Transcription factor TFAP2B up-regulates human corneal endothelial cell-specific genes during corneal development and maintenance.

The corneal endothelium, which originates from the neural crest via the periocular mesenchyme (PM), is crucial for maintaining corneal transparency. The development of corneal endothelial cells (CECs) from the neural crest is accompanied by the expression of several transcription factors, but the contribution of some of these transcriptional regulators to CEC development is incompletely understood. Here, we focused on activating enhancer-binding protein 2 (TFAP2, AP-2), a neural crest-expressed transcription factor. Using semiquantitative/quantitative RT-PCR and reporter gene and biochemical assays, we found that, within the AP-2 family, the TFAP2B gene is the only one expressed in human CECs in vivo and that its expression is strongly localized to the peripheral region of the corneal endothelium. Furthermore, the TFAP2B protein was expressed both in vivo and in cultured CECs. During mouse development, TFAP2B expression began in the PM at embryonic day 11.5 and then in CECs during adulthood. siRNA-mediated knockdown of TFAP2B in CECs decreased the expression of the corneal endothelium-specific proteins type VIII collagen α2 (COL8A2) and zona pellucida glycoprotein 4 (ZP4) and suppressed cell proliferation. Of note, we also found that TFAP2B binds to the promoter of the COL8A2 and ZP4 genes. Furthermore, CECs that highly expressed ZP4 also highly expressed both TFAP2B and COL8A2 and showed high cell proliferation. These findings suggest that TFAP2B transcriptionally regulates CEC-specific genes and therefore may be an important transcriptional regulator of corneal endothelial development and homeostasis.

Anterior segment Scheimpflug imaging for detecting primary angle closure disease.

PURPOSE: To evaluate the capability of anterior segment Scheimpflug imaging for detecting primary angle closure disease (PACD): primary angle closure suspect, primary angle closure, and primary angle closure glaucoma, using cutoff points derived from reference databases of healthy subjects. METHODS: Eighty-seven patients with PACD and 49 age-matched control subjects were included. We evaluated the sensitivity and specificity of anterior chamber depth (ACD), anterior chamber volume (ACV), and anterior chamber angle (ACA) to differentiate patients with PACD from controls. Additionally, the study's raw data was analyzed via receiver operating characteristic curves for comparison. RESULTS: One standard deviation from the normative data's mean values was used as the cutoff point and yielded a sensitivity and specificity of 96.2% and 92.6% for ACD, 97.1% and 75.9% for ACV, and 93.3% and 72.2% for ACA, respectively. Receiver operating characteristic analysis of the raw data showed the area under the curve to be 0.984, 0.975, and 0.931 for ACD, ACV, and ACA, respectively. CONCLUSIONS: Our study demonstrated that the parameters of anterior segment Scheimpflug imaging, particularly ACD, accurately discriminate PACD. This was the first study to validate the device's normative data in a separate population. With its high reproducibility, ease of use, non-invasiveness, and speed, anterior segment Scheimpflug imaging is a potentially powerful screening tool for PACD.

RETINAL MICROVASCULATURE AND VISUAL ACUITY AFTER INTRAVITREAL AFLIBERCEPT IN EYES WITH CENTRAL RETINAL VEIN OCCLUSION: An Optical Coherence Tomography Angiography Study.

PURPOSE: To investigate vascular perfusion and foveal avascular zone area in the superficial capillary plexus (SCP) and deep capillary plexus (DCP) after intravitreal aflibercept therapy in central retinal vein occlusion eyes and their association with best-corrected visual acuity. METHODS: Thirty-five subjects with central retinal vein occlusion and macular edema were evaluated. After macular edema resolution following intravitreal aflibercept, subjects underwent optical coherence tomography angiography to measure SCP and DCP perfusion and the foveal avascular zone within a 3 × 3-mm area. Correlations between best-corrected visual acuity and optical coherence tomography angiography measurements were examined. RESULTS: After intravitreal aflibercept therapy, mean retinal vascular area was 3.41 ± 0.74 mm in the SCP and 3.25 ± 0.91 mm in the DCP. Foveal avascular zone area was 1.03 ± 1.04 mm in the SCP and 1.78 ± 1.73 mm in the DCP. Improved best-corrected visual acuity was significantly associated with better SCP and DCP perfusion (both P < 0.001) and with smaller SCP and DCP foveal avascular zone areas (both P < 0.001). Additionally, SCP and DCP perfusion were negatively correlated with macular edema before treatment (P < 0.05) and ischemia (determined via pretreatment fluorescein angiography, P < 0.05), and positively correlated with photoreceptor integrity (P < 0.001). CONCLUSION: Patients with better retinal perfusion and less retinal ischemia are associated with better visual outcomes after aflibercept in eyes with central retinal vein occlusion.

