Perthes disease, or actually Legg-Calve-Perthes disease (Latin coxa plana) - sterile femoral head necrosis, is one of the many causes of hip pain and walking disorders in children. If the symptoms of Perthes' disease are not noticed in time, the complications caused by it may be irreversible. What is the treatment and rehabilitation of Perthes disease? When is surgery necessary? Is Perthes disease hereditary?

Perthes disease , or actually Legg-Calve-Perthes (Latincoxa plana ), is an inflammatory disease in which the so-called aseptic necrosis of the femoral head. This is where inflammation takes place, but it is not associated with the presence of any microorganism, pathogen, but leads to bone damage and loss.

Perthes disease most often occurs in children between 4 and 8 years of age, usually boys. Its symptoms are often noticed in family members of the patient, but it has not been proven to be a genetic disease. It has also been noticed that it is slightly more common in children living in industrialized areas and in more active people.

Perthes disease: causes

The cause of Perthes disease remains unknown, and research is still ongoing. Currently, it is believed that the main mechanism leading to it is the disturbance of the blood supply to the femoral head, related to the intensive growth of the child.

More specifically, the amount of blood reaching the deep growth cartilage layer of the femoral head is reduced, so that the tissue does not receive the right amount of oxygen and nutrients.

Consequently, bone growth, necrosis and atrophy are disturbed. In some cases, ossification disorders in the wrist and disturbances in the structure of the femoral head on the other side are also observed.

Perthes disease: symptoms

The functions of the hip joint, which includes the head of the femur damaged in Perthes disease, are manifold: it supports a large part of our body ensuring an upright posture, and its motor functions are essential in everyday life, because it enables proper walking and sitting .

The ailments accompanying Perthes' disease are associated withfunctional disturbance of the hip joint.

Perthes disease belongs to the group of diseases of the so-called painful children's hip. This symptom, like limping, should always be disturbing and quickly diagnosed.

A child suffering from this condition reports first of all

  • pain, usually after prolonged activity - running or jumping. Importantly, it is not directly related to the injury. The pain is usually located in the groin, i.e. in the place where the hip joint normally hurts, it can radiate to the thigh and knee
  • limping - moreover, limping is very often the first symptom, it occurs even before the pain begins and is usually reported not by the patient, but by the caregivers

Apart from these symptoms, the following is also noticeable:

  • restriction of mobility in the hip joint
  • slimming of the diseased leg over time due to its saving and muscle wasting

Perthes disease: diagnosis

It is not easy to make a diagnosis. Many diseases cause similar symptoms and it cannot be taken lightly. The differentiation takes into account, among others:

  • hemophilia
  • juvenile rheumatoid arthritis
  • hypothyroidism
  • lymphiaki
  • purulent arthritis
  • osteochondrosis

X-ray changes of the hip joint and correct images of other joints bring the diagnosis of Perthes disease closer. However, if other joints are also damaged, it is rather a systemic disease, i.e. rheumatoid arthritis.

Diagnostics should be carried out in a center experienced in the treatment of this disease, because it is a dangerous disease that can have severe consequences for the rest of your life.

Perthes disease: additional research

In the diagnosis of Perthes' disease, it is necessary to take an X-ray of the hip joint, ideally in two projections, it is also useful for monitoring the progress of the disease and the effectiveness of treatment. After diagnosis, photos are taken every few weeks. This allows you to properly classify the phase of the disease, and over time it can be performed less frequently, every few months.

Ultrasound of the hip joint is also an important diagnostic tool, it is important not so much in making a reliable diagnosis, but should be used for the initial assessment of this area in the event of pain in a child. On this basis, further diagnostics can be undertaken and initial differentiation can be started.

Moreover, in ultrasound of the hip joint, some changes can still be observedbefore they appear on the X-ray - it is extremely important, because it enables early treatment implementation.

Less frequently used tests are computed tomography and magnetic resonance imaging. They facilitate the accurate assessment of the femoral head and the acetabulum and the adjustment of the treatment to their shape. Scintigraphy is sometimes used in the early phase of the disease.

An invasive examination, performed only exceptionally due to the availability of tomography, is arthrography, it allows obtaining similar information, and its conduct is not necessarily associated with the risk of complications.

