Conservative Management of Legg-Calve-Perthes Disease in ...

Evidence-Based Care Guideline for Management of Legg-Calve-Perthes Disease in children aged 3 to 12 years Guideline 39Health Policy & Clinical EffectivenessProgramEvidence-Based Care GuidelineConservative Management of Legg-Calve-Perthes DiseaseIn children aged 3 to 12 years aInclusions:Publication Date October, 2010Target PopulationChildren with diagnosis of Legg-Calve-Perthesdisease (LCP) in disease stages 1-3o Stage 1: Femoral head becomes more densewith possible fracture of supporting bone;o Stage 2: Fragmentation and reabsorption ofbone;o Stage 3: Reossification when new bone hasregrown.Children ages 3-12 years oldExclusions:Children with the following:o Post-operative for LCPo Other diagnosis of avascular necrosis in hipo Femoral head injury or fractureo Slipped capital femoral epiphysiso Femur fractureo Acetabular or pelvis fractureo LCP present with other hip conditiono Acetabular labral tearo Cancer or bone tumor in femuro Arthritis in hipo Hip dysplasiao Cerebral palsya Please cite as: Lee J, Allen M, Hugentobler K, Kovacs C,Monfreda J, Nolte B, Woeste E; Cincinnati Children'sHospital Medical Center:Evidence-based clinical careguideline for Conservative Management of Legg-Calve-Perthes Disease, Occupational Therapy and Physical TherapyEvidence-Based Care Guidelines, Cincinnati Children'sHospital Medical Center, Guideline 39, pages 1-16, October,2010.Target UsersInclude but are not limited to (in alphabetical order): Athletic Trainers Community-based caregivers (e.g. physical educationteachers, school personnel) Nurses Patients and families Physical Therapist Assistants Physical Therapists Primary care providersIntroductionReferences in parentheses ( ) Evidence level in [ ] (See last page for definitions)LCP disease progresses through four stages as seen onradiographs using Modified Waldenstrom Classification.The duration of each stage varies.Stage 1-Initial StageThe capital epiphysis of the femoral head stops growing,causing a smaller ossific nucleus. Waldentrom’s sign, ora fracture in the subchondral area of the femoral head,can be seen on frog leg radiographs in this stage. Thefemoral head appears to be denser. There are cysts andlucencies in the metaphysis that can also be seen onradiographs. The initial stage ends when the lucenciesare in the ossific nucleus. Mean duration of this stage is6 months (Herring 2001 [5]).Stage 2-Fragmentation StageSome areas of the femoral head are sclerotic, whilelucencies remain in other areas. A central area of thefemoral head separates from the medial and lateralportions. The acetabulum also becomes more irregular.The end of this stage occurs when new bone begins todevelop in the subchondral areas of the femoral head.Mean duration of this stage is 8 months (Herring 2001 [5]).Stage 3-Rossification StageHealing of the femoral head begins. New subchondralbone develops in the head of the femur, beginning in thecenter and extending out. The last area to reossify isoften the anterior segment of the femoral head.Eventually the femoral head is replaced with wovenbone, which then remodels to trabecular bone. Theflattening of the femoral head may improve. This stageends when the entire head has reossified and is typicallythe longest of the four, with a mean duration of 51months (Herring 2001 [5]).Copyright © 2010 Cincinnati Children's Hospital Medical Center; all rights reserved. Page 1

