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特发性双侧孟氏孔闭塞:一种临床表现多样的罕见病症。

Idiopathic bilateral occlusion of the foramen of Monro: An unusual entity with varied clinical presentations.

作者信息

Mizrahi Cezar J, Cohen José E, Gomori J M, Shoshan Yigal, Spektor Sergey, Moscovici Samuel

机构信息

Department of Neurosurgery, Hadassah-Hebrew University Medical Center, P.O. Box 12000, Jerusalem 91120, Israel.

Department of Neurosurgery, Hadassah-Hebrew University Medical Center, P.O. Box 12000, Jerusalem 91120, Israel; Department of Radiology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.

出版信息

J Clin Neurosci. 2016 Dec;34:140-144. doi: 10.1016/j.jocn.2016.05.015. Epub 2016 Aug 31.

Abstract

We review our experience with four patients who presented to our Medical Center from 2005-2015 with adult idiopathic occlusion of the foramen of Monro (FM). All patients underwent CT scanning and MRI. Standard MRI was performed in each patient to rule out a secondary cause of obstruction (T1-weighted without- and with gadolinium, T2-weighted, fluid-attenuated inversion recovery [FLAIR] and diffusion-weighted imaging [DWI] protocols). When occlusion of the FM appeared to be idiopathic, further high-resolution MRI with multiplanar reconstructions for evaluation of stenosis or an occluding membrane at the level of the FM was performed (T1-weighted without- and with gadolinium, T2-weighted 3D turbo spin-echo). Occlusion of the FM was due to unilateral stenosis and septum pellucidum deviation in two patients, to an occluding membrane in one, and to bilateral stenosis in one patient. Urgent surgical intervention is mandatory when there are signs of increased intracranial pressure while asymptomatic patients may be managed conservatively. In this patient series, truly bilateral stenotic obstruction of the FM was best managed with ventriculoperitoneal shunt and patients with membranous obstruction or unilateral stenosis with septum deviation were treated endoscopically.

摘要

我们回顾了2005年至2015年期间到我们医疗中心就诊的4例成人特发性Monro孔(FM)闭塞患者的治疗经验。所有患者均接受了CT扫描和MRI检查。对每位患者进行标准MRI检查以排除梗阻的继发原因(T1加权平扫及增强、T2加权、液体衰减反转恢复序列[FLAIR]和弥散加权成像[DWI]方案)。当FM闭塞看似为特发性时,进一步行高分辨率MRI及多平面重建以评估FM水平的狭窄或闭塞膜(T1加权平扫及增强、T2加权三维快速自旋回波序列)。FM闭塞的原因在2例患者中为单侧狭窄和透明隔偏移,1例为闭塞膜,1例为双侧狭窄。当出现颅内压升高迹象时,紧急手术干预是必要的,而无症状患者可采取保守治疗。在这个患者系列中,真正的双侧FM狭窄性梗阻最好采用脑室腹腔分流术治疗,而膜性梗阻或伴有透明隔偏移的单侧狭窄患者则采用内镜治疗。

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