Focal Deficiencies Of The Upper Femur

William R. Eaton, M. D.


Several clinic chiefs have discussed the treatment of children with proximal femoral focal deficiencies in recent issues of the Inter-Clinic Information Bulletin. In the hope that our experiences will also be of interest to those involved in the child amputee research program, I am reporting the treatment procedures we have employed in four cases of focal deficiency of the upper femur - two with surgery and two without.

Each of these four patients had multiple defects and involvement of the opposite femur - one a phocomelia, one coxa vara and two an osteochondrosis. Two had scoliosis which may or may not be congenital. One had involvement of both hips, both arms and a congenital heart lesion. One had bilateral congenital inguinal herniae.

Case Studies

Case One - S.D. Treatment without surgery (Fig. 1 , Fig. 2 , and Fig. 3 ).

This patient was born on June 3, 1956 and admitted to our clinic at the age of a year-and-a-half with a focal deficiency of the left upper femur and a right phocomelia with a complete foot.

An initial fitting attempt with a pylon-type prosthesis was unsuccessful because of multiple problems - external rotation and flexion attitude of the left leg, lack of muscle power in glutei bilaterally and pain on internal rotation. In an effort to maintain the left femoral head near the acetabulum, skeletal traction, followed by a cast, was applied to the left tibia.

At the age of three years and nine months, S. D. was fitted with pylon-type braces with rigid pelvic and thoracic bands. She started walking with short pylons and locked hips. As she became more proficient in walking, the pylons were gradually lengthened to approximate normal height and the hips were unlocked.

A prosthesis on the right phoco-melic extremity has been considered, but no surgery has yet been performed. The plan presently under consideration is conversion of the left side focal deficiency to a below-knee amputation and then the fitting of bilateral prostheses. The patient is very small, however, and this procedure will be delayed in the hope that future study may throw more light on the solution of the problem. S.D now walks fairly well with crutches and bracing.

Case Two - D.S. Treatment without surgery (Fig. 4 and Fig. 5 ).

Born on September 13, 1955, this patient was admitted to the Home for Crippled Children at the age of two years with a diagnosis of congenital malformation of both arms and a focal deficiency of the left femur. She had an abduction and flexion deformity of the left hip. She also has a small septal defect of the heart.

The patient was fitted with an ischial weight bearing brace with an extension caliper and walked satisfactorily Present X-rays reveal attempting development of the femur in the acetabulum plus an osteochondritic process of the right hip.

D. S. is able to derive some use from her right hand but is unable to get either hand to her mouth. To provide this function, her left hand (with non-functioning digits) was fitted with a step-up below-elbow prosthesis.

Case Three - D.D. Treatment with surgery (Fig. 10 , Fig. 11 , Fig. 12 , Fig. 13 , and Fig. 14 ).

This patient was born on June 20, 1950 with a left focal deficiency of the upper femur, right coxa vara and bilateral inguinal hernia. He was admitted to the Home for Crippled Children at the age of six years and nine months.

D. D. had previously walked on a brace-type arrangement for the left leg. This consisted of an ischial weight bearing brace which allowed the foreshortened leg to be suspended between the uprights. The patient developed increased difficulty in brace fitting because of rotation of the foot. He also developed a scoliosis which further complicated his condition. A Syme-type amputation was then performed as a remedial procedure.

The patient was subsequently fitted with a left above-knee type of prosthesis with the knee joint located at the distal end of the tibia. He was also fitted with a spinal brace to correct the scoliosis. The patient learned to walk satisfactorily in spite of the discrepancy in knee joint locations.

The problems yet to be reconciled are the treatment of the right sided coxa vara and the scoliosis. Surgery has not yet been performed in these areas. Conversion of the stump on the left side may be necessary at a future date to correct the discrepancy in leg length. The hernias were corrected during infancy.

Case Four - J. M. Treatment with surgery (Fig. 15 , Fig. 16 , Fig. 17 , Fig. 18 , and Fig. 19 ).

This patient was born on January 8, 1944. After a below-knee amputation had been performed in a hospital in the eastern part of the state because of hip flexion and foot rotation, he was referred to our clinic for prosthesis and training at the age of 15 years and 8 months. His admission evaluation included the following comments:

"This boy is a right lower extremity amputee due to a congenital aplasia of the right upper femur. There is a tibia and fibula and the foot was amputated in order for a prosthesis to be fitted to the stump which simulates a knee disarticulation. A Thomas-ring brace with a pelvic band was worn on the amputated side. Localized muscle testing of the stump at hip level was impossible; however, that of a generalized nature was. He had 10 of abduction with good muscle strength, 45 of flexion with fair plus muscle strength, 25 of adduction with fair muscle strength, and 45 of extension with normal muscle strength.

"There is a congenital deformity of the right elbow. He lacks 70 of muscle extension and has a triceps of fair strength. He has 25 of elbow flexion. He has a left thoraco-lumbar scoliosis."

We were advised that the boy had walked with a brace, gone on hunting trips and taken part in normal activities prior to the amputation.

X-rays revealed a long pointed femur on the right that extended posteriorly and prevented extension, and a healed osteochondrosis on the left. The patient was started on a conditioning program, weight reduction (he weighed 144 pounds), and stretching.

The impingement of the femur in back of the ilium kept him in marked flexion. A month after admission, several inches of the proximal remains of the femur were removed through a posterior incision. There was no soft tissue or cartilaginous attachment to the acetabulum. It was more like a pointed stump.

J. M. was then fitted with an above-knee type of socket and pelvic band. He continued his exercise program until he walked satisfactorily and was returned to the referring institution. Other than the exercises, no attempt was made to correct the scoliosis or the left elbow deformity at this institution.

Conclusions

Four children with proximal femoral focal deficiencies have undergone treatment at our clinic. All had additional complicating defects. Two were fitted with brace type devices without surgery and two were fitted with prostheses following surgery by us or others.

In general, our approach to the management of a child with a proximal femoral focal deficiency tends to be somewhat more conservative than is apparently the practice at some of the other clinics. We have found that brace type appliances without surgery are satisfactory for the very young child. However, in cases where hip flexion, foot rotation and other factors presented insuperable problems, we have resorted to amputation and fitting with a prosthesis.

(Editor's Note: Dr. Eaton's report on "Knee Disarticulation Treated as Above-Knee Amputation" appeared in the April issue of the Inter-Clinic Information Bulletin. His second article, "Focal Deficiencies of the Upper Femur", was also submitted for publication at that time but was deferred until this issue because of space considerations.)

William Eaton is Co-Clinic Chief, Home for Crippled Children, Pittsburgh, Pennsylvania