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Purpose: To report the case and multimodal imaging findings of a healthy young woman who developed paracentral acute middle maculopathy 9 weeks after COVID-19., Methods: Case report. Ultra-widefield fundus photography, macular spectral domain optical coherence tomography, fluorescein angiography, and optical coherence tomography angiography were performed., Results: A 36-year-old woman went to the emergency department with sudden, painless, left eye vision loss. The only relevant medical history was COVID-19 9 weeks before. The best-corrected visual acuity was 20/200, a left eye relative afferent pupillary defect was present, and superficial hemorrhages throughout the macular area and peripheral retina were found. Nearly 4 hours after admission, the left eye best-corrected visual acuity recovered to 20/20 without relative afferent pupillary defect. Five days after presentation in the emergency department, the patient returned with recurrent left eye vision loss, with spontaneous recovery within 12 hours. Macular spectral domain optical coherence tomography revealed hyperreflectivity of the inner plexiform and inner nuclear layers, and the diagnosis of paracentral acute middle maculopathy was established. The patient started oral acetylsalicylic acid and oral prednisolone. The patient did not report any new episodes of vision loss, and there was a progressive resolution of abnormal fundus findings., Conclusion: SARS-CoV-2 infection increases the risk of vascular thrombotic events with possible involvement of the retinal circulation, and paracentral acute middle maculopathy may present as a possible complication. Ophthalmologists should be able to recognize it promptly through multimodal imaging findings.

Congenital ectropion uveae (CEU) is a rare anomaly of the embryonic development of the anterior segment of the eye. We report the case of a 5-year-old child with an undiagnosed CEU who was treated urgently for an acute angle closure attack., Case Description: A 5-year-old child was referred urgently for evaluation of anisocoria with mydriasis of the right eye and severe headache. Brain imaging with contrast injection was initially performed in the pediatric emergency department and ruled out central nervous system pathology. The initial examination of the right eye revealed an intraocular pressure (IOP) of 37mmHg, corneal edema, congenital ectropion uveae, mydriasis with pupillary block, a closed angle on gonioscopy, and a clear lens. The examination of the left eye was unremarkable, with no visible CEU. The initial management consisted of medical treatment with topical glaucoma drops and miotics and acetazolamide at 10mg/kg/d. Re-evaluation under general anesthesia showed persistent mydriasis and no resolution of the pupillary block. Filtering surgery was performed in the absence of a complete response to medical treatment, allowing control of IOP without drops and complete regression of the corneal edema., Discussion: CEU is a rare malformation, and pressure complications represent an insignificant proportion of pediatric glaucoma cases. The acute presentation of acute angle closure in this potentially blinding short-term setting, however, makes detection and management difficult in very young children in a great deal of pain. Only one similar case has been reported in the pediatric literature., Conclusion: Acute angle closure complicating CEU is exceptional and difficult to diagnose in a pediatric context. Parents of children with this predisposing condition should be informed of the need to consult urgently when clinical signs of elevated intraocular pressure appear., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Purpose: Traumatic optic neuropathy can have varying presentations. Blunt focal trauma can lead to optic nerve avulsion with underlying retinal findings. A case of partial optic nerve avulsion after finger poke injury leading to focal retinal ischemia is reported., Methods: Visual acuity, fundus photography with fluorescein angiography, and spectral-domain optical coherence tomography were performed to document the findings in a 16-year-old man who presented after a finger poke injury to the left orbit during a water polo match., Results: On initial presentation, examination revealed decreased visual acuity with a fixed left pupil and afferent pupillary defect by reverse. On slit-lamp examination of the left eye, a hyphema was present. Dilated fundus examination revealed layering vitreous hemorrhage over the posterior pole and an avulsed vitreous base. On follow-up, a gap temporal to the optic nerve head consistent with a partial optic nerve avulsion was noted once the vitreous hemorrhage cleared. Multimodal imaging revealed retinal ischemia temporal to the disc on fluorescein angiography with corresponding changes in the inner retinal layers and retinal nerve fiber layer using spectral-domain optical coherence tomography., Conclusion: Clinicians should have a high suspicion for optic nerve avulsion if a patient presents with new vitreous hemorrhage and afferent pupillary defect after a finger-poke injury. Optic nerve avulsion injury can cause retinal ischemia, likely because of interruption of retinal blood flow as a result of nerve shearing injury. Multimodal imaging can reveal focal retinal injury and aid in proper diagnosis and follow-up.

Abstract: We present a case of ametropia corrected by small incision lenticule extraction (SMILE) in a corectopia eye after cataract surgery. The patient's optical zone, nomogram, and centration were carefully considered. The intended corrections were -6.60 - 1.25 × 175 OD and -6.85 - 0.50 × 10 OS. M-sized and S-sized cones were recorded in the right and left eye, respectively. The diameter of the right eye optical zone was 7.00 mm, whereas that of the left eye was 6.50 mm. The corneal vertex was determined using the film mark method as the scanning center. Three months after surgery, the uncorrected distance visual acuities were 20/20 OD and 20/13 OS. The effective optical zone was 6.5 and 5.5 mm in the right and left eyes, respectively. It is feasible to correct ametropia with SMILE in patients with corectopia after intraocular lens implantation. The large optical zone will compensate for the visual disturbance., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2022 Contact Lens Association of Ophthalmologists.)

