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

FIRST DESCRIBED BY
LEGG (USA), AND
WALDENSTORM IN
1909, AND BY
PERTHES(GERMANY)
AND
CALVE(FRANCE) IN
1910








Disorder of the hip in young children
Usually ages 4-8yrs
As early as 2yrs, as late as teens
Boys:Girls= 4-5:1
Bilateral 10-12%
No evidence of inheritance





Unknown
Past theories:
infection, inflammation, trauma, congenital
Most current theories involve vascular
compromise
◦ Sanches 1973: “second infarction theory”



sometimes called as “coronary artery disease
of hip”


Infants
1. Metaphyseal arteries .
2. Lat epiphyseal arteries
3. Lig teres – insignificant



4 mts – 4 years
1. Lat epiphyseal
2. Metaphyseal art. decrease in number
(due to appearance of growth plate).


4 yrs to 7 years
1. Epiphyseal plate forms a barrier to metaphyseal
vessels.



Pre-adolescent
1. After 7 yrs arteries of lig teres become more
prominent and anastomose with the lateral
epiphyseal vessels.
Susceptible child : delayed bone age
-- Trauma
-- Hereditary factors : controversial(HLA-A
antigens in lymphocytes)
-- Coagulopathy : protein c& s
-- Hyperactivity
-- Passive smoking
-- Synovitis
FACTORS UNLIKELY TO BE ETIOLOGY-- Endocrinopathy
-- Urban envt.
--



Histologic changes described by 1913
Secondary ossification center= covered by
cartilage of 3 zones:
◦ Superficial
◦ Epiphyseal
◦ Thin cartilage zone



Capillaries penetrate thin zone from below


Epiphyseal cartilage in LCP disease:
◦ Superficial zone is normal but thickened
◦ Middle zone has 1)areas of extreme hypercellularity
in clusters and 2)areas of loose fibrocartilaginous
matrix





Superficial and middle layers nourished by
synovial fluid
Deep layer relies on blood supply






Physeal plate: cleft formation, amorphis
debris(Bone dust), blood extravasation
Metaphyseal region: normal bone separated
by cartilaginous matrix
Epiphyseal changes can be seen also in
greater trochanter, acetabulum






Often insidious onset of a limp,excaberated
by activity.
C/O pain in groin, thigh, knee
Few relate trauma hx
Can have an acute onset









Decreased
ROM, especially abduction
and internal rotation:
initially due to muscle
spasm
Abductor limp
Trendelenburg test often
positive
Muscular atrophy of
thigh/buttock/calf
Limb length discrepency
Coxa magna
 Premature physeal growth arrest
Central-short neck,trochanteric overgrowth
Lateral-externally tilted head
trochantric overgrowth
acetublar deformity
Irregular head
Osteochondoirtis dessicans








AP pelvis
Frog leg lateral
Key= view films
sequentially over
course of dz
Arthrography
MRI role
undefined






WALDENSTROM
Modified Elizabethtown Classification
Catterall classification
Salter-Thompson Classification
Lateral Pillar Classification


Four Waldenstrom stages:
◦
◦
◦
◦

1)
2)
3)
4)

Initial stage
Fragmentation stage
Reossification stage
Healed stage
Stage of Avascular Necrosis
Ischemia

A part ( anterior) or whole of capital
femoral epiphysis is necrosed.
On X-ray –
◦ The ossific nucleus looks smaller

◦ Classically of Perthes’, looks
dense
◦ The articular cartilage remains
viable & becomes thicker than
normal
– increased joint space.
Stage of REVASCULARIZATION / FRAGMENTATION


Ingrowths of highly vascular & cellular connective tissue.



Necrotic trabecular debris is resorbed & replaced by vascular
fibrous tissue
the alternating areas of sclerosis and
fibrosis appear on X- ray as fragmentation of epiphysis.



New immature bone laid on intact
necrosed trabeculae by creeping
substitution
further increases
the density of ossific nucleus on
X-ray.
Stage of REVASCULARIZATION / FRAGMENTATION (contd.)
The femoral head may extrude from acetabulum
at this stage.
Stage of Ossification / Healing
New bone starts forming and epiphyseal
density increases in the lucent portions of
the femoral head.


