Indocyanine green angiography-guided management of Vogt-Koyanagi-Harada disease: differentiation between choroidal scars and active lesions.

When following Vogt-Koyanagi-Harada disease (VKH), indocyanine green angiography (ICGA) is crucial in the subacute and convalescent stages of the disease in order to detect subclinical choroiditis and prevent the development of 'sunset glow' fundus. Hypofluorescent dark dots (HDDs) indicate persisting granulomas in the choroid. However, probably as a result of the healing process of choroidal granulomas, stromal choroidal fibrosis can also be shown by HDDs. We present two cases where intravenous corticosteroid administration because of persistent HDDs led to resolution of lesions in one case while they persisted in the other case. We reviewed the medical history of two VKH patients. Complete routine work-up for patients with posterior uveitis was performed. The charts were screened for the presence of HDDs by ICGA in the subacute and convalescent stages of the disease before and after administration of body weight-adapted pulse intravenous methylprednisolone (PIM). The evolution of HDDs was studied and compared in both patients. A female patient presented with a persistent bilateral granulomatous panuveitis compatible with VKH. Cerebrospinal fluid analysis had shown lymphocytic pleocytosis. At presentation, therapy consisted of oral prednisone 80 mg/day. Prednisone was tapered down to 22 mg/day over 3 months, when a recurrence occurred with the presence of disseminated HDDs. PIM was administered, followed by oral corticosteroids. After 8 days of therapy, ICGA showed an almost complete disappearance of HDDs. A girl presented with bilateral panuveitis and widespread depigmented areas of her fundus. Cerebrospinal fluid analysis showed monocytic pleocytosis. Because of relative resistance to oral inflammation suppressive therapy (IST), PIM was administered for 3 days. Nevertheless, ICGA showed persistence of HDDs. Therapy was continued, and 3 months later, a follow-up ICGA still depicted numerous HDDs. Another PIM course was given, which had no effect on ICGA signs. HDDs in this case were interpreted as stromal choroidal scars. ICGA-guided therapy (mainly HDD evolution monitoring) helps to eradicate occult stromal disease in VKH and avoids 'sunset glow' fundus, by allowing precise adjustment of therapy. In some cases, HDDs do not represent active lesions but presumed intrastromal scars which need to be identified. A limited course of maximal IST including PIM can unmask such cases and avoid overtreatment of these patients.