Clumphand refers to a malformation of the forearm that is congenital. A distinction can be made between a so-called radial and an ulnar form, respectively.
What is a club hand?
A club hand is a flexion contracture of the hand, whereby a differentiation can be made between a radial and an ulnar form. The base joints of the fingers also very often show a flexion contracture, whereas the end and middle joints tend more toward extension contractures. In most cases, the hand also retards growth.
Causes
A radial club hand usually occurs spontaneously and is not normally hereditary, but is considered an independent deformity. In this case, the deformity is caused by a missing or shortened radius. As a result, the hand deviates from the normal position, with the radius being affected more frequently. Malformations of the ulna and radius are often genetically determined, whereby the so-called TAR syndrome, VATER syndrome or Rothmund-Thomson syndrome, among others, can cause a radial club hand. Causes of an ulnar club hand include Cornelia de Lange syndrome or femoral-fibula-ulna syndrome.
Symptoms, complaints, and signs
A typical feature of a club hand is the shortened forearm, and the hand also deviates at right angles, radially. As a result, the function of the hand is very limited and grasping can be performed only by pinching things between the forearm and the hand. Most often, there are also anomalies of the carpus or hypoplasia of the long fingers and thumb. Furthermore, a distinction can be made between a primary and a secondary club hand. A primary club hand is a hereditary condition in which certain muscle groups are shortened or poorly differentiated. The bones develop normally. In the radial form, the radial forearm muscles or the biceps are predominantly affected; in the ulnar form, changes occur in the forearm muscles and the triceps. The secondary club hand is due to a congenital ulnar or radial defect. The hand deviates to the side and the deformity coarsens as growth progresses. In addition, deformities of the adjacent joints or fingers may also occur. In an ulnar club hand, the palm faces the body and the wrist and elbow are unstable. There are also pronounced finger defects and accompanying malformations affecting the muscles and skeletal system. In a radial club hand, the back of the hand faces the body, the wrist is luxated, but the elbow is stable. Affected individuals suffer predominantly from thumb defects, whereas the ulnar fingers are formed normally. Concomitant malformations in this case mainly involve the gastrointestinal as well as the cardiorespiratory system.
Diagnosis and course of the disease
A club hand is very noticeable because the arm is shortened and the forearm is bent outward or inward. There are different individual manifestations of this. For example, there is a possibility that the elbow is completely stiff and unstable; in addition, the ulnar fingers may be missing, and the remaining fingers may have what is known as camptodactyly (flexion contracture) or syndactyly (malformation of the phalanges). On X-ray, the malformations of the elbow joint, wrist joint, and on the hand are very visible.
Complications
Club hand usually results in deformity of the lower part of the patient’s arm. This can result in severe limitations in the patient’s daily life. Child development is also often disturbed by this malformation. Likewise, ordinary grasping or lifting can no longer be performed easily. Most patients can only pick up objects by pinching them between the hand and the forearm. It is not uncommon for muscles to be weak as well, and the affected person’s ability to bear weight decreases. It may also no longer be possible to perform certain sporting activities. It is not uncommon for fingers or joints to be affected by the deformities. Children can also suffer from psychological complaints or depression due to the club hand if teasing or bullying occurs. Klumphand can be treated relatively well, with no particular complications.With the help of therapies or surgical interventions, the complaints can be resolved. Further complications do not occur. Life expectancy is also not reduced by a club hand.
When should you see a doctor?
A club hand is usually detected immediately after birth and treated surgically. A mild deformity does not necessarily need to be corrected, but should still be examined by a doctor. The affected child often requires physical therapy as well as orthopedic aids, which must be organized in collaboration with a specialist. Severe deformities must be treated surgically in any case. Parents of affected children should arrange for surgery at an early stage so that the club hand can be corrected before the child grows up and the deformity possibly becomes more severe. If the club hand only develops during the first years of life, medical advice must also be sought. Malformations of the wrist, hand and elbow joints are a reason for a visit to the doctor. Furthermore, a club hand should be taken to the doctor if it occurs in the context of an already diagnosed disease such as TAR syndrome or VATER syndrome. Depending on the cause and severity of the club hand, parents may consult a general practitioner, an orthopedist, or an internist. Hereditary diseases should be examined and treated in a specialized clinic for hereditary diseases.
