Non-syndromic OTX2-associated pattern dystrophy: a 10-year multimodal imaging study.

PURPOSE: To report novel multimodal imaging features and long-term follow-up of Orthodenticle Homeobox 2 (OTX2)-associated pattern Gdystrophy. METHODS: A 14-year-old boy referred with glaucoma suspect and macular pigmentation underwent fundus autofluorescence imaging, optical coherence tomography, fluorescein and indocyanine green angiography, visual field test, microperimetry and electrophysiology over a ten-year period. Next-generation sequencing panel identified a de novo heterozygous likely pathogenic OTX2 variant, c.259G>A, [p.(Glu87Lys)]. RESULTS: Visual acuity was 20/40 OD and 20/30 OS. Examination showed bilateral enlarged optic nerve heads and increased disc cupping, multiple cilioretinal arteries, a pigmentary maculopathy with stellate-shaped region of hypoautofluorescence, shallow serous macular detachment, subretinal deposits and temporal avascular retina. Angiography showed no source of leakage and absence of retinal neovascularisation despite extensive peripheral non perfusion. Electrophysiological assessments demonstrated mild progressive rod and cone pathway abnormalities, reduced light-adapted b:a ratio, and reduced Arden ratio on electro-oculogram. Ten-year follow-up confirmed a stable disease course despite persistent submacular fluid. There was no associated pituitary structural abnormality or dysfunction. CONCLUSIONS: This case study contributes to further understanding of OTX2-associated pattern dystrophy, highlighting its stability over 10 years. Further investigation into inter-individual and intrafamilial variability is warranted.

A planetary collision afterglow and transit of the resultant debris cloud.

Planets grow in rotating disks of dust and gas around forming stars, some of which can subsequently collide in giant impacts after the gas component is removed from the disk1-3. Monitoring programmes with the warm Spitzer mission have recorded substantial and rapid changes in mid-infrared output for several stars, interpreted as variations in the surface area of warm, dusty material ejected by planetary-scale collisions and heated by the central star: for example, NGC 2354-ID8 (refs. 4,5), HD 166191 (ref. 6) and V488 Persei7. Here we report combined observations of the young (about 300 million years old), solar-like star ASASSN-21qj: an infrared brightening consistent with a blackbody temperature of 1,000 Kelvin and a luminosity that is 4 percent that of the star lasting for about 1,000 days, partially overlapping in time with a complex and deep, wavelength-dependent optical eclipse that lasted for about 500 days. The optical eclipse started 2.5 years after the infrared brightening, implying an orbital period of at least that duration. These observations are consistent with a collision between two exoplanets of several to tens of Earth masses at 2-16 astronomical units from the central star. Such an impact produces a hot, highly extended post-impact remnant with sufficient luminosity to explain the infrared observations. Transit of the impact debris, sheared by orbital motion into a long cloud, causes the subsequent complex eclipse of the host star.

Giant pars plana cysts and a diagnostic approach to peripheral retinal elevation with the assistance of multimodal imaging.

A man in his 50s was referred for a suspected superotemporal retinal tear in the right eye. Fundus examination showed multiple areas of elevated retina temporally in both eyes which were immobile and did not reappose with scleral depression. No retinal breaks were present. Ultra-widefield colour fundus photography with Optos captured these areas of elevated retina in both eyes temporally, which on fundus autofluorescence where hypoautofluorescent with no leading hyperautofluorescent edge. On ultrasound biomicroscopy, an immobile lesion with a single hyperechoic convexity towards the vitreous body was noted. The patient was diagnosed with bilateral giant pars plana cysts which were managed conservatively.The multimodal imaging in our case is its distinguishing feature which can be used to help ensure accurate diagnosis when one is presented with an area of peripheral retina elevation.

Direct intralesional n-butyl-cyanoacrylate embolization for intractable vertebral hemangioma bleeding: a salvage technique. Illustrative case.

