Treatment must be individually tailored for each patient and carried out in multidisciplinary clinics in a prioritised and coordinated manner.[20]Noritz G, Davidson L, Steingass K, et al. Providing a primary care medical home for children and youth with cerebral palsy. Pediatrics. 2022 Nov 21;e2022060055.
https://publications.aap.org/pediatrics/article/150/6/e2022060055/190094/Providing-a-Primary-Care-Medical-Home-for-Children
http://www.ncbi.nlm.nih.gov/pubmed/36404756?tool=bestpractice.com
[56]National Institute for Health and Care Excellence. Cerebral palsy in under 25s: assessment and management. January 2017 [internet publication].
https://www.nice.org.uk/guidance/ng62
[63]Novak I, Morgan C, Fahey M, et al. State of the Evidence Traffic Lights 2019: systematic review of interventions for preventing and treating children with cerebral palsy. Curr Neurol Neurosci Rep. 2020 Feb 21;20(2):3.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035308
http://www.ncbi.nlm.nih.gov/pubmed/32086598?tool=bestpractice.com
[106]National Institute for Health and Care Excellence. Cerebral palsy in adults. January 2019 [internet publication].
https://www.nice.org.uk/guidance/ng119
Treatment approaches differ geographically depending on expertise and resources. Patients with genetic syndromes or metabolic aetiologies are referred to geneticists for appropriate management.
One 2019 systematic review of interventions for children with CP (all types) provides a general 'traffic light' guideline to the effectiveness of commonly used interventions, based on literature review and using the GRADE system and the Evidence Alert Traffic Light System.[63]Novak I, Morgan C, Fahey M, et al. State of the Evidence Traffic Lights 2019: systematic review of interventions for preventing and treating children with cerebral palsy. Curr Neurol Neurosci Rep. 2020 Feb 21;20(2):3.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035308
http://www.ncbi.nlm.nih.gov/pubmed/32086598?tool=bestpractice.com
[107]Alonso-Coello P, Schünemann HJ, Moberg J, et al. GRADE Evidence to Decision (EtD) frameworks: a systematic and transparent approach to making well informed healthcare choices. 1: Introduction. BMJ. 2016 Jun 28;353:i2016.
https://www.bmj.com/content/353/bmj.i2016.long
http://www.ncbi.nlm.nih.gov/pubmed/27353417?tool=bestpractice.com
Green (probably effective) interventions include: botulinum toxin, casting, diazepam, hip surveillance, intrathecal baclofen, occupational therapy after botulinum toxin, selective dorsal rhizotomy (also known as selective posterior rhizotomy), and training-based interventions (e.g., bimanual training, constraint-induced movement therapy [CIMT], fitness training, goal-directed training, and treadmill training, among others).
Red (ineffective or harmful) interventions include: craniosacral therapy, hyperbaric oxygen, neurodevelopmental therapy (original passive format), and sensory integration.
Yellow (probably effective) interventions include, but are not limited to: acupuncture, glycopyrrolate, intramuscular alcohol or phenol injection, oral dantrolene or tizanidine, orthoses, physical activity, single-event multilevel surgery, taping, and virtual reality and gaming.
Realistic goals are established by assessing the patient’s physical and intellectual abilities and energy demands. In most cases the focus is on improving function, but in the most severe cases, such as spastic quadriplegia, it may relate to improving care and comfort. Communication and activities of daily living are the priority, while mobility and ambulation are addressed as the needs arise.
Pain is common in patients with CP (caused by the condition itself and/or some treatments), but some patients will have difficulty communicating that they are in pain. Therefore, pain should be assessed regularly and treated as appropriate.[20]Noritz G, Davidson L, Steingass K, et al. Providing a primary care medical home for children and youth with cerebral palsy. Pediatrics. 2022 Nov 21;e2022060055.
https://publications.aap.org/pediatrics/article/150/6/e2022060055/190094/Providing-a-Primary-Care-Medical-Home-for-Children
http://www.ncbi.nlm.nih.gov/pubmed/36404756?tool=bestpractice.com
[56]National Institute for Health and Care Excellence. Cerebral palsy in under 25s: assessment and management. January 2017 [internet publication].
https://www.nice.org.uk/guidance/ng62
[63]Novak I, Morgan C, Fahey M, et al. State of the Evidence Traffic Lights 2019: systematic review of interventions for preventing and treating children with cerebral palsy. Curr Neurol Neurosci Rep. 2020 Feb 21;20(2):3.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035308
http://www.ncbi.nlm.nih.gov/pubmed/32086598?tool=bestpractice.com
[106]National Institute for Health and Care Excellence. Cerebral palsy in adults. January 2019 [internet publication].
https://www.nice.org.uk/guidance/ng119
Early intervention services (0-3 years) include physiotherapy (PT), occupational therapy (OT), and speech therapy, and are directed towards educating and assisting the family in helping the child to achieve developmental milestones, feeding skills, and communication. During school years, treatment is directed at optimising mobility and independence through the use of adaptive equipment, activities of daily living training, strengthening of weak muscles, and stretching of spastic muscles. In later childhood, surgery may be necessary for correction of soft tissue and bony deformities. In adolescence and adulthood, treatment emphasis is on maintaining function, promoting a healthy lifestyle through the use of fitness and exercise, and preventing deformity.
The importance of considering the environment or context of interventions has been highlighted.[63]Novak I, Morgan C, Fahey M, et al. State of the Evidence Traffic Lights 2019: systematic review of interventions for preventing and treating children with cerebral palsy. Curr Neurol Neurosci Rep. 2020 Feb 21;20(2):3.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035308
http://www.ncbi.nlm.nih.gov/pubmed/32086598?tool=bestpractice.com
Context-focused therapy emphasises modification of the task or environment, including the use of compensatory movements, to achieve a functional task identified as a priority by the child and family. Compensatory strategies and environmental adaptations ought to be considered for every child in attempting to improve their overall function.[63]Novak I, Morgan C, Fahey M, et al. State of the Evidence Traffic Lights 2019: systematic review of interventions for preventing and treating children with cerebral palsy. Curr Neurol Neurosci Rep. 2020 Feb 21;20(2):3.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035308
http://www.ncbi.nlm.nih.gov/pubmed/32086598?tool=bestpractice.com
[108]Law MC, Darrah J, Pollock N, et al. Focus on function: a cluster, randomized controlled trial comparing child- versus context-focused intervention for young children with cerebral palsy. Dev Med Child Neurol. 2011 Jul;53(7):621-9.
https://onlinelibrary.wiley.com/doi/full/10.1111/j.1469-8749.2011.03962.x
http://www.ncbi.nlm.nih.gov/pubmed/21569012?tool=bestpractice.com
Spastic CP
This is the most common subgroup and is characterised by a velocity-dependent increase in tonic stretch reflexes, with exaggerated tendon jerks and clonus resulting from hyperexcitability of the stretch reflex.[3]Sanger TD, Delgado MR, Gaebler-Spira D, et al; Task Force on Childhood Motor Disorders. Classification and definition of disorders causing hypertonia in childhood. Pediatrics. 2003 Jan;111(1):e89-97.
http://pediatrics.aappublications.org/content/111/1/e89.full
http://www.ncbi.nlm.nih.gov/pubmed/12509602?tool=bestpractice.com
Spastic CP can be further classified as monoplegia, hemiplegia, diplegia, or quadriplegia.[4]Blair E, Stanley F. Interobserver agreement in the classification of cerebral palsy. Dev Med Child Neurol. 1985 Oct;27(5):615-22.
http://www.ncbi.nlm.nih.gov/pubmed/4065435?tool=bestpractice.com
Spasticity generally presents between ages 1 to 2 years, and manifests when the child attempts activities. Two issues of concern are the spasticity itself and the effects of the spasticity over time. Patients should receive PT, OT, speech therapy, orthoses, and adaptive equipment as required.
Pharmacological therapy
Oral pharmacological therapy (e.g., diazepam, tizanidine, dantrolene, baclofen) may have a role from mid-childhood onwards for significant spasticity interfering with function, and is the first line of treatment in patients with spastic diplegia and quadriplegia.[109]Delgado MR, Hirtz D, Aisen M, et al; Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Practice parameter: pharmacologic treatment of spasticity in children and adolescents with cerebral palsy (an evidence-based review). Neurology. 2010 Jan 26;74(4):336-43.
http://n.neurology.org/content/74/4/336.full
http://www.ncbi.nlm.nih.gov/pubmed/20101040?tool=bestpractice.com
It is not used for the management of mild spastic CP (e.g., gross motor function classification system [GMFCS] level I, II, or possibly III). Oral pharmacological therapy is effective in some patients, but adverse effects such as lethargy may limit tolerability.
Injectable therapy for spasticity includes botulinum toxin type A.[63]Novak I, Morgan C, Fahey M, et al. State of the Evidence Traffic Lights 2019: systematic review of interventions for preventing and treating children with cerebral palsy. Curr Neurol Neurosci Rep. 2020 Feb 21;20(2):3.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035308
http://www.ncbi.nlm.nih.gov/pubmed/32086598?tool=bestpractice.com
[109]Delgado MR, Hirtz D, Aisen M, et al; Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Practice parameter: pharmacologic treatment of spasticity in children and adolescents with cerebral palsy (an evidence-based review). Neurology. 2010 Jan 26;74(4):336-43.
http://n.neurology.org/content/74/4/336.full
http://www.ncbi.nlm.nih.gov/pubmed/20101040?tool=bestpractice.com
[110]Heinen F, Desloovere K, Schroeder AF, et al. The updated European Consensus 2009 on the use of botulinum toxin for children with cerebral palsy. Eur J Paediatr Neurol. 2010 Jan;14(1):45-66.
http://www.ncbi.nlm.nih.gov/pubmed/19914110?tool=bestpractice.com
[111]Love SC, Novak I, Kentish M, et al. Botulinum toxin assessment, intervention and after-care for lower limb spasticity in children with cerebral palsy: international consensus statement. Eur J Neurol. 2010 Aug;17 suppl 2:9-37.
http://www.ncbi.nlm.nih.gov/pubmed/20633177?tool=bestpractice.com
[112]Hoare BJ, Wallen MA, Imms C, et al. Botulinum toxin A as an adjunct to treatment in the management of the upper limb in children with spastic cerebral palsy (UPDATE). Cochrane Database Syst Rev. 2010 Jan 20;(1):CD003469.
