LONG-TERM USE OF ANTI-VASCULAR ENDOTHELIAL GROWTH FACTOR THERAPY FOR NEOVASCULAR AGE-RELATED MACULAR DEGENERATION.

PURPOSE: Although pivotal trials have demonstrated efficacy of anti-vascular endothelial growth factor therapy in neovascular age-related macular degeneration, there is a paucity of clinical data about the long-term (>5 years) treatment. METHODS: Retrospective analysis of all patients with neovascular age-related macular degeneration who were actively treated, had received >40 anti-vascular endothelial growth factor injections, and were followed for ≥5 years. Snellen-corrected visual acuity, initial drug choice, and times elapsed between treatments were collected. Rates of endophthalmitis and outcomes of submacular hemorrhage were also evaluated. RESULTS: A total of 88 patients (162 eyes) met the inclusion criteria: the average patient age was 86.3 years with an average follow-up period of 7.6 years. The average total number of injections per eye was 69 (18.0 SD); a total of 11,208 injections were given throughout the study period, and 6 cases (0.05%) of endophthalmitis were observed. Overall, there was a clinical and statistical difference in average Snellen-corrected visual acuity at Injections #2,#3, #4, #5, #6, #10, and #20, as compared with baseline ( P = 0.03, P < 0.01, P = 0.02, P < 0.01, P = 0.01, P = 0.01, P < 0.01, respectively). Patients in the Snellen-corrected visual acuity subgroup 20/20 to 20/40 maintained vision until injection #30. Seven eyes experienced a visually significant submacular hemorrhage. CONCLUSION: This neovascular age-related macular degeneration cohort received on average eight anti-vascular endothelial growth factor injections per year for approximately 8 years; eyes with good (≥20/40) initial baseline vision maintained their visual acuity, whereas those with worse Snellen-corrected visual acuity (≤20/50) had a robust initial improvement that diminished with time. Most patients were maintained on the same initial drug of choice and the rate of endophthalmitis was low.

Combined epiretinal and internal limiting membrane retracting door flaps for large macular holes associated with epiretinal membranes.

PURPOSE: To assess the closure rate of large full-thickness macular holes (FTMH) associated with epiretinal membrane (ERM) with a combined epiretinal and internal limiting membrane retracting door flap. METHODS: Retrospective chart review of patients treated at a single tertiary retina practice between January 2017 and November 2019. Individuals with FTMH larger than 400 µm and co-diagnosis of ERM who underwent surgical repair with an ERM flap were included. Patients underwent pars plana vitrectomy with peeling of ERM that was positioned as a retracting door flap to cover the FTMH. Primary outcome was closure rate at 6 months following surgery. Final surgical success rate and visual acuity were secondary outcomes. RESULTS: Among 7 eyes of 7 patients, 6 eyes achieved primary surgical success and final surgical success rate was achieved in all 7 eyes with a large FTMH repaired with ERM flap. The mean minimum linear diameter of the FTMH was 681 µm ± 295. All patients had follow-up greater than 6 months, with a mean duration of 17 months (range 14-23 months). Visual acuity improved from a mean of 0.9 ± 0.3 logMar (20/160) before surgery to 0.3 ± 0.5 logMar (Snellen 20/40), postoperatively. CONCLUSION: Large FTMH with concurrent ERM that are managed with an ERM flap have high single-surgery success rate.

Incidence and Visual Outcomes of Endophthalmitis After Intravitreal Injection of Dexamethasone Implant vs Ranibizumab.

Purpose: To compare the incidence and visual outcomes of endophthalmitis after injection of an intravitreal dexamethasone implant and injection of intravitreal ranibizumab. Methods: This retrospective cohort study assessed endophthalmitis in eyes receiving an intravitreal injection of a 0.7 mg dexamethasone implant (DEX group), 0.5 mg ranibizumab (R5 group), or 0.3 mg ranibizumab (R3 group) between January 1, 2016, and May 31, 2018, at 2 large retina practices in the United States. Results: Suspected endophthalmitis occurred in 5 eyes after 4973 DEX injections, 43 eyes after 163 974 R5 injections, and 6 eyes after 18 954 R3 injections. Suspected endophthalmitis was significantly more common in the DEX group (1/995) than in the R5 group (1/3813) (P = .008) but not than in the R3 group (1/3159) (P = .10). Visual acuity outcomes were similar in the 3 groups. Conclusions: Suspected endophthalmitis might be more common after 0.7 mg dexamethasone injections than after 0.5 mg ranibizumab injections. Culture-positive endophthalmitis rates were similar across all 3 medications.

