Legg-Calve-Perthe’s Legg-Calve-Perthe’s DiseaseDisease

Dr.E.Kaizar EnnisDr.E.Kaizar Ennis

DEFINITIONDEFINITION

It is a self-limited non-infectious disease of the hip joint occuring in children characterised pathologicaly by avascular

necrosis of the ossification center of capital femoral epiphysis which is alternately resolved and replaced resulting

in variable degrees of deformity of the femoral head and restricted movements

of the hip joints.

HISTORYHISTORY Described first by Waldenstrom in 1909 who Described first by Waldenstrom in 1909 who

mistakenly ascribed it to tuberculosis.mistakenly ascribed it to tuberculosis.

In 1910In 1910 Arthur Legg , U. S. AArthur Legg , U. S. A

Jacques Calve , FranceJacques Calve , France George Perthes ,GermanyGeorge Perthes ,Germany

described and recognized it as non-infectious in origin.

In 1922 Waldentrom gave the correct In 1922 Waldentrom gave the correct interpretation and described the stages.interpretation and described the stages.

INCIDENCEINCIDENCE

1:1200 1:1200 1:12000 1:12000 4M:1F4M:1F 2- 12 years (mean 7 years)2- 12 years (mean 7 years) 5-20% bilateral5-20% bilateral (In bilateral cases changes appear in one

hip at least one year earlier than the other)

British Isles: Catterall 1970(388 cases)British Isles: Catterall 1970(388 cases)

Aetiological TheoriesAetiological Theories

Arterial occlusionArterial occlusion• Mechanical obstructionMechanical obstruction• TraumaTrauma

Venous congestionVenous congestion Thrombophilia & hypofibrinolysisThrombophilia & hypofibrinolysis Disorder epiphyseal cartilageDisorder epiphyseal cartilage The aetiology may be ultimately

multifactorial Salter & Bell JBJS 1968 Glueck et al JBJS 1996 Ponseti JBJS 1983

BS of the infant femoral head

Metaphyseal vessels Lateral Epiphyseal

vessels Vessels of the

ligamentum teres.

STAGES OF PATHOLOGICAL PROCESS

1. Stage of synovitis

2. Stage of AVN

3. Stage of fragmentation

4. Stage of regeneration

5. Stage of healing.

SIGNSSIGNS Stage of AVN Waldenstrom signWaldenstrom sign

crescent sign crescent sign • Stage of regeneration A growth arrest line that outlines the ossific nucleusA growth arrest line that outlines the ossific nucleus

at the time of initial infarction is represented by aat the time of initial infarction is represented by a

‘‘head within a head’ SIGNhead within a head’ SIGN

The greater Trochanter becomes hypertrophied and elevate proximally, elevation impairs the power of The greater Trochanter becomes hypertrophied and elevate proximally, elevation impairs the power of abductor muscles and leads positive trendelenburg.abductor muscles and leads positive trendelenburg.

• The combination of a short femoral neck and a high greater The combination of a short femoral neck and a high greater

trochanter is considered ‘functional coxa vara’trochanter is considered ‘functional coxa vara’

HEAD AT RISK SIGNS-HEAD AT RISK SIGNS- CLINICAL CLINICAL

LOSS OF HIP MOTION IS PERSISTANT AND LOSS OF HIP MOTION IS PERSISTANT AND PROGRESSIVEPROGRESSIVE

INCREASED ADDUCTION CONTRACTURE INCREASED ADDUCTION CONTRACTURE OF HIPOF HIP

THE OBESE CHILDTHE OBESE CHILD FEMALE CHILDFEMALE CHILD AGE >7YRSAGE >7YRS

SYMPTOMS Mild intermittent pain in the hip,thigh or knee

Onset of pain is acute or insidious

The classical presentation has been described as “painless limp”

Because the presenting symptoms are usually mild, the parents do not seek medical attention long after the onset of symptoms.• A small % of children have a history of trauma

Clinical Examination

Antalgic gait

Tenderness Muscle spasm

Limitation of movement (abduction and internal rotation)

