Perthes ’ disease

1. PERTHES ’ DISEASEPERTHES ’ DISEASE
Dr MANNAN AHMEDDr MANNAN AHMED

2. LEGG – CALVE – PERTHE’SLEGG – CALVE – PERTHE’S
DISEASEDISEASE
 First described byFirst described by
WaldenstormWaldenstorm in 1909in 1909
 Legg, CalveLegg, Calve && Perthe’sPerthe’s inin
19101910

3. PERTHE’S DISEASEPERTHE’S DISEASE
 Coxa planaCoxa plana
 PseudocoxalgiaPseudocoxalgia
 Osteochondritis deformans coxa juvenilisOsteochondritis deformans coxa juvenilis
 Osteochondrosis capital femoral epiphysisOsteochondrosis capital femoral epiphysis

4. Perthe’s DiseasePerthe’s Disease is a condition which isis a condition which is
pathologically characterized by idiopathicpathologically characterized by idiopathic
avascular necrosis of the epiphysis of theavascular necrosis of the epiphysis of the
femoral head in a child.femoral head in a child.
The avascular epiphysis is almost always completelyThe avascular epiphysis is almost always completely
revascularised and replaced but resulting in variablerevascularised and replaced but resulting in variable
degree of deformity of the femoral head and growthdegree of deformity of the femoral head and growth
disturbance.disturbance.

5. EPIDEMIOLOGYEPIDEMIOLOGY
 Common inCommon in Central Europe,Central Europe, less commonless common inin
blacks, Chinese & Indiansblacks, Chinese & Indians..
 Quite frequent in ruralQuite frequent in rural SouthSouth Western coastWestern coast
of India.of India.
10 times more common in Uduppi area of10 times more common in Uduppi area of
Karnataka than Vellore in Tamil Nadu.Karnataka than Vellore in Tamil Nadu.

6. EPIDEMIOLOGYEPIDEMIOLOGY
 Sex: MalesSex: Males are affectedare affected 4-5 times4-5 times
moremore often than females.often than females.
 Age:Age: most commonly seen in agedmost commonly seen in aged 5 – 105 – 10
yrsyrs..
Mean age is higher in South India.Mean age is higher in South India.
9.9 yrs – males9.9 yrs – males
8.7 yrs - females8.7 yrs - females

7. ETIOLOGYETIOLOGY
A temporary and possibly repeated vascularA temporary and possibly repeated vascular
insultinsult
The precise cause of insult is obscure –The precise cause of insult is obscure –
IDIOPATHICIDIOPATHIC
BothBoth 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.

8. PathogenesisPathogenesis
 Histologic changes described by 1913Histologic changes described by 1913
 Secondary ossification center= covered bySecondary ossification center= covered by
cartilage of 3 zones:cartilage of 3 zones:
 SuperficialSuperficial
 EpiphysealEpiphyseal
 Thin cartilage zoneThin cartilage zone
 Capillaries penetrate thin zone from belowCapillaries penetrate thin zone from below

9. Pathogenesis: cartilage zonesPathogenesis: cartilage zones

10. PathogenesisPathogenesis
 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 hypercellularityMiddle zone has 1)areas of extreme hypercellularity
in clusters and 2)areas of loose fibrocartilaginousin clusters and 2)areas of loose fibrocartilaginous
matrixmatrix
 Superficial and middle layers nourished bySuperficial and middle layers nourished by
synovial fluidsynovial fluid
 Deep layer relies on blood supplyDeep layer relies on blood supply

11. PathogenesisPathogenesis
 Physeal platePhyseal plate: cleft formation, amorphis debris,: cleft formation, amorphis debris,
blood extravasationblood extravasation
 Metaphyseal regionMetaphyseal region: normal bone separated by: normal bone separated by
cartilaginous matrixcartilaginous matrix
 Epiphyseal changes can be seen also in greaterEpiphyseal changes can be seen also in greater
trochanter, acetabulumtrochanter, acetabulum

12. PATHOGENESISPATHOGENESIS
4 stages4 stages on the basis of evolution of diseaseon 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

13. PATHOGENESISPATHOGENESIS
Stage of Avascular NecrosisStage of Avascular Necrosis
IschemiaIschemia
A part ( anterior) or whole of capitalA part ( anterior) or whole of capital
femoral epiphysis is necrosed.femoral epiphysis is necrosed.
On X-ray –On X-ray –
 The ossific nucleus looksThe 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
normalnormal
–– increased joint space.increased joint space.

14. PATHOGENESISPATHOGENESIS
Stage ofStage 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 vascularNecrotic trabecular debris is resorbed & replaced by vascular
fibrous tissue the alternating areas of sclerosis andfibrous tissue the alternating areas of sclerosis and
fibrosis appear on X- ray asfibrosis appear on X- ray as fragmentation of epiphysisfragmentation of epiphysis..
 New immature bone laid on intactNew immature bone laid on intact
necrosed trabeculae by creepingnecrosed trabeculae by creeping
substitution further increasessubstitution further increases
the density of ossific nucleus onthe density of ossific nucleus on
X-ray.X-ray.

