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A 10-year-old child had painful periorbital swelling in the left eye. It was diagnosed as preseptal cellulitis and treated with oral antibiotics. Three days later, the ocular condition worsened so the child was referred for further management. On examination, the child had a temperature of 102 °F. Ocular examination revealed proptosis, restricted ocular movements and a relative afferent pupillary defect in the left eye. Ocular examination of the right eye was normal. There was a history of recurrent episodes of cold in the past. CT scan orbit and sinuses revealed signs of orbital cellulitis with sinusitis on the left side. The child was treated with parenteral antibiotics and endoscopic sinus surgery. A child presenting with unilateral periorbital swelling needs to be thoroughly evaluated. It is important to differentiate orbital cellulitis from preseptal cellulitis. Orbital cellulitis is an emergency and delay in diagnosis can lead to vision and life-threatening intracranial complications.

PURPOSE To determine differences in cycloplegic vs. non-cycloplegic refractive error and factors associated with these differences in Chinese school students. METHOD In this cross-sectional school-based study, refractive error was measured in school students using a NIDEK autorefractor before and after administration of 0.5% tropicamide. Spherical equivalent (SER) in diopters (D) was calculated as sphere plus half cylinder. SER differences before vs. after cycloplegia were evaluated using mean, standard deviation (SD), 95% limits of agreement. Univariable and multivariable regression models were used to determine factors associated with SER differences. RESULTS Among 3604 students, 3450 (95.7%) provided data for analysis. Mean age (SD) was 9.7 (3.6) years. The mean SER (SD) was -1.12 (1.97) D before cycloplegia, and -0.20 (2.19) D after cycloplegia, with a mean difference of 0.92 D (95% limits of agreement: -0.93 to 2.78 D). Among 196 eyes with non-cycloplegic SER -6.0 D or worse (e.g., met high myopia definition), 71.4% had cycloplegic SER -6.0 D or worse, and among 3607 eyes with non-cycloplegic SER -0.5 D or worse (e.g., met myopia definition), 62.1% eyes had cycloplegic SER -0.5 D or worse. Cycloplegic SER was more correlated with axial length than non-cycloplegic SER (Pearson r = 0.82 vs. 0.72, p

Objectives: To present and validate the novel grading system for objective classification of corectopia.Subjects and Methods: We evaluated 28 eyes of 28 patients with or without corectopia and validated the grading and classification system for corectopia according to three major criteria: (i) direction, (ii) extent, and (iii) alteration of mydriasis. Intraclass correlation coefficient (ICC) and inter-rater agreement between 7 inexperienced and 1 experienced ophthalmologist against a golden standard (GS) were calculated.Results: The ICC for the 7 inexperienced ophthalmologists regarding the grading of direction and centration of the pupil was 0.83 (95% confidence interval (CI), 0.74 to 0.90; p

Summary Background The clinical presentation of reduced pupillary responses in orbital apex syndrome is currently not well understood clinically. The pupillary light reflex (PLR) is determined using dynamic pupillometry. Case details A newly diagnosed 35-year-old diabetic female patient had ocular symptoms including orbital pain and ptosis, but no visual loss. Cranial nerve autonomic impairment was identified with dynamic pupillometry and compared with cardiac autonomic activity using heart rate variability (HRV). Results PLR showed that pupil size and response were severely affected, with a small resting pupil size, and minimal response to a bright white light flash was seen, due to decreased sympathetic activity. However, HRV showed normal cardiac sympathetic activity. Conclusion These findings suggest that orbital apex syndrome may lead to changes in pupil size and response due to compression of sympathetic fibers at the apex of the orbit. Quantitative PLR measurements help to reveal relevant information on anatomical reflex pathways.

Purpose: To investigate the clinical and histopathological features of congenital fibrovascular pupillary membrane (CFPM) in Chinese patients. Methods: This retrospective study reviewed CFPM cases treated at Beijing Children's Hospital. The clinical manifestations, approaches of treatment, outcomes, and histopathological findings were collected and analyzed. Results: A total of 33 patients with CFPM were reviewed. All patients had unilateral eye involvement. A total of 21 eyes (63.64%) had a white membrane that partially covered the pupil and 12 eyes (36.36%) had a membrane that completely covered the pupil. Of the 12 eyes with a complete pupillary membrane, 6 (50%) had glaucoma. For eyes with a partial pupillary membrane, 11 eyes (52.38%) were followed up at the outpatient clinic without surgery and 10 eyes (47.62%) underwent membranectomy and pupilloplasty due to visual axis blockage. For the 12 eyes with a complete pupillary membrane, 6 eyes (50%) with normal intraocular pressure (IOP) received membranectomy and pupilloplasty combined with iridectomy, and 1 (16.67%) of these 6 eyes underwent a reoperation after 5 months due to a recurrent membrane. Furthermore, 6 eyes (50%) with glaucoma had membranectomy, pupilloplasty, iridectomy, and goniosynechialysis. Among these 6 eyes, 2 eyes (33.33%) underwent a reoperation due to the recurrence of a membrane and 4 eyes (66.67%) had a pale optic disc. The histopathological findings revealed that these membranes were mainly composed of fibrous tissue, lymphocytes, pigment epithelial cells, and vascular tissues. Conclusions: CFPM has diverse manifestations, including a partial pupillary membrane, a complete pupillary membrane with normal IOP, and a complete pupillary membrane with glaucoma. Timely diagnosis and treatment are critical when the visual axis is blocked and/or the IOP is high. [ J Pediatr Ophthalmol Strabismus . 2021;58(2):105–111.]

