Perthes disease Legg Calve Perthes Disease LCPD.ppt

Perthes disease - Legg Calve Perthes Disease History, examination, investigation, management

1. Perthes disease

2. Perthes' disease is a condition of the hip which occurs in some children. It was first
described in 1910 by three separate doctors and so it is sometimes called
Legg-Calve-Perthes
Legg-Calvé-Perthes disease (LPD) is a childhood hip disorder that results in infarction of
the bony epiphysis of the femoral head.
LPD represents idiopathic avascular necrosis of the femoral head.

3. Perthes’ disease, also known as Legg-
Calve-Perthes’ disease was described by
Legg---- an American,
Calve --- a Frenchman,
Perthes----German

4. •Occurs in 4-8 yrs ,male ^ ,low ses
•Lbw , associated inguinal hernias ,genitourinary
anomalies
•Family h/o –6%
•Aetiology –unknown
•Prognosis –imp as 50% donot require treatment
•
•The disease is bilateral in 10-20% When both
hips are involved, they are usually affected
successively, not simultaneously
In adults, the corresponding condition is termed Chandler disease

5. The femoral head is spherical and is covered by articular cartilage.
The fovea is a just-off-center ovoid depression that serves as an
attachment for the ligamentum teres (round ligament).
The femoral head is supplied by a vascular ring at the base of the femoral
neck from branches arising from the lateral and medial circumflex arteries.
The superior and inferior gluteals contribute to the blood supply to a
lesser extent. The anterior aspect of the vascular ring is within the joint
capsule.
Principally, the lateral epiphyseal arteries supply the femoral neck.
The artery in the ligamentum teres supplies one third of the arterial supply
to the femoral head in children but makes only a small contribution in
adults.

6. Pathology
The articular cartilage covering the femoral head is
remarkably resistant to ischemia and usually well preserved
ligamentum teres vessels become nonfunctional, rendering the
head at risk for osteonecrosis
The basic underlying cause of LPD is insufficient blood supply to the
femoral head. The epiphyseal plate acts as a barrier to the supply of
blood in children aged 4-10 years, and the ligamentum teres vessels
become nonfunctional, rendering the head at risk for osteonecrosis.
Healing occurs by revascularization of the necrotic femoral head
The course of revascularization and reconstitution of the femoral head varies

7. The ultimate shape of the reconstituted femoral head depends on several factors,
including the degree and site of necrosis and the magnitude of forces working
across the femoral head
In some patients, the femoral head may return to normal, while in other patients,
coxa plana and shortening and widening of the femoral head may develop.
These changes may be associated with osteoarthrosis and intra-articular loose
bodies
In most patients, the changes occurring in LPD affect only 1 hip. With bilateral
disease, the changes are rarely symmetric.

8. Clinical Details
LPD is a dynamic condition, and results of the physical examination depend on the
stage of the disease at the time of presentation
The child often has a limp with groin, thigh, or knee pain.
Children who present with knee pain must be carefully examined for hip pathology
As the disease progresses, flexion and adduction contractures may develop, and
lateral overgrowth of the femoral head cartilage may cause loss of abduction
Attempts at abduction lead to hinging and possible subluxation of the femoral head.
Eventually, the hip may move in only the flexion-extension plane. Progressive loss of
movement, adduction contractures, flexion with abduction, and obesity are poor
prognostic signs
Lateral subluxation of the femoral head is also associated with a poor outcome

9. The natural history of Perthes’ disease
many children have a good outcome without
any active treatment
In the absence of any foolproof test to distinguish
those that need treatment from those that do not,
the doctor often finds himself in a dilemma.
there are certain factors that need to be considered
when deciding treatment.
If the onset of the disease is before age 4, results tend to be
good, with or without treatment.
The older the child at the onset of disease, the less
favorable the result

10. Is there hip stiffness?
If the doctor discovers stiffness on examination, especially tight hip
adductors (where the leg cannot be brought out away from the
midline),
the results are not favorable.
In these instances, the patient is usually admitted and placed on
traction and physical therapy to relieve the tightness.
Sometimes, surgery (hip adductor release) is performed to correct
the tightness.

