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小脑扁桃体 Chiari I 畸形手术后 1 年出现囊性变性:1 例病例报告及文献复习。

Cystic Degeneration of Cerebellar Tonsil 1 Year After Chiari I Malformation Surgery: A Case Report and Review of the Literature.

机构信息

Department of Neurosurgery, Faculty of Medicine, Tabriz University of Medical Sciences, Tabriz, Iran.

Department of Pathology, Tabriz University of Medical Sciences, Tabriz, Iran.

出版信息

World Neurosurg. 2019 Aug;128:501-505. doi: 10.1016/j.wneu.2019.04.241. Epub 2019 May 8.

Abstract

BACKGROUND

Chiari malformation results from a bony structural anomaly of the skull base. The structural defect causes downward displacement of the cerebellar tonsils through the foramen magnum. The herniated tonsils block the normal flow of cerebrospinal fluid, which causes a wide spectrum of clinical symptoms.

CASE REPORT

In May 2015, a 16-year-old girl was referred to our center because of a 1-year history of occipital headache, most often triggered by exercise and physical activity at school. She had experienced new-onset numbness in both hands, more severe on the right side, associated with some degrees of weakness. Eventually, an evaluation of her condition included magnetic resonance imaging in T1 and T2 sequences, which revealed a 20-mm downward migration of the cerebellar tonsils, associated with a cervical cord syrinx at the level of the fourth and fifth cervical vertebrae. The patient underwent posterior fossa decompression and C1 and partial C2 laminectomies. Postoperatively there were no complications, and the patient was discharged on day 3. Postoperatively, she experienced some improvement in her symptoms. After 2 months of routine outpatient follow-up, she was better, the headaches had subsided, she could resume some activities, and there was no paresis in her limbs.

CONCLUSIONS

In cases of progressive symptoms of Chiari malformation, surgical decompression is important and should be considered after shunt insertion to the hindbrain.

摘要

背景

Chiari 畸形是由颅底骨结构异常引起的。该结构缺陷导致小脑扁桃体通过枕骨大孔向下移位。疝出的扁桃体阻塞了正常的脑脊液流动,从而导致广泛的临床症状。

病例报告

2015 年 5 月,一名 16 岁女孩因 1 年来的枕部头痛就诊于我院,头痛多由运动和学校的体育活动诱发。她还出现了双手新发性麻木,右侧更为严重,伴有一定程度的无力。最终,对其病情进行了评估,包括 T1 和 T2 序列的磁共振成像,结果显示小脑扁桃体向下迁移了 20mm,伴有第四和第五颈椎水平的颈髓空洞。患者接受了后颅窝减压和 C1 及部分 C2 椎板切除术。术后无并发症,患者于第 3 天出院。术后,她的症状有所改善。在常规门诊随访 2 个月后,她的情况有所好转,头痛减轻,她可以恢复一些活动,四肢也没有无力。

结论

对于 Chiari 畸形进行性症状的患者,手术减压很重要,并且应在分流至后脑后考虑进行。

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