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Down Syndrome NSW
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Wednesday 23 June 2010

Revised guidelines on cervical spine instability

The UK and Ireland Down's Syndrome Medical Interest Group has recently revised its guidelines on cervical spine instability (which includes atlantoaxial instability and other conditions) in people with Down syndrome, confirming its long established recommendation that X rays are too unreliable for screening purposes in people without symptoms.  This recommendation is in line with that of the Australian SpineCare Foundation issued in 2004.

The revision also makes clearer recommendations on "different priorities and courses of action for those who are symtomatic and those who are not".

The revised guidelines are intended for professional use.  It would be a useful document  (it is a single sheet) for people with Down syndrome to have in their medical files should it ever need to be referred to, or for families to provide their doctor with a link to the online file:  www.dsmig.org.uk/library/articles/CSI%20from%20BACCH%20newsletter.pdf

It is intended that the revised guidelines will be published by the DSMIG with "fully referenced background notes" later in the year.  The interim document was published by the British Association for Community Child Health, March 2010.

Those interested in the ongoing international differences in recommendations about the value of X-ray screening of people with Down syndrome without screening might be interested in a recent review by neurosurgeons from Columbia University in New York:

Hankinson, TC, Anderson, RC, Craniovertebral Junction Abnormalities in Down Syndrome, Neurosurgery, March 2010 - Volume 66 - Issue 3 - p A32–A38 
  • The abstract and options to purchase the full text of this paper are available here.
Both of these papers emphasise that where surgery is indicated, techniques have developed and improved considerably, and "increasingly good outcomes are being reported where timely intervention is performed in experienced centres" (DSMIG UK).  Hankinson and Anderson conclude that "..... Surgeons performing upper cervical and craniocervical fusion in children with DS should be highly familiar with the surgical anatomy of the region, as these patients frequently harbor congenital anomalies. High resolution imaging is a critical adjunct to successful operative planning."

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