Evidence-Based Care Guideline for Management of Legg-Calve-Perthes Disease in children aged 3 to 12 years Guideline 39Stage 4-Residual StageThere are no changes in the density of the bone, but theshape of the femoral head can change during this phase.The final shape of the femoral head is developed onceskeletal growth is complete. The femoral head may varywidely in shape, from normal to flat, at the conclusion ofthis stage. The acetabulum can also change in shapeduring this stage. Overgrowth of the greater trochantercan occur due to disruption of growth in the capitalphysis (Herring 2001 [5]).Several classification systems to describe the extent ofthe disease have been developed. The Catterall systemconsists of 4 groups classified based on the extent ofinvolvement of the femoral head on radiographs (Wenger1991 [5a]). The Salter-Thompson system classifieschildren into 2 groups based on the extent ofsubchondral fracture and amount of lateral margin of thefemoral epiphysis (Wenger 1991 [5a]). Herring describesclassification based on the involvement of the lateralpillar of the femoral head on radiographs (Herring 2004[4b]).The goal of treatment of children with LCP is to containthe femoral head in the acetabulum to maximize aspherical shape of the femoral head for good jointcongruency (Leach 2006 [5], Wenger 1991 [5a]). However,there has been lack of agreement in treatmentinterventions in children with LCP to achieve this goal(Weinstein 1997 [5a]). Treatment has ranged fromobservation only, performing range of motion (ROM)exercises, use of Petrie casts, use of braces, and surgicalintervention (Leach 2006 [5], Wenger 1991 [5a]). Evidence inthe literature has been mixed regarding the success ofconservative treatment only or surgical intervention(Brech 2006 [3b], Herring 2004 [3b], Canavese 2008 [4b]).Furthermore, there is limited evidence supporting atreatment pathway or methods in conservativemanagement of LCP.The objectives of this guideline are to: Guide and support consistency in delivery ofphysical therapy services for conservativemanagement of patients with LCP Promote and optimize range of motion, strength, andjoint preservation to minimize impairments andmaximize function Maintain and improve patient and familysatisfactionEtiologyLCP affects children 3 to 12 years old and is mostcommon in children 5 to 7 years old (Leach 2006 [5],Wenger 1991 [5a]). Males are affected 3 to 5 times morethan females. There is bilateral hip involvement in 10%to 20% of cases (Wenger 1991 [5a]). Children with LCPdisease typically present with a limp, which is acombination of an antalgic and a Trendelenburg typegait pattern. Pain is often present in the hip or referredto the thigh or knee (Tamai 2004 [5]) . Limp and pain arecommonly made worse with strenuous activities (Tamai2004 [5]) . Range of motion of the hip is limited, with thegreatest limitations typically in hip abduction andinternal rotation (Tamai 2004 [5], Wenger 1991 [5a]).The disease process involves avascular necrosis of thefemoral head due to a loss of blood supply (Leach 2006[5]). As a result, growth is disturbed in the epiphysealand physeal plates, resulting in premature closure. Thiscan lead to a shortened femoral neck and trochantericovergrowth. There is an asymmetric repair processcausing deformity in the femoral head. The deformedfemoral head then leads to a deformed acetabulum,especially the lateral aspect (Weinstein 1997 [5a]). Theetiology of the disease is unclear, but one theory is thereis onset of the disease following an episode of transientsynovitis of the hip (Leach 2006 [5], Wenger 1991 [5a]).Children who present with LCP are typically small fortheir age and have delayed bone age (Leach 2006 [5],Weinstein 1997 [5a], Wenger 1991 [5a]). Maintaining thefemoral head in the acetabulum during the repair processshould yield a more spherical femoral head and overall amore congruous joint (Wenger 1991 [5a]).PrognosisPrognosis of the disease has been determined usingseveral criteria, including age of onset of the disease(Leach 2006 [5], Weinstein 1997 [5a], Wenger 1991 [5a]), extentof involvement of the femoral head (Leach 2006 [5], Wenger1991 [5a]) , amount of incongruity between the femoralhead and acetabulum (Leach 2006 [5]), and amount of hipjoint deformity (Weinstein 1997 [5a]). Onset at a youngage, minimal epiphyseal involvement, and short durationof the disease are favorable prognostic factors. Onset atan older age, greater epiphyseal involvement, lateralsubluxation of the femoral head, and longer duration ofthe disease make up some of the poor prognostic factors(Tamai 2004 [5]).Copyright © 2010 Cincinnati Children's Hospital Medical Center; all rights reserved. Page 2

Evidence-Based Care Guideline for Management of Legg-Calve-Perthes Disease in children aged 3 to 12 years Guideline 39Guideline RecommendationsAssessment and Differential DiagnosisClinical Assessment1. It is recommended that a thorough history andexamination be completed to establish a differentialdiagnosis and individualized plan of care (APTA 2001[5]).2. It is recommended that the following are assessed atthe initial evaluation, on a monthly basis or sooner ifthe patient demonstrates a change in status, and atdischarge (Local Consensus [5]): Pain and symptoms Lower extremity (LE) passive range of motion(PROM) and active range of motion (AROM) Lower extremity strength Gait Balance Outcome measuresPain and symptoms3. It is recommended that pain is assessed using theOucher pain scale (Beyer 2005 [4a]) or NumericalRating Scale (NRS) (Williamson 2005 [1b], von Baeyer2009 [4b]).Lower extremity PROM and AROM4. It is recommended that a fluid filled goniometer beused to measure ROM (Rao 2001 [4b]). Hip motionsto assess include hip flexion, abduction, extension,internal rotation, external rotation.Note 1: Use of a linear goniometer is alsoacceptable based on availability in the clinic (Rao2001 [4b], Clapper 1988 [4b], Local Consensus [5]).Note 2: Children with LCP who have bettercovering of the femoral head in the acetabulumand a more normalized acetabular shape willhave greater hip abduction range of motion(Grzegorzewski 2006 [4b]).5. It is recommended that knee and ankle range ofmotion be assessed at the initial evaluation andthereafter if they are significantly limited.Lower extremity strength6. It is recommended that quantitative muscle testing isperformed using a hand held dynamometer due to itshigh intra- and inter-rater reliability (Gajdosik 2005[4b], Escolar 2001 [4b]). Muscle groups to assessinclude hip flexors, hip abductors, hip extensors, hipinternal rotators, hip external rotators, kneeextensors, knee flexors, and any other muscle groupthat is significantly limitedGait7. It is recommended that gait be qualitatively assessedfor common LCP deviations. Commonly observedgait characteristics seen in children with LCPinclude, but are not limited to: increased hip adduction on stance leg (Westhoff2006 [4b]), trunk lean outside the normal range (Westhoff 2006[4b]), trendelenburg (Westhoff 2006 [4b]) (hip drop onunaffected limb while in swing), compensated trendelenburg/reversetrendelenburg/Duchenne (Westhoff 2006 [4b])(trunk lean to the affected side while in stanceon the affected limb), toe in or toe out (Yoo 2008 [4b]).Note 1: Based on limited accessibility andfeasibility, the gold standard for gait analysis of3-D gait kinematics and kinetics (Toro 2003 [1b]) isnot recommended to be used in the clinic (LocalConsensus [5]).Note 2: There is insufficient evidence and lack ofreliability and validity (Toro 2003 [1b]) to supportuse of observational gait assessment tools withthis population (Local Consensus [5]).Balance8. It is recommended that balance be assessed based onweight bearing status. The desired outcome is thatthe patient maintain balance for age appropriatetimes for safe ambulation and stair negotiation (LocalConsensus [5]).Note: In patients 7 years or older, balance is tobe assessed using the Pediatric Balance Scale(Franjoine 2003 [4b]). If the patient is younger than7 years old, the test is unavailable, time does notpermit its use, or the patient is unable to followcommands, balance is assessed using single legstance on the involved side compared to theuninvolved side (Local Consensus [5]).Outcome measure scores9. It is recommended that the age appropriate PediatricQuality of Life Inventory Version 4.0 (PedsQL)(Varni 2001 [4a])Physical Functioning section isadministered at the initial evaluation, on a monthlybasis for reassessment of patient’s reportedfunctional status, and at discharge (Local Consensus[5]).Copyright © 2010 Cincinnati Children's Hospital Medical Center; all rights reserved. Page 3

