Theodore Bowe, MD and Flavius Beca, MD
Wills Eye Hospital
SESSION 5
Dr. J. Fernando Arevalo and Dr. Jose Pulido moderated the fifth session on Friday at the Atlantic Coast Retina Club.
The first case was presented by Dr. Carol Shields from Wills Eye Hospital who took the opportunity to call attention to her alma mater’s (Notre Dame) advancement to the college football national championship game prior to beginning her talk, “Good clinical skills are important”. She highlighted a case that was referred for retinoblastoma (which was in fact a retinal astrocytic hamartoma). While OCT would clinch the diagnosis, Dr. Shields emphasized that subtle clinical findings of retinal achromic spots and a small skin lesion also pointed to a diagnosis of retinal astrocytic hamartoma. While we have a wide array of imaging modalities that are valuable, a thorough clinical exam is vital.
Dr. John Thompson from Greater Baltimore Medical Center and co-chair of the conference, then presented a case of an immunocompetent patient in their 70s who presented with vitreous hemorrhage, retinitis and hemorrhage that was adjacent to the optic nerve. Infectious and inflammatory workup was negative, and a pauci-cellular vitreous biopsy and MRI were negative for malignancy. Eventual retinal biopsy led to the diagnosis of primary vitreoretinal lymphoma. Dr. Shields outlined some characteristics of the lesion that suggested this might be the diagnosis, and Dr. Francis educated the audience that a negative MRI is insufficient to rule out CNS involvement; an LP must be done as there are many cases of lymphoma that are MRI negative but CSF positive.
Dr. Kenneth Ward from NYU next presented a case of uveitis-glaucoma-hyphema syndrome presenting as vitreous hemorrhage. The lens in question was a PMMA lens that was fixated with a Yamane technique (which has a reported 5% risk of VH). The case highlighted challenges in diagnosis and management of UGH. Dr. Ward had an excellent outcome repositioning and iris suturing the3-piece PMMA lens rather than undergoing a much larger procedure with IOL exchange.
Dr. Pulido from Wills Eye Hospital then pivoted with a presentation that provided an explanation of an OCT artifact that can be seen in some clinical scenarios (ERMs, fovea plana, and others). A “fingerprint sign” has been described, which others have theorized is from corrugations of the Henle fiber layer. Dr. Pulido demonstrated that these are Newton’s rings, which are an optical phenomenon of constructive and destructive interference. Their size can be modified by using various wavelengths of incident light that supported his teaching point. This presentation emphasized that our reliance on advanced imaging can leave us susceptible to misinterpreting optical phenomena as anatomic findings.
The final case of the session was presented by Dr. Miriam-Benigna Chika Amobi, an ocular oncology fellow from Wills Eye Hospital. Dr. Amobi presented an interesting case of a man who presented with multifocal bilateral choroidal masses that were found to be metastases from breast cancer. The ocular oncologists in attendance took the opportunity to note that while retina specialists tend to see more intraocular primary malignancies than metastases, metastases are much more common than primary ocular malignancies. Additionally, metastases from lung cancer are likely the leading cause of ocular metastases in both sexes rather than the classic teaching of breast in women and lung in men.
SESSION 6
The sixth session on Friday focused on ocular oncology and was moderated by Dr. Ivana Kim and Dr. Carol Shields.
The first case of the session, presented by Dr. Brian Marr from Columbia University, was an atypical astrocytic hamartoma case in a young woman with tuberous sclerosis. Dr. Marr highlighted that this so-called “benign” lesion can rarely have significant morbidity and an aggressive presentation with rapid growth, posterior location, and potential for retinal neovascularization and exudation. The case was managed with monthly anti-VEGF injections which appeared to reduce the neovascularization, however, vision was noted to decline after 10 months of treatment. At this point, 2 rounds of full fluence PDT were applied with dramatic improvement in vision. Dr. Freund noted the temporary increase in subretinal and intraretinal fluid associated with treatment and emphasizes the importance of pre-treatment with anti-VEGF given the anticipated increase in retinal edema following PDT. The successful use of PDT in decreasing the tumor size and treatment burden was deemed a significant success.
