This document summarizes the pathogenesis and treatment of Legg-Calve-Perthes disease, a childhood hip condition caused by interrupted blood supply to the femoral head. The disease leads to bone death and deformity of the femoral head. Treatment options are controversial but generally involve either nonoperative casting or operative procedures like osteotomies, depending on the age, stage of disease, and extent of involvement. Current evidence suggests operative treatments may slightly increase the chance of a spherical femoral head developing, but the effect is modest and more research is still needed to develop more effective therapies.

1. The Pathogenesis and
Treatment of
Legg-Calve-Perthes Disease
Talal Ibrahim, and David G. Little
JBJS Reviews
Volume 4(7):e4
July 19, 2016
Dr Asish Rajak
JR, BPKIHS

3. Introduction
childhood hip condition ---- blood supply to the capital
femoral epiphysis is interrupted ---- osteonecrosis and
chondronecrosis ---- progressive deformity of the femoral
head and secondary degenerative osteoarthritis in later life.
Etiology : unclear -- biological and mechanical factors.

4. Treatment : controversial, dependent - the age at
clinical onset, the extent of epiphyseal involvement,
the stage of the disease, and the degree of femoral
head deformity.
Literature -- operative containment in the early stage
of disease.
6 to 7 patients need to be managed to create 1
spherical femoral head that would not have
otherwise occurred.

5. Epidemiology
4-8 yrs. , 5 x Males , 4-32 / 100,000
Caucasians, latitude , low socioeconomic class
higher in less densely populated areas

6. Etiology
Multifactorial – combination of genetic and
environmental factors.
Maternal smoking and passive smoking

7. Pathogenesis
 a single episode or multiple episodes of infarction
 Femoral head biopsy specimens – patients -- dead woven bone
superimposed on dead lamellar bone, with the marrow space
occupied with dead granulation tissue
 Initiation -- disruption of the blood supply to the capital femoral
epiphysis.
 Disruption of the lateral epiphyseal artery at its origin in 68% of
patients in all stages of the disease

8. affects the articular cartilage, epiphysis, physis, and metaphysis
trabecular bone and the marrow space necrotic
Repair : Osteoclastic bone resorption
replaced with fibrovascular tissue
necrotic femoral head with decreased mechanical stiffness
Microfractures (repeated loading)

9. Imaging and Classification
 Waldenstrom : 4 radiographic stages (2 yr. period)
1. The initial stage
2. The fragmentation stage
3. The reossification stage
4. The healed stage
 Dynamic Contrast-enhanced subtraction MRI -- the early stages

12. Natural history
self-healing
- rapid recanalization of existing vessels (weeks)
- formation of new vessels (months-yrs.)

13. Prognosis
Depends on
1. the age of the patient at the time of onset,
2. the stage of the disease,
3. the extent of epiphyseal involvement,
4. the lateral extrusion of the femoral head
Others: heavy patient, stiffness with progressive loss of
hip range of motion, adduction contracture, and a
longer duration from onset to completion of the healing
phase

14. Treatment
Goal : minimize femoral head deformity and risk of
secondary degenerative osteoarthritis in later life
The choice between operative and nonoperative
treatment is based on the concept of containment.

15. Nonoperative Treatment
Earlier
Broomstick Plaster – Harrison et al
Abduction casting with weight-bearing - Petrie and
Bitenc
Removable braces that mimic Petrie casts - Atlanta
and Toronto braces

17. The current evidence in the literature does not support the use
of orthoses for the treatment of LCP disease.
Atlanta Scottish
Rite hospital
brace

18. Non weight bearing
Controversial

19. Bisphosphonates and Anabolic agents
 Bisphosphonates: application in clinical use is unproven
 Osteoprotegerin-immunoglobulin Fc segment complex (OPG-
Fc) that inhibit RANKL (receptor activator of nuclear factor
kappa-B ligand)
 BMPs – experimental

20. Operative Treatment
 Osteotomy – femur / pelvis
 Femoral varus osteotomy (Soeure and De Racker – 1952)
 Innominate osteotomy (Salter, 1962)

21. Initial radiographs, made at the age of 8 years (Figs. 3-A and 3-B), and the most recent
radiographs, made at the age of 14 years (Figs. 3-C and 3-D), showing the hips of a boy with
right Legg-Calvé-Perthes disease who underwent nonoperative treatment.
Talal Ibrahim, and David G. Little JBJS Reviews 2016;4:e4
©2016 by The Journal of Bone and Joint Surgery, Inc.

22. Radiographs of the hips of a boy with bilateral Legg-Calvé-Perthes disease who underwent a
right proximal varus femoral osteotomy with a blade plate.
Talal Ibrahim, and David G. Little JBJS Reviews 2016;4:e4
©2016 by The Journal of Bone and Joint Surgery, Inc.

23. Femoral and Pelvic Osteotomies
Proximal femoral varus osteotomy - most widely,
should be performed before the advanced stage of
fragmentation (Joseph et al).
no differences -- femoral varus osteotomy vs Salter
innominate osteotomy.

24. Discussion
The authors recommended proximal femoral varus
osteotomy in patients with an age of >= 6 years at
the time of diagnosis and > 50% femoral head
necrosis and concluded that abduction orthoses
should be abandoned for the treatment of LCP
disease. (Wiig et al.)

25. Meta-analyses
operative treatment twice as likely to produce
a spherical congruent head than non operative
treatment with patients >=6 yrs, similar results
in <6 yrs (Nguyen et al , 23 studies)

26. Saran et al . (14 NRCT)
> 8 yrs. -- higher likelihood of femoral head
sphericity when treated operatively during or before
the fragmentation phase
6 to 8 years -- transitional group in which the role of
surgery is less obvious
< 6 yrs. -- no effect

27. these studies show that current operative
treatments have a relatively modest treatment
effect, and more research is required to
develop more effective therapies for LCP
disease in the future.

28. Noncontainable hips
late stages of LCP disease
Valgus femoral osteotomy
Shelf acetabuloplasty
( Added Acetabular procedures not
recommended in the young– remodelling)

29. Shelf acetabuloplasty
Meta-analysis :
Kadhim et al – final radiological outcome(Stulberg) –
early vs late intervention --- good outcome 85% -
early vs 69% late
Hsu et al – 13 studies – prevention of sec OA ,
uncertainty

30. Others
Advanced joint preserving surgeries
1. Core decompression
2. Arthrodiastasis: early stage, salvage procedure in
patients older than 8 years of age with advanced
disease

31. 3. Operative hip dislocation:
Intraarticular- osteochondroplasty of the head –
neck junction(femoroacetabular impingement) and
femoral head reduction surgery (coxa magna)
Extra articular- GT overgrowth, femoral neck
shortening
Salvage procedure
Total hip arthroplasty- healed with Sec
Osteoarthritis

32. Summary
 ideal treatment – elusive
 Prognosis - age of the patient at the time of onset, the extent of
epiphyseal involvement, and the stage of disease.
 Current evidence treatment increases the possibility of a spherical
femoral head, but the magnitude of this improvement is small and
the number needed to treat remains high because of a modest
treatment effect
 additional research- epidemiology, pathogenesis, and treatment
 antiresorptive and anabolic agents -- further investigation

33. Thank You