Surgical Management of Full-Thickness Macular Holes in Macular Telangiectasia Type 2: A Global Multicenter Study.

PURPOSE: To report on macular hole repair in macular telangiectasia type 2 (MacTel2). DESIGN: Global, multicenter, retrospective case series. PARTICIPANTS: Patients undergoing surgery for MacTel2-associated full-thickness macular hole (MTMH). METHODS: Standardized data collection sheet distributed to all surgeons. MAIN OUTCOME MEASURES: Anatomic closure and visual outcomes of MTMH. RESULTS: Sixty-three surgeries in 47 patients with MTMH were included from 30 surgeons. Mean age was 68.1 years, with 62% female, 72% White, 21% East or South Asian, 2% African American, and 2% Hispanic or Latino. Procedures included 34 internal limiting membrane (ILM) peeling alone, 22 ILM flaps, 5 autologous retinal transplantations (ARTs), 1 retinotomy, and 1 subretinal bleb. For ILM peeling, preoperative visual acuity (VA) was 0.667 ± 0.423 logarithm of the minimum angle of resolution (logMAR). Minimum hole diameter (MHD) was 305.5 ± 159.4 µm (range, 34-573 µm). Sixteen of 34 ILM peels (47%) resulted in MTMH closure. At postoperative month 6, VA was stable at 0.602 ± 0.516 logMAR (P = 0.65). VA improved by at least 2 lines in 43% and at least 4 lines in 24%. For ILM flaps, preoperative VA was 0.878 ± 0.552 logMAR. MHD was 440.8 ± 175.5 µm (range, 97-697 µm), which was significantly larger than for ILM peels (P < 0.01). Twenty of 22 ILM flaps (90%) resulted in MTMH closure, which was significantly higher than for ILM peels (P < 0.01). At postoperative month 6, VA improved to 0.555 ± 0.405 logMAR (P < 0.05). VA improved by at least 2 lines in 56% and at least 4 lines in 28%. For ARTs, preoperative VA was 1.460 ± 0.391 logMAR. MHD was 390.2 ± 203.7 µm (range, 132-687 µm). All 5 ARTs (100%) resulted in MTMH closure. At postoperative month 6, VA was stable at 1.000 ± 0.246 logMAR (P = 0.08). Visual acuity improved at least 2 lines in 25%. CONCLUSIONS: Surgical closure of macular holes improved VA in 57% of MTMHs. Internal limiting membrane flaps achieved better anatomic and functional outcomes than ILM peeling alone. Autologous retinal transplantation may be an option for refractory MTMHs. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Multimodal imaging risk factors predictive of small choroidal melanocytic lesion growth to melanoma: An educational study and pictorial guide.

BACKGROUND: Risk factors for small choroidal melanocytic lesion growth to melanoma have been redefined using multimodal imaging. We explored provider ability to recognize risk factors for small choroidal melanocytic lesion growth to melanoma before and after image-based education and with and without multimodal imaging. METHODS: Providers were invited to participate in a survey assessing ability to identify risk factors for small choroidal melanocytic lesion growth to melanoma using either fundus imaging or multimodal imaging. Risk factors included thickness >2 mm on ultrasonography, subretinal fluid on optical coherence tomography, presence of orange pigment by autofluorescence, acoustic hollowness by ultrasonography, and diameter >5 mm by fundus imaging. Performance was assessed before and after reviewing an educational PowerPoint providing pictorial examples of risk factors. Comparison between groups was conducted using two-tailed Fisher's exact test. RESULTS: Thirty and 26 providers completed the pre-education and post-education assessments, respectively. Post-education participants were more accurate within ±1 risk factor for lesions with zero risk factors (77% vs. 100%, p = 0.01) or two risk factors (79% vs. 91%, p = 0.03). Following education, participants presented with multimodal imaging more often correctly identified lesions with four (12% vs. 42%, p = 0.03) or five (4% vs. 39%, p = 0.004) risk factors, demonstrated lower mean level of concern for lesions with zero risk factors (2.0 vs. 1.4, p < 0.001), and expressed higher level of concern for lesions with 5 risk factors (2.4 vs. 3.6, p < 0.001). CONCLUSION: Use of multimodal imaging may be more beneficial than education itself to improve accuracy of risk factor identification for small choroidal melanocytic lesions.

