Bifid Pupil Iridodialysis Secondary to Airsoft Gun Injury.

This case report describes a diagnosis of iridodialysis in an 11-year-old boy who presented with decreased vision and inflammation after direct blunt trauma to the left eye.

Leukocoria in a 4-year-old boy.

A healthy 4-year-old boy referred for evaluation of an abnormal red reflex in the left eye was noted, on fundus examination, to have extensive white, striated lesions surrounding the optic nerve that involved the superior and inferior macular arcades. On further examination, he was found to have ipsilateral high myopia and amblyopia. The triad of unilateral myelinated retinal nerve fibers, myopia, and amblyopia led to a diagnosis of Straatsma syndrome, which requires early treatment to prevent permanent vision loss. Treatment comprised a contact lens for refractive correction of the left eye, patching of the right eye, and full-time polycarbonate protective lenses. With 2 years' follow-up, the left eye failed to improve appreciably.

Polymerizing laminins in development, health, and disease.

Polymerizing laminins are multi-domain basement membrane (BM) glycoproteins that self-assemble into cell-anchored planar lattices to establish the initial BM scaffold. Nidogens, collagen-IV and proteoglycans then bind to the scaffold at different domain loci to create a mature BM. The LN domains of adjacent laminins bind to each other to form a polymer node, while the LG domains attach to cytoskeletal-anchoring integrins and dystroglycan, as well as to sulfatides and heparan sulfates. The polymer node, the repeating unit of the polymer scaffold, is organized into a near-symmetrical triskelion. The structure, recently solved by cryo-electron microscopy in combination with AlphaFold2 modeling and biochemical studies, reveals how the LN surface residues interact with each other and how mutations cause failures of self-assembly in an emerging group of diseases, the LN-lamininopathies, that include LAMA2-related dystrophy and Pierson syndrome.

Clinical Presentation and Treatment Outcomes of Pupillary Block Glaucoma Following Vitreoretinal Surgery.

PURPOSE: To describe the clinical presentation and treatment outcomes of pupillary block glaucoma (PBG) following vitreoretinal surgery (VR surgery). MATERIAL AND METHODS: Retrospective observational study of 6941 patients, who underwent VR surgery at a tertiary eye care centre in South India between January 2015 and December 2019. Amongst them, clinical data of 61 patients who developed PBG were taken for statistical analysis. RESULTS: Mean (SD) age was 53.90 (13.4) years and the incidence of PBG was .87%. Median (IQR) time of onset of PBG following VR surgery was 3.33 (1.1-6.6) months and majority were pseudophakic (75%). PBG resolved with Nd:YAG laser peripheral iridotomy (LPI) alone in 50 (82%) patients, whereas 11(18%) patients required additional interventions like surgical iridectomy, trabeculectomy or diode laser cyclophotocoagulation (CPC) either as a stand-alone procedure or in combination with silicone oil removal (SOR). Mean (SD) intraocular pressure at the onset of PBG was 41.61 (14.5) mmHg, which reduced drastically following LPI to 24.28 (14.9) mmHg which further dropped significantly at 6 months follow up to 20.34 (13.9) mmHg. CONCLUSIONS: Incidence of secondary PBG after VR surgery was .87%, and we observed diabetes mellitus, combined cataract and VR surgery, use of 1000cs SO endotamponade, intraoperative endolaser and multiple VR surgical interventions as common associations. Majority of the patients with PBG after VR procedures resolved with LPI and medical management. Few individuals (18%) required additional laser or surgical intervention for IOP control.

Reverse pupillary block with pigment dispersion and elevated intraocular pressure following bilateral phakic intraocular lens implantation.

The authors describe a case of reverse pupillary block with pigment dispersion following sequential phakic intraocular lens (pIOL) implantation for high myopia, in a young female patient. The intraocular pressure (IOP) elevation began 3 weeks postoperatively, for which Nd-YAG laser peripheral iridotomies (PIs) were attempted elsewhere. Despite maximum medical therapy, the IOP was uncontrolled. She was referred to our institute for further management. Examination showed anteriorly displaced iris-pIOL diaphragm, iris pigment dispersion and raised IOP. The PIs were incomplete. Based on clinical evaluation and investigations, we concluded that the excess area of contact of the posterior iris over the pIOL caused a reverse pupillary block and pigment dispersion. The IOPs were controlled by repeating laser iridotomies and with medical therapy. Subsequently, the patient developed a low lens vault leading to bilateral cataract. Sequential explantation of the pIOL along with cataract extraction was performed and her vision was restored.