Significance This case highlights ocular ischemia after hemodialysis resulting in permanent vision loss. Fifteen percent of the U.S. population suffers from chronic kidney disease. Eye care providers should recommend risk factor modifications to their patients with end-stage renal disease before hemodialysis is started to prevent loss of vision. Purpose The purpose of this study was to demonstrate a case of concurrent nonarteritic anterior ischemic optic neuropathy and central retinal artery occlusion in the setting of hemodialysis initiation. Case report A 68-year-old Irish man with end-stage renal disease undergoing dialysis presented, complaining of 3 weeks of progressive vision loss in his left eye. His medical history is complex and includes extensive cardiac disease, bilateral carotid stenosis, and peripheral vascular disease. His surgical history includes a right carotid endarterectomy, bilateral lower extremity amputations, and an aortic valve replacement. Clinical examination revealed light perception vision with an afferent pupillary defect in the left eye and count finger peripheral vision only in the superior temporal quadrant of his vision. The dilated fundus examination showed significant pallid disc edema and focal areas of retina whitening with attenuated peripapillary vasculature in the left eye. This edema was confirmed by optical coherence tomography and supported optic nerve and retinal infarction. A temporal artery biopsy confirmed no evidence of arteritis. Conclusions Hemodynamic disruption during dialysis in patients with end-stage renal disease and overlying anemia can result in optic nerve and retinal infarction. Patients who are predisposed to nonarteritic anterior ischemic optic neuropathy or central retinal artery occlusion should be educated on this before starting dialysis to ensure careful blood pressure monitoring.

Objective For patients with severe traumatic brain injury (sTBI) with bilateral fixed dilated pupils (BFDP), the value of aggressively decompressive craniectomy (DC) treatment is still controversial. The objective of this study was to analyze and validate the outcome of DC in patients with sTBI with BFDP. Methods We retrospectively collected data from 44 patients with sTBI with BFDP who underwent DC treatment from July 2011 to June 2018. Outcomes used as indicators were mortality and favorable outcome. The analysis was based on the Glasgow Outcome Scale score recorded at discharge, 6, and 12 months after trauma. Results The overall survival was 36.4% (16/44) at discharge and 25.0% (11/44) at 6 and 12 months, and the favorable outcome (Glasgow Outcome Scale score = 4–5) at discharge, 6, and 12 months after injury was 9.1% (4/44), 13.6% (6/44), and 20.5% (9/44), respectively. Sex (P = 0.046), preoperative Glasgow Coma Scale (GCS) score (P = 0.031), injury–surgery intervals (P = 0.022), and tracheotomy (P = 0.017) were independent associations to 6 and 12 months follow-up survival, whereas only preoperative GCS score (odds ratio, 6.088; confidence interval, 1.172–31.612; P = 0.032) and injury–surgery intervals (odds ratio, 0.241; confidence interval, 0.065–0.893; P = 0.033) were independent associations with 12 months follow-up favorable outcome. Conclusions BFDP indicates a grave prognostic sign after sTBI, but the higher preoperative GCS score and shorter injury–surgery intervals in patients who underwent DC treatment might independently predict favorable outcome for patients with sTBI with BFDP, and patients might benefit more than expected if the DC treatment were applied more aggressively and positively.

Traumatic brain injury (TBI) is a leading cause of morbidity and mortality in young adults. Automated infrared pupillometry (AIP) has shown promising results in predicting neural damage in aneurysmal subarachnoid hemorrhage and ischemic stroke. We aimed to explore potential uses of AIP in triaging patients with TBI. We hypothesized that a brain injury severe enough to require an intervention would show Neurologic Pupil Index (NPI) changes.We conducted a prospective pilot study at a level-1 trauma center between November 2019 and February 2020. AIP readings of consecutive patients seen in the emergency department with blunt TBI and abnormal imaging findings on computed tomography were recorded by the assessing neurosurgery resident. The relationship between NPI and surgical intervention was studied.Thirty-six patients were enrolled, 9 of whom received an intervention. NPI was dichotomized into normal (≥3) versus abnormal (3) and was predictive of intervention (Fisher exact test; P0.0001). Six of the 9 patients had a Glasgow Coma Scale (GCS) score ≤8 and imaging signs of increased intracranial pressure (ICP) and underwent craniectomy (n = 4) or ICP monitor placement (n = 2) and had an abnormal NPI. Three patients underwent ICP monitor placement for GCS score ≤8 in accordance with TBI guidelines despite minimal imaging findings and had a normal NPI. The GCS score of these patients improved within 24 hours, requiring ICP monitor removal. NPI was normal in all patients who did not require intervention.AIP could be useful in triaging comatose patients after blunt TBI. An NPI ≥3 may be reassuring in patients with no signs of mass effect or increased ICP.

PURPOSE: To present a case where following Implantable Collamer Lens (ICL) implantation the patient developed toxic anterior segment syndrome (TASS) with a subsequent pupillary block as a consequence of the occlusion of the AquaPORT (STAAR Surgical) flow hole. METHODS: Case report and literature review. RESULTS: A V4c toric ICL (STAAR Surgical) was implanted in the left eye of a 32-year-old woman. After 1 week, the lens was 15° off axis and uneventful lens alignment correction was performed. At postoperative day 1, an intraocular pressure (IOP) of 11 mm Hg and mild corneal edema were observed. At postoperative day 7, there was an uncorrected distance visual acuity of 20/800, an IOP of 44 mm Hg, diffuse corneal edema, and fibrin strands in the anterior chamber. TASS was diagnosed and topical steroids, cycloplegia, antiglaucoma drops, and oral acetazolamide were prescribed. At postoperative day 9, dispersed endothelial pigment with a fixed mid-dilated pupil were identified. Anterior segment optical coherence tomography showed strands of fibrin blocking the central ICL hole and angle closure. Pupillary block related to the fibrin occluding the AquaPORT hole consecutive to TASS was diagnosed. The patient underwent ICL removal, but had a persistent atonic, hyporeflexive pupil as a complication. CONCLUSIONS: This case demonstrates that an AquaPORT hole may not be enough to prevent pupillary block in cases with postoperative intraocular inflammation, causing severe postoperative complications such as Urrets-Zavalia syndrome. Clinicians should consider this diagnosis in cases with corneal edema in the early postoperative period following AquaPORT ICL insertion. [ J Refract Surg . 2020;36(12):856–859.]

