A mother of a one-year-old boy, from Dailekh, far western Nepal noticed white glow in the left eye of her son recently. She took her son to the local eye hospital from where he was promptly referred to Tilganga Institute of Ophthalmology for further management. The boy was examined under anesthesia at TIO by us and diagnosed to have Retinoblastoma in both his eyes, larger and multiple tumors in his left retina and two smaller tumors in his left retina. CT scan was done which did not show any extraocular extension or optic nerve invasion. The final diagnosis being RE group B and LE group E Retinoblastoma, Stage 0.
He was referred to Kanti Children Hospital for metastatic workup and standard dose chemotherapy for 6 cycles. In between every chemo cycle, he underwent serial EUAs along with transpupillary thermotherapy (TTT) application to his better eye. After completing 6 doses of chemotherapy, he underwent LE enucleation with an implant with financial aid from Open Eyes Nepal since she could no longer afford her son’s treatment. He received custom fit prosthesis in left socket and further 2 doses of TTT in RE with financial aid from the same organization. Currently, the boy is disease free but on regular 3 monthly follow up.
Retinoblastoma represents 3% of all childhood cancers, and is the most common intraocular malignancy of childhood in the world. It is fatal if left untreated. Early detection can save life and eye. Worldwide prevalence is 1:15000 to 1:20000 live births. Exact prevalence in Nepal is not known but a recent survey done in multiple hospital records suggests that around 50 new cases are detected in Nepal each year. Among them, the Ocular oncology department of TIO sees 35 to 45 new cases per year with around 15 of them having bilateral diseases.
Treatment for Retinoblastoma is long, expensive and psychologically challenging to the parents and children. Stage 1 and 2 disease requires at least 6 months of systemic chemotherapy along with 4 weekly evaluations under anesthesia along with needful local treatment like transpupillary thermotherapy (TTT), cryotherapy and local chemo in the eye (periocular and intravitreal chemotherapy) until the tumor resolves completely. Many times in spite of all the measures, the eye needs to be enucleated eventually to save the child’s life. However, for children who present to hospital late, in stage 3 or 4 disease, there is an increased risk of mortality in spite of high dose 12 cycles of chemotherapy and radiation.
The rate of mortality in retinoblastoma (even with treatment) in the developed world is 5% while it is 40% in the developing world. This large discrepancy is due to early detection in the stage of “white eye reflex”, when life as well as the eye can likely be saved. In countries like Nepal, due to lack of awareness about this cancer among parents and even health care personnel, the children are often brought late in the course of disease. Late presentation means advanced disease requiring longer treatment duration and higher mortality and morbidity even with treatment. All white eye reflexes (leukocoria) may not be retinoblastoma, but it is not the normal color of the pupil. It could be cataract or other congenital retinal problems which also need timely diagnosis and treatment.
Hence as health personnel we should spread an important message that- white reflex is not normal reflex- refer the child immediately to higher center to save the eye and life.
