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PERTHE’S DISEASE
Dr.HARSHA NANDINI TALASILA
Legg-Calvé-Perthes disease (LCPD) is a condition in
which the blood supply of the capital femoral
epiphysis (head) is disrupted, resulting in
epiphyseal osteonecrosis and chondronecrosis with
cessation of growth of the ossific nucleus.
The necrotic epiphyseal bone is eventually
resorbed and replaced by new bone.
During the resorptive phase of the healing, the
mechanical properties of the femoral head are
weakened such that some femoral heads develop a
flattened deformity (coxa plana).
Over time, the femoral head reossifies,resumes
growth with some overgrowing (coxa magna),and
remodels to a variable degree of roundness until
skeletal maturity.
SYNONYMS
• Osteochondritis deformans juvenalis
• Coxa plana
• AGE GROUP : 4–10years
• Male : female – 4:1
• Unilateral more common than bilateral
• Bilateral incidence is 12-16%
BLOOD SUPPLY TO THE FEMOR
HEAD
LESS THAN 4years:
• Metaphyseal artery
• Retinacular artery
4 to 8 years:
• Retinacular arteries
MORE THAN 8years:
• Retinacular arteries
• Foveal artery
Adolescence:
• Retinacular arteries
• Foveal arteries
• Metaphyseal arteries
1. Vascular supply:
- Angiograms and laser studies have shown
medial circumflex artery is missing or obliterated
and obturator artery or the lateral epiphyseal
artery also affected.
2. Increased intra-articular pressure
3. Intraosseous pressure
- Patients has shown that the venous drainage in
the femoral head is impaired, causing an
increase in intraosseous pressure.
4. Coagulation disorder
- Associated with deficiency of factor C or S.
- They normally inhibit coagulation.
5. Social conditions
-Usually belong to lower socioeconomic status,
reflects dietary and environmental factors.
6. Trauma
8.Abnormal growth and development
- Usually shorter than their peers.
- Indicates retarded skeletal growth
9. Genetic factors
-First degree relatives have 35times more risk
CLINICAL FEATURES
• Mild to moderate hip pain radiating to the medial
aspect of thigh and knee.
• Limp is often painless.
• Muscle spasm evident and restricted hip motion,
especially in abduction.
PATHOGENESIS
1. INCIPIENT STAGE OR SYNOVITIS STAGE:
• Synovium is hyperaemic.
2. STAGE OF AVASCULAR NECROSIS:
• Dead trabecular bone
• Collapsed trabeculae
• Thickened articular cartilage
• Physeal disruption
• Cartilage extending from the physis into the
metaphysis
3. FRAGMENTATION STAGE:
• Invasion of vascular granulation tissue.
• New bone forming on old trabeculae.
• Woven new bone formation.
4. HEALING STAGE:
• Normal forming bone alongside replacing slowly
resorbing bone.
• Return to normal architecture.
WALDENSTORM STAGING OF
PERTHE’S DISEASE
1. AVASCULAR NECROSIS:
• Widened joint space
• Femor head is denser than normal and slightly
flattened
• Lateralization of femor head.
2. STAGE OF RESORPTION :
• Femor head breaks into fragments
• Lucent areas in the femor head
• Increased density resolves
• Acetabular contour is more irregular
3. STAGE OF REOSSIFICATION:
• New bone formation occurs in the femor head.
4. HEALING STAGE:
• Heals with or without defect.
• Defects like coxa magna,coxa plana
METAPHYSEAL CHANGES
• An overabundance of fatty marrow
• Circumscribed osteolytic lesions with a
sclerotic border,
• A wide growth plate with disarrayed
ossification and columns of unossified
cartilage coursing down into the
metaphysis.
CLASSIFICATIONS
SALTER THOMPSON
CLASSIFICATION
• TYPE A: extent of the
subchondral fracture
line is less than 50 % of
the superior dome of
the femor head.
• TYPE B: extent of the
subchondral fracture
line is more than 50 %
of the superior dome of
the femor head.
