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一名斜视儿童视盘假性重复,最初类似视神经分叉:病例报告

Pseudoduplication of the optic disc initially resembling a bifurcated optic nerve in a strabismus child: a case report.

作者信息

Huang Liuhui, Zhang Qi, Jin Haiying, Zhao Peiquan

机构信息

Department of Ophthalmology, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Kongjiang Road, No. 1665, Shanghai, 200092, China.

Department of Ophthalmology, Tenth People's Hospital Affiliated to Shanghai Tongji University School of Medicine, Shanghai, China.

出版信息

BMC Ophthalmol. 2020 Mar 14;20(1):101. doi: 10.1186/s12886-020-01369-1.

Abstract

BACKGROUND

Pseudoduplication of the optic disc is a rare clinical condition that is characterized by a circumscribed, disc-like lesion with radiating vessels but only one normal optic nerve. We report a rare case that initially resembled a bifurcated optic nerve in a strabismus child.

CASE PRESENTATION

A 6-year-old female child was initially referred to our hospital due to perceptual exotropia of 15 degrees with poor fixation of the left eye. The visual acuity of the left eye was 3/100 with a refraction of + 1.75/- 1.25 × 175. Fundus images of her left eye revealed a circumscribed and disc-like lesion located one disc diameter (DD) below the true optic disc that showed profound central cupping resembling a second optic disc with a vascular supply. B scan ultrasonography showed an optic nerve with a bifurcated weak-echo region, suggesting that two strands originated from the optic nerve. Optic coherence tomography (OCT) demonstrated a large crater-like depression of the lesion, indicating a colobomatous defect covered by a mysterious membranous structure, a disturbed nerve fibre layer and the absence of regular outer retinal layers. A perimetric examination revealed a relatively superior defect. Magnetic resonance imaging (MRI) revealed the left eye globe showed an abnormal morphology and that the optic nerve was abnormally shaped and shifted nasally in the left eye. Fundus fluorescein angiography (FFA) of the left eye revealed the absence of independent vascular vessels in the disc-like lesion. Hyperfluorescence with patchy fluorescence was evident in the inferotemporal area of the disc. Vascular loops surrounding the temporal region were evident in both eyes. Her right eye was normal except for the vascular loop. We proposed that this represented a case of pseudoduplication of the optic disc. The patient did not undergo any treatment, and her visual acuity remained stable during the follow-up period.

CONCLUSIONS

Our patient presented with a deep and ectatic coloboma below the optic disc that communicated with the true optic nerve and was originally thought to indicate a bifurcated optic nerve. This case suggests that atypical ectatic colobomas should be considered before diagnosing malformations related to the optic nerve in double optic disc cases.

摘要

背景

视盘假性重复是一种罕见的临床病症,其特征为有边界的、盘状病变并有放射状血管,但仅有一条正常视神经。我们报告一例罕见病例,最初在一名斜视儿童中类似视神经分叉。

病例介绍

一名6岁女童因左眼知觉性外斜视15度且注视不良而最初转诊至我院。左眼视力为3/100,验光结果为+1.75/-1.25×175。其左眼眼底图像显示在真正视盘下方一个视盘直径(DD)处有一个边界清晰的盘状病变,该病变有明显的中央凹陷,类似第二个有血管供应的视盘。B超显示视神经有一个分叉的弱回声区,提示有两条束状结构起源于视神经。光学相干断层扫描(OCT)显示病变有一个大的火山口样凹陷,表明是一个被神秘膜状结构覆盖的缺损,神经纤维层紊乱且视网膜外层不规则。视野检查显示相对上方缺损。磁共振成像(MRI)显示左眼眼球形态异常,视神经形态异常并向左眼鼻侧移位。左眼眼底荧光血管造影(FFA)显示盘状病变中无独立血管。视盘颞下区域可见斑片状荧光增强。双眼颞侧区域周围均可见血管袢。除血管袢外,其右眼正常。我们认为这是一例视盘假性重复病例。患者未接受任何治疗,随访期间视力保持稳定。

结论

我们的患者在视盘下方出现一个深部且扩张的缺损,与真正的视神经相通,最初被认为提示视神经分叉。该病例表明,在诊断双视盘病例中与视神经相关的畸形之前,应考虑非典型扩张性缺损。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a71/7071674/4f2c9350cc74/12886_2020_1369_Fig1_HTML.jpg

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