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 presented with sudden onset of pain and decreased vision in the right eye. On examination, she had intraocular pressure (IOP) of 50 mmHg, aggressive iris neovascularization (NVI) and 3-piece IOL. Fundus examination revealed pale disc with tessellated fundus and parapapillary atrophy. Vascular arcades were vertically stretched with avascular ischemic retina starting from the near periphery. Macula appeared thin and atrophic. An intravitreal injection of 0.05 mg/0.1 ml bevacizumab was given to the right eye followed by Ahmed glaucoma valve (AGV) implantation. Assessment of her brother revealed similar posterior segment changes. A subsequent urine analysis showed proteinuria and high albumin to creatinine ratio. Next-generation sequencing for LAMB2 gene revealed a homozygous c.4573 + 1 G > A variant confirming the diagnosis of Pierson syndrome. Conclusion : This case expands our knowledge on retinal ischemia in the setting of Pierson syndrome. Close monitoring after intraocular surgery is recommended to look for the development of NVG.

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., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 by North American Neuro-Ophthalmology Society.)

Purpose: To determine the characteristics and epidemiology of open globe injury (OGI) in a third referral centre over 11 years., Methods: The study was a retrospective observational study. The medical records of 978 OGI cases (a total of 998 eyes), admitted to Chiang Mai University Hospital, Thailand from January 2006 to December 2016, were reviewed., Results: The mean age (SD) of patients was 39.1 (17.5) years. Patients were predominantly male (867, 88.7%). The age with the highest incidence of injury ranged from 41 to 60 years, in both genders. Most injuries happened at the workplace (64.8%), with flying objects from electric grass trimmers (23.9%) being the major injury-related mechanism. Penetration injuries were found in 479 eyes (48.0%). In elderly group (aged >60 years), a higher proportion of a globe rupture injuries were observed (13.3%). In general, intraocular foreign body group presented with better initial visual acuity (VA) compared to other injury types (p< 0.001). In all types of injury, final VA was significantly improved after treatment. However, rupture and perforation globe injuries had significantly worse final VA outcomes, compared to penetration and intraocular foreign body injuries (p< 0.001). With multivariable regression analysis, poor initial VA (p< 0.001), type of injury (p= 0.028), positive relative afferent pupillary defect (RAPD) (p< 0.001), and endophthalmitis (p< 0.001) were associated with poor visual outcomes., Conclusions: Work-related eye injury is a major cause of OGI. Poor initial VA, endophthalmitis, presence of RAPD, and rupture were the predictors for poor final visual outcomes.

Competing Interests: Competing interests: None declared.

Purpose: To report a unique case of acute angle closure glaucoma in the setting of a subarachnoid hemorrhage due to a ruptured cerebral aneurysm., Materials and Methods: Observational case report and review of the literature., Results: A 75-year-old woman presented with blurry vision, nausea, vomiting, and left eye pain. She was found to have a complete third nerve palsy, with ptosis, exotropia, hypotropia, and a fixed mydriasis with resultant acute angle closure glaucoma. Pilocarpine was initiated, and neuroimaging revealed a subarachnoid hemorrhage from a ruptured posterior communicating artery aneurysm. The aneurysm was successfully coiled, and outpatient laser iridotomies were subsequently performed. Only 4 prior cases of acute angle closure glaucoma in the setting of a third nerve palsy have been reported in the literature. To our knowledge, this case is the first report of angle closure glaucoma in the setting of a subarachnoid hemorrhage., Conclusions: This case of a complete third nerve palsy in the setting of a subarachnoid hemorrhage leading to acute angle closure highlights the importance of ruling out this life-threatening diagnosis when neurological signs of increased intracranial pressure and cranial nerve palsies are present.

Background: Detection of a relative afferent pupillary defect (RAPD) by the swinging-light test can be challenging in clinical practice (dark eyes, anisocoria, dark environment). We developed a new method of RAPD quantification based on the recording of the infrared pupillary asymmetry (IPA) with a standard optical coherence tomography (OCT) device., Methods: The diagnostic value of the IPA for detection of the RAPD was determined by receiver-operating characteristic (ROC) curves and area under the curve (AUC)., Results: Twenty-nine subjects were included in this study (17 controls and 12 unilateral optic neuropathies). The IPA was significantly greater in unilateral optic neuropathies (0.39) compared to controls (0.18, p = 0.001). The diagnostic value was good with a ROC-AUC of 0.843. Importantly, the IPA correlated significantly with the inter-eye percentage difference of the macular ganglion cell-inner plexiform layer (mGCIPL) thickness (R = 0.53, p = 0.01). Assessment of the IPA took less than 30 s., Conclusion: The present data show that the IPA is a practical and rapid test that can be applied in a clinical setting. The IPA may be a valuable functional outcome measure for clinical trials, complementing structural retinal OCT data in a biological meaningful way. The IPA should be further investigated for suitability for optic neuritis treatment trials.

