Perthes Disease &

Physiotherapy Management

Dr. D. N. Bid [PT]Senior LecturerSarvajanik College of Physiotherapy, Surat.

FIRST DESCRIBED BY LEGG, AND WALDENSTORM IN 1909,

BY PERTHES AND CALVE IN 1910

DEFINITION

Legg-Calvé-Perthes disease (LCPD) is the name given to idiopathic osteonecrosis of the capital femoral epiphysis in a child.

Increased Joint space

Smaller headDenser headNormal

joint

Epidemiology Prevalence:

77.4%

8.5%5.3% 2.8% 6.0%

Transient synovitis

SCFE

Infection

Perthes' disease

Other

Epidemiology

Race: Caucasians are affected more frequently than persons of other races.

Sex: Males are affected 4-5 times more often than females.

Age: LCPD most commonly is seen in persons aged 3-12 years, with a median age of 7 years.

BLOOD SUPPLY

Causes Exact cause

unknown. Proposed

theories. Inherited protein C

and/or S deficiency.

Venous thrombosis Arterial occlusion Raised intra

osseous pressure

Causes

Proposed theories. Excessive femoral anteversion. Synovitis. Generalized skeletal disorder. Arterial anomalies.

Causes

Pathophysiology The capital femoral epiphysis always is involved. In 15-

20% of patients with LCPD, involvement is bilateral.

The blood supply to the capital femoral epiphysis is interrupted.

Bone infarction occurs, especially in the subchondral cortical bone, while articular cartilage continues to grow. (Articular cartilage grows because its nutrients come from the synovial fluid.)

Revascularization occurs, and new bone ossification starts.

Pathophysiology At this point, a percentage of patients develop

LCPD, while other patients have normal bone growth and development.

LCPD is present when a subchondral fracture occurs. This is usually the result of normal physical activity, not direct trauma to the area

Changes to the epiphyseal growth plate occur secondary to the subchondral fracture.

Pathogenesis Avascular necrosis Temporary cessation of growth Revascularization from periphery Resumption of ossification and trauma Pathological fracture Resorption of underlying bone Replacement of biologically plastic bone Sublaxation Deformity

Clinical History: Symptoms usually have been

present for weeks. Hip or groin pain, which may be

referred to the thigh Mild or intermittent pain in anterior

thigh or knee Limp Usually no history of trauma

Clinical

Physical: Painful gait Decreased range of motion (ROM),

particularly with internal rotation and abduction

Atrophy of thigh muscles secondary to disuse

Muscle spasm Leg length inequality due to collapse

Clinical

Short stature: Children with LCPD often have delayed bone age.

Roll test With patient lying in the supine position,

the examiner rolls the hip of the affected extremity into external and internal rotation.

This test should invoke guarding or spasm, especially with internal rotation.

Differential Diagnosis Listed are some other disorders that should be included

in the differential diagnosis for LCPD: Septic arthritis-This is an infection in the joint Sickle cell-Osteonecrosis of the hip can be a result of

this disease Spondyloepiphyseal Dysplasia Tarda-This disease

typically affects the spine and the larger more proximal joints

Gaucher’s Disease- This is a genetic disorder that often times includes bone pathology

Transient Synovitis-This is an acute inflammatory process and is the most common cause of hip pain in childhood

Workup

Lab Studies: CBC Erythrocyte sedimentation rate - May

be elevated if infection present

WorkupImaging Studies: Plain x-rays of the hip are extremely useful in

establishing the diagnosis. Frog leg views of the affected hip are very helpful. Plain radiographs have a sensitivity of 97% and a

specificity of 78% in the detection of LCPD Multiple radiographic classification systems exist,

based on the extent of abnormality of the capital femoral epiphysis. Waldenstrom, Catterall, Salter and Thompson, and

Herring are the 4 most common classification systems.

No agreement has been reached as to the best classification system.

Radiographic stages

Five radiographic stages can be seen by plain x-ray. In sequence, they are as follows:

Radiographic stages

1.Cessation of growth at the capital femoral epiphysis; smaller femoral head epiphysis and widening of articular space on affected side.

