Overview Legg-Calv-Perthes (LCPD) disease is a childhood hip disorder that results in infarction of the bony epiphysis of the femoral

head. LCPD represents idiopathic avascular necrosis of the femoral head. The disease is bilateral in 10-20% of patients and usually affects children aged 4-8 years. When both hips are involved, they are usually affected successively, not simultaneously. A family history is present in 6% of patients. In adults, the corresponding condition is termed Chandler disease. Although the etiology is unclear, certain risk factors have been identified in children, including sex, socioeconomic group, and the presence of an inguinal hernia and genitourinary tract anomalies. More specifically, boys are affected 3 to 5 times more often than girls, and the incidence increases in low socioeconomic groups and in children with low birth weight. Determining the prognosis is important at the time of presentation, because more than 50% of patients with LCPD do not require treatment. Staging Several staging schema are used to determine severity of disease and prognosis; these include the Catterall, Salter-Thomson, and Herring systems.[4] The Catterall classification is based on radiographic appearances and specifies 4 groups during the period of greatest bone loss. Catterall staging is as follows:

Stage I Histologic and clinical diagnosis without radiographic findings Stage II Sclerosis with or without cystic changes with preservation of the contour and surface of femoral head Stage III Loss of structural integrity of the femoral head Stage IV Loss of structural integrity of the acetabulum in addition

The Salter-Thomson classification simplifies the Catterall classifications by reducing the groups to 2. The first, called group A, includes Catterall groups I and II; for patients in this group, less than 50% of the head is involved. The second, called group B, includes Catterall groups III and IV; for patients in this group, more than 50% of the head is involved. For both classifications, if less than 50% of the ball is involved, the prognosis is better, whereas if more than 50% is involved, the prognosis is potentially poor. The Herring classification addresses the integrity of the lateral pillar of the head. In lateral pillar group A, there is no loss of height in the lateral one third of the head, and there is little density change. In lateral pillar group B, there is a lucency and less than 50% loss of lateral height. Sometimes, the head is beginning to extrude from the socket. In lateral pillar group C, there is a more than 50% loss of lateral height.

Preferred examination Plain radiography remains the major modality for the evaluation of LCPD. Staging of the disease is based on plain radiographic findings.[1, 5, 6] Scintigraphy is a useful technique in early disease when plain radiographic findings may be normal; with scintigraphy, abnormalities become apparent earlier in the course of disease than they do with plain radiography. Computed tomography (CT) scans allow early diagnosis of bone collapse and curvilinear zones of sclerosis early in the disease process when plain radiography is less sensitive. CT scans can also demonstrate subtle changes in the bone trabecular pattern. Ultrasonography is useful in the preliminary diagnosis of transient synovitis of the hip and the onset of LCPD.[7] Hip effusion with capsular distension is well depicted on sonographic images.[8, 9, 10, 11, 12] Magnetic resonance imaging (MRI) is as sensitive as isotopic bone scanning and allows more precise localization of involvement than conventional radiography.[13, 14, 15, 16, 17, 18] Limitations of techniques Plain radiographic findings may be entirely normal in early symptomatic disease. Although abnormalities become apparent with scintigraphy earlier in the course of disease than they do with radiography, abnormal scintigraphic findings are nonspecific; findings may be positive in patients with trauma, synovitis, and infections. The use of CT scanning is limited by the comparatively higher radiation dose. Ultrasonographic diagnosis of LCPD is based on a demonstration of hip effusion, which is a nonspecific finding. On MRI scans, the changes seen as bone marrow edema and joint effusions are nonspecific. Angiography, venography, and arthrography are invasive procedures and do not provide significantly better clinical information for guiding therapeutic options. Early radiographic signs of LCPD include the following:

Small femoral epiphysis (96%) Sclerosis of the femoral head with sequestration and collapse (82%) Slight widening of the joint space caused by thickening of the cartilage, failure of epiphyseal growth, the presence of joint fluid, or joint laxity (60%) (see the images below)

Legg-Calv-Perthes disease. Stage II disease. Note the slight widening of the left hip joint, representing a small joint effusion. Joint widening can also be secondary to hypertrophy of the cartilage.

Legg-Calv-Perthes disease. The left joint effusion is apparent. The femoral head is smaller on the left than the right. The femoral head is also

considerably denser on the left side. Joint widening can also be secondary to hypertrophy of the cartilage.

Legg-Calv-Perthes disease. Stage II disease. Note the slight widening of the left hip joint representing a small joint effusion. Joint widening can also be secondary to hypertrophy of the cartilage.

Legg-Calv-Perthes disease. The left joint effusion is apparent. The femoral head is smaller on the left than on the right. The femoral head is also considerably denser on the left side. Joint widening can also be secondary to hypertrophy of the cartilage.

An absence of destruction of the articular cortex, as occurs in bacterial arthritis (destruction of articular cartilage never occurs in LCPD) Late signs of LCPD on radiographs include the following:

Delayed osseous maturation of a mild degree, a radiolucent crescent line representing a subchondral fracture

Femoral head fragmentation and femoral neck cysts from intramedullary hemorrhage or extension of physeal cartilage into metaphysis, loose bodies, and coxa plana (see the images below)

resence of femoral neck cysts. The femoral head is obviously smaller on the left than on the right.

Legg-Calv-Perthes disease. Image shows flattening and early fragmentation of the left femoral head with the presence of femoral neck cysts. The femoral head is obviously smaller on the left than on the right. Legg-Calv-Perthes disease. Image shows flattening and early fragmentation of the left femoral head with the presence of femoral neck cysts. The femoral head is obviously smaller on the left than on the right.

Legg-Calv-Perthes disease. Image shows flattening and early fragmentation of the left femoral head with the

Coxa magna, or remodeling of the femoral head, which becomes wider and flatter, similar in appearance to a mushroom (see the image below)

Legg-Calv-Perthes disease. Plain radiograph in an adult patient with residual coxa magna and plana deformity with superimposed joint changes.

Plain radiographs have a sensitivity of 97% and a specificity of 78% in the detection of LCPD. Severe osteoarthritis and infective arthritis may mimic the disease.