9-Year Progression of Pentosan Maculopathy with Multimodal Retinal Imaging.

This report describes the progression of pentosan polysulphate maculopathy over almost 10 years of PPS treatment and highlights the importance of NIR as a tool for the diagnosis and monitoring of PPS maculopathy. PPS lesions present as areas of focal RPE thickening with ensuing development of EZ loss and RPE drop-out. The pathophysiology of PPS toxicity is unknown and may either result from primary RPE or choroidal toxicity.