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD003469.pub4/full
http://www.ncbi.nlm.nih.gov/pubmed/20091546?tool=bestpractice.com
[113]Blumetti FC, Belloti JC, Tamaoki MJ, et al. Botulinum toxin type A in the treatment of lower limb spasticity in children with cerebral palsy. Cochrane Database Syst Rev. 2019 Oct 8;(10):CD001408.
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD001408.pub2/full
http://www.ncbi.nlm.nih.gov/pubmed/31591703?tool=bestpractice.com
Phenol or ethyl alcohol may be used for severe spasticity if botulinum toxin is not available.[63]Novak I, Morgan C, Fahey M, et al. State of the Evidence Traffic Lights 2019: systematic review of interventions for preventing and treating children with cerebral palsy. Curr Neurol Neurosci Rep. 2020 Feb 21;20(2):3.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035308
http://www.ncbi.nlm.nih.gov/pubmed/32086598?tool=bestpractice.com
[114]Zafonte RD, Munin MC. Phenol and alcohol blocks for the treatment of spasticity. Phys Med Rehabil Clin N Am. 2001 Nov;12(4):817-32.
http://www.ncbi.nlm.nih.gov/pubmed/11723866?tool=bestpractice.com
An evidence-based review of pharmacological treatment for spasticity can be found in a practice parameter of the American Academy of Neurology.[109]Delgado MR, Hirtz D, Aisen M, et al; Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Practice parameter: pharmacologic treatment of spasticity in children and adolescents with cerebral palsy (an evidence-based review). Neurology. 2010 Jan 26;74(4):336-43.
http://n.neurology.org/content/74/4/336.full
http://www.ncbi.nlm.nih.gov/pubmed/20101040?tool=bestpractice.com
Neurosurgical procedures
Severe spasticity may be ameliorated by intrathecal baclofen delivered via an implantable programmable battery powered pump or selective dorsal rhizotomy.[63]Novak I, Morgan C, Fahey M, et al. State of the Evidence Traffic Lights 2019: systematic review of interventions for preventing and treating children with cerebral palsy. Curr Neurol Neurosci Rep. 2020 Feb 21;20(2):3.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035308
http://www.ncbi.nlm.nih.gov/pubmed/32086598?tool=bestpractice.com
[115]McLaughlin M, Bjornson K, Temkin N, et al. Selective dorsal rhizotomy: meta-analysis of three randomized controlled trials. Dev Med Child Neurol. 2002 Jan;44(1):17-25.
http://www.ncbi.nlm.nih.gov/pubmed/11811645?tool=bestpractice.com
[116]Daunter AK, Kratz AL, Hurvitz EA. Long-term impact of childhood selective dorsal rhizotomy on pain, fatigue, and function: a case-control study. Dev Med Child Neurol. 2017 Jun 15;59(10):1089-95.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5610610
http://www.ncbi.nlm.nih.gov/pubmed/28617943?tool=bestpractice.com
[117]Buizer AI, Martens BHM, Grandbois van Ravenhorst C, et al. Effect of continuous intrathecal baclofen therapy in children: a systematic review. Dev Med Child Neurol. 2019 Feb;61(2):128-34.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7379262
http://www.ncbi.nlm.nih.gov/pubmed/30187921?tool=bestpractice.com
[118]Hasnat MJ, Rice JE. Intrathecal baclofen for treating spasticity in children with cerebral palsy. Cochrane Database Syst Rev. 2015 Nov 13;(11):CD004552.
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD004552.pub2/full
http://www.ncbi.nlm.nih.gov/pubmed/26563961?tool=bestpractice.com
Managing deformity and contractures
Spasticity that interferes with walking may be managed by an orthosis to correct the gait deviation. Spasticity that is not well managed may develop into a fixed contracture.
Contractures are managed by stretching, casting, and ongoing intensive PT.[119]Blackmore AM, Boettcher-Hunt E, Jordan M, et al. A systematic review of the effects of casting on equinus in children with cerebral palsy: an evidence report of the AACPDM. Dev Med Child Neurol. 2007 Oct;49(10):781-90.
http://www.ncbi.nlm.nih.gov/pubmed/17880650?tool=bestpractice.com
[120]Halanski M, Noonan KJ. Cast and splint immobilization: complications. J Am Acad Orthop Surg. 2008 Jan;16(1):30-40.
http://www.ncbi.nlm.nih.gov/pubmed/18180390?tool=bestpractice.com
For resistant contractures or bony deformity, orthopaedic surgery may be necessary.
Dyskinetic CP
Patients present with involuntary, recurring, and occasionally stereotyped movements with a varying muscle tone. Subgroups include dystonia, chorea, and athetosis.
All patients receive PT, OT, speech therapy, orthoses, and adaptive equipment as required.
With dystonia
A therapeutic trial of carbidopa/levodopa may be considered to assess for dystonia due to dopamine-responsive dystonia (also known as Segawa's disease), in which patients show a dramatic response. In many cases, full physical functionality including walking, running, speaking, and writing is restored or preserved.
Diagnosis of dystonia is not made by one definitive test, but by a series of clinical observations and specific biochemical assessments. Defining the exact cause may not be possible.
Dystonia Medical Research Foundation: dopa-responsive dystonia
Opens in new window
If there is a modest response to carbidopa/levodopa, it can be continued and combined with oral medications such as diazepam or trihexyphenidyl.
Non-responsiveness to carbidopa/levodopa does not predict response to the other medications, and an attempt to treat the dystonia on a chronic basis with alternative oral medications should be considered. The drugs may diminish the dyskinesia in selected patients. It is possible to combine drugs to enhance the therapeutic response, while reducing or minimising adverse effects.
One systematic review concluded that trihexyphenidyl is possibly ineffective in reducing dystonia, improving motor function, and easing care-giving in patients with dystonia in CP, and that there is no evidence on the effectiveness of benzodiazepines.[121]Fehlings D, Brown L, Harvey A, et al. Pharmacological and neurosurgical interventions for managing dystonia in cerebral palsy: a systematic review. Dev Med Child Neurol. 2018 Apr;60(4):356-66.
https://onlinelibrary.wiley.com/doi/10.1111/dmcn.13652
http://www.ncbi.nlm.nih.gov/pubmed/29405267?tool=bestpractice.com
One Cochrane review concluded that there is insufficient evidence regarding the effectiveness of trihexyphenidyl for patients with dystonic CP.[122]Harvey AR, Baker LB, Reddihough DS, et al. Trihexyphenidyl for dystonia in cerebral palsy. Cochrane Database Syst Rev. 2018 May 15;(5):CD012430.
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD012430.pub2/full
http://www.ncbi.nlm.nih.gov/pubmed/29763510?tool=bestpractice.com
Injection of botulinum toxin type A, targeted to individual muscles to lessen the force and frequency of dystonic contractions, may be trialled to improve function (e.g., accessing a switch for wheelchair mobility or computer communications) or to facilitate care-giving (e.g., dressing, changing nappies). One systematic review concluded that there is limited evidence on the effectiveness of botulinum toxin for dystonia in CP, but some trials show promising results for the treatment of the upper extremity and cervical muscles for patients with dystonic CP.[121]Fehlings D, Brown L, Harvey A, et al. Pharmacological and neurosurgical interventions for managing dystonia in cerebral palsy: a systematic review. Dev Med Child Neurol. 2018 Apr;60(4):356-66.
https://onlinelibrary.wiley.com/doi/10.1111/dmcn.13652
http://www.ncbi.nlm.nih.gov/pubmed/29405267?tool=bestpractice.com
[123]Sanger TD, Kukke SN, Sherman-Levine S. Botulinum toxin type B improves the speed of reaching in children with cerebral palsy and arm dystonia: an open-label, dose-escalation pilot study. J Child Neurol. 2007 Jan;22(1):116-22.
http://www.ncbi.nlm.nih.gov/pubmed/17608320?tool=bestpractice.com
[124]Yi YG, Kim K, Yi Y, et al. Botulinum toxin type A injection for cervical dystonia in adults with dyskinetic cerebral palsy. Toxins (Basel). 2018 May 16;10(5):203.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5983259
http://www.ncbi.nlm.nih.gov/pubmed/29772695?tool=bestpractice.com
Neurosurgical treatment is indicated for selected patients after less invasive options have been tried. For example:
An intrathecal baclofen pump may be used to reduce dystonia in patients with CP who respond to test dosing.[121]Fehlings D, Brown L, Harvey A, et al. Pharmacological and neurosurgical interventions for managing dystonia in cerebral palsy: a systematic review. Dev Med Child Neurol. 2018 Apr;60(4):356-66.
https://onlinelibrary.wiley.com/doi/10.1111/dmcn.13652
http://www.ncbi.nlm.nih.gov/pubmed/29405267?tool=bestpractice.com
[125]Albright AL, Barry MJ, et.al. Intrathecal baclofen for generalized dystonia. Dev Med Child Neurol. 2001 Oct;43(10):652-7.
http://www.ncbi.nlm.nih.gov/pubmed/11665821?tool=bestpractice.com
Studies on the effect of intrathecal baclofen in children and young adults with dyskinetic CP (GMFCS levels IV and V) found improvements in sitting, communication, fine motor skills, and goal attainment scaling of individual treatment goals.[126]Eek MN, Olsson K, Lindh K, et al. Intrathecal baclofen in dyskinetic cerebral palsy: effects on function and activity. Dev Med Child Neurol. 2017 Nov 17;60(1):94-9.
http://www.ncbi.nlm.nih.gov/pubmed/29148568?tool=bestpractice.com
[127]Bonouvrié LA, Becher JG, Vles JSH, et al. The effect of intrathecal baclofen in dyskinetic cerebral palsy: the IDYS trial. Ann Neurol. 2019 Jul;86(1):79-90.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6617761
http://www.ncbi.nlm.nih.gov/pubmed/31050023?tool=bestpractice.com
Deep brain stimulation, which is used for patients with primary (or genetic) dystonia, may also be a potential treatment in secondary dystonia associated with CP.[121]Fehlings D, Brown L, Harvey A, et al. Pharmacological and neurosurgical interventions for managing dystonia in cerebral palsy: a systematic review. Dev Med Child Neurol. 2018 Apr;60(4):356-66.
