A case of pentosan polysulfate maculopathy originally diagnosed as stargardt disease

PURPOSE: To describe a patient with a past diagnosis of Stargardt disease that was later determined to be pentosan polysulfate (PPS) maculopathy. OBSERVATIONS: The patient had clinical and imaging findings uncharacteristic of Stargardt disease. Rather, her fundus resembled the recently described maculopathy ascribed to PPS. After genetic testing was found to be negative for pathologic variants, the patient was asked to cease usage of PPS. CONCLUSIONS AND IMPORTANCE: This case emphasizes the importance of reviewing patient medication profiles prior to rendering a diagnosis of a retinal dystrophy. It is essential that ophthalmologists catch drug toxicities as early as possible, to minimize risk of further irreversible vision loss due to continued medication exposure.