Download - Legg – calve – perthes disease (2)

Perthes DiseasePerthes Disease

DefinitionDefinition

It is a self limiting disorder of the hip It is a self limiting disorder of the hip produced by ischemia and varying produced by ischemia and varying degrees of necrosis of the femoral degrees of necrosis of the femoral head.head.

SynonymsSynonyms

Coxa PlanaCoxa Plana Pseudocoxalgia (Calve)Pseudocoxalgia (Calve) Arthritis deformans juvenilis Arthritis deformans juvenilis

(Perthes)(Perthes) Osteochondroses of the hip.Osteochondroses of the hip.

Arthur LeggArthur Legg of of the United Statesthe United States

Jacques CalveJacques Calve of of FranceFrance

Georg PerthesGeorg Perthes of of GermanyGermany

Historical aspectHistorical aspect

Parker started the use of broomstick Parker started the use of broomstick cast in 1929.cast in 1929.

Eyre-Brook introduced traction in bed Eyre-Brook introduced traction in bed for 18-24 months.for 18-24 months.

Blood supply to femoral headBlood supply to femoral head

Retinacular arteriesRetinacular arteries Metaphyseal Metaphyseal

arteriesarteries Artery of the round Artery of the round

ligamentligament


Infants Infants 1.1. Metaphyseal arteries .Metaphyseal arteries .

2.2. Lat epiphyseal arteriesLat epiphyseal arteries

3.3. Lig teres – insignificantLig teres – insignificant 4 mts – 4 years4 mts – 4 years

1.1. Lat epiphysealLat epiphyseal

2.2. Metaphyseal art. decrease in numberMetaphyseal art. decrease in number

(due to appearance of growth plate).(due to appearance of growth plate).


4 yrs to 7 years4 yrs to 7 years1.1. Epiphyseal plate forms a barrier to Epiphyseal plate forms a barrier to

metaphyseal vessels.metaphyseal vessels. Pre-adolescentPre-adolescent

1.1. After 7 yrs arteries of lig teres become After 7 yrs arteries of lig teres become more prominent and anastomose with more prominent and anastomose with the lateral epiphyseal vessels.the lateral epiphyseal vessels.


AdolescentAdolescent After skeletal maturity metaphyseal After skeletal maturity metaphyseal

vessels again come into picturevessels again come into picture

IncidenceIncidence Male : Female = 4-5:1Male : Female = 4-5:1 2.5:1 in India2.5:1 in India Age of onset earlier in females.Age of onset earlier in females. Age – Age – Range – 2-13 years.Range – 2-13 years. Most common 4-8 years.Most common 4-8 years. Average – 6 years.Average – 6 years. Bilateral in 10-12 %Bilateral in 10-12 % Incidence more in Caucasians as compared Incidence more in Caucasians as compared

to Negroid, mongoloid.to Negroid, mongoloid.

EtiologyEtiology Etiology not known.Etiology not known. Coagulation disorders.Coagulation disorders. Altered arterial status of femoral head.Altered arterial status of femoral head. Abnormal venous drainage.Abnormal venous drainage. Abnormal growth and development.Abnormal growth and development. Trauma.Trauma. Hyperactivity or attention deficit Hyperactivity or attention deficit

disorder.disorder. Genetic component.Genetic component. Environmental influences.Environmental influences. As a sequelae to synovitis.As a sequelae to synovitis.

Coagulation disordersCoagulation disorders

Protein C or S deficiencyProtein C or S deficiency ThrombophiliaThrombophilia HypofibrinolysisHypofibrinolysis

Altered arterial status Altered arterial status

Angiographic studies have shown Angiographic studies have shown obstruction of obstruction of superior capsular arteries superior capsular arteries and and decreased flow in decreased flow in medial circumflex medial circumflex femoral arteries .femoral arteries .

The The intracapsular ring intracapsular ring has been found to be has been found to be incomplete.incomplete.

Abnormal venous drainageAbnormal venous drainage

Increased venous pressure in theIncreased venous pressure in the

femoral neckfemoral neck Congestion in the metaphysisCongestion in the metaphysis Venous outflow exits more distally in the Venous outflow exits more distally in the

diaphysis.diaphysis.

Abnormal growth and Abnormal growth and developmentdevelopment

A delay in Bone age of 1.5 to 2 A delay in Bone age of 1.5 to 2 years years

Low birth weightLow birth weight

Low levels of somatomedin CLow levels of somatomedin C

Trauma.Trauma.

