Very rare. Considered as a benign (or spontaneously regressive) form of retinoblastoma (see this term), usually asymptomatic and unilateral. Some cases are due to a mutation of the RB1 gene, such as retinoblastomas: these cases are at high risk for developing a secondary tumor such as skin melanoma, osteosarcoma or carcinoma.

Often discovered by chance at fundoscopic examination: white-gray mass with calcified splinters floating in the vitreous. At fluorescein angiography, the mass is almost avascular. The echography shows a calcified tumor projecting an orbital echographic shadow from which calcifications (lining  the detached posterior hyaloid) expand.

Since a malignant transformation of the retinoma into retinoblastoma is possible at any time, long-term follow-up of the lesion (and family screening for retinoblastoma) is essential.

Anesthetic implications: 

frequent ophthalmologicl examinations

References : 

-        Lando L, Mallipatna A, Gallie B. 
A white retinal lesion with calcification in an 11-year-old boy.
JAMA Ophtalmology 2022 ; July

Updated: July 2022