A special thanks goes to Dr. Harry C. Karydes, who contributed to the previous edition.
CODES
ICD9
- 984.0 Toxic effect of inorganic lead compounds
- 984.1 Toxic effect of organic lead compounds
- 984.9 Toxic effect of unspecified lead compound
ICD10
- T56.0X1A Toxic effect of lead and its compounds, accidental, init
- T56.0X4A Toxic effect of lead and its compounds, undetermined, init
LEGG–CALVÉ–PERTHES DISEASE
Sarah V. Espinoza
BASICS
DESCRIPTION
- Idiopathic avascular necrosis of the femoral head in children
- Genetics:
- Increased frequency with factor V Leiden and anticardiolipin antibodies
Pediatric Considerations
Exclusively a pediatric disease
ETIOLOGY
- Successive vascular occlusions causing characteristic findings
- Growing evidence implicating hypercoagulable states
- May be multifactorial
- Risk factors include tobacco smoke, wood smoke, low birth weight, birth length <50 cm
- Progression through 4 stages of disease:
- Initial stage: Dense femoral head causing intermittent synovitis
- Fragmentation: Femoral head becomes soft and deforms causing loss of motion
- Healing: New bone grows into femoral head
- Residual: Healed femoral head with some deformity
- More common in boys: Male > female, 4:1
- More common among Caucasians
- Most commonly occurs between ages of 3 and 7:
- Bilateral in 10–15% of cases
- Associated with short stature, deprived populations, delayed & disproportionate growth
DIAGNOSIS
SIGNS AND SYMPTOMS
History
- Frequently insidious onset
- Limp often presenting complaint
- Pain:
- Aching in hip, groin, anteromedial thigh, or anteromedial knee
- May be mild
- Aggravated by activity, relieved by rest
- Muscle spasm common complaint early in course of disease
Physical-Exam
- Tenderness over anterior aspect of hip joint
- Joint stiffness:
- Limitation of internal rotation seen earliest
- Limited abduction
- Contractures of adductors
- Muscle atrophy and shortening of leg on affected side are late findings
- Otherwise well appearing and afebrile
- May be asymptomatic
ESSENTIAL WORKUP
- Radiographs of hip most important study for diagnosis in ED
- Consider and exclude septic arthritis (usually an acute febrile illness)
DIAGNOSIS TESTS & NTERPRETATION
Lab
- No specific lab studies diagnostic of Legg–Calvé–Perthes (LCP)
- CBC, C-reactive protein (CRP), or ESR, if septic arthritis a concern
Imaging
- Characteristic imaging findings combined with consistent history and physical exam establish diagnosis
- Plain radiographs, MRI, and nuclear scintigraphy (bone scan) are the main diagnostic modalities used
- Hip radiographs:
- AP & Frog lateral view of affected hip
- Image both hips to detect contralateral disease
- Assess stage, extent, and severity
- Can be normal during 1st 3–6 mo
- Usually abnormal at time of presentation
- 5 stages seen in sequence:
- Cessation of growth at the capital femoral epiphysis; smaller femoral head epiphysis and widening of articular space on affected side
- Subchondral fracture
- Resorption of bone
- Reossification of new bone
- Healed stage
- CT:
- Shows precise information on anatomic relationship between femoral head and acetabulum
- May have role in operative planning, staging
- MRI:
- Sensitive in the diagnosis of LCP and provides good anatomic images
- Detects abnormalities earlier than plain radiographs
- Used to assess the extent of femoral head infarction
- Variety of findings depending on imaging protocol used
- Bone scan:
- Precedes x-ray changes by an average of 3 mo
- Evaluates patterns associated with revascularization and recanalization
- US:
- Shows effusion in the hip but is not specific for LCP
- Evaluate for thickening of the synovial membrane
- Evaluate deformity and containment of femoral head
- Arthrography:
- Used to evaluate method of treatment
Diagnostic Procedures/Surgery
Arthrocentesis of hip definitive test to exclude septic arthritis if significant concern for this; may need orthopedic consultation
DIFFERENTIAL DIAGNOSIS
- Unilateral involvement:
- Transient (a.k.a. toxic) synovitis
- Septic arthritis
- Osteomyelitis
- Sickle cell anemia
- Juvenile rheumatoid arthritis
- Rheumatic fever
- Trauma:
- Femoral neck fracture
- Hip dislocation
- Slipped capital femoral epiphysis
- Tuberculosis
- Tumor
- Bilateral involvement:
- Hypothyroidism
- Epiphyseal dysplasia
- Gaucher disease
TREATMENT
PRE HOSPITAL
Clinical course is subacute; less likely to present via ambulance
INITIAL STABILIZATION/THERAPY
Not a life-threatening condition; clinical instability mandates identification of alternative diagnosis
ED TREATMENT/PROCEDURES
- Main ED intervention is pain control
- Restrict from vigorous activity
- May need crutches if weight bearing painful
MEDICATION
First Line
Ibuprofen: 10 mg/kg/dose PO q6–8h PRN pain
Second Line
Diazepam: 0.1–0.2 mg/kg/dose (max. 5 mg) PO q6–8h PRN muscle spasm
FOLLOW-UP
DISPOSITION
Admission Criteria
Need for admission rare, indicated for:
- Severe pain or muscle spasm not controlled by PO medications
- Social considerations; bedrest/care at home not possible
Discharge Criteria
- Adequate pain control with PO medications
- Orthopedic follow-up arranged in 1–2 wk
Issues for Referral
- Age of onset shown to affect outcome; onset younger than 6 with better outcome, if older than 8, shown to have poorer outcome
- For more severe disease, a range of treatment options exists, including conservative treatment, orthotics, traction, and surgical osteotomy, determined by consulting orthopedist
- Degenerative arthritis and possible need of a replacement is the main long-term complication
FOLLOW-UP RECOMMENDATIONS
Orthopedic consultation to determine further management; may be outpatient
PEARLS AND PITFALLS
Abrupt onset, presence of fever, unstable patient, or toxic appearance suggest diagnosis other than LCP.
ADDITIONAL READING
- Daniel AB, Shah H, Kamath A, et al. Environmental tobacco and wood smoke increase the risk of Legg-Calvé-Perthes disease.
Clin Orthop Relat Res
. 2012;470(9):2369–2375.
- Dimeglio A, Canavese F. Imaging in Legg-Calvé-Perthes Disease.
Orthop Clin North Am
. 2011;42:297–302.
- Kim HK, Herring JA. Pathophysiology, classifications, and natural history of Perthes disease.
Orthop Clin North Am
. 2011;42:285–295.
- Nelitz M, Lippacher S, Krauspe R, et al. Perthes disease: Current principles of diagnosis and treatment.
Dtsch Arztebl Int
. 2009;106(31–32):517–523.
- Perry DC, Hall AJ. The epidemiology and etiology of Perthes disease.
Orthop Clin North Am
. 2011;42:279–283.