Identification of Choroidal Vascular Abnormalities in Patients with Hereditary Hemorrhagic Telangiectasia (HHT).

PURPOSE: To evaluate the frequency of ocular imaging in patients with HHT at a large academic medical institution; characterize the choroidal vascular changes on indocyanine green angiography (ICGA) and optical coherence tomography (OCT); and explore the relationship of these ocular features with HHT genotypes and systemic manifestations. METHODS: Retrospective review of patients with HHT who were seen in the Mayo Clinic ophthalmology department between 2011 to 2021. RESULTS: Of the 71 patients with HHT identified, 7 (10%) individuals had qualifying imaging defined as radial or raster spectral-domain OCT scans and/or ICGA. 4 (57%) patients had enlarged choroidal vessels detected on OCT and/or ICGA. 3 (43%) patients had retinal telangiectasias. Patients with choroidal changes (mean age 67 years) had the endoglin gene mutation, gastrointestinal arteriovenous malformations (AVM), and facial and mucocutaneous telangiectasias. In contrast, patients without choroidal changes (mean age 45 years) included the activin A receptor type-like kinase 1 gene mutation. CONCLUSION: Only a small proportion of patients with HHT undergo ophthalmic imaging at our institution. The presence of choroidal abnormalities is associated with the endoglin gene mutation, older age, corroborating retinal vascular changes, and increased systemic manifestations.

Retinal Vasculopathy With Cerebral Leukoencephalopathy and Systemic Manifestations: Critical Role of Retina Specialists.

Purpose: To describe a case of retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) to enhance early recognition of this often-missed diagnosis. Methods: A case report is presented. Results: A 50-year-old woman with a history of Raynaud phenomenon, memory difficulties, and a family history of strokes was referred for evaluation of a bilateral, small-vessel, occlusive disease refractory to immunosuppressive therapy. An extensive workup for treatable causes was unrevealing. Fifteen months after presentation, brain imaging showed white-matter lesions and dystrophic calcification, which led to the discovery of a pathogenic variant in TREX1 and the diagnosis of RVCL-S. Conclusions: Retina specialists play a critical role in the timely diagnosis of RVCL-S. Although the findings in this condition can mimic those in other common retinal vascular disorders, there are key characteristics that increase the suspicion for RVCL-S. Early recognition might decrease unnecessary therapies and procedures.

Acute syphilitic posterior placoid chorioretinitis as the presenting symptom of syphilis in an immunocompetent patient.

Purpose: To report the case of an immunocompetent 62-year old woman with acute syphilitic posterior placoid chorioretinitis. Observations: The patient presented with sudden, painless vision loss in the left eye (OS) four months after self-resolving decreased vision in the right eye (OD) which was incorrectly attributed to ischemic optic neuropathy. At the time of presentation, visual acuity (VA) was hand motion OS and 20/30 OD. The dilated fundus exam demonstrated a flat, yellow-white macular lesion, deep to the retinal vasculature with a temporal, curvilinear demarcation line OS and was unremarkable OD. Trace vitreous cells and veils OS were observed. Optical coherence tomography demonstrated loss of photoreceptor layers. Rapid plasma reagin and fluorescent treponemal antibody absorption were positive. The patient was treated with intravenous penicillin and prednisolone acetate drops with resolution of vitreous cells and return of VA. Conclusions and Importance: Acute syphilitic posterior placoid chorioretinitis can be the single presenting symptom in syphilis. It is imperative for ophthalmologists to consider this relatively uncommon manifestation of syphilis in the differential in immunocompetent patients presenting with unsuspecting histories and perplexing vision loss.

Irreversible Retinal Pigment Epithelium Toxicity Associated with Fibroblast Growth Factor Receptor Inhibitor Therapy.

