Belzutifan for treatment of giant retinal hemangioblastoma with extrascleral extension associated with von Hippel-Lindau syndrome.

PURPOSE: To describe the clinical response and safety profile of the novel HIF-2ɑ inhibitor belzutifan in treating a giant retinal hemangioblastoma (RHB) with extrascleral extension associated with von Hippel-Lindau syndrome (VHL). METHODS: A 71-year-old woman with VHL presented with a giant RHB with extrascleral extension in her only remaining eye. She had no light perception OD and intraocular pressure (IOP) was 48. She requested enucleation due to chronic pain, but, due to concern for significant bleeding given the size of the neoplasm, a trial of belzutifan was initiated. RESULTS: Within three months of treatment initiation, the patient reported an 80% reduction in pain. MRI showed 30% reduction in longest tumor diameter. Dose adjustments were guided by serum hemoglobin levels, allowing the patient to remain on the medication for over a year with continued tumor regression on MRI and avoid enucleation. CONCLUSION: RHB with extrascleral extension is exceedingly rare and its treatment is complex, often requiring enucleation or external beam radiotherapy. This report demonstrates the use of belzutifan to safely and successfully reduce ocular tumor burden of complicated RHB with extrascleral extension, ultimately decreasing the need for enucleation.

Modified Split-Thickness Corneal Patch Graft for Conjunctival Erosions in Patients With the Port Delivery System Implant: Surgical Technique and Long-Term Outcomes.

Purpose: To describe the surgical technique and long-term outcomes of a modified split-thickness corneal patch grafting for conjunctival erosions that can be seen in patients with the Port Delivery System (PDS) implant. Methods: By way of retrospective review of medical records, this interventional case series identified 2 cases in which modified split-thickness corneal patch grafting was used to repair conjunctival erosion in patients with the PDS implant. Results: The surgical approach involved creating a small opening in the corneal graft over the center of the PDS implant to improve visibility and allow for easier access during subsequent refill-exchange procedures. At the last follow-up of 6.9 years and 5.6 years, there was no recurrence of conjunctival erosions in either patient. The PDS implants remained well covered with the split-thickness corneal graft and had undergone multiple implant refills without complication or difficulty. Conclusions: Modified split-thickness corneal patch grafting with central graft aperture offers another option for long-term successful management of conjunctival erosions in patients with a PDS, especially those who have failed prior repair, by allowing sufficient visibility and access for subsequent refill-exchange procedures.

Clinical Applications of Machine Learning in the Management of Intraocular Cancers: A Narrative Review.

Purpose: There is great promise in use of machine learning (ML) for the diagnosis, prognosis, and treatment of various medical conditions in ophthalmology and beyond. Applications of ML for ocular neoplasms are in early development and this review synthesizes the current state of ML in ocular oncology. Methods: We queried PubMed and Web of Science and evaluated 804 publications, excluding nonhuman studies. Metrics on ML algorithm performance were collected and the Prediction model study Risk Of Bias ASsessment Tool was used to evaluate bias. We report the results of 63 unique studies. Results: Research regarding ML applications to intraocular cancers has leveraged multiple algorithms and data sources. Convolutional neural networks (CNNs) were one of the most commonly used ML algorithms and most work has focused on uveal melanoma and retinoblastoma. The majority of ML models discussed here were developed for diagnosis and prognosis. Algorithms for diagnosis primarily leveraged imaging (e.g., optical coherence tomography) as inputs, whereas those for prognosis leveraged combinations of gene expression, tumor characteristics, and patient demographics. Conclusions: ML has the potential to improve the management of intraocular cancers. Published ML models perform well, but were occasionally limited by small sample sizes owing to the low prevalence of intraocular cancers. This could be overcome with synthetic data enhancement and low-shot ML techniques. CNNs can be integrated into existing diagnostic workflows, while non-neural networks perform well in determining prognosis.

Hit and Run: Bilateral Massive Intraocular Lymphomatous Infiltration after CAR-T Therapy.

PURPOSE: We report a case of bilateral intraocular infiltration of DLBCL after CAR-T therapy. METHODS: Retrospective case report. RESULTS: A 62-year-old Caucasian male with medical history of high-grade DLBCL presented with papillitis and vitritis upon completion of CAR-T therapy. Thorough infectious and diagnostic work-ups were performed. Diagnostic vitreous tap revealed intraocular lymphoma. The patient received external beam radiotherapy to both orbits with dramatic improvement in disc edema and vitritis. However, subsequent MRI showed development of intracranial metastatic disease, and the patient died within the same month. CONCLUSION: Atypical intraocular metastasis of DLBCL may occur following CAR-T therapy and may indicate secondary changes in immunosurveillance within immune-privileged sites such as the eye.

