Faculty Bibliography

PURPOSE/UNASSIGNED:To report a unique case of hydroxychloroquine-associated retinal toxicity presenting with cystoid macular edema (CME) and retinal vasculopathy and to discuss its potential pathophysiology. METHODS/UNASSIGNED:A single case was reviewed. CASE REPORT/UNASSIGNED:A 65-year-old woman with a history of hydroxychloroquine use for approximately 5.5 years presented with bilateral CME and parafoveal ellipsoid zone disruption with outer retinal loss on optical coherence tomography. Multimodal imaging demonstrated central bull's-eye maculopathy and peripheral pigmentary changes on fundus autofluorescence and peripheral vascular leakage on fluorescein angiography. The uveitis workup, anti-retinal antibody testing, and genetic testing were negative. As retinal findings were normal at the first hydroxychloroquine screening, the patient was diagnosed with advanced hydroxychloroquine toxicity. CONCLUSIONS/UNASSIGNED:This case expands the phenotypic spectrum of hydroxychloroquine retinal toxicity to include CME associated with retinal vasculopathy. Annual retinal examination should begin promptly in patients receiving hydroxychloroquine at doses exceeding 5 mg/kg/day.

PURPOSE/UNASSIGNED:To describe a rare case of severe anterior and posterior scleritis that led to a diagnosis of high-risk myelodysplastic syndrome. METHODS/UNASSIGNED:We present a case report along with a literature review on cases of paraneoplastic scleritis to highlight commonalities in etiology, presentation, treatment response, and outcomes. RESULTS/UNASSIGNED:A 59-year-old male presented with a severe case of unilateral anterior and posterior scleritis with ciliochoroidal detachment and secondary angle closure. His workup revealed severe macrocytic anemia and thrombocytopenia. A subsequent bone marrow biopsy demonstrated cytogenetic analysis consistent with high-risk myelodysplastic syndrome (MDS). The patient was started on high-dose prednisone but was unable to be tapered below 30 mg without recurrence. Rituximab infusions were initiated followed by resolution of ocular symptoms and a successful taper of prednisone. He initiated chemotherapy and was to receive allogenic bone marrow transplantation. However, while on chemotherapy, he clinically deteriorated and passed away. CONCLUSION/UNASSIGNED:This case of severe scleritis was suspected to be a paraneoplastic syndrome secondary to myelodysplastic syndrome. Paraneoplastic syndromes represent a rare etiology of scleritis. Patients with hematologic abnormalities such as unexplained cytopenias, severe manifestations at presentation, or steroid refractory scleritis may warrant comprehensive evaluation for malignancy, expedited systemic workup with attention to blood counts, and interdisciplinary collaboration between ophthalmology, hematology, and internal medicine. If an underlying malignancy is identified, definitive treatment of the scleritis involves treatment of the cancer.

PURPOSE/OBJECTIVE:We present a patient with a primary vasoproliferative tumor (VPT) accompanied by vitreous haze and an epiretinal membrane (ERM). We report for the first time the vitreous cytokine profile from an eye with a primary VPT to explore the relationship between intraocular inflammation and these tumors. METHODS:Retrospective chart review of a single patient case. RESULTS:25-gauge pars plana vitrectomy with membrane peel and vitreous biopsy was performed. Peripheral vitreous shave exposed an inferior grey-red mass located at the ora serrata, consistent with VPT. Treatment with confluent, long duration endolaser was performed. Vitreous cytology was negative for malignancy. A 13-cytokine panel (Associated Regional and University Pathologists, Inc. Laboratories, Salt Lake City, UT) revealed elevated interleukin 6 (13.3 pg/mL; normal <=2.0) and interleukin 8 (6.0 pg/mL; normal <=3.0). At one month post-operative, visual acuity improved from 20/40 to 20/25 OD, with mild anterior vitreous inflammation and regression of the VPT. CONCLUSION/CONCLUSIONS:Pro-inflammatory and pro-angiogenic cytokines were elevated in the vitreous of this patient's eye with a primary VPT. We suggest that the endothelial cells and macrophages which comprise VPTs could secrete these cytokines into the vitreous, resulting in vitreous haze and an overzealous fibrotic response manifested as ERM formation.