PURPOSE: Erdafitinib is a fibroblast growth factor receptor inhibitor indicated for the treatment of cancer. A case of fibroblast growth factor receptor inhibitor-associated retinopathy that resulted in significant visual symptoms and chronic subretinal abnormalities is reported. METHODS: A 73-year-old male with a history of relapsed multiple myeloma was treated with erdafitinib. Soon after his fourth treatment cycle he developed blurred vision in both eyes. Therapy with erdafitinib was subsequently discontinued. RESULTS: Funduscopic examination and optical coherence tomography of both eyes revealed multifocal subretinal fluid in the macula of both eyes. Eleven weeks after cessation of the erdafitinib, visual acuity improved but the patient reported bilateral annular scotomas. Evaluation was notable for resolution of the subretinal fluid with the development irregular subfoveal thickening in both eyes. The patient's symptoms improved but the subfoveal abnormalities were persistent at 14 months follow up. CONCLUSION: Erdafitinib may be associated with permanent retinal pigment epithelium toxicity.

The reporting of uveal melanoma cases to the cancer registry in North Carolina.

Objective: To ascertain the completeness of reporting of uveal melanoma cases in North Carolina to the state's cancer registry. Methods: This was a retrospective chart review performed at a single institution analyzing the completeness of information reported to the North Carolina Cancer Registry between 2010 and 2015. A list of all patients with uveal melanoma diagnosed, treated and/or followed at UNC-Chapel Hill between 2010-2015 was compared to the list of patients with uveal melanoma reported to the North Carolina Central Cancer registry during the same time frame. Results: Based on ICD 9 and 10 codes, there were 66 patients with ciliary body or choroidal melanomas diagnosed, followed and/or treated at UNC between 2010 and 2015. Of those, 41 (62%) were on the list of cases reported through the UNC Cancer Registry to the NCCCR. A chart review of the excluded cases was performed and the following barriers to reporting of uveal melanoma were identified: lack of diagnostic imaging results, lack of histopathologic confirmation, inconsistent language used to communicate diagnosis, and lack of implementation of the North American Association of Central Cancer Registries' National Interstate Data Exchange Agreement. Conclusion: The diagnosis and treatment of uveal melanoma is unique when compared to other types of cancers. Diagnosis is based on clinical features and characteristic findings on ophthalmic imaging and ultrasound. There is often no pathology report or radiologic imaging which makes it difficult for hospital registrars to recognize and confirm cases of uveal melanoma. This creates significant barriers to reporting cases to state and national cancer registries. The incomplete data makes it difficult to detect changes in the incidence of uveal melanoma in North Carolina. The development of a national uveal melanoma registry should be seriously considered.

Migrainous infarction of the eye: Two cases of monocular ischemic complications associated with retinal migraine.

BACKGROUND: Retinal migraine is defined by fully reversible monocular visual phenomena. We present two cases that were complicated by permanent monocular vision deficits. CASES: A 57-year-old man with history of retinal migraine experienced persistent monocular vision loss after one stereotypical retinal migraine, progressing to finger-count vision over 4 days. He developed paracentral acute middle maculopathy that progressed to central retinal artery occlusion. A 27-year-old man with history of retinal migraine presented with persistent right eye superotemporal scotoma after a retinal migraine. Relative afferent pupillary defect and superotemporal visual field defect were noted, consistent with ischemic optic neuropathy. CONCLUSION: Retinal migraine can complicate with permanent monocular visual loss, suggesting potential migrainous infarction of the retina or optic nerve. A thorough cerebrovascular evaluation must be completed, which was unrevealing in our cases. Acute and preventive migraine therapy may be considered in retinal migraine patients, to mitigate rare but potentially permanent visual loss.

Nuclear Medicine and Molecular Imaging in Nodal Staging and Surveillance of Ocular Melanoma: Case Reports and Review of the Literature.

