Thursday, November 18, 2010

Glad that Children don't Have to Suffer Like That

Retinoblastoma is the most common tumour of the eye in children. It has an incidence of 4 per million population. With a population of 27 millions, one can expect 108 cases per year in Malaysia. Most of the cases were picked up during routine eye screening and the most common sign is the presence of white pupil (leucocoria). However, in places where parents don't have easy access to basic health screening, retinoblastoma can develop into disfiguring tumour pushing the eye out of the eye socket. The most common cause of death is spread of the tumour into the brain. Fortunately, with chemotherapy and radiotherapy most patients have a good chance of survival.
Here are some photos and illustrations of retinoblastomas from the 19th century textbooks. Unfortunately, these presentations can still be seen in many developing countries if you google retinoblastoma.

Illustrations of a child with retinoblastoma while alive. The tumour had spreaded
to the skull and brain. The picture on the right was drawn after the child
died and the skin removed.

Pictures of a child with retinoblastom from 1885.

The same child after operation but no front picture was
shown. Without chemotherapy and radiotherapy, the child
was likely to succumb to the disease within a short time even
if the tumour were removed.

In Malaysia, such advanced presentation are rarely seen. This child from a poor family and lived in remote area presented wtih a left swollen blind eye. Although the social worker arranged for the patient to be treated in Sarawak General Hospital, Kuching, the family repeatedly defaulted until the left eye became very swollen. By the time, we saw the patient the left eye was totally destroyed by the tumour and the tumour occupied the whole eye socket. The patient underwent chemotherapy in the children cancer centre and within a month, the tumour shrunk to nothing. Repeated follow up and regular brain scan are needed to detect any early recurrence.
Left eye swollen and painful caused by retinoblastoma.

Tumour and the eye destroyed by the retinoblastoma
shrunk following chemotherapy.

Unfortunately, this tumour is likely to be with us as it is caused by genetic defects. Patients who survive the tumours can pass the genes on to their next generation. So there is a possibility that we will see more and more of this tumour in the year to come as more and more patients survive. This reminds me of an old English consultant whom I used to work for in the UK. He was a Germanophile and appeared to be a strong supporter of the Third Reich. Whenever, he saw a family with two children having this tumour. He would say "If Hitler were around, these people would not be allowed to reproduce!" He was nicknamed Adolf by the clinic nurses ;).

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