PRESENTER: Dr. ASHWANI PANCHALJSS MEDICAL COLLEGE

MYSORE

BLOOD SUPPLY TO THE FEMORAL HEAD:

At birth:

i) Vessels from lateral side

ii) Vessels from top of ossified shaft.

iii) No ligamentum teres.

4 months to 4 yrs:

i) Epiphyseal ossification begins.

ii) Ascending cervical branches.( metaphyseal and

lateral epiphyseal vessels)

iii) After 4 months metaphyseal branches

decrease.

4 to 7 yrs:i) Epiphyseal plate is firm barrier

between epiphysis and metaphysis.ii) Only source is lateral epiphyseal

arteries.

9-10 yrs:i) Ligamentum teres vessels becomes

prominent.ii) Anastomose with lateral epiphyseal

vessels.

Adoloscent period:i) Trochanter ossified, growth plate

extends beneath both epiphysis.ii) Majority from lateral epiphysis vessels.

Perthes disease may be defined as the “disease

of the hip, limited sharply by age group and

largely by sex, it results from changes in capital

femoral epiphysis, apparently secondary to loss

of an adequate blood supply for at least a

portion of head.”

Age group: 3-10 years

Sex: males 4-5 times more than girls

Bilateral in 10-12% of patients

SYNONYMS

Legg Calve-perthe’s disease

Legg’s stress fracture of femoral head

Osteochondritis deformans juvenalis

Osteochondrosis of hip joint

Pseudocoxalgia

Coxa plana

1. Vascular supply:

- Angiograms and laser studies have shown

medial circumflex artery is missing or obliterated

and obturator artery or the lateral epiphyseal

artery also affected.

2. Increased intra-articular pressure

3. Intraosseous pressure

- Patients has shown that the venous drainage in

the femoral head is impaired, causing an

increase in intraosseous pressure.

4. Coagulation disorder

- Associated with absence of factor C or S.

- Increase in serum levels of lipoproteins,thrombogenic substance.

5. Growth hormones

- Studies have shown reduced levels of growth hormones, somatomedin A and C.

6. Social conditions

- Usually belong to lower socioeconomic status, reflects dietary and environmental factors.

7. Trauma

8.. Abnormal growth and development

- Bone age is lower than chronological age by 1-3 yrs,.

Ex: carpal bone age: 2 yrs (Triquetral and lunate)

- Usually shorter than their peers.

9. Genetic factors

- Inheritance 2-20%;inconsistent pattern.

- More Incidence of low birth weight, abnormal birth

presentations.

- First degree relatives have 35% more risk , 2nd and

3rd degree relatives are 4 times more prone for

perthes disease.

1. TRUETA’S HYPOTHESIS

- Age < 3 yrs: blood supply contributed by

metaphyseal and retinacular arteries.

- Age 4-8 yrs: Retinacular arteries which enters head

as lateral epiphyseal arteries gets compressed by

lateral rotation muscles.

Thus trueta postulates that solitary blood supply

during 4-8 yrs makes vulnerable for AVN of head.

After 8 yrs foveolar arteries of ligamentum terescontribute blood.

Hypothesis into 4 stages

1. Incipient or synovitis stage:

- Lasts for 1-3 weeks.

- Synovium swollen, hyperaemic and oedematous.

2. Stage of avascular necrosis

- Lasts for 6mo to 1 yr.

- involves portion of ossific nucleus or entire

nucleus.

- Bony architecture crushed into minute fragments

and compressed into compact mass.

- Gross appearance and contour remains same.

3. Stage of fragmentation or regeneration

- Lasts for 2-3 yrs

- Characterised by resorption of necrotic bone

and replacement by viable bone.

- Subchondral fractures results in necrotic bone.

4. Healed or residual stage

- Normal bone forms alongside with replacing

slowly resorbing bone.

- Newly formed bone is immature, assuming mushroom shaped contour.

1) Incipient stage or synovitisstage:

- Lasts for 1-3 weeks- Synovium hyperaemic,

swollen

2) Stage of avascular necrosis:- Dead trabecular bone- Collapsed trabeculae- Thickened articular cartilage

Physeal disruption- Cartilage extending from

the physis into the metaphysis

3) Fragmentation stage

- Invasion of vascular

granulation tissue

- New bone forming on old

trabeculae

- Woven new bone formation

4) HEALING STAGE

- Normal forming bone alongside replacing slowly resorbing bone.