15. It is at this stage that there isIt is at this stage that there is
collapse and loss of structuralcollapse and loss of structural
integrity of the femoral head asintegrity of the femoral head as
it is sort of softened due to boneit is sort of softened due to bone
resorption, collapse of necroticresorption, collapse of necrotic
bone and persistence ofbone and persistence of
fibro-vascular tissue leading tofibro-vascular tissue leading to
deformation of epiphysis.deformation of epiphysis.
The femoral head mayThe femoral head may extrudeextrude from the acetabulumfrom the acetabulum
at this stage.at this stage.
Stage ofStage of REVASCULARIZATION / FRAGMENTATION (contd.)REVASCULARIZATION / FRAGMENTATION (contd.)

16. PATHOGENESISPATHOGENESIS
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.

17. PATHOGENESISPATHOGENESIS
 Remodeling / Residual stageRemodeling / Residual stage
This is the stage of remodeling and there is noThis is the stage of remodeling and there is no
additional change in the density of the femoraladditional change in the density of the femoral
head.head.
Depending on the severity of the disease theDepending on the severity of the disease the
residual shape of the head may be sphericalresidual shape of the head may be spherical
or distorted.or distorted.

18. CLINICAL PICTURECLINICAL PICTURE
 Typically a boy, 5-10 years old.Typically a boy, 5-10 years old.
 Characteristic presentation is aCharacteristic 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 beThe child appears to be wellwell & not sick.& not sick.
 The hip looks to be deceptively normal – there may beThe hip looks to be deceptively normal – there may be
little wasting.little 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.

19. DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS
UnilateralUnilateral
 Tuberculosis hipTuberculosis hip
 SynovitisSynovitis
 Slipped femoral capitalSlipped femoral capital
epiphysisepiphysis
 LymphomaLymphoma
 Eosinophilic granulomaEosinophilic granuloma
BilateralBilateral
 HypothyroidismHypothyroidism
 Multiple epiphysealMultiple epiphyseal
dysplasiadysplasia
 SpondyloepiphysealSpondyloepiphyseal
dysplasiadysplasia
 Sickle cell diseaseSickle cell disease

20. IMAGING STUDIESIMAGING STUDIES
Perthe’s disease is suspected clinically butPerthe’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

21. IMAGING STUDIESIMAGING STUDIES
 Stages of Avascular necrosis, Fragmentation, Ossification & ResidualStages 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 prematureAbnormal growth and premature
closure leading to short & wide neck.closure leading to short & wide neck.
Greater Trochanter –Greater Trochanter –
Elevated proximally d/to retardationElevated proximally d/to retardation
of the longitudinal growth of femoralof the longitudinal growth of femoral
neck – abductor insufficiency.neck – abductor insufficiency.

22. IMAGING STUDIESIMAGING STUDIES
Sagging rope signSagging rope sign
 A rope like radiodense line overlying the proximalA rope like radiodense line overlying the proximal
femoral metaphysis.( intertrochantric area)femoral metaphysis.( intertrochantric area)
 Is infact the anterior portion of the overlarge femoralIs infact the anterior portion of the overlarge femoral
head as it projects on a shortened and wide proximalhead as it projects on a shortened and wide proximal
femoral metaphysis.femoral metaphysis.

23. IMAGING STUDIESIMAGING STUDIES
Hinge abductionHinge abduction
 The Articular surface of the head and acetabulum areThe Articular surface of the head and acetabulum are
not concentric.not concentric.
 TheThe femoral head hingesfemoral head hinges at the acetabulum when limb isat 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.

24. IMAGING STUDIESIMAGING STUDIES
Head In Head Sign

25. RADIOGRAPHIC CLASSIFICATIONRADIOGRAPHIC CLASSIFICATION
 Radiographic picture varies with the stage andRadiographic picture varies with the stage and
severity of the disease.severity of the disease.
 Number of classification systems have beenNumber of classification systems have been
developed to estimate the severity of the diseasedeveloped 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

26. 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.

27. Catterall I Catterall II
GROUP I & II Have A Good PrognosisGROUP I & II Have A Good Prognosis

28. Groups III and IV have a poor prognosisGroups III and IV have a poor prognosis
Catterall IVCatterall III

29. SALTER AND THOMPSONSALTER AND THOMPSON
 A more simple classification.A more simple classification.
 Recognized that Catterall first two groups & nextRecognized 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

30. HERRING LATERAL PILLAR CLASSIFICATIONHERRING LATERAL PILLAR CLASSIFICATION
Lays importance on the structural integrity ofLays 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 pillarno 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 ofThere 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 ofThere 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

31. HERRING LATERAL PILLAR CLASSIFICATION
Group AGroup A Group CGroup C

32. MANAGEMENTMANAGEMENT
No general agreement on the “correct” courseNo 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 theis to prevent deformation of the
femoral head.femoral head.
 Prevention of stiffness and maintenance ofPrevention 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

33. MANAGEMENTMANAGEMENT
Main cause of deformationMain cause of deformation -- extrusionextrusion of theof the
femoral head.femoral head.
TheThe treatmenttreatment when needed is to try to prevent thiswhen needed is to try to prevent this
deformation .deformation .
ContainmentContainment of the femoral head within the acetabulum.of the femoral head within the acetabulum.
The socket, thus, acts as a mould to keep the headThe 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.

