What is Retinoblastoma?

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This week’s blog on ‘Retinoblastoma’ has been contributed by Dr. Namir Kafil-Hussain, Consultant Paediatric Ophthalmologist, Specialist in Children’s Eye Diseases, Strabismus (Squint) and Paediatric Cataract Surgery
Retinoblastoma is an eye cancer that occurs most commonly in childhood under the age of 5. . Two thirds of children are diagnosed before the age of two and almost 95% by the age of 5.
It is estimated that 5000-8000 children develop retinoblastoma worldwide every year. The incidence rate of retinoblastoma in United States and Europe is approximately 1:15000 live births. However, the rate appears to be higher in Africa and India.
Its present in two forms, bilateral (hereditary) form (25% of all cases) and unilateral (75% of all cases, 90% of which are non-hereditary).
The most common presentation is leucocoria which is an abnormal white reflection in the eye (60%) and 20% as squint, the misaligned position of the eye).
The best approach to manage this condition is based on early detection and early diagnosis.
It involves Paediatric Ophthalmologist, Ophthalmic Oncologist and Paediatric Oncologist. The aim of the treatment is primarily to preserve the life of the child. Secondly, to preserve vision and finally, to preserve the eye. It is highly curable if identified in early stages; more than 90% of children survive in high income countries and up to 40% in less privileged countries. Treatment includes radiotherapy, chemotherapy and sometimes enucleation of the eye with tumor. Children with bilateral disease and gene mutation are at higher risk for secondary cancers. This increases with radiation therapy.
It is very important to for parents, Pediatricians, Ophthalmologists and other healthcare professional involved in childcare to detect abnormal white reflection (absence of red reflex) during routine checkups of the children.
Leucocoria can be seen in the eye with the tumor due to the reflection of the white flash light from abnormal white retina in the back of the eye.
In the modern phone cameras, a red eye reduction feature is included so that the red reflex is prevented in the photos. There are few iPhone applications (apps) which are designed to detect the white reflex. However, there are certain limitations with these apps.
A thorough eye examination by a Paediatric Ophthalmologist is essential to detect the tumor in its early stages when concerns arise by parents with these apps or iPhone photos.