Acute hydrops with a 180-degree massive edematous cavern demonstrated by three dimensional view of anterior segment optical coherence tomography in a patient with pellucid marginal corneal degeneration, a case report.

BACKGROUND: Pellucid marginal corneal degeneration is a non-inflammatory disorder complicated by severe inferior corneal thinning. The central portion of the cornea, consequently, appears to protrude outwards, decreasing vision by means of an irregular stigmatism. Additionally, acute hydrops can occur in case of Descemet's membrane rupture. CASE PRESENTATION: A 41-year-old Japanese woman presenting with severe visual loss in the left eye was examined and observed to have had full thickness corneal perforation as well as a Descemet membrane rupture with massive edema in the corneal stroma. Anterior segment optical coherence tomography-based corneal topography revealed a distorted crab claw sign indicating pellucid marginal corneal degeneration. The Descemet membrane rupture allowed acute hydrops to occur which was especially noteworthy given the scale of edema present within the stroma, rotating 180 degrees along the limbus, causing a smiley-face like lesion. We visualized it via a gonioscopic three-dimensional optical coherence tomography to build a three-dimensional video. Patient history revealed a previous acute hydrops in the right eye as well, which was ultimately treated with anterior lamellar keratoplasty, suggesting the pellucid marginal corneal degeneration had a classic bilateral involvement, which was also characterized with bilateral acute hydrops. CONCLUSION: This appears to be a very rare and interesting presentation of bilateral pellucid marginal degeneration, wherein not only acute hydrops formed bilaterally, but the cavity within the cornea stroma was exceptionally large with an unusual shape. Using the gonioscopic three-dimensional optical coherence tomography imaging, we were able to easily visualize the massive intrastromal cavern, and appropriately planned the crescent-shaped anterior lamellar keratoplasty. The 3d video constructed using this data is particularly elucidative compared to 2d images. As such, we recommend utilizing 3d imaging in cases where more conventional topography is not as explanatory with respect to precise nature of deformation.

Hemorrhagic hypopyon as presenting feature of intravascular lymphoma, a case report.

BACKGROUND: Herpes uveitis has been previously reported to present with hyphema, but hemorrhagic hypopyon is rarely reported as a herpetic uveitis manifestation. We report a case of herpes simplex virus (HSV) presenting with hemorrhagic hypopyon, and speculate on the underlying pathophysiology with relation to an intravascular lymphoma which was subsequently diagnosed as a result. CASE PRESENTATION: We present a case wherein a 62-year-old Japanese rheumatoid arthritis woman, with HSV uveitis, presented with hemorrhagic hypopyon in the anterior chamber and a fever with photophobia. Patient was treated with antiviral drugs which improved the hyphema and corneal lesions, but lesions recurred 3 months later. This rare presentation of HSV induced uveitis, and its subsequent recurrence, aroused suspicion of an additional hypopyon-inducing pathology. On account of previous history of lung opacities and elevated LDH, intravascular lymphoma was eventually diagnosed via lung biopsy. She was treated for the lymphoma which also completely resolved all ocular symptoms without any recurrence as of 1.5 years later. CONCLUSION: The exceedingly rare presentation of hemorrhagic hypopyon may have been enabled by an interaction of the HSV with the intravascular lymphoma. HSV involvement was indicated by the dendritic lesions, IgG assay, and response to anti-viral drugs. The ocular involvement of the intravascular lymphoma seems to be indicated by virtue of the anti-tumor drugs completely resolving all ocular symptoms.

Echocardiographic imaging of a bifurcated double barrel coronary sinus.

