Perthes ’ disease

PERTHES ’ DISEASEPERTHES ’ DISEASE

Dr MANNAN AHMEDDr MANNAN AHMED

LEGG – CALVE – PERTHE’S LEGG – CALVE – PERTHE’S DISEASEDISEASE

First described by First described by WaldenstormWaldenstorm in 1909 in 1909

Legg, Calve Legg, Calve && Perthe’s Perthe’s in in 19101910

PERTHE’S DISEASEPERTHE’S DISEASE

Coxa plana Coxa plana PseudocoxalgiaPseudocoxalgia Osteochondritis deformans coxa juvenilis Osteochondritis deformans coxa juvenilis Osteochondrosis capital femoral epiphysisOsteochondrosis capital femoral epiphysis

Perthe’s Disease Perthe’s Disease is a condition which is is a condition which is pathologically characterized by idiopathic pathologically characterized by idiopathic avascular necrosis of the epiphysis of the avascular necrosis of the epiphysis of the femoral head in a child.femoral head in a child.

The avascular epiphysis is almost always completely The avascular epiphysis is almost always completely

revascularised and replaced but resulting in variable revascularised and replaced but resulting in variable degree of deformity of the femoral head and growth degree of deformity of the femoral head and growth disturbance.disturbance.

EPIDEMIOLOGYEPIDEMIOLOGY

Common inCommon in Central Europe, Central Europe, less commonless common inin blacks, Chinese & Indiansblacks, Chinese & Indians..

Quite frequent in rural Quite frequent in rural SouthSouth Western coast Western coast of India.of India.

10 times more common in Uduppi area of 10 times more common in Uduppi area of Karnataka than Vellore in Tamil Nadu.Karnataka than Vellore in Tamil Nadu.

EPIDEMIOLOGYEPIDEMIOLOGY

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

Age:Age: most commonly seen in aged most commonly seen in aged 5 – 10 yrs 5 – 10 yrs..

Mean age is higher in South India.Mean age is higher in South India.

9.9 yrs – males 9.9 yrs – males

8.7 yrs - females 8.7 yrs - females

ETIOLOGYETIOLOGY A temporary and possibly repeated vascular A temporary and possibly repeated vascular

insultinsult

The precise cause of insult is obscure – The precise cause of insult is obscure – IDIOPATHICIDIOPATHIC

Both Both developmental and environmental factorsdevelopmental and environmental factors which make the child susceptible to disease.which make the child susceptible to disease.

Proposed theoriesProposed theories.. Inherited protein C and/or S deficiency.Inherited protein C and/or S deficiency. Venous thrombosis.Venous thrombosis. Arterial occlusion / anomalies.Arterial occlusion / anomalies. Raised intra osseous pressure.Raised intra osseous pressure. Synovitis of hip joint.Synovitis of hip joint. Generalized skeletal disorder.Generalized skeletal disorder.

PathogenesisPathogenesis

Histologic changes described by 1913Histologic changes described by 1913 Secondary ossification center= Secondary ossification center=

covered by cartilage of 3 zones:covered by cartilage of 3 zones: SuperficialSuperficial EpiphysealEpiphyseal Thin cartilage zoneThin cartilage zone

Capillaries penetrate thin zone from Capillaries penetrate thin zone from belowbelow

Pathogenesis: cartilage Pathogenesis: cartilage zoneszones


Epiphyseal cartilage in LCP disease:Epiphyseal cartilage in LCP disease: Superficial zone is normal but thickenedSuperficial zone is normal but thickened Middle zone has 1)areas of extreme Middle zone has 1)areas of extreme

hypercellularity in clusters and 2)areas hypercellularity in clusters and 2)areas of loose fibrocartilaginous matrixof loose fibrocartilaginous matrix

Superficial and middle layers Superficial and middle layers nourished by synovial fluidnourished by synovial fluid

Deep layer relies on blood supplyDeep layer relies on blood supply


Physeal platePhyseal plate: cleft formation, : cleft formation, amorphis debris, blood extravasationamorphis debris, blood extravasation

Metaphyseal regionMetaphyseal region: normal bone : normal bone separated by cartilaginous matrixseparated by cartilaginous matrix

Epiphyseal changes can be seen also Epiphyseal changes can be seen also in greater trochanter, acetabulumin greater trochanter, acetabulum

PATHOGENESISPATHOGENESIS

4 stages4 stages on the basis of evolution of disease on the basis of evolution of disease

