先天性蛛网膜囊肿致慢性小脑扁桃体下疝并梗阻性脑积水1例
APPLIED JOURNAL OF GENERAL PRACTICE(2004)
Abstract
1 病例介绍
患者女性,17岁,因头痛1年加重伴行走不稳1月余入院,查体:神志清,双侧瞳孔等大等圆,光反应灵敏,眼球水平性震颤,咽反射减弱,颈部活动受限,四肢肌力Ⅳ级,腱反射亢进,双Babianki(一),指鼻不准,Romberg征(+).MRI示脑室系统均扩大,侧脑室最宽径达4cm,三脑室球形扩大约2.3×3cm,以小脑幕缘为基底于小脑蚓部见3.2×2.8cm大小囊肿,呈脑脊液信号,小脑扁桃体疝入环椎.入院5天后行手术治疗,先行脑室腹腔分流术,再行后颅窝减压及小脑囊肿~枕大池分流术,术中探查见小脑幕发育不良,呈窄条镰状,小脑幕切迹扩大,见小脑扁桃体已疝入环椎下缘,枕大池受压移位.咬除部分环椎后弓,打开小脑蚓部蛛网膜囊肿,将两端带多孔的分流管置于囊肿与枕大池之间并固定在蛛网膜上,10天后复查MRI示小脑囊肿消失,脑积水明显改善,各基底池显影清楚,头痛及行走不稳症状消失.
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