Background: Unilateral isolated optic disc edema (UIODE) represents a challenging clinical presentation that frequently precipitates an extensive diagnostic work-up. Patients without an apparent diagnosis despite appropriate investigations are often categorized as having "papillophlebitis," an entity that is poorly defined in the existing literature. Our aim was to describe the characteristics of a series of patients with paucisymptomatic UIODE, determine the optimal diagnostic approach to such cases, and clarify the clinical features of presumed papillophlebitis.
Methods: We retrospectively identified 29 patients with UIODE who were seen by neuro-ophthalmologists at a single center between 2005 and 2019. Each patient presented with isolated, unilateral disc edema that was either entirely asymptomatic or associated with minimal visual symptoms. Patients underwent a comprehensive neuro-ophthalmic evaluation and several ophthalmic and systemic investigations. Data from the initial visit and all subsequent clinical visits were collected, including patient demographics, examination findings, and details of the diagnostic work-up.
Results: Our 29 patients with UIODE were found to have a variety of underlying diagnoses including unilateral papilledema due to idiopathic intracranial hypertension (10 patients), optic nerve sheath meningioma (5), incipient nonarteritic anterior ischemic neuropathy (4), vitreopapillary traction (3), orbital masses (2), a peripapillary choroidal neovascular membrane (1), and presumed papillophlebitis (4). The duration of disc edema varied considerably based on the etiology, but most patients had favorable visual outcomes.
Conclusions: A systematic approach to the evaluation of UIODE, combined with long-term follow-up, led to a definite diagnosis in a majority of patients, with only 4 patients presumed to have papillophlebitis, a diagnosis the actual existence of which remains controversial.
Copyright © 2020 by North American Neuro-Ophthalmology Society.