Hemiplegic or Unilateral CerebralPalsy Gait

Freeman Miller

ContentsIntroduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2

Natural History and Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2Etiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2

Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3Hemiplegia Type 0 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4Hemiplegia Type 1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5Hemiplegia Type 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5Outcome of Plantar Flexor Tendon Lengthening . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6Rotational Deformities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7Hemiplegia Type 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7Stiff Knee Gait . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8Rotational Deformities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8Hemiplegia Type 4 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9Rotational Deformities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9Limb Length Discrepancy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10

Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10

Cases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11

Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18

AbstractHemiplegic pattern cerebral palsy (CP) meansthe motor lesion is primarily located on oneside of the body usually involving both the armand a leg. Another synonymous term is unilat-eral CP. There are many children who haveprimary involvement on one side of the body;

however, they may have also some contralat-eral abnormalities. There are no clear defini-tions of when unilateral or hemiplegic patternCP becomes bilateral or diplegia or quadriple-gic pattern CP. Hemiplegic pattern CP makesup approximately one third of all children withthe diagnosis. The vast majority of childrenwith hemiplegic pattern CP tend to be high-functioning community ambulators with GrossMotor Function Classification System(GMFCS) I or II. Large majority of individuals

F. Miller (*)AI DuPont Hospital for Children, Wilmington, DE, USA

# Springer International Publishing AG 2018F. Miller et al. (eds.), Cerebral Palsy,https://doi.org/10.1007/978-3-319-50592-3_101-1

1

with hemiplegic pattern CP become full andnormal independent functioning members inthe society. The functional ability of individ-uals with unilateral CP tends to be much moreinfluenced by concurrent cognitive disabilityor epilepsy and then motor impairment. Manychildren with unilateral CP do develop muscu-loskeletal deformities impairing their gait pat-tern and upper extremity function, which areamendable to surgical correction. The Winter’sclassification divides hemiplegic gait into fourpatterns. Type 1 has ankle plantar flexion inswing phase with an inactive or very weaktibialis anterior, which is the cause of the plan-tar flexion. Type 2 has an equinus gait patternbut with spastic or contracted plantar flexors,which overpower an active dorsiflexor. Type3 includes the ankle position of type 2, furtheradding abnormal function of the knee joint.Type 4 includes all problems of type 3 withthe addition of abnormal function of the hipjoint muscles. The separation of these types isusually easy through a combination of physicalexamination, EMG, kinematic evaluation, andkinetic data. As with all biological groups,however, there are intermediate patients. Thegoal of this chapter is to review the naturalhistory and treatment plan for the individualwith hemiplegic pattern CP.

KeywordsCerebral palsy · Hemiplegia · Unilateral ·Winter’s classification

Introduction

Hemiplegic pattern cerebral palsy (CP) means themotor lesion is primarily located on one side of thebody usually involving both the arm and a leg.Another synonymous term is unilateral CPa. There are many children who have primaryinvolvement on one side of the body; however,they may have also some contralateral abnormal-ities. There are no clear definitions of when uni-lateral or hemiplegic pattern CP becomes bilateralor diplegia or quadriplegic pattern CP. Althoughmost publications present these groupings as

absolute distinct, there is not a clear separationbetween the groups, and as a consequence, a sig-nificant number of children can be categorizedeither way. Hemiplegic pattern CP makes upapproximately one third of all children with thediagnosis (Andersen et al. 2008). The vast major-ity of children with hemiplegic pattern CP tend tobe high-functioning community ambulators withGross Motor Function Classification System(GMFCS) I or II. Large majority of individualswith hemiplegic pattern CP become full indepen-dent normal functioning member in the society.From the perspective of the International Classifi-cation of Functioning, Disability, and Health(ICF), these individuals will often have high par-ticipation rates. The functional ability of individ-uals with unilateral CP tends to be much moreinfluenced by concurrent cognitive disability orepilepsy and then motor impairment (Beckunget al. 2008). The goal of this chapter is to reviewthe natural history and treatment plan for the indi-vidual with hemiplegic pattern CP.

Natural History and Pathophysiology

Etiology

The central nervous system pathology whichcauses hemiplegic pattern CP tends to be primar-ily localized to one hemisphere of the brain. Thereare multiple etiologies, which can create this uni-lateral lesion, but a common cause is third trimes-ter anterior cerebral artery infarction (Darmency-Stamboul et al. 2012). A risk factor for this lasttrimester infarction may be a familial hyper-coagulable state. There are questions about howaggressive the work-up should be for childrenwith hemiplegia to determine the presence of afamilial hypercoagulable state especially lookingfor issues such as factor V Leiden deficiency,protein S, and protein C. One study has reportedthat screening for these in a population of childrenwith hemiplegic CP compared with a normalcohort of children found a similar incidence(Turedi Yildirim et al. 2015). Based on currentevidence, the required work-up for a child whopresents with a typical hemiplegic pattern CP is

2 F. Miller

still not clear. There are no clear benefits to doingany hematologic work-up if the family historydoes not suggest a hypercoagulable state.

