how to approach a case of perthes disease .

1. CASE DISCUSSION OF PERTHES DISEASE
Dr. Siddharth Deshwal

2. • I am presenting the case of child name_________,a
_____yrs.(usually 4-10 yrs.) old male/female(M:F-4:1) child of lower
socioeconomic status(usually) with chief complains of-
1.Painless limp x ______duration(mostly)
2.In some cases can present with-
• Pain and stiffness ± in right/left groin region radiating to thigh or
knee .
3. Sometimes only knee pain(referred pain) can be the only chief
complain .

3. HOPI
• Patient was apparently well _____time back when parents
noticed /child experienced pain (if present) in his /her left/right
side groin region which was insidious/sudden in onset,
mild/moderate in intensity ,radiating to thigh/knee ,aggravated
on activity(playing, running etc.) and relieved on taking rest and
medications.

4. PAST HISTORY&TREATMENT
HISTORY
• There can be similar history in the past of about ____weeks
back which recovered spontaneously/with ______treatment.

5. POSITIVE HISTORY
• There can be a positive history of minor trauma around
hip_______ weeks back .

6. NEGATIVE HISTORY
• Other joint involvement.
• Chronic cough.
• Tuberculosis history in family ,friends or neighbors.
• Low grade fever , night pain .
• Significant trauma requiring prolonged bed rest or
hospitalization .
• Severe pain in any hip with high grade fever requiring
hospitalization/treatment .

7. BIRTH HISTORY
• There can be history of –
• Low birth weight.
• Later born child(3rd or 4th order child).
• Abnormal birth presentation.
• Any associated congenital anomaly like –
• Congenital cardiac anomalies
• Pyloric stenosis
• Inguinal hernia
• Genitourinary disorders
• Undescended kidneys

8. FAMILY HISTORY
• Positive in 10% cases .

9. EXAMINATION
• General examination –
• The child appears to be undersized and of short stature .
• Gait –
• Antalgic or Trendelenburg .
• Trendelenburg sign±

10. LOCAL EXAMINATION
• INSPECTION (Positive findings)
• Lumbar lordosis can be exaggerated in fixed flexion deformity of
hip .
• Wasting of gluteal region muscles /thigh muscles/both of affected
side .
• Limb length discrepancy can be present(usually apparent
shortening due to muscle spasm ,but can be true in case of
established severe coxa vara deformity).

11. • PALPATION(Positive findings)
• Tenderness in groin region(anterior hip point) of the affected
side.
• Roll test positive (Adductor spasm ±).

12. • MOVEMENTS(Positive findings)
• Fixed flexion deformity of hip ( ___⁰ ) can be present .
(measured by doing Thomas hip knee flexion test )
• Abduction in flexion of hip( ___⁰ ) and internal rotation in flexion
of hip ( ___⁰ ) are decreased wrt. opposite side .
• Sectoral sign may be positive .
• Range of motion of knee - full .

13. • MEASUREMENTS-(Positive findings)
Circumferential- thigh wasting of __cms on (R/L)side ___cms
above the medial joint line as compared from normal side.
Linear –apparent/true shortening of ___cms on (R/L)side as
compared from normal side .

14. DIFFERENTIAL DIAGNOSIS
• TRANSIENT SYNOVITIS(IRRITABLE HIP)-
• Restriction of all movements with pain at the extreme range of movement .
• Symptoms last for 1-2 weeks and then subsides spontaneously.
• Xray findings are normal .
• EARLY TUBERCULOUS HIP-
• Extreme of movements are painful ,thigh wasting is gross , ESR ↑, TLC count ↑ ,differential count( relative
lymphocytosis) .
• LOW GRADE SEPTIC ARTHRITIS HIP-
• The affected limb may be held absolutely still and all attempts at moving the hip are resisted .
• The diagnosis is confirmed by aspirating the pus or fluid from joint and submitting it for laboratory examination and
bacterial culture .

15. DIFFERENTIAL DIAGNOSIS
• SLIPPED CAPITAL FEMORAL EPIPHYSIS-
• The patient is usually a child around puberty, typically overweight or very tall and thin.
• On x-ray –lateral view the femoral epiphysis is tilted backward .
• MONOARTICULAR JUVENILE RHEUMATOID ARTHRITIS-
• The hallmark of the disease is progressive bone destruction on both sides of the joint without any reactive osteophyte
formation .
• All movements are restricted and painful .
• On X-ray-acetabulum and femoral head are eroded.
- protrusio acetabuli is common .

