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Management of Spasticity

in Children With Cerebral PalsyAnn Tilton, MD

Spasticity and other forms of muscle overactivity caused by cerebral palsy may impair

function or ease of care or may cause discomfort or poor body image. The treatment

program for a child with spasticity may include allied health therapy, exercise, casting,

constraint-induced therapy, oral medications, chemodenervation, intrathecal baclofen, se-

lective dorsal rhizotomy, and orthopedic surgery. Techniques may be combined for greater

efficacy and better tailoring to the needs of the child. This article provides an overview of

each approach, with a review of significant research findings in support of each.

Semin Pediatr Neurol 16:8289 2009 Published by Elsevier Inc.

Properly defined, spasticity is a motor disorder character-ized by a velocity-dependent increase in tonic stretchreflexes (muscle tone) with exaggerated tendon jerks, result-

ing from hyperexcitability of the stretch reflex, as one com-

ponent of the upper motor neuron syndrome.1 Along with

the other positive motor phenomena of the upper motor

neuron syndromeincluding phasic stretch reflexes (clonus

and hyperreflexia), flexor and extensor spasms, cocontrac-

tion, dystonia, and associated phenomenaspasticity can

have a significant functional impact on a child with cerebral

palsy (CP). These various forms of muscle overactivity (forwhich spasticity is often a convenient, although overly sim-

plified, shorthand term) represent important therapeutic

targets in the attempt to provide functional gains for the

child. Nonetheless, it is important to realize that the negative

symptomsweakness and lack of dexteritymay be far

more functionally disabling and are far less amenable to treat-

ment. Even so, tone reduction in the properly selected pa-

tient can lead to important gains, and acceptance of the limits

is not a reason to avoid trying therapy.

The range of treatments for excess tone is large, from sim-

ple stretching exercises to oral and injectable therapies to

surgeries. In this article, we review treatment planning forspasticity in CP and provide an overview of treatment op-

tions.

Evaluation andTreatment Planning

The treatment program begins with a careful and thoroughevaluation to determine whether muscle overactivity is inter-fering with some aspect of function, comfort, cosmesis, orcare. If it is not, no treatment is necessary, and it should notbe undertaken. Additionally, whether the patients spasticityis aiding functionfor instance, whether lower extremitystiffness actually improves transfer ability in the face of un-derlying leg muscle weaknessshould be determined. Re-duction of such useful spasticity may possibly be counter-productive; on the other hand, when combined with musclestrengthening and appropriate orthotics, reduction of spas-ticity may lead to overall functional benefit and therefore canbe contemplated. Thorough evaluation requires the input ofthe entire spasticity management team, including the patientand caregivers, physicians, physical and occupational thera-pists, nurses, orthopedists, and orthotists, as well as surgeonsand other professionals in some cases. Psychologists, socialworkers, and educators may round out the team.

Spasticity in CP can be classified by affected body region:

Spastic diplegia (both legs involved, more than arms) Hemiplegia (involves an arm and a leg on the same side

of the body) Double hemiplegia (both arms involved, more than

legs) Tetraplegia and/or quadriplegia (all 4 limbs involved,

usually severely)

Within this broad scheme, it is important to document theparticular pattern of spastic involvement (eg, scissoringthighs, adducted shoulder) and the degree of severity.2,3

Department of Neurology, Louisiana State University Health and Sciences

Center, New Orleans, LA.

Address reprint requests to Ann Tilton, MD, Louisiana State University

Health and Sciences Center, New Orleans, LA 70,118. E-mail:atilto@

aol.com

82 1071-9091/09/$-see front matter 2009 Published by Elsevier Inc.doi:10.1016/j.spen.2009.03.006
mailto:[email protected]:[email protected]:[email protected]:[email protected]


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Tone is typically measured with the Ashworth Scale, a sub-jective but nonetheless useful and easily administered clinicalscale that rates tone from 1 (no hypertonia) to 4 (rigid inflexion or extension).

Evaluation includes not only measurement of tone withthe Ashworth Scale, but also one or more measures of motorperformance and functional ability, such as the Gross Motor

Function Measure (GMFM), the Functional IndependenceMeasure, or the Barthel Index. Such measures should be cho-sen not only for their relevance to the clinical picture andtreatment plan but also for their ease of use, keeping in mindthat a measure is only as good as the consistency with whichit is used during follow-up.

