Salvage Value Of Elective Amputation
John C. Allen, M.D.
In our standard management of children's prosthetics problems, we are concerned with the problem of creating function through the use of prosthetic mechanisms of one type or another. Ingenious adaptations or variations from standard prosthetic equipment have been made to accommodate a multitude of anomalous limbs. Perhaps some of the most ingenious accommodations have been in the utilization of minimal remnants of an extremity to activate control switches, or to control joints in upper extremity fittings. Certainly a great many methods of carrying weight have been used in lower extremity problems. More recently the use of external power in developmental prosthetics is proving to be increasingly effective.
In children's prosthetics we have also used adaptive surgery to convert a congenital anomaly, which is extremely difficult to fit to an amputation-type stump which is more easily fitted, when reconstructive surgery is not feasible. In general, the more nearly conventional the stump that can be created, the more routinely available will be the subsequent prosthetic equipment. By and large, the prosthetists connected with the children's prosthetics program are appreciably more adaptable in their techniques and more capable of producing the unusual prosthesis to fit the unusual situation than are their colleagues dealing with older patients. Therefore, as these youngsters become adults and are no longer covered by specialized children's prosthetics services, the likelihood that they can continue to be fitted adequately is uncertain. With improvements in the spread of knowledge and technical ability, hopefully this problem may not be as acute as it has been in the past.
Another concept which needs to be kept in mind is the place of elective amputation as a salvage procedure. In this paper, four cases are presented to illustrate possibilities in the application of this concept.
N.B., a 16-year-old white female, was originally seen at Newington Hospital for Crippled Children at the age of 9 years 4 months with a diagnosis of lymphangioma of the right ankle and foot. This condition was quite apparent at birth, this youngster being the product of a normal full-term pregnancy. When the child was one year old, the medial portion of this tumor mass was removed. Healing occurred completely, but at the age of 2 years 6 months an attempt to remove surgically the lateral portion of this mass was unsuccessful. The operative area did not heal and drainage was constant from that time on. The patient incurred multiple infections between the toes. The right foot was thick and appreciably larger than the left. It would not tolerate the wearing of shoes or weight-bearing. At the age of 9 years 3 months, N.B. was again reviewed critically at the original hospital. Consideration was given to the possibilities for plastic reconstruction and, if necessary, elective amputation. She was admitted to Newington Hospital for Crippled Children without surgery one month later, for another opinion and for probable management.
Decision to Amputate
On admission the entire right lower extremity was severely atrophic but the leg lengths were equal. Atrophy of both the thigh and the calf was two centimeters. The child was unable to dorsiflex her foot above a 30-degree angle of equinus. The inter-digital spaces between the first and second, and the second and third toes were very much diminished. A large weeping area approximately five centimeters in diameter was evident over the lateral surface of the ankle with an open granulating center approximately two centimeters in diameter, also numerous venous channels on the dorsum as well as on the plantar surface of the foot. Culture from the granulating area grew out hemolytic staphylococcus aureus, coagulase positive, which was sensitive to all antibiotics tested. X-rays of the foot and ankle showed extensive osteoporosis of all bones but with no radiolucent areas. Apparently no tumor involvement of bone had occurred. The medical work-up was otherwise within normal limits with a normal blood picture and normal blood sugar. The patient was presented at the Tumor Clinic with the recommendation that all soft tissue be removed from the dorsum of the foot and toes, and that the area be covered with split thickness grafts. Our consultant in plastic surgery predicted discouraging results from any reconstructive procedure and recommended a below-knee amputation. Psychiatric review and opinion indicated that the youngster and the parents had adequate stability to consider the amputation and to handle it emotionally. When the child was seen in the Amputee Clinic, an amputation was recommended as the procedure of choice. She was then presented to the Orthopedic Teaching Conference where amputation was also recommended, unless the tumor could be eradicated by X-ray therapy. The radiology consultant was of the opinion that this tumor should not be handled by radiation therapy.
Amputation and Prosthesis
N.B., therefore, had a right below-knee amputation at the age of 9 years 5 months (Fig. 1 ) with primary healing. Psychiatric guidance and support with pre-prosthetic preparation followed, and when the stump was ready, she was fitted with a right below-knee patellar-tendon-bearing prosthesis, cuff suspension and a SACH foot (Fig. 2 ). She developed total mobility within a matter of three weeks and very promptly she was able to go on to such activities as ice-skating and dancing.
Now 16 years old, N.B. probably has reached full growth. There have been no recent problems. She has a very suitable cosmetic as well as functional replacement and is living a totally normal life. This youngster has matured both physically and in personality and is completely accepted by her peers. This is a considerably different child from the one who was limited to crutch mobility or the sick bed and was segregated from the usual children's activities because of the tumor problem.
D.K. is presently 15 years 8 months of age with a diagnosis of hemangio-lymphangioma. This condition was apparent at birth, being described as a mass in the groin, buttock and medial aspect of the right foot. At the age of six weeks a surgical procedure was undertaken for removal of the groin mass which involved the scrotum. At this time part of the mass in the medial side of the foot was also removed and X-ray therapy was undertaken in the attempt to control the tumor. This procedure was ineffective and was abandoned.
