The diseases that can occur even in childhood include not only disorders of the metabolism or other health impairments. The bone system can also be affected, so extensive treatment must be initiated. One of these bone diseases is Perthes disease.
What is Perthes disease?
A typical disease of the bone system is the damage known as Perthes disease. The disorders associated with Perthes disease are localized to a specific functional part of the thigh, the caput femoris. Perthes disease involves what is known as aseptic necrosis of the bone. Aseptic in this reference means a death of bone tissue not associated with infection. As in Perthes disease, bone necrosis occurs when bone cells are no longer functional and perish due to various causes. Usually, this process results from the cell walls being destroyed, as is the case in Perthes disease.
Causes
In finding the causes of Perthes disease, it is important to categorize the triggers for it, which lead to the death of bone cells. These are poorly understood in this context and, to some extent, are probably based on an undersupply of blood to the affected areas of the bone. The technical term for this process is ischemia. In Perthes disease, this is limited to the head area of the femur, which actually holds the femur in the hip joint. Like the entire bone, this zone is also supplied with blood. If this is not ensured, there is a breakdown of the cartilage cells and thus of the head of the femur. Unfortunately, at present it is not yet possible to determine the definite causes of the development of Perthes’ disease, so medical sciences still have to do research on it.
Symptoms, complaints and signs
Perthes disease is characterized by increasing joint and bone pain. The disease progresses in episodes, and the symptoms can be divided into four stages. In the first stage, affected children complain of pressing or throbbing pain in the knee and thighs. In the second stage, the affected leg can no longer be moved properly – the typical dragging when walking occurs. Parents often describe these complaints as “walking laziness.” In the third stage, the hip joint continues to degenerate and muscle atrophy occurs. This is accompanied by chronic pain that persists during periods of rest. Patients are already limping badly at this stage. This can lead to bullying and exclusion, which often results in psychological problems. Finally, the pain is so severe that the affected leg can no longer be moved. As a result, patients with Perthes disease adopt an incorrect posture, which can lead to joint wear and chronic pain, among other things. The symptoms of the bone disease develop progressively, becoming more severe and lasting longer. Typically, Perthes disease presents itself in early childhood. Externally, the disorder, aside from gait disturbances, is not apparent.
Diagnosis and course
Basically, the course of Perthes disease is characterized by a relapsing progression of necrotic processes and is therefore divided into four stages. Within these stages, children experience painful discomfort in the thigh to the knee and a slight dragging of the affected leg when walking. Children with Perthes disease are often “lazy to walk” and complain of pain in the groin. The range of motion of the hip joint increases in Perthes disease, and the muscle mass in this area decreases significantly. The diagnosis of Perthes disease involves a variety of imaging technologies in addition to the visual assessment of the child by the specialist and the visualization of the walking motor function. In addition to x-rays, computed tomography may be helpful in this regard to assess the destruction of the bone structure in Perthes disease.
Complications
As a result of Perthes disease, those affected suffer primarily from pain. These can occur thereby in different regions of the body and lead thereby to strong impairments in the everyday life of the concerning. As a rule, pain occurs in the knees and hips. This can also result in restricted mobility, causing patients to limp and hobble.Furthermore, it is not uncommon for Perthes disease to cause a difference in leg length, which results in gait disturbances. Children in particular may be bullied or teased because of the gait disturbances. If the pain also occurs at night, Perthes disease can lead to sleep problems or depressive moods. The daily life of the affected person is significantly restricted and the quality of life of the patient is considerably reduced by the disease. The treatment of this disease takes place with the help of physiotherapies and by taking painkillers. Furthermore, there is no reduction in the life expectancy of the affected person. In many cases, surgical intervention is also necessary so that a prosthesis can be inserted.
When should you go to the doctor?
Doctor visits are usually at the beginning of a longer series of consultations for Perthes disease. The problem is that the symptoms of this circulation-related growth disorder are initially confused with other diseases or joint weaknesses and misdiagnosed. In addition, the symptom presentation is not uniform among affected children. The individual symptoms of Perhes disease are difficult to assess, especially in the early stages. In the second stage of the disease, Perthes disease is usually correctly diagnosed. Since the increasing symptoms are a cause for concern in many cases, parents should consult an orthopedist as soon as their child experiences unilateral leg problems, relieving postures or expressions of pain. Whether the present complaints require treatment or whether the matter will grow out with time varies. Often, the affected joints only need to be spared. It often makes sense to get a second opinion. For severe joint pain, some physicians may want to prescribe pain medication. The question, however, is whether it is actually Perthes’ disease or just a temporary hip condition. If the diagnosis of Perthes disease is confirmed, there is no alternative to appropriate treatment with regular follow-up examinations because of the possible joint damage. Depending on the stage of the disease and the symptoms, the treating physician can decide between conservative or surgical therapy. The goal of therapy is to prevent destruction of the affected joint.
