Atlantic Coast Retina Club 2022 Part 2: Streaks, Fluid, Kids, Tumors, and Boom Boom

Ollya Fromal, MD
Wills Eye Hospital

The morning session of the ACRC continued with stellar presentations from current Wills Eye Hospital/Mid Atlantic Retina fellows. Dr. Abtin Shahlaee, a first year retina fellow, presented a case of a secondary retinal neovascularization due to angioid streaks. Dr. Shahlaee highlighted the importance of a thorough systemic examination in patients with angioid streaks, as careful skin examination of his patient revealed neck papules, leading to a diagnosis of pseudoxanthoma elasticum.

Dr. Meera Sivalingam, a first year Wills Eye retina fellow, skillfully employed multimodal imaging to showcase a case of a chronic bilateral central serous chorioretinopathy (CSCR) with macula edema and extensive serous choroidal detachments – rare findings in CSCR. Resolution of the edema and choroidals were achieved with oral eplerenone. This led to a heated discussion amongst the crowd regarding the use of eplerenone as well as the role of PDT in these eyes with extensive peripheral lesions.

Dr. Samir Patel, a second year Wills Eye retina fellow, shared a case of a patient with a high-grade metastatic endometrial cancer who developed vision loss soon after initiating pembrolizumab. Positive anti-recoverin antibodies aided in the diagnosis of cancer associated retinopathy in this case. Patient was effectively treated with infliximab infusions and was able to continue his anticancer therapy with pembrolizumab. Dr. Jose Pulido offered the clinical pearl that whenever anti-recoverin antibodies are found, it is almost always cancer-associated retinopathy. Helpful in this case in which there was debate whether the patient’s findings were related to pembrolizumab or the underlying malignancy.

Taku Wakabayashi, MD, a pediatric retina postdoctoral fellow, presented a curious case of severe bilateral stage 5 tractional retinal detachment in 18P deletion syndrome with concurrent Frizzled-4 mutation – a novel association. Complex vitrectomy with membrane peeling was effective in reattaching the posterior pole in this 6-month-old boy.

A lively ocular tumor session of the ACRC was moderated by Dr. Carol Shields and Dr. Sara Lally and included a case of an RPE adenoma arising from CHRPE (Andrea Laiton, MD), unilateral chorioretinal folds leading to a diagnosis of a dermoid cyst (Mrittika Sen, MD), as well as a case of a large metastatic choroidal carcinoma in a patient who initially denied history of breast cancer, despite having undergone chemotherapy and surgery several years prior (Konica Singla, MD).

Dr. Ana Velasco, Wills Eye ocular oncology research fellow, reviewed treatment options for retinal hemangioblastoma, as well as discussed an oral HIF-2α inhibitor belzutifan as a novel treatment for von Hippel-Lindau disease. Drs. Carol Shields and Jose Pulido described this medication as a complete game changer in the management of VHL.

Dr. Kushal Agrawal, an ocular oncology fellow at Wills Eye, discussed management of primary uveal lymphoma emphasizing high sensitivity of this tumor to radiation. Dr. Agrawal advocated for ultra-low-dose radiotherapy for primary uveal lymphoma, cleverly termed “boom-boom” therapy as the length of the treatment is only two days – boom and boom.