Legg calve perthes
disease
Coxa plana,
osteochondritis deformans juvenilis,
osteochondrosis of the hip joint
 Perthe’s Disease is a condition which is pathologically characterized
by idiopathic avascular necrosis of the epiphysis of the femoral head
in a child.
 The avascular epiphysis is almost always completely revascularised
and replaced but resulting in variable degree of deformity of the
femoral head and growth disturbance.
EPIDEMIOLOGY
 Common in Central Europe, less common in blacks, Chinese & Indians.
 Quite frequent in rural South Western coast of India.
 10 times more common in Uduppi area of Karnataka than Vellore in Tamil
Nadu.
 Sex: Males are affected 4-5 times more often than females.
 Age: most commonly seen in aged 5 – 10 yrs.
 Mean age is higher in South India -9.9 yrs – males
-8.7 yrs - females
ETIOLOGY
 A temporary and possibly repeated vascular insult
 Proposed theories.
- Trueta’s hypothesis: arterial supply in infants and children younger
than 3 years of age have dual blood supply to the femoral head namely
metaphyseal and retinacular arteries. Between the ages of 4 to 8 years, each
femoral head has single arterial supply through retinacular arteries. After 8 years
of age, foveolar arteries of the ligamentum teres contribute blood supply
providing double sourse and there incidence of LCDP begins to decline at this
age.
- Caffey’s hypothesis: Coagulation abnormalities have been postulated
to cause recurrent thrombosis leading to ischemia and necrosis.
PATHOLOGY
 4 stages on the basis of evolution of disease
- Stage of Avascular Necrosis
- Stage of Revascularization / Fragmentation
- Stage of Ossification / Healing
- Remodeling / Residual 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.
 It is at this stage that there is collapse and loss of structural
integrity of the femoral head as it is sort of softened due to
bone resorption, collapse of necrotic bone and persistence of
fibro-vascular tissue leading to deformation of epiphysis.
 The femoral head may extrude from the 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.
CLINICAL PICTURE
 Typically a boy, 5-10 years old.
 Characteristic presentation is a painless limp.
 May present with limp along with pain.
 The child appears to be well & not sick.
 The hip looks to be deceptively normal – there may be little
wasting.
 Abduction & Internal rotation are nearly always limited.
 Antalgic gait in the irritable phase or Trendelenburg gait.
DIFFERENTIAL DIAGNOSIS
 Unilateral 
-Tuberculosis hip
-Synovitis
-Slipped femoral capital epiphysis
-Lymphoma
-Eosinophilic granuloma
 Bilateral
-Hypothyroidism
-Multiple epiphyseal dysplasia
-Spondyloepiphyseal dysplasia
-Sickle cell disease
IMAGING STUDIES
 Perthe’s disease is suspected clinically but diagnosis rests on plain X-rays.
- Pelvis with both hips – AP view
- Frog leg Lateral view of the hip
 Stages of Avascular necrosis, Fragmentation, Ossification &
Residual stage.
 Other radiological changes
 Metaphyseal changes – Hyperemia & osteoporosis Cystic
changes – poor prognosis
 Changes in physis – Abnormal growth and premature closure
leading to short & wide neck.
 Greater Trochanter – Elevated proximally d/to retardation of
the longitudinal growth of femoral neck – abductor
insufficiency.
 Sagging rope sign A rope like radiodense line overlying the

proximal femoral metaphysis.( intertrochantric area). Is infact
the anterior portion of the overlarge femoral head as it
projects on a shortened and wide proximal femoral metaphysis.
 Head in head sign
MANAGEMENT
 No general agreement on the “correct” course of treatment for all cases.
 Aims of treatment : Primary aim is to prevent deformation of the femoral
head.
 Prevention of stiffness and maintenance of good range of movements.
 Prevent or correct growth disturbances -greater trochanteric
overgrowth
 Main cause of deformation - extrusion of the femoral head.
- The treatment when needed is to try to prevent this deformation.
Containment of the femoral head within the acetabulum.
- The socket, thus, acts as a mould to keep the head spherical while still
it is in the softened state.
(a) Conservative methods
 Weight relief & rest In the past, treatment was
primarily directed at avoiding weight by bed rest for
prolonged period (up to 2 yrs) or weight relieving
calipers to prevent head deformation. Little evidence
for efficacy.
 Containment by bracing & casting
- Plaster cast in abd. & internal rotation
– broomstick casts
- Braces to keep hip in desired position.
- Weight bearing is allowed in braces.
- Casts - temporary form of
containment till definitive treatment
undertaken.
SURGICAL METHODS
 FEMORAL OSTEOTOMY
-Up to 12 years of age an open wedge
osteotomy may be
performed without the risk of delayed
union / non-union.
-Also the amount of shortening is
minimized.
-Pre-requisites – near normal hip
movements.
 PELVIC OSTEOTOMY
-Redirectional Osteotomy - Salter’s
osteotomy to reorient the acetabulum
-Shelf Operation - To create a bony shelf
to cover the extruded part of the epiphysis.
-Displacement Osteotomy - Chiari
osteotomy is another way to improve the
coverage. Guidelines
Thank You

PERTHES.pptx.............................