HERRING LATERAL PILLAR
CLASSIFICATION
• GROUP A: no involvement of the pillar
• GROUP B: 50% of the pillar is maintained
• GROUP C: less than 50% of the pillar is maintained
CATERRAL’S CLASSIFICATION
• GROUP 1: anterior part of the head is involved
• GROUP 2: anterior and partial lateral part
involved,sequestrum and mild metaphyseal
changes
• GROUP 3:Anterior and lateral head
involved,sequestrum,diffuse metaphyseal
changes,coxa magna
• GROUP 4:complete head involvement and collapse
of the head
STULBERG’S CLASSIFICATION
• GRADE 1: round head,normal hip
• GRADE 2: round head,coxa magna
• GRADE 3: oval,mushroom shaped head,coxa magna
• GRADE 4: flat head,congruent with acetabulum
• GRADE 5: flat head,incongruent
CATERALL’S HEAD AT RISK SIGNS
1. Lateral subluxation of the femoral head from
acetabulum.
2. Speckled calcification lateral to the capital
epiphysis.
3. Diffuse metaphyseal reactions or metaphyseal
cysts.
4. Horizontal physes
5. Gage sign: a radioluscent v shaped defect in the
lateral epiphyses and adjacent metaphyses.
• GAGE SIGN:radiolucent
V shaped defect in the
lateral epiphysis and
adjacent metaphysis.
DIFFERENTIAL DIAGNOSIS
• AVASCULAR NECROSIS:
Sickle cell syndrome
Thalassemia
Down syndrome
Achondroplasia
Steroid medication
• EPIPHYSEAL DYSPLASIA
Comparison chart
PERTHES DISEASE EPIPHYSEAL DYSPLASIA
Unilateral Bilateral involvement
If B/L, marked asymmetry, disease
in differing stages and severity
Symmetrical findings
No involvement of other joints Involvement of other joints or
spine.
Sclerotic and cystic changes in
femoral head and cystic changes in
metaphysis
Few sclerotic changes in femoral
head.
More tendency towards lateral
calcification and subluxation
Little tendency.
INVESTIGATIONS
• X rays
• MRI to delineate the extent of head involvement
• ARTHROGRAPHY: whether containment is possible
MANAGEMENT
GOALS OF TREATMENT
1. Elimination of hip irritability
2. Restoration and maintenance of good range of hip
motion.
3. Prevention of epiphyseal extrusion and
subluxation.( containment).
4. Attainment of spherical femoral head on healing.
OBSERVATION
 Onset <6yrs of age, regardless of extent of capital
femoral epiphyseal involvement.
 Age<6yrs of age: Catterall’s group 1 and 2 or
herring group A
 They should have clinical and radiographic
examination at frequent intervals( 3 months).
 If unsuccessful, may necessaite a short course (2-
6 months) of non surgical treatment.
SYMPTOMATIC TREATMENT
• BED REST
• SKIN TRACTION
• NSAIDS
• ABDUCTION BRACING
DEFINITIVE TREATMENT
• Age at clinical onset 6yrs or older.
• Catterall Group 3 or 4/ Salter thomson Group
B.
• When loss of containment manifested by
extrusion seen on AP view.
Containment of femoral head
• Placing the femoral head into the acetabulum as fully as
possible so that it retains its Sphericity during period of
remodelling, healing.
• It can be
-non surgical
-surgical containment
Non surgical containment by orthotics
• Put affected Hip in Abduction
• Some allow Flexion and control rotation of limb
• It can be by casts or braces
• Before starting Containment it is important to restore normal
range of hip motion by Bedrest , Traction ,reduced weight
bearing.
• Throughout the bracing period hip range of motion must be
preserved.
Petrie casts
• Hips were abducted to 45 degrees and 5-10 degrees internally
rotated and casting done with bars inbetween.
• Casts changed every 3-4 months and adductor tenotomy done
when required.
Toronto brace
• Heavy and complicated brace which
keeps hip in abduction while allowing
hip and knee flexion
Atlanta scottish rite brace
• Commonly used today
• Consists of metallic pelvic band,thigh
cuffs,hip hinges and bar between thigh
cuffs
Femoral osteotomy: VARUS
DEROTATIONAL OSTEOTOMY
Indications :
-Age of onset > 6 yrs
-Hips with “at risk” signs even if head is not severely deformed
Pre requisites
-Good to full range of hip motion
-Minimally deformed head
-Usually done in Early and Fragmentation stages
Contraindications:
-Significant flattening with no containment in abduction and
internal rotation
Level of osteotomy
Insertion of guide pin and reaming
of femur
First depth marking flush with lateral
cortex
Removal of wedge to customize it
Plate and compression screw
application
Insertion of bone screws.
