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Show Poster 148 The Spectrum of Pauci-Symptomatic Isolated Unilateral Optic Nerve Head Edema (IUONHE) Eman Hawy1, Jason Peragallo1, Beau Bruce1, Michael Dattilo1, Nancy Newman1, Valerie Biousse1 1 Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia, USA Introduction: Isolated unilateral optic nerve head edema (IUONHE) with normal visual function is relatively rare, often prompting numerous investigations. Negative workup frequently presumes a diagnosis of "papillophlebitis." We report 28 patients with IUONHE, describing the natural history and final diagnoses. Methods: 28 IUONHE patients with detailed systematic workup and ≥1 year of followup were identified. Demographics, details of workup, final diagnosis, duration of IUONHE prior to resolution or visual loss, and visual outcome were recorded. Results: 9/28 patients had intracranial hypertension with unilateral papilledema related to idiopathic intracranial hypertension (IIH).All IIH patients were obese and had signs of intracranial hypertension on MRI/MRV. Diagnosis of IIH was made 1-32 months after presentation. 5/28 patients had an optic nerve sheath meningioma (ONSM) with evidence of IUONHE for 1-240 months before diagnosis of ONSM, with poor visual outcomes in patients with delayed diagnosis (lack of appropriate imaging at presentation). 2/28 patients had an orbital mass (one vascular malformation, one orbital extension of an intracranial mass). 3/28 patients had vitreopapillary traction (VPT), diagnosed 1 week-7 months after presentation. 4/28 patients had incipient nonarteritic anterior ischemic optic neuropathy (NAION); all were >60 yo with discs-at-risk. IUONHE spontaneously resolved within 3-11 months in 3/4 patients; one developed visual loss from NAION 4 months after presentation. 1 patient had a peripapillary choroidal neovascular membrane (CNV) diagnosed 6 weeks after presentation. 4/28 patients (age 21-37yo, 4 women) remained without definite diagnosis and were classified as presumed "papillophlebitis". All had persistent IUONHE with normal visual function up to 42 months after presentation. Conclusions: A systematic approach to the evaluation of IUONHE led to a definite diagnosis in 24/28 patients. The diagnosis of "papillophlebitis" remains controversial and poorly defined. The good visual prognosis of patients with unexplained IUONHE remains reassuring. A systematic diagnostic approach should result in earlier diagnosis and improved outcomes. References: None. Keywords: Orbit, Pseudotumor Cerebri, Neuroimaging, Optic neuropathy, Tumors Financial Disclosures: No conflict of interest Supported by Research to Prevent Blindness Grant Support: Supported by Research to Prevent Blindness 2018 Annual Meeting | 294 |
