all-hook construct versus pedicle screw construct. Objectives. To compare the clinical and radiographic results of selective posterior thoracic fusion using hooks versus pedicle screws in patients with major thoracic-compensatory lumbar C modifier AIS curves. Summary of Background Data. Although spontaneous lumbar curve correction often occurs following a selective thoracic spinal fusion, there are few reports that focus on selective posterior thoracic spinal fusion in the presence of a lumbar C modifier curve. Methods. Sixty-six consecutive patients with major thoracic-compensatory lumbar C modifier AIS curves underwent selective posterior thoracic fusion to T12 or L1 at a single institution (1987–2001). Hooks were used for instrumentation in 32 patients and pedicle screws were used in 34 patients. Patients were evaluated at a minimum 2-year follow-up. To test for differences between groups analysis of covariance (ANCOVA) was used. Results. There was no statistical difference between the preoperative thoracic and lumbar Cobb values for the hook group versus the pedicle screw group. The amount of correction obtained surgically of the thoracic Cobb and the amount of spontaneous lumbar Cobb correction were significantly greater in the pedicle screw group (P < 0.001). The incidence of postoperative coronal decompensation, with a greater than 20 mm shift to the left of the C7 plumbline, was higher in the hook group (13 patients) as compared with the pedicle screw Group 4 patients (P < 0.005). There were no complications or reoperations in either group. Conclusion. Selective thoracic fusion of main thoracic-compensatory lumbar C modifier AIS curves with pedicle screws allowed for better thoracic correction and less postoperative coronal decompensation than seen with hooks.

Complications of titanium elastic nails for pediatric femoral shaft fractures.

Limited data exist about complications of titanium elastic nails (TNs) for femur fracture management in pediatric patients. Thirty-nine patients with 43 femoral shaft fractures were identified whose average age was 6.0 years. There were 21 complications (1 intraoperative, 20 postoperative) in 43 femur fractures (49%). There were two major postoperative complications: one septic arthritis after nail removal and one hypertrophic nonunion. Minor postoperative complications were pain at the nails in 13 extremities, nail erosion through the skin in 4, and one delayed union. There was an association between the prominence of TNs and nail pain or skin erosion. Fracture angulation and outcome were associated with the patients weight and size of the nails implanted. Technical pitfalls exist with this implant and can be minimized by leaving less than 2.5 cm of nail out of the femur and by using the largest nail sizes possible.

Update on Clubfoot: Etiology and Treatment

AbstractAlthough clubfoot is one of the most common congenital abnormalities affecting the lower limb, it remains a challenge not only to understand its genetic origins but also to provide effective long-term treatment. This review provides an update on the etiology of clubfoot as well as current treatment strategies. Understanding the exact genetic etiology of clubfoot may eventually be helpful in determining both prognosis and the selection of appropriate treatment methods in individual patients. The primary treatment goal is to provide long-term correction with a foot that is fully functional and pain-free. To achieve this, a combination of approaches that applies the strengths of several methods (Ponseti method and French method) may be needed. Avoidance of extensive soft-tissue release operations in the primary treatment should be a priority, and the use of surgery for clubfoot correction should be limited to an “a la carte” mode and only after failed conservative methods. Level of Evidence: Level V, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Anterior/posterior Spinal Instrumentation versus Posterior Instrumentation Alone for the Treatment of Adolescent Idiopathic Scoliotic Curves More Than 90°

