One-Bone Forearm Procedure for Hajdu–Cheney Syndrome: A Case Report. (December 2013)

• Record Type: Journal Article
• Authors: Bachoura, Abdo; Jacoby, Sidney M.; Osterman, A. Lee
• Is Part Of: Hand. Volume 8:Number 4(2013)
• Page Start: 479
• Page End: 482
• Publication Date: 2013-12
• Subjects: Hand -- Surgery -- Periodicals; Hand -- Surgery; Periodicals; 617.57005
• DOI: 10.1007/s11552-013-9542-5
• Languages: English
• ISSNs: 1558-9447

Abstract

One-bone forearm surgery is generally regarded as one of the last available salvage procedures that could be used to treat patients with longitudinal forearm instability secondary to a congenital, oncologic, or a post-traumatic etiology. We performed this procedure on a 23-year-old patient with longitudinal forearm instability secondary to Hajdu–Cheney syndrome, which is a rare genetic disorder characterized by generalized ligamentous laxity, skeletal dysplasia, acro-osteolysis, and generalized osteoporosis. The patient developed shoulder pain secondary to overuse 28 months following treatment, and was managed conservatively. Eight years after surgery, the patient had not undergone any additional procedures, had no pain, reported a Quick Disabilities of the Arm Shoulder and Hand score of 21, and was completely satisfied with treatment. Although OBF procedure is a radical first-line salvage option, in unique circumstances and appropriate patient selection, it may provide acceptable, durable, and predictable results.