Perthes disease
Perthes disease
- Perthes disease is found in children between 4 and 10 years of age.
- It affects four times as many boys as girls.
- Twenty percent of cases are familial.(The incidence of the disease is 1:35 in family members compared with 1:20,000 in the general population.)
- The disease affects both hips in 20% of patients.
- The low birth weight (especially boys less than 5.5 pounds) is correlated with the disease
Because many disease processes have signs and symptoms similar to those of perthes disease, it is diagnosed by x-rays. The first x-ray may not be helpful. Because characteristic signs may not have appeared. Bone scans and magnetic resonance images (MRI’s) can be used to detect the amount of blood circulation in the femoral head.
A two-group classification for perthes has been developed. Group A consists hips with less than half of the femoral head involved. This group generally needs little treatment and recovers well. Group B includes hips with more than half of the femoral head involved. Patients in this group need treatment to do well.
The aim of treatment is to prevent flattening of the femoral head. There are several methods of treatment: bracing, short periods of rest, soft- tissue releases, and operations on the bone. The choice depends on factors such as the severity of the problem and the stage of the disease.
Treatment is divided into five categories: (1) observation, (2) intermittent symptomatic treatment, (3) definitive early treatment (to prevent deformity), (4) surgery to make the femur head round again, and (5) late surgery for arthritis.
Observation consists of testing the hip stiffness and taking x-ray every few months. It is suitable for children at the time of onset of perthes disease, regardless of the amount of femoral head involvement, provided there is no limitation of movement or subluxation (partial dislocation) of the hip. Observation is also appropriate for children who have Group A perthes with a good range of hip motion and no evidence of femoral head collapse (as indicated by x-rays).
This treatment consists of rest and exercise when the hip hurts, and may be used in conjunction with observation. It includes muscle-stretching exercise to maintain the joint’s range of motion, and periodic bed rest, with or without traction. X-rays at 3-4 month intervals are necessary during the early months after the first signs and symptoms appear.
This type of treatment is used in cases of clinical onset at 6 years of age or older, a Group B hip, and loss of femoral head containment. It involves the use of containment methods (either surgical or non-surgical) early in the disease process.
Containment can be pictured as an ice cream scoop encasing soft ice cream. When the ice cream is enveloped by the scoop (like the head of the femur in the acetabulum or hip socket), the ice cream remains round and undented. Uncontainment occurs when the scoop covers only half of the ice cream. The ice cream outside the scoop dents and the remainder then seeps out. Similarly, the femur is no longer within the acetabulum or socket and a dent develops so the femur is not rounded.
- Nonsurgical containment (braces and casts)
This type of containment is carried out by abducting or drawing away from the body the affected limb in order to place the femoral head back into the acetabulum. The joint is allowed to move, and the movement gradually molds the head into a round shape. Casts and/or braces may be used to keep the femoral head within the joint, and crutches may be used to limit weight bearing on the joint.
Abduction casts consist of long leg casts applied to both lower limbs and held apart by sticks.
A variety of abduction apparatuses and braces are also available. They are lighter, removable, less cumber-some, but also more expensive than casts. Twelve to 18 months of this type of treatment may be required.
2.Surgical containment
Surgical containment is indicated when nonsurgical treatment is not feasible or when it is not possible to contain the femoral head in the acetabulum (hip joint) in an abducted position.
A procedure known as an innominate osteotomy is performed. It takes about 2 hours. The purpose of this operation is to use the normal movement of the hip to mold the soft bone into a round shape.
The bones are moved into the desired position and held together with pins. The pins are removed after 6-12 weeks in a minor operation, usually done on an outpatient basis. After surgery the child is often put into a cast or brace until the joint is stable (i.e., the femoral head well contained). After an 8-week postoperative recovery and a short rehabilitation period, the child is able to resume normal activities. The entire process usually takes about 3-4 months.
When there is significant femoral head deformity that cannot be successfully treated with the methods already described, alternative techniques must be sought.
One procedure used is known as a muscle release (of adductors, muscles that draw the limb in toward the centre of the body).this is followed by the use of an abduction cast to hold the limb out. After the patient has worn the cast 3-4 months, enough abduction should be gained so that the femoral head can be placed into the acetabulum through a surgical osteotomy.
A second technique involves a combination of femoral and pelvic osteotomies. If the deformed femoral head cannot be contained within the acetabulum, part of the head may have to be removed.
In general, the younger the child at the age of onset of perthes disease, the better the results of treatment. Many long-term studies have shown that most children with clinical onset at less than 6 years of age have an excellent prognosis. However, they must be evaluated through clinical and radiological (x-ray) examinations at intervals of 2 to 4 months. If loss of either motion or containment is noted, a short course (3-6 months) of nonsurgical treatment may be necessary.
The extent of femoral head involvement has much to do with prognosis. Children with less than half of the femoral head involved (Group A) show better treatment results. As loss of containment may still occur, these children must also be examined every 2-4 months.