BILATERAL ENDOGENOUS ENDOPHTHALMITIS FROM STREPTOCOCCUS PNEUMONIAE.

PURPOSE: To report a case of bilateral endogenous endophthalmitis from Streptococcus pneumoniae with devastating sequelae. METHODS: Interventional case report. RESULTS: A 56-year-old man presented with acute bilateral blurred vision and floaters with fever and hemodynamic instability. Previously, he was diagnosed with acute otitis externa and reported manually extracting several of his own teeth. He underwent a vitreous tap and intravitreal antibiotic injections. Blood and vitreous cultures were positive for S. pneumoniae. The patient later developed life-threatening medical sequelae. His final visual acuity was no light perception in the right eye and 20/25 in the left eye. CONCLUSION: Diagnosing endogenous endophthalmitis early is essential to initiating a systemic evaluation for potentially life-threatening medical conditions, including sepsis, endocarditis, and osteomyelitis. A high degree of suspicion, expeditious treatment, and interdisciplinary collaboration are essential to maximizing patient outcomes.

REFRACTIVE OUTCOMES AFTER SUTURELESS INTRASCLERAL FIXATION OF INTRAOCULAR LENS WITH PARS PLANA VITRECTOMY.

PURPOSE: To evaluate the refractive outcomes of sutureless intrascleral fixation of intraocular lens with pars plana vitrectomy. METHOD: A retrospective, consecutive cohort from multiple surgeons of a single center. Primary outcomes included spherical equivalent (SEQ) and change in SEQ (ΔSEQ) from preoperative intraocular lens power calculations. Secondary outcomes included refractive outcomes of fixation at 1.5 mm, 2 mm, and 2.5 mm posterior to the limbus. RESULTS: In total, 84 eyes of 80 patients were included. Preoperative logarithm of the minimum angle of resolution visual acuity was 1.21 ± 0.68 (20/320). The mean follow-up time was 2.33 ± 1.36 years. At 3 months, SEQ was -0.50 ± 1.59 D and ΔSEQ was 0.58 ± 1.49 D. At 1 year, SEQ was -0.55 ± 1.32 D and ΔSEQ was 0.39 ± 1.42 D. At the last follow-up, logarithm of the minimum angle of resolution visual acuity was 0.34 ± 0.34 (20/40), SEQ was -0.51 ± 1.44 D, and ΔSEQ was 0.57 ± 1.27 D. There was no difference between SEQ or ΔSEQ throughout follow-up (P = 0.97 and P = 0.96, respectively). At fixation distances more posterior to the limbus, mean ΔSEQ was more hyperopic at 3 months, 1-year, and the last follow-up (P = 0.02, P = 0.01, and P = 0.006, respectively). CONCLUSION: Refractive outcomes for sutureless intrascleral fixation of intraocular lens with pars plana vitrectomy were favorable and showed stability postoperatively. These results may aid surgeons achieve better desired refractive outcomes for this technique.

Paraneoplastic ocular sarcoidosis in the setting of recurrent rectal carcinoid tumor diagnosed by F18-fluorodeoxyglucose PET CT.

PURPOSE: Nuclear medicine imaging can provide a noninvasive means of distinguishing inflammatory mass lesions from oncologic intraocular tumors. OBSERVATION: We report a case of paraneoplastic ocular sarcoidosis with choroidal mass lesions that was initially concerning for choroidal metastasis of a primary carcinoid tumor. PET CT was utilized with two different tracers to characterize the choroidal mass as being FDG-avid, consistent with a sarcoid-like lesion, and lacking the Gallium (Ga-68) DOTATAE uptake of carcinoid tumor metastases. CONCLUSIONS AND IMPORTANCE: Functional imaging is valuable to distinguish clinically similar inflammatory verses oncologic intraocular pathology.

Influenza-Induced Acute Macular Neuroretinopathy With Cerebral Involvement in a Ten-Year-Old Boy.

