Paracentral Acute Middle Maculopathy as a Presenting Sign of CRAO in Sickle Cell Disease Treated with Tissue Plasminogen Activator.

PURPOSE We report the case of a 27-year-old monocular woman with history of sickle cell disease who received intra-arterial tissue plasminogen activator (tPA) after presenting with acute painless vision loss secondary to incomplete central retinal artery occlusion presenting as paracentral acute middle maculopathy (PAMM) in her left eye. METHODS Ultra-widefield fundus photography, ultra-widefield fluorescein angiography, en face ocular coherence tomography (OCT), and ocular coherence tomography angiography (OCTA) were obtained and reviewed, followed by cerebral angiography and infusion of intra-arterial tissue plasminogen activator (tPA). RESULTS A patient with a history of sickle cell disease presented with new onset of a dense central scotoma and visual acuity diminished to 20/200 from baseline 20/20 in her left eye. Fluorescein angiogram was non-diagnostic. OCT revealed perifoveal hyperreflective bands in the inner nuclear layer in a pattern characteristic of PAMM. The patient received intra-arterial tPA through her left ophthalmic artery shortly after presentation, resulting in a gradual restoration of visual acuity to 20/20 in the three months following the procedure. CONCLUSIONS This is the first report describing the use of intra-arterial tPA to treat incomplete central retinal artery occlusion presenting as a PAMM lesion in a patient with sickle cell disease.