- New bone, woven and lamellar

- Mushroom shaped contour.

- Soft tissues fibrotic, motion restricted.

- Return to normal architecture

Changes in greater trochanter

- Becomes strinkingly hypertropic and elevated

proximally.

- Growth discrepancy between femoral head and

neck with trochanter.

- Impairs power of pelvitrochantric muscles resulting

in abductor insufficiency.

1) An overabundance of fatty marrow,

2) Circumscribed osteolytic lesions with a sclerotic

border,

3) A wide growth plate with disarrayed ossification

and columns of unossified cartilage coursing

down into the metaphysis, and

4) Extension of the growth plate down the side of

the neck of the femur.

Onset is insidous and prolonged course.

Age group and sex preponderance:

Painless limp is the earliest symptom and gait

antalgic.

Pain in medial aspect of thigh and inner knee.

Aggravated by movement of hip, increased

walking and relieved on rest.

Stiffness.

PHYSICAL FINDINGS

- Short stature

- In early stages- Muscle spasm evident and restricted hip motion, especially in abduction.

- Proximal thigh and gluteal muscle atrophy.

- Child may hold extremity in slight flexion and abduction, tenderness elicited over anterior aspect of joint.

- As disease progresses moderate amount of restricted motion, slight shortening and insignificant limp is present.

Waldenstrom observed that clinical course is

variable.

He divided into 4 stages

1. Patients age:

- Most consistent factor affecting course of the disease.

Age< 6 yrs: Mild disease

Age 6-9yrs: moderate symptoms.

Age > 9yrs: most severe.

2 . Disease severity:

- Varies from mild to severe, with most children

experiencing moderate symptoms for 12-18 months,

followed by complete resolution.

3. Extent of radiographic changes:

4. Outcome:

Hematological parameters

ESR

CRP

RA factor.

Coagulability profile.

X-rays

CT scan

MRI

Arthrography

Scintigraphy.

I) Stage 1(stage of increased density)

- Ossific nucleus initially smaller; femoral head becomes uniformly dense;

- Convex rounded enlargement develops at superior ,margin of neck( Gage’s sign).

- A subchondral fracture may be seen;

- radiolucencies appear in the metaphysis

II) Stage 2(fragmentation stage)

- Lucency appear in epiphysis;

- Segments (pillars) of the femoral head demarcate the femoral head may flatten and widen;

- Metaphyseal changes resolve;

- Acetabular contour may change

III) Stage 3(healing or reossification stage)

- New bone appears in femoral head which gradually

reossifies;

- Epiphysis becomes homogeneous.

IV) Stage 4( healed or remodelling stage)

- Femoral head is fully reossified and remodels to

maturity;

- Acetabulum also remodels

Radiographic changes in metaphysis.

- Apparent very early in the disease process.

- Changes are of prognostic changes, hips with

cystic changes were twice likely to have poor

outcomes as hips without cysts.

Sagging rope sign

Changes in neck of femur

- Deformity in neck can develop earlier than head.

- Upper part of neck is expanded and metaphyseal

end becomes rounded.

- neck progressively becomes shorter and wider.

Changes in acetabular cavity

- Distance between medial pole of head and floor

of socket is increased(Waldenstrom’s sign)

- Ligamentum teres grossly swollen and congested.

Changes in acetabular cavity

- Floor is altered to adapt shape of head, hollowed

out abruptly.

- There may be irregular ossification, cystic and

increased radiodense areas.

Effective means of diagnosing perthes disease in

its early stages much before radiographic changes

are apparent.

It provides more accurate information about the

extent of necrosis, reveals revascularization and

the stage of the disease.

Scintigraphy also used to classify revascularisation

as either recanalization or neovascularization.

Classified into two pathways

A track indicates uncomplicated revascularisation of

femoral head,(Caterrall score 2.4), good prognosis.

Track B represents slower rate of revascularisation

and healing(caterrall score of 3.5) poor prognosis.

It provides accurate three- dimensional images of

femoral head and acetabulum.

Classified into three types

Group A

Group B

Group C

CT is of benefit in later stages of the disease to

evaluate pain, locking of joint and other

mechanical symptoms

Usg used in early stages of perthes disease to

demonstrate joint effusion and in later stages to

assess shape of femoral head

A four stage classification

It has a role in evaluation of blood flow in femoral

head.

