Atypical Mid-Late Phase ICGA Hyperfluorescence in a Secondary MEWDS Case: A Distinct Disease or a Shift in Our Understanding of MEWDS?

Abstract

Purpose: To report a secondary multiple evanescent white dot syndrome (MEWDS) case with peculiar indocyanine green angiography (ICGA) findings.

Method: Report of a patient with sickle cell disease (SCD) and a longstanding macular hole who developed an atypical form of secondary MEWDS. Analysis of multimodal imaging findings was performed.

Results: A 33-year-old female with SCD presented with a longstanding full-thickness macular hole in the left eye, but then was lost to follow-up. Upon her return nine months later, fundus examination revealed new, asymptomatic, yellowish lesions at the level of the outer retina or retinal pigment epithelium (RPE). ICGA showed a peculiar hyperfluorescence in mid-to-late phases, while fundus autofluorescence (FAF) highlighted distinct temporal patterns of hyper-autofluorescence. Some of the ICGA hyperfluorescent lesions displayed a central hypofluorescent core. The multimodal imaging findings suggest a distinct disease or a sequential mechanism in MEWDS pathophysiology. The process may begin with an initial photoreceptoritis, marked by primary self-resolving hyper-autofluorescence on FAF, followed by an early RPE dysfunction with choroidal hyperpermeability evidenced by mid-late phase ICGA hyperfluorescence, then more marked RPE dysfunction shown by late-phase ICGA hypofluorescence and FAF hyper-autofluorescence.

Conclusion: This case describes an unusual secondary MEWDS presentation with unique imaging findings. The peculiar ICGA behavior and evolving FAF patterns may suggest either a distinct disease or a shift in our understanding of MEWDS involving photoreceptoritis, early RPE dysfunction, followed by more advanced RPE damage. The findings underscore the need for a nuanced approach to interpreting white-dot syndromes.