BACKGROUND AND OBJECTIVE: Pierson syndrome is a rare genetic disease defined by congenital nephrotic syndrome in association with microcoria. The authors aim to describe the posterior segment and retinal features in Pierson syndrome. PATIENTS AND METHODS: A retrospective chart review of nine patients diagnosed with Pierson syndrome was ascertained. Details of ophthalmic history, ocular examination, retinal imaging, and surgical interventions were obtained during a median duration of 17 months of follow-up (range: 6 to 60 months). Retinal interventions included scatter laser photocoagulation and surgical retinal repair. RESULTS: Sixteen eyes of nine patients were included. The axial length of five eyes with flat retina was 26.59 mm ± 0.99 mm. Highly myopic features including tessellated fundus with accompanying optic disc pallor, unidentifiable cup, and abnormal retinal vascular emanation from the disc were observed in all eyes (100%), whereas 12 eyes (75%) had parapapillary chorioretinal atrophy. Features of abnormal retinal vascularization included avascular peripheral retina on fluorescein angiography, aberrant course of the temporal arcades in 13 eyes (81.3%), and straightened nasal retinal blood vessels in 12 eyes (75%). Tortuous retinal blood vessels were observed in three eyes (18.75%). Surgical repair was performed in five out of seven eyes with rhegmatogenous retinal detachment (RRD). Recurrence was observed in all eyes, which required two to three procedures to achieve final reattachment. CONCLUSIONS: Combined features of high axial myopia with incomplete peripheral vascular maturation characterize the posterior segment in Pierson syndrome. Careful posterior segment examination is essential to detect RRD or retinal neovascularization. [ Ophthalmic Surg Lasers Imaging Retina . 2020;51:618–627.]

BackgroundFor the prehospital diagnosis of raised intracranial pressure (ICP), clinicians are reliant on clinical signs such as the Glasgow Coma Score (GCS), pupillary response and/or Cushing’s triad (hypertension, bradycardia and an irregular breathing pattern). This study aimed to explore the diagnostic accuracy of these signs as indicators of a raised ICP.MethodsWe performed a retrospective cohort study of adult patients attended by a Helicopter Emergency Medical Service (Air Ambulance Kent, Surrey Sussex), who had sustained a traumatic brain injury (TBI), requiring prehospital anaesthesia between 1 January 2016 and 1 January 2018. We established optimal cut-off values for clinical signs to identify patients with a raised ICP and investigated diagnostic accuracy for combinations of these values.ResultsOutcome data for 249 patients with TBI were available, of which 87 (35%) had a raised ICP. Optimal cut-off points for systolic blood pressure (SBP), heart rate (HR) and pupil diameter to discriminate patients with a raised ICP were, respectively, >160 mm Hg,5 mm. Cushing criteria (SBP >160 mm Hg and HR ConclusionTraditional clinical signs of raised ICP may under triage patients to prehospital treatment with hyperosmolar drugs. Further research should identify more accurate clinical signs or alternative non-invasive diagnostic aids in the prehospital environment.

Background Visual acuity has been a significant outcome measure in clinical trials for patients suffering from neuro-ophthalmological diseases and multiple sclerosis; however, there are limited data on the comparison of various testing strategies in pediatric patients with these disorders. Clinical trials using vision as an outcome could include a variety of tools to assess the acuity, including 2-m and 4-m standardized retroilluminated charts. Methods We investigated the difference in Early Treatment Diabetic Retinopathy Study (ETDRS) scores obtained using 2-m and 4-m charts, as well as the impact of optic neuritis, use of vision correction, age, and gender on visual acuity data from 71 patients with pediatric neuroimmunological conditions in a cross-sectional study. Results We determine that the ETDRS letter scores obtained using 4-m charts are on average 3.43 points less (P = 0.0034) when testing monocular ETDRS letter scores and on average 4.14 points less (P = 0.0008) when testing binocular ETDRS letter scores, relative to that obtained using the 2-m charts. However, we find that when performing monocular testing, optic neuritis in the eye being tested did not result in a statistically significant difference between 2-m and 4-m ETDRS letter scores. Conclusions Although visual acuity charts are formatted by the distance, there are significant differences in the number of letters correctly identified between 2-m and 4-m charts. Although the differences may not impact the clinical acuity, research protocols should consider these differences before collapsing data across disparate studies.

To measure the predictive values of relative afferent pupillary defect (RAPD) assessed semi-quantitatively, and visual acuity (VA) at onset of central retinal vein occlusion (CRVO), for neovascularization.Retrospective analysis of the TROXHEMO trial that included patients with CRVO within 30 days after the onset. Inclusion criteria were as follows: semi-quantitative RAPD assessment at diagnosis and/or at one month. RAPD was 'severe' if ≥ 0.9 log. Exclusion criteria were as follows: prophylactic panretinal photocoagulation (PRP) before neovascularization.Among the 119 patients enrolled in the main centre, 101 were analysed. 26 had a neovascular complication during the twelve months of follow-up: rubeosis (19), glaucoma (7) and posterior neovascularization (15). The mean time to onset of a neovascular complication was 4.7 months (1 to 12, median 3 months). All the patients who had a neovascular complication had RAPD at first examination or at one month (negative predictive value (NPV) = 100%) but the positive predictive value (PPV) was low (31%, 95% CI [21%; 42%]). The association 'severe RAPD or VA 35 letters (ETDRS) at inclusion or at one month' was the best compromise between PPV (53%, [39%; 68%]) and NPV (96%, [92%; 100%]).To predict neovascularization, RAPD should be routinely evaluated with filters: the risk of neovascular complication is (a) almost nil if there is no RAPD, (b) very low if there is no severe RAPD and if VA is higher than 35 letters, and (c) higher than 50% if RAPD is ≥ 0.9 log or if VA is less than 35 letters.

The importance of the glomerular basement membrane (GBM) in glomerular filtration is underscored by the manifestations of Alport and Pierson syndromes, caused by defects in type IV collagen α3α4α5 and the laminin β2 chain, respectively. Lamb2 null mice, which model the most severe form of Pierson syndrome, exhibit proteinuria prior to podocyte foot process effacement and are therefore useful for studying GBM permselectivity. We hypothesize that some LAMB2 missense mutations that cause mild forms of Pierson syndrome induce GBM destabilization with delayed effects on podocytes. While generating a CRISPR/Cas9-mediated analogue of a human LAMB2 missense mutation in mice, we identified a 44-amino acid deletion (LAMB2-Del44) within the laminin N-terminal domain, a domain mediating laminin polymerization. Laminin heterotrimers containing LAMB2-Del44 exhibited a 90% reduction in polymerization in vitro that was partially rescued by type IV collagen and nidogen. Del44 mice showed albuminuria at 1.8-6.0 g/g creatinine (ACR) at one to two months, plateauing at an average 200 g/g ACR at 3.7 months, when GBM thickening and hallmarks of nephrotic syndrome were first observed. Despite the massive albuminuria, some Del44 mice survived for up to 15 months. Blood urea nitrogen was modestly elevated at seven-nine months. Eight to nine-month-old Del44 mice exhibited glomerulosclerosis and interstitial fibrosis. Similar to Lamb2−/− mice, proteinuria preceded foot process effacement. Foot processes were widened but not effaced at one-two months despite the high ACRs. At three months some individual foot processes were still observed amid widespread effacement. Thus, our chronic model of nephrotic syndrome may prove useful to study filtration mechanisms, long-term proteinuria with preserved kidney function, and to test therapeutics.

