LEGG-CALVÉ-

PERTHES DISEASE
MODERATORS : DR. J. VENKATESHWARLU ( PROFESSOR & HEAD OF DEPARTMENT )
DR. B. VISHWANATH NAIK ( ASSOCIATE PROFESSOR )
DR. RAM MOHAN ( ASSISTANT PROFESSOR )
DR. SIRISH ( ASSISTANT PROFESSOR )
DR. KESHAV ( ASSISTANT PROFESSOR )

PRESENTED BY
DR. SAMUAL SHELSON P H
1ST YR ORTHO POSTGRADUATE
 DEFINITION
• LEGG-CALVÉ-PERTHES DISEASE(LCPD) IS A SELF-LIMITING DISEASE
OF HIP IN CHILDREN PRODUCED BY VARYING DEGREES OF
IDIOPATHIC OSTEONECROSIS OF THE CAPITAL FEMORAL
EPIPHYSIS AND OFTEN THE MOST PROXIMAL PART OF THE
METAPHYSIS.
• THE CAUSE OF LCPD REMAINS UNKNOWN. LCPD HAS BEEN
THOUGHT TO BE AN INFLAMMATORY DISEASE, SECONDARY TO
TRAUMA OR A DEVELOPMENTAL DISORDER OR TOXIC SYNOVITIS
 SYNONYMS
• MORBUS LEGG-CALVÉ-PERTHES,
• COXA PLANA ,
• PSEUDO-COXALGIA (CALVE),
• ISCHEMIC NECROSIS OF THE HIP,
• JUVENILE/IDIOPATHIC AVASCULAR NECROSIS OF THE HEAD OF
THE FEMUR
• PRECOXALGIA ,
• COXA VARA CAPITALIS (LEVY),
• OSTEOCHONDRITIS COXAE JUVENILIS.
 EPIDEMIOLOGY
• MOST COMMON - 4 TO 8 YEAR
• BUT HAS BEEN REPORTED IN CHILDREN AS YOUNG AS 2 YEARS OF AGE
• BOYS > GIRLS BY A RATIO OF 4:1
• THE INCIDENCE OF BILATERALNESS - 10 TO 12%.
• POSITIVE FAMILY HISTORY OF ABOUT 10%.
• HIGH ASSOCIATION OF ABNORMAL BIRTH PRESENTATION, SUCH AS BREECH
OR TRANSVERSE LIE.
• THERE ARE ALSO RACIAL AND ETHNIC FACTORS - MORE COMMON IN
JAPANESE, ESKIMOS, AND CENTRAL EUROPEANS AND UNCOMMON IN NATIVE
AUSTRALIANS , AMERICAN INDIANS, AND BLACKS.
 ETIOLOGY
• INFLAMMATORY DISEASE
• SECONDARY TO TRAUMA OR A DEVELOPMENTAL DISORDER.
• TOXIC SYNOVITIS
• 3RD BORN OR LATER CHILD OF OLDER THAN AVERAGE PARENTS.
• EXPOSURE TO SMOKE
• FACTOR V LEIDEN DEFICIENCY.
• LOCAL ANATOMICAL ABNORMALITIES—THERE IS FINDING OF INCREASED
ANTEVERSION AT THE AFFECTED HIP.
• ASSOCIATIONS OF INGUINAL HERNIA, UNDESCENDED TESTIS, RENAL
ABNORMALITIES (FEMALES WITH BILATERAL AFFECTION), PYLORIC STENOSIS,
CONGENITAL HEART DISEASE, IN SOME CASES.
PROPOSED THEORIES
• INHERITED PROTEIN C AND/OR S DEFICIENCY.
• VENOUS THROMBOSIS.
• ARTERIAL OCCLUSION / ANOMALIES.
• RAISED INTRA OSSEOUS PRESSURE.
