Presenter: Elon H.C Van Dijk The Netherlands
A 68-year-old women presented with a relatively large macular area of complete absence of the retinal pigment epithelium (RPE) monolayer, in association with a chronic, persistent, and relatively stable subretinal fluid accumulation.
Case Summary:
A 68-year-old women presented with a relatively large macular area of complete absence of the retinal pigment epithelium (RPE) monolayer, in association with a chronic, persistent, and relatively stable subretinal fluid accumulation overlying this area of absent RPE. Fluorescein angiography showed RPE window defects and fluorescein pooling, without leakage. No leakage could be detected on indocyanine green angiography. Based on clinical characteristics and multimodal imaging of this patient (and 2 other patients that visited our clinic) with subretinal fluid associated with a striking absence of the RPE in the macula, we propose serous maculopathy with absence of the retinal pigment epithelium (SMARPE) as a new phenotype of serous maculopathy.
SMARPE is a slowly progressive macular disease, that is unresponsive to commonly used treatments such as intravitreal injections with anti-vascular endothelial growth factor receptor medication and photodynamic therapy. SMARPE appears clinically distinct from previously described clinical entities with serous subretinal fluid accumulation in the macula. The exact pathophysiology of SMARPE is unclear, and it remains to be elucidated if it is a single clinical entity or a common endpoint of different etiologies.
https://onlinelibrary.wiley.com/doi/epdf/10.1111/aos.14995
