bluuebunny/crossref_metadata_2025_split huggingface dataset:
Sudden monocular vision loss after sneezing as captured on widefield swept source optical coherence tomography-angiography
Authors: Moon Jade Y. , Garg Itika , Miller John B. , Ludwig Cassie A. 
Published: February 2023

Abstract

Purpose:
We report an unusual case of likely combined retinal artery occlusion and retinal vein occlusion following a sneeze. Widefield swept source optical coherence tomography-angiography (WF-SS-OCTA) was used to analyze retinal changes at one-month follow-up.

Methods:
The patient reported visual symptoms in his left eye immediately after sneezing five times in a row. Fundus examination showed an embolus along the superior arcade. Stroke workup revealed moderate carotid artery stenosis and a subtherapeutic INR. At one-month follow-up, fundus photography and SS-OCTA were obtained.

Results:
Fundus photography showed superior retinal whitening and arteriole attenuation, an inferior perifoveal cotton wool spot, and inferotemporal intraretinal hemorrhages. SS-OCTA showed loss of the deep capillary plexus superiorly and inferotemporally, most consistent with combined retinal artery and retinal vein occlusion.

Conclusion:
We report the first case of sneeze-induced combined retinal artery and retinal vein occlusion. The retina is a uniquely accessible window that provides insight into human physiology, and SS-OCTA is a powerful tool that can give further insight into the pathophysiology of an occlusion.
Novel retinal findings in a patient with Autosomal recessive Cutis Laxa type 2A
Authors: Abdullah Mohammad , Alabduljalil Talal 
Published: February 2023

Abstract

Purpose:
To report a case of Autosomal recessive Cutis Laxa type 2A with novel retinal findings

Methods:
Case Report

Results:
22-year-old female patient presented with a longstanding history of reduced visual acuity in her right eye. She has generalized redundant skin, downslanting of palpebral fissures, and long philtrum. Ophthalmic examination showed ptosis in her right eye and visual acuity of 20/2000 in the right eye and 20/30p in the left eye. Funduscopic examination showed a round macular scar lesion in the right eye macula and a chorioretinal scar superonasally in the left eye. Multimodal imaging showed macular atrophy in the right eye with speckled hypoautoflorescence of the described lesions. Genetic testing showed a homozygous splice acceptor variant of the ATP6V0A2 gene.

Conclusions:
The natural history of the presented pigmentary lesions is not known and further follow up is needed to assess any progressive nature. Our case adds to the variability of ophthalmic manifestations reported in ARCL2A and therefore to the importance of regular ophthalmic surveillance in patients with Cutis Laxa.
Recurrent optic disc pit maculopathy due to Valsalva's maneuver
Authors: Reis Cyrino Francyne Veiga , Guzzi Marques Joao Pedro , Jorge Rodrigo 
Published: February 2023

Abstract

Purpose:
We report a patient with recurrent optic disc pit maculopathy after Valsalva’s maneuver and discuss its pathophysiology. We also hypothesize the role of Valsalva’s maneuver in its genesis.

Method:
Case report of one patient, male, 12 years old.

Results:
Serous retinal detachment may occur in association with the optic disc pit, a developmental anomaly of the optic nerve head. Histopathologically, it consists of a dysplastic retina herniation into a pocket extending posteriorly through a defect in the lamina cribrosa into the subarachnoid space. These three different compartments – intraocular space, optic nerve head and subarachnoid space – and the dynamic interactions among them should be understood as the key factors for the occurrence of optic disc pit-related serous retinal detachment.

Conclusion:
Based on the possibility that serous retinal detachment secondary to the ODP could be related to the Valsalva's maneuver, we strongly recommend that patients with ODP be advised to avoid intense physical or work activities that may increase abdominal, thoracic and cerebral pressure, and to refrain from playing wind instruments in order to avoid pre, per and post treatment intercurrences.
Bilateral diffuse uveal melanocytic proliferation mistaken for nivolumab-induced VKH-like syndrome
Authors: Dallinga Marchien Geesje , Bolhuis Karen , Bins Adriaan , de Hoog Joeri 
Published: February 2023

Abstract

Purpose:
To describe a case of bilateral diffuse melanocytic proliferation (BDUMP) that was mistaken for nivolumab-induced Vogt-Koyanagi-Harada-like syndrome.

