Legg-Calvé-Perthes syndrome
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| ICD-10 | M91.1 |
|---|---|
| ICD-9 | 732.1 |
Legg-Calvé-Perthes syndrome is a degenerative disease of the hip joint, where a loss of bone mass leads to some degree of collapse of the hip joint, that is, to deformity of the ball of the femur and the surface of the hip socket. The disease is typically found in young children and small dogs, though in extremely rare cases it also affects adults.
It is the idiopathic avascular osteonecrosis of the capital femoral epiphysis of the femoral head. It is caused by an interruption to the blood supply of the head of the femur close to the hip joint.
It is also known as Perthes disease, ischemic necrosis of the hip, coxa plana, osteochondritis and avascular necrosis of the femoral head, or Legg-Perthes Disease or Legg-Calve-Perthes Disease (LCPD).
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[edit] Cause
The direct cause is a reduction in blood flow to the joint, though what causes this is unknown. Genetics does not appear to be a determining factor, though it may be involved. When the disease is genetic in origin, it typically runs along the male line. Some evidence suggests that parental smoking may be a factor, though this is not yet proven, or more recently that a deficiency of some blood factors used to disperse blood clots may lead to blockages in the vessels supplying the joint.
[edit] Signs and symptoms
Symptoms are hip or groin pain, exacerbated by hip/leg movement. There is a reduced range of motion at the hip joint and a painful 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, though can also be felt in the knee (so-called 'referred pain'). It is predominantly a disease of boys (around 4 in 5 cases), and is generally diagnosed between 5 and 12 years of age. Typically the disease is only seen in one hip, though appearance in both hips is not uncommon (about 10-15% of cases).
[edit] Diagnosis
X-Rays of the hip joint are absolutely necessary. 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.
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] Treatment
The goal of treatment is to avoid severe degenerative arthritis.
Treatment involves bedrest to take weight off the joint. It may require traction, leg braces, a plaster cast and physiotherapy. Surgery is only rarely necessary.
Orthopedic assessment is crucial. Younger children have a better prognosis than older children.
There are no drugs for treatment of Perthes. Analgesic medication should be given as necessary.
Treatment has traditionally centered on removing pressure from the joint until the disease has run its course. Options have included bed rest and traction (to separate the femur from the pelvis and reduce wear), often for several months or even years. Braces were also popular, again to isolate the joint. Recent evidence suggests that these methods are not effective, and treatment seems to be moving towards a mixture of careful monitoring, and surgical intervention when needed.
Perthes is a long-term problem - treatment is aimed at minimizing damage while the disease runs its course, not at 'curing' the disease. As sufferers age problems in the knee and back can arise, as a result of the abnormal posture and stride adopted to protect the affected joint. The condition is also linked to arthritis of the hip and other joints, 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] Incidence
Perthes is a relatively unusual condition, with only 1 in 9,000 children being affected.
Caucasians are affected more frequently than other races, males are affected 4-5 times more often than females and it is most commonly seen in persons aged 3-12 years, with a median of 7 years of age. In the US, 1 in 1200 children younger than 15 years will have this disease while in the UK 1 in 18,000 suffer from it.
[edit] Prognosis
Children that have been diagnosed with Perthes' Disease after the age of 10 are at a very high risk of developing osteoarthritis.
[edit] Legg-Calvé-Perthes disease in dogs
This is also known as aseptic necrosis of the femoral head. Yorkshire Terriers, Pugs, Jack Russell Terriers, and Dachshunds can be affected. Bilateral hip pain is usually seen by the age of 6 to 8 months. X-rays confirm the diagnosis. The recommended treatment is surgical removal of the head of the femur.[1]
[edit] References
- ^ Ettinger, Stephen J.;Feldman, Edward C. (1995). Textbook of Veterinary Internal Medicine, 4th ed., W.B. Saunders Company. ISBN 0721667953.
[edit] External links
- Perthes Association (UK)
- Because of its relatively low profile, much of the available literature on the subject is written from a medical viewpoint. A useful first step for parents or others affected by the disease is the Legg-Calvé-Perthes Disease page of the National Osteonecrosis Foundation
- eMedicine radio/387
- MedlinePlus Encyclopedia 001264
- DDB 9891
- Bill Shannon, a dancer with Legg-Calve-Perthes Syndrome who incorporates his crutches into his routines, which frequently take place in public with the intent of challenging peoples' perceptions of disability.
