Assessment andTreatment of Children

with Cerebral Palsy

Gilbert Chan, MDa,*, Freeman Miller, MDbKEYWORDS

Orthopedics Cerebral palsy Hips Spine Spasticity Neuromuscular Feet

KEY POINTS

Children with cerebral palsy are prone to development of musculoskeletal deformities. The underlying neurlogic insult may results in a loss of selective motor control, an increase inunderlying muscle tone, and muscle imbalance, which lead to abnormal deforming forces actingon the immature skeleton.

The severely involved child is one who is at increased risk for developing progressive musculoskel-etal deformities.

Close surveillance and evaluation are key to addressing the underlying deformity and improving andmaintaining overall function.INTRODUCTION

Orthopedic management of children with cerebralpalsy is a challenging task. The presentation ishighly variable, ranging from those with mildclinical manifestations to those who are severelyinvolved. The critical part in the initial assessmentof children with cerebral palsy is the identificationof risk factors for development of deformities sothat attempts can be made to circumvent theseevents. This, in turn, maintains or improves achilds overall function.

Cerebral palsy is characterized by an injury orinsult to the immature brain. This may occurbefore, during, or up to 5 years after birth. The pa-thology in the brain is permanent and nonprogres-sive. It results in a wide variety of postural andmovement disorders. Clinical manifestations aredetermined by the timing of the injury and whetherthey occur in the preterm (immature) or term(mature) infant. The underlying pathology canThe authors have nothing to disclose.a Childrens Orthopedics of Louisville, Kosair ChildrensLouisville, KY 40207, USA; b Alfred I. DuPont Hospital19803, USA* Corresponding author.E-mail address: Gilbert.Chan@nortonhealthcare.org

Orthop Clin N Am 45 (2014) 313325http://dx.doi.org/10.1016/j.ocl.2014.03.0030030-5898/14/$ see front matter 2014 Elsevier Inc. Allpoint to probable patterns of involvement. Animmature or preterm infant with periventricular leu-komalacia typically presents with spastic diplegia,whereas a child with periventricular hemorrhage ismore likely to present with hemiplegia. Cerebellarinvolvement may present with hypotonia andataxia. Occasionally, more than one lesion existsin the brain, resulting in a mixed presentation. Afull-term child with watershed ischemia betweenthe anterior and middle cerebral artery presentswith quadriparesis whereas a focal ischemic injuryin a full-term child presents with hemiparesis.Although the brain lesion is static, its manifesta-tions are progressive. The primary manifestationsof the neurologic insult include loss of selectivemotor control alteration in muscular balanceand muscle tone abnormalities. This results in sec-ondary manifestations of abnormal growth anddevelopment of the musculoskeletal system. Itsignificantly affects a childs function, includingHospital, 3999 Dutchmans Lane, Plaza 1, 6th Floor,for Children, 1600 Rockland Road, Wilmington, DE

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Chan & Miller314abnormalities in gait and ambulation. The com-pensations that children undertake to overcomeor adapt to these secondary manifestations aretermed, tertiary manifestations.1 It is in addressingthe secondary and tertiary manifestations whereorthopedists take a lead role, with the goal of cor-recting lever arm dysfunction, preventing progres-sion of deformity, and optimizing overall function.In a sense, the role of orthopedic surgeons is tomaintain, improve, or optimize a childs functionand alter the natural history of the condition.CLASSIFICATION

Classification systems help define and quantifythe underlying pathology. They help determineand guide clinicians toward the most appropriatetreatment and aid in communication between cli-nicians. Several classification systems have beenproposed, dating as far back as the 1800s. Themost comprehensive of these is the classificationproposed by Minear in 1956,2 which takes intoconsideration every aspect of a child, includingphysiologic, topographic, etiologic, traumatic,neuroanatomic, functional, and therapeutic in-volvement. Bax and colleagues3 proposed asimpler classification system based on motorabnormalities, associated impairments, anatomicand radiographic findings, and causation andtiming. Functional classification schemes arealso commonly used and these are based on achilds overall functional capability. The GrossMotor Functional Classification System (GMFCS)is the most widely used functional scheme. It di-vides children into 5 groups based on the overallfunctional capability (Table 1).4 The FunctionalMobility Scale (FMS) describes motor functioninto 6 levels in 3 domains based on typicalwalking distance of 5, 50, or 500 m. This systemis used to monitor change in motor function overtime.5Table 1Gross motor function classification system

