MACULAR HOLE IN A PATIENT WITH PENTOSAN POLYSULFATE MACULOPATHY

Pentosan polysulfate (PPS), a drug used for interstitial cystitis, has recently been detected to cause maculopathy in a dose-dependent manner. Outer retinal atrophy is the hallmark of this condition.

History, examination, and multimodal imaging were used to guide diagnosis and management.

We report a case of PPS-related maculopathy in a 77-year-old lady, who presented with florid retinal atrophy at the posterior pole in both eyes, and a concurrent macular hole in the left eye. She had been diagnosed with interstitial cystitis several years before for which she was prescribed PPS (Elmiron). She had noticed a drop in vision 5 years after initiation of PPS and self-discontinued the drug after 24 years of use. A diagnosis of PPS-related maculopathy with a macular hole was made. She was counselled regarding the prognosis and was advised to avoid PPS. Surgery for macular hole was deferred in view of the severe retinal atrophy.

Pentosan polysulfate-related maculopathy can lead to severe retinal atrophy and a subsequent degenerative macular hole. A high index of suspicion is required for early detection and cessation of drug to prevent this irreversible vision loss.