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[视神经肿瘤作为视神经炎鉴别诊断的临床及影像学特征]

[Clinical and imaging characteristics of optic nerve tumors as the differencial diagnosis of optic neuritis].

作者信息

Sun C B, Jiang B, Liu G H, Xiao Q

机构信息

Eye Center, Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou 310009, China.

Department of Ophthalmology, First Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou 310009, China.

出版信息

Zhonghua Yan Ke Za Zhi. 2023 May 11;59(5):367-375. doi: 10.3760/cma.j.cn112142-20220529-00268.

Abstract

To investigate the clinical and imaging features of optic nerve tumors that require differential diagnosis from optic neuritis. A retrospective case series study was conducted. Clinical data of patients diagnosed with optic nerve tumors from January 2017 to December 2021 at the Second Affiliated Hospital of Zhejiang University School of Medicine were collected. A total of 29 patients (39 eyes) with clinical and magnetic resonance imaging (MRI) findings similar to optic neuritis or optic neuropathy were included. There were 15 cases of optic nerve sheath meningioma (ONSM) (17 eyes), 4 cases of optic nerve glioma (ONG) (5 eyes), and 10 cases of infiltrative optic nerve lesions (ION) (17 eyes). All patients underwent best-corrected visual acuity (BCVA), anterior and posterior segment examinations, visual field examination, and orbital or cranial MRI examination. Patient data were observed and analyzed, treatment and follow-up information were recorded, and clinical and imaging features were summarized and compared with those of optic neuritis or optic neuropathy. Among the 29 patients with optic nerve tumors, 10 were male and 19 were female, with an average age of (43.3±13.8) years and a range of 11 to 72 years. The follow-up time was 6.8 (2.0, 11.0) months, with a range of 1 to 33 months. Sixteen patients (21 eyes) with optic nerve tumors were initially misdiagnosed as having acute optic neuritis and showed poor response to steroid treatment. Of these, 9 cases (11 eyes) were ONSM, 4 cases (6 eyes) were ION, and 3 cases (4 eyes) were ONG. The diagnostic delay time was 7.1 (1.5, 12.0) months, with a range of 1 to 24 months. The main clinical symptoms of all affected eyes were acute vision loss in 23 eyes, slow vision loss in 5 eyes, transient blackouts in 4 eyes, and no obvious visual symptoms in 7 eyes. The median BCVA of all affected eyes was 0.1, ranging from light perception to 1.0. Fundus examination results showed optic disc edema in 34 eyes and normal optic disc in 5 eyes among the 39 eyes with optic nerve tumors. A total of 27 patients (33 eyes) completed visual field examinations, which revealed an enlarged physiological blind spot in 11 eyes, a concentric or tubular visual field contraction in 8 eyes, a diffuse decrease in light sensitivity in 7 eyes, an arcuate scotoma in 4 eyes, and a normal visual field in 3 eyes. All affected eyes completed orbital or cranial MRI examinations, which showed mild optic nerve thickening in 22 eyes, significant thickening in 6 eyes with distortion, and no significant thickening in 6 eyes. Contrast-enhanced T1-weighted imaging (T1WI) MRI showed optic nerve parenchymal thickening in 5 eyes, all of which were ONG, and 2 of them had optic nerve parenchymal enhancement. Optic nerve sheath thickening and enhancement without optic nerve parenchymal thickening or enhancement were observed in 28 eyes, including 17 eyes of ION and 11 eyes of ONSM. There were 6 eyes with no obvious optic nerve thickening, which were all ONSM, showing mild or significant thickening and enhancement of the optic nerve sheath without optic nerve parenchymal thickening or enhancement. Optic nerve tumors can present with ophthalmic clinical features similar to optic neuritis, such as optic disc edema, and demonstrate MRI findings that resemble those of optic neuritis. Therefore, differentiation between the two is crucial, based on differences in their natural course and response to steroid therapy.

摘要

探讨需要与视神经炎进行鉴别诊断的视神经肿瘤的临床及影像学特征。进行了一项回顾性病例系列研究。收集了2017年1月至2021年12月在浙江大学医学院附属第二医院被诊断为视神经肿瘤的患者的临床资料。共纳入29例(39只眼)临床及磁共振成像(MRI)表现与视神经炎或视神经病变相似的患者。其中视神经鞘膜瘤(ONSM)15例(17只眼),视神经胶质瘤(ONG)4例(5只眼),浸润性视神经病变(ION)10例(17只眼)。所有患者均接受了最佳矫正视力(BCVA)、眼前节和后节检查、视野检查以及眼眶或头颅MRI检查。观察和分析患者数据,记录治疗及随访信息,并总结临床及影像学特征,与视神经炎或视神经病变的特征进行比较。29例视神经肿瘤患者中,男性10例,女性19例,平均年龄(43.3±13.8)岁,年龄范围为11至72岁。随访时间为6.8(2.0,11.0)个月,范围为1至33个月。16例(21只眼)视神经肿瘤患者最初被误诊为急性视神经炎,对类固醇治疗反应不佳。其中,ONSM 9例(11只眼),ION 4例(6只眼),ONG 3例(4只眼)。诊断延迟时间为7.1(1.5,12.0)个月,范围为1至24个月。所有患眼的主要临床症状为:23只眼急性视力丧失,5只眼视力缓慢下降,4只眼短暂性黑矇,7只眼无明显视觉症状。39只视神经肿瘤患眼中,眼底检查结果显示34只眼视盘水肿,5只眼视盘正常。27例(33只眼)患者完成了视野检查,结果显示11只眼生理盲点扩大,8只眼呈同心性或管状视野收缩,7只眼光敏感度弥漫性降低,4只眼呈弓形暗点,3只眼视野正常。所有患眼均完成了眼眶或头颅MRI检查,结果显示22只眼视神经轻度增粗,6只眼明显增粗伴形态改变,6只眼无明显增粗。对比增强T1加权成像(T1WI)MRI显示5只眼视神经实质增粗,均为ONG,其中2只眼视神经实质强化。28只眼观察到视神经鞘增粗并强化,但视神经实质无增粗或强化,包括ION 17只眼和ONSM 11只眼。6只眼无明显视神经增粗,均为ONSM,表现为视神经鞘轻度或明显增粗并强化,但视神经实质无增粗或强化。视神经肿瘤可表现出与视神经炎相似的眼科临床特征,如视盘水肿,并在MRI上表现出与视神经炎相似的表现。因此,基于两者自然病程及对类固醇治疗反应的差异,对两者进行鉴别至关重要。

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