GMFCSLevel Description

I No functional impairment

II Functional limitation, may needassistive device

III Assistive device needed forambulation

IV Limited self-mobility, wheelchairoften required

V Wheelchair boundASSESSMENT

Assessment of children with cerebral palsy cen-ters on a complete history and physical evaluation.The history must include birth history and associ-ated underlying medical conditions. The clinicalevaluation should consist of a clinical evaluationof gait, both barefoot and with the use of orthotics,if any. Rotational profile should be checked toevaluate underlying torsional malalignment. Rangeof motion should be checked for the presence ofany contractures. The specific underlying muscletone is evaluated and recorded. A more detailedevaluation may include strength testing and anevaluation for selective motor control. Orthotics,assistive devices, and wheelchairs are evaluatedto ensure that they fit properly.Radiographs should be taken on the first ortho-

pedic visit to establish a baseline; this is parti-cularly true of the hips and pelvis. The severity ofinvolvement and amount of deformity presentdictate the frequency and need for sequential im-aging studies on follow-up visits. Other advancedimaging techniques may be required prior to plan-ning of reconstructive procedures.A comprehensive gait analysis can be per-

formed to obtain an objective assessment of thegait pattern that could be measured and quanti-fied. This information can be further assessed tohelp in preoperative planning. It also provides apermanent record to compare the outcome ofsurgery. Studies have shown that gait analysismay aid and improve in surgical decision makingin children with cerebral palsy.HIPS

The hips in children with cerebral palsy are normalat birth. The deformity occurs from loss of selec-tive motor control and abnormalities in muscletone and balance. Such deformities includecoxa valga, femoral anteversion, and acetabulardysplasia. The muscular imbalance is typicallydue to strong hip flexors and adductors overpow-ering the hip extensors and abductors. The rate ofhip subluxation in cerebral palsy has been re-ported as high as 75%.615 It is related to a childslevel of function. Lonstein and Beck7 found therate of hip subluxation 11% in ambulators and57% in nonambulators. Root8 reported the inci-dence of dislocation to be 8% and subluxation38%. Soo and colleagues16 found no dis-placement in children who were GMFCS 1% andfound 90% displacement in children who wereGMFCS V. Increased femoral anteversion andcoxa valga were also noted to be related to chil-drens GMFCS level; Robin and colleagues17

Treatment of Children with Cerebral Palsy 315found femoral anteversion and neck shaft angles30 and 135.9, respectively, in those who wereGMFCS I and 40 and 163, respectively, in thosewho were GMFCS V.

Close surveillance of the hip joint is necessary.Identifying the hip at risk can lead to early andappropriate intervention to prevent the long-termsequelae of an untreated hip. Initial evaluationshould include the hip range of motion; the pres-ence or absence of contractures; pelvic obliquity;spinal deformity, if any; and femoral anteversion.Radiographic evaluation of the hip should quantifythe amount of subluxation, if any. This is best as-sessed by the Reimer migration index (migrationpercentage) (Fig. 1), which is the measurementof the width of the uncovered femoral head relativeto the total width of the femoral head. In childrenwith cerebral palsy, the migration index is believedwithin acceptable limits if it is below 30. Anincreasing migration index has been correlated tothe increased risk for hip dislocation. Hagglundand colleagues9 reported that hips with a migra-tion index greater than 40 had a high risk for dislo-cation and require treatment. Miller and Bagg18

reported that children with a migration index below30 were at low risk for dislocation and those with amigration index of greater than 60% had completedislocation. The acetabular index is used to eval-uate and quantify acetabular dysplasia (seeFig. 1). Acetabular index of less than 20 is consid-ered normal in adulthood. In children below 5 yearsFig. 1. Anteroposterior radiograph taken of the pelvisexhibiting measures of both the acetabular index (A)and Reimer migration index; (B and C) the angle sub-tended by Hilgenreiner line and the acetabular roofforms the acetabular index (A). The amount of thefemoral head extruded (B) divided by the entire widthof the femoral head (C) multiplied by 100 equates tothe Reimer migration index.of age, 25 is considered normal. An increasedangle may denote the need to address the pelviccomponent of the deformity during surgical recon-struction. Radiographically, the amount of coxavalga may be assessed by measuring the neckshaft angle, which is typically increased. The ra-diographs must be taken with the hips in internalrotation to get an accurate measurement of theproximal femur.19 In more complex deformities, aCT scan may be useful in preoperative planning.