Stage of Avascular NecrosisStage of Avascular Necrosis Stage of Revascularization / FragmentationStage of Revascularization / Fragmentation Stage of Ossification / HealingStage of Ossification / Healing Remodeling / Residual stageRemodeling / Residual stage


Stage of Avascular NecrosisStage of Avascular Necrosis IschemiaIschemia

A part ( anterior) or whole of capital A part ( anterior) or whole of capital femoral epiphysis is necrosed.femoral epiphysis is necrosed. On X-ray – On X-ray –

The ossific nucleus looks The ossific nucleus looks smallersmaller Classically of Perthes’, Classically of Perthes’, lookslooks densedense TheThe articular cartilage remainsarticular cartilage remains viable & becomes thicker thanviable & becomes thicker than normal normal – – increased joint space.increased joint space.


Stage of Stage of REVASCULARIZATION / FRAGMENTATIONREVASCULARIZATION / FRAGMENTATION Ingrowths of highly vascular & cellular connective tissue.Ingrowths of highly vascular & cellular connective tissue.

Necrotic trabecular debris is resorbed & replaced by vascular Necrotic trabecular debris is resorbed & replaced by vascular

fibrous tissue the alternating areas of sclerosis and fibrous tissue the alternating areas of sclerosis and

fibrosis appear on X- ray as fibrosis appear on X- ray as fragmentation of epiphysisfragmentation of epiphysis..

New immature bone laid on intact New immature bone laid on intact

necrosed trabeculae by creeping necrosed trabeculae by creeping

substitution further increases substitution further increases

the density of ossific nucleus on the density of ossific nucleus on

X-ray.X-ray.

It is at this stage that there is It is at this stage that there is collapse and loss of structural collapse and loss of structural integrity of the femoral head asintegrity of the femoral head asit is sort of softened due to bone it is sort of softened due to bone resorption, collapse of necrotic resorption, collapse of necrotic bone and persistence of bone and persistence of fibro-vascular tissue leading to fibro-vascular tissue leading to deformation of epiphysis.deformation of epiphysis.

The femoral head mayThe femoral head may extrudeextrude from the acetabulum from the acetabulum at this stage.at this stage.

Stage of Stage of REVASCULARIZATION / FRAGMENTATION (contd.)REVASCULARIZATION / FRAGMENTATION (contd.)


Stage of Ossification / HealingStage of Ossification / Healing New bone starts formingNew bone starts forming

and epiphyseal densityand epiphyseal density

increases in the lucentincreases in the lucent

portions of the femoral head.portions of the femoral head.


Remodeling / Residual stageRemodeling / Residual stage

This is the stage of remodeling and there is no This is the stage of remodeling and there is no additional change in the density of the femoral additional change in the density of the femoral head.head.

Depending on the severity of the disease the Depending on the severity of the disease the residual shape of the head may be spherical residual shape of the head may be spherical

or distorted.or distorted.

CLINICAL PICTURECLINICAL PICTURE Typically a boy, 5-10 years old.Typically a boy, 5-10 years old.

Characteristic presentation is a Characteristic presentation is a painless limp.painless limp.

May present with limp along with pain.May present with limp along with pain.

The child appears to be The child appears to be wellwell & not sick. & not sick.

The hip looks to be deceptively normal – there may be little The hip looks to be deceptively normal – there may be little wasting.wasting.

Abduction & Internal rotation are nearly always limited.Abduction & Internal rotation are nearly always limited.

Antalgic gait in the irritable phase or Trendelenburg gait.Antalgic gait in the irritable phase or Trendelenburg gait.

DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS

UnilateralUnilateral

Tuberculosis hipTuberculosis hip SynovitisSynovitis Slipped femoral capital Slipped femoral capital

epiphysisepiphysis LymphomaLymphoma Eosinophilic granuloma Eosinophilic granuloma

BilateralBilateral

HypothyroidismHypothyroidism

Multiple epiphyseal Multiple epiphyseal dysplasiadysplasia

Spondyloepiphyseal Spondyloepiphyseal dysplasiadysplasia

Sickle cell diseaseSickle cell disease

IMAGING STUDIESIMAGING STUDIES

Perthe’s disease is suspected clinically but Perthe’s disease is suspected clinically but diagnosis rests on plain X-rays.diagnosis rests on plain X-rays.

