【摘要】 目的 探讨神经纤维脂肪性错构瘤(FLH)的MRI表现。方法 回顾性分析2011年1月～2021年8月10例FLH的临床及MRI资料。男7例,女3例,年龄1～37岁,中位年龄21.5岁。10例患者均行MRI平扫。结果 9例累及正中神经,MRI表现为正中神经远段及分支增粗,在轴位MRI图像上,增厚的神经外膜包裹多条增粗的神经束,低信号的神经束被大量高信号脂肪组织分隔,呈“同轴电缆”表现;在冠状位或矢状位MRI图像上可见圆柱状增粗的神经束,走行迂曲,低信号的神经束与高信号的脂肪交替排列,呈“意大利面”改变;3例合并巨指畸形。1例累及双侧三叉神经,MRI表现为双侧三叉神经、三叉神经节及右侧眼神经终末段增粗,三叉神经周围可见环形增厚的脂肪信号,三叉神经节及右侧眼神经内可见高信号脂肪分隔增粗的低信号神经束,呈“同轴电缆”表现;同时合并右侧上眼睑皮下脂肪增多。结论 FLH好发于正中神经及其分支,典型MRI表现为受累神经增粗,神经内有大量纤维脂肪组织浸润,也可伴周围软组织内脂肪沉积增多或巨指畸形。更多还原

【Abstract】 Objective To explore the MR imaging findings of neural fibrolipomatous hamartoma(FLH). Methods The clinical data and unenhanced MRI of 10 patients(7 males, 3 females; age range: 1-37 years; median age 21.5 years) with pathologically confirmed FLH were retrospectively reviewed. Results Of 9 lesions involving the median nerves, multiple enlarged median nerve bundles encased in thickened epineural fibrous tissue were noted on MRI. On axial sections, the hypointense nerve bundles were separated by hyperintense fat, thus creating a coaxial-cable-like appearance. On coronal or sagittal sections, the nerve bundles appeared as hypointense serpentine tubules alternating with hyperintense fat, creating a spaghetti-like appearance. Three of the 9 patients had macrodactyly. On MRI, the trigeminal nerve, trigeminal ganglion, and the distal right ophthalmic nerve were all enlarged in 1 patient with trigeminal nerve FLH. The fat was mainly distributed in the periphery of the trigeminal nerve and between the nerve bundles in the trigeminal ganglion and distal ophthalmic nerve, creating a coaxial-cable-like appearance. Fat deposition in the upper eyelid was also noted. Conclusion FLH most commonly affecting the median nerve and its branches. The typical MRI findings are enlarged nerve with increased fibrofatty tissue interspersed among thickened nerve bundles. FLH may be associated with adjacent soft tissue fat deposition or macrodactyly.更多还原