The most common presentation of hemiplegicpattern CP is parents noticing that the child ispreferentially using one hand for upper extremityactivities. This may be observed as early as 6–-9 months of age, when typical hand dominanceshould not be presenting until 18–24 months.There may also be a delay in independent walkingespecially with asymmetric toe walking. It is com-mon for the initial toe walking to be more sym-metric then suggested by the physicalexamination of mild increased muscle tone andlikely reduced range of motion in the affectedankle. The hand is often in a fisted position withthe thumb held in the palm. When the child iscrawling, they may be crawling on the fistedhand or occasionally even utilizing the dorsumof the right wrist as a support. In this early stagebetween 9 and 18 months of age, the wrist isusually in a neutral position at the elbow held inflexion. As the child develops good independentambulation, the unaffected limb tends to comedown to the side and by 3–4 years of age developsreciprocal swing with the contralateral leg. Theinvolved upper extremity tends to be held withslight shoulder flexion and internal rotation andelbow flexion, and then gradually the wrist willbecome slightly flexed, and the fingers start toopen up from the flexed finger position. By5–6 years of age, the child holds the plegic armin the typical position that is usually recognized ashemiplegia with slight shoulder flexion and inter-nal rotation, elbow flexion, and forearm pronationand wrist in mild flexion with fingers extendedand thumb adducted. During late childhood andinto adolescence, the shoulder flexion and internalrotation and elbow-flexed posture of the upperextremity during gait tend to relax, and the armcomes down to the side developing more normalposture. It is also during this time that the forearmpronates more; the wrist may become more flexedwith the thumb adducted with the fingers havingmore difficulty with grasping. This change is usu-ally seen as cosmetic improvement but not asfunctional improvement (Riad et al. 2007a,

2011) (chapter “▶The Upper Extremity in Cere-bral Palsy: An Overview).

The ability to clearly classify a child withhemiplegia as opposed to diplegia or quadriplegiamay be ambiguous. Many children by 18 monthsof age with classic presentation of asymmetricearly hand use and toe walking with classic asym-metric physical examination can be given with thediagnosis of hemiplegic CP. There is a subgroupof children who will make very large gains and by4 or 5 years of age may essentially look normal.Another group of children with similar findingsmay develop much more apparent skeletal mani-festations and even some abnormality on the con-tralateral side making the pattern identification anasymmetric diplegia. Another group of earlyambulators appeared to be very symmetric andappeared to have a diagnosis of bilateral CP but,as the child grows especially by age 5–6 years,may present as unilateral CP. Therefore, it is wisenot to be overly confident in explaining to familieswith young children under age 3 as to the exactlong-term outcome. However, it is clear that if thechild is independently ambulating, one can havegreat confidence that the child will be GMFCS I orII as an adult.

Treatment

Many children with unilateral CP do developmusculoskeletal deformities impairing their gaitpattern and upper extremity function, which areamendable to surgical correction. A few children,usually with severe mental retardation, do notbecome functional ambulators (GMFCS III)(Beckung et al. 2008). Often, inability to havefunctional ambulation (GMFCS IVor V) is relatedto poor function in the upper extremity, whichmakes the use of an assistive device difficult.There have been several attempts to classify pat-terns of hemiplegic gait (Hullin et al. 1996; Win-ters et al. 1987), but the classification of Winterset al. (1987) is easy to remember and has the mostdirect implications for treatment (Fig. 1). Thisclassification divides hemiplegic gait into fourpatterns. Type 1 has ankle plantar flexion inswing phase with an inactive or very weak tibialis

Hemiplegic or Unilateral Cerebral Palsy Gait 3

anterior as the etiology of the plantar flexion. Thepattern is most common in adult stroke or pero-neal nerve palsy. Type 2 has an equinus gait pat-tern but with spastic or contracted plantar flexors,which overpower an active dorsiflexor. Type3 includes the ankle position of type 2, furtheradding abnormal function of the knee joint. Theknee has increased spasticity in the hamstring orrectus with increased forefoot contact and stancephase knee flexion with or without decreasedswing phase knee flexion. Type 4 includes allproblems of type 3 with the addition of abnormalfunction of the hip joint muscles. Type 4manifestswith increased tone in the adductors and hipflexors and with internal femoral torsion. Theseparation of these types is usually easy througha combination of physical examination, EMG,kinematic evaluation, and kinetic data. As withall biological groups, however, there are interme-diate patients. A modification of the system wasthe addition of type 0 added by Riad et al.(2007b). This is a group of patients who are notable to clearly be placed in either type 1 or

2 because they have relatively neutral ankle posi-tion. These tend to be very mild patients who havedecreased ankle dorsiflexion but have a foot flatstrike and come to some dorsiflexion during thesecond rocker. Patients who have been treated fortype 2 with plantar flexor lengthenings often fallinto this pattern. The Winter’s classification sys-tem does not consider transverse plane deformi-ties; however, most children with significantresidual internal femoral torsion are classified astype 4. Tibial torsion is uncommon but may occurwith types 2, 3, and 4.

Hemiplegia Type 0

Patients with hemiplegic type 0 pattern CP are thesecond most common group. Many of thesepatients were originally classified as type 2 buthave been treated with plantar flexor lengthen-ings, so they have an ankle that is in a neutralposition typically with some limited dorsiflexionand also some reduced active plantarflexion (Riad

Fig. 1 The best classification of hemiplegia is that ofWinters et al. (1987) in which type 1 is due to a weak orparalyzed ankle dorsiflexor causing a drop foot. Type 2 hasequinus foot position due to a contracture of the gastrocne-mius or gastrocsoleus preventing dorsiflexion. Type 3 hasspastic or contracted hamstrings or quadriceps muscles in

addition to type 2 ankle. Type 4 has spastic or weak hipmuscles in addition to type 3 deformity. Almost all patientsare relatively easy to classify into one or the other type,which is then helpful for planning treatment. Transverserotational plane malalignments do not fit into this classifica-tion and should be seen as an additional problem

4 F. Miller

et al. 2007b). The moments generated at the ankleare usually reduced in magnitude compared to theunaffected side, but they have relatively normaltiming of the moment. The primary treatment forthis group is ongoing monitoring and focusing onactivity especially muscle strength training (Leeet al. 2008). If these children are still in middlechildhood under the age of 10, there is a reason-ably high risk that during the adolescent growth,spurt equinus will reoccur and they’ll fall backinto the type 2 pattern. The use of orthotics to limitplantarflexion is somewhat controversial. It islikely possible with the diligent use of orthoticsto probably prevent recurrent equinus contracture;however, the risk is increasing muscle weakness,which is always present. All attempts should bemade to avoid the weakness making it worse.Children with hemiplegia tend to migrate theirpower generation to the proximal muscles, soeven the uninvolved side has a tendency to doless power generation and be somewhat weaker.Strength training should focus on the whole lowerextremity bilaterally and not only on a singleinvolved calf muscle (Riad et al. 2008).

