Incidence, Management, and Surgical Outcomes of Macular Splitting Rhegmatogenous Retinal Detachment.

BACKGROUND AND OBJECTIVE: Limited knowledge exists regarding macular splitting retinal detachment (RD). The purpose of this study is to investigate clinical features and outcomes of macular splitting RD. PATIENTS AND METHODS: This was a retrospective case series performed at a single practice. Macular splitting RD was identified clinically and on optical coherence tomography (OCT). Primary outcomes were anatomical and functional success, and secondary outcomes were factors associated with postoperative visual acuity. RESULTS: The overall number of patients with OCT-confirmed macular splitting RD was 16 of 664, which is an incidence rate of 2.4%. Preoperative and final logMAR were 0.33 and 0.13, respectively (P = .002). Presenting visual acuity (VA) (P = 0.015) and duration of symptoms (P = .007) were associated with final VA, whereas time to surgery was not significant (P = .581). CONCLUSION: The incidence of macular splitting RD is higher than previously reported. Anatomical and functional outcomes were excellent in this study. [Ophthalmic Surg Lasers Imaging Retina. 2021;52:602-608.].

Pars Plana Vitrectomy Outcomes for Rhegmatogenous Retinal Detachment Qualifying for Pneumatic Retinopexy.

PURPOSE: To investigate real-world outcomes of pars plana vitrectomy (PPV) for eyes with primary rhegmatogenous retinal detachments (RRD) eligible for pneumatic retinopexy (PnR). METHODS: This was a single center retrospective case series looking at consecutive patients with primary RRDs. A database was created on all patients with a primary RRD from 2010 to 2018 based on billing code 67108. Eyes anatomically eligible for PnR were reviewed for preoperative, intraoperative and postoperative characteristics. The main outcome assessed was single surgery anatomical success (SSAS), final anatomical success (FAS), and postoperative LogMAR vision. RESULTS: A total of 720 eyes (age, 62.9 ± 9.1 years; 61.7% were male) met inclusion criteria for PnR and underwent PPV. SSAS was 94.0% and FAS was 99.9%. Preoperative and final LogMAR vision was 0.853 and 0.293 (P<0.001) in eyes with SSAS vs 0.714 and 0.648 (P=0.686) in eyes with primary failure. PVR was the most common etiology of primary surgical failure (n=21, 49%). Patients who failed primary repair had a mean of 1.12 additional surgeries with a median time of 45 days between surgeries. CONCLUSION: A robust single surgery success rate with good visual outcomes was achieved across 8 years and multiple surgeons utilizing PPV to treat primary RRDs in eyes which anatomically qualified for pneumatic retinopexy in a real-world setting.

Increased Reoperation Rate in Surgical Treatment of Rhegmatogenous Retinal Detachment with Coexistent Macular Hole.

PURPOSE: To examine the surgical outcomes in patients with coexistent macular hole (MH) and rhegmatogenous retinal detachment (RRD). DESIGN: Retrospective case series. PARTICIPANTS: All patients who underwent surgical repair of concomitant MH and retinal detachment (MHRD) between January 2014 and December 2016 in our facility were examined. At least 1 retinal break was noted in all MHRD cases. Exclusion criteria included MHRD related to high myopia without peripheral retinal tears. METHODS: Retrospective chart review. MAIN OUTCOME MEASURES: Data collected included presence of proliferative vitreoretinopathy (PVR) and classification at time of surgical repair, details of surgical repair including whether internal limiting membrane (ILM) peeling was achieved, type of ILM staining used, presence of retinal detachment (RD) in the fellow eye, and duration of follow-up. Outcomes evaluated included visual acuity comparisons, reoperation rate, final anatomic success, and MH closure rate. RESULTS: Over the study period, MHRD cases accounted for 17 of 745 (2.3%) of all repaired RDs in our practice. Proliferative vitreoretinopathy was present in 53% of MHRD cases. Reoperation rates for MHRD were significantly higher than our practice average for all RD repairs (29% vs. 9.7%; P = 0.01). Final anatomic success with RD was achieved in 100% of patients. Internal limiting membrane peeling was performed in 15 of 17 patients. Macular hole closure rate was 71% after initial surgery. Although 82% of patients experience equal or improved vision, only 24% of patients achieved visual acuity better than 20/80. Retinal detachment in the contralateral eye was noted in 3 of 16 patients (19%) included before initial presentation or during the follow-up period. CONCLUSIONS: Visual outcomes in MHRD cases were underwhelming because of high rates of presentation with PVR macula-off RRD, high reoperation rates, and relatively low MH closure rates. We suggest aggressive surgical techniques to repair MHRD.