Remodeling / Residual stage
This is the stage of remodeling and there is no
additional change in the density of the femoral
head.
Depending on the severity of the disease the
residual shape of the head may be spherical
or distorted.
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








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I Sclerotic
A: no loss of height
B: loss of height
II Fragmentation
A: early
B:late
III Healing
A: peripheral
B:>1/3epiphysis
IV Healed
Stages



220 days



240 days



255 days




Stage Ia - the initial stage of the
disease, characterized by sclerosis of the
epiphysis without any loss of epiphyseal
height .
Stage Ib - epiphysis is sclerotic and there is
loss of height of the epiphysis. In this stage
the epiphysis is still in a single piece and no
fragmentation is visible in either
anteroposterior or lateral views .
Stage Iia- epiphysis has just begun to
fragment; one or two vertical fissures in the
epiphysis are seen in either view .
In stage IIb - fragmentation of the epiphysis
is advanced, but there is no evidence of new
bone formation lateral to the fragmented
epiphysis .
In stage IIIa - evidence of new bone formation
at the periphery of the necrotic fr agment;
the new bone is not of normal texture and
covers less than one-third the circumference
of the epiphysis .
In stage IIIb - new bone is of normal texture
and covers more than one-third the
circumference of the epiphysis .
In stage IV the healing is complete and there is
no radiologically identifiable avascular bone




extent of epiphyseal involvement and
percentage of collapse as seen in x-ray (both
AP and Lateral view)
most commonly used
◦ 4 groups based on amount of femoral head
involvement
◦ Also presence of sequestrum, metaphyseal
rxn, subchondral fx


3 groups:
◦ A) no lateral pillar involvment
◦ B) >50% lat height intact
◦ C) <50% lat height intact






Simplification of Catterall
Based on status of lateral margin of capital
femoral epiphysis
Group A (Catterall I & II equivalent)
Group B (Catterall III & IV equivalent)
Clinical

Radiographic
(1)





Progressive loss
of movement
more of
ABduction
Pain

(2)

(3)

(4)
(5)

lateral subluxation of
the femoral head from
the acetabulum,
speckled calcification
lateral to the capital
epiphysis,
diffuse metaphyseal
reaction (metaphyseal
cysts),
a horizontal physis
Gage sign


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

Bilateral









Tuberculosis hip
Synovitis
Slipped femoral capital
epiphysis
Lymphoma
Eosinophilic granuloma

Hypothyroidism



Multiple epiphyseal
dysplasia



Spondyloepiphyseal
dysplasia



Sickle cell disease



60% of kids do well without tx
AGE is key prognostic factor:
◦ <6yo= good outcome regardless of tx
◦ 6-8yo= not always good results with just
containment
◦ >9yo= containment option is questionable, poorer
prognosis, significant residual defect
◦ --Flat femoral head incongruent with acetabulum=
worst prognosis


CONTAINMENT of the femoral head



Minimize enlargement of the femoral head



Prevent or correct GT overgrowth



Prevent secondary degenerative arthritis of
the hip


Weight Relief



Containment by bracing or casting



Surgical Containment



Greater trochanteric arrest







disease progresses and resolves stage
wise, which cannot be bypassed or hurried.
Improve ROM 1st
Bracing:
Removable abduction orthosis
Pietrie cst

-Wean from brace when improved x-ray healing
signs
 Check serial radiographs
◦ Q3-4 mos with ROM testing



Orthotic treatment is discontinued when the
disease enters the reparative phase and healing
is established


Atlanta Scotish Rite
Brace
petrie abduction


The radiographic evidence of healing are

1. Appearance of irregular ossification in the
femoral head.
2 . Increased density of femoral head should
disappear.
3 . Medial segment of femoral head should
increase in size and height.

4 . Metaphyseal rarefaction involving the lateral
cortex of the metaphysis should ossify.
5 . There should be intact lateral column.