Ocular melanoma (OM) is a rare noncutaneous malignancy and consists of 2 different subtypes based on the anatomic location in the eye: uveal melanoma and conjunctival melanoma. Like cutaneous melanoma, OM benefits from nuclear medicine and molecular imaging in nodal staging and clinical management. Through the illustration of 2 distinctive cases, we aim to demonstrate the complementary roles of standard lymphoscintigraphy, advanced SPECT/CT, 18F-FDG PET/CT, and 18F-FDG PET/MRI in accurate nodal staging and surveillance of OM. We also review the epidemiology, existing staging guidelines, and management of uveal melanoma and conjunctival melanoma.

Autologous Retinal Transplantation for Primary and Refractory Macular Holes and Macular Hole Retinal Detachments: The Global Consortium.

PURPOSE: To report the anatomic and functional outcomes of autologous retinal transplantation (ART). DESIGN: Multicenter, retrospective, interventional, consecutive case series. PARTICIPANTS: One hundred thirty eyes of 130 patients undergoing ART for the repair of primary and refractory macular holes (MHs), as well as combined MH-rhegmatogenous retinal detachment (MH-RRD), between January 2017 and December 2019. METHODS: All patients underwent pars plana vitrectomy and ART, with surgeon modification of intraoperative variables. A large array of preoperative, intraoperative, and postoperative data was collected. Two masked reviewers graded OCT images. Multivariate statistical analysis and subgroup analysis were performed. MAIN OUTCOME MEASURES: Macular hole closure rate, visual acuity (VA), external limiting membrane and ellipsoid zone (EZ) band integrity, and alignment of neurosensory layers (ANL) on OCT. RESULTS: One hundred thirty ART surgeries were performed by 33 vitreoretinal surgeons worldwide. Patient demographics were: mean age of 63 ± 6.3 years, 58% female, 41% White, 23% Black, 19% Asian, and 17% Latino. Preoperative VA was 1.37 ± 0.12 logarithm of the minimum angle of resolution (logMAR; Snellen equivalent, approximately 20/500), which improved significantly to 1.05 ± 0.09 logMAR (Snellen equivalent, approximately 20/225; P < 0.001) after surgery (mean follow-up, 8.6 ± 0.8 months). Autologous retinal transplantation was performed for primary MH repair in 27% of patients (n = 35), for refractory MH in 58% of patients (n = 76; mean number of previous surgeries, 1.6 ± 0.2), and for MH-RRD in 15% of patients (n = 19). Mean maximum MH diameter was 1470 ± 160 µm, mean minimum diameter was 840 ± 94 µm, and mean axial length was 24.6 ± 3.2 mm. Overall, 89% of MHs closed (78.5% complete; 10% small eccentric defect), with a 95% closure rate in MH-RRD (68.4% complete; 26.3% small eccentric defect). Visual acuity improved by at least 3 lines in 43% of eyes and by at least 5 lines in 29% of eyes. Reconstitution of the EZ (P = 0.02) and ANL (P = 0.01) on OCT were associated with better final VA. Five cases of ART graft dislocation (3.8%), 5 cases of postoperative retinal detachment (3.8%), and 1 case of endophthalmitis (0.77%) occurred. CONCLUSIONS: In this global experience, patients undergoing ART for large primary and refractory MHs and MH-RRDs achieved good anatomic and functional outcomes, with low complication rates despite complex surgical pathologic features.

Genomic profiling proves metastasis of cutaneous melanoma to vitreal fluid.

Cutaneous melanoma metastases can contribute to visual disturbances through a variety of factors, including metastasis to the vitreal fluid. The optimum management of metastatic cutaneous melanoma to the vitreal fluid is unknown, but can include radiation therapy or systemic therapy including immunotherapy. A high degree of suspicion is necessary to consider this complication while working with patients with cutaneous melanoma.

Recurrence of Acute Macular Neuroretinopathy.

A 31-year-old female with a 2-month history of a central scotoma was diagnosed with acute macular neuroretinopathy (AMNR). Her symptoms resolved spontaneously, only to recur 2 years later with progressively worsening visual field deficits that did not improve with a trial of oral prednisone. The authors report a case of AMNR that is distinguished from other reports by its recurrence in the same eye after complete resolution of the first episode. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:962-968.].

