终丝牵拉综合征患儿临床表现和终丝病变类型的特点

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目的通过观察终丝牵拉综合征(TFTS)患儿临床表现和MRI显示终丝病变类型的特点,提高对TFTS的认识,为TFTS临床诊断与治疗提供依据。方法回顾性分析37例TFTS患儿临床资料,包括体格检查、全脊柱X线片、全脊柱螺旋CT、全脊髓MRI、尿流动力学、术后病理结果及术后恢复效果。根据MRI终丝影像学表现,将其分为2组:一组为终丝脂肪变,另一组为终丝增粗(直径>2 mm),比较2组患儿之间的差异。结果 37例TFTS患儿的MRI显示终丝脂肪变24例(64.9%),终丝增粗(直径>2 mm)13例(35.1%)。2组患儿在下肢畸形、脊柱发育畸形和术后恢复效果方面无明显差异,但在腰背部皮肤异常和大小便功能障碍方面有较明显差别,腰骶部皮肤异常改变23例(62.3%)。其中终丝增粗组5例(13.5%),终丝脂肪变组18例(48.8%);大小便功能障碍26例(70.2%)。其中终丝增粗组7例(18.9%),终丝脂肪变组19例(51.3%)。37例患儿尿流动力学均提示有异常。术后病理结果提示终丝脂肪及纤维组织增生。结论 TFTS患儿的终丝存在异常改变,MRI显示终丝病变类型多为直径增粗和脂肪变,病变终丝切断对改善症状有效。诊断TFTS需综合临床信息,其中尿流动力学和MRI是诊断的关键指标。 Objective To observe the clinical manifestations of children with terminal suture pull-off syndrome (TFTS) and the characteristics of the type of terminal lesion by MRI, and to improve the understanding of TFTS and provide the basis for the clinical diagnosis and treatment of TFTS. Methods Retrospective analysis of 37 cases of TFTS clinical data, including physical examination, total spine X-ray, Spinal CT, spinal cord MRI, urodynamics, postoperative pathological results and postoperative recovery. According to MRI end-stage radiographic findings, they were divided into two groups: one was terminal steatosis and the other was terminal thickened (diameter> 2 mm). The differences between the two groups were compared. Results Thirty-seven patients with TFTS showed thirteen cases (64.9%) of terminal steatosis and 13 (35.1%) of terminal thickened (> 2 mm diameter) patients. There were no significant differences between the two groups in the lower limb deformity, spine deformity and postoperative recovery effect, but there were significant differences in the skin abnormalities and urinary dysfunction in the lower back. Twenty-three patients (62.3%) had abnormal skin lumbosacral changes, . Among them, 5 cases (13.5%) were terminal suture thickening group, 18 cases (48.8%) were terminal sliver steatosis group, and 26 cases (70.2%) were stool dysfunction. Among them, 7 cases (18.9%) were terminal suture thickening group and 19 (51.3%) terminal fat slipping group. Urinary flow dynamics in 37 children were all suggestive of abnormalities. Postoperative pathological findings prompted the terminal fat and fibrous tissue hyperplasia. Conclusion There is an abnormal change in the terminal filament of TFTS. MRI shows that the type of terminal filamentous lesion is mostly diameter enlargement and steatosis. The end of filament lesion is effective in improving symptoms. Diagnosis of TFTS need to be integrated clinical information, including urodynamics and MRI are key indicators of diagnosis.
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