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PERTHES DISEASE AND SCFE
DR ARUNODAYA SIDDARTHA
DEFINITION
HISTORY
Arthur Legg described prominent
characteristics of the disorder: onset
between 5 and 8 years, history of
trauma, painless limp, minimal or no
spasm or shortening of affected limb.
Jacques Calve noted that affected
individuals had minimal atrophy of
the leg and no palpable hip swelling;
abnormal or delayed bone formation
Georg Perthes observed the disorder as “a
self limiting, non inflammatory condition,
affecting the capital femoral epiphysis with
stages of degeneration and regeneration,
leading to restoration of the bone nucleus”
Henning Waldenstrom reported radiographic
changes associated with the disorder in
1909; thought the disease was a form of
Tuberculosis.
Epidemiology
 Incidence 1-4/10,000
 Age 4 - 10years; average 7 yrs
 As early as 2yrs as late as teens
 Boys : girls 4:1
 Bilateral 10-12%
 No evidence of inheritance
 Common in Caucasians; rare in black races
PERTHES AND SCFE.ppt
ETIOLOGY
1. Vascular supply: Medial circumflex artery is missing or obliterated and obturator
artery or lateral epiphyseal artery also affected.
2. Increased intra-articular pressure
3. Increased intraosseous pressure: Impaired venous drainage in femoral head
4. Coagulation disorder: Absence of factor C or S, increase in serum levels of
lipoproteins, thrombogenic substance, Factor V Leiden mutation.
5. Growth hormones- Reduced levels of growth hormones, somatomedin A and C.
6. Social conditions- Lower socioeconomic status, dietary and environmental
factors.
7. Trauma: Risk of vascular interruption increased due to narrow passage and
thick cartilage of femoral head penetrated by lateral epiphyseal arteries
8. Abnormal growth and development: Bone age is lower than chronological
age by 1-3 yrs (Radiological pause). Usually shorter than their peers.
9. Genetic factors
- Inheritance 2-20%; inconsistent pattern.
- Low birth weight, abnormal birth presentations.
- Ratio of affected 1st, 2nd, 3rd and 4th degree relatives to general
population of the same set – 35: 4: 4:1
Pathophysiology
 Rapid growth occurs in relation to devlopment of blood supply
 Interruption of blood supply results in necrosis, removal of necrotic
tissue, and its replacement with new bone.
 Bone replacement may be so complete and perfect that completely
normal bone may result
 The adequacy of bone replacement depends on
 Age of the patient
 Congruity of the involved joint
Sources of blood supply
 Up to 4years
 Metaphyseal vessels
 Retinacular vessels
 Ligamentum teres – scanty
 4 to 7 years
 Metaphyseal vessels ceases
 Above 7years
 Vessels in ligamentum teres have developed
Pathology
 Goes through stages which may last 3 to 4 years
 Stage1
 Ischaemia and bone death, cartilage thickens
 Stage 2
 Revascularization and repair
 Dead marrow replaced by granulation tissue
 Bone revascularized and new bone laid down
 Dead bone resorbed, replaced by fibrous tissue, fragmentation
 Stage 3
 Distortion and remodelling
 Restoration of femoral archtecture or collapse
 Femoral head displaces laterally in relation to acetabulum
PERTHES AND SCFE.ppt
PERTHES AND SCFE.ppt
Classification
 Waldenstrom classification
 Catterall classification
 Salter and thompson classification
 Herring classification
PERTHES AND SCFE.ppt
Stage Ia Stage Ib Stage IIa Stage IIb
Stage IIIa Stage
IIIb
Stage
IV
Catterall classification
 Group I: Only anterior portion of epiphysis
affected
 Group II: More of anterior segment involved;