Muscle wasting

Leg length inequality

DIFFERENTIAL DIAGNOSIS UNILATERAL CASES Septic arthritis

Sponondyloepiphyseal dysplasia

Rheumatoid arthritis

Tuberculosis

Juvenile rheumatoid arthritis

BILATERAL CASES

Hypothyroidism

Multiple epiphyseal dysplasia

Mucopolysacharidosis

Sickle cell disease

Spondyloepiphyseal dysplasia

InvestigationsInvestigations CBCCBC ESRESR CRPCRP Urine REUrine RE RADIOGRAPHYRADIOGRAPHY BONE SCINTIGRAPHYBONE SCINTIGRAPHY MRIMRI USGUSG ARTHROGRAMARTHROGRAM ANGIOGRAPHYANGIOGRAPHY

Classification systemsClassification systems

CatterallCatterall Salter and ThompsonSalter and Thompson Herring’s lateral pillarHerring’s lateral pillar StulbergStulberg

Catterall ClassificationCatterall Classification

Group 1 –anterior portion of the Group 1 –anterior portion of the epiphysisepiphysis

Group 2-anterior and centralGroup 2-anterior and central Group 3-Most of the epiphysis is Group 3-Most of the epiphysis is

sequestratedsequestrated Group 4-All of the epiphysis is Group 4-All of the epiphysis is

sequestratedsequestrated

Salter Thompson classificationSalter Thompson classification

Type A Type A • Extent of subchondral fracture < 50% of Extent of subchondral fracture < 50% of

superior dome of HOFsuperior dome of HOF• Good prognosisGood prognosis

Type BType B• Extent of subchondral fracture > 50 % Extent of subchondral fracture > 50 %

of superior dome of HOFof superior dome of HOF• Fair or poor prognosisFair or poor prognosis

Lateral Pillar Classification by Lateral Pillar Classification by HerringHerring

Based on radiographic changes in Based on radiographic changes in the lateral portion of the femoral the lateral portion of the femoral head when it enters the head when it enters the fragmentation stage as seen in AP fragmentation stage as seen in AP viewview

Herring’s…Herring’s…

Group A-Minimal density change in the Group A-Minimal density change in the lateral pillar-good outcomelateral pillar-good outcome

Group B- height loss upto 50%- moderate Group B- height loss upto 50%- moderate outcomeoutcome

Group C- height loss > 50%-worst Group C- height loss > 50%-worst outcomeoutcome

Stulberg ClassificationStulberg Classification

Group I – Shape of HOF is normalGroup I – Shape of HOF is normal Group II –within 2 mm to a concentric Group II –within 2 mm to a concentric

circlecircle Group III –more than 2 mm (congruous Group III –more than 2 mm (congruous

incongruity)incongruity) Group IV – HOF flattened area > 1 cm in Group IV – HOF flattened area > 1 cm in

length(congruous incongruity)length(congruous incongruity) Group V – Collapse of HOF Group V – Collapse of HOF

(incongruous incongruity)(incongruous incongruity)

Determination of final outcomeDetermination of final outcome

MOSE-MOSE-AP & LAT VIEWS with Mose templateAP & LAT VIEWS with Mose template

Final shape of the head may be compared to a Final shape of the head may be compared to a perfect circle:perfect circle:

When the head contour is within 1 mm of a given When the head contour is within 1 mm of a given circle ,the result is deemed good.circle ,the result is deemed good.

  When the head contour is within 2 mm ,the result is When the head contour is within 2 mm ,the result is deemed fair. deemed fair. 

When the head contour is greater than 2 mm ,the result is When the head contour is greater than 2 mm ,the result is deemed poor.deemed poor.

Head at Risk Factors-RadiologicalHead at Risk Factors-Radiological

1.1. Lateral subluxation of femoral headLateral subluxation of femoral head

2.2. Gage’s sign – a radioluscent ‘V’ in Gage’s sign – a radioluscent ‘V’ in the lateral aspect of the epiphysisthe lateral aspect of the epiphysis

3.3. Calicification lateral to the epiphysis Calicification lateral to the epiphysis (*Cage sign)(*Cage sign)

4.4. A horizontal physeal lineA horizontal physeal line

5.5. Diffuse metaphyseal reaction Diffuse metaphyseal reaction (Metaphyseal cyst)(Metaphyseal cyst)

Gage SignGage Sign

Small osteoporotic Small osteoporotic segment forming a segment forming a translucent V- translucent V- shaped trough in shaped trough in the lateral part of the lateral part of the epiphysisthe epiphysis

CAGE SIGNCAGE SIGN

Calcification of the Calcification of the lateral epiphysis.lateral epiphysis.