In the broadest terms, the goals of any spasticity treatmentplan are to maximize active function, ease care, and preventsecondary problems such as pain, subluxation, and contrac-ture. Compromises are often required to navigate amongmultiple potentially conflicting goals. Because the patientmay have goals and dreams and because the caregiver plays

the central role in the day-to-day management of the child,each should be a central figure in setting treatment goals.

When setting treatment goals, it is often vital to temper thecaregivers overly ambitious hopes and to focus on realistic,attainable goals. This may become even more important aftera successful round of treatment, when excitement over im-proved function leads to unrealistic expectations for furtherimprovement. At each stage of goal setting, treatment plan-ning, and plan modification, it is vital to obtain full informa-tion from the caregiver regarding the effects of previous treat-ments and the details of the childs current situation. Todevelop the best treatment plan, these details must go beyonda strictly clinical picture to include how the child is function-ing in the home and school, interests of the child (eg, sportsor other activities) that may be affected by treatment, andother factors important to the childs life. Equally important,the needs of the caregiver must be considered in formulatingtreatment. For instance, the tone reduction that will improveease of hygiene is likely to be greater than that which willimprove transfers.

Multiple other factors influence treatment plan and timing.The age of the child, presence of comorbidities such as sei-zures or cognitive impairment, the ability of the family tocarry out home treatments or return for regular follow-ups,financial concerns, and other issues are all considered in

determining the best treatment.Although every case is unique, several broad parameters

can help shape decision making.3 Orthopedic surgery shouldbe delayed until the gait is mature. Meanwhile, stretching,physical therapy (PT), and orthotics are used to maintainrange of motion. Oral medications, botulinum toxin injec-tions, intrathecal baclofen, and/or rhizotomy may also beappropriate. When gait is mature (between ages 6 and 10),gait analysis and clinical examination can be used to deter-mine whether surgery is necessary. To avoid multiple surger-ies and periods of immobility, one should try to perform allsurgery at one time and remobilize the child as soon as pos-

sible. Continued stretching and medical treatment are usedas needed to maintain range of motion and mobility.

PT and Orthoses

The importance of regular stretching in maintaining fullrange of motion and preventing contracture cannot be over-stated. Luckily, children want to move, and much of the needfor range-of-motion exercises can be met by the daily activi-ties of a reasonably active child. However, when impairments

are dealt with by compensatory strategies that minimizemovement of the affected joint, the potential for contractureincreases. Thus, regular stretching of all affected limbs isgenerally prescribed. Stretching can reduce severity of tonefor several hours, providing a short-term, but not long-term,antispasticity action.

PT for the child with CP may encompass a regular exerciseprogram,4 horseback riding,5 and a variety of modalities, in-cluding biofeedback6 and electrical stimulation.7-10

Spastic muscles are often weak. The efficacy of lower limbstrength training has been examined in several controlledclinical trials.11-14 The results of these trials suggest that when

added to a well-designed PT program, strength training canimprove gait parameters without worsening spasticity.The intensity and constancy of PT needed to maximize

gains have been the subject of several studies. Bower et alcompared the effects of the usual amount of PT to intensivetherapy (1 hour per day, 5 days per week) over 6 months inchildren between ages 3 and 12.15 They found no significantdifference between the groups in either function (as mea-sured by the GMFM) or performance (measured by the GrossMotor Performance Measure) at the end of the 6-month treat-ment, though inclusion of age and severity covariates sug-gested a trend toward significance. Six months after the endof the trial, there was no significant difference betweengroups, even with inclusion of these covariates. The authorsconcluded that such intensive therapy was not superior to thenormal therapy that children were already receiving, at leastin its long-term effects. A similar result was shown by Wein-dling et al, who compared 6 months of standard PT, standardPT plus extra PT delivered by a trained assistant for 1 hourper week, and standard PT plus a home visit from a familysupport worker. They found no evidence that additional PTaffected motor function, developmental status, or adaptivefunction. Moreover, there was no benefit seen from interven-tion from the family support worker on parental stress orfamily needs. The authors concluded that extra intervention

is not necessarily beneficial, although they recognized thatresearch is still needed to determine what constitutes ade-quate intervention for children of different needs.16 Chris-tiansen et al compared 2 regimens of PTintermittent (4times a week for 4 weeks, alternating with a 6-week hiatus) orcontinuous (once or twice a week, every week)both for 30weeks.17 They found no difference in GMFM between the 2regimens and noted that compliance was higher in the inter-mittent group.

