Surgical treatment of type 0 radial longitudinal deficiency

The purpose of this study was 2-fold: (1) to describe the surgical anatomy associated with type 0 radial longitudinal deficiency (radially deviated hand in the presence of a normal-length radius) and (2) to report the results of a surgical procedure designed to improve the alignment of the hand and forearm.

Since 1986 there have been 6 cases of type 0 radial longitudinal deficiency in 5 children seen at the St. Louis Shriner's Hospital. These children were treated with a surgical procedure to release the radial soft tissues and correct the alignment with tendon transfers. Age at surgery ranged from 12 to 40 months, with an average age of 21 months. The average follow-up period was 21 months. The tight radial wrist extensors were detached at their distal insertion and the tight radial wrist capsule was released dorsally and volarly, thus relieving the radial tether. The extensor carpi ulnaris (ECU) tendon also was detached just proximal to its insertion and sutured to the dorsal wrist capsule to augment wrist extension. The radial wrist extensor tendon was reattached to the ECU tendon at its insertion, converting it into an ulnar deviator.

In each case the radial wrist extensor was noted to be hypoplastic and the radial wrist capsule was noted to be tight, tethering the hand in radial deviation. In 4 cases there was only a single radial wrist extensor tendon. Average radial deviation at rest improved from 58 degrees to 12 degrees . Improvement in radial deviation at rest averaged 47 degrees , ranging from 25 degrees to 75 degrees . Improvement in active wrist extension averaged 53 degrees and improvement in passive wrist extension averaged 28 degrees .

For children with type 0 radial longitudinal deficiency a soft-tissue procedure that releases the radial tether and augments ulnar deviation and wrist extension by tendon transfer satisfactorily improves wrist alignment with minimal morbidity.