RETINAL DETACHMENT IN EYES WITH BOSTON TYPE 1 KERATOPROSTHESIS: Surgical Techniques and Mid-Term Outcomes.

PURPOSE: To evaluate the mid-term outcomes of pars plana vitrectomy performed for retinal detachment (RD) repair after Boston Type 1 keratoprosthesis (KPro) implantation. METHODS: Retrospective review of medical records of KPro implanted at the Stein Eye Institute presenting with RD and treated by pars plana vitrectomy. Functional success was defined as a postoperative visual acuity maintained within 2 Snellen lines of the corrected distance visual acuity measured before the development of the RD (baseline) and anatomical success as an attached retina after the pars plana vitrectomy. Kaplan-Meyer survival analyses were performed. RESULTS: Among the 224 KPro performed, 28 (15.2%) RD were identified; of which, 21 (9.4%) were included. The mean follow-up was 42.5 ± 27.3 months. Vitreoretinal proliferation was present in 18 of 21 eyes (85.7%). Surgical techniques were adapted to the complex anterior segment anatomy of KPro eyes. Anatomical success was achieved in 18 of 21 eyes (85.7%). Functional success occurred in 17 of 21 eyes (81.0%), and 5 of 21 eyes (23.8%) reached 20/400 or better visual acuity at the final follow-up. The KPro was retained in 11 in 21 eyes (52.4%). The retention rate decreased from 94.7% at 1 year to 53.5% at 5 years. The most frequent complications were retroprosthetic membrane (47.6%) and corneal melt (23.8%). CONCLUSION: Modified pars plana vitrectomy techniques resulted in relatively good mid-term anatomical, functional, and retention rate outcomes, given the severity of RD at presentation and the numerous preoperative comorbidities of KPro eyes.

EARLY DETECTION OF RETINAL HEMANGIOBLASTOMAS IN VON HIPPEL-LINDAU DISEASE USING ULTRA-WIDEFIELD FLUORESCEIN ANGIOGRAPHY.

PURPOSE: To study the use of ultra-widefield fluorescein angiography (UWF FA) in the detection and management of retinal capillary hemangioblastomas in patients with von Hippel-Lindau disease. METHODS: This is a retrospective study of patients with von Hippel-Lindau disease who underwent UWF FA using the Optos camera at a single center from June 2009 to May 2015. The clinical use of UWF FA was reviewed, and the number of hemangioblastomas identified on UWF FA was compared with ophthalmoscopy and a simulated seven standard field (7SF) FA montage. RESULTS: Twenty eyes of 10 patients were identified. Only 33% of lesions seen on UWF FA were also found on ophthalmoscopy, and 88% of lesions visualized on UWF FA were located outside the 7SF overlay. In 5 eyes that had gaze steering, 18% of lesions could be visualized only on gaze-steered images. For the 14 eyes with data available, 6 had procedures recommended and 8 eyes observed based on data from UWF FA. One of 20 eyes had a lesion on ophthalmoscopy that was missed by imaging. CONCLUSION: Ultra-widefield FA using the Optos camera is helpful for the evaluation and management of patients with von Hippel-Lindau disease. The UWF FA with gaze steering appears to detect more hemangioblastomas than ophthalmoscopy and conventional angiography.

NEW ULTRA-WIDE-FIELD ANGIOGRAPHIC GRADING SCHEME FOR RADIATION RETINOPATHY AFTER IODINE-125 BRACHYTHERAPY FOR UVEAL MELANOMA.

