Case Report: Pentosan Polysulfate Sodium Maculopathy Originally Diagnosed as Pattern Macular Dystrophy

Area of Science:

• Ophthalmology
• Toxicology

Background:

• Pentosan polysulfate sodium (PPS) is indicated for interstitial cystitis/painful bladder syndrome.
• PPS maculopathy is a clinical entity characterized by pigmentary maculopathy from chronic PPS exposure.
• Interstitial cystitis/painful bladder syndrome affects up to 2% of the population, necessitating awareness of potential visual side effects.

Observation:

• An observational case report details a patient initially diagnosed with pattern macular dystrophy.
• The patient presented with painless, bilateral vision loss and bilateral pigmentary maculopathy.
• Multimodal imaging, including OCT, NIR, FAF, and angiography, was utilized.

Findings:

• A comprehensive review of medical history and medication revealed chronic PPS exposure.
• The diagnostic workup ultimately identified Pentosan polysulfate sodium (PPS) maculopathy.
• Genetic studies were also performed as part of the diagnostic process.

Implications:

• Early screening and recognition of PPS maculopathy are critical in primary eye care.
• Discontinuation of Pentosan polysulfate sodium may prevent severe, irreversible vision loss.
• Ongoing surveillance with multimodal imaging is recommended for at-risk patients.