Congenital Absence Of The Radius And Digital Deformities: Club-Hand - (Paraxial Heklmelia Radialis)

J. Leonard Goldner, M.D.


Congenital absence of the radius or "club-hand" results from an embryological deficiency involving the somites of the upper extremity. The degree of deformity varies from partial absence of the radius with resulting radial deviation of the hand and moderate soft tissue contracture and little or no digital deformity to complete absence of the radius with a more severe hand deformity such as absence of the thumb and changes in adjacent digits associated with limited elbow and shoulder motion.

Associated Congenital Deformities

Numerous other associated congenital deformities have been mentioned in the literature and these include harelip, cleft palate, clubfoot, hydrocephalus, hernia, kyphosis, torticollis, rib deformities, scoliosis. Aplasia of the lung on the affected side and dextrocardia have also been noted. In our group, instances of severe congenital heart disease have been observed. Fanconi's syndrome has also been noted, particularly the hypoplastic anemia.

Corrective Measures

Riordan's publications in 1955 and 1965 emphasized that soft tissue release and placement of the hand toward or over the distal end of the ulna will diminish radial soft tissue contractures and maintain the hand in a straighter position in relation to the forearm. Use of a fibular bone graft with proximal epiphysis attached has not been successful in maintaining the hand in a corrected position. Evidence is against continued growth of the transferred epiphyseal plate. Stability afforded by the bone graft has not been superior to placement of the hand dis tally and maintenance of elongation of skin, tendons and ligaments by splinting and repeated surgical release.

Surgical Indications

Our experience during the past ten years indicates that the earlier the soft tissue release is done, the less difficult is the maintenance of correction and the less severe is the final deformity. The initial surgery can be done when the child is six months old provided there are no other physical reasons for deferring anesthesia. For various reasons, operations on children in this Center have usually been at age twelve to eighteen months.

Indications for surgical treatment include: (1) unilateral involvement with a reasonably good hand and satisfactory elbow motion. (2) Bilateral involvement with flexible elbows and functional hands suggests an early effort at placement of the dominant hand over the distal ulna. If the length of the extremity and degree of elbow motion is such that the hand can be brought to the mouth by the flexed elbow with the hand in the corrected position, then the other side should also be done. (3) The absence of other severe congenital abnormalities such as palate deformities, congenital heart defects and other problems. These conditions take precedence over surgery of the hand and may be the reason for considerable delay in doing the hand surgery. (4) Need for consideration of emotional conflicts. The closer the hand can be placed toward the normal position the less difficulty the child will have in the later years, not only from the physical but also from the psychological standpoint. (5) The indication for surgery of the digits depends on the degree and extent of the involvement and the potential for improved function and appearance.

Contraindications

Surgical treatment is contra-indicated in certain special instances: (1) Bilateral "club-hand" untreated in the adolescent, teen-age or adult patient. Exceptions do exist and bone surgery may occasionally allow placement of one hand followed by arthrodesis, but generally soft tissue releases in the older patient are unsuccessful. (2) Limited elbow motion requiring maintenance of wrist motion. If correction of the hand deviation necessitates soft tissue and bone surgery, it is likely that severe limitation of motion will occur at the wrist. An immobile elbow and a limited wrist in a fore-shortened extremity is an undesirable combination. If both elbows are limited in motion neither extremity should be operated upon. (3) Hand deformity of such severity that individual digits are not useful requires maintenance of wrist motion to utilize the hand as a single unit. (4) Unwillingness of the family to continue appropriate follow-up, which would include the early use of plaster (beginning shortly after the birth of the child), intermittent surgical construction, and use of plastic splinting.

Method of Treatment

(1) Application of plaster to forearm, hand and arm a few weeks following birth: The elbow is placed at right angles, the hand is held toward neutral position and the plaster is applied from the axilla over the elbow and down to the wrist, and allowed to set. The ulnar side of the wrist and hand are then cleared of plaster and the hand is padded so that it is held toward maximum correction while the last plaster splints and rolls are applied. The fingers are left free for function. The elbow must be bent or the cast will slip. The flexed elbow also allows the baby to bring the hand to the mouth. The plaster is changed at regular intervals varying from two to four weeks with the routine continued for several months.

(2) If correction is possible and if the hand can be placed over the distal ulna with plaster treatment, this method of holding correction is continued until the child is approximately twelve to eighteen months old. A plastic forearm splint in the form of a split socket is then constructed over a plaster mold. This has several obvious procedural advantages in providing a removable splint and allowing increased elbow motion.

(3) By the time the child is six to twelve months old, limitations of plaster correction will become obvious. Soft tissue release may be necessary and this is done when the child is about one year old. The specific operations have included a large single or double Z-plasty, fascial incision, tendon lengthening, intercarpal ligament release and arthrotomy of the radial-carpal joint. In all instances, a second incision has been made over the ulnar-carpal joint in order to remove from the distal ulna the plicated capsule which blocks placement of the hand over the ulna. Kirschner wire fixation has been done to maintain the hand in the corrected position and plaster treatment again follows for several additional months. Once maximum correction has been obtained, the plaster is replaced with a plastic socket.

(4) Second stage surgical correction may take the form of osteotomy of the ulna, partial carpalectomy, or ulnar-carpal fusion, but the latter is to be avoided until maximum growth has been obtained, which may be age twelve to fourteen. The necessity of carpal or ulnar surgery usually depends on the age of the child, with the indication for bone and/or joint surgery increasing with age.

Given the proper prerequisites, the projected goals should be attainable, including: (1) elongation of the forearm and hand as a single unit, (2) improved activity of the fingers, (3) increased hand and forearm strength, (4) cosmetic improvement, and (5) diminution of future emotional conflicts .

During the past ten years this pattern of treatment has been followed at this center and the results obtained imply that the procedures should be continued. Approximately twenty patients with congenital clubhand have been observed during that time. The ages have ranged from newborn to age fifty. All, of course, have not been operated upon, and the comparison of the treated to untreated regarding variations in activity of the patients and their appearance has provided a basis for our conclusions.

An Illustrative Case

The accompanying figures show a patient now under active treatment.

(Fig. 1A ), (Fig. 1B ), (Fig. 2A ), (Fig. 2B ), (Fig. 3A/3B )

Summary

Congenital club-hand should be treated immediately following birth and treatment should be vigorous. Surgery, if indicated, should be performed by the time the child is a year old. Maintenance of correction or improvement can be accomplished successfully by alternating plaster, necessary surgical treatment, and a removable plastic socket.

J. Leonard Goldner, M.D. is associated with the Duke Orthopedic Amputee Clinic Duke Medical Center Durham, North Carolina

References:
1. Kato, Katsuji: "Congenital Absence of Radius." J. Bone and Joint Surg., 6: 589-626, July, 1924. 
2. Starr, D. E,: "Congenital Absence of the Radius." A Method of Surgical Correction. J. Bone and Joint Surg., 27: 572-577, October, 1945. 
3. Riordan, D. C,: "Congenital Absence of the Radius." J. Bone and Joint Surg., 37-A, No. 6 - 1129-1139, 1955. 
4. Riordan, D. C: "Congenital Absence of the Radius," Inter-Clinic Information Bulletin, April 1965.