A patient with multiple hereditary osteochondromas may have any of
several severe deformities of the forearm, the most common of which are
ulnar deviation of the wrist associated with relative shortening of the
ulna, bowing of either or both of the bones of the forearm, shortening of
the forearm, and late dislocation of the radial head. The natural history
of these deformities is progression, with variable weakness, functional
impairment, and cosmetic deformity of the extremity. We describe the
results, after a follow-up of at least two years, in eighteen patients who
underwent major surgical procedures: excision of the osteochondromas (ten
patients), ulnar lengthening with excision of the osteochondromas (three
patients), and ulnar lengthening with radial hemiepiphyseal stapling (seven
forearms of five patients). Early excision of the osteochondromas alone did
not slow the progression of the deformity. Ulnar lengthening did, on
occasion, correct the ulnar drift at the wrist, but the relative shortening
of the ulna recurred. Distal radial hemiepiphyseal stapling should
accompany ulnar lengthening if radiocarpal angulation or subluxation of the
lunate occurs with ulnar shortening. Deformities of the forearm should be
treated early and aggressively to prevent disability.