BACKGROUND: The coronary sinus (CS) is the terminal collecting vessel of the myocardial venous network, which returns deoxygenated blood used by the heart to the right atrium. The advent of high-fidelity imaging via CT and transesophageal echocardiography (TEE) has further defined the anatomy of the CS and its multiple tributaries. Understanding this anatomy is crucial for cardiac surgical cases that require the cannulation of the coronary sinus to deliver retrograde cardioplegia. However, anatomical variants of the CS may frustrate surgical retrograde catheter placement, in turn increasing the risk of CS injury or leading to inadequate cardioplegia delivery. Here, we present an especially unique CS presentation, a bifurcated, double-barrel CS, which was discovered via intraoperative TEE imaging that revealed a CS with two smaller lumens instead of the singular large os. CASE PRESENTATION: A 67-year-old male presented for ascending aortic dissection repair, aortic valve replacement, and single vessel coronary artery bypass graft. On the pre-bypass TEE exam, the anesthesiologist noted a bifurcated CS with two small lumens. The surgeon utilized this information to select a smaller diameter retrograde catheter to avoid damage or perforation of the vessel. With TEE guidance, the surgeon successfully cannulated one of the CS lumens. However, it was noted upon dosing of retrograde cardioplegia that all tributary vessels attached to the non-cannulated lumen remained devoid of cardioplegia. The surgeon was forced to repeatedly administer anterograde cardioplegia via a handheld catheter through the coronary ostium throughout the case. The operative field was also flooded with topical ice saline slush to ensure cardiac protection. Ultimately, the operation was completed without incident despite the non-ideal conditions resulting from this anatomic variant. CONCLUSIONS: Discovery of this patient's double-barrel CS during the pre-bypass TEE was incidental, showing that such anatomical variants may be completely asymptomatic and benign in the non-operative setting. However, the delivery of cardioplegia proved challenging for this patient, highlighting some degree of risk with certain cardiac interventions. This case demonstrates the utility of intraoperative TEE to quickly ascertain unforeseen anatomical variants of the CS which could compromise the safety of cardiac surgery cases.

Delayed Anaphylactic Reaction to Midazolam in the Absence of Immediate Respiratory or Skin Manifestations.

Anaphylaxis, a type 1 hypersensitivity reaction, is a feared but uncommon complication of medications administered in the perioperative period. The incidence of perioperative hypersensitivity reactions has been reported to range from 1 in 20,000 to 1 in 1,361. Anesthesiologists are well aware of common causes of hypersensitivity such as paralytics and antibiotics; however, less common triggers of anaphylaxis need to be considered as well. Midazolam, a short acting benzodiazepine metabolized by cytochrome P450 enzymes, is considered very safe with a minimal risk profile. Previous reports have described adverse reactions to occur within seconds to minutes following the administration of midazolam. We describe a patient with no known history of asthma or allergies who underwent elective hydrocelectomy with spinal analgesia without incident until 42 minutes later at the conclusion of the procedure, when they experienced circulatory collapse necessitating immediate emergency treatment. This case emphasizes the necessity to improve knowledge and awareness of delayed hypersensitivity reactions following the administration of perioperative medications such as midazolam.

Burnout and depression among anesthesiology trainees in the United States: An updated National Survey.

STUDY OBJECTIVE: A high prevalence of burnout, depression and suicidal ideation has been reported among anesthesiology trainees. Over the last decade, there has been a significant emphasis on the development of wellness programs in academic departments to mitigate the prevalence and effects of burnout during anesthesiology training. Therefore, we aimed to reevaluate the prevalence of burnout and depression in anesthesiology trainees in the United States. DESIGN: Cross-sectional survey. SETTING: Anesthesiology Department. PATIENTS: A nationally representative sample of 1000 anesthesiology trainees in the United States. MEASUREMENTS: A 33-item questionnaire consisting of 1) burnout, 2) depression, 3) job satisfaction, 4) family support, 5) work characteristics, 6) demographic factors, 7) self-reported errors, and 8) impact of COVID-19 pandemic. RESULTS: We received 384 responses. Twenty-four percent (91/384) of the respondents met the criteria for high burnout risk and 58/384 (15%) of the respondents screened positive for depression. Multivariable analysis revealed that: (1) hours per week > 70, (odds ratio [OR; 95% confidence interval {CI}] = 3.1 [1.4-6.8], P=0.005 and (2) overnight calls per month > 7 (OR [95% CI] = 2.5 [1.0- 6.0], P=0.03 were independent factors for increased odds of burnout whereas the presence of (3) married/domestic partnership (OR [95% CI} = 0.52 [0.32-0.85], P=0.01 was associated with lower odds of burnout and/or depression. Ten percent (4/40) of high burnout/depression residents reported that they often times fall short in the quality of care provided to patients compared to 0.36% (1/275) residents with low burnout/depression scores, P < 0.001. Similarly, 22% (9/40) of high burnout/depression residents reported that they often times did not have enough attention to their patients compared to 4% (11/275) residents with low burnout/depression scores, P < 0.001. CONCLUSIONS: We report a still concerning but significantly lower rate of burnout and depression in anesthesiology trainees than previously reported a decade ago. This suggests that efforts on wellness implemented by academic programs have positively impacted the work experience of anesthesia trainees.