This patient was the product of a normal delivery following a full-term normal pregnancy. The youngster was basically in good health. He had had measles and chickenpox but no other diseases and no other congenital abnormalities were present. The admission examination at Newington Hospital for Crippled Children at the age of 4 years 10 months revealed a much enlarged right buttock and leg, which were more than twice the size of the normal left side with gross excess of subcutaneous soft tissue. The right lower extremity was a half inch longer than the left. One testicle was in the scrotum. A previous surgical scar was well healed. Full motion was present in all joints. The boy was able to walk but did so with a marked limp.
Excision of Tumor and Amputation
Surgical correction consisting of a radical circumferential excision of the tumor of the ankle and foot with free grafting to cover the area was undertaken (Fig. 3 ). Good healing was obtained in five weeks and the youngster reestablished ambulation using an elastic stocking for support and a special shoe.
At the age of 6 years 1 month D.K. was readmitted to the hospital because of repeated infections and cellulitis including thrombophlebitis controlled by antibiotics. These infections had been recurring with increasing frequency and were increasingly difficult to control. Consultations were held with general, plastic, and orthopedic surgeons, as well as with the Amputee Clinic Team. The decision was unanimous that amputation of the extremity would be a precarious procedure but was the only possible way of controlling infection. Consequently at the age of 6 years 2 months an above-knee amputation was completed. The tumor was found to extend throughout the lateral and posterior musculature of the thigh. Cultures taken at surgery were sterile.
The wound healed primarily. The patient's convalescence was good, although he had required nine units of blood at operation since the procedure was done without tourniquet control. Two weeks prior to discharge he developed a small vesicular rash on the posterior pelvic region and also on the posterior thigh stump (Fig. 4 ). He also ran a high fever. This rash was determined to be due to the underlying tumor and was the probable portal of entry for the infection. The prognosis was uncertain and extreme care relative to cleansing and protection of the skin became mandatory.
No Prosthetic Replacement
It was obvious that prosthetic replacement was not a possibility because of the existence of tumor in the groin, scrotum and the buttock area and the boy's inability to tolerate the pressure of a prosthetic socket. However, he learned to use crutches very effectively and returned to regular school. The one restriction placed on his activities was exclusion from the gym program. He has been followed in the Tumor Clinic. Many episodes of weeping from various areas of the thigh stump, the groin and the scrotum with high temperature elevations have occurred, but have been controlled by antibiotic therapy. Soft tissues have slowly become less turgid. He has run into an odor problem from contamination of his clothes by discharge and is now protecting his clothes with Saran Wrap and using an acid mantle cream which has been very effective. He has been able to participate with his peers in regular school, although on crutches, with his odor problem controlled. He continues on penicillin maintenance.
In the opinion of those who have worked with this boy the amputation has made possible the mobility which he did not previously have, because of the bulk and gigantism of the affected leg. Moreover, the amputation was probably a lifesaving procedure.
R.G. is a 22-year-old white female who has a diagnosis of lumbar myelomeningocele with paralysis and spotty sensation distal to the knees bilaterally. A significant degree of mental retardation is also present.
This patient was the product of a normal pregnancy and delivery. A lumbar myelomeningocele was closed surgically within the first few days of life. At the age of 16 months a triple arthrodesis was done on the left side and at the age of 5 years the same procedure was accomplished on the right foot. A dysplastic right hip was reduced and has remained stable. All of these procedures were done at another hospital. She also was able to develop effective sphincter control.
When first seen at Newington Hospital for Crippled Children at the age of 16 years 11 months, her chief complaint was pain at the left ankle and foot. This pain was caused by an avascular necrosis of the talus with a moderately severe fixed equinovarus deformity on which she was trying to walk. A tibio-calcaneal fusion was done and after 12 weeks it appeared that an excellent union had been achieved. She was mobilized with progressive weight-bearing protected by crutches and was discharged ambulant.
At the age of 17 years 8 months, a pseudoarthrosis of the fusion was apparent (Fig. 5 ). A further attempt was made to surgically repair the pseudoarthrosis and also at that point an arthrodesis of the midtarsal joints was undertaken. This surgery was complicated by wound dehiscence and subsequent soft tissue infection, the organism being Pseudomonas. A cross leg skin graft was accomplished in multiple stages and the girl was again discharged, ambulant on crutches.
Osteomyelitis and Amputation
At the age of 18 years 7 months, an obvious chronic osteomyelitis involving the left ankle was evident. A sequestrectomy was accomplished at this time, although the possibility of amputation was discussed. The sequestrectomy procedure healed but, by the age of 20 years 11 months, the patient had an unstable left ankle with chronic infection, plus an infected great toe. Poor function of the right lower extremity required the continuing use of a short leg brace.
A left below-knee amputation was accomplished. The amputation stump healed primarily and at the age of 21 years 2 months the patient was fitted with a left patellar-tendon-bearing, below-knee prosthesis with a cuff suspension and a SACH foot (Fig. 6 ). This prosthesis permitted complete ambulation in the house without other assistive mechanisms other than the short leg brace on the right side. For walking longer distances, she has been in the habit of using a Canadian-type crutch also. When last seen at the age of 22 years, R.G. was completely stable and mobile. She lived in her parent's home, carried on an active life, socialized effectively, and was without distress .