Expanding the spectrum of LAMB2: Pierson syndrome associated with neuromuscular junction disorder in two patients.

LAMB2 gene disorders present with different phenotypes. Pierson syndrome (PS) is a common phenotype associated with LAMB2 variants. Neuromuscular phenotype has been reported including hypotonia and developmental delay. However, neuromuscular junction abnormalities represented as congenital myasthenic syndrome (CMS) was reported in one adult patient only. Here, in this paper, we present two pediatric cases with a severe presentation of PS and have CMS so expanding the knowledge of LAMB2 related phenotypes. The first patient had hypotonia and global developmental delay. Targeted genetic testing panel demonstrated homozygous pathogenic variant in the LAMB2 gene (c.5182C>T, pGln1728*) which was reported by Maselli et al. 2009. Repetitive nerve stimulation (RNS) showed a decremental response at low frequency of 3 Hz. On the other hand, the second patient had profound weakness since birth. Tri-Whole exome sequencing showed homozygous pathogenic variant in the LAMB2 gene c.2890C>T, pArg964*. A trial of salbutamol did not improve the symptoms. Both patients passed away from sequala of PS. The spectrum of phenotypic changes associated with LAMB2 mutations is still expanding, and further investigation into the various clinical and morphologic presentations associated with these mutations is important to better identify and manage affected individuals.

Leukocoria Due to Persistent Hyperplastic Primary Vitreous.

This case report discusses a diagnosis of persistent hyperplastic primary vitreous presenting as leukocoria in a boy aged 50 days.

Reverse pupillary block, in contemporary scleral intraocular lens procedures.

BACKGROUND: To evaluate the frequency and anterior segment optical coherence tomography parameters of patients with scleral fixated intraocular lenses (IOL) and reverse pupillary block (RPB). METHODS: Retrospective analysis at a tertiary care centre (Department for Ophthalmology and Optometry, Medical University of Vienna, Austria). We researched our records for patients who underwent scleral fixated IOL implantation from January 2018 till February 2023. Patients were included only if there was at least one adequate post-operative scan of anterior segment optical coherence tomography (AS-OCT) available. Initially, AS-OCT scans were assessed for IOL tilt and decentration employing a 3D scan and then later for anterior chamber angle (ACA), aqueous anterior chamber depth (AQD), pupil diameter and iris-IOL distance using the 2D scan at a 0° angle. Both an iris-IOL distance of 0 or less and an ACA of more than 70° were required to define an RPB. RESULTS: A total of 110 patients met the inclusion criteria, 41 were treated using the Carlevale, 33 the four flanged, 24 the Yamane and 12 the Scharioth technique, respectively. RPB was found in 32 patients (29%). Twenty patients with RPB were treated using YAG peripheral iridotomy, mean ACA decreased from 91.91° ± 13.77 to 61.02° ± 8.52, (p < 0.001), mean AQD decreased from 4.67 mm ± 0.47 to 4.31 ± 0.36 mm (p < 0.001) and mean iris-IOL distance increased from -0.09 ± 0.04 to 0.33 ± 0.30 (p < 0.001). CONCLUSIONS: RPB is found in a third of eyes who have undergone scleral fixated IOL implantation without iridectomy. YAG peripheral iridotomy is a potent option to treat RPB, and subsequently reduce the risk of iris chafing and secondary inflammation or glaucoma.

Diagnostic Value of Point-of-Care Ultrasound-Guided Assessment of Relative Afferent Pupillary Defect in Adult Ocular Trauma Patients Presenting to the Emergency Department: A Prospective Cohort Study.

