AIM: To investigate the influence of unilateral congenital ptosis on the development of the eye and vision in children. METHODS: In this prospective observational study, 41 patients with unilateral congenital ptosis were enrolled (age range 3-15y). The blepharoptosis was divided into 3 subgroups according to the margin reflex distance-1 (MRD-1), including mild group (MRD-1≥2 mm), moderate group (0≤MRD-1<2 mm), and severe group (MRD-1<0 mm). The fellow eyes served as controls. All subjects underwent ocular examinations, including axial length, keratometry, and refractive error. RESULTS: The incidence of astigmatism (ptotic eyes: 58.5% vs fellow eyes: 24.4%, P=0.002) and magnitude of cylindrical power (ptotic eyes: -0.86±0.79 D vs fellow eyes: -0.43±0.63 D, P=0.003) differed significantly between the ptotic eyes and the fellow eyes. The spherical equivalent refraction (P=0.006), spherical power (P=0.01), cylindrical power (P=0.011), axial length-corneal radius (AL/CR) ratio (P=0.009), frequency of hyperopia (P=0.002) and astigmatism (P=0.004) were significantly different among the ptotic eye subgroups and the fellow eye group. In addition, in patients with congenital ptosis, the incidence of amblyopia is 43.9% and the incidence of anisometropia is 24.4%. More importantly, the ratio of AL/CR showed significantly positive correlation with the severity of ptosis (P=0.002). CONCLUSION: Congenital ptosis may lead to a delayed eyeball development in the aspect of AL/CR. The risk of amblyopia is also increased due to visual deprivation and aggravated anisometropia, particularly in severe ptosis case.
by
Elliot M Frohman;
Nicole R Villemarette-Pittman;
Esther Melamed;
Roberto Alejandro Cruz;
Reid Longmuir;
Thomas C Varkey;
Lawrence Steinman;
Scott S Zamvil;
Teresa C Frohman
The coronavirus disease 2019 (COVID-19) pandemic has produced a world-wide collapse of social and economic infrastructure, as well as constrained our freedom of movement. This respiratory tract infection is nefarious in how it targets the most distal and highly vulnerable aspect of the human bronchopulmonary tree, specifically, the delicate yet irreplaceable alveoli that are responsible for the loading of oxygen upon red cell hemoglobin for use by all of the body's tissues.
In most symptomatic individuals, the disease is a mild immune-mediated syndrome, with limited damage to the lung tissues. About 20% of those affected experience a disease course characterized by a cataclysmic set of immune activation responses that can culminate in the diffuse and irreversible obliteration of the distal alveoli, leading to a virtual collapse of the gas-exchange apparatus.
Here, in Part I of a duology on the characterization and potential treatment for COVID-19, we define severe COVID-19 as a consequence of the ability of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) to trigger what we now designate for the first time as a ‘Prolific Activation of a Network-Immune-Inflammatory Crisis’, or ‘PANIC’ Attack, in the alveolar tree. In Part II we describe an immunotherapeutic hypothesis worthy of the organization of a randomized clinical trial in order to ascertain whether a repurposed, generic, inexpensive, and widely available agent is capable of abolishing ‘PANIC’; thereby preventing or mitigating severe COVID-19, with monumental ramifications for world health, and the global pandemic that continues to threaten it.
by
Elliot M Frohman;
Nicole R Villemarette-Pittman;
Roberto A Cruz;
Reid Longmuir;
Vernon Rowe;
Elizabeth S Rowe;
Thomas C Varkey;
Lawrence Steinman;
Scott S Zamvil;
Teresa C Frohman
Here, in Part II of a duology on the characterization and potential treatment for COVID-19, we characterize the application of an innovative treatment regimen for the prevention of the transition from mild to severe COVID-19, as well as detail an intensive immunotherapy intervention hypothesis.
We propose as a putative randomized controlled trial that high-dose methotrexate with leucovorin (HDMTX-LR) rescue can abolish ‘PANIC’, thereby ‘left-shifting’ severe COVID-19 patients to the group majority of those infected with SARS-CoV-2, who are designated as having mild, even asymptomatic, disease. HDMTX-LR is endowed with broadly pleiotropic properties and is a repurposed, generic, inexpensive, and widely available agent which can be administered early in the course of severe COVID-19 thus rescuing the critical and irreplaceable gas-exchange alveoli.