Treatment and therapy
Because Perthes disease is represented by individually divergent courses, therapy also varies. Depending on the age of the patient and the extent of the necrotic processes, drug and surgical procedures are used in a treatment in addition to conventional, conservative therapy. Conservative therapy in Perthes disease includes relief of the thigh and hip by special splinting, fitting of a plaster support bandage or a so-called Synder Sling or orthoses. Extension treatment and walking aids are also common. Medications prescribed for Perthes disease are analgesics and nonsteroidal anti-inflammatory drugs such as ibuprofen during the acute stages. Surgical procedures are useful for Perthes disease when the goal is to obtain an artificial femoral head. Several surgical procedures are implemented in practice, which are represented by straightening the neck of the femur, “pivoting” the roof of the socket, or artificially paralyzing parts of the thigh muscles with Botox.
Outlook and prognosis
As a rule, a scientifically justifiable prognosis cannot be made for Perthes disease. However, it depends strongly on the quality of the existing congruence or incongruence between the two joint surfaces after healing. In most cases, the physician can make a prognosis at the end of growth based on the shape of the femoral head. The more spherical the femoral head and the more congruent it is with the acetabulum, i.e. the more exact the fit between the two joint bodies, the better the prognosis. Aspherical congruence, in which the joint partners fit together, is associated with an increased risk of hip joint arthrosis (coxarthrosis). However, this usually only becomes apparent after the age of 50. In the case of incongruence, hip joint arthrosis usually develops more quickly. Severe deformity is also associated with further joint damage, increasing limitations in joint mobility and pain. Prognostically unfavorable is also a disease onset at a later age.Young children have a better regeneration potential compared to older children. Therefore, the prognosis for children with the disease before the age of 5 is very good in the majority of cases. If left untreated or treated too late, Perthes disease leads to irreversible (irreversible) hip deformity and, possibly, extensive joint wear at a young age.
Prevention
Unfortunately, no prevention of Perthes disease is currently possible. However, the following facts should say something about the prognosis of Perthes disease: the sooner treatment begins and the younger the children are, the better the prognosis, a deformation of the head of the femur can be reduced. In most cases, the chances of surgical reconstruction of the hip and thigh joint in particular are good. Due to the progressive course of Perthes disease, which cannot yet be stopped, implantation of a high-quality artificial joint is usually unavoidable to establish an appropriate quality of life.
Aftercare
Perthes disease requires comprehensive follow-up care. After treatment of the condition has been completed, children with the disease require physiotherapy care. During physiotherapy, the joints are mobilized to increase the range of motion in the long term. This is usually done using forearm support crutches. Follow-up care usually lasts for twelve weeks. In the first six weeks, for example, full relief of the femoral head takes place. In the second six weeks, partial weight-bearing is performed using the so-called 4-point gait. The detailed measures required and the period of time over which they must be carried out depend on the severity of the Perthes’ disease. The physiotherapist in charge draws up the aftercare plan together with the attending physician and the patient. Once Perthes’ disease has been cured and the ability to move has been optimized, comprehensive aftercare is no longer necessary. The child must undergo regular medical examinations. Typical measures such as X-rays or ultrasound are used here. In addition, testing and, if necessary, adjusting the child’s pain medication is part of the follow-up care. Parents of affected children should discuss follow-up care with the physician at an early stage so that therapy can be continued seamlessly after the operation.
What you can do yourself
Children who have Perthes disease need support in everyday life. Depending on the severity of the disease, those affected are dependent on walking aids, wheelchairs and other aids in order to be able to move around in everyday life without discomfort. As a rule, patients are not allowed to engage in sports. In particular, sports that place stress on the hips should be avoided. Despite these measures, several operations must be performed, often over a period of several years, as the disease progresses progressively. The repeated interventions as well as the condition itself often also place a psychological burden on those affected. This makes it all the more important to deal openly with the disease. Parents should inform the child early on about the symptoms and the manifestations of the condition and visit a specialist clinic together with the child or talk to an orthopedist. The necessary psychosocial support is provided within the framework of self-help groups, where everyday problems can be discussed and experiences exchanged. Self-help also includes adherence to medical guidelines after treatment. For example, prescribed painkillers must be taken as prescribed to minimize the risk of side effects and interactions.