Dr Jasmine Francis’ mystery case from Memorial Sloan Kettering Cancer Center provided this retina attuned audience on a detour in the world of medicine. A middle aged man presented with subtle bilateral outer retinal loss noted on OCT. Drs. Shields and Freund were invited to offer ideas. Dr. Shields inquired about medication use: none. Dr. Freund considered a placoid-like process but admitted, “I don’t know.” Dr. Francis then showed an ICG with venous distension, facial spider veins, a macrocytic anemia, and a wildly elevated GGT level seen in cases of liver toxicity, like in alcohol use disorder. Through this case, Dr. Francis offered a compelling albeit not universally accepted hypothetical mechanism for central serous via venous overload from portal hypertension while also highlighting the importance of recognizing alcohol use disorder as the 3rd leading cause of cancer.
Dr. Yannis Paulus from Wilmer Eye Institute then presented a case of diffuse choroidal hemangioma in a man with Sturge Weber syndrome who presented with a large bullous inferior exudative detachment. The case was managed with anti-VEGF injections which successfully reduced the size of the lesion and subretinal fluid though not the vision. The audience, led by Dr. Jampol who again confirmed in jest that he is not an ocular oncologist, debated the merits of PDT in similar cases. Dr. Marr confirmed having success with a similar case despite initial skepticism in line with Dr. Jampol. However, Dr. Shields posited why should we worry about the risks of radiation as these cases require the lowest levels of radiation of any ocular tumors, well below the threshold for retinopathy, while allowing treatment of the entire tumor and no need for repeat treatment.
Dr. Benjamin Kim from University of Pennsylvania presented a typical choroidal nevus with a subfoveal location that was managed conservatively for many years. However, once the lesion was noted to produce subretinal fluid, it was managed with 3 monthly anti-VEGF injections. Dr. Shields confirms that in her practice she begins with 4 monthly bevacizumab injections, even cases that she suspects may ultimately require PDT for management of fluid. Although the fluid resolved, continued follow up over the next 2-3 years resulted in subfoveal retinal atrophy. Dr. Shields answered what many in the audience were thinking: would earlier PDT have led to better vision outcomes? Probably not as the treatment itself may have induced the same atrophy that was observed later. She added that unfortunately as her 2007 paper showed, the subfoveal location likely would have led to this result eventually. Dr. Freund posited that the mechanism of retinal atrophy in these benign nevi is secondary to a compressive effect of the lesion on the choriocapillaris, rather than from the presence of subretinal fluid. Therefore, aggressive treatment likely would not avoid the eventual atrophy.
The final case of the session, presented by Dr. David Abramson from Memorial Sloan Kettering, was of a large retinoblastoma. Dr. Abramson offered 2 important clinical pearls of how to recognize intraocular seeding by these large lesions as well as the importance of careful examination of the second eye where identification of another tumor leads to recognition that this implies a germline mutation. Dr. Abramson reviewed the advances in treatment, while highlighting intra-arterial chemotherapy as the current treatment of choice. This case, however, received intravitreal topotecan due to social circumstances limiting care. The conversation then shifts to a discussion of the role of MRI screening for “trilateral” disease. After reviewing the costs and risks of MRI and gadolinium contrast, Dr. Abramson states that no case of early screening prevented the eventual death of the patient, except for this patient. He ended on the somber note that he anticipated this patient too may ultimately succumb to his disease burden, though the topotecan treatment may have provided some additional time.
Read All Atlantic Coast Retina Club / Macula 2025 Articles:
Mystery Cases 1 & 2
Mystery Cases 3 & 4
Mystery Cases 5 & 6
Mystery Cases 7, 8, 9
Imaging, GA, and IRDs
Keynote Lectures
DR and DME
Choroidal Neovascularization
Retinal Vascular Diseases
Ocular Oncology
Special Topics & Women in Retina