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脊髓圆锥部脂肪瘤相关的脊髓积水:病例报告。

Hydromyelia associated with spinal lipoma of the conus: case report.

机构信息

Division of Pediatric Neurological Surgery, Department of Pediatrics, University of Padua, Padua, Italy.

出版信息

Spine (Phila Pa 1976). 2010 Sep 15;35(20):E1069-71. doi: 10.1097/BRS.0b013e3181ed0b50.

Abstract

STUDY DESIGN

A case report and literature review of the treatment of "noncommunicating" syringomyelia. OBJECTIVE.: The aim of this report is to document the timing and the treatment of hydromyelia holocord after surgical treatment for both tethering and retethering of spinal lipoma.

SUMMARY OF BACKGROUND DATA

Syringomyelia associated with spinal lipoma presents a different pathogenesis and treatment in comparison to the "communicating" hydromyelia in the myelomeningocele. After the primary retethering operation performed in symptomatic patients, recurrent retethering can occur with an increase of the syringomyelia signs and symptoms.

METHODS

Syringomyelia treated with a thin silastic tube passed from the syrinx to the subarachnoidal space for drainage and decompression. Prior operations were: (1) initial untethering at birth, (2) second untethering at 5 years of age, (3) posterior fossa and cervical decompression.

RESULTS

Magnetic resonance imaging 6 months post shunt operation demonstrated decompression of the hydromyelia holocord and syringobulbia with improvement of motor function of the legs and improvement in sensory symptoms.

CONCLUSION

Usefulness of syrinx-subarachnoidal shunt is demonstrated in this case report after unsuccessful decompression and detethering. When the enlargement of the ependymal channel is greater than 50% of the spinal cord's diameter, neurologic, and urological symptoms are evident and the patient benefitted from cord untethering and syrinx drainage. (1) The terminal "noncommunicating" syringomyelia in lumbar sacral lipoma has been reported to be associated with retethering in spinal lipoma in the 25% of the cases. (2) The rise of distal syringomyelia isn't only linked to the kind of the spinal lipoma, but also to the difficulty to obtain the untethering and a smooth cerebrospinal fluid flow between the subarachnoidal space and the ependymal canal. (3) In patients with hydromyelia holocord greater than the 50% of the spinal cord's diameter a myelotomy and insert an ependymal channel/syrinx to the subarachnoidal space shunt can resolve of the syrinx. In this case, the enlargement of the ependymal channel in "noncommunicating" syringomyelia associated with lumbosacral lipoma is greater than 50% of the spinal cord's diameter; neurologic and urological symptoms occurred and the patient benefited from cord untethering and concurrent syrinx drainage.

摘要

研究设计

“非交通性”脊髓空洞症治疗的病例报告及文献复习。

目的

本报告旨在记录脊髓脊膜膨出术后脊髓空洞症全脊髓空洞形成的治疗时机和治疗方法,该病例患者同时存在脊髓栓系和再栓系。

背景资料总结

与脊髓脊膜膨出相关的脊髓空洞症与“交通性”脊髓空洞症的发病机制和治疗方法不同。在对有症状的患者进行初次松解术后,脊髓空洞症的体征和症状可能会再次出现,并伴有脊髓空洞症的复发。

方法

采用细硅胶管从脊髓空洞引流至蛛网膜下腔进行引流和减压。先前的手术包括:(1)出生时初次松解,(2)5 岁时第二次松解,(3)后颅窝和颈椎减压。

结果

分流术后 6 个月磁共振成像显示全脊髓空洞减压,下肢运动功能改善,感觉症状改善。

结论

在本例不成功的减压和松解后,显示出脊髓空洞蛛网膜下腔分流术的有效性。当室管膜通道的扩大超过脊髓直径的 50%时,会出现神经和泌尿症状,患者从脊髓松解和脊髓空洞引流中获益。(1)终末期“非交通性”脊髓空洞症与腰骶部脊膜膨出相关的脊髓栓系在 25%的病例中与脊髓脊膜膨出的再栓系有关。(2)远端脊髓空洞的上升不仅与脊髓脊膜膨出的类型有关,而且与获得松解和蛛网膜下腔与室管膜通道之间的脑脊液流畅度有关。(3)对于全脊髓空洞大于脊髓直径 50%的患者,行脊髓切开术并将室管膜通道/脊髓空洞插入蛛网膜下腔分流可解决脊髓空洞问题。在这种情况下,腰骶部脊膜膨出相关的“非交通性”脊髓空洞症的室管膜通道扩张大于脊髓直径的 50%;出现神经和泌尿症状,患者从脊髓松解和同时进行的脊髓空洞引流中获益。

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