Purpose: To compare intraoperative complication rates, 1-year visual outcomes, and postoperative complication rates over the first postoperative year in eyes with and without pseudoexfoliation undergoing cataract surgery., Design: Prospective, comparative, interventional study., Participants: Nine hundred thirty eyes with cataract and uncomplicated pseudoexfoliation (without phacodonesis, clinically shallow anterior chambers, or pupil size <4 mm) and 476 controls with cataract but without pseudoexfoliation recruited from 4 centers of the Aravind Eye Care System in Southern India. The 2 groups were randomized separately to receive either a single-piece acrylic intraocular lens (IOL; SA60AT; Alcon Laboratories, Fort Worth, TX) or a 3-piece acrylic IOL (MA60AS; Alcon Laboratories). The pseudoexfoliation group also was randomized to receive or not receive a capsular tension ring., Methods: All eyes underwent phacoemulsification with IOL implantation and were followed up at 1 day, 1 month, 3 months, and 1 year after surgery., Main Outcome Measures: Association of pseudoexfoliation status with intraoperative complication rates, 1-year best-corrected visual acuity, and any other complications., Results: Mean ages were 63.0±6.9 years and 57.9±7.3 years in the pseudoexfoliation and control groups, respectively (P < 0.001). Pseudoexfoliation patients were more likely to be men (P = 0.014), to have a nuclear opalescence grade of more than 4 (P = 0.001), and to have a pupil size of less than 6 mm (P < 0.001) when compared with controls. Intraoperative complication rates were 2.9% and 1.9% in the pseudoexfoliation and control groups, respectively (P = 0.29). One-year postoperative best-corrected visual acuity was comparable (P = 0.09). Complication rates at 1 year were 2.7% and 2.5% in the pseudoexfoliation and control groups, respectively (P = 0.82). Average endothelial cell loss was 14.7% in the pseudoexfoliation group and 12.7% in the control group at 1 year (P = 0.066) when adjusting for age and nuclear opacity., Conclusions: Pseudoexfoliation eyes without shallow anterior chamber, small pupils, or apparent zonulopathy may represent eyes with lower risks of complications. Despite smaller pupils and denser cataracts, pseudoexfoliation eyes without clinically apparent preoperative zonulopathy were not at a higher risk of intraoperative or postoperative complications or worse visual outcomes after cataract surgery., (Copyright © 2018 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

A 7-year-old girl presented with watering and redness in the left eye for 2 months. Her parents reported poor vision in both eyes for 4 years. Visual acuity was finger counting at 1 m and finger counting close to face in the right and left eyes, respectively. Slit lamp examination of the right eye revealed corectopia, aphakia in the pupillary area, temporally subluxated clear crystalline lens, persistent pupillary membrane, irido-hyaloidal adhesion and poorly dilating pupil. Left eye revealed central corneal oedema with descemet scarring, anteriorly dislocated clear crystalline lens with lenticulo-corneal touch. Ultrasound examination of the left eye was normal. Hence a diagnosis of ectopia lentis et pupillae with left eye spontaneous anterior dislocation of the lens along with corneal decompensation was made. The child underwent microscope integrated intraoperative optical coherence tomography guided intralenticular lens aspiration with optical iridectomy in the left eye. Postoperative visual rehabilitation was done with aphakic glasses., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Background Laminin α 5 β 2 γ 1 (LM-521) is a major component of the GBM. Mutations in LAMB2 that prevent LM-521 synthesis and/or secretion cause Pierson syndrome, a rare congenital nephrotic syndrome with diffuse mesangial sclerosis and ocular and neurologic defects. Because the GBM is uniquely accessible to plasma, which permeates endothelial cell fenestrae, we hypothesized that intravenous delivery of LM-521 could replace the missing LM-521 in the GBM of Lamb2 mutant mice and restore glomerular permselectivity. Methods We injected human LM-521 (hLM-521), a macromolecule of approximately 800 kD, into the retro-orbital sinus of Lamb2 -/- pups daily. Deposition of hLM-521 into the GBM was investigated by fluorescence microscopy. We assayed the effects of hLM-521 on glomerular permselectivity by urinalysis and the effects on podocytes by desmin immunostaining and ultrastructural analysis of podocyte architecture. Results Injected hLM-521 rapidly and stably accumulated in the GBM of all glomeruli. Super-resolution imaging showed that hLM-521 accumulated in the correct orientation in the GBM, primarily on the endothelial aspect. Treatment with hLM-521 greatly reduced the expression of the podocyte injury marker desmin and attenuated the foot process effacement observed in untreated pups. Moreover, treatment with hLM-521 delayed the onset of proteinuria but did not prevent nephrotic syndrome, perhaps due to its absence from the podocyte aspect of the GBM. Conclusions These studies show that GBM composition and function can be altered in vivo via vascular delivery of even very large proteins, which may advance therapeutic options for patients with abnormal GBM composition, whether genetic or acquired., (Copyright © 2018 by the American Society of Nephrology.)

Our aim is to study the dynamics of pupillary abnormalities in varying severity of diabetic retinopathy. A non-interventional case-control study with 405 eyes of 244 subjects with diabetes, and 41 eyes of 26 subjects with no history of diabetes was done. Diabetes group was classified according to retinopathy severity: no retinopathy, mild non-proliferative diabetic retinopathy (NPDR), moderate NPDR, severe NPDR and proliferative diabetic retinopathy (PDR). After dark adaptation, pupil size and flashlight response were captured with an infrared camera. Baseline Pupil Diameter (BPD), Amplitude of Pupillary Constriction (APC), Velocity of Pupillary Constriction (VPC) and Velocity of Pupillary Dilatation (VPD). Compared to controls, mean BPD decreased with increasing severity of diabetic retinopathy. Mean APC in control group was 1.73 ± 0.37 mm and reduced in mild NPDR (1.57 ± 0.39, p = 1.000), moderate NPDR (1.51 ± 0.44, p = 0.152) and found to be significant reduced in severe NPDR (1.43 ± 0.48, p = 0.001) and PDR (1.29 ± 0.43, p = 0.008). Compared to controls, mean VPC decreased progressively with increasing severity of retinopathy, with a maximal difference in the PDR group. Mean VPD as compared to the control group was significantly reduced in the no DR (p = 0.03), mild NPDR (p = 0.038), moderate NPDR (p = 0.05), PDR group (p = 0.02). We found pupillary dynamics are abnormal in early stages of diabetic retinopathy and progress with increasing retinopathy severity.