central sequestrum present
 Group III: Most of the epiphysis sequestrated,
with unaffected portions located medial and
lateral to central segment
 Group IV: Entire epiphysis sequestrated
BENIGN
PROGNOSIS
REQUIRE
TREATMENT
CATTERALL classification
Group I Group
II
Group III Group IV
HEAD-AT-RISK FACTORS
 Lateral subluxation of femoral head
 Radiolucent V in the lateral aspect of
epiphysis (Gage sign)
 Calcification lateral to epiphysis
 Horizontal physeal line
 Metaphyseal cyst
Salter-Thompson
classification
 Based on extent of subchondral fracture on AP
and lateral views
 Group A: Less than half of femoral head
involvement
 Group B: More than half of femoral head
involvement
 Advantage: Applicable at an earlier time point
than Catterall or lateral pillar classification
 Disadvantage: Subchondral fracture present in
only 30 % of patients
SALTER sign
 As disease progresses, a
subchondral fracture may be seen
on anterolateral aspect of femoral
capital epiphysis
 Crescent sign/ Caffey’s sign
 Early radiographic feature best
seen on frog leg lateral view
PERTHES AND SCFE.ppt
HERRING’s LATERAL PILLAR
Classification
Bilateral involvement
 More severe than unilateral
 Boys and girls equally affected
 Independent event
 Bone age delayed in perthes disease
ClInical features
 Incidence: higher latitude, western coastal region of South India
 Onset: 18 months to skeletal maturity (Most prevalent: 4-8 years)
 Male sex prevalence: Boys 4-5 times more susceptible
 Involvement: Bilateral in 10-12 %
SYMPTOMS
 Limp
 Pain
 History of antecedent trauma
 Waxing and waning symptoms
SIGNS
 Small stature
 Atrophy of gluteus, quadriceps and
hamstring muscles
 Abductor limp ( Antalgic +
Trendelenburg gait)
 Decreased hip ROM especially abduction
and internal rotation (Transient early in
disease, persistent later on)
 Positive Trendelenburg test
 Resistance to logroll
Investigations
 Blood tests
 haemogram, ESR, CRP
 Imaging
 Plain X-rays
 Hip U/S
 Bone scintigraphy
 MRI
 Dynamic arthrography
 Assess spherity of femoral head
 Hinge abduction
 Bilateral perthes
 Skeletal survey as part of work-up
MRI
 Accurate for early diagnosis and for
visualising configuration of femoral head
and acetabulum
 More reliable information about true
extent of femoral head necrosis than
radiography or scintigraphy
 Gadolinium enhanced subtraction MRI as
effective as scintigraphy in delineating
epiphyseal necrosis early
 De Sanctis classification:
Group A: < 50 % head necrosis
Group B: > 50 % head necrosis
B0- B3 based on degrees of lateral
extrusion and physeal disruption
TREATMENT OPTIONS
1. SYMPTOMATIC THERAPY
 Bed rest
 Non weight bearing on affected limb
 Short term use of NSAIDs
 Traction:
 Simple longitudinal traction
 Balanced suspension and traction
 Russell traction
 “Slings and springs”
PERTHES AND SCFE.ppt
CONTAINMENT BY BRACING OR CASTING:
 Aims at repositioning extruded anterolateral part of femoral
epiphysis into confines of acetabulum
 Achieved by abducting and flexing or internally rotating the hip
 Needs to be ensured until healing progresses beyond late part
of stage of regeneration ( upto 2 years)
 Femoral head reforms in a concentric manner- Biological
plasticity
Broomstick plasters (Petrie casts)
Snyder sling
A-frame
brace
Toronto brace
Treatment: Two main choices
 Conservative
 Pain control
 Gentle exercises
 Regular re-assessment
 Avoid sport and strenous activities
 Containment
 Hold hips widely abducted in cast/brace >1yr
 Operation
 Varus osteotomy of femur
 Innominate osteotomy of pelvis
 Both
