LUNATOMALACIA ASSOCIATED WITH CONGENITAL SHORTENING OF THE ULNA IN LANGER-GIEDION-SYNDROME - A CASE-REPORT

A 22-year-old patient with type I trichorinophalangeal Langer-Giedion syndrome presented a stage 2 osteonecrosis of the lunate associated wi th congenital shortening of the ulna and carpal tunnel syndrome. The p atient was treated by shortening osteotomy of the radius and median ne rve neurolysis, with an excellent result, This case provides an additi onal piece of evidence associating ulnar-minus variance with lunatomal acia, and another argument in favor of the theory that Kienbock's dise ase results from microfractures sustained by the lunate under an abnor mal stress distribution situation.