2. Overview
Definition
Vascular supply of femoral head
Natural History of the disease
Pathogenesis
Classification
Symtoms
Diagnosis
Management
Conservative Management
Containment procedures
Salvage procedures
3. Perthes disease may be defined as the self
limiting form of osteochondrosis of capital
femoral epiphysis of unwnown etiology that
develops in children
Age group: 3-10 years
Sex: males 4-5 times more than girls
Bilateral in 10-12% of patients
DEFINITION
4. SYNONYMS
Legg Calve-perthe’s disease
Legg’s stress fracture of femoral head
Osteochondritis deformans juvenalis
Osteochondrosis of hip joint
Pseudocoxalgia
Coxa plana
5. Epidemiology
M : F ratio 4 : 1
Children between 4- 8 years of age.Occasionally as
young as 2 years and teenagers have been reported.
10 % of the cases have a positive family history.
Abnormal presentations breech, tranverse lie.
more common in Japanese, Eskimos, and Central
Europeans and uncommon in native Australians,
Polynesians, American Indians, and blacks.
Low socio economic status, 3rd or the 4th child ususally
affected. Mean parental age is also higher than normal
6. Affected children have short stature, retarded bone
age, delay in skeletal maturation
Anthropometric measurements confirmed the affected
children were smaller in all dimensions except the
head circumference with distal extremities affected
more than the proximal ones.
Onset of disease at an early age may culminate in
normal growth in adult life, but a child with later onset
tends to remain small throughout life.
Epidemiology
8. Etiology
Coagulation disorders.
Arterial status of femoral head.
Abnormal venous drainage.
Abnormal growth and development.
Trauma.
Hyperactivity or attention deficit disorder.
Genetic component.
Environmental influences.
As a sequel to synovitis.
9. 1. Vascular supply
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.
4. Coagulation disorder
- Associated with absence of factor C or S.
- Increase in serum levels of
lipoproteins,thrombogenic substance.
ETIOLOGY
10. 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-the lateral epiphyseal artery which courses
through a narrow passage is susceptible to
damage
11. 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.
12. Blood supply to femoral head
Retinacular arteries
Metaphyseal arteries
Artery of the teres
ligament
13.
14. Blood supply to femoral head
Infants
1. Medial ascending cervical or inferior metaphyseal
arteries of trueta.
2. Lat epiphyseal
3. Lig teres – insignificant
4 mts – 4 years
1. Lat epiphyseal
2. Med epiphyseal decrease in number.
15. Blood supply to femoral head
4 yrs to 7 years
Epiphyseal plate forms a barrier to metaphyseal vessels.
Pre-adolescent
After 7 yrs arteries of lig teres become more prominent
and anastomose with the lateral epiphyseal vessels.
16. Truetta’s Hypothesis
He postulated that the solitary blood supply in the age
group 4-8 yrs makes them suceptible to ischemia.
Compression of Lat epiphyseal arteries by ext.rotators.
17. CAFFEY’S HYPOTHESIS
this theory is incompatible with the high predominance of
the disease in males, since the vascular supply is
identical in both sex
The radiologic features are more consistent with an AVN
resulting from intraepiphyseal compression of blood
supply to the ossification center
Recently in POSICON 2016 they have come across DVT as
one of the cause for vasularity.
19. PATHOGENESIS
1) INCIPIENT OR SYNOVITIS STAGE
lasts for 1 to 3 weeks.
The synovium is swollen, hyperemic and odematous.
There is notable absence of inflammatory cells, joint
fluid is increased
20. 2) STAGE OF AVASCULAR NECROSIS
lasts for 6 months to 1 year.
It involves only a portion of the ossific nucleus usually
situated anteriorly or involves the entire nucleus.
The bone architecture remains normal but lacunae
are vacant
Bone trabeculae are crushed into minute fragments
and compressed into a compact mass.
The gross appearance and contour of the femoral
head remains unchanged
21.
22. 3) STAGE OF FRAGMENTATION OR RESORPTION
Lasts for 2 to 3 years and characterised by resorption of
the necrotic bone and replacement by viable bone.