Radiographic stages

2. Subchondral fracture; linear radiolucency within the femoral head epiphysis

Radiographic stages

3. Resorption of bone

Radiographic stages

4. Re-ossification of new bone

Radiographic stages

5. Healed stage

Catterall classification

Catterall Group I: Involvement only of the anterior epiphysis (therefore seen only on the frog lateral film)

Catterall Group II: Central segment fragmentation and collapse. However the lateral rim is intact and thus protects the central involved area.

Catterall Group III: The lateral head is also involved or fragmented and only the medial portion is spared. The loss of lateral support worsens the prognosis.

Catterall Group IV: The entire head is involved. Catterall's classification has a significant inter and intra

observer error.

Catterall: I – anterior epiphysis

II – up to 50% with collapse

III – only small posterior portion not involved

IV – whole head involvement

Catterall I:

Catterall II:

Catterall III:

Catterall IV:

Catterall classification

Groups I and II had a good prognosis (in 90%) and required no intervention.

Groups III and IV had a poor prognosis (in 90 %) and required treatment.

The classification is applied to the frog lateral and AP film during the fragmentation phase

Salter and Thompson Classification

Salter and Thompson recognized that Catterall's first two groups and second two groups were distinct and therefore proposed a two part classification.

Salter & Thompson Group A: Less than 1/2 head involved.

Salter & Thompson Group B: More than 1/2 head involved.

Again the main difference between these two groups is the integrity of the lateral pillar.

(Herring) Lateral Pillar Classification

Lateral Pillar Group A: There is no loss in height of the lateral 1/3 of the head and minimal density change. Fragmentation occurs in the central segment of the head.

Lateral Pillar Group B: There is lucency and loss of height in the lateral pillar but not more that 50% of the original (contralateral) pillar height. there may be some lateral extrusion of the head.

Lateral Pillar Group C: There is greater than 50% loss in the height of the lateral pillar. The lateral pillar is lower than the central segment early on.

Herring A:

Herring B: Herring C:

And now there is “B/C Border” since the ability to differentiate B / C proved elusive

Unilateral Perthes with entire head involvement and fragmentation. The reossification phase has not yet begun.

Unilateral Perthes disease with widening of the medial joint space, blurring of the physis, increased density of the head and lucency between the medial and central 1/3's of the head corresponding to early fragmentation phase.

Unilateral Perthes in the reossification phase with a visible subchondral line similar to Waldenstrom's sign. However Waldenstrom's subchondral fracture is seen very early in the disease process, before fragmentation. In this case the lateral pillar has maintained some integrity.

“Head at risk signs”

1. Gage's sign. a V shaped lucency in the lateral epiphysis.

2. lateral calcification (lateral to the epiphysis) (implies loss of lateral support)

3. lateral subluxation of the head.(implies loss of lateral support)

4. A horizontal growth plate.(implies a growth arrest phenomenon and deformity)

Workup Technetium 99 bone

scan - Helpful in delineating the extent of avascular changes before they are evident on plain radiographs. The sensitivity of

radionuclide scanning in the diagnosis of LPD is 98%, and the specificity is 95%.

Dynamic arthrography - Assesses sphericity of the head of the femur.

Ultrasonography in transient synovitis and early Perthes' disease

Ultrasonography may provide significant diagnostic clues to differentiate early Perthes' from transient synovitis.

T Futami, Y Kasahara, S Suzuki, S Ushikubo, and T Tsuchiya Journal of Bone and Joint Surgery - British Volume, Vol 73-B, Issue 4, 635-

639

CT Scan Staging determined

by using plain radiographic findings is upgraded in 30% of patients.

Not as sensitive as nuclear medicine or MRI.

CT may be used for follow-up imaging in patients with LPD.

MRI It allows more precise

localization of involvement than conventional radiography.

MRI is preferred for evaluating the position, form, and size of the femoral head and surrounding soft tissues.

MRI is as sensitive as isotopic bone scanning.

Outcome variables

Age Extent of involvement Duration Remodeling potential Premature physeal closure Type of treatment Stage of disease at treatment.

Treatment

Goals of treatment Achieve and maintain ROM Relieve weight bearing Containment of the femoral epiphysis

within the confines of the acetabulum Traction

Rational behind "containment"

Containment of the head within the acetabulum is reported to encourage spherical remodelling during the reossification and subsequent phases.

However if there is total head involvement and the lateral pillar collapses then the effect of containment is probably less.