Methods:
We present the case of a 58-year-old Caucasian male with metastatic renal clear cell carcinoma for which he was palliatively treated with intravenous nivolumab immunotherapy. Patient developed subacute onset of blurry vision and grey spots in the visual fields of both eyes, macular subretinal fluid, thickening of the RPE and swollen optic nerve heads. Differential diagnosis included nivolumab-induced Vogt-Koyanagi-Harada-disease-like syndrome (nVKH) and patient was initially treated with steroids, which gave no improvement. Investigation showed the development of dark-grey patches in the peripheral retina of both eyes and cataract, which prompted re-evaluation of the diagnosis, deeming BDUMP most likely. The patient was successfully treated with plasmapheresis.

Discussion:
The initial presentation of the case was incorrectly diagnosed as nVKH. Upon repeat studies of the patients’ symptoms and imaging, we concluded we had missed signs of BDUMP.

Conclusion:
The diagnosis BDUMP was missed in the first evaluation. We present this case to discuss the similarities and differences between this disease and nVKH. More importantly, we want to highlight that re-evaluation of the diagnosis upon worsening of a disease was key in this unusual case.
Rate of Endothelial Cell Loss and Graft Survival in Descemet Membrane Endothelial Keratoplasty in Eyes Requiring a Rebubble
Authors: Tapley Jeffrey L. , Hill Jordan R. , Bauer Alex J. , Straiko Megan M. W. , Straiko Michael D. , Terry Mark A. 
Published: February 2023

Purpose:
We aimed to compare the rate of 6-month endothelial cell loss (ECL) and 6-month graft survival in eyes that did not require a postoperative rebubble with eyes that did require a postoperative rebubble after Descemet membrane endothelial keratoplasty (DMEK) surgery.

Methods:
A consecutive series of DMEK surgeries performed from September 2013 to March 2020 was retrospectively analyzed. Eyes that did not require a rebubble for graft detachment were compared with eyes with 1 rebubble and eyes with 2 or more rebubbles for 6-month ECL and graft survival. A subanalysis of the rebubble rate for different indications for transplantation was also performed.

Results:
One thousand two hundred ninety-eight eyes were included in this study. The 6-month ECL for eyes with no rebubbles, 1 rebubble, and ≥2 rebubbles was 29.3% ± 16.2% (n = 793), 36.4% ± 18.6% (n = 97, P = 0.001), and 50.1% ± 19.6% (n = 28, P < 0.001), respectively. The 6-month graft survival rate for eyes with no rebubbles, 1 rebubble, and ≥2 rebubbles was 99.5%, 97.8% (P = 0.035), and 81.8% (P < 0.001), respectively. When compared to the rebubble rate for DMEK for Fuchs dystrophy (156/1165 eyes = 13.4%), the rebubble rates were statistically higher for DMEK for failed penetrating keratoplasty (28.5%, P = 0.021) and pseudophakic bullous keratopathy (28.0%, P = 0.036).

Conclusions:
Eyes undergoing any rebubble procedure in the postoperative period after DMEK have an increased risk of endothelial cell loss and graft failure at 6 months postoperative. DMEK in eyes for failed penetrating keratoplasty and failed DMEK had the highest rebubble rate, with the former reaching statistical significance.
Corneal and Whole-Eye Higher Order Aberrations Do Not Correlate With Ocular Residual Astigmatism in Prepresbyopic Refractive Surgery Candidates
Authors: Balparda Kepa , Maya-Naranjo María Isabel , Mesa-Mesa Sara , Herrera-Chalarca Tatiana 
Published: February 2023

Purpose:
Ocular residual astigmatism (ORA) is the proportion of manifest astigmatism that is not explained by anterior corneal astigmatism. The role of higher order aberrations (HOAs) in the level of ORA has not been profoundly studied. The purpose of this study was to evaluate the effect of different corneal and whole-eye HOAs on levels of ORA using a multivariate modeling approach.

Methods:
This is a retrospective analytical study including a sample of healthy refractive surgery candidates. One eye of every patient was randomly selected. A total of 294 eyes from an equal number of patients were included. Corneal and whole-eye HOAs were measured with a Pentacam AXL Wave, and subjective manifest refraction was taken. Astigmatism values were converted into power vectors and ORA was calculated. The Spearman rank-order correlation was initially used to explore correlation between HOA and ORA. All variables with a P value under 0.10 were included into a multiple linear regression model to explore this correlation adjusting for confounding variables.

