Commentary: Profiling Children With Cerebral Visual Impairment (CVI) Using Multiple Methods of Assessment to Aid in Differential Diagnosis.

PROFILING CHILDREN WITH CEREBRAL VISUAL IMPAIRMENT USING MULTIPLE METHODS OF ASSESSMENT TO AID IN DIFFERENTIAL DIAGNOSIS: Amanda H. Lueck , Gordon N. Dutton , Sylvie Chokron Seminars in Pediatric Neurology Volume 31, October 2019, Pages 5-14 Cerebral (cortical) visual impairment (CVI), the primary cause of visual impairment in chil dren in high-income countries, is increasing globally due to improved life-saving measures for premature and full-term infants. Yet the consequences of this condition are only begin ning to be understood and addressed. According to the topography, site, and the extent of the pathology, the deficit may variably concern central visual functions, visual field, percep tion of movement, visual analysis, visual exploration, attention, or visual memory, as well as visual guidance of movement. Each affected child has a unique clinical picture, which needs to be identified and individually profiled. This is probably the underlying reason that CVI is commonly underdiagnosed or misdiagnosed, especially in children, and, as a consequence, the full range of potential behavioral outcomes are not identified and adequately addressed. The present paper shows how the use of multiple methods of assessment can improve understanding of children with CVI.

From vision to cognition: potential contributions of cerebral visual impairment to neurodevelopmental disorders.

Vision has a crucial role to play in human development and functioning. It is, therefore, not surprising that vision plays a fundamental role in the development of the child. As a consequence, an alteration in visual function is, therefore, likely to hinder the child's development. Although ocular disorders are well known, diagnosed and taken into account, cerebral visual impairments (CVI) resulting from post-chiasmatic damage are largely underdiagnosed. However, among the disorders resulting from an episode of perinatal asphyxia and/or associated with prematurity, or neonatal hypoglycaemia, CVIs are prominent. In this article, we focus on the role of the possible effects of CVI on a child's learning abilities, leading to major difficulty in disentangling the consequences of CVI from other neurodevelopmental disorders (NDD) such as dyslexia, dyscalculia, dysgraphia, attention-deficit/hyperactivity disorder (ADHD), developmental coordination disorder (DCD) and autism spectrum disorders (ASD). Although we focus here on the possible overlap between children with CVI and children with other NDD, De Witt et al. (Wit et al. Ear Hear 39:1-19, 2018) have raised exactly the same question regarding children with auditory processing disorders (the equivalent of CVI in the auditory modality). We underline how motor, social and cognitive development as well as academic success can be impaired by CVI and raise the question of the need for systematic evaluation for disorders of vision, visual perception and cognition in all children presenting with a NDD and/or previously born under adverse neurological conditions.

A new look at the safety and tolerability of prostaglandin analogue eyedrops in glaucoma and ocular hypertension.

INTRODUCTION: In the last 25 years, topical prostaglandin analogues (PGAs) have emerged to become first line and first choice therapeutic options in the management of glaucoma and ocular hypertension (OHT). Although the short-term efficacy and safety of PGAs has been extensively investigated, less is known about their long term safety and tolerability. This gap in current knowledge is clinically relevant, because treatment-related adverse events and long-term tolerability issues are key determinants of the overall success of long-term therapy and the final outcome of a lifelong, symptomless disease like glaucoma. AREAS COVERED: We include selected evidence pertaining to the safety and tolerability of available and emerging PGA formulations. We also outline PGA formulations with different concentrations of the active ingredient, different preservatives, and preservative-free (PF) options. EXPERT OPINION: Undoubtedly PGAs will continue to play a major role in the medical therapy of glaucoma and OHT. Despite extensive literature and prolonged clinical experience with these agents worldwide, a number of areas that warrant further research have been identified in the present review. Recently launched novel PGAs, or those still in development offer new opportunities and future challenges.

Cortical Visual Impairments and Learning Disabilities.

