perthes disease by Dr jaskaran Singh Sandhu MS ortho

1. Perthes disease
by Dr. Jaskaran Singh Sandhu

2. A condition in which the
blood supply of the
capital femoral epiphysis
(head) is disrupted
- epiphyseal osteonecrosis
- chondronecrosis with
cessation of growth of the
ossific nucleus

3. • Common in 4 -8 years old
• Male:Female 4.5:1
• Unilateral more common than bilateral (10-
12%)
• No involvement of the other joints and spine

4. Aetiology
• The exact cause is still unknown
• Several theories have been proposed but none
definitely proved
• Theories :
 Compromised vascular supply :medical
circumference artery is missing and
obliterated in many cases , obturator artery
and lateral epiphyseal artery are also affected
in some cases

5.  Increased intra articular pressure
Raised intraosseous pressure
Coagulation disorder :Protein C or S deficiency
Social conditions : low socioeconomic status
Genetic factor : first degree relatives are 35
times more likely to suffer
Environmental factor : Passive tobacco smoke,
firewood smoke also associated

9. Clinical features :
• Limp (often painless)
• Mild to moderate hip, thigh, knee pain
• The range of motion of the hip is restricted, in
particular abduction and internal rotation .
• Positive Trendelenberg test
• Slight shortening

10. Radiographs :
• Early
 Joint space widening (
due to hypertrophy of the
articular cartilage )
 Increased density of
femoral epiphysis ( due
to loss of vascularity )
 Subcondral fracture (
salter’s sign or crescent
sign ) seen on lateral
radiograph ( signifies
beginning of collapse .

11. • Midstage
 Fragmentation and
flattening of head
 Widening of physis
 Femoral neck cysts
(appears as a result of
metaphyseal changes as
the part of disease
process )
 Extrusion of the femoral
head (due to flattening of
the head and increase in
medial space )

12. Late
 Coxa magna
 High riding trochanter ( as
a result of retarded growth
in the capital physes )
 Flattened femoral head
 Irregular articular surface
 Bi-compartmentalization of
acetabulum ( secondary
remodeling of the
acetabulum over the
flattened and subluxed
femoral head )

13. Waldenstrom staging
Stage Characteristics
1 Avascular stage Joint space widened (waldenstron sign )
Lateralisation of femoral head
Femoral head appears slightly denser than normal and slightly
falttened
2 Stage of resorption
(Fragmentation )
Femoral head breaks up into fragments
Lucent areas appears in the femoral head
Increased density resolves
Acetabular contour is more irregular
3. Stage of reossification The femoral head is rebuilt
New bone formation occurs in the femoral head
4. Healing stage End stage with or without defect healing

15. CATERALL CLASSIFICATION
• This grading system is applied
during the fragmentation stage of
disease as described by
Waldenstrom and is based on the
amount of epiphyseal involvement.
• Grade 1 – represents less than 25%
involvement, usually involving the
anterior portion of the epiphysis
with no metaphyseal reaction.
• Grade 2 – involves 25–50% and
progresses towards lateral
epiphysis. A sequestrum is often
present along with anterior and
lateral metaphyseal reaction and a
subchondral fracture line in the
anterior half of the epiphysis

16. • Grade 3 – involves 50–75%
with sequestrum and
posterior subchondral
fracture line.
• Grade 4 – involves 100% of
the epiphysis with diffuse
metaphyseal involvement.
• This grading system has
demonstrated poor inter-
observer reliability; however,
a grade of 3 or 4 has been
shown highly predictive of
poor outcomes

17. • Caterall also described several
‘head-at-risk’ signs on
radiography that correlated with
a more severe disease course
and poorer outcomes.
These include :
• Lateral subluxation of the
femoral head,
• Speckled calcification lateral to
the capital epiphysis,
• A horizontal physis,
• Metaphyseal cyst formation and
• Gage’s sign : Radiolucent V
shaped defect in the lateral
epiphysis and adjacent
metaphysis .

18. SALTER AND THOMPSON
CLASSIFICATION
• The classification is based on subchondral fracture .
• This system can be applied once a fracture line is present, often up to 8
months before full fragmentation when the Caterall classification can be
applied.
• • Type A disease – involves less than 50% of the femoral head and has a
good prognosis.
• • Type B disease – involves over 50%, often with associated lateral pillar
collapse and suggests poor outcomes.
• The main drawback of this classification is that up to two-thirds of patients
do not demonstrate a clear subchondral fracture line.

19. HERRING CLASSIFICATION
• This is the most commonly used classification system and it can be
applied early in the fragmentation stage, usually within the first 6
months of clinical symptoms.
• The Herring classification is based on involvement of the lateral pillar
of the epiphysis, leading to loss of pillar height on AP radiograph.
• Group A – no lateral pillar involvement.
• Group B – over 50% of lateral pillar height
is maintained.
• Group B/C – 50% loss of height
• – B/C-1: the lateral pillar is less than 2–3 mm wide
• – B/C-2: there is minimal ossification
• – B/C-3: the lateral pillar is depressed in relation to the central pillar
of the femoral head.
• Group C – there is collapse and loss of over 50% of lateral pillar

20. • This classification has demonstrated strong
prognostic value and inter-observer reliability.
Group A is associated with universally good
outcomes;
• Group B hips generally have poor outcomes in
children older than 6 years and
• Group C hips have universally poor outcomes.

