Madelung and pseudo Madelung deformities-Pictorial essay

Abstract

Madelung deformity of the wrist is a rare disorder. This often presents with a short forearm. This entity is also known as mesomelic dwarfism of Leri and Weill. Female preponderance is noted and is autosomal dominant. This may be bilateral in 50-66% of patients. Clinical diagnosis is often suggested but radiological confirmation is essential. Pseudo Madelung deformity simulates Madelung deformity except in the former there is negative ulnar variance with the distal articular surface of the ulna articulating with the medial cortex of the distal radial metaphysis. It also includes ‘reverse Madelung deformity’. Three major causes of deformity exist: 1. Idiopathic, 2. Hereditary, 3. Acquired. Radiologically, shortening and bowing of radius is noted with dysgenesis of the distal radial epiphysis. Subluxation of the distal ulna is noted as well as dislocation at the elbow. Surgical correction is the only treatment with genetic consultation to the parents.

Keywords

• Madelung deformity
• short radius
• dislocation of the distal ulna
• proximal radius
• Leri weill
• dyschondrosteosis