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[对从患有 Chiari I 型畸形合并脊髓空洞症患者切除的小脑扁桃体疝进行组织病理学分析]

[Histopathological analysis of herniated cerebellar tonsils resected from the patients with Chiari type I malformation with syringomyelia].

作者信息

Koga H, Mukawa J, Nakata M, Miyazato H, Ishikawa Y, Sakuta O, Hokama A, Terada Y

机构信息

Department of Neurosurgery, University of the Ryukyus School of Medicine, Okinawa, Japan.

出版信息

No To Shinkei. 1995 Nov;47(11):1075-9.

PMID:7495613
Abstract

Cerebellar tonsillectomy is a controversial treatment for Chiari type I malformation combined with syringomyelia. To demonstrate the validity of this procedure, we evaluated the postoperative clinical course, the histopathological finding in the resected cerebellar tonsils and the MR images of four patients. The patients were all women, ranging in age from 27 to 58 years old (mean: 43 years). The postoperative follows-up period lasted from 28 to 51 months (average: 38 mos), and the neurological symptoms and signs improved in all patients. Histopathological examination of the resected tonsils revealed a loss of Purkinje cells and granule cells. Vacuolated degeneration and chromatolysis were also seen, and modified Bielschowsky stain revealed axonal degeneration. These findings appeared to be irreversible. Postoperative T1-weighted MR images of the cervical and thoracic spine demonstrated a decrease in the size of the syrinx and the disappearance of evidence of tonsillar herniation in all patients. Postoperative phase-contrast MR images showed good CSF pulsation in the subarachnoid space at the craniocervical junction in all three patients examined. It was possible to avoid surgical complications by using careful microsurgical techniques. Based on these results, we concluded that cerebellar tonsillectomy is an adequate surgical strategies for treating Chiari type I malformation associated with syringomyelia.

摘要

小脑扁桃体切除术是治疗 Chiari I 型畸形合并脊髓空洞症的一种存在争议的治疗方法。为了证明该手术的有效性,我们评估了 4 例患者的术后临床病程、切除的小脑扁桃体的组织病理学发现以及磁共振成像(MR)图像。患者均为女性,年龄在 27 至 58 岁之间(平均:43 岁)。术后随访期为 28 至 51 个月(平均:38 个月),所有患者的神经症状和体征均有改善。对切除扁桃体的组织病理学检查显示浦肯野细胞和颗粒细胞缺失。还可见空泡变性和染色质溶解,改良 Bielschowsky 染色显示轴索变性。这些发现似乎是不可逆的。颈椎和胸椎的术后 T1 加权 MR 图像显示,所有患者的脊髓空洞大小减小,扁桃体疝的迹象消失。术后相位对比 MR 图像显示,在接受检查的所有 3 例患者中,颅颈交界处蛛网膜下腔的脑脊液搏动良好。通过采用精细的显微外科技术可以避免手术并发症。基于这些结果,我们得出结论,小脑扁桃体切除术是治疗与脊髓空洞症相关的 Chiari I 型畸形的一种合适的手术策略。

相似文献

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[Histopathological analysis of herniated cerebellar tonsils resected from the patients with Chiari type I malformation with syringomyelia].[对从患有 Chiari I 型畸形合并脊髓空洞症患者切除的小脑扁桃体疝进行组织病理学分析]
No To Shinkei. 1995 Nov;47(11):1075-9.
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Histological findings in cerebellar tonsils of patients with Chiari type I malformation.
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Childs Nerv Syst. 2007 Apr;23(4):427-9. doi: 10.1007/s00381-006-0252-y. Epub 2006 Nov 22.