If non-op tx cannot maintain containment
Surgically ideal pt:
◦
◦
◦
◦

6-9yo
Catterral II-III
Good ROM
In collapsing phase


Indicator of acetabular depth



It is the angle formed by a




perpendicular line through the
midpoint 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
Surgical Containment

Femoral VDRO
osteotomies

Varus 20
Derotation 20-30

Pelvic

Shelf
Redirectional
Displacement
Contd.


varus osteotomy :◦ INDICATIONS- patients with a spherical femoral head,
little or no acetabular dysplasia (center-edge angle of at
least 15 to 20 degrees),lateral overloading, and a valgus
neck-shaft angle of more than 135 degrees.
◦ DISADVANTAGES-varus angulation that may not correct
with growth (especially in an older child),
◦ further shortening of an already shortened extremity,
◦ the possibility of a gluteus lurch produced by decreasing
the length of the lever arm of the gluteal musculature,
◦ the possibility of nonunion of the osteotomy,
◦ requirement of a second operation to remove the
internal fixation










ADVANTAGE-Anterolateral coverage of the femoral
head, lengthening of the extremity (possibly
shortened by the avascular process), and avoidance
of a second operation for plate removal.
DISADVANTAGES-1)inability sometimes to obtain
proper containment of the femoral head, especially
in older children;
2)an increase in acetabular and hip joint pressure
that may cause further avascular changes in the
femoral head;
3)an increase in leg length on the operated side
compared with the normal side that may cause a
relative adduction of the hip and uncover the
femoral head.
Eg.-Salter’s ostoeotomy


Aims of treatment
Relieve pain
Correct Trendelenburg gait
Minimize the risk of development of
degenerative arthritis


Valgus osteotomy



Joint distraction



Surgical dislocation and
osteochondroplasty



Cheilectomy(Osteochondroplasty)



Arthrodesis


Greater trochanter advancement



Lengthening of the femoral neck



Improving acetabular coverage of the femoral
head by periacetabular osteotomy









Valgus extension osteotomy
indication -hinge abduction of hip
Cheilectomy
indication – malformed femoral head with lateral
protuberance Coxa plana
Chiari osteotomy
indication – malformed femoral head with lateral
subluxation
Trochanteric advancement
indication – premature capital femoral physeal arrest
Greater trochanteric epiphysiodesis
indication – premature capital femoral physeal arrest
Shelf augmentation procedure
indication – coxa magna coxa magna & lack of acetabular
coverage








Patients presenting at 8+yrs
Have a worse prognosis
Severe femoral head deformity more likely
Deformity at maturity predicts outcome
Particularly if Catterall III or IV Or Herring C
(B/C)
Girls have a poorer prognosis







In some patients collapse was more
pronounced in the middle pillar rather than
the lateral.
Neither the Catterall grouping nor the Herring
grading correlated with the final outcome
Osteoporosis
premature fusion of: the triradiate cartilage,
trochanteric growth plate and the capital
femoral growth plate.




The outcome of the disease in adolescents is
poor.
Many of the patients with the destructive
pattern required salvage surgery to relieve
pain. It is likely that patients with the other
patterns of the disease will develop
degenerative changes in due course.
Thank you for attention !

UMY

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Legg-Calvé-Perthes Disease: A Concise Guide