https://onlinelibrary.wiley.com/doi/10.1111/dmcn.13652
http://www.ncbi.nlm.nih.gov/pubmed/29405267?tool=bestpractice.com
[128]Sanger TD. Deep brain stimulation for cerebral palsy: where are we now? Dev Med Child Neurol. 2020 Jan;62(1):28-33.
https://onlinelibrary.wiley.com/doi/10.1111/dmcn.14295
http://www.ncbi.nlm.nih.gov/pubmed/31211420?tool=bestpractice.com
[129]Krauss JK, Loher TJ, Weigel R, et al. Chronic stimulation of the globus pallidus internus for treatment of non-dYT1 generalized dystonia and choreoathetosis: 2-year follow up. J Neurosurg. 2003 Apr;98(4):785-92.
http://www.ncbi.nlm.nih.gov/pubmed/12691403?tool=bestpractice.com
With athetosis
Oral medications such as levodopa, diazepam, dantrolene, and tetrabenazine may be tried, although evidence for efficacy is limited.[130]Masson R, Pagliano E, Baranello G. Efficacy of oral pharmacological treatments in dyskinetic cerebral palsy: a systematic review. Dev Med Child Neurol. 2017 Dec;59(12):1237-48.
https://onlinelibrary.wiley.com/doi/10.1111/dmcn.13532
http://www.ncbi.nlm.nih.gov/pubmed/28872668?tool=bestpractice.com
Ataxic CP
Patients present with loss of muscular coordination with abnormal force and rhythm, and impairment of accuracy.
There is no specific treatment for ataxic CP. All patients receive PT, OT, speech therapy, orthoses, and adaptive equipment as required. Treatment is directed towards strengthening of weak muscles (e.g., the proximal musculature of the pelvis, scapula, and trunk) and coordination and balance activities to improve the patient's innate balance.
Pharmacotherapy may sometimes be used to treat accompanying symptoms such as tremor. Emotional support and medication to treat any associated depression should also be considered.
Mixed CP
Most patients with CP have a predominant motion disorder but when that cannot be determined, patients are classified as having a mixed-type CP.
All patients receive PT, OT, speech therapy, orthoses, and adaptive equipment as required. Pharmacological treatments appropriate for both spasticity and dyskinesia may be used; however, diminution of one type may unmask or appear to amplify the other. This may be seen following treatments to decrease spasticity, such as oral baclofen or botulinum toxin, when an increase in dystonic posturing may be observed.
Occupational therapy (OT), physiotherapy (PT), and speech therapy
Children should begin therapy as young as possible (early intervention is 0-3 years) while the clinical picture is evolving and parents are learning about their child's needs and abilities. Therapy should be directed towards facilitating the child's strengths in order to optimise independence and participation in school, work, and the community.
The American College of Sports Medicine has published exercise guidelines for children, adolescents, and adults with CP.[131]Verschuren O, Peterson MD, Balemans AC, et al. Exercise and physical activity recommendations for people with cerebral palsy. Dev Med Child Neurol. 2016 Aug;58(8):798-808.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4942358
http://www.ncbi.nlm.nih.gov/pubmed/26853808?tool=bestpractice.com
Occupational therapy and physiotherapy: children
All children with CP require OT and PT, but it is particularly important in those with spasticity. Therapy is directed towards strengthening of weak muscles, stretching of shortened muscles, and coordination activities, with an emphasis on bimanual upper-extremity activities and symmetry of gait and posture.[132]Gordon AM, Schneider JA, Chinnan A, et al. Efficacy of a hand-arm bimanual intensive therapy (HABIT) in children with hemiplegic cerebral palsy: a randomized control trial. Dev Med Child Neurol. 2007 Nov;49(11):830-8.
http://www.ncbi.nlm.nih.gov/pubmed/17979861?tool=bestpractice.com
This is combined with positioning, adaptive equipment, and splinting.
Therapies are tailored to the individual patient. Evidence supports the use of training-based interventions, such as bimanual training, CIMT, goal-directed training, mobility training, and treadmill training.[63]Novak I, Morgan C, Fahey M, et al. State of the Evidence Traffic Lights 2019: systematic review of interventions for preventing and treating children with cerebral palsy. Curr Neurol Neurosci Rep. 2020 Feb 21;20(2):3.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035308
http://www.ncbi.nlm.nih.gov/pubmed/32086598?tool=bestpractice.com
Bottom-up, generic, and/or passive motor interventions (e.g., neurodevelopmental treatment) are less effective or ineffective.[63]Novak I, Morgan C, Fahey M, et al. State of the Evidence Traffic Lights 2019: systematic review of interventions for preventing and treating children with cerebral palsy. Curr Neurol Neurosci Rep. 2020 Feb 21;20(2):3.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035308
http://www.ncbi.nlm.nih.gov/pubmed/32086598?tool=bestpractice.com
The emphasis is on practising real-life tasks and activities, using active movements, targeted at achieving an identified goal.
There is some evidence that exercise interventions and fitness programmes have positive effects on function, social participation, self-perception, and quality of life.[133]Fowler EG, Kolobe TH, Damiano DL, et al; Section on Pediatrics Research Summit Participants; Section on Pediatrics Research Committee Task Force. Promotion of physical fitness and prevention of secondary conditions for children with cerebral palsy: section on pediatrics research summit proceedings. Phys Ther. 2007 Nov;87(11):1495-510.
https://academic.oup.com/ptj/article/87/11/1495/2742266
http://www.ncbi.nlm.nih.gov/pubmed/17895351?tool=bestpractice.com
[134]Demuth SK, Knutson LM, Fowler EG. The PEDALS stationary cycling intervention and health-related quality of life in children with cerebral palsy: a randomized controlled trial. Dev Med Child Neurol. 2012 Jul;54(7):654-61.
http://www.ncbi.nlm.nih.gov/pubmed/22582760?tool=bestpractice.com
[135]Franki I, Desloovere K, De Cat J, et al. The evidence-base for conceptual approaches and additional therapies targeting lower limb function in children with cerebral palsy: a systematic review using the ICF as a framework. J Rehabil Med. 2012 May;44(5):396-405.
http://www.ncbi.nlm.nih.gov/pubmed/22549647?tool=bestpractice.com
[136]Ryan JM, Cassidy EE, Noorduyn SG, et al. Exercise interventions for cerebral palsy. Cochrane Database Syst Rev. 2017 Jun 11;(6):CD011660.
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD011660.pub2/full
http://www.ncbi.nlm.nih.gov/pubmed/28602046?tool=bestpractice.com
Even in children who are non-ambulatory, as little as 6 weeks of exercise intervention may improve gross motor function.[137]Bryant E, Pountney T, Williams H, et al. Can a six-week exercise intervention improve gross motor function for non-ambulant children with cerebral palsy? A pilot randomized controlled trial. Clin Rehabil. 2013 Feb;27(2):150-9.
http://www.ncbi.nlm.nih.gov/pubmed/22850757?tool=bestpractice.com
CIMT is a treatment approach for children with hemiplegic CP that involves restraining the use of the less affected limb (e.g., using a splint, mitt, or sling) and intensive therapeutic practice of the more affected limb. One Cochrane review concluded that CIMT was more effective at improving bimanual performance and unimanual capacity in children with hemiplagic CP than standard care or other low-intensity interventions (e.g., OT), and was as effective as intensive or dose-matched comparators (e.g., individualised OT, bimanual therapy).[138]Hoare BJ, Wallen MA, Thorley MN, et al. Constraint-induced movement therapy in children with unilateral cerebral palsy. Cochrane Database Syst Rev. 2019 Apr 1;(4):CD004149.
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD004149.pub3/full
http://www.ncbi.nlm.nih.gov/pubmed/30932166?tool=bestpractice.com
Therapy combining soft constraint, bimanual play, reach training with a sticky mitten, graduated motor-sensory training, and parent education was effective in children aged under 2 years with asymmetric CP. These therapeutic approaches avoid sensory deprivation that may occur in other forms of constraint therapy.[139]Maitre NL, Jeanvoine A, Yoder PJ, et al; BBOP Group. Kinematic and somatosensory gains in infants with cerebral palsy after a multi-component upper-extremity intervention: a randomized controlled trial. Brain Topogr. 2020 Nov;33(6):751-66.
http://www.ncbi.nlm.nih.gov/pubmed/32748303?tool=bestpractice.com
[140]Chorna O, Heathcock J, Key A, et al. Early childhood constraint therapy for sensory/motor impairment in cerebral palsy: a randomised clinical trial protocol. BMJ Open. 2015 Dec 7;5(12):e010212.
https://bmjopen.bmj.com/content/5/12/e010212.long
http://www.ncbi.nlm.nih.gov/pubmed/26644127?tool=bestpractice.com
Mechanically assisted walking training aims to help children with CP to walk further by using equipment such as a treadmill, a gait trainer (a wheeled walking aid), or a robotic training device. One Cochrane review concluded that mechanically assisted walking training probably improves walking speed (with or without body weight support) and may improve motor function (with body weight support). There was some evidence of a greater beneficial effect of such training without body weight support compared with overground walking for improving speed and function. However, the clinical significance of the results is unclear.[141]Chiu HC, Ada L, Bania TA. Mechanically assisted walking training for walking, participation, and quality of life in children with cerebral palsy. Cochrane Database Syst Rev. 2020 Nov 18;(11):CD013114.