Therefore it seems that the extent of involvement of the head is the critical factor and containment simply optimizes the situation.

Medications Medical treatment does not stop or reverse

the bony changes. Appropriate analgesic medication should be

given. Nonsteroidal anti-inflammatory drugs

Ibuprofen Adult dose: 200-400 mg PO q4-6h; not to exceed 3.2 g/d. Pediatric dose: 6 months to 12 years: 20-40 mg/kg/d PO divided

tid or qid; start at lower end of dosing range and titrate upward; not to exceed 2.4 g/d>12 years: Administer as in adults.

Non surgical containment

Scottish Rite abduction brace

LCPD – Petrie broomstick POP

Petrie-1971

LCPD - Braces

LCPD – Atlanta “Scottish Rite”

LCPD – Unilateral Brace-Birmingham

LCPD – Bilateral Brace

LCPD – Bicycle Containment

LCPD – Bike and Brace

Japanese modification of Petrie abduction cast

Surgical containment

Greater trochanteric overgrowth

The trochanteric overgrowth can be dramatic on radiographs but several studies have shown that a Trendelenberg gait does not always occur.

If it does occur, and is significant, then trochanteric advancement may improve the gait.

An alternative is to perform a trochanteric arrest at an earlier date but this assumes that the first statement will not apply to the particular child.

RECONSTRUCTIVE SURGERY

INDICATIONS: Hinge abduction. valgus subtrochanteric

osteotomy.

Malformed femoral head in late group III or residual group IV. Garceau’s cheilectomy.

Coxa magna. shelf augmentation A large malformed

femoral head with lateral sublaxation. Chiari’s pelvic osteotomy.

Capital femoral physeal arrest. trochanteric advancement or arrest.

SUMMARY

For patients less that 6 years old the prognosis is good for the majority.

If they are stiff or painful they respond to bed rest, traction and pain relieving anti-inflammatory medication.

There is no evidence that abduction splints or surgical intervention is warranted in the majority of these younger patients.

SUMMARY For patients between 6 and 8 years but with a

bone age less than 6 and an intact lateral pillar (Herring A and B) the prognosis is similar to that for the first group and observation is as good as surgical intervention for the majority.

If they have bone ages greater than 6 years and Herring lateral pillar classification B then "containment" of the head within the acetabulum seems to be warranted.

This may be done by abduction bracing, femoral varus osteotomy or a pelvic osteotomy.

SUMMARY If they are between 6 and 8 and are in lateral

pillar group C then the result of intervention are equivocal.

Children presenting with Perthes disease at age 9 or older often have lateral pillar B or C and a poor prognosis.

The trend is towards early containment of these hips although stiffness can be a problem following early pelvic (Salter's) osteotomy.

Follow-up

Initially, close follow-up is required to determine the extent of necrosis.

Once the healing phase has been entered, follow-up can be every 6 months.

Long-term follow-up is necessary to determine the final outcome.

Prognosis The younger the age of onset of LCPD, the

better the prognosis.

Children older than 10 years have a very high risk of developing osteoarthritis.

Most patients have a favorable outcome.

Prognosis is proportional to the degree of radiologic involvement.

Outcome Measures The Lower Extremity Functional Scale is one that

measures how this disease is affecting the child in a functional way. Since this questionnaire does ask about certain activities the child may not be allowed to perform (i.e. running, hopping, etc), it may not be the outcome measure of choice.

The Harris Hip score is another questionnaire that has more to do with a lower level of functional activities such as walking, stair climbing, donning/doffing shoes, sitting, etc.

Questionnaires that test the patient on a functional level are useful to provide a baseline and monitor functional progress in the patient’s activities.

Examination 1. The limp:

The limp is usually antalgic.[4]

It is possible that the child has a Trendelenburg gait (a positive Trendelenburg test on the affected side) which is marked by a pelvic drop on the unloaded side during single stance. [9]

The child can also have a Duchenne gait, which is marked by a trunk lean toward the stance limb with the pelvis level or elevated on the unloaded side. [9]

2. Range of motion: [4]

The restriction of hip motion is variable in the early stages of the disease;Many patients, may only have a minimal loss of motion at the extremes of internal rotation and abduction.At this stage there usually is no flexion contracture.Loss of hip ROM in patients with early Perthes’ disease without intra-articular incongruity is due to pain and muscle spasm. [10]

This is why, if the child is examined for instance after a night of bed rest, the range will be much better then later in the day.