Purpose To determine the test-retest reliability and diagnostic accuracy of a binocular optical coherence tomography (OCT) prototype (Envision Diagnostics, El Segundo, California, USA) for pupillometry. Design Assessment of diagnostic reliability and accuracy. Methods Fifty participants with relative afferent pupillary defects (RAPDs) confirmed using the swinging flashlight method (mean age 49.6 years) and 50 healthy control subjects (mean age 31.3 years) were examined. Participants twice underwent an automated pupillometry examination using a binocular OCT system that presents a stimulus and simultaneously captures OCT images of the iris-pupil plane of both eyes. Participants underwent a single examination on the RAPDx (Konan Medical, Irvine, California, USA), an automated infrared pupillometer. Pupil parameters including maximum and minimum diameter, and anisocoria were measured. The magnitude of RAPD was calculated using the log of the ratio of the constriction amplitude between the eyes. A pathological RAPD was above ±0.5 log units on both devices. Results The intraclass correlation coefficient was >0.90 for OCT-derived maximum pupil diameter, minimum pupil diameter, and anisocoria. The RAPDx had a sensitivity of 82% and a specificity of 94% for detection of RAPD whereas the binocular OCT had a sensitivity of 74% and specificity of 86%. The diagnostic accuracy of the RAPDx and binocular OCT was 88% (95% confidence interval 80%-94%) and 80% (95% confidence interval 71%-87%) respectively. Conclusions Binocular OCT-derived pupil parameters had excellent test-retest reliability. The diagnostic accuracy of RAPD was inferior to the RAPDx and is likely related to factors such as eye movement during OCT capture. As OCT becomes ubiquitous, OCT-derived measurements may provide an efficient method of objectively quantifying the pupil responses.

Background Despite automated pupillometry's (AP) improved detection of relative afferent pupillary defects (RAPDs) compared with the Swinging Flashlight Test (SFT), AP remains uncommon in clinical practice. This study examined barriers to routine use of AP in evaluation of acute vision loss. Methods (1) Ophthalmologists and optometrists' perceptions of AP were captured via electronic survey. (2) Ophthalmologists were presented with clinical vignettes to assess their use of AP in clinical decision-making. (3) Patients presenting with decreased vision to an ophthalmology urgent care clinic underwent manual SFT and AP screening to evaluate ophthalmologists' perceptions of the device. Results Surveys indicated that clinicians were "neutral" to "somewhat likely" to use AP. In clinical vignettes, more physicians proceeded with workup for optic nerve pathology when presented with an RAPD by AP than SFT (77% vs 26%, P = 0.003). When SFT and AP results were discordant, more physicians proceeded with workup for optic nerve disease when AP was positive and SFT was negative than vice versa (61% vs 18%, P = 0.008). In the clinical study of 21 patients, 50% of RAPDs detected by AP were not detected by SFT, although ophthalmologists rated AP's usefulness as only "neutral" to "somewhat useful." Conclusion Clinicians value pupillary examination and trust AP over SFT; however, widespread adoption and perceived value of AP may depend on its impact on clinical outcomes. Within a comprehensive diagnostic device, AP may be an important tool, but is not necessary to screen for optic nerve disease or evaluate acute vision loss.

Objectives To test the feasibility of adding driving simulation tasks to measure visuospatial ability and processing speed to an existing neurocognitive battery for breast cancer survivors (BCSs). Sample & setting 38 BCSs and 17 healthy controls from a cross-sectional pilot study conducted at the University of Kansas Medical Center. Methods & variables Exploratory substudy measuring pupillary response, visuospatial ability, and processing speed during two 10-minute driving simulations (with or without n-back testing) in a sample of BCSs with self-reported cognitive complaints and healthy controls. Results Feasibility of measurement of pupillary response during driving simulation was demonstrated. No between-group differences were noted for pupillary response during driving simulation. BCSs had greater visuospatial ability and processing speed performance difficulties than healthy controls during driving simulation without n-back testing and slower n-back response time. Implications for nursing Preliminary evidence showed a possible link between cancer/treatment on visuospatial ability and processing speed in BCSs.

Pineal masses commonly present with Parinaud syndrome, a triad consisting of upgaze restriction, convergence-retraction nystagmus, and pupillary light-near dissociation from compression of the quadrigeminal plate.

An Intraocular Lens (IOL) fixated on the iris either anteriorly, as a phakic IOL, or posteriorly, as an aphakic IOL, can influence pupil motility. In this interventional case series study, we evaluated pupil size under different levels of illumination (scotopic = 0.04 lx, low-mesopic = 0.4 lx and high-mesopic = 4 lx) for anterior iris-claw IOL fixation for correcting myopia or hyperopia (IFPH), retropupillary iris-claw IOL fixation to correct aphakia or as treatment for late in-the-bag IOL dislocation/subluxation (IFRP), and capsular-fixation IOL in-the-bag implantation (IB). Pupil size was measured preoperatively for the IFPH- and IB-group as well as 6 months after surgery for all groups. We analyzed a total of 70 eyes: 22 eyes of 11 patients with phakic IOLs, 22 eyes of 20 patients in the IFRP group and 26 eyes of 13 patients in the IB group. Both IFPH and IB showed a smaller postoperative scotopic pupil size, compared with the preoperative values. When compared to postoperative values of IB and IFPH, IFRP showed a significantly smaller postoperative scotopic pupil size (IFPH: 5.89 ± 0.83 mm, IFRP: 4.37 ± 0.83 mm, IB: 5.34 ± 0.98 mm, p