Pierson syndrome is a congenital nephrotic syndrome with eye and neurologic defects caused by mutations in laminin β 2 ( LAMB2 ), a major component of the glomerular basement membrane (GBM). Pathogenic missense mutations in human LAMB2 cluster in or near the laminin amino-terminal (LN) domain, a domain required for extracellular polymerization of laminin trimers and basement membrane scaffolding. Here, we investigated an LN domain missense mutation, LAMB2-S80R, which was discovered in a patient with Pierson syndrome and unusually late onset of proteinuria. Biochemical data indicated that this mutation impairs laminin polymerization, which we hypothesized to be the cause of the patient's nephrotic syndrome. Testing this hypothesis in genetically altered mice showed that the corresponding amino acid change (LAMB2-S83R) alone is not pathogenic. However, expression of LAMB2-S83R significantly increased the rate of progression to kidney failure in a Col4a3 -/- mouse model of autosomal recessive Alport syndrome and increased proteinuria in Col4a5 +/- females that exhibit a mild form of X-linked Alport syndrome due to mosaic deposition of collagen α 3 α 4 α 5(IV) in the GBM. Collectively, these data show the pathogenicity of LAMB2-S80R and provide the first evidence of genetic modification of Alport phenotypes by variation in another GBM component. This finding could help explain the wide range of Alport syndrome onset and severity observed in patients with Alport syndrome, even for family members who share the same COL4 mutation. Our results also show the complexities of using model organisms to investigate genetic variants suspected of being pathogenic in humans., (Copyright © 2018 by the American Society of Nephrology.)

Background: To evaluate the mechanisms of acute angle closure (AAC) other than the pupillary block using ultrasound biomicroscopy., Design: Retrospective chart review., Participants: All patients who were diagnosed with AAC in Ramathibodi Hospital, Bangkok, Thailand, between June 2011 and February 2015 were enrolled., Methods: Seventy-two patients who were diagnosed with AAC underwent a detailed ocular examination. The diagnosed mechanism of AAC was confirmed by UBM and ocular biometry., Main Outcome Measures: Primary mechanism responsible for acute angle closure., Results: In 72 patients, the mean age was 62.33 ± 10.4 years, 18 (25%) patients were male and 54 (75%) patients were female. The primary mechanism of AAC was iridolenticular wrapping (crowded-angle (CR) plus anterior lens subluxation (LS)) in 49 eyes (68.1%), pupillary block (PB) in 17 (23.6%) eyes, and plateau iris (PL) in 6 (8.3%) eyes. Thirty (41.7%) out of 72 eyes without previous iridotomy before UBM examination were analyzed. The most common primary mechanism in this group was iridolenticular wrapping (20 eyes, 66.7%). There were seven (23.3%) eyes that had a pupillary block, and only three (10.0%) eyes had plateau iris syndrome as the primary mechanism of AAC. There was a single mechanism in 14 (46.7%) eyes, and there were combined mechanisms in 16 (53.3%) eyes., Conclusions: The most common mechanism contributing to AAC development in this Thai population was iridolenticular wrapping. From this study, we suggest that iridolenticular wrapping was the most common hidden mechanism beyond pupillary block among Thai patients., (© 2016 Royal Australian and New Zealand College of Ophthalmologists.)

Purpose: To evaluate the changes in dynamic pupillometry in patients with idiopathic overactive bladder (OAB)., Methods: The study included 40 female patients with idiopathic OAB and 40 healthy female volunteers as a control group. Demographic and clinical data were recorded. Dynamic pupillometric parameters were measured with a commercially available unit (MonPack One, Metrovision, France) at baseline and on the 30th day of treatment with an antimuscarinic treatment (drug-agent) (solifenacin 5 mg daily). Initial, minimum, maximum and mean pupil diameters, the latency and duration of contraction and dilatation of the pupil, the amplitude of contraction and dilatation velocity were automatically measured and compared between the groups., Results: There were no significant differences between two groups with respect to age and body mass index (p = 0.288, 0.755, respectively). The measurements of initial, minimum and mean pupil diameters were significantly lower in patients with OAB compared to healthy controls (p = 0.007, 0.002, 0.001, respectively). OAB patients had significantly longer latency of pupil dilatation, latency of pupil contraction and shorter duration of pupil contraction than control group (p = 0.028, 0.029, 0.021, respectively). After the antimuscarinic treatment, latency of pupil contraction, latency of pupil dilatation and duration of pupil contraction shortened significantly (all p < 0.001). Pupil dilatation velocity increased significantly during the treatment (p < 0.001)., Conclusions: The dynamic pupillometric findings in this study imply impaired autonomic dysfunction, mostly the increased parasympathetic action, in OAB patients and the modulatory effects of antimuscarinic treatment.