OBJECTIVES: Early diagnosis of relative afferent pupillary defects (RAPDs) in patients with ocular trauma is crucial for timely management and improved outcomes. However, clinical examination can be challenging for patients with periorbital ecchymosis. This study aimed to compare the diagnostic accuracy of point-of-care ultrasound (POCUS) and clinical examination by emergency physicians for detecting RAPD in adult ocular trauma patients and to evaluate the proportion of RAPD in patients with ocular trauma who presented to the ED. METHODS: This prospective cohort study was conducted at an academic emergency department in South India. Adult ocular trauma patients were assessed for RAPD using clinical examinations by emergency physicians and POCUS. The diagnostic accuracies of both methods were compared, with the ophthalmologist's final diagnosis serving as the gold standard. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were calculated for both techniques. RESULTS: A total of 376 patients (median age, 35 years) were included in this study. RAPD was identified in 14.63% of the patients. The sensitivity and specificity of POCUS in detecting RAPD were 92.73% and 99.38%, respectively, which were higher than those of clinical examination, with a sensitivity of 81.82% and specificity of 99.07%. The PPV and NPV of the clinical examination were 93.75% and 96.95%, respectively, whereas the PPV and NPV of POCUS were 96.23% and 98.76%, respectively. POCUS accurately diagnosed RAPD in patients with periorbital ecchymosis. CONCLUSION: POCUS-guided RAPD assessment proves to be a better diagnostic adjunct compared to clinical examination in patients with ocular trauma presenting to the emergency department.

Isolated ectopia lentis with partial anterior dislocation and pupillary block: a case report.

BACKGROUND: Ectopia lentis is the dislocation of the natural crystalline lens and usually presents in the setting of trauma or other systemic diseases. Herein, we describe a case of an otherwise healthy four-year-old boy with isolated ectopia lentis whose partial lens dislocation was captured on a smartphone by the patient's father several days prior. CASE PRESENTATION: A four-year-old boy with no past medical, developmental, or trauma history presented with bilateral partial anterior lens dislocation with pupillary block. Initial ophthalmic evaluation two months prior was notable for uncorrected visual acuity at 20/100 OD, 20/250 OS, bilateral iridodenesis, and partially dislocated lenses inferonasally OD and inferiorly OS on slit lamp. Genetic testing found no abnormalities. Ten months later, the patient developed sudden onset of left eye pain. A dislocated lens and temporarily dilated left pupil were captured on a smartphone by the patient's father. He was evaluated 3 days later after a second episode and found to have hand motion vision OS, a fixed 8 mm left pupil with the crystalline lens subluxed into the pupil space and accompanying intraocular pressure OS of 40 mmHg. The lens was surgically removed with a limited anterior vitrectomy. Four and a half years after surgery, visual acuity was 20/125 OS with aphakic correction. The right eye eventually underwent prophylactic lensectomy and was 20/30 in aphakic correction. CONCLUSIONS: This report presents a unique presentation of isolated ectopia lentis with anterior lens dislocation and pupillary block and illustrates the role of smartphone photography in assisting in the triage of eye emergencies.

Surgical approach to and morphology of visually significant persistent pupillary membranes.

PURPOSE: To characterize the morphology of persistent pupillary membranes (PPMs) in pediatric patients and explore the corresponding surgical approaches. SETTING: Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangzhou, China. DESIGN: Prospective observational study. METHODS: Consecutive pediatric patients with PPMs who underwent surgery from April 2020 to July 2022 were included. PPM morphology was assessed and categorized according to its anatomic relationship with crystalline lens and distribution of iris strands. The surgical approaches for different morphologies of PPMs were described in detail. The visual outcome and operation-related complications were recorded. RESULTS: 31 eyes from 19 patients were included with the mean age of 7.2 years. 3 morphological variants of PPMs were observed: type I (51.6%, 16/31), a spider-like appearance and no adhesion to the anterior lens capsule (ALC); type II (38.7%, 12/31), a loose central adherence to the ALC and partially thick iris strands attached to the iris collarette; type III (9.7%, 3/31), a tight central adherence to the ALC and only silk-like iris strands. Surgeries were performed with a natural pupil size in type I, while dilated pupil in the other types. The adhesions between PPM and the ALC were separated by viscoelastic injection in type II and by discission needles in type III. The corrected distance visual acuity was significantly improved from 0.34 ± 0.18 logMAR preoperatively to 0.17 ± 0.09 logMAR postoperatively ( P < .001). No operation-related complications were observed during 9.5-month follow-up. CONCLUSIONS: PPMs were categorized into 3 types according to their different morphologies, which helped to determine the best surgical strategy.