PURPOSE: Radiation retinopathy remains incompletely characterized and may cause severe vision loss. Ultra-wide-field fluorescein angiography provides a pan-fundus view of vascular alterations caused by radiation treatment and may predict visual and ocular outcomes. We have developed a grading scheme to describe pan-fundus severity and to predict the progression of radiation retinopathy in patients treated for uveal melanoma with iodine-125 brachytherapy. METHODS: A retrospective review of patients treated with standard iodine-125 brachytherapy for uveal melanoma at the Ophthalmic Oncology Center at the University of California, Los Angeles, who had undergone both baseline and postbrachytherapy ultra-wide-field fluorescein angiography. A grading scheme was devised based on observations of vascular leakage, retinal perfusion status, and retinal proliferation. The correlation of grade severity with patient characteristics, tumor features, visual acuity, optical coherence tomography findings, and neovascular glaucoma was measured with chi-square and one-way analysis of variance analyses. RESULTS: Sixty-seven patients were identified for review. Consistent wide-field angiographic patterns after brachytherapy were observed and graded as follows: Grade 0: normal; Grade 1: late foveal leakage; Grade 2: late peripheral leakage; Grade 3: presence of nonperfusion; and Grade 4: retinal neovascularization. Six eyes (8.9%) were Grade 0; 16 (23.8%) were Grade 1; 25 (37.3%) were Grade 2; 16 (23.4%) were Grade 3; and 4 (6.0%) were Grade 4. Higher grade radiation severity correlated significantly with duration of follow-up (P < 0.02); younger age (P = 0.035); worse visual acuity (P = 0.001); cystoid macular edema or atrophy on optical coherence tomography (P < 0.0001); and neovascular glaucoma (P = 0.003). CONCLUSION: Wide-field fluorescein angiography revealed distinct fundus-wide patterns of vascular damage, which were progressive in nature in eyes treated with iodine-125 brachytherapy for uveal melanoma and correlated with signs of progressive vascular injury. This grading scheme may have prognostic value to predict the progression of radiation retinopathy and to prognosticate visual outcomes in patients undergoing brachytherapy.

Juxtapapillary and circumpapillary choroidal melanoma: globe-sparing treatment outcomes with iodine-125 notched plaque brachytherapy.

PURPOSE: Managing juxtapapillary and circumpapillary choroidal melanoma with brachytherapy is challenging because of technical complications with accurate plaque placement and high radiation toxicity given tumor proximity to the optic nerve. We evaluated our center's experience using ultrasound-guided, Iodine (I)-125 notched plaque brachytherapy for treating choroidal melanoma contiguous with (juxtapapillary) and at least partially surrounding the optic disc (circumpapillary). METHODS: All cases of choroidal melanoma treated with I-125 notched plaque brachytherapy at our center from September 2003-December 2013 were retrospectively reviewed. Only patients with ≥18 months of follow-up who had lesions contiguous with the optic disc (0 mm of separation) were included. The tumor apex prescription dose was 85 Gy. Outcomes evaluated included local control, distant metastasis-free survival (DMFS), cancer-specific survival (CSS), overall survival (OS), visual acuity, and radiation toxicity. RESULTS: Thirty-four patients were included with a median follow-up of 44.1 months (range 18.2-129.0). AJCC T-category was T1 in 58.8%, T2 in 26.5%, and T3 in 14.7%. Median circumferential optic disc involvement was 50% (range 10%-100%). Eye retention was achieved in 94.1%. Actuarial 2- and 4-year rates of local recurrence were 3.1% and 7.6%, DMFS were 97.0% and 88.5%, CSS were 97.0% and 92.8%, and OS were 97.0% and 88.9%, respectively. In addition, 23.5% had visual acuity ≥20/200 at last follow-up. CONCLUSIONS: I-125 notched plaque brachytherapy provides high eye preservation rates with acceptable longer-term post-treatment visual outcomes. Based on our experience, choroidal melanoma directly contiguous with and partially encasing the optic disc may be effectively treated with this technique.

VITRECTOMY-ASSISTED BIOPSY FOR MOLECULAR PROGNOSTICATION OF CHOROIDAL MELANOMA 2 MM OR LESS IN THICKNESS WITH A 27-GAUGE CUTTER.