Evidence-Based Care Guideline for Management of Legg-Calve-Perthes Disease in children aged 3 to 12 years Guideline 39Note 1: The Parent Report for Toddlers isadministered for children 2 to 4 years old (Varni2007a [4a]).Note 2: The Young Child Self Report isadministered for children 5 to 7 years old (Varni2007a [4a]).Note 3: The Child Self Report is administeredfor children 8 to12 years old (Varni 2007a [4a]).Note 4: For patients who are 5 to 12 years oldand unable to conceptually understand thequestionnaire tool, it is acceptable for the parentto complete the parent proxy form for the youngchild (age 5 to 7 years old) or child (age 8 to 12years old (Varni 2007b [4a]).Classification of Phases of Rehabilitation10. It is recommended that the Classification Instrumentin Perthes (CLIPer) be used to place the patient intoa rehabilitation classification phase uponexamination (Local Consensus [5]) (See Appendix 1 forinstructions and score sheet).Note 1: Conservative physical therapy treatmentwill be guided using the rehabilitationclassification phases (Local Consensus [5]).11. It is recommended that the patient be re-examinedusing the CLIPer on a monthly basis to determinethe appropriate progression through therehabilitation classification stages (Local Consensus[5]).Note 1: The patient is to be re-examined soonerif there is a change in status (Local Consensus [5]).Note 2: Pain, ROM, strength, gait, and balancewill be monitored at each treatment visit todetermine need for reassessment andreclassification using the CLIPer (Local Consensus[5]). (See Appendix 1 for instructions and scoresheet).12. It is recommended the patient is referred back to theorthopedic surgeon if the patient’s status worsensover two consecutive PT sessions (Local Consensus[5]).Management RecommendationsPhysical Therapy Interventions13. It is the recommendation that the patient diagnosedwith LCP and is a candidate for conservativemanagement receive supervised physical therapyintervention (Local Consensus [5]). (See Appendix 2 foralgorithm).Note: Physical therapy interventions have beenshown to improve ROM and strength in thispatient population (Brech 2006 [3b]). Individualswho participate in supervised clinic visitsdemonstrate greater improvement in musclestrength, functional mobility, gait speed, andquality of exercise performance than those whoreceive a home exercise program alone or noinstruction at all (Friedrich 1996 [2b]). Individualswho receive regular positive feedback from aphysical therapist are more likely to be compliantwith a supplemental home exercise program(Sluijs 1993 [4b]).14. It is recommended that supervised physical therapyis supplemented with a customized written homeexercise program (Friedrich 1996 [2b]) in all phases ofrehabilitation15. It is recommended that the physical therapist engagein ongoing communication with the patient, family,and referring physician regarding the diseaseprocess and plan of care (Local Consensus [5]).16. It is recommended that the patient begin physicaltherapy upon diagnosis of LCP (Local Consensus [5]).17. It is recommended that advancement through thephases of rehabilitation follow a goal based ratherthan a time based progression.Note: Treatment is to focus on containment ofthe femoral head in the acetabulum throughouteach phase of the disease (Leach 2006 [5]).Phases of RehabilitationSevere Involvement Phase (CLIPer score 14 to 24)18. It is recommended that goals of this phase include(Local Consensus [5]): Reduce pain to less than 7/10 Increase ROM to 50% or greater of theuninvolved side Increase strength to 50% or greater of theuninvolved side Patient to be independent with the appropriateassistive device and weight bearing precautionsImprove balance to 50% or greater of themaximum Pediatric Balance Scale score orsingle limb stance of the uninvolved side.19. It is recommended that supervised Physical Therapyservices are provided weekly (Bailes 2008 [5a]) at afrequency of 2 times per week (Local Consensus [5]).Copyright © 2010 Cincinnati Children's Hospital Medical Center; all rights reserved. Page 4