Background: Few studies have established surgical landmarks for endoscopic endonasal dissection of the intraconal orbital apex (OA)., Objective: We describe the optic nerve (ON) anatomy and its relationships, as seen during a fully endoscopic, endonasal approach to the medial intraconal OA., Methods: The study question was approached through a cadaver dissection and a radiographic study. Four formalin-fixed, latex-injected cadaver heads were dissected using transnasal endoscopic techniques. Dissection was performed using 0 degree and 30 degree nasal endoscopes and standard endoscopic sinus surgical instrumentation. A bi-nostril 4-handed technique was used. The anatomy of 8 medial OAs was evaluated and recorded. As the radiographic portion, 100 consecutively enrolled patient magnetic resonance images were evaluated, with particular attention given to the relationship of the ON to the medial rectus muscle (MRM) in 200 orbits., Results: Intraconally, the ON consistently coursed along the superior half of the MRM. Interestingly, the nerve was more easily identified from a superior approach after retracting the MRM inferiorly. With the identification of the nerve at the OA, carrying the dissection of the medial OA was easily accomplished with the ON as the guiding landmark. The radiographic portion of this study revealed a consistent relationship between the superior edge of the ON and the MRM. This relationship was maintained in the orbital apex in 98.4%-100% of the orbits examined., Conclusion: The superior edge of the optic nerve is consistently found coursing along the superior half of the MRM, facilitating facile identification and further dissection navigation., (Copyright © 2016 by the Congress of Neurological Surgeons)

Cyclic oculomotor nerve paresis is a rare and usually congenital disorder. It is characterized by unilateral third nerve paresis with periodic spasms causing eyelid elevation, miosis, and contraction of 1 or more of the extraocular muscles innervated by the third nerve. We report a 20-month-old girl who presented initially with a congenital partial right third nerve paresis without ptosis. She subsequently developed isolated cyclic spasms of the pupil followed several months later by permanent partial ptosis.

Background: Anecdotally, increasing number of patients are seen at Korle Bu Teaching Hospital (KBTH) with brain tumour. Neuro-ophthalmic symptoms and signs may help in timely diagnosis and intervention., Objective: To evaluate the neuro-ophthalmic and clinical characteristics of brain tumour in patients presenting at a tertiary hospital in Ghana., Study Design: A prospective case series involving 36 consecutive patients newly diagnosed with brain tumour from November 2010 to October 2011, at the Ophthalmology, Neurosurgery and Endocrine units of KBTH, Ghana. All patients had clinical diagnosis of brain tumour with confirmation by computerized tomography (CT) or magnetic resonance imaging (MRI). Thirteen patients had histological confirmation of diagnosis., Outcome Measures: Presenting Visual acuity, Colour vision, Visual fields and Cranial nerve deficits., Results: Data of 36 patients were analyzed. Ages ranged from 3 to 69 years, mean (SD) 42.56 (±16.6 years). Twenty-six (72%) were females. Tumours included pituitary adenoma (20, 55.5%), meningioma(10, 27.8%), choroid plexus tumour(1, 2.8%), medulloblastoma(1, 2.8%), craniopharyngioma(1, 2.8%), haemangioblastoma(1, 2.8%), thalamic tumour(1, 2.8%) and haemangioma(1, 2.8%). Histologically confirmed tumours included pituitary adenoma (9, 69.2%), meningioma (3, 23.1%), craniopharyngioma (1, 7.7%). One patient had both a pituitary adenoma and meningioma. Blurred vision (30, 83.3%), headache (28, 77.8%) and photophobia (13, 36.1%) were predominant symptoms. Commonest neuro-ophthalmic signs were impaired colour vision (62 eyes, 88.6%), optic atrophy (26, 74.3%), unilateral or bitemporal hemianopia (15, 41.5%) and relative afferent pupillary defect (12, 34.3%). Seven (19.4%) patients were visually impaired and nine (25%) blind. Thirty-three of 72 (45.8%) eyes had monocular blindness., Conclusions: Common neuro-ophthalmic characteristics were blurred vision, headache, impaired colour vision, optic atrophy, and relative afferent pupillary defect (RAPD). Significant numbers of patients were blind or visually impaired at presentation.

Frequently, in literature and curent practice, accessory iris membrane (AIM) and persistant pupillary membrane (PPM) are confused. Both AIM and PPM are congenital iris anomalies in which fine or thick iris strands arrise form the collarette and obscure the pupil. AIM, which is also called iris duplication, closely resembles the normal iris tissue in color and thickness and presents a virtual second pseudopupil aperture in the centre while PPM even in its extreme forms presents as a translucent or opaque membranous structure that extends across the pupil and has no pseudopupil. Mydriatiscs, laser treatment or surgery is used to clear the visual axis and optimize visual development. Surgical intervention is reserved for large, dense AIMs and PPMs. Our patient, a 29 year old male, has come with bilateral dense AIM, bilateral compound hyperopic astigmatism, BCVA OD = 0.6, BCVA OS = 0.4, IOP OU = 17 mmHg. To improve the visual acuity of the patient we decided to do a bilateral membranectomy, restoring in this way transparency of the visual axis. After surgery, the visual acuity improved to BCVA OD= 0.8, BCVA OS=0.8.

Purpose of Review: To summarize the multiple mechanisms responsible for angle closure in uveitis and to outline the management principles and treatment modalities., Recent Findings: Angle closure in uveitis is a heterogeneous disease with multiple mechanisms. Recent advances in anterior segment imaging have provided insights into the mechanisms of angle closure in uveitis. Uveitic eyes with angle closure from pupil block require surgical iridectomy with mobilization of the peripheral iris and viscogoniosynechiolysis of both posterior synechiae and peripheral anterior synechiae. Systemic conditions associated with uveitis can result in anterior displacement of the iris-lens diaphragm, and present as acute angle closure. Pupil block is not the predominant mechanism in these eyes, and management is primarily medical. Data are limited on the optimal treatment of angle closure in uveitis, and the role of glaucoma filtration surgery, cataract extraction, minimally invasive glaucoma surgery and newer modalities of cycloablation require evaluation., Summary: The management of angle closure in uveitis should adhere to the principles of managing both uveitic glaucoma and angle closure. Identification of the mechanism of angle closure in uveitic eyes may enable treatment to be targeted at the responsible mechanism.

