Kristel Eunice F.

Caperal
4Nu03

Legg–Calvé–Perthes syndrome is a degenerative disease of the hip joint, where growth/loss of bone

mass leads to some degree of collapse of the hip joint and to deformity of the ball of the femur and the
surface of the hip socket. The disease is characterized by idiopathicavascular osteonecrosis of the capital
femoral epiphysis of the femoral head leading to an interruption of the blood supply of the head of
thefemur close to the hip joint. The disease is typically found in young children, and it can lead to
osteoarthritis in adults. The effects of the disease can sometimes continue into adulthood. It is also
known more simply as Perthes disease[1], ischemic necrosis of the hip, coxa plana, osteochondritis and
avascular necrosis of the femoral head, Legg–Perthes Disease or Legg–Calve-Perthes Disease (LCPD).
It is named for Arthur Legg, Jacques Calvé and Georg Perthes.[1][2][3][4]
Cause
Although no-one has identified the cause of Perthes disease it is known that there is a reduction in blood
flow to the joint. It is thought that the artery of the ligamentum teres femoris closes too early, not
allowing time for the medial circumflex femoral artery to take over.[citation needed] For example, a child may
be 6 years old chronologically but may have grown to 4 years old in terms of bone maturity. The child
may then engage in activity appropriate for a child of 6 but may not yet have the bone strength of an
older child, leading to flattening or fracture of the hip joint. Genetics do not appear to be a determining
factor, but it has been suggested that a deficiency of some blood factors used to disperse blood clots
may lead to blockages in the vessels supplying the joint, but these have not been proven.

Signs and symptoms

Common symptoms include hip, knee, or groin pain, exacerbated by hip/leg movement. The pain feels
like a tooth ache, possibly severe. There is a reduced range of motion at the hip joint and a painful
or antalgic 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, but can
also be felt in the knee ('referred pain'). In some cases, pain is felt in the unaffected hip and leg [citation
needed]
, due to the child favoring the injured side and placing the majority of their weight on the "good"
leg. It is predominantly a disease of boys (4:1 ratio). Whereas Perthes is generally diagnosed between 5
and 12 years of age, it has been diagnosed as early as 2 years of age. Typically the disease is only seen in
one hip, but bilateral Perthes is seen in about 8-10% of children diagnosed.
Onset of pain may be up to 4 hours after inactivity. Knee pain is felt in the back of the knee rather than
in the front, not unlike a localized charley horse. This lasts for an hour or so and returns nightly on
inactivity.

Diagnosis
X-Rays of the hip confirm the diagnosis. X-rays usually demonstrates a flattened and later fragmented
head of femur. A bone scan or MRI may be useful in making the diagnosis in those cases where x-rays
are inconclusive. Neither bone scan or MRI offer any additional useful information beyond that of x-rays
in an established case. If MRI or bone scans are necessary a positive diagnosis relies upon patchy areas
of vascularity to the capital femoral epiphysis (the developing femoral head).

Treatment
The goal of treatment is to avoid severe degenerative arthritis. Orthopedic assessment is crucial.
Younger children have a better prognosis than older children.
Treatment has traditionally centered on removing pressure from the joint until the disease has run its
course. Options include traction (to separate the femur from the pelvis and reduce wear) braces (often
for several months, with an average of 18 months) to restore range of motion, physiotherapy, and
surgical intervention when necessary because of permanent joint damage. To maintain activities of daily
living, custom orthotics may be used. These devices internally rotate the femoral head and abduct the
leg(s) at 45 degrees. Orthoses can start as proximal as the lumbar spine (LSO), and extend the length of
the limbs to the floor. Most functional bracing is achieved using a waist belt and thigh cuffs derived from
the Scottish-Rite Orthosis.[5] These devices are typically prescribed by a physician and implemented by a
certified orthotist. For older children, the distraction method has been found to be a successful
treatment, using an external fixator which relieves the hip from carrying the body's weight. This allows
room for the top of the femur to regrow. Many children need no intervention at all and are simply asked
to refrain from contact sports or games which impact the hip. The Perthes Association has a "library" of
equipment which can be borrowed to assist with keeping life as normal as possible, newsletters, a
helpline, and events for the families to help children and parents to feel less isolated.
Modern treatment focuses on removing pressure from the joint to increase blood flow, in concert with
physiotherapy. Pressure is minimized on the hip through use of crutches or a cane, and the avoidance of
running-based sports. Swimming is highly recommended, as it allows exercise of the hip muscles with
full range of motion while reducing the stress to a minimum. Cycling is another good option as it also
keeps stress to a minimum. Physiotherapy generally involves a series of daily exercises, with weekly
meetings with a physiotherapist to monitor progress. These exercises focus on improving and
maintaining a full range of motion of the femur within the hip socket. Performing these exercises during
the healing process is essential to ensure that the femur and hip socket have a perfectly smooth
interface. This will minimize the long term effects of the disease. Use of zoledronic acid has also been
investigated.[6]
Perthes disease is self limiting, but if the head of femur is left deformed there can be a long-term
problem. Treatment is aimed at minimizing damage while the disease runs its course, not at 'curing' the
disease. It is recommended not to use steroids or alcohol as these reduce oxygen in the blood which is
needed in the joint. As sufferers age, problems in the knee and back can arise secondary to abnormal
posture and stride adopted to protect the affected joint. The condition is also linked to arthritis of the
hip, 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.

Prognosis
Children younger than 6 have the best prognosis since they have time for the dead bone to revascularize
and remodel. Children who have been diagnosed with Perthes' Disease after the age of 10 are at a very
high risk of developing osteoarthritis and Coxa Magna.