• SYNOVITIS OF HIP JOINT.
• GENERALIZED SKELETAL DISORDER.
BLOOD SUPPLY OF FEMORAL
HEAD
 PATHOGENESIS
• DISRUPTION OF THE NORMAL BLOOD SUPPLY TO THE FEMORAL
HEAD IS THE KEY PATHOGENIC EVENT THAT AFFECTS THE
ARTICULAR CARTILAGE, BONY EPIPHYSIS AND IN SOME PATIENTS
THE METAPHYSIS AND PHYSIS .
• THE COMPROMISE OF BLOOD SUPPLY MAY ARISE FROM TAMPONADE
EFFECT ON RETINACULAR VESSELS OR TRANSIENT SYNOVITIS
TEMPORARILY CAUSING OBSTRUCTION TO RETINACULAR BLOOD
FLOW FROM TENSE INTRA-ARTICULAR EFFUSION IN A HIP WITH
BORDERLINE BLOOD SUPPLY (NO FOVEOLAR SUPPLY).
• THE NECROTIC CHANGES IN PERTHES DISEASE ARE MAINLY
OBSERVED IN THE DEEPER LAYER OF THE CARTILAGE WHICH IS
RESPONSIBLE FOR THE CIRCUMFERENTIAL GROWTH OF THE BONY
EPIPHYSIS.
• DUE TO ISCHEMIA THERE IS CESSATION OF THE ENDOCHONDRAL
OSSIFICATION AT CARTILAGE-SUBCHONDRAL BONE JUNCTION.
SEPARATION OF THE CARTILAGE FROM UNDERLYING BONE
FOLLOWED BY VASCULAR INVASION OF THE CARTILAGE AND
ACCESSORY OSSIFICATION CENTERS OCCURS LEADING TO BONE
RESORPTION AND DEPOSITION OF REACTIVE FIBROUS TISSUE.
• THE BONY EPIPHYSIS SHOWS NECROTIC CHANGES FOLLOWED BY
FRACTURE OF THE TRABECULAE AND SUBSEQUENT THICKENING OF
CHARACTERISTIC CHANGES IN
THE BLOOD SUPPLY OF
FEMORAL HEAD IN CHILDREN
MAY BE PARTIALLY
RESPONSIBLE FOR
DEVELOPMENT OF DISEASE:
CHILDREN < 4 YEARS HAVE TWO
MAJOR ARTERIES SUPPLYING
THE HEAD—THE METAPHYSEAL
AND RETINACULAR SYSTEM
FROM 4 TO 8 YEARS—THE
METAPHYSEAL SUPPLY IS
OBLITERATED WITH DEVELOPMENT
OF CAPITAL PHYSIS SO ONLY THE
RETINACULAR SYSTEM IS THE
PREDOMINANT BLOOD SUPPLY THAT
ENTERS THE HEAD. THE LATERAL
EPIPHYSEAL SYSTEM IS DIVIDED
INTO POSTEROSUPERIOR AND
POSTERIOR INFERIOR SYSTEMS.
OBLITERATION OF THE FORMER IS
RESPONSIBLE FOR OSTEONECROSIS
OF ANTEROLATERAL ASPECT OF
> 8 YEARS THE
DEVELOPMENT OF
FOVEOLAR SYSTEM GIVES
THE HEAD A DUAL SUPPLY
AGAIN (FOVEOLAR AND
RETINACULAR).
 FROM 16 TO 18 YEARS AS
THE GROWTH PLATE
DISAPPEARS
ALL THREE GROUPS
BECOME RE-ESTABLISHED
TO SUPPLY THE
FEMORAL HEAD
(FOVEOLAR, RETINACULAR
AND METAPHYSEAL
BLOOD SUPPLY)
 WALDENSTROM STAGES OF NATURAL
PROGRESSION OF PERTHES

