A 5 Year’S Retrospective Case Series On The Clinical Profile And Management Of Retinoblastoma At Maputo Central Hospital, Mozambique

Abstract

Background: Retinoblastoma is the commonest intraocular malignancy in childhood. The national epidemiological characteristics of retinoblastoma in Mozambique are not clearly known. Early diagnosis and appropriate treatment leads to a favourable outcome while a delayed diagnosis and inappropriate treatment can be fatal. Objective: To determine the pattern of presentation and management of retinoblastoma patients at Maputo Central Hospital during a 5 year period (1/01/ 2010 to 31/12/ 2014) Methodology: The study was a descriptive retrospective case series carried out at the ophthalmology Services, Paediatric Oncology ward and theatres of Maputo Central Hospital. Records of all retinoblastoma patients within the identified timeframe were retrieved with the help of the specific file number. Demographic characteristics, clinical presentation, investigations and management modalities of retinoblastoma patients as well as discharge/death records were retrieved. Data was extracted and entered into a structured questionnaire and analysed. Results: A total of 57 patients from 7 different provinces of Mozambique had clinical, histological or both clinical and histological diagnosis of retinoblastoma. The overall mean age at presentation was 30.5 months (CI 24.65 - 36.05) (SD 21.5); 82.4 % of cases had unilateral retinoblastoma while 12.3 % of cases had bilateral (mean age at presentation was 30.2 months and 26.4 months respectively).The sex was almost equally represented among the patients with an F: M ratio of 1.2:1. The main presenting complaint was white reflex(62.5 %) followed by eye swelling (50 %) , eye redness (40 %) and poor vision (32.5%) while the main clinical sign were leukocoria (55%) followed by proptosis (50%) and eye redness (27.5%). Out of the 51 eyes who had surgery: 96 % did not have documentation of the choroid and 98 % of the scleral, but 54.9 % of the eyes had optic nerve involovement, 33.3 % had resecction margin involvement and 31.4% had periocular tissue involvement. The main modalities of management were enucleation (93.9%), chemotherapy (24.5%) and exenteration (18%). The eligible patients for chemotherapy were 67.7% but only 35.3% of the patients received chemotherapy. Conclusion: Many patients presented at older ages and late with advanced orbital disease. Majority of the patients had unilateral retinoblastoma. Significant number of patients had optic nerve, resection margin and periocular tissue involvement noted on histology. The main modalities of management were enucleation, exenteration and chemotherapy, although there were no clear eligibility criteria for chemotherapy in retinoblastoma patients who needed