Left Atrial Dissection Secondary to Retrograde Coronary Sinus Cannulation During a Stanford Acute Type-A Aortic Dissection Repair: A Case Report.

Left atrial (LA) dissections are rare phenomena, often iatrogenic, caused by blood flow into a false lumen, potentially obstructing the pulmonary veins or flow into the left ventricle. Severity can range from an incidental observation or complete circulatory collapse. While LA dissections are often associated with mitral valve repair, there are 2 reported cases of LA dissections associated with retrograde cardioplegia cannula insertion through the coronary sinus. Here, we present a large LA dissection that was directly visualized and tracked to a coronary sinus injury from the retrograde cardioplegia cannula. The clinical presentation and echocardiography findings informed our subsequent management.

Successful Anesthetic Management of an Adult with Sotos Syndrome.

Sotos syndrome is a rare genetic disorder presenting with craniofacial abnormalities, profound hypotonia, and cardiac abnormalities, giving rise to several potential challenges and concerns for an anesthesiologist. When preparing for a Sotos syndrome patient's case, we consulted the literature for precedents on how to plan the anesthetic, to which we were only able to find a few reports and nothing in the age group our patient fell within. We present our case of an adult in addition to examining the previous cases so as to document a precedent when encountering patients with this syndrome in the operating room. We describe a unique case of a nonverbal adult with hypotonia and severe craniofacial abnormalities who successfully underwent multiple dental extractions under general anesthesia, with no complications other than a delay of emergence attenuated by naloxone. Our case and the seven previous documented cases over the past several decades demonstrate anesthesia, including paralytics and intubation itself safe despite obvious concerns given the common features of the syndrome for both pediatric patients and the one adult we described in this report.

Two Endotracheal Tubes in One Trachea with a Traumatic Injury.

BACKGROUND: Traumatic airway injuries often require improvising solutions to altered anatomy under strict time constraints. We describe here the use of two endotracheal tubes simultaneously in the trachea to facilitate securing an airway which has been severely compromised by a self-inflicted wound to the trachea. Case Presentation: A 71-year-old male presented with a self-inflicted incision to his neck, cutting deep into the trachea itself. An endotracheal tube was emergently placed through the self-inflicted hole in the trachea in the ED. The patient was bleeding profusely, severely somnolent, and desaturating upon arrival to the operating room. Preservation of the tenuous airway was a priority while seeking to establish a more secure one. A video laryngoscope was used to gain a wide view of the posterior oropharynx and assist with oral intubation using a fiberoptic scope loaded with a second endotracheal tube. The initial tube's cuff was deflated as the second tube was advanced over the fiberoptic scope, thereby securing the airway while a completion tracheostomy was performed. CONCLUSIONS: Direct penetrating airway trauma may necessitate early, albeit less secure, intubations though the neck wounds prior to operating room arrival. The conundrum is weighing the risk of losing a temporary airway while attempting to establish a more secure airway. Here, we demonstrate the versatility of common anesthesia tools such as a video laryngoscope and a fiberoptic bronchoscope and the welcome discovery of the trachea's ability to accommodate two endotracheal tubes simultaneously so as to ensure a patent airway at all points throughout resuscitation.

Anterior segment optical coherence tomography and in vivo confocal microscopy in cases of mucopolysaccharidosis.

PURPOSE: To report anterior segment evaluation in patients with mucopolysaccharidosis 1 using anterior segment optical coherence tomography and in vivo confocal microscopy. OBSERVATIONS: Case 1 involved a 26-year-old man with mucopolysaccharidosis 1 Hurler-Scheie syndrome who presented with a corrected distance visual acuity of 20/67 in the right eye and 20/50 in the left eye because of bilateral corneal opacification. He had undergone bone marrow transplantation at the age of 7 years. Anterior segment optical coherence tomography revealed a very narrow anterior chamber angle; a flat, thickened cornea; and astigmatism with the rule. Confocal microscopy demonstrated hyper-refractive deposits in the corneal epithelial basal layer, decreased subepithelial nerves, and depletion of keratocytes in the anterior and posterior stroma. Endothelial cells were not visible.Case 2 involved an 18-year-old woman who presented with increased corneal opacity in both eyes. Her medical history included mucopolysaccharidosis 1 Hurler syndrome and bone marrow transplantation at the age of 2 years. Her corrected distance visual acuity could not be measured because of severe intellectual disability. Anterior segment optical coherence tomography demonstrated a flat and thick cornea. CONCLUSIONS AND IMPORTANCE: Anterior segment optical coherence tomography can provide detailed morphological information for the anterior segment in patients with severe corneal opacification associated with mucopolysaccharidosis 1. Moreover, in vivo confocal microscopy facilitates the detailed observation of corneal cellular changes. Thus, these tools can provide data that would contribute to an increased understanding of corneal changes in patients with mucopolysaccharidosis 1.

