Trilateral retinoblastoma is the association of hereditary bilateral or unilateral retinoblastoma with a pineal neuroblastic tumour. We describe two cases of trilateral retinoblastoma from a total of 141 cases of retinoblastoma seen over an 8.5 year period. Both had a fatal outcome, with survival times of only 4 and 8 months respectively.
Retinoblastoma was clinically diagnosed in 38 patients in the University Hospital between 1968 to 1988. White pupil or cat's eye reflex, found in 25 patients, was the most common symptom. Four patients refused any form of treatment. Of the 27 histopathologically confirmed cases, 16 were males and 11 were females. There was no ethnic group predominance, although a male preponderance was observed among Malay patients (M:F 3:1, p less than 0.05). Treatment in cases with unilateral disease, without extra ocular extension consisted of enucleation of the involved eye, with close examination of the contralateral eye in follow-up. In cases presenting with bilateral tumour the worse eye was removed. Of 20 cases of histopathologically proven unilateral retinoblastoma treated surgically, 12 patients survived for a mean period of 4.5 years (Range: 1-19 years). The defaulter rate for this group of patients was 55%. None of the 7 cases of bilateral retinoblastoma survived beyond 5 years (Mean survival 2.5 years).
Retinoblastoma is a childhood ocular cancer. The aim of this paper is to describe the clinical and epidemiological characteristics of patients with retinoblastoma in a major paediatric ophthalmology center in the country. Retrospective information was collected through the retinoblastoma registry. Late presentation with advanced staging is a major problem.
Retinoblastoma, the most common primary intraocular malignancy of childhood, usually presents in the first three years of life. Atypical presentation of retinoblastoma can masquerade as virtually any ocular or orbital pathology, which may lead to diagnostic dilemmas especially in the presence of other systemic diseases. We report a 20-month-old boy who was diagnosed with coronary aneurysm as a complication of Kawasaki disease, and presented with sudden left eye redness. His mother noticed the presence of white pupillary reflex three months earlier. Atypical acute ocular presentation secondary to Kawasaki disease was initially suspected, but the presence of multiple calcification and mild proptosis on imaging suggested characteristics of advanced retinoblastoma. Histopathological examination of the enucleated eye, which revealed a classical rosette pattern appearance, confirmed the diagnosis. Atypical presentations of retinoblastoma are usually associated with advanced disease. The presence of other systemic conditions further complicates the diagnosis. Early diagnosis is important to reduce the mortality and morbidity.