PURPOSE/UNASSIGNED:To discuss the pathophysiology, etiology, and current management strategies of uveitis-glaucoma-hyphema (UGH) syndrome. METHODS/UNASSIGNED:Literature review. RESULTS/UNASSIGNED:The classic UGH syndrome associated with anterior chamber intraocular lenses (ACIOL) have decreased in incidence with the modernization of IOL design and surgical techniques. The current UGH syndrome is increasing in prevalence largely related to a parallel increase in late onset dislocations of intraocular lenses (IOLs) and the developing techniques to remedy that condition. The modern features of UGH can present as cystoid macular edema, intraocular pressure elevation typically not attributed to UGH, and recurrent vitreous hemorrhage, unlike the original description as described by Ellingson in 1978. Medical management to control inflammation, reduce intraocular pressure, and reduced the bleeding diathesis are mainstays of therapy. However, surgery with IOL repositioning or exchange should be reserved for cases that are refractory to or progressing despite medical treatment. CONCLUSIONS/UNASSIGNED:UGH syndrome is an increasingly common, poorly understood, and often subtle, manifestation of an anatomic disturbance post intraocular surgery that persists with continued evolution of intraocular surgical techniques and new imaging modalities to aid in its diagnosis.

Purpose : To examine cases of new onset uveitis-glaucoma-hyphema (UGH) syndrome after posterior chamber intraocular lens (PCIOL) repositioning with iris-sutured IOL, differentiating between in-the-bag versus out-of-the-bag dislocations. Methods : Retrospective chart review was performed on 99 patients with a posteriorly dislocated IOL operated upon by a single surgeon from 2018-2020. Patients with previously diagnosed UGH, retained lens fragment, early dislocation, or lost-to-follow-up after one month were excluded. Variables of interest included 1-piece versus 3-piece IOL, in-the-bag versus out-of-the bag dislocation, and history of pxeudoexfoliation. UGH was defined as having at least one of the following: elevated intraocular pressure >25 mmHg, anterior uveitis, hyphema, or post-operative cystoid macular edema (CME). Results : 24 patients were eligible for the study. 18 patients were noted to have in-the-bag dislocations (75%). A total of 12 patients developed UGH (50%). Two of the 12 patients (16.67%) exhibited uveitis, glaucoma, and CME. No patients had hyphema. Among patients with UGH, mean age was 73 years old. 7 of 12 cases were in-the-bag dislocations (58.3%), and 6 of 12 cases (50%) had pre-existing pxeudoexfoliation (PXE). Among in-the-bag dislocations with UGH, 5 out of 7 patients had 1-piece IOLs (71%); whereas among the 5 cases of out-of-the-bag dislocations with UGH, all had 3-piece IOLs. Moreover, none of the patients with out-of-the-bag dislocation had pre-existing PXE. A final logMAR VA of 0.314 for in-the-bag dislocations with UGH (p = 0.832) was not found to be statistically significant compared to out-of-the bag UGH patients. Mean follow-up time was 4.5 months. Patients were treated with topical steroids(9 out of 12, 75% ), topical nonsteroidal antiinflammatory (8 out of 12, 67%), and pressure lowering drops (9 out of 12, 75%). Conclusions : UGH is a notable complication of iris sutured PCIOL repositioning. Preexisting PXE may be a predisposing factor for in-the-bag dislocations. There was a tendency toward UGH with single-piece in-the-bag dislocations, and three-piece out-of-thebag dislocations. Moreover, both in-the-bag and out of-the-bag dislocations appear to have similar visual outcomes. However, a larger sample size with longer follow-up time is necessary to draw more definitive conclusions

PURPOSE/OBJECTIVE:To describe the multimodal imaging findings of retinal lesions that clinically resemble retinal astrocytic hamartomas (RAHs), but also have unique characteristics that we believe represent a novel variant. METHODS:Observational study. Five eyes in five patients with solitary retinal lesion evaluated at the retina division of three institutions. We describe the multimodal imaging findings including fundus photography, fundus autofluorescence, fluorescein angiography, spectral-domain optical coherence tomography (OCT), swept-source OCT, swept-source OCT angiography and ultrasonography. RESULTS:The retinal lesions described shared similar appearance to RAHs but demonstrated unique features such as glistening granular appearance on fundus photographs with perivascular hyperreflectivity with OCT and OCT angiography. CONCLUSION/CONCLUSIONS:The lesions described herein appear to have unique characteristics that warrant a designation as a novel RAH variant. The name presumed retinal pericapillary astrocytic hamartoma is suggested.