Medical advances in neonatology have improved the survival rate of premature infants, as well as children who are born under difficult neurological conditions. As a result, the prevalence of cerebral dysfunctions, whether minimal or more severe, is increasing in all industrialized countries and in some developing nations. Whereas in the past, ophthalmological diseases were considered principally responsible for severe visual impairment, today, all recent epidemiological studies show that the primary cause of blindness and severe visual impairment in children in industrialized countries is now neurological, with lesions acquired around the time of birth currently comprising the commonest contributor. The resulting cortical or cerebral visual impairments (CVIs) have long been ignored, or have been confused either with other ophthalmological disorders causing low vision, or with a range of learning disabilities. We present here the deleterious consequences that CVI can have upon learning and social interaction, and how these can be given behavioral labels without the underlying visual causes being considered. We discuss the need to train and inform clinicians in the identification and diagnosis of CVI, and how to distinguish the diagnosis of CVI from amongst other visual disorders, including the specific learning disorders. This is important because the range of approaches needed to enhance the development of children with CVI is specific to each child's unique visual needs, making incorrect labeling or diagnosis potentially detrimental to affected children because these needs are not met.

Perceptual visual dysfunction in children - An Indian perspective.

Perceptual visual dysfunction (PVD) comprises a group of vision disorders resulting from dysfunction of the posterior parietal and/or temporal lobes. Often, affected children have normal/near normal visual acuities and/or visual fields, but have difficulties in activities of daily living involving the use of vision. PVDs are known to be common among children with risk factors such as a history of prematurity and/or neurodevelopmental disorders. The inferior temporal lobes and ventral stream transform visual signals into perception, while the posterior parietal lobes and dorsal stream transform visual signals to non-consciously map the scene to guide action and facilitate attention. Dysfunction of these can lead to specific visual impairments that need to be identified during history taking, triggering ascertainment of further details by a structured inventory approach. Clinical tests to elicit dorsal and ventral stream visual dysfunctions have good specificity but low sensitivity. Neuropsychologists are rarely available in the developing world to perform detailed assessments, but there are a few tests that can be used by eye care professionals with some training. Optical coherence tomography (OCT) showing thinning of the ganglion cell layer and retinal nerve fiber layer is being explored as a potential tool for rapid assessment in the clinic. The behavioral outcomes of PVD can mimic psychological conditions including autism spectrum disorder, attention deficit hyperactivity disorder, specific learning disability, and intellectual impairment, and one needs to be aware of overlap among these differential diagnoses. A practical functional approach providing working solutions for each child's set of difficulties in day-to-day activities is needed.

The treatment of glaucoma using topical preservative-free agents: an evaluation of safety and tolerability.

Introduction: Preservative-free (PF) medications represent a valuable treatment strategy in the lifelong management of glaucoma. By removing preservative toxicity, PF formulations provide tangible clinical benefits to glaucoma patients worldwide. They improve tolerability and adherence, leading to a positive impact in long-term intraocular pressure (IOP) control.Areas covered: A critical review of the subject is provided, including selected evidence on the safety and tolerability of currently available topical PF formulations. Cumulative evidence confirms that topical PF medications are at least equally efficacious to their preserved equivalents. There is convincing short-term evidence for superior tolerability and safety of PF formulations compared to preserved medications. The long-term benefits and success of PF therapy requires further elucidation.Expert opinion: Successful stepwise administration of medical therapy for glaucoma remains elusive. There is a greater risk for ocular toxicity and therapy failure with preserved topical glaucoma therapy. Currently available and emerging PF therapy options potentially optimize lifelong stepwise glaucoma therapy and may enhance outcome. To avert complications from preservatives leading to poor adherence, ideally, future antiglaucoma therapy should become 100% PF. There are still key aspects of PF therapy that warrant further investigation.

Simultanagnosia as a cause of visual disturbance following Posterior Reversible Encephalopathy Syndrome (PRES): A case report.

Simultanagnosia resulting from dorsal stream dysfunction is an under recognized condition. In this case report we describe the case of a young woman who developed posterior reversible encephalopathy syndrome (PRES), and who recovered visual acuities of 20/20 in each eye, along with normal visual fields and contrast sensitivities, yet experienced persistent symptoms of perceptual dysfunction. Detailed and systematic history taking revealed consistent visual difficulties typical of dorsal stream dysfunction. After a detailed explanation of her symptomatology and training in a range of strategies to cope, the patient experienced a great improvement in her day-to-day functioning.

Profiling Children With Cerebral Visual Impairment Using Multiple Methods of Assessment to Aid in Differential Diagnosis.