STAGES
1. INITIAL - stage of increased radiodensity
2. FRAGMENTATION – resorptive stage
3. REOSSIFICATION – healing / repair stage
4. RESIDUAL – healed stage
4 STAGES OF PERTHES DISEASE
 INITIAL STAGE ( INCREASED
RADIODENSITY )
• SMALLER OSSIFIC NUCLEUS,
• INCREASED RADIODENSITY OF THE
NUCLEUS,
• INFARCTION PRODUCES A SMALLER,
SCLEROTIC EPIPHYSIS WITH MEDIAL
JOINT SPACE WIDENING,
• RADIOGRAPHS MAY REMAIN
OCCULT FOR
 FRAGMENTATION STAGE
( RESORPTION )
• FEMORAL HEAD APPEARS TO FRAGMENT OR
DISSOLVE
• RESULT OF A REVASCULARIZATION PROCESS
AND BONE RESORPTION PRODUCING
COLLAPSE AND SUBSEQUENT INCREASED
DENSITY
• HIP-RELATED SYMPTOMS ARE MOST
PREVALENT
• LATERAL PILLAR CLASSIFICATION BASED ON
THIS STAGE
 REOSSIFICATION (HEALING / REPAIR)

• OSSIFIC NUCLEUS UNDERGOES

REOSSIFICATION AS NEW BONE
APPEARS AS NECROTIC BONE IS
RESORBED
• MAY LAST UP TO 18 MONTHS
 RESIDUAL STAGE ( HEALED STAGE )

• FEMORAL HEAD REMODELS UNTIL

SKELETAL MATURITY IS ACHIEVED AND
OSSIFIC NUCLEUS IS COMPLETELY
REOSSIFIED
• REOSSIFIED TRABECULAR PATTERN
RETURNS
• MAY DEMONSTRATE FLATTENING OF
ARTICULAR SURFACE, ESPECIALLY
SUPERIORLY AND WIDENING OF THE
HEAD & NECK OF FEMUR.
 CLINICAL FEATURES
• LIMP
• PAIN IS TYPICALLY PRESENT IN THE GROIN REGION.
• EXAMINATION, THERE IS FREQUENT LIMITATION OF INTERNAL ROTATION AND
ABDUCTION (ESPECIALLY IN FLEXION) AND IN ADVANCED STAGES THE LEG-
LENGTH DISCREPANCY MAY BE OBSERVED. LIMITATION OF ABDUCTION IN
FLEXION IS THE EARLIEST CLINICAL SIGN PRESENT IF CAREFULLY ELUCIDATED.
• MILD TO MODERATE ATROPHY OF THE THIGH.
• GAIT IS ANTALGIC THAT ALSO DEVELOPS TRENDELENBURG COMPONENT LATER.
• IN BILATERAL CASES, THE DISEASE IS CHARACTERISTICALLY ASYMMETRIC AND
BEGINS 1 YEAR PRIOR TO OTHER HIP.
 DIFFERENTIAL DIAGNOSIS
• COXITIS FUGAX,
• JUVENILE IDIOPATHIC ARTHRITIS,
• OSTEOMYELITIS,
• EPIPHYSEAL DYSPLASIA,
• MEYER’S DYSPLASIA,
• SPONDYLOEPIPHYSEAL DYSPLASIA,
• CHONDROBLASTOMA,
• STEROID-INDUCED OSTEONECROSIS.
 RADIOLOGICAL CHANGES