PERTHES AND SCFE.ppt
Herring Guidelines to treatment
 Children <6years
 Symptomatic treatment
 Children >6years; bone age more imp than chronological age
 Bone age at or <6yrs
 Lateral pillar A or B/ caterall I and II
 Symptomatic treatment
 Lateral pillar C/ Caterall III and IV
 Bone over 6years
 Herring A and B/Caterall I and II
 Abduction brace or osteotomy
 Herring C/Caterall III and IV
 Outcome unaffected by treatment
 Children 9yrs and older
 Except in very mild cases, operative containment is the treatment of choice
Prognostic features
 Age
 <6yrs; good regardless of treatment
 6-9years; not always satisfactory with containment
 >10yrs; questionable benefit from containment, poor prognosis
 Gender
 Girls have worse prognosis
 Classification grade
 Herrings lateral pillar classification
 Salter and thompson grade B worse prognosis
 Caterral classification grade
 Caterral “head-at-risk” signs
 The five signs carry worse prognosis
 Others
 Body weight, decreased ROM
SLIPPED CAPITAL FEMORAL EPIPHYSIS
SCFE
Slipped capital femoral epiphysis (SCFE) is an condition of the proximal
femoral physis that leads to slippage of the metaphysis relative to the
epiphysis, and is most commonly seen in adolescent obese males
EPIDEMIOLOGY
 Most common disorder affecting adolescent hips
 Found in 10 per 100,000
 More common in
 Obese children, associated with puberty
 Males (male to female ratio is 2:1.4)
 Specific ethnicities - African Americans, Pacific islanders, Latinos
 During period of rapid growth (10-16 years of age)
 location
 left hip is more common
 bilateral in 17% to 50% (~25%)
 RISK FACTORS
 Obesity
 Single greatest risk factor
 Recent data shows trend towards younger age and increased frequency of
bilaterality at presentation, possibly related to increased rates of
childhood obesity
 Acetabular retroversion and femoral retroversion
 Increased mechanical shearing forces at the physis
 History of previous radiation therapy to the femoral head region
PERTHES AND SCFE.ppt
PATHOPHYSIOLOGY
 Due mechanical forces acting on a susceptible physis
 Pathoanatomy
 Slippage occurs though the hypertrophic zone of the physis
PATHOPHYSIOLOGY
 Perichondrial ring thins and weakens
 Undulating mammillary processes in physis unlocks, further
destabilizing the physis
 Physis is still vertical in this age group (160° at birth to 125° at skeletal
maturity), resulting in increased shearing forces
 Epiphyseal tubercle can provide a rotational pivot point
 Anatomic structure in the posterior superior epiphysis that shrinks with
skeletal maturity
 Cartilage in the hypertrophic zone acts as a weak spot
PATHOPHYSIOLOGY
 Angulation
 Metaphysis translates anterior
and externally rotates
 Epiphysis remains in the
acetabulum, lies posterior to the
translated metaphysis
 Similar to Salter-Harris type
I fracture
PRESENTATION
CLINICAL FEATURES
 Abnormal gait / limp
 Antalgic, waddling, externally rotated
gait or Trendelenburg gait
 Decreased hip motion
 Obligatory external rotation during
passive flexion of hip (Drehmann sign)
 Due to a combination of synovitis and
impingement of the displaced anterior-
lateral femoral metaphysis on the
acetabular rim
CLINICAL FEATURES
 Loss of hip internal rotation, abduction, and flexion
 Abnormal leg alignment
 Externally rotated foot progression angle
 Weakness
 Thigh atrophy
ASSESSMENT XRAY
PERTHES AND SCFE.ppt
CLASSIFICATION
TREATMENT
 Percutaneous in situ
fixation.
 Contralateral prophylactic
insitu fixation.
 Severe cases:
 open epiphyseal
reduction and fixation.
