Treatment At Abteilung 10--Dysmelien: A Preliminary Letter Report

Professor Dr. G. Hauberg


Ward 10-Deformed Limbs

The chief malformations presented at our clinic from 1960 to 1962 were ectro-melias, phocomelias, and amelias; plus some peromelias. These malformations were the result of the thalidomide tragedy, which left 3,000 deformed babies in West Germany. To accommodate our large case load and to meet the urgent need for comprehensive rehabilitation facilities, we converted a former male tuberculosis ward into a special ward for children with deformed limbs: Abteilung 10--Dysmelien.

Our basic premise was that such children should not be compelled to spend their lives in institutions but should be taught to function as soon as possible, so that they could take their place in society. We also believed that artificial limb fitting should not be postponed until after the period of rapid growth, but that the child should grow up with his prosthesis, learning to experience it as part of himself. On the other hand, we avoided operations to remove seemingly functionless protrusions of tissue as long as possible, since most of these protrusions contain muscles that may be developed to provide invaluable assistance in manipulating artificial limbs.

The thalidomide babies were admitted in batches of thirty at a time and lived in the clinic for from two to three months. During the last two weeks of a child's stay, the mother also became a clinic resident and wore the same style blue coat as did the staff nurses and therapists. The child thus learned to identify his mother with the clinic staff, while simultaneously receiving from her what we call nest warmth. The mothers also attended group psychotherapy sessions, to help lessen their feelings of guilt and anxiety.

Prosthesis for Amélie Child

For children between one and a half and four years old with amelia, we use the Heidelberg externally powered prosthesis. The arm is suspended from the trunk by a metal frame covered with a porous textile, which we have found more satisfactory than the plastic shell (Fig. 1 , Fig. 2A/2B ). Among the advantages of the metal frame are good fixation, lightweight construction, reduced perspiration, easier fitting, exact positioning of the valves, and closer contact between the shoulder joint and the body. Special joints have been constructed, and the prosthesis is riveted to the frame.

For a nine-year-old child with bi-.lateral amelia we constructed the following prostheses: The arms are suspended from the metal frame, which is not covered with textile material. Two small joints in the back link the two halves of the frame and prevent them from twisting (Fig. 3A/3B ). The weight of the prosthesis is borne comfortably, without any pressure being exerted on the sensitive areas of the cervical column. The scapula and the clavicle are left free so that they may be used for valve control.

External power is used to provide five functions: grip, pronation and supination, elbow flexion, and upper-arm rotation. The ball shoulder joint has proved to be very satisfactory: it is small and lightweight, moves smoothly, needs little maintenance, and can be used with either a metal frame or a plastic shell (Fig. 4A Fig. 4B Fig. 4C Fig. 4D ).

We are convinced that when the child with congenital amelia has sound legs, he has at his command a complete functional system, and therefore we should be careful not to overemphasize the importance of the prosthesis. The children at our clinic do not wear their artificial arms all day long and are permitted to use their feet, chin, shoulders, and mouth to perform a variety of activities- -usually more effectively than they do with their prosthesis. Considerable research still remains to be done before we will have available an upper-extremity prosthesis that can compete functionally with the feet.

For unilateral upper amelia we provide a conventional (body-powered) prosthesis; or a metal frame may be used (Fig. 5A Fig 5B ). However, the frame must be supported in front of the shoulder to counteract the pull of the hook. The elbow lock can be operated by shoulder elevation, but the prosthesis must be made somewhat heavier than the force required to lock.

Self-Portable Arm for Phocomelia

For patients with short phocomelias we use the so-called "self-portable arm on demand," developed at Edinburgh University (Fig. 6A/6B Fig. 6C ). The weight of the prosthesis is transmitted by a spring to the trunk, freeing the arm from the pull of gravity. Thus the malformed arm, often lacking a shoulder joint, can easily bring the prosthesis to the desired position. The spring power can be regulated according to the weight of the forearm, clothing, etc. "On demand" means that the prosthesis can be called upon to perform functions beyond the scope of the phocomelic hand. The valves controlling the prosthesis are activated by the fingers.

The Functional Hand for Children

In an effort to combine the functional value of a hook with the cosmetic advantages of a hand, we put the hook into the hand (Fig. 7A/7B Fig. 7C/7D Fig. 7E ). As shown in the illustration, the thumb and the ring and small fingers are cosmetic appendages only. The two tines of the hook are contained within the other two fingers. The mechanical design is simple, and the spring power can be easily regulated. We are now testing this hand and hope to start producing it commercially during the coming year.

Professor Dr. G. Hauberg is the Chief at Orthopedic Clinic "Annastift" and Helmut John, Research Division Hannover-Kleefeld, Germany