Legg Calve Perthes Disease: Symptoms, Types, Causes and Treatment

Legg-Calve-Perthes Disease (LCPD) is a childhood hip disorder that can have a profound impact on a child's mobility, comfort, and long-term joint health. Although the condition is relatively rare, its effects can be long-lasting and sometimes debilitating if not addressed early and appropriately. In this article, we explore the symptoms, types, causes, and treatment options for LCPD, synthesizing the latest evidence and insights from clinical research.

Symptoms of Legg Calve Perthes Disease

Legg-Calve-Perthes Disease often presents with subtle and gradually evolving symptoms, making early diagnosis challenging. Recognizing these symptoms is crucial in seeking timely intervention and improving outcomes for affected children.

Symptom	Description	Prevalence/Onset	Reference
Pain	Gradual, mild, activity-related hip/knee pain	Common, early stages	1, 3, 4
Limping	Lameness or Trendelenburg gait	Frequently observed	1, 3, 4
Limited Motion	Restricted hip abduction and internal rotation	Often present	1, 3, 4
Muscle Atrophy	Muscle wasting around the affected hip	Possible, later stage	3

Table 1: Key Symptoms

Understanding the Symptoms

The clinical presentation of LCPD can be variable, but several hallmark symptoms tend to appear as the disease progresses.

Gradual Onset Hip and Knee Pain

• Pain is usually mild and related to activity, often starting insidiously in the hip, thigh, or even the knee, which may mislead both caregivers and clinicians 1, 3, 4.
• Children may describe discomfort after play or physical activity, and pain may subside with rest.

Limping and Gait Changes

• Limping is a characteristic early sign, sometimes manifesting as a Trendelenburg gait in advanced cases 1, 3, 4.
• Limping may be subtle at first, often attributed to minor injuries or growing pains, but can become more pronounced over time.

Limited Hip Mobility

• Loss of range of motion, particularly in hip abduction and internal rotation, is a key diagnostic feature 1, 3, 4.
• Stiffness or reluctance to move the hip may be noticed by parents during activities like dressing or climbing stairs.

Muscle Atrophy

• Over time, muscle wasting around the affected hip can develop, especially if the child reduces activity to avoid discomfort 3.
• This is more common in advanced or untreated cases.

Additional Presentation Notes

• Symptoms may appear suddenly or gradually, with some children presenting with acute symptoms and others with a slow, insidious progression 3.
• Radiographic changes often lag behind clinical symptoms, making early diagnosis dependent on high clinical suspicion 1, 3.

Types of Legg Calve Perthes Disease

LCPD is not a uniform condition; it varies in severity, stage, and long-term outcome. Medical professionals have developed several classification systems to describe these differences, which are essential for guiding treatment and predicting prognosis.

Type/Class	Defining Feature	Prognosis/Outcome	Reference
Catterall (I–IV)	Extent of femoral head involvement	I–II: Good, III–IV: Poor	4
Herring (A–C, B/C)	Lateral pillar height (A: >50%, C: <50%, B/C: 50%)	A: Best, C: Worst, B/C: Intermediate	5, 7, 14
Stulberg (I–V)	Femoral head sphericity after healing	I–II: Good, III–V: Poor	5, 7, 14
Bilateral/Unilateral	One or both hips affected	Bilateral: Often better outcome	7

Table 2: Disease Types and Classifications

Classification Systems in Detail

Understanding the "type" of LCPD a child has is crucial for both prognosis and treatment planning.

Catterall Classification

• Divides LCPD into four groups based on the extent of femoral head involvement seen on X-ray 4.
  • Groups I & II: Less involvement, generally a good prognosis.
  • Groups III & IV: Extensive involvement, higher risk for poor long-term outcome.

Herring (Lateral Pillar) Classification

• Focuses on the integrity of the lateral pillar of the femoral head during the fragmentation stage 5, 7, 14.
  • Group A: Minimal loss of height—best outcomes.
  • Group B: <50% loss—intermediate outcome.
  • Group B/C: Exactly 50% loss—prognosis varies.
  • Group C: >50% loss—poorest outcomes.

Stulberg Classification

• Assesses femoral head shape and congruence at skeletal maturity 5, 7, 14.
  • Class I/II: Spherical head, good function.
  • Class III: Ovoid head, fair outcome.
  • Class IV/V: Flat or irregular head, poor outcome with higher risk for arthritis.

Bilateral vs. Unilateral Disease

• While most cases are unilateral, up to 10–15% can be bilateral 3, 7.
• Interestingly, children with simultaneous bilateral disease may have a better prognosis than those with severe unilateral involvement 7.

Disease Stages

• Avascular Necrosis: Initial insult with bone death 4, 13.
• Fragmentation: Bone is resorbed and replaced 4, 13.
• Re-ossification/Healing: New bone forms and the femoral head regains strength 4, 13.
• Remodeling: Final shape and congruence are established 4, 13.

Types and Prognosis

• Severity, age at onset, and bilateral involvement are key determinants of long-term joint health 4, 5, 7, 14.

Causes of Legg Calve Perthes Disease

Despite over a century of study, the precise cause of LCPD remains elusive. Modern research points to a multifactorial origin, involving a combination of biological, mechanical, and environmental factors.