Constraint-induced therapy (also called forced-use ther-apy) has received much attention during the past decade. Inthis protocol, a hemiparetic childs better-functioning upper

extremity is constrained to force use of the more poorly func-tioning one. This is believed to lead to plastic changes in the

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brain that improve the function of the less able limb. Ran-domized trials have shown the potential of this therapy forincreased acquisition of new motor skills and increased dex-terity that may last for at least 6 months.18-22 Compliance maybe a significant issue, one addressed by Charles et al, whotested a child friendly schedule of 6 hours per day of con-straint for 10 of 12 consecutive days.19 They found this reg-

imen was effective in ways similar to a more restrictive one.These results were retained over the long term and could beimproved upon by a further course of treatment.23

Ankle-foot orthoses (AFOs) are commonly used to treatdynamic equinus in CP. Carlson et al have shown with gaitanalysis that AFOs could significantly reduce ankle excursionand increase dorsiflexion angle at foot strike, as well as pro-vide other benefits, although neither stride length norwalking speed were improved.24AFOs can also improve thesit-to-stand transition in preambulatory children whosestanding is impaired by equinus.25 Bjornson has suggestedthat dynamic AFOs are most effective in younger children.26

One cautionary note is in order: the patient or caregivermay expect use of any of the new modalities, such as botuli-num toxin, intrathecal baclofen, or selective dorsal rhizot-omy, to substitute for an individualized PT program. Expe-rience has shown that patients who do not continue with aprogram of strengthening and stretching do not realize thepotential benefits of the intervention.

Oral Medications

Oral antispasticity agents have the advantage of ease of usebut the disadvantages of systemic effect and significant side

effects. Thus, they are most appropriate in children for whomonly mild tone reduction is needed or in whom spasticity iswidespread. Unfortunately, most studies on the efficacy ofthese agents are old, did not address measures of function,and employed trial designs less rigorous than the best prac-tices of today. In addition, most antispasticity trials have beencarried out in adults with spastic disorders, and relatively fewtrials have been conducted in children. Thus, the choice ofagent is more a matter of personal experience and trial anderror than of rigorous, evidence-based medicine. Continueduse of any particular agent must be justified by clear benefit tothe patient.

BaclofenBaclofen is a GABA-B agonist, and oral baclofen is often thedrug of choice for spasticity of spinal cord origin in adults. Itmay be useful in selected pediatric patients, although littleresearch has been conducted to support use in this popula-tion, and it does not have Food and Drug Administrationapproval for use in CP. Milla et al showed in a double-blindcrossover trial that baclofen reduced spasticity significantlybetter than placebo and improved both passive and activemovement.27 More recently, Scheinberg et al showed thatwhereas there was no effect on spasticity as measured by the

Modified Tardieu scale or on the Pediatric Evaluation of Dis-ability Inventory, there was improvement vs placebo on the

Goal Attainment Scale, a functional assessment tool.28 Thetendency of Baclofen tendency to cause confusion and seda-tion limits the dose, although these effects may improve overseveral weeks of treatment. A typical starting dose in a child is2.5 mg/d, which can be gradually titrated up to a maximumof 20-60 mg/d, depending on body size.29 Weaning off ofBaclofen must be gradual to avoid a withdrawal syndrome.

TizanidineTizanidine is a centrally acting alpha-2 noradrenergic agonistthat has been shown to reduce tonic stretch reflexes and toenhance presynaptic inhibition in animals. A Russian studyin 30 diplegic children of tizanidine dosed at 6 mg/d reportedimproved motor ability, which was confirmed with electro-neuromyography.30 Side effects were reported to be well tol-erated. To date, no clinical trials of this agent in children havebeen published in the English-language literature. Sedationand the requirement for frequent dosing have been limitingfactors in tizanidine use. Nevertheless, the sedative quality

can be an advantage when the medication is delivered atnight. Improved initiation of sleep and reduced tone are po-tential benefits.

