1,018 Children With Skeletal Limb Deficiencies

Editor's Note: A full report of this study conducted by New York University will be published this summer. The following summary, prepared by Miss Heidi Vorchheimer, indicates the scope and some of the principal findings of the study. Copies of the complete report may be obtained by writing to Dr. Sidney Fishman, Project Director, Prosthetic and Orthotic Studies, New York University Post-Graduate Medical School, 317 East 34th Street, New York, N.Y. 10016.

A group of 1,018 children with limb deficiencies treated at 17 juvenile amputee centers were studied in terms of their age, sex, and cause of amputation (and/or anomaly), the number of limbs affected, and the level of amputation or anomaly. Data concerning the extent of prosthetic utilization and phantom limb experiences were also obtained. The study provides detailed descriptive data on a reasonably representative sample of the juvenile population requiring and obtaining prosthetic services during the years 1959-1963. This report confirms and elaborates findings obtained in surveys conducted during the period from 1953 to 1958. 1,2

The group was divided into two broad categories based on the etiology of the limb deficiencies: congenital and acquired.

Congenital Deficiencies

Limb losses of congenital origin were further subdivided into types according to the nature of the deficiency, viz., amputation (terminal transverse) - 67%, anomaly-29%, and combined-4%. Thus, approximately one-third of the children with congenital limb deficiencies did not present a true amputation-type stump for prosthetic fitting.

Surgical conversion of the anomalous limb to an amputation-type stump was performed on nearly half of the total anomaly group. The ratio of lower-extremity to upper-extremity anomalies was 3:1, but surgical intervention occurred in the lower-extremity group 12 times as frequently (98 out of 122 lowers; 12 out of 40 uppers). Surgical conversion of the lower extremities did not always resuit in a stump which was analogous to an amputation at the same anatomical level, since frequently the proximal bones and/or joints were also abnormal. For example, ablation of the foot in some cases of proximal femoral focal deficiency typically produces a stump which anatomically is at the be low-knee or Syme's level, but for purposes of prosthetic fitting is the equivalent of an above-knee stump.

By far the greatest number of children had an amputation-type congenital involvement of a single upper extremity at the short or very short below-elbow level-398 of 733 congenitally affected children had unilateral upper-extremity amputation-type deficiencies, and 219 of these were at the level noted. The male-to-female ratio was approximately equal in the total amputation-type group, although in the below-elbow category, girls outnumbered boys 127:92. Upper-extremity deficiencies occurred much more frequently than lower (12:1 in unilateral and 2:1 in bilateral involvement) . In the case of anomaly-type deficiencies, a different situation obtained. Here, males outnumbered females by approximately 40%; and lower-extremity deficiencies, both unilateral and bilateral, outnumbered those of the upper extremity by 3:1 and 2:1 respectively.

No information as to the specific causes of the congenital deficiencies was obtained. However, the groups of children who had bilateral and quadruple involvements were reviewed as to the levels of the deficiencies, and the findings were compared with those related to thalidomide etiology, which had been reported by Dr. Mongeau. 3 Dr. Mongeau's subjects had what he called "symmetrical" (bilaterally equivalent) deficiencies in 18 of 20 upper-extremity cases and in all three bilateral lower-extremity cases. Two of three quadruple amputees in his sample had symmetrical deficiencies in both upper and lower extremities, and the third child was symmetrically involved in the upper extremities only.

In our sample, there were 42 bilateral upper-extremity, 43 bilateral lower-extremity, and nine quadruple deficiencies in the amputation and anomaly groups. Of the 42 bilateral upper-extremity patients, 27 (19 of 28 amputation types and eight of 14 anomaly types) were apparently symmetrical; ten (nine amputation types and one anomaly type) were apparently not symmetrical; and the remainder (five anomaly types) were doubtful. The bilateral lower-extremity group showed apparent symmetry in 35 instances (11 of 11 amputation types, and 24 of 32 anomaly types), no symmetry in five (all anomaly types), and three doubtful cases (also all anomaly types). In the quadruple group, both upper and lower extremities were apparently symmetrical in two of nine amputation types, the lower limbs only were at equivalent levels in three instances, and the upper limbs only in one.

No more definite statement than "apparent symmetry" can be made because of the number of anomalous types for which no standard or complete description was available. Nevertheless, if symmetrical limb deficiencies are characteristic effects of a known teratogen (thalidomide) acting during the first trimester of pregnancy, it might be suspected that many of the "prethali-domide" children in the study had also been affected by some teratogenic agent at a similar stage of fetal life.

