This document summarizes Legg-Calvé-Perthes disease, a childhood hip disorder characterized by temporary loss of blood supply to the femoral head. It describes the disease stages including initial sclerosis, fragmentation as new bone forms, and healing. Risk factors include age 4-8 years and delayed bone age. Symptoms include limping and pain. Treatments aim to contain the femoral head through bracing or surgery to prevent joint damage. Prognosis depends on age and deformity, with older children and severe deformity having worse outcomes.
Presentation contain etiology, blood supply of femoral head & neck,pathogenesis ,classification system ,clinical features,diagnosis,managment, pelvic & femoral osteotomies in detail
Presentation contain etiology, blood supply of femoral head & neck,pathogenesis ,classification system ,clinical features,diagnosis,managment, pelvic & femoral osteotomies in detail
Dr Omar Mohamad M. Abdallah
Legg Calvé perthes
sinal de Caffey
Salter - Thompson: corresponde á extensão da fratura subcondral - sinal de Caffey
Herring - acometimento do pilar lateral
Caterall – Fase tardia - Extensão do envolvimento da cabeça do fêmur
Stulberg - Avalia possibilidade de desenvolvimento de artrose futura
osteotomia de Salter
osteotomia varizante do fêmur proximal
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Legg calve perthes disease-UMY
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
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
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
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
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
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.
Susceptible child: Abnormal Growth and Development: The “Predisposed Child”Hyperactivity or Attention Deficit Disorder
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
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