Multimodal imaging features of butterfly-like pattern retinal pigment epithelial dystrophy

Abstract:

AIM: To explore the multimodal imaging features of butterfly-like retinal pigment epithelial dystrophy(BPD)patients.

METHODS: Retrospective analysis of the multimodal imaging of 18 BPD patients(36 eyes)from January 2016 to July 2019, including fundus color photography, infrared photography, autofluorescence, fundus fluorescein angiography, indocyanine green angiography, optical coherence tomography(OCT), and optical coherence tomography angiography(OCTA).

RESULTS: A typical fundus color photography showed the appearance of a butterfly-like lesion caused by abnormal pigmentation. The appearance of the butterfly was not obvious after the lesion progressed, and the corresponding region shrinks; Infrared photography showed the yellow lesions clearly in the BPD patients, which are white highlight images; Autofluorescence showed patchy, spotted butterfly wing performance. When the lesion progressed, autofluorescence did not show typical butterfly changes, but it showed the damage of pigment epithelial cells; Fundus fluorescein angiography and choroidal angiography, in addition to showed butterfly lesions, can more accurately display vascular lesions, especially choroidal neovascularization(CNV); OCT showed lesions located between the retinal pigment epithelial(RPE)layer and the photoreceptors. As the lesion progressed, the pigment epithelium showed enlarged lesions correspondingly. Secondary CNV patients can be seen to break RPE; OCTA showed that the lesions were not obvious at the deep and superficial layers of the retina. But the choroidal blood flow signals were lost in some degrees in OCTA, and the blood flow images of CNV can be detected sensitively.

CONCLUTION: Multimode imaging technology can provide imaging features of progression in BPD patients, which helps clinicians to understand the disease more deeply.

Key words:pattern retinal pigment epithelial dystrophy; butterfly-like pattern; multimodal imaging; lipofuscin; features