Evidence-Based Care Guideline for Management of Legg-Calve-Perthes Disease in children aged 3 to 12 years Guideline 3920. It is recommended that treatment interventions ofthe Severe Involvement Phase include: Paino Hot pack for pain management withstretching (Nadler 2004 [1b])o Cryotherapy (Nadler 2004 [1b])o Medications as prescribed by the referringphysician for pain (Local Consensus [5]).ROM (See Appendix 3 for exerciseprescription)o Static stretch for LE musculature (Moseley2005 [2A], Bandy 1998 [2a], Davis 2005 [2b]) withor without use of a hot pack based onpatient preference and comfort.o Dynamic ROM(Bandy 1998 [2a])and activeassistive range of motion (AAROM) maybe appropriate if the patient is muscleguarding due to pain and unable to achieveend range motion with static stretch (LocalConsensus [5]).o Perform AROM and AAROM followingpassive stretching to maintain newly gainedROM (Depino 2000 [2b]).oPerform stretching in a position that issuitable for the individual and include hipIR, hip ER, hip abduction, hip extension,and any other lower extremity motion thatis significantly limited (Local Consensus [5]).Strength (See Appendix 4 for exerciseprescription)o Begin with isometric exercise and progressto isotonic exercises in a gravity lessenedposition with further progression to isotonicexercises against gravity. It is appropriateto include concentric and eccentriccontractions (Brech 2006 [3b]).o Begin with 2 sets of 10 to 15 repetitions ofeach exercise (Rhea 2002 [2b], Faigenbaum 1996[4b]), with progression to 3 sets of eachexercise to be used (Rhea 2002 [2b]).Note: If the patient is unable to perform 2sets of 10 repetitions of an exercise, thedifficulty of the exercise is to be decreasedeither through weight or type of exercise(Local Consensus [5]).o Focus on strengthening of hip abductors,hip flexors, hip external rotators, hipinternal rotators, hip extensors, or any otherlower extremity muscle group that displaysoooosignificant strength deficits (Westhoff 2006[4b], Bolgla 2005 [4b]). Special attention is tobe given to the gluteus medius in order tominimize intraarticular pain (Plasschaert 2006[4b]) and for pelvic control during single legactivities and ambulation (Westhoff 2006 [4b],Bolgla 2005 [4b]).Performance of weight bearing vs. nonweightbearing exercises is based on theweight bearing status as determined by thephysician, patient’s tolerance to weightbearing positions, and safety (Local Consensus[5]).Weight bearing activities can be performedwhen the patient can apply weight on theinvolved extremity (Bolgla 2005 [4b]) andsafely perform the exercise in a standingposition.Closed chain double limb exercises withlight resistance (less than full body weight)(e.g. Total Gym ®) may be performed(Bolgla 2005 [4b]).It is not recommended to perform singlelimb closed chain exercises on the involvedside (Local Consensus [5]) due to increasedintra-articular pressure in the hip joint(Levangie 1992 [5]).Balanceo Activities that include double limb stanceand a narrowed base of support on stablesurfaces may be performed if weightbearing status and symptoms allow (LocalConsensus [5]).o It is not recommended to perform singlelimb activities (Local Consensus [5])due toincreased intra-articular pressure in the hipjoint (Levangie 1992 [5]) .Gaito Follow the referring physician’s guidelinesfor weight bearing status (Local Consensus [5]).o Begin gait training with the appropriateassistive device given weight bearing statusas determined by the referring physician orbased on the patient’s tolerance to fullweight bearing due to pain or safety (LocalConsensus [5]).Moderate Involvement Phase (CLIPer score 6 to 13)21. It is recommended that goals of this phase include:Copyright © 2010 Cincinnati Children's Hospital Medical Center; all rights reserved. Page 5