34. MANAGEMENTMANAGEMENT
 Essential that intervention to preventEssential that intervention to prevent
deformation of head is instituted before thisdeformation of head is instituted before this
complication develops / any irreparablecomplication develops / any irreparable
deformationdeformation
 When does deformation occur & till whenWhen does deformation occur & till when
is it reversible ?is it reversible ?
Deformation occurs during the phase ofDeformation occurs during the phase of
revascularization (fragmentation) & earlyrevascularization (fragmentation) & early
regeneration (ossification).regeneration (ossification).
It would therefore follow that if the containment isIt would therefore follow that if the containment is
to succeed, it would need to be performedto succeed, it would need to be performed
before the late phase of fragmentation, i.e., inbefore the late phase of fragmentation, i.e., in
stages of AVN or early fragmentation.stages of AVN or early fragmentation.

35.  How long containment?How long containment?
Needs to be ensured until the healing processNeeds to be ensured until the healing process
and beyond the stage where epiphysis isand beyond the stage where epiphysis is
vulnerable to deformation that is until the latevulnerable to deformation that is until the late
stage ofstage of regeneration phase ( 2 yrs)regeneration phase ( 2 yrs)

36.  Symptomatic treatmentSymptomatic treatment
 CONTAINMENT OF HEADCONTAINMENT OF HEAD
(a) Conservative methods(a) Conservative methods
(b) Surgical methods(b) Surgical methods
ManagementManagement

37. CONSERVATIVE METHODSCONSERVATIVE METHODS
Weight relief & restWeight relief & rest
In the past, treatment was primarily directed at avoidingIn the past, treatment was primarily directed at avoiding
weight by bed rest for prolonged period (up to 2 yrs) orweight by bed rest for prolonged period (up to 2 yrs) or
weight 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 & casting
Plaster cast in abd. & internal rotation – broomstick castsPlaster 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.

38. HIP ABDUCTION BRACE / CASTSHIP ABDUCTION BRACE / CASTS
Broom stick casts
Scottish Rite orthosis

39. BROOMSTICK CASTSBROOMSTICK CASTS

40. SURGICAL METHODSSURGICAL METHODS
Femoral osteotomyFemoral osteotomy – S/T or I/T.– S/T or I/T.
Innominate osteotomyInnominate osteotomy –– Anterolateral coverageAnterolateral coverage
Operative reconstruction provides the advantage ofOperative reconstruction provides the advantage of
improved containment & early mobilization and is aimproved containment & early mobilization and is a
preferred method.preferred method.
No end point for discontinuing the treatment because theNo end point for discontinuing the treatment because the
improved containment is permanent.improved containment is permanent.
Short term studies suggest an improvement in the naturalShort term studies suggest an improvement in the natural
course of the disease process with femoral osteotomy.course of the disease process with femoral osteotomy.
(Salter’s )

41. FEMORAL OSTEOTOMYFEMORAL OSTEOTOMY
 Technically less demanding than innominate osteotomyTechnically less demanding than innominate osteotomy
 Usually 20Usually 2000
varus angulation & 20varus angulation & 2000
IR appears sufficient.IR appears sufficient.
 Good to decide abduction, internal rotation or flexion on aGood to decide abduction, internal rotation or flexion on a
pre-operative arthrogram.pre-operative arthrogram.

42. FEMORAL OSTEOTOMYFEMORAL OSTEOTOMY
 Up to 12 years of age anUp 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.

43. PELVIC OSTEOTOMYPELVIC OSTEOTOMY
 Redirectional OsteotomyRedirectional Osteotomy
 Salter’s osteotomy toSalter’s osteotomy to
reorient the acetabulumreorient the acetabulum
 Shelf OperationShelf Operation
 To create a bony shelf toTo create a bony shelf to
cover the extruded part ofcover the extruded part of
the epiphysis.the epiphysis.
 Displacement OsteotomyDisplacement Osteotomy
 Chiari osteotomy isChiari osteotomy is
another way to improveanother way to improve
the coveragethe coverage..

44. 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 affectedwhole head affected
with some lateral extrusionwith 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 review
Surgical
containment
• No specific treatmentNo specific treatment otherother
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 femoralGrade 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

45. 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 planaprotuberance Coxa plana
 Chiari osteotomyChiari osteotomy
indication – malformed femoral head with lateralindication – malformed femoral head with lateral
subluxationsubluxation
 Trochanteric advancementTrochanteric 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

46. 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 – mostPresence of epiphyseal extrusion – most
important factor influencing outcome.important factor influencing outcome.
 Metaphyseal translucencies.Metaphyseal translucencies.
 Head at risk signs.Head at risk signs.

47. ““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 ofLateral 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)

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

49. GAGEGAGE``S SIGNS SIGN

50. Thank YouThank You