Complex congenital scoliosis: brace treatment. What can we expect?

Abstract Background Treatment of congenital hemivertebra is challenging and data on long-term follow-up (≥ 5 years) are lacking. This study evaluated the surgical outcomes of posterior thoracolumbar hemivertebra resection and short-segment fusion with pedicle screw fixation for treatment of congenital scoliosis with over 5-year follow-up. Methods This study evaluated 27 consecutive patients with congenital scoliosis who underwent posterior thoracolumbar hemivertebra resection and short-segment fusion from January 2007 to January 2015. Segmental scoliosis, total main scoliosis, compensatory cranial curve, compensatory caudal curve, trunk shift, shoulder balance, segmental kyphosis, and sagittal balance were measured on radiographs. Radiographic outcomes and all intraoperative and postoperative complications were recorded. Results The segmental main curve was 40.35° preoperatively, 11.94° postoperatively, and 13.24° at final follow-up, with an average correction of 65.9%. The total main curve was 43.39° preoperatively, 14.13° postoperatively, and 16.06° at final follow-up, with an average correction of 60.2%. The caudal and cranial compensatory curves were corrected from 15.78° and 13.21° to 3.57° and 6.83° postoperatively and 4.38° and 7.65° at final follow-up, with an average correction of 69.2% and 30.3%, respectively. The segmental kyphosis was corrected from 34.30° to 15.88° postoperatively and 15.12° at final follow-up, with an average correction of 61.9%. A significant correction (p < 0.001) in segmental scoliosis, total main curve, caudal compensatory curves and segmental kyphosis was observed from preoperative to the final follow-up. The correction in the compensatory cranial curve was significant between preoperative and postoperative and 2-year follow-up (p < 0.001), but a statistically significant difference was not observed between the preoperative and final follow-up (p > 0.001). There were two implant migrations, two postoperative curve progressions, five cases of proximal junctional kyphosis, and four cases of adding-on phenomena. Conclusion Posterior thoracolumbar hemivertebra resection after short-segment fusion with pedicle screw fixation in congenital scoliosis is a safe and effective method for treatment and can achieve rigid fixation and deformity correction.

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Cross-sectional analysis and trend of vertebral and associated anomalies in Chinese congenital scoliosis population: a retrospective study of one thousand, two hundred and eighty nine surgical cases from 2010 to 2019

International Orthopaedics ◽

10.1007/s00264-021-05061-x ◽

2021 ◽

Author(s):

Guanfeng Lin ◽

Xiran Chai ◽

Shengru Wang ◽

Yang Yang ◽

Jianxiong Shen ◽

...

Keyword(s):

Retrospective Study ◽

Congenital Scoliosis ◽

Associated Anomalies ◽

Cross Sectional ◽

Cross Sectional Analysis ◽

Sectional Analysis

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Novel FGFR1 Variants Are Associated with Congenital Scoliosis

Genes ◽

10.3390/genes12081126 ◽

2021 ◽

Vol 12 (8) ◽

pp. 1126

Author(s):

Shengru Wang ◽

Xiran Chai ◽

Zihui Yan ◽

Sen Zhao ◽

Yang Yang ◽

...

Keyword(s):