Evidence-Based Care Guideline for Management of Legg-Calve-Perthes Disease in children aged 3 to 12 years Guideline 39 Reduce pain to less than 4/10 Increase ROM to greater than 75% of theuninvolved side Increase strength to greater than 75% of theuninvolved side Progress from use of an assistive device ifapproved by physician and without adverseeffects Independence with a step to pattern on stairswithout UE support Improved efficiency in walking Improved balance to greater than 75% of themaximum Pediatric Balance Scale score orsingle limb stance of the uninvolved side(Local Consensus [5])22. It is recommended that supervised Physical Therapyservices are provided on a weekly basis (Bailes 2008[5a]) at a frequency of 1 to 2x/week (Local Consensus[5]).23. It is recommended that treatment interventions ofthe Moderate Involvement Phase include: Paino Hot pack for pain management withstretching (Nadler 2004 [1b])o Cryotherapy (Nadler 2004 [1b])o Medications as prescribed by the referringphysician for pain (Local Consensus [5]).ROM (See Appendix 3 for exerciseprescription)o Static stretch for LE musculature (Moseley2005 [2A], Bandy 1998 [2a], Davis 2005 [2b]) withor without use of a hot pack based onpatient preference and comforto Dynamic ROM (Bandy 1998 [2a])andAAROM may be appropriate if the patientis muscle guarding due to pain and unableto achieve end range motion with staticstretch (Local Consensus [5]).o Perform AROM and AAROM followingpassive stretching to maintain newly gainedROM (Depino 2000 [2b]).oPerform stretching in a position that issuitable for the individual and include hipIR, hip ER, hip abduction, hip extension,and any other lower extremity motion thatis significantly limited (Local Consensus [5]).Strengthening (See Appendix 4 for exerciseprescription.)o Perform isotonic exercises in gravitylessened and against gravity positions, andinclude concentric and eccentriccontractions (Brech 2006 [3b]).o Weight bearing and non-weight bearingactivities can be used in combination basedon the patient’s ability (Jacobs 2008 [4b]) andgoals of the treatment session.o Upper extremity supported functionaldynamic single limb activities (e.g. step ups,side steps) may be performed (LocalConsensus [5]).o Double limb closed chain exercises may beused with light resistance (e.g. mini-squats,wall-sits) if the weight bearing status allows(Local Consensus [5]).Balanceo Activities that include double limb stancewith narrowed base of support on unstablesurfaces may be performed as weightbearing status and patient comfort allow(Local Consensus [5]).o Limit prolonged single limb activities (LocalConsensus [5]) due to excessive jointcompressive forces (Levangie 1992 [5]).Gaito Continue to follow the referring physician’sguidelines for weight bearing status (LocalConsensus [5]).o Progress to gait training without use of anassistive device as appropriate, focusing onminimizing deficits and improvingefficiency of walking (Local Consensus [5]).o Stair negotiation and other functionalmobility (Local Consensus [5]).Mild Involvement Phase (CLIPer score 0 to 5)24. It is recommended that goals of this phase include: Reduce pain to 1/10 or less Increase range of motion to 90% or greater ofthe uninvolved side Increase strength to 90% or greater of theuninvolved side Improve balance to 90% or greater of themaximum Pediatric Balance Scale score orsingle limb stance of the uninvolved side Ambulation with a non-painful limp withnormal efficiencyCopyright © 2010 Cincinnati Children's Hospital Medical Center; all rights reserved. Page 6

Evidence-Based Care Guideline for Management of Legg-Calve-Perthes Disease in children aged 3 to 12 years Guideline 39o Negotiation of stairs utilizing a reciprocalpattern(Local Consensus [5])25. It is recommended that supervised physical therapyservices are provided on a periodic or bimonthlybasis (Bailes 2008 [5a]) at a frequency of 1 to 2x/month(Local Consensus [5]).26. It is recommended that treatment interventions ofthe Mild Involvement Phase include: Paino Hot pack for pain management withstretching (Nadler 2004 [1b])o Cryotherapy (Nadler 2004 [1b])o Medications as prescribed by the referringphysician for pain (Local Consensus [5])ROM (See Appendix 3 for exerciseprescription.)o Static stretch for LE musculature (Moseley2005 [2A], Bandy 1998 [2a], Davis 2005 [2b]) withor without use of a hot pack based onpatient preference and comfort (Nadler 2004[1b])o Dynamic ROM (Bandy 1998 [2a])andAAROM may be used if the patient ismuscle guarding due to pain and is unableto achieve end range of motion with staticstretch (Local Consensus [5]).o Perform AROM and AAROM followingpassive stretching to maintain newly gainedROM (Depino 2000 [2b]).oPerform stretching in a position that issuitable for the individual and include hipIR, hip ER, hip abduction, hip extension,and any other lower extremity motion thatis significantly limited (Local Consensus [5]).Strengthening (See Appendix 4 for exerciseprescription)o Isotonic exercises in gravity lessened andagainst gravity positions may be used andinclude concentric and eccentriccontractions (Brech 2006 [3b])o Functional dynamic single limb activitieswith UE support as needed for patientsafety (e.g. step ups, sidesteps) may beperformed (Local Consensus [5])o Closed kinetic chain single limb exerciseswith light resistance (leg press) may beperformed (Bolgla 2005 [4b]).Balanceo Double limb stance with narrowed base ofsupport on unstable surfaces withperturbations may be performed as weightbearing status and patient comfort allow(Local Consensus [5]).o Limit prolonged single limb activities (LocalConsensus [5]) due to excessive jointcompressive forces (Levangie 1992 [5]).Gaito Continue to follow the referring physician’sguidelines for weight bearing statuso Progress to gait training without the use ofan assistive device as appropriate, focusingon minimizing deficits and improving theefficiency of walking.o Stair negotiation and other functionalmobility.o Progress to walking on uneven surfaceswith an emphasis on safety.(Local Consensus [5])Discharge Criteria27. It is recommended that children may be dischargedfrom physical therapy at the time of surgicalintervention or when four of the five followingcriteria have been met: Pain rating 0 to 1/10 Range of motion 90 to 100% of the uninvolvedside Strength 90 to 100% of the uninvolved side Balance 90 to 100% of the maximum score onthe Pediatric Balance Scale or maintainingbalance with SLS 90 to 100% of the uninvolvedside Gait presents with a non-painful limp and uses areciprocal pattern on the stairs.(Local Consensus [5])28. It is recommended that following discharge fromsupervised physical therapy, the patient continue ahome exercise program to maintain improvementsin strength, balance, locomotor function, and painmanagement (Local Consensus [5]) until it has beendetermined by the physician that the disease processis complete (Local Consensus [5]).29. It is recommended that physical therapy services areprovided as needed on a consultative basis (Bailes2008 [5a]) to manage flare up of symptoms or otherconcerns (Local Consensus [5]).Copyright © 2010 Cincinnati Children's Hospital Medical Center; all rights reserved. Page 7

