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Reading List: Surgery for ambulant children with cerebral palsy by Mr Robert Jeffery and Professor Jonathan Marsden

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Introduction

Like other children, those with cerebral palsy benefit from a varied, active lifestyle and medical management should be unobtrusive. Simple measures such as activities, muscles stretches and orthoses minimise the need for surgical intervention and drugs. Potential walking ability is predominantly determined by the nature of the damage to the brain. Lack of volition, strength and control are not amenable to limb surgery. Management decisions depend on understanding gait abnormalities, interpretation of the available evidence, the clinician’s philosophy of management and the families’ preferences. On-going multidisciplinary team work is required to manage muscle tone, prevent contractures and optimise the mechanical environment. Surgery may have a role in correcting established bony torsional abnormalities and joint contractures. Preparation and rehabilitation are essential for success. We have chosen a selection of recent papers from the Journal and presented them as for a journal club, with more questions than answers – let the authors speak for themselves!

Graham HK, Selber P. Musculoskeletal aspects of cerebral palsy. J Bone Joint Surg [Br] 2003;85-B:157-66.

Graham and Selber’s excellent and well-referenced review ends with a warning to orthopaedic surgeons to assemble evidence that operative intervention improves outcomes or to risk withdrawal of funding. The authors highlight the importance of weakness and loss of selective control in determining walking dysfunction and the possible role of strength training.


Management of abnormal muscle tone

Ramachandran M, Eastwood DM. Botulinum toxin and its orthopaedic applications. J Bone Joint Surg [Br] 2006;88-B:981-7.

Ramachandran and Eastwood present an overview of the mechanism of action of botulinum toxin, its effects and the uncertainties regarding its use. Intramuscular injections have been used in many sites and doses, sometimes outside the licensed indications. Check the data sheet of the preparation used in your hospital. Concerns were raised by two speakers at the meeting of the British Society of Children’s Orthopaedic Surgery in January 2007 about atrophic histological features after injection of botulinum toxin,1 but details have not yet been published and the significance of these findings is not clear.

Metaxiotis D, Wolf S, Doederlein L. Conversion of biarticular to monoarticular muscles as a component of multilevel surgery in spastic diplegia. J Bone Joint Surg [Br] 2004;86-B:102-09.

Metaxiotis et al examine the role of surgery in converting biarticular fast-twitch muscles, which are more susceptible to abnormal tone and shortening, to monoarticular muscles. The back-kneeing in some patients is worrying and the minimum follow-up was only two years. Do you think that the authors make the case for converting biarticular muscles to monoarticular ones as opposed to lengthening them? Are the primary outcome measures clearly identifiable? Can you separate the effect of the operations on the gastrocnemius and semitendinosus from the other soft tissue and bony operations performed? Where do you think muscle surgery fits in with use of physical treatments, selective dorsal rhizotomy, intrathecal baclofen and other drugs in the management of muscle tone?


Multilevel and bony surgery

Gough M, Schneider P, Shortland AP. The outcome of surgical intervention for early deformity in young ambulant children with bilateral spastic cerebral palsy. J Bone Joint Surg [Br] 2008;90-B:946-51.

Sutherland’s classic work on the maturation of gait2 is cited as a reason to delay surgery until after the age of seven. Conversely, children may learn to walk in a different way more easily if surgery is performed at a younger age. Healing and rehabilitation can be quicker, but recurrent deformity may be more common. Gough et al report the results of surgery in children aged seven or younger. Consider the control group, outcome measures, physiotherapy input and length of follow-up. What is the relationship of popliteal angle (fixed flexion of the knees with the hips flexed) to the minimum knee flexion in the stance phase of gait and pelvic tilt? What is the significance of the wide range of outcomes in the control group?

Khan MA. Outcome of single-event multilevel surgery in untreated cerebral palsy in a developing country. J Bone Joint Surg [Br] 2007;89-B:1088-91.

Khan’s work challenges the belief that it is futile to perform surgery if children cannot achieve sitting balance by the age of two and cannot walk by the time they are five. Only 21% of subjects underwent bony procedures. The figure seems low, especially when Gordon and Simkiss3 found that 60% of children who could not walk by the age of five developed hip subluxation. Do you think that this represents possible regional variation or a different management philosophy? Would instrumented gait analysis have altered the clinical decision making process? Do you consider the subsequent performance of femoral derotation osteotomies to represent a failure of the original surgery? Could the model of rehabilitation be replicated and funded in your healthcare environment?


Conclusion

The studies show some important methodological difficulties with studying this patient population, namely:

  • The difficulties of measuring the effect of one intervention when it is performed along side other interventions (eg as part of multi-level surgery.)
  • The difficulty in obtaining a control group and taking into account the effects of time and growth on the parameters of gait. One possibility is to compare two interventions.
  • The small numbers involved and the need to perform multi-centre trials.
  • The long follow up period required (and the difficulties obtaining funding for such long periods).
  • Defining the outcome measures used eg specific aspects of walking kinematics / kinetics/ global walking scales or measures of activity / participation. Recently, Viehweger et al4 describe a national attempt to agree a range of validated outcome measures from different domains including function, energy expenditure, gait analysis and quality of life. Interestingly, quality of life correlated least well with the other measures.

There is plenty of work to do!

References
1. Sewry C, Roberts A, Patrick J. The long term histological effects of botulinum toxin in gastrocnemius affected by cerebral palsy diplegia. [abstract] J Bone Joint Surg [Br] 2008;90-B(Suppl III):520.
2. Sutherland DH, Olshen R, Cooper L, Woo SL. The development of mature gait. J Bone Joint Surg [Am] 1980;62-A:336-53.
3. Gordon GS, Simkiss DE. A systematic review of the evidence for hip surveillance in children with cerebral palsy. J Bone Joint Surg [Br] 2006;88-B:1492-6.
4. Viehweger E, Haumont T, de Lattre C, et al. Multidimensional outcome assessment in cerebral palsy: is it feasible and relevant? J Pediatr Orthop 2008;28:576-83.

Further reading

Gage JR. Treatment of gait problems in cerebral palsy. Second Ed. Mac Keith Press 2004.

McNee AE, Will E, Lin JP, et al. The effect of serial casting on gait in children with cerebral palsy: preliminary results from a crossover trial. Gait Posture 2007;25:463-8.

Baker R, Jasinski M, Maciag-Tymecka I, et al. Botulinum toxin treatment of spasticity in diplegic cerebral palsy: a randomized, double-blind, placebo-controlled, dose-ranging study. Dev Med Child Neurol 2002;44:666-75.

Seniorou M, Thompson N, Harrington M, Theologis T. Recovery of muscle strength following multi-level orthopaedic surgery in diplegic cerebral palsy. Gait Posture 2007;26:475-81.


Robert Jeffery MA FRCS(orth)
Consultant Orthopaedic Surgeon
Derriford Hospital, Plymouth

Jonathan Marsden PhD MSc MCSP
Professor of Rehabilitation
Human Movement and Function Laboratory
School of Health Professions
Plymouth University

Correspondence to robert.jeffery{at}phnt.swest.nhs.uk

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Hip, Knee, Trauma, Upper limb, Foot & Ankle, Paediatrics, Oncology, Spine, Arthroplasty, General