Hemiplegia Type 1

In CP hemiplegia type 1 is the least commonpattern of involvement. Type 1 occurs more withadult stroke or with a peripheral nerve injury. Ifthis type is identified in a child with CP, thephysical examination will demonstrate full pas-sive dorsiflexion; however, no active dorsiflexioncan be elicited. The kinematic examination willshow plantar flexion at initial contact and nodorsiflexion in swing phase. The EMG will dem-onstrate a tibialis anterior that is silent or nearlysilent. The primary treatment for type 1 hemiple-gia is a relatively flexible leaf spring AFO (Case 1).In very rare situations where the tibialis posteriorhas normal tone and normal phasic firing, thetibialis posterior can be transferred through theinterosseous membrane to the dorsum of thefoot. However, this transfer is mainly used withperipheral nerve palsy. With central lesions,relearning is difficult as this is an out-of-phasetransfer, and transfer of the spastic tibialis

posterior through the interosseous membraneleads to very severe foot deformities over timeand should be avoided.

Hemiplegia Type 2

The most common subtype of hemiplegia is type2, making up approximately 75% of all childrenwith hemiplegia. Typically, children learn to walkindependently between 15 and 20 months of age,either with toe walking or foot flat with aplanovalgus. The early treatment is to providethe children support through the use of an orthotic,usually starting with a solid ankle AFO and thenwith an articulated AFO for the second orthotic. Ifa child has a very spastic gastrocsoleus, botulinumtoxin injection for two or three cycles can helpparents apply the AFO and make AFO wear morecomfortable for the child. Usually, by 4–7 years ofage, the gastrocsoleus contracture has become sosevere that brace wear is no longer possible. Onphysical examination, children often demonstratea contracture of both the gastrocnemius andsoleus. The kinematic examination will showequinus throughout the gait cycle, and knee flex-ion at foot contact may be increased as childrenpreposition the knee to avoid high external exten-sion moments from the ground reaction forceduring weight acceptance. Often, these childrenwill be toe walking on the unaffected side as well,and a careful assessment is required to make surethat this is compensatory toe walking and not mildspastic response in a limb that was erroneouslythought to be normal. The physical examinationand kinematic evaluation are most useful for thisassessment. The unaffected ankle should haveadequate dorsiflexion measuring 5–10� withknee extension. The ankle moment should shownormal late stance phase plantar flexion momentor a variable moment, one or two of which lookalmost normal. The affected ankle will also bemore consistently abnormal with high early plan-tar flexion moments. If children have been allo-wed to walk on the toes until late middlechildhood, their unaffected ankles will oftendevelop plantar flexion contractures from persis-tent toe walking. The physical examination will

Hemiplegic or Unilateral Cerebral Palsy Gait 5

show a reduced ankle range of motion, and theankle moment will still show the same variabilitywith much better power generation than theaffected ankle. The step length of the affectedside is usually longer, and the stance phase timeof the normal limb is longer. These changes occurbecause the affected leg has a normal swing phase,due to normal knee and hip motor control, but ismore unstable in stance phase. If the normal anklehas a fixed contracture, it will need a gastrocne-mius lengthening, or this normal ankle willbecome a driving force toward toe walking aftercorrection of the contracture on the primarilyinvolved side (Case 2).

For patients with severe equinus contractures,typically, those who are unable to come within�20� to plantigrade will have significantly over-lengthened tibialis anterior muscle and tendon.After appropriate plantar flexor lengthening usu-ally with an open tendon Achilles lengthening, thetibialis anterior is not efficient to provide adequatedorsiflexion because of its redundancy. The ten-don will slowly readjust its length; however, asignificant period of time will be required duringwhich time the individual has to wear an articu-lated or leaf spring AFO to prevent foot drop inswing phase. For a child under age 10, this periodof time may be a number of months; however, foran older individual past skeletal maturity, the timeframe is often a couple of years. This long-termneed for orthotic use further magnifies the weak-ness that is already present in the plantar flexormuscles. Based on this, for an individual past theage of 10, we would recommend considering aplication of the tibialis anterior tendon to precludethis long-term need for orthotic management.Results of this procedure have documented goodpostoperative active ankle dorsiflexion (Rutz et al.2011; Tsang et al. 2016). We have found that it iswise to make sure there is at least 10�

plantarflexion after the tendon has been shortened,because too much shortening can create a calca-neus gait pattern if the child loses too muchplantarflexion.