RESUMO Objetivo: Calcular as velocidades médias da dilatação de pupila para classificar a gravidade da lesão derivada da escala de coma de Glasgow, estratificada por variáveis de confusão. Métodos: Neste estudo, analisaram-se 68.813 exames das pupilas para determinar a velocidade normal de dilatação em 3.595 pacientes com lesão cerebral leve (13 - 15), moderada (9 - 12) ou grave (3 - 8), segundo a escala de coma de Glasgow. As variáveis idade, sexo, raça, tamanho da pupila, tempo de permanência na unidade de terapia intensiva, pressão intracraniana, uso de narcóticos, classificação pela escala de coma de Glasgow e diagnóstico foram consideradas confundidoras e controladas para análise estatística. Empregou-se regressão logística com base em algoritmo de classificação com aprendizado de máquina para identificar os pontos de corte da velocidade de dilatação para as categorias segundo a escala de coma de Glasgow. Resultados: As razões de chance e os intervalos de confiança desses fatores se mostraram estatisticamente significantes em sua influência sobre a velocidade de dilatação. A classificação com base na área sob a curva mostrou que, para o grau leve, na escala de coma de Glasgow, o limite da velocidade de dilatação foi de 1,2mm/s, com taxas de falsa probabilidade de 0,1602 e 0,1902 e áreas sob a curva de 0,8380 e 0,8080, respectivamente, para os olhos esquerdo e direito. Para grau moderado na escala de coma de Glasgow, a velocidade de dilatação foi de 1,1mm/s com taxas de falsa probabilidade de 0,1880 e 0,1940 e áreas sob a curva de 0,8120 e 0,8060, respectivamente, nos olhos esquerdo e direito. Mais ainda, para o grau grave na escala de coma de Glasgow, a velocidade de dilatação foi de 0,9mm/s, com taxas de falsa probabilidade de 0,1980 e 0,2060 e áreas sob a curva de 0,8020 e 0,7940, respectivamente, nos olhos esquerdo e direito. Esses valores foram diferentes dos métodos prévios de descrição subjetiva e das velocidades de dilatação previamente estimadas. Conclusão: Observaram-se velocidades mais lentas de dilatação pupilar em pacientes com escores mais baixos na escala de coma de Glasgow, indicando que diminuição da velocidade pode indicar grau mais grave de lesão neuronal. ABSTRACT Objective: To calculate mean dilation velocities for Glasgow coma scale-derived injury severity classifications stratified by multiple confounding variables. Methods: In this study, we examined 68,813 pupil readings from 3,595 patients to determine normal dilation velocity with brain injury categorized based upon a Glasgow coma scale as mild (13 - 15), moderate (9 - 12), or severe (3 - 8). The variables age, sex, race, pupil size, intensive care unit length of stay, intracranial pressure, use of narcotics, Glasgow coma scale, and diagnosis were considered as confounding and controlled for in statistical analysis. Machine learning classification algorithm-based logistic regression was employed to identify dilation velocity cutoffs for Glasgow coma scale categories. Results: The odds ratios and confidence intervals of these factors were shown to be statistically significant in their influence on dilation velocity. Classification based on the area under the curve showed that for the mild Glasgow coma scale, the dilation velocity threshold value was 1.2mm/s, with false probability rates of 0.1602 and 0.1902 and areas under the curve of 0.8380 and 0.8080 in the left and right eyes, respectively. For the moderate Glasgow coma scale, the dilation velocity was 1.1mm/s, with false probability rates of 0.1880 and 0.1940 and areas under the curve of 0.8120 and 0.8060 in the left and right eyes, respectively. Furthermore, for the severe Glasgow coma scale, the dilation velocity was 0.9mm/s, with false probability rates of 0.1980 and 0.2060 and areas under the curve of 0.8020 and 0.7940 in the left and right eyes, respectively. These values were different from the previous method of subjective description and from previously estimated normal dilation velocities. Conclusion: Slower dilation velocities were observed in patients with lower Glasgow coma scores, indicating that decreasing velocities may indicate a higher degree of neuronal injury.

Purpose: To report the presenting signs of retinoblastoma in a large cohort of patients who underwent orthoptic assessment at presentation. Methods: A retrospective medical chart review was conducted on 131 patients with retinoblastoma who presented consecutively to a single institution during a 6-year period. The main outcome measure was the presenting sign(s) of the disease. Results: Of 131 patients with retinoblastoma, 88 presented with unilateral disease and 43 presented with bilateral disease (mean ages: 22.7 and 14.8 months, respectively). Leukocoria was the presenting sign in 56% of patients, leukocoria and strabismus in 18%, strabismus in 13%, inflammation in 8%, and “other” signs in 5%. The fovea was affected by the retinoblastoma tumor or its sequelae in 75% of patients. Patients who presented with strabismus were significantly more likely to have foveal involvement than patients who presented with leukocoria alone ( P = .001). Thirty-one percent of patients had strabismus as a component of their presentation; 63% had exotropia, 23% had esotropia, and 14% had variable strabismus. The percentage of patients with strabismus increased to 66% when small angle and variable strabismus were also considered. Patients with inflammation had worse ocular survival ( P < .05). Conclusions: This study assessed the combination of leukocoria and strabismus as presenting features of retinoblastoma. Foveal involvement is common in patients who have strabismus and may influence decision-making regarding globe salvage. The authors confirmed that exotropia is more common than esotropia in retinoblastoma in the largest cohort to have undergone an orthoptic assessment. [ J Pediatr Ophthalmol Strabismus . 2021;58(5):324–330.]

Purpose The occurrence of relative afferent pupillary defect (RAPD) secondary to optic nerve diseases and widespread retinal disorders is well established. However, only very few reports of RAPD in macular disorders exist in the literature. In this study, we used automated pupillometer to evaluate RAPD in eyes with macular lesions. Methods It was a prospective cross-sectional study. A total of 82 patients with choroidal neovascular membrane (CNVM) - 65 unilateral and 17 bilateral macular lesions - were enrolled. RAPD was assessed with an automated pupillometer and macular lesions evaluated with optical coherence tomography (OCT). The length of the ellipsoid zone disruption was measured as the longest length of lesion on the horizontal raster scans and the area of macular lesion was measured manually, mapping the affected area of ellipsoid zone on the enface images. Results : RAPD scores showed good correlation with the intereye difference in length of maximum ellipsoid zone disruption (r-value = 0.84, P value

Purpose: To report a patient with Pierson syndrome who presented with neovascular glaucoma (NVG) after cataract surgery. Methods: Retrospective case report. Results: A 17-year old monocular female ...

