SPINAL-FUSION IN DUCHENNES MUSCULAR-DYSTROPHY

The Women's and Children's Hospital experience with Luque spinal fusio n in Duchenne's muscular dystrophy was reviewed from its Commencement in 1983 to the present with a view to assessing the clinical and radio logic outcome and safety of the procedure. Seventeen boys have undergo ne spinal fusion. L-rod instrumentation was used in 10, six of whom ha d significant problems with sitting imbalance or progression of the sc oliosis or both. In seven cases, distal instrumentation was taken to t he pelvis with a Galveston construct and rigid crosslinking. Apart fro m some progression and sitting imbalance in the L-rod group, there wer e few complications. In the Galveston group, pelvic obliquity was corr ected by a mean of 63%, and there was better maintenance of correction . There were no pseudoarthroses or instrument failures in the Galvesto n group. Of the total group, four patients had forced vital capacity ( FVC) values <25% predicted, and two required ventilation postoperative ly (<48 h). There were no other respiratory complications. The effect of surgery on respiratory function remains uncertain. Spinal fusion wi th the Luque rod construct and pelvic fixation is a safe procedure. It provided a mean correction of 60% and control of pelvic obliquity wit hout significant postoperative deterioration. In our experience, surge ry can be safely performed with FVC values down to 20% predicted. On t he basis of these data, our current practice is to instrument to the p elvis with a Galveston construct and Texas Scottish Rite Hospital cros s-linking.