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A 56-year-old man with a medical history of alcoholic cirrhosis presented with acute bilateral loss of vision. On admission, the patient had pale skin and low arterial pressure. Ophthalmic examination demonstrated a visual acuity of 6/9 in the right eye and the absence of light perception in the left. Automated perimetry revealed a superior altitudinal defect in the right eye. Optic disc swelling, flame-shaped hemorrhages, and several cotton-wool spots were evident in both eyes on fundoscopy. Lab results confirmed severe anemia. Following prompt correction of the anemia, the altitudinal defect remained unchanged. However, visual acuity in the right eye improved significantly in a few days. The potential association of anemia with both papilledema and non-arteritic anterior ischemic optic neuropathy (NA-AION) is discussed, with a focus on possible pathophysiological mechanisms. The necessity for routine anemia screening, encompassing complete blood count, serum iron, and vitamin B12 levels, and subsequent rapid correction in these patients, is emphasized.

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Dolichoectatic arterial compression of the anterior visual pathway is a rare but recognized condition. The functional impact, however, is still less clear. We describe a case of a 56-year-old male with diminished visual acuity in the left eye and incongruent inferior visual defect in both eyes caused by dolichoectasia due to a congenital hypoplasia of one horizontal segment of the circle of Willis and subsequent arterial compression of the chiasm and left optic nerve.

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Emerging anticancer agents such as the pan-FGFR Inhibitor have achieved remarkable improvements in the survival of patients with metastatic malignancies. Nevertheless they are still associated with specific ophthalmic toxicities. Understanding their pathophysiology can lead us to better clinical practice of life-threatening and vision-threatening circumstances. To investigate choroidal alterations as a potential pathophysiological mechanism of a serous detachment in bilateral pan-FGFR Inhibitor-Associated Retinopathy (FGFRAR), the morphology of the choroid and choriocapillaris were assessed. The choroidal thickness (ChT) and choriocapillaris flow void were measured by macular optical coherence tomography (OCT) and angiography (OCT-A), respectively. Data were collected at the baseline, then at one-month and two-months follow-ups after starting erdafitinib, in a single case of pulmonary angiosarcoma. Choroidal and choriocapillaris morphology showed stable ChT and choriocapillaris flow void at FGFRAR onset and relapse. To the best of our knowledge, this is the first analyzed case reported with flow-void OCT-angiography. Considering these results, FGFRAR in this patient does not seem to match the pachychoroid spectrum disorder definition; rather, an intracellular mechanism based on intracellular transduction pathways may be at work.

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BACKGROUND: The use of immunomodulating therapy to treat various cancers has been on the rise and these immune checkpoint inhibitors are known to cause ocular side effects. In this article a case of acute exudative polymorphous vitelliform maculopathy (AEPVM) is reported which developed during a first line treatment with pembrolizumab. CASE PRESENTATION: A 54-year-old woman was referred because of blurry vision in both eyes with a yellow spot in the central visual field of the left eye. These symptoms started after four treatments with pembrolizumab (a monoclonal antibody against the programmed cell death receptor-1) for a metastatic recurrent vaginal mucosal melanoma. Her best corrected visual acuity was 10/10 in both eyes with a correction of + 2.00 bilaterally. There were no inflammatory findings in the anterior segment or the vitreous. Fundoscopy revealed an attenuation of the foveal reflex with subtle yellow-white subretinal macular deposits (vitelliform lesions) in both eyes. Fluorescein angiography did not show staining or leakage in the mid-phase, neither a late staining. Spectral-domain optical coherence tomography of the macula illustrated bilateral neurosensory retinal detachment with a thick, highly reflective band at the outer photoreceptor segment. En face structural OCT at the level of the photoreceptors showed focal areas of increased signal corresponding to hyperreflective vitelliform material. The treatment with pembrolizumab was ceased immediately. During the following visits we slowly saw an improvement of the neurosensory retinal detachment. After almost four months a total resolution of the subretinal fluid was visualized in both eyes without the use of additional treatment, though the vitelliform deposits persisted. CONCLUSIONS: The development of AEPVM in melanoma patients could be triggered by treatment with Pembrolizumab. Pembrolizumab has the potential to disturb indirectly the retinal pigment epithelium homeostasis with accumulation of lipofuscin deposits and subretinal fluid, both signs of AEPVM.

