Prosthetic Fitting of the Congenital Quadrilateral Amputee: A Rehabilitation-Team Approach to Care
RICHARD A. SULLIVAN, M.D. FELICE CELIKYOL, O.T.R.
This article describes the effect of a full, comprehensive, rehabilitation-team approach to the problem of the quadrilateral congenital amputee. A specific case is presented with discussion of the prosthetic, physical, and social problems that were faced during the rehabilitation process. The patient has progressed to ambulation with four prostheses and crutches.
One of the most challenging problems which the physician, therapist, and prosthetist face in any children's congenital-amputee clinic is that of the quadrilateral amputee. It is obviously a problem which varies from case to case depending on the extent of the amputations of the individual limbs. The presence or absence of one or more of the key joints determines the functional result which will be achieved. As incongruous as it may seem, it is often the case in the lower-limb problem that a complete lack of a limb can be more easily handled prosthetically than some of the apparently less disabling combinations of amputations, hip-joint deficiencies, and added distal deformity problems. The fact that so many of these multiple problems are also symmetrical is also of aid when one is planning the prosthetic solution.
At the Kessler Institute for Rehabilitation these multiple-congenital-amputee problems are evaluated at a special Multilimb Amputee Clinic held every three months. This clinic involves, in addition to the usual members, both the upper-limb and lower-limb prosthetic specialists and the Social Service Department of the hospital. These clinics are held in addition to the weekly upper-limb and lower-limb amputee clinics to provide a more comprehensive approach to these cases.
We are presenting here a case of a congenital quadrilateral amputee with whom we have been able to work since the age of 3. In spite of upper-limb deficiencies bilaterally above-elbow and despite lack of both lower limbs, a young man has progressed to a high level of functional capacity in both the upper and lower limbs through consistent and innovative prosthetic management. We feel that it is a combination of a comprehensive rehabilitation-team approach, the introduction of devices at an early age, the intelligence and the desire of the patient to achieve, and the understanding and assistance of a supportive family which have combined to bring about such a good functional result.
The child, who is now age 6, was first examined in our children's amputee clinic at age 3 in April 1974. He was classified on admission as a transverse hemimelia (above elbow) bilateral, and a bilateral amelia of both lower limbs. X-rays revealed normal scapular and clavicular development bilaterally with the additional advantage of normal shoulder joints. The humerus on the left was complete, and although there was soft-tissue growth below the elbow suggesting some partial elbow formation, neither radius nor ulna was present. The right humerus was maldeveloped and foreshortened.
In the lower body there was maldevelopment of the pelvis with widening of the symphysis pubis and hypoplasia of the acetabulae. The entire pelvis was foreshortened and flared, and there were soft-tissue prominences present at both hips. There were no lower-limb bony parts present.
The patient ( Figure 1 ) is the fourth child of a Colombian family of five children and was born while the family lived in South America. He was the product of a full-term pregnancy with normal delivery and milestone development. There is no history of maternal illness or drug ingestion during pregnancy to account for the multiple amputations. The family immigrated to the United States in 1973 and sought aid from numerous centers and agencies before being referred to the Kessler Institute by one of our United States Senators. It appears that the financial needs for his care could not be solved at these first centers. Our Social Service Department was activated, and with effort the financial problems were finally solved, at first temporarily and later permanently. This situation again underlines the need for a team approach in handling complex medical-social cases such as this.
Since the child and his family were aliens, they did not qualify for governmental-agency assistance. Funds were received from a local women's club and were supplemented by the Kessler Institute's Patient Aid Funds. These funds covered the cost of the original prostheses and training sessions. The child is now eligible for state-agency sponsorship and is being sponsored by the Child Health Services Program of New Jersey after much Social Service effort.
Prostheses were prescribed at the April 1974 clinic and were received at the end of October. The prostheses consisted of a standard left elbow-disarticulation prosthesis and a right above-elbow prosthesis, both containing flexion wrist units and 12P hooks, connected with a bilateral harness.
Training was provided in the Occupational Therapy Department, with emphasis on terminal-device operation. Because elbow-lock control was poor at this time and was frustrating to the patient, this function was performed manually for him. Developmental toys were used in training; and self-feeding was stressed using a special scoop dish, a nonskid pad, and an adapted spoon (metal loop on handle). He progressed slowly because of language and transportation problems which affected his ability to communicate and his attendance at training sessions. Assistance was provided by the Hackensack Neighborhood Center, an outreach program on the Bergen County Committee Action Program.
