Guidelines

Spasticity in children and young people with non-progressive brain disorders: summary of NICE guidance

BMJ 2012; 345 doi: http://dx.doi.org/10.1136/bmj.e4845 (Published 26 July 2012)
Cite this as: BMJ 2012;345:e4845

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We are grateful to Dr Crilly for his comments. There are differing opinions on the place of selective dorsal rhizotomy (SDR) in the management of spasticity in children. In a recent debate in the House of Commons SDR was strongly advocated, a speaker saying “At this time, medical evidence shows that SDR is more robust than any other treatment for cerebral palsy.”[1] Families have been going to the USA at considerable cost to access this treatment.

The guideline developers were acutely aware of the lack of high quality clinical evidence for SDR, particularly in relation to important functional benefits and long-term outcomes. Crilly cites a meta-analysis of three randomised controlled trials,[2] each of which was included in the developers’ deliberations. A recent prospective study also failed to demonstrate improved Gross Motor Function Classification System (GMFCS) scores during a 10 year follow-up.[3] However, the developers were aware of reports of children with spasticity experiencing sustained improvement in motor function following SDR. They believed that children whose walking was primarily impaired by spasticity rather than weakness, contractures or poor muscle control would have the potential to benefit. SDR is an irreversible procedure which could cause harm. The developers therefore recommended that teams offering this procedure should participate in a coordinated national agreed programme to collect information on short- and long-term outcomes on all patients assessed for SDR, whether or not performed. In addition they made a research recommendation for the urgent evaluation of long-term outcomes (including adverse effects) using a range of designs, including randomised controlled trials. A similar plea has been made by others.[4] The NHS can provide an ideal environment in which to carry out such research, and determine which children with spasticity may benefit from SDR to achieve improved participation and quality of life.

We also thank Willoughby and Graham for their comments regarding the need for early detection of hip dislocation in children with spasticity. The guideline developers recognised that this was of crucial importance. The guideline does not advise that the diagnosis of dislocation should depend on symptoms and physical signs, but it was of course considered important to make a recommendation regarding the recognition of such clinical manifestations. Specific recommendations are provided with regard to radiological monitoring in those at increased risk of dislocation, but it is important to strike a balance between the risk of delayed diagnosis and the hazards associated with increased radiation exposure. As Willoughby and Graham indicate, the guideline recommends that hip X-ray be offered at 24 months for all with bilateral cerebral palsy, but it does go further than this. It recommends that consideration be given to repeating the X-ray annually in those at GMFCS level III, IV or V. Additionally, it is recommended that consideration be given to repeating the hip X-ray sooner (after 6 months) in children and young people where the initial hip migration is greater than 30%, and then to consider repeating the hip X-ray every 6 months after this if the hip migration is increasing by more than 10 percentage points per year.

[1] Selective Dorsal Rhizotomy, Daily Hansard, 18 Apr 2012: Columns 474-482, http://www.publications.parliament.uk/pa/cm201212/cmhansrd/cm120418/debt...

[2] McLaughlin J, Bjornson K, Temkin N, Steinbok P, Wright V, Reiner A, Roberts T, Drake J, O'Donnell M, Rosenbaum P, Barber J, Ferrel A. Selective dorsal rhizotomy: meta-analysis of three randomized controlled trials. Dev Med Child Neurol. 2002;44(1):17-25

[3] Motor function after selective dorsal rhizotomy: a 10-year practice-based follow-up study Josenby AL, Wagner P, Jarnlo G-B, Westbom L, Nordmark E Dev Med Child Neurol 2012; 4(5):429-435

[4] McLaughlin J Motor function after dorsal rhizotomy Dev Med Child Neurol 2012; 54(5) 389-390

Competing interests: None declared

Moira A Mugglestone, Director of Guideline Development

Paul Eunson, M Stephen Murphy

National Collaborating Centre for Women's and Children's Health, 2-16 Goodge Street, London W1T 2QA

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Dear Sir

Re: Spasticity in children and young people with non-progressive brain disorders: summary of NICE guidance. BMJ 2012;345:e4845

We were surprised to note in the Practice Guideline by Mugglestone et al, the old myth that hip displacement in children and young people with non-progressive brain disorders should be diagnosed on the basis of symptoms or signs on physical examination (Box 1). By the time symptoms or signs are apparent, the vast majority of hips will have severe displacement and irreversible changes in the shape of the femoral head and/or acetabulum with extensive loss of cartilage i.e. premature degenerative arthritis.