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A 5-year-old boy presented with periorbital swelling due to craniofacial emphysema after a dental procedure. Computed tomography also revealed emphysema of the mediastinum. Pneumoparotitis and anesthesia mumps were considered in the differential diagnosis; however, Stensen's duct as well as the parotid itself appeared normal. It was determined that the most probable cause was dissection of soft tissues around the tooth using air-driven dental handpieces or air syringes, causing air to spread from the buccal, sublingual, and submandibular spaces to the mediastinal compartment inferiorly and cervicofacial compartment superiorly.

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PURPOSE: The present study investigates by optical coherence tomography angiography (OCTA) the retinal capillary plexus and choriocapillaris flow voids and their possible correlation with MEKAR. METHODS: 34 eyes of 17 patients (61.5 years [30.4-77.4]) with stage IV cutaneous melanoma were included prospectively. All patients showed disease progression under treatment with Nivolumab/Ipilimumab and were subsequently treated with the MEK-inhibitor Trametinib 2 mg once daily. At the start and every 6 weeks during follow-up of 4 months, patients underwent a complete ophthalmologic exam, OCTA and when needed fluorescein angiography. RESULTS: Statistical analysis was performed on 17 eyes of 9 patients. Eight patients were excluded due to missing OCTA images or due to drop-out because of decease or change of treatment. Comparing vessel area density (P = .625 and 0.681, respectively), vessel skeleton density (P = .996 and 0.766, respectively) of the superficial and deep capillary plexus, flow void number and total flow void area (mm2 and %) (P = .495; 0.197 and 0.298, respectively) of choriocapillaris slab, before and after treatment, revealed no significant difference. The evolution of choriocapillaris flow void parameter did not significantly differ in patients, who developed MEKAR compared to patients who did not. CONCLUSION: In patients receiving MEK-inhibitor with and without MEKAR, no significant different characteristics of the retinal capillary plexus and choriocapillaris were found. These data suggest that the development of MEKAR, has no correlation with vascular alteration.

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ABSTRACT: Immune checkpoint inhibition has improved the clinical outcomes for numerous patients with cancer. However, the downside is a whole new spectrum of immune-related adverse events. We report a 68-year-old man with a history of nonsmall cell lung cancer presenting with a spontaneous corneal perforation in the right eye after 22 cycles of pembrolizumab. In addition, a chronic central nonhealing epithelial defect developed after performing a penetrating keratoplasty. Treatment with autologous serum drops resulted in complete healing of the corneal ulcer, where other conventional therapies had no effect. One month after reinitiating pembrolizumab therapy, our patient presented again with a corneal perforation in the fellow eye. This case describes relapsing sterile ulcerations associated with pembrolizumab use and presents an unexpected cure.

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PURPOSE: Besides chiasmal hemidecussation, interhemispheric connections are likely important in human binocularity. The corpus callosum (CC) is the major fiber bundle in the mammalian brain which mostly connects homologous cortical areas in the two hemispheres. Visual interhemispheric connections were found abnormal in strabismic cats. No studies have investigated these pathways in humans with infantile strabismus. METHODS: Diffusion tensor imaging was used in four subjects with infantile esotropia (IE) and nine control subjects with normal binocularity, in order to study interhemispheric fibers in the CC connecting the right and left primary visual cortical areas. RESULTS: The number of callosal fibers linking both visual cortical areas between the hemispheres was found to be higher in subjects with IE. Also in IE, the amount of visual callosal fibers found after analysis from the primary visual cortical areas on one side appeared significantly different from the amount starting from the contralateral primary visual areas. The distribution area on one side is wider. CONCLUSION: We show callosal visual fibers to be abnormal in human IE. Subjects with IE showed abnormal numbers of transcallosal fibers connecting the visual cortical areas on both sides which likely results from an abnormal elimination process during development. Pruning of these fibers in IE favors the side of the visual cortex ipsilateral to the dominant eye. This study underlines the likely role of the CC in the development of human binocularity.