  • 1.
  • 2.  FIRST DESCRIBED BY LEGG (USA), AND WALDENSTORM IN 1909, AND BY PERTHES(GERMANY) AND CALVE(FRANCE) IN 1910
  • 3.       Disorder of the hip in young children Usually ages 4-8yrs As early as 2yrs, as late as teens Boys:Girls= 4-5:1 Bilateral 10-12% No evidence of inheritance
  • 4.    Unknown Past theories: infection, inflammation, trauma, congenital Most current theories involve vascular compromise ◦ Sanches 1973: “second infarction theory”  sometimes called as “coronary artery disease of hip”
  • 5.
  • 6.
  • 7.  Infants 1. Metaphyseal arteries . 2. Lat epiphyseal arteries 3. Lig teres – insignificant  4 mts – 4 years 1. Lat epiphyseal 2. Metaphyseal art. decrease in number (due to appearance of growth plate).
  • 8.  4 yrs to 7 years 1. Epiphyseal plate forms a barrier to metaphyseal vessels.  Pre-adolescent 1. After 7 yrs arteries of lig teres become more prominent and anastomose with the lateral epiphyseal vessels.
  • 9. Susceptible child : delayed bone age -- Trauma -- Hereditary factors : controversial(HLA-A antigens in lymphocytes) -- Coagulopathy : protein c& s -- Hyperactivity -- Passive smoking -- Synovitis FACTORS UNLIKELY TO BE ETIOLOGY-- Endocrinopathy -- Urban envt. --
  • 10.   Histologic changes described by 1913 Secondary ossification center= covered by cartilage of 3 zones: ◦ Superficial ◦ Epiphyseal ◦ Thin cartilage zone  Capillaries penetrate thin zone from below
  • 11.
  • 12.  Epiphyseal cartilage in LCP disease: ◦ Superficial zone is normal but thickened ◦ Middle zone has 1)areas of extreme hypercellularity in clusters and 2)areas of loose fibrocartilaginous matrix   Superficial and middle layers nourished by synovial fluid Deep layer relies on blood supply
  • 13.    Physeal plate: cleft formation, amorphis debris(Bone dust), blood extravasation Metaphyseal region: normal bone separated by cartilaginous matrix Epiphyseal changes can be seen also in greater trochanter, acetabulum
  • 14.     Often insidious onset of a limp,excaberated by activity. C/O pain in groin, thigh, knee Few relate trauma hx Can have an acute onset
  • 15.      Decreased ROM, especially abduction and internal rotation: initially due to muscle spasm Abductor limp Trendelenburg test often positive Muscular atrophy of thigh/buttock/calf Limb length discrepency
  • 16. Coxa magna  Premature physeal growth arrest Central-short neck,trochanteric overgrowth Lateral-externally tilted head trochantric overgrowth acetublar deformity Irregular head Osteochondoirtis dessicans 
  • 17.      AP pelvis Frog leg lateral Key= view films sequentially over course of dz Arthrography MRI role undefined
  • 18.      WALDENSTROM Modified Elizabethtown Classification Catterall classification Salter-Thompson Classification Lateral Pillar Classification
  • 19.  Four Waldenstrom stages: ◦ ◦ ◦ ◦ 1) 2) 3) 4) Initial stage Fragmentation stage Reossification stage Healed stage
  • 20. Stage of Avascular Necrosis Ischemia A part ( anterior) or whole of capital femoral epiphysis is necrosed. On X-ray – ◦ The ossific nucleus looks smaller ◦ Classically of Perthes’, looks dense ◦ The articular cartilage remains viable & becomes thicker than normal – increased joint space.
  • 21. Stage of REVASCULARIZATION / FRAGMENTATION  Ingrowths of highly vascular & cellular connective tissue.  Necrotic trabecular debris is resorbed & replaced by vascular fibrous tissue the alternating areas of sclerosis and fibrosis appear on X- ray as fragmentation of epiphysis.  New immature bone laid on intact necrosed trabeculae by creeping substitution further increases the density of ossific nucleus on X-ray.
  • 22. Stage of REVASCULARIZATION / FRAGMENTATION (contd.) The femoral head may extrude from acetabulum at this stage.
  • 23. Stage of Ossification / Healing New bone starts forming and epiphyseal density increases in the lucent portions of the femoral head.
  • 24.  Remodeling / Residual stage This is the stage of remodeling and there is no additional change in the density of the femoral head. Depending on the severity of the disease the residual shape of the head may be spherical or distorted.
  • 25.           I Sclerotic A: no loss of height B: loss of height II Fragmentation A: early B:late III Healing A: peripheral B:>1/3epiphysis IV Healed Stages  220 days  240 days  255 days
  • 26.   Stage Ia - the initial stage of the disease, characterized by sclerosis of the epiphysis without any loss of epiphyseal height . Stage Ib - epiphysis is sclerotic and there is loss of height of the epiphysis. In this stage the epiphysis is still in a single piece and no fragmentation is visible in either anteroposterior or lateral views .
  • 27. Stage Iia- epiphysis has just begun to fragment; one or two vertical fissures in the epiphysis are seen in either view . In stage IIb - fragmentation of the epiphysis is advanced, but there is no evidence of new bone formation lateral to the fragmented epiphysis .
  • 28. In stage IIIa - evidence of new bone formation at the periphery of the necrotic fr agment; the new bone is not of normal texture and covers less than one-third the circumference of the epiphysis . In stage IIIb - new bone is of normal texture and covers more than one-third the circumference of the epiphysis . In stage IV the healing is complete and there is no radiologically identifiable avascular bone
  • 29.   extent of epiphyseal involvement and percentage of collapse as seen in x-ray (both AP and Lateral view) most commonly used ◦ 4 groups based on amount of femoral head involvement ◦ Also presence of sequestrum, metaphyseal rxn, subchondral fx
  • 30.
  • 31.
  • 32.
  • 33.
  • 34.  3 groups: ◦ A) no lateral pillar involvment ◦ B) >50% lat height intact ◦ C) <50% lat height intact
