DISCUSSION
Late presentation is a major challenge in management of
Table 1Age distribution of 34 children with retinoblastoma(%)
Age (years)SexMaleFemaleTotalPercent1123921031338393123541013511266101372026Total25934100
retinoblastoma in developing countries. Greater majority of the patients in our series presented at advanced stages with extraocular extension and orbital involvement. Most parents seek alternative medicine treatment modality first and resort to orthodox treatment when everything has failed.
In developed countries, initial detection of abnormalities in the eye suggestive of retinoblastoma by family members rather than physicians is associated with a higher incidence of enucleation[3]. Preservation of eye was greatly improved when physicians first detected the abnormalities. This, however, entailed serial examination under anaesthesia[2,3].
The serial examination under anaesthesia is designed for patients with family history of retinoblastoma. In the current series, none of the patients admitted a positive family history of the disease. This may stem from denial or ignorance. Moreover, most retinoblastoma patients in rural Africa do not survive to the age of reproduction.
Six patients (18%) had bilateral disease which is more often associated with familial disease. Unaffected parents of a child with bilateral retinoblastoma with no family history have a 40% chance of producing another affected child[1]. Such siblings should benefit from serial screening. This may not be achievable in rural Africa due to poverty. The patients and their caregivers travel several kilometres to get to the urban communities where the ophthalmologists practice. Although modern treatment modalities, such as photocoagulation, cryotherapy, external beam irradiation and episcleral radioactive plaque are not widely available in developing countries, enucleation at such early stages cures the patient of cancer[6].
The patients in this series were offered enucleation or exenteration and were subsequently referred to the paediatric oncology unit for chemotherapy. Cyclophosphamide, Vincristine, Methotrexate and Prednisolone were used. Carboplatin and Etoposide were not available. There is a need to provide these drugs. It is advocated that cancer patients in developing countries should be offered free treatment.
Extraocular disease whether to the orbit or central nervous system is associated with increased mortality[79]. Twentythree patients (68%) had exenteration because of spread to the orbit. Availability of effective cytotoxic agents can help achieve chemoreduction, enucleation rather than disfiguring extenteration can now be carried out[10]. After enucleation and excision of all visible residual orbital tumour, orbital radiotherapy followed by four more courses of chemotherapy is advocted[10]. Centres for radiotherapy are not widely available in Nigeria. Poverty is a limiting factor for referrals to such treatment centres. Also facilities for proper evaluation of these patients for metastatic disease such as technetium bone scan, CT scan or magnetic resonance imaging are not widely available. Another setback in the management of retinoblastoma in a developing country is failures in followup. There was no record of followup till death in any of the patients and this has made it difficult to assess the survival rate.
In conclusion, late presentation, poverty and unavailability of modern treatment facilities are some of the challenges in managing retinoblastoma in a developing country like Nigeria. Health education, early diagnosis and provision of modern treatment facilities will go a long way in improving the management of retinoblastoma in developing countries.
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