16. MANAGEMENT
• Investigations:-
1. Radiological
• Xray pelvis with both hip Ap view (for comparison with other
hip).
• Frog leg lateral view of affected hip.
2. Blood investigations-
• Complete blood count-normal and to rule out other
differentials(septic arthritis , tb hip)
• ESR-normal or mildly raised in perthes (very high in septic
arthritis where TLC is also increased ,with increased
polymorphs in DLC.)

17. IMAGING
• In Perthes disease the diagnosis is made and the clinical
course assessed by plain radiographs taken in the
anteroposterior and frog-leg lateral view.
• The extent of the epiphyseal involvement (Caterall group I – IV ,
Salter Thompson type A or B , Lateral Pillar group A,B or C) and
the stage of the disease can be determined.

18. MODIFIED WALDENSTROM CLASSIFICATION
• Radiographic staging of disease evolution : Based on the
modified version of Waldenstrom’s classification
4 STAGES :-
1. Initial stage
2. Fragmentation stage
3. Reossification stage
4. Residual stage

19. INITIAL STAGE(3-6 MONTHS)
• Widening of the medial joint space
• Lateralization of femoral head in the acetabulum
• Smaller ossific nucleus due to cessation of growth of the
capital epiphysis
• WALDENSTROM’S SIGN : Linear fracture in the
subchondral area of femoral head ( frog leg lateral view)

20. INITIAL STAGE

21. FRAGMENTATION STAGE(6-12 MONTHS)
• Lucencies develop in ossific nucleus
• Central segment gets demarcated from medial and lateral
segments of the femoral head
• Acetabular contour becomes more irregular
• End of this stage is marked by appearance of new bone in
subchondral area of femoral head

22. REOSSIFICATION STAGE/HEALING STAGE (12-18 MONTHS)
• Reossification of nucleus begins peripherally and progress
centrally  necrotic bone is fully removed .
• Lucent portions of femoral head fill in with woven bone
• Finally head regains roundness .

23. REOSSIFICATION STAGE/HEALING STAGE

24. RESIDUAL STAGE/REMODELLING STAGE
• Head fully reossified
• Remodeling of head continues until skeletal maturity when
the permanent contour is established .
• Acetabulum remodels as well .

25. RESIDUAL STAGE/REMODELLING STAGE

26. CATERALL CLASSIFICATION
• Applied during fragmentation stage of disease as described by
waldenstrom .
• Based on amount of epiphyseal involvement .

27. GRADING :
Grade I -
• represents <25 % epiphyseal involvement .
• usually anterior portion of the epiphysis is involved with no
metaphyseal reaction.
Grade II –
• 25-50 % epiphyseal involvement .
• Progress towards lateral epiphysis.
• Sequestrum +nt.
• Metaphyseal reaction present (anterior and lateral part)
• Subchondral fracture line in anterior half of epiphysis.

28. GRADING:
Grade III-
• Involves 50-75% of the epiphysis with sequestrum.
• Posterior subchondral fracture line present .
Grade IV-
• 100% epiphyseal involvement.
• Diffuse metaphyseal involvement.

29. “Head at Risk "signs
Radiological signs
1. Gage sign( V shape osteoporotic segment in lateral epiphysis and adjacent metaphysis)
2. Lateral subluxation of femoral head
3. Speckled calcification in lateral epiphysis
4. Horizontal physis
5. Metaphyseal cyst formation
Clinical signs
1. Loss of hip motion is persistent and progressive
2. Increased adduction contracture of hip
3. The obese child
4. Female child
5. Age >7yrs

30. HEAD AT RISK SIGNS(RADIOLOGICAL)

31. HEAD AT RISK SIGN –GAGE SIGN

32. HEAD AT RISK SIGN -METAPHYSEAL CYSTS

33. HEAD AT RISK SIGN -CALCIFICATION LATERAL TO EPIPHYSIS

34. SAGGING ROPE SIGN
• Radio dense line overlying the proximal femoral metaphysis produced by
growth plate damage associated with metaphyseal
response.

35. SALTER AND THOMSON CLASSIFICATION

36. HERRING’S LATERAL PILLAR CLASSIFICATION

37. STULBERG’S CLASSIFICATION

38. MRI OF AFFECTED HIP
• It is postulated that gadolinium-enhanced MRI may be able to
predict the prognosis at an early stage .

39. ULTRASOUND OF AFFECTED HIP
• May be able to differentiate between -transient synovitis with
capsular distention due to synovial effusion and Perthes
Disease with thickening of the synovial membrane.
• It can demonstrate irregularity of the anterior and superior
surface of the femoral capital epiphysis consistent with Perthes
disease .

40. ARTHROGRAM
• It is useful primarily in demonstrating any flattening of the
femoral head that may not be seen on X-Ray .
• In conjunction with plain film or CT may be useful in diagnosing
Osteochondritis dissecans following Perthes disease .
• It is most useful for assessing head shape and the relation to
the acetabulum that would be necessary for treatment decisions
.