This study aims to identify predictive factors associated with surgical intervention and the visual outcome of orbital cellulitis and to evaluate the treatment outcomes. A retrospective study involving 66 patients (68 eyes; 64 unilateral and 2 bilateral) diagnosed with bacterial orbital cellulitis was conducted between November 2005 and May 2019. The mean (± standard deviation) age was 42.1 (± 25.8) years (range: 15 days–86 years). Sinusitis was the most frequent predisposing factor, occurring in 25 patients (37.9%), followed by skin infection in 10 patients (15.2%), and acute dacryocystitis in 9 patients (13.6%). Subperiosteal abscesses were found in 24 eyes and orbital abscesses in 19 eyes. Surgical drainage was performed in 31 eyes. Regarding the abscess volume for surgical drainage, a cut-off of 1514 mm3 showed 71% sensitivity and 80% specificity. There was significant improvement in visual acuity (VA) and decrease in proptosis after treatment (for both, P ≤ .001). Only pre-treatment VA ≤20/200 was a significant predictor for post-treatment VA of 20/50 or worse (adjusted odds ratio: 12.0, P = .003). The presence of a relative afferent pupillary defect was the main predictor of post-treatment VA of 20/200 or worse (adjusted odds ratio: 19.0, P = .003). The most common predisposing factor for orbital cellulitis in this study was sinusitis. VA and proptosis significantly improved after treatment. We found that the abscess volume was strongly predictive of surgical intervention. Pre-treatment poor VA and the presence of relative afferent pupillary defect can predict the worst visual outcome. Hence, early detection of optic nerve dysfunction and prompt treatment could improve the visual prognosis.

A 42-year-old Algerian man presented for binocular oblique diplopia, hypersomnolence with drop attacks, bilateral hearing loss, and thoracic pain. He had a right thalamomesencephalic hemorrhage due to an underlying cavernous malformation treated with subtotal surgical resection. On neuro-ophthalmic examination, the patient had a left relative afferent pupillary defect and a right oculosympathetic efferent pupillary defect (i.e., Horner syndrome) in addition to other thalamomesencephalic eye and neurologic signs (right fourth nerve palsy, hearing loss, hemiparesis, and thalamic pain). Clinicians should recognize the localizing value of this unique constellation of mesencephalic afferent and efferent pupillary defects.

Background Aneurysm rerupture and bilateral fixed and dilated pupils (bFDPs) typically have a poor prognosis across all age groups. The synchronous occurrence of both features in the infantile demographic has not been previously reported. Case Description We describe the near-fatal clinical course of a 7-month-old infant who experienced an acute giant aneurysm rerupture with signs of bFDPs. The patient was rapidly managed with neurosurgical intervention and has achieved a favorable outcome 1 year later. Conclusions This case highlights that survival of infants who present with intracranial aneurysmal rerupture and bFDPs is amenable to rapid neurosurgical intervention and should be remembered in clinical practice.

Introduction: The failure rate of both filtration surgery and of aqueous shunt implantation is higher for iridocorneal endothelial syndrome than in other scenarios, due to the continuous proliferation of abnormal endothelial cells over the trabecular meshwork and the filtration area and also due to the more pronounced cicatrizing response shown by these young patients. We present the first case ever described in the literature of a pregnant patient with iridocorneal endothelial syndrome and uncontrolled ocular hypertension who was implanted an Ex-PRESS mini-shunt. Clinical case: A 35-year-old female presented with diminution of vision in the left eye for 2 months. She was 20 weeks pregnant. Her visual acuity was 20/20 in right eye and 20/25 in left eye, and intraocular pressure was 11 mmHg in right eye and 34 mmHg in left eye. Slit lamp biomicroscopic examination revealed no alterations in right eye, whereas left eye showed corectopia and uveal ectropion, stroma of iris’ sectoral atrophy and moderate corneal epithelial edema. Gonioscopy showed some anterior iris synechiae in left eye. Fundus evaluation was normal. Based on clinical features and examination, the diagnosis of left eye iridocorneal endothelial syndrome with decompensated intraocular pressure was made. She was prescribed topical timolol (0.5%) and dorzolamide. As a result of uncontrolled intraocular pressure and the impossibility to prescribe other hypotensive treatment available due to her being pregnant, it was decided to perform surgery in left eye using an Ex-PRESS mini-shunt and Ologen®; 6 months post surgery, intraocular pressure was 9 mmHg with no need for hypotensive treatment. The cornea was transparent, and the patient maintained her left eye visual acuity. Conclusions: Ex-PRESS mini-shunt can be considered a surgical option for iridocorneal endothelial syndrome. Its composition allows the ostium to remain open and the device triggers a milder postoperative inflammatory response. In our particular case, taking into account that the subject was a young, phakic, pregnant woman, whose intraocular pressure had to be closely controlled and we had to ensure that her postoperative care included as few drugs and as few reoperations as possible, we thought that using this device was the most appropriate option.

Purpose: The relative afferent pupillary defect (RAPD) is an important sign of asymmetrical retinal ganglion cell damage. The purpose of this study was to quantify RAPD by a pupillometer (RAPiDo, Neuroptics) and assess its correlation with asymmetric glaucoma and manual pupillary assessment. Methods: A total of 173 subjects were enrolled in the study and categorized into glaucoma, n = 130, and control, n = 43. Subjects were all recruited in the Glaucoma Clinic of the Aravind Eye Hospital in Madurai during their follow-up. They were 18 years and older, with best corrected visual acuity of 6/36 or better. Exclusion criteria included all retinal pathologies, optic atrophies, ocular injuries, severe uveitis, cloudy corneas, dense cataracts, or use of mydriatics or miotic drugs. RAPD was assessed in all subjects using an automated pupillometer and the results were compared with the swinging flash light test conducted on the same subjects by an experienced ophthalmologist. We looked at the correlation between RAPD and the intereye difference in cup-to-disc ratio (CDR), mean deviation (MD) of visual field testing, and retinal nerve fiber layer (RNFL) thickness. Sensitivity and specificity were assessed by area under the receiver operator characteristic (AUROC) analysis. Results: Glaucoma patients had significant RAPD (0.55 ± 0.05 log units) when compared with the controls (0.25 ± 0.05 log units), P < 0.001. Significant intereye differences in CDR, MD, and RNFL between glaucoma and control (P < 0.001) were seen. There was a good correlation between the magnitude and sign of RAPD and these intereye differences in CDR (r = 0.52, P < 0.001), MD (r = 0.44, P < 0.001) and RNFL thickness (r = 0.59, P < 0.001). When compared with the experienced ophthalmologist, AUROC was 0.94, with 89% sensitivity and 91.7% specificity. Conclusion: The good correlation between the magnitude of RAPD, as measured by the automated pupillometer, and intereye differences in MD, CDR, and RNFL thickness in glaucomatous, and the good sensitivity and specificity when compared with the experienced ophthalmologist, suggest that pupillometry may be useful as a screening tool to assess asymmetric glaucoma.

