PERTHES disease defination of perthes disease clinical features of perthes treatment of perthes

1. PRESENTER: Dr. ASHWANI PANCHAL
JSS MEDICAL COLLEGE
MYSORE

2.  BLOOD SUPPLY TO THE FEMORAL HEAD:
At birth:
i) Vessels from lateral side
ii) Vessels from top of ossified shaft.
iii) No ligamentum teres.
4 months to 4 yrs:
i) Epiphyseal ossification begins.
ii) Ascending cervical branches.( metaphyseal and
lateral epiphyseal vessels)
iii) After 4 months metaphyseal branches
decrease.

3.  4 to 7 yrs:
i) Epiphyseal plate is firm barrier
between epiphysis and metaphysis.
ii) Only source is lateral epiphyseal
arteries.
9-10 yrs:
i) Ligamentum teres vessels becomes
prominent.
ii) Anastomose with lateral epiphyseal
vessels.
Adoloscent period:
i) Trochanter ossified, growth plate
extends beneath both epiphysis.
ii) Majority from lateral epiphysis vessels.

4.  Perthes disease may be defined as the “disease
of the hip, limited sharply by age group and
largely by sex, it results from changes in capital
femoral epiphysis, apparently secondary to loss
of an adequate blood supply for at least a
portion of head.”
 Age group: 3-10 years
 Sex: males 4-5 times more than girls
 Bilateral in 10-12% of patients

5.  SYNONYMS
 Legg Calve-perthe’s disease
 Legg’s stress fracture of femoral head
 Osteochondritis deformans juvenalis
 Osteochondrosis of hip joint
 Pseudocoxalgia
 Coxa plana

6. 1. Vascular supply:
- Angiograms and laser studies have shown
medial circumflex artery is missing or obliterated
and obturator artery or the lateral epiphyseal
artery also affected.
2. Increased intra-articular pressure
3. Intraosseous pressure
- Patients has shown that the venous drainage in
the femoral head is impaired, causing an
increase in intraosseous pressure.

7. 4. Coagulation disorder
- Associated with absence of factor C or S.
- Increase in serum levels of
lipoproteins,thrombogenic substance.
5. Growth hormones
- Studies have shown reduced levels of growth
hormones, somatomedin A and C.
6. Social conditions
- Usually belong to lower socioeconomic status,
reflects dietary and environmental factors.
7. Trauma

8. 8.. Abnormal growth and development
- Bone age is lower than chronological age by 1-3 yrs,.
Ex: carpal bone age: 2 yrs (Triquetral and lunate)
- Usually shorter than their peers.
9. Genetic factors
- Inheritance 2-20%;inconsistent pattern.
- More Incidence of low birth weight, abnormal birth
presentations.
- First degree relatives have 35% more risk , 2nd and
3rd degree relatives are 4 times more prone for
perthes disease.

9. 1. TRUETA’S HYPOTHESIS
- Age < 3 yrs: blood supply contributed by
metaphyseal and retinacular arteries.
- Age 4-8 yrs: Retinacular arteries which enters head
as lateral epiphyseal arteries gets compressed by
lateral rotation muscles.
 Thus trueta postulates that solitary blood supply
during 4-8 yrs makes vulnerable for AVN of head.
 After 8 yrs foveolar arteries of ligamentum teres
contribute blood.

10.  Hypothesis into 4 stages
1. Incipient or synovitis stage:
- Lasts for 1-3 weeks.
- Synovium swollen, hyperaemic and oedematous.
2. Stage of avascular necrosis
- Lasts for 6mo to 1 yr.
- involves portion of ossific nucleus or entire
nucleus.
- Bony architecture crushed into minute fragments
and compressed into compact mass.
- Gross appearance and contour remains same.

11. 3. Stage of fragmentation or regeneration
- Lasts for 2-3 yrs
- Characterised by resorption of necrotic bone
and replacement by viable bone.
- Subchondral fractures results in necrotic bone.
4. Healed or residual stage
- Normal bone forms alongside with replacing
slowly resorbing bone.
- Newly formed bone is immature, assuming
mushroom shaped contour.