In the developing femur (4 – 7 In the developing femur (4 – 7 yrs),the major yrs),the major lateral epiphyseal lateral epiphyseal vessels vessels must course through a must course through a narrow passage narrow passage ,which could make it ,which could make it susceptible to trauma.susceptible to trauma.

Hyperactivity or attention Hyperactivity or attention deficit disorderdeficit disorder

Genetic componentGenetic component

Familial association.Familial association.

X-Linked recessive inheritance. X-Linked recessive inheritance.

Environmental influencesEnvironmental influences

Low socioeconomic status.Low socioeconomic status.

Sequel to synovitisSequel to synovitis

Synovitis of the hip occurs early in Synovitis of the hip occurs early in Perthes disease.Perthes disease.

Increased pressure in synovitis may Increased pressure in synovitis may cause a cause a tamponade effect tamponade effect on the on the vasculaturevasculature

PathogenesisPathogenesis

Waldenstrom Waldenstrom staged the staged the pathological process of the disease pathological process of the disease asas

1.1. Initial or ischaemic stageInitial or ischaemic stage

2.2. Resorption or fragmentation stageResorption or fragmentation stage

3.3. Reparative stageReparative stage

4.4. Remodelling stageRemodelling stage

PathogenesisPathogenesis Ischaemic stageIschaemic stage

- Necrosis- Necrosis

- Crushing of trabaculae.- Crushing of trabaculae.

- degeneration of basal layer of - degeneration of basal layer of

articular cartilagearticular cartilage

- Thickening of peripheral - Thickening of peripheral

cartilagenous cap.cartilagenous cap.

- Shape of head maintained.- Shape of head maintained.

Ischaemic stageIschaemic stage

Pathogenesis cont…Pathogenesis cont…

Resorption stageResorption stage

- Invasion of vascular connective tissue.- Invasion of vascular connective tissue.

- Resorption of dead bone by- Resorption of dead bone by

Osteoclasts.Osteoclasts.

- loss of epiphyseal height due to- loss of epiphyseal height due to

1) Collapse of bony trabaculae.1) Collapse of bony trabaculae.

2) Resorption of dead bone2) Resorption of dead bone

Resorption stageResorption stage

Pathogenesis cont…Pathogenesis cont…

Reparative stageReparative stage

- pathological fracture.- pathological fracture.

- creeping substitution and - creeping substitution and

apposition of viable bone in deadapposition of viable bone in dead

trabaculae.trabaculae.

Reparative stageReparative stage

Remodelling stageRemodelling stage(replacement by biologically (replacement by biologically

plastic bone)plastic bone)

If treatedIf treated

Femoral head is Femoral head is

congrouscongrous

If untreatedIf untreated

Subluxation and Subluxation and deformitydeformity

Clinical FeaturesClinical Features Painless limp leads to painful limpPainless limp leads to painful limp

Pain in the groin,anterior hipPain in the groin,anterior hip

or greater trochanteror greater trochanter

Referred pain to the kneeReferred pain to the knee

Combination of antalgic & trendelenburg Combination of antalgic & trendelenburg

gait.gait.

Decreased range of motion Decreased range of motion especially abduction and internal especially abduction and internal rotation.rotation.

Atrophy of thigh muscles.Atrophy of thigh muscles.

ShorteningShortening

InvestigationInvestigation

X-Ray –AP & Frog leg Lat view X-Ray –AP & Frog leg Lat view (Lowenstein view)(Lowenstein view)

USGUSG ArthrographyArthrography Bone ScanBone Scan MRIMRI

X-RayX-Ray

Ossific nucleus Ossific nucleus smallersmaller

X-RayX-Ray

Cresent Sign or Cresent Sign or Salters sign or Salters sign or Caffey’s signCaffey’s sign

X-RayX-Ray

Increased Radio Increased Radio opacity of femoral opacity of femoral head due to head due to collapse, new bone collapse, new bone formation and formation and calcification of calcification of dead marrow.dead marrow.

X-RayX-Ray

Fragmentation of Fragmentation of epiphysisepiphysis

X-RayX-Ray

Metaphyseal Metaphyseal widening and widening and cystic changes in cystic changes in femoral neckfemoral neck

X-RayX-Ray

Lateral extrusion of Lateral extrusion of femoral head.femoral head.