11. Complications:
Osteoarthritis, or degenerative arthritis in adulthood are the most common complications
resulting from Legg-Calve-Perthes disease.
The risk for degenerative arthritis in adulthood correlates with the restoration of the
sphericity of the femoral head at the resolution of disease.
Older children with residual femoral head deformity are at the greatest risk of
developing degenerative arthritis in adulthood.

12. X-rays of the hips show partial or total head involvement?
If the whole head of the femur is involved, the prognosis is less favorable.
If only part of the head is involved, and the lateral part of the head is not
involved, risk of collapse is much less, and long-term results tend to be better.

If X-rays show that the head is well-contained in the acetabulum, results tend
to be good.
If the head is too large for the acetabulum, or if part of the head extrudes from
under the cup, results tend to be poor.

13. The Catterall classification is based on radiographic appearances and
specifies 4 groups during the period of greatest bone loss
.
Catterall staging is as follows:
Stage I - Histologic and clinical diagnosis without radiographic findings
Stage II - Sclerosis with or without cystic changes with preservation of the
contour and surface of femoral head
Stage III - Loss of structural integrity of the femoral head
Stage IV - Loss of structural integrity of the acetabulum in addition
Several staging schema are used to determine severity of disease and prognosis;
these include the Catterall, Salter-Thomson, and Herring systems.

14. •Salter-Thomson
classification
•The Herring classification
•addresses the integrity of
the lateral pillar of the
head.
group A, includes Catterall groups I
and II and indicates that less than 50%
of the head is involved. –better
prognosis
group B, includes Catterall groups III
and IV and is used when more than
50% of the head is involved. –bad
prognosis
In Lateral Pillar group A, there is no
loss of height in the lateral one third of
the head and little density change
In Lateral Pillar group B, there is a
lucency and loss of height of less
than 50% of the lateral height.
Sometimes, the head is beginning to
extrude the socket.
In Lateral Pillar group C, there is
more than 50% loss of lateral height

16. Stage I - Histologic and
clinical diagnosis
without radiographic
findings
Stage II - Sclerosis with or
without cystic changes with
preservation of the contour
and surface of femoral head
Stage III - Loss of structural
integrity of the femoral head
Stage IV - Loss of structural
integrity of the acetabulum in
addition

17. Plain radiography remains the major modality for the evaluation of
LPD. Staging of the disease is based on plain radiographic findings
Plain radiograph findings may be entirely normal in early symptomatic disease.
Although abnormal scintigraphic findings antedate abnormal radiograph findings,
they are nonspecific, and findings may be positive in patients with trauma,
synovitis, and infections.

18. The diagnosis of Legg-Calve-Perthes disease is essentially a radiological one.
AP and frog-leg lateral films of the hips should be taken and evaluated carefully, as
LCPD is easily missed.
Radiologic films can show:
Early disease can present with devascularization around the capital femoral epiphysis
Mild disease can present with fragmentation and collapse of the CFE
Moderate disease shows reossification
Resolving disease can show remodeling

19. Radiograph of the hip.
X-ray pictures can show a femoral head which is broken or damaged.
A typical appearance is a 'flattened' femoral head when it should normally look
rounded in the hip socket
. However, the X-ray can be normal in the early stages of the disease before the
'softened' bone breaks.
An X-ray every few months can show the progress of the breakdown, and then
healing as new fresh bone is made and gradually 'remodelled'.

Bone scan. This may be done to confirm Perthes' disease in the early stages when
the X-ray picture is normal, but symptoms suggest that Perthes' disease is the
problem.

An MRI scan or other tests are sometimes used to asses the extent of the damage

20. • Findings
•Early signs on radiographs include the following:
•Small femoral epiphysis (96%)
•Sclerosis of the femoral head with sequestration
and collapse (82%)
•Slight widening of the joint space due to thickening
of the cartilage, failure of epiphyseal growth, the
presence of joint fluid, or joint laxity (60%)
•No destruction of the articular cortex as in
bacterial arthritis (destruction of articular cartilage
never occurs in LPD)

21. •Late signs on radiographs include the following:
•Delayed osseous maturation of a mild degree, a
radiolucent crescent line representing a subchondral
fracture
•
•Femoral head fragmentation and femoral neck cysts from
intramedullary hemorrhage or extension of physeal
cartilage into metaphysis, loose bodies, and coxa plana
•Coxa magna, or remodeling of the femoral head, which
becomes wider and flatter and similar in appearance to a
mushroom
•Degree of Confidence
•Plain radiographs have a sensitivity of 97% and a
specificity of 78% in the detection of LPD.
•False Positives/Negatives
•Severe osteoarthritis and infective arthritis may mimic
LPD.