PURPOSE: Fine-needle aspiration biopsy of small choroidal melanomas 2 mm or less in thickness may be associated with a low biopsy yield. This report describes our center's experience using the 27-gauge vitreous cutter to perform tumor sampling in these small melanomas. METHODS: This was a retrospective analysis of all patients who underwent a 27-gauge vitreous cutter biopsy for molecular prognostication at the time of iodine-125 plaque placement for local treatment of choroidal melanoma ≤2.0 mm in height. RESULTS: Seventeen consecutive patients with a mean baseline tumor height of 1.67 mm (range, 1.31-2.03 mm) and median follow-up time of 7.0 months (range, 3.3-19.5 months) were included. The 27-gauge cutter biopsy yielded sufficient material for multiplex-ligation probe amplification (MLPA) or gene expression profiling (GEP) in 17/17 (100%) patients. Complications were limited to focal vitreous hemorrhage in 13/17 patients and diffuse vitreous hemorrhage in 1/17 patients, with no instance of retinal detachment or nonclearing vitreous hemorrhage. CONCLUSION: Tumor sampling of small choroidal melanoma with a 27-gauge vitreous cutter is safe and offers excellent biopsy yield for molecular prognostication when cancer prognosis is desired by patients.

Tumor-height regression rate after brachytherapy between choroidal melanoma gene expression profile classes: effect of controlling for tumor height.

PURPOSE: To evaluate the relationship between choroidal melanoma regression rate and its gene expression profile class after iodine-125 brachytherapy at 3 and 6 months, controlling for baseline tumor height. METHODS: Patients from October 2012 to January 2015 at a single Ophthalmic Oncology Center who had undergone iodine-125 brachytherapy for the treatment of choroidal melanoma and who had a gene expression profile test result obtained from intraoperative fine-needle aspiration biopsy at the time of plaque surgery were retrospectively reviewed. Baseline patient and tumor characteristics were obtained, including tumor height and gene expression profile test result. Tumor height at 3 and 6 months following treatment was obtained. Regression rate was analyzed with two-way analysis of variance to class type and baseline pre-operative tumor height. Class 2 patients were matched to class 1 patients by tumor height and resulting distributions of paired regression rate differences were compared. RESULTS: A total of 114 patients were studied. When preoperative tumor height was controlled for in the comparative analysis, neither group of patients at 3 or 6 months had a significant dependency between gene expression profile class and tumor regression rate. Additionally, class 1 and class 2 patients matched for pre-operative tumor height did not express different regression rates. CONCLUSIONS: Our study adds to a growing body of evidence that tumor regression rate does not necessarily depend on gene expression profile class type in choroidal melanoma after brachytherapy at 3 and 6 months when controlling for baseline tumor height.

23-mm iodine-125 plaque for uveal melanoma: benefit of vitrectomy and silicone oil on visual acuity.

PURPOSE: To review outcomes in mostly large uveal melanoma treated with a 23-mm-diameter iodine-125 plaque, the largest size available at our center, and the influence of vitrectomy and silicone oil 1000 centistokes for radiation attenuation. METHODS: A one-to-one matched case-control comparison was performed. Case patients were treated with a 23-mm-diameter iodine-125 plaque and vitrectomy with silicone oil 1000-cSt placement. Control cases, treated with 23-mm plaque alone, were matched to cases with respect to tumor size and distance from tumor apex to optic nerve and fovea. Postoperative complications, visual acuity and metastasis were reviewed. RESULTS: Twenty case patients with uveal melanoma treated with a 23-mm plaque were identified. The final logMAR vision was 0.83 in case patients and 2.06 in control patients (P = 0.0064); the change from pre-treatment to last follow-up logMAR vision was 0.70 in cases and 1.62 in controls (P = 0.019). Of good vision outcomes, 65 % of cases and 25 % of controls achieved vision ≥20/200 (P = 0.025). Of poor vision outcomes, 35 % of cases and 80 % of controls achieved vision <20/200 (P = 0.0053), and 5 % of cases and 35 % of controls achieved "light perception" or "no light perception" vision (P = 0.044). Thirty-nine of the 40 eyes (98 %) achieved local tumor control. Metastasis occurred in 15 % of cases and 45 % of controls (P = 0.082). CONCLUSIONS: Iodine-125 brachytherapy for mostly large uveal melanoma is effective in achieving local tumor control. Furthermore, combining brachytherapy with vitrectomy and silicone oil 1000-cSt for radiation attenuation significantly improves vision over the use of plaque alone.

IMPACT OF INTERNAL LIMITING MEMBRANE PEELING ON MACULAR HOLE REOPENING: A Systematic Review and Meta-Analysis.