In girls, perthes disease is more likely to involve over half of the femoral head, and so more vigorous treatment is usually needed.
Regardless of the method of treatment chosen, it is essential that a good range of motion be achieved for the hip and maintained throughout treatment. The outcome should then be satisfactory.
Tips for parents
- Don’t panic! Many surgical and treatment advances have been made in this field. Doctors who treat this condition are familiar with its details and have repeatedly demonstrated high levels of success in treating it.
- Ask questions. You should discuss your child’s problem thoroughly with your doctor. Do not hesitate to ask him all the questions you may have. Write down questions ahead of time.
- Become involved in your child’s treatment. We will teach you what you need to know to help in your child’s recovery.
- Follow directions about casting, braces and harnesses carefully. Remove them only as directed by the physician.
- If your child is in a cast or brace, keep it clean.
- Continue to monitor your child’s problem with your physician. This will allow for early detection and treatment should the condition recur.
- Make a conscious effort to treat your child as normally as possible.
- Encourage your child to resume normal activities as soon as is medically recommended.
- Use all available resources. Remember you are not in this alone. Many professionals are available to answer questions or direct you to people who can help you.
Legg-Calve-Perthes Disease
Legg-Calvé-Perthes disease is a temporary condition in children in which the ball-shaped head of the thigh bone, referred to as the femoral head, loses its blood supply. As a result, the femoral head collapses. The body will absorb the dead bone cells and replace them with new bone cells. The new bone cells will eventually reshape the femoral head of the thigh bone. Legg-Calvé-Perthes disease causes the hip joint to become painful and stiff for a period of time.
Legg-Calvé-Perthes disease goes through four phases of changes that affect the head of the femur. The phases include:
- Phase 1 – Blood supply is absent to the femoral head and the hip joint becomes inflamed, stiff, and painful. Portions of the bone turn into dead tissue. The ball of the thigh bone becomes less round in appearance on x-rays. This phase can last from several months up to one year.
- Phase 2 – The body cleans up the dead bone cells and replaces them with new, healthier bone cells. The femoral head begins to remodel into a round shape again. The joint is still irritated and painful. This phase can last from one to three years.
- Phase 3 – The femoral head continues to model itself back into a round shape with new bone. This phase lasts for one to three years.
- Phase 4 – Normal bone cells replace the new bone cells. This last phase can last a few years to complete the healing process.
The cause of Legg-Calvé-Perthes disease is unknown. It is four times more likely to occur in boys than girls.
Legg-Calvé-Perthes disease commonly affects first-born children and is typically seen in children 4 to 8 years of age. The majority of cases affect only one hip.
The child typically complains of pain in his/her hip that is aggravated by activity. Sometimes, they will also experience pain in their thigh or knee area. The child usually walks with a limp and reports that rest will alleviate the pain.
The symptoms of Legg-Calvé-Perthes disease may resemble other conditions or medical problems of the hip. Always consult your child’s physician for a diagnosis.
In addition to a complete medical history and physical examination, diagnostic procedures for Legg-Calvé-Perthes disease may include:
- x-rays – a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
- bone scans – a nuclear imaging method to evaluate any degenerative and/or arthritic changes in the joints; to detect bone diseases and tumors; to determine the cause of bone pain or inflammation.
- magnetic resonance imaging (MRI) – a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.
- arthrograms – a diagnostic imaging test to study the non-bony structures of joints.
- blood tests
Specific treatment for Legg-Calvé-Perthes disease will be determined by your child’s physician based on:
- your child’s age, overall health, and medical history
- the extent of the condition
- your child’s tolerance for specific medications, procedures, or therapies
- expectations for the course of the condition
- your opinion or preference
The goal of treatment is to preserve the roundness of the femoral head and to prevent deformity while the condition runs its course. Treatment options are dependent upon the amount of hip pain, stiffness, and x-ray changes over time, as well as how much of the femoral head has collapsed.
Typically, the first step of treatment is to regain hip motion and eliminate pain that results from the tight muscles around the hip and the inflammation inside the joint. Treatment may include:
- rest
- activity restrictions
- medications
- bed rest and traction
- casting or bracing (to hold the femoral head in the hip socket, permit limited joint movement, and allow the femur to remold itself into a round shape again)
- surgery (to hold the femoral head in the hip socket)
- physical therapy (to keep the hip muscles strong and to promote hip movement)
- crutches or wheelchair (in some cases)
The ultimate goal in Legg-Calvé-Perthes disease is to diagnose the condition early in order to allow as much time as possible to let the femoral head remodel back into a round shape. Other treatment goals include controlling pain, maintaining hip motion, and preventing continued hip deformity.
The two most critical factors that determine the outcome are the child’s age and how much of the femoral head is affected by this condition.
The more severe the case, the greater the likelihood that the child may experience limited hip motion, differences in leg lengths, and further hip problems in adulthood.