Further into the disease process;Children with mild disease may maintain a minimal loss of motion at the extremes only and thereafter regain full mobility.Those with more severe disease will progressively lose motion, in particular abduction and internal rotation. Late cases may have adduction contractures and very limited rotation, but the range of flexion and extension is only seldom compromised.

3. Pain: [4]

Pain occurs during the acute disease.[1] The pain may be located in the groin, anterior hip area, or around the greater trochanter. Referral of pain to the knee is common.

4. Atrophy: [4]

In most cases there is atrophy of the gluteus, quadriceps[11] and hamstring muscles, depending upon the severity and duration of the disorder.

PHYSIOTHERAPY MANAGEMENT There is no consensus concerning the possible

benefits of physiotherapy in LCP disease, or in which phase of the development of the health problem it should be used.

Some studies mention physiotherapy as a pre- and/or postoperative intervention, while others consider it a form of conservative treatment associated with other treatments, such as skeletal traction, orthesis, and plaster cast.

In studies comparing different treatments[12], physiotherapy was applied in children with a mild course of the disease.

The characteristics of the patients were: Children with less than 50% femoral head necrosis

(Catterall groups 1 or 2) [12]

Children with more than 50% femoral head necrosis, under six years, whose femoral head cover is good (>80%)[12]

Herring type A or B[13]

Salter Thompson type A[13]

For patients with a mild course, physiotherapy can produce improvement in articular range of motion, muscular strength and articular dysfunction.

The physiotherapeutic treatment included: Passive mobilisations for musculature stretching of the involved

hip. Straight leg raise exercises, to strengthen the musculature of the

hip involved for the flexion, extension, abduction, and adduction of muscles of the hip.

They started with isometric exercises and after eight session, isometric exercises.

A balance training initially on stable terrain, and later on unstable terrain. 

For children over 6 years at diagnosis with more than 50% of femoral head necrosis, proximal femoral varus osteomy gave a significantly better outcome than orthosis and physiotherapy[12].

Goals of PT Rx

The main goals of conservative management of LCPD are to promote and optimize range of motion, strength, and joint preservation to minimize impairments and maximize function.

Containment of the femoral head in the acetabulum is the most important focus during each stage of LCPD.

Relieving Pain• Hot Pack for pain management with stretching2

• Cryotherapy for 20min at a time2

Increasing Strength• Hip abductors• Hip flexors• Hip extensors• Hip internal & external rotators• Gluteus medius

**Avoid single limb stance activities due to increased force through hip joint during severe involvement stage

Improving Range of Motion**ROM is typically lost in this population due to muscle guarding secondary to pain from incongruent joint surfaces

• Static Stretching of LE musculature• Dynamic ROM and AAROM for muscle guarding due to pain and if unable to achieve through static stretching• AROM & AAROM following passive stretching to maintain newly gained ROM• Stretching should include the following muscle groups: hip internal and external rotators, hip abductors, hip extensors, and any other LE motion that is significantly limited.

Functional Activities• Single leg dynamic function activities such as side steps and step ups, according to WB status and involvement phase

Home Exercise/Modality Use• Cryotherapy for 20min at a time may be used to decrease pain and inflammation• The HEP should be based on orthopedist recommendations due to the staging of the disease, age of onset of the disease, radiographic data, and patient presentation

Post-Surgical Physical Therapy - There is no evidence on the best post-op physical therapy interventions following femoral osteotomy in children with LCPD• The child will most likely be non-weight bearing approximately 6-8 weeks3

• During the non-weight bearing phase AROM of the joints above and below the surgical site should be done to prevent stiffness and undue weakness3

• Once the cast is removed, AAROM, AROM, and resistive exercises should be done to restore strength lost from immobility3

• Static stretches held for 30 sec+ and contract-relax stretches should be done every day followed by AROM exercises to restore and maintain full ROM

References http://www.physio-pedia.com/Legg-Calve-Perth

es_Disease Conservative Management of Legg-Calve-Perth

es Disease-pdf

Post-Operative Management of Legg-Calve-Perthes Disease-pdf

Evidence based guideline for LCPD from NGC