perthes disease lcpd

Dr. ANOOP G.C. Junior Resident in orthopedics

MCH Kozhikkode

Legg Calve Perthes Disease

Dr.Anoop G.C.,JR,Orthopaedics,GMCK

SYNONYMS

Coxa plana

Osteochondritis deformans coxa juveniles

Pseudocoxalgia

Osteochondrosis of hip joint

Childhood Aseptic Necrosis of Femoral Head

Osteochondritis dessicans of Hip

Legg’s stress fracture


DEFINITION

• PERTHES DISEASE : is a self-limiting form of osteochondrosis of the femoral capital epiphysis

• of unknown etiology that develops in children commonly between the ages of 4 – 12 years

• caused by impaired circulation in the femoral head

• necrosis of the femoral epiphysis and its replacement by new bone

• resulting in deformation of the femoral head.


HISTORY Described first by Waldenstrom in 1909

who mistakenly ascribed it to tuberculosis.

In 1910 was independently described by

Arthur Legg , U. S. A - February

Jacques Calve , France - July

George Perthes ,Germany - October

Hence name – “Legg Calve Perthes Disease”

In 1922 Waldenstrom gave the correct

interpretation and described the stages .


LEGG CALVE PERTHES


ETIOLOGICAL FACTORS - that play a

role in development of illness

Vascular supply

Increased intra-articular pressure

Intraosseous pressure

Coagulation disorder

Growth hormones

Skeletal Growth

Social conditions

Genetic factors

Attention deficit Disorders

TRAUMA


FEMORAL HEAD - vascular supply

EXTRINSIC OR EXTRAOSSEOUS ANATOMY (Crock & Chung)

1. Extra capsular ring – base of neck - Formed by Medial circumflex femoral (Major)and Lateral

circumflex femoral arteries - Branches ascend in 4 groups as Ascending cervical(Extra

capsular) or Retinacular(intra capsular) arteries

- Lateral group most important. - The Lateral group pierce the capsule and enter epiphysis to

become Lateral epiphyseal arteries

2. Intra capsular ring – base of head - incomplete in 57% males and 31% females. - Formed by branches of ascending cervical & retinacular

arteries. - They contribute to lateral epiphyseal arteries

3. Artery of Ligamentum teres Dr.Anoop G.C.,JR,Orthopaedics,GMCK


INTRINSIC OR INTRAOSSEOUS ANATOMY

(Trueta & Harrison)

1. Lateral epiphyseal Arteries

- Formed by the Lateral group of Ascending cervical or

Retinacular arteries

- Supplies 2/3 rd of femoral head

- Exclusive supply of suprolateral head

2. Medial epiphyseal or Artery of Ligamentum teres

- Negligible to ½ of epipysis

3. Metaphyseal arteries



ADULT Dr.Anoop G.C.,JR,Orthopaedics,GMCK

TRUETA’S HYPOTHESIS • Infants and children <4 yrs : 2 arterial

blood supplies to femoral head- the retinacular or ascending cervical arteries (Lateral epiphyseal artery) & the metaphyseal arteries

• 4-12 yrs : single areterial supply by Lateral epiphyseal artery. This solitary blood supply makes this age period vulnerable to ischaemic necrosis due to compression by external rotators and extreme movements.

• After 12 yrs: foveolar arteries of Ligamentum teres contribute blood along with Lateral epiphyseal arteries & incidence decreases. (In blacks it occurs earlier)


TRUETA’S HYPOTHESIS In adults :cartilage growth plate disappears

& the metaphyseal arteries enter the epiphysis and thus the adult pattern of blood supply by

• Foveolar

• Retinacular

• Metaphyseal arteries occurs .

Hence the disease doesnot occur in adults & rare after 12 year.

Supply by ligamentum teres occurs earlier in blacks - disease is rare in blacks


FEMORAL HEAD - The Blood Supply of

the Immature Femoral Head

CHILD

Metaphyseal or no

other vessels cross

epiphyseal plate

Artery of

Ligamentum teres

is not developed

Solely dependent

on Lateral

epiphyeal arteries


CAFFEY’S HYPOTHESIS

Avascular necrosis of femoral head

results from intraepiphyseal

compression of blood supply to the

ossification center and not due to

external compression of vessel.


