Guidelines for Management of Scoliosis in Rett Syndrome Patients Based on Expert Consensus and Clinical Evidence

@article{Downs2009GuidelinesFM,
  title={Guidelines for Management of Scoliosis in Rett Syndrome Patients Based on Expert Consensus and Clinical Evidence},
  author={Jenny Anne Downs and Anke K Bergman and Philippa J Carter and Alison Anderson and Greta M. Palmer and David P Roye and Harold J.P. van Bosse and Ami Bebbington and Eva Larsson and Brian G Smith and Gordon Baikie and Susan Fyfe and Helen Margaret Leonard},
  journal={Spine},
  year={2009},
  volume={34},
  pages={E607-E617}
}
Study Design. Modified Delphi technique. Objective. To develop guidelines for the clinical management of scoliosis in Rett syndrome through evidence review and consensus expert panel opinion. Summary of Background Data. Rett syndrome is a rare disorder and clinical expertise is thus with small case series. Scoliosis is a frequent association and the evidence base dealing with scoliosis management in this syndrome is limited. Parents of affected girls and women have expressed needs for more… 
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References

SHOWING 1-10 OF 76 REFERENCES
Parental experiences of scoliosis management in Rett syndrome
TLDR
The perspectives of parents provided useful insights into the complexities of decision-making regarding scoliosis treatment in Rett syndrome and the provision ofScoliosis information by clinicians should be more family-centred.
Managing Scoliosis in a Young Child with Rett Syndrome: A Case Study
TLDR
The present study describes a new management approach implemented with a girl with Rett syndrome: asymmetrical activation of trunk muscles through equilibrium reactions, which suggested that the intervention was successful in reversing the progress of the scoliosis for the above-mentioned child.
Scoliosis in Rett Syndrome: Clinical and Biological Aspects
TLDR
TheScoliosis in Rett syndrome is of a neurogentic type, and it develops earlier than idiopathic scoliosis, and the development of scoliotic progression is dependent more on stage of disease than on age.
Results of Surgery for Scoliosis in Rett Syndrome
TLDR
Recommendations include planning for surgery when the curve passes 40°, ensuring optimal nutrition before and after surgery, robust fixture of the whole spine in two stages, familiarization of the surgical team with the individual and the disorder before the operation, and inclusion of the main carer in the hospital care team.
THE INCIDENCE AND NATURAL HISTORY OF SCOLIOSIS IN RETT SYNDROME
TLDR
A survey on spinal deformity was conducted by means of a questionnaire mailed to the 350 American families who are members of the International Rett Syndrome Association, finding that scoliosis was present in 119 patients.
Spinal Cord Monitoring for Scoliosis Surgery in Rett Syndrome: Can These Patients Be Accurately Monitored?
TLDR
Patients with Rett syndrome undergoing scoliosis surgery can be successfully monitored with SSEPs, which can accurately alert the surgeon to potential intraoperative spinal cord compromise and, therefore, decrease postoperative morbidity.
Scoliosis in Rett syndrome.
TLDR
Surgical intervention in Rett syndrome should include fusing the scoliotic and the excessively kyphotic segments, and anterior discectomy, interbody fusion, and posterior fusion with instrumentation can achieve improved correction in young adolescents with significant curves.
Predictors of Scoliosis in Rett Syndrome
TLDR
Children with compromised early development before 6 months, those who were less mobile at 10 months, and those who never walked were more likely to have an earlier onset of scoliosis, and the R294X mutation appeared to provide some protective effect against the development ofScoliosis.
Imaging in scoliosis: what, why and how?
TLDR
Although magnetic resonance imaging has had a significant impact in the understanding of the scoliotic deformity, it is still evolving and it may well eventually play a very important role in uncovering the underlying aetiology of 'idiopathic' scoliosis.
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