A 10-year-old male presented with 1-week duration of painless bilateral central vision loss after having been diagnosed with influenza A. Optical coherence tomography revealed superficial retinal nerve fiber layer infarcts, hyperreflectivity of the inner nuclear layer consistent with paracentral acute middle maculopathy (PAMM), and outer nuclear layer hyperreflectivity and disruption of the ellipsoid zone suggesting acute macular neuroretinopathy (AMN). Brain MRI revealed enhancement of the right basal ganglia consistent with focal encephalitis. He was diagnosed with presumed influenza-induced leukocytoclastic vasculitis (LCV) and treated with intravenous steroids. Influenza-induced LCV is a rare phenomenon and can present with AMN, PAMM, and encephalitis. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:293-297.].

Optical Coherence Tomography Angiography Findings in Patients With Acute Syphilitic Posterior Placoid Chorioretinopathy.

Purpose: Acute syphilitic posterior placoid chorioretinopathy (ASPPC) is an uncommon and distinct manifestation of ocular syphilis necessitating immediate treatment. ASPPC is attributed to disruption of the choriocapillaris, retinal pigment epithelium, and photoreceptors. Optical coherence tomography angiography (OCTA) can evaluate choriocapillaris vascular flow and may provide further information about ASPPC's disease process. Methods: OCTA images from 7 eyes of 4 patients with ASPPC were compared before and after treatment when available. Results: All eyes demonstrated increased choriocapillaris vascular flow voids in the distribution of the ASPPC lesions at initial testing. Following treatment, decreased placoid lesion size was associated with decreased flow voids on OCTA along with improved ellipsoid zone integrity in 2 patients. Conclusions: Disruption of choriocapillaris vascular flow in ASPPC that causes outer retinal changes can improve following treatment as suggested by OCTA imaging. Some cases may continue to demonstrate decreased flow even after appropriate therapy.

BASAL CELL NEVUS SYNDROME PRESENTING AS EPIRETINAL MEMBRANE AND MYELINATED NERVE FIBER LAYER.

PURPOSE: To report a case of epiretinal membrane and myelinated nerve fiber layer, which preceded the diagnosis of basal cell nevus syndrome, in a young girl. METHODS: Observational case report. RESULTS: A 12-year-old girl was referred for an asymptomatic epiretinal membrane. Examination revealed epiretinal membrane in the right eye without posterior vitreous separation or vitreous abnormality and bilateral myelinated nerve fiber layer. Subsequent workup yielded pathologic diagnosis of multiple skin basal cell carcinoma and odontogenic keratocysts in the jaw. Genetic testing revealed a frameshift mutation in the PTCH1 gene. CONCLUSION: Basal cell nevus syndrome is a rare autosomal dominant disease that affects multiple organ systems, including the eyes. Recognition of common ocular findings in children with basal cell nevus syndrome can lead to systemic diagnosis. Early diagnosis is critical to initiate early screening for known neoplastic associations and lifelong minimization of sun exposure to reduce the incidence and severity of basal cell carcinoma.

Histone deacetylase inhibitor treatment dramatically reduces cholesterol accumulation in Niemann-Pick type C1 mutant human fibroblasts.

Niemann-Pick type C (NPC) disease is predominantly caused by mutations in the NPC1 protein that affect intracellular cholesterol trafficking and cause accumulation of unesterified cholesterol and other lipids in lysosomal storage organelles. We report the use of a series of small molecule histone deacetylase (HDAC) inhibitors in tissue culture models of NPC human fibroblasts. Some HDAC inhibitors lead to a dramatic correction in the NPC phenotype in cells with either one or two copies of the NPC1(I1061T) mutation, and for several of the inhibitors, correction is associated with increased expression of NPC1 protein. Increased NPC1(I1061T) protein levels may partially account for the correction of the phenotype, because this mutant can promote cholesterol efflux if it is delivered to late endosomes and lysosomes. The HDAC inhibitor treatment is ineffective in an NPC2 mutant human fibroblast line. Analysis of the isoform selectivity of the compounds used implicates HDAC1 and/or HDAC2 as likely targets for the observed correction, although other HDACs may also play a role. LBH589 (panobinostat) is an orally available HDAC inhibitor that crosses the blood-brain barrier and is currently in phase III clinical trials for several types of cancer. It restores cholesterol homeostasis in cultured NPC1 mutant fibroblasts to almost normal levels within 72 h when used at 40 nM. The findings that HDAC inhibitors can correct cholesterol storage defects in human NPC1 mutant cells provide the potential basis for treatment options for NPC disease.