成人型卵黄样黄斑营养不良的多模式影像特征观察

Multimodal imaging characteristics of adult-onset foveomacular vitelliform dystrophy

摘要:

目的：观察成人型卵黄样黄斑营养不良(AFVD)的多模式影像特征。

方法：回顾性研究，观察2015-01/2018-04在郑州市第二人民医院就诊并确诊为成人型卵黄样黄斑营养不良的患者6例11眼，其中男2例3眼，女4例8眼，年龄52.5±1.7(43～67)岁。眼前节、眼压及ERG均未见异常，无家族史。最佳矫正视力(BCVA)：0.08～0.8，EOG：9眼正常，2眼轻度下降。所有患者均进行了眼底彩照、荧光血管造影(FFA)、吲哚菁绿血管造影(ICGA)、光学相干断层扫描血管成像(OCTA)检查。对其影像资料进行分析，总结其主要特征。

结果：眼底表现为黄斑区视网膜见约≤1PD大小的黄色斑块； 卵黄样物质沉积处在FFA及ICGA均呈低荧光，部分在FFA晚期出现荧光积存； OCTA B-scan示卵黄样物质沉积在感光细胞层和视网膜色素上皮层(RPE)之间，随着疾病的进展，感光细胞层逐渐被破坏，外核层变薄，RPE-Bruch膜复合体层变薄或消失； OCTA脉络膜毛细血管层见暗区，周围见强反射信号，En-face 图像见暗区。

结论：多种成像模式相结合有助于AFVD的诊断，减少漏诊、误诊，能够更好地理解其发病机制，协助临床制定治疗方案，准确评价预后。

Abstract:

AIM: To observe the multimodal imaging characteristics of adult-onset foveomacular vitelliform dystrophy(AFVD).

METHODS: Retrospective study. Six cases(11 eyes)of AFVD in Zhengzhou Second People Hospital from January 2015 to April 2018 were included. Amongst all of them, there were 2 men 3 eyes, 4 women 8 eyes, aged from 43-67 years(average 52.5±1.7 years). No abnormality was found in the anterior segment, intraocular pressure and electroretinogram(ERG). There was no family history. Best corrected visual acuity(BCVA)was from 0.08-0.8, EOG was normal in 9 eyes, slightly decreased in 2 eyes. Fundus color photography, optical coherence tomography angiography(OCTA), fundus fluorescein angiography(FFA)and indolecyanine green angiography(ICGA)of the patients were examined and analyzed.

RESULTS: The fundi of all patients showed less than 1PD vitelliform lesions, showing hypofluorescence in FFA and ICGA. In the late phase of FFA,fluorescence accumulated in the lesion in some patients. In B-scan of OCTA, there was vitelliform substance which deposited between the photoreceptor and retinal pigment epithelium(RPE )layer. As the disease continues to progress, photoreceptor layer became thinner, RPE-Bruch layer became thinner or disappeared. In OCTA there was dark spot with strong reflection around it in choriocapillary layer. There was dark spot in En-face image.

CONCLUSION: Multimodal imaging assist in diagnosing AFVD, reducing missed diagnosed and misdiagnosis, better understanding the pathogenesis, developing the treatment plan, judging prognosis.