卵黄样黄斑营养不良的多模式影像特征观察

Multimodal imaging characteristics of Best vitelliform macular dystrophy

摘要:

目的：观察卵黄样黄斑营养不良(BVMD，别称Best病)的多模式影像特征。

方法：收集2016-06/2022-10于南京医科大学眼科医院确诊为Best病Ⅰ-Ⅳ期的患者30例60眼的临床资料进行回顾性分析，均双眼发病。所有患者均行最佳矫正视力(BCVA)、裂隙灯显微镜、间接眼底镜、眼压、眼底彩色照相、频域光学相干断层扫描(SD-OCT)、眼底自发荧光(FAF)、荧光素眼底血管造影(FFA)、眼电图(EOG)及光学相干断层扫描血管成像(OCTA)。

结果：共纳入患者30例60眼，其中Ⅰ期8眼，Ⅱ期24眼，Ⅲ期22眼，Ⅳ期6眼，眼底彩色照相、FAF、FFA、SD-OCT的影像特征与既往文献报道基本一致，EOG显示Arden比均<1.55，OCTA能够发现早期病灶，观察到卵黄样物质、光感受器外节、液体的位置分布及有无CNV形成。

结论：多模式影像有助于Best病的诊断，减少临床上漏诊、误诊，其中OCTA较其他检查有明显优势，快捷无创是其最大优势。

Abstract:

AIM: To observe the multimodal imaging characteristics of Best vitelliform macular dystrophy(BVMD).

METHODS:The clinical data of 30 patients(60 eyes)diagnosed as BVMD at stage Ⅰ to Ⅳ in Nanjing Medical University Affiliated Eye Hospital from June 2016 to October 2022 were collected for a retrospective analysis, and all patients are binocular involved. All patients underwent best corrected visual acuity(BCVA), slit lamp microscopy, indirect ophthalmoscopy, intraocular pressure, fundus photography, spectral-domain optical coherence tomography(SD-OCT), fundus autofluorescence(FAF), fundus fluorescein angiography(FFA), electro-oculogram(EOG)and optical coherence tomography angiography(OCTA).

RESULTS: A total of 30 patients(60 eyes)were included, with 8 eyes at stage Ⅰ, 24 eyes at stage Ⅱ, 22 eyes at stage Ⅲ and 6 eyes at stage Ⅵ. The imaging characteristics of fundus photography, FAF, FFA and SD-OCT were basically consistent with previous literature reports. EOG showed Arden ratio <1.55. OCTA could detect early lesions, observe the location of vitelliform substance, external segment of photoreceptor, fluid and choroidal neovascularization(CNV).

CONCLUSION: Multimodal imaging assisted in diagnosing BVMD, reducing missed diagnosis and misdiagnosis, among which OCTA had significant advantages over other examinations, and fast and non-invasive were its biggest advantages.