The contribution of indocyanine green angiography to the appraisal and management of Vogt-Koyanagi-Harada disease

Objective: The goal of this study was to analyze indocyanine green angiographic (ICGA) findings in Vogt-Koyanagi-Harada (VKH) disease and to determine their value in assessing choroidal involvement as well as their use for diagnostic and follow-up purposes. Design: Retrospective and prospective observational, interventional case series. Participants: Ten patients with VKH disease documented with, for the retrospective cases, at least one concomitant fluorescein and indocyanine green angiogram and, for the prospective cases, follow-up angiograms performed regularly. Testing: Indocyanine green angiography was performed according to a standard protocol used for inflammatory disorders. Systemic steroids were used for treatment. Main Outcome Measures: Indocyanine green angiographic findings were correlated with funduscopy, fluorescein angiography, inflammatory activity, disease stage, and response to systemic steroids. Results: In newly diagnosed acute disease with exudative retinal detachments, the main features observed in all three patients were: (1) signs indicating choroidal inflammatory vasculopathy, including choriocapillaris perfusion delay in the very early angiographic phase, perivascular leakage of individual vessels in the early phase, diffusely leaking fuzzy vessels in the intermediate phase, and diffuse choroidal hyperfluorescence in the late phase; (2) hypofluorescent dark dots during the intermediate phase of angiography, either becoming isofluorescent in the late phase of the angiogram or remaining hypofluorescent, probably representing partial or full-thickness granuloma; (3) disc hyperfluorescence indicating severe papillitis; and (4) hyperfluorescent pinpoints in the area of exudative retinal detachment. Recurrences in the six patients with chronically evolving disease did not show the hyperfluorescent pinpoints. Otherwise, they showed the same features, albeit less pronounced, together with peripheral atrophic hypofluorescent lesions. In the two patients with "healed" disease for whom high-dose steroids had been initiated at an early stage, only dark hypofluorescent areas in the intermediate and late phases on the fluorescein angiogram were seen, probably representing choroidal scarring. Conclusions: Consistent ICGA findings in 10 VKH patients allowed the authors to establish a fairly precise pattern of choroidal involvement. Indocyanine green angiography was especially useful to observe the evolution of choroidal inflammatory involvement and to monitor the effect of steroid therapy. Ophthalmology 2001;108:54-64 (C) 2001 by the American Academy of Ophthalmology.