Factor	Description	Evidence Summary	Reference
Vascular Insult	Blood supply interruption to femoral head	Central event in pathogenesis	1, 4, 13, 17
Thrombophilia	Tendency for abnormal blood clotting	Factor V Leiden, anticardiolipin antibodies associated	2, 9
Genetics	Family/twin studies	No major role, but predisposition possible	10, 11, 12
Environmental	Socioeconomic, obesity, latitude, trauma	May contribute, not sole cause	4, 8, 10, 11
Mechanical	Repetitive microtrauma, ischemia	Supports avascular necrosis hypothesis	11, 12

Table 3: Proposed Causes and Risk Factors

Multifactorial Etiology

Vascular and Mechanical Theories

• The central event in LCPD is an interruption of blood flow to the capital femoral epiphysis, resulting in bone and cartilage death (osteonecrosis and chondronecrosis) 1, 4, 13, 17.
• Mechanical factors like repetitive trauma or anatomical predispositions (e.g., acetabular retroversion) may increase susceptibility 4, 8, 11, 12.

Thrombophilia and Coagulation Abnormalities

• Recent research has shown higher rates of thrombophilic markers (such as Factor V Leiden mutation and anticardiolipin antibodies) in LCPD patients compared to controls, suggesting a role for abnormal blood clotting 2, 9.
• Low antithrombin levels and elevated Factor VIII have also been observed in some studies 2.

Genetic and Environmental Influences

• Genetic factors do not appear to be a dominant cause, as twin studies and family studies do not show strong hereditary patterns 10, 11.
• However, a congenital or acquired predisposition cannot be fully excluded 11, 12.
• Environmental factors—such as socioeconomic status, obesity, and even geographic latitude—may play contributing roles, but are not sufficient causes on their own 4, 8, 10, 11.

Summary

• The cause of LCPD is best described as multifactorial, with both intrinsic (biological, mechanical) and extrinsic (environmental, possibly genetic) factors interacting to predispose certain children to this avascular necrosis event 10, 11, 12.

Treatment of Legg Calve Perthes Disease

Treatment for LCPD is complex, individualized, and often controversial. The main goals are to relieve pain, maintain hip mobility, prevent femoral head deformity, and optimize long-term joint function.

Treatment	Target/Indication	Typical Use/Outcome	Reference
Observation	Mild cases, young children	Good prognosis, minimal intervention	7, 14, 15
Physical Therapy	Pain relief, range of motion	Early and ongoing in all severities	3, 14
Bracing/Casting	Containment of femoral head	Used selectively, variable benefit	6, 8, 14
Surgery	Severe cases, older children, B/C–C hips	Osteotomy, arthrodiastasis, etc.	1, 4, 6, 14, 16, 17
Pain Management	Symptom control	Short-term, adjunctive	1, 3

Table 4: Main Treatment Approaches

Principles and Approaches

Non-Operative Management

• Observation and Activity Modification: Many young children with mild disease (especially under age 6) do well with minimal intervention 7, 14, 15.
• Physical Therapy: Central to all treatment plans; aims to preserve hip motion, prevent muscle atrophy, and reduce pain 3, 14.
• Bracing and Casting: Devices like the Petrie cast or Scottish Rite orthosis are designed to "contain" the femoral head within the socket during healing, but evidence for their long-term benefit is mixed 6, 8, 14.
  • Bracing is not universally recommended and is often reserved for selected cases 8, 14.

Surgical Management

• When is surgery indicated?
  • Older children (>8 years at onset)
  • Severe lateral pillar involvement (B/C border or C)
  • Poor response to non-operative care 6, 14, 17
• Surgical options:
  • Femoral or pelvic osteotomy: Reorients the hip to better contain the femoral head 1, 4, 6, 14
  • Arthrodiastasis: Use of external fixators to offload the femoral head and promote healing, especially in late-onset cases 16
  • Reconstructive procedures: In skeletally mature patients with residual deformity 6

Outcome and Prognosis

• Age and severity at onset are the most important prognostic factors 5, 14. Children under 8 years with milder disease have a favorable outlook regardless of treatment type 7, 14.
• Surgical treatment can improve outcomes in children over 8 years with moderate (B/B–C) disease, but not in those with the most severe involvement (C) 14.
• Global variations exist in treatment preferences, but final outcomes are similar across regions 15.

Emerging and Adjunct Therapies

• Pain management and short-term medication use for symptom control may be needed 1, 3.
• Ongoing research is focused on biological and mechanical interventions that may better target the disease process 12, 17.

Conclusion

Legg-Calve-Perthes Disease is a complex pediatric condition with a variable course and outcome. Early recognition of symptoms and understanding of disease types are essential for prognosis and treatment. While the exact cause remains unclear, research points to a multifactorial etiology involving vascular, mechanical, and possibly coagulation abnormalities. Treatment is tailored to the individual, with age and disease severity guiding decisions between observation, physical therapy, bracing, and surgery.

Key Takeaways:

• Symptoms include gradual hip/knee pain, limping, restricted motion, and sometimes muscle atrophy.
• Types of LCPD are classified by severity and radiographic findings (Catterall, Herring, Stulberg), influencing prognosis.
• Causes are multifactorial—vascular insult, mechanical factors, and possible clotting disorders play a role.
• Treatment depends on age and severity: young children with mild disease often do well with conservative management, while older children or those with severe involvement may benefit from surgery.

By understanding the nuances of LCPD, caregivers and clinicians can work together to ensure the best possible outcomes for affected children.