Legg-Calve-Perthes disease

Legg-Calve-Perthes disease

OVERVIEW:

Idiopathic necrosis of capital femoral epiphysis of the femoral head. 10-20% of cases are bilateral.

System(s) affected: Musculoskeletal
Genetics: No known genetic pattern identified
Incidence/Prevalence in USA: Incidence 15/100,000; prevalence 75/100,000
Predominant age: Susceptible age 2-12 years. However, approximately 80% occur between the ages of 4 and 9 years
Predominant sex: Males > Females (4:1). In bilateral cases males predominate 7:1. However, females seem to have more severe involvement.

SIGNS AND SYMPTOMS:

• Primarily hip or groin pain although with referred pain to the knee and thigh not uncommon
• Range of motion limited, especially in internal rotation and abduction
• Atrophy of thigh musculature due to disuse
• Leg length discrepancy secondary to collapse of the femoral head

CAUSES:

• Etiology unclear
• Felt to be related to interruption of blood flow to femoral epiphysis
• Role of biochemical factors remains to be established

RISK FACTORS:

• No genetic risk factors
• Increased incidence in children with low birth weight and delayed physical maturation

DIAGNOSIS

DIFFERENTIAL DIAGNOSIS:

• Unilateral - septic arthritis, toxic synovitis, juvenile rheumatoid arthritis
• Bilateral - spondyloepiphyseal dysplasia, metaphyseal dysplasia

LABORATORY:

• CBC
• Sedimentation rate (elevated in infection)

Drugs that may alter lab results: N/A
Disorders that may alter lab results: N/A

PATHOLOGICAL FINDINGS:

• Early (necrosis, resorption) stage - necrosis of bone with subchondral bone fracture and subsequent collapse of subchondral bone
• Late (healing) stage - revascularization by creeping substitution of necrotic bone

SPECIAL TESTS:

IMAGING:

• Serial radiographs, AP and frog lateral, of the pelvis are crucial for determining of extent of involvement and progression of healing.
• Full extent of involvement may not be evident for several months as radiographic findings lag symptoms
• Technetium 99 bone scan - helpful in delineating the extent of avascular changes
• MRI - Most sensitive test; facilitates early diagnosis of necrosis and visualization of articular surface
• Dynamic arthrography - used to assess sphericity of femoral head

DIAGNOSTIC PROCEDURES:

Hip aspiration to rule out septic arthritis

TREATMENT

APPROPRIATE HEALTH CARE:

• When necessary, a pediatric orthopaedic consultation
• Ambulatory treatment is usual, however, some patients may require inpatient traction or surgical procedures

GENERAL MEASURES:

• Goals of treatment:
• Relieve weight bearing across affected hip, thus reducing irritability of the hip
• Obtain and maintain hip range of motion
• Maximize regeneration and spherical development of the femoral head by containing the femoral epiphysis within the acetabulum

SURGICAL MEASURES:

• Adductor tenotomy to help restore range of motion secondary to adductor contracture
• Femoral and/or pelvic osteotomy to help contain femoral epiphysis within the confines of the acetabulum

ACTIVITY:

• Ambulatory status depends on extent/stage of disease
• Limit weight bearing in cases of hip irritation

DIET:

No special diet

PATIENT EDUCATION:

• Legg-Calve-Perthes disease is a self-limited disease with revascularization occurring within 3 years
• Treatment is directed at maintaining an appropriate range of motion and maximizing the containment of the femoral head

MEDICATIONS

DRUG(S) OF CHOICE:

• Ibuprofen 10 mg/kg tid-qid

Contraindications: Allergy to ibuprofen
Precautions: GI irritation
Significant possible interactions: N/A

ALTERNATIVE DRUGS:

N/A

FOLLOW UP

PATIENT MONITORING:

• Initially, close followup, with radiographic evaluation, is needed to determine extent of necrosis
• Once healing phase entered, followup can be every 6 months
• Long-term followup necessary to determine final outcome

PREVENTION/AVOIDANCE:

Since etiology is not clearly understood, prevention is not possible

POSSIBLE COMPLICATIONS:

• Permanent distortion of the femoral head
• Distorted joint susceptible to early degenerative joint disease

EXPECTED COURSE AND PROGNOSIS:

• Most patients have a favorable outcome
• Outcome is dependent on the patient's age at the time of the diagnosis (the younger the better)
• Prognosis is also related to the degree of involvement of the femoral head (as determined by radiography)

MISCELLANEOUS

ASSOCIATED CONDITIONS:

N/A

AGE-RELATED FACTORS:

Pediatric:
• Physical maturation is delayed
• The younger the patient at the time of diagnosis, the greater the chance for remodeling
Geriatric: N/A
Others: N/A

PREGNANCY:

N/A

SYNONYMS:

N/A

ICD-9-CM:

732.1 Juvenile osteochondrosis of hip and pelvis

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Medical Diagnosis » L » Legg-Calve-Perthes disease