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罕见的中脑发育性静脉异常因导水管狭窄导致梗阻性脑积水。

Unusual mesencephalic developmental venous anomaly causing obstructive hydrocephalus due to aqueductal stenosis.

作者信息

Guhl Susanne, Kirsch Michael, Lauffer Heinz, Fritsch Michael, Schroeder Henry W S

机构信息

Department of Neurosurgery, Greifswald University, Greifswald, Germany.

出版信息

J Neurosurg Pediatr. 2011 Oct;8(4):407-10. doi: 10.3171/2011.7.PEDS114.

Abstract

Developmental venous anomalies (DVAs) are benign vascular malformations that rarely become symptomatic. They are anatomical variations of the venous drainage system and most are incidentally discovered. Mechanical (obstruction and compression of cerebral and neural structures) and flow-related pathological mechanisms have been described in rare cases of symptomatic DVAs. The authors present the case of a 10-month-old boy with a mesencephalic DVA compressing the aqueduct and causing occlusive hydrocephalus. Endoscopic inspection confirmed the venous malformation causing aqueductal stenosis. The authors successfully performed endoscopic third ventriculostomy, resulting in decrease in the size of the ventricles. At the 6-month follow-up after surgery, the patient had significantly progressed in his psychomotor development. One year postsurgery the patient is doing fine, with no neurological or developmental deficits.

摘要

发育性静脉异常(DVAs)是良性血管畸形,很少出现症状。它们是静脉引流系统的解剖变异,大多数是偶然发现的。在少数有症状的DVAs病例中,已描述了机械性(脑和神经结构的阻塞和压迫)和与血流相关的病理机制。作者报告了一例10个月大的男孩,患有中脑DVA压迫导水管并导致梗阻性脑积水。内镜检查证实静脉畸形导致导水管狭窄。作者成功实施了内镜下第三脑室造瘘术,脑室大小减小。术后6个月随访时,患者的精神运动发育有显著进步。术后一年患者情况良好,无神经或发育缺陷。

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