Acquired Deficiencies

The group of children with acquired amputations were subdivided on the basis of general cause, i.e., accident or disease. Of the total of 285 children, 191 amputations were the result of trauma and 94 the result of disease. The ratio of boys to girls in the accident-caused group approached 3:1, presumably reflecting the generally more adventurous nature of the male child. The ratio was nearly equal (1.2:1) in the disease-caused group. It is noted that the largest number of amputations due to accident occurred between the ages of two and four years (69 out of the total of 191), while the largest number due to disease occurred before the age of one year (29 of 94). The disease precipitating 13 of these 29 amputations was tumor, the remainder being classified as birth defects of various kinds, presumably with superimposed gangrene or other complications.

Tumors were the precipitating cause of amputation in 60 of the total of 94 amputations due to disease. Of the 94 cases, 86 involved amputations of a single extremity, with lower-extremity amputations occurring more than twice as frequently as those of the upper extremity (61 vs 25). Seven of the remaining eight children had amputations of both lower extremities, and one of all four extremities, primarily because of birth defects.

The high proportion of single-extremity losses also held for the accident group (171 of 191), but in this group the proportion of upper-extremity to lower-extremity losses was the reverse of the "disease" group (102:69). The remaining 20 children in the accident-caused group had had amputation of two limbs-three bilateral uppers, 14 bilateral lowers, and three ipsilateral doubles (i.e., one upper and one lower limb). It would appear then that accidents produce more unilateral upper-extremity losses, and disease more lower-extremity losses, with the total number of acquired unilateral losses being equally divided between uppers and lowers.

Accidents were the primary cause of multiple-limb losses, accounting for 20 out of 23 (disregarding five children who were amputated because of complications superimposed on birth defects). Vehicular accidents were the cause of 13 multiple amputations, and burns the cause of five. It was noted, however, that tumor-caused amputations exceeded those attributable to vehicular accidents.

With regard to sites of amputation in the "acquired" group as a whole, the largest numbers were at the above-elbow and above-knee levels, in contrast with the below-elbow and below-knee levels predominating in the congenital group.

Prosthetic Utilization

The data indicate that congenitally deficient children were usually fitted with prostheses before the age of six years, the average for unilateral upper- and lower-extremity deficiencies, for example, being four years two months and three years seven months respectively. The average for the multiply involved children was somewhat lower. It would be expected that the age of initial fitting for children with congenital limb deficiencies is now lower than was the case when the data for this study were gathered, reflecting the more recent practice of fitting both upper- and lower-extremity prostheses during the first year of life.

The median elapsed time between amputation and prosthetic fitting for children with acquired amputations was nine months for unilateral upper-extremity losses and seven months for unilateral lower and multiple limb losses. Obviously, this median time lapse could be reduced through the acceptance of the early prosthetic fitting concept, better case-finding techniques, and more expeditious financing.

As far as the number of prostheses fitted to children with multiple deficiencies was concerned, it was reported that only 50% of children with bilateral upper-extremity deficiencies were fitted with two prostheses, whereas 96% of children with bilateral lower-extremity amputations were so fitted.

Phantom Limb Sensation

The analysis of data on phantom limb sensation revealed that for both the unilateral upper- and lower-limb amputation group the incidence increased very substantially as the age at amputation increased; the average incidence rate for the entire group being approximately 45%. The data also suggest that awareness of the phantom limb tended to decrease as the time since amputation increased. Children whose congenital anomalies had been surgically converted indicated a lower incidence of phantom sensation than was the case in children whose amputations were acquired. This finding, however, may have been a function of a limited sample. Three instances of reported phantom sensation by children with congenital amputation-type deficiencies could not be substantiated through further interrogation of the children or parents. An analysis of the incidence of phantom sensation in relation to time lapse between amputation and prosthetic fitting failed to reveal any relationship.

In conclusion, the data obtained in this study has:

  1. Enlarged our understanding of the nature of the child amputee population.

  2. Provided information which may give direction to the development of the most urgently needed prosthetic components and amputee management procedures.

  3. Established a basis of comparison for future survey-type studies.

  4. Raised questions regarding the definitive teratological etiology of congenital deficiencies.

-Heidi Vorchheimer

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References:
1 Surveys of Child Amputees at the Mary Free Bed Hospital, Grand Rapids, Michigan, New York University College of Engineering, Report No. 115.21, May 1957.
2 The Clinical Treatment of Juvenile Amputees 1953-1956, New York University College of Engineering, Report No. 115.26C, August 1958.
3 Mongeau, Maurice: Our Experience With the Thalidomide Children-An Interim Report, Inter-Clinic Information Bulletin, VI:3-7, 12 (Jan.) 1967.