Legg-Calve-Perthes Disease Background

Legg-Calve-Perthes disease (LCPD) is avascular necrosis of the proximal femoral head resulting from compromise of the tenuous blood supply to this area. LCPD usually occurs in children aged 4-10 years. The disease has an insidious onset and may occur after an injury to the hip. In the vast majority of instances, the disorder is unilateral. Both hips are involved in less than 10% of cases, and the joints are involved successively, not simultaneously.

Image in an 8-year-old boy with Herring type C Legg-CalvPerthes disease shows evidence of lateral extrusion. Symptoms began 18 months before this view

was obtained. Image courtesy of Dennis P. Grogan, MD. Anteroposterior (AP) radiograph in a 9-year-old boy with Legg-Calv-Perthes disease. Image

courtesy of Dennis P. Grogan, MD. Frog-leg lateral radiograph of same 9-year-old boy with Legg-Calv-Perthes disease as in Image above. Image courtesy of Dennis P. Grogan, MD.

Epidemiology
Frequency

Legg-Calv-Perthes disease usually occurs in children aged 4-10 years, with a mean age of 7 years. It occurs more commonly in boys than in girls, with a male-to-female ratio of 4:1. The condition is rare, occurring in approximately 4 of 100,000 children.

Etiology
The cause is not known, but children with Legg-Calv-Perthes disease (LCPD) have delayed bone age, disproportionate growth, and a mildly shortened stature. LCPD may be idiopathic, or it may result from a slipped capital femoral epiphysis, trauma, steroid use, sickle-cell crisis, toxic synovitis, or congenital dislocation of the hip.

Pathophysiology
Rapid growth occurs in relation to development of the blood supply of the secondary ossification centers in the epiphyses, creating an interruption of adequate blood flow and making these areas prone to avascular necrosis. Interruption of the blood supply to the bone results in necrosis, removal of the necrotic tissue, and its replacement with new bone. Bone replacement may be so complete and perfect that completely normal bone may result. The adequacy of bone replacement depends on the age of the patient, the presence of associated infection, congruity of the involved joint, and other mechanical and physiologic factors. Necrosis may occur after trauma or infection, but idiopathic lesions can develop during periods of rapid growth of the epiphyses.

Presentation
The earliest sign of Legg-Calv-Perthes disease (LCPD) is an intermittent limp (abductor lurch), especially after exertion, with mild or intermittent pain in the anterior part of the thigh. LCPD is the most common cause of a limp in the 4- to 10-year-old age group, and the classic presentation has been described as a painless limp. The patient may present with limited range of motion of the affected extremity. The most common symptom is persistent pain. Hip pain may develop and is a result of necrosis of the involved bone. This pain may be referred to the medial aspect of the ipsilateral knee or to the lateral thigh. The quadriceps muscles and adjacent thigh soft tissues may atrophy, and the hip may develop adduction flexion contracture. The patient may have an antalgic gait with limited hip motion. Pain may be present with passive range of motion and limited hip movement, especially internal rotation and abduction. Children with LCPD can have a Trendelenburg gait resulting from pain in the gluteus medius muscle. Laboratory studies and radiography may supplement medical history taking and physical examination in the assessment of a child with a limp.[1]

Differential diagnoses include osteomyelitis, pyogenic arthritis, transient synovitis, abscess of the psoas muscle, juvenile rheumatoid arthritis, hemophilia, slipped capital femoral epiphysis, and neoplasm.

Laboratory Studies
Determination of the CBC with a differential and the erythrocyte sedimentation rate (ESR) are recommended, but the findings may be normal.

Imaging Studies
Obtain hip radiographs, including anteroposterior and frog-leg lateral views of the pelvis to establish the diagnosis.[1, 2] Initial radiographs can be normal, but radiographic changes can be divided into 5 distinct stages representing a continuum of the disease process.

Stage 1 reveals cessation of femoral epiphyseal growth. Stage 2 is a subchondral fracture. Stage 3 shows resorption. Stage 4 demonstrates reossification.

Stage 5 is the healed or residual stage. Image in an 8-year-old boy with Herring type C Legg-Calv-Perthes disease shows evidence of lateral extrusion. Symptoms began 18 months before this view was obtained. Image courtesy of Dennis P. Grogan,

MD. Anteroposterior (AP) radiograph in a 9-year-old boy with Legg-Calv-Perthes disease. Image courtesy of Dennis P. Grogan, MD.

Frog-leg lateral radiograph of same 9-year-old boy with LeggCalv-Perthes disease as in Image above. Image courtesy of Dennis P. Grogan, MD.

