Scoliosis Combined With Congenital Deficiencies of the Upper Limb: The Effect of Prosthesis Wearing

N. LeROY HAMMOND, III, M.D. RICHARD L. LEVITT, M.D. JAMES M. HUNTER, M.D.


Kato, in 1924, was the first to report scoliosis associated with congenital deficiencies of the upper limb3. He reviewed 253 cases of radial hemimelia and mentioned that five of these patients had scoliosis. Since then occasional reports have cited the association of scoliosis with congenital upper-limb deficiencies. Specifically this association has been reported by Kuhns and Hor-mell4, Epps1, Wynne-Davies 8 , and Pardini 7 .

More recently Makley and Heiple6, in 1970, investigated this problem and noted a 48-percent occurrence of scoliosis in patients with severe upper-limb deficiencies. Subsequently Lambert and Sciora5 reported this phenomenon in 13 children.

In our amputee clinic we are currently investigating this association of congenital upper-limb deficiency and scoliosis. We are placing special emphasis on the effect of prosthesis wearing in relation to spinal curvature. Because of the relatively small number of patients evaluated, the findings in the present study should be considered as preliminary.

Materials and Methods

From 1962 to 1972, 116 children with congenital upper-limb deficiencies were seen in our clinic. To date, 60 of these have been specifically evaluated for scoliosis by means of both erect and supine x-rays of the spine. We have defined a significant curve as one which measured ten degrees or more in the erect position and which persisted in direction and location in the supine position. Using these criteria, it is possible that some early or mild structural curves were classified as functional. Rather than over-report the incidence of scoliosis, we elected to disregard such curves in evaluating our results.

In classifying the deficiencies an attempt was made to follow the nomenclature of Frantz and O'Rahilly 2 . Because of the relatively small number of patients evaluated, we did not distinguish between complete and incomplete, or between terminal and intercalary defects. Seven children with bilateral deficiencies were classified according to the more severely affected limb.

Of the 60 patients in this study, prostheses have been prescribed for 35. Each of these 35 patients was designated as either a prosthesis wearer or a non-wearer. This classification was done after interviewing the patient and his parents, reviewing the child's clinic chart, and watching him use his prosthesis. Only those patients who wore their prostheses on a daily basis, and used them actively, were classified as wearers. Patients who wore their prostheses for cosmesis only, or who wore them only occasionally, were classified as non-wearers. Patients for whom prostheses were not prescribed were also classified as non-wearers.

Results

Sixty patients with congenital skeletal upper-limb deficiencies were evaluated. Twenty-five of these were prosthesis wearers and 35 were non-wearers. The two groups were compared with respect to age, sex. and type of deficiency.

The patients ranged in age from one to 22 years. The age distribution in both groups is shown in Table 1 . The four age groups shown are those commonly used in classifying scoliosis. The average age of the wearers was 9.1 years; that of the non-wearers was 9.4 years.

Table 2 indicates the composition of the two groups with respect to sex. The female to male ratio in the group of prosthesis wearers was 1.5 to 1. The same ratio in the group of non-wearers was 1.2 to 1.

The incidence of the various types of upper-limb deficiencies in the two groups is summarized in Tables 3 & 4 . While the two groups cannot be considered precisely matched samples, we feel that they are sufficiently similar for the purpose of this study.

Tables 3 & 4 shows the incidence of scoliosis in this series. Thirty-six percent of prosthesis wearers developed significant structural scoliosis, as did 34 percent of non-wearers. This difference is not significant. The overall incidence of scoliosis in this series was 35 percent.

Discussion

Previous reports5,6 have indicated a high incidence of scoliosis in children with congenital deficiencies of the upper limb. Our data support these findings. Of the 60 patients evaluated, 21 (35%) had significant scoliosis.

The mechanism of production of such scoliosis is unknown. Perhaps it is the result of mechanical or muscle imbalance caused by the upper-limb deficiency. If this were the case, the active use of a prosthesis by the growing child might confer a degree of protection from such spinal curvature. On the basis of this study, however, we are unable to show any significant difference in the incidence of scoliosis in prosthesis wearers as compared to non-wearers. It would appear, then, that the risk of scoliosis in these children is not decreased by prosthesis wearing.

Summary

There is a very high incidence of structural scoliosis associated with congenital skeletal deficiencies of the upper limb. Prosthesis wearing does not seem to influence the incidence of such scoliosis.

References:
1. Epps, C. H., Upper-extremity limb deficiency with concomitant infantile structural scoliosis, Inter-Clin. Information Bull., 5 :2:1-9, November 1965.
2. Frantz, C. H., and Ronan O'Rahilly, Congenital skeletal limb deficiencies. J. Bone and Joint Surg., 43-A :8:1202-1224, December 1961.
3.. Kato, Katsiyi, Congenital absence of the radius. With a review of the literature and report of three cases. J. Bone and Joint Surg., 6 :598-626, July 1924.
4. Kuhns, J. G., and R. S. Hormell, Management of congenital scoliosis. A review of one hundred and seventy cases. Arch. Surg., 65 :250-263, 1952.
5. Lambert, Claude N., and Jean Sciora, The Incidence of Scoliosis in the Juvenile Amputee Population. [nter-Clin. Information Bull., 11 :2:1-6, November 1971.
6. Makley, J. T„ and K. G. Heiple, Scoliosis associated with congenital deficiencies of the upper extremity. J. Bone and Joint Surg., 52-A :2:279-287, March 1970.
7. Pardini, A. G , Radial dysplasia. Clin. Orthop., 57-A : 153-177, 1968.
8. Wynne-Davies, Ruth, Familial idiopathic scoliosis. J. Bone and Joint Surg., 50-B 24-30, February 1968.