12. 1) Incipient stage or synovitis
stage:
- Lasts for 1-3 weeks
- Synovium hyperaemic,
swollen
2) Stage of avascular necrosis:
- Dead trabecular bone
- Collapsed trabeculae
- Thickened articular cartilage
Physeal disruption
- Cartilage extending from
the physis into
the metaphysis

13. 3) Fragmentation stage
- Invasion of vascular
granulation tissue
- New bone forming on old
trabeculae
- Woven new bone
formation

14. 4) HEALING STAGE
- Normal forming bone
alongside replacing slowly
resorbing bone.
- New bone, woven and
lamellar
- Mushroom shaped
contour.
- Soft tissues fibrotic, motion
restricted.
- Return to normal
architecture

15.  Changes in greater trochanter
- Becomes strinkingly hypertropic and elevated
proximally.
- Growth discrepancy between femoral head and
neck with trochanter.
- Impairs power of pelvitrochantric muscles resulting
in abductor insufficiency.

16. 1) An overabundance of fatty marrow,
2) Circumscribed osteolytic lesions with a sclerotic
border,
3) A wide growth plate with disarrayed ossification
and columns of unossified cartilage coursing
down into the metaphysis, and
4) Extension of the growth plate down the side of
the neck of the femur.

22.  Onset is insidous and prolonged course.
 Age group and sex preponderance:
 Painless limp is the earliest symptom and gait
antalgic.
 Pain in medial aspect of thigh and inner knee.
 Aggravated by movement of hip, increased
walking and relieved on rest.
 Stiffness.

23.  PHYSICAL FINDINGS
- Short stature
- In early stages- Muscle spasm evident and
restricted hip motion, especially in abduction.
- Proximal thigh and gluteal muscle atrophy.
- Child may hold extremity in slight flexion and
abduction, tenderness elicited over anterior aspect
of joint.
- As disease progresses moderate amount of
restricted motion, slight shortening and
insignificant limp is present.

24.  Waldenstrom observed that clinical course is
variable.
 He divided into 4 stages

25. 1. Patients age:
- Most consistent factor affecting course of the disease.
Age< 6 yrs: Mild disease
Age 6-9yrs: moderate symptoms.
Age > 9yrs: most severe.
2 . Disease severity:
- Varies from mild to severe, with most children
experiencing moderate symptoms for 12-18 months,
followed by complete resolution.
3. Extent of radiographic changes:
4. Outcome:

26.  Hematological parameters
 ESR
 CRP
 RA factor.
 Coagulability profile.
 X-rays
 CT scan
 MRI
 Arthrography
 Scintigraphy.

27. I) Stage 1(stage of increased density)
- Ossific nucleus initially smaller; femoral head
becomes uniformly dense;
- Convex rounded enlargement develops at superior
,margin of neck( Gage’s sign).
- A subchondral fracture may be seen;
- radiolucencies appear in the metaphysis
II) Stage 2(fragmentation stage)
- Lucency appear in epiphysis;
- Segments (pillars) of the femoral head demarcate
the femoral head may flatten and widen;
- Metaphyseal changes resolve;
- Acetabular contour may change

28. III) Stage 3(healing or reossification stage)
- New bone appears in femoral head which gradually
reossifies;
- Epiphysis becomes homogeneous.
IV) Stage 4( healed or remodelling stage)
- Femoral head is fully reossified and remodels to
maturity;
- Acetabulum also remodels

29.  Radiographic changes in metaphysis.
- Apparent very early in the disease process.
- Changes are of prognostic changes, hips with
cystic changes were twice likely to have poor
outcomes as hips without cysts.
Sagging rope sign

30.  Changes in neck of femur
- Deformity in neck can develop earlier than head.
- Upper part of neck is expanded and metaphyseal
end becomes rounded.
- neck progressively becomes shorter and wider.
 Changes in acetabular cavity
- Distance between medial pole of head and floor
of socket is increased(Waldenstrom’s sign)
- Ligamentum teres grossly swollen and congested.

31.  Changes in acetabular cavity
- Floor is altered to adapt shape of head, hollowed
out abruptly.
- There may be irregular ossification, cystic and
increased radiodense areas.

32.  Effective means of diagnosing perthes disease in
its early stages much before radiographic changes
are apparent.
 It provides more accurate information about the
extent of necrosis, reveals revascularization and
the stage of the disease.

33.  Scintigraphy also used to classify revascularisation
as either recanalization or neovascularization.
 Classified into two pathways
 A track indicates uncomplicated revascularisation of
femoral head,(Caterrall score 2.4), good prognosis.
 Track B represents slower rate of revascularisation
and healing(caterrall score of 3.5) poor prognosis.

34.  It provides accurate three- dimensional images of
femoral head and acetabulum.
 Classified into three types
 Group A
 Group B
 Group C
 CT is of benefit in later stages of the disease to
evaluate pain, locking of joint and other
mechanical symptoms

35.  Usg used in early stages of perthes disease to
demonstrate joint effusion and in later stages to
assess shape of femoral head
 A four stage classification
 It has a role in evaluation of blood flow in femoral
head.
 USG with microbubble contrast enhancement
used to evaluate vascularisation of femoral head.