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD013114.pub2/full
http://www.ncbi.nlm.nih.gov/pubmed/33202482?tool=bestpractice.com
Occupational therapy and physiotherapy: adolescents and adults
In adolescents and adults with CP, ongoing PT and OT are rare except following an intervention, but should be available if required for patients with complex needs.[106]National Institute for Health and Care Excellence. Cerebral palsy in adults. January 2019 [internet publication].
https://www.nice.org.uk/guidance/ng119
A home exercise programme and healthy lifestyle that includes an exercise regimen of stretching, strengthening, and endurance is recommended.[106]National Institute for Health and Care Excellence. Cerebral palsy in adults. January 2019 [internet publication].
https://www.nice.org.uk/guidance/ng119
[142]Rogers A, Furler BL, Brinks S, et al. A systematic review of the effectiveness of aerobic exercise interventions for children with cerebral palsy: an AACPDM evidence report. Dev Med Child Neurol. 2008 Nov;50(11):808-14.
https://onlinelibrary.wiley.com/doi/full/10.1111/j.1469-8749.2008.03134.x
http://www.ncbi.nlm.nih.gov/pubmed/18811714?tool=bestpractice.com
[Evidence C]4408c6cd-9ac0-4bfd-b6d4-d095bc6a37baguidelineCWhat are the effects of physical activity, physiotherapy, or occupational therapy to improve or maintain physical function in adults with cerebral palsy?[106]National Institute for Health and Care Excellence. Cerebral palsy in adults. January 2019 [internet publication].
https://www.nice.org.uk/guidance/ng119
One study suggested that a lifestyle intervention programme including physical fitness, counselling focused on physical behaviour, and sports participation over a 6-month period could improve quality of life in adolescents and young adults, as well as decrease fatigue and bodily pain.[143]Slaman J, van den Berg-Emons HJ, van Meeteren J, et al. A lifestyle intervention improves fatigue, mental health and social support among adolescents and young adults with cerebral palsy: focus on mediating effects. Clin Rehabil. 2015 Jul;29(7):717-27.
http://www.ncbi.nlm.nih.gov/pubmed/25352613?tool=bestpractice.com
Another study showed no lasting effect of a lifestyle intervention programme on physical activity.[144]Slaman J, Roebroeck M, Dallmijer A, et al. Can a lifestyle intervention programme improve physical behaviour among adolescents and young adults with spastic cerebral palsy? A randomized controlled trial. Dev Med Child Neurol. 2015 Feb;57(2):159-66.
https://onlinelibrary.wiley.com/doi/full/10.1111/dmcn.12602
http://www.ncbi.nlm.nih.gov/pubmed/25303096?tool=bestpractice.com
In one European multi-centre study, self-reporting patients noted a similar quality of life in children and adolescents with and without CP, but adolescents with CP had significantly lower scores in social support and peer relationships.[145]Colver A, Rapp M, Eisemann N, et al. Self-reported quality of life of adolescents with cerebral palsy: a cross-sectional and longitudinal analysis. Lancet. 2015 Feb 21;385(9969):705-16.
https://www.thelancet.com/journals/lancet/article/PIIS0140-6736(14)61229-0/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/25301503?tool=bestpractice.com
[146]Michelsen SI, Flachs EM, Damsgaard MT, et al. European study of frequency of participation of adolescents with and without cerebral palsy. Eur J Paediatr Neurol. 2014 May;18(3):282-94.
https://www.ejpn-journal.com/article/S1090-3798%2813%2900206-7/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/24412031?tool=bestpractice.com
Elevated blood pressure has been linked to a sedentary lifestyle in children and adolescents with CP.[147]Ryan JM, Hensey O, McLoughlin B, et al. Reduced moderate-to-vigorous physical activity and increased sedentary behavior are associated with elevated blood pressure values in children with cerebral palsy. Phys Ther. 2014 Aug;94(8):1144-53.
https://academic.oup.com/ptj/article/94/8/1144/2735645
http://www.ncbi.nlm.nih.gov/pubmed/24700137?tool=bestpractice.com
Speech therapy
Speech therapy is directed towards educating and assisting the family in helping the child achieve developmental milestones and overcome communication difficulties, which may be underestimated because of the severity of motor disability.
Orthoses
Orthoses may be used to improve function and maintain range of motion during walking or upper-extremity activities, particularly in patients with spastic CP.[148]Lintanf M, Bourseul JS, Houx L, et al. Effect of ankle-foot orthoses on gait, balance and gross motor function in children with cerebral palsy: a systematic review and meta-analysis. Clin Rehabil. 2018 Sep;32(9):1175-88.
https://www.doi.org/10.1177/0269215518771824
http://www.ncbi.nlm.nih.gov/pubmed/29714066?tool=bestpractice.com
[149]Ries AJ, Novacheck TF, Schwartz MH. The efficacy of ankle-foot orthoses on improving the gait of children with diplegic cerebral palsy: a multiple outcome analysis. PM R. 2015 Sep;7(9):922-9.
http://www.ncbi.nlm.nih.gov/pubmed/25771349?tool=bestpractice.com
[150]Jackman M, Novak I, Lannin N. Effectiveness of hand splints in children with cerebral palsy: a systematic review with meta-analysis. Dev Med Child Neurol. 2014 Feb;56(2):138-47.
https://onlinelibrary.wiley.com/doi/10.1111/dmcn.12205
http://www.ncbi.nlm.nih.gov/pubmed/23848480?tool=bestpractice.com
[151]Betancourt JP, Eleeh P, Stark S, et al. Impact of ankle-foot orthosis on gait efficiency in ambulatory children with cerebral palsy: a systematic review and meta-analysis. Am J Phys Med Rehabil. 2019 Sep;98(9):759-70.
http://www.ncbi.nlm.nih.gov/pubmed/30920399?tool=bestpractice.com
Casting is used to correct deformity, while splinting is used to maintain correction and prevent recurrence. Bracing and casting are also used to enhance function and to address any contractures that develop.
Care must be taken to determine the specific impairment causing the gait deviation to ensure the most appropriate form of orthosis is used.
Excessive plantar flexion in patients with spastic hemiplegia may require an ankle-foot orthosis to assist with dorsiflexion during swing or to prevent excessive plantar flexion during stance. One systematic review concluded that ankle-foot orthoses improved step length and dorsiflexion angle in children with CP; step length is a key component of walking speed.[151]Betancourt JP, Eleeh P, Stark S, et al. Impact of ankle-foot orthosis on gait efficiency in ambulatory children with cerebral palsy: a systematic review and meta-analysis. Am J Phys Med Rehabil. 2019 Sep;98(9):759-70.
http://www.ncbi.nlm.nih.gov/pubmed/30920399?tool=bestpractice.com
In patients with spastic diplegia, orthoses that limit dorsiflexion may improve stability when plantar flexor weakness is the primary cause; however, remaining contributors such as hip or knee flexion contractures and tight hamstrings creating a crouched gait must also be addressed.
Lower-extremity orthoses have not been shown to be effective or well tolerated in patients with ataxic CP, and in some cases their use may limit the patient's ability to compensate distally for proximal impairment.
Adaptive equipment
Therapy is directed at providing patients with necessary equipment or aids depending on their physical abilities.
Walking aids
Aids for walking include braces, crutches, canes, and walkers. Patients with functional upper extremities who require assistance for balance and stability may use a standard or reverse walker with wheels. Those with poor trunk control or persistent scissoring may use a gait trainer, which also provides trunk and pelvic stability. Children with difficulty advancing the limbs during swing may use a reciprocating gait orthosis. Gait trainers and reciprocating orthoses are typically used with assistance for therapeutic purposes and are not considered independent mobility aids.
Mobility aids for non-ambulatory patients and those with walking limitations
Specially adapted scooters, tricycles, and automobiles, as well as positioning equipment for electric and non-electrically driven wheelchairs, may be used.
One study of 858 children with CP aged 0 to 11 years demonstrated that only 10% of children self-propelled their manual wheelchairs, regardless of age, motor function, range of motion or manual ability, while 90% were pushed. In contrast, 75% of 166 children using power mobility outdoors were independent.[152]Rodby-Bousquet E, Paleg G, Casey J, et al. Physical risk factors influencing wheeled mobility in children with cerebral palsy: a cross-sectional study. BMC Pediatr. 2016 Oct 10;16(1):165.
https://bmcpediatr.biomedcentral.com/articles/10.1186/s12887-016-0707-6
http://www.ncbi.nlm.nih.gov/pubmed/27724880?tool=bestpractice.com
This emphasises the need to consider power mobility at an early age to promote independence for children with CP.
Communication aids
Adaptive technology both to enhance communication and to enable access to computers should be provided as needed.
Appropriate augmentative and alternative communication systems should be made available to children at an early age if required. Provision should be re-assessed regularly to ensure that aids are still appropriate as the patient’s needs and abilities change.[56]National Institute for Health and Care Excellence. Cerebral palsy in under 25s: assessment and management. January 2017 [internet publication].
https://www.nice.org.uk/guidance/ng62
[153]Kristoffersson E, Dahlgren Sandberg A, Holck P. Communication ability and communication methods in children with cerebral palsy Dev Med Child Neurol. 2020 Aug;62(8):933-8.
https://onlinelibrary.wiley.com/doi/10.1111/dmcn.14546
http://www.ncbi.nlm.nih.gov/pubmed/32281100?tool=bestpractice.com
[154]Light J, McNaughton D, Beukelman D, et al. Challenges and opportunities in augmentative and alternative communication: research and technology development to enhance communication and participation for individuals with complex communication needs. Augment Altern Commun. 2019 Mar;35(1):1-12.
http://www.ncbi.nlm.nih.gov/pubmed/30648903?tool=bestpractice.com
Adaptive equipment for patients with ataxic CP
Adaptive equipment such as weighted devices, modified utensils, and walking aids may compensate for the poor proximal control that often contributes to instability in patients with ataxic CP. Protective gear, such as helmets, may be used to protect those prone to frequent falling. Equipment such as weighted vests or visual cues may provide additional sensory feedback to improve stability and safety. In more severe cases, canes, crutches, or wheelchairs may be necessary for safe mobility.
