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

Abstract

To describe the characteristic pattern of progression of pentosan polysulphate (PPS) maculopathy with multimodal retinal imaging in 2 patients, including one with over 9 years of follow up. Two patients with PPS maculopathy were sequentially evaluated with near-infrared reflectance (NIR) and optical coherence tomography (OCT). NIR showed characteristic centrifugal progression of the parafoveal hyperreflective lesions towards the vascular arcades with the development of hyporeflective areas in both cases. OCT demonstrated focal retinal pigment epithelium (RPE) thickening that corresponded to the hyperreflective lesions on NIR. On subsequent OCT scans, the hyperreflective areas resolved with the development of ellipsoid zone (EZ) attenuation, retinal pigment epithelial (RPE) disruption and atrophy, which co-localized with hyporeflectivity on NIR. 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.