 proximal femoral
osteotomy
TREATMENT
a) MCQ’s
1)Average age of onset of perthes disease is :
a) 5y
b) 6y
c) 7y
d) 8y
2)Which of the following is true:
a) Autosomal dominant
b) Bilateral in 1-2%
c) Boys : girls - 1 : 4
d) Self limiting
3)Which of the following is head at risk sign:
a) Salters sign
b) Gazes sign
c) Caffeys sign
d) Crescent sign
4)Gazes sign means:
a) Radiolucent V in lateral aspect of epiphysis
b) Metaphyseal crest
c) Lateral subluxation
d) Horizontal physeal line
5)Metaphyseal vessels ceases at :
a) At birth
b) 1-2y
c) 4-7y
d) 10y
6)Perthes disease is a self limiting disease, it ,ay last for:
a) 2-3 months
b) 1-2y
c) 3-4y
d) 7-8y
7)What sign is this:
a) Gazes sign
b) Salters sign
c) Galeazzi sign
d) Caffeys sign
8)Which of the following is false with respect to SCFE:
a) Common in males
b) Seen in obese children
c) Not associated with puberty
d) Incidence : Left > bilateral > right
9)In SCFE, slippage occurs through which zone of physis:
a) Proliferative
b) Reserve
c) Hypertrophic
d) Spongiosa
10)What sign is this:
a) Salters sign
b) Crescent sign
c) Drehmann sign
d) Trethowan sign
THANK YOU
PERTHES AND SCFE.ppt
PERTHES AND SCFE.ppt

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PERTHES AND SCFE.ppt

  • 1. PERTHES DISEASE AND SCFE DR ARUNODAYA SIDDARTHA
  • 3. HISTORY Arthur Legg described prominent characteristics of the disorder: onset between 5 and 8 years, history of trauma, painless limp, minimal or no spasm or shortening of affected limb. Jacques Calve noted that affected individuals had minimal atrophy of the leg and no palpable hip swelling; abnormal or delayed bone formation
  • 4. Georg Perthes observed the disorder as “a self limiting, non inflammatory condition, affecting the capital femoral epiphysis with stages of degeneration and regeneration, leading to restoration of the bone nucleus” Henning Waldenstrom reported radiographic changes associated with the disorder in 1909; thought the disease was a form of Tuberculosis.
  • 5. Epidemiology  Incidence 1-4/10,000  Age 4 - 10years; average 7 yrs  As early as 2yrs as late as teens  Boys : girls 4:1  Bilateral 10-12%  No evidence of inheritance  Common in Caucasians; rare in black races
  • 7. ETIOLOGY 1. Vascular supply: Medial circumflex artery is missing or obliterated and obturator artery or lateral epiphyseal artery also affected. 2. Increased intra-articular pressure 3. Increased intraosseous pressure: Impaired venous drainage in femoral head 4. Coagulation disorder: Absence of factor C or S, increase in serum levels of lipoproteins, thrombogenic substance, Factor V Leiden mutation. 5. Growth hormones- Reduced levels of growth hormones, somatomedin A and C.
  • 8. 6. Social conditions- Lower socioeconomic status, dietary and environmental factors. 7. Trauma: Risk of vascular interruption increased due to narrow passage and thick cartilage of femoral head penetrated by lateral epiphyseal arteries 8. Abnormal growth and development: Bone age is lower than chronological age by 1-3 yrs (Radiological pause). Usually shorter than their peers. 9. Genetic factors - Inheritance 2-20%; inconsistent pattern. - Low birth weight, abnormal birth presentations. - Ratio of affected 1st, 2nd, 3rd and 4th degree relatives to general population of the same set – 35: 4: 4:1
  • 9. Pathophysiology  Rapid growth occurs in relation to devlopment of blood supply  Interruption of blood supply results in necrosis, removal of necrotic tissue, and its replacement with new bone.  Bone replacement may be so complete and perfect that completely normal bone may result  The adequacy of bone replacement depends on  Age of the patient  Congruity of the involved joint
  • 10. Sources of blood supply  Up to 4years  Metaphyseal vessels  Retinacular vessels  Ligamentum teres – scanty  4 to 7 years  Metaphyseal vessels ceases  Above 7years  Vessels in ligamentum teres have developed
  • 11. Pathology  Goes through stages which may last 3 to 4 years  Stage1  Ischaemia and bone death, cartilage thickens  Stage 2  Revascularization and repair  Dead marrow replaced by granulation tissue  Bone revascularized and new bone laid down  Dead bone resorbed, replaced by fibrous tissue, fragmentation  Stage 3  Distortion and remodelling  Restoration of femoral archtecture or collapse  Femoral head displaces laterally in relation to acetabulum