Outcome of Plantar Flexor TendonLengthening

The need for postoperative orthotic use varies, butbraces are not routinely needed. If children do notgain foot flat at initial contact by 3–6 months aftersurgery, an AFO should be used, usually an AFOthat allows dorsiflexion to encourage the tibialisanterior to gain function. This AFO can be eitheran articulated AFO or a half-height wraparoundAFO with an anterior ankle strap. With appropri-ate early treatment, most children with type2 hemiplegic pattern CP can be free of an orthosisby early grade school. Some children will developan equinus contracture again in late childhood oradolescence. If an adolescent is willing to toleratethe orthosis, another round of Botox injectionsand orthotic wear can delay surgery until he/sheis near the completion of growth. Approximately25% of type 2 hemiplegics will need a secondgastrocnemius or tendon Achilles lengthening inadolescence (Joo et al. 2011). Younger age at thefirst lengthening and severe equinus deformity arerelated to recurrence. It is also important to recog-nize that having a recurrent equinus deformity ismuch preferred over having an overlengthenedplantar flexor that is incompetent. The risk ofoverlengthening is less in individuals with hemi-plegia and then with bilateral involvementbecause they have at least one good limb tostand on. Adolescents or young adults with type2 hemiplegia should seldom need to wear anorthosis after their last lengthening. Routinelong-term use of an AFO should be avoided asthis will weaken the muscle and make the personpermanently brace dependent. Long toe flexorspasticity may also be present, but this seldomneeds surgical treatment.

In early childhood, the feet are often in aplanovalgus position; however, as children gainincreased tone, gastrocnemius and soleus equinusdevelop. Often, this equinus causes theplanovalgus to correct and sometimes even over-correct. Children with type 2 hemiplegia developplanovalgus that needs treatment less frequentlyand then those with bilateral involvement. Surgi-cal treatment should not be considered until8–10 years of age because this planovalgus may

6 F. Miller

continue to slowly improve unless it is verysevere. The predominant problem for childrenwith type 2 hemiplegia is equinovarus, usuallydue to a spastic or overactive tibialis posterior. Inoccasional children, equinovarus is due to a spas-tic tibialis anterior. The diagnosis of the specificetiology of the varus between these two tendonsrequires a combination of physical examinationand EMG data. The physical examination willoften demonstrate increased tone in the musclemost responsible. The EMG should show a nor-mal tibialis anterior that is active during preswingand initial swing phase and again in terminalswing at initial contact. Significant activity duringmidstance is abnormal. The tibialis posterior maybe active throughout stance phase, more so interminal stance, and should be silent in swingphase (Renders et al. 1997). Most commonly, thetibialis posterior is constantly active on EMG andspastic on physical examination, although thereare cases where it is only active in swing phase. Ifthe subtalar motion is supple, allowing full cor-rection of the varus, a split transfer of the tibialisposterior to the peroneus brevis on the lateral sideis performed. If the tibialis anterior is mostaffected, it is split transferred to the cuboid or toa slip of the peroneus longus. If both tendons areabnormal, both can have a split transfer performedat the same time. If the subtalar joint is not allo-wing overcorrection into some valgus, a calcanealosteotomy may be required, although this is rarein type 2 hemiplegia.

Rotational Deformities

Transverse plane torsional deformities are notcommon in type 2 hemiplegia and are usuallymild, similar to torsional deformities in normalchildren. Because the torsional deformities aremild, surgical treatment should not be considereduntil late middle childhood or adolescence. Limblength discrepancy is usually approximately 1 cmshorter on the involved side, which is anatomi-cally perfect. Shoe lifts should not be given, asthey will only require children to make an adap-tation, which increases the difficulty of swinging

the leg through. This degree of shortness causesno short-term or long-term problems.

Treatment of the spasticity, which is limited tothe plantar flexors in type 2 hemiplegia, requiresonly local measures such as tendon lengthening,Botox, and/or bracing. There is no role for dorsalrhizotomy or intrathecal baclofen because thelocal treatments are effective and much simpler.Because both the gastrocnemius and soleus seemto contract together in many of these children, it isreasonable to consider nighttime orthotic wear totry to stretch the soleus and perhaps the gastroc-nemius. A nighttime orthosis is usually attemptedwhen contractures are present; however, mostchildren object to this orthosis because they areunable to fall asleep, and therefore, in practice,this seldom works. The nighttime orthotic use hasto include keeping the knee fully extended, so thegastrocnemius gets stretched as well as the soleus.

Hemiplegia Type 3

Children with type 3 involvement have all theconcerns and problems of the children with type2 involvement. Children with type 3 hemiplegiatend to start walking slightly later than with type2, usually at 18–24 months of age. They almost allstart with toe walking on both feet and usually willnot need assistive devices to start walking. Thediagnosis of type 3 hemiplegia requiresestablishing evidence that the knee is involved inthe pathology. On physical examination, theremay be increased tone in the hamstrings or rectusmuscles and increased hamstring contracture,usually at least 20� and often 30–40� more thanthe unaffected side. Knee flexion at initial contactwill be high, more than 25�. In midstance, theknee flexion continues to be increased. Almostall type 3 patterns have abnormal hamstring activ-ity. On the EMG, this activity is usually prematureonset in swing phase and prolonged activity instance phase. The presence of a fixed knee flexioncontracture of more than 5� is also evidence ofhamstring involvement. The step length is usuallyshorter than the normal side due to the overactiv-ity of the hamstrings, and the stance time is vari-able, sometimes longer and sometimes shorter

Hemiplegic or Unilateral Cerebral Palsy Gait 7

depending on the stability of stance phase (Case 3).Treatment of the hamstring contractures and over-activity may use botulinum toxin injections forseveral cycles in young children, along with gas-trocnemius injections. When the hamstring con-tracture is causing progressive knee flexioncontracture, surgical lengthening should beperformed. If the gastrocsoleus contractures needto be addressed, the hamstrings should also belengthened at the same time, or knee flexion inmidstance will draw these children to either toewalk again or stand with a crouched gait on theaffected side, which also draws the unaffectedside into a crouched gait pattern with increasedknee flexion in stance.