DiazepamDiazepam is a benzodiazepine that facilitates the postsynap-tic action of GABA. A series of trials in the 1960s demon-strated its ability to reduce spasticity in children with CP.31-35

More recently, Mathew et al showed the ability of diazepamto reduce muscle overactivity in comparison to placebo in arandomized trial of 180 children.36 Diazepam has also beencompared with and used in conjunction with dantrolene so-

dium;37 in the same small trial, the 2 agents appeared to beequally effective, and the combination was more effectivethan either alone. The daily dose of diazepam is usually 0.12-0.8 mg/kg, divided into 3-4 doses. The known sedation pro-file of this class of medications must be considered. Diaze-pam at bedtime may aid sleep without carry-over daytimesedation.38

Dantrolene SodiumUnlike other oral antispasticity agents, dantrolene sodiumworks at the muscle level, inhibiting calcium release from thesarcoplasmic reticulum, causing muscle weakness. In dou-

ble-blind crossover studies, it has been shown to reduce spas-ticity in children with CP.37,39 It can cause global weaknessand, despite its peripheral mode of action, sedation. It alsohas the potential for hepatotoxicity in approximately 1% ofpatients. It does not have long-term stability in liquid form,which limits its administration primarily to children able totake capsules.

In general, the cognitive and sedative side effects of oralmedications can be said often to overshadow any improve-ment in spasticity, leaving the patient with minimal globalgain in function. Thus, other types of treatments often play amore important role in tone management in the child with

CP. Oral agents may be most useful as adjuncts in childrenwith seizures, sleep disturbance, or other conditions in which

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the other effects of these medications, besides the antispasticeffects, are useful.

Botulinum Toxin,Phenol, and Alcohol

Chemodenervation (also called neurolysis or neuromuscularblockade) refers to the use of an injectable therapy to preventnerve-muscle transmission. Two strategies are in current use:perineural injection of phenol or ethyl alcohol and intramus-cular injection of botulinum toxin (BoNT). All 3 are appro-priate for focal spasticity or for targeting specific problemmuscles in more generalized spasticity.

Both phenol and alcohol have been used in children withCP, although neither has been widely or rigorously tested.Phenol is typically injected at a concentration of 3%-6%aqueous solution, whereas absolute alcohol is diluted to30%-50%. The target nerve is identified with electrical stim-

ulation, a procedure that may be poorly tolerated in children,making sedation or anesthesia necessary. The agent is in-

jected perineurally, where it promotes denervation via axonaldegeneration. The effect is not permanent, with functionalreinnervation occurring over months to years. Studies haveshown the benefits for spasticity in CP of both alcohol40 andphenol.41,42 Benefit ranges from a few weeks to 2 years ormore, with no factors conclusively identified in the literatureas determining the duration. Adverse effects of both agentsinclude a significant risk of pain or paresthesia when target-ing a mixed nerve, which may persist. This, combined withthe high degree of skill required to target the nerve and theavailability of botulinum toxin as an alternative, has keptphenol and alcohol from assuming a larger role in focal spas-ticity management. Nonetheless, the absence of immunoge-nicity and the lower cost compared to BoNT make theseagents more attractive in some settings.43

Botulinum toxin is an exotoxin produced by Clostridiumbotulinum, the same organism responsible for tetanus. Thereare 7 naturally occurring serotypes of the toxin, A-G, all ofwhich are zinc proteases that target the synaptic vesicle fu-sion machinery at the neuromuscular junction. Denervationoccurs because vesicles cannot fuse with the synaptic mem-brane, and therefore, acetylcholine cannot be released. The 7serotypes differ in the specific component of the fusion ma-

chinery they target, their duration of action, their unit po-tency, and their immunogenic potential. Two serotypes, Aand B, are commercially available. BoNT-A is marketed un-der the trade names Botox (Allergan; throughout the world),Dysport (Ipsen; in Europe and elsewhere), and Xeomin(Merz; in Europe and elsewhere). BoNT-B is marketed byElan Biopharmaceuticals in the United States as Myobloc andin Europe as NeuroBloc. At effective antispasticity doses, all 4types have roughly the same duration of clinical action, ap-proximately 3 months. Potency is expressed in mouse units,the amount of toxin required to kill 50% of mice in a stan-dardized assay. Because of differences in molecular formula-

