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.

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.

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.].

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.

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.