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1型神经纤维瘤病患儿肋骨小头脱位致椎管狭窄

Rib head dislocation causing spinal canal stenosis in a child with neurofibromatosis, type 1.

作者信息

Chen Alan M, Neustadt Jeffrey B, Kucera Jennifer Neville

机构信息

Department of Radiology, University of South Florida College of Medicine, Tampa, FL, USA.

Department of Orthopedic Surgery, Johns Hopkins All Children's Hospital, St. Petersburg, FL, USA.

出版信息

J Radiol Case Rep. 2017 Aug 31;11(8):8-15. doi: 10.3941/jrcr.v11i8.3113. eCollection 2017 Aug.

Abstract

A 10-year-old female with neurofibromatosis type 1 and severe dystrophic scoliosis presented with a two-month history of difficulty ambulating due to low back pain. The patient did not have any neurological symptoms. MRI of the thoracolumbar spine demonstrated subluxation of the right posterior tenth and eleventh ribs through their respective neural foramina, with mild mass effect on the thecal sac without abnormal cord signal or cord compression. Groups of neurofibromas were present along the right ribs and paravertebral soft tissues around these levels. CT evaluation was completed for greater osseous definition. Cases of rib head dislocation into the central canal in the setting of dystrophic scoliosis were documented in only a limited sample of case reports. The angulated short-segment curvature in dystrophic scoliosis causes vertebral body rotation, foraminal enlargement, spindling of transverse processes, and penciling of the apical ribs. These changes can alter the articulation of the rib along the transverse process. The enlarged foramina can also create a larger space into which a rib may displace. As a result, in most reported cases, the subluxed ribs were on the convex apex of the curve in the mid-to-lower thoracic region. The risk of cord injury from rib head dislocation makes the complete depiction of the anatomy essential for proper surgical management.

摘要

一名患有1型神经纤维瘤病和严重营养不良性脊柱侧凸的10岁女性,因腰痛出现行走困难2个月。患者无任何神经症状。胸腰椎MRI显示右侧第十和十一肋骨通过各自的神经孔半脱位,对硬脊膜囊有轻度占位效应,但脊髓信号无异常或脊髓受压。在这些层面的右侧肋骨和椎旁软组织中可见神经纤维瘤群。为了更清晰地显示骨质情况,完成了CT评估。在营养不良性脊柱侧凸背景下肋骨头部脱位至椎管内的病例仅在有限的病例报告样本中有记录。营养不良性脊柱侧凸的成角短节段弯曲会导致椎体旋转、椎间孔扩大、横突纺锤样变以及顶椎肋骨铅笔样变。这些改变会改变肋骨与横突的关节连接。扩大的椎间孔也会形成一个更大的空间,肋骨可能会移位到这个空间中。因此,在大多数报道的病例中,半脱位的肋骨位于胸段中下部曲线的凸侧顶点。肋骨头部脱位导致脊髓损伤的风险使得完整描绘解剖结构对于正确的手术管理至关重要。

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本文引用的文献

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