tion and other variables, the potency of a single unit variesgreatly among the commercial types. Although a unit of Bo-

tox is roughly as potent as 1 unit of Xeomin, 4 units ofDysport, and 40-50 units of Myobloc, it is important to rec-ognize that there is not a simple ratio of dosing equivalencies.Because of these differences, it is critical to specify the com-mercial brand when discussing units for dosing recommen-dations.

Most published research in CP has been conducted with

BoNT-A, although a small number of studies have examinedthe effect of BoNT-B in this population. Sanger et al injectedBoNT-B into the biceps and brachioradialis or 1 or both armsin 7 children with CP and upper extremity dystonia. In thisopen-label trial, reaching speed improved in response totreatment, although no dose-dependent effect was ob-served.44 BoNT-B and BoNT-A have both been combinedsuccessfully with phenol injection, allowing an increase inthe number of treated muscles per injection session.45

BoNT-B has a tendency to cause more autonomic side effectsthan BoNT-A, and should be used with this caution inmind.46

Although earlier studies of BoNT-A concentrated mainlyon demonstrating spasticity reduction via lowered Ashworthscores,47,48 more recent work has focused on determining thefunctional benefit from injections. Fehlings et al showed thatupper extremity injections of BoNT-A plus occupationaltherapy were superior to occupational therapy alone on theQuality of Upper Extremity Skills Test,49 an effect they cor-related with preinjection grip strength.50 Lower extremityfunction has also been shown to improve significantly; 51-53

however, this may not correlate with an improvement inhealth-related quality of life.54

Injections can also ease pain55 and have been shown byone group to be equivalent to fixed casting for improvementof dynamic calf tightness 56 but inferior to casting by oth-ers.57,58 The 2 can be combined, which some studies haveshown to produce better results than either alone.59-62 Cast-ing is usually delayed until the peak effect of the toxin, about2-4 weeks after injection, which produces superior results toimmediate casting.63

The functional improvements seen in randomized trialsare somewhat modest, but this may be more attributable tolimitations of study design than to the limitations of the ther-apy itself. Focal improvements in spasticity are difficult tocapture with global measures of function such as the GMFM,and the individualized dosing and injection patterns that

optimize therapy are usually not possible in rigorous trials.Practitioners and caregivers usually agree that the benefitsthey see from BoNT injection are greater than those revealedin clinical trials, although family satisfaction may not alwaysaccompany measurable functional gains.64 Even thoughthere may be no progressive improvement over the course ofrepeated injection cycles, the benefits of a single injectionseen early in treatment can persist for many injection cycleswithout waning.65

Dosing guidelines for BoNT-A as Botox, for adults andchildren, have been developed by consensus.3,66 With time,the recommended ceiling doses used by experienced practi-

tioners have increased from 4 to 16 U/kg (Botox) in somecases. In children, maximum dosing should take into consid-



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eration the childs weight, muscle bulk, and degree of spas-ticity. The lowest effective dose with an injection interval of atleast 3 months or more should be used to minimize the risk ofantibody development. Children may need a topical anes-thetic or light sedation during injection. Electromyography(EMG) or electrical stimulation is sometimes used to helptarget difficult-to-localize muscles, such as in the upper

extremities, although clinical examination is sufficient fordetermining affected muscles in many situations. Injection ofthe psoas requires visualization by ultrasound or othermeans. Some injectors potentiate the effects of injection withneuromuscular electrical stimulation applied over the injec-tion site for the first 3 days after injection. The family can beinstructed to perform this.