Subchondral fracture of necrotic bone result in
multiple trabecular fragments being compressed
together
23. 4) HEALED OR RESUDIAL STAGE
The normal bone is forming along side and
replacing slowly resorbing bone
The newly formed bone is immature formed of
slender trabeculae and early compressed together with
necrotic fragments
The entire ossific nucleus may be deformed assuming
mushroom shaped contour
Finally an enlarged femoral head (coxa magna)
emerges varying in contour from a normally spherical
and concentrically lodged head to a deformed
flattened and eccentrically placed head
24. APPERANCE OF GREATER
TROCHANTER:- It becomes strikinly large in
some cases. Since longitudinal growth of the
femoral neck may cease completely at 12 -14 years of
age , whereas growth of the greater trochanter
continues until 17 -18 years, a discerpancy in growth
neck and the greater trochanter may result.The
elevation impairs the power of pelvitrochanteric
abducter muscles, manifested by positive
trendelberg sign.
25. CLINICAL FEATURES
SYMPTOMS
Most childern present with mild and intermittent pain in the
thigh or a limp or both.
The onset of pain may be acute or insidious
The classical presentation is described as a “painles limp” the
child limps but does not complains of discomfort.
Pain is agrravated by movement of hip and relived by rest.
H/o of trauma usually a mild is present.
26. EXAMINATION:-
Antalgic gait
Muscle spasm secondary to irritable hip.
Limitation of abduction and internal rotation
Short stature
Ffd may be present
DIFFERENTIAL ROTATION .
TRENDELENBERG TEST POSITIVE
28. Arthrography
Indicated to know the contour of head and congruity
of articular surface
Provides reliable information regarding containment.
We can assess congruity of hip in many different
positions.
Not routinely used .
Arthrography is important only in the fragmentatory
and reparative stages
29. CT SCAN
Not as sensitive as nuclear
medicine or MRI.
CT may be used for
follow-up imaging in
patients with LPD.
30. MRI SCAN
It allows more precise
localization of involvement
than conventional radiography.
MRI is preferred for evaluating
the position, form, size of the
femoral head and surrounding
soft tissues.
MRI is as sensitive as isotopic
bone scanning.
31. Bone Scan
Indicated to diagnose in early stages and to classify the
severity.
Diagnosis possible months before signs appear on X-
Ray.
Avascular areas show cold spots.
Revascularisation can be detected much before
radiographic evidence.
33. 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
WALDENSTROM’S CLASSIFICATION BASED ON
RADIOGRAPHIC CHANGES
34. 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
35. CRESCENT/CAFFEY SIGN
'segmental fracture' also
termed by Caffey as
submarginal fracture which is
represented by a localized area of
increased density continuous
with the remainder of the EOC.
36. Sagging rope sign
-radio dense line overlying
proximal femoral metaphysis,
a result of growth plate
damage with metaphysial
response.
37. WALDENSTROM SIGN
-Slight lateral displacement of the femoral
head and smallness of theE0C
-best determined in the Lauenstein (frogleg)
view
38. Radiographic changes in metaphysis.
- Apparent very early in the disease process.
- Changes are of prognostic value, hips with cystic
changes were twice likely to have poor outcomes as
hips without cysts.
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
39. 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.
- Floor is altered to adapt shape of head, hollowed out
abruptly.
- There may be irregular ossification, cystic and
increased radiodense areas
40. Catterall classification (1971)
I – only anterior portion of epiphysis affected.
II – anterior segment involved, central
sequestrum present
III – most of epiphysis sequestered with
unaffected portions located medial and lateral to
central segment
IV – all of epiphysis sequestered.
41. Catterall's Classification
GRADE 1 : Only the
anterior part of the
epiphysis is involved. It
differs from the other
group that no collapse
occurs and there is
complete absorption of the
involved segment without
sequestrum formation and
height of the epiphysis is
maintained.
42. Catterall's Classification
GROUP 2 :- In this variety more of the anterior part of the
epiphysis is involved
Collapse with the formation of a dense collapsed segment
or sequestrum.
Despite collapse occurs the viable fragments maintain the
epiphyseal height.
Metaphyseal change -usually a well defined cyst which is
transitory and disappears with healing.
The sequestrum is separated from the viable posterior--
segments by a V which when present, is characteristic of
this group.