  • 14. Classification  Waldenstrom classification  Catterall classification  Salter and thompson classification  Herring classification
  • 16. Stage Ia Stage Ib Stage IIa Stage IIb Stage IIIa Stage IIIb Stage IV
  • 17. Catterall classification  Group I: Only anterior portion of epiphysis affected  Group II: More of anterior segment involved; central sequestrum present  Group III: Most of the epiphysis sequestrated, with unaffected portions located medial and lateral to central segment  Group IV: Entire epiphysis sequestrated BENIGN PROGNOSIS REQUIRE TREATMENT
  • 19. Group I Group II Group III Group IV
  • 20. HEAD-AT-RISK FACTORS  Lateral subluxation of femoral head  Radiolucent V in the lateral aspect of epiphysis (Gage sign)  Calcification lateral to epiphysis  Horizontal physeal line  Metaphyseal cyst
  • 21. Salter-Thompson classification  Based on extent of subchondral fracture on AP and lateral views  Group A: Less than half of femoral head involvement  Group B: More than half of femoral head involvement  Advantage: Applicable at an earlier time point than Catterall or lateral pillar classification  Disadvantage: Subchondral fracture present in only 30 % of patients
  • 22. SALTER sign  As disease progresses, a subchondral fracture may be seen on anterolateral aspect of femoral capital epiphysis  Crescent sign/ Caffey’s sign  Early radiographic feature best seen on frog leg lateral view
  • 25. Bilateral involvement  More severe than unilateral  Boys and girls equally affected  Independent event  Bone age delayed in perthes disease
  • 26. ClInical features  Incidence: higher latitude, western coastal region of South India  Onset: 18 months to skeletal maturity (Most prevalent: 4-8 years)  Male sex prevalence: Boys 4-5 times more susceptible  Involvement: Bilateral in 10-12 %
  • 27. SYMPTOMS  Limp  Pain  History of antecedent trauma  Waxing and waning symptoms
  • 28. SIGNS  Small stature  Atrophy of gluteus, quadriceps and hamstring muscles  Abductor limp ( Antalgic + Trendelenburg gait)  Decreased hip ROM especially abduction and internal rotation (Transient early in disease, persistent later on)  Positive Trendelenburg test  Resistance to logroll
  • 29. Investigations  Blood tests  haemogram, ESR, CRP  Imaging  Plain X-rays  Hip U/S  Bone scintigraphy  MRI  Dynamic arthrography  Assess spherity of femoral head  Hinge abduction  Bilateral perthes  Skeletal survey as part of work-up
  • 30. MRI  Accurate for early diagnosis and for visualising configuration of femoral head and acetabulum  More reliable information about true extent of femoral head necrosis than radiography or scintigraphy  Gadolinium enhanced subtraction MRI as effective as scintigraphy in delineating epiphyseal necrosis early  De Sanctis classification: Group A: < 50 % head necrosis Group B: > 50 % head necrosis B0- B3 based on degrees of lateral extrusion and physeal disruption
  • 31. TREATMENT OPTIONS 1. SYMPTOMATIC THERAPY  Bed rest  Non weight bearing on affected limb  Short term use of NSAIDs  Traction:  Simple longitudinal traction  Balanced suspension and traction  Russell traction  “Slings and springs”
  • 33. CONTAINMENT BY BRACING OR CASTING:  Aims at repositioning extruded anterolateral part of femoral epiphysis into confines of acetabulum  Achieved by abducting and flexing or internally rotating the hip  Needs to be ensured until healing progresses beyond late part of stage of regeneration ( upto 2 years)  Femoral head reforms in a concentric manner- Biological plasticity
  • 34. Broomstick plasters (Petrie casts) Snyder sling A-frame brace Toronto brace
  • 35. Treatment: Two main choices  Conservative  Pain control  Gentle exercises  Regular re-assessment  Avoid sport and strenous activities  Containment  Hold hips widely abducted in cast/brace >1yr  Operation  Varus osteotomy of femur  Innominate osteotomy of pelvis  Both
  • 37. Herring Guidelines to treatment  Children <6years  Symptomatic treatment  Children >6years; bone age more imp than chronological age  Bone age at or <6yrs  Lateral pillar A or B/ caterall I and II  Symptomatic treatment  Lateral pillar C/ Caterall III and IV  Bone over 6years  Herring A and B/Caterall I and II  Abduction brace or osteotomy  Herring C/Caterall III and IV  Outcome unaffected by treatment  Children 9yrs and older  Except in very mild cases, operative containment is the treatment of choice
