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In children with cerebral palsy, does spinal fusion surgery for scoliosis improve lung function?
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  1. Katherine Lehovsky
  1. West Hertfordshire Hospitals NHS Trust, Watford, UK
  1. Correspondence to Dr Katherine Lehovsky, West Hertfordshire Hospitals NHS Trust, Watford WD18 0HB, UK; katherine.lehovsky{at}nhs.net

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Scenario

While in a paediatric respiratory outpatient clinic, you meet a 12-year-old boy with Gross Motor Function Classification System Level IV cerebral palsy. He has a left-sided scoliosis (80° Cobb angle T11–L4) and is being considered for scoliosis correction surgery. You have been asked to see him for preoperative assessment of respiratory function. The consultant paediatrician comments that the aim of surgery is to improve quality of life; you wonder if there is evidence that it can also improve lung function?

Structured clinical question

In children with cerebral palsy (population), does spinal fusion surgery for scoliosis (intervention) improve lung function (outcome)?

Search

  • Secondary sources: 3 indirectly relevant.1 2

  • The Cochrane Library: (cerebral palsy AND scoliosis AND lung)—1 reference, 0 directly relevant to question.

  • PubMed: (cerebral palsy AND scoliosis) AND (lung OR respiratory)—44 references, 2 directly relevant to question.

  • National Health Service evidence: (cerebral palsy AND scoliosis AND lung)—21 references, 0 directly relevant to question.

Summary

See tables 1 and 2

Table 1

Directly relevant

Table 2

Indirectly relevant

Commentary

Respiratory problems are widely recognised as a common cause of hospital admission, morbidity and mortality in children with cerebral palsy. This emphasises the importance of actively addressing lung function in their management. Given that scoliosis has been shown to cause reduced chest compliance, intuitively one might expect that correction through surgery would improve lung function.3 However, there is very limited research available to address this question in patients with cerebral palsy with only two directly relevant papers. Goussard et al describe resolution of lung collapse caused by bronchial compression post-surgery. This single case of qualitative radiological improvement in lung volume is somewhat anecdotal and does not indicate if there is any global change in lung function.1 The second study is a cohort study of neuromuscular scoliosis which contains a subgroup of 17 patients with cerebral palsy, which did not find a decrease in annual incidence of pneumonia after surgery.2 This highlights the paucity of clinical data and draws attention to the need for further research to guide preoperative planning for this high-stakes surgery in a complex group of patients.

Broadening this search to include the effect of spinal fusion surgery on patients with other neuromuscular disorders, unfortunately, only yields limited data. However, there has been more extensive research in Duchenne muscular dystrophy (DMD) when compared with cerebral palsy. This is likely to be because patients with DMD are able to more reliably perform spirometry. The effect of spinal fusion on lung function in DMD remains controversial. There are conflicting reports with some authors claiming that pulmonary function continues to decline but at a decreased rate after surgery while others have found no such improvement.4–6

These results must be interpreted within the confines of their limited applicability to cerebral palsy. DMD is a progressive primary muscle disorder of genetic origin. In contrast, cerebral palsy arises due to a non-progressive injury of the central nervous system of multifactorial origin predominantly attributed to environmental factors. The distinct pathogenesis of these two conditions limits the construct validity of using patients with DMD as a model for cerebral palsy. In particular, DMD is recognised to have a progressive pathology hence a linear decline in lung function is predicted. This is unlikely to be representative of cerebral palsy which is a non-progressive condition. Furthermore, the use of spirometry as a metric in cerebral palsy is flawed by the inability to collect reliable data, hence it is arguable that an alternative measure should be used, such as number of hospital admissions or number of courses of antibiotics. These arguments point against extrapolation of data between distinct forms of neuromuscular scoliosis. Further observational studies of large cohort size are clearly necessary to draw an evidence-based conclusion as to the effect of spinal fusion surgery on lung function in children with cerebral palsy.

Clinical bottom line

  • There are insufficient data available to draw an evidence-based conclusion on the effect of spinal fusion surgery on lung function in cerebral palsy.

  • The risks and benefits of surgery should be weighed up on an individual patient basis, without including lung function as a proven benefit.

Ethics statements

Patient consent for publication

References

Footnotes

  • 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 Not commissioned; externally peer reviewed.

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