Chu Luan Nguyen1,2, David Pahau1

Purpose: Retinoblastoma is the most common primary ocular malignancy in children. It is fatal without treatment, however there is potential for high survival rates with early presentation. This case report highlights the impact that socioeconomic factors have on delayed presentations of retinoblastoma, and its management.

Method: A case report set in Wewak, Papua New Guinea. A three-year-old girl from a rural village, presented to a district hospital with a right fungating globe and leukocoria of the left eye.

Results: Fundus examination of the left eye under general anaesthesia showed a large white mass in the posterior pole of the retina, and B-scan ocular ultrasound showed a hyperechoic intraocular lesion with irregular contours. The clinical diagnosis was bilateral retinoblastoma with orbital invasion in the right eye. Exenteration was performed on the right side. Imaging, histopathology analysis, and other treatment modalities were not available. Surgery on the fellow eye in the future was discussed, but the parents refused further treatment.

Conclusion: The case illustrates the devastating natural history of retinoblastoma if diagnosis is delayed, and highlights the impact that socioeconomic factors have on late presentations of retinoblastoma. The case is a reminder that while ocular salvage is a priority in developed countries, morbidity from delayed presentation of retinoblastoma and limited management options is still a major issue in least developing countries.