神经纤维瘤病Ⅰ型的头颈部CT和MRI表现

CT and MRI Diagnosis of Craniocerebral and Jugular Neurofibromatosis-type 1

目的探讨神经纤维瘤病Ⅰ型(NF-1)的CT和MRI表现。资料与方法回顾性分析33例经病理证实的NF-1患者的临床资料及头颈部CT、MRI特征。结果单侧听神经瘤2例;单侧视神经瘤1例及单发凸面脑膜瘤1例;视神经增粗4例;眼球体积增大伴外凸(青光眼)6例;脉络丛粗大钙化3例;面颊部肿块2例;颌面骨及周围改变6例;蝶翼缺损及颞叶疝21例,其他部位颅骨缺损8例;颈深部肿块2例。结论CT与MRI相结合能全面显示NF-1的头颈部病变,对NF-1具有重要诊断价值。

Objective To study the CT and MRI features of neurofibromatosis type 1 in order to improve the diagnostic accuracy.Materials and Methods The clinical symptoms and radiological appearances of 33 cases of neurofibromatosis type1,which were confirmed by clinical and pathological data,were analyzed retrospectively.Results The imaging findings of 33 cases included solitary acoustic neuroma in 2 cases,single optic nerve meningiomas in 1 case,single meningiomas located in the convexity of the brain in 1 case,thickening of optic nerve in 4 cases,augmentation and evagination（glaucoma） of the eyeballs in 6 cases,bulky calcify of the choroid plexus in 3 cases,tumours in the cranial region in.2 cases.6 cases had deformities in the maxillofacial bones and surrounding area.21 cases had defect of the sphenoid wings and temporal lobe hernia.8 cases had defect of other parts of the cranial bone.2 cases had tumours in the cervical deep part.Conclusion CT combined with MRI have important value in the diagnosis of NF-1.