Emergency decompressive surgery in patients with transtentorial brain herniation and pupillary abnormalities: the importance of improved pupillary response after osmotherapy and surgery.

OBJECTIVE: The predictors of survival and functional recovery following emergency decompressive surgery in patients with transtentorial brain herniation, particularly those with pupillary abnormalities, have not been established. In this study, the authors aimed to assess the outcome of patients with intracranial mass lesions, transtentorial brain herniation, and nonreactive mydriasis, following emergency surgical decompression. METHODS: A retrospective chart review was performed of all patients with transtentorial herniation and pupillary abnormalities who underwent craniotomy or craniectomy at two trauma and stroke centers between 2016 and 2022. The functional outcome was determined using the modified Rankin Scale (mRS). RESULTS: Forty-three patients, 34 men and 9 women with a mean age of 47 years (range 16-92 years), were included. The underlying etiology was traumatic brain injury in 33 patients, hemorrhagic stroke in 8 patients, and tumor in 2 patients. The median preoperative Glasgow Coma Scale score was 3 (range 3-8), and the median midline shift was 9 mm (range 1-29 mm). Thirty-two patients (74.4%) had bilaterally fixed and dilated pupils. The median time to surgery (from pupillary changes) was 133 minutes (mean 169 minutes, range 30-900 minutes). Eighteen patients (41.9%) died postoperatively. After a median follow-up of 12 months (range 3-12 months), 11 patients (26.8%) had a favorable functional outcome, while 10 remained severely disabled (mRS score 5). On univariate analysis, younger age (p < 0.001), less midline shift (p = 0.049), and improved pupillary response after osmotic therapy (p < 0.01) or decompressive surgery (p < 0.001) were associated with favorable outcomes at 3 months. CONCLUSIONS: With aggressive medical and surgical management, patients with transtentorial brain herniation, including those with bilaterally fixed and dilated pupils, may have considerable rates of survival and functional recovery. Young age, less midline shift, and improved pupillary response following osmotic therapy or decompressive surgery are favorable prognosticators.

Comparison of cycloplegia with atropine 1% versus cyclopentolate 1.

PURPOSE: Cycloplegic refraction is mandatory for children to know the eye's refractive status. In this study, we compared cycloplegia induced by cyclopentolate 1% to that induced by atropine 1% by means of retinoscopy. METHODS: In this parallel-designed interventional study, we included 67 children aged between 4 and 17 years. After the initial retinoscopy under cyclopentolate 1% (used twice in each eye), we repeated it a week later under atropine ointment 1% (used twice a day for 3 days); both were done by the same trained optometrist masked to the drug. Each eye's refraction was converted to spherical equivalents (SEs), and the values averaged between the two eyes of each child under each drug. We compared SE with paired t-test (JASP 16.4). In addition, we performed correlational analysis, and looked for agreement using the Bland-Altman plot. Significance was set at P < 0.05. Wherever possible, 95% confidence intervals (CIs) are quoted. RESULTS: The mean SE with atropine was +1.93 ± 2.0 D, compared to +1.75 ± 1.95 D under cyclopentolate. On average, atropine induced greater cycloplegia by a mere 0.18 D (95% CI: 0.07 to 0.29 D, P value 0.002). The two cycloplegic refractions correlated significantly (Pearson's r: 0.975, P < 0.001). The Bland-Altman plot revealed the limits of agreement as 1.06 and -0.71 D. CONCLUSION: Our study suggests that cyclopentolate works for the most part as well as atropine to attain cycloplegia. Atropine may be considered for children less than 15 years of age with greater than 5.0 D of hyperopia. Cycloplentolate, with its advantages of quick action and short duration, should form the first go-to topical cycloplegic in busy outpatient clinics.

Comparison of the new self-contained darkroom refractive screener versus table-top autorefractor and cycloplegia retinoscopy in detecting refractive error.

