Presentation of retinoblastoma at a paediatric eye clinic in Ghana

Abstract

Background: Retinobalstoma, the commonest childhood malignant intraocular tumour, is usually diagnosed early with over 90% survival rate in
developed countries. In developing countries, the diagnosis is late resulting in less than 50% survival.
Objective: To determine retinoblastoma stages at presentation and patients¡¦ outcomes.
Design: Retrospective case series.
Methods: The clinical and histopathological records of children with retinoblastoma seen from May 2004 to December 2005 were studied. Data was analysed for mode of presentation, laterality, clinical staging using
Reese-Ellsworth (R-E) classification, histopathological high risk features (HHRF) for metastasis, and patient outcome.
Setting: Ophthalmology Unit, Korle-Bu Teaching Hospital, Ghana.
Results: Twenty-three patients presented with retinoblastoma over the period. Males were 12(52.2%). The age range from 1 to 84 months, mean 36.3(„b22.15) and median (36 months). Nineteen (82.6%) had
unilateral and 4(17.4%) bilateral disease. The common clinical presentations were leukocoria in 20(87.0%), proptosis 8(34.8%), strabismus 5(21.7%) and red eye 5(21.7%). The clinical features were commensurate with R-E stage V in 20(87.0%) patients, 2(8.7%) with
orbital recurrence and 1(4.3%) with post-enucleation anophthalmos. HHRFs were present in 9(75%) enucleated eyes with invasion of optic nerve as the
commonest site (7/9). The patients were followed up for 1 day to 19 months. Eight abandoned treatment, 2 were discharged for palliative treatment, 2 out of 5 with metastasis died and 6 had no metastases at their last visit. Common sites for metastasis were the bone
marrow, brain and orbit.
Conclusion: Majority of the patients presented with advanced disease manifesting as leukocoria, proptosis, RE stages V disease and poor outcome.