ETIOLOGICAL FACTORS

Vascular : General reduction in blood flow with a significant reduction in medial circumflex artery

Normally venous drainage occurs through medial circumflex vein

In Perthes disease increased venous pressure in affected neck associated venous congestion in metaphysis


ETIOLOGICAL FACTORS

Increased intra-articular pressure : – Animal experiments have shown that an

ischemia similar to that in Perthes disease can be generated by increasing the intra-articular pressure.

– However, the condition of transient synovitis of the hip does not appear to be a precursor stage of Perthes disease as the increased pressure resulting from the effusion in transient synovitis does not lead to vessel closure


ETIOLOGICAL FACTORS

Intraosseous pressure: – The measurement of intraosseous pressure in Perthes

patients has shown that the venous drainage in the femoral head is impaired, causing an increase in intraosseous pressure.

Coagulation disorder : • Study have found a coagulation disorder in 75%

children with Perthes disease.

• In most cases the disorder was thrombophilia.

• protein C or protein S deficiency, elevated serum lipoprotein, Factor-V Leiden mutation, Anticardiolipin antibodies were also noted.


ETIOLOGICAL FACTORS Growth hormones :

– While earlier studies found reduced levels of the growth hormone.

– Recent studies have not shown any difference from control groups in respect of hormone status

Skeletal Growth:

– Children with Perthes disease are shorter, on average, than their peers of the same age & show a retarded skeletal age (cartilaginous dysplasia).

– The maturation disorder occurs between the ages of 3 and 5 years.

– Both the trunk and extremities lag behind in terms of growth.

– But pick up to attain normal skeletal maturity by adulthood.


ETIOLOGICAL FACTORS

Social conditions:

– Studies in the UK have shown that Perthes disease is more common in the lower social status.

– The authors suggest a poorer diet during pregnancy as one possible explanation for this phenomenon.

– A recent study did not confirm this theory

Genetic factors:

– Studies have shown that first degree relatives of children with Perthes disease are 35 times more likely to suffer from the condition than the normal population.

– Even second- and third-degree relatives show a fourfold increased risk.


ETIOLOGICAL FACTORS

Attention deficit Hyperkinetic Disorders : more prone to trauma

Synovitis : predisposed due to increased intra articular pressure

TRAUMA :

– Trauma in the predisposed child ppts AVN OF FEMORAL HEAD AND development of Perthes disease

– the lateral epiphyseal artery which courses

through a narrow passage is susceptible to damage


HERIDITARY ASSOCIATIONS

Congenital abnormalities Hemivertebrae Deafnes Imperforate anus Pyloric stenosis Epilepsy

Cong heart disease

Short tibia

Undescended testis


PATHOLOGY

By Waldenstrom in 1922

4 stages

based on microscopic and gross pathology

Paul_Petter_Waldenström Dr.Anoop G.C.,JR,Orthopaedics,GMCK

Stages

Stage 1 : Incipient or synovitis stage

• Lasts for 1-3 week

• synovium is swollen edematous and hyperemic

• joint fluid is increased

• Inflammatory cell are notably absent


Stages Stage 2 : Avascular necrosis

• Lasts for 6 month to 1year

• Significant necrosis of bone

• trabeculae are crushed into minute fragments.

• Absent/pyknotic nuclei in the osteocytes

• No evidence of bone regeneration

• Degenerative changes in the basal layer of

articular cartilage

• Thickened peripheral cartilagenois cells

• Gross contour of femoral head is unchanged


Stages

Stage 3 : Fragmentation or Regeneration

– Lasts for 2- 3 year.

– Dead bone infested with vascular connective tissue was actively resorbed by osteoclasts and replaced by newly formed immature bone.

– Loss of epiphyseal height due to collapse of bony trabeculae and resorption of fragmented necrotic bone


Stages

Stage 4 : Healed or Residual Stage

• Normal bone starts replacing necrotic bone.

• Ossific nucleus is deformed assuming mushroom contour

• Femoral head enlarges, flattens and subluxate.


EPIDEMIOLOGY

• ONSET : 18 months - skeletal maturity

• Most prevalent : 4-12 yrs

• Male: female : 4 : 1

• Bilateral in 10 - 20%

• No evidence of inheritance but 35 time risk in 1st degree relative.