A severely involved child who is at an increasedrisk for the development of hip dysplasia andsubsequent dislocation should be observedclosely, with radiographs taken at regular intervals(approximately 6 months). In more functionalambulatory children, a baseline radiograph shouldbe obtained and the need for follow-up radio-graphs should be at the discretion of the physicianbased on a childs clinical evaluation. This maydepend on whether there are initial concerns onthe radiographs or if there are changes in the rangeof motion of the hip during regular evaluations.Nonoperative modalities should focus on main-taining hip range of motion, with or without formalphysical therapy. Hip abduction bracing maybe at-tempted, but it may be difficult to maintain in thepresence of a childs underlying tone. Focal spas-ticity management may be performed to improverange of motion and allow children to toleratebracing better. Short-term studies have showninitial benefit with the use of botulinum toxin,particularly in younger children. These findingshave not been substantiated, however, by otherstudies. In a randomized study, Graham and col-leagues20 reported a 1.4% decrease in the rateof hip displacement and they did not recommendbotulinum toxin. In a long-term study, Willoughbyand colleagues21 showed that botulinum toxin in-jection combined with abduction bracing did notsignificantly reduce the rate of hip reconstructivesurgery or influence the development of the hipsat skeletal maturity. The current recommendationis to get one anteroposterior pelvis radiograph be-tween 2 to 4 years of age for GMFCS I and II (inde-pendent ambulators) and one radiograph everyyear until age 8 and then every 2 years until skel-etal maturity for GMFCS III, IV, and V as long asthe migration index is less than 30. If the migrationindex is more than 30, more frequent radiographsand possible intervention should be planned.

Operative modalities are intended to preventprogression of deformity or to address an estab-lished deformity, subluxation, and/or dislocation.Soft tissue procedures may be carried out to main-tain and improve hip range of motion. Miller andcolleagues22 performed iliopsoas and adductorlengthening in children with hip abduction of less

Chan & Miller316than or equal to 30 and migration percentages ofgreater than or equal to 25%. At a mean follow-upof 39 months, 54% had good and 34% had fairoutcome. The investigators concluded that earlydetection and intervention can lead to satisfactoryoutcome in 80% of children with spastic hips. Thisstudy, however, did not answer the question as tohow many children would eventually require bonyreconstruction. In a later long-term follow-upstudy, Presedo and colleagues23 reported thatsoft tissue releases were effective for preventionof hip dislocation. The best predictors of goodoutcome in their study were ambulatory statusand migration percentage. In those children whodevelop progressive subluxation or dislocation,hip reconstruction may be required (Fig. 2). Duringsurgery, the deformity of the proximal femur andthe acetabulum are assessed and addressed. Inchildren with progressive subluxation or disloca-tion without significant acetabular dysplasia, afemoral varus derotation osteotomy combinedwith appropriate soft tissue procedures is oftensufficient. In those with significant acetabularinvolvement, a pelvic osteotomy may be required.The osteotomy is performed to address the defi-ciency, which, in children with cerebral palsy, ismore commonly posterior.24,25 Outcomes afteroperative intervention have been favorable.McNerney and colleagues26 reviewed 104 hipswith a mean follow-up of 6.9 years. A total of95% of the hips remained well reduced and therewere no redislocations. Similarly, Miller and col-leagues,27 in a review of 49 subluxated and 21 dis-located hips, reported 2 hip redislocations at amean follow-up of 34 months; 82% of cases hadcomplete pain relief. The current recommenda-tions are as follows: children under the age of8 years and with migration index between 30%Fig. 2. (A) A 7-year-old child with quadriplegic cerebral pwith significant coxa valga and acetabular dysplasia; a dePatient underwent correction of both hips. Soft tissue releosteotomies and bilateral pelvic osteotomies to correct thtive radiographs taken show excellent correction of deforand 60% should undergo adductor and iliopsoaslengthening, and children over the age of 8 yearswith migration percentage greater than 40% andall children with migration index greater than60% should be recommended for hip reconstruc-tion with femoral varus shortening osteotomy, pel-vic osteotomy, and adductor lengthening.In the skeletally mature hip, treatment is more