Adverse effects of injection are usually mild and transient,consisting of pain on injection, occasionally a mild flulikesyndrome, and excess weakness.67,68 The botulinum neuro-toxin complex is immunogenic, and repeated exposure canlead to immunoresistance. Rates of immunoresistance in

spasticity treatment have not been published, but the ac-cepted rate in adults with dystonia is approximately 3%.Recently, Allergan introduced a newbatch of Botox to replacethe original commercial batch that had been the source of allcommercial Botox until then. Initial reports indicate that thenew batch may be less immunogenic.69 If immunoresistancedevelops, it is possible to switch serotypes, although immu-noresistance to the second may develop more quickly than tothe first.70

Rhizotomy

Selective dorsal rhizotomy (SDR) was introduced in NorthAmerica in the early 1980s, and has since become a com-monly used treatment for reduction of moderate to severelower-extremity spasticity. During the procedure, the patientis positioned prone and the dural sac is exposed so that nerveroots from L2 to S2 can be identified. Individual nerve root-lets are identified and stimulated independently. Those affer-ent rootlets that elicit excessive activity, monitored via EMGand visual observation, are cut. Typically 25%-50% of root-lets are cut.71 Controversy exists about how accurately EMGidentifies the appropriate rootlets. Hays et al found no con-sistent relationship between the proportion of abnormally

responding rootlets and the degree of spasticity and grossmotor abnormality. They concluded that EMG monitoring asit is typically performed is unlikely to identify accuratelythose neural elements that contribute to spasticity in childrenwith CP.72

Results from clinical trials of SDR have indicated that itreduces spasticity, although the magnitude of the effect maybe variable. Engsberg et al showed that ankle spasticity couldbe reduced almost to normal by SDR, although the standarddeviations were large, indicating high response in some pa-tients and minimal response in others.73 The same groupobtained similar results in a measure of hip spasticity con-

ducted later.74

In a prospective study, Buckon et al demon-strated that SDR and orthopedic surgery can each contribute

significantly to improvements in movement over the longterm.75

The relationship of spasticity reduction to functional im-provement has also been controversial with this treatment, aswith others, and 2 investigator-masked trials of similar de-sign have reached opposite conclusions. Both groups ran-domized candidates to receive surgery plus PT or PT alone.

Both groups quantified functional changes with the GMFMand used a variety of measures to quantify spasticity changes.McLaughlin et al76 included 21 surgical and 17 PT-only pa-tients, with a 24-month follow-up, whereas Wright et al77

included 12 surgical and 12 PT-only patients, with a 12-month follow-up. Evaluators in both studies were blindedto surgical status. They differed in one significant aspect:McLaughlin et al sectioned a mean of 25% of rootlets vsapproximately 50% for Wright. In addition, patients in the

Wright study were significantly more functionally impairedat baseline than those in the McLaughlin study. Both groupsshowed significant postoperative reduction in spasticity with

SDR plus PT compared to that with PT alone, which, in thewords of McLaughlin, was judged by blinded investigators tobe clinically obvious and meaningful. However, in thatstudy, no long-term functional differences were seen be-tween the 2 groups, despite a 24-month follow-up in whichspasticity reduction might have been expected to afford theSDR group an advantage. In contrast, Wright et al showed a7.7 point difference in GMFM scores after 12 months thatfavored the rhizotomy group. Further studies will be neededto determine whether the different outcomes in these 2 stud-ies were attributable to differences in patient population, sur-gical technique, or some other variable.

Reduction in strength following SDR has historically beena concern, although more recent studies have not found ob-

jective loss of strength.73,74,78 A retrospective study of 158children who underwent SDR in a single center showed thatpostoperative complications occurred in up to 30% of pa-tients. Approximately 10% of patients had back pain starting6 months or more after surgery, sensory changes, and neu-rogenic bladder or bowel problems.79

In our experience, the best candidates for SDR are childrenfrom ages 4-8 for whom spasticity is more significant in thelegs than in the arms, with reasonably well-preserved legstrength and mobility, and whose spasticity is interferingwith that mobility. SDR is followed by intensive PT to remo-

bilize the child and improve strength. Despite best manage-ment, most children who require SDR will also eventuallyrequire orthopedic surgery to correct spasticity-induced de-formities. However, SDR may reduce the number of suchsurgeries required.80 Overall, the use of SDR has declinedwith the expanding indications for intrathecal baclofen.