USG with microbubble contrast enhancement

used to evaluate vascularisation of femoral head.

Demonstrates actual contour of femoral head and

state of congruity of articular surfaces.

It provides reliable information regarding

containment of femoral head within acetabulum.

Major advantage is that examiner can assess

congruity of hip in different positions.

Often used in early diagnosis of hinge abduction

of hip.

Useful in fragmentation and reparative stages.

Accurate imaging modality for early diagnosis of perthes disease.

Evaluated congruity of articular surfaces, femoral head containment, joint effusion and synovial hypertrophy.

Epiphyseal involvement clearly visualised on MRI 3 to 8 months after first symptoms.

Diagnostic accuracy: 97-99%.

Also provides earlier and reliable information on revascularisation and extent of femoral head necrosis.

AVN caused by variety of conditions

- Sickle cell anemia

- Other hemoglobinopathies

- Thalassemia

- Steroid medication

- After traumatic hip dislocation

- Treatment of developmental dysplasia of hip

1) Transient synovitis

2) Slipped femoral epiphysis

3) Congenital dysplasia of hip

4) Congenital coxa vara

5) Early Tuberculosis

6) Rheumatoid arthritis

EPIPHYSEAL DYSPLASIAS

- Multiple epiphyseal dysplasia

- Sponyloepiphyseal dysplasia

- Mucopolysaccharidosis

- Hypothyroidism

Comparison chart

PERTHES DISEASE EPIPHYSEAL DYSPLASIA

Unilateral Bilateral involvement

If B/L, marked asymmetry, disease in differing stages and severity

Symmetrical findings

No involvement of other joints Involvement of other joints or spine.

Acetabulum not involved Involved

Sclerotic and cystic changes in femoral head and cystic changes in metaphysis

Few sclerotic changes in femoral head.

More tendency towards lateral calcification and subluxation

Little tendency.

POOR IF,1) Extensive involvement of EOC2) More than 6yrs of age.3) Early closure of epiphyseal plate4) Advanced stage of disease when first seen.5) Female patient.6) Calcification lateral to epiphysis7) Horizontal epiphyseal line.

Short term prognosis: concerns femoral head deformity at completion of healing stage.

Long term prognosis: concerns with late development of secondary degenerative OA of hip in adult life.

GOALS OF TREATMENT

1) Elimination of hip irritability

2) Restoration and maintenance of good range of

hip motion.

3) Prevention of epiphyseal extrusion and

subluxation.( containment).

4) Attainment of spherical femoral head on healing.

- Lateral pillar classification coupled with age of

onset provides useful information regarding

prognosis and treatment modalities.

Observation1) Onset <6yrs of age, regardless of extent of capital

femoral epiphyseal involvement.

2) Age<6yrs of age: Catterall’s group 1 and 2. or

Salter thomson group A.

3) They should have clinical and radiographic

examination at frequent intervals( 3 months)

4) If unsuccessful, may necessaite a short course (2-

6 months) of non surgical treatment.

The two primary means of symptomatic treatment are bed rest and traction.

NSAIDS and crutches Stretching exercises with observation

used. Beneficial effects are greatest

around time of development of subchondral fracture.

Various traction methods include simple longitudinal traction with leg on bed, balanced suspension and traction and “slings and springs”.

Hip irritability with decrease of hip motion:

1-2 week period of bed rest with abduction traction

if recurs

2-3 months period of surgical non containment to

decrease risk of extrusion.

X-ray taken bi-monthly for evaluation.

Indications

Age at clinical onset 6yrs or older.

Catterall Group 3 or 4/ Salter thomson Group B.

When loss of containment manifested by extrusion seen on AP view.

Contraindications

Group 1,2,3 cases less than 5 yrs, with no signs of head at risk.

Severe flattening of head

Healed cases and cases with hinged abduction.

Use of orthosis

1. Atlanta Scottish Rite Brace.:Post. Coverage

2. Toronto Brace of Bobechko

3. Hughston A frame

4. Broom stick plaster

5. Petrie and Bitenc Abduction cast: ant & lat.

coverage

6. Newington Abduction

7. Ambulation Brace.

Containment index used for evaluation.

Toronto braceNewington brace incorporates a metal A-

frame with a central support for the thighs.

Preliminary traction given

Extremity placed in brace (abd: 45 & int. rot.)