CLINICAL FEATURE - Symptoms

Described by Sundt in 1920 – limp-exacerbated by activity and relieved by rest – Pain-located in

• Groin • Anterior hip region • Laterally around greater trochanter • Referred pain to knee (may obscure the true

nature of the disorder) • Aggravated by physical activity • Night pain

– H/o of antecedent trauma – Waxing & waning of symptoms


CLINICAL FEATURE - Signs

Typical limp : combination of antalgic and Trendelenburg gait

Signs of old injuries - scars, old fractures

Reduction of hip motion( muscle spasm)

– Minimal noted at abduction & internal rotation(earlier)

– Greater loss of motion with more severe disease

– Abduction contractures

– Lose all rotations in very severe cases

– FLEXION/EXTENSION SELDOM AFFECTED


CLINICAL FEATURE - Signs

Wasting of gluteus, quadriceps & hamstrings

Positive Trendelenburg on the involved side

Classicaly :

A small

Thin

Extremely active

Constantly running and jumping child

Who develops limping after strenuous physical

activities


NATURAL HISTORY

DISEASE SEVERITY:

Mild to severe

Majority will have moderate symptoms for 12- 18 months followed by complete resolution of symptom

Finally return to normal activities

PATIENT’S AGE : MOST CONSISTENT FACTOR AFFECTING COURSE OF THE DISEASE

– Early onset (before 6 yrs) : mild

– Onset 6-9 yrs :moderate

– Late Onset after 9 yrs: most severe course and worst outcome


NATURAL HISTORY

EXTENT OF RADIOGRAPHIC CHANGES :

Poorest results seen in hips with the

greatest degree of involvement

OUTCOME: affected by the duration from

onset of disease to complete resolution

The shorter the duration the better the

outcome


ASSOCIATION BETWEEN CLINICAL

FINDINGS & STAGES

Stage 1 – Initial stage

Waxing/waning of symptoms & signs

Mild limp and pain

Episodes of moderate discomfort(weeks)

Stage 2 – Fragmentation stage

Limp and pain more

Greater loss of range of motion


ASSOCIATION BETWEEN CLINICAL

FINDINGS & STAGES

Stage 3 – Reossification/healing stage

Pain /limp resolves

Mild limitation of joint motion

Resumes normal activities

Stage 4 – Residual/healed stage

Symptoms disappear as head completely reossifies


PROGNOSIS – poor in

• Female child

• Obese child

• Age of Onset > 8 years

• Adduction contractures

• Progressive decrease in range of movements

• Presence of “Head at risk Sign’s” on Radiographs


DIFFERENTIAL DIAGNOIS

• Transient synovitis • Slipped femoral epiphysis • Congenital dysplasia of hip • Congenital coxa vara • Early Tuberculosis • Rheumatoid arthritisAcute and chronic sepsis • Acetabular dysplasia • Epiphyseal dysplasia • Histiocytosis • Gaucher’s disease


INVESTIGATIONS

• Bloods – FBC, ESR, CRP, Blood Culture – r/o other diseases.

• Radiographs

• Ultrasound Scan

• CT scan

• Bone Scan – Decreased bone scan uptake before radiographic changes

• MRI

–Earlier diagnosis than plain radiography – More information regarding extent of necrosis than

bone scanning

• Arthrography


IMAGING STUDIES

Radiographic staging of disease evolution:

- Based on Anteroposterior and Lateral frog leg views

- the modified version of WALDENSTROM’S classification

4 STAGES

1. Initial stage

2. Fragmentation stage

3. Reossification stage

4. Residual stage


RADIOGRAPHY

AP View FROG LEG View


Intial stage (6 months)

Lateralization of femoral head in the acetabulum

Smaller ossific nucleus due to cessation of growth of the capital epiphysis

Apparent widening of medial joint space (synovitis & hypertrophy of articular cartilage)

WALDENSTROM’S SIGN : Linear fracture in the subchondral area of femoral head ( frog leg lateral view)

Increased density of femoral head : 20 accumulation of new bone on the dead bone trabeculae in the head .

Metaphyseal cysts & lucencies


Fragmentation stage (8 months)

Lucencies develop in the ossific nucleus/ other

sections remain sclerotic.

