Legg-Calve-Perthes disease

Other Names: Coxa plana, LCPD, Legg-Calve-Perthes syndrome, Osteochondritis deformans, Perthes disease See more

Categories: Congenital and Genetic Diseases, Connective tissue diseases, Musculoskeletal Diseases

Grouped Under

Osteochondrosis, Legg-Calve-Perthes disease

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Description

Legg-Calve-Perthes disease (LCPD) occurs when blood supply to the ball of the thighbone in the hip (femoral head) is disrupted. Without an adequate blood supply, the bone cells die. LCPD usually occurs in children between the ages of 4 and 10. Early symptoms may include limping; pain in the hip, thigh or knee; and reduced range of hip motion. Later in the disease course, there may be leg length discrepancy (one leg longer than the other) and wasting of the muscles around the hip. The condition can last for several years before new bone formation (re-ossification) and eventual healing occurs. Some people with LCPD go on to develop degenerative arthritis in adulthood.

The underlying cause of LCPD is often not known. Sometimes it results from a slipped capital femoral epiphysis, trauma, steroid use, sickle-cell crisis, toxic synovitis, or congenital hip dysplasia. It usually is not caused by genetic factors, but a few familial cases with autosomal dominant inheritance have been reported. In a small percentage of cases, mutations in the COL2A1 gene were found to be responsible.

Treatment aims to keep the thigh bone inside the hip socket. Treatment options may include rest or medication for pain; physical therapy; using a brace; or surgery.

Source: GARD Last updated on 05-01-20

Symptoms

Symptoms and their Known Frequencies

We're currently performing maintenance on our symptoms information from GARD. Please check back at a later time for updates.

Is Legg-Calve-Perthes disease inherited?

Legg-Calve-Perthes disease (LCPD) is usually not caused by genetic factors (thus is usually not inherited), but there are some cases where LCPD affects more than one family member. In a small percentage of these familial cases, changes or mutations in the COL2A1 gene have been found to cause LCPD. When mutations in COL2A1 gene are the cause of LCPD, the disease is inherited in an autosomal dominant manner. In some cases, it appears that genetic and environmental factors interact to increase a person's chance to develop LCPD.

Last updated on 05-01-20

Can extreme weakness in the hips be associated with having had Legg-Calve- Perthes disease?

We are unable to say whether extreme weakness in the hips might be due to having had Legg-Calve-Perthes disease (LCPD) and/or having arthritis, or whether the weakness could be due to a separate underlying problem.

It has been reported that people who have had LCPD can develop abductor muscle weakness in addition to pain, impaired range of motion, and osteoarthritis of the hip. However, we were unable to locate information specifically describing extreme weakness being associated with LCPD. Although many long- term follow-up studies about LCPD have been published, the number of recent studies with a follow-up longer than 40 years is scarce.

Lower extremity muscle weakness has been directly associated with osteoarthritis of the hip, which develops in the majority of people with LCPD by the sixth or seventh decade of life. The mechanisms underlying the muscle weakness may include a combination of reduced muscle size (atrophy), muscle inhibition, and decreased muscle quality.

Last updated on 05-01-20

What is the long-term outlook for people with Legg-Calve-Perthes disease?

The prognosis for people with Legg-Calve-Perthes disease (LCPD) depends on the extent and severity of bone involvement, and residual deformity. Overall, the prognosis for recovery and sports participation after treatment is very good for most people. Generally, a younger age at diagnosis is associated with a better outcome.

For people who are younger than age 5 when LCPD develops, the incidence of degenerative arthritis later in life is reportedly very low. The more deformed the femoral head is during healing, the greater the risk of osteoarthritis of the hip later in life. The risk is also higher for those with metaphyseal defects (where the shaft of the bone flares out); and for those who develop LCPD late in childhood (at age 10 or older). Nearly 100% of people with complex involvement of the femoral head and residual deformity will develop degenerative arthritis. Total hip replacement in early adulthood may be needed in some cases.

Last updated on 05-01-20

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