2. HISTORY
Jacques Calve France
1875 -1954
Arthur Legg USA
1874 - 1939
George Perthes Germany
1869 - 1927
3. DEFINITION
A self limiting non-inflammatory condition, affecting
the capital femoral epiphysis with stages of
degeneration and regeneration, leading to restoration
of the bone nucleus.
Osteonecrosis of the proximal femoral epiphysis in a
growing child caused by poorly understood non
genetic factors
4. Epidemiology
Usually ages 4 – 8 years
As early as 2 years, as late as teens
Boys:Girls 4 : 1
Bilateral 10 - 12%
No evidence of inheritance ?? 10%
5. Etiology
Unknown
Past theories:
-infection, inflammation,
-trauma,
-hereditary, environmental,
-predisposed child, attention deficit disorder.
Most current theories involve vascular compromise
-Coagulation abnormalities
-Sanches : “second infarction theory”
7. Pathogenesis
Histological changes described by 1913
Secondary ossification center= covered by cartilage of
3 zones:
Superficial
Epiphyseal
Thin cartilage zone
Capillaries penetrate thin zone from below
9. Pathological stages
Stage 1, ischaemia:
-dead trabecular bone
-collapsed trabecular bone
-thickened articular cartilage
-physeal disruption
-cartilage extending from the physis into the
metaphysis
10. Pathological stages
Stage 2, resorption, fragmentation, re-
vascularisation, and repair:
-invasion of vascular granulation tissue
-new bone forming on dead trabeculae
-woven new bone formation
Stage 3, re-ossification and resolution:
-new bone, woven and lamellar
Stage 4, re-modelling:
-return to normal architecture
13. Presentation
Often insidious onset of a limp
Pain in groin, thigh, knee
17% relate trauma history
Can have an acute onset
14. Physical Exam
Decreased ROM, especially abduction and internal
rotation
Trendelenburg test often positive
Adductor contracture (due to long standing spasm)
Muscular atrophy of thigh/buttock/calf
Limb length discrepency (due to head collapse)
15. Differential Diagnosis
CAUSES OF AVASCULAR NECROSIS
- sickle cell disease
- thalassemia
- other hemoglobinopathies
- steroid medication
- after traumatic hip disloction
EPIPHYSEAL DYSLASIAS
- multiple epiphyseal dysplasia
- spondyloepiphyseal dysplasia
17. Imaging
AP pelvis
Frog leg lateral
Key = view films
sequentially over
the course of
disease
18. CLASSIFICATION
The classifications for LCPD can be divided into
- the one that defines the stage of the disease and
- the ones used to prognosticate outcome.
Waldenstrom’s radiographic classification defines 4
stages of LCPD during the active phase of the disease
19. CLASSIFICATION
Three radiographic classification systems, namely
- the Catterall,
- Salter-Thompson, and
- lateral pillar,
have been developed as prognosticators of outcome that
are to be applied at the stage of fragmentation.
21. Initial Stage
Early radiographic signs:
Failure of femoral ossific nucleus to grow
"Waldenstrom's sign" (increased joint space and
apparent mild pseudosubluxation)
a subchondral fracture may be seen in the early stages in
the infracted area (crescent, Salter's or Caffrey's sign).
Irregular physeal plate
Blurry/ radiolucent metaphysis
23. Fragmentation Stage
Bony epiphysis begins to fragment
Areas of increased lucency and density
osteolysis of the superolateral portion of the femoral
head (Gage sign on x-ray)
Evidence of repair aspects of disease
35. Salter-Thompson Classification
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)
36. Lateral Pillar Classification
(Herring Classification)
3 groups:
A) no lateral pillar
involvment
B) >50% lat height
intact
C) <50% lat height
intact
38. Stulberg Classification
Class 1
completely normal
Class 2
Spherical head with enlargement, short neck, or steep
acetabulum
Class 3
Non-spherical head (ovoid, mushroom, or umbrella
shaped)
Class 4
Flat femoral head, flat acetabulum
Class 5
Flat femoral head, round acetabulum
39. Mose Classification (1980)
A radiolucent template, with concentric circles, is
placed over the femoral head on AP and lateral
radiographs.
-If the femoral head spheroid does not deviate more
that 1 mm from the template, the result is considered
as good.
-On the other hand, femoral heads deviating within 2
mm or more than 3 mm from the template are
considered to be fair and poor, respectively.
41. Magnetic Resonance Imaging
(MRI) with conventional sequencing is used
- to assess the extent of femoral head infarction.
- provide a good anatomic picture of the cartilaginous
femoral head including flat or round shape,
- the degree of extrusion of the femoral head,
- the degree of superolateral displacement of the
femoral head (subluxation),
- The eversion of the labrum, and the extent of
necrosis.