Evidence-Based Care Guideline for Management of Legg-Calve-Perthes Disease in children aged 3 to 12 years Guideline 3930. It is recommended that the post-operative guidelinefor LCP be followed when a patient is dischargedfrom conservative management physical therapydue to surgical intervention. (Local Consensus [5]).Future Research Agenda1. In children ages 3 to 12 years old with Legg-Calve-Perthes disease, what is the reliability and validity ofthe Classification Instrument in Perthes (CLIPer)?2. In children ages 3 to 12 years old with Legg-Calve-Perthes disease, what are the effects of weightbearing on symptoms, joint preservation, andfunction?Copyright © 2010 Cincinnati Children's Hospital Medical Center; all rights reserved. Page 8

Evidence-Based Care Guideline for Management of Legg-Calve-Perthes Disease in children aged 3 to 12 years Guideline 39Appendix 1Classification Instrument in Perthes (CLIPer)Domains of Assessment DescriptionScorePain with ADLs7 to10/10 44 to 6/10 20 to 3/10 0Hip ROMLess than 50% of uninvolved side for the majority of directions 650 to 75% of uninvolved side for the majority of directions 376 to 100% of uninvolved side for the majority of directions 0Hip StrengthLess than 50% of uninvolved side for the majority of muscle groups 650 to 75% of uninvolved side for the majority of muscle groups 376 to 100% of uninvolved side for the majority of muscle groups 0BalancePediatric balance score less than 50% of best score (best score=56) OR SLSwith eyes open less than 50% of time on uninvolved sidePediatric balance score 50 to 75% of best score (best score=56) OR SLSwith EO of uninvolved side 50 to 75% length of timePediatric balance score 76 to 100% of best score (best score=56) OR SLSwith EO 76 to 100% of uninvolved side420GaitNWB and uses an assistive device and without AD, displays excessive gaitdeficits with decreased efficiencyNo assistive device and displays excessive deficits without a decrease inefficiencyUses step to pattern on stairsNon-painful limpAble to perform reciprocal pattern on stairs420Rehabilitation Classification PhaseScore total 14 to 24: Severe InvolvementScore total 6 to 13: Moderate InvolvementScore total 0 to 5: Mild InvolvementTOTAL:Instructions for useUpon examination, assess pain with ADL’s, hip range of motion, hip strength, balance, and gait. Assign a correlatingscore for each domain of assessment based on examination results and total the sum. Place the patient in a rehabilitationclassification phase based on the total score to guide physical therapy treatment.Copyright © 2010 Cincinnati Children's Hospital Medical Center; all rights reserved. Page 9

Evidence-Based Care Guideline for Management of Legg-Calve-Perthes Disease in children aged 3 to 12 years Guideline 39Appendix 2Start: Patient has beendiagnosed with Legg-Calve-Perthes DiseaseIs classification score14 to 24?NoYesFollow severe involvement phasetreatmentIs classification score6 to 13?NoYesFollow moderate involvement phasetreatmentIs classification score0 to 5?YesNoDoes patient meetdischarge criteria?YesDiscontinue PT.Continue with HEP.Follow consultativemodel as needed.Follow mild involvement phasetreatmentPt to have immediatesurgical procedure?NoYesDiscontinue preoperativePT.Guidelinediscontinued.Reassess monthly or sooner if patient demonstrates a change in status: Pain, ROM, Strength, Balance, and GaitHas classification scorechanged?YesDoes condition worsenover 2 consecutive PTsessions?YesRefer back toOrthopaedicsurgeon.NoNoContinue PT undercurrent rehabilitationphasePlace patient in newclassification phasebased on new scorefor continuedtreatmentCopyright © 2010 Cincinnati Children's Hospital Medical Center; all rights reserved. Page 10

Evidence-Based Care Guideline for Management of Legg-Calve-Perthes Disease in children aged 3 to 12 years Guideline 39Appendix 3ROM Exercise PrescriptionIntervention Parameters Intensity Notes Muscle GroupsStatic Stretch 2 minutes of stretchingper day, per musclegroup (Santonja Medina 2007[2b], Cipriani 2003 [4b]).Gentle static hold DynamicStretch30 second hold time,doing 4 repetitions permuscle group (Bandy 1994[2a], Reid 2004 [2b], Depino2000 [2b]).If not tolerated, may do10 to 30 second hold timewith repetitions adjustedto meet 2 minuterequirement (e.g. ifholding 15 seconds,would do 8 stretches)(Cipriani 2003 [4b], Roberts1999 [4b]).5 second hold, done with24 repetitions permuscle group per day tomeet 2 minute stretchingtime required (Bandy 1998[2a], Santonja Medina 2007[2b], Cipriani 2003 [4b]).Should be within patientpain tolerance andwithout muscle guardingso as to prevent tissuedamage and inflammatoryresponse (Bandy 1994 [2a],Santonja Medina 2007 [2b],Davis 2005 [2b]).Self selected intensity bypatient as long as notcausing painThis is the preferredmethod of stretching togain flexibility and/orROM (Moseley 2005 [2A],Bandy 1994 [2a], Davis 2005[2b]).stretching be done afterwarm up, but prior toactive exercises tomaintain newly gainedROM (Depino 2000 [2b]).Dynamic stretch is donewith patient activation ofantagonistic muscle group(Bandy 1998 [2a]).This is done if patientdoes not tolerate staticstretchAny limited motions ortight muscles in the lowerextremities should beaddressed, but withparticular emphasis on thefollowing: hip adductors hip internal rotators hip external rotators hip flexors(Local Consensus [5])Copyright © 2010 Cincinnati Children's Hospital Medical Center; all rights reserved. Page 11

