A dissertation submitted to the Faculty of Health Sciences, University of the
Witwatersrand, Johannesburg, in fulfillment of the requirements for the degree
of Master of Medicine in Ophthalmology
Johannesburg, 2014 / Retinoblastoma is the most frequent malignant intraocular tumour of
childhood, and in Africa it is the commonest and most important life
threatening ocular neoplasm. In resource-rich countries such as the United
States and Canada, survival rates for patients with retinoblastoma approach
100%. This is in stark contrast to developing countries where survival rates
may be less that 20%. Despite the vast majority of affected patients living in
less developed countries, there is little attention and published literature on
disease characteristics in these populations.
OBJECTIVES: To characterise retinoblastoma in the South African population
through a 20-year retrospective analysis of patient records at two tertiary
academic hospitals in Johannesburg.
DESIGN AND METHOD: 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.
RESULTS: The total number of retinoblastoma cases identified was 282, with
245 of these meeting the study inclusion criteria. Retinoblastoma comprised
6.9% of total paediatric 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), for
unilateral 39.4 months (median 33.0 months) and for bilateral 19.7 months
(median 17.0 months). The mean delay to presentation overall was 7.0
months (median 4.0 months), for unilateral 8.5 months (median 5.0 months)
and for 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 (using the 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 (data not available in
4.9%). 26.5% of patients defaulted care. The five-year survival rate 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.
CONCLUSION: The five-year survival rate for Stage I disease is similar to the
overall five-year survival rates reported in the developed world. This suggests
that treatment standards at the study hospitals are comparable with those of
developed countries and that similar overall survival rates may be achieved if
patients were to present as early. Delay to presentation, disease stage at
presentation and defaulting care were identified as key factors contributing to
the poor overall survival rate. This study provides information regarding
patient demographics, social challenges in management, and patient
outcomes in comparison to developed countries and to reports from other
African populations. It also highlights the need for educational campaigns and
screening initiatives to address poor survival outcomes as a result of late
presentation and high rates of patients defaulting care. Finally, this report
serves as a platform for comparison with future research in this area.
| Identifer | oai:union.ndltd.org:netd.ac.za/oai:union.ndltd.org:wits/oai:wiredspace.wits.ac.za:10539/17502 |
| Date | 22 April 2015 |
| Creators | Goolam, Saadiah |
| Source Sets | South African National ETD Portal |
| Language | English |
| Detected Language | English |
| Type | Thesis |
| Format | application/pdf |
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