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Masquerade syndrome

Photo by Dr. Yew, Dr. Vanitha and Professor Chua

A 12 year-old boy presented with a poor right vision. Slit-lamp examination showed a dense cataract with 3+ cells in the anterior chamber. Following lens aspiration and implant, a white mass was found in the superior retina. Cytology of the cells from the anterior chamber revealed Flexner-Wintersteiner rosettes strongly suggestive of retinoblastoma. There was no family history of any ocular tumours. CT scan showed no evidence of calcification or extra-ocular extension. Cryotherapy was performed but the cells in the anterior chamber increased and formed pseudo-hypopyon. Repeated cytology confirmed the presence of rosettes. Enucleation was performed with a long stalk of optic nerve. Bisection of the globe showed a ring of tumour at the anterior retina just behind the ciliary body. Histology showed no optic nerve invasion but tumour cells were found in the choroidal space. He is currently under the care of the paediatric oncologist.

 

Right pseudo-hypopyon.   Right eye showing pseudo-hypopyon and tumour infiltrating the iris.   A close-up view of the iris showing clusters of tumour cells.   Enucleated globe with a long optic nerve. CT scan showed no signs of calcification.   Bisected globe showing a ring of tumour behind the ciliary body. The greyish white area on the right was caused by cryotherapy.

Atlas of Paediatric Ophthalmology