  • 1.
  • 2.
    Coxa plana, osteochondritis deformansjuvenilis, osteochondrosis of the hip joint  Perthe’s Disease is a condition which is pathologically characterized by idiopathic avascular necrosis of the epiphysis of the femoral head in a child.  The avascular epiphysis is almost always completely revascularised and replaced but resulting in variable degree of deformity of the femoral head and growth disturbance.
  • 3.
    EPIDEMIOLOGY  Common inCentral Europe, less common in blacks, Chinese & Indians.  Quite frequent in rural South Western coast of India.  10 times more common in Uduppi area of Karnataka than Vellore in Tamil Nadu.  Sex: Males are affected 4-5 times more often than females.  Age: most commonly seen in aged 5 – 10 yrs.  Mean age is higher in South India -9.9 yrs – males -8.7 yrs - females
  • 4.
    ETIOLOGY  A temporaryand possibly repeated vascular insult  Proposed theories. - Trueta’s hypothesis: arterial supply in infants and children younger than 3 years of age have dual blood supply to the femoral head namely metaphyseal and retinacular arteries. Between the ages of 4 to 8 years, each femoral head has single arterial supply through retinacular arteries. After 8 years of age, foveolar arteries of the ligamentum teres contribute blood supply providing double sourse and there incidence of LCDP begins to decline at this age. - Caffey’s hypothesis: Coagulation abnormalities have been postulated to cause recurrent thrombosis leading to ischemia and necrosis.
  • 5.
    PATHOLOGY  4 stageson the basis of evolution of disease - Stage of Avascular Necrosis - Stage of Revascularization / Fragmentation - Stage of Ossification / Healing - Remodeling / Residual stage
  • 6.
    Stage of AvascularNecrosis 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.
  • 7.
    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.  It is at this stage that there is collapse and loss of structural integrity of the femoral head as it is sort of softened due to bone resorption, collapse of necrotic bone and persistence of fibro-vascular tissue leading to deformation of epiphysis.  The femoral head may extrude from the acetabulum at this stage.
  • 8.
    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.
  • 9.
    CLINICAL PICTURE  Typicallya boy, 5-10 years old.  Characteristic presentation is a painless limp.  May present with limp along with pain.  The child appears to be well & not sick.  The hip looks to be deceptively normal – there may be little wasting.  Abduction & Internal rotation are nearly always limited.  Antalgic gait in the irritable phase or Trendelenburg gait.
  • 10.
    DIFFERENTIAL DIAGNOSIS  Unilateral -Tuberculosis hip -Synovitis -Slipped femoral capital epiphysis -Lymphoma -Eosinophilic granuloma  Bilateral -Hypothyroidism -Multiple epiphyseal dysplasia -Spondyloepiphyseal dysplasia -Sickle cell disease
  • 11.
    IMAGING STUDIES  Perthe’sdisease is suspected clinically but diagnosis rests on plain X-rays. - Pelvis with both hips – AP view - Frog leg Lateral view of the hip
  • 12.
     Stages ofAvascular necrosis, Fragmentation, Ossification & Residual stage.  Other radiological changes  Metaphyseal changes – Hyperemia & osteoporosis Cystic changes – poor prognosis  Changes in physis – Abnormal growth and premature closure leading to short & wide neck.  Greater Trochanter – Elevated proximally d/to retardation of the longitudinal growth of femoral neck – abductor insufficiency.  Sagging rope sign A rope like radiodense line overlying the  proximal femoral metaphysis.( intertrochantric area). Is infact the anterior portion of the overlarge femoral head as it projects on a shortened and wide proximal femoral metaphysis.  Head in head sign
  • 13.
    MANAGEMENT  No generalagreement on the “correct” course of treatment for all cases.  Aims of treatment : Primary aim is to prevent deformation of the femoral head.  Prevention of stiffness and maintenance of good range of movements.  Prevent or correct growth disturbances -greater trochanteric overgrowth  Main cause of deformation - extrusion of the femoral head. - The treatment when needed is to try to prevent this deformation. Containment of the femoral head within the acetabulum. - The socket, thus, acts as a mould to keep the head spherical while still it is in the softened state.
  • 14.
    (a) Conservative methods Weight relief & rest In the past, treatment was primarily directed at avoiding weight by bed rest for prolonged period (up to 2 yrs) or weight relieving calipers to prevent head deformation. Little evidence for efficacy.  Containment by bracing & casting - Plaster cast in abd. & internal rotation – broomstick casts - Braces to keep hip in desired position. - Weight bearing is allowed in braces. - Casts - temporary form of containment till definitive treatment undertaken.
  • 15.
    SURGICAL METHODS  FEMORALOSTEOTOMY -Up to 12 years of age an open wedge osteotomy may be performed without the risk of delayed union / non-union. -Also the amount of shortening is minimized. -Pre-requisites – near normal hip movements.  PELVIC OSTEOTOMY -Redirectional Osteotomy - Salter’s osteotomy to reorient the acetabulum -Shelf Operation - To create a bony shelf to cover the extruded part of the epiphysis. -Displacement Osteotomy - Chiari osteotomy is another way to improve the coverage. Guidelines
  • 16.