Effect of surgical modality on visual outcomes for young patients with primary rhegmatogenous retinal detachments: a retrospective cohort study.

OBJECTIVE: To examine outcomes of different surgical modalities for correcting primary rhegmatogenous retinal detachments in patients younger than 50 years of age. METHODS AND ANALYSIS: A single-centre, retrospective, cohort study of 754 patients who underwent retinal surgery at the University of Virginia Hospital between 1 July 2012 and 1 July 2020 was conducted. Exclusion criteria were patients less than 18 or over 50 years of age, repeat detachments, second eyes of patients with bilateral detachments and follow-up less than 3 months. A multivariate regression model was used to compare overall outcomes in patients. RESULTS: 86 patients met inclusion criteria and of those, 38 (44%) underwent vitrectomy, 22 (26%) underwent scleral buckling, 13 (15%) underwent pneumatic retinopexy and 13 (15%) underwent combined scleral buckle and vitrectomy repair. Comparison of eye-level parameters among the procedure groups shows difference with respect to macular involvement (p<0.05) but not regarding clock hour involvement or giant tear status (p>0.05). Preoperative visual acuity was superior in the scleral buckle group compared with vitrectomy (p<0.001). Mean postoperative visual acuity improved with all procedures and all repair procedures had comparable rates of complication. The mean overall anatomical success rate was 73% (n=63) and comparable among all modalities. CONCLUSIONS: Vitrectomy, scleral buckle, pneumatic retinopexy or combined procedures are viable repair options for rhegmatogenous retinal detachments in patients younger than 50 years of age. Selection of the repair modality should be guided on baseline clinical features of the patient and detachment.

Pars Plana Vitrectomy for Endophthalmitis: Microbiologic Spectrum and Clinical Outcomes.

Purpose: To report visual outcomes, microbiologic spectrum, and antibiotic resistance in endophthalmitis patients undergoing pars plana vitrectomy (PPV).Methods: Retrospective study of 32 patients who underwent PPV with microbial analysis. Linear mixed models were utilized to compare visual acuity (VA).Results: Streptococcal species and coagulase-negative staphylococcus (CoNS) were the most common organisms. No resistance to vancomycin or fluoroquinolones was observed. Culture-negative individuals had better VA and lower incidences of retinal detachment (RD) and hypotony and better VA than culture-positive group at post-surgical month 3 (p = .025) and marginally at month 12 (p = .098). CoNS endophthalmitis (final logMAR VA 0.80) was associated with better VA than Streptococcal endophthalmitis (final logMAR VA 2.36) (p = .001). Secondary RD was observed in 33.3% of non-cataract endophthalmitis.Conclusion: No organisms were resistant to vancomycin or fluoroquinolones. Culture-negative endophthalmitis had better VA and lower rates of RD and hypotony than culture-positive group. A high rate of RD was observed in non-cataract endophthalmitis.

Effects of Intravitreal Anti-VEGF Therapy on Glaucoma-like Progression in Susceptible Eyes.

PRéCIS:: Intravitreal anti-vascular endothelial growth factor (VEGF) injections may accelerate glaucomatous change in patients with preexisting glaucoma or ocular hypertension (OHT). The safety of long-term injections in this specific population may be reflected in the need for additional glaucoma interventions. PURPOSE: The purpose of this study was to investigate whether repeated anti-VEGF injections accelerate structural and functional glaucomatous change in eyes with preexisting glaucoma or OHT. MATERIALS AND METHODS: This is a retrospective, observational study of injected and noninjected fellow eyes. A total of 28 patients with preexisting glaucoma or OHT, who received ≥6 unilateral anti-VEGF injections for concurrent neovascular retinal disease, were selected for chart review. Primary outcome measures were rate of visual field loss in dB/year, rate of change in retinal nerve fiber layer (RNFL) thickness in microns/year, and need for additional glaucoma medications, surgery, or laser. RESULTS: The number of eyes requiring additional glaucoma surgery or laser was 8 of 28 (28.6%) for the injected group and 2 of 28 (7.1%) for the noninjected group. A significantly greater proportion of injected eyes required invasive glaucoma intervention (P=0.034). Average rate of decline in mean deviation and change in pattern standard deviation were both significantly greater in injected eyes (P=0.029; P=0.019). Estimated mean rate of global retinal nerve fiber layer change was -4.27 µm/y for the injected group and -1.17 µm/y for the noninjected group and was significant only for injected eyes (P=0.014). Only the superior quadrant exhibited thinning that was significantly different between groups (P=0.030). CONCLUSIONS: Intravitreal injections were associated with accelerated functional and structural glaucoma-like change in susceptible eyes. Clinicians should assess the need for glaucoma medications or other interventions over the course of anti-VEGF therapy.