Further, we describe a preventative treatment intervention regimen for those designated as having mild to moderate COVID-19 disease, but who exhibit features which herald the transition to the severe variant of this disease. Both of our proposed hypothesis-driven questions should be urgently subjected to rigorous assessment in the context of randomized controlled trials, in order to confirm or refute the contention that the approaches characterized herein, are in fact capable of exerting mitigating, if not abolishing, effects upon SARS-CoV-2 triggered ‘PANIC Attack’. Confirmation of our immunotherapy hypothesis would have far-reaching ramifications for the current pandemic, along with yielding invaluable lessons which could be leveraged to more effectively prepare for the next challenge to global health.
By 2040, one third of worldwide cases of age-related macular degeneration (AMD) will originate from Asia.1 Although both geographic atrophy (GA) and neovascular AMD (nAMD) can result in irreversible blindness, currently no treatment exists for GA. Thus, understanding the global epidemiologic characterists of GA can better guide allocations of health care resources and future research. To address the limited knowledge regarding the prevalence of GA in the Asian population, Rim et al2 (see page 1371) performed a cross-sectional meta-analysis of 22 population-based studies from the Asian Eye Epidemiology Consortium. A total of 97,213 individuals from 10 Asian countries were included.
Objectives
To evaluate the impact of the novel coronavirus pandemic on practice patterns, clinical behavior, personal health, and emotional/psychological concerns of rhinologists.
Methods
A 15-question survey was sent out to the American Rhinologic Society's (ARS) membership to determine the impact of COVID-19 during the crisis. Demographic factors and practice patterns were collected and evaluated.
Results
There were 224 total respondents out of 835 ARS members queried (26.8% response rate). Study queries were sent in April 2020. Notably, 17.8% reported illness in themselves or their staff and 74.4% noted a psychological/emotional impact. A plurality of rhinologists noted their practice volume and in-office procedure volume has become 20.0% and 0.0% of their prior volumes, respectively. In addition, 96.2% were noted to be using telemedicine in our subspecialty.
Conclusion
In addition to severely impacting volume and the perception of future decreases in patients and revenue, the COVID-19 pandemic has had a physical and emotional impact on rhinologists in ways that need to be further studied. These data include significantly novel and objective information. The COVID-19 crisis also reveals the important role of telemedicine in rhinology. Guidelines regarding personal protective equipment for in-office visits, nasal endoscopy, and other in-office and operating room procedures would be particularly helpful as future waves are expected.
To determine whether the fellow eye of children who have undergone unilateral cataract extraction in the first year of life are at increased risk of injury and vision loss, the 10.5-year data on 109 of 114 children enrolled in the Infant Aphakia Treatment Study were examined. Based on this limited data, it was estimated that the fellow eye is at greater risk of injury than the operated eye. Our data do not support the risk being higher in children with the worst vision in the treated eye.[Formula presented]
Purpose. To investigate the healing process and functional recovery of neuroretina after idiopathic macular hole surgery, as well as analyzing the influencing factors. Methods. Thirty-six eyes of 31 patients with full-thickness idiopathic macular hole (IMH) were enrolled in this retrospective study. All of them were operated using 23-gauge or 25-gauge vitrectomy with inner limiting membrane peeling and air tamponade. Spectral-domain optical coherence tomography was performed before surgery and after surgery to observe the structural changes of neuroretina. Results. Twenty eyes (55.56%) had the macular hole closed at 3 to 5 days after surgery (closed group), beginning from the inner retina based on OCT. Holes of 16 eyes (44.44%) remained unclosed and progressed to larger holes at 13 to 15 days (t = -2.811, P=0.013) after surgery (unclosed group). Compared with the eyes in the closed group, the eyes in the unclosed group had significantly larger hole diameter (t = -2.882, P=0.007). Postoperative BCVA was significantly improved in the closed group (t = 2.573, P=0.019) and not improved in the unclosed group (t = 0.606, P=0.554) at the 6-month follow-up. Conclusion. Full-thickness IMHs could achieve anatomic closure 3 to 5 days after surgery with first-step inner retina tissue bridging. Otherwise, they were not able to achieve hole closure and opened to larger holes about 2 weeks postoperatively. Macular hole diameter was an important factor affecting the healing of the holes. The delayed restoration of fovea detachment and ellipsoid area deficiency were responsible for poor vision outcomes after surgery.