Pelvis with both hips – AP viewPelvis with both hips – AP view

Frog leg Lateral view of the hipFrog leg Lateral view of the hip

IMAGING STUDIESIMAGING STUDIES Stages of Avascular necrosis, Fragmentation, Ossification & Residual Stages of Avascular necrosis, Fragmentation, Ossification & Residual

stage.stage. Other radiological changesOther radiological changes

Metaphyseal changes –Metaphyseal changes –

Hyperemia & osteoporosisHyperemia & osteoporosis

Cystic changes – poor prognosisCystic changes – poor prognosis

Changes in physis –Changes in physis –

Abnormal growth and premature Abnormal growth and premature

closure leading to short & wide neck.closure leading to short & wide neck.

Greater Trochanter –Greater Trochanter –

Elevated proximally d/to retardation Elevated proximally d/to retardation

of the longitudinal growth of femoral of the longitudinal growth of femoral

neck – abductor insufficiency.neck – abductor insufficiency.


Sagging rope sign Sagging rope sign A rope like radiodense line overlying the proximal A rope like radiodense line overlying the proximal

femoral metaphysis.( intertrochantric area)femoral metaphysis.( intertrochantric area) Is infact the anterior portion of the overlarge femoral Is infact the anterior portion of the overlarge femoral

head as it projects on a shortened and wide proximal head as it projects on a shortened and wide proximal femoral metaphysis.femoral metaphysis.

IMAGING STUDIESIMAGING STUDIESHinge abductionHinge abduction The Articular surface of the head and acetabulum are The Articular surface of the head and acetabulum are

not concentric.not concentric. The The femoral head hingesfemoral head hinges at the acetabulum when limb is at the acetabulum when limb is

abducted – the medial joint space is increased.abducted – the medial joint space is increased. Best diagnosed on arthrography.Best diagnosed on arthrography.


Head In Head Sign

RADIOGRAPHIC CLASSIFICATIONRADIOGRAPHIC CLASSIFICATION

Radiographic picture varies with the stage and Radiographic picture varies with the stage and severity of the disease.severity of the disease.

Number of classification systems have been Number of classification systems have been developed to estimate the severity of the disease developed to estimate the severity of the disease based on the radiographic findings .based on the radiographic findings .

Catterall, Catterall, Salter and Thompson, Salter and Thompson, HerringHerring

CATTERALL CLASSIFICATIONCATTERALL CLASSIFICATION

Catterall Group ICatterall Group I: : anterioranterior portion ofportion of epiphysis.epiphysis. no collapse.no collapse.

Catterall Group IICatterall Group II: Anterior segment (<50 %).: Anterior segment (<50 %). CentralCentral segment fragmentation & collapse. segment fragmentation & collapse. The lateral weight bearing segment intact .The lateral weight bearing segment intact .

Catterall Group IIICatterall Group III: Most of the nucleus is involved. : Most of the nucleus is involved. Only a small posterior segment viable.Only a small posterior segment viable. Fragmen. & collapse including lateral part. Fragmen. & collapse including lateral part. Metaphyseal resorption.Metaphyseal resorption.

Catterall Group IVCatterall Group IV: The: The entire headentire head is involved. is involved.

Catterall I Catterall II

GROUP I & II Have A Good PrognosisGROUP I & II Have A Good Prognosis

Groups III and IV have a poor prognosisGroups III and IV have a poor prognosis

Catterall IVCatterall III

SALTER AND THOMPSONSALTER AND THOMPSON A more simple classification.A more simple classification.

Recognized that Catterall first two groups & next Recognized that Catterall first two groups & next two groups are distinct with a different prognosis.two groups are distinct with a different prognosis.

Group AGroup A: < 1/2 head involved : < 1/2 head involved

favorable prognosisfavorable prognosis

Group BGroup B: > 1/2 head involved : > 1/2 head involved

unfavorable prognosisunfavorable prognosis

HERRING LATERAL PILLAR CLASSIFICATIONHERRING LATERAL PILLAR CLASSIFICATION

Lays importance on the structural integrity of Lays importance on the structural integrity of superolateral – the principal load bearing part of the head.superolateral – the principal load bearing part of the head.

Lateral Pillar Group A:Lateral Pillar Group A: no loss in height of the lateral pillar no loss in height of the lateral pillar minimal density change. minimal density change.

Lateral Pillar Group B:Lateral Pillar Group B: There is lucency & < 50% loss of There is lucency & < 50% loss of height in the lateral pillar.height in the lateral pillar.

Lateral Pillar Group C:Lateral Pillar Group C: There is > 50% loss in the height of There is > 50% loss in the height of the lateral pillar, severe collapse.the lateral pillar, severe collapse. Outcome relates strongly to the integrity of the lateral pillar

Group A faring the best & Group C the worst prognosis

HERRING LATERAL PILLAR CLASSIFICATION

Group AGroup A Group CGroup C

MANAGEMENTMANAGEMENT

No general agreement on the “correct” course No general agreement on the “correct” course of treatment for all cases.of treatment for all cases.

