摘要
目的探讨神经纤维瘤病I型(NF1)累及眶部的MRI特征。方法回顾性分析2004年1月至2011年5月首都医科大学附属北京同仁医院80例符合国际医疗组织临床诊断标准的NF1型患者眼眶MRI表现,86侧眼眶受累。80例患者均行眼眶和(或)头颅MR成像,其中71例同时行增强扫描。结果79例85侧眶内从状神经纤维瘤,累及颞窝61侧,海绵窦61侧,翼腭窝51侧,颞下窝31侧,增强后71侧轻度一中等强化;蝶骨大翼发育不良所致眶壁骨质缺损79侧,蝶骨小翼发育不良72侧;眼球增大32侧,视神经胶质瘤5侧;伴发脑实质多发T2WI高信号灶53例。结论NF1容易累及眼眶,MRI可直接显示眶部受累范围以及伴发的其他异常。
Objective To explore the magnetic resonance imaging (MRI) features of neurofibromatosis type 1 (NF1) with orbital involvement. Methods We retrospectively reviewed the MRI findings of orbital abnormalities in 80 NF1 patients (86 orbits). The diagnosis was confirmed by the updated National Institute of Health ( NIH ) criteria. All of them underwent MRI scans of orbit or brain while 71 patients had contrast enhancement. Results The orbital abnormalities were documented in 80 patients. And 79 cases (85 orbits) had plexiform neurofibroma in orbit and/or adjacent regions. The orbital involvements extended to superficial temporal fossa ( n = 61 ) , infratemporal fossa ( n = 31 ) , cavernous sinus ( n = 61 ) and pterygopalatine fossa (n = 51 ). There was mild-to-moderate enhancement in 71 orbits on contrastenhanced MRI examinations. There were localized defects of orbital walls because of sphenoid bone dysplasia of greater wing (n =79), sphenoid bone dysplasia of lesser wing (n = 72), augmentation (glaucoma) of eyeballs (n = 32) and optic nerve gliomas (n = 5 ). And 53 cases had hyperintense lesions on T2-weighted brain images. Conclusion Orbital involvement is common for NF1. And MRI is a useful tool of evaluating the extent of involvements and other abnormalities in the NF1 patients.
出处
《中华医学杂志》
CAS
CSCD
北大核心
2012年第29期2042-2045,共4页
National Medical Journal of China