The Incidence of Scoliosis in the Juvenile Amputee Population

CLAUDE N. LAMBERT, M.D. JEAN SCIORA, R.N, B.S.P.H.N


The incidence of scoliosis occurring in children with congenital deficiencies of the upper extremities has been mentioned by Kato 5 , Kuhns and Hormell 6 , Riordan 9 , Harrison, Pearson and Roaf 3 , Frantz and O'Rahilly 2 , Pardini 8 , and Wynn-Davies 11 . Relatively little attention, however, has been paid to scoliosis as a complicating factor in the prosthetics management of congenital limb deficiencies until recently. In 1965 Epps1 reported on four children with upper-extremity limb deficiencies who had concomitant infantile structural scoliosis. More recently Makley and Heiple 7 found a significant incidence of scoliosis in children with the congenital upper-extremity anomalies of radial and ulnar hemimelia, phocomelia, and amelia.

Our experience at the University of Illinois Clinics has also indicated the need to be alert to the possibility of scoliosis developing in children with congenital upper-extremity deficiencies. We have found scoliosis in our juvenile amputee population with acquired amputations as well as those with congenital deficiencies. The review of our cases which follows emphasizes this need for awareness that scoliosis may develop in the juvenile amputee population regardless of the etiology of the limb deficit. This population of developing children appears to be a high-risk group.

Case Histories

Unilateral Terminal Transverse, Partial Hemimelia, Upper Extremity

In all but one of the six children in this category, the left arm was affected; three were males and three were females:

S H. has been followed in clinic since she was 11 months of age. She also has bilateral tibial torsion and metatarsus varus more severe on the right-for which she has had several surgical procedures. She had also bitten into a connected electric cord and has had plastic surgery to her mouth A slight idiopathic right dorsal, left lumbar, curve was noted when she was 9 years of age It is being kept under observation

C.W , another female, has been followed in the amputee clinic since she was 20 months of age. At 12 years, 6 months of age, she complained of pain in the lumbar area. A left thoracolumbar curve (D 10 - L 4 = 15°) was noted. It is compensatory to a leg-length discrepancy. This condition has been controlled by a ¾-in. lift on the left heel

A.B., a male, has been followed in clinic since he was 3 years, 5 months of age His right dorsal, left lumbar, idiopathic scoliosis was noted when he was 17 years of age It has not progressed.

C.H., a male, has been followed in clinic since he was 5 months of age. He has a slight (10°) right thoracic idiopathic scoliosis, but his greater problem has been dorsal round back, which was noted at the time he was 11 years of age and has been controlled by hyperextension exercises. The mother stated there is a considerable history of round back on the paternal side.

G.P., a male, has been followed in clinic since he was 4 years, 4 months of age. A mild right thoracic, left lumbar, idiopathic scoliosis was noted when he was 14 years, 3 months of age. This patient is also being kept under observation.

M.D., a female, and the only one of this group with the right arm involved, has been followed in clinic since she was 2 years of age. She also has pronated feet and wears shoe corrections. When she was 10 years, 9 months of age, a slight right thoracic, left lumbar, curve was noted. About eight months later, a varus deformity of the right knee was noted. X-rays revealed an epiphyseal mass in the proximal tibial epiphysis, which increased in size. This mass was removed in the summer of 1969, with lateral stapling of the epiphysis. The varus was corrected, and leg lengths equalized. Her scoliosis has not progressed.

Other Congenital Anomalies

Seven additional children with other congenital limb deficiencies were reviewed. Four were males; three were females:

T.J., a male with bilateral amelia upper, has been followed in the clinic since he was 1 ½ years of age. At that time he was noted to have a mild right thoracic scoliosis, and at 3 years of age X-rays revealed dysplasia of the right hip with retarded growth of the femoral capital epiphysis. He began to walk at 2 ½ years of age. His curve had been noted prior to that time so, although he had a leg-length discrepancy (the right leg was shorter than the left), his curve was an idiopathic one. Interestingly enough, there is history of scoliosis in the family on both the maternal and paternal sides, and the mother asked whether the scoliosis was hereditary. This boy was seen in the clinic irregularly, as the family did not keep appointments consistently. The family was then lost to follow-up, and when located again 3½ years later the child had had no orthopedic follow-up and the scoliosis had increased. About a year later, the family moved out of state, where the boy had a spinal fusion.

K.J., a female, was first seen in the amputee clinic when she was 15 years, 3 months of age (she had been followed in various facilities as a military dependent). She has a right intercalary distal phocomelia (upper). Her right thoracic scoliosis was noted at her initial examination. She was then mature biologically and skeletally, and further progression of the curve was not expected.