36.  Demonstrates actual contour of femoral head and
state of congruity of articular surfaces.
 It provides reliable information regarding
containment of femoral head within acetabulum.
 Major advantage is that examiner can assess
congruity of hip in different positions.
 Often used in early diagnosis of hinge abduction
of hip.
 Useful in fragmentation and reparative stages.

37.  Accurate imaging modality for early diagnosis of
perthes disease.
 Evaluated congruity of articular surfaces, femoral
head containment, joint effusion and synovial
hypertrophy.
 Epiphyseal involvement clearly visualised on MRI
3 to 8 months after first symptoms.
 Diagnostic accuracy: 97-99%.
 Also provides earlier and reliable information on
revascularisation and extent of femoral head
necrosis.

39.  AVN caused by variety of conditions
- Sickle cell anemia
- Other hemoglobinopathies
- Thalassemia
- Steroid medication
- After traumatic hip dislocation
- Treatment of developmental dysplasia of hip

40. 1) Transient synovitis
2) Slipped femoral epiphysis
3) Congenital dysplasia of hip
4) Congenital coxa vara
5) Early Tuberculosis
6) Rheumatoid arthritis

41.  EPIPHYSEAL DYSPLASIAS
- Multiple epiphyseal dysplasia
- Sponyloepiphyseal dysplasia
- Mucopolysaccharidosis
- Hypothyroidism

42. Comparison chart
PERTHES DISEASE EPIPHYSEAL DYSPLASIA
Unilateral Bilateral involvement
If B/L, marked asymmetry, disease
in differing stages and severity
Symmetrical findings
No involvement of other joints Involvement of other joints or
spine.
Acetabulum not involved Involved
Sclerotic and cystic changes in
femoral head and cystic changes in
metaphysis
Few sclerotic changes in femoral
head.
More tendency towards lateral
calcification and subluxation
Little tendency.

43.  POOR IF,
1) Extensive involvement of EOC
2) More than 6yrs of age.
3) Early closure of epiphyseal plate
4) Advanced stage of disease when first seen.
5) Female patient.
6) Calcification lateral to epiphysis
7) Horizontal epiphyseal line.
Short term prognosis: concerns femoral head deformity at
completion of healing stage.
Long term prognosis: concerns with late development of
secondary degenerative OA of hip in adult life.

44.  GOALS OF TREATMENT
1) Elimination of hip irritability
2) Restoration and maintenance of good range of
hip motion.
3) Prevention of epiphyseal extrusion and
subluxation.( containment).
4) Attainment of spherical femoral head on healing.
- Lateral pillar classification coupled with age of
onset provides useful information regarding
prognosis and treatment modalities.

45.  Observation
1) Onset <6yrs of age, regardless of extent of capital
femoral epiphyseal involvement.
2) Age<6yrs of age: Catterall’s group 1 and 2. or
Salter thomson group A.
3) They should have clinical and radiographic
examination at frequent intervals( 3 months)
4) If unsuccessful, may necessaite a short course (2-
6 months) of non surgical treatment.

46.  The two primary means of
symptomatic treatment are bed rest
and traction.
 NSAIDS and crutches
 Stretching exercises with observation
used.
 Beneficial effects are greatest
around time of development of
subchondral fracture.
 Various traction methods include
simple longitudinal traction with leg
on bed, balanced suspension and
traction and “slings and springs”.

47.  Hip irritability with decrease of hip motion:
1-2 week period of bed rest with abduction traction
if recurs
2-3 months period of surgical non containment to
decrease risk of extrusion.
X-ray taken bi-monthly for evaluation.

48. Indications
 Age at clinical onset 6yrs or older.
 Catterall Group 3 or 4/ Salter thomson Group B.
 When loss of containment manifested by
extrusion seen on AP view.
Contraindications
 Group 1,2,3 cases less than 5 yrs, with no signs of
head at risk.
 Severe flattening of head
 Healed cases and cases with hinged abduction.

49.  Use of orthosis
1. Atlanta Scottish Rite Brace.:Post. Coverage
2. Toronto Brace of Bobechko
3. Hughston A frame
4. Broom stick plaster
5. Petrie and Bitenc Abduction cast: ant & lat.
coverage
6. Newington Abduction
7. Ambulation Brace.
Containment index used for evaluation.