Virtual reality, video games, and web-based therapies
Interactive adaptive gaming offers the opportunity for participation and social interaction.[155]Howcroft J, Fehlings D, Wright V, et al. A comparison of solo and multiplayer active videogame play in children with unilateral cerebral palsy. Games Health J. 2012 Aug;1(4):287-93.
http://www.ncbi.nlm.nih.gov/pubmed/26191632?tool=bestpractice.com
There is some evidence that virtual reality training may be as effective as conventional interventions for improving motor function in children with CP.[156]Bonnechère B, Jansen B, Omelina L, et al. Can serious games be incorporated with conventional treatment of children with cerebral palsy? A review. Res Dev Disabil. 2014 Aug;35(8):1899-913.
http://www.ncbi.nlm.nih.gov/pubmed/24794289?tool=bestpractice.com
[157]Chen Y, Fanchiang HD, Howard A. Effectiveness of virtual reality in children with cerebral palsy: a systematic review and meta-analysis of randomized controlled trials. Phys Ther. 2018 Jan 1;98(1):63-77.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6692882
http://www.ncbi.nlm.nih.gov/pubmed/29088476?tool=bestpractice.com
[158]MacIntosh A, Desailly E, Vignais N, et al. A biofeedback-enhanced therapeutic exercise video game intervention for young people with cerebral palsy: a randomized single-case experimental design feasibility study. PLoS One. 2020;15(6):e0234767.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7307764
http://www.ncbi.nlm.nih.gov/pubmed/32569284?tool=bestpractice.com
Injectable pharmacological therapy
Botulinum toxin type A[109]Delgado MR, Hirtz D, Aisen M, et al; Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Practice parameter: pharmacologic treatment of spasticity in children and adolescents with cerebral palsy (an evidence-based review). Neurology. 2010 Jan 26;74(4):336-43.
http://n.neurology.org/content/74/4/336.full
http://www.ncbi.nlm.nih.gov/pubmed/20101040?tool=bestpractice.com
[110]Heinen F, Desloovere K, Schroeder AF, et al. The updated European Consensus 2009 on the use of botulinum toxin for children with cerebral palsy. Eur J Paediatr Neurol. 2010 Jan;14(1):45-66.
http://www.ncbi.nlm.nih.gov/pubmed/19914110?tool=bestpractice.com
Used to lessen spasticity in patients with spastic CP, lessen the force and frequency of dystonic contractions in patients with dystonic CP, and treat chronic sialorrhoea (excessive drooling).[63]Novak I, Morgan C, Fahey M, et al. State of the Evidence Traffic Lights 2019: systematic review of interventions for preventing and treating children with cerebral palsy. Curr Neurol Neurosci Rep. 2020 Feb 21;20(2):3.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035308
http://www.ncbi.nlm.nih.gov/pubmed/32086598?tool=bestpractice.com
[112]Hoare BJ, Wallen MA, Imms C, et al. Botulinum toxin A as an adjunct to treatment in the management of the upper limb in children with spastic cerebral palsy (UPDATE). Cochrane Database Syst Rev. 2010 Jan 20;(1):CD003469.
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD003469.pub4/full
http://www.ncbi.nlm.nih.gov/pubmed/20091546?tool=bestpractice.com
[113]Blumetti FC, Belloti JC, Tamaoki MJ, et al. Botulinum toxin type A in the treatment of lower limb spasticity in children with cerebral palsy. Cochrane Database Syst Rev. 2019 Oct 8;(10):CD001408.
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD001408.pub2/full
http://www.ncbi.nlm.nih.gov/pubmed/31591703?tool=bestpractice.com
Botulinum toxin type A products are approved for use in adults with upper limb spasticity and cervical dystonia. OnabotulinumtoxinA (botulinum toxin type A) and abobotulinumtoxinA (botulinum toxin type A) are approved for upper limb and lower limb spasticity caused by CP in children aged 2 years and over. IncobotulinumtoxinA (botulinum toxin type A) is approved for the treatment of chronic sialorrhoea in adults and children aged 2 years and older. OnabotulinumtoxinA is also used for drooling in adults and children; however, this is an off-label use and there are no approved dosing schedules.
Acts locally in the injected muscle. Target muscles are identified by clinical examination, aided by instrumented gait analysis including electromyogram (EMG). Deeper muscles (e.g., posterior tibialis or iliopsoas) can be identified by EMG, electric stimulation using a teflon-covered needle, or ultrasound examination augmenting surface anatomy.
There is a delayed onset of action of 1 to 2 days; muscle consistency is altered by day 10. It temporarily weakens muscle but is followed by re-innervation, accounting for the short duration of therapeutic effects (typically 3-4 months).
Injection of upper-extremity muscles is followed by OT, splinting, and/or casting. Injection of lower-extremity muscles is followed by serial casting, orthoses, and intensive PT.[63]Novak I, Morgan C, Fahey M, et al. State of the Evidence Traffic Lights 2019: systematic review of interventions for preventing and treating children with cerebral palsy. Curr Neurol Neurosci Rep. 2020 Feb 21;20(2):3.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035308
http://www.ncbi.nlm.nih.gov/pubmed/32086598?tool=bestpractice.com
[112]Hoare BJ, Wallen MA, Imms C, et al. Botulinum toxin A as an adjunct to treatment in the management of the upper limb in children with spastic cerebral palsy (UPDATE). Cochrane Database Syst Rev. 2010 Jan 20;(1):CD003469.
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD003469.pub4/full
http://www.ncbi.nlm.nih.gov/pubmed/20091546?tool=bestpractice.com
[113]Blumetti FC, Belloti JC, Tamaoki MJ, et al. Botulinum toxin type A in the treatment of lower limb spasticity in children with cerebral palsy. Cochrane Database Syst Rev. 2019 Oct 8;(10):CD001408.
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD001408.pub2/full
http://www.ncbi.nlm.nih.gov/pubmed/31591703?tool=bestpractice.com
One Cochrane review concluded that there is a high level of evidence that botulinum toxin type A is effective as an adjunct to OT in managing upper limb spasticity in children with CP.[112]Hoare BJ, Wallen MA, Imms C, et al. Botulinum toxin A as an adjunct to treatment in the management of the upper limb in children with spastic cerebral palsy (UPDATE). Cochrane Database Syst Rev. 2010 Jan 20;(1):CD003469.
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD003469.pub4/full
http://www.ncbi.nlm.nih.gov/pubmed/20091546?tool=bestpractice.com
One Cochrane review found limited evidence that botulinum toxin type A is more effective than placebo or a non-placebo control at improving lower limb spasticity, gait, joint range of motion, and satisfaction in children with CP. Evidence indicated that botulinum toxin type A is not more effective than ankle serial casting for treating ankle contractures, but is more effective than orthotics for improving range of motion and spasticity.[113]Blumetti FC, Belloti JC, Tamaoki MJ, et al. Botulinum toxin type A in the treatment of lower limb spasticity in children with cerebral palsy. Cochrane Database Syst Rev. 2019 Oct 8;(10):CD001408.
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD001408.pub2/full
http://www.ncbi.nlm.nih.gov/pubmed/31591703?tool=bestpractice.com
Botulinum toxin type A has been used as an effective treatment for the control of pain in children with hip spasms.[159]Lundy CT, Doherty GM, Fairhurst CB. Botulinum toxin type A injections can be an effective treatment for pain in children with hip spasms and cerebral palsy. Dev Med Child Neurol. 2009 Sep;51(9):705-10.
https://onlinelibrary.wiley.com/doi/full/10.1111/j.1469-8749.2009.03315.x
http://www.ncbi.nlm.nih.gov/pubmed/19459910?tool=bestpractice.com
It may delay, but not eliminate, the need for surgical hip intervention.[160]Graham HK, Boyd R, Carlin JB, et al. Does botulinum toxin A combined with bracing prevent hip displacement in children with cerebral palsy and "hips at risk"? A randomized, controlled trial. J Bone Joint Surg Am. 2008 Jan;90(1):23-33.
http://www.ncbi.nlm.nih.gov/pubmed/18171954?tool=bestpractice.com
Mild transient side effects lasting 1 to 2 days (local rash, flu-like symptoms, constipation, weakness) may occur. Rare adverse reactions, possibly dose-related, include respiratory distress and dysphagia, although a direct causal relation to botulinum toxin has not been established.[112]Hoare BJ, Wallen MA, Imms C, et al. Botulinum toxin A as an adjunct to treatment in the management of the upper limb in children with spastic cerebral palsy (UPDATE). Cochrane Database Syst Rev. 2010 Jan 20;(1):CD003469.
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD003469.pub4/full
http://www.ncbi.nlm.nih.gov/pubmed/20091546?tool=bestpractice.com
[113]Blumetti FC, Belloti JC, Tamaoki MJ, et al. Botulinum toxin type A in the treatment of lower limb spasticity in children with cerebral palsy. Cochrane Database Syst Rev. 2019 Oct 8;(10):CD001408.
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD001408.pub2/full
http://www.ncbi.nlm.nih.gov/pubmed/31591703?tool=bestpractice.com
There is some evidence of muscle damage and incomplete recovery after botulinum toxin type A treatment, particularly in shorter-term animal models.[161]Multani I, Manji J, Tang MJ, et al. Sarcopenia, cerebral palsy, and botulinum toxin type A. JBJS Rev. 2019 Aug;7(8):e4.
https://journals.lww.com/jbjsreviews/Fulltext/2019/08000/Sarcopenia,_Cerebral_Palsy,_and_Botulinum_Toxin.7.aspx
http://www.ncbi.nlm.nih.gov/pubmed/31415277?tool=bestpractice.com
[162]Sätilä H. Over 25 years of pediatric botulinum toxin treatments: what have we learned from injection techniques, doses, dilutions, and recovery of repeated injections? Toxins (Basel). 2020 Jul 6;12(7):440.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7404978
http://www.ncbi.nlm.nih.gov/pubmed/32640636?tool=bestpractice.com
Following recommended dosing schedules, including repeating injections no more than twice a year, along with accurate placement of the medication using ultrasound and/or electrical stimulation, there are ways to minimise damage. Studies suggest that humans recover up to 90% of pre-injection muscle strength and volume at 1 year and that injections should be accompanied by an organised exercise programme for muscle strengthening.[162]Sätilä H. Over 25 years of pediatric botulinum toxin treatments: what have we learned from injection techniques, doses, dilutions, and recovery of repeated injections? Toxins (Basel). 2020 Jul 6;12(7):440.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7404978
http://www.ncbi.nlm.nih.gov/pubmed/32640636?tool=bestpractice.com
Use of botulinum toxin type A is contraindicated in children with respiratory compromise, severe dysphagia, or presence of neuromuscular junction disease (e.g., Eaton-Lambert syndrome, myasthenia gravis). It must not be given with aminoglycoside antibiotics or tubocurarine-type muscle relaxants. AbobotulinumtoxinA is contraindicated in patients with hypersensitivity to any form of botulinum toxin or to cow’s milk protein. IncobotulinumtoxinA is contraindicated in patients with hypersensitivity to sucrose or albumin.
It is important for practitioners to note that doses differ for each botulinum toxin type A and they are not interchangeable.