M.O., a female, has been followed at clinic since she was 9 months of age. She has a right amelia upper, and a left terminal transverse hemimelia, upper. She also has congenital shortening of the left lower extremity. When she was 7½ years of age a left lumbar compensatory scoliosis was noted. There was sufficient discrepancy in leg length for her to wear a lift on the left shoe. At 10½ years of age she had stapling of the right femoral epiphyses. She now wears a one-inch heel lift.

R.R., a male, has been followed in clinic since he was 2 months of age. He has bilateral upper amelia and bilateral lower intercalary transverse proximal phocomelia; also left equinovarus, right calcaneovalgus, and bilateral inguinal hernias. He was noted to have a mild scoliosis-no bony deficiencies being noted in his spine. His feet were treated with casts and braces, and he was fitted with a left shoulder-disarticulation-type prosthesis and a plastic jacket. The curve progressed, and by 5 years of age he had a severe left dorsal, right lumbar, curve. Surgery was being considered, but family was undecided and shortly thereafter moved out of state.

E.E., a male, has right terminal longitudinal complete paraxial ulnar hemimelia and bilaterally fused elbows, a deformed left radius and ulna, and partial adactylia bilaterally. He was referred to the Scoliosis Clinic by the physical therapist in the special school because of his progressive scoliosis-left thoracolumbar, (T 5 - L 3 = 35°) at 9 years of age. According to the parents, scoliosis had been noted early in his infancy. He was fitted with a Milwaukee brace.

D,M„ a male, has a left upper intercalary distal phocomelia. He was first seen in the Scoliosis Clinic at 12 years of age. His curve was noted by the physician on a routine physical examination about two months before his first clinic visit. He used his phoco-melic hand well, and was not interested in any prosthetic fitting. He had a right thoracolumbar idiopathic scoliosis (T 8 - L 3 = 83°) and a spinal fusion with Harrington Rod instrumentation.

S.S., a female, has bilateral upper terminal longitudinal complete radial hemimelia. She also has bilateral cleft lip and palate, and is mentally retarded. She has been under the care of the Center for Craniofacial Anomalies. Her parents failed to keep numerous appointments in both that Center and local clinics of the Division of Services for Crippled Children. When the girl was 13 years, 3 months of age, her parents contacted the Division and reported that they had noticed a scoliosis developing for about a year, with marked progression during the last six months. She was seen in the Scoliosis Clinic at 13 years, 4 months of age. She had a right thoracic (T 7 - T 12 = 95°), and a left lumbar (T 12 - L 4 = 110°) curve. She has had a halo hoop and double femoral traction followed by spinal fusion with Harrington Rod instrumentation.

Acquired Amputations

Five children with acquired amputations were also reviewed (two males and three females):

D.M., a male, has a short left above-elbow amputation as a result of a gunshot wound received at the age of 10 years, 6 months His right dorsal, left lumbar, curve was noted when he was 16 years of age. Because of his skeletal age he was fitted with a Milwaukee brace, which he later wore at night only.

S.N., a female, had a left hip-disarticulation amputation secondary to neurofibrosarcoma at 11 months of age. At 9 years, 10 months of age, her scoliosis (idiopathic) was noted on a routine chest film. At that time (right T 3 - L 1 ) it was 10 deg , but later it increased to 35 deg. She was fitted with a Milwaukee brace at 10 years, 3 months of age, but wore the brace only at night because she wore a Canadian hip-disarticulation prosthesis in the daytime. She recently received her second Milwaukee brace with the new throat mold at 14 years, 4 months of age. She continues to wear it at night only. This has controlled her curve. In the brace her curve measures 12 deg. There is no familial history of scoliosis. However, a spine x-ray of the mother reveals she has a 10-deg. thoracic curve ( Fig. 1 , Fig. 2 , Fig. 3 , Fig. 4 ).

A.G., a male, was first seen in Amputee Clinic at the age of 4 years for bilateral gigantism of the feet secondary to hemangiomata. He also had a hemangioma of the left thorax with a left "C"-shaped curve. He had a left Syme's-type amputation at the age of 11 years, and right Syme's-type amputation at 13 years of age. His curve, which is attributed to the increased vascularity of the chest wall hemangioma, is being kept under observation.

K.H., a female, was followed in Amputee Clinic for treatment of pseudarthrosis of the right tibia with a right below-knee amputation performed at the age of 13 years, 5 months. She was lost to follow-up in 1959 when the family moved out of state. She returned to clinic this year at the age of 25 years with a severe thoracolumbar scoliosis (T 10 - L 3 = 72°) for which surgical correction was recommended. A diagonosis of neurofibromatosis has now been made and most likely the pseudarthrosis of the right tibia was secondary to neurofibromatosis. The family is undecided as to acceptance of the recommended surgery.