Purpose: To investigate the relation between erectile dysfunction (ED) severity and pupillary functions in patients with diabetes mellitus (DM). Methods: This prospective and observational study included 90 patients with type 2 DM and ED. Patients divided into three subgroups according to severity of ED: (i) Mild ED, (ii) Moderate ED and (iii) Severe ED groups. Thirty age-matched healthy subjects formed the control group. Main outcome measures were pupil diameter and average speed of pupil dilation. Static and dynamic pupillometry analysis was performed using the Sirius Topographer (CSO, Firenze, Italy). Results: Mean pupil diameter during static and dynamic pupillometry analysis were significantly greater in the control group than in the all study groups (P < 0.05). Mean pupil diameter in static pupillometry analysis was significantly different in each study group and pupil was more miotic in the Severe ED group than in the both Moderate and Mild ED groups (P < 0.05 for each). Dynamic pupillometry analysis revealed that mean pupil diameter and mean average dilation speed were significantly different in each study group throughout measurement period and the highest speed was observed in the Mild ED group and the lowest speed was observed in the severe ED group (P < 0.005 for each). Conclusion: Our study results suggest that abnormal pupil functions due to diabetic autonomic neuropathy may indicate the associated ED in patients with DM.

Symptoms of autonomic dysfunction are common in Fabry disease. In this study we aimed to evaluate alterations in the pupillary response to white light stimulation in patients with Fabry disease and their association with the severity of autonomic symptoms. Fourteen consecutive patients with Fabry disease and 14 healthy control participants were enrolled in this cross-sectional study. The Mainz Severity Score Index (MSSI) was used to measure the severity of Fabry disease and the Composite Autonomic Symptom Scale 31 (COMPASS 31) questionnaire was used to evaluate the severity of autonomic symptoms. The pupil light responses were assessed with an infrared dynamic pupillometry unit. There were significant reductions in the amplitude (P = 0.048) and duration (P = 0.048) of pupil contraction, and the latency of pupil dilation (P = 0.048) in patients with Fabry disease compared to control subjects. The total weighted COMPASS 31 score correlated with MSSI (r = 0.592; P = 0.026) and the duration of pupil dilation (ρ = 0.561; P = 0.037). The pupillomotor weighted sub-score of the COMPASS 31 correlated inversely with the duration of pupil contraction (r = − 0.600; P = 0.023) and latency of pupil dilation (ρ = − 0.541; P = 0.046), and directly with the duration of pupil dilation (ρ = 0.877; P r = 0.533; P = 0.049). In conclusion, abnormal pupillary function is demonstrated in patients with Fabry disease, which is associated with the severity of autonomic symptoms.

Background The purpose of this study was to evaluate the shape of the crystalline lens in terms of biometry and diopters before and after cycloplegia using the CASIA2 swept-source (SS) optical coherence tomography (OCT) system on the anterior segment. Methods This was a retrospective study. Children and adolescents (26 males and 29 females, aged 4–21 years) with simple ametropia were selected for optometry and CASIA2 imaging at 2 separate visits before and after cycloplegia. Diopter values were derived from the spherical power (S) obtained by optometry. Biometric parameters of the crystalline lens, including the anterior chamber depth (ACD), anterior and posterior curvature of the lens (ACL and PCL), lens thickness (LTH), lens decentration (LD), lens tilt (LT), and equivalent diameter of the lens (LED), were measured by the CASIA2 system. The differences in these parameters after compared with before cycloplegia were determined, and their relationships were analyzed. Results Fifty-five participants (106 eyes) were initially enrolled. There was a significant difference (P t=-7.026, P ), ACD (t=-8.796, P ), ACL (t=-13.263, P ) and LTH (t = 7.363, P ) after compared with before cycloplegia. The change in the PCL (t = 1.557, P = 0.122), LD (t = 0.876, P = 0.383), LT (t = 0.440, P = 0.661) and LED (t=-0.351, P = 0.726) was not statistically significant (P > 0.05). There was a significant (P r = 0.466, P ), LTH (r=-0.592, P ), and LED (r = 0.223, P = 0.021) but not the PCL (r = 0.19, P = 0.051), LD (r=-0.048, P = 0.0628) or LT (r=-0.022, P = 0.822). Furthermore, the change in the ACD was closely related to the change in crystalline morphology. However, in children and adolescents, we found that the change in crystalline morphology was unrelated to age. Conclusions Changes in lens morphology after compared with before cycloplegia are mainly related to the ACL and LTH, but there is no difference in the PCL, LD, LT, or LED. In the adolescent population, change in the S is related to change in the ACL, LED and LTH. However, age is unrelated to the shape and tendency of the crystalline lens. Further research is required to determine whether the same conclusion applies to different age groups and different refractive states (myopia, hyperopia, emmetropia) .