Purpose : We primarily aim to determine the incidence of uveitis-glaucoma-hyphema (UGH) after surgical repair of late, in-the-bag posterior chamber intraocular lens (PCIOL) dislocations treated by PCIOL-capsular complex repositioning and iris-suture fixation technique. Secondarily, we aim to characterize the outcomes of patients with this complication. Methods : We retrospectively reviewed the course of 212 patients with a lens repositioning surgical procedure by a single-surgeon in a four-year period, from 2014-2018. 68 patients with a posteriorly dislocated, in the capsular bag, PCIOL treated with iris-sutured lens repositioning with a follow-up duration of at least 3 months were included. Patients with pre-existing UGH, early dislocation within three months of cataracts extraction, or with retained lens material were excluded. UGH was defined as iritis or a combination of hyphema, high intraocular pressure, or vitreous hemorrhage. Results : After a median follow-up of 8.0 months, six (8.8% of 68) patients developed UGH at a median onset of 82.5 days from surgery. Baseline characteristics of these patients were the following: mean age of 70, initial intraocular pressure (IOP) of 18 +/- 4, initial logarithm of the minimum angle of resolution visual acuity (logMAR VA) of 0.64 +/- 0.56, and 1-piece IOL dislocation (83.3%). UGH was treated with topical corticosteroids in 5 patients (83.3%) and pressure lowering drops in 4 patients (66.7%). No patient required reoperation, nor had diminished vision. At a median follow-up of 8.0 months within this cohort with UGH, the final logMAR VA was 0.22 +/- 0.06 (versus baseline, p=0.104), and the final IOP was 23 +/- 10 (versus baseline, p=0.193). Conclusions : UGH is a rare but notable complication of iris sutured PCIOL repositioning. Patients who develop UGH can be effectively treated with medical therapy without surgical intervention and achieve good final visual acuity outcomes. A syndrome combining signs of iritis, VH, hyphema, and sustained IOP elevation particularly with single piece PCIOLs should prompt consideration of a UGH diagnosis and early treatment intervention to optimize patient outcomes. Future prospective clinical trials are needed to compare overall complication rates and outcomes of in-the-bag lens repositioning surgery with scleral versus iris sutured fixation technique

Purpose : To characterize outcomes for patients with re-operated macular holes using a novel technique after failed closure in the initial surgery Methods : A retrospective chart review was conducted on 401 eyes that had undergone macular hole surgery in a private retina practice from 2013 to 2018. Those patients that underwent re-operation for persistently open macular hole were then identified. The initial macular hole surgery was done using pars plana vitrectomy (PPV), hyaloid elevation, internal limiting membrane (ILM) peeling with indocyanine green staining (ICG) with wide removal to the arcades, SF6 gas, and prone positioning for 6 days. For those patients that underwent a successive operation, 25-gauge PPV and a Tano diamond-dusted membrane scraper or Finesse flex loop was used to brush the retina in an outside-in manner in attempts to reduce the hole diameter. No attempt was made to restain the ILM, and C3F8 gas was used for re-operation. Age, race, gender, lens status, initial visual acuities before initial surgery, time to reoperation, and final visual outcomes were recorded. The optical coherence tomography (OCT) images of the initial hole and re-operated hole were analyzed. Results : Eight eyes were re-operated from 2013 2018. Visual outcomes improved by at least 3 lines from the initial prior to surgery to the most recent following re-operation in 7 (87.5%) patients, with all 8 (100%) eyes having macular hole closure. The re-operated eyes had larger hole diameters of >500 microns compared to eyes with successful primary macular hole operation. Conclusions : Previous studies have reported few techniques for re-operation include wider ILM peel/rexis and intraoperative macular hole fluid drainage. In this study, we utilized a technique of brushing the retina in an outside-in manner to help facilitate anatomic closure of the hole. Inner retinal brushing with hole diameter reduction and long-acting gas appears to be a successful treatment for persistent macular holes or possibly as initial surgery for large macular holes