PURPOSE: To assess the literature regarding macular hole reopening rates stratified by whether the internal limiting membrane (ILM) was peeled during vitrectomy surgery. METHODS: Systematic review and meta-analysis of studies reporting on macular hole reopenings among previously surgically closed idiopathic macular holes. A comprehensive literature search using the National Library of Medicine PubMed interface was used to identify potentially eligible publications in English. The minimum mean follow-up period for reports to be included in this study was 12 months. Analysis was divided into eyes that underwent vitrectomy with and without ILM peeling. The primary outcome parameter was the proportion of macular hole reopenings among previously closed holes between the two groups. Secondary outcome parameters included duration from initial surgery to hole reopening and preoperative and postoperative best-corrected correct visual acuities among the non-ILM peeling and ILM peeling groups. RESULTS: A total of 50 publications reporting on 5,480 eyes met inclusion criteria and were assessed in this meta-analysis. The reopening rate without ILM peeling was 7.12% (125 of 1,756 eyes), compared with 1.18% (44 of 3,724 eyes) with ILM peeling (odds ratio: 0.16; 95% confidence interval: 0.11-0.22; Fisher's exact test: P < 0.0001). There were no other identifiable associations or risk factors for reopening. CONCLUSION: The results of this meta-analysis support the concept that ILM peeling during macular hole surgery reduces the likelihood of macular hole reopening.

Postinjection endophthalmitis in the comparison of age-related macular degeneration treatments trials (CATT).

OBJECTIVE: To describe the incidence and outcomes of endophthalmitis after intravitreal injections of anti-vascular endothelial growth factor agents in the Comparison of Age-Related Macular Degeneration Treatments Trials (CATT) and to assess the effect of prophylactic topical antimicrobials on incidence. DESIGN: Cohort study within a randomized clinical trial. PARTICIPANTS: Patients enrolled in CATT. METHODS: Patients with neovascular age-related macular degeneration received intravitreal injections of ranibizumab or bevacizumab under 1 of 3 dosing regimens. The study protocol specified preinjection preparation to include use of a sterile lid speculum and povidone iodine (5%). Use of preinjection and postinjection antibiotics was at the discretion of the treating ophthalmologist. Patients were followed up monthly for 2 years. MAIN OUTCOME MEASURES: Development of endophthalmitis and visual acuity. RESULTS: Endophthalmitis developed after 11 of 18 509 injections (1 per 1700 [0.06%]; 95% confidence interval, 0.03%-0.11%), and in 11 of 1185 patients (0.93%; 95% confidence interval, 0.52-1.66). Incidence of endophthalmitis was 0.15% among injections with no antibiotic use, 0.08% among injections with preinjection antibiotics only, 0.06% among injections with postinjection antibiotics only, and 0.04% among injections with preinjection and postinjection antibiotics (P = 0.20). All eyes were treated with intravitreal antibiotics and 4 underwent vitrectomy. Among the 11 affected eyes, the final study visual acuity was 20/40 or better in 4 eyes (36%), 20/50 to 20/80 in 2 eyes (18%), 20/100 to 20/160 in 3 eyes (27%), and worse than 20/800 in 2 eyes (18%). The final visual acuity was within 2 lines of the visual acuity before endophthalmitis in 5 eyes (45%). CONCLUSIONS: Rates of endophthalmitis were low and similar to those in other large-scale studies. Use of topical antibiotics either before or after injection does not seem to reduce the risk for endophthalmitis.

Dexamethasone implant anterior chamber migration: risk factors, complications, and management strategies.