Complications
-Excessive varus
-Shortening of limb
-Delayed / Nonunion
Salter’s osteotomy
Indications
-Age > 6 yrs
-Moderate - Severe femoral head involvement
Contra indication
-Pre existing femoral head deformity
Complications :
Loss of fixation
Lengthening of limb
Joint stiffness
Arthrodiastasis
• DISTRACTION OF THE JOINT:
widens joint space.
reduces the pressure on the femoral head
allows fibrous repair of articular cartilage defects
preserves congruency of the femoral head.
1.HINGED ABDUCTION:
Valgus subtrochanteric osteotomy
2.Malformed femoral head
Cheilectomy
3.Coxa magna
shelf augmentation would provide coverage
4.Large malformed femoral head with subluxation laterally
pelvic osteotomy
5.Capital femoral physeal arrest with trochanteric overgrowth
trochanteric advancement
Indications for reconstructive surgery
Valgus osteotomy
• For treating hinged abduction in which flattened head
interferes with abduction
Shelf arthroplasty
Indications
• Lateral subluxation of head
• Hinge abduction of hip
Complications
• Loss of hip flexion
Chiari osteotomy
• Reserved for healing femoral head that remains lateralised
• An osteotomy of the pelvis is performed at the superior
margin of the acetabulum.
• Pelvis inferior to the osteotomy along with the femur is
displaced medially.
• The superior fragment becomes a shelf, and the capsule is
interposed between it and the femoral head.
Cheilectomy
• Surgically removal of protruding femoral head fragments
• Done after closure of capital physis
• Procedure weakens attatchment of femoral head to neck
resulting in slipped epiphysis.
For malformed head
-Mushroom head
-Complete flattened head
-Lateral subluxated head
TROCHANTRIC OVERGROWTH
-Elevation of trochanter decreases tension
and mechanical efficiency of pelvic and
trochantric muscles.
-Shortened femoral neck moves trochanter
closer to centre of rotation of hip, line of pull of
muscles becomes more vertical.
-Impingement of head to the roof limiting
abduction
Trochanteric advancement
-Trochanteric osteotomy done and attached to lateral femoral cortex
Poor prognostic factors
1. Female sex
2. Age > 10yr
3. Uncovering of the femoral head.
4. > 50% head involved
5. Loss of the hip range of movement
6. Premature physeal closure
REFERENCES:
• Tachdjian’s pediatric orthopaedics
• Campbell’s operative orthopaedics
THANKYOU

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Perthes

  • 2. Legg-Calvé-Perthes disease (LCPD) is a condition in which the blood supply of the capital femoral epiphysis (head) is disrupted, resulting in epiphyseal osteonecrosis and chondronecrosis with cessation of growth of the ossific nucleus.
  • 3. The necrotic epiphyseal bone is eventually resorbed and replaced by new bone. During the resorptive phase of the healing, the mechanical properties of the femoral head are weakened such that some femoral heads develop a flattened deformity (coxa plana). Over time, the femoral head reossifies,resumes growth with some overgrowing (coxa magna),and remodels to a variable degree of roundness until skeletal maturity.
  • 4. SYNONYMS • Osteochondritis deformans juvenalis • Coxa plana
  • 5. • AGE GROUP : 4–10years • Male : female – 4:1 • Unilateral more common than bilateral • Bilateral incidence is 12-16%
  • 6. BLOOD SUPPLY TO THE FEMOR HEAD LESS THAN 4years: • Metaphyseal artery • Retinacular artery 4 to 8 years: • Retinacular arteries MORE THAN 8years: • Retinacular arteries • Foveal artery
  • 7. Adolescence: • Retinacular arteries • Foveal arteries • Metaphyseal arteries
  • 8. 1. Vascular supply: - Angiograms and laser studies have shown medial circumflex artery is missing or obliterated and obturator artery or the lateral epiphyseal artery also affected. 2. Increased intra-articular pressure 3. Intraosseous pressure - Patients has shown that the venous drainage in the femoral head is impaired, causing an increase in intraosseous pressure.
  • 9. 4. Coagulation disorder - Associated with deficiency of factor C or S. - They normally inhibit coagulation. 5. Social conditions -Usually belong to lower socioeconomic status, reflects dietary and environmental factors. 6. Trauma
  • 10. 8.Abnormal growth and development - Usually shorter than their peers. - Indicates retarded skeletal growth 9. Genetic factors -First degree relatives have 35times more risk
  • 11. CLINICAL FEATURES • Mild to moderate hip pain radiating to the medial aspect of thigh and knee. • Limp is often painless. • Muscle spasm evident and restricted hip motion, especially in abduction.
