title: Lunatomalacia associated with congenital shortening of the ulna in Langer-Giedion syndrome: a case report.
abstract: A 22-year-old patient with type I trichorinophalangeal Langer-Giedion syndrome presented a stage 2 osteonecrosis of the lunate associated with congenital shortening of the ulna and carpal tunnel syndrome. The patient was treated by shortening osteotomy of the radius and median nerve neurolysis, with an excellent result. This case provides an additional piece of evidence associating ulnar-minus variance with lunatomalacia, and another argument in favor of the theory that Kienböck's disease results from microfractures sustained by the lunate under an abnormal stress distribution situation.
authors.affiliation: Department of Orthopedics and Traumatology, University of Brussels Clinic, Erasme Hospital, Belgium.
authors.forename: F A
authors.initials: FA
authors.lastname: Schuind
authors.forename: D
authors.initials: D
authors.lastname: Schiedts
authors.forename: E
authors.initials: E
authors.lastname: Fumiere
authors.forename: J L
authors.initials: JL
authors.lastname: Pasteels
authors.forename: F
authors.initials: F
authors.lastname: Burny
mesh_terms: D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging*; D000328:Adult; D002349:Carpal Tunnel Syndrome / Q000209:etiology*; D006801:Humans; D015826:Langer-Giedion Syndrome / Q000151:congenital*; D012667:Lunate Bone; D008279:Magnetic Resonance Imaging; D008297:Male; D008475:Median Nerve / Q000601:surgery; D010007:Osteochondritis / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery; D010027:Osteotomy; D011859:Radiography; D011884:Radius / Q000601:surgery; D014457:Ulna / Q000002:abnormalities*