PURPOSE: By comparing the results of the new self-contained darkroom refractive screener (YD-SX-A) versus table-top autorefractor and cycloplegic retinoscopy, to evaluate the performance of the YD-SX-A in detecting refractive error in children and adolescents and then judge whether it can be used in refractive screening. METHODS: Cross-sectional study. 1000 participants between the ages of 6 and 18 who visited the Optometry Center of the People's Hospital of Guangxi Zhuang Autonomous Region from June to December 2022 were selected. First, participants were instructed to measure their diopter with a table-top autorefractor (Topcon KR8800) and YD-SX-A in a noncycloplegic setting. After cycloplegia, they were retinoscopy by a professional optometrist. The results measured by three methods were collected respectively. To avoid deviation, only the right eye (1000 eyes) data were used in the statistical analysis. The Bland-Altman plots were used to evaluate the agreement of diopters measured by the three methods. The receiver operating characteristic (ROC) curves was used to analysis effectiveness of detecting refractive error of YD-SX-A. RESULTS: The average age of participants was 10.77 ± 3.00 years, including 504 boys (50.4%) and 496 girls (49.6%). When YD-SX-A and cycloplegia retinoscopy (CR) were compared in the myopia group, there was no statistical difference in spherical equivalent (SE) (P > 0.05), but there was a statistical difference in diopter spherical (DS) and diopter cylinder (DC) (P < 0.05). Comparing the diopter results of Topcon KR8800 and CR, the difference between each test value in the myopia group was statistically significant (P < 0.05). In the hyperopia group, the comparison between YD-SX-A and CR showed no statistically significant differences in the DC (P > 0.05), but there were significant differences in the SE and DS (P < 0.05). In the astigmatism group, the SE, DS, and DC were statistically different, and the DC of YD-SX-A was lower than that of CR and Topcon KR8800. Bland-Altman plots indicated that YD-SX-A has a moderate agreement with CR and Topcon KR8800. The sensitivity and specificity of YD-SX-A for detecting myopia, hyperopia and astigmatism were 90.17% and 90.32%, 97.78% and 87.88%, 84.08% and 74.26%, respectively. CONCLUSION: This study has identified that YD-SX-A has shown good performance in both agreement and effectiveness in detecting refractive error when compared with Topcon KR8800 and CR. YD-SX-A could be a useful tool for large-scale population refractive screening.

Ross syndrome with chronic cough and RF positivity: a case report.

Ross syndrome is a rare disorder of unknown etiology, characterized by the triad of segmental anhidrosis, tonic pupil, and areflexia/hyporeflexia. Ross syndrome is thought to be a limited and selective ganglioneuropathy. Its etiology has not been fully elucidated. Autonomic findings may also accompany. We wanted to present our 25-year-old patient who was diagnosed with Ross syndrome and presented with complaints of inability to sweat, heat intolerance, headache, diarrhea and chronic cough. Keyword: cough, tonic pupil, anhidrosis, compensatory.

Comparability of the Retinomax K-plus 3 handheld autorefractometer in quick mode versus on-table autorefractometer in standard mode.

INTRODUCTION: This study analyzes the comparability of measurements taken by a Retinomax K-plus 3 handheld autorefractometer in Quick mode and a Topcon KR-800 on-table autorefractometer in standard mode on the pediatric population, and establishes their correlation. METHODS: It is a retrospective comparative study. Spherical diopter power (SPH), cylindrical diopter power (CYL), angle of cylindrical axis (AX), and spherical equivalent (SE) were measured with the Retinomax in Quick mode and with the Topcon in standard mode. Each patient was evaluated in cycloplegic and non-cycloplegic conditions by both autorefractometers. Student's t-test was performed between the two instruments for SPH, CYL, and SE. The Pearson correlation coefficient was calculated and the dispersion was represented using Bland-Altman graphs, also evaluating the subgroup of patients under 4 years of age. A descriptive analysis of the percentages of measures that differed was performed. RESULTS: It included 98 eyes of 49 subjects (age range: 3-16 years). The data for HPS without cycloplegia are virtually identical, whereas with cycloplegia there is a hyperopic bias of +0.5 diopters measured with Retinomax. CYL results are very similar with and without cycloplegia. There is a high Pearson correlation for both instruments (>0.91) and a low degree of dispersion in the Bland-Altman plots under cycloplegia. CONCLUSION: The Retinomax data were consistent with those obtained by Topcon. The Retinomax is a useful instrument for detecting refractive errors in children between 3 and 16 years of age.