Article Text

Download PDFPDF
Beware the syndromic spine
  1. Vivienne Campbell1,
  2. Jonathan Lucas2
  1. 1 Chailey Clinical, Sussex Community Foundation NHS Trust, Sussex, UK
  2. 2 Evelina London Childrens Hospital, Guys and St Thomas’ NHS Foundation Trust, London, UK
  1. Correspondence to Dr Vivienne Campbell, Chailey Clinical, Sussex Community Foundation NHS Trust, Brighton BN2 3EW, UK; vivienne.campbell{at}nhs.net

Statistics from Altmetric.com

‘Of all the challenges we have managed—her scoliosis appearing as we were discussing transition just seemed to come out of nowhere’. The comments of parents of a child with complex disability who developed a scoliosis in their late teens and benefited from surgical correction.

The management of scoliosis lies with our spinal and orthopaedic colleagues. Awareness of the presentation and characteristics of children and young people (CYP) who may develop scoliosis and what they may need if they do is very much with in the paediatric remit and the paper by Homans et al 1 adds considerably to this knowledge.

The authors present a large two centre prevalence study to explore the presence and characteristics of scoliosis in more than 1000 CYP with 22q11.2 deletion syndrome. Although the data were available to them, the authors did not include CYP who had additional diagnoses or were known to have congenital bone anomalies. For this reason, their data can be assumed to be as ‘pure’ as possible for this one genetic condition. There are a number of findings that are helpful to the paediatrician both for this condition and also to consider when managing children with disabilities.

  1. The curvatures in this group were progressive—less than 5% of children aged 6 years had a scoliosis, 19% of children aged 10–12 years and at least 48% aged 16–18 years had a scoliosis. This is a condition where the majority of scoliosis develops late in childhood.

  2. Within the group who developed a scoliosis the range of deformity varied considerably—from small curves which would be unlikely to cause difficulty to significant curves where spinal surgery was performed.

  3. The presence of congenital heart disease requiring thoracotomy or sternotomy did not …

View Full Text

Footnotes

  • Contributors Article drafted by VC with JL adding information and redrafting.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Commissioned; internally peer reviewed.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

Linked Articles