We note that this summary of the NICE guidelines mentions “offering hip radiography to assess for hip displacement if there are clinical concerns about possible hip displacement or at 24 months in children with bilateral cerebral palsy”. However, this does not go far enough. The only way to reduce the severe long-term sequelae of neglected hip displacement in children with cerebral palsy and other non-progressive brain disorders is for regular radiographic surveillance and early intervention.[1,2] The risk of hip displacement according to GMFCS is now well known. From this, guidelines for regular clinical and radiographic surveillance can be established based on the patient’s specific risk.[3] For the majority of children who are non-ambulant, this involves radiographic examination every 6-12 months, beginning at 12-24 months of age and continuing to skeletal maturity.[1,2] The summary of the NICE guidelines fails to highlight this important aspect of surveillance. A single radiographic examination at 24 months would be falsely reassuring in many children and fail to reduce the preventable morbidity associated with neglected hip displacement.

References:
[1] Dobson F, Boyd RN, Parrott J, Nattrass GR, Graham HK. Hip surveillance in children with cerebral palsy: Impact on the surgical management of spastic hip disease. J Bone Joint Surg 2002;84-B;5:720-726

[2] Hagglund G, Andersson S, Duppe H, Lauge-Pedersen, Nordmark E, Westbom L. Prevention of dislocation of the hip in children with cerebral palsy. The first ten years of a population-based prevention programme. J Bone Joint Surg 2005;87-B:95-101.

[3] Soo B, Howard J, Boyd RN, Reid S, Lanigan A, Wolfe R, Reddihough D, Graham HK. Hip displacement in cerebral palsy: A population based study of incidence in relation to motor type, topographical distribution and gross motor function. J Bone Joint Surgery 2006;88-A:121-129

Competing interests: None declared

Kate L Willoughby, Physiotherapist

Prof H Kerr Graham

Orthopaedic Department, The Royal Children's Hospital, 50 Flemington Road, Parkville, Victoria, Australia 3052

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Dorsal rhizotomy is an experimental surgical procedure

The recent NICE guideline regarding the clinical management of children with cerebral palsy advises clinicians to consider the invasive and irreversible surgical procedure of selective dorsal rhizotomy (SDR) to improve walking.[1] Unfortunately this NICE guidance is not based on the best available evidence since it omits several important publications concerning the limited data concerning the benefits and harms of SDR.[2-4] If SDR were a new drug then it would not receive a license for use in children with cerebral palsy.

Only three small randomised clinical trials (RCTs) have assessed the effectiveness of SDR (combined with post-operative physiotherapy), compared to physiotherapy alone, for improving motor function. A meta-analysis of the pooled individual patient data from these RCTs (83 children; age range 3 to 7 years) was published in 2002 and reported that SDR increased ‘gross motor function measure’ (GMFM) by 4.5 (95%CI 2.1 to 6.9; calculated from the data provided) after 9-12 months.[2] However the clinical collaborators concluded that this demonstrated only a “small positive effect on motor function”.[2] This was because the trialists had agreed in advance a ‘minimal clinically important benefit’ (MCIB) of between 5 to 10 points for the primary outcome GMFM. The full NICE review notably fails to identify a MCIB for SDR.[1]

A systematic review (published June 2011) of adverse longer term outcomes (>=5 years) associated with SDR found only one small study (21 children) with an appropriate comparison group.[3] Six studies reported on the occurrence of spinal abnormalities and chronic back pain in children following SDR, but without appropriate comparison groups it was impossible to know if such problems were attributable to SDR.[3]

The published literature indicates that SDR remains an experimental surgical procedure which requires further evidence from a much larger RCT in order to establish its clinical effectiveness. If children, parents, clinicians and policymaker are not going to remain forever in the dark concerning the effectiveness of SDR, then clinicians must now insist on only referring potentially eligible children to centres which will randomise them into a definitive trial, and policymakers should only use public money to fund SDR within such a trial.

[1] Mugglestone MA, Eunson P, Murphy MS; on behalf of the Guideline Development Group. Spasticity in children and young people with non-progressive brain disorders: summary of NICE guidance.
BMJ. 2012;345:e4845. doi: 10.1136/bmj.e4845.

[2] McLaughlin J, Bjornson K, Temkin N, Steinbok P, Wright V, Reiner A, Roberts T, Drake J, O'Donnell M, Rosenbaum P, Barber J, Ferrel A. Selective dorsal rhizotomy: meta-analysis of three randomized controlled trials. Dev Med Child Neurol. 2002;44(1):17-25.

[3] Grunt S, Becher JG, Vermeulen RJ. Long-term outcome and adverse effects of selective dorsal rhizotomy in children with cerebral palsy: a systematic review. Dev Med Child Neurol. 2011;53(6):490-8. doi: 10.1111/j.1469-8749.2011.03912.x.

Competing interests: The author works for both Aberdeen University and NHS Grampian. He has previously been asked by NHS Grampian to review the evidence concerning the effectiveness of 'selective doral rhizotomy' (SDR) for children with cerebral palsy.

Michael A Crilly, Senior Lecturer in Clinical Epidemiology.

Aberdeen University Medical School., Polwarth Building, Aberdeen, AB25 2ZD.

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