Hinged abduction.Hinged abduction.

X-RayX-Ray

Sagging rope sign Sagging rope sign in adults with in adults with history of pertheshistory of perthes

UltrasoundUltrasound

Synovial effusionSynovial effusion

Cartilage hypertrophy in early stagesCartilage hypertrophy in early stages

ArthrographyArthrography

Shows configuration of the femoral Shows configuration of the femoral head and its relation with the head and its relation with the acetabulum.acetabulum.

ContainmentContainment CongruityCongruity Not routinely used .Not routinely used .

Bone ScanBone Scan

Diagnosis possible months before Diagnosis possible months before signs appear on X-Ray.signs appear on X-Ray.

Avascular areas show cold spots.Avascular areas show cold spots.

Bone ScanBone Scan

Convay et al Convay et al classificationclassification

Stage 1 is total Stage 1 is total lack of uptakelack of uptake

Bone Scan( stage 2)Bone Scan( stage 2)

Revascularisation of a Revascularisation of a lateral columnlateral column

Failure to Failure to revascularise revascularise at lat at lat column is a grave signcolumn is a grave sign

Also called Also called ““scintigraphic head at scintigraphic head at risk signrisk sign””

Precedes radiographic Precedes radiographic head at risk sign by 2-head at risk sign by 2-3 mths3 mths


Gradual filling of Gradual filling of anterolateral partanterolateral part


Return to normalReturn to normal

MRIMRI

Accurate in early diagnosis.Accurate in early diagnosis.

Shows Shows congruity,containment,synovial congruity,containment,synovial hypertrophy well.hypertrophy well.

ClassificationClassification

Waldenstroms classification.Waldenstroms classification.

Catterall classificationCatterall classification

Salter classificationSalter classification

Herrings lateral pillar classification.Herrings lateral pillar classification.

Waldenstroms classificationWaldenstroms classification

(Pathological classification)(Pathological classification)

Catterall classificationCatterall classification(based on x ray AP and Lat view(based on x ray AP and Lat view).).

I – only anterior portion of epiphysis I – only anterior portion of epiphysis

affected.affected. II – anterior segment involved central II – anterior segment involved central

sequestrum presentsequestrum present III – most of epiphysis sequestered withIII – most of epiphysis sequestered with

unaffected portions located medial unaffected portions located medial

and lateral to central segmentand lateral to central segment IV – all of epiphysis sequestered.IV – all of epiphysis sequestered.

Salter ClassificationSalter Classification

Type A = I & II CatterallType A = I & II Catterall Type B = III & IV Catterall.Type B = III & IV Catterall.

Herring Lat PillarHerring Lat Pillar

Group-A no involvement of the lateral Group-A no involvement of the lateral pillar, with no density changes and no loss pillar, with no density changes and no loss of height of the lateral pillarof height of the lateral pillar

Group-B hips have lucency in the lateral Group-B hips have lucency in the lateral pillar and may have some loss of height , pillar and may have some loss of height , but not exceeding 50% of the original but not exceeding 50% of the original height.height.

Group-C hips are those with more lucency Group-C hips are those with more lucency in the lateral pillar and >50% loss of in the lateral pillar and >50% loss of heightheight

Prognostic FactorsPrognostic Factors1.1. Age at diagnosisAge at diagnosis

<6 yrs – good <6 yrs – good

6 – 9 yrs – fair6 – 9 yrs – fair

>9 yrs - poor>9 yrs - poor

1.1. Extent of involvementExtent of involvement

2.2. SexSex

3.3. Catterall “head at risk” signsCatterall “head at risk” signs

Catterall “head at risk” Catterall “head at risk” signssigns

ClinicalClinical

RadiographicRadiographic

ClinicalClinical1.1. Progressive loss of hip motion more Progressive loss of hip motion more

so abduction.so abduction.

2.2. Obese childObese child

RadiographicRadiographic

1.1. Gage signGage sign

2.2. Calcification lateral to epiphysisCalcification lateral to epiphysis

3.3. Diffuse metaphyseal rarefactionDiffuse metaphyseal rarefaction

4.4. Lateral extrusion of femoral headLateral extrusion of femoral head

5.5. Growth disturbance of physis Growth disturbance of physis

Physeal disruptionPhyseal disruption

Metaphyseal rarefactionMetaphyseal rarefaction

Femoral head extrusionFemoral head extrusion

Gage’s signGage’s sign

Rarefaction in the Rarefaction in the lateral part of the lateral part of the epiphysis and epiphysis and subjacent subjacent metaphysis.metaphysis.

