Much of the information available on this topic is confusing and conflicting.  Approximately 15% of people with Down syndrome will have a misalignment of the cervical vertebrae C-1  and in the neck – this exposes them to the possibility of injury if they participate in activities that hyperextend or radically flex the neck or upper spine.

Special Olympics restrict competition in some sports until an x-ray is provided.  Screening is not necessary for every child as X-ray testing is not definitive and there is no effective treatment available to stabilise the bones satisfactorily if AAI exists.  It is more important for parents to take a common sense approach and if they recognise the symptoms of AAI – persitent neck pain, recent disturbance of gait and loss of previously controlled urination – to seek medical care.

The Spine Society of Australia recommends to:

  • take care to support the head and body of the person with Down syndrome in the car – use a good booster seat for as long as   children are able to fit in one.
  • be wary at the time of a general anaesthetic – parents should make the possibility of AAI known to the anaesthetist as people with Down syndrome undergoing operations that require the lateral  cervical spine in full flexation or full extension may need to be x-rayed before surgery ( such operations are usually denoidectomies and tympanostamies (ENT) surgery).  There have also been RARE reports of neck injury during placement of the breathing tube (Down Syndrome News, June 1991, p61).
  • take care when trampolining
  • take children for an annual exam with a GP of Paediatrician who has been responsible for their long-term care to detect any signs of spinal cord compression eg changes in strength, tone and reflexes in the limbs.

Articles and Information

Atlantoaxial Instability in Down Syndrome : Controversy and Commentary – by Dr Len Leshin

Atlantoaxial Instability – notes for parents and other carers and Atlantoaxial Instability Information Pack

Policy for the participation of children with Down syndrome in sport – the question of C1-2 instability (AAI)

Resources

Selikowitz, Mark  Down Syndrome : the facts

Pueschel, Siegfried  A Parent’s Guide to Down Syndrome

Medical and Surgical Care for Children with Down Syndrome

Links

Spine Society of Australia www.spinesociety.org.au

Special Olympics www.specialolympics.org