OBJECTIVE: To describe the risk factors, clinical course, and complications of migration of a dexamethasone (DEX) intravitreal implant (OZURDEX; Allergan, Inc., Irvine, CA) into the anterior chamber and subsequent management strategies. DESIGN: Retrospective, observational case series. PARTICIPANTS: Fifteen patients had 18 episodes of migration of the DEX implant into the anterior chamber. METHODS: The medical records of 15 patients with spontaneous migration of a DEX implant were retrospectively reviewed. MAIN OUTCOME MEASURES: Migration of the DEX implant into the anterior chamber. RESULTS: Migration of a DEX intravitreal implant into the anterior chamber occurred in 6 patients who were aphakic, 4 patients with an anterior chamber intraocular lens, 2 patients with a scleral-fixated posterior chamber intraocular lens (PCIOL), 2 patients with a PCIOL, and 1 patient with an iris-fixated PCIOL. All 15 patients had prior pars plana vitrectomy, and 14 patients (93%) had no lens capsule. The average interval from DEX implant injection to detection of the implant migration into the anterior chamber was 13 days (range, 5-44 days). In 14 patients, corneal edema developed. Among those eyes undergoing surgical removal of the implant, earlier intervention reduced the likelihood of permanent corneal edema (0.5 days [from diagnosis of migration to surgical removal of the implant] vs. 5.5 days; P = 0.04). Aspiration was necessary to remove the implant in 6 patients. Among the 14 patients with corneal edema, the corneal edema did not resolve in 10 patients (71%), 6 (43%) of whom required corneal transplantation. CONCLUSIONS: Absence of lens capsule and prior vitrectomy are risk factors for migration of the DEX implant into the anterior chamber. Early removal of the implant may be necessary to minimize the risk of chronic corneal edema.

Spectral domain optical coherence tomography findings in patients with acute syphilitic posterior placoid chorioretinopathy.

PURPOSE: To describe the appearance of acute syphilitic posterior placoid chorioretinitis, a rare ocular manifestation of syphilis, on spectral domain optical coherence tomography (SD OCT) both before and after treatment. METHODS: Ophthalmic examination and imaging studies of 30 eyes of 19 confirmed cases were analyzed both at the time of presentation and at each follow-up visit. Patients with SD OCT and fluorescein angiography at the time of presentation, and at least three documented follow-up visits after initiation of therapy, were included in the study. Standard treatment of neurosyphilis was given to each patient, including 4 million units of penicillin G administered intravenously every 4 hours for 14 days. RESULTS: Fundus examination and imaging studies were consistent with previous reports and confirmed the diagnosis of acute syphilitic posterior placoid chorioretinitis. In 13 eyes (43.3%), baseline SD OCT scans were performed within 1 to 2 days of presentation and revealed a small amount of subretinal fluid, disruption of the inner segment/outer segment junction, and hyperreflective thickening of the retinal pigment epithelium (RPE). All 30 eyes were again scanned between Days 7 and 9 after presentation and revealed loss of the inner segment/outer segment and OS/RPE bands, and irregular hyperreflectivity of the RPE with prominent nodular elevations but without subretinal fluid. Early disruption of the external limiting membrane and punctate choroidal hyperreflectivity were seen in 1 of the 30 eyes (3.3%) and 14 of the 30 eyes (46.6%), respectively. Vision improved and the outer retinal abnormalities normalized in 28 of the 30 eyes (93.3%) after the treatment of neurosyphilis. The external limiting membrane, inner segment/outer segment band, and/or linear outer segment/RPE junction remained substantially abnormal despite treatment in 2 eyes left with 20/200 vision. CONCLUSION: Patients with acute syphilitic posterior placoid chorioretinitis show characteristic outer retinal abnormalities on SD OCT imaging, including disruption of the inner segment/outer segment band, nodular thickening of the RPE with loss of the linear outer segment/RPE junction, and, in some cases, loss of the external limiting membrane, accumulation of subretinal fluid, and punctate hyperreflectivity in the choroid. Vision improved and these abnormalities reversed after treatment of neurosyphilis in most of the patients. Persistently, poor vision despite treatment was associated with long-term loss or disruption of outer retinal anatomy on SD OCT.

Intravitreal injection technique and monitoring: updated guidelines of an expert panel.

PURPOSE: To review evidence and provide updated guidelines on intravitreal (IVT) injection technique and monitoring. METHODS: A review of the published literature on IVT injection from 2004 to 2014 formed the basis for round table deliberations by an expert panel of ophthalmologists. RESULTS: The dramatic increase in the number of IVT injections has been accompanied by a comparable increase in evidence surrounding IVT practice patterns and techniques. The expert panel identified a number of areas that have evolved since publication of the original IVT injection guidelines in 2004, the most notable of which were a lack of evidence to support the routine use of pre-, peri-, and postinjection antibiotics to reduce the risk of endophthalmitis, and the role of aerosolized droplets containing oral contaminants from the patient and/or providers as a potential source of infection. The panel emphasized the continued importance of applying povidone-iodine to and avoiding eyelid contact with the intended injection site and needle. CONCLUSION: Updated guidelines on IVT injection technique and monitoring are proposed based on a review of published literature and expert panel deliberations.

