Posters

Retinal astrocytic hamartoma as part of Bourneville's tuberous sclerosis (BTS)

Poster Details

First Author: A.El Bouaychi MOROCCO

Co Author(s): A. Ridallah   F. Boudguigue   S. Jouahri   L. Harrak   O. Cherkaoui        

Abstract Details

Purpose:

Assess the utility of fluorescein angiography and SD-OCT in the diagnosis of astrocytic hamartoma

Setting:

Hopital des spécialités, Ophtalmology A service, Rabat

Methods:

29 years old patient, having as antecedent: iron deficiency anemia undergoing treatment, followed in dermatology for skin lesions with angiofibromas in the chin and the wings of the nose (in butterfly wing) causing suspicion of BTS, and in pneumology for dyspnea with a chest CT showing bilateral pulmonary micro-cystic images. The patient was referred to us for ophthalmological examination to support the diagnosis of BTS.

Results:

the patient's best corrected visual acuity is 10/10 with a refraction of -1 in both eyes the anterior segment is normal the fundus in the left eye shows a raspberry tumor, below the upper temporal branch, whitish, partially calcified, of 1.5 papillary diameter, evocative of an astrocytoma type 2. Fluorescence angiography showed hypofluorescence at early frames followed by hyperfluorescence originating from leakage in late frames SD-OCT of the tumor showed hyperreflectivity at its surface, internal retinal disorganization with no view of deeper layers due to shadowing, discrete internal moth-eaten optically empty spaces representing intralesional calcification or intratumoral cavities

Conclusions:

Retinal lesions during BTS are dominated by the presence of astrocytic hamartomas essentially around the papillae which can be easily detected by angiography, especially type 1 lesions which are difficult to visualize by funduscopy and characteristic moth-eaten spaces, and posterior shadowing in SD-OCT.  They are often an incidental diagnosis and evolve slowly. Except in complicated cases, their prognosis is usually satisfactory.

Financial Disclosure:

None