Scoliosis Correction in an Adolescent with a Rigid Spine Syndrome
Bibliographic record
Abstract
In Brief Study Design. Details of presentation and approach to the treatment of scoliosis in a case of a Rigid Spine Syndrome (RSS). Objective. To report on the results of conservative and operative treatment of scoliosis associated with RSS, and, based on this, to propose an assessment and treatment protocol for this condition. Summary of Background Data. Congenital muscular dystrophies (CMD) are a group of disorders marked by hypotonia at birth and a generally nonprogressive course of muscle weakness. Spinal rigidity is present in a number of patients with CMD. RSS is classified as a CMD, and is characterized by early rigidity of the spine, limb contractures, and restrictive respiratory dysfunction. An approach to the treatment of scoliosis in RSS has not been established. Methods. Details of history, diagnostic tests, and treatment of an adolescent with RSS associated with progressive scoliosis and cervical spine extension contracture is presented. The role of brace treatment, Botox® (Allergan, Inc., Irvine, CA), and details of operative correction, including histology of back muscles, is defined. Results. In this case of RSS with rigid scoliosis nonresponsive to brace therapy and Botox® injections, an anterior thoracolumbar spine fusion with instrumentation was successfully performed for correction of the scoliosis, and the cervical spine was addressed through a posterior approach and an occiput-cervical fusion. We outline the details of surgical procedure and restoration of spinal balance in both sagittal and coronal planes. Conclusions. The chromosomal bases of CMD and characteristic features of RSS, including diagnostic tests, have been reviewed. Surgical intervention with spinal deformity fusion, correction, and instrumentation is indicated in RSS with progressive spinal imbalance not responding to conservative therapy is safe and can give promising results. Congenital muscular dystrophies (CMD) are a group of disorders marked by hypotonia at birth and a generally nonprogressive course of muscle weakness. Spinal rigidity may be present in a number of patients with CMD. Rigid spine syndrome (RSS) is categorized under the group of CMD. RSS is characterized by early rigidity of the spine, generally associated with muscle weakness, limb-joint contractures, and respiratory dysfunction. We present a case of a 14-year-old girl with rigid spine syndrome associated with severe progressive thoracolumbar scoliosis and progressive cervical extension contracture that did not respond to brace treatment. We further outline the surgical treatment and briefly review the literature.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.000 |
| Insufficient payload (model declined to judge) | 0.002 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".