challenging. Pelvic osteotomies, such as theDega osteotomy or the Bernese periacetabular os-teotomy, could be performed2832 but the resultsmight not be as optimal. Often the severity ofdeformity dictates the most appropriate course.The results of primary reconstruction in hips withchronic degenerative changes and significantdeformity (Fig. 3) may be dismal because painmay persist despite reconstruction. A fixed, pain-ful, subluxated, or dislocated hip in a more maturepatient with cerebral palsy often presents with atreatment conundrum. Typically, the hip showsdenudation and loss of cartilage. Hip replacementcan be performed and has the advantage ofpreserving hip motion. There are, however, sub-stantial risks of hip dislocation, infection, andimplant-related complications. Raphael and col-leagues33 reported on 56 patients (59 hips) whounderwent total hip arthroplasty. Patients wereroutinely placed in a unilateral hip spica postoper-atively for 3 weeks. All patients had a minimumfollow-up of 2 years (mean 9.7 years). There wascomplete pain relief in 48/59 (81%) hips and 52/59 (88%) returned to their prepain GMFCS level.Revision rate was 15%. There were 8 dislocations(14%). Two-year implant survivorship was 95%,and the10-year survivorship was 85%. Alter-natively, Gabos and colleagues34 performedinterpositional arthroplasty in 11 GMFCS V (nonweight-bearing) patients (14 hips) and achievedalsy, exhibiting progressive subluxation of both hipscision was made to undergo surgical intervention. (B)ases were done followed by bilateral varus derotatione acetabular component of the deformity. Postopera-mity.

Fig. 3. (A, B) Photographs of a resected hip from an 15-year-old child with quadriplegic cerebral palsy. He hadpresented with significant hip pain and a windswept deformity and a decision was made to perform a resectionof the hip. Resected specimen shows denudation of the superolateral portion of the hip with complete loss ofcartilage.

Treatment of Children with Cerebral Palsy 317improvement in pain in 10 of 11 cases. Proxi-mal femoral resection and interpositional arthro-plasty were initially introduced by Castle andSchneider.35 McCarthy and colleagues36 revisedthe technique to perform the resection at the levelof the ischial tuberosity; in their series, all but 1patient achieved improvement in seating. The con-cerns related to proximal femoral resection areheterotopic ossification, increased time to pain re-lief, prolonged hospital stay, and migration of theproximal femur. To address the issue of hetero-topic ossification, Egermann and colleagues37

used femoral head to cap the proximal femurand showed a decreased rate of heterotopic ossi-fication. Another option in lieu of proximal femoralresection is a valgus osteotomy (McHale proce-dure). The goal of this procedure is to aim thefemoral head away from the acetabulum whileallowing for an indirect transfer of load. Severalstudies have shown an improvement in seatingand pain relief.38,39 Leet and colleagues40 com-pared the results of proximal femoral resectionwith a valgus osteotomy with a mean follow-upof 3.4 years. Those treated with the McHale proce-dure had a shorter hospital stay and decreasedproximal femoral migration. Both groups achievedimproved seating and caretaker satisfaction.KNEES

Anterior knee pain may present as a significantissue in children with cerebral palsy. It is oftenrelated to the patellofemoral joint. The commoncauses of anterior knee pain include patella alta,patellar subluxation or dislocation, quadricepsweakness, angular deformity, and rotational mala-lignment. Senaran and colleagues41 looked atpatients with anterior knee pain secondary topatellafemoral symptoms; in their study, the pa-tients were classified based on patella alta, frac-ture of the inferior pole of the patella, and patellarsubluxation or dislocation. The investigators advo-cated aggressive treatment to prevent futuredeterioration.

