Choroidal ganglioneuromas in Francois variant neurofibromatosis-1: A rare retinoblastoma mimic
Indian J Ophthalmol
;
2022 Jul; 70(7): 2602-2605
Article
| IMSEAR
| ID: sea-224439
ABSTRACT
Neurofibromtosis-1 (NF-1) is the commonest oculo-neuro-cutaneous syndrome with multiple ocular manifestations. Reporting three children who presented with unilateral glaucoma (buphthalmos), ipsilateral facial hemihypertrophy, and eyelid plexiform neurofibroma completing the triad of François syndrome, a rare NF1 variant. Two presented with leukocoria and were referred to as retinoblastoma suspects. Histopathology showed ganglioneuroma, a benign choroidal tumor, associated with NF-1, which does not need treatment. Knowledge of this rare condition avoids misdiagnosis of retinoblastoma, prevents aggressive management, and the associated psychological impact.
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Index:
IMSEAR (South-East Asia)
Journal:
Indian J Ophthalmol
Year:
2022
Type:
Article
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