Results:
The mean age was 28.81 ± 5.40 years. Simple bivariate correlation was significant for root mean square (RMS) total, RMS lower order aberrations, RMS HOAs, defocus, and vertical astigmatism. After being included into a multivariate regression model adjusting for confounding variables, the only variable that was significant was RMS total [F(3, 282) = 78.977; P < 0.001; adjusted R2 = 0.451]. For every μm increase in corneal RMS total, ORA increases by 0.135 diopters. About 45% of variability in ORA can be explained by corneal RMS total, corneal J0, and manifest J0. No individual HOA correlated with ORA in the multivariate regression model.

Conclusions:
ORA increases with general corneal irregularity, especially regular astigmatism. Irregular astigmatism expressed by corneal and whole-eye HOAs does not seem to be a significant contributor of ORA in healthy refractive surgery candidates.
Anthropometric Measures and Their Relationship to Steep Cornea in the United States Population
Authors: Valluru Girish , Henick Daniel , Klawe Janek , Liu Bian , Pasquale Louis R. , Ahmad Sumayya 
Published: February 2023

Purpose:
The aim of this study was to determine the relationship between anthropometric measures and steep cornea.

Methods:
Participants from the 1999 to 2008 US National Health and Nutrition Examination Survey visual examination were included (20,165 subjects). Cases had a mean dioptric power, averaged across the meridians, ≥ 48.0 diopters (n = 171). Separate multivariable models assessed body mass index, height, and weight in relation to steep cornea. Analyses included both overall and sex-stratified populations.

Results:
A relationship between BMI and steep cornea in the overall population was not detected (P for trend = 0.78). There was a strong inverse relationship between height (adjusted for weight) and steep cornea in the overall population (P for trend <0.0001) and in women (P for trend <0.0001). For every 1-inch increase in height, there was a 16% reduced odds of steep cornea in the overall population (OR, 0.84; 95% CI: 0.77–0.92). A relationship between weight and steep cornea was not detected in the overall population (P for trend = 0.79).

Conclusions:
Greater height was associated with a lower risk of steep cornea.
Etiology-Specific Comparison of the Long-Term Clinical Outcome of Repeat Deep Anterior Lamellar Keratoplasty for Optical Indications
Authors: Yasu-Mimura Ririko , Hirayama Masatoshi , Kasamatsu Hirotsugu , Yamaguchi Takefumi , Shimazaki Jun 
Published: February 2023

Purpose:
The aim of this study was to evaluate the etiology-specific clinical outcomes and complications of repeat deep anterior lamellar keratoplasty (DALK) after failed DALK.

Methods:
This retrospective case study included 32 eyes of 27 patients who underwent repeat DALK of 450 cases of DALK performed for optical indications between 1997 and 2013. The patients were divided into 4 etiology-specific subgroups (the corneal dystrophy, ocular surface disease, stromal scar, and others) or those with or without limbal stem cell deficiency (LSCD). The clinical outcomes evaluated were graft survival, best-corrected visual acuity, endothelial cell density, and complications.

Results:
The mean postoperative follow-up duration was 69.6 ± 54.8 months. The 1-, 3-, and 5-year overall graft survival rate were 76.7%, 57.5%, and 38.8% respectively. The graft survival rate was the highest in the corneal dystrophy group (P = 0.0014) and was significantly (P = 0.0010) higher in eyes without LSCD than in eyes with LSCD. There were no significant differences in the graft survival rates between the previous and current DALK groups. The postoperative best-corrected visual acuity of all subjects improved significantly. The postoperative endothelial cell density did not decrease after repeat DALK. There were no significant differences in the incidence of complications between patients with and without LSCD, except the incidence of persistent epithelial defects.

Conclusions:
Repeat DALK had favorable outcomes in all etiology-specific groups, whereas eyes with LSCD required careful assessment of the ocular surface to avoid graft failure due to persistent epithelial defects.
En Bloc Keratolimbal Allograft and Central Penetrating Keratoplasty: A Novel Surgical Technique in Severe Limbal Stem Cell Deficiency
Authors: Karimian Farid , Hassanpour Kiana 
Published: February 2023

Purpose:
The purpose of this study was to present a novel surgical technique combining 360-degree keratolimbal allograft (KLAL) and simultaneous central keratoplasty termed en bloc KLAL with the central penetrating keratoplasty (PKP) performed in those cases with total limbal stem cell deficiency (LSCD) and corneal scars.

Methods:
Nine eyes of 9 patients underwent en bloc KLAL and central PKP between 2014 and 2016. All patients had bilateral total LSCD with total corneal opacity due to different etiologies. The exclusion criteria were previous limbal stem cell transplantation and the presence of active and uncontrolled ocular surface inflammation. The same donor globe was used for harvesting 360-degree KLAL and central PKP. The 1-piece integrity of the KLAL and PKP graft was preserved during the described technique. All patients received modified immunosuppressive regimens compatible with the Cincinnati solid organ transplantation protocol.