BACKGROUND: Surgical resection of vertebral hemangiomas in the setting of cord compression can be technically difficult and has the potential for life-threatening hemorrhage. The authors report a case of intraoperative direct intralesional n-butyl-cyanoacrylate embolization for intractable vertebral hemangioma bleeding. OBSERVATIONS: A 53-year-old woman presented for repeat surgery of a residual vertebral hemangioma after a previous debulking, laminectomy, and fixation that were without problems with bleeding. The second surgery was complicated by intractable hemorrhage. Bleeding was controlled with direct intralesional n-butyl-cyanoacrylate embolization after fluoroscopy without accompanying endovascular embolization. LESSONS: Aggressive vertebral hemangiomas should ideally be managed in centers where transarterial embolization is available. If such centers are not available or there is still intractable intraoperative bleeding despite preoperative embolization, direct intralesional embolization may be considered as a potential salvage technique.

Exocomets size distribution in the [Formula: see text] Pictoris planetary system.

The star [Formula: see text] Pictoris harbors a young planetary system of about 20 million years old, which is characterized by the presence of a gaseous and dusty debris disk, at least two massive planets and many minor bodies. For more than thirty years, exocomets transiting the star have been detected using spectroscopy, probing the gaseous part of the cometary comas and tails. The detection of the dusty component of the tails can be performed through photometric observations of the transits. Since 2018, the Transiting Exoplanet Survey Satellite has observed [Formula: see text] Pic for a total of 156 days. Here we report an analysis of the TESS photometric data set with the identification of a total of 30 transits of exocomets. Our statistical analysis shows that the number of transiting exocomet events (N) as a function of the absorption depth (AD) in the light curve follows a power law in the form [Formula: see text], where [Formula: see text]. This distribution of absorption depth leads to a differential comet size distribution proportional to [Formula: see text], where [Formula: see text], showing a striking similarity to the size distribution of comets in the Solar system and the distribution of a collisionally relaxed population ([Formula: see text]).

Conjunctival amelanotic melanoma presenting as a multifocal pink lesion.

Conjunctival amelanotic malignant melanoma is a rare form of melanoma, which lacks visible pigment and is commonly located underneath the eyelids in the bulbar conjunctiva. In this report, we described a case of a Caucasian women in her 70s who presented with unilateral irritation and tenderness following cataract surgery. On eversion of the eyelid, two elevated pink lesions were noted. Tumour - Node - Metastasis staging with the American Joint Committee on Cancer staging system eighth edition was T3C and required multiple excisions and reconstruction procedures. This case exemplified the diagnostic pitfall of conjunctival amelanotic malignant melanoma, which is a potentially life-threatening disease and the importance of histopathology in the diagnostic process.

A wide-orbit giant planet in the high-mass b Centauri binary system.

Planet formation occurs around a wide range of stellar masses and stellar system architectures1. An improved understanding of the formation process can be achieved by studying it across the full parameter space, particularly towards the extremes. Earlier studies of planets in close-in orbits around high-mass stars have revealed an increase in giant planet frequency with increasing stellar mass2 until a turnover point at 1.9 solar masses (M⊙), above which the frequency rapidly decreases3. This could potentially imply that planet formation is impeded around more massive stars, and that giant planets around stars exceeding 3 M⊙ may be rare or non-existent. However, the methods used to detect planets in small orbits are insensitive to planets in wide orbits. Here we demonstrate the existence of a planet at 560 times the Sun-Earth distance from the 6- to 10-M⊙ binary b Centauri through direct imaging. The planet-to-star mass ratio of 0.10-0.17% is similar to the Jupiter-Sun ratio, but the separation of the detected planet is about 100 times wider than that of Jupiter. Our results show that planets can reside in much more massive stellar systems than what would be expected from extrapolation of previous results. The planet is unlikely to have formed in situ through the conventional core accretion mechanism4, but might have formed elsewhere and arrived to its present location through dynamical interactions, or might have formed via gravitational instability.

The 13CO-rich atmosphere of a young accreting super-Jupiter.

