Surgical treatment of the thumb in the Rubinstein-Taybi syndrome.

In a review of 530 individuals with Rubinstein-Taybi syndrome, 182 (34%) were found to have thumbs in severe radial angulation ("hitch-hiker thumbs"), which prevented opposition and functional gripping strength. Surgery has been performed on 35 thumbs (from 20 patients), usually to correct a delta phalanx deformity. The preferred approach was a closing wedge osteotomy, with a Z-plasty on the concave side to straighten the thumb. In eight and possibly 11 of the 35 thumbs, the angulation deformity or stiffness recurred, emphasizing the importance of proper and careful surgery. We conclude that surgical correction of the deformity is best done before the age of two, so that the thumb is functional during the initial development of hand-eye coordination. Deviation persisting at the age of ten can be corrected by fusing the metacarpophalangeal joint.