Different strains of toxin may be effective when a specific strain fails. Non-responders may be as frequent as 1 in 6 patients due to multiple factors or immunogenicity.[163]Naumann M, Boo LM, Ackerman AH, et al. Immunogenicity of botulinum toxins. J Neural Transm (Vienna). 2013 Feb;120(2):275-90.
https://link.springer.com/article/10.1007%2Fs00702-012-0893-9
http://www.ncbi.nlm.nih.gov/pubmed/23008029?tool=bestpractice.com
Botulinum toxin type B
Phenol and ethyl alcohol
Used for severe spasticity in patients with spastic CP when botulinum toxin is not available, but phenol and ethyl alcohol are less effective and associated with more adverse effects than botulinum toxin.[63]Novak I, Morgan C, Fahey M, et al. State of the Evidence Traffic Lights 2019: systematic review of interventions for preventing and treating children with cerebral palsy. Curr Neurol Neurosci Rep. 2020 Feb 21;20(2):3.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035308
http://www.ncbi.nlm.nih.gov/pubmed/32086598?tool=bestpractice.com
Phenol is used mainly for motor nerves such as the obturator nerve or during open surgical procedures where branches of nerves may be electrically stimulated to ensure they are purely motor. For persistent elbow flexion due to spasticity, phenol may be injected into the musculocutaneous nerve.
Phenol can also be employed as an alternative to botulinum toxin to lessen the force and frequency of dystonic contractions in dystonic CP.
Phenol has a higher rate of complications than botulinum toxin type A, including failure of nerves to recover and persistent dysesthesias when sensory nerves are inadvertently injected.[164]Tilton AH. Injectable neuromuscular blockade in the treatment of spasticity and movement disorders. J Child Neurol. 2003 Sep;18 suppl 1:S50-66.
http://www.ncbi.nlm.nih.gov/pubmed/13677571?tool=bestpractice.com
[165]Gooch JL, Patton CP. Combining botulinum toxin and phenol to manage spasticity in children. Arch Phys Med Rehabil. 2004 Jul;85(7):1121-4.
http://www.ncbi.nlm.nih.gov/pubmed/15241761?tool=bestpractice.com
Phenol denatures protein indiscriminately and can cause sloughing of surrounding tissues, scarring, and contractures. Duration of action is approximately 6 months.
Ethyl alcohol, in lower concentrations, acts as a local anaesthetic. At higher concentrations it denatures proteins indiscriminately. It is preferably injected into motor points or directly into a nerve by open surgical visualisation. Duration of effect varies with strength, concentration, and accuracy of the injection, but lasts 1 week to 6 months. General anaesthesia is required secondary to pain and the need for EMG localisation of the motor point.
Neurosurgical techniques
Intrathecal baclofen (ITB)
An implanted, titratable, and programmable pump delivers baclofen to the spinal cord via a catheter within the thecal space. ITB is more effective than oral baclofen with fewer systemic side effects, although risk of baclofen overdose and withdrawal exist. Location of catheter tip and a diffusion gradient with drug dilution at the brain level are important.
ITB is used in patients with spastic diplegia or quadriplegia. It decreases spasticity, improves endurance, comfort, and ease of care, and reduces fatigue and pain.[118]Hasnat MJ, Rice JE. Intrathecal baclofen for treating spasticity in children with cerebral palsy. Cochrane Database Syst Rev. 2015 Nov 13;(11):CD004552.
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD004552.pub2/full
http://www.ncbi.nlm.nih.gov/pubmed/26563961?tool=bestpractice.com
[166]Kraus T, Gegenleitner K, Svehlik M, et al. Long-term therapy with intrathecal baclofen improves quality of life in children with severe spastic cerebral palsy. Eur J Paediatr Neurol. 2017 Feb 11;21(3):565-9.
http://www.ncbi.nlm.nih.gov/pubmed/28237420?tool=bestpractice.com
[167]Kolaski K, Logan LR. A review of the complications of intrathecal baclofen in patients with cerebral palsy. NeuroRehabilitation. 2007;22(5):383-95.
http://www.ncbi.nlm.nih.gov/pubmed/18162701?tool=bestpractice.com
ITB is also employed in dystonic CP to reduce dystonia in patients with CP who respond to test dosing.[121]Fehlings D, Brown L, Harvey A, et al. Pharmacological and neurosurgical interventions for managing dystonia in cerebral palsy: a systematic review. Dev Med Child Neurol. 2018 Apr;60(4):356-66.
https://onlinelibrary.wiley.com/doi/10.1111/dmcn.13652
http://www.ncbi.nlm.nih.gov/pubmed/29405267?tool=bestpractice.com
[125]Albright AL, Barry MJ, et.al. Intrathecal baclofen for generalized dystonia. Dev Med Child Neurol. 2001 Oct;43(10):652-7.
http://www.ncbi.nlm.nih.gov/pubmed/11665821?tool=bestpractice.com
[126]Eek MN, Olsson K, Lindh K, et al. Intrathecal baclofen in dyskinetic cerebral palsy: effects on function and activity. Dev Med Child Neurol. 2017 Nov 17;60(1):94-9.
http://www.ncbi.nlm.nih.gov/pubmed/29148568?tool=bestpractice.com
[127]Bonouvrié LA, Becher JG, Vles JSH, et al. The effect of intrathecal baclofen in dyskinetic cerebral palsy: the IDYS trial. Ann Neurol. 2019 Jul;86(1):79-90.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6617761
http://www.ncbi.nlm.nih.gov/pubmed/31050023?tool=bestpractice.com
Before implantation, a trial dose is administered via lumbar puncture. A trial is considered effective if the modified Ashworth scale scores decrease 1 or more points in most affected muscles 2 to 4 hours after injection.[83]Bohannon RW, Smith MB. Interrater reliability of a modified Ashworth scale of muscle spasticity. Phys Ther. 1987 Feb;67(2):206-7.
http://www.ncbi.nlm.nih.gov/pubmed/3809245?tool=bestpractice.com
Pump doses are started at the bolus dose and may be titrated 10% to 15% at each follow-up. Typically, if the first dose is unsuccessful, a second dose 25 micrograms higher may be administered 24 hours later.[168]Ridley R, Rawlins PK. Intrathecal baclofen therapy: ten steps towards best practice. J Neurosci Nurs. 2006 Apr;38(2):72-82.
http://www.ncbi.nlm.nih.gov/pubmed/16681287?tool=bestpractice.com
Dosage is based on patient and family report and evidence of spasticity. Side effects include somnolence and excessive weakness.[169]Campbell WM, Ferrel A. Long term safety and efficacy of continuous intrathecal baclofen. Dev Med Child Neurol. 2002 Oct;44(10):660-5.
http://www.ncbi.nlm.nih.gov/pubmed/12418790?tool=bestpractice.com
Maintaining a sterile procedure during pump refills avoids infection.[170]Dario A, Scamoni C, Picano M, et al. The infection risk of intrathecal drug infusion pumps after multiple refill procedures. Neuromodulation. 2005 Jan;8(1):36-9.
http://www.ncbi.nlm.nih.gov/pubmed/22151381?tool=bestpractice.com
The overall rate of infection may be as high as 9.5% over a protracted course of treatment.[171]Bayhan IA, P Sees J, Nishnianidze T, et al. Infection as a complication of intrathecal baclofen treatment in children with cerebral palsy. J Pediatr Orthop. 2016 Apr-May;36(3):305-9.
http://www.ncbi.nlm.nih.gov/pubmed/26296219?tool=bestpractice.com
Baclofen withdrawal can be caused by failure of the pump or battery; failure to refill drug before scheduled alarm date; or a leak, a disconnection, or breakage in the catheter. Withdrawal can result in exaggerated rebound spasticity, rhabdomyolysis, and multiple organ failure. The condition may resemble autonomic dysreflexia, sepsis, malignant hyperthermia, and neuroleptic-malignant syndrome.[172]Coffey RJ, Edgar TS, et al. Abrupt withdrawal from intrathecal baclofen: recognition and management of a potentially life-threatening syndrome. Arch Phys Med Rehabil. 2002 Jun;83(6):735-41.
http://www.ncbi.nlm.nih.gov/pubmed/12048649?tool=bestpractice.com
[173]de Assis Gondim F, Lopes AC, et al. Gastric emptying and gastrointestinal motility abnormalities after spinal cord injury. Arch Phys Med Rehabil. 2002 Oct;83(10):1479.
http://www.ncbi.nlm.nih.gov/pubmed/12370892?tool=bestpractice.com
Although not always leading to withdrawal, the reported incidence of catheter-related complications ranges from 7% to 9%.[174]Motta F, Buonaguro V, Stignani C. The use of intrathecal baclofen pump implants in children and adolescents: safety and complications in 200 consecutive cases. J Neurosurg. 2007 Jul;107(1 suppl):32-5.
http://www.ncbi.nlm.nih.gov/pubmed/17644918?tool=bestpractice.com
[175]Gooch JL, Oberg WA, et al. Complications of intrathecal baclofen pumps in children. Ped Neurosurg. 2003 Jul;39(1):1-6.
http://www.ncbi.nlm.nih.gov/pubmed/12784068?tool=bestpractice.com
Catheter micro-cracks can occasionally lead to underdosage or overdosage.[176]Dawes WJ, Drake JM, Fehlings D. Microfracture of a baclofen pump catheter with intermittent under- and overdose. Pediatr Neurosurg. 2003 Sep;39(3):144-8.
http://www.ncbi.nlm.nih.gov/pubmed/12876394?tool=bestpractice.com
Selective dorsal rhizotomy (SDR)
Also called selective posterior rhizotomy, SDR is a one-time, irreversible treatment to ameliorate spasticity. It is occasionally also used in severe refractory cases to enhance care and relieve pain. The principle of SDR is to balance the loss of inhibition from higher centres in the brain by decreasing sensory stimulation coming from the periphery.
SDR does not address factors other than spasticity that may impede walking. Among patients with spastic diplegia, the best candidates are those with preserved voluntary selective motor control, strong trunk and pelvic muscles, and good dynamic balance.
Post-operative weakness is common and patients require long-term, aggressive PT. Ankle-foot orthoses are recommended post-operatively for at least 6 months to protect weak plantar flexor muscles from overstretch.