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Over 150 million years, modern reptiles, birds and mammals evolved. Predatory birds and mammals have eyes on the front of their heads. In these animals, binocular correspondence, voluntary eye movements, eye-hand coordination, partial decussation at the optic chiasm and cortical interhemispheric pathways could develop at the cost of a smaller visual field. The subsequent enlargement of the cerebral neocortex and the hemispheric pathways are of central importance in binocular vision, sensory fusion and infantile strabismus. To investigate visual interhemispheric fibers, tractography was used in subjects with infantile esotropia (IE), callosal agenesis and control subjects with normal binocularity. In human callosal agenesis, normal binocularity could be explained by a different interhemispheric connection. In subjects with infantile esotropia, the analyses starting from the primary visual area on one side appeared different from the analysis from the other side. The distribution areas are asymmetrical between sides. Binocularity and alignment not only rely on correspondence between crossed and uncrossed hemi-representations but, in addition the corpus callosum, the most important interhemispheric pathway in placental mammals, seems important in the development of human binocularity. When correspondence is possible between the hemi-representations of the crossed and the uncrossed pathway on both sides of the cortex, the uncrossed pathway finds its way via the corpus callosum toward the contralateral hemi-representation of the same eye. A vertical midline must come out of this process. Meanwhile, however, the other uncrossed pathway strives to find the vertical midline of the other eye. Likely, the corpus callosum is important during the tuning of the pathways involved in sensory fusion. The anterior commissure might be an alternative in callosal agenesis. A failure in the offset will result in strabismus and crossed dominance after V1. Consequently, the normal binocular development of the brainstem (i.e., the superior colliculus), will not take place and latent nystagmus, as well as dissociated divergence, may ensue.

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PURPOSE: Two Caucasian Belgian families were diagnosed with autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). The ophthalmological findings in both ARSACS disease and carriers are described. METHODS: In addition to a complete ophthalmological assessment, in both patients and carriers, spectral-domain Optical Coherence Tomography scans of the peri-papillary retinal nerve fiber layer were performed. RESULTS: Molecular analysis revealed a missense mutation which has not been reported before. Besides patients with ARSACS, who also presented additional ophthalmological abnormalities i.e. eye movement problems, ARSACS carriers demonstrated thickening of the retinal nerve fiber layer. CONCLUSION: The most conspicuous ophthalmological feature of ARSACS is an increased thickness of the peri-papillary retinal nerve fiber layer. Retinal striation and thickening of the nerve fiber layer on spectral-domain Optical Coherence Tomography appeared also in carriers of the ARSACS-gene. Other ophthalmological features encountered, were gaze-evoked nystagmus and rebound nystagmus.

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Aim: In humans, images in the median plane of the head either fall on both nasal hemi-retinas or on both temporal hemi-retinas. Interhemispheric connections allow cortical cells to have receptive fields on opposite sides. The major interhemispheric connection, the corpus callosum, is implicated in central stereopsis and disparity detection in front of the fixation plane. Yet individuals with agenesis of the corpus callosum may show normal stereopsis and disparity vergence. We set out to study a possible interhemispheric connection between primary visual cortical areas via the anterior commissure to explain this inconsistency because of the major role of these cortical areas in elaborating 3D visual perception. Methods: MRI, DTI and tractography of the brain of a 53-year old man with complete callosal agenesis and normal binocular single vision was undertaken. Tractography seed points were placed in both the right and the left V1 and V2. Nine individuals with both an intact corpus callosum and normal binocularity served as controls. Results: Interhemispheric tracts through the anterior commissure linking both V1 and V2 visual cortical areas bilaterally were indeed shown in the subject with callosal agenesis. All other individuals showed interhemispheric visual connections through the corpus callosum only. Conclusion: Callosal agenesis may result in anomalous interhemispheric connections of the primary visual areas via the anterior commissure. It is proposed here that these connections form as alternative to the normal callosal pathway and may participate in binocularity.