Those with more severe fixed knee flexioncontractures, especially those with more than 20�

lacking full knee extension, will need to have thisaddressed as well. The options for addressingyour fix knee flexion contracture may includeknee extension osteotomy, posterior kneecapsulotomy, or anterior femoral epiphysiodesis.For a young child under age 10, the knee flexioncontracture can often be stretched with aggressivetherapy and orthotic use following hamstringlengthening. If there is limited therapy availabilityor the contracture is more stiff, a posterior kneecapsulotomy is a good solution in this age group(Taylor et al. 2016). The use of guided growthwith anterior epiphysiodesis has been reported(Macwilliams et al. 2011); however, in our expe-rience, the deformity correction is too slow, and itadds further to the length discrepancy which isalready a problem in some children. For the ado-lescent and young adult in whom the flexion con-tracture is stiff and more than 20�, a kneeextension osteotomy is the preferred approach(Stout et al. 2008).

Stiff Knee Gait

Some children with type 3 hemiplegia haveinvolvement of the rectus. This involvement willbe noted by the parents as a complaint of toedragging, frequent tripping, and rapid shoe wear,especially on the anterior aspect of the shoe box.The physical examination may or may not

demonstrate increased rectus tone and a positiveEly test. The kinematic evaluation will showswing phase peak knee flexion to be less than thenormal, usually less than 50�, and the peak is oftenlate, close to midswing. For children with late orlow knee flexion in swing, when the EMG activityof the rectus muscle in swing phase is increasedand evidence of complaints of toe dragging ispresent, then a distal transfer of the rectus is indi-cated. This transfer is almost always performedwith hamstring lengthening and gastrocnemius ortendon Achilles lengthening. Similar to type2 hemiplegia, approximately 25% of the childrenwill need two plantar flexor tendon lengthenings,one at age 4–7 years and the other at adolescence.A few children will need three lengthenings (Jooet al. 2011). These tend to be children who neededthe first lengthening very early due to more severecontracture, sometimes as early as the third year oflife. The goal of delaying the first tendon length-ening is to try to avoid the second or third tendonlengthening, although there is no physical docu-mentation that this strategy is effective.

Rotational Deformities

Transverse plane deformities are more commonwith type 3 hemiplegic involvement and then type2. If tibial torsion and femoral anteversion arecausing increased tripping or are very cosmeti-cally objectionable by 5–7 years of age, surgicalcorrection can be considered. Although rare intype 3 hemiplegia, if children have a very asym-metric pelvic rotation as an adaptation for unilat-eral torsion usually tibial, correction should beconsidered as early as age 5–7 years. Becausethe functional impairment is greater, the limblength discrepancy tends to be slightly greaterthan for type 2 hemiplegia, often between 1 and2 cm at maturity. For most children, this limblength discrepancy works perfectly well to helpwith foot clearance during swing phase in a limbthat does not have a good ability to shorten duringpreswing and initial swing phase. A shoe liftshould not be used, and radiographic monitoringof limb length is needed only with a discrepancyof over 1.5 cm. If the knee flexion contracture is

8 F. Miller

more than 10�, additional shortening will occur.To prevent further leg shortening, knee flexioncontracture prevention is important. Like type2 hemiplegia, there is no role for the global treat-ment of spasticity in type 3 hemiplegia.

Hemiplegia Type 4

Type 4 hemiplegia is the third most commonpattern, however, making up less than 5–10% ofall children with hemiplegia. It is relatively com-mon to find type 4 hemiplegia that overlaps withasymmetric diplegia or mild quadriplegia, and it isrelatively uncommon to find a child with type4 hemiplegia who is completely normal on thecontralateral side. Most children with type 4 hemi-plegia are either GMFCS II or GMFCS III levelambulators. The children who are GMFCS IIIusually are using a single lofstrand crunch in theuninvolved limb. Children with type 4 involve-ment walk later, between the ages of 2 and 3 years.Many children will use a walker during the learn-ing period of walking. The walker may need to befitted with an arm platform on the involved side.The diagnosis of type 4 hemiplegia is made by thepresence of increased tone in the adductor or hipflexor muscles and by evidence on the kinematicexamination of decreased hip extension in mid-stance. Increased internal rotation of the hip isvery common. Both the stance time and the steplength will be shortened as the involved limb. Thelimb can neither swing normally nor is very stablein stance phase. All the problems and consider-ations of type 2 and type 3 have to now be addedinto the treatment of type 4. In addition, concernfor overactivity and contracture of the adductorsand hip flexors has to be considered as well.Increased internal rotation of the hip is also com-mon secondary to increased femoral anteversion.It is important to recognize that children with type4 hemiplegia can develop spastic hip disease, sothey have to be monitored by physical examina-tion and radiographs for hip dysplasia. This is thehighest-risk group of GMFCS I or GMFCS IIfunctional ambulators who develop hip subluxa-tion during the adolescent growth (Abousamraet al. 2016; Rutz et al. 2012) (chapter “▶Natural

History and Surveillance of Hip Dysplasia inCerebral Palsy”).

