Diseases

Madelung Deformity – Causes, Symptoms & Treatment

Madelung deformity

A growth disorder in the forearm that leads to a misalignment of the arm and an abnormally long ulna is called a Madelung deformity . The bone formation of the patients is disturbed and causes a dysostosis, which becomes noticeable in adolescence, depending on the load. The deformity can be corrected surgically.

What is a Madelung deformity?

The Madelung deformity belongs to the group of congenital deformities. A growth disorder on the forearm, which typically leads to a significant misalignment of the arm, is characteristic of the clinical picture. The surgeon Otto Wilhelm Madelung first described the deformity in the 19th century. The surgeon is also the namesake of the Madelung hand deformity, which is clearly distinguishable from the Madelung deformity.

Before Madelung described the growth disorder with the resulting deformity of the forearm, six other physicians had already tried to describe it, such as Dupuytren . The deformity occurs in the context of various symptom complexes and clinical pictures. The idiopathic form of Madelung deformity is extremely rare. In addition to enchondromatosis, syndromes such as Ullrich-Turner syndrome and Léri Weill dyschondrosteosis are often associated with the deformity.

causes

The Madelung deformity does not appear to occur sporadically but appears to run in families. An autosomal dominant inheritance could be observed several times. The deformity is caused by dysostosis caused by disrupted enchondral bone formation. As the radius grows, the distal radial metaphysis is left on the ulnar and dorsal side, and therefore causes the radius to grow bent and shorter than the ulna.

In addition, in the idiopathic Madelung deformity, the ligament structure is abnormal due to a Vickers ligament between the proximal carpal and the distal end of the radius, causing a subluxation of the carpal row. The abnormal Vickers ligament consists of fibrotic and fibrocartilaginous structures. Patients with Léri Weill dyschondrosteosis regularly suffer from the Madelung deformity because they are affected by a deficit in SHOX proteins.

Symptoms, Ailments and Signs

The ulna of patients with a Madelung deformity protrudes dorsally at the wrist. Your spoke bends in a wide arc to the ulnar and volar and causes the so-called bayonet malposition with a subluxation at the wrist. The mobility of the wrist is restricted in a volar or radially tilted joint position. The same applies to the forearm rotation movement, which restricts the patient, especially in supination and pronation.

Dorsiflexion and abduction in the ulnar direction are also impeded, primarily by bony inhibition. Although the Madelung deformity is a congenital deformity, it is not apparent until adolescence and does not appear to be present in childhood. The first complaints of the patients are caused by the prominent ulna. Most of those affected initially complain of stress-related difficulties.

The Madelung deformity does not always cause symptoms. Some patients even remain asymptomatic for life. In the long term, however, the Madelung deformity can promote secondary diseases. The most common secondary disease is arthrosis , which is favored by the misalignment and the associated incorrect loading of the joints.

causes

The Madelung deformity is usually diagnosed through imaging. The first suspicion of the deformity overtakes the doctor as a rule in the anamnesis, whereupon X-rays are usually ordered. X-rays of the wrist, for example, show a severe imbalance on the distal surface of the radial joint. The joint appears tilted in the ulnar and volar direction.

Imaging also provides evidence of the overly long ulna of those affected. In most cases, the X-ray also shows a greatly enlarged gap between the radius and ulna, which often appears wedge-shaped. Sometimes the lunar bone slips into the gap and thus deforms in a similar wedge-shaped manner. All other carpal bones are subluxated. The prognosis for patients with Madelung deformity is relatively favorable.

therapy and treatment

When treating a Madelung deformity, improving the mobility of the joints is the most important goal. In most cases, growth must be completed before therapy can begin. If the bones stop growing, surgical intervention can take place. Surgical treatment is only indicated if the patient perceives the restricted movement as an impairment or feels pain when moving.

The surgical intervention used to be a shortening of the ulna or a Suave-Kapangi-Lowenstein operation. However, modern medicine uses corrective osteotomies on the distal radius, since this area of ​​the radius is the causative deformity. The Vickers ligament is cut as standard during the operation so that the patient can move the wrist without pain in the future.

During the operation, a metaphyseal arcuate corrective osteotomy is performed via an anterior approach, which enables a three-dimensional correction by tilting the distal spoke fragment. Steinmann pins inserted radially hold the correction. After the operation, the affected arm is immobilized with an upper arm cast for approximately two months.

The surgical correction of the deformity is carried out in specialist pediatric hand surgery centers. If the correction takes place before the growth phase is complete, recurrences usually occur as the growth progresses. The younger the patient, the more likely it is that the symptoms will recur. Due to the risk of arthrosis, correction of the deformity can also be useful in patients who have not had any symptoms up to now.

Over-the-counter medication for arm pain

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prevention

The Madelung deformity cannot be prevented, since in most cases it is an autosomal dominant inherited deformity. Complications such as arthrosis can be prevented by timely correction.

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Hello! I am Lisa Newlon, and I am a medical writer and researcher with over 10 years of experience in the healthcare industry. I have a Master’s degree in Medicine, and my deep understanding of medical terminology, practices, and procedures has made me a trusted source of information in the medical world.