Retinoblastoma presenting as congenital glaucoma.

An infant girl was referred to our paediatric ophthalmology service as a case of congenital glaucoma not responding to topical medication, presenting with asymmetrical globe enlargement, epiphora, corneal oedema and right eye elevated intraocular pressure of 29 mm Hg. Examination under anaesthesia of the right eye revealed iris neovascularisation, corneal oedema and a retrolental vascularised dome-shaped mass. Examination of the left eye was normal. B-scan ultrasonography demonstrated a bi-lobulated retinal lesion with calcifications extending into the anterior hyaloid to the lens. Since the findings were highly suggestive of retinoblastoma, urgent MRI was arranged which demonstrated a right globe intraocular mass with no optic nerve or intracranial extension. The tumour was classified as Group E retinoblastoma, based on the International Intraocular Retinoblastoma Classification (IIRC) classification system. She underwent right eye enucleation and was fitted with a prosthesis. At nearly 1 year of follow-up, the patient remains clinically well and undergoes regular follow-ups with our retinoblastoma service.