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Beware the syndromic spine
  1. Vivienne Campbell1,
  2. Jonathan Lucas2
  1. 1Chailey Clinical, Sussex Community Foundation NHS Trust, Sussex, UK
  2. 2Evelina 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}

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‘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 al1 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 …

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