Neuro-Ophthalmic Presentations of Polycythemia

Ocular manifestations of hyperviscosity, particularly associated with the hemodynamic changes in polycythemia, can lead to various ocular vascular events, ranging from transient monocular blindness to potentially irreversible vision loss if left untreated.

This is a multicenter, retrospective case series and review of literature involving 4 patients presenting with neuro-ophthalmic symptoms related to underlying polycythemia. A thorough search for prior reported cases with similar manifestations to ours was conducted through the PubMed database.

Four patients, 3 men and 1 woman, ranging in age from 68 to 71 years, were evaluated in the neuroophthalmology clinic. The patients had variable clinical presentations, ranging from transient unilateral or bilateral painless vision loss, nonarteritic anterior ischemic optic neuropathy, and acephalgic migraine with precipitation of visual auras. Polycythemia was secondary to testosterone therapy in 3 cases and primary in 1 case of polycythemia vera. Diagnostic challenges and treatment strategies are discussed in the context of each case. We also describe an additional 14 cases published in prior literature, with the majority presenting with variable symptoms of vision loss associated with polycythemia, and 1 case of amaurosis fugax attributed to a migrainous visual phenomenon.

This case series highlights the role of polycythemia as a precipitating factor for ocular vascular events, necessitating a more careful approach in clinical assessment and management of such patients. Further research is needed to better understand the mechanisms underlying the hemodynamic alterations in polycythemia and their implications for ocular health to optimize therapeutic approaches.