Department of Orthopaedic Surgery and Spine Center, Meijo Hospital.
Department of Orthopaedic Surgery, Nagoya University Graduate School of Medicine, Nagoya, Japan.
J Pediatr Orthop B. 2021 May 1;30(3):235-238. doi: 10.1097/BPB.0000000000000745.
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.
在马凡综合征中，腰骶部脊柱的硬脑膜扩张和椎体变薄很常见。在停止生长的中年患者中，硬脑膜扩张保持不变。因此，必须从儿童期到成年期进行评估。最终随访时发现 16 例硬脑膜扩张患者[4 名男性和 12 名女性；平均年龄：17.7（8.6-33.5）岁]。最终随访时平均随访时间为 10.4 年（5.0-16.7 年）。在硬脑膜扩张水平的 L5 至 S3 中线矢状位切片上的 MRI 或 CT 图像上测量硬脑膜囊直径（DD）/椎体直径（VD）。使用 Fattori 分级评估硬脑膜囊最宽处：0 级，正常；1 级，轻度；2 级，中度；3 级，严重硬脑膜扩张。最终随访时，L5 处观察到硬脑膜扩张 3 例，S1 处 9 例，S2 处 14 例，S3 处 11 例。从初次随访到最终随访，DD/VD 的相应变化为：L5：21.7-24.2mm/29.1-27.9mm；S1：17.0-18.6mm/21.0-19.5mm；S2：15.5-19.6mm（P<0.01）/9.6-7.5mm；S3：15.6-17.9mm（P<0.05）/6.2-3.3mm（P<0.05）。4 例患者硬脑膜扩张为 0 级，7 例为 1 级，2 例为 2 级，3 例为 3 级。9 例青少年至年轻成年患者的分级增加，但成年患者未增加。在随访期间，我们系列中青少年至年轻成年马凡综合征患者的硬脑膜扩张大小发生了变化，特别是在 S2-S3 水平。
