Increase in dural ectasia size in scoliosis patients with Marfan syndrome.

Abstract

Increased dural ectasia and vertebral thinning in the lumbosacral spine are common in Marfan syndrome. Dural ectasia is unchanged in middle-aged patients, who have stopped growing. Therefore, evaluation from childhood to adulthood is mandatory. Sixteen patients [four men and 12 women; mean age:17.7 (8.6-33.5) years] with dural ectasia at final follow-up [mean follow-up: 10.4 (5.0-16.7) years] were enrolled. Dural sac diameter (DD)/vertebral diameter (VD) were measured on MRI or CT images of midline sagittal slices at the level of dural ectasia between L5 and S3. The widest part of the dural sac was evaluated with Fattori grading: grade 0, normal; 1, mild; 2, moderate; and 3, severe dural ectasia. At final follow-up, dural ectasia was observed at L5 in 3, S1 in 9, S2 in 14, and S3 in 11 patients. Respective changes in DD/VD from primary to final follow-up were L5: 21.7-24.2 mm/29.1-27.9 mm; S1: 17.0-18.6 mm/21.0-19.5 mm; S2: 15.5-19.6 mm (P < 0.01)/9.6-7.5 mm; and S3: 15.6-17.9 mm (P < 0.05)/6.2-3.3 mm (P < 0.05). Four patients had grade 0 dural ectasia, seven had grade 1, two had grade 2, and three had grade 3. Grade was increased in nine patients with juvenile to young adult patients but did not with adults. During follow-up, dural ectasia size changed in the juvenile to young adult patients with Marfan syndrome, particularly at the S2-S3 level in our series.