The clinical manifestation and operative treatment for the scoliosis of Marfan's syndrome / 中华骨科杂志

ABSTRACT

Objective To evaluate the clinical manifestation and operative treatment for the scolio-sis of Marfan syndrome, and analyze its clinical outcomes as well. Methods The retrospective study cov-ered 10 patients including 8 females and 2 males with an average age of 12.9 years (9 to 16 years), who had undergone operations from January 1990 to December 2002. The Cobb's angle in the coronal and sagittal plane, the trunk shift, the apex rotation and shift were evaluated both before and after operation respectively. In the group, there were four cases with family history. Of the 10 cases, the classification of scoliosis was single curve in two cases, double curves in six cases and three curves in two cases. Results All the pa-tients were followed up for a mean time of 15 months, ranged from 4 months to 3 years. The trunk shift changed from the mean distance of 2.17 cm to 1.41 cm. The apex rotation decreased about 1?. The apex shift changed from the mean distance of 4.57 cm to 2.14 cm. The mean Cobb's angle in the coronal plane changed from 88? to 42?. The correction rate was about 46.38% (18.18% to 81.54%). The Cobb's angle on the final follow-up was 46?( 11? to 96?), and the mean lost angle was 4?. The mean Cobb's angle of the thoracic kyphosis was 20?(-10? to 52?) preoperatively and 23?(0? to 35?)postoperatively, and 24?( 0? to 35?) on the final follow-up. The Cobb's angles of the thoracolumbar kyphosis of 5 cases improved from 85? to 10? after operations, and there was no angle loss on the follow-up. Conclusion The three dimensional corrective instrumentation can be used effectively for the correction of the scoliosis of Marfan syndrome. The critical points for the successful operation are the simultaneous correction of the coronal and sagittal plane deformities, prevention of the dural expansion in the lumbar or lumbosacral canal, rigid and multi-segmental internal fixation, extensive and ample bone fusion, and correct external fixation after operation.