People with Down's Syndrome have a small risk of acute or chronic neurological problems caused by cervical spine instability.

Down's Syndrome Medical Interest Group (UK)

Basic medical surveillance essentials for people with Down's Syndrome.

One of a set of guidelines drawn up by the Down's Syndrome Medical Interest Group:
www.dsmig.org.uk

  1. People with Down's Syndrome have a small risk for acute or chronic neurological problems caused by cervical spine instability (1,2).
  2. Currently there is no screening procedure which can predict those at risk. In particular, cervical spine X-rays in children have no predictive validity for subsequent acute dislocation/subluxation at the atlantoaxial joint (3,4,5,6,7)
  3. Children with Down's syndrome should not be barred from sporting activities because there is no evidence that participation in sports increases the risk of cervical spine injury any more than for the general population (6,8).
  4. Although the risk of injury is small, if any child or adult needs an anaesthetic the anaesthetist and recovery room staff must always be reminded of the diagnosis so that appropriate care can be taken to avoid cervical injury whilst manipulating the head and neck in the unconscious subject (9).
  5. Although the risk of injury is small, if a person with Down's syndrome is involved in a road traffic accident personnel involved in their care should be alerted to the possibility of cervical spine instability and of the need for particular care relative to this (1,5).
  6. If a person with Down's syndrome develops pain behind the ear or elsewhere in the neck, abnormal head posture, torticollis, deterioration of gait, manipulative skills, or bowel and/or bladder control they should be referred immediately to an appropriate specialist (usually a neurologist or a spinal orthopaedic surgeon).

References - Cervical spine instability:

  1. Davidson,RG.,(1988). Atlantoaxial Instability in Individuals With Down Syndrome: A Fresh Look at the Evidence. Pediatrics: 81: 857-865
  2. Saad,KFG., (1995) A lethal case of atlantoaxial dislocation in a 56-year-old woman with Down's syndrome. J.Intellectual. Disability Research. 39: 447-449
  3. Selby,KA.,Newton,RW.,Gupta,S.,Hunt,L. (1991) Clinical predictors and radiological reliability in atlantoaxial subluxation in Down's syndrome. Archives of Disease in Childhood. 66: 876-878.
  4. Cremers,MJG.,Ramos,L.,Bol,E.,van.Gijn,J. (1993) Radiological assessment of the atlantoaxial distance in Down's syndrome. Archives of Disease in Childhood. 69. 347-350.
  5. Morton,RE.,Ali Khan,M.,Murray-Leslie,C.,Elliott,S.(1995) Atlantoaxial instability in Down's syndrome: a five year follow up study plus Chapman,S.,Commentary. Archives of Disease in Childhood, 72: 115-119
  6. Department of Health.(1995). Cervical spine instability in people with Down syndrome. CMO Update 7.p4.
  7. American Academy of Paediatrics Committee on Genetics.(1994) Health Supervision for children with Down syndrome. Pediatrics 93: 855-859
  8. Cremers,MJG.,Bol,E.,de Roos,F.,van Gijn,J. (1993) Risk of sports activities in children with Down's syndrome and atlantoaxial instability. Lancet. 342: August 28th. 511-514.
  9. Casey,AT.,O’Brien,M.,Kumar,V.,Hayward,RD.,Crockard,HA. (1995) Don't twist my child’s head off : iatrogenic cervical dislocation. BMJ. 311: 4 November. p1212-1213.

Enquiries to:
Down’s Syndrome Medical Information Services
Children’s Centre, City Hospital Campus, Nottingham NG5 1PB. UK
Tel (0)115 962 7658. Ext 45667. (0)115 934 5502 (answerphone). Fax (0)115 962 7915
Email:info@dsmig.org.uk

This article was first published on the site in 2002.