Knee flexion contractures frequently occur inchildren with cerebral palsy. It is more severe innonambulatory children. In younger children (age

Chan & Miller318Several options exist to correct crouch gait.Initially, a ground reaction ankle foot orthosismay be used to aid in achieving knee extension.Surgical intervention for correction of crouchincludes lengthening of the contracted muscletendon units. Rethlefsen and colleagues51

showed, however, that repetitive hamstringlengthening does not result in long-term correctionof crouch. A complete evaluation of children needsto be undertaken prior to surgical intervention.Correction of all underlying deformities and resto-ration of the knee extensor mechanism are es-sential in correction of crouch gait. Stout andcolleagues52 showed that adequate correctioncan be achieved by addressing the knee flexioncontracture through a distal femoral extension os-teotomy (DFEO) and the patella alta with anadvancement and transfer of the patellar tendon.In their series, children who underwent a com-bined DFEO with patellar tendon advancementdid better than children who underwent DFEOalone or patellar tendon advancement alone.53 Ina majority of cases of undergoing a DFEO,hamstring lengthening does not seem required.54

Correction of the other aspects of lower extremitymalalignment, such as rotational malalignment(tibial torsion or femoral anteversion), planovalgusfeet and muscle imbalance should be undertakento restore and improve overall function. In asmaller series, Rodda and colleagues55 showedthat the use of multilevel orthopedic surgery waseffective in improving the knee extensor mecha-nism, relieving knee pain, and achieving improvedfunction in children with crouch gait. Childrenshould not be allowed to develop severe crouchto the point where they lose function and ambula-tory potential and become wheelchair bound.Correction is indicated when the crouch increasesor a childs functional ability decreases.Stiff knee gait results from increase spasticity of

the rectus femoris muscle and is one of the com-mon gait patterns seen in cerebral palsy. It inter-feres with the swing phase of gait by keeping theknee in extension, hence interfering with foot clear-ance. During the normal gait cycle, the rectus fem-oris is active during toe-off and inactive duringmidswing; it then reactivates during terminal swingand early stance to allow and prepare the limb forload acceptance. In stiff knee gait, the rectus fem-oris seems active during the entire swing phase ofgait.56,57 Stiff knee gait is defined as decreasedmagnitude of peak knee flexion of less than 45,decreased range of knee flexion, and delay inpeak knee flexion.58 This may cause frequent trip-ping and falling. Other contributors to stiff knee gaitinclude femoral torsional malalignment, poor push-off from the ankle, and weak hip flexor power.Indications for treatment of stiff knee gait includedecreased peak knee flexion, delay in time to peakknee flexion, and rectus femoris activity duringswing phase of the gait. Transfer of the rectus fem-oris is the treatment of choice for stiff knee gait.The area or the site to where the rectus femoris istransferred does not seem to show any differencein the outcome of treatment when the area ofthe transfer is evaluated.59,60 It seems that theimprovement in peak knee flexion is maintainedlong term.61 Although the results of simple releaseof rectus femoris in the treatment of stiff knee gaithave not been good, there have been good resultswith a rectus femoris tendon resection of 4 to6 cm62; rectus femoris transfer still seems superiorto a release of the muscle in treatment of stiff kneegait.63,64 It seems that when the rectus femoris isfiring out of phase, when it fires predominantly dur-ing swing as documented by electromyography, iswhen maximal benefit can be achieved with arectus femoris transfer.65FEET

The feet are an essential part of gait providing astable base of support to allow for standing andambulation. The foot consists of 2 columnsthelateral and the medial columns, and 3 partsfore-foot, midfoot, and the hindfoot. It is essential thatall these segments be assessed to correct theunderlying deformity. The underlying muscularimbalance in cerebral palsy plays a large role inthe development and progression of deformity.The necessity of correcting the foot in ambulatorychildren relies on restoring the lever arm to allowfor appropriate push off, especially in childrenwith a crouch gait pattern. In nonambulatory chil-dren, correction of deformity is centered onachieving a corrected foot that allows for shoewear, bracing, standing, and pain relief. If thedeformity is flexible, it may be amenable to bracingor soft tissue procedures. Radiographs may beused to document malalignment in various footsegments. Gait analysis with an electromyogrammay be performed to assess and evaluate dy-namic muscle imbalance. Dynamic pedobaro-graphs may be used to assess plantar pressuredistribution.Planovalgus and equinovarus are the common

deformities seen in cerebral palsy. In achievingappropriate correction of the foot, the role of thegastrocnemius cannot be stressed enough. Thetightness of the Achilles tendon exacerbates thedeformity, preventing the calcaneus from comingdown to achieve an appropriate correction; mostof this contracture tends to occur in the gastrocne-mius and not the soleus. Lengthening of the