Early radiographic changes may reveal only a nonspecific effusion of the joint associated with slight widening of the joint space, metaphyseal demineralization (decreased bone density around the joint), and periarticular swelling (bulging capsule). This is the acute phase, and it may last 12 weeks. Decreasing bone density in and around the joint is noted after a few weeks. With advancement of the disease, the joint space between the ossified head and acetabulum widens as the necrotic ossification center appears denser than the surrounding structures. Narrowing or collapse of the femoral head causes it to appear widened and flattened (coxa plana). A varus deformity of the femoral neck may occur as a result of damage to the femoral head growth center and overgrowth of the greater trochanteric apophysis. Eventually, the disease may progress to collapse of the femoral head, increase in the width of the neck, and demineralization of the femoral head. The final shape of this area depends on the extent of necrosis and the degree of collapse. All of the findings are correlated with disease progression and the extent of necrosis. This is the active phase, and it can last 12-40 months. A bone scan can be used to evaluate the site for avascular necrosis.

Medical Therapy
Consultation with an orthopedist is recommended. Treatment goals include eliminating hip irritability, restoring and maintaining good range of motion in the hip, preventing femoral epiphyseal collapse, and attaining a spherical femoral head when the hip heals. Initial therapy includes minimal weight bearing and protection of the joint, which is accomplished by maintaining the femur abducted and internally rotated so that the femoral head is held well inside the rounded portion of the acetabulum. Abduction and rotation of the femur is accomplished either by the use of orthotic devices (bracing) or surgery (osteotomy). The Scottish Rite brace achieves containment by abduction while allowing free knee motion.

Surgical Therapy
Results of surgical containment appear to be better than those of nonsurgical containment (orthosis). Surgical approaches include either femoral osteotomy to redirect the involved portion

within the acetabulum or innominate osteotomy. Both procedures produce equal results, but femoral osteotomy may cause shortening of the limb, leading to a chronic limp.[3, 4, 5, 6, 7, 8, 9] Surgery does not speed healing of the femoral head, but it does cause the head to reossify in a more spherical fashion. In a Norwegian study of Perthes' disease (Legg-Calve-Perthes' disease), Wiig et al followed 358 patients for 5 years and determined that proximal femoral varus osteotomy provided the best results in children 6 years and older with hips having more than 50% femoral head necrosis at the time of diagnosis. They found no significant difference between physiotherapy and abduction orthosis and therefore suggested abandoning abduction orthosis for Perthes' disease. For children younger than 6 years, no difference in outcome was determined for any of the 3 treatments.[3] One study by Kim et al suggest that, contrary to conventional belief, a greater varus angulation may not produce better preservation of the femoral head following proximal femoral varus osteotomy.[10]

Follow-up
For excellent patient education resources, visit eMedicineHealth's First Aid and Injuries Center. Also, see eMedicineHealth's patient education article Repetitive Motion Injuries.

Complications
Because Legg-Calv-Perthes disease is a local self-healing disorder, treatment consists of protection of the joint by allowing new bone formation to occur and regain a spherical femoral head. The goal is to prevent (1) irregular contouring, flattening, or mushrooming of the head; (2) shortening and broadening of the neck; and (3) flattening of the vertical wall of the acetabulum. The development of any of these conditions can result in osteoarthritis at an early age.

Outcome and Prognosis

The prognosis for patients with Legg-Calv-Perthes disease can be good and depends on the completeness of involvement of the epiphyseal center. The severity of involvement of the femoral head, its subsequent healing, and proper joint space preservation all determine the degree and timing for an athlete to participate in sports. The functional result depends on the amount of deformity that develops when the structure is softened. Overall, the prognosis for recovery and sports participation after treatment is very good for most individuals. The patient's short-term prognosis is related to femoral head deformity at the completion of the healing stage. Risk factors include a clinical onset at an older age, extensive femoral epiphyseal involvement, femoral head containment, reduced range of motion in the hip, and premature closure of the growth plate. The long-term prognosis is related to the potential for osteoarthritis of the hip as an adult. In patients with metaphyseal defects, in those in whom the disease develops late in childhood (age 10 y or older), and in those who have more complex involvement of the femoral head with

residual deformity, the prognosis is worse, and degenerative arthritis occurs in nearly 100% of these patients. This rate is in comparison to those patients who are younger than 5 years when the problem develops. The incidence of degenerative arthritis is negligible in this younger population. The results of one small retrospective study found that total hip replacement can be considered a possible option for patients who had Legg-Calv-Perthes disease in childhood. Though a high rate of neurologic complications was noted, these were possibly related to inadequate soft-tissue release or excessive limb lengthening.[