Iliolumbar Fusion In The Management Of Sacral Agenesis A Preliminary Report

Earl E. VanDerwerker, Jr., M.D.

Typically the management of a child with sacral agenesis entails amputation of the afunctional lower extremities and the subsequent fitting of bilateral prostheses to permit ambulation. The majority of these children have died at a relatively early age, primarily because renal complications, secondary to neurogenic and mechanical problems, have resulted in chronic renal infection.

Recently, when we had the opportunity to treat a case of sacral agenesis, we decided to attempt to stabilize the pelvis and thus prevent it from telescoping into the abdominal cavity. If this stabilization could be accomplished, we believed that the kinking of the ureters would be decreased and the patient's vital capacity would be improved when he was ambulating.

The patient was four years and one month of age at the time of surgery and exhibited the typical findings of flexion contractures at the hips and knees (Fig. 1 and 2 ). Little sensory perception and no motor function were present in the lower extremities. Bowel and bladder functions were impaired, although the patient did have some voluntary control.

On August 20, 1965, bilateral subtrochanteric amputations of the lower extremities were performed (Fig. 3 ). The lower extremities were "filleted" and the bone preserved in the bone bank. Approximately one month later (September 24, 1965), an iliolumbar stabilization was attempted through a mid-line incision using the autogenous bone. The procedure was somewhat difficult technically because of the anterior placement of the ilia as compared with the normal. The possibility of placing a graft between the ilia was discarded when a large mass of fibrofatty tissue was found to be interposed. Placement of a graft would have necessitated excision of this mass, in which lumbar and sacral roots might be present (Fig. 4 , November 9, 1965).

The postoperative course of the amputation was uneventful. For five months immobilization with a body jacket was attempted. Although minimal support was obtained, the jacket at least helped maintain recumbency. X-rays showed progressive absorption or revascularization of the graft (Fig. 5 -8 ). However, a considerable amount of stability has been obtained. Although the pelvis is permitted to move anteriorly in relation to the spine like a hinge joint, the marked telescoping in the longitudinal plane (such as occurred preoperatively) is no longer present.

Intravenous pyelograms were taken on June 4, 1965 (Fig. 9 ), and when compared with those taken six months later on January 12, 1966 (Fig. 10 ), showed an apparent decrease in the mobilization of the ureters, accomplishing, we hope, a part of the goal for which the procedure was undertaken.

The child has been fitted with a prosthetic appliance consisting of a bucket with bilateral Canadian hip joints. Knee joints have not yet been added (Fig. 11 ). He is now beginning ambulation with a swing-through gait.

Earl E. VanDerwerker, Jr., M.D. is associated with the Newington Hospital for Crippled Children Newington, Connecticut