Treatment of Children with Cerebral Palsy 319gastrocsoleus mechanism should be undertakenwith great caution in diplegic children, becauseoverlengthening may lead to exacerbation orworsening of crouch gait from weakening of theplantar flexors.48 Correction of the planovalgusfoot begins with correction of segmental malalign-ment. This is best achieved by lengthening of thelateral column.6668 Once the hindfoot and lateralcolumn are lengthened and the forefoot is broughtover, the medial side of the foot should be as-sessed. If full correction of the foot is achieved,then the initial lengthening of the lateral columnmay be all that is needed. If residual deformity inthe forefoot is still present and the first ray remainsin supination, a plantar flexion osteotomy of thefirst ray may be performed either through thecuneiform or the first metatarsal. In more severecases, a talonavicular arthrodesis may be re-quired.69 The specific method of correction isdependent on the severity of the deformity and achilds age. Seldom should surgery be consideredfor children younger than 8 years of age, becausethe deformity can usually be managed with or-thotics. In children up to 4 years of age, significantcorrection can occur as part of the natural historyof the disease, with no treatment required. Mostsurgery for planovalgus deformity correctionshould be done after age 10 years, becausecorrection is much better maintained. The specificcorrection for lateral column lengthening can bedone through lengthening of the calcaneus. Thisprocedure works well for children with excellentfunctional gait, usually in those who ambulatewithout any assistive device. For children with hy-potonia, poor muscle control, and more severegait problems requiring walkers or doing standingtransfers, lateral lengthening is best done with cor-rect reduction of the calcaneus to the talus fol-lowed by a subtalar fusion. Medial columncorrection must correct the forefoot supination;in mild cases, this correction may be achievedwith excision of the navicular tuberosity andadvancement of the tibialis posterior. In more se-vere cases, an osteotomy and correction at theapex of the deformity can be done, which is usu-ally at the cuneiform to bring the first ray down; afusion at the talonavicular joint can also be per-formed. Usually, a fusion of the joint at the apexhas the best long-term results in the authorsexperience. Often the gastrocnemius is con-tracted with large differences between foot dorsi-flexion with the knee flexed and with the kneeextended. Usually, a lengthening of the gastrocne-mius is required, but lengthening at the level of theAchilles tendon should be avoided, if possible,because this only further lengthens the soleusunnecessarily.The equinovarus foot is more common in hemi-plegic children. The treatment relies on balancingor removing the deforming force and addressingthe bony deformities. In mild cases, soft tissuecorrection may suffice; this may take the form ofsplit tendon transfers.70,71 Complete tendon trans-fers are not advocated. In the more severe cases,soft tissue procedures in combination with bonyprocedures, such as a sliding calcaneal osteotomyor a closing wedge osteotomy for residual varus isrequired. In the most severe cases, an arthrodesismay be required.72 Posterior tibialis tendon sur-gery prior to age 8 years should be avoidedbecause of a high rate of overcorrection. In hemi-plegia and children over age 8 years, the tibialisposterior is the most common overactive tendonand a split transfer to the peroneus brevis is a reli-able procedure. When the varus is accentuatedduring the swing phase of gait or if varus seemsmainly from the forefoot, then a split transfer ofthe tibialis anterior is a good option. Dynamic elec-tromyogram is helpful to sort out these 2 options.Almost all varus deformities are associated withequinus and need to have a lengthening of thegastrocnemius or tendo-Achilles depending onthe source of contracture. An open Z-lengtheningof the tendo-Achilles is safer then blind percuta-neous approaches.

Forefoot deformities also occur often in combi-nation with other deformities. Hallux valgus canbe managed with toe straps in the brace. Surgicalcorrection is reserved for recalcitrant deformities.Dorsal bunions may be secondary to an underlyingmuscle imbalance or from an iatrogenic injury.Surgery is reserved for recalcitrant cases thatinterfere with shoe wear or bracing.SPINE

The development of spinal deformity has been welldescribed in children with cerebral palsy. Spineinvolvement, as with the development of any otherdeformity in cerebral palsy, is related to the func-tional level of the child. Scoliosis is the most com-mon form of spinal deformity in cerebral palsy; itsincidence has been reported as high as 77% insome studies.7376 A majority of these studiesagree that the incidence of scoliosis increaseswith the severity of involvement of a child.Koop73 found a higher incidence of scoliosis in pa-tients with quadriplegic involvement. Their studyshowed that 30% of quadriplegic childrendeveloped scoliosis of greater than 40 at skeletalmaturity compared with only 2% in children withhemiplegia. In a more recent study, Persson-Bunke and colleagues77 analyzed the relationshipof the development of scoliosis with childrens