From the perspective of children’s gait, thedecisions about surgery are usually based mostlyon the function at the level of the ankle and knee.Based on the evaluation of these joints, surgery ofthe hip has to be considered as an additionalprocedure. Adductor lengthening is only neededoccasionally. If the abduction is greater than 20�

on physical examination and abduction is presentat foot contact, surgery is seldom indicated.Iliopsoas lengthening is indicated if hamstringlengthening is to be done, a hip flexion contractureof more than 20� is present, anterior pelvic tilt ismore than 25�, and there is less than 10� of hipflexion at maximum extension in mid- or terminalstance. Usually, these hip lengthenings are neededonly once; however, additional lengthenings,especially hamstring and gastrocnemius lengthen-ings, are very commonly needed. Probably75–90% of children with type 4 hemiplegia needat least two lengthening procedures, and approx-imately 25% may need a third lengthening proce-dure. Treatment of the distal problems follows thepattern of type 2 and type 3; however, the muscletone and contractures tend to be worse. Soft tissuebalancing procedures may improve the gait oftype 4 hemiplegia, but it will not address the hipdysplasia (Rutz et al. 2012).

Rotational Deformities

Transverse plane deformities, especially increasedfemoral anteversion, are common in type 4 hemi-plegia. Usually, this is added to the neurologictendency for pelvic rotation with the affectedside rotated posteriorly. In occasional children,this pelvic rotation may be so severe that theypresent with almost sideways walking. This side-ways walking pattern can also be described ascrab walking. This gait pattern is very ineffectiveand should be addressed at the young age of5–7 years. Femoral derotation is required toallow the pelvis to rotate anteriorly on the affectedside creating a more symmetric gait pattern. Fem-oral derotation should be considered if the pelvicrotation is more than 15–20� on the involved side

Hemiplegic or Unilateral Cerebral Palsy Gait 9

and the physical examination shows an asymmet-ric femoral rotation with more internal rotation onthe affected side. Femoral derotation can be com-bined with all the other soft tissue lengtheningsthat may be needed. Children with type 4 hemiple-gia may develop foot deformities similar to diple-gia in which the planovalgus improves intomiddle childhood but then gets worse again inadolescence. It is important for children withtype 3 and 4 hemiplegia to have a full detailedanalysis of their gait and then to plan to do asingle-event multilevel surgical (SEMLS) correc-tion. The outcome of SEMLS in hemiplegia issimilar to the outcome in bilateral CP (Schranzet al. 2016).

Limb Length Discrepancy

Limb length discrepancy is an active concern intype 4 hemiplegia, because many children have2–2.5 cm of shortness on the affected side. Thefunctional impact of the limb shortness isincreased with the tendency for knee and hipflexion deformities to add more functional short-ening to the real shortening. Also, this leg lengthdiscrepancy may be further complicated byadductor contractures that may limit hip abduc-tion allowing the pelvis to drop on the affectedside, which further magnifies the limb lengthinequality. If the limb length cannot be function-ally accommodated, the use of a shoe lift isrecommended for type 4 hemiplegia. This groupalso merits close radiographic monitoring of limblength with the goal in some children of doing adistal femoral epiphysiodesis to arrest growth orto use epiphysiodesis plates for temporary growtharrest on the noninvolved side. The goal in type4 hemiplegia is to have the affected limb lengthequal to 1 cm longer than the noninvolved sidebecause of the functional impact of the inability toaccommodate for joint positions during stancephase, which take precedence over swing phasedysfunction (Case 4). There is benefit to having alonger affected limb only in definite type 4 hemi-plegia. In all other types, which make up morethan 90% of hemiplegia, the affected limb should

be approximately 1 cm shorter for maximumfunction (Fig. 2).

In some children with type 4 hemiplegia, theuse of intrathecal baclofen can be considered fortreating severe spasticity even though it is unilat-eral. We have not used intrathecal baclofen in thispopulation except for those with severe hemiple-gic dystonia, and there are no reports specificallyaddressing its use. The local treatment of thedegree of spasticity present in many childrenwith type 4 hemiplegia is not very effective.

In severe type 4 hemiplegia GMFCS III andGMFCS IV, an assistive device is needed longterm for ambulation. These children withGMFCS IV require a platform walker and thosewith GMFCS III who usually walk with onecrutch or cane. The most functional device isfound by trial and error in physical therapy. Inthe children who are GMFCS IV with limitedambulation, wheelchairs are needed. Because ofthe presence of one normal arm, a double-rim one-arm-drive chair should be considered, ordepending on the environment and personalneed, a power wheelchair may bemore functional.

Complications

Complications related to the management of chil-dren and young adults with hemiplegic CP tend tobe most related to either poor timing of treatmentor lack of treatment. Allowing severe equinuscontractures to develop only incurs significantsecondary osseous deformity which should beavoided (Fig. 3). Appropriate timing of correctionof equinus contractures may incur the risk ofneeding repeat lengthening; however, this is lessdeforming in the long term than the secondarybone deformities which occur with neglected con-tractures. Attempts should also be made to avoidlong-term orthotic use because this will create anorthotic dependence due to weakness of the mus-cle. Care should be taken to avoid over-lengthening muscles and to creating externalfemoral torsion as these deformities becomeimpairments in their own right. However, it isless functionally devastating in the person withhemiplegia to have overlengthening than it is in

10 F. Miller

children with diplegia. Children with hemiplegiastill have one good leg they can stand on anddepend on for function.

Cases

Case 1 TaniaTania, an 18-year-old girl, had hemiplegiaas a result of a traumatic brain injurysustained at age 8 years. Her main com-plaint was that she could not lift her foot.Physical examination of her right ankledemonstrated an active toe extensor and

some apparent activity of the tibialis ante-rior on withdrawal stimulus of a pin stick onthe sole. Ankle dorsiflexion was 10� withknee flexion and 20� with knee extension.Ankle kinematics showed no activedorsiflexion in swing phase and no EMGactivity of the tibialis anterior (Fig. C.1).Observation of her gait demonstrated anextended hallux in swing phase, but noapparent dorsiflexion was in swing phase.Knee and hip motion appeared to be normal.She was ordered a leaf spring AFO thatworked well when it was worn.