Classification of resultsClassification of results Uniplanar methodsUniplanar methods - CE angle of Weiberg.- CE angle of Weiberg. - Salters extrusion Index.- Salters extrusion Index. - Salters extrusion angle.- Salters extrusion angle. - Epiphyseal index.- Epiphyseal index. - Epiphyseal quotient.- Epiphyseal quotient. Biplanar methodsBiplanar methods - Mose classification.- Mose classification. - Stulberg classification.- Stulberg classification.

CE angle of WeibergCE angle of Weiberg Indicator of Indicator of acetabular depthacetabular depth It is the It is the angle formed by a angle formed by a

perpendicular line through perpendicular line through the midpoint of the femoral the midpoint of the femoral head and a line from the head and a line from the femoral head center to the femoral head center to the upper outer acetabular upper outer acetabular margin. margin.

Normal = 20 to 40 degrees Normal = 20 to 40 degrees Angle >25 = good, 20-25= Angle >25 = good, 20-25=

fair, < 20 = poorfair, < 20 = poor

Salters extrusion IndexSalters extrusion Index

If AB is more If AB is more than 20% of than 20% of CD it CD it indicates a indicates a poor poor prognosis prognosis

Salters extrusion angleSalters extrusion angle

Normal is 50 Normal is 50 degrees or degrees or moremore

Epiphyseal index & quotientEpiphyseal index & quotient

Epiphyseal index = greatest height Epiphyseal index = greatest height of the epiphysis divided by its width.of the epiphysis divided by its width.

Epiphyseal quotient = Epiphyseal Epiphyseal quotient = Epiphyseal index of involved hip divided by the index of involved hip divided by the index for uninvolved hip.index for uninvolved hip.

>0.6 = good>0.6 = good 0.4-0.6 = fair0.4-0.6 = fair <0.4 = poor<0.4 = poor

Mose ClassificationMose Classification

Good < 1 mmGood < 1 mm Fair < 2 mmFair < 2 mm Poor > 2 mmPoor > 2 mm

Stulberg classificatonStulberg classificaton Class I – Shape of the femoral head wasClass I – Shape of the femoral head was basically normal.basically normal. Class II – Loss of head height but within 2 mmClass II – Loss of head height but within 2 mm to a concentric circle on AP and frogto a concentric circle on AP and frog leg X-Rayleg X-Ray Class III – Deviates more than 2 mm andClass III – Deviates more than 2 mm and acetabulum contour matchesacetabulum contour matches the head contourthe head contour Class IV – Head Flattened, Flattened area Class IV – Head Flattened, Flattened area <1cm. Acetabulum contour matches<1cm. Acetabulum contour matches the head contourthe head contour Class V – Collapse of femoral head, AcetabularClass V – Collapse of femoral head, Acetabular contour does not changecontour does not change

Stulberg classificatonStulberg classificaton

Class I & IIClass I & II – Spherically congruent. – Spherically congruent.

Class III & IVClass III & IV – Congruous Incongruity – Congruous Incongruity OROR Aspherical congruity.Aspherical congruity.

Class VClass V – Incongruous incongruity – Incongruous incongruity OROR Aspherically incongruent.Aspherically incongruent.


Class I Class I Shape of Shape of the the femoral femoral head is head is basically basically normal.normal.


Class II Class II Loss of Loss of head head height height but but within 2 within 2 mmmm


Class III Class III Deviates Deviates more more than 2 than 2 mm mm


Class IV Class IV Head Head FlattenFlatteneded


Class V Class V Collapse of Collapse of femoral femoral head, head, Acetabular Acetabular contour contour does not does not changechange

Differential diagnosisDifferential diagnosis

Tuberculosis of the hipTuberculosis of the hip AVN due to leukemia, lymphoma, AVN due to leukemia, lymphoma,

gauchers disease, gauchers disease, hemoglobinopathies etchemoglobinopathies etc

Meyers dysplasiaMeyers dysplasia AVN following dislocation.AVN following dislocation. Transient synovitisTransient synovitis

TreatmentTreatment

ObjectivesObjectives - To produce a normal femoral - To produce a normal femoral head and neckhead and neck - To produce a normal acetabulum- To produce a normal acetabulum - A congruous hip which is fully - A congruous hip which is fully mobilemobile - To prevent degenerative arthritis - To prevent degenerative arthritis of the hip later in lifeof the hip later in life

TreatmentTreatment Treatment efforts are directed Treatment efforts are directed

towardstowards

- Restoration and maintenance of - Restoration and maintenance of

full mobility of the hipfull mobility of the hip

- Containment of the femoral- Containment of the femoral

head.head.