Cerebral (cortical) visual impairment (CVI), the primary cause of visual impairment in children in high-income countries, is increasing globally due to improved life-saving measures for premature and full-term infants. Yet the consequences of this condition are only beginning to be understood and addressed. According to the topography, site, and the extent of the pathology, the deficit may variably concern central visual functions, visual field, perception of movement, visual analysis, visual exploration, attention, or visual memory, as well as visual guidance of movement. Each affected child has a unique clinical picture, which needs to be identified and individually profiled. This is probably the underlying reason that CVI is commonly underdiagnosed or misdiagnosed, especially in children, and, as a consequence, the full range of potential behavioral outcomes are not identified and adequately addressed. The present paper shows how the use of multiple methods of assessment can improve understanding of children with CVI.

Cerebral visual impairment is a major cause of profound visual impairment in children aged less than 3 years: A study from tertiary eye care center in South India.

Purpose: The purpose of this study was to evaluate causes for profound visual impairment in children ≤3 years of age at a tertiary eye care center in Andhra Pradesh, India. Methods: A retrospective study was conducted for all the children (≤3 years) who attended the pediatric ophthalmology service between January 2012 and February 2017. Results: A total of 428 severely visually impaired children aged ≤3 years were seen during the study period: 264 (62%) of them were boys and I64 (38%) were girls. The average age at presentation was 14.02 months. The causes of visual impairment were cerebral visual impairment (CVI) 142 (33%), a combination of CVI and ocular visual impairment (OVI) 48 (11%), and OVI only 236 (56%), which included congenital cataract 56 (13.1%), retinopathy of prematurity 52 (I2.6%), optic atrophy 17 (4.5%), congenital nystagmus (4.4%), congenital globe anomalies 2I (5.2%), and high refractive errors - 10 (2.8%). Delays in different areas of development were seen in 103 out of 142 children with CVI (72.5%), which included motor delay 53 (51.5%), cognitive delay 15 (14.6%), speech delay in 3 (2.9%), and delay in multiple areas of development (like combination of motor, cognitive, and speech delay) in 32 (31.1%). Conclusion: In children under 3 years of age, CVI is a major cause of profound visual impairment in our area and the majority of them manifest delay in several areas of development.

The effect of visual support strategies on the quality of life of children with cerebral palsy and cerebral visual impairment/perceptual visual dysfunction in Nigeria: study protocol for a randomized controlled trial.

BACKGROUND: Cerebral visual impairment (CVI), including perceptual visual dysfunction (PVD), is common in children with cerebral palsy (CP). Inventories of questions relating to practical aspects of visual perception in everyday life, in particular the closed-ended Insight Questions Inventory (IQI), can be used to assess CVI/PVD. Studies linking responses to the inventory with specific visual support strategies, aimed at modifying the child's environment and/or behaviour to minimize the impact of the CVI/PVD, have been piloted. The IQI and tailored strategies have not been used in an African population, nor have they been tested in a controlled trial. This trial will compare the effectiveness of the IQI and linked visual support strategies versus general supportive treatments on the quality of life of children with CVI/PVD and CP through a randomized controlled trial. METHODS/DESIGN: This is a prospective, double-blind, parallel-arm, randomized controlled trial. The primary outcome is change in quality of life scores between the two arms of the trial at 6 weeks, assessed using the Paediatric Quality of Life Inventory (PedsQL) generic 4.0 and CP 3.0 module. All children will undergo baseline assessment including the Open Questions Inventory, IQI, PedsQL 3.0, PedsQL 4.0 generic, and the Strengths and Difficulties Questionnaire (SDQ). Eligible children with CP aged 4 years to < 16 years will be stratified and blocked by the age groups 4-9 and 10 to < 16 years and by Gross Motor Function Classification System (GMFCS) levels 1-3 and 4-5. Families in the intervention arm will receive tailored insight visual support strategies and telephone calls during the 6-week trial period. The control arm will receive standard treatment and the intervention after the 6-week trial period. Follow-up interviews will be performed in both arms at 6 weeks with a repeat administration of the PedsQL CP 4.0 and 3.0, the IQI and the SDQ. Secondary outcomes include a change in functional vision. DISCUSSION: This randomized controlled trial will provide evidence of the effectiveness of this intervention for children with CP in a resource-poor setting. TRIAL REGISTRATION: Pan African Clinical Trials Registration, PACTR201612001886396 . Registered on 3 December 2016.