Child encouraged to walk because weight bearing movements are essential for successful remodelling

X-rays taken at regular intervals

Discontinued when evidence of new subchondral bone seen(20 months)

Broom stick plaster and abduction cast by Patric

and Bitenc consists of long leg casts with 30-40

degrees of abduction and 5 of internal rotation.

Disadvantages:

I) Stiffness of knee and ankle.

II) Restricted ambulation

III) Pressure sores

IV) Need for frequent changes.

Femoral osteotomy has been used to contain

femoral head with perthes disease.

Better results with lateral pillar B/C border hips, in

children more than 8 yrs of age

Indications:

- Age more than 6 yrs.

- Head at risk signs on radiography

- Failed conservative methods

Pre operative measures:

Age at surgery:

- Should be done in the increased density or

early fragmentation phase.

- Very early or late surgeries have led to

premature closure of epiphysis.

Trochantric epiphysiodesis.

Advantages

- Ability to obtain containment of head which

enhance remodelling.

- Period of restriction is for 2 months.

- No end point is necessary for treatment as the containment is permanent

First performed by Salter (‘62)

Indications:

Onset < 6 yrs

Moderately or severly affected head.

Loss of containment

Pre- op requistes:

Minimum deformity of femoral head

Non irritable hip

No significant restriction of hip motion

Advantages:

- Anterolateral coverage of femoral head.

- Lengthening of extremity

- Avoidance of plate removal

Disadvantages:

- Inability to obtain proper containment of femoral head

- Increase in acetabular and hip joint pressure that may cause furher necrosis.

- Increase in leg length

- Relative adduction of hip and uncovert femoral head

Willett recommenned first for older children

because of insufficient remodelling capacity, and

likelihood that shortening femur would cause persistent limp.

i)Curved incision below iliac crest, strip glutei.ii) Mobilize and divide reflected head of rectus femoris

iii) Trough in bone above insertion of capsule.iv) Strips of cancellous bone inserted into trough so that they form a

canopy on superior surface of hip joint.v) Pack web space between flap and graft canopy with gratft

vi)Repair rectus and lose the wound.

Advocated by Axer, Craig et al.

Advantages:

- Ability to obtain maximal coverage of femoral head.

- Ability to correct excessive femoral anteversion.

Disadvantages:

- Excessive varus angulation that may not correct with growth.

- Shortening of already shortened limb

- gluteal lurch

- Non-union, implant removal, premature closure of epiphysis.

Procedure of choice

- When containment is necessary and cant

be achieved by brace.

- Child of 8-10yrs old, without leg-length

discrepancy.

- On arthrogram,MRI femoral head is uncovered

- When there is significant amount of femoral

anteversion.

Level of osteotomy

Insertion of guide pin and reaming of femur

First depth marking flush with lateral cortex

Removal of wedge to customize it

Plate and compression screw application

Insertion of bone screws.

I) LATERAL WEDGE OSTEOTOMY

Axer developed for children of 5 yrs and younger,

prebent plate is used.

II)OPENING WEDGE OSTEOTOMY

III)REVERSED WEDGE OSTEOTOMY

Rationale is that distraction of joint widens and

unloads joint space and reduces femoral head,

allows repair of articular cartilage.

It preserves congruency of femoral head.

Allows 50 degrees of flexion.

VALGUS EXTENSION OSTEOTOMY:

- used mainly in patients with hinge abduction.

- It is an abnormal movement occurs when the deformed femoral head fails to slide within the acetabulum

VALGUS FLEXION INTERNAL ROTATION OSTEOTOMY

- Kim et al. in 3D CT observed “Functional retroversion”.

- Recommended valgus flexion internal rotation femoral osteotomy plus acetabuloplasty.

Advantages:

1) Corrects functional coxa vera

2) Establishes normal articulation between posteromedial portion of head and acetabulum.

3) Corrects external rotation deformity.

4) Improves joint congruity and head coverage

CHEILECTOMY

- Ocassionally child is left with malformed femoral

head, large mushroom shaped/ lateral

protuberance.

- Erard and Dvaric observed good results at short

term follow up, deteriorated with time, and

increased pain after 2-4yrs of surgery.

Pre op evaluation:

- determine whether protrubence is anterior or

posterior.

Lateral incision

Muscle seperated, nerve

secured.