Perthes disease: phases of the disease

For the proper growth and development of all joints, proper articular surfaces of all bones that make up the joint are essential, because they influence each other and stimulate the formation of an optimal shape for movement.

If any surface takes a different form, it causes a change in the structure of the entire joint - the original damaged surface is distorted, which in turn exerts irregular pressure on the opposite surface, which causes it to grow asymmetrically. As a result, the normal function of the joint is disturbed.

Based on the radiological image, Perthes disease was divided into several stages, i.e. stages:

1. necrosis phase - characterized by a reduction in the femoral head and a widening of the hip joint fissure

2. reconstruction phase - new bone tissue is created within the old bone tissue, which causes fragmentation of the femoral head

3. repair phase - in this phase changes in the shape of the head and neck of the femur are observed

4. healing phase - the disease process has stopped, permanent changes are visible, deformation of the femoral head, e.g. its enlargement

The effects of Perthes disease in the fourth phase of the disease arise through several mechanisms: bone growth is disturbed due to primary ischemia, subsequent inflammatory processes cause tissue dislocation and irregular bone growth due to the previously described mechanism.

In addition, the acetabulum distorts the weakened cartilage of the femur depending on applied pressure and load. All these processes significantly distort the femur, which results in deep walking disorders, including disability.

The radiological image, as well as determining the phase of the disease, is used to classify it, it is important because it enables the determination of the extent of the changes, prognosis of disease progression and, above all, the choice of the treatment method.

For this purpose it is usedseveral classifications: Catterall, S alter, Thompson and Herring. In addition to assigning disease activity to one of the classification groups, various other factors have prognostic value:

  • primarily the bone age at which the disease appeared - in younger children, under 8 years of age, the prognosis is better. Bone age is literally the age of a child's bones, it is determined on the basis of an X-ray of the wrist
  • deformation of the femoral head visible in the photo
  • growth disorders associated with damage to the growth cartilage
  • long duration of illness
  • sex, the prognosis for girls is worse

Perthes disease: treatment and prognosis

Pharmacological methods are ineffective because the exact mechanism of disease development is not known, so the disease cannot be treated causally.

The goal of the therapy is to reduce tissue tension and fluid pressure in the hip joint. Thanks to this, proper regeneration and reconstruction of the femoral head following the necrosis phase is possible, which in turn protects against its inappropriate shape and ensures the proper anatomical structure of the hip joint.

If this therapeutic goal is achieved, there is a very good chance of eliminating or minimizing gait disturbances, which in the case of disturbances in the structure of the femoral head would certainly occur.

Depending on the severity of the disease and its activity, various activities are undertaken - from restricting movements in this joint to surgical treatment.

There is no causal treatment, there is relief of the affected limb and surgery, which is necessary in most patients to ensure the proper function of the sick joint.

In the acute phase of the disease, it is recommended to relieve the limb, sometimes including lying under the hood, for up to 6 weeks or until the leg has a full range of motion and movement is not painful.

It is very important to properly position the limb on the lift to ensure optimal pressure of the synovial fluid, and therefore proper regeneration and growth.

Gypsum dressings (due to the patient's lack of comfort) or orthotics (due to the high cost) are much less often used for this purpose.

In the later phase of the disease - repair, surgery can be applied to obtain the correct position of the femoral head in the acetabulum, i.e. consistency.

There are many methods of surgery (eg S alter's osteotomy or Sanger's surgery). The author of one of them is a Polish orthopedist, prof. Dega.

As it is easy to guess the mentioned methods of treatmentare based, inter alia, on the previously described mechanism of joint development.

In cases where the course of the disease is very severe or the patient comes too late and the methods described do not allow for cure, corrective operations are performed, e.g. pelvic osteotomy, proximal femoral osteotomy.

They allow for the correction of the incorrect position of the leg in the hip joint. If the disease causes disturbance in the growth of the femur, limb lengthening methods are used.

Most patients, many years after the onset of the disease, have no pain symptoms and the mobility of the joint is normal. Unfortunately, in the case of flattening, irregularities of the femoral head, that is, in people who see a doctor too late, were improperly treated or had a very severe disease, there is pain when moving, as well as limitation, and eventually loss of joint function.

Category:

Anatomy