A B

Figs A and B: The radiology of Perthes disease. (a) Increased width of epiphysis, (b) Lateral
extrusion of physis, (c) Broadening and shortening of femoral neck, (d) Decreased neck shaft
angle, (e) Convex proximal femoral growth plate, (f ) Step-like irregularities in growth plate and
bony epiphysis, (g) Increased width of articular cartilage, and (h) Increased radius of acetabulum,
flattened lateral lip of acetabulum
 RADIOLOGICAL WORK UP
• X-RAYS OF PELVIS WITH BOTH HIPS – AP AND LATERAL VIEWS
• ULTRASONOGRAPHY CAN BE USED TO INVESTIGATE THE
DIFFERENTIALS OF PERTHES LIKE EFFUSION IN COXITIS FUGAX OR
OSTEOMYELITIS.
• MAGNETIC RESONANCE IMAGING (MRI) CAN BE USED TO
DIAGNOSE THE DISEASE EARLY (IDENTIFICATION OF NECROTIC
AREAS AND ASSESSMENT OF FEMORAL HEAD FOR SHAPE CHANGES)
AND DYNAMIC MRI CAN BE USED TO ASSESS “HINGED ABDUCTION”
THAT IS OFTEN DIFFICULT TO IDENTIFY IN THE UNOSSIFIED
 CHARACTERISTIC
RADIOGRAPHIC SIGNS
• SMALLER SIZE OF OSSIFIC NUCLEUS OF CAPITAL FEMORAL EPIPHYSIS ALONG
WITH WIDENED JOINT SPACE (THIS IS THE “EARLIEST RADIOLOGICAL SIGN”)
- DUE TO TEMPORARY CESSATION OF ENDOCHONDRAL OSSIFICATION
- THE ARTICULAR CARTILAGE CONTINUES TO GROW AS IT DERIVES
NUTRITION FROM SYNOVIAL FLUID.
• THE ANTEROLATERAL REGION IS AFFECTED DUE TO:
- MAXIMUM WEIGHT BEARING STRESSES
- MAXIMUM BONE RESORPTION SEEN HERE DUE TO AREA SUPPLIED BY
POSTEROSUPERIOR VESSEL DISTRIBUTION.
SUBCHONDRAL FRACTURE (CAFFEY SIGN, CRESCENT SIGN) IS A
RADIOLUCENT LINE SEEN IN THE ANTEROLATERAL REGION OF FEMORAL HEAD
BEST VISUALIZED IN LOWENSTEIN VIEW
- REQUIREMENT FOR TRUE PERTHES DISEASE
- MINIMAL EXTENSION OF FRACTURE OR THIS LINE IS SEEN AFTER INITIAL
VISUALIZATION
- TRANSIENT FINDING EARLY IN COURSE OF DISEASE WITH AVERAGE OF 2–9
MONTHS
GAGE SIGN
–– THIS IS A V-SHAPED DEFECT IN
THE LATERAL PART OF EPIPHYSIS
AND ADJOINING METAPHYSIS
–– IT INDICATES EXCESS
GROWTH OF CARTILAGE
LATERALLY THAT CAUSES
RAREFACTION IN THE ADJOINING
EPIPHYSIS AND METAPHYSIS.
SAGGING ROPE
SIGN
THIS IS A CURVILINEAR
SCLEROTIC LINE
RUNNING
HORIZONTALLY ACROSS
THE FEMORAL NECK.
IT IS CONFIRMED BY 3D
CT STUDIES.
IT IS A FINDING IN AP
RADIOGRAPH IN A
 BONE SCAN
THE FOUR STAGES SEEN ON A TYPICAL BONE SCAN
FOR PERTHES DISEASE ARE:
TOTAL LACK OF UPTAKE
 LATERAL COLUMN UPTAKE INDICATING
VASCULARIZATION
 GRADUAL FILLING OF ANTEROLATERAL PART OF
EPIPHYSIS
 ARTHROGRAPHY
• ARTHROGRAPHY OFFERS AN OPPORTUNITY TO EVALUATE
COVERAGE AND MOBILITY UNDER DIRECT VISION.
LAREDO CLASSIFIED HIPS INTO 5 TYPES DEPENDING
• UPON ARTHROGRAPHIC FINDINGS.
 TYPE 1 HIPS ARE NORMAL
 TYPE 2 HIPS HAVE SPHERICAL FEMORAL HEAD WHICH IS
LARGER THAN NORMAL
 TYPE 3 HIPS HAVE OVOID FEMORAL HEAD
TYPE 4 HAS LARGE AND FLATTENED FEMORAL HEAD,
WITH STRAIGHT AND ELEVATED LABRUM, HINGED
ABDUCTION MAY BE PRESENT
TYPE 5 HIPS SHOW A FEMORAL HEAD LARGER THAN
NORMAL AND SADDLE-SHAPED, THE LABRUM IS
STRAIGHT AND ELEVATED.
ALL HIPS FROM TYPE 2 TO TYPE 5 SHOW FEMORAL HEAD
EXTRUSION IN NEUTRAL POSITION AND NORMAL
COVERAGE AT ABOUT 30DEGREE OF ABDUCTION WITH
SOME INTERNAL ROTATION.
 CATTERALL
CLASSIFICATION
IT EMPHASIZES THE RELATIONSHIP BETWEEN
THE EXTENT OF HEAD INVOLVEMENT AND
OUTCOME.
• GROUP I - (25%INVOLVEMENT),
• GROUP II - (50% INVOLVEMENT),
• GROUP III - (75% INVOLVEMENT),
Group I :
Involvement of the
anterior epiphysis
only
Group II :
Involvement of the
anterior epiphysis with
clear sequestrum
Group III :
Only a small part of the
epiphysis is not
involved
Group IV :
Total head involvement