Crescent sign/Salter sign/CaffreysCrescent sign/Salter sign/Caffreys

Trabeculae in dead Trabeculae in dead bone may fracture bone may fracture in subchondral in subchondral regionregion

Lateral subluxationLateral subluxation

Sagging Rope SignSagging Rope Sign

TREATMENTTREATMENT

Goals of TreatmentGoals of Treatment

1.1. Elimination of hip irritabilityElimination of hip irritability

2.2. Restoration & maintenance of hip Restoration & maintenance of hip motionmotion

3.3. Prevention of extrusion and collapse.Prevention of extrusion and collapse.

4.4. Attainment of a spherical HOFAttainment of a spherical HOF

PRINCIPLESPRINCIPLES

Full mobilityFull mobility

Containment of the femoral Containment of the femoral headhead

Resume wt. bearing & activity Resume wt. bearing & activity as soon as possible.as soon as possible.

Phases of TreatmentPhases of Treatment

Phase 1 – Initial phasePhase 1 – Initial phase Phase 2 – Containment and Phase 2 – Containment and

maintenance of HOF within maintenance of HOF within acetabulum and restoration of full acetabulum and restoration of full ROMROM

Phase 3 – Reconstructive surgeryPhase 3 – Reconstructive surgery

Phase 1Phase 1

Restore full ROMRestore full ROM Traction at homeTraction at home B/L counterpoised split Russel’s B/L counterpoised split Russel’s

traction with a medial rotation stop traction with a medial rotation stop on the thighon the thigh

InvestigationInvestigation AnalgesicsAnalgesics ObservationObservation

Phase 2(Containment by Phase 2(Containment by orthoses/surgery) orthoses/surgery)

Containment using OrthosesContainment using Orthoses• All braces abduct the affected hip, All braces abduct the affected hip,

allows for hip flexion and control allows for hip flexion and control rotation of the limbrotation of the limb

• Before starting containment, restore Before starting containment, restore normal ROM bynormal ROM by

Bed restBed rest TractionTraction AnalgesicsAnalgesics Decrease weight bearing by crutchesDecrease weight bearing by crutches

ORTHOSESORTHOSES

PREREQUISITESPREREQUISITES FULL RANGE OF MOTION WITH RELIEF OF FULL RANGE OF MOTION WITH RELIEF OF

MUSCLE SPASMMUSCLE SPASM ENTIRE FEMORAL HEAD SHOULD BE ENTIRE FEMORAL HEAD SHOULD BE

CONCENTRIC AND FULLY CONTAINED WITH CONCENTRIC AND FULLY CONTAINED WITH IN THE ACETABULUM IN THE ACETABULUM

MOTOR STRENGTH AND BALANCE TO USE MOTOR STRENGTH AND BALANCE TO USE THE ORTHOSES THE ORTHOSES

REASSESS EVERY 4-8 WEEKS FORREASSESS EVERY 4-8 WEEKS FOR GAITGAIT RANGE OF MOTIONRANGE OF MOTION XRAYXRAY

TYPES OF ORTHOSISTYPES OF ORTHOSIS

NON AMBULATORYNON AMBULATORY• BROOMSTICK PLASTIC CASTBROOMSTICK PLASTIC CAST• BIVALVED HIP SPICA CASTBIVALVED HIP SPICA CAST

AMBULATORYAMBULATORY• STATICSTATIC

• HARRISON HIP CONTAINTMENTHARRISON HIP CONTAINTMENT

• DYNAMICDYNAMIC BILATERALBILATERAL

• NEWINGTON HIP CONTAINMENTNEWINGTON HIP CONTAINMENT• SCOTTISH RITESCOTTISH RITE

UNILATERALUNILATERAL• TRILATERAL SOCKET HIP ABDUCTIONTRILATERAL SOCKET HIP ABDUCTION

Adujustable broomstick plasterAdujustable broomstick plaster

NEWINGTON HIP CONTAINMENTNEWINGTON HIP CONTAINMENT

Atlanta Scottish Rite BraceAtlanta Scottish Rite Brace

Containment by orthosis:Signs Containment by orthosis:Signs of healingof healing

Appearance of irregular ossification Appearance of irregular ossification in the capital femoral epiphysisin the capital femoral epiphysis