43.
44. Catterall's Classification
Group-3 : Only a small part of the posterior epiphysisis
is involved.
Ap view shows a appearance of a "head within a head".
In the later stages there is a collapsed sequestrum
centrally placed with very small amount of normal
appearing bone on the medial and lateral sides.
Metaphyseal changes - more generalised and when
extensive are frequently associated with broadening of
the neck.
45.
46. Catterall's Classification
GROUP 4 : whole epiphysis is sequestrated. On AP
view total collapse of the epiphysis may be seen
producing a dense fine.
Displacement of the epiphysis can occur not anteriorly
but posteriorly producing a mushroom like
apperance of the head.
The metaphyseal changes may be extensive
47.
48. HEAD AT RISK
Gage's sign –triangular section of osteoporosis on
lateral femoral head
lateral calcification
lateral subluxation
horizontal alignment of the growth plate
Diffuse metaphyseal reaction.
55. Prognostic Factors
1. Age at diagnosis
2. Extent of involvement
3. Sex
4. Catterall “head at risk” clinical signs
Clinical
1. Progressive loss of hip motion
2. Increasing abduction contracture
3. Obese child
56. Classification of Prognosis
Uniplanar methods
- CE angle of Weiberg.
- Salters extrusion Index.
- Epiphyseal index.
- Epiphyseal quotient.
Biplanar methods
- Stulberg classification.
57. CE angle of Weiberg
Indicator of acetabular depth It is
the angle formed by a
perpendicular lines through the
midportion of the femoral head
and a line from the femoral head
center to the upper outer
acetabular margin.
Normal = 20 to 40 degrees
Angle >25 = good,
20-25= fair,
< 20 = poor
59. Epiphyseal index & quotient
Epiphyseal index = greatest height of the epiphysis
divided by its width.
Epiphyseal quotient = Epiphyseal index of involved hip
divided by the index for uninvolved hip.
>0.6 = good
0.4-0.6 = fair
<0.4 = poor
61. Stulberg Classification Prognosis
Prognosis is poor if;
Extensive involvement of EOC
More than 6 years of age.
Early closure of epiphyseal plate
Advanced stage of disease when first seen.
Female patient.
The short term prognosis - Concerns femoral head
deformity at the completion of healing stage.
The long term prognosis - concern with the late
development of secondary degenerative osteoarthritis of
the hip in adult life.
62. Differential diagnosis
Tuberculosis of the hip
SCFE
Transient synovitis
Spondylodysplasia
AVN due to leukemia, lymphoma, gauchers disease,
Hemoglobinopathies etc
64. Treatment
Objectives
- To produce a normal femoral head and
neck(prevent deformation & enlargement)
- To produce a normal acetabulum
- A congruous hip which is fully mobile
- To prevent degenerative arthritis of the hip later in
life
65. Treatment
Perthes disease is a local, self - healing disorder
Goals of Treatment:
Containment of the head.
Elimination of hip irritability.
Restoration and maintenance of a good range of hip
motion.
Prevention of epiphyseal extrusion and subluxation.
Attainment of a spheric femoral head on healing
66. CONTAINMENT
CONTAINMENT aims at repositioning the extruded
anterolateral part of the femoral epiphysis into the
confines of the acetabulum
This can be achieved by ABDucting & flexing or
ABDucting & Internal Rotating the hip
Containment needs to be ensured until the healing
process beyond the stage where the epiphysis is
vulnerable to deformation(stage 3b)
67. Treatment
Caterall group 1 and
group 2 ( < 7 years)
No active
Herring group 1 & Treatment
group 2 (< 6 years)
68. Treatment
Treatment is divided into 3 phases
Initial Phase – restore & maintain mobility
Active Phase – Containment and maintainance of full
mobility.
Reconstructive phase – correct residual deformities.
69. 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.
Observation
70. 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”.
Symptomatic treatment
71. 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.
72. 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 1cases,group2&3cases less than 5 yrs, with
no signs of head at risk.
Severe flattening of head
Healed cases and cases with hinged abduction.
