Management of Legg-Calvé-Perthes Disease
Children diagnosed with Legg-Calvé-Perthes disease should be immediately referred to a pediatric orthopedic surgeon for specialized management, as this is the standard of care. 1
Diagnosis and Assessment
- Legg-Calvé-Perthes disease (LCPD) is a self-limiting idiopathic avascular necrosis of the capital femoral epiphysis in children 2
- Key prognostic factors that determine treatment approach:
- Age at disease onset (older children have worse prognosis)
- Degree of limitation of range of motion
- Extent of femoral head involvement
- Presence of "head-at-risk" signs on radiographs 3
Treatment Principles
The primary goal of treatment is to prevent femoral head deformity and premature coxarthrosis (hip arthritis) by maintaining or restoring joint congruence while biological plasticity is still present - known as the "containment principle" 3.
Treatment Algorithm:
Initial Assessment Phase
- Determine disease stage (Waldenstrom Stage I, II, or III)
- Assess lateral pillar classification (Herring classification)
- Evaluate range of motion and pain level
- Determine extent of femoral head involvement
Treatment Selection Based on Severity:
Mild Cases:
- Observation with frequent follow-up
- Regular radiographic monitoring
- Maintenance of range of motion through physical therapy
Moderate to Severe Cases:
- Non-surgical containment methods:
- Activity modification
- Bracing
- Physical therapy to maintain hip mobility
Severe Cases with Poor Prognosis:
- Surgical containment methods:
- Femoral osteotomy
- Pelvic osteotomy
- In some cases, soft tissue release with joint distraction using external fixation 4
Specific Treatment Approaches
Non-surgical Management:
- Weightbearing and activity restrictions are commonly prescribed during active disease stages
- Restrictions range from non-weightbearing with cast/brace to full weightbearing with activity limitations
- Important consideration: While mobility scores are lower with more severe restrictions, mental health measures (depression, anxiety) and social health (peer relationships) are not significantly affected by these restrictions 5
Surgical Management:
- Surgical intervention aims to achieve containment when non-surgical methods are insufficient
- Options include:
- Femoral varus osteotomy
- Pelvic osteotomy (Salter or triple)
- Soft tissue release with joint distraction using external fixation
- The latter has shown good results even in older children with typically poor prognosis 4
For Older Patients with Painful Sequelae:
- Palliative operations can be effective for adults with painful old Perthes' disease:
- Subcutaneous adductor tenotomy
- Cheilectomy of femoral head
- Multiple drilling for femoral head decompression
- Muscle-pedicle bone grafting
- These approaches have shown good to excellent results in most cases 6
Follow-up and Monitoring
- All children with LCPD require individualized treatment and continued follow-up into adulthood 3
- Regular monitoring of:
- Hip range of motion
- Pain levels
- Radiographic changes in femoral head shape
- Development of limb length discrepancy
Important Considerations and Pitfalls
- Early diagnosis and management are crucial to prevent femoral head collapse, progressive deformity, and impingement 2
- The variable course of LCPD means treatment must be determined individually in each case 3
- Age at onset is a critical prognostic factor - children diagnosed after age 8 typically have worse outcomes
- Avoid delaying referral to pediatric orthopedic specialists as early intervention may improve outcomes
- Consider the impact of restrictive treatments on quality of life, particularly physical mobility 5