SHM Converge Scientific Abstract ePoster Gallery-Not Seeing It: A Rare Cause of Retinal Vasculitis

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This case describes an 18-year-old girl with photophobia, vomiting, headache, and later blurry vision. She had multiple emergency visits and was initially treated for status migrainosus, but ophthalmologic evaluation revealed bilateral optic nerve edema and peripheral retinal vasculitis with hemorrhages and whitening. Her lumbar puncture showed lymphocytic pleocytosis, elevated protein, and high opening pressure, suggesting aseptic meningitis. MRI brain and cerebral arteriogram were normal.<br /><br />An extensive infectious and autoimmune work-up was negative, including HIV, HSV, VZV, syphilis, TB, enterovirus, cryptococcus, toxoplasma, CMV, ANA, ANCA, dsDNA, SSA, and SSB. Based on the retinal findings, bilateral involvement of arteries and veins, and broad perivascular whitening, the presentation was consistent with frosted branch angiitis (FBA), a rare form of retinal vasculitis.<br /><br />FBA is uncommon, with only about 50 reported cases, and can be associated with infections such as HSV, EBV, VZV, CMV, COVID-19, or with autoimmune disease such as SLE. Its cause is uncertain but may involve a hypersensitivity reaction with immune complex deposition. Patients often have vitritis as well, as in this case.<br /><br />Treatment of retinal vasculitis generally involves corticosteroids and management of any underlying cause. Although the optimal therapy for FBA is not fully established, most reported cases improve with systemic steroids, sometimes rapidly.<br /><br />In this patient, the combination of aseptic meningitis, retinal vasculitis, lack of systemic autoimmune features, and negative testing supported a rare diagnosis of idiopathic FBA.

Asset Subtitle

Ann M. Kumfer

Meta Tag

Author List Ambika Viswanathan, Ann M. Kumfer, Merete Lund

Category Clinical Vignettes

Concept Retinal Vasculitis

Concept Frosted Branch Angiitis

Concept Infectious Etiology

Concept Corticosteroid

Concept Blurry Vision

Distinguished Non-Finalist

Presenter Organization University of North Carolina Hospital

Presenting Author Ann M. Kumfer

Track Adult

Keywords

frosted branch angiitis

retinal vasculitis

aseptic meningitis

optic nerve edema

photophobia

bilateral vision loss

vitritis

lymphocytic pleocytosis

systemic corticosteroids

idiopathic inflammation

Retinal Vasculitis

Frosted Branch Angiitis

Infectious Etiology

Corticosteroid

Blurry Vision