22. Legg-Calvé-Perthes disease. Stage II disease. Note the slight widening of the left
hip joint representing a small joint effusion (see also Image 2). Joint widening can
also be secondary to hypertrophy of the cartilage
Widening of
joint space s

23. Legg-Calvé-Perthes disease. The left subchondral radiolucency is more
readily demonstrated on a frog-leg view and represents subchondral fracture
Subchondr
al fracture

24. Legg-Calvé-Perthes disease. Image shows left femoral subchondral
sclerosis and radiolucency
Sclerosis
+radiolucenc
y

25. Image shows left femoral subchondral sclerosis and radiolucency (see

26. flattening and early fragmentation of the left femoral head with the presence
of femoral neck cysts. The femoral head is obviously smaller on the left than
the right.

27. fragmentation of the left femoral head. Also note widening and shortening of the
left femoral neck.

28. loss of structural integrity of the right femoral head. Also note lateral
extrusion of the right femoral head.

29. loss of structural integrity of the left femoral head. Also note the widening and
shortening of the femoral neck, and more importantly, the severe subluxation.

30. Plain radiograph in an adult patient with residual coxa magna and plana deformity
with superimposed joint changes

31. Ultrasonography
1)useful in establishing the diagnosis of transient synovitis of the hip and the
onset of LPD.
2)Hip effusion, which results in capsular distension, is
accurately documented on sonograms
3) It allows aspiration of joint fluid for laboratory
examination
4)Sonography-guided aspiration allows the selection of only
those patients with septic arthritis for surgical drainage and
shortens the procedure.
5) Negative sonographic findings allow the exclusion of
septic arthritis but not osteomyelitis

32. Capsular distension lasting longer than 6 weeks is associated with
LPD.
A chronologic, 4-part staging of LPD has been proposed on the
basis of the sonographic findings.
The stages reflect the degree of flattening and fragmentation and
the reconstitution of the femoral head.
Thickening of articular cartilage, associated synovitis, and lateral
extrusion of the femoral head can be documented.
Joint effusion is present in 74% of patients in stages I-II.
Lateral extrusion increases from stage II onwards until the healing
stage.

33. Degree of Confidence
Although not performed routinely, sonographic evaluation of patients with LPD
1) is a simple and standardized procedure that can be useful for staging the
disease and monitoring its course.
2) It can also spare the patient from radiation exposure and lower treatment
costs.

3) Lateral extrusion and the onset of healing in patients with LPD can be
shown earlier with sonograms than with radiographs.
False Positives/Negatives
Changes similar to those of LPD can be found in transient synovitis and other
causes of hip joint effusions.
Joint effusion is not always present in patients with LDP.

34. Ultrasound 1: Hip sonography, ventral. Irregular contours, right femoral
head with irregular echo pattern with areas of high echogenicity and low
echogenicity. Femoral head is altogether lower in height. Epiphyseal gap
is fuzzy with increased echogenicity. Slight joint effusion to the right.

35. Ultrasound 2: Hip sonography, ventral. Right and left sides for
comparison.

36. Findings
Early signs on CT scans include the following:
Bone collapse
Curvilinear zones of sclerosis
Subtle changes in bone trabecular pattern

Disruption of an area of condensation of bone formed by a
compressive group of trabeculae (abnormal asterisk sign)

37. Late signs on CT scans include the following:
Central or peripheral areas of decreased attenuation
Intraosseous cysts
Coronal reconstructions can show subchondral fractures, subtle buckling, or
collapse of the articular surface.
Degree of Confidence
With CT scans, the staging determined by using plain radiographic findings
is upgraded in 30% of patients.
CT is not as sensitive as nuclear medicine or MRI.
CT may be used for follow-up imaging in patients with LPD.
False Positives/Negatives
CT findings of osteoarthritis and infective arthritis may mimic those of LPD.

38. Axial nonenhanced CT scan through the hip joints in the same patient , clearly
shows the loss of structural integrity of the right femoral head.

