Autofluorescence and Angiographic Findings of Retinal Astrocytic Hamartomas in Tuberous Sclerosis

OBJECTIVE: To describe fundus autofluorescence (AF), fluorescein angiography (FA) and indocyanine green angiography (ICGA) in different types of retinal astrocytic hamartomas in tuberous sclerosis (Morbus Bourneville-Pringle). METHODS: Two eyes with 8 lesions, i.e. type 1 (n = 7) and type 3 (n = 1), were examined. AF pictures were taken prior to injection, FA and ICGA images were obtained in the early and the late phase. To achieve additional cases, a systematic literature review with exten- sive Internet and library search was performed. RESULTS: Strong AF was seen in type 2 and type 3 retinal astrocytic hamartomas, whereas type 1 lesions blocked the physiologic fundus AF. Fluorescence angiography of all types of lesions revealed hypofluorescence in early frames and hyperfluorescence originating from leakage in late frames. ICGA showed a subtle blockade in type 1, a total blockade in type 2 and in the central part and a partial blockade in the peripheral part in type 3 lesions. CONCLUSIONS: Retinal astrocytic hamartomas in tuberous sclerosis can be easily detected by angiography, especially type 1 lesions which are difficult to visualize by funduscopy. Early- and late-phase fluorescein angiography and ICGA are helpful to differentiate the three lesion types.