A Case of Alport Syndrome Presented with Bilateral Anterior Lenticonus

Abstract

Retinoblastoma is the most common primary intraocular tumor in children with an incidence of 1: 16,000 to 18,000 live birth. Every year worldwide newly detected cases are about 8000 and in India about 1400. It represents 11% of cancer that develop in the first year of life. Current revolutionary management strategy has increased the survival rate of retinoblastoma above 95% in developed country and it is the highest among all pediatric cancer. But still it is a deadly cancer worldwide. Survival from retinoblastoma based on income >90% vs 40% (in high to low income countries). Occurrence of metastases is higher in low-income countries (32% vs 12% in middle-income). Forty three (43%) of global burden lives in 6 countries of Asia (India, China, Indonesia, Pakistan, Bangladesh & Philippines). Mortality rate varies in different continents. An estimated worldwide death rate is more than 40% and most of them from Asia and Africa. Bangladesh is one of the developing countries of the South- East Asia region and retinoblastoma constitutes 83% of all pediatric cancer bellow 4 years of age. For proper management of retinoblastoma with an international standard, establishment of retinoblastoma center consisting of Ocular oncologist, Clinical oncologist, Radiation oncologist, Pediatrician, Oculoplastic surgeon, Retina specialist, Pediatric ophthalmologist, and Ocularist is needed. Management include proper diagnosis, treatment of the disease, genetic counseling, regular follow up, rehabilitation of survivor and screening of sibling. Chittagong Eye Infirmary & Training Complex which is a tertiary center and one of the referral centers of the Bangladesh is treating retinoblastoma since its inception. Due to demand of time the hospital has been reorganized the various facilities to serve retinoblastoma patients with a team approach from 2017. From January 2017 to March 2022 total 304 patients were diagnosed. Among them 132 received VEC (Vincrisrine, Etoposide, Carbplatin) chemotherapy from this center and 79 underwent enucleation with long optic nerve. The hospitals also screen sibling, provide visual and psycho-social rehabilitation of the RB survivors and run awareness program in community level.