The deformity varies in degree from a slight protrusion of the lower end of the ulna, to complete dislocation of the inferior radio-ulnar joint with marked ulnar deviation of the hand.
At this age, the relatively slower growth of the ulnar and palmar part of the radius, leads to an increasingly progressive deformity.
[citation needed] Leri-Weill dyschondrosteosis is a pseudoautosomal dominant disorder which occurs more frequently in females and is due to a mutation, deletion or duplication of the SHOX gene.
The SHOX gene plays a particularly important role in the growth and maturation of bones in the arms and legs.
It occurs predominantly in adolescent females, who present with pain, decreased range of motion, and deformity.
This is an abnormal ligament formed between the lunate bone of the wrist and the radius and is found in 91% of cases of Madelung's deformity.
[citation needed] First options for treatment are conservative, using hot or cold packs, rest and NSAID's at first.
[citation needed] Physiolysis Purpose of the treatment is the removal of the epiphysis that causes the abnormal growth of the wrist.
The approach is made passing the Flexor carpi radialis with detachment of the Pronator quadratus muscle from the radius.
The dome shape of the osteotomy allows adequate bony contact for stability and a subperiosteal void for rapid healing.
[3] Ulna reduction Adults with Madelung’s deformity may suffer from ulnar-sided wrist pain.
However, if patients have a positive ulnar variance and focal wrist pathology, it’s possible to treat with an isolated ulnar-shortening osteotomy.
An oblique segment is removed from the ulna, after which the distal radial-ulnar joint is freed, making sure structures stay attached to the styloid process.
A guide wire is then inserted in the medullary canal of the ulna, allowing centralization for a cannulated drill bit.