G.A., a female, has been followed in Amputee Clinic since she had her right hip-disarticulation amputation at the age of 9 years, 8 months, secondary to osteogenic sarcoma of the right femur. When she was 12 years, 3 months of age, a single metastasis was noted in the right chest with a right basilar resection done the following month. Her right dorsal, left lumbar, scoliosis was noted 13 months later when she was 13 years, 5 months of age, on a routine chest film. The curve was kept under observation and did not progress further She is now 20 years of age.

Discussion

In the group of 13 children with congenital limb deficiencies, the scoliosis was noted in early infancy in three of the children, and in the remainder when they were from 7½ years to 17 years of age. Three of these children had compensatory scoliosis secondary to leg-length discrepancy. The rest appeared to be idiopathic.

In four of the children with acquired amputations, the scoliosis was noted when the patients were from 4 years to 16 years of age. The age of onset was not known in the patient who returned to clinic at the age of 25 years with a severe scoliosis. Of this group, two were idiopathic. The others were secondary to hemangiomata, to neurofibromatosis, and to thoracic surgery.

Although the juvenile census of the University of Illinois Amputee Clinic is less than 1,000 (862 from April 1952 to April 1971), at least 18 children have been found to have scoliosis. Treatment varied from observation, exercises, surgery and/or shoe lifts to correct leg-length discrepancies, and Milwaukee braces, to spinal fusion.

Surveys as to the incidence of scoliosis in the general population yield varied results but all present a lower incidence than that noted in this clinic. Wynne-Davies 11 found 1.3 per thousand in the under-eight-years-of-age group, 1.8 per thousand in the over-eight-years-of-age group, and 0.39 per cent in the general population. Kane and Moe4 in their Minnesota study indicated a 0.133 per cent prevalence of scoliosis requiring referral to an orthopedist. Shands and Eis-berg10, in their study of 50,000 chest minifilms, found a 1.9 per cent incidence in the population over 14 years of age.

Summary

The cases presented in this report reveal that although the Amputee Clinic staff is primarily concerned with management related to amputated limb(s), there is need for careful examination of the entire child on both initial and follow-up visits, with further evaluations and prompt institution of appropriate treatment measures whenever indicated. This paper is not presented as a therapeutic outline for scoliosis, but seeks mainly to draw attention to the fact that scoliosis is not an uncommon finding in children with amputations, no matter what the etiology. Closer observation will, no doubt, reveal many more cases of scoliosis than are customarily identified.

Acknowledgments

The close cooperation between services within the University in the management of these amputees with scoliosis and especially the assistance rendered by Dr. Ronald L. DeWald, Chief, University of Illinois Scoliosis Clinic, and Dr. Edward F. Lis, Director of the University of Illinois Division of Services for Crippled Children, are gratefully acknowledged.

University of Illinois Amputee Clinic Chicago, Illinois

References:
1.Epps, C. H., Upper-extremity limb deficiency with concomitant infantile structural scoliosis. Inter-Clin. Information Bull., 5:2:1-9, Nov. 1965.
2.Frantz, C. H., and Ronan O'Rahilly, Congenital skeletal limb deficiencies. J. Bone and Joint Surg., 43-A:1202-1224, Dec. 1961.
3.Harrison, R. G., M. A. Pearson, and Robert Roaf, Ulnar dimelia. J. Bone and Joint Surg., 42-B:549-555, Aug. 1960.
4.Kane, W. J., and John H. Moe, A scoliosis-prevalence survey in Minnesota. Clin. Orthopaedics and Related Research, 69:216-218, March-April 1970
5.Kato, Katsiyi, Congenital absence of the radius. With a review of the literature and report of three cases. J. Bone and Joint Surg., 6:589-626, July 1924.
6.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.
7.Makley, J. T., and K. G. Heiple, Scoliosis associated with congenital deficiencies of the upper extremity. J. Bone and Joint Surg., 52-A :279-287, Mar. 1970.
8.Pardini, A. G., Radial dysplasia. Clin. Orthop., 57:153-177, 1968.
9.Riordan, D. C, Congenital absence of the radius. J. Bone and Joint Surg., 37-Ar 1129-1140, Dec. 1955.
10 Shands, A. R., Jr., and H. B. Eisberg, The incidence of scoliosis in the State of Delaware. A study of 50,000 minifilms of the chest made during a survey of tuberculosis. J. Bone and Joint Surg., 37-A: 1243-1249, Dec. 1955.
11. Wynne-Davies, Ruth, Familial idiopathic scoliosis. J. Bone and Joint Surg., 50-B: 24-30, Feb. 1968.