Clinical presentation and management of retinoblastoma at Queen Elizabeth Central Hospital, Blantyre, Malawi

Abstract

Objective: Retinoblastoma is the commonest intraocular malignancy in childhood worldwide commonly affecting children in the first 5 years of life. The primary goal of retinoblastoma treatment is to improve survival. This is achievable through early detection. We conducted this study to report the clinical presentation and management of retinoblastoma in Blantyre, Malawi.
Methodology: This was a retrospective case series in which all files of patients who presented with a clinical diagnosis of retinoblastoma from 1st January to 31st December 2017 were reviewed. Data extracted included clinical presentation, lag time, examination findings, investigations and treatment provided. Categorical variables were summarized as percentages and frequencies whilst continuous variables were analysed as medians.
Results: A total of 43 patient records were retrieved. The median age was 31 months with a median lag time of 3 months (IQR 0.1 – 48 months) with most patients (n =37, 86%) presenting to the retinoblastoma treatment unit within two weeks of being referred. There was no gender preponderance with a male to female ratio of 1:1 Most patients presented with leukocoria (n = 29, 67.4%) and almost a third had proptosis (n = 13, 30.2%).Thirty six patients (83.7%) had a unilateral presentation. Patients were clinically staged using the ICRB staging. There was limited availability of facilities for thorough patient evaluation with 21 (48.8%) patients being examined under anaesthesia, B-scan being performed in 14 (32.6%) patients and only 6 (13.9%) patients undergoing Magnetic Resonance Imaging (MRI). Enucleation and chemotherapy were the only treatment options available with chemotherapy being given to 40 (93%) of the patients and 28 (65.1%) children underwent enucleation apart from chemotherapy over the study period.
Conclusions: The median lag time to presentation was 3 months and most of the delay in presentation was at the level of the patient before the first contact with the health system. There is a need to improve treatment options for retinoblastoma at Queen Elizabeth Central Hospital whilst also improving community awareness and early case detection at the primary level of health care.