Chan & Miller320GMFCS level. In their series, only children withGMFCS level IV and V developed significant scoli-osis of greater than 40.A majority of children with cerebral palsy mani-

fest with a long C-shaped curve that often involvesthe pelvis. This curve pattern is more typical inthose who are severely involved, in particularquadriplegics. Other curve types, similar to thetypes seen in idiopathic scoliosis, do occur in themore functional individuals. The natural history ofscoliosis in cerebral palsy is well described.Severely involved children who develop scoliosisat a younger age (

Fig. 4. (A) Initial radiographs taken of a 15-year-old boy with quadriplegic cerebral palsy. He was noted to havean XX degree curve; continued observation was advised. (B, C) On follow-up PA and lateral radiographs taken XXmonths later, he was noted to have progression of his curvature to XX degrees. (D) Traction films taken showexcellent correction of his curvature with improvement of pelvic obliquity. (E, F) Postoperative films after aposterior spinal fusion was performed, which shows excellent correction of the curvature and pelvic obliquity.The child is sitting comfortably with a well-balanced spine.

Treatment of Children with Cerebral Palsy 321often required to control and correct pelvic obliq-uity.85 In most cases, the goals of surgery can beachieved through a posterior only approach,although occasionally, for a large, rigid curve, ananterior and posterior approach may be required.The necessity to go anterior can be determinedby both clinical methods and radiographicmethods. Clinically, the deformity and pelvis canbe assessed for flexibility by applying traction orby using the side bending test.86 Radiographically,flexibility films can be used to determine spinalflexibility.87,88 Traction films are particularly usefulin evaluating pelvic flexibility. Inability to correctthe pelvis often necessitates an anterior release

Chan & Miller322to provide additional flexibility to the spine.89 Inaddition to adding flexibility, an anterior approachmay be required in addressing the spine inyounger children to prevent crankshaft phenome-non of the spine. Performing an anterior surgeryis not without its risks. Significant postoperativecomplications may occur after an anteriorapproach. Pulmonary complications can occurespecially if the thoracic cage is entered. The sur-gery may be performed either on the same day orin a staged fashion. Increased blood loss, pro-longed operative time, and higher complicationrates have been noted with sequential (single-stage) procedure.90 Given the increased risk andmorbidity associated with an anterior procedure,proper patient selection, patient preparation, andpreoperative planning are required prior to sur-gery. Ideally, if surgery can be performed earlywith the spine maintaining adequate flexibility,then an anterior procedure can be obviated.The choice of instrumentation is surgeon depen-

dent. Several instrumentation techniques havebeen used successfully to address the deformity;these include the unit rod, Luque construct,Galveston technique, Cotrel-Dubousset instru-mentation, third-generation instrumentation, andcombinations thereof, which have been used suc-cessfully in the treatment of neuromuscular scoli-osis.9196 The results after treatment with thevarious instrumentation systems have been com-parable. When choosing instrumentation systems,surgeons must be reminded that the goal is not toachieve a straight spine but to correct the pelvicobliquity, achieve a balanced spine, restoreseating ability, and relieve pain. The significanteconomic cost of newer-generation instrumenta-tion must also be considered. Another factor forconsideration is implant prominence, becausesoft tissue coverage may prove an issue in someof these children and, because most of these chil-dren are severely involved, osteopenia and poorbone quality in the areas of fixation may be a keyissue during surgery. At the end of the day, thechoice of instrumentation system relies on sur-geon familiarity and preference.SUMMARY

The orthopedic manifestations of children with ce-rebral palsy are wide and varied, with the clinicalpresentation directly correlating with the severityof a childs involvement. Although the underlyinginjury is static, its effects on a childs musculo-skeletal system are progressive throughout thegrowing years. Close clinical surveillance andobservation are needed to address these defor-mities as they develop and progress. The goal oftreatment should not focus on the specific defor-mity but on the child as a whole, in an attempt toimprove overall function.REFERENCES

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Assessment and Treatment of Children with Cerebral PalsyKey pointsIntroductionClassificationAssessmentHipsKneesFeetSpineSummaryReferences