Hemianopia and Features of Bálint Syndrome following Occipital Lobe Hemorrhage: Identification and Patient Understanding Have Aided Functional Improvement Years after Onset.

Introduction. Cerebral visual impairment (CVI) can present around birth or any time thereafter. Homonymous hemianopia is a common feature. The concept that functional improvement is unattainable augurs against active management. Dorsal stream dysfunction (or Bálint syndrome when severe) results from bilateral posterior parietal dysfunction but may go undetected, especially in children. Case Presentation. At 16 the patient suffered spontaneous left occipital lobe brain hemorrhage from a ruptured arteriovenous malformation. This was surgically excised. Short lived right upper limb intermittent jerking, with additional left sided weakness, ensued. Anomalous EEG recordings, with right-sided bias, arose from the posterior temporoparietal area. A right homonymous hemianopia was evident. During the ensuing 17 years she experienced multiple complex difficulties, until, at a lecture describing how to identify and support children with CVI, she realized she herself had many of the difficulties described. Visual assessment identified hemianopia and dorsal stream dysfunction. Discussion. Following identification, characterization, and explanation of the impact of her visual difficulties, she both gained greater awareness of her visual difficulties and their impact and developed a range of strategies leading to functional improvement of her visual field loss and amelioration of her dorsal stream dysfunction, with great improvement in quality of life.

Psychophysical and neuroimaging responses to moving stimuli in a patient with the Riddoch phenomenon due to bilateral visual cortex lesions.

Patients with injury to early visual cortex or its inputs can display the Riddoch phenomenon: preserved awareness for moving but not stationary stimuli. We provide a detailed case report of a patient with the Riddoch phenomenon, MC. MC has extensive bilateral lesions to occipitotemporal cortex that include most early visual cortex and complete blindness in visual field perimetry testing with static targets. Nevertheless, she shows a remarkably robust preserved ability to perceive motion, enabling her to navigate through cluttered environments and perform actions like catching moving balls. Comparisons of MC's structural magnetic resonance imaging (MRI) data to a probabilistic atlas based on controls reveals that MC's lesions encompass the posterior, lateral, and ventral early visual cortex bilaterally (V1, V2, V3A/B, LO1/2, TO1/2, hV4 and VO1 in both hemispheres) as well as more extensive damage to right parietal (inferior parietal lobule) and left ventral occipitotemporal cortex (VO1, PHC1/2). She shows some sparing of anterior occipital cortex, which may account for her ability to see moving targets beyond ~15 degrees eccentricity during perimetry. Most strikingly, functional and structural MRI revealed robust and reliable spared functionality of the middle temporal motion complex (MT+) bilaterally. Moreover, consistent with her preserved ability to discriminate motion direction in psychophysical testing, MC also shows direction-selective adaptation in MT+. A variety of tests did not enable us to discern whether input to MT+ was driven by her spared anterior occipital cortex or subcortical inputs. Nevertheless, MC shows rich motion perception despite profoundly impaired static and form vision, combined with clear preservation of activation in MT+, thus supporting the role of MT+ in the Riddoch phenomenon.

Diurnal and 24-h Intraocular Pressures in Glaucoma: Monitoring Strategies and Impact on Prognosis and Treatment.

The present review casts a critical eye on intraocular pressure (IOP) monitoring and its value in current and future glaucoma care. Crucially, IOP is not fixed, but varies considerably during the 24-h cycle and between one visit and another. Consequently, a single IOP measurement during so-called office hours is insufficient to characterize the real IOP pathology of a patient with glaucoma. To date IOP remains the principal and only modifiable risk factor for the development and progression of glaucoma. Only by evaluating IOP characteristics (mean, peak and fluctuation of IOP) at diagnosis and after IOP-lowering interventions can we appreciate the true efficacy of therapy. Unfortunately, a major limiting factor in glaucoma management is lack of robust IOP data collection. Treatment decisions, advancement of therapy and even surgery are often reached on the basis of limited IOP evidence. Clearly, there is much room to enhance our decision-making and to develop new algorithms for everyday practice. The precise way in which daytime IOP readings can be used as predictors of night-time or 24-h IOP characteristics remains to be determined. In practice it is important to identify those at-risk glaucoma patients for whom a complete 24-h curve is necessary and to distinguish them from those for whom a daytime curve consisting of three IOP measurements (at 10:00, 14:00 and 18:00) would suffice. By employing a staged approach in determining the amount of IOP evidence needed and the rigour required for our monitoring approach for the individual patient, our decisions will be based on more comprehensive data, while at the same time this will optimize use of resources. The patient's clinical picture should be the main factor that determines which method of IOP monitoring is most appropriate. A diurnal or ideally a 24-h IOP curve will positively impact the management of glaucoma patients who show functional/anatomical progression, despite an apparently acceptable IOP in the clinic. The potential impact of nocturnal IOP elevation remains poorly investigated. The ideal solution in the future is the development of non-invasive methods for obtaining continuous, Goldmann equivalent IOP data on all patients prior to key treatment decisions. Moreover, an important area of future research is to establish the precise relationship between 24-h IOP characteristics and glaucoma progression.

