About lcpd

What is lcpd?

Legg-Calvé-Perthes Disease (LCPD) is one of a group of disorders known as the Osteochondroses. The Osteochondroses typically are characterized by degeneration (avascular necrosis) and subsequent regeneration of the growing end of a bone (epiphyses). In Legg-Calvé-Perthes Disease, the growing end (epiphysis) of the upper portion (capital) of the thigh bone (femur) is affected. Researchers believe that an unexplained interruption of the blood supply (ischemia) to the capital femoral epiphysis results in degeneration (avascular necrosis) and deformity of the thigh bone in this area. Symptoms may include a limp with or without pain in the hip, knee, thigh, and/or groin; muscle spasms; delayed maturation of the femur (delayed bone age); mild short stature; and/or limited movements of the affected hip. The disease process seems to be self-limiting as new blood supplies are established (revascularization) and new healthy bone forms (reossifies) in the affected area. Most cases of Legg-Calvé-Perthes Disease occur randomly for no apparent reason (sporadically).

What are the symptoms for lcpd?

The age of onset, severity, duration, and associated complications of Legg-Calvé-Perthes disease vary greatly from one person to another. Generally, the onset is slow and the progression is gradual. The disorder usually affects children between the ages of eight and ten years, with some instances occurring as early as age two or as late as age 12. The disease typically affects one leg (unilateral); however, in approximately 10% of cases, the condition may subsequently develop in the other leg (bilateral).

In most children, the first symptom is a limp with or without pain. Affected children may experience Pain in the hip, knee, thigh, and/or groin. In some cases, the Pain may be limited (localized) to the knee or inner part of the thigh. In addition, affected children may also experience muscle spasms of the legs; loss of muscle mass (atrophy) of the front thigh muscles; limited or restricted movements of the hips; and/or Inflammation of the membrane lining the hip joint (synovitis). As affected children age, they may also exhibit slightly differing lengths of their two legs (leg length discrepancy).

In all cases, blood supply to the capital femoral Epiphysis is re-established and degenerative (necrotic) bone is resorbed and rehardens (re-ossifies). This occurs without therapeutic intervention (spontaneously). The regenerated bone may be only slightly abnormal, misshapen, or significantly misshapen (e.g., abnormally flattened or unusually enlarged). In almost all cases, some shortening of the leg occurs, and in more severe cases, the femoral head may be deformed. The length of time between onset of LCPD and new bone formation may be as short as 18 months or as long as four years.

When onset occurs during the ages of four to nine years, it is, depending on treatment, less likely that osteoArthritis will develop later in life than it is in children with onset after the age of 10. Affected individuals who present significant deformity during re-ossification of the capital femoral Epiphysis have an increased risk of developing osteoArthritis later in life.

What are the causes for lcpd?

The symptoms of Legg-Calvé-Perthes disease develop due to damage to the growing portion (epiphysis) of the upper end of the thigh bone. This damage results from interruption of the blood supply (ischemia) to this region. This damages the bone-forming cells (osteoblasts) and resident bone cells (osteocytes) and results in degeneration (necrosis) and softening of the bone in this area. The upper end of thigh bone becomes fragile as bone mass is lost. It is possible that this fragile area may “fracture” internally, causing deformity. A thin line of decreased density (Caffey sign) may be apparent on the epiphysis, which may represent such a “fracture” within the bone (subchondral). Damaged bone may fragment and cause irregularities when blood flow to the affected area eventually resumes (revascularization). As the bone regrows and rehardens (re-ossifies), it may deform, resulting in permanent malformation of the upper thigh bone (e.g., abnormally flattened or unusually enlarged epiphysis).

The exact, underlying cause of the interrupted blood flow is not fully understood. Multiple factors including environmental and genetic ones may play a role in the development of the disorder. Some risk factors or conditions that may play a role in the development of LCPD include low birth weight, delayed skeletal maturity, trauma, adverse social and economic conditions, exposure to tobacco smoke, or a positive family history of the disorder. Some researchers have theorized that disorders that disrupt or affect how the blood clots (coagulation disorders) such as thrombophilia play a role in some individuals. These disorders could lead to abnormal blood clot formation would cause the interrupted blood flow that characterizes LCPD.

No one theory has been proven as the underlying or contributing cause(s) of LCPD and it is possible that different individuals may have a different underlying causes. More research is necessary to determine the complex interactions that ultimately bring about the disorder.

In a handful of extremely rare familial instances, mutations in the COL2A1 gene have been linked to avascular necrosis of the head of the thigh bone in children.

What are the treatments for lcpd?

