Autofluorescence and Angiographic Findings of Retinal Astrocytic Hamartomas in Tuberous Sclerosis

Abstract

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.

Autofluorescence of intraocular tumours

“…Astrocytic hamartoma is an uncommon retinal tumour found in patients with tuberous sclerosis complex or as a sporadic condition [38]. This benign retinal lesion is classified into three morphological groups: types 1, 2 and 3.…”

Drusen Characteristics Revealed by Spectral-Domain Optical Coherence Tomography and Their Corresponding Fundus Autofluorescence Appearance in Dry Age-Related Macular Degeneration

“…Changes in the normal FAF appearance may be among the first signs of retinal disease before they are evident funduscopically [4] . Alterations on FAF images have been reported to correlate with reduced photoreceptor function and retinal sensitivity [15][16][17][18] .…”

Protein S deficiency manifesting simultaneously as central retinal artery occlusion, oculomotor nerve palsy, and systemic arterial occlusive diseases

“…7 Though our case is sporadic, it has features of type 1 on FA and type 2 on ICG, suggesting incomplete calcification. OCT features are also consistent with retinal astrocytic hamartoma.…”