神经纤维瘤病眼眶受累影像学诊断(附12例分析)

目的：探讨神经纤维瘤病眼眶受累的影像学表现。方法：回顾性分析12例经病理或临床表现和影像学证实的神经纤维瘤病眼眶受累患者的影像学资料,12例均行眼眶CT检查,其中5例行CT增强,6例行MRI或增强检查。结果：12例神经纤维瘤病病例中,Ⅰ型8例,Ⅱ型4例。眼眶内受累10例,视神经受累2例,眼球内受累3例,眼环增厚伴点状钙化1例,泪腺2例,视神经孔2例,海绵窦2例,眼睑部6例。CT表现为眶内不规则形等密度影,眼球内半月形等密度影,视神经孔开大,邻近骨质缺损或增生硬化。T1WI上病灶呈等信号,T2WI上呈高信号,增强后中度增强。MRI增强后脂肪抑制序列更能清晰显示病变。结论：神经纤维瘤病眼眶受累的CT和MRI表现多种多样,结合临床和其他部位影像学资料,可提高其诊断符合率。

Imaging diagnosis of neurofibromatosis with orbit involvement (analysis of 12 cases)

Objective：To discuss the imaging manifestations of neurofibromatosis with orbit involvement.Methods：The clinical and imaging manifestations of 12 cases with pathology/clinical and imaging materials confirmed neurofibromatosis with orbit involvement were analyzed retrospectively.All of the 12 patients had CT scanning of orbit,5 patients had contrast enhancement;MRI or contrast enhanced scanning were performed in 6 patients.Results：Of the 12 patients with neurofibromatosis,there were typeⅠ（8 patients）,type Ⅱ（4 patients）;intraorbital involvement（10 patients）,optic nerve involvement（2 patients）,intraocular involvement（3 patients）,thickening of wall of eyeball with spotty calcifications（1 patient）,involvement of lacrimal gland and cavernous sinus（2 patients for each）,eyelids involvement was showed in 6 patients.CT findings included intra-orbital irregular iso-attenuated shadows,intraocular iso-attenuated crescent shaped mass,enlargement of optic foramen and adjacent bone defect or hyperostosis.The lesions were iso-intense on MR T1WI and hyper-intense on T2WI,moderately enhanced after contrast administration.Lesions could be more clearly assessed on post-constrast fat-suppressed sequence.Conclusion：Various CT and MRI manifestations of neurofibromatosis with orbit involvement could be revealed,the diagnostic accuracy could be improved as the imaging findings were in combination with clinical data and imaging materials of the other parts of the body.