Abstract
Purpose :
Frosted branch angiitis (FBA) is a rare condition defined by diffuse retinal vasculitis with perivascular whitish infiltration, first described in 1976 by Ito. The causes of FBA are infections, neuro-Behçet, lymphomatous and leukemic affections. There is a risk of severe visual or vital prognosis. We report the detailed semiology of FBA cases their etiology and their evolution.
Methods :
All consecutive patients with FBA treated in a tertiary center between 2014 and 2018 were reported. The ophthalmologic examination, visual acuity (VA), etiology and evolution were detailed. An anterior chamber tap (ACT) for herpesviruses or Toxoplasma gondii analysis was performed in all cases to eliminate an infection.
Results :
Four patients were included. A 34-year-old man had an unilateral painless visiual loss. The anterior segment showed granulomatous keratic precipitates (KP) and the fundus disclosed a FBA. The work-up revealed a Castelman's disease. Corticosteroids and immunosuppressors have been efficient. A 33-year-old woman presented a severe visual loss in both eyes. Slit-lamp examination found granulomatous KP and diffuse FBA. The assessment found a systemic Hodgkin's lymphoma. Under chemotherapy the lesions decreased but VA remained low. A 70-year-old man was referred for a bilateral visual loss. The fundus showed a focal area of retinitis on the right eye and FBA on the left one associated with fine KP. ACT identified a cytomegalovirus infection. Antiviral therapy allowed vision improvement in both eyes. A 42-year-old man had an unilateral vision loss. The KP were fine and the fundus showed a FBA; ACT was positive for varicella zoster virus. VA did not improve under antiviral therapy.
Conclusions :
In our case series, patients who presented a hematological cause showed granulomatous KP contrary to the viral etiology with fine KP. The type of KP and the relation to an etiology must be confirmed in larger observational studies. This may be useful as management of FBA is an emergency. Specific therapy remains essential. Long-term monitoring is required to detect complications like retinal ischemia. The visual prognosis seems to depend on on the severity of vascular involvement and the location of lesions.
This is a 2020 ARVO Annual Meeting abstract.