50. Toronto brace
Newington brace incorporates a metal A-
frame with a central support for the thighs.

51.  Preliminary traction given
 Extremity placed in brace (abd: 45 & int. rot.)
 Child encouraged to walk because weight bearing
movements are essential for successful remodelling
 X-rays taken at regular intervals
 Discontinued when evidence of new subchondral bone
seen(20 months)

52.  Broom stick plaster and abduction cast by Patric
and Bitenc consists of long leg casts with 30-40
degrees of abduction and 5 of internal rotation.
Disadvantages:
I) Stiffness of knee and ankle.
II) Restricted ambulation
III) Pressure sores
IV) Need for frequent changes.

53.  Femoral osteotomy has been used to contain
femoral head with perthes disease.
 Better results with lateral pillar B/C border hips, in
children more than 8 yrs of age
 Indications:
- Age more than 6 yrs.
- Head at risk signs on radiography
- Failed conservative methods

54.  Pre operative measures:
 Age at surgery:
- Should be done in the increased density or
early fragmentation phase.
- Very early or late surgeries have led to
premature closure of epiphysis.
 Trochantric epiphysiodesis.

55.  Advantages
- Ability to obtain containment of head which
enhance remodelling.
- Period of restriction is for 2 months.
- No end point is necessary for treatment as the
containment is permanent

56.  First performed by Salter (‘62)
 Indications:
Onset < 6 yrs
Moderately or severly affected head.
Loss of containment
Pre- op requistes:
Minimum deformity of femoral head
Non irritable hip
No significant restriction of hip motion

57.  Advantages:
- Anterolateral coverage of femoral head.
- Lengthening of extremity
- Avoidance of plate removal
Disadvantages:
- Inability to obtain proper containment of femoral
head
- Increase in acetabular and hip joint pressure that
may cause furher necrosis.
- Increase in leg length
- Relative adduction of hip and uncovert femoral
head

58.  Willett recommenned first for older children
because of insufficient remodelling capacity, and
likelihood that shortening femur would cause
persistent limp.

59. i)Curved incision below iliac crest, strip glutei.
ii) Mobilize and divide reflected head of rectus femoris
iii) Trough in bone above insertion of capsule.
iv) Strips of cancellous bone inserted into trough so that they form a
canopy on superior surface of hip joint.
v) Pack web space between flap and graft canopy with gratft
vi)Repair rectus and lose the wound.

60. Advocated by Axer, Craig et al.
Advantages:
- Ability to obtain maximal coverage of femoral
head.
- Ability to correct excessive femoral anteversion.
Disadvantages:
- Excessive varus angulation that may not
correct with growth.
- Shortening of already shortened limb
- gluteal lurch
- Non-union, implant removal, premature closure
of epiphysis.

61.  Procedure of choice
- When containment is necessary and cant
be achieved by brace.
- Child of 8-10yrs old, without leg-length
discrepancy.
- On arthrogram,MRI femoral head is uncovered
- When there is significant amount of femoral
anteversion.

62. Level of osteotomy
Insertion of guide pin and reaming
of femur
First depth marking flush with lateral
cortex
Removal of wedge to customize it

63. Plate and compression screw
application
Insertion of bone screws.

64. I) LATERAL WEDGE OSTEOTOMY
 Axer developed for children of 5 yrs and younger,
prebent plate is used.
 II)OPENING WEDGE OSTEOTOMY
 III)REVERSED WEDGE OSTEOTOMY

65.  Rationale is that distraction of joint widens and
unloads joint space and reduces femoral head,
allows repair of articular cartilage.
 It preserves congruency of femoral head.
 Allows 50 degrees of flexion.

66.  VALGUS
EXTENSION
OSTEOTOMY:
- used mainly in
patients with hinge
abduction.
- It is an abnormal
movement occurs
when the deformed
femoral head fails to
slide within the
acetabulum

67.  VALGUS FLEXION INTERNAL ROTATION
OSTEOTOMY
- Kim et al. in 3D CT observed “Functional
retroversion”.
- Recommended valgus flexion internal rotation
femoral osteotomy plus acetabuloplasty.
Advantages:
1) Corrects functional coxa vera
2) Establishes normal articulation between
posteromedial portion of head and acetabulum.
3) Corrects external rotation deformity.
4) Improves joint congruity and head coverage

68.  CHEILECTOMY
- Ocassionally child is left with malformed femoral
head, large mushroom shaped/ lateral
protuberance.
- Erard and Dvaric observed good results at short
term follow up, deteriorated with time, and
increased pain after 2-4yrs of surgery.
 Pre op evaluation:
- determine whether protrubence is anterior or
posterior.