Ophthalmic surgery simulator training improves resident performance of capsulorhexis in the operating room.

PURPOSE: To assess the effect of a Capsulorhexis Intensive Training Curriculum (CITC) on the rates of errant, continuous, curvilinear capsulorhexes (CCCs) during cataract surgery among resident surgeons at a teaching hospital. DESIGN: Retrospective educational interventional case series. PARTICIPANTS AND CONTROLS: A total of 1037 consecutive cataract surgeries performed at Harbor-UCLA Medical Center during 4 consecutive academic years were considered. The baseline cohort consists of 434 cataract surgeries performed during the 2 academic years before the intervention. The postintervention cohort consists of 603 cataract surgeries performed during the following 2 consecutive academic years. INTERVENTION: The principal intervention was the introduction of the CITC for residents on the Eyesi (VRmagic, Mannheim, Germany) ophthalmic virtual reality surgical simulator. MAIN OUTCOME MEASURES: The main outcome measure was the rate of errant CCCs among the capsulorhexes performed during resident surgical cases. Errant CCCs were defined as attempted CCCs that resulted in the attending physician taking over, radialization of the CCC, conversion to can-opener capsulorhexis, or any combination of the 3 aforementioned conditions. Secondary measures included the use of trypan blue during CCC and correlating errant CCC and surgeons' level of training (postgraduate year [PGY]). RESULTS: There were 68 errant CCCs (15.7%) in the baseline cohort and 30 errant CCCs (5.0%; P<0.0001) in the postintervention cohort, a 3.2-fold or 68% reduction. The use of trypan blue increased from 55.3% in the baseline cohort to 76.0% in the postintervention cohort (P<0.00001), but within each cohort there was no significant difference in the rate of errant CCCs whether trypan blue was used or not. In the baseline cohort, there was a statistical trend toward fewer errant CCCs among PGY 4 (14.6%) compared with PGY 3 (22.8%) surgeons (P = 0.12). The postintervention cohort showed no significant difference in errant CCC rates between PGY 3 (4.4%) and PGY 4 (5.1%) surgeons (P = 1.00). CONCLUSIONS: This study strongly suggests that virtual reality surgical simulation training with the CITC on the Eyesi reduces the rate of errant capsulorhexes. The incorporation of a formal program for surgical training via virtual reality simulation should be strongly considered in ophthalmology resident surgical education to reduce the unnecessary risk of complications for live patients.

Adoption of electronic health records and preparations for demonstrating meaningful use: an American Academy of Ophthalmology survey.

OBJECTIVE: To assess the current state of electronic health record (EHR) use by ophthalmologists, including adoption rate, user satisfaction, functionality, benefits, barriers, and knowledge of meaningful use criteria. DESIGN: Population-based, cross-sectional study. PARTICIPANTS: A total of 492 members of the American Academy of Ophthalmology (AAO). METHODS: A random sample of 1500 AAO members were selected on the basis of their practice location and solicited to participate in a study of EHR use, practice management, and image management system use. Participants completed the survey via the Internet, phone, or fax. The survey included questions about the adoption of EHRs, available functionality, benefits, barriers, satisfaction, and understanding of meaningful use criteria and health information technology concepts. MAIN OUTCOME MEASURES: Current adoption rate of EHRs, user satisfaction, benefits and barriers, and availability of EHR functionality. RESULTS: Overall, 32% of the practices surveyed had already implemented an EHR, 15% had implemented an EHR for some of their physicians or were in the process of implementation, and another 31% had plans to do so within 2 years. Among those with an EHR in their practice, 49% were satisfied or extremely satisfied with their system, 42% reported increased or stable overall productivity, 19% reported decreased or stable overall costs, and 55% would recommend an EHR to a fellow ophthalmologist. For those with an electronic image management system, only 15% had all devices integrated, 33% had images directly uploaded into their system, and 12% had electronic association of patient demographics with the image. CONCLUSIONS: The adoption of EHRs by ophthalmology practices more than doubled from 2007 to 2011. The satisfaction of ophthalmologists with their EHR and their perception of beneficial effects on productivity and costs were all lower in 2011 than in 2007. Knowledge about meaningful use is high, but the percentage of physicians actually receiving incentive payments is relatively low. Given the importance of imaging in ophthalmology, the shortcomings in current image management systems need to be addressed. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Peripheral polypoidal choroidal vasculopathy as a cause of peripheral exudative hemorrhagic chorioretinopathy: a report of 10 eyes.