Aims of treatment :Aims of treatment : Primary aimPrimary aim is to prevent deformation of the is to prevent deformation of the

femoral head.femoral head. Prevention of stiffness and maintenance of Prevention of stiffness and maintenance of

good range of movements.good range of movements. Prevent or correct growth disturbances- Prevent or correct growth disturbances-

greater trochanteric overgrowthgreater trochanteric overgrowth

MANAGEMENTMANAGEMENT

Main cause of deformationMain cause of deformation - - extrusionextrusion of the of the femoral head.femoral head.

The The treatmenttreatment when needed is to try to prevent this when needed is to try to prevent this deformation . deformation .

Containment Containment of the femoral head within the acetabulum. of the femoral head within the acetabulum.

The socket, thus, acts as a mould to keep the head The socket, thus, acts as a mould to keep the head

spherical while still it is in the softened state.spherical while still it is in the softened state.

MANAGEMENTMANAGEMENT Essential that intervention to prevent Essential that intervention to prevent

deformation of head is instituted before this deformation of head is instituted before this complication develops / any irreparable complication develops / any irreparable deformationdeformation

When does deformation occur & till when When does deformation occur & till when is it reversible ?is it reversible ?

Deformation occurs during the phase of Deformation occurs during the phase of revascularization (fragmentation) & early revascularization (fragmentation) & early regeneration (ossification).regeneration (ossification).

It would therefore follow that if the containment is It would therefore follow that if the containment is to succeed, it would need to be performed to succeed, it would need to be performed before the late phase of fragmentation, i.e., in before the late phase of fragmentation, i.e., in stages of AVN or early fragmentation.stages of AVN or early fragmentation.

How long containment?How long containment? Needs to be ensured until the Needs to be ensured until the

healing process and beyond the healing process and beyond the stage where epiphysis is vulnerable stage where epiphysis is vulnerable to deformation that is until the late to deformation that is until the late stage of stage of regeneration phase ( 2 regeneration phase ( 2 yrs)yrs)

Symptomatic treatmentSymptomatic treatment

CONTAINMENT OF HEADCONTAINMENT OF HEAD

(a) Conservative methods (a) Conservative methods

(b) Surgical methods(b) Surgical methods

ManagementManagement

CONSERVATIVE METHODSCONSERVATIVE METHODSWeight relief & restWeight relief & restIn the past, treatment was primarily directed at avoidingIn the past, treatment was primarily directed at avoidingweight by bed rest for prolonged period (up to 2 yrs) orweight by bed rest for prolonged period (up to 2 yrs) orweight relieving calipers to prevent head deformation.weight relieving calipers to prevent head deformation.Little evidence for efficacy.Little evidence for efficacy.

Containment by bracing & castingContainment by bracing & castingPlaster cast in abd. & internal rotation – broomstick casts Plaster cast in abd. & internal rotation – broomstick casts Braces to keep hip in desired position. Braces to keep hip in desired position. Weight bearing is allowed in braces.Weight bearing is allowed in braces.Casts - temporary form of containment till definitiveCasts - temporary form of containment till definitive treatment undertaken.treatment undertaken.

HIP ABDUCTION BRACE / CASTSHIP ABDUCTION BRACE / CASTS

Broom stick castsScottish Rite orthosis

BROOMSTICK CASTSBROOMSTICK CASTS

SURGICAL METHODSSURGICAL METHODSFemoral osteotomyFemoral osteotomy – S/T or I/T. – S/T or I/T.

Innominate osteotomyInnominate osteotomy – – Anterolateral coverageAnterolateral coverage

Operative reconstruction provides the advantage of Operative reconstruction provides the advantage of improved containment & early mobilization and is aimproved containment & early mobilization and is apreferred method.preferred method.No end point for discontinuing the treatment because theNo end point for discontinuing the treatment because theimproved containment is permanent.improved containment is permanent.

Short term studies suggest an improvement in the naturalShort term studies suggest an improvement in the naturalcourse of the disease process with femoral osteotomy.course of the disease process with femoral osteotomy.

(Salter’s )

FEMORAL OSTEOTOMYFEMORAL OSTEOTOMY

Technically less demanding than innominate osteotomyTechnically less demanding than innominate osteotomy

Usually 20Usually 200 0 varus angulation & 20varus angulation & 200 0 IR appears sufficient.IR appears sufficient.