42. ARTHROGRAPHY
may be used to evaluate possible methods of
treatment.
opportunity to evaluate coverage and mobility under
direct vision during fluoroscopy
helpful in assessing containability before any
treatment is started.
can help identify the best position for femoral head
containment and
demonstrate absence of hinge abduction prior to
containment surgery.
43. Laredo arthrographic classification
identifies 5 types of hip;
Type 1 hips , are normal.
Type 2 hips, the femoral head is still spherical but is
larger than normal.
Type 3 hips, the femoral head is ovoid in shape.
Type 4 hips have a large and flattened femoral head,
and the labrum loses its concavivity.
Moreover, hinge abduction is present.
Type 5 hips show a femoral head larger than normal
and saddle shaped; the labrum is still elevated
44. Computed Tomography
Allow early diagnosis of bone collapse, curvilinear
zones of sclerosis,
Identify intraosseous cysts in later stages of LCP
disease.
Moreover CT provides precise information about the
anatomic relationship between femoral head and
acetabulum.
Allow study of the 3-dimensional nature of the
deformity.
45. Bone Scintigraphy
Precedes radiographic changes by an average of 3
months.
Does not describe the extent of femoral head
involvement, rather
The scintigraphic patterns are associated with the
revascularization versus recanalization process.
46. PROGNOSIS
60% of kids do well without Rx
AGE is key prognostic factor:
<6y = good outcome regardless of Rx
6-8y = not always good results with just containment
>9y = containment option is questionable, poorer
prognosis, significant residual defect
47. Poor Prognosis
Sex
Bone age
Uncovering of Femur head
Percentage involvement
Adduction contracture
Weight
48. Catterall’s Head at risk signs
Along with his classification system, Catterall also
described head-at-risk signs associated with a poor
outcome.
Lateral subluxation of the head
Whole of the head involved
Calcification lateral to epiphysis
Metaphyseal cysts
Gage’s sign
Horizontal physis
49. Management of Perthes Disease
The primary long-term goal of treatment of Legg-
Calve´ -Perthes disease is to try to prevent secondary
degenerative arthritis of the hip in adult life by
achieving the short-term goal.
The primary short-term goal of treatment of Legg-
Calve´ -Perthes disease is to try to ensure that when
the disease is completely healed the femoral head is
spherical, and minimally enlarged.
50. TIME FRAMES
The treatment of Legg-Calve´ -Perthes disease needs
to be divided into 3 distinct time frames:
a.) Early in the course of the disease: from the onset of
the disease to the early fragmentation stage
b.) Late in the course of the disease: from the late
fragmentation stage to full reossification of the
femoral head (complete healing)
c.) After complete healing: after the disease has healed
and residual sequelae are present
51. TREATMENT EARLY IN THE COURSE
OF THE DISEASE
The goal of treatment early in the course of the disease
is to retain the normal shape of the femoral head by:
a.) Identifying patients at risk for a poor outcome as
soon as possible
b.) Containing the femoral head as early as possible in
patients at risk of a poor outcome
52. TREATMENT EARLY IN THE COURSE
OF THE DISEASE
Containment may be achieved by nonoperative or
operative means and surgical options include femoral
and /or pelvic surgery
Containment may or may not be combined with
weight relief
In order for containment to be successful, it should be
achieved before the late stage of fragmentation
Containment should be maintained until the late
reconstitution (reossification) stage
53. METHODS OF CONTAINMENT
Bed rest and range of motion exercises
Casts and Bracing:
Removable abduction orthosis
Pietrie casts
Hips abducted and internally rotated
56. CONTAINMENT BY SALTER
OSTEOTOMY
The Salter osteotomy is a transverse osteotomy of the
pelvis along a line from the sciatic notch to just above
the anterior inferior iliac spine.
The acetabulum is then rotated laterally and anteriorly
using the pubic symphysis as a hinge.
58. CONTAINMENT BY SHELF
PROCEDURE
Shelf acetabuloplasty is a procedure where the margin
of the acetabulum is extended to provide more
coverage for the femoral head
One advantage of the shelf acetabuloplasty is
preservation of limb length without excessive
trochanteric prominence.
Another advantage is long-term improved coverage of
the enlarged femoral head that develops following
Perthes disease.
59. CONTAINMENT BY TRIPLE PELVIC
OSTEOTOMY
The triple pelvic osteotomy combines
- the transverse osteotomy of Salter with
- complete osteotomies of the superior pubic ramus
and ischium.
This allows greater mobility of the acetabulum without
interfering with growth of the triradiate cartilage.