 “head at risk” correlated

positively with poor results,
especially in patients in
groups II, III, and IV
head-at-risk signs include :
1)lateral subluxation of the femoral head from
the acetabulum,
2) speckled calcification lateral to the capital
epiphysis,
3) diffuse metaphyseal reaction (metaphyseal
cysts),
4) a horizontal physis, and
5) the Gage sign
Catterall recommended
older children in groups II, III,  containment by
femoral
and IV with head-at-risk signs varus derotational
osteotomy
Contraindications : already malformed femoral head
and delay of treatment of >8 months from onset of
symptoms.
Surgery is not recommended for any group I children or
any child without the head-at-risk signs.
SALTER-THOMPSON CLASSIFICATION
Class A : Crescent sign involves <½ of femoral head
Class B : Crescent sign involves >½ of femoral head
Lateral Pillar (Herring) Classification
HERRIN LATERAL PILLAR AGE PROGNOSIS
G
GROUP
A Uninvolved All ages Good outcome
B <50% Involved < 8 - 9 years  Good outcome
Maintains >50% height > 8 - 9 years  Less favorable
results
B/C Lateral pillar is narrowed (2-3 All ages Bad outcome
border mm) or poorly ossified with
approximately 50% height
C Less than 50% of lateral pillar All ages Worst
height is maintained outcome
 TREATMENT
In the early stage (active phase) :
 4 years and older  explaining to the parents the natural
history and expected duration of the disease (24 to 36
months).
 Children 2 to 3 years  observed and do not need
aggressive treatment
 Once synovitis resolves  daily home physical therapy
program, including active and active-assisted range-of-motion
and muscle stretching exercises to the hip and knee, is
recommended to try to maintain a normal hip range of motion.
 If loss of hip motion is significant, and subluxation laterally
is occurring, bed rest, skin traction, progressive passive and
active physical therapy, abduction exercises, pool therapy, or
bracing if possible, are indicated.
 If there is no improvement  closed reduction under GA and
percutaneous adductor longus tenotomy,

followed by an ambulatory abduction

cast (Petrie) for 6 weeks or more
 femoral head involvement was severe (lateral pillar B, B/C)
surgical procedures - Varus derotational osteotomy and
Shelf arthroplasty (lateral labral support )
 Catterall III or IV;
 lateral pillar B, B/C, C
 in the early stages (fragmentation),
with incorporation of the shelf graft into the pelvis as a
result of continued growth of the lateral acetabular
structures

Distraction of the hip joint (arthrodiastasis) by an

external
fixator for 4 months has been described in older children
Complications, such as pin breakage and pin track infections,
have been reported with this procedure, and presently
its use seems to be limited to the most severe cases.

A combined osteotomy (pelvic osteotomy and varus femoral

osteotomy) used as a salvage procedure for severe Legg-
Calvé-Perthes disease has the theoretical advantage of
obtaining maximal femoral head containment while avoiding
the complications of either procedure alone, such as limb
shortening, extreme neck-shaft varus angulation, and
associated abductor weakness.
Stevens et al. described guided
growth of the trochanteric
apophysis using a “tether”
with an eight-plate and soft-
tissue release as part of
nonosteotomy management
strategy for select children with
progressive symptoms and
related radiographic changes.
INNOMINATE OSTEOTOMY