Characterization of the Progression Pattern in Retinopathy of Prematurity Subtypes.

PURPOSE: To evaluate differences in the progression pattern among subtypes of retinopathy of prematurity (ROP). DESIGN: Retrospective cohort study. PARTICIPANTS: Premature infants screened for ROP. METHODS: Medical records of 578 premature infants who were screened at the neonatal intensive care unit from September 2009 through March 2016 were reviewed. We matched for the number of patients, gestational age at birth, and postmenstrual age at the first examination between infants with spontaneously regressed ROP and those with treated ROP. A total of 133 premature infants who were born before 27 weeks' gestation were included. MAIN OUTCOME MEASURES: The mean age at onset of any ROP and the duration from the initial examination to onset were compared between infants with regressed ROP and those with treated ROP. The mean age at treatment and the duration from onset to treatment were compared between infants with type 1 ROP and those with aggressive posterior ROP (AP-ROP). Data were analyzed for 1 randomly selected eye for each infant. RESULTS: Of 133 premature infants with any ROP, 67 regressed spontaneously, 43 demonstrated type 1 ROP, and 23 demonstrated AP-ROP. Individual trajectories of ROP progression over time showed that AP-ROP progressed through the stages in a steep linear manner in most cases. In contrast, the type 1 ROP and regressed ROP developed in a slower, stepwise manner. CONCLUSIONS: In infants with ROP, the disease trajectories across ROP stages are different based on the ROP subtype, despite postmenstrual age at onset being comparable across subtypes. Our findings could be useful for managing follow-up screening.

Transient extremely shallow anterior chamber caused by ciliochoroidal detachment in a patient with Mycobacterium chelonae keratitis.

PURPOSE: To report a case of Mycobacterium chelonae keratitis that resulted in a transient reduction of anterior chamber depth. OBSERVATIONS: A 46-year-old man with keratoconus and reduced visual acuity (20/286) in his left eye presented with ciliary injection 16 months after femtosecond laser-assisted penetrating keratoplasty (PK). A slit-lamp examination showed a corneal ulcer with infiltrates and edema in both the host and graft between the 3 o'clock and 6 o'clock positions. Microbiologic tests confirmed the presence of M. chelonae. Topical arbekacin and moxifloxacin, erythromycin/colistin ointment, and oral clarithromycin were prescribed. We monitored anterior chamber depth by anterior segment optical coherence tomography (AS-OCT) throughout the recovery period. The anterior chamber depth was normal before treatment, with an intraocular pressure (IOP) of 7 mmHg. Although ciliary injection and infiltrates were gradually resolved, slit-lamp examination and AS-OCT revealed an extreme reduction of anterior chamber depth without corneal perforation, 1 month after beginning treatment. The IOP was 5 mmHg, and ciliochoroidal detachment (CCD) was present. The anterior chamber increased with the resolution of CCD and keratitis. Although hypotony continued despite the resolution of CCD and keratitis, the IOP eventually recovered to ≥10 mmHg at 1 month after remission. Onset and resolution of transient reduction of anterior chamber depth presumably occurred by anterior rotation and recovery of the ciliary body, respectively. Subsequent PK triple surgery enabled visual recovery to 20/100. CONCLUSIONS AND IMPORTANCE: severe anterior segment inflammation due to infectious keratitis may cause CCD and subsequent reduction of anterior chamber depth due to anterior rotation. AS-OCT is a non-invasive and efficient tool for the evaluation of iridociliary structure and the anterior chamber in patients with infectious keratitis.

Retinal Microvasculature and Visual Acuity after Intravitreal Aflibercept in Diabetic Macular Edema: An Optical Coherence Tomography Angiography Study.