Good to decide abduction, internal rotation or flexion on a Good to decide abduction, internal rotation or flexion on a pre-operative arthrogram.pre-operative arthrogram.

FEMORAL OSTEOTOMYFEMORAL OSTEOTOMY Up to 12 years of age an Up to 12 years of age an open wedge osteotomyopen wedge osteotomy

may be performed without the risk of delayed union / may be performed without the risk of delayed union / non-union.non-union.

Also the amount of shortening is minimized.Also the amount of shortening is minimized. Pre-requisites – near normal hip movements.Pre-requisites – near normal hip movements.

PELVIC OSTEOTOMYPELVIC OSTEOTOMY Redirectional OsteotomyRedirectional Osteotomy

Salter’s osteotomy to Salter’s osteotomy to reorient the acetabulumreorient the acetabulum

Shelf OperationShelf Operation To create a bony shelf to To create a bony shelf to

cover the extruded part of cover the extruded part of the epiphysis.the epiphysis.

Displacement OsteotomyDisplacement Osteotomy Chiari osteotomy is Chiari osteotomy is

another way to improve another way to improve the coveragethe coverage..

Guidelines To Specific Treatment : Guidelines To Specific Treatment :

Present trendPresent trend

FavorableFavorable outcomeoutcome< ½ head affected < ½ head affected

with no extrusionwith no extrusion (Catterall I & II, Herring A,B)(Catterall I & II, Herring A,B)

Unfavorable outcomeUnfavorable outcome whole head affected whole head affected with some lateral extrusion with some lateral extrusion (Catterall III & IV, Herring C,(Catterall III & IV, Herring C, Head at risk signs)Head at risk signs)

<7 year > 7 year

• Containment with braces

• Periodic reviewSurgical containment

• No specific treatmentNo specific treatment other other than symptomatic treatment.than symptomatic treatment.• Require a periodicRequire a periodic radiological review. radiological review.

Grade the patients acc. to likely outcome, of the shape of the femoral Grade the patients acc. to likely outcome, of the shape of the femoral head - determined by radiographic features in the early stagehead - determined by radiographic features in the early stage

TREATMENTTREATMENT Reconstructive proceduresReconstructive procedures

Valgus extension osteotomyValgus extension osteotomy indication -hinge abduction of hipindication -hinge abduction of hip CheilectomyCheilectomy indication – malformed femoral head with lateralindication – malformed femoral head with lateral protuberance Coxa plana protuberance Coxa plana Chiari osteotomyChiari osteotomy indication – malformed femoral head with lateralindication – malformed femoral head with lateral subluxationsubluxation Trochanteric advancement Trochanteric advancement indication – premature capital femoral physeal arrestindication – premature capital femoral physeal arrest Greater trochanteric epiphysiodesisGreater trochanteric epiphysiodesis indication – premature capital femoral physeal arrestindication – premature capital femoral physeal arrest Shelf augmentation procedureShelf augmentation procedure indication – coxa magna coxa magna & lack of acetabularindication – coxa magna coxa magna & lack of acetabular coveragecoverage

PROGNOSTIC FACTORSPROGNOSTIC FACTORS

Age of the child at presentation.Age of the child at presentation. Sex : girls have poor prognosis.Sex : girls have poor prognosis. Extent of epiphyseal involvement. Extent of epiphyseal involvement. Range of movement at the hip.Range of movement at the hip. Presence of epiphyseal extrusion – most Presence of epiphyseal extrusion – most

important factor influencing outcome.important factor influencing outcome. Metaphyseal translucencies.Metaphyseal translucencies. Head at risk signs.Head at risk signs.

““HEAD AT RISK SIGNS”HEAD AT RISK SIGNS”

Gage's signGage's sign :- :- a V shaped lucency in the lateral epiphysis. a V shaped lucency in the lateral epiphysis. Lateral calcification (lateral to the epiphysis) (implies loss of Lateral calcification (lateral to the epiphysis) (implies loss of

lateral support)lateral support) Lateral subluxation of the head. (implies loss of lateral support)Lateral subluxation of the head. (implies loss of lateral support) A horizontal growth plate. (implies a growth arrestA horizontal growth plate. (implies a growth arrest

phenomenon and deformity)phenomenon and deformity)

Lat subluxation / Calcification lat Lat subluxation / Calcification lat to epipiphysis – HEAD AT RISKto epipiphysis – HEAD AT RISK

GAGEGAGE`̀S SIGNS SIGN

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Perthes ’ disease