Freehand correction of scoliosis in Rett's syndrome.
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ABSTRACT:
SUBMITTER: Mehdian H
PROVIDER: S-EPMC3585620 | biostudies-literature | 2013 Mar
REPOSITORIES: biostudies-literature
Publications
Freehand correction of scoliosis in Rett's syndrome.
Mehdian Hossein H Elsayed Sherief S
European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society 20130301 3
We present the case of an 18-year-old girl with a scoliosis (long C-shaped curve) in association with an atypical Rett's syndrome. In order to attain a full correction and to provide her with adequate posture and sitting balance, segmental instrumentation was utilised from the high thoracic spine to the pelvis. We describe the procedure including the relevant pre-operative, intra-operative and post-operative imaging. ...[more]
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Project description:BackgroundSurgical correction of neuromuscular scoliosis can be associated with high complication rates, including such associated with pelvic fixation. Up to now it is debated whether and when to include the pelvis into the fusion construct. Therefore, we aimed to illuminate when pelvic fixation is beneficial in surgical correction of neuromuscular scoliosis.MethodsA prospective cohort of 49 patients (mean age 13 ± 3 y, 63% females, follow up 56 months, range 24-215) who underwent correction of neuromuscular scoliosis including S1/the ileum (n = 18) or without (n = 31) pelvic fixation were included. The outcome was measured with analysis of radiological parameters, clinical improvement and complication/revision rates. Subgroup analysis was performed to find if non-ambulatory patients with gross motor function classification system (GMFCS) levels >III, with larger scoliotic curves (>60°) and moderate pelvic obliquities up to 35° benefit from pelvic fixation.ResultsThere was no significant difference in complications when comparing patients with (9 out of 18 patients, 50%) or without (9 out of 31 patients, 29%) fixation to the pelvis (p = .219). Wheelchair bound patients (GMFCS >III) with cobb angles greater than 60° and pelvic obliquity less than 35° (n = 20) revealed no differences in amount of clinical improvement of ambulation with (n = 9) or without (n = 11) pelvic fixation (p: n.s.). And even complication or revision rates where not different in those two groups.ConclusionPelvic fixation does not seem obligatory in wheelchair bound patients per definition. Even with pelvic obliquities up to 35° and large scoliotic curves >60°, avoiding pelvic fixation does not result in higher revision rate or worse clinical outcomes.
| S-EPMC9126953 | biostudies-literature
Project description:ObjectiveRecently, minimally invasive surgery (MIS) has been included among the treatment modalities for scoliosis. However, literature comparing MIS to open surgery for scoliosis correction is limited. The objective of this study was to compare outcomes for scoliosis correction patients undergoing MIS versus open approach.MethodsWe retrospectively collected data on demographics, procedure characteristics, and outcomes for 207 consecutive scoliosis correction surgeries at our institution between 2009 and 2015.ResultsMIS patients had lower number of levels fused (p < 0.0001), shorter surgeries (p = 0.0023), and shorter overall lengths of stay (p < 0.0001), were less likely to be admitted to the ICU (p < 0.0001), and had shorter ICU stays (p = 0.0015). On multivariable regression, number of levels fused predicted selection for MIS procedure (p = 0.004), and multiple other variables showed trends toward significance. Age predicted ICU admission and VTE. BMI predicted any VTE, and DVT specifically. Comorbid disease burden predicted readmission, need for transfusion, and ICU admission. Number of levels fused predicted prolonged surgery, need for transfusion, and ICU admission.ConclusionsPatients undergoing MIS correction had shorter surgeries, shorter lengths of stay, and shorter and fewer ICU stays, but there was a significant selection effect. Accounting for other variables, MIS did not independently predict any of the outcomes.
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Project description:BackgroundScoliosis is prominent in Rett syndrome (RTT). Following the prior report from the US Natural History Study, the onset and progression of severe scoliosis (≥40° Cobb angle) and surgery were examined regarding functional capabilities and specific genotypes, addressing the hypothesis that abnormal muscle tone, poor oral feeding, puberty, and delays or absence of sitting balance and ambulation may be responsible for greater risk in RTT.MethodsThe multicenter RTT Natural History Study gathered longitudinal data for classic RTT, including mutation type, scoliosis, muscle tone, sitting, ambulation, hand function, and feeding. Cox regression models were used to examine the association between scoliosis and functional characteristics. All analyses utilized SAS 9.4; two-sided P values of <0.05 were considered significant.ResultsA total of 913 females with classic RTT were included. Scoliosis frequency and severity increased with age. Severe scoliosis was found in 251 participants (27%), 113 of whom developed severe scoliosis during the follow-up assessments; 168 (18%) had surgical correction. Severe MECP2 mutations (R106W, R168X, R255X, R270X, and large deletions) showed a higher proportion of scoliosis. Individuals developing severe scoliosis or requiring surgery were less likely to sit, ambulate, or use their hands and were more likely to have begun puberty. Significant differences were absent for epilepsy rates, sleep problems, or constipation.DiscussionScoliosis requires vigilance regarding the risk factors noted, particularly specific mutations and the role of puberty and motor abilities. Bracing is recommended for moderate curves and surgery for severe curves in accordance with published guidelines for scoliosis management.
