Nursing Care in the Treatment of a Child With Lumbosacral Agenesis

YVONNE MAR, R.N. MARCELLE LAURENDEAU, R.N. JEANNETTE HUTCHISON, O.T.


In the past 10-15 years a number of articles dealing with congenital absence of the lumbar spine and sacrum have appeared in the literature 1,2,3,4,5 . Invariably, these articles have been concerned primarily with etiological, surgical and prosthetic considerations. It is obvious, however, that children with these defects have multiple problems. These problems are frequently so diverse that their solution requires a closely coordinated team effort on the part of all personnel involved in the treatment program.

This report describes the care of a young child with lumbosacral absence and emphasizes the role of the pediatric nurse as a member of the treatment team.

Case History

CP., a female now three years old, was born without lumbar spine and sacrum, and with consequent paraplegia of the lower limbs. Her hips were flexed at 80 deg. and there was severe webbing in the popliteal areas.

Weighing only 3 lb. 10 oz. at birth, CP. was transferred to a children's hospital where she remained until she was referred to the Rehabilitation Institute of Montreal (RIM) for evaluation and treatment.

At the time of her admission, the child was 37 weeks old, weighed 10 lbs., was alert and normally nourished. She suffered fecal incontinence, the semiliquid stool dripping constantly by gravity.

Upon her arrival at RIM, a program to stimulate and develop her tolerance in the standing position and encourage her psycho-motor development was formulated. She was, and still is, followed regularly by our pediatric and urology consultants.

To develop standing balance the child was put into a vitrathene "bucket" which extended to her thorax. This "bucket" was built at our prosthetic laboratory and later served as the socket for a swivel walker ( Fig. 1 ).

At first, the child was encouraged to maintain the vertical position in the O.T. Department. When her tolerance had reached a reasonable level, the training was continued in the pediatric ward until she was able to maintain that position for approximately one hour without complaint.

During her stay at RIM, CP. suffered frequent urinary infections and was referred for genitourinary investigation. An intravenous pyelogram performed on May 22, 1970, showed a derotation of the two ureters and the beginning of damage to the left kidney. In August 1970, the child was referred to a children's general hospital, for investigation of the urinary system. An ileal loop conduit was recommended and was done in the same month ( Fig. 2 ).

CP. was readmitted at the Rehabilitation Institute of Montreal on Oct. 5, 1970. After a period of readjustment to the surroundings she continued standing training in her swivel walker which was provided with a window to prevent pressure on the ileal bag. It could remain in place for three days. Because of her fecal incontinence, her diapers had to be changed frequently and frequent irritations of her buttocks required constant nursing care. CP. has developed normally considering her physical condition

The strength of C.P.'s upper limbs had increased sufficiently to allow her to move about on the floor when she was not using the swivel walker. She could bring herself from the lying to a sitting position. Her vocabulary was now sufficient to allow her to communicate. Her legs were still flexed and without sensitivity and therefore were obstacles to moving about ( Fig. 3 ).

Because of these latter factors, amputation of the lower limbs was recommended in order to permit the child to be more mobile and eventually use prostheses for walking.

Following this decision, the patient and her parents were prepared for the amputations in close collaboration with the staff psychologists. At their suggestion, CP. was given a doll which had had both legs amputated. She was also prepared for a temporary separation with the pediatric staff to whom she was strongly attached.

Surgery

On Apr. 22, 1971, CP. was again transferred to a children's general hospital. On May 11, 1971, she underwent a left hip disarticulation and on June 29, 1971, the same operation was performed on the right hip. The child suffered septicemia and remained at the hospital until she had completely recovered ( Fig. 4 ).

During her stay at the general hospital, the RIM pediatric staff visited their young patient frequently in order to maintain good relationships.

On Aug. 3, 1971, CP. was readmitted at the Rehabilitation Institute of Montreal. She adapted herself easily to the surroundings and to staff members whom she recognized spontaneously. She was talking fairly well and could be understood. She resumed all her treatments and became increasingly agile now that her legs were no longer an obstacle ( Fig. 5 ). She climbed up on her bed and went up and down from her wheelchair independently.

In January 1972, she was measured for two hip-disarticulation prostheses. The "bucket" used was similar to the one she had worn previously on her swivel walker, but this one was made of laminated plastic. The limbs had a hip joint which was very stable and the artificial feet were shod with white boots ( Fig. 6 ).

The prostheses were well accepted by CP., and she was given gait training first in O.T., then on the ward. She now moves about easily with her prostheses, although at times she refuses to wear the limbs because she can perform some activities better without them.

She is still followed by our urologist and the ileal bag is no longer a problem. When she has grown up, she will learn how to install it herself. Her fecal incontinence still persists but defecation is less frequent. The stools are more solid which facilitates the prevention of skin irritations. We have tried without success different means of reducing the frequency of stools, medicinally with "Kaopectate," and mechanically with "Tampax."

CP. stayed at our Institute much longer than was expected, but for social reasons the long stay could not be avoided. She is now three and a-half years old, is intelligent, active and partially independent in her activities of daily living. Her prostheses will need to be modified or changed as she grows up. This child should be able to live normally and not be dependent because of her malformations.

Conclusion

Nursing staff members should participate actively in the rehabilitation program of any physically disabled child who is hospitalized, and should be integral members of the rehabilitation team, particularly in dealing with children who have multiple handicaps.

O.T. REG. Rehabilitation Institute of Montreal Montreal, Quebec, Canada

O.T. REG. Rehabilitation Institute of Montreal Montreal, Quebec, Canada

O.T. REG. Rehabilitation Institute of Montreal Montreal, Quebec, Canada

References:
1. Blumel, Johanna, E. B. Evans, and G. W. N. Eggers, Partial and complete agenesis or malformation of the sacrum with associated anomalies: Etiologic and clinical study with special reference to heredity: A preliminary report J. Bone and Joint Surg., 41-A:3:497-518, April 1959
2. Elting, James J., and John C Allen, Management of the young child with bilateral anomalous and functionless lower extremities. J. Bone and Joint Surg., 54-A:7:1523-1530, October 1972.
3. Frantz, Charles H? Complete absence of the lumbar spine and sacrum. Selected lower-limb anomalies?surgical and prosthetics management. A symposium. National Academy of Sciences, Washington, D.C., 1971 pp 29-48.
4. Frantz, C. H., and G. T. Aitken, Complete-absence of the lumbar spine and sacrum. J, Bone and Joint Surg., 49-A:8:1531-1540, December 1967.
5. Russell, H. E., and G. T. Aitken, Congenital absence of the sacrum and lumbar vertebrae with prosthetic management: A survey of the literature and presentation of five cases. J. Bone and Joint Surg., 45-A:3:501-508, April 1963
Descriptors: nursing; lumbosacral agenesis; prostheses; swivel walker.