Treatment and therapy
Treatment of a club hand is either surgical or conservative. Conservative treatment is usually indicated for milder forms, and in this form of therapy, the goal is to counteract the contracture and compensate for the shortening of the arm. The arm is fixed in place by means of a splint or plaster cast. More severe deformities, however, can usually only be corrected by surgery. These corrections are carried out from the first year of life, whereby the fingers are separated or bony parts are removed during an operation. Today, the following surgical procedures are primarily used for this purpose:
- Radius hypoplasia: At the age of two to three years, this method involves lengthening the radius. It may be necessary to repeat the operation due to the growth of the child.
- Subtotal or total radiusplasia: Here, centralization of the ulna is performed at the age of two to three months, six months or in the third or fourth year of life.
Often in a clumphand also the thumb is missing or it is degenerated. Since a gripping function without the thumb is not possible, this must therefore be reconstructed. There are two possibilities for reconstruction: Either a finger is converted into a thumb or a toe is transplanted. The surgical procedure in which a thumb is reconstructed is called pollicization, and the index finger is usually used for this purpose. Operations on the elbow and forearm are normally not performed until puberty, so that the growth plates are not endangered. An exception is the surgical method according to Walther Blauth, which is already performed at pre-school age. In this case, the surgeon cuts through the soft tissues at the wrist and then bolts the ulna into the carpus. After the operation, the arm is immobilized for about four to six weeks, and constant monitoring by a specialist is also required until growth is complete. The therapy of infantile deformities of the hand often proves to be extremely lengthy and can take years. Due to the growth, new corrective interventions are often necessary. In the case of very severe deformities, usually only the functions can be improved, but not the cosmetic result, so psychological care is also part of the therapy concept.
Outlook and prognosis
Club hand is a congenital malformation of the skeletal system. This can be changed neither by the measures of self-help nor by the use of alternative or natural remedies. Relief of the symptoms can only be achieved with the possibilities of conventional medicine. Without the use of medical care, the impairments may increase in the course of life. The movement restrictions worsen, there is a threat of pain and a strong emotional burden. In a surgical intervention or through the use of conservative procedures, a change and optimization of the skeletal system is sought.The application of the methods used is associated with various risks and side effects. In addition, several interventions are often necessary within the child’s growth and development process. Complications and consequential damage can occur in the process. Under optimal conditions, doctors achieve good functionality of the arm, hand as well as fingers in a long-term treatment. Frequently, fingers are reconstructed and subsequently mobilized in targeted training sessions. Nevertheless, psychological irregularities are to be expected in many patients with the disease due to the visual abnormalities and restrictions in everyday life. These must be taken into account when making the overall prognosis, as they are often protracted and associated with a reduced quality of life.
Prevention
Because a club hand is a congenital malformation, it cannot be prevented.
Aftercare
The actual aftercare takes place until the end of puberty. After that, growth comes to a halt at the latest. Reduction of the condition of the club hand is then unlikely. The patient can live with a cosmetic improvement in everyday life, but mobility remains limited. Thus, the goal of follow-up care for adults cannot be to prevent a recurrence of the disease, as is usually the case. The hand treated in the growth phase has a rigid form. Affected persons must spend their daily lives with the corresponding restrictions in their private and professional lives. If mental suffering results from the immovable conditions, psychotherapy may be indicated. Children must be monitored after an initial intervention. This is because the club hand not infrequently brings with it the need for further surgery. Even afterwards, the growth phase can cause some changes in the affected hand. In order to achieve the desired result, quarterly or semi-annual check-ups with the treating physician are recommended. This is the only way to avoid complications and unforeseen developments. Orthoses or splints can be adapted to the changed conditions at short notice. X-rays, which clearly visualize the malformations, serve primarily as diagnostic instruments.
What you can do yourself
Mild forms of clubfoot can often be treated with conservative methods. For example, less pronounced deformities can be corrected by wearing a cast or splint. Accompanying this, the doctor will recommend that the patient rest the affected hand. If surgery is necessary, the affected hand must then be rested for at least four to six weeks. In addition, constant monitoring by the specialist is required. Only when the physician gives his or her consent may major physical work be performed again. In the case of children with a club hand, the parents should initiate surgery as early as possible, otherwise further complications may arise in the developmental process. If the club hand puts a lot of stress on the patient and may have already caused psychological problems, therapeutic treatment is indicated. Participation in a self-help group can give the affected person courage and thus make it easier to deal with the deformity. A club hand should always be monitored by a doctor. Even in milder forms, complications such as premature joint wear or circulatory disorders can arise, which must be clarified and treated if necessary. A physician should be consulted in the event of sudden onset of symptoms.