Evidence-Based Care Guideline for Management of Legg-Calve-Perthes Disease in children aged 3 to 12 years Guideline 39Appendix 4Strengthening Exercise PrescriptionIntervention Parameters Intensity Notes Muscle GroupsIsometricStrengthening 10 second hold with 10repetitions per muscle Performed atapproximately 75% Performed with hip inneutral position,Any lower extremitymuscle that demonstratesgroup, for a total of 100 maximal contraction o 0 degrees hip weakness should beseconds (Local Consensus (Local Consensus [5])flexion/extension, addressed, however,[5]).o 0 degrees hipspecial attention should be Can adjust hold time to 5abduction/adductiongiven to the following:seconds, would thenand hip abductorsadjust to 20 repetitions too 0 degrees hip(especially gluteusmeet 100 secondexternal/internal medius)requirementrotation hip internal rotators(Local Consensus [5]) hip external rotatorsIsotonic High repetitions (10 to Low resistance hip flexorsStrengthening 15 reps) and 2 to 3 sets(Campos 2002 [2b],Faigenbaum 1999 [2b]).Perform both concentricand eccentric contractions(Brech 2006 [3b])Rest 1 to 3 minutesbetween sets (Campos 2002[2b], Jacobs 2008 [4b], Bolgla2005 [4b])(depending onstrength and endurance)Rest can include exerciseof a different musclegroup or cessation ofactivityIf patient is unable toperform 2 sets of 10repetitions with properform, exercise intensityshould be decreased eitherthrough weight or type ofexercisehip extensors(Plasschaert 2006 [4b],Westhoff 2006 [4b], Bolgla2005 [4b])Copyright © 2010 Cincinnati Children's Hospital Medical Center; all rights reserved. Page 12

Evidence-Based Care Guideline for Management of Legg-Calve-Perthes Disease in children aged 3 to 12 years Guideline 39Members of Legg-Calve-Perthes DiseaseTeam 2010Division of Occupational Therapy and Physical TherapyGuideline Development TeamJulie A. Lee, PT, DPT, Team Leader, Division of OccupationalTherapy and Physical TherapyMichael D. Allen, PT, OCS, Division of Occupational Therapy andPhysical TherapyKathleen Hugentobler, PT, DPT, Division of Occupational Therapyand Physical TherapyChristopher Kovacs, PT, DPT, CSCS, Division of OccupationalTherapy and Physical TherapyJessica King Monfreda PT, DPT, Division of Occupational Therapyand Physical TherapyBarbara Nolte, PT, Division of Occupational Therapy and PhysicalTherapyElizabeth Woeste, MPT, Division of Occupational Therapy andPhysical TherapySenior Clinical DirectorRebecca D. Reder OTD, OTR/L, Division of Occupational Therapyand Physical TherapyDivision of Health Policy & Clinical Effectiveness SupportEloise Clark, MPH, FacilitatorDanette Stanko, MA, MPH, EpidemiologistEdward Donovan, MD, Medical Director, Clinical EffectivenessUma Kotagal, MBBS, MSc, VP, Division DirectorAll Team Members and Clinical Effectiveness support staff listedabove have signed a conflict of interest declaration and none werefound.Ad hoc AdvisorsMichelle Kiger, OTR/L, Division of Occupational Therapy andPhysical TherapyMary Gilene, MBA, Division of Occupational Therapy and PhysicalTherapyBarbarie Hill MLS, Pratt LibraryKaren Vonderhaar, MS, RN, MethodologistDevelopment ProcessThe process by which this guideline was developed is documented inthe Guideline Development Process Manual; a Team Bindermaintains minutes and other relevant development materials. Therecommendations contained in this guideline were formulated by aninterdisciplinary working group which performed systematic searchand critical appraisal of the literature, using the Table of EvidenceLevels described following the references, and examined current localclinical practices.To select evidence for critical appraisal by the group for thisguideline, the Medline, EmBase and the Cochrane databases weresearched for dates of January 1970 to April 2009 to generate anunrefined, “combined evidence” database using a search strategyfocused on answering clinical questions relevant to Legg-Calve-Perthes Disease and employing a combination of Boolean searchingon human-indexed thesaurus terms (MeSH headings using an OVIDMedline interface) and “natural language” searching on searching onhuman-indexedthesaurus terms (MeSH headings using an OVID Medline interface)and “natural language” searching on words in the title, abstract, andindexing terms. The citations were reduced by: eliminatingduplicates, review articles, non-English articles, and adult articles.The resulting abstracts were reviewed by a methodologist to eliminatelow quality and irrelevant citations. During the course of theguideline development, additional clinical questions were generatedand subjected to the search process, and some relevant review articleswere identified.Tools to assist in the effective dissemination and implementation ofthe guideline may be available online athttp://www.cincinnatichildrens.org/svc/alpha/h/health-policy/evbased/default.htm. Experience with the implementation of earlierpublications of this guideline has provided learnings which have beenincorporated into this revision.Once the guideline has been in place for three years, the developmentteam reconvenes to explore the continued validity of the guideline.This phase can be initiated at any point that evidence indicates acritical change is needed.Recommendations have been formulated by a consensus processdirected by best evidence, patient and family preference and clinicalexpertise. During formulation of these recommendations, the teammembers have remained cognizant of controversies anddisagreements over the management of these patients. They havetried to resolve controversial issues by consensus where possible and,when not possible, to offer optional approaches to care in the form ofinformation that includes best supporting evidence of efficacy foralternative choices.The guideline has been reviewed and approved by clinical experts notinvolved in the development process, distributed to seniormanagement, and other parties as appropriate to their intendedpurposes.The guideline was developed without external funding. All TeamMembers and Clinical Effectiveness support staff listed have declaredwhether they have any conflict of interest and none were identified.Copies of this Evidence-based Care Guideline (EBCG) and its anyavailable implementation tools are available online and may bedistributed by any organization for the global purpose of improvingchild health outcomes. Website address:http://www.cincinnatichildrens.org/svc/alpha/h/healthpolicy/ev-based/default.htmExamples of approved uses of theEBCG include the following:• copies may be provided to anyone involved in the organization’sprocess for developing and implementing evidence based careguidelines;• hyperlinks to the CCHMC website may be placed on theorganization’s website;• the EBCG may be adopted or adapted for use within theorganization, provided that CCHMC receives appropriateattribution on all written or electronic documents; and• copies may be provided to patients and the clinicians who managetheir care.Notification of CCHMC at HPCEInfo@cchmc.org for any EBCG,or its companion documents, adopted, adapted, implemented orhyperlinked by the organization is appreciated.Copyright © 2010 Cincinnati Children's Hospital Medical Center; all rights reserved. Page 13