Purpose: To report a case of an angle-closure glaucoma in a patient with Miller Fisher syndrome (MFS) without pupillary dysfunction., Methods: We present a case report of a 75-year-old male presenting with total ophthalmoplegia, complete bilateral ptosis, and gait disturbance. He was diagnosed with MFS without pupillary dysfunction, which precipitated unilateral acute angle-closure glaucoma (AACG) due to complete lid ptosis., Results: The initial ocular examination revealed hand motion in the right eye. Intraocular pressure, as assessed by Goldmann applanation tonometry, was 50 mm Hg, and gonioscopic findings revealed a closed angle on the right eye. After maximal tolerated medical therapy, laser peripheral iridotomy was performed. The unilateral AACG with MFS resolved without further incident., Conclusions: This is the first reported case of a patient with MFS without autonomic dysfunction and AACG. We believe that pupillary dysfunction or lid ptosis due to neurological disorders may increase the possibility of AACG.

Congenital fixed dilated pupils (congenital mydriasis) is characterized by hypoplasia or aplasia of the iris muscles, with absence of iris between the collarette and pupillary border, creating a scalloped pupillary margin. This condition has been reported in a multisystemic smooth muscle cell dysfunction syndrome, combined with congenital patent ductus arteriosus, cerebrovascular disease (Moya-moya-like), coronary artery disease, thoracic aorta aneurysm, and dysfunction of smooth muscle cells in organs throughout the body. All affected individuals carry a p.R179H heterozygous mutation in the ACTA2 gene. We add to the ophthalmologic involvement with 3 more patients. Congenital fixed dilated pupils is a rare condition and should alert ophthalmologists to the possibility of the coexistence of systemic life-threatening disorders.

We report a case in which laser-assisted cataract surgery was performed safely and successfully following manual division of posterior synechiae and mechanical dilation of the pupil with a Malyugin ring in a patient with an irregular, small stuck-down pupil., (Copyright © 2013 ASCRS and ESCRS. Published by Elsevier Inc. All rights reserved.)

Introduction: Orbital teratoma is a known cause of proptosis in neonates but it is rare in adults., Case Report: An adult female presented with primary orbital teratoma with a well formed tooth inside the multicystic mass. Anterior orbitotomy with cyst excision was done with retrieval of a canine tooth., Comment: This report documents a unique case of orbital teratoma presenting in an adult with well formed canine tooth inside. Microcornea, corectopia and cataract were other distinctive coexisting features of our case.

Purpose: To evaluate the efficacy of different treatment modalities in eyes with small pupils before femtosecond laser-assisted cataract surgery., Setting: Ruhr University Eye Clinic, Bochum, Germany., Design: Prospective clinical trial., Methods: Eyes with an intraoperative pupil size smaller than 5.5 mm received sequential treatments to achieve a pupil larger than 5.5 mm in 3 steps: (1) intracameral administration of epinephrine solution, (2) additional viscomydriasis, and (3) implantation of a Malyugin ring pupil expander. When a step enlarged the pupil to at least 5.5 mm, femtosecond laser-assisted cataract surgery with an anterior capsulotomy diameter of at least 4.5 mm and 350 μm nuclear fragmentation grids, ultrasound phacoemulsification, and intraocular lens implantation were performed. Main outcome measures were achieved preoperative pupil size in each sequential treatment group and analysis of intraoperative complications., Results: Of 850 eyes scheduled for cataract surgery, 40 received sequential treatments. To achieve a pupil larger than 5.5 mm, epinephrine was sufficient in 7% of the eyes; additional viscomydriasis was necessary in 25%, and the pupil expander was implanted in 68%. The most frequent comorbidities were pseudoexfoliation of the lens capsule (30.0%) and intraoperative floppy-iris syndrome (12.5%). Tongue-like lesions of the capsulotomies were detected in 5 eyes., Conclusions: The 3-step treatment allowed the surgeon to increase the efficiency and safety of femtosecond-assisted cataract surgery in eyes with a small preoperative pupil, providing good safety margins at the pupil boundary for the capsulotomy and the softened nuclear volume., (Copyright © 2013 ASCRS and ESCRS. Published by Elsevier Inc. All rights reserved.)

Purpose: To evaluate the results of phacoemulsification in eyes with a narrow pupil dilated with Malyugin Ring, in comparison with manual pupillary stretching with hooks., Setting: Department of Ophthalmology, Medical University of Lodz, Poland., Materials and Methods: The examined group (group 1) consisted of 23 eyes of 23 patients whose pupil was dilated using Malyugin Ring. The reference group (group 2) consisted of 17 eyes of 17 patients whose pupil was dilated manually by stretching with hooks. All patients underwent uneventful standard phacoemulsification. Patients were examined preoperatively, 1 day, and 1 month after the surgery. Best-corrected visual acuity (BCVA), intraocular pressure, anterior and posterior segment of the eye, and corneal endothelial cell density were evaluated. Statistical analysis was done using nonparametric tests., Results: Mean preoperative BCVA in group 1 was 0.31±0.27 and in group 2 it was 0.26±0.26 (p>0.05). In both groups there was a significant improvement in BCVA after the surgery (p<0.05). Mean postoperative BCVA in group 1 was 0.75±0.30 and in group 2 it was 0.56±0.56 (p<0.05). Mean corneal endothelial cell loss measured 30 days postoperatively amounted to 9.35±11% in group 1 and 13.77±8.0% in group 2 (p<0.05). No serious complications were found., Conclusions: In eyes with narrow pupil, the use of Malyugin Ring makes the surgery easier and allows for better functional results and smaller corneal endothelial cell loss in comparison with manual pupillary stretching with 2 hooks.