L.N. is a 16-year 7-month-old white female, with extensive third-degree burns of both legs, abdomen, right upper extremity and both hands.
This patient was first seen at the age of ten months. At the age of three months she had been involved in a fire in a housing project and survived the acute problems of extensive burns at another hospital. She had had extensive multiple skin grafting, also amputation of the lateral four toes of the right foot and partial amputation of the great toe. When first seen, she had developed severe scarring with fixed flexion contractures involving the popliteal area, the right ankle and a severe equinovarus deformity of the right foot (Fig. 7 ). The left leg showed no contractures other than some tightness in the popliteal region. The right elbow was densely scarred with fibrous bands laterally and extensive scarring was present across the lower abdomen. During her first admission at Newington Hospital for Crippled Children, L.N. underwent soft tissue releases and skin grafting to correct the contractures of the popliteal area and the great toe. At her subsequent admission at the age of 2 years 6 months, further soft tissue releases for the right ankle and foot were accomplished, while at the age of 5 years 11 months further releases were required for the right popliteal region so that full extension of the knee was again obtained. However, it was apparent that the right ankle was ankylosed and that the right leg was 3.5 cm. shorter than the left. A corrective shoe was applied successfully.
Evaluation for Amputation
At the age of 8 years 9 months, the problem of shoeing had become increasingly acute as a result of the extremely poor skin, severe scarring and the poor position of the foot. Because of this difficulty plus the problem of recurrent flexion deformity at the knee and a leg length discrepancy, she was seen in the Children's Prosthetic Clinic and evaluated for a possible Syme's or other amputation. It was felt that because of the unstable home situation an amputation should not be undertaken at that time. In the course of the ensuing two years, a good deal of work was done to evaluate and stabilize the home environment and when she was again presented to the Orthopedic Teaching Clinic, opinion was mixed concerning the suitability of amputation.
It was not until the girl was 10 years 7 months old that amputation was felt to be appropriate. Because of the status of the skin, group opinion was also divided concerning the level of amputation. The possibility of an above-knee amputation with ischial weight-bearing in the prosthesis was compromised by scarring in the area of the tuberosity. Hence the decision to do a Syme's-type amputation. Since there was further soft tissue tightness and contracture at the right knee, a z-plasty was also done. Ischemia caused delayed healing over the distal end of the stump and two z-plasties were subsequently required to control the spot breakdowns due to this factor. With very careful attention to minor changes in the prosthesis, the patient became ambulant with a Canadian Syme's prosthesis incorporating the patellar-tendon-bearing principle.
During the ensuing two years, several short admissions were necessary resulting from the combined problems of ischemia and weight-bearing through the prosthesis. These problems were handled by socket modifications. Modifications were also necessary to adjust for growth.
At the age of 13 years 4 months, ischemia over the lateral distal portion of the Syme's stump was evident because of pull by a linear fibrous band when the knee was fully extended. This band extended well above the knee to the area of the ischial tuberosity so that multiple z-plasties, further skin grafting, and revision of the bony stump were undertaken together with provision of a new prosthesis. The girl again became completely independent and ambulant. This independence was carried to excess so that at the age of 14 years she was again admitted because of ulcer formation in the area of the fibular head and the further development of linear fibrous bands. At that point, the girl was labeled as a sociopathic personality, since she was going to extremes in teenage activities and dancing. This situation stabilized with the addition of a long thigh corset to the previous below-knee prosthesis, and she did well.
At the age of 14 years 10 months, L.N. was admitted for further plastic work involving the left thumb and web space. She also needed a new prosthetic socket. She was instructed to pay more attention to personal hygiene and cleanliness of the extremely irregular skin areas of the entire extremity and again became totally independent. Since that time, she has had minor adjustments in the prosthesis to keep her positioned properly and to allow for growth (Fig. 8 ).
L.N. was seen briefly at 16 years 5 months because of irritation at the lower pole of the patella. She had been doing a good deal of kneeling and had compressed the upper anterior lip of the socket. This resulted in soft tissue irritation which cleared on proper management. It was felt that some further psychiatric assistance and closer supervision in the Prosthetic Clinic was necessary.
We have presented four patients for whom elective amputation has been the determining feature in the establishment of effective independence. Three of them are using prosthetic equipment. One of them continues to use crutch ambulation exclusively. All four have attained essentially normal mobility.
We believe that elective amputation should not be relegated to a "last resort" status. In all four of the reported cases, this procedure might possibly have been undertaken earlier, to the advantage of the individual and the achievement of a more normal childhood. Possibly it would have permitted more activity during the period of development. We do not believe that elective amputation should be viewed as an indication that previous surgery or treatment was inadequate, but that it is actually the procedure of choice in selected instances.
John C. Allen, M.D. is associated with the Amputee Clinic Newington Hospital for Crippled Children Newington, Connecticut