Fig. 2 In hemiplegic types 1–3, it is better to have a mildshortness of the affected limb. Naturally, this ends up beingbetween 1 and 2 cm, which helps limb clearance in swing.However, in type 4, there is a tendency to have increasedhip adduction and flexion contractures that greatly magnifyany other leg shortness. Also, hip extension and abductionare major mechanisms for accommodating leg length

shortness, and when this is deficient in type 4 hemiplegia,the limb shortness becomes an impairment in its own right.Therefore, careful attention should be paid to limb lengthin type 4 with a goal usually of having symmetric limblengths. An occasional patient may even function betterwith a longer limb on the affected side

Hemiplegic or Unilateral Cerebral Palsy Gait 11

Case 2 ChristianChristian, a boy with hemiplegia, startedwalking at 17 months of age. He used asolid ankle AFO until he was 2.5 yearsold. He then used articulating AFOs untilhe was 4 years old, when he complainedthat the orthotics caused him pain. Aftermultiple attempts to make the orthoticscomfortable, he was allowed to walk with-out orthotics for 1 year until age 5 years,when he had a full analysis. The physicalexamination demonstrated that he had pop-liteal angles of 35� bilaterally, and ankledorsiflexion on the right was only �25�

with both knee flexion and extension. Onthe left, he had ankle dorsiflexion to 20�

with knee flexion but only 5� with kneeextension. The observation of his gaitshowed that he was toe walking bilaterally,although it is higher on the right than theleft. It was recommended that he have anopen Z-lengthening of the tendon Achilles.Postoperatively, he used an articulated AFOfor 1 year, and following this, he developedgood active dorsiflexion with a plantigradefoot position (Fig. C2.1).

Fig. 3 A 13-year-old boy first presented with severe fixedequinus with maximum dorsiflexion being minus 60�. Hehad many episodes of casting and botulinum toxin whichwere clearly insufficient for the severity of the deformity.He now has developed secondary deformities, fixed kneeflexion contracture, and rigid cavus. He is currently in hismid-30s, and these secondary deformities continue tocause him significant disability. This could all have beenavoided with appropriate surgical management at theappropriate age before secondary deformities developed

Fig. C.1

12 F. Miller

Case 3 KwameKwame, an 18-month-old boy, was initiallyseen with a complaint that he was late inlearning to walk. He was reported to havebeen premature by 8 weeks but had beenhealthy since discharge from the hospital.On physical examination, he had increasedtone through the lower and upper extremi-ties, but it seemed worse on the left side. Hewas placed in an AFO, and over the next6 months, he started walking. By age

5 years, he was developing significant inter-nal rotation of the femur and having a stiffknee gait as well as significant toe walkingbilaterally. At this time, the physical exam-ination showed that he had hip abduction of25� on the left and 45� on the right andinternal rotation on the left of 75� and onthe right of 60�. The popliteal angle on theleft was 68� compared with 50� on the right.The left ankle dorsiflexion with the kneeextended was �20�, while on the right itwas 4�. The knee flexed ankle dorsiflexionon the left was�8�, while on the right it was11�. The kinematics demonstrated low nor-mal knee flexion in swing phase, increasedknee flexion at foot contact, and bilateralearly ankle dorsiflexion in stance phase,with less total dorsiflexion on the left side.Internal rotation of the left femur was alsonoted (Fig. C3.1). The EMG showed muchless clear activity patterns on the left withthe rectus having high variability and thehamstring having very early initiation onthe left. The right side looked normal(Fig. C3.2). Except for the internal rotationof the hip, the primary pathology seemed tobe in the left knee and ankle; therefore, thisis a type 3 hemiplegia. Based on this, thefemur was derotated, hamstring lengthened,distal rectus transferred to the sartorius, anda tendon Achilles lengthening performed(Fig. C3.3). He did well for 4 years, butthen he again developed a significant ankleequinus requiring a second tendon Achillesand distal hamstring lengthening. As heentered puberty, he was doing well with anearly symmetric gait pattern.

Case 4 JeremyWhen Jeremy was 9 years old, his parentscomplained that he tripped over his right legand could not run. Jeremy had moderate

(continued)

Fig. C2.1

Hemiplegic or Unilateral Cerebral Palsy Gait 13

mental retardation and no other history ofmedical problems. The left side was normalon physical examination, but on the rightside, he had weakness, especially at the hip

abductors and extensors. He had no spastic-ity of the gastrocnemius but increased tonein the hamstrings with a popliteal angle of50� on the right and 30� on the left. Ankle

(continued)

Fig. C3.1

14 F. Miller

dorsiflexion on the right was 15� with kneeflexion and 5� with knee extension. Hipabduction was limited to 10� on the right,full flexion was present, and a 2.5 cm short-ness was noted on the right side (Fig. C4.1).Jeremy was put in an AFO and given a1.5 cm shoe lift, which improved the trip-ping symptoms. An adductor and hamstringlengthening was performed, and the leglength was monitored with annualscanograms. Because this was believed to

represent a type 4 hemiplegia without muchcompensation attempted by toe walking, afemoral epiphysiodesis was planned whenhis remaining growth would leave the rightleg approximately equal to 1 cm long. Atage 12.5 years, the epiphysiodesis wasperformed (Fig. C4.2), and by age16 years, he was left with several millime-ters of increased lengthening on the rightside (Fig. C4.3). He was weaned off of the

(continued)

Fig. C3.2

Hemiplegic or Unilateral Cerebral Palsy Gait 15

shoe lift and out of the AFO. At the com-pletion of growth, he walked without assis-tance. This is the typical limb lengthproblem of type 4 hemiplegia, which shouldbe managed to gain equal limb lengtheningto slightly overlengthening on the involvedside. With the other types of hemiplegia, the

goal is to leave the child with a 1 to 2 cmshortness on the involved side, which willhelp with limb clearance and accommodatefor the tendency for premature heel risefrom gastrocnemius spasticity orcontracture.