39. Nonenhanced axial CT section through the hip joints obtained at a different level
in the same patient. Scan once again shows the loss of structural integrity of the
right femoral head. Note the acetabular subchondral sclerosis

40. Coronal reconstruction shows flattening, sclerosis, and early fragmentation
of the right femoral head

41. Findings mri
Early in the disease, irregular low signal intensity foci or linear
segments replace the normal high signal intensity of bone
marrow in the femoral epiphysis on T1- and T2-weighted images.
Other findings include
1) an intra-articular effusion and a small laterally displaced
ossification nucleus, labral inversion, and femoral head
deformity.

Fat-suppressed or short-tau inversion recovery (STIR) sequences
are more accurate than plain radiographs in showing
degenerative changes of the articular cartilage.
These MRIs demonstrate the influx of fluid into areas of
articular cartilage irregularity.

42. The asterisk sign
1)is defined as replacement of normal marrow signal intensity
with low signal intensity on T1-weighted images and high
signal intensity on T2-weighted images.
2) hypo –T1
3) hyperintense—T2
4)The double-line sign occurs in as many as 80% of patients and
represents the sclerotic rim, which appears as a signal void.
This sign is demonstrated as a line between necrotic and viable
bone edges with a hyperintense rim of granulation tissue.

43. Degree of Confidence
MRI is as sensitive as isotopic bone scanning, and it allows more
precise localization of involvement than conventional radiography.
MRI is preferred for evaluating the position, form, and size of the
femoral head and surrounding soft tissues.
The differential diagnosis includes
severe osteoarthritis
infective arthritis
other causes of bone marrow edema and joint effusions

44. Coronal T2-weighted MRIs show irregularity and flattening of cortical margins of the left
femoral epiphysis. Also note a mild joint effusion and subluxation and hinge deformity of
the left

45. Coronal T1-weighted MRIs show the loss of normal high signal intensity in left
femoral epiphysis, which now has low signal intensity.

46. Axial T1-weighted MRIs through the femoral heads show low signal
intensity in the left femoral head.

47. Technetium-99m diphosphonate uptake depends on the stage of the disease, but
it does play a role in the diagnosis
.1) Characteristic features include a photopenic void in proximal femoral epiphyses,
when compared with the contralateral side, which usually can be seen by using a
pinhole camera with the hip in maximal medial rotation, obviating single-photon
emission CT (SPECT).
Scintigraphy may be helpful in early diagnosis.
Initially, uptake is decreased in the femoral head due to an interruption in the blood
supply.
Later, uptake is increased in the femoral head due to revascularization, bone repair,
and degenerative osteoarthritis. In addition, acetabular activity can be increased with
associated joint disease.
Degree of Confidence
The sensitivity of radionuclide scanning in the diagnosis of LPD is 98%, and the
specificity is 95%.
False Positives/Negatives
Similar activity patterns may occur with osteoarthritis or infective or inflammatory
arthritis. The presence of a large joint effusion can simulate diminished perfusion due
to osteonecrosis.

48. technetium-99m diphosphonate bone scan shows a photon-deficient defect in
right femoral head.

49. Zoomed images of the same patient show the photopenic defect more clearly

50. Radiograph obtained at same time as the radionuclide scans illustrates the
usefulness of isotope bone scans. The radiographic changes of subchondral
lucency are subtle.

51. Coronal T2-weighted MRI shows irregularity and flattening of cortical margins of
the right femoral epiphysis and the loss of normal signal intensity. Also, note a
mild effusion in the joint

52. MRI and conventional radiography in two planes are considered the most
important methods of investigation for early diagnosis and for assessment of
the course of the disease
. MRI can reveal
1) the early marrow oedema, thus allowing early differential diagnosis
against diseases that are similar in clinical appearance (coxitis fugax,
epiphyseal dysplasia).
2) The extent of the necrotic area within the epiphysis, the most important
indicator of the prognosis of the disease and thus for the therapeutic
management, can be assessed earlier and more reliably with MRI than with
other techniques.
3) The loss of containment can be visualized because depiction of the
cartilaginous structures is possible earlier than with conventional
radiography.
4) Staging of LCPD is also possible with MRI, especially in stages I and II.
Radiography shows the reossification and the osseous remodelling of
the epiphysis better.
A disadvantage of MRI seems to be the occasional need for sedation or
anaesthesia of the child to avoid motion artefacts.