  • 12. PATHOGENESIS 1. INCIPIENT STAGE OR SYNOVITIS STAGE: • Synovium is hyperaemic. 2. STAGE OF AVASCULAR NECROSIS: • Dead trabecular bone • Collapsed trabeculae • Thickened articular cartilage • Physeal disruption • Cartilage extending from the physis into the metaphysis
  • 13. 3. FRAGMENTATION STAGE: • Invasion of vascular granulation tissue. • New bone forming on old trabeculae. • Woven new bone formation.
  • 14. 4. HEALING STAGE: • Normal forming bone alongside replacing slowly resorbing bone. • Return to normal architecture.
  • 15. WALDENSTORM STAGING OF PERTHE’S DISEASE 1. AVASCULAR NECROSIS: • Widened joint space • Femor head is denser than normal and slightly flattened • Lateralization of femor head. 2. STAGE OF RESORPTION : • Femor head breaks into fragments • Lucent areas in the femor head • Increased density resolves • Acetabular contour is more irregular
  • 16. 3. STAGE OF REOSSIFICATION: • New bone formation occurs in the femor head. 4. HEALING STAGE: • Heals with or without defect. • Defects like coxa magna,coxa plana
  • 17. METAPHYSEAL CHANGES • An overabundance of fatty marrow • Circumscribed osteolytic lesions with a sclerotic border, • A wide growth plate with disarrayed ossification and columns of unossified cartilage coursing down into the metaphysis.
  • 19. SALTER THOMPSON CLASSIFICATION • TYPE A: extent of the subchondral fracture line is less than 50 % of the superior dome of the femor head. • TYPE B: extent of the subchondral fracture line is more than 50 % of the superior dome of the femor head.
  • 20. HERRING LATERAL PILLAR CLASSIFICATION • GROUP A: no involvement of the pillar • GROUP B: 50% of the pillar is maintained • GROUP C: less than 50% of the pillar is maintained
  • 21. CATERRAL’S CLASSIFICATION • GROUP 1: anterior part of the head is involved • GROUP 2: anterior and partial lateral part involved,sequestrum and mild metaphyseal changes • GROUP 3:Anterior and lateral head involved,sequestrum,diffuse metaphyseal changes,coxa magna • GROUP 4:complete head involvement and collapse of the head
  • 22. STULBERG’S CLASSIFICATION • GRADE 1: round head,normal hip • GRADE 2: round head,coxa magna • GRADE 3: oval,mushroom shaped head,coxa magna • GRADE 4: flat head,congruent with acetabulum • GRADE 5: flat head,incongruent
  • 23. CATERALL’S HEAD AT RISK SIGNS 1. Lateral subluxation of the femoral head from acetabulum. 2. Speckled calcification lateral to the capital epiphysis. 3. Diffuse metaphyseal reactions or metaphyseal cysts. 4. Horizontal physes 5. Gage sign: a radioluscent v shaped defect in the lateral epiphyses and adjacent metaphyses.
  • 24. • GAGE SIGN:radiolucent V shaped defect in the lateral epiphysis and adjacent metaphysis.
  • 25. DIFFERENTIAL DIAGNOSIS • AVASCULAR NECROSIS: Sickle cell syndrome Thalassemia Down syndrome Achondroplasia Steroid medication • EPIPHYSEAL DYSPLASIA
  • 26. Comparison chart PERTHES DISEASE EPIPHYSEAL DYSPLASIA Unilateral Bilateral involvement If B/L, marked asymmetry, disease in differing stages and severity Symmetrical findings No involvement of other joints Involvement of other joints or spine. Sclerotic and cystic changes in femoral head and cystic changes in metaphysis Few sclerotic changes in femoral head. More tendency towards lateral calcification and subluxation Little tendency.
  • 27. INVESTIGATIONS • X rays • MRI to delineate the extent of head involvement • ARTHROGRAPHY: whether containment is possible
  • 29. GOALS OF TREATMENT 1. Elimination of hip irritability 2. Restoration and maintenance of good range of hip motion. 3. Prevention of epiphyseal extrusion and subluxation.( containment). 4. Attainment of spherical femoral head on healing.
  • 30. OBSERVATION  Onset <6yrs of age, regardless of extent of capital femoral epiphyseal involvement.  Age<6yrs of age: Catterall’s group 1 and 2 or herring group A  They should have clinical and radiographic examination at frequent intervals( 3 months).  If unsuccessful, may necessaite a short course (2- 6 months) of non surgical treatment.
