Definition: Legg-Calv-Perthes Disease (Coxa Plana) is an idiopathic form of avascular necrosis of the femoral head occurring in children.

This disorder is usually self-limited whereby the femoral head undergoes aseptic necrosis and replacement resulting in some flattering of the femoral head (coxa plana). A variable amount of permanent deformity and restricted motion usually result. Etiology The etiology of Legg-Calv-Perthes disease remains unclear; however, the following scenario generally is accepted: The blood supply to the capital femoral epiphysis is interrupted. Bone infarction occurs, especially in the subchondral cortical bone, while articular cartilage continues to grow. (Articular cartilage grows because its nutrients come from the synovial fluid.) Revascularization occurs, and new bone ossification starts. At this point, a percentage of patients develop LCPD, while other patients have normal bone growth and development. LCPD is present when a subchondral fracture occurs. This is usually the result of normal physical activity, not direct trauma to the area Changes to the epiphyseal growth plate occur secondary to the subchondral fracture.

Epidemiology Perthes is rare, occurring in approximately 4 of 100,000 children. Caucasians are affected more frequently than other races. Males are affected 4-5 times more often than females, suggesting a partial sex-linked genetic inheritance of the syndrome. Children of sufferers of the disease are themselves at an increased risk; 1 in 100 male children of adults with LeggCalvPerthes syndrome also exhibit the syndrome. It is most commonly seen in persons aged 312 years, with a median of 6 years of age.[citation needed] In the US, 1 in 1200 children younger than 15 years will have this disease, while the incidence is higher in the UK, with Ireland having the highest percentage. It is also found in Latin Americans, Asians and Inuit Indians. Anatomy and Physiology The femoral head is spherical and is covered by articular cartilage. The fovea is a justoff-center ovoid depression that serves as an attachment for the ligamentum teres (round ligament). The femoral head is supplied by a vascular ring at the base of the femoral neck from branches arising from the lateral and medial circumflex arteries. The superior and inferior gluteals contribute to the blood supply to a lesser extent. The anterior aspect of the vascular ring is within the joint capsule. Principally, the lateral epiphyseal arteries supply the femoral neck. The artery in the ligamentum teres supplies one third of the arterial supply to the femoral head in children but makes only a small contribution in adults. Pathophysiology The primary pathologic abnormality in patients with LPD is osteonecrosis resulting in flattening and collapse of the femoral head. The articular cartilage covering the femoral

head is remarkably resistant to ischemia and is usually well preserved. The basic underlying cause of LPD is insufficient blood supply to the femoral head. The epiphyseal plate acts as a barrier to the supply of blood in children aged 4-10 years, and the ligamentum teres vessels become nonfunctional, causing the head to be at risk for osteonecrosis. Healing occurs by revascularization of the necrotic femoral head. The course of revascularization and reconstitution of the femoral head varies. The ultimate shape of the reconstituted femoral head depends on several factors, including the degree and site of necrosis and the magnitude of forces working across the femoral head. In some patients, the femoral head may return to normal, whereas in other patients, coxa plana and shortening and widening of the femoral head may develop. These changes may be associated with osteoarthrosis and intra-articular loose bodies. In most patients, the changes occurring in LPD affect only 1 hip. With bilateral disease, the changes are rarely symmetric. Manifestations Common symptoms include hip, knee, or groin pain, exacerbated by hip/leg movement. The pain feels like a tooth ache, possibly severe. There is a reduced range of motion at the hip joint and a painful or antalgic gait. There may be atrophy of thigh muscles from disuse and an inequality of leg length. In some cases, some activity can cause severe irritation or inflammation of the damaged area including standing, walking, running, kneeling, or stooping repeatedly for an extended period of time. The first signs are complaints of soreness from the child, which are often dismissed as growing pains, and limping or other guarding of the joint, particularly when tired. The pain is usually in the hip, but can also be felt in the knee ('referred pain'). In some cases, pain is felt in the unaffected hip and leg[citation needed], due to the child favoring the injured side and placing the majority of their weight on the "good" leg. It is predominantly a disease of boys (4:1 ratio). Whereas Perthes is generally diagnosed between 5 and 12 years of age, it has been diagnosed as early as infancy. Typically the disease is only seen in one hip, but bilateral Perthes is seen in about 8-10% of children diagnosed. Onset of pain may be up to 4 hours after inactivity. Knee pain is felt in the back of the knee rather then in the front, not unlike a localized charley horse. This lasts for an hour or so and returns nightly on inactivity. Diagnostic Test X-Rays of the hip joint are absolutely necessary. X-rays will show a small, flattened and fragmented head of femur. A bone scan may be useful in helping determine the extent of the avascular changes. A hip aspiration may be performed if there is suspicion of a septic arthritis. The diagnostic finding will show patchy areas of avascular necrosis and eventually fragmentation and flattenning in the femoral head. Diagnosis is made predominantly by X-ray study, together with physical examination (MRIs have also been found useful for judging the extent of the deformity). Sufferers typically have limited range of motion in their hip, particularly when rotating the joint.

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