Purpose: To report the clinical presentation, treatment course, and outcome of a case of bilateral frosted branch angiitis (FBA) and neuroretinitis associated with acute Epstein-Barr virus (EBV) infection in a pediatric patient with Turner Syndrome.
Methods: Case report with multimodal ocular imaging and extensive systemic workup.
Results: A 16-year-old female with Turner syndrome presented with acute bilateral vision loss, hearing loss, and ataxia. Ocular examination showed bilateral extensive perivascular sheathing, optic disc edema, and macular exudates. Fluorescein angiography showed focal retinal ischemia and late optic disc leakage in both eyes. Macular optical coherence tomography was remarkable for intraretinal and subretinal fluid with scattered hyperreflective foci in both eyes. MRI brain and orbits showed a supratentorial T2 signal without enhancement, consistent with inactive vasculopathy. Results of a broad systemic workup were notable only for elevated EBV viral capsid antigen IgM with undetectable EBV viral capsid antigen IgG. After treatment with intravenous methylprednisolone followed by oral prednisone, the patient's best corrected visual acuity in both eyes improved from 20/200 to 20/20.
Conclusion: Ocular symptoms and imaging biomarkers in this case of EBV-associated FBA resolved after high-dose systemic corticosteroid monotherapy. Although treatment with antiviral medications is reported in other FBA cases associated with acute EBV infection, this case underscores the treatable nature of this disease without antivirals, the use of which are not supported by the broader literature for EBV infection or post-viral inflammation.