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Page 1: Legg-Calve´-Perthes’ disease Prof. J. Sahoo

Legg-Calve´-Perthes’ Legg-Calve´-Perthes’ diseasedisease

Prof. J. Sahoo Prof. J. Sahoo

Page 2: Legg-Calve´-Perthes’ disease Prof. J. Sahoo

DeffinationDeffination Perthes’ disease is shelf limited condition Perthes’ disease is shelf limited condition

of capital femoral epiphysis due to lack of capital femoral epiphysis due to lack of adequate blood supply with sharp of adequate blood supply with sharp limitation of age as well as sex.limitation of age as well as sex.

Incidence:Incidence: Age 5-10 (3-12)Age 5-10 (3-12) Sex M:f (4:1)Sex M:f (4:1) Bilateral rare about 10%.Bilateral rare about 10%.

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Synonyms: Synonyms: CoxaplanaCoxaplanaOsteochondritis deformans Osteochondritis deformans

coxajuvenitiscoxajuvenitisPseudocoxalgiaPseudocoxalgia

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This condition was found out by three This condition was found out by three important Orthopedics surgeons important Orthopedics surgeons synchronouslysynchronously

Arthur LeggArthur Legg Jacques Calve’Jacques Calve’ George Perthes George Perthes

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BLOOD SUPPLY TO FEMORAL HEADBLOOD SUPPLY TO FEMORAL HEADAnatomyAnatomy• Medial and lateral femoral circumflex arteries.Medial and lateral femoral circumflex arteries.• Lateral ascending cervical artery- After Lateral ascending cervical artery- After

penetrating the lateral capsul in posterior penetrating the lateral capsul in posterior trochanteric fossa.trochanteric fossa.

• Intracapsular ring has been found to be Intracapsular ring has been found to be incomplete more often in boys than girls.incomplete more often in boys than girls.

• Minimal blood is supplied through the Minimal blood is supplied through the ligamentum teres.ligamentum teres.

• (5-7 years) the supply from the lateral (5-7 years) the supply from the lateral epiphysial artery is receding and from midial epiphysial artery is receding and from midial side arterial supply is yet to be developed. side arterial supply is yet to be developed.

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Legg-Calve´-Perthes disease Legg-Calve´-Perthes disease Aseptic, idiopathic osteonecrosis of the Aseptic, idiopathic osteonecrosis of the

femoral epiphysisfemoral epiphysis Growth of the ossific nucleus stops and Growth of the ossific nucleus stops and

the bone becomes densethe bone becomes dense It is subsequently resorbed and replaced It is subsequently resorbed and replaced

by new bone by new bone

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Legg-Calve´-Perthes’ Legg-Calve´-Perthes’ diseasedisease Etiology Etiology

TraumaTrauma Hereditary factorsHereditary factors CoagulopathyCoagulopathy IdiopathicIdiopathic Altered arterial status of femoral headAltered arterial status of femoral head Abnormal venous drainageAbnormal venous drainage Abnormal growth and developmentAbnormal growth and development As a sequelae to synovitis.As a sequelae to synovitis.

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Level of affection according Level of affection according to possible pre disposing to possible pre disposing

factors.factors.

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PathogenesisPathogenesis Articular cartilage becomes thick getting Articular cartilage becomes thick getting

nutrition from synovial fluid where as nutrition from synovial fluid where as deeper zone of epiphysis is under deeper zone of epiphysis is under nurished thus it become thin, wide and nurished thus it become thin, wide and cyst formation in metaphsis (Ischaemia).cyst formation in metaphsis (Ischaemia).

Impaired and uneven revascularation Impaired and uneven revascularation following repeated infarction being following repeated infarction being aggravated by mechanical forces aggravated by mechanical forces following subluxed head- deformed head following subluxed head- deformed head (Resorption, repair and remodel).(Resorption, repair and remodel).

