Radial longitudinal deficiency encompasses a wide spectrum of anomalies affecting the thumb side of the limb ranging from a small thumb to complete absence of the radius bone. When the radius is short or absent, the wrist will show deviation deformity. The forearm will be shorter than the other limb. The elbow flexion may or may not be limited. The thumb may be small or absent. The other fingers may not be functioning well. About 50% of the children have bilateral involvement.
Picture and X ray of a child with radial longitudinal deficiency of both hands with underdevelopment of the thumbs
The condition can be a part of a variety of syndromes. Some of the syndromes are:
Thrombocytopenia Absent Radius (TAR) Syndrome
Fanconi anemia
VACTERL - Vertebral anomalies, anal atresia, cardiovascular anomalies, tracheo-esophageal fistula, renal and radial/limb anomalies
Holt-Oram syndrome.
A wide variety of treatment options are available. But to choose the right one, a complete evaluation by the hand surgeon is required.
The condition may or may not be genetic. They are mostly sporadic.
The child needs complete evaluation as the condition is often associated with syndromes that affect the blood counts, heart and other systems like the gastrointestinal system/vertebrae/renal system.
Non-operative care starts early in the newborn period. Stretching exercises and splinting are advised after examining the child.
The choice of surgery depends on various clinical signs. The options available for correction of the deviated wrist are surgical release, balancing of the tendons, radialization (correction of the wrist deformity) and distraction lengthening. The surgery is done at 6-9 months of age.
Surgery for correction of the wrist deformity on the left side
Surgery for the thumb may or may not be needed. Surgery if needed, depends on the degree of thumb hypoplasia. Surgery for the thumb can be done at 18-24 months of age.
For mild to moderate degrees of underdevelopment of the thumb, surgeries to reconstruct the tendons and ligaments are done.
Pollicization is the procedure of choice in cases of an absent or floating thumb. The severely underdeveloped thumb is removed, and the index finger is surgically moved to the thumb position to create a new thumb.
The floating thumb on the left side was removed and Pollicization was done
The hand will be immobilized in an above elbow Plaster of Paris cast. The stitches are usually absorbable. The cast will be cut open at 2-3 weeks. The wrist will be then supported by a splint to keep it in the corrected position.
The child after correction of the left wrist deformity and pollicization for both the hands