Central dense fragment gets demarcated from the

medial & lateral segments of the head

Increased density resolves

acetabular contour more irregular

End of this stage is marked by appearance of

new bone in the subchondral area of femoral head


Reossification stage / healing stage

(51 months) Starts in the Centre of the femoral head and

expands medially and laterally

Last areas to reossify

- anterior segment of head

- Centre of head

Lucent portions of femoral head fill in with woven bone

Over the time the new bone remodels into trabecular bone

Head regains roundness


Residual stage

Head fully reossified

Remodeling of head continues until

skeletal maturity when the permanent

contour is established

Acetabulum remodels as well


CHANGES IN THE METAPHYSIS

Gill (1940) reported the changes as “HOLES OF DECALCIFICATION” due to metaphyseal necrosis

Metaphyseal Cyts :Ponseti described cystic changes caused by tongues of fibrillated cartilage stretching deep into the neck

Sagging Rope Sign

Radiodense line overlying the proximal femoral metaphysis

Produced by growth plate damage associated with metaphyseal response.


CHANGES IN THE METAPHYSIS

Metaphyseal cyst

Sagging rope sign Dr.Anoop G.C.,JR,Orthopaedics,GMCK

CHANGES IN PHYSIS

Abnormal growth of proximal femoral physis

Premature physeal closure(25% cases) causing

Lateral extrusion of capital nucleus

Medial bowing of femoral neck

Greater trochanter overgrowth

lateral X-ray showed a bulge in the metaphysis (a step shaped irregularity) comparable to changes observed in Blount’s disease


CHANGES IN THE ACETABULUM

BICOMPARTMENTALIZATION :When the femoral head protrudes from the acetabulum , the medial wall may form what looks like a second compartment for the head (Yngve and Roberts)

by early closure of triradiate cartilage

its an indicator of poor out come

Resolves during the healing stage

Osteoporosis of the roof of the acetabulum

Position of the head rather than it’s shape has been the most significant factor in the growth & remodeling of acetabulum


CHANGES IN THE ACETABULUM

BICOMPARTMENTALIZATION Dr.Anoop G.C.,JR,Orthopaedics,GMCK

ULTRASONOGRAPHY

To demonstrate joint effusion

Provide a good profile of cartilaginous

femoral head and subsequent deformation

of head can be assessed


CT SCAN

3D Images of head & acetabulum

Useful in later stages

– To evaluate pain

– Locking of joint

– Mechanical symptoms


BONE SCAN

Effective means of diagnosis in early stages, before associated radiographic findings are apparent

To classify the severity of disease

Reveals revascularization and consequently the stage of the disease

To classify revascularization as either recanalization or neovascularization


MRI

Accurate imaging modality for early diagnosis

Visualization of configuration of the femoral head and acetabulum

Determine the extent of revascularization

Epiphyseal involvement more clearly visualized

Earlier and reliable information about the true extent of femoral head necrosis

Finding the degree of involvement during the early phases of this disorder


ARTHROGRAPHY Provides reliable information regarding

containment of femoral head within the acetabulum

Examiner can assess the congruity of the hip in many different position.

Most often used in the early diagnosis of HINGE ABDUCTION OF HIP in which the head hinges out of acetabulum when the hip is abducted – It occurs early in the course of the disease

– The longer it remains untreated ,the worst is the outcome .

– Treatment :traction initially to relieve hinging and later surgery to contain the head .


ARTHROGRAPHY


RADIOLOGICAL CLASSIFICATION

• AP and FROG LEG views required

• Depending on Extent of lesions

• Important in deciding treatment method.