Innominate osteotomy using

full thickness 2X3cm
quadrangular graft from
wing of ilium for Legg-
Calvé-Perthes disease
 Three years after innominate osteotomy.
Eight weeks after innominate Femoral head is contained without
osteotomy with fixation using evidence of subluxation. Center-edge
three pins angle is 28 degrees, and femoral head is
concentric but slightly enlarged.
 ADVANTAGES OF INNOMINATE OSTEOTOMY :
• include 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 OF INNOMINATE
OSTEOTOMY:
• the inability sometimes to obtain adequate containment of
the femoral head, especially in older children;
• an increase in acetabular and hip joint pressure that may cause further
avascular changes in the femoral head;
• an increase in leg length on the operated side compared with the
normal side that may cause a relative adduction of the hip and
POSTOPERATIVE CARE The patient is immobilized for10 to 12
weeks in a spica cast before the pins are removed. Range-of-motion
exercises and full weight-bearing ambulation are started, and
radiographic evaluation is repeated.
 LATERAL SHELF PROCEDURE
 Make a curved incision below the iliac crest, passing 1.5cm
below the anterior superior iliac spine to avoid the lateral
cutaneous nerve of the thigh. Strip the glutei subperiosteally
from the outer table of the ilium to the level
 of insertion of the joint capsule. Mobilize and divide the
reflected head of the rectus femoris.
 Create a trough in the bone immediately above the insertion
of the capsule (Fig A). Raise a bony flap 3 cm wide × 3.5
cm long superiorly from the outer cortex of the ilium.
A to C, Operative technique for lateral shelf
acetabuloplasty in Legg-Calvé-Perthes disease.
 Cut strips of cancellous graft from the ilium above the flap,
and insert them into the trough so that they form a canopy on
the superior surface of the hip joint (Fig B). Pack the web-
shaped space between the flap and the graft canopy with
cancellous bone graft (Fig C).
 Repair the reflected head of the rectus femoris over the
created shelf. Close the wound in the usual manner, and
apply a spica cast.
POSTOPERATIVE CARE : The spica cast is worn for 8 weeks.
Protective weight bearing in a single spica cast is continued for
6 additional weeks.
labral support procedure early argue that
it has 3 beneficial effects:
(1)lateral acetabular growth stimulation,
(2) prevention of subluxation,
(3) shelf resolution after femoral epiphyseal
reossification.
 VARUS DEROTATIONAL OSTEOTOMY
The advantages of varus derotational osteotomy
Ability to obtain maximal coverage of the femoral head,
Ability to correct excessive femoral anteversion with the same
osteotomy
The disadvantages of varus derotational osteotomy
o excessive varus angulation that may not correct with growth and cause
further shortening of an already shortened extremity,
o gluteus lurch produced by decreasing the length of the lever arm of the
gluteal musculature,
o nonunion of the osteotomy, and
o the requirement of a second operation to remove the internal fixation.
Preoperative radiograph After varus osteotomy and
fixation
 Insertion of guide pin

Reaming of femur

First depth marking Removal of wedge to customize Plate and compression screw application
flush with lateral cortex fit
Plate and compression screw application Insertion of bone screws

POSTOPERATIVE CARE The

spica cast is worn for 8 to12
weeks, until union is achieved.
The internal fixation can be
removed 12 to 24 months after
Insertion of bone screws the osteotomy if desired.
 ARTHRODIASTASIS
The rationale behind arthrodiastasis is that distraction of the
joint not only widens but also unloads the joint space, reduces
the pressure on the femoral head, allows fibrous repair of
articular cartilage defects, and preserves congruency of the
femoral head. The articulated fixator allows 50 degrees of
hip flexion.
Recent reports have described significant complications with
this procedure; it should not be taken lightly and used only for
the most severely involved hips with severe subluxation.
Radiographic and clinical appearance of hinged external
fixator for hip arthrodiastasis in a patient with Legg-Calvé-
Perthes disease.
POSTOPERATIVE CARE : Flexion and extension
exercises
are encouraged with the fixator in place, and the patient
is kept non–weight bearing. The fixator is left in place for
4 to 5 months until lateral pillar reossification appears. The
fixator is removed in the operating room, and a hip
arthrogram is obtained.
After removal of the frame, the patient continues protective
non–weight bearing and intensive physical therapy and
hydrotherapy for an additional 6 weeks. At this stage, full
weight bearing is allowed with continued physiotherapy for
VALGUS EXTENSION OSTEOTOMY

A to C, Valgus osteotomy to
reduce hinge abduction
and increase flexion of hip;
osteotomy is fixed with
pediatric screw and side plate.
 CHIARI OSTEOTOMY
 We have used the pelvic osteotomy described by Chiari as a
salvage procedure to accomplish coverage of a large flattened
femoral head in an older child when the femoral head is
subluxating and painful
 It is a supra-acetabular medial displacement pelvic osteotomy.
 Place the extra-articular buttress of bone to the lateral
acetabulum over the subluxed femoral head.
 It Increases weight bearing surface and covers femoral head
with fibrocartilage (metaplastic bone) not articular cartilage.
R
A. Residual Legg-
R
Calvé-Perthes disease
(coxa plana) and
subluxation in hip on
right.

B. Eight months after

Chiari osteotomy
with good coverage
of femoral head.
THANK YOU