Köhler’s disease I refers to a fairly rare, aseptic necrosis of the scaphoid bone. Köhler’s disease I occurs primarily in boys who are between three and eight years old. However, many physicians do not make the diagnosis until later in life; by then, in many cases, osteoarthritis has already developed in the tarsus.
What is Köhler’s disease I?
First of all, there is Köhler’s disease I as well as Köhler’s disease II; while Köhler’s disease I represents a necrosis of the scaphoid bone, a tarsal bone, the metatarsal bone (second, third or even fourth ray) dies in Köhler’s disease II. Characteristic of Köhler I disease is spontaneous death or spontaneous formation of necrosis. This means that neither an infection nor any other external influence is responsible for the formation of the necrosis. So far, it is unclear what causes the death. The disease becomes noticeable through pain in the foot, without anything being externally visible. The physician can make a diagnosis by means of X-rays.
Causes
The reasons why Köhler’s disease I develops have not yet been clarified. However, many experts have been discussing possible models of development for years or are of the opinion that they have found favoring factors that could cause Köhler I disease. It is striking that Köhler’s disease I always develops when the affected person has a so-called growth spurt. For this reason, physicians assume that the ossification of the skeleton sometimes has problems and cannot keep up with the growth. For this reason, bone instability develops in the affected region. Another theory is based on similar diseases, which are sometimes characterized by circulatory disorders. However, the fact that reduced blood flow only occurs at an advanced age or in adulthood speaks against this assumption, so that this factor is not present in the context of Köhler I disease. Furthermore, doctors are of the opinion that an overload of the bone can also be the decisive reason for Köhler’s disease. For which reason however exclusively children and preferably boys fall ill, could not be answered – in the context of that theory.
Symptoms, complaints, and signs
A first sign is pain. The affected person complains of sudden pain during exertion (such as walking or running). However, external injuries or changes in the foot cannot be detected. Furthermore, patients complain of pain when pressure is applied to the navicular bone. If the foot is not spared, swelling may occur in the region of the scaphoid, which also suggests that it may sometimes be Köhler I disease.
Diagnosis and course of the disease
If there is a suspicion that it is Köhler’s disease I, a diagnosis must be confirmed – in addition to the so-called tentative diagnosis. Confirmation of the diagnosis is made by means of radiography. The affected foot is examined from the side and from above. If Köhler’s disease I is indeed present, the physician can recognize a narrowing and at the same time a compression of the scaphoid. Sometimes it may also already be displaced, which gives a further indication that Köhler’s disease I is very likely to be present. Other examinations are not necessary. However, when confirming the diagnosis, the physician must make sure that he can definitely exclude Köhler’s disease II. Köhler I disease has a relatively good prognosis. Although the healing process is lengthy and takes several years, it is promising. The physician mainly opts for conservative therapy; surgery is only necessary in the rarest of cases. Damage that has occurred due to Köhler’s disease I usually heals – without late consequences.
Complications
Due to Köhler’s disease I, the patient primarily experiences very severe pain. This pain occurs mainly during exertion and can thus considerably restrict the daily life of the affected person. As a rule, patients suffer from pain primarily when walking or running, although this can not infrequently occur in the form of pain at rest. In this context, pain at rest at night can lead to sleep problems and possibly to depression. Likewise, no external injuries or other peculiarities can be detected.It is not uncommon for Köhler’s disease I to also lead to severe swelling in the affected regions. These can occur if the legs continue to be loaded even in the presence of pain. As a rule, the affected person’s quality of life is significantly restricted by this disease. The patient’s daily life is also restricted, so that physically strenuous activities or sports can no longer be performed. Especially in children, Köhler’s disease I can therefore lead to restrictions in development. Treatment of the disease does not lead to further complications. Above all, physiotherapy measures are necessary to alleviate the symptoms. However, a completely positive course of the disease cannot be guaranteed in every case.
When should one go to the doctor?
The risk group for Koehler’s disease I consists of boys between the ages of three and eight. Although often the correct diagnosis is usually not made until several years after the first signs of a health irregularity, a visit to the doctor should be made as soon as the initial symptoms appear. If children complain of pain in the bones or during locomotion, a doctor should be consulted to clarify the symptoms. There is a risk of confusion with natural growing pains. For this reason, it is particularly important to have a careful examination initiated as soon as a discrepancy is suspected in direct comparison with children of the same age. If the child appears to be particularly whiny or supposedly snivelling, a check-up visit to a doctor is necessary. If there are peculiarities in movement patterns or general motor skills, a doctor is needed. A decreased level of physical performance, premature exhaustion, or low resilience in the child should be investigated and clarified. If instability of the bones is noticed, limping occurs, or signs of circulatory disturbance present themselves, a doctor’s visit is required. A particularly pale skin in the affected region or cold feet may indicate a health impairment. If they persist unabated for a prolonged period, consultation with a physician is recommended.
