Abstract

Aim: To ascertain the demographics, clinical features, and treatment options for children with retinoblastoma. 
Methods: This was a hospital-based cohort study of all children with retinoblastoma presenting from 1 January 2008 to 31 December 2008. Demographic data, presenting features, family history, and findings identified during examination were recorded for each patient. The tumours were classified using the Reese- Ellsworth classification system. Documentation of the tumours included examination under anesthesia, ultrasound B- scan, and computed tomography scan as well as histopathological reporting of enucleated eyes.
Results: Twenty seven new patients with retinoblastoma were identified and 36 eyes were affected, with a bilaterality rate of 33%. The frequency was 4.1, with a bimodal peak of presentation of younger than 1 year
and 4 years. The mean age at diagnosis was 3.2 years (SD, 2.5 years; range, 3 months to 8 years). Most of the patients were boys, (59%) and most were in the age range of 1 to 5 years (66%). There was a male preponderance (5:1) amongst patients younger than 1 year and the male to female ratio was equal (1:1) in the 1 to 5 years age range. Leukocoria was the main presentation in 38% of eyes and 19% presented with proptosis. Enucleation/exenteration was performed for 17 unilateral eyes (94%) and 5 bilateral eyes (27%) with group V disease. Groups I and II tumours (n = 1 and n = 7 for unilateral and bilateral eyes, respectively) were treated conservatively with cryotherapy or chemotherapy alone or in combination. The commonest treatment for bilateral disease was enucleation of 1 eye and chemotherapy for the fellow eye (22%). Radiotherapy was advised for patients with histopathological evidence of optic nerve involvement (25%).
Conclusions: Retinoblastoma usually affects infants and children younger than 5 years, with no sexual or racial predilection. One-third of patients demonstrate bilaterality, with leukocoria as the commonest presenting feature and proptosis as a feature of late presentation. In bilateral retinoblastoma, primary enucleation of one eye with conservative chemotherapy and/or cryotherapy of the fellow eye is advocated.