We investigated changes in retinal vascular area and the foveal avascular zone (FAZ) after intravitreal aflibercept in diabetic macular edema (DME) and the association of these changes with visual outcomes. The retinal vascular area in the superficial capillary plexus (SCP) and the deep capillary plexus (DCP) and the FAZ area were measured using optical coherence tomography angiography (OCTA) in 23 eyes of 23 patients with DME, before and after intravitreal aflibercept. Overall, there was no significant change in retinal vascular area or FAZ. Better BCVA after treatment was significantly associated with larger retinal vascular area in the SCP and the DCP, both at baseline (R2 = 0.512, P < 0.001 and R2 = 0.361, P = 0.002, respectively) and after intravitreal aflibercept (R2 = 0.717, P < 0.001 and R2 = 0.618, P < 0.001, respectively). MAs were observed in the DCP in 20 eyes (87%), but only detected in four eyes (17%) in the SCP before treatment. The number of eyes with MAs in the DCP significantly decreased to 13 (57%) after treatment (P = 0.049). The persistence of DME was associated with persistent MAs (P = 0.019) and less visual gain (P = 0.031) following treatment. Thus, preserving retinal perfusion and the resolution of MAs are associated with better vision and resolution of the DME after intravitreal aflibercept.

Diagnostic evaluation of optical coherence tomography angiography and fundus autofluorescence in bilateral diffuse uveal melanocytic proliferation.

PURPOSE: To demonstrate the utility of optical coherence tomography angiography (OCTA) in visualizing the choroidal vasculature in bilateral diffuse uveal melanocytic proliferation (BDUMP), so as to elucidate pathophysiology and also aid in diagnosis. Additionally, to recommend autofluorescence (AF) over traditional angiography for purposes of noninvasive diagnosis. OBSERVATIONS: Three BDUMP cases are examined using AF, and two are examined using OCTA. Additionally, the cases vary in etiology and include a case with iris cysts, which we believe to have only been recorded once before in scientific literature, steroids were successfully used to treat two cases and anti-tumor drugs were used to treat the third case. OCTA revealed altered choroidal vasculature in the two cases tested, and AF was successfully used to diagnose all three cases regardless of etiology. CONCLUSIONS AND IMPORTANCE: We believe the OCTA findings are potentially elucidative regarding the pathophysiology at the choroidal layer, where BDUMP lesions primarily exist. Given the limited number of recorded BDUMP cases and relatively unknown pathophysiology, OCTA may prove to be invaluable in visualizing disease progression. Also we were able to use AF to diagnose all three cases ranging from extremely rare iris cysts to a more conventional presentation, indicating its utility regardless of etiology.

Changes in Retinal Microvasculature and Visual Acuity After Antivascular Endothelial Growth Factor Therapy in Retinal Vein Occlusion.

Purpose: To investigate the changes in the retinal microvasculature during the course of anti-VEGF therapy in eyes with macular edema due to retinal vein occlusion (RVO) and their association with visual outcomes. Methods: The vessel density (VD) and foveal avascular zone (FAZ) area in the superficial capillary plexus (SCP) and deep capillary plexus (DCP) were quantitatively measured by optical coherence tomography angiography (OCTA) in 48 consecutive eyes with RVO before and 1, 3, 6, 9, and 12 months after anti-VEGF therapy. Anti-VEGF therapy was performed either with ranibizumab or aflibercept following a pro re nata (PRN) regimen. The correlation between post-treatment best-corrected visual acuity (BCVA) and changes in the retinal microvasculature evaluated by OCTA were assessed. Results: The BCVA improved significantly at 12 months (P < 0.001). Better BCVA at 12 months was significantly associated with a better VD in the SCP and DCP both at baseline (R2 = 0.524, P < 0.001 and R2 = 0.457, P < 0.001, respectively) and at 12 months (R2 = 0.521, P < 0.001 and R2 = 0.662, P < 0.001, respectively). Overall, both VD and FAZ did not change significantly during the 12 months. However, the progression of nonperfusion was observed in the SCP in 6 (13%) eyes and in the DCP in 10 (21%) eyes. The number of macular edema recurrence was significantly associated with a decrease in the VD (P = 0.006 [SCP] and P < 0.001 [DCP]) and less visual gain (P = 0.02) after treatment. Conclusions: Anti-VEGF therapy maintains retinal perfusion in most patients with RVO. Preserving retinal perfusion is crucial for better visual outcomes.

Novel PAX6 mutation reported in an aniridia patient.

An aniridia patient was found to have a novel PAX6 mutation. A genetic duplication within PAX6, which caused a frameshift mutation, ultimately created a nonsense stop codon and premature truncation of the protein. Consequently, the patient presented with a clouded cornea as a result of partial limbal stem cell deficiency, foveal hypoplasia, nystagmus and a pale, cupped optic disc caused by glaucoma.