The International Scientific Society on Scoliosis Orthopaedic and Rehabilitation Treatment (SOSORT) produced its first guidelines in 2005 and renewed them in 2011. Recently published high-quality clinical trials on the effect of conservative treatment approaches (braces and exercises) for idiopathic scoliosis prompted us to update the last guidelines’ version. The objective was to align the guidelines with the new scientific evidence to assure faster knowledge transfer into clinical practice of conservative treatment for idiopathic scoliosis (CTIS). Physicians, researchers and allied health practitioners working in the area of CTIS were involved in the development of the 2016 guidelines. Multiple literature reviews reviewing the evidence on CTIS (assessment, bracing, physiotherapy, physiotherapeutic scoliosis-specific exercises (PSSE) and other CTIS) were conducted. Documents, recommendations and practical approach flow charts were developed using a Delphi procedure. The process was completed with the Consensus Session held during the first combined SOSORT/IRSSD Meeting held in Banff, Canada, in May 2016. The contents of the new 2016 guidelines include the following: background on idiopathic scoliosis, description of CTIS approaches for various populations with flow-charts for clinical practice, as well as literature reviews and recommendations on assessment, bracing, PSSE and other CTIS. The present guidelines include a total of 68 recommendations divided into following topics: bracing (n = 25), PSSE to prevent scoliosis progression during growth (n = 12), PSSE during brace treatment and surgical therapy (n = 6), other conservative treatments (n = 2), respiratory function and exercises (n = 3), general sport activities (n = 6); and assessment (n = 14). According to the agreed strength and level of evidence rating scale, there were 2 recommendations on bracing and 1 recommendation on PSSE that reached level of recommendation “I” and level of evidence “II”. Three recommendations reached strength of recommendation A based on the level of evidence I (2 for bracing and one for assessment); 39 recommendations reached strength of recommendation B (20 for bracing, 13 for PSSE, and 6 for assessment).The number of paper for each level of evidence for each treatment is shown in Table 8. The 2016 SOSORT guidelines were developed based on the current evidence on CTIS. Over the last 5 years, high-quality evidence has started to emerge, particularly in the areas of efficacy of bracing (one large multicentre trial) and PSSE (three single-centre randomized controlled trials). Several grade A recommendations were presented. Despite the growing high-quality evidence, the heterogeneity of the study protocols limits generalizability of the recommendations. There is a need for standardization of research methods of conservative treatment effectiveness, as recognized by SOSORT and the Scoliosis Research Society (SRS) non-operative management Committee.

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2016 SOSORT guidelines: orthopaedic and rehabilitation treatment of idiopathic scoliosis during growth.

STUDY DESIGN Retrospective chart review. OBJECTIVES To assess outcome and complications in pediatric patients with a vital capacity < or =45% of predicted who underwent reconstructive spine surgery for severe thoracic scoliosis. SUMMARY OF BACKGROUND DATA Despite the known sequelae of untreated spine deformity, pediatric patients with severe restrictive lung disease are frequently not considered good operative candidates for surgical treatment on the basis of their underlying lung disease. Only a few reports have examined surgical treatment in this population. METHODS A retrospective chart review was conducted on 21 pediatric patients with restrictive lung disease (average age 11.5 years, range 2-21 years). Eighteen patients completed pulmonary function testing, all with vital capacity < or =45%. The remaining three patients had clinical features of restrictive lung disease but could not perform pulmonary function testing. Eighteen patients underwent combined anterior and posterior spinal fusions. Before surgery, all patients were seen by the pulmonary, cardiology, pediatric, and anesthesia services, as well as neurology and gastroenterology services if indicated. Outcome variables examined were as follows: 1) days on oxygen, 2) need for tracheostomy, 3) days on ventilatory support, 4) mortality rate, 5) pulmonary complications, 6) blood loss, 7) other complications relative to the surgical procedure, and 8) length of stay. RESULTS For the 21 patients, the preoperative average vital capacity was 32% predicted (range 18-43%). After surgery, the median time on supplemental oxygen was 3 days (range 0-90 days). Only one patient with a vital capacity of 29%, who was successfully extubated on postoperative day 4, developed a deep wound infection, which required incision and drainage, as well as recurrent right lung collapse, and subsequently required a tracheostomy to facilitate ventilator weaning. Four patients were on assisted ventilation before the surgery and continued to require it after surgery. Two additional patients required noninvasive positive pressure ventilation for a short period of time following the surgery. Pneumonia occurred in two patients, and pleural effusion occurred in two patients. Four patients developed postoperative atelectasis. There was one reoperation for implant failure. There were no mortalities or adverse neurologic outcomes. Major thoracic scoliosis measured an average of 82 degrees (range 40-140 degrees) before surgery, with correction to an average of 35 degrees (range 6-75 degrees). CONCLUSIONS Reconstructive spine surgery in pediatric patients with severe restrictive lung disease and significant spinal deformity is well tolerated. Familiarity with different surgical techniques of salvage reconstruction and perioperative multidisciplinary management should be emphasized. Routine preoperative tracheostomy is not indicated.

