神经纤维瘤病Ⅰ型的头颈部CT和MRI诊断价值

CT and MRI diagnosis of craniocerebral and jugular neurofibromatosis - Ⅰ

目的探讨神经纤维瘤病Ⅰ型（NF-1）的CT和MRI表现，提高对该病的正确诊断率。方法回顾性分析33例经临床确诊和病理证实的神经纤维瘤病Ⅰ型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；多发脑膜瘤1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相结合能全面显示神经纤维瘤病Ⅰ型的头颈部病变，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. Methods The clinical symptoms and radiological appearances of 33 cases of neurofibromatosis - Ⅰ were analyzed retrospectively, which were confirmed by clinic and pathology. Results The imaging findings within 33 cases： 2 cases had single acoustic neuroma. One case had single optic nerve meningiomas and single meningiomas located in the convexity of the brain. Four cases had thickening of optic nerve. Six cases had augmentation and evagination （glaucoma） of the eyeballs. One case had multiple meningiomas. Three cases had bulky calcify in the choroidplexus. Two cases had tumours in the cranial region. Six cases had changes in the maxillofacial bones and entourage of them. Twenty - one cases had defect of the wings of the sphenoid and temporal lobe hernia. Eight cases had de- fect of other pats of the cranial bone. Two cases had tumours in the cervical deep part. Conclusions CT and MRI have important diagnostic value on NF -Ⅰ because they can find the pathological changes of head and neck roundly.