Abstract
Purpose: :
To describe the features of retinal astrocytic hamartomas (RAHs) in Tuberous Sclerosis (TS) using Spectral-Domain Optical Coherence Tomography (SD-OCT) and Autofluorescence (AF)
Methods: :
Features of SD-OCT and AF (HRA SPECTRALIS, Heidelberg Engineering) images of 14 patients affected by TS with RAHs.
Results: :
There were 14 Caucasian patients with TS (27 eyes), 7 females and 7 males, the mean age was 25.5 years (SD 5.32, range 19-32). The localization of the RHAs was 45.5% superotemporal, 18.2% inferotemporal, 27.3% peripapillary, 9% paramacular. 63.63% of the RHAs showed hypoautofluorescence and 46, 37% showed hyperautofluorescence consistent with intratumoral calcification. By SD-OCT 4 types of RHAs were identified: Type I RHAs (38%) were mildly elevated inner retinal lesions without retinal traction, with a gradual gently sloping transition from a normal retina into the tumours, Type II RHAs (38%) demonstrated retinal traction and retinal disorganization limited to inner retina, Type III RHAs (20%) showed dome shaped, moth-eaten appearance with intralesional calcifications, Type IV RHAs (6%) showed a dome shaped lesion, with an optically empty, cavitary, intralesional space.
Conclusions: :
RHAs showed characteristics features on AF and SD-OCT. With AF it was possible to classify the tumours in hypoautoflourescent lesions, consistent with Type I and II, and hyperautofluorecent lesions as a result of calcification typical of Type III. SD-OCT showed gradual transition from a normal retina into an optically hyper-reflective mass with retinal disorganisation, characteristic moth-eaten spaces and posterior shadowing. It has also been recognized a new particularly feature of RHAs characterized by a cavitary space within the astrocytic hamartoma (Type IV).
Keywords: tumors • retina • imaging methods (CT, FA, ICG, MRI, OCT, RTA, SLO, ultrasound)