结节性硬化症眼部病变CT及MRI表现

目的描述结节性硬化症患者眼部病变CT、MRI影像表现，并评价其临床价值。设计回顾性病例系列。研究对象CT和MRI影像学检查发现眼部病变的7例结节性硬化症患者。方法经临床诊断及头部CT检查确诊结节性硬化症7例，通过眼眶CT平扫、MRI平扫及增强检查观察视网膜病变大小、形态、密度以及增强后强化情况；结合临床直接、间接眼底镜检查结果。主要指标眼球CT、MRI表现。结果头部CT平扫发现室管膜下及皮质多发钙化或，和非钙化结节7例（100％）。眼眶CT和／或MRI发现：眼底扁平型视网膜错构瘤4例（57．1％）；眼底分叶状或结节状视网膜错构瘤3例（42．9％）；错构瘤内可见钙化2例（28．6％）；患侧小眼球2例（28．6％）；增强后病变未见明显强化7例（100％）；随访检查发现视网膜错构瘤呈进行性生长1例（14．3％）。结论CT、MRI可以发现较大的视网膜错构瘤，结合颅脑检查对定性诊断有重要价值。对于进行性生长的视网膜错构瘤病例CT、MRI影像学的系统评价可以为选择适当的治疗方法提供依据。（眼科，2009，18：257-260）

Ophthalmic manifestations of tuberous sclerosis: CT and MRI findings

Objective To describe CT and MRI imaging findings of retinal hamartomas with tuberous sclerosis and evaluate their clinical value. Design Retrospective case series. Participants 7 patients of tuberous sclerosis complex (TSC) who have ocular lesions found with CT and MRI examination. Methods 7 patients with tuberous sclerosis were diagnosed by clinical data and head CT. The size, morphology, density and enhancing situation of the retinal lesions were observed with CT and MR.I, as while as with direct or indi-rect fundoscopy. Main Outcome Measures CT and MRI features of ocular lesions. Results 7 cases displayed intraeranial calcification and/or non-calcified tubercles, situating in subendyrna (7 cases, accounting for 100%) by head CT. Orbital CT and MRI showed 4 cases (57.1%) of flat retinal hamartomas 3 cases (42.9%) 9f retinal lobular and nodular hamartomas, 2 cases(28.6%) scattered calcification patches in hamartoma, and 2 cases (28.6%) of nanophthalmos. The lesions displayed unenhancement on contrast-enhanced MR images in 7 cases(100%) . There was 1 case (14.3%) of progressive retinal astrocytic hamartoma. Conclusion The examination of CT and MRI may find the bigger retinal hamartomas, which can play a very important role at the diagnosis with the help of whole brain examination. For progressive retinal nstrocytic hamartoma,systemic evaluation with CT and MRI can provide evidence for choosing appropriate thera-pies. (Ophthalmol CHN, 2009, 18: 257-260)