  • 35.
  • 36.     Simplification of Catterall Based on status of lateral margin of capital femoral epiphysis Group A (Catterall I & II equivalent) Group B (Catterall III & IV equivalent)
  • 37. Clinical Radiographic (1)   Progressive loss of movement more of ABduction Pain (2) (3) (4) (5) lateral subluxation of the femoral head from the acetabulum, speckled calcification lateral to the capital epiphysis, diffuse metaphyseal reaction (metaphyseal cysts), a horizontal physis Gage sign
  • 38.
  • 39.
  • 40.  Rarefaction in the lateral part of the epiphysis and subjacent metaphysis.
  • 41.
  • 42. Unilateral Bilateral       Tuberculosis hip Synovitis Slipped femoral capital epiphysis Lymphoma Eosinophilic granuloma Hypothyroidism  Multiple epiphyseal dysplasia  Spondyloepiphyseal dysplasia  Sickle cell disease
  • 43.   60% of kids do well without tx AGE is key prognostic factor: ◦ <6yo= good outcome regardless of tx ◦ 6-8yo= not always good results with just containment ◦ >9yo= containment option is questionable, poorer prognosis, significant residual defect ◦ --Flat femoral head incongruent with acetabulum= worst prognosis
  • 44.  CONTAINMENT of the femoral head  Minimize enlargement of the femoral head  Prevent or correct GT overgrowth  Prevent secondary degenerative arthritis of the hip
  • 45.  Weight Relief  Containment by bracing or casting  Surgical Containment  Greater trochanteric arrest
  • 46.      disease progresses and resolves stage wise, which cannot be bypassed or hurried. Improve ROM 1st Bracing: Removable abduction orthosis Pietrie cst -Wean from brace when improved x-ray healing signs  Check serial radiographs ◦ Q3-4 mos with ROM testing  Orthotic treatment is discontinued when the disease enters the reparative phase and healing is established
  • 49.  The radiographic evidence of healing are 1. Appearance of irregular ossification in the femoral head. 2 . Increased density of femoral head should disappear. 3 . Medial segment of femoral head should increase in size and height. 4 . Metaphyseal rarefaction involving the lateral cortex of the metaphysis should ossify. 5 . There should be intact lateral column.
  • 50.   If non-op tx cannot maintain containment Surgically ideal pt: ◦ ◦ ◦ ◦ 6-9yo Catterral II-III Good ROM In collapsing phase
  • 51.  Indicator of acetabular depth  It is the angle formed by a   perpendicular line through the midpoint 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
  • 52. Surgical Containment Femoral VDRO osteotomies Varus 20 Derotation 20-30 Pelvic Shelf Redirectional Displacement
  • 53.
  • 54.
  • 55. Contd.  varus osteotomy :◦ INDICATIONS- patients with a spherical femoral head, little or no acetabular dysplasia (center-edge angle of at least 15 to 20 degrees),lateral overloading, and a valgus neck-shaft angle of more than 135 degrees. ◦ DISADVANTAGES-varus angulation that may not correct with growth (especially in an older child), ◦ further shortening of an already shortened extremity, ◦ the possibility of a gluteus lurch produced by decreasing the length of the lever arm of the gluteal musculature, ◦ the possibility of nonunion of the osteotomy, ◦ requirement of a second operation to remove the internal fixation
  • 56.
  • 57.      ADVANTAGE-Anterolateral coverage of the femoral head, lengthening of the extremity (possibly shortened by the avascular process), and avoidance of a second operation for plate removal. DISADVANTAGES-1)inability sometimes to obtain proper containment of the femoral head, especially in older children; 2)an increase in acetabular and hip joint pressure that may cause further avascular changes in the femoral head; 3)an increase in leg length on the operated side compared with the normal side that may cause a relative adduction of the hip and uncover the femoral head. Eg.-Salter’s ostoeotomy
  • 58.
  • 59.
  • 60.
  • 61.
  • 62.
  • 63.
  • 64.
  • 65.
  • 66.  Aims of treatment Relieve pain Correct Trendelenburg gait Minimize the risk of development of degenerative arthritis
  • 67.  Valgus osteotomy  Joint distraction  Surgical dislocation and osteochondroplasty  Cheilectomy(Osteochondroplasty)  Arthrodesis
  • 68.  Greater trochanter advancement  Lengthening of the femoral neck  Improving acetabular coverage of the femoral head by periacetabular osteotomy
  • 69.
  • 70.
  • 71.
  • 72.       Valgus extension osteotomy indication -hinge abduction of hip Cheilectomy indication – malformed femoral head with lateral protuberance Coxa plana Chiari osteotomy indication – malformed femoral head with lateral subluxation Trochanteric advancement indication – premature capital femoral physeal arrest Greater trochanteric epiphysiodesis indication – premature capital femoral physeal arrest Shelf augmentation procedure indication – coxa magna coxa magna & lack of acetabular coverage
  • 73.       Patients presenting at 8+yrs Have a worse prognosis Severe femoral head deformity more likely Deformity at maturity predicts outcome Particularly if Catterall III or IV Or Herring C (B/C) Girls have a poorer prognosis
  • 74.     In some patients collapse was more pronounced in the middle pillar rather than the lateral. Neither the Catterall grouping nor the Herring grading correlated with the final outcome Osteoporosis premature fusion of: the triradiate cartilage, trochanteric growth plate and the capital femoral growth plate.
  • 75.   The outcome of the disease in adolescents is poor. Many of the patients with the destructive pattern required salvage surgery to relieve pain. It is likely that patients with the other patterns of the disease will develop degenerative changes in due course.
  • 76. Thank you for attention ! UMY

