An Exchange Of Viewpoints On Managing The Patient With Upper-Extremity Limb Deficiency And Scoliosis

Editor's Note: The article "Upper-Extremity Limb Deficiency With Concomitant Infantile Structural Scoliosis," by Charles H. Epps, Jr., M.D., appeared in the November, 1965, issue of the ICIB. In the belief that clinics should pool their information wherever possible, Dr. J. Leonard Goldner wrote Dr. Epps about his own, somewhat dissimilar experience with this difficult problem. His letter to Dr. Epps and the letter's reply are reprinted below with their permission. We invite further comments from our readers.

Dear Dr. Epps:

Your recent report on upper-extremity limb deficiency with concomitant scoliosis clearly presented the problems attending this difficult condition. Our experience with these patients has also been limited. We have fitted one or two prostheses on some children with bilateral amelia, but for the various reasons you have encountered, plus the fact that most of these children do exceedingly well with their feet, the prostheses have generally been discontinued. This is particularly true among patients with bilateral amelia without any residual phocomelic segment.

During the past two years, as a result of our previous ten years' experience, we have stopped fitting bilateral amelia cases with any type of prosthesis. So far I have not seen a single child I would want to fit The only time I provide such a prosthesis is when the parents wish it strongly - and then only to show them that the child will probably not continue to wear it.

We have observed patients with scoliosis both with and without hemivertebrae. One of these children moved to California and subsequently underwent spinal fusion when about seven or eight years old.

In going over your case histories, it seems to me that in Case No. 1, with progression through 46 , consideration of fusion in the near future would certainly be warranted. This could be followed after a reasonable time in plaster by a Milwaukee brace. The surface-area problem would be minimal if the pelvic covers were aerated and if no lateral pressure pad was used.

I don't believe that interference with the prosthesis is a valid reason for not doing the spinal fusion. As already indicated, most of these children don't do well with the prosthesis anyhow, and treatment of the scoliosis is much more important than the prosthesis.

Furthermore, as the rotation becomes more severe, the scoliosis is much more difficult to correct. Thus it seems reasonable to go ahead with the correction even at age five. Although the condition may recur, an aggressive attitude, including re-expiration at eight to twelve weeks, refusion if the curve begins to move, osteotomies, and so on, are certainly most essential in this group of individuals.

Case 2 falls in the same category, the curve being 80° and the child six years old. I wonder why you are delaying surgery, since spinal fusion was performed on Case 3 in 1962 when the child was five years old, and progression of the curve appears to have been slowed or halted.

Case 4 appears to be mild enough to warrant continuing the present routine, particularly since this child has so many deformities and the spinal condition is not severe at the present time.

I am particularly interested in your comments about prostheses and the fact that the children wear them "with their former skill." if you have obtained a consistent level of performance in bilateral amelia with one or two prostheses, I would be very interested in knowing how long the patients have been wearing them and what they actually use them for. Are the parents forcing the child to wear the prostheses or is the child really cooperative?

There is a great deal to learn about this problem, and certainly pooling information from the various clinics will be helpful.

J. Leonard Goldner, M. D.

Professor of Orthopaedic Surgery Chief,

Child Amputee Clinic Duke

University Medical Center Durham, North Carolina

Dear Dr. Goldner:

I want to thank you for your interest in my recent report concerning upper-extremity limb deficiency and scoliosis.

Our experience with bilateral amelia and phocomelia has been interesting. Until five years ago, we routinely attempted bilateral fittings. Since then, we have fitted one side (hopefully the dominant side), feeling that very young patients do not have the neuromuscular coordination or physical landmarks for successful operation of bilateral prostheses. Older patients are given a second arm, usually with a cosmetic hand, if function is not practical for the second side. The level of skill our patients achieve is undoubtedly a tribute to the competence and tireless efforts of our therapists. We have been fortunate also in having the cooperation and support of parents in most instances.

Patients with bilateral amelia or other anomalous appendages with little or no intrinsic functional ability have been fitted satisfactorily. We have not discouraged use of the feet at home for play, etc., but we have stressed development of prosthetic function for eating, writing, and toileting when possible. Our feeling has been that these skills make for a more satisfactory school adjustment and more acceptable function in social situations.

While we have one outstanding failure in an eleven-year-old bilateral transverse hemimelia, we have had notable success in a fifteen-year-old with left amelia and very short right transverse hemimelia (stump less than four inches). The failure, we feel, was due to rejection of the prostheses on the part of the parents, with strong transference of attitudes to the patient. The other patient, who was not presented in the report because his scoliosis was mild and nonprogressive, attends regular school, using public transportation, eats, writes, and performs limited toileting in school. He has a standard prosthesis for a very short above-elbow stump on the right, with dual control, flexion wrist, and single-load voluntary-opening hook. The left(amelia) side was fitted in the past six months with an SD-tye prosthesis with nudge control for elbow and perineal strap for terminal device operation. This patient is relatively independent and I suspect that the presence of a very short stump on the right has added materially to his functional ability.

Case No. 1 learned to eat and to color within lines with a standard shoulder disarticulation prosthesis. Special utensils were used, but he performed these two activities -independently in a manner that was gratifying to him and acceptable in a kindergarten situation. Prosthetic function was a must for this patient because his phocomelic feet were not functional. The patient died unexpectedly in December, 1965, of infection, but at the time of his death was attending the local health school and doing satisfactorily. We prepared a 16 mm movie demonstrating his skill. This film was used to orient his first teacher. I will be happy to send the film so that you can see what was accomplished.

The scoliosis in Case No. 2 has progressed to a point that makes surgery an urgent consideration. His problem is presently under review. Although he is almost seven years old, he is rather small for his age. Thank you for your comments.

Case No. 3 is fitted unilaterally at present. Initially, she had bilateral fitting, but the left was abandoned five years ago when it was noted that she was using her phocomelic finger through an opening in the humeral section of her prosthesis. She uses her nonstandard shoulder disarticulation type prosthesis in school for eating, writing, and distant prehension. At home she frequently eats with her left extremity and uses the two fingers for other activities requiring close prehension.

As you can see, we have tried to vary and individualize our approach to each patient within the guidelines stated above.

It was good of you to respond to my paper. We have much to learn about these patients. Exchanging ideas among the clinics, I am sure, will enhance our ability to deal with this and other difficult problems.

Charles H. Epps, Jr., M. D.

Clinic Chief

Juvenile Amputee Clinic

Department of Public Health

District of Columbia General Hospital

Washington, D. C.