Protein Function ◽

Hypogonadotropic Hypogonadism ◽

Congenital Scoliosis ◽

Wild Type ◽

Conservation Analysis ◽

Gene Assay ◽

Protein Expressions ◽

Patient Series ◽

Reduced Activity

FGFR1 encodes a transmembrane cytokine receptor, which is involved in the early development of the human embryo and plays an important role in gastrulation, organ specification and patterning of various tissues. Pathogenic FGFR1 variants have been associated with Kallmann syndrome and hypogonadotropic hypogonadism. In our congenital scoliosis (CS) patient series of 424 sporadic CS patients under the framework of the Deciphering disorders Involving Scoliosis and COmorbidities (DISCO) study, we identified four unrelated patients harboring FGFR1 variants, including one frameshift and three missense variants. These variants were predicted to be deleterious by in silico prediction and conservation analysis. Signaling activities and expression levels of the mutated protein were evaluated in vitro and compared to that of the wild type (WT) FGFR1. As a result, the overall protein expressions of c.2334dupC, c.2339T>C and c.1261A>G were reduced to 43.9%, 63.4% and 77.4%, respectively. By the reporter gene assay, we observed significantly reduced activity for c.2334dupC, c.2339T>C and c.1261A>G, indicating the diminished FGFR1 signaling pathway. In conclusion, FGFR1 variants identified in our patients led to only mild disruption to protein function, caused milder skeletal and cardiac phenotypes than those reported previously.

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Pre-operative halo-gravity traction in severe neurofibromatosis type 1 and congenital scoliosis with thoracic rotatory subluxation

Clinical Neurology and Neurosurgery ◽

10.1016/j.clineuro.2019.105548 ◽

2019 ◽

Vol 187 ◽

pp. 105548 ◽

Cited By ~ 1

Author(s):

Benlong Shi ◽

Liang Xu ◽

Yang Li ◽

Zhen Liu ◽

Xu Sun ◽

...

Keyword(s):

Neurofibromatosis Type 1 ◽

Congenital Scoliosis ◽

Neurofibromatosis Type ◽

Rotatory Subluxation

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4:03 The growth of the thoracic spine in congenital scoliosis after expansion thoracoplasty

The Spine Journal ◽

10.1016/s1529-9430(02)00317-0 ◽

2002 ◽

Vol 2 (5) ◽

pp. 71-72

Author(s):

Robert Campbell ◽

A.K. Hell-Vocke

Keyword(s):

Thoracic Spine ◽

Congenital Scoliosis ◽

Expansion Thoracoplasty

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Survey to describe variability in early onset scoliosis cast practices

Journal of Children s Orthopaedics ◽

10.1302/1863-2548.12.170207 ◽

2018 ◽

Vol 12 (4) ◽

pp. 406-412 ◽

Cited By ~ 2

Author(s):

A. Grzywna ◽

A. McClung ◽

J. Sanders ◽

P. Sturm ◽

L. Karlin ◽

...

Keyword(s):

Early Onset ◽

Congenital Scoliosis ◽

Patient Characteristics ◽

Study Groups ◽

Early Onset Scoliosis ◽

Radiographic Evaluation ◽

Level Of Evidence ◽

Major Curve ◽

Radiographic Measurements ◽

Multicentre Research

Purpose To investigate paediatric orthopaedists’ cast practices for early onset scoliosis regarding patient selection, cast application, radiographic evaluation, treatment cessation and adjunctive bracing. Methods A casting survey was distributed to all paediatric orthopaedists in Children’s Spine and Growing Spine Study Groups (n = 92). Questions included physician and patient characteristics, technique, treatment, outcomes, radiographic measurements and comparison to other treatments. A total of 55 orthopaedists (60%) responded, and descriptive statistics were calculated on the subset who cast (n = 45). Results A majority of respondents use cast treatment for idiopathic and syndromic scoliosis patients, but not for neuromuscular or congenital scoliosis patients. Major curve angle ranked most important in orthopaedists’ decision to commence cast treatment, in comparison with rib-vertebra angle difference or clinical observations. The major curve angle threshold to initiate casting was a median of 30° (20° to 70°), and the minimum patient age was median ten months (3 to 24). First in-cast and out-of-cast radiographs are taken standing, supine, awake, under anesthesia and/or in traction. In all, 58% consistently cast over or under the arm, while 44% vary position by patient. Respondents were divided about the use of a brace after cast treatment: 22% do not prescribe a brace, 31% always do and 36% do in some patients. Conclusions Future multicentre research studies must standardize radiographic practices and consider age and major curve angle at cast initiation and termination, scoliosis aetiology, shoulder position and treatment duration. Practices need to be aligned or compared in these areas in order to distinguish what makes for the best cast treatment possible. Level of Evidence V, Expert opinion

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