Research On The Treatment Of Complex Syndrome With Scoliosis

Captain 1: The efficacy of brace treatment for thoracolumbar kyphosis in patients with achondroplasiaObjectives Brace treatment has been used to correct thoracolumbar kyphosis(TLK)in patients with achondroplasia.However,there was a paucity of knowledge concerning its effectiveness and related factors.The primary aim of the current study was to evaluate theoutcome of brace treatment in the correction of TLK for patients with achondroplasia and to determine the factors associated with bracing efficacy.Methods 33 achondroplasic patients treated by bracing between January 2002 and February 2015 were included in this study.Radiographic parameters including TLK,lumbar lordosis(LL),curve magnitude,apical vertebral translation(AVT),percentage of apical vertebral wedging,pelvic tilt(PT)and pelvic incidence(PI)were recorded for each patient at the visit.Comparison of these parameters between the initial visit and the final visit was performed using the Student t test.Factors associated with the correction of TLK were evaluated using the logistic regression analysis.Results The mean age at presentation was 57.5 ± 23.4 months.The mean period of treatment was 32.2 ± 15.7 months,and the mean period of follow-up was 25.7 ± 11.3 months.At the initial visit,the mean value of TLK and the percentage of apical vertebral wedging were 41.7 ± 15.4 degrees and 61.4% ± 16.2%,respectively.At the final visit,the TLK and apical vertebral wedging were remarkably reduced to 29.5 ± 20.8 degrees and 52.1% ± 18.7%,respectively.The logistic regression analysis showed that initial TLK,apical vertebral translation,percentage of apical vertebral wedging,and pelvic tilt were independent factors associated with the correction of TLK.Conclusions Brace treatment can effectively correct TLK and restore the morphology of apical vertebral body for patients with achondroplasia.Large TLK,severe apical vertebral wedging,presence of AVT and low PT may be indicative of an unfavorable outcome,which should be taken into account at the initiation of bracing.Captain 2:Posterior spinal osteotomy for correction of progressive thoracolumbar kyphosis in patients with achondroplasiaObjective Progressive thoracolumbar kyphosis(TLK)is a common manifestation in patients with achondroplasia.To date,few papers have investigated the outcome of correction surgery for TLK in this type of patients.The primary aim of the present study was to evaluate the outcome of posterior spinal osteotomy surgery for TLK in pediatric patients with achondroplasia.Methods 14 achondroplastic patients undergoing one-stage posterior surgery for progressive TLK were reviewed.The osteotomy procedures included Smith-Petersen osteotomy(SPO)in 12 patients and pedicle subtraction osteotomy(PSO)in 2 patients.Correction rate of kyphotic deformity,fusion levels,density of pedicle screw,surgical complications,and patient satisfactory index(PSI)were evaluated for each patient.Results The mean age at surgery was10.4±2.1 years(range,8-14),with an average follow-up period of 47.8 ± 16.3 months(range,24-84).The mean preoperative kyphotic angle was 58.3 ± 17.1(range,42°-91°),which was corrected to 14.7 ± 6.4 with a mean correction rate of 74.8%.The mean curve magnitude was 40.5 ± 16.7,which was corrected to 11.5 ± 6.8 with a mean correction rate of 71.6%.The average percentage of apicalvertebral wedging was improved from 49.4% before surgery to 26.6% at the final follow up.There was no case with significant loss of correction or neurological symptoms during the follow-up.12 patients were satisfied with the surgical results with a PSI of 92.9%.The perioperative complications included one case of dural tear concomitant with transient neurologic impairment and one case of infection at the incision site.Conclusions The one-stage posterior osteotomy surgery with segmental instrumentation is a safe and effective surgical option for the progressive TLK in pediatric patients with achondroplasia,whichcan not be prevented throughconservative treatment such as braces.The wedged apical vertebral can be spontaneously improved in the long-term follow-up.We recommended that early surgical intervention in childhood should be performed for achondroplastic patients with progressive deformities.Chapter 1:Risk factors of impaired pulmonary function in arthrogryposis multiplex congenital patients with concomitant scoliosis: a comparison with adolescent idiopathic scoliosisObjectives Arthrogryposis multiplex congenital(AMC)patients are generally believed to have impaired pulmonary function.However,the severity of respiratory morbidity and the associated risk factors have not been reported.The aim of this study was to investigate the severity of pulmonary function impairment for AMC patients with concomitant scoliosis and to determine risk factors associated with the impaired pulmonary function in these patients.Methods The pulmonary function tests(PFTs)data including the percentage predicted values of forced vital capacity(%FVC),forced expiratory volume in 1 second(%FEV1),and the ratio of FEV1 to FVC(%FEV1/FVC)were reviewed for 48 AMC patients with secondary scoliosis and 48 patients with adolescent idiopathic scoliosis(AIS).The radiographic parameters of coronal and sagittal plane deformities and body mass index(BMI)were measured.The PFTs data was compared between the AMC and the AIS patients with the student t test.Correlation analysis and regression analysis were performed to determine risk factors associated with impaired pulmonary function.Results AMC patients with concomitant scoliosis had significant lower mean %FVC,%FEV1 and %FEV1/FVC than AIS patients(48.8 vs.70.3 for %FVC,p < 0.001;45.3 vs.69.7 for %FEV1,p < 0.001;92.1 vs.96.9 for %FEV1/FVC,p = 0.02,respectively).70% of AMC patients had severe pulmonary function impairment.A positive correlation was found between BMI and %FVC and %FEV1(r = 0.44 for %FVC,P = 0.002;r = 0.48 for %FEV1,P = 0.001)and between hypokyphosis and %FVC and %FEV1(r = 0.32 for %FVC,P = 0.03;r = 0.36 for %FEV1,P = 0.02).A negative correlation was found between coronal angle and pulmonary function(r =-0.33 for %FVC,P = 0.02;r =-0.29 for %FEV1,P = 0.04).Multiple regression analysis showed that all of the three variables were independent predictors associated with a reduced pulmonary function and with a combination of these variables,the multiple regression model could account for 38.7% of the variance in %FVC and 41.5% in %FEV1.Conclusions There exists severe impairment of pulmonary function in AMC patients with concomitant scoliosis.The scoliotic curve,hypokyphosis and BMI were independent risk factors associated with the pulmonary dysfunction in these patients.As the majority of the variability of impaired PFT is still poorly understood,more risk factors await to be uncovered in future studies.