Shields, Carol L.

Purpose: To report two cases of macular choroidal macrovessel with description of multimodal imaging and review of published cases. Methods: Medical and imaging records were retrospectively reviewed. A literature review was performed to identify other cases of macular choroidal macrovessel published between 1990 and 2018. Results: There were 2 patients referred for evaluation of a potential choroidal tumor, including a 55-year-old white woman with no visual symptoms and a 68-year-old white woman with blurred vision. Funduscopic examination in each case revealed a focal area of choroidal elevation in the temporal foveal area with a single, dilated, slightly tortuous choroidal vessel, estimated to measure 250 μm and 300 μm, respectively, at widest girth, and extending temporally toward the equator with a tapering width. Optical coherence tomography showed an optically hollow choroidal lesion elevating the retinal pigment epithelium with overlying ellipsoid zone mottling. Both cases demonstrate shallow subretinal fluid, sparing the foveola. Fluorescein angiography showed faint staining along the course of the vessel in both cases. Indocyanine green angiography demonstrated clear visualization of the ectatic vessel with early filling and late staining without leakage. Both patients retained visual acuity of 20/25 in the affected eye and did not require treatment. Conclusion: Macular choroidal macrovessel is a rare vascular anomaly presenting as a single, dilated, tortuous choroidal vessel, originating in the temporal foveal region and extending temporally to the equator. This finding can simulate a choroidal neoplasm or parasitic track. Optical coherence tomography and indocyanine green angiography have diagnostic value demonstrating the vascular nature of this lesion and delineating the vascular course.

Purpose: To review immune checkpoint inhibitor indications and ophthalmic side effects. Methods: A literature review was performed using a PubMed search for publications between 1990 and 2017. Results: Immune checkpoint inhibitors are designed to treat system malignancies by targeting one of three ligands, leading to T-cell activation for attack against malignant cells. These ligands (and targeted drug) include cytotoxic T-lymphocyte antigen-4 (CTLA-4, ipilimumab), programmed death protein 1 (PD-1, pembrolizumab, nivolumab), and programmed death ligand-1 (PD-L1, atezolizumab, avelumab, durvalumab). These medications upregulate the immune system and cause autoimmune-like side effects. Ophthalmic side effects most frequently manifest as uveitis (1%) and dry eye (1–24%). Other side effects include myasthenia gravis (n = 19 reports), inflammatory orbitopathy (n = 11), keratitis (n = 3), cranial nerve palsy (n = 3), optic neuropathy (n = 2), serous retinal detachment (n = 2), extraocular muscle myopathy (n = 1), atypical chorioretinal lesions (n = 1), immune retinopathy (n = 1), and neuroretinitis (n = 1). Most inflammatory side effects are managed with topical or periocular corticosteroids, but advanced cases require systemic corticosteroids and cessation of checkpoint inhibitor therapy. Conclusion: Checkpoint inhibitors enhance the immune system by releasing inhibition on T cells, with risk of autoimmune-like side effects. Ophthalmologists should include immune-related adverse events in their differential when examining cancer patients with new ocular symptoms.

Purpose: To report a case of retinal cavernous hemangioma with intralesional phleboliths, simulating retinoblastoma. Methods: A healthy 5-month-old girl developed left esotropia and was noted to have atraumatic vitreous hemorrhage with underlying partially calcified mass, suspicious for retinoblastoma. Results: On examination, the visual acuity was fix and follow in the right eye and absent fixation in the left eye. Evaluation of the right eye revealed normal findings. The left eye demonstrated healthy anterior segment and dense vitreous hemorrhage with no view of the postequatorial structures, but with hazy view of the flat peripheral retina and a superonasal retinal mass, covered with fresh hemorrhage. Three white intralesional flecks, consistent with calcification, each measuring 300 μm, were visualized. B-scan ultrasonography confirmed the dense mass with several foci of calcification, suspicious for retinoblastoma, despite poor visualization on funduscopy. Prophylactic intravenous chemotherapy was delivered for globe salvage and systemic protection. At 12-month follow-up, the hemorrhage showed resolution, revealing a superonasal dark blue multilobulated mass with saccular aneurysms, measuring 16 mm in diameter, and with 3 phleboliths (intralesional calcification). Fluorescein angiography demonstrated early and midphase hypofluorescence with late-phase filling and with plasma–erythrocyte separation in some larger aneurysms, characteristic of retinal cavernous hemangioma. Conclusion: Retinal cavernous hemangioma can be associated with intralesional calcification (phleboliths).