Isotope abundance ratios have an important role in astronomy and planetary sciences, providing insights into the origin and evolution of the Solar System, interstellar chemistry and stellar nucleosynthesis1,2. In contrast to deuterium/hydrogen ratios, carbon isotope ratios are found to be roughly constant (around 89) in the Solar System1,3, but do vary on galactic scales with a 12C/13C isotopologue ratio of around 68 in the current local interstellar medium4-6. In molecular clouds and protoplanetary disks, 12CO/13CO ratios can be altered by ice and gas partitioning7, low-temperature isotopic ion-exchange reactions8 and isotope-selective photodissociation9. Here we report observations of 13CO in the atmosphere of the young, accreting super-Jupiter TYC 8998-760-1 b, at a statistical significance of more than six sigma. Marginalizing over the planet's atmospheric temperature structure, chemical composition and spectral calibration uncertainties suggests a 12CO/13CO ratio of [Formula: see text](90% confidence), a substantial enrichment in 13C with respect to the terrestrial standard and the local interstellar value. As the current location of TYC 8998-760-1 b at greater than or equal to 160 astronomical units is far beyond the CO snowline, we postulate that it accreted a substantial fraction of its carbon from ices enriched in 13C through fractionation.

Impact of time-variant turbulence behavior on prediction for adaptive optics systems.

For high-contrast imaging systems, the time delay is one of the major limiting factors for the performance of the extreme adaptive optics (AO) sub-system and, in turn, the final contrast. The time delay is due to the finite time needed to measure the incoming disturbance and then apply the correction. By predicting the behavior of the atmospheric disturbance over the time delay we can in principle achieve a better AO performance. Atmospheric turbulence parameters, which determine wavefront phase fluctuations, have time-varying behavior. We present a stochastic model for wind speed and model time-variant atmospheric turbulence effects using varying wind speeds. We test a low-order, data-driven predictor, the linear minimum mean square error predictor, for a near-infrared AO system under varying conditions. Our results show varying wind can have a significant impact on the performance of wavefront prediction, preventing it from reaching optimal performance. The impact depends on the strength of wind fluctuations with the greatest loss in expected performance being for high wind speeds.

The transiting dust clumps in the evolved disc of the Sun-like UXor RZ Psc.

RZ Psc is a young Sun-like star, long associated with the UXor class of variable stars, which is partially or wholly dimmed by dust clumps several times each year. The system has a bright and variable infrared excess, which has been interpreted as evidence that the dimming events are the passage of asteroidal fragments in front of the host star. Here, we present a decade of optical photometry of RZ Psc and take a critical look at the asteroid belt interpretation. We show that the distribution of light curve gradients is non-uniform for deep events, which we interpret as possible evidence for an asteroidal fragment-like clump structure. However, the clumps are very likely seen above a high optical depth midplane, so the disc's bulk clumpiness is not revealed. While circumstantial evidence suggests an asteroid belt is more plausible than a gas-rich transition disc, the evolutionary status remains uncertain. We suggest that the rarity of Sun-like stars showing disc-related variability may arise because (i) any accretion streams are transparent and/or (ii) turbulence above the inner rim is normally shadowed by a flared outer disc.

Measuring individuality in habitat use across complex landscapes: approaches, constraints, and implications for assessing resource specialization.

Many mobile marine species are presumed to utilize a broad spectrum of habitats, but this seemingly generalist life history may arise from conspecifics specializing on distinct habitat alternatives to exploit foraging, resting/refuge, or reproductive opportunities. We acoustically tagged 34 red drum, and mapped sand, seagrass, marsh, or oyster (across discrete landscape contexts) use by each uniquely coded individual. Using 144,000 acoustic detections, we recorded differences in habitat use among red drum: proportional use of seagrass habitat ranged from 0 to 100%, and use of oyster-bottom types also varied among fish. WIC/TNW and IS metrics (previously applied vis-à-vis diet specialization) consistently indicated that a typical red drum overlapped >70% with population-level niche exploitation. Monte Carlo permutations showed these values were lower than expected had fish drawn from a common habitat-use distribution, but longitudinal comparisons did not provide evidence of temporally consistent individuality, suggesting that differences among individuals were plastic and not reflective of true specialization. Given the range of acoustic detections we captured (from tens to 1,000s per individual), which are substantially larger sample sizes than in many diet studies, we extended our findings by serially reducing or expanding our data in simulations to evaluate sample-size effects. We found that the results of null hypothesis testing for specialization were highly dependent on sample size, with thresholds in the relationship between sample size and associated P-values. These results highlight opportunities and potential caveats in exploring individuality in habitat use. More broadly, exploring individual specialization in fine-scale habitat use suggests that, for mobile marine species, movement behaviors over shorter (≤weeks), but not longer (≥months), timescales may serve as an underlying mechanism for other forms of resource specialization.