Notas do Editor

  1. Susceptible child: Abnormal Growth and Development: The “Predisposed Child”Hyperactivity or Attention Deficit Disorder
  2. Proposed by Benjamin Joseph from India.Deformation of femoral head occurs during the late stage of fragmentation and early stage of revascularization. Hence the surgery for containmentof femoral head can be performed before the late stage of fragmentation
  3. Prevent deformation of the femoral headThis is the most important aim of treatment of Perthes’ disease. In order to plan treatment aimedat preventing this complication it is necessary to understand the pathogenesis of femoral headdeformation. Weight-bearing and muscular contraction produce stresses that are transmittedacross the acetabular margin onto the extruded part of the avascular femoral capital epiphysis.The avascular epiphysis is particularly vulnerable to deformation when subjected to thesestresses. Studies have shown that if extrusion of the femoral head exceeds 20 percent by thetime the disease has progressed to the latter part of the stage of fragmentation there is a highrisk of femoral head deformation3,4 (Figure 68.3). Hence every effort must be made to preventextrusion of the femoral head, and if extrusion does occur it should be corrected before the latterpart of the stage of fragmentation if deformation of the femoral head is to be prevented.8• Minimize enlargement of the femoral headSince the degree of enlargement of the femoral head is related to the severity of deformation ofthe femoral head, intervention that succeeds in preventing femoral head deformation is likely tosucceed also in minimizing the extent of enlargement of the femoral head.9,10• Prevent or correct greater trochanteric overgrowthAlthough the interference with normal femoral capital physeal growth appears to be related tothe severity of the disease, there is no way of identifying which children will develop this