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A 72-year-old woman presented with a painful right eye. A few weeks before, she had noticed a red, swollen area in the conjunctiva of the same eye. On slit lamp examination, it appeared as chemosis and vascular injection; artificial tears were prescribed. A month later, a firm mass developed on the superotemporal orbital rim, in the area of the lacrimal gland. A CT scan revealed infiltrative structures in both the left and right orbit, with contrast staining in the right lacrimal gland and near the left optic nerve. A biopsy was taken of the conjunctival swelling as well as of the lacrimal gland. Both tissues showed infiltration with lobular breast carcinoma metastases.

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A closer look at the evolution of the eye and the brain provides a possible explanation for both the origin of infantile esotropia and its motor characteristics. In the course of evolution, the eyes have moved from a lateral to a frontal position. Consequently, the monocular visual fields started to overlap resulting in a binocular visual field. In lateral-eyed animals, the retinae project to the contralateral visual cortices only. These projections are also found in binocular mammals and birds with binocular visual fields but in addition there are uncrossed projections from the temporal retinae to the visual cortex. The partial chiasmal decussation and the corpus callosum provide the necessary structure that allows binocular vision to develop. Disruption of normal binocular development causes a loss of binocularity in the primary visual cortex and beyond. Beyond the primary visual cortex, the contralateral eye dominates while the temporal retinal signal appears to lose influence. Loss or absence of binocular vision in infantile esotropia may be caused by inadequate retinotopic matching between the nasal and temporal retinal signals like in albinism with an abnormal or asymmetric chiasmal decussation or agenesis of the corpus callosum. Dominance of the crossing retinal signal might also explain the motor characteristics of infantile esotropia (asymmetric OKN, latent nystagmus, DVD). A normal binocular cortical signal will predominate over the evolutionary older, originally non-binocular, retinal projections to the superior colliculi (CS) and the accessory optic system (AOS). A suppressed temporal retinal signal paves the way for the re-emergence of eye movements driven by one eye, as in lateral-eyed non-binocular animals.

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Both see-saw nystagmus and dissociated vertical divergence are cyclovertical eye movements characterized by vertical disconjugation and torsional conjugation. See-saw nystagmus is known to occur with chiasmal disorders and bitemporal hemianopia. Dissociated vertical divergence is commonly encountered in the infantile strabismus syndrome. A hypothetical model is presented in which both conditions are explained. The basic organization of the oculomotor system is most likely monocular and synchronous eye movements may have developed by neuronal coupling of the symmetrical oculomotor structures. The vertical dissociation of both eye movement disorders is explained by insufficiently developed neuronal coupling between the superior colliculi. A functional differentiation between crossed and uncrossed retinal ganglion cells fibers is assumed to cause this diminished binocular coupling in the case of see-saw nystagmus. The interstitial nucleus of Cajal may well play a pivotal role in explaining the distinct torsional eye movements in both conditions.

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Dissociated vertical divergence (DVD) is commonly encountered in the infantile strabismus syndrome. The movement is said to be dissociated since alignment differs between right and left eye fixation. It has been hypothesized that DVD is caused by a primitive reflex present in fish. Visual pathways in the fish brain indeed are dissociated with complete crossing at the optic chiasm and projection to the superior colliculus instead of a visual cortex. Neuroanatomical data and clinical characteristics of DVD, however, contradict this hypothesis. From these data a new hypothesis arises: corticotectal lateralization. The absence of binocular development in the cerebral cortex in infantile strabismus gives rise to dissociated activation of subcortical pathways through individual less tightly linked colliculi.

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