Rb-NET Challenges 2023
Thank you for participating in the second Rb-NET Challenges Webinar! During the event, we had the privilege of having Prof. Alfred Sommer from the Johns Hopkins Bloomberg School of Public Health, as our guest speaker. He enlightened us with his groundbreaking research on the correlation between Vitamin A deficiency, blindness, and childhood mortality rates. Subsequently, we delved into case presentations by prominent experts in the field of retinoblastoma.
Vera Essuman – Ghana
Presented a case of a 5-year-old girl with suspected hypopyon in the left eye, who was admitted for pediatric treatment due to a recent URI with cough. She had a three-year history of an “eye condition” involving surgery on the other eye. When asked the audience, “what would you do next?” most opted for a full eye examination, which revealed retinoblastoma with active lesions, despite previous treatments (including enucleation of the contralateral eye, local and systemic therapy, which resulted in a chest infection.) This case emphasizes the crucial need to examine the contralateral eye in every child, especially when the pathology is not clear.
Ashwin Reddy – UK
Presented a case involving a 2-year-old boy with a suspicious yellowish exophytic mass and abnormal vasculature in the left eye. Upon asking the audience, “what would you do next,” various options, including enucleation and systemic chemotherapy, were considered. Mr. Reddy emphasized the importance of continued follow-up since the mass did not clinically resemble retinoblastoma. At the 3-month follow-up, with additional examinations such as IV Fluorescein, Coat’s disease was diagnosed. This case underscores the necessity of a thorough ophthalmic evaluation and ancillary testing before initiating treatment, given the diverse potential diagnoses in cases resembling retinoblastoma.
Francis Munier – Switzerland
Presented a case of a 1-year-old with bilateral Group D and Group B retinoblastoma, who underwent various treatments, including intravitreal and intra-arterial chemotherapy, proton therapy, and secondary enucleation of the right eye. Although the child was tumor-free in the retina and vitreous, aqueous seeding was observed and supported by UBM. When asked, “what would you do next?” most audience members suggested intracameral chemotherapy. Dr. Munier highlighted the novel approach of intracameral chemotherapy for isolated aqueous disease, as well as visually demonstrated his technique for injecting chemotherapy into the anterior chamber. The child received four intracameral injections along with concurrent intravitreal chemotherapy to prevent inadvertent posterior spread. At 17-months follow up, the child maintained 20/20 vision with no complications and no metastasis
Purnima Rajkarnikar – Nepal
Presented a case of a 1-year-old boy with leukocoria, diagnosed as Group E retinoblastoma with an intraocular tumor and calcification. Due to parental objection to enucleation, systemic chemotherapy was initiated. However, despite initial improvement, the tumor size increased after six cycles and the optic disc was not visible due to extensive vitreous hemorrhage. When asked, “what would you do next?” half the audience suggested IAC, but enucleation was deemed appropriate, revealing optic nerve involvement on pathology, which upgraded the tumor stage. The child continued high-dose chemotherapy and orbital radiation. This case highlights the fine line between Group E and orbital Rb, stressing the need for proactive treatment decisions, particularly when the optic disc is not visible.
Andrew Stacy – USA
Presented a 4-month-old girl with leukocoria in the right eye, diagnosed with group D equatorial retinoblastoma in the right eye and group B multifocal (including the macula) retinoblastoma in the left eye. Intra-arterial chemotherapy was administered bilaterally for vision salvage, but after one treatment, sudden Phthisis developed in the right eye. Dr. Stacy, after asking the audience, opted to enucleate the right eye and switch to intravenous chemotherapy. Emphasizing the room for discussion in management, he highlighted the uncertainty in pathology from a phthisis eye. Unconvinced that there were no high-risk features, systemic chemotherapy was chosen and intra-arterial chemotherapy was avoided in order to mitigate similar risks in the left eye. The child underwent 6 cycles of adjuvant intravenous chemotherapy with local consolidation therapy. At the last follow-up, the tumor remained stable, and the child showed satisfactory vision.
A video recording of the meeting, below
Thank you to all panelists and attendees from across the world, and stay tuned for the Rb-NET Challenges 2024!