- Resumption of weight bearing- Resumption of weight bearing

and full activity as soon as and full activity as soon as

possiblepossible

TreatmentTreatment

Caterall group 1 andCaterall group 1 and

group 2 ( < 7 years)group 2 ( < 7 years)

NoNo Herring group 1 &Herring group 1 & TreatmentTreatment

group 2 (< 6 years)group 2 (< 6 years)

TreatmentTreatmentTreatment is divided into 3 phasesTreatment is divided into 3 phases

Initial Phase – restore & maintain Initial Phase – restore & maintain mobilitymobility

Active Phase – Containment and Active Phase – Containment and maintainance of full mobility.maintainance of full mobility.

Reconstructive phase – correct Reconstructive phase – correct residual deformities.residual deformities.

Treatment ( Initial Phase )Treatment ( Initial Phase ) Physiotherapy – active and passive Physiotherapy – active and passive

range of motionrange of motion

exercises to restoreexercises to restore

motionmotion Traction – B/L skin traction and Traction – B/L skin traction and

gradually abducting over 1-2 gradually abducting over 1-2

weeks till full abduction isweeks till full abduction is

regained.regained.

Treatment ( Active Phase )Treatment ( Active Phase )

Consists of containment of the Consists of containment of the femoral head within the acetabulum. femoral head within the acetabulum. This can be achieved by This can be achieved by

orthosis orthosis

or byor by

surgerysurgery

Treatment (Orthosis)Treatment (Orthosis) Non Ambulatory weight releivingNon Ambulatory weight releiving

1.1. Abduction broomstick plaster castAbduction broomstick plaster cast

2.2. Hip pica castHip pica cast

Ambulatory Both limbs includedAmbulatory Both limbs included

1.1. Petrie Abduction castPetrie Abduction cast

2.2. Toronto orthosisToronto orthosis

3.3. Newington orthosisNewington orthosis

4.4. Birmingham braceBirmingham brace

5.5. Atlanta Scotish Rite Brace Atlanta Scotish Rite Brace

Ambulatory unilateralAmbulatory unilateral

1.1. Tachdjian trilateral socket orthosisTachdjian trilateral socket orthosis

Treatment (Orthosis)Treatment (Orthosis)

Atlanta Scotish Rite Atlanta Scotish Rite BraceBrace


Newington orthosisNewington orthosis


Birmingham braceBirmingham brace


Toronto BraceToronto Brace


Tachdjian trilateral Tachdjian trilateral socket orthosissocket orthosis


Orthotic treatment is Orthotic treatment is discontinueddiscontinued when when the disease enters the disease enters the reparative phase the reparative phase and healingand healing is established. is established.

The radiographic evidence of healing areThe radiographic evidence of healing are1.1. Appearance of irregular ossification in the Appearance of irregular ossification in the

femoral head.femoral head.

2 . Increased density of femoral head should 2 . Increased density of femoral head should disappear.disappear.

3 . Medial segment of femoral head should 3 . Medial segment of femoral head should increase in size and height.increase in size and height.

4 . Metaphyseal rarefaction involving the lateral 4 . Metaphyseal rarefaction involving the lateral cortex of the metaphysis should ossify.cortex of the metaphysis should ossify.

5 . There should be intact lateral column.5 . There should be intact lateral column.

Treatment ( Surgical)Treatment ( Surgical)

Femoral varus osteotomy.Femoral varus osteotomy. Inominate osteotomy.Inominate osteotomy. Combined femoral and inominate Combined femoral and inominate

osteotomyosteotomy Valgus osteotomyValgus osteotomy Shelf arthroplastyShelf arthroplasty

Femoral varus osteotomyFemoral varus osteotomy

Recent AdvancesRecent Advances

AnticoagulantAnticoagulant

IbadronateIbadronate

Thank YouThank You

Download - Legg – calve – perthes disease (2)

Top Related