Outcome of pediatric patients with severe restrictive lung disease following reconstructive spine surgery.

https://www.pmr.theclinics.com/article/S1047-9651(12)00082-4/pdf

Management of Pulmonary Complications in Neuromuscular Disease

Study Design. Prospective clinical study of three-dimensional thoracic growth in children with idiopathic scoliosis and a reference group. Objective. To measure thoracic dimensions and volume in relation to growth and to verify the influence of scoliosis. of Background Data. Scoliosis represents a three-dimensional spinal deformity leading to thoracic deformity. Vertical expandable prosthetic titanium rib distraction primarily treats this deformity, but little is known about thoracic growth in scoliosis. Methods. One hundred and thirty children with scoliosis (20 boys, 110 girls; 4-16 years; curves 15°-45°; rib hump 5-25 mm) were compared with 126 children without spinal deformity (61 boys, 65 girls). The ORTEN system was used for optical trunk surface data acquisition. Thoracic volume, perimeter, anterior-posterior and transversal diameters, T1-T12 and sternal lengths were calculated and related to age, weight, standing and sitting height. Results. There was no significant difference in thoracic volume between the 2 groups (P > 0.05). The correlation between thoracic volume (3-16 dm 3 ) and growth parameters was greater (R 2 > 0.70) than with age. At 4 years, thoracic volume represents 33%, and at 10 years, it represents 55% of its volume at 16 years. It triples between 4 and 16 years and doubles during puberty. These relationships were constant in all groups. During growth, the transversal diameter corresponds to 30%, the anterior-posterior diameter represents 20% and the thoracic perimeter 100% of sitting height. Conclusion. The thoracic deformity in scoliosis is measurable with ORTEN, but scoliosis s45° with a rib hump ≤25 mm does not significantly influence thoracic volume. Thoracic parameters should be related to sitting height rather than age. Established relationships give a reliable indication of thoracic proportions, which are useful for understanding global spinal and thoracic deformity and for optimizing braces or surgical strategies. Further investigations are required for curves >45°, leading to major thoracic asymmetry.

Influence of idiopathic scoliosis on three-dimensional thoracic growth.

Adolescent idiopathic scoliosis (AIS) is a clinically significant disorder with high heritability that affects 2–4% of the population. Genome-wide association studies have identified LBX1 as a strong susceptibility locus for AIS in Asian and Caucasian populations. Here we further dissect the genetic association with AIS in a Caucasian population. To identify genetic markers associated with AIS we employed a genome-wide association study (GWAS) design comparing 620 female Caucasian patients who developed idiopathic scoliosis during adolescence with 1,287 ethnically matched females who had normal spinal curves by skeletal maturity. The genomic region around LBX1 was imputed and haplotypes investigated for genetic signals under different inheritance models. The strongest signal was identified upstream of LBX1 (rs11190878, Ptrend = 4.18×10-9, OR = 0.63[0.54–0.74]). None of the remaining SNPs pass the genome-wide significance threshold. We found rs11190870, downstream of LBX1 and previously associated with AIS in Asian populations, to be in modest linkage disequilibrium (LD) with rs11190878 (r2 = 0.40, D' = 0.81). Haplotype analysis shows that rs11190870 and rs11190878 track a single risk factor that resides on the ancestral haplotype and is shared across ethnic groups. We identify six haplotypes at the LBX1 locus including two strongly associated haplotypes; a recessive risk haplotype (TTA, Controlfreq = 0.52, P = 1.25×10-9, OR = 1.56), and a co-dominant protective haplotype (CCG, Controlfreq = 0.28, P = 2.75×10-7, OR = 0.65). Together the association signals from LBX1 explain 1.4% of phenotypic variance. Our results identify two clinically relevant haplotypes in the LBX1-region with opposite effects on AIS risk. The study demonstrates the utility of haplotypes over un-phased SNPs for individualized risk assessment by more strongly delineating individuals at risk for AIS without compromising the effect size.

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Haplotypes at LBX1 Have Distinct Inheritance Patterns with Opposite Effects in Adolescent Idiopathic Scoliosis

In kyphoscoliosis restrictive ventilatory defect occurs. In idiopathic scoliosis vital capacity failure is significantly correlated with Cobb angle, vertebral rotation, and thoracic lordosis. Maximum voluntary ventilation is the most affected measurement. Forced expiratory volume in 1 second is reduced. Residual volume remains longtime normal. Hypoxemia due to decrease of diffusing capacity occurs, with initially reflex hyperventilation hypocapnia, and secondary hypercapnia. Pulmonary hypertension and cor pulmonale is related to hypoventilation and hypoxia. The lung situated on the concave side of the scoliosis curve shows a more functional derangement. Ventilatory pattern consists of low tidal volume and high respiratory rate with increase of ventilatory work. Scoliosis that appears in the earlier stage of the life has the worst respiratory prognosis (before 5 years of age) with impairement of lung and thoracic growth. To stimulate pulmonary and thoracic growth, intermittent ventilatory assistance by pressure preset ventilator should be performed as soon as possible and pursued up to 8 years of age, at least, more if necessity. In over 60 degrees angle idiopathic scoliosis, respiratory failure appears after 40 to 50 years of age. Non invasive ventilatory assistance with preset pressure ventilator by oral way in moderate cases and nocturnal nasal ventilation by volume ventilator or inspiratory assistance ventilator, in the most severe cases are efficient. In very severe and acute respiratory insufficiency (scoliosis over 90 degrees) ventilation by intubation then tractheostomy may be required. Earlier orthopedic management and surgical procedure to correct and stabilize spinal deformities is the best to prevent respiratory insufficiency. For scoliosis below 60 degrees, post operative pulmonary complications are very low, with no requirement of post operative ventilatory support. In very severe respiratory insufficiency treatment of respiratory failure precedes, and follows, orthotic treatment and surgical procedures; it shouldle pursued afterwards.

A Barois

Papers

Respiratory problems in severe scoliosis