DEFINITIVE EARLY TREATMENT
73. Orthosis
Non Ambulatory weight releiving
1. Abduction broomstick plaster cast
2. Hip spica cast
3. Milgram hip abduction orthosis
Ambulatory Both limbs included
1. Petrie Abduction cast
2. Toronto orthosis
3. Newington orthosis
4. Birmingham brace
5. Atlanta Scotish Rite Brace
Ambulatory unilateral
1. Tachdjian trilateral socket orthosis
74. Orthosis
CONTRAINDICATION
Incompliant patient.
Psycho socially unacceptable for the patient or parents.
Bilateral involvement at different times requring
prolonged brace wear.
Disadvantages
Stiffness of the knee and ankle joint with adaptive
articular changes,
Restricted ambulation,
Pressure sores and need for frequent changes
75. 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)
Treatment regimen at Newington
Children’s hospital
79. Birmingham brace
Knealing bar & chain to
keep the foot off the
ground while a specially
altered crutch allows the
abducted, internally
rotated limb to clear the
body when patient walks
82. SURGICAL CONTAINMENT
• INDICATIONS:
– Age of clinical onset > 8yrs of age
– Herring type B
– Radiological evidence of loss of containment by conservative
modes
• CONTRAINDICATIONS:
– Herring’s type A and C
– Herring’s type B if child less than 8 yrs
– Healed cases.
– Hinged abduction
• ADVANTAGES
– Ability to obtain permanent containment of head.
– Period of Restriction is only 2 months.
Age at surgery:
- Should be done in the increased density or early
fragmentation phase
83. COMPLICATIONS
Femoral
Shortening
Stiffness
Malrotation
Limp
Positive trendelenburg
Pelvic
Lenghtening
Stiffness
Chondrolysis
Failure of
containment
Femoral-ABD+IR/ Flexion
Pelvic-augment acetabulum / reorient to cover ant
lateral part of epiphysis
85. CONTAINMENT SURGERIES
– Varus Derotational Femoral Osteotomy
–Innominate or Salter osteotomy
–Shelf procedure
–Combination of femoral and innominate osteotomy
–Combination of innominate osteotomy and shelf
-Trochanteric arrest
86. VARUS DEROTATION FEMORAL OSTEOTOMY (VDO)
Procedure of choice in 8 – 10 yrs without limb shortening
Prerequsite-reasonable ROM
osteotomy in increased density or early fragmentation stage
• Types - Open wedge or closed wedge
• Technique - Osteotomy at subtrochantric level & Distal
femur is fixed in varus(ADD) and external rotation using plate
and screws
20 -30 degree derotation & 20 degree varus
Hip spica for 8-12 weeks
87. varus derotational osteotomy
Limit varus correction 10-15degree at early stage
At later stage-greater varus correction needeed
If only internal rotation restricted-varus extension osteotomy
Complications
1. Persistant ext.rotation
2. Shortening of extremity
3. Incresed abductor lurch
4. Trochanteric over growth
5. Delayed or non union
6. Excessive varus
88. Level of osteotomy
Insertion of guide pin and reaming of
femur
First depth marking flush with lateral
cortex
Removal of wedge to customize it
90. Inominate osteotomy
Indications:
1.>6 yrs
2.mod Or severely affected head with loss of containment
Requirement:
• Able to abduct 45 deg
• femoral head to be contained in position
Complications :
loss of fixation, leg-lengthening ,dec. hip flx, jt.stiffness,
second procedure for k wire removal
91. INNOMINATE OSTEOTOMY – SALTER
• Advantages:
– Anterolateral coverage
– Lengthening of shortened limb
– No second operation for I/R
• Technique
– Iliac osteotomy is made just above acetabulum extending
from
greater sciatic notch to anterior inferior Iliac pine
– Entire acetabulum with pelvis is rotated downward and
outwards
– Bone graft from ilium is applied to osteotomy site
• Hip Spica for 8-12 weeks.
97. SHELF PROCEDURE
• Catterall proposes this as the primary method of management in
children over 8 years of age
Used to improve acetabular lateral cover of femoral head
INDICATION:
• Lateral subluxation
• Insufficient coverage
• Hinged abduction
Contra indication: Dysplastic hip with spherical congruity
COMPLICATION:
• Loss of hip flexion
• lateral femoral cutaneous nerve injury
Technique:
• bone graft is harvested from the ilium and inserted into the roof of
the acetabulum.