69.  Lateral incision
 Muscle seperated, nerve
secured.
 Capsule cut, protuberance
exposed
 Osteotome directed away
from lateral edge of
proximal femur physis.
 Excised.
 Check range of motion.

70.  CHIARI OSTEOTOMY
- Used as salvage procedure to accomplish
coverage of large flattened head, in older children
when head is subluxating and painful.
- Osteotomy of pelvis performed at superior margin
of acetabulum
- Pelvis inferior to osteotomy along with femur
displaced medially.
- Superior fragment then becomes shelf and
capsule interposed between it and femoral head.

71.  TROCHANTRIC OVERGROWTH
 Causes
 Result
 Consequence:
- Elevation of trochanter decreases tension and
mechanical efficiency of pelvic and trochantric
muscles.
- Shortened femoral neck moves trochanter closer to
centre of rotation of hip, line of pull of muscles
becomes more vertical.
- Impingement of head to the roof limiting abduction.

72.  Manicol and Makris described ‘Gear stick sign’
of trochantric impingement that is used for pre op
evaluation.
 Sign is based on observation that hip abductor is
limited by impingement of greater trochanter on
ilium when the hip is extended but full abduction
is possible when hip fully flexed

73. Normal growth pattern
Long. Growth arrested, greater
trochanter continues

74.  Muscle release and abduction casts
- If healing femoral head is moderately flattened,
an AP view reveals extruded anterolateral portion
of head impinging on acetabulum.
- Adductor tenotomy, iliopsoas release and
arthrotomy of hip joint can be done.
- Later Patric casts for 3-4 months usually permits
remodelling.

75.  Two types:
1)CAM Type (FAI).
- Occurs because of abnormal head with
increased radius at the base of neck, causes
abutting the acetabulum during extreme flexion.
- Causes acetabular cartilage abrasion or avulsion
and detachment of labrum.
- Coxa magna with large head are especially
prone.

76. 2) PINCER TYPE FAI
- Occurs due to direct contact between acetabular
rim and head neck junction.
- Labrum is first structure to get injured and may
become hypertropied / ossified.
- Pincer type is usually not the primary type.
Symptoms
1) Pain,
2) Arthritis.

77.  MANAGEMENT.(surgical)
 Indications.
 Patient mainly complains groin pain worse on
flexion type activities.
 Impingement sign. And its importance
 Imaging studies:
- AP standing view,
- Lateral view of each pelvis.
On AP, underlying pathologic conditions and
acetabular version to be identified.

78.  Cross over sign:
Intersection of anterior wall
and posterior wall medial to
lateral edge of acetabulum.
 Posterior wall sign:
Posterior wall projects more
medially than the centre of
hip.

79.  Surgical treatment.
- Surgical hip dislocation described by Ganz.
- Treatment involves removal of any non spherical
portion of head to provide greater clearance
during motion.
- Pincer type FAI needs temporary detachment of
central portion of labrum followed by recession of
anterior of acetabulum using osteotome.

80.  3 STAGES
1) Good:
- Hip asymptomatic, good ROM
- Head round and centered, no acetabular change.
2) Fair:
- Hip asymptomatic, ROM slightly restricted.
-Femoral head round with slight broadening.
- One fifth of head uncovered, some acetabular changes
accepted.
3) Poor:
- Hip symptomatic, motion restricted,
- Head flat ,broad.
- Gross acetabular changes, joint space widened at the
inferior medial aspect

81.  MEASUREMENT OF SPHERICITY OF
FEMORAL HEAD
- Obtained by transparent templates on which
inscribed series of 28concentric circles 2mm
apart.
- By this, deviation from circularity can be
measured in each projection.
- Acceptable when
i) NO deviation, radii equal, head spherical
ii) Radii<2mm, other parameters normal.

82.  2 LINES
i) Vertical line
passing through
centre of head©
ii) CE line from centre
of head to the edge
of acetabulum
CE angle <20
degrees on weight
bearing AP indicates
lateralisaton or
subluxation.

83. GRADING BY MOSE
1) Good: Femoral head spherical and same radius on
AP and lateral view.
- CE angle 20degrees or more.
2) Fair:
-NO more than 2mm deviation from sphericity on AP
and lateral view and CE angle of 15-19 degrees
3) Poor:
- Greater than 2mm variation from sphericity on either
AP or lateral view and CE angle less than 15degrees.

84.  References
1) Tachdjian’s pediatric orthopaedics
2) Cambell’s operative orthopaedics
3) Hefti’s pediatric orthopaedics
4) Mercer’s orthopaedics
5) Turek’s orthopaedics
6) Gray’s anatomy

85. THANK YOU