The Pathogenesis and Treatment of

Legg-Calve-Perthes DiseaseTalal Ibrahim, and David G. Little

JBJS Reviews

Volume 4(7):e4

July 19, 2016

Dr Asish Rajak

JR, BPKIHS

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.

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.

Epidemiology 4-8 yrs. , 5 x Males , 4-32 / 100,000

Caucasians, latitude , low socioeconomic class

higher in less densely populated areas

Etiology

Multifactorial – combination of genetic and environmental factors.

Maternal smoking and passive smoking

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

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)

Imaging and Classification Waldenstrom : 4 radiographic stages (2 yr. period) 1. The initial stage2. The fragmentation stage3. The reossification stage4. The healed stage

Dynamic Contrast-enhanced subtraction MRI -- the early stages

Natural history

self-healing rapid recanalization of existing vessels

(weeks) formation of new vessels (months-yrs.)

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

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.

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

The current evidence in the literature does not support the use of orthoses for the treatment of LCP disease.

Atlanta Scottish Rite

hospital brace

Non weight bearing

Controversial

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

Operative Treatment

Osteotomy – femur / pelvis

Femoral varus osteotomy (Soeure and De Racker – 1952)

Innominate osteotomy (Salter, 1962)

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.

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.

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.

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.)

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)

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

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.

Noncontainable hips

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

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

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

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

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

Thank You