Legg calve perthes disease

Legg calve perthes disease (LCPD) is a rare hipbone disease that annually occurs in many children around the world. Read and know all about the disease as well as its causes, symptoms, diagnosis and treatment.

What is Legg calve perthes disease?

It is a degenerative disorder that affects the hip joint. It occurs when there is an insufficiency in the volume of blood reaching the ball of the thighbone, causing death of the bone.

Picture 1 – Legg calve perthes disease

In medical world, the condition is also known as Ischemic Necrosis of the Hip or Avascular Necrosis of the Hip.

Legg calve perthes disease Incidence

The condition commonly occurs in individuals aged between 3 and 12 years, with the highest incidence being observed in 6 year olds. The disease is five times more likely in males than females. The symptoms mostly emerge in individuals aged 14. One out of 100 male children with parents having this condition is found to suffer from it at a later time. In the US, one out of 1200 children below 15 years can have this condition while 1 out of 2000 in the UK are diagnosed with it. Around 5 out of 100,000 children are found to have this condition every year.

Legg calve perthes disease Symptoms

Patients with this disease usually limp while walking, which is the first symptom of this condition. The disorder is generally painless. In some cases, patients may experience mild pain that comes and disappears. The disease is generally unilateral (affects one hip). However, it may also be bilateral, meaning it may cause problems in both the hips at the same time.

Other signs and symptoms may involve:

• Knee pain
• Constant pain in groin or thigh
• Limited range of movement
• Hip stiffness, which limits motion in the hip
• Reduction of muscles in the upper thigh
• Shortening of the leg or unequal length of legs

In healthy people with normal circulation, all parts of the human body get supplied with nutrient-rich, oxygenated blood. In people with LCPD, however, proper blood supply to the hip joint gets impaired. Due to lack of nourishment, cells inside the hip joint begin to die at once. The death of blood-famished bone cells leads to the onset of bone degeneration. This deterioration leaves the afflicted bone matter compromised despite the arrival of fresh, healthy cells. This ultimately results in instability of the hip joint, thus making it susceptible to fractures. The hip joint becomes unable to heal itself as it would in normal cases.

Legg calve perthes disease Causes

It is not clear what exactly gives rise to this condition. As aforesaid, the condition arises due to insufficient supply of blood to the femoral head (hip joint ball). The absence of proper blood supply naturally results in a deterioration of the femoral head. As new bone cells replace the dying cells, the bone turns unstable. It may easily crack and heal very slowly. According to some medical organizations, insufficient blood supply to the hip joint results from secondhand exposure to smoke.

Legg calve perthes disease Diagnosis

During a physical examination, the health care provider will look for a loss in hip motion and a typical limp. A hip x-ray or pelvis x-ray may show signs of Legg-Calve-Perthes disease. An MRI scan may be needed.

Legg-Calve-Perthes disease may be diagnosed at any point during childhood, but it is most commonly detected during the second year of a child’s life. The earlier the disease is detected, the better the child’s prognosis. Following a comprehensive consultation and physical examination, a battery of imaging tests is usually performed. Bone scans and X-rays are frequently conducted to evaluate the condition of the hip joint and to check for bone abnormalities or changes.

Legg calve perthes disease Differential Diagnosis

The differential diagnosis of LCPD includes ruling out the possibility of the symptoms being caused by other disorders, such as:

• Juvenile idiopathic arthritis
• Sickle cell disease
• Septic arthritis
• Transient synovitis of the hip

Legg calve perthes disease Treatment

Treatment for Legg Calve Perthes disease mainly aims at preventing long-term damage to the joint of the hip. This involves:

• Physical therapy – Regular physical therapy helps reduce stiffness in the hip joint. The affected child may be taught some exercises that can be practiced at home. These can help maintain the range of motion in the hip.
• Anti-inflammatory medicines – Medications, such as Ibuprofen, can help provide relief from stiffness as well as inflammation in the hip joint.
• Supportive equipments – In many cases, crutches, braces or nighttime traction devices may be used to immobilize the bone and ease limping in patients.
• Limiting weight – If there is worsening of the limp or too much pain in the hip, it may help to restrict activities such as running or reducing the amount of weight placed on the leg.
• Bed rest – Although LCPD patients are not recommended to take several months of bed rest any more, severe pain can be alleviated with a short period of bed rest.

LCPD treatment often uses a process known as “Containment” that holds the thighbone ball within the socket. This is done to ensure that the hip has good range of movement. In some cases, braces may be used to help the technique.

Even after treatment, it is essential to regularly follow-up the progress with an orthopedic specialist or doctor.

Legg calve perthes disease and Surgery

Surgery may be required if all other treatment options fail. The form of surgery depends on the shape of the hip joint ball as well as the severity of the problem. Surgery may also be needed to adjust the hip joint or correct muscle constriction.

Osteotomy

This surgical technique has various forms and involves cutting and repositioning of the bone. The process is performed at various stages of the condition, depending on the requirement. In some cases, softening of the hip joint ball increases the possibility of its slipping out of the socket. This can be prevented by a Femoral Varus Osteotomy. Whether conducted with rotation or without it, the process partly redirects the joint ball back into the socket.

Osteotomy may also be performed above the hip socket. This lets surgical repositioning of the hip socket in a manner that leaves little possibilities for the femoral head to become deformed. Shelf arthroplasty provides the ball with additional coverage from the top lip of the socket. Both shelf and innominate arthroplasty helps in reshaping the ball joint.

Tenotomy

A “Tenotomy” is a surgery that is performed to release an atrophied muscle that has shortened due to limping. Once released, a cast is applied allowing the muscle to regrow to a more natural length. Cast time is about six to eight weeks.

Legg calve perthes disease Prognosis

The outcome of the disease depends on the age of the patient as well as the severity of the condition. Generally, the younger the patient, the better is the prognosis.

Legg calve perthes disease Complications

Kids who are less than 6 years of age and receive timely treatment are more likely to end up with a normal hip joint. Children over 6 years of age commonly suffer from deformities in hip joint, despite treatment. Such patients may develop Osteoarthritis or Degenerative Arthritis at a later stage in life. Generally, early diagnosis and proper treatment of LCPD helps minimize complications.

Legg-Calve-Perthes disease Risk Factors

Even though it may affect children of about any age group, the disorder is most common among those aged between 2 and 12. It is five times more likely in boys than girls. When girls develop this condition, it is supposed to be more serious. The disease also seems to have a racial preference as it is most common in Eskimos, Asians and white people. It is also common in

• Children who are physically active
• Kids who are physically small for their age
• Children who are exposed to passive smoke

Legg calve perthes disease Prevention

The condition cannot be prevented as its exact cause is still unknown. However, one may still take adequate measures that may help minimize or prevent the collapse of the femoral head. This can be discussed with doctors and physiotherapists.

Legg calve perthes disease in Children

Children affected with LCPD often walk with a limp and report of pain on the impacted side. A restricted range of hip motion often induces stiffness that passes down from the hip to the knee. With passing time, limping may lead to constriction of muscles in the affected limb. In some cases, children report of an improvement in the symptoms by resting the joint. Keeping the joint immobile can ease painful symptoms in the joint. If symptoms are left untreated, permanent damage may occur in the hip joint.

If your child is showing up any of the symptoms of this disorder, immediately get in touch with a professional health care provider. Timely detection and treatment will go a long way to ensure quick recovery without any risk of future complications.

References:

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002244/

http://emedicine.medscape.com/article/826935-overview

http://www.mayoclinic.com/health/legg-calve-perthes-disease/DS00654

http://www.orthoseek.com/articles/perthes.html