No new radio opaque areasNo new radio opaque areas Medial segment of femoral head Medial segment of femoral head

should increase in heightshould increase in height There should be an intact lateral There should be an intact lateral

columncolumn

COMPLICATIONSCOMPLICATIONS

PERSISTENT OR RECURRENT LOSS PERSISTENT OR RECURRENT LOSS OF HIP MOTIONOF HIP MOTION

PROGRESSIVE COLLAPSE AND PROGRESSIVE COLLAPSE AND SUPEROLATERAL EXTRUSION OF SUPEROLATERAL EXTRUSION OF FEMORAL HEADFEMORAL HEAD

SURGICAL CONTAINMENTSURGICAL CONTAINMENT

SURGERYSURGERY

ADVANTAGESADVANTAGES• NO END POINT OF TREATMENT IS NO END POINT OF TREATMENT IS

REQUIREDREQUIRED• RAPID RESUMPTION OF ACTIVITYRAPID RESUMPTION OF ACTIVITY

DIS ADVANTAGESDIS ADVANTAGES• SECOND OPERATION TO REMOVE SECOND OPERATION TO REMOVE

IMPLANTIMPLANT• COMPLICATION OF SURGERYCOMPLICATION OF SURGERY

PREREQUISITESPREREQUISITES

ABSENCE OF IRRITABILITY OR ABSENCE OF IRRITABILITY OR RESTRICTION OF HIP MOTIONRESTRICTION OF HIP MOTION

ABSENCE OR MINIMAL DEFORMITY ABSENCE OR MINIMAL DEFORMITY OF FEMORAL HEAD OF FEMORAL HEAD

CONCENTRIC CONTAINMENT BY CONCENTRIC CONTAINMENT BY ABD,MEDIAL ROTATION AND ABD,MEDIAL ROTATION AND FLEXIONFLEXION

CONTAINMENT PROCEDURESCONTAINMENT PROCEDURES

Innominate Osteotomy (Salter)Innominate Osteotomy (Salter) Femoral OsteotomyFemoral Osteotomy Lateral opening wedge osteotomyLateral opening wedge osteotomy

Varus derotation osteotomyVarus derotation osteotomy

Combined Femoral & Innominate osteotomyCombined Femoral & Innominate osteotomy

LATERAL SHELF PROCEDURELATERAL SHELF PROCEDURE

ArthrodiastasisArthrodiastasis

Phase 3Phase 3Reconstructive SurgeriesReconstructive Surgeries

LOWER LIMB INEQUALITYLOWER LIMB INEQUALITY GREATER TROCHANTER GREATER TROCHANTER

OVERGROWTH AND COXA BREVAOVERGROWTH AND COXA BREVA HINGED ABDUCTION HINGED ABDUCTION COXA MAGNACOXA MAGNA INCONGROUS HIPINCONGROUS HIP OSTEOCHONDRITIS DISSECANSOSTEOCHONDRITIS DISSECANS

Phase 3Phase 3Reconstructive SurgeriesReconstructive Surgeries

Valgus extension osteotomyValgus extension osteotomy Valgus flexion and internal rotation Valgus flexion and internal rotation

osteotomyosteotomy CheilectomyCheilectomy Shelf procedure by StaheliShelf procedure by Staheli

Salvage proceduresSalvage procedures

Garceau’s cheilectomyGarceau’s cheilectomy Chiari’s osteotomyChiari’s osteotomy Trochanteric epiphyseodesisTrochanteric epiphyseodesis Trochanteric advancementTrochanteric advancement Valgus osteotomyValgus osteotomy Hip replacementHip replacement

PROGNOSISPROGNOSIS

AGEAGE EXTENTEXTENT PROTRUSION OF FEMORAL HEADPROTRUSION OF FEMORAL HEAD GROWTH DISTURBANCE OF PHYSISGROWTH DISTURBANCE OF PHYSIS METAPHYSEAL CHANGESMETAPHYSEAL CHANGES STAGE IN THE NATURAL COURSE OF THE STAGE IN THE NATURAL COURSE OF THE

DISEASEDISEASE PERSISTENT LOSS OF HIP MOTIONPERSISTENT LOSS OF HIP MOTION OBESE CHILDOBESE CHILD

THANK YOU.THANK YOU.