Study Design. A retrospective review of patients with adolescent idiopathic scoliosis (AIS), with curves more than 90° treated with either a combined anterior/posterior spinal fusion or a posterior spinal fusion alone. Objectives. To assess the results of spinal fusion for AIS curves >90° and determine whether the use of a posterior-only approach with an all-pedicle screw construct can decrease the need for anterior release surgery. Summary of Background Data. Treatment of AIS curves >90° often consists of anterior release and posterior fusion to improve coronal correction and fusion rate. However, the use of pedicle screws has allowed improved coronal curve correction rates even in large curves, which may decrease the need for anterior release surgery. Methods. A total of 54 consecutive patients with AIS with curves >90° who underwent a spinal fusion procedure at 1 institution between 1987 and 2001, with either a combined anterior/posterior spinal fusion (hooks and screws) or a posterior spinal fusion alone with an all-pedicle screw construct, were included for analysis. All patients had a minimum 2-year follow-up (mean 6.0; range 2.0–14.5), and were analyzed radiographically as well as with pulmonary function tests. Statistical analyses were performed between groups using the Wilcoxon-Mann-Whitney tests. Results. There were 20 patients treated with an anterior/posterior spinal fusion and 34 with a posterior spinal fusion alone. There were no statistically significant differences between the groups for gender, age, number of levels fused, preoperative coronal/sagittal Cobb measurements, coronal curve flexibility, or amount of postoperative coronal Cobb correction. There was less of a negative effect on pulmonary function in the group treated with posterior spinal fusion versus the group treated with a combined anterior/posterior spinal fusion (P < 0.005). There were no complications/reoperations in either group. Conclusion. In this patient population with often restrictive preoperative pulmonary function, a posterior-only approach with the use of an all-pedicle screw construct has the advantage of providing the same correction as an anterior/posterior spinal fusion, without the need for entering the thorax and more negatively impacting pulmonary function.

CHD7 gene polymorphisms are associated with susceptibility to idiopathic scoliosis

Idiopathic scoliosis (IS) is the most common spinal deformity in children, and its etiology is unknown. To refine the search for genes underlying IS susceptibility, we ascertained a new cohort of 52 families and conducted a follow-up study of genomewide scans that produced evidence of linkage and association with 8q12 loci (multipoint LOD 2.77; P=.0028). Further fine mapping in the region revealed significant evidence of disease-associated haplotypes (P<1.0 x 10-4) centering over exons 2-4 of the CHD7 gene associated with the CHARGE (coloboma of the eye, heart defects, atresia of the choanae, retardation of growth and/or development, genital and/or urinary abnormalities, and ear abnormalities and deafness) syndrome of multiple developmental anomalies. Resequencing CHD7 exons and conserved intronic sequence blocks excluded coding changes but revealed at least one potentially functional polymorphism that is overtransmitted (P=.005) to affected offspring and predicts disruption of a caudal-type (cdx) transcription-factor binding site. Our results identify the first gene associated with IS susceptibility and suggest etiological overlap between the rare, early-onset CHARGE syndrome and common, later-onset IS.

Genome-wide association studies of adolescent idiopathic scoliosis suggest candidate susceptibility genes

Adolescent idiopathic scoliosis (AIS) is an unexplained and common spinal deformity seen in otherwise healthy children. Its pathophysiology is poorly understood despite intensive investigation. Although genetic underpinnings are clear, replicated susceptibility loci that could provide insight into etiology have not been forthcoming. To address these issues, we performed genome-wide association studies (GWAS) of ∼327 000 single nucleotide polymorphisms (SNPs) in 419 AIS families. We found strongest evidence of association with chromosome 3p26.3 SNPs in the proximity of the CHL1 gene (P < 8 × 10(-8) for rs1400180). We genotyped additional chromosome 3p26.3 SNPs and tested replication in two follow-up case-control cohorts, obtaining strongest results when all three cohorts were combined (rs10510181 odds ratio = 1.49, 95% confidence interval = 1.29-1.73, P = 2.58 × 10(-8)), but these were not confirmed in a separate GWAS. CHL1 is of interest, as it encodes an axon guidance protein related to Robo3. Mutations in the Robo3 protein cause horizontal gaze palsy with progressive scoliosis (HGPPS), a rare disease marked by severe scoliosis. Other top associations in our GWAS were with SNPs in the DSCAM gene encoding an axon guidance protein in the same structural class with Chl1 and Robo3. We additionally found AIS associations with loci in CNTNAP2, supporting a previous study linking this gene with AIS. Cntnap2 is also of functional interest, as it interacts directly with L1 and Robo class proteins and participates in axon pathfinding. Our results suggest the relevance of axon guidance pathways in AIS susceptibility, although these findings require further study, particularly given the apparent genetic heterogeneity in this disease.