Treatment and therapy
If the physician diagnoses Köhler’s disease I, he or she has various therapeutic options at his or her disposal. Predominantly, the physician opts for conservative treatment. The leg should therefore often be spared; orthotics can provide relief. Sometimes the leg can also be immobilized in a cast. Above all, physiotherapeutic treatments are important. This is because any tension, muscle hardening or other stiffening can be prevented. Massages or specific therapies dealing with relaxation and movement of the foot help to ensure that the course of the disease is positive and that no late effects are to be expected at the end of the healing process. With hyperbaric oxygen therapy, the physician can regulate the ambient pressure for the patient – using the hyperbaric chamber – and supply 100 percent oxygen. Records have shown that patients diagnosed with Köhler’s disease I while it was still in its early stages were especially able to benefit from the treatment. Hyperbaric oxygen therapy is also often used as part of physiotherapeutic treatment and is one of the conservative treatment methods. Surgery is rarely necessary, but is required if conservative treatments do not bring the desired success or if Köhler’s disease I could only be diagnosed at a later stage. In this case, the physician attempts to stiffen the joint; this variant becomes necessary when advanced arthrosis has already developed, causing the patient enormous pain. However, there are also surgical measures that can be taken at the beginning of Koehler’s disease I. The physician decides on the surgical procedure. In this case, the physician decides to use the so-called Pridie drill. This means that the physician drills into the affected bone and thus initiates revascularization. This method is rarely performed and, if so, only in the early stages of Koehler I disease.
Outlook and prognosis
In most cases, Köhler I disease can be treated with promising results. Scientifically based treatments are available that allow complete resolution of the symptoms. However, the healing process can take a long time. The treatment spectrum ranges from months to years.It is recognized that early initiation of treatment provides the best results. On the other hand, anyone who forgoes treatment or does not attach importance to typical symptoms risks developing osteoarthritis in adulthood. This results in instabilities and movement restrictions that place a heavy burden on everyday life. The disease is mostly diagnosed in male children. The body is still growing at this age. Relief of the metatarsal and other conservative procedures are usually already sufficient to achieve complete healing. The body has tremendous self-healing power at this time, making surgery unnecessary. This results in a favorable prognosis for Köhler’s disease I. If complaints remain in rare cases, they can be virtually eliminated by means of remedies. Affected persons do not have to suffer any significant restrictions in their quality of life. There is no shortening of life span due to Köhler I disease.
Prevention
Preventive measures, since no causes have yet been found for why Köhler I disease develops, are not known.
Follow-up care
In many cases, the measures and options for direct aftercare for Köhler I disease are significantly limited. Sometimes it is not even available to affected individuals because of the severity of the disease. For this reason, patients should ideally see a doctor at a very early stage and also initiate treatment to prevent further symptoms and complications. It is also not possible for the disease to heal itself, so treatment by a doctor is always necessary. Due to the genetic origin of Koehler’s disease I, those affected should have a genetic examination and counseling if they wish to have children, in order to prevent the recurrence of the disease in their descendants. In most cases, patients with the disease rely on the measures of physical therapy and physiotherapy to alleviate symptoms. Many of the exercises from such therapies can be repeated in the patient’s own home, thus accelerating the treatment. Likewise, it is not uncommon that the support of one’s own family is very important in the daily life of the affected person. Depression and other psychological upsets can also be prevented. In this context, the disease Köhler I does not usually reduce the life expectancy of the patient.
What you can do yourself
With the disease, the affected person has little room for maneuver and possibilities of action for self-help. In everyday life, the physical limits should be perceived and respected. Especially during the period of the growth spurt, there may be a sudden decrease in physical performance, which should be taken into account. In case of pain and discomfort, the organism should be spared. Sufficient rest periods should be observed so that a regeneration of strength can take place. The footwear should be adapted to the size of the foot. They should be neither too small nor too large. High heels should not be worn during the child’s entire period of growth and development, as they can aggravate complaints. Sturdy footwear, the size of which is checked and adjusted at regular intervals, is important and necessary. The body weight of the affected person should be in the normal range it BMI. Overweight should be avoided, as it has a negative influence on the development of the bone structure and the joints. It can lead to postural deformities or discomfort, which cause an impairment of well-being. If there is pain at rest or a persistent experience of pain, a visit to the doctor should be made as medical attention is needed. Competitive sports or the practice of extreme sports should be refrained from. They represent an additional burden that has a negative effect on the course of the disease.