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Thus Waldenstorm staged the Thus Waldenstorm staged the pathological process into 4 stages:pathological process into 4 stages:

Initial or Ischemic stageInitial or Ischemic stage Resorption or fragmentation stageResorption or fragmentation stage Reparative stageReparative stage Remodelling stageRemodelling stage

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Legg-Calve´-Perthes diseaseLegg-Calve´-Perthes disease Pathologic AnatomyPathologic Anatomy

Stage of increased density Fragmentation phaseFragmentation phase Healing phaseHealing phase

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Legg-Calve´-Perthes diseaseLegg-Calve´-Perthes disease Stage of increased densityStage of increased density

Areas of necrotic bone Areas of necrotic bone Subchondral fracture -Subchondral fracture -Collapsed Collapsed

trabeculaetrabeculae Thickened articular cartilageThickened articular cartilage

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Legg-Calve´-Perthes diseaseLegg-Calve´-Perthes disease Fragmentation phase Fragmentation phase

Signs of repair are found Signs of repair are found ““creeping substitution” creeping substitution” Loss of height of the femoral headLoss of height of the femoral head Growth plate is irregular and disrupted Growth plate is irregular and disrupted

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Legg-Calve´-Perthes diseaseLegg-Calve´-Perthes disease Healing phase Healing phase

Both woven and lamellar, predominates Both woven and lamellar, predominates Trabeculae and marrow spaces regain a Trabeculae and marrow spaces regain a

normal architecture normal architecture

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Legg-Calve´-Perthes diseaseLegg-Calve´-Perthes disease Changes soft tissue of the hip joint

Synovitis Articular cartilage hypertrophy

Irreversible femoral head deformation Stresses of weight‑bearing pass across

the acetabular margin Incapable of withstanding physiological

stresses

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Classification of Perthes Classification of Perthes diseasedisease

According to stage of disease –According to stage of disease – Waldenstrom classificationWaldenstrom classification According to Prgnosticate outcome-According to Prgnosticate outcome-

Catterall classificationCatterall classificationSalter and Thompson classificationSalter and Thompson classificationHerring lateral pillar classificationHerring lateral pillar classification

According to definning outcome-According to definning outcome-Stulberg classificationStulberg classification

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Waldenström ClassificationWaldenström Classification Stage-I: Stage of increased densityStage-I: Stage of increased density Stage-II: Fragmentation stageStage-II: Fragmentation stage Stage-III: Healing or reossification stageStage-III: Healing or reossification stage Stage-IV: Healed or remodeling stageStage-IV: Healed or remodeling stage

Catterall classificationCatterall classification Group-I Group-I Group-II Group-II Group-IIIGroup-III Group-IVGroup-IV

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Group-I Group-I Affection of only a small part of the anterior Affection of only a small part of the anterior

epiphysisepiphysis Group-IGroup-III

More of the anterior segment is involved More of the anterior segment is involved Central sequestrum is present Central sequestrum is present Epiphyseal height is preserved.Epiphyseal height is preserved.

Group-IIIGroup-III Most of the epiphysis is “sequestrated”Most of the epiphysis is “sequestrated” Unaffected portions located medial and lateral Unaffected portions located medial and lateral

to the central segment to the central segment Group-IVGroup-IV

The whole epiphysis is sequestrated The whole epiphysis is sequestrated

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Herring Lateral Pillar Classification Herring Lateral Pillar Classification A- Minimal density change, no loss of A- Minimal density change, no loss of

heightheight B- Some density change, B- Some density change, Height ≥50%, Height ≥50%,

Central pillar collapseCentral pillar collapse C- C- Height < 50%Height < 50%

The Stulberg Classification System Group-I: Femoral head normal Group-II: Femoral head round, within 2

mm of circle, same circle both views Group-III: Femoral head ovoid,

acetabulum matches head

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Group-IV: Femoral head flattened more than 1 cm on weight-bearing areas, acetabulum also flattened

Group-V: Femoral head collapsed, acetabulum not flattened

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Clinical FeaturesClinical Features

Painless limp leads to painful limpPainless limp leads to painful limp Pain in the groin very often refer to kneePain in the groin very often refer to knee Antalgic & trendelenburg gaitAntalgic & trendelenburg gait Decrease range of motion especially abd, Decrease range of motion especially abd,

internal rotation, to some extent flexioninternal rotation, to some extent flexion Atrophy of thigh muscleAtrophy of thigh muscle Short limbShort limb

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Investigation requiredInvestigation required X-ray-AP & frog lateral view X-ray-AP & frog lateral view

