Anterior Segment Involvement in Polycythemia Vera: A Case Report and Review of Literature

To describe the clinical and tomographic features of anterior segment involvement as the initial manifestation of previously undiagnosed Polycythemia Vera (PV), using slit-lamp examination and anterior segment optical coherence tomography (AS-OCT) before and after treatment.

Observational case report and literature review of ocular involvement in PV.

A 42-year-old Caucasian woman presented with a long-standing history of unilateral conjunctival and episcleral hyperemia and vascular tortuosity unresponsive to topical therapy, showing clinical features inconsistent with ocular inflammation. Slit-lamp examination revealed conjunctival and episcleral vascular congestion that did not blanch with phenylephrine drops. AS-OCT imaging demonstrated ectasia and dilatation of the superficial and deep episcleral vascular plexuses. Laboratory work-up confirmed a diagnosis of PV. Following initiation of appropriate therapy, the episcleral vascular hyperemia and engorgement fully resolved without residual sequelae. A review of the literature identified two cases reported between 1971 and the present. Their presentations and clinical courses are reviewed herein.

This report characterizes anterior segment involvement in PV using slit-lamp and AS-OCT imaging, highlights its distinctive clinical features, and underscores the importance of laboratory investigations in evaluating atypical ocular presentations potentially caused by hematological masquerade disorders.