What is Legg-Calve-Perthes disease?

Legg-Calve-Perthes disease is a rare condition affecting the hip in children aged 2 to 12. It is caused when the head of the femur bone, known as the "ball" in the "socket" in the hip joint, temporarily loses its blood supply. When the bone is deprived of blood, the tissue turns into necrosis. After some time, blood flow is restored to heal the bone slowly; however, it does not regain its original shape and size.

Legg-Calve-Perthes disease is a type of disorder called an osteochondrosis. This refers to a degeneration and regeneration of the growing end of a bone. It occurs unexpectedly and for unknown reasons. It progresses over stages, and the process, as a whole, may take several years. How it impacts your child will depend on how much their bone changes. With treatment, the long-term prognosis is good.

There are several other names associated with Legg-Calve-Perthes disease, including:

• Perthes disease.
• Perthes syndrome.
• Legg Calve.
• Legg-Perthes.
• Calve-Perthes.
• Coxa plana.

How rare is Perthes disease?

Approximate figures suggest that Legg-Calve-Perthes disease affects approximately 1 in 12,000 children. It is five times more common in children assigned male at birth compared with children assigned female at birth.

At what age is Legg-Calve-Perthes the most prevalent?

The average age of the most frequently reported cases of Perthes disease lies within kids aged 5-7. It could still happen sometimes in a child as small as 2 and sometimes up to an older child as 12.

Is Legg-Calve-Perthes disease permanent?

The degeneration and regeneration process in your child's femur bone from the syndrome caused by Perthes can last for years. The condition is reversible but can bring about permanent changes.

Can Perthes disease lead to problems later on?

If the shape of your child's femur head changes significantly, it might not fit into its hip socket again. Treatment will help, but if it continues not to fit, your child may develop some form of hip problem later in life.

What is Legg-Calve-Perthes disease and what are its signs and symptoms?

Like Perthes itself, the signs and symptoms of Perthes seem to develop over time, often even without a clear onset. You or your child will likely start wondering what's wrong when symptoms appear and never improve.

Early symptoms may include:

• Limp: Your child may prefer to bear weight on one leg when walking to avoid the affected side. Typically a limp develops before the child knows they have pain. The child does not know why he or she is limping.
• Hip pain: If your child has pain, the time is likely to be early in the course of this disease, when the bone has died and is beginning to break down. Pain is worse with activity.
• Referred pain: Some children may have pain in their knees, thighs, pelvises, or abdomens.
• Leg cramps: Children may have spasm in the leg muscles where these muscles are attached at your child's hip joints.

Some later signs and symptoms of LCPD include:

• Trendelenburg gait: This malignant form of walking suggests hip problems. Weak hip muscles cause the pelvis to tilt downward on the affected side as your child walks.
• Limited range of motion: Your child may have trouble moving their hip. They might not be able to open their thighs open as much or easily turn their knees inward as they once could.
• Muscle wasting: You may be able to notice that your child's thighs and buttocks are shrinking due to loss of muscle mass.
• Leg length difference: One leg might look shorter than the other.

What causes Legg-Calve-Perthes disease?

In Perthes disease, the blood supply to the head of the femur bone temporarily slows or ceases. As the bone relies on the blood supply to bring oxygen and nutrients, its death and breakdown occur during the absence of these life-giving agents. This process is, therefore, termed avascular necrosis or osteonecrosis.

Normally, ischemia may develop if a blood vessel becomes obstructed or compressed. It might be blocked inside by artery disease or a clot, or compressed from the outside by swelling. But so far, the cause of ischemia in LCPD is not known. It may be different in different children.

What are the risk factor for Legg-calve-Perthes disease?

Research shows that nearly half of the children who have LCPD suffer from some blood clotting disorder. Another supposed cause is a traumatic injury or repetitive strain injury which caused enough swelling to cut off blood flow to the bone. About 10% appears to be related to an inherited gene mutation.

Factors that increase the risk for your child developing Legg-Calve-Perthes include:

• Delayed skeletal growth
• Low birth weight.
• Short stature.
• Abnormal teeth.
• Low socioeconomic status.
• Secondhand smoke exposure.
• Blood clotting disorder.
• HIV infection.
• Some form of athleticism, such as sports or gymnastics.
• Genetic mutations in the COL2A1 gene.

How will Perthes disease affect my child?

When the condition remains active, the head of your child's femur bone in their hip socket would degrade (break down) and then regenerate (rebuild). The breaking down phase could last up to a year, and rebuilding and remodeling would last two to five years.

Some children lose more tissue than others. Perhaps their ischemia is worse or lasts longer, or perhaps it's simply that they are using their bone too much when it is weakened, producing secondary damage. In some children, the rounded heads of their femurs collapse and become flattened, making them a poor fit in their hip sockets.

At that point, the bone gets another chance to shape and fit properly. However, unlike beads in a jar, each child's growth is unique. Some femurs never achieve the size and dimensions they had before. Others overgrow the hip socket. Your surgeon will make efforts to control those results.

Some children will have to be pinned, with their hips held in place with a cast for a while, and others will need to have surgery. These interventions can assist the bone in repairing itself properly. Those children who do not have hip joints that fit together perfectly will continue to have their symptoms. In some cases, the disordered fit of Legg-Calve-Perthes disease can cause other problems, like arthritis, to develop later in life.

How is Legg-Calve-Perthes disease diagnosed?

A healthcare provider will begin with a proper physical examination and then later take X-rays to examine your child's bones. The X-rays may show signs of Perthes syndrome, stage, and severity. In some cases, they may order an MRI to examine specific things in greater detail.

How is Legg-Calve-Perthes disease treated?

Legg-Calve-Perthes treatment will depend on the severity of the disease, its stage, and the child's age. Typically, an early child has rapid bone growth and does better with less invasive treatment, whereas an older one may need more. A health care team will build an appropriate treatment plan for your child.

This can include:

Limited activity and weight bearing on their injured hip. Your child may need to avoid or limit walking for some time, or use an assistive walking device, such as crutches, to get around.

Nonsteroidal anti-inflammatory drugs (NSAIDs). These OTC drugs, such as aspirin and ibuprofen, can help manage the pain and inflammation of the hip.

• Physical therapy: A physical therapist will work with your child to rehab the hip by developing special exercises. You may have to assist your child with practicing their exercises.
• Traction: An orthopedic traction device gently and progressively stretches or pulls the hip joint into place over several days.
• Rods or casts: Your doctor may make you wear a cast or rod during the remodeling process to stabilize the bone in the socket. The socket can help form the bone as it re-erupts.
• Surgery: Some children will need surgical intervention to better shape and restore the bone in the socket. This is called osteotomy. Others may need arthroplasty to lengthen or enlarge the hip socket.

Can Perthes disease be prevented?

Unfortunately, no. No one knows how to prevent Perthes disease.

What is the prognosis for Legg-Calve-Perthes disease?

If your child has acquired Legg-Calve Perthes, a health journey several years long with successive stages will interrupt their childhood to some extent—less so for some than for others. However, when it's over, most can go back to whatever they were doing without any restrictions or lingering symptoms.

If your child is less than 7, he or she is likely to heal completely from conservative treatments. However, if your child surpasses 7 years, then he is likely to need surgical interventions for the femur bones. If the proper treatment is not given at the right time, if the femur bones continue to be distorted, then later in life, these kids are likely to contract hip osteoarthritis.

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