21. STULBERG GRADING
Stulberg grading is based on congruency and sphericity of the femoral
head at skeletal maturity.
• Grade I - normal, round femoral head.
• Grade II – Round head , coxa magna, coxa-breva or an abnormally steep
acetabulum , 15 % increase in risk of osteoarthritis .
• Grade III – Oval mushroom shaped head , , coxa magna, coxa-breva or an
abnormally steep acetabulum
• Grade IV – Flat head congurent with acetabulum .
• Grade V – Flat head incongurant

22. Modified Elizabeth town classification
• 1A : Epiphysis is avascular and appears dense, no loss of
height
• 1B : There is some height loss of the dense sclerotic
epiphysis
• 2A : Sclerotic epiphysis begins to fragment
vertical 1 or 2 fissure present
• 2B : Fragmentation advanced
• 3A : New bone formation , cover less than half width of
epiphysis
• 3B : New bone , normal texture grow over one third of
the epiphysis .
Containment gives best results when performed before
stage 2B

25. Prognostic features :
• Perthes is self limiting illness and 60-80 % good
excellent outcome .
• Asphericity of the femoral head predisposing to
future osteoarthritis .
• Age at presentation – 6yr better outcome
- 8yr poorer outcome
• Heavy child , progressive loss of hip movement ,
adduction contracture in the hip is poor prognosis
• Longer duration from the onset of disease to
complete healing – poor prognosis

26. Investigation
• Diagnosis can be made on plain xray . Staging
system are also based on the plain x rays .
• MRI – not important in the diagnosis of the
perthes disease . The advantage include
ability to visualised the location and extend of
femoral head ,physis , articular cartilage
involvement and before radiological diagnosis
• Arthrography : invasive and difficult to repeat
at follow ups .

27. Differential diagnosis
1 Epihyseal dysplasia :
• Bilateral involvement
• Largely symmetrical finding
• Possible involvement of other joints or spine
• Possible involvement of the acetabulum
• Few cystic and sclerotic changes in the femoral
head
• Little tendency towards lateral calcification and
lateral subluxation
• Typical stages like perthes disease are not appears.

28. 2. Osteochondritis dissencans of the femoral head
3. Chondroblastoma of the femoral head :
• The height of the femoral head is not initially
reduced , nor the cartilage thickened
• The pressure of non load related pain .
4. Meyers dysplasia :
• Causes collapse of the femoral head is similar
to perthes disease
• More common in boys younger than 4 yr of age
• Xrays shows delayed and similar ossification
centers , a seprated or cracked epiphysis , cystic
changes

29. • Subcondral fractures , fragmentatoion and
subluxation not seen .

30. Disorders associated with AVN of
femoral head in children
• Sickle cell anemia
• Thalassemia
• Klinefelter syndrome
• Down syndrome ( trisomy 21)
• Achondroplasia
• Gauchers disease
• Myelomeningocoele
• Hemophilia
• Congenital tibial arthrosis

31. Treatment
Goal : Relief of symptoms , restoration of ROM ,
containment of the hip .
•Most current therapeutic approaches are based
on the concept of containment, which, over the
years, has evolved to include nonoperative as well
as operative treatment methods.

32. Initial stage (Active )
• Symptomatic treatment for children with onset
on or before the eighth years
• Nonoperative or operative containment
treatment for children with onset on or before
the eighth years if they develop a loss of hip
abduction and lateral extrusion of the femoral
head
• Consideration of surgical treatment for
children with onset after the eighth year if
they present at the initial stage of disease and
show >50% head involvement

33. Preferred Treatment Approach for
the Fragmentation Stage
• Symptomatic treatment for children with onset after
eighth year, lateral pillar group A
• Surgical treatment for children with onset after eighth
year, if they present with lateral pillar groups B and B/C
border
• Nonoperative containment treatment for children with
onset after eighth year who present with lateral pillar
group C
• Nonoperative treatment choices: prolonged non–weight
bearing, Petrie casts, and wide abduction (A-frame) brace
• Surgical choices: femoral varus osteotomy, Salter
innominate osteotomy, both osteotomies combined (onset
after age 9 years)

34. Preferred Treatment Approach for
the Healing or Healed Stage
• Late measures: femoral valgus osteotomy
for established head and acetabular
flattening, adducted hip, short leg gait;
surgical hip dislocation with possible
trochanteric advancement and
osteochondroplasty for impingement
• Mechanical symptoms: hip arthroscopy
with removal of osteochondrotic fragment

35. Symptomatic therapy
• Bed rest
• Non weight bearing on the affected hip
• NSAIDS for pain
• Traction

36. Non surgical containment using
orthotic devices
• Before staring containment therapy , it is
important to restore normal range of motion
of the hip .

43. Surgical treatment
• Containment –
 Femoral osteotomy – proximal varus derotation
femoral osteotomy
 Pelvic Osteotomy – Salter, triple , Dega , pembertan
• Salvage –
Valgus femoral osteotomy for hinge abduction
Chiari and /or shelf pelvic osteotomies for the hips that can
no longer be contained

44. Femoral osteotomy
• Done in initial stages of the disease
• The groups with better results after surgery
were children older than 8 years of age at
onset who developed lateral pillar B or B/C
border hip in the fragmentation stage
• Lateral pillar C hip had the highest incidence
of poor outcome and were not shown to have
better results with the surgical management .