53. •Meyer dysplasias are simple
developmental disorder of hip manifested by delayed
irregular ossification of the femoral epiphyseal
•Nucleus .it is seen 2
nd
decade of life disappears by 6 yrs
without any treatment
MEYER dysplasia is a symptomless developmental disorder of the hip
manifested by delayed, irregular ossification of the femoral epiphysial nucleus
.1 The dysplasia is noted during the second year of life and usually disappears
by the end of the sixth year without treatment
.2 This rare condition may easily be mistaken for other hip problems, leading to
unnecessary diagnostic procedures and treatments.

54. Anteroposterior view of hip joint, demonstrating the various appearances of Meyer dysplasia. A, Patient 1,
demonstrating radiolucent defect in the femoral head. B, Patient 2, showing flattening, sclerosis, and a
radiolucent focus in the femoral head. C, Patient 4, demonstrating fragmentation of the femoral head.

55. The initial diagnosis in patients 1 and 2 was acute osteomyelitis, based on the clinical
presentation and the presence of an elevated sedimentation rate with leukocytosis and
radiolucent lesions of the femoral head.
However, the normal findings on bone scan ruled out this possibility and supported the
diagnosis of Meyer dysplasia.
The 3 other patients (patients 3, 4, and 5) were first suspected to have Perthes
disease, based on the clinical presentation, lack of signs of infection, and the
presence of epiphysial changes on the hip radiographs.
Again, normal bone scan findings excluded this diagnosis. Because of a family history
of Perthes disease in patient 4, magnetic resonance imaging was performed, revealing
multiple centers of ossification of the femoral heads.
The normal bone marrow intensity in all sequences ruled out edema and ischemia
and confirmed the diagnosis of Meyer dysplasia (

56. Magnetic resonance imaging scan, T2-weighted coronal cut of the hip joints,
demonstrating multiple centers of ossification of the femoral heads. The bone
marrow shows normal signal intensity.

57. In the past,
children with Perthes’ disease were treated
1) with non-weight bearing. They were placed on prolonged bed-rest and cructches,
since the thinking was that weight bearing causes the head collapse. This thinking is
no longer valid.
2) The contemporary concept is "containment" of the head in the
acetabulum, which can be achieved by use of casting and bracing, or surgery.
Examples of casting and bracing are
the Petrie cast and the Scottish-Rite (Atlanta) brace.
These are usually worn for a period of 18 to 24 months.
Femoral osteotomy
involves cutting the femur just below the hip, and redirecting the head into the
acetabulum.
The osteotomy is stabilized with surgical hardware, usually plate and screws.
Pelvic osteotomy involves cutting the pelvic bone adjacent to the acetabulum, and
redirecting the acetabulum to contain the head.

58. Arthrography of the hip is important in tailoring treatment
Hip arthrography in children is used
1)for dynamic imaging to determine congruency and containment of the
femoral head by the acetabulum
2)to rule out "hinge" abduction Hinge abduction means failure of the femoral
head to move medially in abduction, with blocking of the severe flattened
femoral head on the lateral acetabular margin
primary goal in treatment
1)is to improve containment and joint congruity ,information obtained from
hip arthrography may help in selecting candidates for periacetabular and
intertrochanteric osteotomy Detection of hinge abduction is of paramount
importance.
2) Hinging must be relieved to assure normal femoral development, which
requires contact between the femoral head and the normally formed
acetabulum. This relief can frequently be accomplished by appropriate
surgery
3)arthrography allows assessment of joint congruity when immobilization
during the process of epiphyseal healing is used as treatment

59. MR imaging is a valuable tool in the diagnosis of early or radiographically occult
Legg-Calvé-Perthes disease.
It shows the extent of epiphyseal involvement
However, dynamic examination of the hip with MR imaging is hampered by the
closed architecture of conventional MR systems.
With the increasing availability of open-configuration MR systems, dynamic
imaging of joints in various positions has become feasible.

60. 7-year-old boy with Legg-Calvé-Perthes Catterall [6] group III disease. Arthrogram (A)
and dynamic T2-weighted fast spin-echo MR image (TR/TE, 5000/133)
(B) taken in neutral position show overgrowth of femoral head and aspheric flattened
subluxated femoral head.
Note pooling of contrast material and joint fluid (arrow), respectively, indicating
incongruency of articular surfaces.