Spinal deformities have been reported, but these need to be compared with the rates in similar patients who have not undergone SDR in order to be considered post-operative complications.[177]Wheelwright M, Selvey PJ, Steinbok P, et al. Systematic review of spinal deformities following multi-level selective dorsal rhizotomy. Childs Nerv Syst. 2020 May;36(5):1025-35.
http://www.ncbi.nlm.nih.gov/pubmed/31595313?tool=bestpractice.com
[178]Veerbeek BE, Lamberts RP, Fieggen AG, et al. A long-term follow-up study of spinal abnormalities and pain in adults with cerebral palsy and spastic diplegia more than 25 years after selective dorsal rhizotomy. J Neurosurg Spine. 2020 Oct 16;1-8.
http://www.ncbi.nlm.nih.gov/pubmed/33065536?tool=bestpractice.com
[179]Lundkvist Josenby A, Westbom L. No support that early selective dorsal rhizotomy increase frequency of scoliosis and spinal pain - a longitudinal population-based register study from four to 25 years of age. BMC Musculoskelet Disord. 2020 Nov 27;21(1):782.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7697382
http://www.ncbi.nlm.nih.gov/pubmed/33246436?tool=bestpractice.com
The long-term outcomes of SDR are still being investigated, but most patients achieve lasting spasticity reduction. Some require adjunctive treatment for residual spasticity.[180]Tedroff K, Hägglund G, Miller F. Long-term effects of selective dorsal rhizotomy in children with cerebral palsy: a systematic review. Dev Med Child Neurol. 2020 May;62(5):554-62.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7187377
http://www.ncbi.nlm.nih.gov/pubmed/31342516?tool=bestpractice.com
Deep brain stimulation
The use of electrical stimulation by electrodes placed in the posteroventral lateral globus pallidus internus to decrease extra-pyramidal movement disorders.
Deep brain stimulation has been successful in patients with primary (or genetic) dystonia, and some evidence suggests it may be successful in patients with secondary dystonia resulting from CP.[121]Fehlings D, Brown L, Harvey A, et al. Pharmacological and neurosurgical interventions for managing dystonia in cerebral palsy: a systematic review. Dev Med Child Neurol. 2018 Apr;60(4):356-66.
https://onlinelibrary.wiley.com/doi/10.1111/dmcn.13652
http://www.ncbi.nlm.nih.gov/pubmed/29405267?tool=bestpractice.com
[128]Sanger TD. Deep brain stimulation for cerebral palsy: where are we now? Dev Med Child Neurol. 2020 Jan;62(1):28-33.
https://onlinelibrary.wiley.com/doi/10.1111/dmcn.14295
http://www.ncbi.nlm.nih.gov/pubmed/31211420?tool=bestpractice.com
[129]Krauss JK, Loher TJ, Weigel R, et al. Chronic stimulation of the globus pallidus internus for treatment of non-dYT1 generalized dystonia and choreoathetosis: 2-year follow up. J Neurosurg. 2003 Apr;98(4):785-92.
http://www.ncbi.nlm.nih.gov/pubmed/12691403?tool=bestpractice.com
Criteria for patient selection are undefined.
Orthopaedic surgery
The goals are to prevent progressive deformity and remove mechanical or anatomic barriers in order to maximise mobility and function.
Single-event multilevel surgery (i.e., combining several surgeries into a single surgical event) is recommended in order to minimise total recovery time, rather than 'birthday surgery' (a procedure nearly every year).[63]Novak I, Morgan C, Fahey M, et al. State of the Evidence Traffic Lights 2019: systematic review of interventions for preventing and treating children with cerebral palsy. Curr Neurol Neurosci Rep. 2020 Feb 21;20(2):3.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035308
http://www.ncbi.nlm.nih.gov/pubmed/32086598?tool=bestpractice.com
[181]Õunpuu S, Solomito M, Bell K, et al. Long-term outcomes after multilevel surgery including rectus femoris, hamstring and gastrocnemius procedures in children with cerebral palsy. Gait Posture. 2015 Sep;42(3):365-72.
http://www.ncbi.nlm.nih.gov/pubmed/26260009?tool=bestpractice.com
Multilevel surgery has been shown to improve gait function in the long term in ambulatory patients with bilateral spastic CP, although 39% of patients required additional surgery during follow-up.[182]Dreher T, Thomason P, Švehlík M, et al. Long-term development of gait after multilevel surgery in children with cerebral palsy: a multicentre cohort study. Dev Med Child Neurol. 2018 Jan;60(1):88-93.
https://onlinelibrary.wiley.com/doi/full/10.1111/dmcn.13618
http://www.ncbi.nlm.nih.gov/pubmed/29171016?tool=bestpractice.com
Orthopaedic surgery is often part of a comprehensive treatment approach, which includes spasticity reduction and muscle strengthening through intensive physiotherapy in the post-operative period. Orthopaedic surgery is ideally offered in specialty CP clinics where all the manifestations of a patient’s condition can be identified and prioritised.
Orthopaedic surgery is unpredictable in patients with dystonic CP or ataxic CP and is usually avoided; exceptions are tailored to individual circumstances.
Upper limbs
The goals are to improve function, appearance, and hygiene. For functional improvement, adequate selective motor control and sensation (stereognosis) must be present. Tendon transfers to improve wrist and finger/thumb extension, along with first web-space release and flexion contracture release, can improve hand function. Wrist fusion to address residual wrist-flexion deformity is often sought prior to a patient beginning university or participating in the workplace. It may improve function as a 'helping hand' as well.
For hygiene issues, tendon lengthening and long-term bracing usually suffice. Release of contractures is also considered to facilitate placement of hand in space and prevent hygiene problems.
If deformity is long-standing, more extensive releases, including release of the joint capsules, may be needed. Elbow flexor and forearm pronation contracture release may help an adolescent fit in socially.
Upper-extremity contractures are rare in diplegia.
Lower limbs
Spastic diplegia can result in accentuated crouching gait in adolescence. Goals are to keep the hips located, if possible, and to address later multilevel contractures.
Hips are normally located at birth but may drift out of the sockets in many children starting around age 3 years. Early identification is by screening programmes and periodic x-rays from age 3 years, with frequency related to the severity of the condition as classified by the GMFCS.[183]Larnert P, Risto O, Hägglund G, et al. Hip displacement in relation to age and gross motor function in children with cerebral palsy. J Child Orthop. 2014 Mar;8(2):129-34.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3965763
http://www.ncbi.nlm.nih.gov/pubmed/24595560?tool=bestpractice.com
Treatment ranges from tendon releases through correction of rotational deformities (e.g., femoral anteversion, internal or external tibial torsion) along with acetabular osteotomies to provide more strategic coverage of the hips.[184]Hägglund G, Andersson S, Düppe H, et al. 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 Br. 2005 Jan;87(1):95-101.
http://www.ncbi.nlm.nih.gov/pubmed/15686244?tool=bestpractice.com
Tendon releases should ideally take place before age 5 years, and correction of rotational deformities with osteotomy should be carried out between ages 3 and 7 years. Late surgeries are less effective in preventing or reducing sequelae such as pain and progressive dislocation.
Late-presenting dislocation (age 9+ years) requires either observation or surgical reduction, depending on the shape of the acetabulum and how recently the hip was last known to be located. In non-ambulatory children, severity of hip dislocation, as measured by migration percentage, is associated with a lower health-related quality of life (HRQoL); hip reconstructive surgery is associated with improvements in HRQoL.[185]DiFazio R, Shore B, Vessey JA, et al. Effect of hip reconstructive surgery on health-related quality of life of non-ambulatory children with cerebral palsy. J Bone Joint Surg Am. 2016 Jul 20;98(14):1190-8.
http://www.ncbi.nlm.nih.gov/pubmed/27440567?tool=bestpractice.com
Hip resection to treat pain in older children is frequently complicated by heterotopic ossification; post-operative traction or indomethacin administration does not seem to alter outcomes.[186]Dartnell J, Gough M, Paterson JM, et al. Proximal femoral resection without post-operative traction for the painful dislocated hip in young patients with cerebral palsy: a review of 79 cases. Bone Joint J. 2014 May;96-B(5):701-6.
http://www.ncbi.nlm.nih.gov/pubmed/24788508?tool=bestpractice.com
[187]Dartnell J, Paterson JM, Magill N, et al. Proximal femoral resection for the painful dislocated hip in cerebral palsy: does indomethacin prevent heterotopic ossification? J Pediatr Orthop. 2014 Apr-May;34(3):295-9.
http://www.ncbi.nlm.nih.gov/pubmed/24590343?tool=bestpractice.com
Adult presentation depends on symptoms: hip replacement, osteotomy, or resection for significant pain; or extreme osteotomy to position the femoral head away from the acetabulum.
The severity of contracture and deformity tends to be less in hemiplegia than with more global involvement. However, while hip dysplasia and deformity is less frequent in hemiplegic patients, it should not be ignored.[85]Hägglund G, Lauge-Pedersen H, Wagner P. Characteristics of children with hip displacement in cerebral palsy. BMC Musculoskelet Disord. 2007 Oct 26;8:101.
https://bmcmusculoskeletdisord.biomedcentral.com/articles/10.1186/1471-2474-8-101
http://www.ncbi.nlm.nih.gov/pubmed/17963501?tool=bestpractice.com
[188]Hogan KA, Blake M, Gross RH. Subtrochanteric valgus osteotomy for chronically dislocated, painful spastic hips. J Bone Joint Surg Am. 2006 Dec;88(12):2624-31.
http://www.ncbi.nlm.nih.gov/pubmed/17142412?tool=bestpractice.com
If possible, surgery to correct knee flexion and ankle equinus contractures is best deferred until adolescence to avoid weakness, but recalcitrant cases will require earlier treatment.
