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Legg-Calve-Perthes disease - Adventist HealthCare adventisthealthcare.com | Legg-Calve'-Perthes Disease peds-ortho.com | Your Orthopaedic Connection: Perthes Disease orthoinfo.aaos.org | Perthes' Disease return2fitness.net |
Legg–Calvé–Perthes syndrome is a degenerative disease of the hip joint, where growth/loss of bone mass leads to some degree of collapse of the hip joint and to deformity of the ball of the femur and the surface of the hip socket. The disease is characterized by idiopathic avascular osteonecrosis of the capital femoral epiphysis of the femoral head leading to an interruption of the blood supply of the head of the femur close to the hip joint. The disease is typically found in young children, and it can lead to osteoarthritis in adults. The effects of the disease can sometimes continue into adulthood. It is also known more simply as Perthes disease[1], ischemic necrosis of the hip, coxa plana, osteochondritis and avascular necrosis of the femoral head, Legg–Perthes Disease or Legg–Calve-Perthes Disease (LCPD). It is named for Arthur Legg, Jacques Calvé and Georg Perthes.[1][2][3][4]
[edit] CauseAlthough no-one has identified the cause of Perthes disease it is known that there is a reduction in blood flow to the joint. It is thought that the artery of the ligamentum teres femoris closes too early, not allowing time for the medial circumflex femoral artery to take over.[citation needed] For example, a child may be 6 years old chronologically but may have grown to 4 years old in terms of bone maturity. The child may then engage in activity appropriate for a child of 6 but may not yet have the bone strength of an older child, leading to flattening or fracture of the hip joint. Genetics do not appear to be a determining factor, but it has been suggested that a deficiency of some blood factors used to disperse blood clots may lead to blockages in the vessels supplying the joint, but these have not been proven. [edit] Signs and symptomsCommon 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 2 years of age. 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 than in the front, not unlike a localized charley horse. This lasts for an hour or so and returns nightly on inactivity. [edit] DiagnosisX-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. [edit] TreatmentThe goal of treatment is to avoid severe degenerative arthritis. Orthopedic assessment is crucial. Younger children have a better prognosis than older children. Treatment has traditionally centered on removing pressure from the joint until the disease has run its course. Options include traction (to separate the femur from the pelvis and reduce wear) braces (often for several months, with an average of 18 months) to restore range of motion, physiotherapy, and surgical intervention when necessary because of permanent joint damage. To maintain activities of daily living, custom orthotics may be used. These devices internally rotate the femoral head and abduct the leg(s) at 45 degrees. Orthoses can start as proximal as the lumbar spine (LSO), and extend the length of the limbs to the floor. Most functional bracing is achieved using a waist belt and thigh cuffs derived from the Scottish-Rite Orthosis [5]. These devices are typically prescribed by a physician and implemented by a certified orthotist. For older children, the distraction method has been found to be a successful treatment, using an external fixator which relieves the hip from carrying the body's weight. This allows room for the top of the femur to regrow. Many children need no intervention at all and are simply asked to refrain from contact sports or games which impact the hip. The Perthes Association has a "library" of equipment which can be borrowed to assist with keeping life as normal as possible, newsletters, a helpline, and events for the families to help children and parents to feel less isolated. Modern treatment focuses on removing pressure from the joint to increase blood flow, in concert with physiotherapy. Pressure is minimized on the hip through use of crutches or a cane, and the avoidance of running-based sports. Swimming is highly recommended, as it allows exercise of the hip muscles with full range of motion while reducing the stress to a minimum. Cycling is another good option as it also keeps stress to a minimum. Physiotherapy generally involves a series of daily exercises, with weekly meetings with a physiotherapist to monitor progress. These exercises focus on improving and maintaining a full range of motion of the femur within the hip socket. Performing these exercises during the healing process is essential to ensure that the femur and hip socket have a perfectly smooth interface. This will minimize the long term effects of the disease. Use of zoledronic acid has also been investigated.[6] Perthes disease is self limiting, but if the head of femur is left deformed there can be a long-term problem. Treatment is aimed at minimizing damage while the disease runs its course, not at 'curing' the disease. It is recommended not to use steroids or alcohol as these reduce oxygen in the blood which is needed in the joint. As sufferers age, problems in the knee and back can arise secondary to abnormal posture and stride adopted to protect the affected joint. The condition is also linked to arthritis of the hip, though this appears not to be an inevitable consequence. Hip replacements are relatively common as the already damaged hip suffers routine wear; this varies by individual, but generally is required any time after age 50. [edit] EffectsPerthes disease can lead to pain in the affected area later in life and can also lead to bone spurs in the region. Other than surgery; NSAIDs such as Motrin help with the pain inflicted. [edit] IncidencePerthes is rare, occurring in approximately 5.5 of 100,000 children. Caucasians are affected more frequently than other races. Males are affected 3-4 times more often than females, suggesting a partial sex-linked genetic inheritance of the syndrome.[citation needed] Children of sufferers of the disease themselves have a very slightly increased risk; 1 in 100 male children of adults with Legg–Calvé–Perthes syndrome also exhibit the syndrome. It is most commonly seen in persons aged 3–12 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.[citation needed] It is also found in Latin Americans, Asians and Inuit Indians. [edit] PrognosisChildren younger than 6 have the best prognosis since they have time for the dead bone to revascularize and remodel. Children that have been diagnosed with Perthes' Disease after the age of 10 are at a very high risk of developing osteoarthritis and Coxa Magna. [edit] Legg–Calvé–Perthes disease in dogsThis is also known as aseptic necrosis of the femoral head. Toy and small breeds, particularly Toy Poodles,Yorkshire Terriers, Pugs, Jack Russell Terriers, and Dachshunds can be affected. Hip pain is usually seen by the age of 6 to 8 months.[7] The disease is bilateral in 12 to 16 percent of cases.[8] X-rays are necessary to make the diagnosis and show increased opacity and focal lysis in the head of the femur, and later in the disease, collapse and fracture of the neck of the femur. The recommended treatment is surgical removal of the head of the femur, but conservative treatment (rest, exercise restriction, and pain medication) may be effective in a limited number of cases (less than 25 percent, according to some studies).[8] The prognosis is excellent with surgery. [edit] References
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