Perthes’ Disease / Legg-Calvé-Perthes’ Disease (LCPD)

Perthes’ disease is an important differential diagnosis of a limping child.

A limp in a child may be due to a minor cause, but the serious differentials has to be exclude to arrive at a proper diagnosis.

Differential diagnosis of a limping child includes….



Perthe’s disease is a condition affecting the hip joint in children which occur due compromised blood supply to the femoral head leading to avascular necrosis of the femoral epiphysis of the femoral head.

Incidence : Rare condition, 4 of 100 000 children develops this condition

Prognosis of Perthes’ Disease is good. A very small proportion may develop osteoarthritis of the hip join in adult life.


Aetiology Of Perthes’ Disease

Exact cause is unknown
May occur following….

  • Trauma
  • Corticosteroid use
  • Sickle cell anaemia
  • Developmental dysplasia of the hip 



Pathophysiology Of Perthes’ Disease

Idiopathic interruption of the blood supply to the femoral head
                 ↓ 
Bone infarction — Avascular necrosis
                 ↓
Reduced range of motion of the hop joint
Increased risk of fracture
                 ↓
New blood vessels formation
Ossification begins to occur
                 ↓
Bone remodeling takes place 
                 ↓
Normally functioning hip joint (Only in some cases)


 Clinical Presentation Of Perthes’ Disease

  • Commonly affects children at 4 10 years old age
  • Males : Female ratio is 4 : 1
  • Mostly unilateral, but 20% of cases are bilateral 
  • Limping gait – Specially after exertion 
    • Hip abduction is more affected than other movements
  • Pain in hip joint, sometimes referred to the knee and thigh
  • Pain worsens with activity
    • Pain begins to occur with the onset of necrosis of the femoral head
  • Ultimately the child develops an antalgic gait with reduced hip movements 
  • These symptoms have “on and off” periods 



Investigations Of Perthes’ Disease

  • Plain X-Ray – Diagnostic 
    • AP view of the hip join
    • Frog-leg lateral view of the pelvis
       
  • These investigations are done only in some occasions 
    • Technetium bone scan 
    • MRI 



Staging Of Perthe’s Disease

Catterall Staging

  • Stage 1 – Clinical and histological features only 
  • Stage 2 – Sclerosis with / without cystic changes and preservation of articular surface
  • Stage 3 – Loss of structural integrity of the femoral head
  • Stage 4 – Loss of acetabular integrity 


Radiological staging

  • Stage 1 – Cessation of femoral epiphyseal growth 
  • Stage 2 – Subchondral fracture 
  • Stage 3 – Resorption 
  • Stage 4 – Reossification 
  • Stage 5 – Healed or residual stage



Management Of Perthes’ Disease

60% of cases heal with only conservative management
But the patient has to be followed up in the clinic every 4 monthly, till the patient is completely healed
Disease process may persist for years with “on and off” periods

Conservative management

  • Minimize weight bearing on the hip joint – To avoid injury and to promote regeneration 
    • Rest
    • Clutches 
    • Braces 
  • Analgesics
  • Physiotherapy

Surgical management
  • Done in patients with fracture of femoral head
  • Osteotomy is performed 
  • Surgical intervention has proved to have a better outcome than non surgical techniques 
  • But the rate of recovery is the same 


Complications Of Perthes’ Disease

  • During conservative management, some cases fail to achieve the spherical shape of the femoral head
    This leads to reduced range of motion of the hip joint
  • Surgery may result in a chronic limp

  

  

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