Severe Spondylolisthesis and Scoliosis in Association with Marfan??s Syndrome

Abstract

Scoliosis in Marfan's syndrome may be painful, but severe spondylolisthesis with a cauda equina tension syndrome as the cause of pain has only recently been recognized. Scoliosis may be associated with spondylolisthesis. The scoliosis may be sciatic or structural. These may be true structural curves or secondary to the asymmetric slip of the spondylolithesis. Grade 4 spondylolithesis was treated with an in situ ala-transverse fusion combined with posterior decompression. The tension signs were rapidly dissipated. A plaster spica was applied for six months. Solid fusion was achieved without progression of spondylolisthesis or further rotation. Subsequently, the structural scoliosis was treated with a two-stage anterior release and posterior Harrington fusion. The progression of spondylolisthesis in Marfan syndrome may be related to the poor quality of the surrounding musculoligamentous structures. Sciatic scoliosis resolves after fusion of the spondylolithesis. The results of in situ fusion are satisfactory. Reduction of the spondylolithesis with combined anterior and posterior fusion offers the most satisfactory basis for subsequent treatment of a structural scoliosis but carries a significant risk of neurologic damage and recurrence of slip.