98. 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.
99. Combined femoral & innominate osteotomy
For severely affected hips at risk of poor outcome
Indications
Lateral subluxation & calcification
Considerable changes in epiphysis
100. SALVAGE OPTIONS
It is used for pain relief
Valgus osteotomy
Chiari osteotomy
Cheilecteomy
Trochanteric advancement
Arthrodesis
101. Recommended Indications for diff. surgeries
Hinged abduction - valgus subtrochanteric osteotomy
Severly Mal formed femoral head – cheilectomy
Coxa magna – shelf augmentation
A large malformed femoral head with lat.subluxation –
Chiari’s pelvic osteotomy
Capital femoral physeal arrest – Trochanteric
advancement
103. Chiari osteotomy
It is used as a salvage procedure to accomplish
coverage of a large flattened femoral head, in an older
child when the femoral head is subluxating
acetabulum and the pelvis inferior to the osteotomy
along with the femur is displaced medially.
The superior fragment of the osteotomy then becomes
a shelf and capsule is interposed between it and the
femoral head.
104. Chiari osteotomy
Capsular interpositional
arthroplasty
Deepens the deficient
acetabulum by medial
displacement of distal
pelvic fragment and
improves sup.lat.femoral
coverage.
109. ARTHRODISTRACTION
Illizarov half ring fixator in late onset perthes with
total head involvement
Unloads joint space
Preserve congruency of femoral head
FENETRATION OF EPIPHYSEAL GROWTH PLATE
Fenestration made in neck anteriorly with 3 or 4 drill
holes or a curet through epiphyseal growth plate.
Reduced time for resorption and regeneration.
110. GROWTH FACTORS AND INDUCING DRUGS
Stimulate healing to prevent deformity. Newer drugs
that will slow down bone loss and stimulate new bone
formation. (bisphosphonates)
HLA 1 RELATED TO CHILDREN WITH PERTHES
DISEASE SHOWS IMMUNOLOGICAL RELATION
WITH PERTHES
GENE THERAPY - Stahl gene responsible for perthes,
further studies are yet to understand
111. Criteria of Final Result
Grading of the final result is done after four year of onset.
Good: The hip is asymptomatic with full range of motion,
the femoral head is round and well centered, there is no
acetabular change joint shape is not increased. A slight loss
of epiphyseal height is permissible.
Fair: The hip is asymptomatic with motion is slightly
restricted, especially in medial rotation. The femoral head
is round with slight broadening that is not fully contained;
less than one fifth of is uncovered some adaptive acetabular
change is acceptable provided that head is round,
epiphyseal height is reduced.
112. Poor: The hip is symptomatic with motion always
restricted femoral head is flat, broad, and irregular
and at least one fifth is uncovered. There are adaptive
acetabular changes and the joint space is widened at
the inferior medial aspect.
113. ASSESSMENT OF END RESUTLT
Grading (Mose)
Good : Femoral head spherical and of the same radius
on AP and lateral view. CE angle of 20° or more.
FAIR : no more than 2mm deviation from sphericity
on ap and lateral view and CE angle of 15 to 19 degree
POOR : greater than 2mm variation from sphericity
on either ap or lateral view and CE angle less than 15
degree
114.
115. Recent Advances
Anticoagulant
Botulinum toxin
Ibadronate :this has shown there importance in rat
model by increase spericity of femoral head
116.
117.
118. Take home message
Localised manifestation of an generalized epiphyseal
disorder
Non weight bearing & weight bearing have shown
similar results
Always classify the disease grade & jump to treatment
options
Age at onset,sex & amount of involvement for
prognosis
At Birth – Physeal plate is porous. Metaphyseal vessels easily penetrate to supply the head. Lateral and medial epiphyseal vessels contribute. Acetabular contribution absent
4 months to 4 years - Lateral epiphyseal vessels predominate, penetrating vessels gradually reduce as epiphyseal plate develops
4- 7 years only lateral and medial epiphyseal vessels supply the head.
Above 7 years – artery of ligamentum teres also contributes to vascularity.