Ultra-widefield fundus autofluorescence in age-related macular degeneration.

BACKGROUND: Establish accuracy and reproducibility of subjective grading in ultra-widefield fundus autofluorescence (FAF) imaging in patients with age-related macular degeneration (AMD), and determine if an association exists between peripheral FAF abnormalities and AMD. METHODS: This was a prospective, single-blinded case-control study. Patients were consecutively recruited for the study. Patients were excluded if there was a history of prior or active ocular pathology other than AMD or image quality was insufficient for analysis as determined by two independent graders. Control patients were those without any evidence of AMD or other ophthalmic disease apart from cataract. Using the Optos 200Tx (Optos, Marlborough, MA, USA), a ResMax central macula and an ultra-widefield peripheral retina image was taken for each eye in both normal color and short wavelength FAF. Ultra-widefield photographs were modified to mask the macula. Each ResMax and ultra-widefield image was independently graded by two blinded investigators. RESULTS: There were 28 AMD patients and 11 controls. There was a significant difference in the average age between AMD patients and control groups (80 versus 64, respectively P<0.001). There was moderate, statistically significant agreement between observers regarding image interpretation (78.4%, K = 0.524, P<0.001), and 69.0% (K = 0.49, P<0.001) agreement between graders for FAF abnormality patterns. Patients with AMD were at greater risk for peripheral FAF abnormalities (OR: 3.43, P = 0.019) and patients with FAF abnormalities on central macular ResMax images were at greater risk of peripheral FAF findings (OR: 5.19, P = 0.017). CONCLUSION: Subjective interpretation of FAF images has moderate reproducibility and validity in assessment of peripheral FAF abnormalities. Peripheral FAF abnormalities are seen in both AMD and control patients. Those with AMD, poor visual acuity, and macular FAF abnormalities are at greater risk.

Focal choroidal excavation associated with polypoidal choroidal vasculopathy.

A 48-year-old woman presented with blurred vision in her right eye for 6 weeks. Visual acuity was 20/300 and 20/25 in the right and left eyes, respectively. Fundus examination showed subretinal hemorrhage in the superonasal macula in the right eye, whereas the left eye was normal. Fluorescein angiography showed blocked fluorescence from hemorrhage and a round distinct hypofluorescent spot along the inferotemporal arcade. Indocyanine green angiography revealed hyperfluorescent tubular and aneurysmal dilatations consistent with polypoidal choroidal vasculopathy in the superior macula. Spectral-domain optical coherence tomography showed retinal pigment epithelial irregularities and detachment. Scans through the round area of hypofluorescence revealed a conforming focal choroidal excavation and thinning of the underlying choriocapillaries. Because the pathogenesis of focal choroidal excavation is currently unclear, the authors propose the possibility of an acquired etiology related to loss of choriocapillaries from perfusion abnormalities as evidenced here.

Automated segmentation of intraretinal cystoid fluid in optical coherence tomography.

Cystoid macular edema (CME) is observed in a variety of ocular disorders and is strongly associated with vision loss. Optical coherence tomography (OCT) provides excellent visualization of cystoid fluid, and can assist clinicians in monitoring the progression of CME. Quantitative tools for assessing CME may lead to better metrics for choosing treatment protocols. To address this need, this paper presents a fully automated retinal cyst segmentation technique for OCT image stacks acquired from a commercial scanner. The proposed method includes a computationally fast bilateral filter for speckle denoising while maintaining CME boundaries. The proposed technique was evaluated in images from 16 patients with vitreoretinal disease and three controls. The average sensitivity and specificity for the classification of cystoid regions in CME patients were found to be 91% and 96%, respectively, and the retinal volume occupied by cystoid fluid obtained by the algorithm was found to be accurate within a mean and median volume fraction of 1.9% and 0.8%, respectively.

Indocyanine green angiographic features in a case of alkali retinopathy.