The treatment of Legg-Calvé-Perthes disease is directed toward the specific symptoms presented by each individual. Treatment varies with the severity of symptoms and may require the coordinated efforts of a team of specialists. Pediatricians usually refer patients to specialists who diagnose and treat disorders of the skeleton, joints, muscles, and related tissues (orthopedists); orthopedic surgeons, especially those that specialized in pediatrics; physical therapists; and other health care professionals may need to systematically and comprehensively plan an affected child’s treatment.

Degenerative bone changes associated with LCPD usually heal on their own without intervention (spontaneously). Affected children should be closely monitored so the progression of the disease may be evaluated to ensure the bone reforms properly. Physical therapy may be recommended to maintain a good range of motion of the joint. Radiologic studies (e.g., MRI, arthrography, and bone scan) may be used to assist in monitoring the progression of the disease.

Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen can be used for mild to moderate pain.

In other cases, decreased physical activity, bedrest (if pain is present and prolonged) with or without traction, and/or stretching exercises (if stiffness occurs), particularly exercises in which the legs are rotated away from the body (abduction exercises), may be recommended to decrease pain in the hip and maintain proper mobility of the joint. In addition, as an affected child ages and regrowth of the epiphysis occurs, physicians must ensure that epiphyseal growth is contained within the cavity of the hip bone (acetabulum) where the thigh bone (femur) and hip normally meet (articulate).

Nonsurgical treatment may include crutches or a special brace or cast to keep the ball of the joint completely within the socket (acetabulum) so that it will not deform while healing.

Surgical treatments may include various procedures designed to place and keep the ball within the socket. Surgery is done mainly to improve and preserve the shape of the hip joint in order to prevent or reduce the chances of arthritis later during life. A femoral or pelvic osteotomy is often performed. Osteotomy is a surgical procedure in which bone is cut to shorten, lengthen, or change the alignment (i.e. reposition or reorient the bone). Some adults may ultimately require a total hip replacement surgery (total hip arthroplasty) later in life due to osteoarthritis.

Physicians may rely upon specific classification systems to make certain decisions concerning treatment and predict an affected individual’s long-term progress. For example, the Stulberg Classification System, created in 1981, defines five outcome categories based upon the shape of the femoral head and its relationship with the hip socket (as seen upon x-ray imaging studies). However, the variability of the disease and long term follow up needed to understand the outcome of treatment have contributed to the lack of establishing a universal treatment strategy for children with this disorder.

What are the risk factors for lcpd?

Legg-Calvé-Perthes disease affects males approximately four to five times as often as females (4-5:1). According to reports in the medical literature, when the disorder occurs sporadically, males are predominantly affected. However, in families with more than one affected member (kindreds), the disorder appears to occur relatively equally among males and females. Reported cases of LCPD include several individuals within multiple, multigenerational families.

The disorder usually affects children between the ages of six and 10 years; however, associated symptoms and findings may become apparent as early as age two or as late as age 12. The incidence and prevalence of LCPD is unknown and estimates vary greatly from one country to another. The disorder is more common in Caucasians.

Is there a cure/medications for lcpd?

Several medications can be used to treat Legg-Calvé-Perthes disease (LCPD). The type of medication you will receive depends on the severity of your condition and whether you have had it for a long time.

The most common LCPD medications include:

1. Pain relievers: These include acetaminophen, ibuprofen, and naproxen sodium. These medications can help you feel less pain when exercising or doing daily activities.

2. Corticosteroids: These medications may be given as an injection into your hip to reduce inflammation. Corticosteroids are not recommended for children who are younger than 12 years old because they can cause growth problems.

3. Bisphosphonate: This medication reduces bone loss by slowing down how fast your body breaks down bone tissue. This is a good option if you have had LCPD for a long time or if your bones are very weak due to a lack of calcium in them (osteopenia).

4. Aspirin (acetylsalicylic acid): This medication is typically given in low doses over a long period of time. It's important not to give aspirin when there's an active infection or fever because it can make your child more likely to get sick.

5. Anticoagulants such as heparin or warfarin are also used in some cases if your child has a high risk of bleeding problems or has undergone surgery recently. These medications prevent blood clots from forming inside their body's arteries and veins by slowing down how fast their blood clots form; however, they can also cause major side effects, so they're only used when absolutely necessary (e.g., when there's a high risk of bleeding).

Stiffness in the hip or thigh,Pain in the hip or thigh,Limping or a limp,Unable to walk,Swelling of the joint in the hip or thigh,Decreased range of motion (ROM) of the joint in the hip or thigh
Bone Fracture,Bone Infection,Osteomyelitis
Corticosteroids,Anticoagulants,Anti-inflammatory drugs,Vitamin D analogues, Calcium and vitamin D supplements

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