Multimodal Imaging Observation in Different Progressive Types of Bietti Crystalline Dystrophy

Shengjuan Zhang; Lifei Wang; Yanhui Wang; Yanxia Shang; Xin Wang; Lizhen Ma; Zanzhang Yang; Chen Xing; Xiaoyan Peng

doi:10.1155/2022/7426052

Multimodal Imaging Observation in Different Progressive Types of Bietti Crystalline Dystrophy

J Ophthalmol. 2022 May 24:2022:7426052. doi: 10.1155/2022/7426052. eCollection 2022.

Authors

Shengjuan Zhang^{1

2}, Lifei Wang², Yanhui Wang², Yanxia Shang², Xin Wang², Lizhen Ma², Zanzhang Yang², Chen Xing², Xiaoyan Peng¹

Affiliations

¹ Beijing Institute of Ophthalmology, Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China.
² Hebei Provincial Eye Hospital, Hebei Provincial Key Laboratory of Ophthalmology, Hebei Provincial Eye Institute, Xingtai, Hebei 054001, China.

Abstract

Objective: 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.

Methods: 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.

Results: 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.

Conclusions: 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.