• 3 classifications are

– Catterall

– Salter & Thompson

– Herring


Catterall

• Most common

• Based on extent of femoral head lesion

• IV groups


Catterall Group I

25% involvement

No metaphyseal Reaction

No sequestrum

No subchondral fracture line Dr.Anoop G.C.,JR,Orthopaedics,GMCK

Catterall Group II

50% involvement

Sequestrum present - junction Clear

Metaphyseal reaction - antero lateral

Subchondral fracture line - anterior half Dr.Anoop G.C.,JR,Orthopaedics,GMCK

Catterall Group III

75% involvement

Sequestrum large - junction sclerotic

Metaphyseal reaction - diffuse - antro lateral area

Subchondral fracture line - posterior half Dr.Anoop G.C.,JR,Orthopaedics,GMCK

Catterall Group IV

Whole head involvement

Metaphyseal reaction - central or diffuse

Posterior remodelling Dr.Anoop G.C.,JR,Orthopaedics,GMCK

Catterall’s - Head at Risk Signs

• Lateral epiphyseal calcification

• Lateral subluxation

• Gage’s sign

• Cage sign

• Caffey’s or Salter Sign

• Metaphyseal cysts

• Horizontal growth plate


Lateral subluxation


GAGE’S SIGN

• small osteoporotic segment forming a translucent V- shaped trough in the lateral part of the epiphysis


CAGE SIGN

• Calcification of the lateral epiphysis


Salter’s or Caffey’s sign • a subchondral # may occur in the anterolateral

aspect of the femoral capital epiphysis. This produces a crescentic radiolucency known as the crescent, Salter’s or Caffey’s sign


Salter and Thompson • Extend of sub chondral fracture

• Subchondral fracture correlates with eventual extent of resorption

– GROUP A : Subchondral # involving <50% of the femoral dome

– GROUP B : Subchondral # involving >50% of the femoral dome


Herring

The femoral head pillars are

derived by noting the lines of

demarcation between the

central sequestrum and the

remainder of the epiphysis on

the anteroposterior radiograph


Herring

Group A Normal Height of lateral

pillar maintained


Herring

Group B > 50% of lateral pillar

height maintained


Herring

Group C < 50% of lateral pillar

height maintained


TREATMENT • AIM OF TREATMENT :

• The etiology of Perthes disease is unknown and consequently treatment is not in any way directed to achieving a cure.

• If it’s a elf limiting process of degeneration and then regeneration why do we need to treat it ?


AIM OF TREATMENT

• The long-term aim of treatment of Perthes’

disease is to prevent the onset of secondary degenerative arthritis of the hip.

• Several long-term studies have shown that loss of sphericity of the femoral head is the most important factor related to development of secondary degenerative arthritis of the hip.


GOALS OF TREATMENT • Elimination of hip irritability.

• Containment of the head.

• Restoration good ROM • Prevent the femoral head from getting

deformed or enlarged

• Prevent trochanteric overgrowth

• Ensure Psychological & Physical development


TREATMENT PROTOCOL

Observation only

Intermittent symptomatic treatment

Early definitive treatment

Conservative

surgical

Late reconstructive surgical


Observation only • All children < 6 years

– Irrespective of the extent of involvement of femoral capital epiphysis

– Provided there is no limitation of motion or subluxation

• All children > 6year – Under Caterll group I & II or Salter – Thompon

group A – Provided there is no limitation of motion or

subluxation or collape

• Do not require active management but require frequent evaluation clinically & Radiologically every 3 months.


Intermittent symptomatic treatment • All children under observation group

developing persistent loss of Motion or containment.

• Temporary or periodic treatment for 1 or 2 weeks with – Bed ret in abduction

– Traction in abduction

– Physiotherapy

• Once motion is regained and irritability subsides child can resume normal activities

• Bi monthly evaluation is required. Dr.Anoop G.C.,JR,Orthopaedics,GMCK

Early definitive treatment • All children in intermittent symptomatic

group with 2 – 3 recurrent episodes of irritability.

• All children > 6 years under Caterall group III & IV or Salter – Thompson group B.

• Any child with severe loss of motion or evidence of Extrusion.

• Contraindicated in severe flattening of head , healed cases and hinged acetabulum.

• Principle is CONTAINMENT of femora head in acetabulum – Non surgical or surgical.


CONTAINMENT - Non surgical

• Braces & orthoses – Preliminary traction is applied to overcome muscle

spasm while the hip is gradually abducted and internally rotated

– Brace are applied with lower limbs in approximately 45 degree abduction and light internal rotation.

– Walking is encouraged since weight bearing movement are evential to remodeling.

– Bed time exercises preferably done under water.

