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Saadiah Goolam, Nicky D Welsh, Ismail Mayet; Retinoblastoma in South Africa - A 20-year retrospective study at two tertiary academic hospitals in Johannesburg. Invest. Ophthalmol. Vis. Sci. 2015;56(7 ):82.
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To characterise retinoblastoma in the South African population through a 20-year review of patient records at two tertiary academic hospitals in Johannesburg
Retrospective clinical case series analysis of medical records of patients with retinoblastoma presenting to Charlotte Maxeke Johannesburg Academic Hospital and Chris Hani Baragwanath Academic Hospital between 01 January 1992 and 31 December 2011
The total number of cases identified was 282, with 245 meeting the study inclusion criteria. Retinoblastoma comprised 6.9% of total pediatric oncology presentations. 65.3% were unilateral, 34.3% bilateral and 0.4% trilateral. The overall male to female ratio was 1.08. Mean age at presentation overall was 32.6 months (median 28.0 months), unilateral 39.4 months (median 33.0 months) and bilateral 19.7 months (median 17.0 months). The mean delay to presentation overall was 7.0 months (median 4.0 months), unilateral 8.5 months (median 5.0 months) and bilateral 4.4 months (median 3.0 months). The most frequent presenting symptoms were leukocoria (37.1%) and proptosis (34.7%). Distribution of disease stage at presentation (International Retinoblastoma Staging System) was 1.6% with Stage 0, 24.1% with Stage I, 27.8% Stage II, 16.3% Stage III and 25.3% Stage IV. 26.5% of patients defaulted care. The five-year survival rate using the Kaplan-Meier survival curve was 57.7% in the overall study population, and according to disease stage at presentation: 95.3% - Stage I, 84.8% - Stage II, 49.7% - Stage III and 5.7% - Stage IV
This study showed that delay in presentation of retinoblastoma cases remains a significant barrier to effective treatment in this African setting. Intervention to streamline referrals is indicated and may include outreach programs and education of referring hospitals
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