41. • It is helpful in determining containability before any treatment.
• It is also useful in determining the best position of containment .

42. Radionuclide Bone Scanning
• Bone Scanning with technetium and pin hole columnation may
be helpful in early stages of the disease when the diagnosis is
in question. Here uptake of the normal side compared with the
disease side .
• The technique gives improved definition of the acetabular roof,
femoral capital epiphysis , physis and greater trochanter .

43. TREATMENT OBJECTIVES
• To produce a normal femoral head and neck(prevent
deformation & enlargement) .
• To produce a normal acetabulum .
• A congruous hip which is fully mobile .
• To prevent degenerative arthritis of the hip later in life .

44. • Goals of Treatment:
• Containment of the femoral head.
• Elimination of hip irritability.
• Restoration and maintenance of a good range of hip motion.
• Prevention of epiphyseal extrusion and subluxation.
• Attainment of a spherical femoral head on healing .

45. PRINCIPLES OF CONTAINMENT
• The objective of containment treatment is to hold the femoral
head in the acetabulum during the period of “biological
plasticity” while necrotic bone is resorbed and living bone is
restored through the process of “creeping substitution “,so
that when repair and remodeling occurs , the head becomes
almost spherical .

46. • Containment is useful only in revascularization and repair
phase.
• Prerequisites of containment -full range of motion .
• Hinged abduction is a contraindication to containment.
• Containment should start before the late fragmentation stage
and should continue till late in the repair/remodelling phase .

47. • Containment can be done-
Conservatively by-
• Orthosis-which holds the affected hip in abduction and internal
rotation.
Surgically by-
Osteotomies
1. Femoral (Varus subtrochanteric derotation) osteotomy
2. Pelvic osteotomy
3. Combined femoral +pelvic osteotomy

48. Orthosis
Non Ambulatory weight relieving
1. Abduction broomstick plaster cast
2. Hip Spica cast
3. Milgram hip abduction orthosis
 Ambulatory Both limbs included(bilateral)
1. Petrie Abduction cast
2. Toronto orthosis
3. Newington orthosis
4. Birmingham brace
5. Atlanta Scottish Rite Brace
 Ambulatory unilateral
• Tachdjian trilateral socket orthosis

49. DIFFERENT TYPE OF ORTHOSIS USED IN
PERTHES DISEASE TREATMENT
NEWINGTON BRACE
TORONTO
ORTHOSIS

50. Orthosis
CONTRAINDICATIONS
• Incompliant patient.
• Psycho socially unacceptable for the patient or parents.
• Bilateral involvement at different times requring prolonged
brace wear.
DISADVANTAGES
• Stiffness of the knee and ankle joint with adaptive articular
changes.
• Restricted ambulation.
• Pressure sores and need for frequent changes.

51. SURGICAL CONTAINMENT
INDICATIONS:
– Age of clinical onset > 8yrs of age
– Herring type B
– Radiological evidence of loss of containment by conservative modes
CONTRAINDICATIONS:
– Herring’s type A and C
– Herring’s type B if child less than 8 yrs
– Healed cases.
– Hinged abduction
 Age at surgery: - Should be done in the increased density or early fragmentation phase.

52. •COMPLICATIONS
• Femoral
• Shortening
• Stiffness
• Malrotation
• Limp
• Positive Trendelenburg test .
• Pelvic
• Lengthening
• Stiffness
• Chondrolysis
• Failure of containment

54. RECENT
ADVANCES

55. • ARTHRODISTRACTION
• FENESTRATION EPIPHYSEAL GROWTH PLATE
• DRUGS AND GROWTH FACTORS
• GENETIC

56. ARTHRODISTRACTION
• New method of treatment in perthes disease.
• Tries to neutralize muscular and weight bearing forces on the
femoral epiphysis ,induce neovascularization, and prevent
femoral head distraction.

57. FENESTRATION OF EPIPHYSEAL GROWTH PLATE
• Fenestration made in neck anteriorly with 3 or 4 drill holes or a curet through
epiphyseal growth plate reduce time for resorption and regeneration .

58. GROWTH FACTORS AND INDUCING DRUGS
Low plasma levels of insulin-like growth factor 1(IGF-1) stimulate healing to prevent
deformity .
Bisphosphonates:
-The third-generation bisphosphonate (zoledronic acid) is currently being explored as a
treatment for children presenting with Perthes disease .
-Ibandronate : shown there importance in rat model by increase sphericity of femoral head.

59. GENETIC ANALYSIS
• A protective effect was seen with HLA-A 2 and HLA-Cw3 .

60. THANK YOU