Pentosan-associated maculopathy: Early detection using OCT angiography and choriocapillaris flow deficit analysis.

Abstract

Purpose : Pentosan maculopathy may progress despite discontinuation of the drug exposure. The identification of earlier imaging markers of toxicity may be critical to prevent vision loss. Hence, we compared the choriocapillaris flow deficit (CC FD) from optical coherence angiography (OCT) angiography in eyes of patients treated with high cumulative dosages of pentosan polysulfate sodium (PPS) with no signs of retinal toxicity versus healthy age-matched controls

Methods : Patients treated with PPS for interstitial cystitis with a cumulative dosage of more than 1000 grams underwent multimodal imaging screening to exclude evidence of PPS maculopathy or other retinal abnormalities. All study patients and age-matched healthy controls completed OCT angiography (OCTA) using the Solix device (Solix; Optovue Inc, Fremont, California, USA), with a 3x3 mm volume cube scan centered on the fovea. En face OCTA images at the level of the CC were exported, and CC FDs were computed and compared between groups.

Results : Fifteen patients treated with PPS and fifteen age-matched controls were included. The mean PPS cumulative dose was 1974±666.4 grams over a mean of 17.6 ±6.8 treatment years. All patients had visual acuity of 20/25 or better and normal fundus autofluorescence (FAF), OCT, multicolor, near-infrared reflectance (NIR), and ultra-widefield fundus color and AF images. The CC FD was 32.7±3.6% in the PPS group compared to 28.6±4.3% in the control group (p=0.023).

Conclusions : PPS treated patients show significant CC flow impairment before the development of overt signs of macular toxicity compared to healthy age-matched controls. Thus, the choroid may be the earliest manifestation of ocular toxicity, predating the development of clinically evident RPE injury. The subsequent RPE disruption may be the result of choriocapillaris impairment or primary PPS toxicity. Regardless, assessment of the CC on OCTA may be a useful tool for early detection of toxicity, though further longitudinal studies are required.

This abstract was presented at the 2022 ARVO Annual Meeting, held in Denver, CO, May 1-4, 2022, and virtually.