Results:
The average age of patients was 58.6 ± 18.6 years. The diagnosis was mustard gas keratopathy in 6, herpes simplex keratitis in 1, and severe acid chemical burn in 2 patients. Seven patients were male. An integrated ocular surface without epitheliopathy and a clear cornea was achieved in 8 patients (88.8%) with an average of 6.5 years in follow-up. The average best-corrected visual acuity was 1.89 ± 0.18 (20/1600) preoperatively which improved to 1.02 ± 0.64 (20/200) logMAR in the postoperative period. Endothelial immune rejection episodes were observed in 3 patients. KLAL rejection was not observed in any patient. One patient required repeat PKP due to corneal graft failure.

Conclusions:
En bloc 360-degree KLAL and central PKP could simultaneously be performed in patients with total LSCD and corneal opacification.
Investigating the Link Between Visual Quality and Vision-Related Quality of Life in Patients With Keratoconus
Authors: Schümmer Laura , Kreps Elke O. , Pesudovs Konrad , Berwouts Jesse , Estramil Natalia , Mathysen Danny G. P. , Rozema Jos J. 
Published: February 2023

Purpose:
The purpose of the study was to investigate the optical and visual determinants of vision-related quality of life (VR-QoL) in patients with keratoconus.

Methods:
A cross-sectional case–control study was conducted at the Department of Ophthalmology, Antwerp University Hospital, Belgium. Patients previously diagnosed with keratoconus and healthy emmetropic or ametropic volunteers were included. Patients younger than 18 years, with ametropia of more than ±10 D, or with a history of corneal surgery or relevant ocular comorbidity limiting visual acuity were excluded. Assessment included autorefraction, high-contrast visual acuity testing, corneal imaging, intraocular straylight analysis, contrast sensitivity, aberrometry, and the National Eye Institute Visual Functioning Questionnaire (NEI VFQ-25). The Rasch-modified visual functioning scale (VFS) and socio-emotional scale were used to quantify VR-QoL. Stepwise linear regression was used to investigate the association between the clinical variables and VR-QoL.

Results:
Seventy-seven patients with keratoconus (77 pairs of eyes) and 77 age-matched and sex-matched controls were included in the study. The scores on the VFS and the SES were significantly lower in patients with keratoconus compared with controls (P < 0.001). Higher-order and lower-order aberrations, high-contrast visual acuity, and contrast sensitivity were poorer in patients with keratoconus (P < 0.001). Bivariate analyses showed that spectacle-corrected high-contrast visual acuity, higher spatial frequency contrast sensitivity, and higher-order aberration metrics were strong predictors of SES (P < 0.001) and of VFS (P < 0.001). Higher-order aberration of the worse eye was the strongest predictor for both SES (b = 0.310, P < 0.001) and VFS (b = 0.638, P < 0.001) on stepwise regression. Contrast sensitivity was not included in the stepwise regression because of insufficient data in the keratoconus group (33/77 patients; 42.9%).

Conclusions:
Both higher and lower aberration showed a strong correlation with VR-QoL, surpassing high-contrast visual acuity. These findings underline the importance of visual quality measures within keratoconus research and clinical care.
Recurrence of Guttae and Endothelial Dysfunction After Successful Descemet Stripping Only in Fuchs Dystrophy
Authors: Kaufman Aaron R. , Bal Sila , Boakye Jeffrey , Jurkunas Ula V. 
Published: February 2023

Purpose:
There are limited data about long-term durability of endothelial rejuvenation after Descemet stripping only (DSO). This study reports a case of bilaterally recurrent endothelial dysfunction and guttae formation after initially successful DSO in combination with cataract extraction (DSO-CE).

Methods:
This is a retrospective case report. A 49-year-old man with Fuchs endothelial corneal dystrophy with bilateral visually significant endothelial guttae (predominantly confluent centrally) and concomitant cataract underwent DSO-CE bilaterally. Postoperative course to long-term outcome at 6 years was analyzed.