The canonical Wnt signaling antagonist DKK2 is an essential effector of PITX2 function during normal eye development.

Local control of cell signaling activity and integration of inputs from multiple signaling pathways are central for normal development but the underlying mechanisms remain poorly understood. Here we show that Dkk2, encoding an antagonist of canonical Wnt signaling, is an essential downstream target of the PITX2 homeodomain transcription factor in neural crest during eye development. Canonical Wnt signaling is ectopically activated in central ocular surface ectoderm and underlying mesenchyme in Pitx2- and Dkk2-deficient mice. General ocular surface ectoderm identity is maintained during development in Dkk2-deficient mice but peripheral fates, including conjunctival goblet cells and eyelash follicles, are ectopically permitted within more central structures and eyelids are hypomorphic. Loss of DKK2 results in ectopic blood vessels within the periocular mesenchyme and PITX2 expression remains persistently high, providing evidence for a negative feedback loop. Collectively, these data suggest that activation of Dkk2 by PITX2 provides a mechanism to locally suppress canonical Wnt signaling activity during eye development, a paradigm that may be a model for achieving local or transient inhibition of pathway activity elsewhere during embryogenesis. We further propose a model placing PITX2 as an essential integration node between retinoic acid and canonical Wnt signaling during eye development.

Genetic markers and biomarkers for age-related macular degeneration.

Age-related macular degeneration (AMD) is the leading cause of visual impairment and blindness in the USA. Although the treatment of AMD has evolved to include laser photocoagulation, photodynamic therapy, surgical macular translocation and antiangiogenesis agents, treatment options for advanced AMD are limited. Furthermore, the dry form of AMD, albeit less devastating than the wet form, has even fewer viable treatment options. This review summarizes the various biomarkers of AMD and analyzes whether or not they may one day be exploited to determine risks of disease onset, measure progression of disease or even assess the effects of treatment of AMD. Potential biomarkers are important to identify since some might be utilized to reflect the disease state of a particular patient and to individualize therapy. Although studies have yielded promising results for nutrient and inflammatory biomarkers, these results have been inconsistent. At present, the best available markers of AMD risk are single nucleotide polymorphisms (SNPs). SNPs in complement factor H (CFH) and PLEKHA1/ARMS2/HtrA1 capture a substantial fraction of AMD risk and permit the identification of individuals at high risk of developing AMD.

Murine ccl2/cx3cr1 deficiency results in retinal lesions mimicking human age-related macular degeneration.

PURPOSE: Senescent Ccl2(-/-) mice are reported to develop cardinal features of human age-related macular degeneration (AMD). Loss-of-function single-nucleotide polymorphisms within CX3CR1 are also found to be associated with AMD. The authors generated Ccl2(-/-)/Cx3cr1(-/-) mice to establish a more characteristic and reproducible AMD model. METHODS: Single Ccl2- and Cx3cr1-deficient mice were crossbred to obtain Ccl2(-/-)/Cx3cr1(-/-) mice. Funduscopy, histopathology, retinal A2E quantification, proteomics, RT-PCR gene expression assay, immunochemistry, and Western blotting were used to examine the retina and to evaluate gene expression within the retinal tissue. RESULTS: By 6 weeks of age, all Ccl2(-/-)/Cx3cr1(-/-) mice developed AMD-like retinal lesions, including drusen, retinal pigment epithelium alteration, and photoreceptor degeneration. Furthermore, choroidal neovascularization occurred in 15% of the mice. These degenerative lesions progressed with age. A2E, a major lipofuscin fluorophore that accumulated during AMD progression, was significantly higher in the Ccl2(-/-)/Cx3cr1(-/-) retina than in the wild-type retina. Complement cofactor was higher in the Ccl2(-/-)/Cx3cr1(-/-) RPE. Proteomics data indicated that four proteins were differentially expressed in Ccl2(-/-)/Cx3cr1(-/-) retina compared with control. One of these proteins, ERp29, an endoplasmic reticulum protein, functions as an escort chaperone and in protein folding. CONCLUSIONS: The authors concluded that Ccl2(-/-)/Cx3cr1(-/-) mice develop a broad spectrum of AMD abnormalities with early onset and high penetrance. These observations implicate certain chemokines and endoplasmic reticulum proteins in AMD pathogenesis. Similar to the mechanism of neurodegeneration caused by dysfunction of endoplasmic reticulum proteins, decreased chaperoning may cause misfolded protein accumulation, leading to drusen formation and retinal degeneration.