SIGNIFICANCE After a dilated eye examination, many patients experience symptoms of prolonged light sensitivity, blurred vision, and cycloplegia associated with pharmacological mydriasis. Phentolamine mesylate ophthalmic solution (PMOS) may expedite the reversal of mydriasis in patients, potentially facilitating return to functional vision and reducing barriers to obtaining dilated eye examinations. PURPOSE The protracted reversal time after pharmacologically induced pupil dilation impairs vision. We tested the hypothesis that PMOS rapidly reduces pupil diameter in this acute indication. METHODS In this double-masked placebo-controlled, randomized, two-arm crossover phase 2b trial, we evaluated the effects of one drop of 1% PMOS applied bilaterally in subjects who had their pupils dilated by one of two common mydriatic agents: 2.5% phenylephrine or 1% tropicamide. End points included change in pupil diameter, percent of subjects returning to baseline pupil diameter, and accommodative function at multiple time points. RESULTS Thirty-one subjects completed the study (15 dilated with phenylephrine and 16 with tropicamide). Change in pupil diameter from baseline at 2 hours after maximal dilation with 1% PMOS was -1.69 mm and was significantly greater in magnitude compared with placebo for every time point beyond 30 minutes (P < .05). At 2 hours, a greater percentage of study eyes given 1% PMOS returned to baseline pupil diameter compared with placebo (29 vs. 13%, P = .03), which was this also seen at 4 hours (P < .001). More subjects treated with PMOS in the tropicamide subgroup had at least one eye returning to baseline accommodative amplitude at 2 hours (63 vs. 38%, P = .01). There were no severe adverse events, with only mild to moderate conjunctival hyperemia that resolved in most patients by 6 hours. CONCLUSIONS Phentolamine mesylate ophthalmic solution at 1% reversed medically induced pupil dilation more rapidly than placebo treatment regardless of which mydriatic was used (adrenergic agonists and cholinergic blockers) with a tolerable safety profile.

Myelinated retinal nerve fiber layer is a benign retinal condition that is usually an incidental finding in a healthy patient. Visual acuity is typically not compromised, but some cases have other associated ocular conditions that can affect vision. Diagnosis in challenging atypical cases requires imaging studies to rule out other pathologies. The authors describe the case of a 2-year-old girl who presented with atypical optic nerve fiber myelination. [ J Pediatr Ophthalmol Strabismus . 2020;57:e56–e58.]

Pierson syndrome (PIERSS) is a rare autosomal recessive disorder characterized by the combination of congenital nephrotic syndrome (CNS) and extrarenal symptoms including ocular malformations and neurodevelopmental deficits. PIERSS is caused by biallelic pathogenic variants in the LAMB2 gene leading to the defects of β2-laminin, the protein mainly expressed in the glomerular basement membrane, ocular structures, and neuromuscular junctions. Severe complications of PIERSS lead to the fatal outcome in early childhood in majority of the cases. We report a case of 5-year-old girl with severe phenotype of PIERSS caused by biallelic functional null variants of the LAMB2 gene. Due to consequences of CNS, the patient required bilateral nephrectomy and peritoneal dialysis since early infancy. The course was additionally complicated by tubulopathy, life-threatening infections, severe hypertension, erythropoietin-resistant anemia, generalized muscular hypotonia, neurogenic bladder, profound neurodevelopmental delay, epilepsy, gastrointestinal problems, secondary hypothyroidism, and necessity of repeated ocular surgery due to microcoria, cataract, and nystagmus. Due to multidisciplinary efforts, at the age of 4 years, the kidney transplantation was possible. Currently, the renal graft has an excellent function; however, the girl presents severe neurodevelopmental delay. The report presents a unique long-term follow-up of severe PIERSS with a few new phenotypical findings. It highlights the clinical problems and challenges in management of this rare condition.

PReCIS:: The potential parameters for differentiating pupillary block (PB) from plateau iris configuration (PIC) on anterior segment optical coherence tomography (ASOCT) are lens/pupil size parameters and angles. Further study is needed to determine a landmark peripheral to the centroid of the iris. Purpose Investigate anterior segment parameters to distinguish between 2 mechanisms of angle closure, PB and PIC, using swept-source Fourier domain ASOCT. Patients and methods Retrospective ASOCT images from narrow angle eyes were reviewed. PIC was defined either by ultrasound biomicroscopy and/or clinically when an iridoplasty was performed. Images were read by a masked reader using Anterior Chamber Analysis and Interpretation software to identify scleral spur landmarks and calculate anterior chamber, peripheral angle, iris size, iris shape, and lens/pupil size parameters. ASOCT parameters were summarized and compared using the 2-sample t test. Thresholds and area under receiver operating characteristic curve were calculated using logistic regression analysis. Results One hundred eyes (66 PB and 34 PIC) of 100 participants were reviewed. Of all ASOCT parameters, iris length in each quadrant, pupil arc, lens/pupil parameters (pupil arc, lens vault, and pupil diameter), all pupillary margin-center point-scleral spur landmark (PM-C-SSL) parameters, and all except superior central iris vault parameters were significantly different between PB and PIC. On threshold evaluation, lens/pupil parameters had the greatest area under receiver operating characteristic curve values (0.77 to 0.80), followed by PM-C-SSL angles (0.71 to 0.75). Conclusions We propose that the pupil size parameters and PM-C-SSL angle are the most reliable novel ASOCT parameters to distinguish between PB and PIC eyes. These parameters do not rely on the visibility of the posterior iris surface, which is difficult to identify with ASOCT, but may be ambient lighting dependent.

Purpose To evaluate the changes in pupil diameter in women and men after cataract surgery. The correlation of pupillary changes with the variables age and anterior chamber depth will be analyzed. Methods The values of 109 randomized eyes who underwent cataract surgery were obtained and divided into two groups, 71 women and 38 men. Pupil diameter was measured preoperatively and 3-months postoperatively using the pupillometer software of the Topolyzer Vario (Wavelight Laser Technologie AG). Anterior chamber depth was obtained with Pentacam® (Oculus). Differences in pupillary diameters were investigated and correlations with age and anterior chamber depth were analyzed. Results For mesopic pupils, the male group had greater reduction in their postoperative pupillary diameter, -0.56 mm (-12.4%), than the female group, -0.38 mm (-8.2%), P = 0.025. Photopic postoperative pupils reduced to a lesser extent, yet more in men than in women (-0.11mm [-4.5%] vs. -0.04 [-1.6%], P = 0.048). Weak significant negative correlation was found between photopic pupillary changes in women with age (r = -0.24, P = 0.041), and positive correlation for mesopic pupillary changes in men with age (r = +0.34, P = 0.039). Conclusions Patients experience pupil reduction after cataract surgery in general, but more in men than in women and for both photopic and mesopic lighting conditions. The differences are statistically significant and have moderate clinical relevance. Concerning pupillary changes, weak but opposite sign correlations were found between male/female gender and age. This work was supported by the Spanish Ministerio de Economía y Competitividad and FEDERfunds under project Ref. DPI2016-76019-R