Results:
Baseline central corneal thickness (CCT) was 568 μm in OD and 582 μm in OS. Preoperatively, both eyes had no countable central endothelial cells but good peripheral endothelial mosaic. In both eyes, the cornea clinically cleared at approximately 1 month postoperatively after DSO-CE. In short-term follow-up (OD postoperative month 6 and OS postoperative month 3), CCT was 556 μm in OD and 561 μm in OS and central endothelial cell density was 1352 cells/mm2 in OD and 880 cells/mm2 in OS. The patient returned to our center in postoperative year 6 OU. At this time, OU had interval formation of guttae within the descemetorhexis, with increased CCT (OD 631 μm and OS 609 μm) and decreased central endothelial cell density (OD 728 cells/mm2 and OS 609 cells/mm2).

Conclusions:
After DSO, progressive endothelial dysfunction with new guttae formation can occur within the descemetorhexis region of repopulated endothelium. Larger analyses with longer follow-up are needed to better characterize long-term outcomes of DSO.
Refractory Keratolimbal Allograft Rejection in Autoimmune Polyglandular Syndrome–Associated Keratopathy Treated With Intravenous Immunoglobulin
Authors: Thakur Shambhawi , Dandar Rachel , Restaino Irene G. , Cheung Albert Y. 
Published: February 2023

Purpose:
The aim of this study was to describe the use of intravenous immunoglobulin (IVIG) in the management of a 20-year-old woman with autoimmune polyglandular syndrome–associated keratopathy who developed acute transplant rejection after keratolimbal allograft (KLAL) surgery.

Case:
Nine weeks after KLAL surgery, a 20-year-old woman with autoimmune polyglandular syndrome–related limbal stem cell deficiency presented with graft injection, hemorrhage, and an epithelial rejection line. This was concerning for acute rejection in the setting of triple-agent systemic immunosuppression (albeit nonadherence at times). There was initial reversal of the rejection process with a sub-Tenon's injection of triamcinolone, frequent topical corticosteroids, increase in oral prednisone, and optimization of systemic immunosuppression medications; however, recurrence of the epithelial rejection line and symptoms were noted whenever the prednisone dose was tapered. This was accompanied by ocular surface decompensation (late staining, neovascularization, and persistent epithelial defects). She was found to have weakly positive HLA Class 1 antibodies. The patient was treated with a pulsed corticosteroid infusion and 2 monthly IVIG infusions. This led to resolution of the acute rejection. However, there was a subsequent rejection episode 4 months later after tapering the prednisone. Monthly IVIG for 6 more months led to final resolution with successful prednisone tapering and no further rejection.

Conclusions:
Treatment with prolonged IVIG showed better improvement in a case of acute rejection refractory to traditional treatments, especially in the setting of HLA antibodies. The case demonstrates that close follow-up with a corneal specialist and collaboration with a transplant specialist is important to monitor for postoperative KLAL rejection.
Allogeneic Ocular Surface Stem Cell Transplantation Outcomes With Decreased or No Systemic Immunosuppression in the Elderly
Authors: Cheung Albert Y. , Jeffrey Joseph H. , Govil Amit , Kinosz Elizabeth , Sarnicola Enrica , Denny Matthew R. , Reinisch Cameron B. , Holland Edward J. 
Published: February 2023

Purpose:
The aim of this study was to report outcomes after allogeneic ocular surface stem cell transplantation (OSST) for limbal stem cell deficiency in the setting of decreased or no systemic immunosuppression (SI) in the elderly.

Methods:
A retrospective chart review was performed of all eyes that underwent OSST for limbal stem cell deficiency between 2005 and 2020 at CVP Physicians. Inclusion criteria included patients who were (1) at least 70 years at the time of (2) allogeneic OSST. Postoperative SI regimens were assessed. Outcome measures included improvement in visual acuity, ocular surface stability, and adverse effects.

Results:
There were 14 eyes of 14 patients that met the inclusion criteria with mean follow-up of 3.0 (range 0.4–7.0) years. SI was run at a lower level for 6 patients, and 8 patients did not receive any SI. Nine eyes underwent keratolimbal allograft, 1 had a living-related conjunctival limbal allograft, and 4 had combined OSST. Most eyes (85.7%) attained improvement in visual acuity during their follow-up. At the last follow-up, 57.1% maintained a stable ocular surface. Six eyes developed acute rejection or late failure. Minimal adverse events were noted.

Conclusions:
Elderly patients administered less or no SI exhibit overall favorable outcomes after allogeneic OSST. Although not significantly different, surface stability and duration of improved vision was greater with low SI. No SI may be an option that still achieves improved vision in a high proportion for at least part of their follow-up. Decreasing SI after OSST in this population can improve quality of life while minimizing adverse effects.