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Project description:BackgroundTo identify the pelvic parameters affecting in-brace correction (IBC) in patients with idiopathic scoliosis (IS).MethodsPatients with IS receiving Chêneau brace treatment in our scoliosis center from January 2019 to November 2019 were retrospectively analyzed. Pelvic rotation parameters, including pelvic incidence (PI), sacral slope (SS), pelvic tilt (PT), L/R ratio, were collected. Other radiographic data, such as Risser sign, coronal and sagittal balance, curve location, kyphosis, lordosis of each patient were also recorded to analyze their correlations with IBC. Correlation analyses were performed to identify the classified variables influencing IBC. The principal component analysis was used to extract common factors of radiographic parameters to eliminate interaction effects. The linear regression equation was established using principal components, the variables influencing IBC were identified.ResultsA cohort of 44 patients with IS (36 girls and 8 boys) were included in the present study. The mean IBC was 49.87% (range, 3%-100%). IBC of lumbar IS was negatively correlated with apical rotate factor (ARF, B = -0.385), mainly consisted of pelvic coronal plane rotation (PCPR, 0.449), Cobb angle (CA, 0.575), apical vertebral rotation (AVR, 0.918), and pelvic rotate factor (PRF, B = -0.387), mainly consisted of PT (0.861), PI (0.728), PCPR (-0.570). The regression equation of lumbar IS had statistical significance (F = 6.500, P = 0.005, R2 = 0.317), whereas statistically significance was not found in the regression equation of thoracic IS (F = 2.913, P = 0.106). The remaining parameters were not related to IBC.ConclusionsFor lumbar IS, ARF and PRF have negative effects on IBC, coronal and sagittal rotation of the pelvis is related to IBC.
| S-EPMC7495828 | biostudies-literature
Project description: Not available
| S-EPMC7571623 | biostudies-literature
Project description:The three-dimensional correction of severe rigid scoliosis has been improved by segmental pedicle screw instrumentation. However, there can be significant difficulty related to the use of a rigid rod, especially in the apex region of severe scoliosis. This study is a retrospective matched cohort study to evaluate the advantages of Nitinol shape memory alloy (SMA) rod-based correction by comparing the clinical and radiographic results obtained from using a temporary SMA rod and those from a standard rod in the correction of severe scoliosis. From May 2004 to September 2006, patients with matched curve type, ages at surgery, operative methods and fusion levels in our institute and instrumented with either SMA rods (n = 14) or traditional correction techniques (n = 16) were reviewed. In SMA group, the SMA rods served as a temporary intraoperative tool for deformity correction and were replaced by standard rods. The blood loss at surgery averaged 778 +/- 285 ml in the traditional group and 585 +/- 188 ml in the SMA group (P < 0.05). Operative time averaged 284 +/- 53 min in the SMA group and 324 +/- 41 min in the traditional group (P < 0.05). In the SMA group, the preoperative major curve was 92.6 degrees +/- 13.7 degrees with a flexibility of 25.5 +/- 7.3% was corrected to 29.4 degrees +/- 5.7 degrees demonstrating a 68.4% immediate postoperative correction. In the traditional group, the preoperative major curve was 88.6 degrees +/- 14.6 degrees with a flexibility of 29.3 +/- 6.6% was corrected to 37.2 degrees +/- 7.3 degrees demonstrating a 57.8% immediate postoperative correction. There was a statistic difference between the SMA group and traditional group in correction rate of the major thoracic curve. In the SMA group, one case suffered from deep infection 2 months postoperatively. In the traditional group, 6 of 16 cases suffered pedicle screw pull out or loosening during placement of the standard rod at the apex vertebrae on the concave side. In three cases, the mono-axial pedicle screws near the apex were abandoned and in five cases replaced with poly-axial pedicle screws. This study shows that the temporary use of SMA rod may reduce the operative time, blood loss, while improve the correction of the coronal plane when compared with standard techniques.
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