Capsule cut, protuberance

exposed

Osteotome directed away

from lateral edge of

proximal femur physis.

Excised.

Check range of motion.

CHIARI OSTEOTOMY

- Used as salvage procedure to accomplish

coverage of large flattened head, in older children

when head is subluxating and painful.

- Osteotomy of pelvis performed at superior margin

of acetabulum

- Pelvis inferior to osteotomy along with femur

displaced medially.

- Superior fragment then becomes shelf and

capsule interposed between it and femoral head.

TROCHANTRIC OVERGROWTH

Causes

Result

Consequence:

- Elevation of trochanter decreases tension and

mechanical efficiency of pelvic and trochantric

muscles.

- Shortened femoral neck moves trochanter closer to

centre of rotation of hip, line of pull of muscles

becomes more vertical.

- Impingement of head to the roof limiting abduction.

Manicol and Makris described ‘Gear stick sign’

of trochantric impingement that is used for pre op

evaluation.

Sign is based on observation that hip abductor is

limited by impingement of greater trochanter on

ilium when the hip is extended but full abduction is possible when hip fully flexed

Normal growth pattern

Long. Growth arrested, greater trochanter continues

Muscle release and abduction casts

- If healing femoral head is moderately flattened,

an AP view reveals extruded anterolateral portion

of head impinging on acetabulum.

- Adductor tenotomy, iliopsoas release and

arthrotomy of hip joint can be done.

- Later Patric casts for 3-4 months usually permits

remodelling.

Two types:

1)CAM Type (FAI).

- Occurs because of abnormal head with

increased radius at the base of neck, causes

abutting the acetabulum during extreme flexion.

- Causes acetabular cartilage abrasion or avulsion

and detachment of labrum.

- Coxa magna with large head are especially

prone.

2) PINCER TYPE FAI

- Occurs due to direct contact between acetabular

rim and head neck junction.

- Labrum is first structure to get injured and may

become hypertropied / ossified.

- Pincer type is usually not the primary type.

Symptoms

1) Pain,

2) Arthritis.

MANAGEMENT.(surgical)

Indications.

Patient mainly complains groin pain worse on

flexion type activities.

Impingement sign. And its importance

Imaging studies:

- AP standing view,

- Lateral view of each pelvis.

On AP, underlying pathologic conditions and

acetabular version to be identified.

Cross over sign:

Intersection of anterior wall

and posterior wall medial to

lateral edge of acetabulum.

Posterior wall sign:

Posterior wall projects more

medially than the centre of

hip.

Surgical treatment.

- Surgical hip dislocation described by Ganz.

- Treatment involves removal of any non spherical

portion of head to provide greater clearance

during motion.

- Pincer type FAI needs temporary detachment of

central portion of labrum followed by recession of

anterior of acetabulum using osteotome.

3 STAGES1) Good:

- Hip asymptomatic, good ROM- Head round and centered, no acetabular change.

2) Fair:- Hip asymptomatic, ROM slightly restricted.-Femoral head round with slight broadening.- One fifth of head uncovered, some acetabular changes accepted.

3) Poor:- Hip symptomatic, motion restricted,- Head flat ,broad.- Gross acetabular changes, joint space widened at the inferior medial aspect

MEASUREMENT OF SPHERICITY OF

FEMORAL HEAD

- Obtained by transparent templates on which

inscribed series of 28concentric circles 2mm

apart.

- By this, deviation from circularity can be

measured in each projection.

- Acceptable when

i) NO deviation, radii equal, head spherical

ii) Radii<2mm, other parameters normal.

2 LINES

i) Vertical line passing through centre of head©

ii) CE line from centre of head to the edge of acetabulum

CE angle <20 degrees on weight bearing AP indicates lateralisaton or subluxation.

GRADING BY MOSE

1) Good: Femoral head spherical and same radius on

AP and lateral view.

- CE angle 20degrees or more.

2) Fair:

-NO more than 2mm deviation from sphericity on AP

and lateral view and CE angle of 15-19 degrees

3) Poor:

- Greater than 2mm variation from sphericity on either

AP or lateral view and CE angle less than 15degrees.

References

1) Tachdjian’s pediatric orthopaedics

2) Cambell’s operative orthopaedics

3) Hefti’s pediatric orthopaedics

4) Mercer’s orthopaedics

5) Turek’s orthopaedics

6) Gray’s anatomy

THANK YOU