  • 38. Prognostic features  Age  <6yrs; good regardless of treatment  6-9years; not always satisfactory with containment  >10yrs; questionable benefit from containment, poor prognosis  Gender  Girls have worse prognosis  Classification grade  Herrings lateral pillar classification  Salter and thompson grade B worse prognosis  Caterral classification grade  Caterral “head-at-risk” signs  The five signs carry worse prognosis  Others  Body weight, decreased ROM
  • 40. SCFE Slipped capital femoral epiphysis (SCFE) is an condition of the proximal femoral physis that leads to slippage of the metaphysis relative to the epiphysis, and is most commonly seen in adolescent obese males
  • 41. EPIDEMIOLOGY  Most common disorder affecting adolescent hips  Found in 10 per 100,000  More common in  Obese children, associated with puberty  Males (male to female ratio is 2:1.4)  Specific ethnicities - African Americans, Pacific islanders, Latinos  During period of rapid growth (10-16 years of age)  location  left hip is more common  bilateral in 17% to 50% (~25%)
  • 42.  RISK FACTORS  Obesity  Single greatest risk factor  Recent data shows trend towards younger age and increased frequency of bilaterality at presentation, possibly related to increased rates of childhood obesity  Acetabular retroversion and femoral retroversion  Increased mechanical shearing forces at the physis  History of previous radiation therapy to the femoral head region
  • 44. PATHOPHYSIOLOGY  Due mechanical forces acting on a susceptible physis  Pathoanatomy  Slippage occurs though the hypertrophic zone of the physis
  • 45. PATHOPHYSIOLOGY  Perichondrial ring thins and weakens  Undulating mammillary processes in physis unlocks, further destabilizing the physis  Physis is still vertical in this age group (160° at birth to 125° at skeletal maturity), resulting in increased shearing forces  Epiphyseal tubercle can provide a rotational pivot point  Anatomic structure in the posterior superior epiphysis that shrinks with skeletal maturity  Cartilage in the hypertrophic zone acts as a weak spot
  • 46. PATHOPHYSIOLOGY  Angulation  Metaphysis translates anterior and externally rotates  Epiphysis remains in the acetabulum, lies posterior to the translated metaphysis  Similar to Salter-Harris type I fracture
  • 48. CLINICAL FEATURES  Abnormal gait / limp  Antalgic, waddling, externally rotated gait or Trendelenburg gait  Decreased hip motion  Obligatory external rotation during passive flexion of hip (Drehmann sign)  Due to a combination of synovitis and impingement of the displaced anterior- lateral femoral metaphysis on the acetabular rim
  • 49. CLINICAL FEATURES  Loss of hip internal rotation, abduction, and flexion  Abnormal leg alignment  Externally rotated foot progression angle  Weakness  Thigh atrophy
  • 53. TREATMENT  Percutaneous in situ fixation.  Contralateral prophylactic insitu fixation.  Severe cases:  open epiphyseal reduction and fixation.  proximal femoral osteotomy
  • 55. a) MCQ’s 1)Average age of onset of perthes disease is : a) 5y b) 6y c) 7y d) 8y
  • 56. 2)Which of the following is true: a) Autosomal dominant b) Bilateral in 1-2% c) Boys : girls - 1 : 4 d) Self limiting
  • 57. 3)Which of the following is head at risk sign: a) Salters sign b) Gazes sign c) Caffeys sign d) Crescent sign
  • 58. 4)Gazes sign means: a) Radiolucent V in lateral aspect of epiphysis b) Metaphyseal crest c) Lateral subluxation d) Horizontal physeal line
  • 59. 5)Metaphyseal vessels ceases at : a) At birth b) 1-2y c) 4-7y d) 10y
  • 60. 6)Perthes disease is a self limiting disease, it ,ay last for: a) 2-3 months b) 1-2y c) 3-4y d) 7-8y
  • 61. 7)What sign is this: a) Gazes sign b) Salters sign c) Galeazzi sign d) Caffeys sign
  • 62. 8)Which of the following is false with respect to SCFE: a) Common in males b) Seen in obese children c) Not associated with puberty d) Incidence : Left > bilateral > right
  • 63. 9)In SCFE, slippage occurs through which zone of physis: a) Proliferative b) Reserve c) Hypertrophic d) Spongiosa
  • 64. 10)What sign is this: a) Salters sign b) Crescent sign c) Drehmann sign d) Trethowan sign