General Principles Of Surgical Treatment Of The Congenitally Deformed Hand
Ernest M. Burgess, M.D. Alfred I. Blue, M.D.
The treatment of severe congenital deformities of the upper extremity frequently involves consideration of reconstructive surgery of the hand, particularly in children. Sound principles of hand surgery must be followed, and growth and the child's adaptability should also be taken into account. The presence of a congenital hand deformity is, in itself, neither an invitation to, nor an indication for surgery. Function is of paramount importance. The surgeon should be constantly alert for the possible functional loss that might be incurred in an attempt to improve appearance. For this reason, we do not believe it possible to departmentalize or specifically catalogue the surgical treatment of these deformities in children. Each hand requires thorough individual evaluation. Nevertheless, it is easier to discuss the surgical treatment of congenital hand deformities in children if they are placed in certain general categories.
Syndactylism is probably the most common congenital abnormality of the hand. A complete examination of the entire upper extremity is mandatory since associated abnormalities such as absence of the pectoralis major muscle, Apert's syndrome, and other conditions occur in association with syndactylism. When surgery is to be undertaken, it is our usual practice to attempt to repair the hand prior to the time the child enters school. We do not believe syndactylism should be treated as an emergency or semi-emergency procedure in the very young child.
One exception may be that condition in which the terminal phalanges are fused, resulting in a progressive deformity because of the different rates of growth in the fingers. In this circumstance, the finger tips may be divided initially, and the rest of the fingers operated upon at a later date. This surgical timing and approach will bring about a more satisfactory result than complete division at a very young age. The minor correction thus obtained is not dependent on nerve supply since the digital nerves can be separated into the adjacent fingers; rather, the correction is dependent upon the blood supply to the fingers.
The treatment of syndactylism is illustrated by two cases - one repaired by surgery and the other not repaired.
L. M. (Figures 1 and 2) has multiple congenital abnormalities involving both the upper and lower extremities. At the time of the picture shown in Fig. 1 , an operation had been performed on the right upper extremity and an attempt was made to provide a deeper web for the thumb.
In Fig. 2 , the child, now one year older, is wearing a left upper arm prosthesis and a left lower extremity prosthesis. Her function with her arm prosthesis was excellent and, in fact, was regarded as superior to that of her other hand. The possibility of a Krukenberg type of procedure is now being considered for L.M.
M.G. (Fig. 3 ) has multiple congenital abnormalities. A syndactyly between the thumb and what is thought to be an index finger can be noted in the photograph. This has not been corrected as yet because she is functioning satisfactorily with the thumb and index fingers acting as one digit. We are concerned that correction of the syndactyly might impair the use of the hand. However, consideration is being given to the possibility of reducing the cleavage between the index and middle fingers and providing a deeper web for the thumb. This will probably be done at a later date.
If syndactylism is the most common congenital abnormality of the hand, polydactylism certainly is second. If doubt exists as to what portion of the finger or which member should be removed, the surgeon should wait until function of the hand can be fully ascertained. We have seen surgery performed for cosmetic reasons before function had been thoroughly evaluated, and the wrong digit removed, as in the case of a bifid thumb. When the supernumerary digit is removed, surgery should not interfere with the function of the remaining parts of the hand.
Fig. 4 illustrates some considerations in the treatment of a Polydactyly. A supernumerary digit on the left hand had been excised at an earlier age but reoperation is believed necessary. The initial operation did not produce meaningful results and we now feel that it should have been deferred until the child was older, at which time a definitive procedure could have been planned.
The ulnar deviation of each thumb was due to a triangular shaped extra phalanx which was excised to avoid an increasing deformity.
Radial Club Hand
This anomaly is always due to a defect of the distal radius or a total radial defect. To our knowledge, no cases have been reported in which the defect was in the central portion of the radius alone. The anomaly may occur in varying degrees from loss of portions of the thumb to the usually associated clinical syndrome of complete radial club hand. We reposition the hand as early as possible, the time being gauged by the physiological age of the child. A critical evaluation of the final result of surgery in radial club hand may reveal that very little actual functional improvement has been obtained. The surgery, then, despite glowing reports of improvement, has been in essence a cosmetic procedure. In a number of instances, improvement is actually attributable to the growth of the patient, rather than to the surgery.
Surgery should consist of replacement of the hand upon the ulna at an early age in an attempt to secure a pseudoarthrosis. Growth and stress changes can alter the distal ulna greatly to provide stability, such as one sees when the fibula is replaced into the tibial position for congenital absence of the tibia. The actual surgical technique has been carefully detailed by Starr1 and Riordan2 and it is not necessary to repeat it here.
Pollex Varus or the thumb flexed hand is a rare anomaly, thought to be inherited. The thumb is characteristically held up in the hand with marked flexion at the metacarpophalangeal joint. There is an associated contracture of the superficial tissues. Ordinarily, the extensor pollicus brevis is hypoplastic or absent. Our treatment for this anomaly consists of immobilizing the thumb out of the palm with splinting, if possible. If this is impossible, then a tendon transplant will have to be done to replace the function of the extensor pollicus brevis. The flexor surface will require skin correction which may be either a Z-plasty or a full thickness graft.
Congenital Trigger Thumb
Congenital trigger thumb, another and different entity, is easily treated when diagnosed, by a small incision parallel to the flexion crease. Care should be taken not to damage the digital nerves. The full restoration of function promptly results.
Ectrodactylism or cleft hand is best managed by observation over a prolonged period. There may be an associated syndactylism of the digits on the radial or ulnar side. After the hand has been thoroughly evaluated over a period of months, improvement may be obtained by surgically treating the syndactylism. Certainly, the hand should not be closed in an attempt to eliminate the "claw". This is sometimes done for the sake of cosmetic improvement, with unfortunate reduction of function.
S.A. (Fig. 5 ) has an ectrodactyly associated with a syndactyly. The syndactyly repair, visible between the little and ring fingers, was not undertaken until the patient was 16 years of age. While deferment of repair until this age is considered unnecessary, this case indicates that a procedure of this type can be performed at a late date and a satisfactory cosmetic and functional result still achieved.
Megalodactylism is an extremely difficult problem to handle. We have attempted to treat this complicated anomaly by multiple excision of tissues. When the digit or digits have reached the size of anticipated full growth or normal, we have attempted epiphysiodesis with some success. We have no recommended treatment for brachydactylism at the present time other than observation of the patient. After thorough evaluation, function may be improved by amputation of the part to provide a better-fitting prosthesis. Fortunately, this condition is relatively rare.
We have not utilized the Krukenburg operation at our clinic and have no personal experience with it. Observation of Krukenburg procedures performed by others would indicate that it has a real but limited field of usefulness in the bilateral amputee, especially one who is blind or mentally deficient.
The basic tenet to governing the surgical treatment of the congenital deformed hand in children is the fact that function, not form, should govern operative intervention. With this fact in mind plus an awareness of the great adaptability of children and the potentialities of growth, one can approach these complicated problems with confidence and gratification in the surgical improvement which can be obtained with properly timed, careful reconstructive surgery.
The Amputation and Congenital Deficiency Service, Children's Orthopedic Hospital and Medical Center, Seattle, Washington. Ernest Burgess is Clinic Chief.
1. Starr, D.E., "Congenital Absence of the Radius; Method of Surgical Correction", Journal of Bone and Joint Surgery, 27: 572-77, October, 1945.
2. Riordan, Daniel C., "Congenital Absence of the Radius", Journal of Bone and Joint Surgery, 37-A, 1129-40, December, 1955.