Crescent signCrescent signSalters signSalters signCaffey’s signCaffey’s sign

USGUSG ArthrographyArthrography Bone scanBone scan MRIMRI

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Co-relation between clinico, Co-relation between clinico, radio & pathological stateradio & pathological state

First stageFirst stage Clin.- Pain, complain around knee or Clin.- Pain, complain around knee or almost almost

normal.normal. Rad.- Dense head Rad.- Dense head Path.- Interrupted blood vessels, more Path.- Interrupted blood vessels, more

venous obstruction with few bone cell deatvenous obstruction with few bone cell deat Second stage:Second stage:

Clin.- Restriction of abd, internal rotation at times Clin.- Restriction of abd, internal rotation at times flexion. (abd in flex.) Mild atrophy flexion. (abd in flex.) Mild atrophy

Rad.- Increased density and flattening.Rad.- Increased density and flattening. Path.- collapse of trabecular bone.Path.- collapse of trabecular bone.

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Third stage:Third stage: Clin.- Pain and stiff hipClin.- Pain and stiff hip Rad.- Fragmentation(ant, sup & lateral aspect of Rad.- Fragmentation(ant, sup & lateral aspect of

epiphysis) epiphysis) Path.- Osteoclast invasion.Path.- Osteoclast invasion.

Fourth stage:Fourth stage: Clin.- Recovery of some movements of hip with Clin.- Recovery of some movements of hip with

reduction reduction of pain. of pain. Rad.- Mushroom head, coxa plana.Rad.- Mushroom head, coxa plana. Path.- Revascularization & recanalisation.Path.- Revascularization & recanalisation.

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Changes of NeckChanges of Neck First Stage- NormalFirst Stage- Normal Second Stage- Some cystic change & wideSecond Stage- Some cystic change & wide Third Stage- Looks bend.Third Stage- Looks bend. Fourth Stage- Short & Bent (Coxa vara)Fourth Stage- Short & Bent (Coxa vara)Changes of acetabular cavityChanges of acetabular cavity Ist- Increased ( Thick articular cartilage)Ist- Increased ( Thick articular cartilage) Iind & IIIrd- More increased ( Hypertrophy Iind & IIIrd- More increased ( Hypertrophy

of ligamentum Teres). of ligamentum Teres).

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CHANGES OF ACETABULUM CHANGES OF ACETABULUM In late stage ( III & IV) becomes irregular In late stage ( III & IV) becomes irregular

and coveted, secondary to changes of and coveted, secondary to changes of head.head.

CHANGES OF ARTICULAR CARTILAGE CHANGES OF ARTICULAR CARTILAGE OF HEADOF HEAD

Hypertrophy in IInd and IIIrd stageHypertrophy in IInd and IIIrd stageCHANGES IN SYNOVIAL MEMBRANECHANGES IN SYNOVIAL MEMBRANE Hyper plasia in IInd & IIIrd stageHyper plasia in IInd & IIIrd stageCHANGES IN CAPSULE & MUSCLECHANGES IN CAPSULE & MUSCLE Contracted & atrophy in IIIrd & IVth stageContracted & atrophy in IIIrd & IVth stage

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Cateral “ Head at Risk” Cateral “ Head at Risk” SignsSigns

Clinical:Clinical: Progressive loss of hip motion more so Progressive loss of hip motion more so

abductionabduction Obese childObese child

Radiological:Radiological: Gage signGage sign Calcification lateral to epiphysisCalcification lateral to epiphysis Diffuse metaphyseal rarefactionDiffuse metaphyseal rarefaction Lateral extrusion of femoral headLateral extrusion of femoral head Growth disturbance of physisGrowth disturbance of physis

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DIFFERENTIAL DIFFERENTIAL DIAGNOSISDIAGNOSIS

Transient synovitisTransient synovitis Coxavara (ICV & ACV)Coxavara (ICV & ACV) Tuber culosis of hipTuber culosis of hip Limp (Clinical high suspicious index)Limp (Clinical high suspicious index)

0 to 1year 0 to 1year CDHCDH 1 To 5years1 To 5years ICVICV 5 to 10years5 to 10years LCPLCP 10 to 15years10 to 15years TB TB

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Treatment :Treatment : AIMAIM

Restoration and full mobility of hipRestoration and full mobility of hip Active containment of femural headActive containment of femural head Resumption of weight bearing and full Resumption of weight bearing and full

activity as soon as possible.activity as soon as possible. ObjectivesObjectives

To produce a normal femoral head and neckTo produce a normal femoral head and neck To produce a normal acetabulumTo produce a normal acetabulum A congruous hip which is fully mobileA congruous hip which is fully mobile To prevent degenerative arthiritis of the hip To prevent degenerative arthiritis of the hip

later in lifelater in life

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Factors influencing the Factors influencing the prognosisprognosis

Younger the age of onset better is Younger the age of onset better is the prognosis.the prognosis.

