Aim: This paper presents a 20-year review of retinoblastoma in Johannesburg, South Africa, aiming to better characterize the disease in this sub-Saharan setting. Methods: The study represents a retrospective case series of retinoblastoma patients presenting to Charlotte Maxeke Johannesburg Academic Hospital and Chris Hani Baragwanath Academic Hospital between January 1, 1992, and December 31, 2011. Results: The total number of cases identified was 282, with 245 meeting the study inclusion criteria. Retinoblastoma comprised 6.9% of the total pediatric oncology presentations; 65.3% were unilateral, 34.3% bilateral, and 0.4% trilateral. The overall male-to-female ratio was 1.08. The mean age at presentation overall was 32.6 months (median 28.0), in the unilateral group 39.4 months (median 33.0), and in the bilateral group 19.7 months (median 17.0). The mean delay to presentation overall was 7.0 months (median 4.0). The most frequent presenting symptoms were leukocoria (37.1%) and proptosis (34.7%). The distribution of disease stages at presentation (International Retinoblastoma Staging System) was 1.6% stage 0, 24.1% stage I, 27.8% stage II, 16.3% stage III, and 25.3% stage IV. 26.5% defaulted care. The 5-year Kaplan-Meier survival estimate was 57.7% overall. Conclusion: This study shows that delayed presentation and refusal of therapy remains a significant barrier to effective treatment in this African setting.

Purpose:The purpose of this study was to report the medium-term outcome of our index case of Descemet stripping only (DSO) in the clinical setting of Fuchs endothelial corneal dystrophy with pancorneal guttae.Methods:This was a retrospective case report.Results:A 44-year-old woman with bilateral Fuchs endothelial corneal dystrophy was referred for consideration of DSO. At initial slit-lamp examination, widespread guttae were observed with no clear zone visible. Confocal microscopic examination also failed to isolate a population of undisturbed endothelial cells. DSO with supplemental ripasudil was performed with corneal clearance achieved at 2.5 months. A stable result was obtained for 18 months with a subsequent slow decline in vision and return of diurnal fluctuation. At 3.5 years after DSO, DMEK was performed with resolution of symptoms.Conclusions:Medium-term failure in this clinical setting is further evidence that DSO is likely best offered to patients with central guttae but a clear corneal periphery, indicative of a healthy cell reservoir.