PURPOSE: Polypoidal choroidal vasculopathy (PCV) is characterized by polyp-like sub-retinal pigment epithelium vascular abnormalities predominantly found in the macula and peripapillary region. Less commonly, PCV can be found peripherally and be a cause of peripheral exudative hemorrhagic chorioretinopathy (PEHCR). We sought to further describe the clinical spectrum of this ill-defined subgroup of PEHCR. METHODS: A retrospective observational case series, of 10 eyes of 8 patients diagnosed with PEHCR caused by peripheral PCV, was conducted. In all cases, the presence of PCV was confirmed with indocyanine green angiography and/or fluorescein angiography and optical coherence tomography. The clinical presentation, natural history, and clinical outcomes with or without intervention were studied. RESULTS: Patients with PEHCR caused by peripheral PCV were most commonly men, white, asymptomatic, and had a concomitant diagnosis of age-related macular degeneration. The mean age was 70 years (range, 59-82 years) with a mean follow-up of 32.5 months (range, 4-91 months). Four patients had unilateral involvement with minimal subretinal hemorrhage that resolved spontaneously, one patient had unilateral involvement outside the macula that responded to anti-vascular endothelial growth factor therapy, one patient had unilateral involvement with subretinal hemorrhage threatening the macula that responded to anti-vascular endothelial growth factor therapy, and two patients had extensive bilateral subretinal hemorrhage requiring surgical intervention. Both patients with multiple lesions in one eye had bilateral lesions (two of eight patients). Lesions were most commonly located in the temporal periphery (8 of 10 eyes). CONCLUSION: A new subclassification is proposed that includes both eyes with polyps and those without polyps within the spectrum of disease described previously as PEHCR. Within the spectrum of disease described previously as PEHCR exists a subgroup of lesions caused by peripheral PCV, which has not been well defined before this report. The largest case series to date of eyes with PEHCR due to peripheral PCV, a unique form of type 1 neovascularization, is further classified and described. These eyes have a spectrum of disease, including small, medium-sized, and large lesions. Although most eyes with PEHCR from peripheral PCV experience a benign course with spontaneous resolution, a subset of eyes may experience macula-threatening hemorrhage, requiring treatment with laser-based therapies, anti-vascular endothelial growth factor injections, or surgical intervention.

Primary Autologous Stem Cell Transplantation for Unilateral Primary Central Nervous System Lymphoma-Ophthalmic Variant (Primary Vitreoretinal Lymphoma).

Purpose: To describe the long-term outcomes of 2 cases of primary autologous stem cell transplantation (ASCT) for the treatment of primary central nervous system lymphoma-ophthalmic variant (PCNSL-O) or primary vitreoretinal lymphoma (PVRL). Methods: Two cases and their findings were analyzed. A review of the histopathology, systemic treatment, and multimodal ocular imaging was performed. Results: A 52-year-old woman and 56-year-old woman were referred for vitritis and retinal lesions suspicious for PCNSL-O. The initial vitreous biopsies were inconclusive. Both patients had subsequent chorioretinal biopsies that confirmed the diagnosis of diffuse large B-cell lymphoma. No systemic or central nervous system involvement was found on systemic workup. Both patients received intravitreal and systemic chemotherapy followed by ASCT, and both remained in complete remission 7 and 8 years later. Conclusions: These cases show the long-term survival of patients diagnosed with PVRL when primary ASCT, the primary treatment for PCNSL, is performed.