Training continued sporadically, and at approximately 4 and 1/2 years of age he was able to control selective opening of the terminal devices, especially with the left upper limb, which was now his dominant limb. He had also learned elbow-lock control on that side. His control was only fair in elbow lock/unlock positions on the right. Hook prepositioning was emphasized at this time. The training program included self-feeding skills and preschool activities. He had now begun attending a nursery school, and close contact was maintained with the school to coordinate the training programs.
In the Spring of 1975 the patient was seen at the Kessler Institute's Quarterly Clinic for Amputee Children with multiple-limb involvement. The recommendation was made for a parapodium to be adapted with a bucket for support to allow standing. Further analysis, however, revealed so many extensive revisions of the parapodium would be required that such a device was not feasible. A special bucket-pedestal support was then fabricated ( Figure 2 and Figure 3 ) by our consulting prosthetist at his expense. It was innovative in design and filled the patient's need at that time. It was delivered in October 1975, and a standing table was provided for home use. The patient soon demonstrated good balance and control. He then progressed to maneuvering in this bucket on a level surface by using trunk rotation similar to the movement used to propel a parapodium. Ambulation of a limited form was therefore achieved ( Figure 4 )!
In addition, the pedestal was constructed with a modified hip joint which allowed both sitting and standing. This arrangement allowed the patient to wear the prosthesis continually throughout the day, both when standing and ambulating and when sitting in the wheelchair or at a desk.
A CAPP cart was considered for use in the nursery school but was found to be inappropriate for that setting. He was then given a standard wheelchair with the recommendation that a powered chair be considered at a later time, since it was still felt that ambulation with prostheses might not be an achievable goal.
In March 1976 he was enrolled in a local school containing a multiply handicapped program unit. This school provided occupational and physical therapy; and he was, therefore, able to receive prosthetic training regularly. In addition to improving upper-limb prosthetic use, he began wheelchair propulsion with use of the arm prostheses. Vertical logs were added to the handrims for improved control. The pedestal was of value to him in the wheelchair, since it helped provide more sitting stability when propelling the chair, thus increasing his speed of propulsion.
A decision was then needed regarding the feasibility of lower-limb prosthetic fitting and crutch ambulation. The child was presented as a problem case at the Clinic Chiefs' meeting in Miami in October 1976. No clear-cut decision was received, although the sense of the discussion was that a trial of ambulation was certainly indicated in view of his history to date. Therefore, bilateral leg prostheses were prescribed in March 1977 and were delivered in September. The prostheses consisted of bilateral Canadian hip-disarticulation prostheses with single-axis locking knees and SACH feet ( Figure 5 ). A manual knee lock was custom made and handtooled to adapt to the single-axis knee joint for this small child ( Figure 6 and Figure 7 ). Axillary crutches were prescribed for use in ambulation. He was able to progress quickly to an independent drag-to gait. Sling-top crutches were evaluated and provided him with better control and are now used full time ( Figure 8 ).
As a result of innovative early prosthetic fitting and comprehensive training, a quadrilateral amputee with profound disability progressed to a level of functional ambulation that was at first felt beyond his capacity, using standard upper-limb prostheses, lower-limb Canadian prostheses, and sling crutches. An initial fitting with a special bucket-pedestal support allowed independence in both sitting and standing and was found to provide limited ambulation through rotary trunk movements. This ability to "ambulate" provided the clue to the patient's future success with standard prostheses attached to all four limbs, allowing a functional gait performance and independence in upper-limb skills.
A combination of early fitting, innovative design, detailed training, an intelligent patient, and a supportive family produced an exceptional functional result. The socioeconomic problems were faced and gradually solved. Prosthetic training programs were integrated with the local community agencies and school district with excellent results. The case again stresses the need for a complete rehabilitation-team approach to the problems of the severely handicapped child, bringing in the expertise of the physician, prosthetist, physical and occupational therapists, social workers, local social agencies, state agencies, and school personnel. The availability of this entire team in the amputee clinic is vital to provide the comprehensive care such patients require and to provide access to community social agencies and educational facilities required to complete the rehabilitation process. This case underlines the need for such comprehensive care.