Intrathecal Baclofen

Intrathecal baclofen (ITB) is delivered to the intrathecal spacevia a catheter attached to an implanted pump. Because ofdirect delivery to the central nervous system, the required

dose is less than 1% of that delivered orally, thus limiting theside effect of lethargy, which is especially of concern in this

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population. ITB has been shown to reduce spasticity in CP inseveral large trials.81-83 Some functional improvement hasbeen demonstrated as well.83,84 ITB is expensive, but a recentmodeling of cost-effectiveness indicated that over a 5-yearperiod, ITB, despite increasing cost of care by $49,000 com-pared to alternative treatment, added 1.2 quality-adjustedlife-years, which is considered good value for the money. 85

ITB is typically indicated for patients with lower-extremityspasticity, although studies have shown significant benefit onupper-extremity spasticity as well. The dimensions of thepumpabout the size and shape of a hockey pucklimit theuse of ITB in the very young children, although a smallerpump is available and has been implanted in children asyoung as age 3. Typically, candidates are screened with abolus injection of baclofen delivered via lumbar puncture. Acatheter trial with incremental dosing over several days isbeneficial in certain patients, especially those with dystoniaor other movement disorders. A negative response is an in-dication that further clinical evaluation may be necessary, for

instance, to rule out fixed contracture as the source of muscletightness.

The pump is implanted subcutaneously or subfacially inthe abdomen, and the catheter is advanced to the high tho-racic region. The pump contains a refillable reservoir, and analarm sounds when the reservoir is low. Refills, deliveredtranscutaneously, are required every 2-3 months. The pumpis programmable via a telemetry wand, and the dose may beadjusted to serve different functions over the course of theday, for instance, increasing at night to aid in comfort forsleep and decreasing in the morning to aid transfers.

Chronic ITB use carries a small but significant risk of seri-ous complications. Overdose from a programming error ispossible, and may lead to respiratory depression and coma.

Abrupt withdrawal caused by emptying of the reservoir,pump failure, or catheter withdrawal, kinking, or breakage ismore common. More than a dozen cases of a withdrawalsyndrome and 6 deaths have been reported in the literature.86

Symptoms develop over 1-3 days and include prodromalitching or paresthesias, rebound spasticity, priapism in men,tachycardia, hypotension or labile blood pressure, dysphoriaevolving to decreased consciousness, and the potential forseizures. The most effective treatment is the prompt restora-tion of ITB therapy. Intravenous benzodiazepines and high-dose oral or enteral baclofen, along with life-support mea-

sures, may be needed until restoration is possible.

Orthopedic Surgery

Despite best medical and rehabilitation management, chil-dren with spastic CP will often eventually require orthopedicsurgery to correct deformities induced by muscle overactiv-ity. Uncorrected deformities can cause pain, interfere withmobility or care, and lead to subluxation. It is estimated thathip subluxation or dislocation occurs in up to 25% of chil-dren with CP.87 Correction typically involves multiple teno-tomies and transfers of the thigh adductors and, in severe

cases, femoral osteotomy. Equinovarus foot is the most com-mon deformity seen in CP, resulting in a flexed ankle and

turned-in foot, sometimes with toe curling. Treatment ofteninvolves tendon lengthening. An advance is the SPLATTsplit anterior tibial transferin which the tendon is splitalong its length and the distal end of the lateral half is trans-ferred to the cuneiform and cuboid bones, where it can exerta corrective pull across the joint. This procedure is typicallycombined with Achilles tendon lengthening and toe flexor

release. Treatment may also be prescribed for the knee andthe upper extremities, depending upon the degree of defor-mity in these joints.

Orthopedic procedures are best performed sometime aftergait has matured, usually between the ages of 6 and 10. As faras possible, the multilevel procedures are performed all atonce to minimize postsurgical recovery times, and thechild ismobilized as quickly as possible. PT remains a central part oftreatment to strengthen and improve range of motion innewly realigned limbs.

Conclusions

Muscle overactivity can be a significant source of functionaldisability in a child with CP. Treatment planning is centeredon improving function, comfort, and care; reducing pain;and preventing or correcting deformity. Oral medications,chemodenervation, rhizotomy, intrathecal baclofen, and or-thopedic surgery may all play a role in treatment of the prop-erly selected child. Physical and occupational therapy arecentral to any treatment plan.

AcknowledgmentsThe author thanks Richard Robinson for his assistance in

preparing this manuscript.

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