Madelung's deformity

The Madelung deformity is a growth disorder of the forearm, which is associated with a characteristic deformity. It was first described comprehensively in 1878 by the German surgeon Otto Wilhelm Madelung, after the Madelungsche Handdeformität and Madelungsche fat neck were named. However, there was also previously at least six descriptions of misalignment, including by Guillaume Dupuytren.

Pathogenesis

It is a dysostosis, which is caused by the abnormal flow of endochondral ossification. In the course of growth, there is a lag of the distal radius metaphysis, especially on the ulnar and dorsal side, which is why the spoke is growing bent after volar and ulnar. Characterized the ulna is longer than the radius.

In addition, we find in idiopathic Madelung deformity, abnormal rigid band structure (Vickers - band ) between the proximal and distal carpal spoke end, which is responsible for a subluxation of the proximal carpal row. This up to 5-7 mm thick strip was first described in 1989 by Vickers and Nielsen and is found in almost all idiopathic Madelung deformity. It arises in a mostly in the X-ray image clearly visible small pit on the volar - ulnar side of the spoke metaphysis and runs under the pronator quadratus to the lunate bone, where it inserted volar to the original approach of the radio lunar band, as well as the triangular fibrocartilage complex between Elle and wrist. The band consists of fibrotic, hyaline cartilage and fibrocartilaginous structures.

A Madelung 's deformity can occur secondary to a distal broken spoke, after a bone infection and in a multiple Enchondromatosis.

The idiopathic Madelung deformity is very rare and accounts for less than 2 % of all childhood Hand Deformities from. In two of three cases the deformity can be found on both forearms. Girls and women are affected about four times more frequently. Often there is a family history, often with an autosomal dominant inheritance pattern.

The Madelung deformity is found more frequently in girls with Turner syndrome (prevalence 7.5%).

In patients with a Dyschondrosteosis Léri Weill there is almost always a Madelung deformity, here the cause is a deficiency of the SHOX protein (short stature homeobox -containing gene)

Clinical features

The ulna protrudes at the dorsal side of the wrist and the spoke is bent weitbogig after volar and ulnar. This leads to so-called bayonet deformity of the hand with subluxation of the carpus.

The Madelung deformity the mobility of the tipped- after volar and radial wrist and the forearm rotational movement are restricted in supination and pronation. The dorsiflexion and abduction ulnar be hindered by bony inhibition.

The Madelung deformity occurs only during adolescence, it is not yet present in childhood ( although it is often referred to as congenital deformity ).

Symptoms occur only in late adolescence on, they are often given to the prominent Elle, and are mostly dependent on load. Partial results only the deformity without discomfort for diagnosis.

In radiographs of the wrist shows a strong inclination of the distal radial articular surface, which is tilted to ulnar and volar. The cubit is about long ( ulnar plus variant) The gap between the radius and ulna may be greatly enlarged and look wedge-shaped (English chevron carpus ) be such that the lunate is in slips and wedge-shaped deformed. The other carpal bones are subluxated volar side. Very rarely is there an inverse Madelung deformity in which the spoke is bent dorsally instead of volar subluxated and the proximal carpal row back of hand side.

Therapy

After completion of growth surgical treatment at a perceived as impeding movement restriction and pain should be considered. Previously shortening of the ulna were performed, or surgery by Suave Kapangi - Lowenstein with distal radioulnar arthrodesis and distal ulnar osteotomy, now done but essentially a corrective osteotomy of the distal radius as a place of the actual deformity. An ulnar shortening is only carried out at distinct excess length of the ulna, and only in conjunction with a corrective Radiusosteotomie.

In addition, has established itself as the standard to cut through even the Vickers - band, and presumably this is severing the crucial step to obtain a pain-free wrist while the Radiusosteotomie mainly corrects the deformity. In the technique by Harley, which was introduced in 2006, access is via a front ( anterior ) metaphyseal arcuate corrective osteotomy, creating a three-dimensional correction by tilting of the distal fragment is possible, along with a transection (release) of the volar ligaments. The osteotomy is with two radially introduced transcutaneous Steinmann pins held and immobilized the arm for six weeks in the arm plaster.

A Texas case series of 19 in the technology operated by Harley patients and 31 operated wrists pointed to the middle eleven years consistently good to excellent results. The remaining reduction in forearm rotational movement is compensated for by an increased rotation of the wrist itself, due to the presence of Gelenklaxizität and rarely poses a problem

The operations should be carried out in specialized children's hands surgical centers. Complications from a procedure are rare, it was carpal tunnel syndrome, and at a correction of an inverse Madelung deformity, compression of the ulnar nerve described. In addition, there may be a relapse, especially among young patients who underwent surgery.

Especially in children with a Léri Weill Dyschondrosteosis is recommended that an early epiphyseolysis after Langenskjöld with fatty tissue interposition carried out on the affected ulnar - volar side of the distal radius growth plate with transection of the Vickers belt.

The Madelung deformity avascular osteonecrosis of the wedge-shaped deformed lunate ( lunatomalacia ) was occasionally observed. In the long term, the Madelung deformity lead to osteoarthritis of the wrist, with no studies on the long-term course available.