Abstract
Intravitreal chemotherapy (IVitC) in the management of retinoblastoma has increased the rate of globe salvage, specifically in patients with recurrent disease and associated vitreous seeds. A significant number of children with retinoblastoma in developing countries present late, resulting in higher intraocular tumour-stage at presentation. Treatment requirements for such tumours usually include intravenous chemotherapy (IVC) and/or intra-arterial chemotherapy (IAC). While IVC has a long usage track record and a good efficacy, it has been reported to be associated with higher recurrence rates in a significant number of patients. Intra-arterial chemotherapy has the advantage of lower recurrence rates but requires personnel with advanced interventional radiology skills and has limited efficacy in treating intravitreal seeds. Intravitreal chemotherapy has gained popularity recently, largely because of its superior efficacy in the management of vitreous seeds, subretinal seeds and recurrent retinal tumour. An 8-month-old male infant initially presented with bilateral retinoblastoma, International Classification System for Intraocular Retinoblastoma (ICRB) Group E in the right eye and Group B on the left eye. The right eye was enucleated and currently has a prosthesis. The left eye had tumours that initially responded to brachytherapy and transpupillary thermotherapy (TTT). Approximately two years later his tumours recurred with vitreous seeds and were successfully managed with the use of cryotherapy and intravitreal chemotherapy. The simplicity of the technique of IVitC and its efficacy in controlling vitreous seeds and recurrent retinal tumours makes this route of regional chemotherapy a viable one in areas with limited expertise and resources such as South Africa.
Highlights
Retinoblastoma is the most common intraocular malignancy reported in children.[1]
We present a retinoblastoma patient whose management included intravitreal chemotherapy (IVitC) for recurrences and vitreous seeds
The child was referred to the only facility that offered brachytherapy in South Africa at the time, three months from initial presentation, where he received brachytherapy to the three tumours and transpupillary thermotherapy (TTT) to another tumour, all in his left eye. He underwent regular examination under anaesthesia (EUA) and RetCam photos for a period of two years and four months, during which time the tumours remained inactive. He was later observed to have recurrences with overlying vitreous seeds, when he presented for his routine follow up (Figure 1 – RetCam photographs showing lesions of concern) and was started on cryotherapy to the lesions and multiple three weekly IVitC injections of both Melphalan (20 μg in 0.1 mL) and Topotecan (20 μg in 0.1 mL) over a period of ten months
Summary
Retinoblastoma is the most common intraocular malignancy reported in children.[1]. It accounts for 2% – 4% of childhood cancers in the developed world compared with 10% – 15% in Africa where it is a significant life-threatening neoplasm.[1,2,3] The overall 5-year survival rate in a 20-year review of the disease in Johannesburg, South Africa was reportedly 57.7%. He underwent regular EUAs and RetCam photos for a period of two years and four months, during which time the tumours remained inactive He was later observed to have recurrences with overlying vitreous seeds, when he presented for his routine follow up (Figure 1 – RetCam photographs showing lesions of concern) and was started on cryotherapy to the lesions and multiple three weekly IVitC injections of both Melphalan (20 μg in 0.1 mL) and Topotecan (20 μg in 0.1 mL) over a period of ten months. The tumours and the vitreous seeds regressed with this treatment and remained inactive at two months follow up (Figure 2 – RetCam photographs showing regression of tumours with a calcified appearance) This treatment afforded functional vision in his left only eye at the age of four years
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