Deformities and problems of the forearm in children with multiple hereditary osteochondromata

Multiple hereditary osteochondromata is the most common benign bone tumor. Deformities of the forearm are the most frequent cause of functional impairment in these patients. The most common deformity is a combination of relative shortening of the ulna, bowing of the radius and/or ulna, increased ulnar tilt of the distal radial epiphysis, ulnar deviation of the hand, progressive ulnarward translocation of the carpus, and subluxation/dislocation of the proximal radial head. In addition to relative shortening and cosmetic displeasure, these deformities commonly produce limited forearm rotation, limited wrist motion, and, when the radial head dislocates, both elbow pain and loss of motion. This article reviews all surgical procedures currently in use, and attempts to synopsize the current thinking about how best to treat these deformities. Preventing and reducing progression of deformity and functional impairment, particularly radial head dislocation, remain paramount goals, and aggressive management is advocated.