Zhang Shengjuan, Wang Lifei, Wang Yanhui, Shang Yanxia, Wang Xin, Ma Lizhen, Yang Zanzhang, Xing Chen, Peng Xiaoyan
Beijing Institute of Ophthalmology, Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China.
Hebei Provincial Eye Hospital, Hebei Provincial Key Laboratory of Ophthalmology, Hebei Provincial Eye Institute, Xingtai, Hebei 054001, China.
J Ophthalmol. 2022 May 24;2022:7426052. doi: 10.1155/2022/7426052. eCollection 2022.
The aim of the study is to observe the difference in progression between type 1 and type 2 Bietti crystalline dystrophy (BCD) using multimodal imaging.
A retrospective clinical study was performed with six BCD patients who underwent multimodal imaging twice in Hebei Provincial Eye Hospital from October 2015 to December 2020. Multimodal imaging includes color fundus photography, fundus autofluorescence (AF), infrared autofluorescence (IRAF), fundus fluorescein angiography (FFA), and spectral domain optical coherence tomography (SD-OCT). The fundus lesion progression difference was observed in 3 patients with type 1 BCD and 3 patients with type 2 BCD.
In type 1 BCD, the range of hypoautofluorescence (hypo-AF), hypoinfrared autofluorescence (hypo-IRAF), and hypofluorescence in the posterior pole was enlarged, and FFA showed that the lesions in the posterior pole and periphery extended to the middle periphery. SD-OCT revealed retinal and choroidal thinning, progressive loss of the outer nuclear layer and ellipsoid zone, and reduction of the choroid macrovascular diameter. In type 2 BCD, the range of hypo-AF was enlarged, but there was no significant change in the macula area. The uniform hypo-IRAF in the posterior pole showed no significant change. FFA showed no significant change with the progression of the disease in the macula area and the hypofluorescence around it expanded. SD-OCT revealed no obvious change in the macula area.
The retinal choroid atrophy in the macula area of type 1 BCD continued to worsen, and the choroid great vessels became narrower. The macular lesions of type 2 BCD can remain unchanged for a long time.
本研究旨在通过多模态成像观察1型和2型贝氏结晶状视网膜变性（BCD）进展的差异。
对2015年10月至2020年12月在河北省眼科医院接受两次多模态成像检查的6例BCD患者进行回顾性临床研究。多模态成像包括彩色眼底照相、眼底自发荧光（AF）、红外自发荧光（IRAF）、眼底荧光血管造影（FFA）和频域光学相干断层扫描（SD-OCT）。观察3例1型BCD患者和3例2型BCD患者的眼底病变进展差异。
在1型BCD中，低自发荧光（低AF）、低红外自发荧光（低IRAF）和后极部低荧光范围扩大，FFA显示后极部和周边部病变扩展至中周边部。SD-OCT显示视网膜和脉络膜变薄，外核层和椭圆体带逐渐丧失，脉络膜大血管直径减小。在2型BCD中，低AF范围扩大，但黄斑区无明显变化。后极部均匀的低IRAF无明显变化。FFA显示黄斑区疾病进展无明显变化，其周围低荧光区扩大。SD-OCT显示黄斑区无明显变化。
1型BCD黄斑区视网膜脉络膜萎缩持续恶化，脉络膜大血管变窄。2型BCD黄斑病变可长期保持不变。