Objective: To demonstrate a unique abnormality of the pupillary light reflex in patients with autoimmune autonomic ganglionopathy (AAG)., Design: Case series., Setting: Autonomic clinics at 2 university hospitals (University of Texas Southwestern Medical Center and Beth Israel Deaconess Medical Center)., Participants: Seven patients with antibody-positive AAG., Interventions: All patients with AAG underwent either monocular or binocular infrared pupillometry using a standard 2-second light stimulus at a defined intensity. Findings were compared with those from healthy control subjects and patients with other autonomic disorders. The light stimulus used in this study was selected to eliminate the normal phenomenon of pupil escape., Main Outcome Measures: The time to onset of redilation as well as other indices of pupillary constriction to light stimulus., Results: Patients with AAG exhibited premature pupillary redilation (mean [SD], 1.02[0.20] seconds) compared with healthy control subjects (mean [SD], 2.24 [0.10] seconds) and other patients with autonomic disorders (mean [SD], 2.30 [0.12] seconds) (P.001). In healthy control subjects and patients with other autonomic disorders, pupillary redilation always followed the termination of the light stimulus; in patients with AAG, redilation consistently occurred during the light stimulus. In 1 patient, serial repetitive light stimulation further decreased the time to onset of redilation., Conclusions: Premature redilation of the pupil is a unique physiological feature seen only in patients with AAG. This phenomenon appears to be a manifestation of pupillary fatigue, a clinical correlate of defective synaptic transmission at the level of autonomic ganglia in antibody-positive AAG.

Pupillary abnormalities are commonly seen in patients presenting with severe traumatic brain injury (TBI). The objectives of this study were to determine the underlying condition responsible, the natural history of recovery of third nerve palsy and the ultimate clinical outcome in 60 patients admitted to a regional neurosurgical centre with a diagnosis of TBI and unilateral or bilateral fixed, dilated pupils (FDP). In approximately three-quarters of cases, some form of road traffic incident was the mechanism of injury. In patients presenting with a unilateral FDP, the CT-defined condition was most commonly diffuse brain injury (49%) with no obvious lateralising condition. In 34% of cases CT demonstrated a lateralising condition ipsilateral to the side of the FDP and in 9% cases the FDP was contralateral to the side of the CT abnormality. Of those patients who survived an FDP, 72% were left with some form of ophthalmological deficit. Most patients with bilateral FDP did not survive (88%); however, of those who did survive, none was left in a persistent vegetative state or with any ophthalmological sequelae. A FDP is a grave prognostic sign following TBI commonly resulting in long term ophthalmological sequelae; however, a favourable outcome is still attainable.

Aim: To investigate the functional and cosmetic outcomes of keratopigmentation (KTP) in cases of moderate to severe visual dysfunctions owing to different iris disorders., Methods: 11 eyes with moderate to severe visual disabilities related to iris defects underwent KTP for functional and cosmetic restoration using micronised mineral pigments and assisted with modern technologies such as femtosecond laser and new automated keratopigmentation instruments for the intrastromal and superficial application of the pigments., Results: Following the KTP surgery, the visual-function-related symptoms improved in all cases, from significant improvement to total elimination. Eight patients were asymptomatic after the surgery. In two patients, minimal non-disabling symptoms remained after surgery. One patient with traumatic aniridia complained of significant residual glare at the 3-month postoperative visit and was reoperated to reduce the simulated pupil to 4 mm. The cosmetic outcomes were analysed and classified as excellent in eight patients and good in three., Conclusion: KTP using new micronised mineral pigments and new surgical protocols has proven in this series to be an effective surgical technique for the management of moderate to severe visual dysfunctions related to iris defects.

Background: Previous authors have suggested that an afferent pupillary defect (APD) may serve as an effective screening tool for some specific eye diseases, especially glaucomatous optic neuropathy, since the disease usually presents asymmetrically. Its success as a screening tool for glaucoma has never been previously prospectively evaluated in a large population., Methods: In this study, the authors carry out assessments for the presence of APD as it relates to the diagnosis of glaucoma in an existing population-based eye study in southern India. The authors calculate the sensitivity and specificity for APD, both at the level of a village/household screening and in a more comprehensive/hospital setting, as it pertains to its ability to predict glaucomatous optic neuropathy., Results and Discussion: The authors find that APD assessed via the swinging flashlight test is a poor screening tool for glaucoma in this setting.