Evidence-Based Care Guideline for Management of Legg-Calve-Perthes Disease in children aged 3 to 12 years Guideline 39NOTE: These recommendations result from review of literatureand practices current at the time of their formulations. Thisguideline does not preclude using care modalities provenefficacious in studies published subsequent to the current revisionof this document. This document is not intended to imposestandards of care preventing selective variances from therecommendations to meet the specific and unique requirementsof individual patients. Adherence to this guideline is voluntary.The clinician in light of the individual circumstances presented bythe patient must make the ultimate judgment regarding thepriority of any specific procedure.For more information about this guideline, its supporting evidencesand the guideline development process, contact the Health Policy &Clinical Effectiveness office at: 513-636-2501 orHPCEInfo@cchmc.org ..Copyright © 2010 Cincinnati Children's Hospital Medical Center; all rights reserved. Page 14

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J Pediatr Orthop,28(7): 717-22, 2008, [4b] http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=18812896Note: Full tables of evidence grading system available in separatedocument: Table of Evidence Levels of Individual Studies by Domain,Study Design, & Quality (abbreviated table below) Grading a Body of Evidence to Answer a Clinical Question Judging the Strength of a Recommendation (abbreviated tablebelow)Table of Evidence Levels (see note above)Quality level Definition1a† or 1b†Systematic review, meta-analysis, or metasynthesisof multiple studies2a or 2b Best study design for domain3a or 3b Fair study design for domain4a or 4b Weak study design for domainOther: General review, expert opinion, case5report, consensus report, or guideline†a = good quality study; b = lesser quality studyTable of Recommendation Strength (see note above)StrengthDefinition“Stronglyrecommended”“Recommended”No recommendationmadeThere is consensus that benefits clearlyoutweigh risks and burdens(or visa-versa for negativerecommendations).There is consensus that benefits are closelybalanced with risks and burdens.There is lack of consensus to directdevelopment of a recommendation.Dimensions: In determining the strength of a recommendation, thedevelopment group makes a considered judgment in a consensusprocess that incorporates critically appraised evidence, clinicalexperience, and other dimensions as listed below.1. Grade of the Body of Evidence (see note above)2. Safety / Harm3. Health benefit to patient (direct benefit)4. Burden to patient of adherence to recommendation (cost, hassle,discomfort, pain, motivation, ability to adhere, time)5. Cost-effectiveness to healthcare system (balance of cost /savings of resources, staff time, and supplies based on publishedstudies or onsite analysis)6. Directness (the extent to which the body of evidence directlyanswers the clinical question [population/problem, intervention,comparison, outcome])7. Impact on morbidity/mortality or quality of lifeCopyright © 2010 Cincinnati Children's Hospital Medical Center; all rights reserved. Page 16