> 9- Poor> 9- Poor Extent of involvement of headExtent of involvement of head Male- Good prognosisMale- Good prognosis Catterall “head at risk” signsCatterall “head at risk” signs Passive containment Passive containment Type of treatment renderType of treatment render

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TREATMENT PROTOCOL TREATMENT PROTOCOL

Initial phase- Restoration of normal Initial phase- Restoration of normal looking head and maintain looking head and maintain mobility.mobility.

Active phase- Active containment and Active phase- Active containment and maintenance of full mobility.maintenance of full mobility.

Reconstructive phase- Correction of Reconstructive phase- Correction of residual deformities residual deformities

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Types of treatment Types of treatment adoptedadopted

ConservativeConservative SurgicalSurgical Traction Traction Inominate osteotomyInominate osteotomy Plaster castPlaster cast Femoral varus osteotomyFemoral varus osteotomy OrthosisOrthosis Combination of bothCombination of both

Valgus Valgus osteotomyosteotomy

Arthroplasty Arthroplasty

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TREATMENT ACCORDING TO TREATMENT ACCORDING TO AGEAGE

< 5yrs: Traction in 20 degree < 5yrs: Traction in 20 degree abduction abduction followed by weight followed by weight relieving relieving caliper caliper

> 5yrs: Without head at risk sign > 5yrs: Without head at risk sign conservative treatment (Plaster conservative treatment (Plaster spica followed by caliper)spica followed by caliper)

> 5yrs: With head at risk- varus > 5yrs: With head at risk- varus osteotomy ( inominate osteotomy osteotomy ( inominate osteotomy of of salter does not give gratifying salter does not give gratifying result result alone)alone)

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Treatment according to the stage of perthes’ disease

Initial phase - Physiotherapy - Active and passive ROM exercises to restore motion Traction - B/L skin traction and gradually abducting over 1-2 weeks till full abduction is regainedWeight relieving caliperActive phaseAim is to have containment Conservative a.Ambulatory - wt relieving caliper (Toronto orthosis, Newington orthosis. Birmingham brace etc) b.Non-ambulatory - Abduction Broomstick, plaster cast ,Hip spica cast Surgical – different osteotomies

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Utility of brace

• Keep head in acetabulum• Pressure of acetabular rim on head is

avoided • Head is equally pressurized • Maintain good range of movement • Perpetuate formation of spherical head

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Indication of different osteotomy procedures

•Passive abduction leading to full containment – subtrochanteric adduction osteotomy•With hinged acetabulum, (Deformed head is obstructed to enter into acetabular cavity – valgus osteotomy)•Inominate osteotomy is indicated with flat head (usually is combined with axer)•Chilectomy is indicated to relive the prominent lateral part to achieve containment

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Total hip joint replacement is the

gold standard for neglected

perthes’ disease with degenerative

changes found in both components

of hip joint in elderly patients

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Critical biomechanical observation of different deformities observed with

perthes’ disease Head - Flat, irregular, deformed with loss of spherocity Neck – wide, short and in varus position Trochanter – broad, prominent (beaking)Limb as a hole is shortAll these deformities could be explained with one preposition and i.e.,………………..

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Biomechanical analysis of different changes in prox femurAdd Force >>>> ABD

ForceCapital femoral epiphysis pushed laterally

Horizontal position of metaphyseal plate

Lateral subluxation of capital femoral epiphysis

Irregular growth of head in relation to normal growth of trochanter-thus beaking

Lilfting of abductor attachment

Weakening of abductor force

Vicious Cycle revolves

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Thus its seems that the hole

pathological deformities scenario could

be changed tackling the disparity

between adductor and abductor force

at the outset to nip the problem in bud


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