PURPOSE: To report the fluorescein and indocyanine green angiographic features of a patient who developed posterior segment complications after an ocular alkali burn. METHODS: Clinical examination, fluorescein angiography, and indocyanine green angiography were performed on a patient with an ocular alkali injury. RESULTS: Clinical examination revealed an area of conjunctival and scleral necrosis in the injured eye. There was an area of retinal necrosis, which corresponded to the area of external necrosis. Fluorescein angiography demonstrated late leakage and obliteration of the retinal vessels in the area of necrosis. There was nonperfusion of the choroid with a surrounding rim of late indocyanine green leakage. CONCLUSION: Alkaline substances can penetrate sclera. Ocular alkali burns can result in choroidal ischemia and retinal necrosis. It is important to suspect posterior segment complications in all ocular alkali injuries. This case emphasizes the necessity of prompt irrigation of the conjunctival cul de sac and the removal of any solid chemical remnants after ocular alkali injuries.

Subretinal hemorrhage after lens-sparing vitrectomy in infants born at or under 24 weeks gestation.

PURPOSE: To describe short term outcomes in a case series of infants with rapidly progressive retinopathy of prematurity (ROP) requiring laser and lens-sparing vitrectomies (LSV) at early postmenstrual ages (PMAs). METHODS: Retrospective chart review of infants born at or under 24 0/7 weeks consecutively referred for management of severe ROP between August 2006 and January 2007. RESULTS: Five female infants (mean gestational age 23 4/7 weeks, mean birthweight 514.4 g) required laser treatment bilaterally for zone 1 or posterior zone II ROP at 35-37 weeks PMA. LSV was performed for progressive stage 4 ROP in eight eyes at 37-39 weeks PMA (mean 38.2 weeks). Subretinal hemorrhage occurred after LSV in five eyes, with three developing subretinal fibrosis preventing complete retinal reattachment. CONCLUSION: Premature infants in this series had rapidly progressive ROP requiring laser and LSV at early PMAs. Subretinal hemorrhage occurred and may be related to the amount of laser necessary to treat large zones of avascular retina, and the early timing of vitrectomy when postoperative contracting preretinal vitreous no longer has the natural tamponading force of formed gel against it. Improved therapies for premature infants of very young gestational ages and large avascular zones are needed.

Purtscher's retinopathy that occurred 6 months before acute pancreatitis.

PURPOSE: To report Purtscher's retinopathy in a patient with chronic pancreatitis 6 months before the development of fulminant acute pancreatitis. DESIGN: Observational case report. METHODS: Review of clinical chart, photographs, fluorescein angiography, and optical coherence tomography. RESULTS: A 45-year-old man with a history of alcohol abuse with a 3-day history of decreased vision in both eyes was examined. Diffuse retinal whitening and intraretinal hemorrhages that were consistent with Purtscher's retinopathy were present in both eyes. Serum amylase and lipase levels were normal. Six months later, he experienced intractable abdominal pain. Serum amylase and lipase levels were elevated markedly. Abdominal computed tomography and endoscopic retrograde cholangiopancreatography confirmed acute pancreatitis, with evidence of coexisting chronic pancreatitis. His funduscopic examination after the development of acute pancreatitis was improved, with almost complete resolution of retinal whitening and hemorrhages. Visual acuity remained poor because of retinal ischemia. CONCLUSIONS: Purtscher's retinopathy can be associated with chronic pancreatitis and can precede the development of fulminant acute pancreatitis.

Posterior scleritis secondary to Pseudomonas aeruginosa keratitis.

PURPOSE: To report a case of Pseudomonas aeruginosa keratitis that progressed to posterior scleritis. METHODS: Observational case report. RESULTS: A 64-year-old woman was treated for P. aeruginosa keratitis. Twelve days later, she was diagnosed with posterior scleritis and treated with oral ciprofloxacin. Her condition progressed, ultimately resulting in enucleation. Histopathology confirmed posterior scleritis, a previously unreported complication of P. aeruginosa keratitis. CONCLUSION: P. aeruginosa keratitis may progress to posterior scleritis, a potentially devastating complication.