61. TREATMENT EARLY IN THE COURSE
OF THE DISEASE
The decision to consider containment treatment early in
the course of the disease is primarily governed by the
age of onset of the disease with patients divided into 4
age groups.
- < 5 yrs
- 5 to 8 yrs
- 8 to 12 yrs
- > 12 yrs
62. TREATMENT EARLY IN THE COURSE
OF THE DISEASE
Children less than 5 years of age at the onset of the
disease:
treatment is seldom needed regardless of severity of
involvement of the femoral head.
(However, if femoral head extrusion occurs treatment
will be needed)
63. TREATMENT EARLY IN THE COURSE
OF THE DISEASE
Children 5 years or older but less than 8 years of age:
a. Early containment is indicated if it is possible to
determine that more than half the femoral epiphysis is
necrotic.
b. Early determination cannot be made in most patients.
These patients should be monitored with periodic
(4-monthly) radiographs to detect early extrusion of
the femoral head.
64. TREATMENT EARLY IN THE COURSE
OF THE DISEASE
c. Containment treatment should be considered as soon
as extrusion of the femoral head is detected.
d. Extrusion is determined by a break in the Shenton
line.
e. No containment is needed in this age group when
extrusion does not occur
65. TREATMENT EARLY IN THE COURSE
OF THE DISEASE
Children >8 years but < 12 years of age:
a. Should be treated by containment as soon as the
disease is diagnosed regardless of the extent of
necrosis. Containment should be initiated before the
fragmentation stage and before extrusion whenever
possible
b. Alternative methods should be considered when the
patient presents in the late stage of fragmentation
66. TREATMENT EARLY IN THE COURSE
OF THE DISEASE
Children 12 years of age or older at the onset of the
disease:
Containment should NOT be considered in these
adolescents as it does not work.
Treatment considerations should be similar to
treatment of adults with osteonecrosis
67. TREATMENT LATE IN THE COURSE
OF THE DISEASE
The goal of treatment of Legg-Calve´ - Perthes late in
the course of the disease is to attempt to minimize the
extent of deformation of the femoral head that has
already developed.
The treatment in the late fragmentation stage may be
remedial or salvage depending on
- the deformity of the femoral head or
- the presence of hinge abduction
68. TREATMENT LATE IN THE COURSE
OF THE DISEASE
In children who have hinge abduction the goal of
treatment is
-to correct hinge abduction and
-facilitate some remodeling of the femoral head.
Containment may be considered if the femoral head
can be contained without hinge abduction.
If hinge abduction is present. A valgus femoral
osteotomy is a reliable choice to improve motion and
reduce pain.
69. TREATMENT AFTER HEALING OF
THE DISEASE
The goals of treatment of adolescents or young adults
with healed Legg-Calve´ - Perthes disease and
deformity of the femoral head is to
- improve function,
- relive pain, and
- delay the onset of secondary degenerative arthritis
The treatment approach depends on the specific cause
of pain, dysfunction, or deformity
70. TREATMENT AFTER HEALING OF
THE DISEASE
If the femoral head is spherical or ovoid and there is
coxa brevis with a Trendelenburg gait, consider
trochanteric advancement with or without
lengthening the femoral neck
If there is pain on account of femoro-acetabular
impingement consider repairing the labral pathology
and/or correcting impingement
72. TREATMENT AFTER HEALING OF
THE DISEASE
A deficient acetabular roof may require
- labral support or
- pelvic osteotomy with or without realignment of the
proximal femur
Symptoms caused by osteochondritis dessicans can
sometimes be relieved by removing the loose fragment
73. TREATMENT AFTER HEALING OF
THE DISEASE
The role of reshaping a grossly deformed femoral head
is uncertain although in a few selected cases of
moderate deformity, it may be of benefit
-Femoral head reduction osteotomy done for coxa
megna.
75. TREATMENT AFTER HEALING OF
THE DISEASE
When the articular surface is severely damaged salvage
procedures such a total hip replacement should be
considered
76. ROLE OF CORE DECOMPRESSION
May be a useful adjunct to current methods of
treatment.
Younger children with early stage disease may benefit
from multiple small drillings,
Largecore decompression, debridement, and grafting
for Perthes disease in older children with larger lesions
and more advanced stages of disease.
schwartz-jampel syndrome is a autosomal recessive disorder of 2 types. Femoral epiphyseal dysplasia is a part of this.
1 n 2 good prognosis.3 n 4 chances of mild to moderate arthritis5 developpainfull arthritis
Atlanta scottish rite brace
epiphysealavascular necrosis of the proximal femoral physis causes an alteration of the longitudinal growth of the femoral neck that allows for a relative “greater trochanteric overgrowth.” This results in a foreshortened femoral neck termed coxabrevis
