Radius Wedge Osteotomy in Madelung Deformity of Leri-Weill Syndrome

• Published in: Orthopedics & Traumatology Vol. 47; no. 4; pp. 1207 - 1212
• Main Authors: Kanaya, Fuminori, Toyohara, Issaku, Futenma, Chojo, Ibaraki, Kunio
• Format: Journal Article
• Language: English
• Published: West-Japanese Society of Orthopedics & Traumatology 1998
• Subjects: Leri-Weill syndrome; Madelung deformity; mesomelic dwarfism; opening wedge osteotomy; radius osteotomy
• ISSN: 0037-1033; 1349-4333
• DOI: 10.5035/nishiseisai.47.1207

Leri-Weill syndrome is a hereditary disorder transmitted as an autosomal dominant trait. Appearing in the form of mesomelic dwarfism, it consists of mild shortness of stature and Madelung deformity of the wrist. We here in report the results of surgical treatment on 3 patients (4 wrists). All cases were female affected bilaterally, and their ages at operation were 9, 25 and 31. 2 adult patients complained of pain and deformity in the right wrist, and a skeletally immature girl complained of deformity of the bilateral wrists. Dorsal dislocation of the distal ulna was observed in all patients. Radiograph revealed excessive ulnar and volar angulation of the distal articular surface of the radius in all patients. The mean radial inclination was 56.3° (45-60°), and volar tilt was 32.8° (20-43°). Radius opening wedge osteotomy and iliac bone graft were performed to correct radial inclination and volar tilt in 2 adults. Fixation was achieved with the A-O plate and tension band wiring in each. Wedge osteotomy (radial closing and ulnar opening) was performed in the bilateral wrists of the girl fixed with the tension band wiring. The follow-up period ranged from 1 to 4 years. The prominence of the ulnar head diminished and correction of the radius was preserved in 2 adults. The girl however showed progressive loss of correction in the bilateral wrists with growth.