  • 31. SYMPTOMATIC TREATMENT • BED REST • SKIN TRACTION • NSAIDS • ABDUCTION BRACING
  • 32. DEFINITIVE TREATMENT • Age at clinical onset 6yrs or older. • Catterall Group 3 or 4/ Salter thomson Group B. • When loss of containment manifested by extrusion seen on AP view.
  • 33. Containment of femoral head • Placing the femoral head into the acetabulum as fully as possible so that it retains its Sphericity during period of remodelling, healing. • It can be -non surgical -surgical containment
  • 34. Non surgical containment by orthotics • Put affected Hip in Abduction • Some allow Flexion and control rotation of limb • It can be by casts or braces • Before starting Containment it is important to restore normal range of hip motion by Bedrest , Traction ,reduced weight bearing. • Throughout the bracing period hip range of motion must be preserved.
  • 35. Petrie casts • Hips were abducted to 45 degrees and 5-10 degrees internally rotated and casting done with bars inbetween. • Casts changed every 3-4 months and adductor tenotomy done when required.
  • 36. Toronto brace • Heavy and complicated brace which keeps hip in abduction while allowing hip and knee flexion
  • 37. Atlanta scottish rite brace • Commonly used today • Consists of metallic pelvic band,thigh cuffs,hip hinges and bar between thigh cuffs
  • 38. Femoral osteotomy: VARUS DEROTATIONAL OSTEOTOMY Indications : -Age of onset > 6 yrs -Hips with “at risk” signs even if head is not severely deformed Pre requisites -Good to full range of hip motion -Minimally deformed head -Usually done in Early and Fragmentation stages Contraindications: -Significant flattening with no containment in abduction and internal rotation
  • 39. Level of osteotomy Insertion of guide pin and reaming of femur First depth marking flush with lateral cortex Removal of wedge to customize it
  • 40. Plate and compression screw application Insertion of bone screws.
  • 42. Salter’s osteotomy Indications -Age > 6 yrs -Moderate - Severe femoral head involvement Contra indication -Pre existing femoral head deformity
  • 43. Complications : Loss of fixation Lengthening of limb Joint stiffness
  • 44. Arthrodiastasis • DISTRACTION OF THE JOINT: widens joint space. reduces the pressure on the femoral head allows fibrous repair of articular cartilage defects preserves congruency of the femoral head.
  • 45. 1.HINGED ABDUCTION: Valgus subtrochanteric osteotomy 2.Malformed femoral head Cheilectomy 3.Coxa magna shelf augmentation would provide coverage 4.Large malformed femoral head with subluxation laterally pelvic osteotomy 5.Capital femoral physeal arrest with trochanteric overgrowth trochanteric advancement Indications for reconstructive surgery
  • 46. Valgus osteotomy • For treating hinged abduction in which flattened head interferes with abduction
  • 47. Shelf arthroplasty Indications • Lateral subluxation of head • Hinge abduction of hip Complications • Loss of hip flexion
  • 48. Chiari osteotomy • Reserved for healing femoral head that remains lateralised • An osteotomy of the pelvis is performed at the superior margin of the acetabulum. • Pelvis inferior to the osteotomy along with the femur is displaced medially. • The superior fragment becomes a shelf, and the capsule is interposed between it and the femoral head.
  • 49. Cheilectomy • Surgically removal of protruding femoral head fragments • Done after closure of capital physis • Procedure weakens attatchment of femoral head to neck resulting in slipped epiphysis. For malformed head -Mushroom head -Complete flattened head -Lateral subluxated head
  • 50. TROCHANTRIC OVERGROWTH -Elevation of trochanter decreases tension and mechanical efficiency of pelvic and trochantric muscles. -Shortened femoral neck moves trochanter closer to centre of rotation of hip, line of pull of muscles becomes more vertical. -Impingement of head to the roof limiting abduction
  • 51. Trochanteric advancement -Trochanteric osteotomy done and attached to lateral femoral cortex
  • 52. Poor prognostic factors 1. Female sex 2. Age > 10yr 3. Uncovering of the femoral head. 4. > 50% head involved 5. Loss of the hip range of movement 6. Premature physeal closure
  • 53. REFERENCES: • Tachdjian’s pediatric orthopaedics • Campbell’s operative orthopaedics