Fig. C3.3

16 F. Miller

Fig. C4.1

Fig. C4.2

Hemiplegic or Unilateral Cerebral Palsy Gait 17

Cross-References

▶ Foot Deformities Impact on Cerebral Palsy Gait▶Natural History and Surveillance of Hip Dys-plasia in Cerebral Palsy

▶The Upper Extremity in Cerebral Palsy: AnOverview

References

Abousamra O, Er MS, Rogers KJ, Nishnianidze T, DabneyKW, Miller F (2016) Hip reconstruction in childrenwith unilateral cerebral palsy and hip dysplasia. JPediatr Orthop 36:834–840

Andersen GL, Irgens LM, Haagaas I, Skranes JS, MebergAE, Vik T (2008) Cerebral palsy in Norway: preva-lence, subtypes and severity. Eur J Paediatr Neurol12:4–13

Beckung E, Hagberg G, Uldall P, Cans C, Europe Surveil-lance of Cerebral Palsy in (2008) Probability of walk-ing in children with cerebral palsy in Europe. Pediatrics121:e187–e192

Darmency-Stamboul V, Chantegret C, Ferdynus C,Mejean N, Durand C, Sagot P, Giroud M, Bejot Y,Gouyon JB (2012) Antenatal factors associated withperinatal arterial ischemic stroke. Stroke 43:2307–2312

Hullin MG, Robb JE, Loudon IR (1996) Gait patterns inchildren with hemiplegic spastic cerebral palsy. JPediatr Orthop B 5:247–251.

Joo SY, Knowtharapu DN, Rogers KJ, Holmes L Jr, MillerF (2011) Recurrence after surgery for equinus foot

deformity in children with cerebral palsy: assessmentof predisposing factors for recurrence in a long-termfollow-up study. J Child Orthop 5:289–296

Lee JH, Sung IY, Yoo JY (2008) Therapeutic effects ofstrengthening exercise on gait function of cerebralpalsy. Disabil Rehabil 30:1439–1444

Macwilliams BA, Harjinder B, Stevens PM (2011) Guidedgrowth for correction of knee flexion deformity: aseries of four cases. Strategies Trauma Limb Reconstr6:83–90

Renders A, Detrembleur C, Rossillon R, Lejeune T,Rombouts JJ (1997) Contribution of electromyo-graphic analysis of the walking habits of children withspastic foot in cerebral palsy: a preliminary study. RevChir Orthop Reparatrice Appar Mot 83:259–264. SRC– GoogleScholar

Riad J, Coleman S, Lundh D, Brostrom E (2011) Armposture score and arm movement during walking: acomprehensive assessment in spastic hemiplegic cere-bral palsy. Gait Posture 33:48–53

Riad J, Coleman S, Miller F (2007a) Arm posturing duringwalking in children with spastic hemiplegic cerebralpalsy. J Pediatr Orthop 27:137–141

Riad J, Haglund-Akerlind Y, Miller F (2007b) Classifica-tion of spastic hemiplegic cerebral palsy in children. JPediatr Orthop 27:758–764

Riad J, Haglund-Akerlind Y, Miller F (2008) Power gen-eration in children with spastic hemiplegic cerebralpalsy. Gait Posture 27:641–647

Rutz E, Baker R, Tirosh O, Romkes J, Haase C, Brunner R(2011) Tibialis anterior tendon shortening in combina-tion with Achilles tendon lengthening in spasticequinus in cerebral palsy. Gait Posture 33:152–157

Rutz E, Passmore E, Baker R, Graham HK (2012) Multi-level surgery improves gait in spastic hemiplegia but

Fig. C4.3

18 F. Miller

does not resolve hip dysplasia. Clin Orthop Relat Res470:1294–1302

Schranz C, Kruse A, Kraus T, Steinwender G, Svehlik M(2016) Does unilateral single-event multilevel surgeryimprove gait in children with spastic hemiplegia? Aretrospective analysis of a long-term follow-up. GaitPosture 52:135–139

Stout JL, Gage JR, Schwartz MH, Novacheck TF (2008)Distal femoral extension osteotomy and patellar tendonadvancement to treat persistent crouch gait in cerebralpalsy. J Bone Joint Surg Am 90:2470–2484

Taylor D, Connor J, Church C, Lennon N, Henley J,Niiler T, Miller F (2016) The effectiveness of posteriorknee capsulotomies and knee extension osteotomies in

crouched gait in children with cerebral palsy. J PediatrOrthop B 25:543–550

Tsang ST, McMorran D, Robinson L, Herman J, Robb JE,Gaston MS (2016) A cohort study of tibialis anteriortendon shortening in combination with calf musclelengthening in spastic equinus in cerebral palsy. GaitPosture 50:23–27

Turedi Yildirim A, Sutcu R, Koroglu M, Delibas N,Kisioglu N, Akar N, Ergurhan Ilha I (2015) The roleof prothrombotic factors in children with hemiplegiccerebral palsy. Minerva Pediatr 67:279–284

Winters TF Jr, Gage JR, Hicks R (1987) Gait patterns inspastic hemiplegia in children and young adults. J BoneJoint Surg Am 69:437–441

Hemiplegic or Unilateral Cerebral Palsy Gait 19