– Plastic abduction night splint

– Generally discontinued at 20 month or if evidence of new layer of subchondral bone in radiograph


Braces and orthoses

• An

TORONTO BRACE NEWINGTON BRACE


Braces and orthoses

TACHDJIAN BRACE

BIRMINGHAM BRACE


Braces and orthoses

Snyder sling Patten botom brace Dr.Anoop G.C.,JR,Orthopaedics,GMCK

Braces and orthoses

Ambulatory abduction orthosis Dr.Anoop G.C.,JR,Orthopaedics,GMCK

Plaster Casting

• Petrie or “Broomstick cast”

– By Patric and Bitenc.

– Long leg cats are applied to both extremities in 30 – 40 degree abduction and 5 degree internal rotation.

– And secured by two wooden bars.

• Disadvantages

– Knee and ankle stiffness with adaptive articular changes

– Restricted ambulation and pressure sores


Petrie or “Broomstick cast”


SURGICAL CONTAINMENT • INDICATIONS:

– Age of clinical onset > 8yrs of age

– Herring type B

– Radiological evidence of loss of containment by conservative modes

• CONTRAINDICATIONS: – Herring’s type A and C

– Herring’s type B if child less than 8 yrs

– Healed cases.

– Hinged abduction

• ADVANTAGES – Ability to obtain permanent containment of head. – Period of Restriction is only 2 months.


CONTAINMENT SURGERIES

– Varus Derotational Femoral Osteotomy

– Innominate or Salter osteotomy

–Shelf procedure

–Combination of femoral and

innominate osteotomy

–Combination of innominate

osteotomy and shelf


VARUS DEROTATION FEMORAL OSTEOTOMY (VDO)

• Initially advocated by Axer in 1965 • Procedure of choice in 8 – 10 yrs without limb

shortening • Uncovered head on MRI / Arthrogram • Excessive femoral anteversion • Types - Open wedge or closed wedge • Technique - Osteotomy at subtrochantric level & Distal femur is fixed in varus and external rotation using plate and screws • Hip spica for 8-12 weeks


VARUS DEROTATION FEMORAL OSTEOTOMY (VDO)


INNOMINATE OSTEOTOMY – SALTER • Initially advocated by Salter in 1966

• Advantages:

– Anterolateral coverage

– Lengthening of shortened limb

– No second operation for I/R

• Disadvantages:

– Improper coverage in older child

– Limb length inequality

– AVN due to raised pressure in joint

• Technique

– Iliac osteotomy is made just above acetabulum extending from greater sciatic notch to anterior inferior Iliac pine

– Entire acetabulum with pelvis is rotated downward and outwards

– Bone graft from ilium is applied to osteotomy site

• Hip Spica for 8-12 weeks.


INNOMINATE OSTEOTOMY – SALTER


SHELF PROCEDURE • Formerly used as a salvage procedure

• Catterall proposes this as the primary method of

management in children over 8 years of age

• INDICATION:

• Lateral subluxation

• Insufficient coverage

• Hinged abduction

• COMPLICATION:

• Loss of hip flexion

• lateral femoral cutaneous nerve injury

• Technique:

• bone graft is harvested from the ilium and

inserted into the roof of the acetabulum.


SHELF PROCEDURE


LATE RECONSTRUCTIVE SURGERIES • Done for Healed Perthes with permanent

deformities

• Valgus osteotomy - Hinged abduction

• Shelf acetabuloplasty - Coxa magna

• Garceau cheilectomy - Malformed head in catterall group 3

• Trochanteric advancement or arrest - Capital physeal arrest & trochantric overgrowth

• Chiari osteotomy– Significant femoral head flattening.


ASSESSMENT OF END RESUTLT

• Assessment of end result is done at 4 years after onset.

• Based on sphericity and containment of femoral head.

• Good – no arthritis develops

• Fair – mild to moderate arthritis will develop in late adulthood

• Poor – severe arthritis will develop before age of forty.



SPHERICITY OF HEAD

MOSE CLASSIFICATION: Based on fitting of contour of healed femoral head into template of concentric circles in both AP & Frog leg lateral views

• Good - < 1 mm • Fair - < 2 mm • Poor - > 2 mm



CONTAINMENT OF HEAD

CE Angle of Wiberg:

- A line is drawn from center of head C and edge of acetabulum E called CE line

- The angle between CE line and vertical through center of head is called the CE angle.

Good - >20

Fair- 15-19

Poor- < 15

E

C

Vertical


THANK YOU


perthes disease lcpd

Health & Medicine