Knee flexion contracture or ankle equinus is acceptable in non-ambulatory patients up to the point at which care or positioning is impaired. In ambulatory patients (GMFCS stage I, II, or III) tendon releases or transfers at the knee or ankle should ideally be preceded with a gait study, including EMG and kinematic data. The more mobile the patient, the more likely tendons will be transferred and lengthened rather than released. Aggressive lengthening of the triceps surae in young ambulatory children should be avoided, as it may contribute to a crouched gait as the child ages.[189]Rutz E, McCarthy J, Shore BJ, et al. Indications for gastrocsoleus lengthening in ambulatory children with cerebral palsy: a Delphi consensus study. J Child Orthop. 2020 Oct 1;14(5):405-14.
https://online.boneandjoint.org.uk/doi/full/10.1302/1863-2548.14.200145
http://www.ncbi.nlm.nih.gov/pubmed/33204348?tool=bestpractice.com
When possible in diplegia, the soleus is left intact, and only the gastrocnemius fascia is lengthened.[189]Rutz E, McCarthy J, Shore BJ, et al. Indications for gastrocsoleus lengthening in ambulatory children with cerebral palsy: a Delphi consensus study. J Child Orthop. 2020 Oct 1;14(5):405-14.
https://online.boneandjoint.org.uk/doi/full/10.1302/1863-2548.14.200145
http://www.ncbi.nlm.nih.gov/pubmed/33204348?tool=bestpractice.com
Excessive dorsiflexion may also be due to hypermobility in the subtalar joint that requires stabilisation. In adults, the incidence of tripping may be lessened in those with a stiff knee gait pattern (failure to clear the floor in swing) by distal rectus tenotomy.[190]Drefus LC, Buckland MA, Backus SI, et al. The functional effect of a distal rectus femoris tenotomy in adults with cerebral palsy. Gait Posture. 2014;40(1):145-9.
http://www.ncbi.nlm.nih.gov/pubmed/24742707?tool=bestpractice.com
In patients with spastic diplegia, significant knee flexion contractures should be addressed before adolescence in order to prevent the development of a severely crouched gait.[191]Stout JL, Gage JR, Schwartz MH, et al. Distal femoral extension osteotomy and patellar tendon advancement to treat persistent crouch gait in cerebral palsy. J Bone Joint Surg Am. 2008 Nov;90(11):2470-84.
http://www.ncbi.nlm.nih.gov/pubmed/18978417?tool=bestpractice.com
[192]Novacheck TF, Stout JL, Gage JR, et al. Distal femoral extension osteotomy and patellar tendon advancement to treat persistent crouch gait in cerebral palsy. Surgical technique. J Bone Joint Surg Am. 2009 Oct 1;91 suppl 2:271-86.
http://www.ncbi.nlm.nih.gov/pubmed/19805590?tool=bestpractice.com
[193]Healy MT, Schwartz MH, Stout JL, et al. Is simultaneous hamstring lengthening necessary when performing distal femoral extension osteotomy and patellar tendon advancement? Gait Posture. 2011 Jan;33(1):1-5.
http://www.ncbi.nlm.nih.gov/pubmed/21145748?tool=bestpractice.com
This frequently involves distal femoral shortening extension osteotomies with advancement of the patellar tendons.[191]Stout JL, Gage JR, Schwartz MH, et al. Distal femoral extension osteotomy and patellar tendon advancement to treat persistent crouch gait in cerebral palsy. J Bone Joint Surg Am. 2008 Nov;90(11):2470-84.
http://www.ncbi.nlm.nih.gov/pubmed/18978417?tool=bestpractice.com
[194]Boyer ER, Stout JL, Laine JC, et al. Long-term outcomes of distal femoral extension osteotomy and patellar tendon advancement in individuals with cerebral palsy. J Bone Joint Surg Am. 2018 Jan 3;100(1):31-41.
http://www.ncbi.nlm.nih.gov/pubmed/29298258?tool=bestpractice.com
Lever disease or the malpositioning of the functional plane of motion of the knee or ankle can be corrected with femoral or tibial rotational osteotomies, or both.
Foot deformities may be treated with a bony operation because of the unpredictability of the deforming forces. Selective motor control evaluation can be a useful guide: the poorer the control, the more likely the need for a bony operation; the better the control, the more likelihood of tendon surgery being successful. Foot deformities or contractures of ankles or toes in patients with ataxic CP can be treated orthopaedically, if necessary, to prolong walking and alleviate pain.
Spine
Abnormalities are less common, but observation is still indicated. Abnormalities are more common as the severity of CP increases and are much more likely in patients at GMFCS levels IV and V.[7]Palisano R, Rosenbaum P, Walter S, et al. Development and reliability of a system to classify gross motor function in children with cerebral palsy. Dev Med Child Neurol. 1997 Apr;39(4):214-23.
http://www.ncbi.nlm.nih.gov/pubmed/9183258?tool=bestpractice.com
Surgical treatment should be avoided until at least age 7 or 8 years, and preferably until age 12 years, to allow lung maturation.
If the spinal curve is significant or progressive, surgical fusion from sacrum to upper thorax may be indicated. Posterior fixation devices alone are usually sufficient, although anterior release may also be required for severe deformities, depending on the flexibility of the curve.
Previously, spine fusions were frequently performed on both the anterior and posterior aspects of the spine. With improved posterior spinal instrumentation incorporating pedicle screws, the posterior approach by itself now seems effective.[195]Funk S, Lovejoy S, Mencio G, et al. Rigid instrumentation for neuromuscular scoliosis improves deformity correction without increasing complications. Spine (Phila Pa 1976). 2016 Jan;41(1):46-52.
http://www.ncbi.nlm.nih.gov/pubmed/26352742?tool=bestpractice.com
Complementary and alternative treatments
The National Center for Complementary and Integrative Health (NCCIH) of the US National Institutes of Health (NIH) defines complementary and alternative treatments as “a group of diverse medical and health care systems, practices and products that are not considered to be part of conventional or allopathic medicine”.[196]National Center for Complementary and Integrative Health. Are you considering a complementary health approach? September 2016 [internet publication].
https://www.nccih.nih.gov/health/are-you-considering-a-complementary-health-approach
Most complementary and alternative treatments are used in conjunction with, rather than as a true alternative to, conventional therapies. These methods are not evidence-based and, consequently, are not usually employed by mainstream clinicians in day-to-day practice; however, lack of evidence does not necessarily imply lack of effectiveness. Parents may be desperate to try these methods despite the long odds of success and high costs if traditional medical care has been ineffective for symptom management.[197]ResearchGate. Complementary and alternative medicine in cerebral palsy. October 2020 [internet publication].
Non-traditional therapies with some evidence of benefit for patients with CP include hippotherapy (therapeutic horseback riding), acupuncture, and other animal-assisted therapy.[63]Novak I, Morgan C, Fahey M, et al. State of the Evidence Traffic Lights 2019: systematic review of interventions for preventing and treating children with cerebral palsy. Curr Neurol Neurosci Rep. 2020 Feb 21;20(2):3.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035308
http://www.ncbi.nlm.nih.gov/pubmed/32086598?tool=bestpractice.com
Other therapies (e.g., massage, yoga, reflexology, conductive education) show no evidence of benefit for improving motor skills, but may help some patients in improving quality of life and symptoms such as pain or constipation.[63]Novak I, Morgan C, Fahey M, et al. State of the Evidence Traffic Lights 2019: systematic review of interventions for preventing and treating children with cerebral palsy. Curr Neurol Neurosci Rep. 2020 Feb 21;20(2):3.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035308
http://www.ncbi.nlm.nih.gov/pubmed/32086598?tool=bestpractice.com
Methods such as conductive education emphasise the acquisition of practical daily skills over traditional learning activities while promoting social interaction (as patients engage in group therapies), and may have benefits for social skills and quality of life.[63]Novak I, Morgan C, Fahey M, et al. State of the Evidence Traffic Lights 2019: systematic review of interventions for preventing and treating children with cerebral palsy. Curr Neurol Neurosci Rep. 2020 Feb 21;20(2):3.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035308
http://www.ncbi.nlm.nih.gov/pubmed/32086598?tool=bestpractice.com
Hyperbaric oxygen treatment, threshold electrical stimulation, and craniosacral therapy should not be used as they showed no evidence of benefit in trials and may be associated with various adverse effects.[63]Novak I, Morgan C, Fahey M, et al. State of the Evidence Traffic Lights 2019: systematic review of interventions for preventing and treating children with cerebral palsy. Curr Neurol Neurosci Rep. 2020 Feb 21;20(2):3.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035308
http://www.ncbi.nlm.nih.gov/pubmed/32086598?tool=bestpractice.com
[198]Collet JP, Vanasse M, Marois P, et al. Hyperbaric oxygen for children with cerebral palsy: a randomised multicentre trial. HBO-CP Research Group. Lancet. 2001 Feb 24;357(9256):582-6.
http://www.ncbi.nlm.nih.gov/pubmed/11558483?tool=bestpractice.com
[199]Dali C, Hansen FJ, Pedersen SA, et al. Threshold electrical stimulation (TES) in ambulant children with CP: a randomized double-blind placebo-controlled clinical trial. Dev Med Child Neurol. 2002 Jun;44(6):364-9.
https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1469-8749.2002.tb00830.x?sid=nlm%3Apubmed
http://www.ncbi.nlm.nih.gov/pubmed/12088304?tool=bestpractice.com
[200]Wyatt K, Edwards V, Franck L, et al. Cranial osteopathy for children with cerebral palsy: a randomised controlled trial. Arch Dis Child. 2011 Jun;96(6):505-12.
https://adc.bmj.com/content/96/6/505.long
http://www.ncbi.nlm.nih.gov/pubmed/21349889?tool=bestpractice.com
There is no evidence that cannabinoids are effective in treating spasticity associated with CP.[201]Fairhurst C, Kumar R, Checketts D, et al. Efficacy and safety of nabiximols cannabinoid medicine for paediatric spasticity in cerebral palsy or traumatic brain injury: a randomized controlled trial. Dev Med Child Neurol. 2020 Sep;62(9):1031-9.
https://onlinelibrary.wiley.com/doi/10.1111/dmcn.14548
http://www.ncbi.nlm.nih.gov/pubmed/32342496?tool=bestpractice.com
[202]Nielsen S, Murnion B, Campbell G, et al. Cannabinoids for the treatment of spasticity. Dev Med Child Neurol. 2019 Jun;61(6):631-8.
https://onlinelibrary.wiley.com/doi/10.1111/dmcn.14165
http://www.ncbi.nlm.nih.gov/pubmed/30680713?tool=bestpractice.com
Further trials are planned.[203]ClinicalTrials.gov. Full-spectrum medical cannabis for treatment of spasticity in patients with severe forms of cerebral palsy (HemPhar). 18 November 2020 [internet publication].
https://clinicaltrials.gov/ct2/show/NCT04634136