Ocular coloboma is sometimes accompanied by corectopia in humans and therefore ectopic pupil may indicate ocular coloboma in experimental animals. The RCS strain of rats has a low incidence of microphthalmia. We found that inferior ectopic pupil is associated exclusively with small-sized eyes in this strain. The objective of the current study was to evaluate whether inferior ectopic pupil is associated with iridal coloboma and other types of ocular coloboma in RCS rats. Both eyes of RCS rats were examined clinically, and those with inferior ectopic pupils underwent morphologic and morphometric examinations. In a prenatal study, coronal serial sections of eyeballs from fetuses at gestational day 16.5 were examined by using light microscopy. Ectopic pupils in RCS rats were found exclusively in an inferior position, where the iris was shortened. Fundic examination revealed severe chorioretinal coloboma in all cases of inferior ectopic pupil. The morphologic characteristics closely resembled those of chorioretinal coloboma in humans. Histopathologic examination of primordia showed incomplete closure of the optic fissure in 4 eyeballs of RCS fetuses. Neither F(1) rats nor N(2) (progeny of RCS × BN matings) displayed any ocular anomalies, including ectopic pupils. The RCS strain is a suitable model for human ocular coloboma, and inferior ectopic pupil appears to be a strong indicator of ocular coloboma.

Purpose: Observations of multiple ocular malformations together with heterozygosity for galactosaemia in siblings and homozygosity in one child are highly unusual. In these case histories, a series of investigations in one family are reported., Methods: Members of a family of two brothers and one sister and their children were pre- and post-surgically examined over several years. Blood examination was carried out in a laboratory specializing in investigation into genetic diseases (Dr Podskarbi, Munich)., Results: Two brothers and one sister suffered from cataract-induced visual deterioration at 38, 34 and 35 years of age, respectively. All three siblings reported having had bilateral poor vision since early childhood. The three siblings' parents had no congenital ocular malformations, nor was there any parental consanguinity. One child, the 10-year-old son of the 35-year-old sister, exhibited classic galactosaemia and normal ocular findings. This sister's other child was healthy. All three siblings presented congenital lens luxation, axial myopia, cataract and iridodonesis. In addition, the 34-year-old brother showed unilateral right corectopia and left coloboma adjacent to the optic disc. The 38-year-old brother revealed myopic fundus changes, but no coloboma. The three siblings experienced a distinct increase in visual acuity after cataract surgery. Both eyes of the patients were partially or distinctly amblyopic, respectively. We assume an autosomal-recessive transmission. Molecular genetic examination of the 10-year-old child with classic galactosaemia showed homozygosity for the mutation Q188R with a complete galactose-1-phosphate-uridyltransferase (GALT) deficiency. Because of his galactose-free diet, the child showed normal values for galactose-1-phosphate. The 35-year-old mother showed compound heterozygosity for Q188R and G1391A (D2/G). The 10-year-old boy's father also revealed heterozygosity for galactosaemia caused by GALT deficiency. The two children of the 38-year-old brother were heterozygous for G1391A. They did not show any clinical abnormality. None of the family members had clinical signs of Marfan's syndrome or homocysteinuria. The three siblings' parents were not consanguineous., Conclusions: Patients with worsening cataracts occurring at a pre-senile age should be examined for galactosaemia. We describe for the first time the molecular genetic findings in congenital ectopia lentis et pupillae. Early treatment in conjunction with a galactose-free diet is mandatory in patients with galactosaemia. Members of a family with heterozygosity for galactosaemia should be advised to attend a human genetic consultation., (© 2010 The Authors. Journal compilation © 2010 Acta Ophthalmol.)

Intraoperative floppy iris syndrome (IFIS) occurring during cataract surgery in adults has been widely reported in association with tamsulosin and other α-1(a) adrenergic antagonists; however, only one case of pediatric IFIS has been previously reported and was associated with congenital cataract. We report a case of a 1-month old girl with IFIS associated with bilateral persistent pupillary membranes without cataracts and the use of preoperative topical phenylephrine and pilocarpine for IFIS prophylaxis., (Copyright © 2011 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

Pediatric glaucoma may coexist with other anterior segment disorders, and when 2 surgeries are indicated in children, they are generally performed on separate days. We present 3 cases, documented with video, that illustrate the combination of anterior segment surgery (including cataract extraction and papillary membrane removal) with fiber-optic microcatheter trabeculotomy in infants. In one case, the microcatheter procedure could not be completed, and a trabeculotome was used to complete the trabeculotomy. One patient developed a mild hyphema after microcatheter trabeculotomy, which resolved. All cases were performed without other complications and with satisfactory results., (Copyright © 2011 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

Purpose: To study the mechanisms of pupillary block in eyes with occludable angle by ultrasound biomicroscopy., Methods: Initially, a pilot study of 13 eyes with acute primary angle-closure without medication was executed. Ultrasound biomicroscopy measurements of the angle, posterior chamber depth and iris thickness were performed in the temporal quadrant under light and dark conditions. Afterwards, ultrasound biomicroscopy measurements of iris-lens contact distance and iris-lens angle in the temporal quadrant and central anterior chamber depth were made in 32 eyes with acute primary angle-closure or intermittent primary angle-closure without medication, under light and dark conditions before and after laser peripheral iridectomy., Results: In the pilot study, a significant decrease in the angle as well as a significant increase in the iris thickness occurred when comparing light to dark conditions. Before and after laser peripheral iridectomy (second study), significant differences were found in iris-lens contact distance (P<0.001) and iris-lens angle (P<0.001) under light and dark conditions. Also, significant differences were found in light and dark conditions, before laser peripheral iridectomy, in iris-lens angle (P=0.005), and after laser peripheral iridectomy, in iris-lens contact distance (P<0.001). No significant change occurred with anterior chamber depth., Conclusions: A decreased angle was correlated to an increase in iris thickness. After laser peripheral iridectomy, acute primary angle-closure or primary angle-closure eyes had an increased iris-lens contact distance and a decreased iris-lens angle. The anterior chamber depth did not change. These findings contradict the theory that pupillary block is the mechanism of acute primary angle-closure.