Legg-Calve-Perthes Disease

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Clinically Relevant Anatomy

This disease involves the femoral head.

Mechanism of Injury / Pathological Process

The origin of this disease is unknown. The pathology of the disease is, however, accepted and is as follows.
First, there is interrupted blood supply to the capital femoral epiphysis. After this, an infarction of the subchondral bone occurs. Next, revascularization of the area occurs and new bone ossification begins. This is the turning point where a percentage of patients will have normal bone growth and development; while others will develop Legg Calve Perthes Disease. (LCPD). This disease is present when a subchondral fracture occurs. Usually, there is no trauma to cause this scenario. LCPD is most commonly the result of normal physical activity. Because of the subchondral fracture, changes occur to the epiphyseal growth plate.

Classification:
Severity and prognosis of the disease is determined by using a variety of classification systems.
Two of the classification systems are listed here.
In the Catteral Classification there are four different groups to define the radiographic appearance during the period of greatest bone loss.
The Catteral Classification specifies four different groups to define radiographic appearance during the period of greatest bone loss.
These four groups are reduced down to two by the Salter-Thomson Classification. The first group, which is Group A (Catteral I,II) shows less than 50% of the ball is involved. Group B (Catteral III, IV) shows that more than 50% of the ball is involved. If there is less than 50% involvement the prognosis is good; if there is more than 50% there is usually a poor prognosis.
The Herring Classification is based on the integrity of the lateral pillar of the ball. Group A of this classification shows no loss of height in the lateral 1/3 of the head and little density change. In Lateral Pillar Group B, there is less than 50% loss of lateral height and lucency is present in the joint. In some cases, the ball is beginning to extrude the socket. In Lateral Pillar Group C, there is more than 50% loss of lateral height.

There are four phases of Legg-Calve Perthes Disease which are as follows:

1.  Increased density of femoral head possibly leading to fractures

2.  Bone undergoes fragmentation and reabsorption

3.  Growth of new bone

4.  Reshaping of new bone

Clinical Presentation

This disease presents in children 2-13 years of age and there is a four times greater incidence in males compared to females. The average age of occurrence is six years.
A psoatic limp is typically present in these children secondary to weakness of the psoas major.
The child will show a decrease in extension and abduction active ranges of motion.
There is usually no traumatic event to initiate symptoms

Diagnostic Procedures

An MRI is usually obtained to confirm the diagnosis; however x-rays can also be of use to determine femoral head positioning.

Outcome Measures

The Lower Extremity Functional Scale is one that measures how this disease is affecting the child in a functional way. Since this questionnaire does ask about certain activities the child may not be allowed to perform (i.e. running, hopping, etc), it may not be the outcome measure of choice. The Harris Hip score is another questionnaire that has more to do with a lower level of functional activities such as walking, stair climbing, donning/doffing shoes, sitting, etc. Questionnaires that test the patient on a functional level are useful to provide a baseline and monitor functional progress in the patient’s activities.

Management / Interventions

Medications include acetaminophen for pain as well as NSAIDS for pain and/or inflammation.
Physical therapy can help with joint protection strategies, improving joint mechanics, building a safe aerobic endurance component, and regaining functional flexibility, strength, coordination, endurance and gait training.

Differential Diagnosis

Listed are some other disorders that should be included in the differential diagnosis for LCPD:
Septic arthritis-This is an infection in the joint
Sickle cell-Osteonecrosis of the hip can be a result of this disease
Spondyloepiphyseal Dysplasia Tarda-This disease typically affects the spine and the larger more proximal joints
Gaucher’s Disease- This is a genetic disorder that often times includes bone pathology
Transient Synovitis-This is an acute inflammatory process and is the most common cause of hip pain in childhood

Key Evidence

According to a research article in the Journal of Bone and Joint Surgery, Legg Calve Perthes is best treated with surgery if the child is eight years old or older at the time of onset; and second if the child falls into a category B or B/C border group of the laterall pillar system (herring).
In other studies, good prognosis of a child with this disease under the age of eight has been shown to be up to 80%. This is with minimal treatment given to the patient. In this study, children between the ages of 4 and 5 and 11 months had a less favorable chance of a good outcome.

Resources

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Case Studies

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Recent Related Research (from Pubmed)

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References

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