by
Vedakumar Tatavarty;
Alejandro Torrado Pacheco;
Chelsea Groves Kuhnle;
Heather Lin;
Priya Koundinya;
Nathaniel J. Miska;
Keith B. Hengen;
Florence F. Wagner;
Stephen D. Van Hooser;
Gina G. Turrigiano
Mutations in Shank3 are strongly associated with autism spectrum disorders and neural circuit changes in several brain areas, but the cellular mechanisms that underlie these defects are not understood. Homeostatic forms of plasticity allow central circuits to maintain stable function during experience-dependent development, leading us to ask whether loss of Shank3 might impair homeostatic plasticity and circuit-level compensation to perturbations. We found that Shank3 loss in vitro abolished both synaptic scaling and intrinsic homeostatic plasticity, deficits that could be rescued by treatment with lithium. Further, Shank3 knockout severely compromised the in vivo ability of visual cortical circuits to recover from perturbations to sensory drive. Finally, lithium treatment ameliorated a repetitive self-grooming phenotype in Shank3 knockout mice. These findings demonstrate that Shank3 loss severely impairs the ability of central circuits to harness homeostatic mechanisms to compensate for perturbations in drive, which in turn may render them more vulnerable to such perturbations.
by
Shuo Zhang;
Paul Lyuboslavsky;
Jendayi Azeezah Dixon;
Micah A. Chrenek;
Jana T. Sellers;
Jessica M. Hamm;
P Michael Iuvone;
Christophe P. Ribelayga;
Zhijing Zhang;
Yun Z. Le
PURPOSE. The present study tested the hypothesis that connexin-36 (Cx36) and gap junctions between photoreceptor cells contribute to the circadian rhythm of the photopic electroretinogram (ERG) b-wave amplitude. METHODS. Cone-specific disruption of Cx36 was obtained in mice with a floxed Gjd2 gene and human red/green pigment promoter (HRGP)-driven Cre recombinase. Standard ERG, spectral-domain optical coherence tomography (SD-OCT) and histochemical methods were used. RESULTS. HRGPcreGjd2fl/fl mice had a selective reduction in Cx36 protein in the outer plexiform layer; no reduction in Cx36 was observed in the inner plexiform layer. Cx36 disruption had no effect on the number of cones, the thickness of the photoreceptor layer, or the scotopic ERG responses. However, there was a reduction of the photopic ERG circadian rhythm, with b-wave amplitudes in the day and the night locked in the daytime, light-adapted state. In HRGPcreGjd2+/+ and Gjd2fl/fl controls, the circadian rhythm of light-adapted ERG persisted, similar to that in wild type mice. CONCLUSIONS. Cx36 regulation contributes to the circadian rhythm of light-adapted ERG; in the absence of photoreceptor gap junctions, mice appear to be in a fully light-adapted state regardless of the time of day. The higher amplitudes and reduced circadian regulation of the b-wave of HRGPcreGjd2fl/fl mice may be due to increased synaptic strength at the cone to ON bipolar cell synapse due to electrotonic isolation of the terminals lacking gap junctions.
We illustrate the growing power of the BXD family of mice (recombinant inbred strains from a cross of C57BL/6J and DBA/2J mice) and companion bioinformatic tools to study complex genome-phenome relations related to glaucoma. Over the past 16 years, our group has integrated powerful murine resources and web-accessible tools to identify networks modulating visual system traits—from photoreceptors to the visual cortex. Recent studies focused on retinal ganglion cells and glaucoma risk factors, including intraocular pressure (IOP), central corneal thickness (CCT), and susceptibility of cellular stress. The BXD family was exploited to define key gene variants and then establish linkage to glaucoma in human cohorts. The power of this experimental approach to precision medicine is highlighted by recent studies that defined cadherin 11 (Cdh11) and a calcium channel (Cacna2d1) as genes modulating IOP, Pou6f2 as a genetic link between CCT and retinal ganglion cell (RGC) death, and Aldh7a1 as a gene that modulates the susceptibility of RGCs to death after elevated IOP. The role of three of these gene variants in glaucoma is discussed, along with the pathways activated in the disease process.