Fast & Furious focal-plane wavefront sensing.

We present two complementary algorithms suitable for using focal-plane measurements to control a wavefront corrector with an extremely high-spatial resolution. The algorithms use linear approximations to iteratively minimize the aberrations seen by the focal-plane camera. The first algorithm, Fast & Furious (FF), uses a weak-aberration assumption and pupil symmetries to achieve fast wavefront reconstruction. The second algorithm, an extension to FF, can deal with an arbitrary pupil shape; it uses a Gerchberg-Saxton (GS)-style error reduction to determine the pupil amplitudes. Simulations and experimental results are shown for a spatial-light modulator controlling the wavefront with a resolution of 170×170 pixels. The algorithms increase the Strehl ratio from ∼0.75 to 0.98-0.99, and the intensity of the scattered light is reduced throughout the whole recorded image of 320×320 pixels. The remaining wavefront rms error is estimated to be ∼0.15 rad with FF and ∼0.10 rad with FF-GS.

Performance characterization of a broadband vector Apodizing Phase Plate coronagraph.

One of the main challenges for the direct imaging of planets around nearby stars is the suppression of the diffracted halo from the primary star. Coronagraphs are angular filters that suppress this diffracted halo. The Apodizing Phase Plate coronagraph modifies the pupil-plane phase with an anti-symmetric pattern to suppress diffraction over a 180 degree region from 2 to 7 λ/D and achieves a mean raw contrast of 10(-4) in this area, independent of the tip-tilt stability of the system. Current APP coronagraphs implemented using classical phase techniques are limited in bandwidth and suppression region geometry (i.e. only on one side of the star). In this paper, we introduce the vector-APP (vAPP) whose phase pattern is implemented through the vector phase imposed by the orientation of patterned liquid crystals. Beam-splitting according to circular polarization states produces two, complementary PSFs with dark holes on either side. We have developed a prototype vAPP that consists of a stack of three twisting liquid crystal layers to yield a bandwidth of 500 to 900 nm. We characterize the properties of this device using reconstructions of the pupil-plane pattern, and of the ensuing PSF structures. By imaging the pupil between crossed and parallel polarizers we reconstruct the fast axis pattern, transmission, and retardance of the vAPP, and use this as input for a PSF model. This model includes aberrations of the laboratory set-up, and matches the measured PSF, which shows a raw contrast of 10(-3.8) between 2 and 7 λ/D in a 135 degree wedge. The vAPP coronagraph is relatively easy to manufacture and can be implemented together with a broadband quarter-wave plate and Wollaston prism in a pupil wheel in high-contrast imaging instruments. The liquid crystal patterning technique permits the application of extreme phase patterns with deeper contrasts inside the dark holes, and the multilayer liquid crystal achromatization technique enables unprecedented spectral bandwidths for phase-manipulation coronagraphy.

Calibrating a high-resolution wavefront corrector with a static focal-plane camera.

We present a method to calibrate a high-resolution wavefront (WF)-correcting device with a single, static camera, located in the focal-plane; no moving of any component is needed. The method is based on a localized diversity and differential optical transfer functions to compute both the phase and amplitude in the pupil plane located upstream of the last imaging optics. An experiment with a spatial light modulator shows that the calibration is sufficient to robustly operate a focal-plane WF sensing algorithm controlling a WF corrector with 40,000 degrees of freedom. We estimate that the locations of identical WF corrector elements are determined with a spatial resolution of 0.3% compared to the pupil diameter.