Cerebral visual impairment in children: Causes and associated ophthalmological problems.

Purpose: The aim of this study is to identify common causes, associated ophthalmological abnormalities, and systemic comorbidities in children in Andhra Pradesh, India, with cerebral visual impairment (CVI). Methods: A retrospective review of case records of all children aged <16 years with diagnosis of CVI seen between January 2016 and December 2016 was carried out. Data were collected for their age, gender, cause of CVI, refraction, accommodation, anterior and posterior segment examination findings, and systemic problems. Results: A total of 124 patients were identified and studied (80 boys and 44 girls, mean age 5.23 years, 44.8% aged <2 years). The most common causes of CVI were hypoxic-ischemic encephalopathy (HIE) (34.4%), undetermined etiology (32.8%), neonatal seizures, and infantile spasms (16% each). The most common presenting complaints were poor vision (76%) and squint (11.2%). Profound visual impairment was seen in 88.8%, and 11.2% had high functioning CVI. Fifty-eight (46.4%) patients had significant refractive errors, 40 (32.25%) had strabismus, 4 (3.2%) had visually significant cataract, and 40 (32%) had optic atrophy. Motor delay was observed in 39.5%, speech delay was evident in 22.4%, and cognitive delay in 16%. Conclusion: HIE is the most common cause (one-third) of CVI in our population, and the majority of them presented at age <2 years (44.8%) with profound visual impairment (88.8%). A significant number of them have treatable ophthalmic conditions such as refractive errors (46.4%), accommodative insufficiency (12.1%), and cataract (3.2%), and more than one-third of them also have delay in other areas of development.

Response to correction of refractive errors and hypoaccommodation in children with congenital Zika syndrome.

PURPOSE: To describe the immediate response to correction of refractive errors and hypoaccommodation in children with congenital Zika syndrome (CZS). METHODS: Children born between May and December 2015 with a confirmed diagnosis of CZS and enrolled in a multidisciplinary early intervention program were included in this study. All children received a comprehensive ophthalmic examination, including dynamic retinoscopy and cycloplegic refraction. Children were prescribed their full correction if they met the criteria for refractive error, and additional plus 3.00 overcorrection for strabismus, accommodative dysfunction, and/or low vision. Monocular and binocular visual responses to Lea Grating Test at 30 cm, with and without eyeglasses, were measured on day 1 of glasses wear. RESULTS: A total of 60 children were evaluated (mean age at evaluation, 11.5 ± 1.1 months; range, 9.0-16.0 months). Lea Grating Test responses were abnormal in all children prior to spectacle correction. Hypoaccommodation was present in 17 of 21 children (81%). Overcorrection was prescribed for all children. Visual responses were subnormal even with glasses use; however, immediate improvement in binocular vision was found in 37 children (62%) and in 74 of 119 eyes (62.2%). For the monocular visual improvement, 27 of 115 eyes (23.5%) had structural abnormalities, and 44 of 115 eyes (38.3%) were structurally normal. There was a statistical difference between the cycloplegic refraction of the children in August and in November, including emmetropia (P = 0.001), hyperopia (P = 0.000), myopia (P = 0.007), and astigmatism (P = 0.004). CONCLUSIONS: Eyeglasses can improve visual acuity in children with CZS. Significant changes in their refractive status over time requires periodic updates.

Pediatric Balint's Syndrome Variant: A Possible Diagnosis in Children.

Balint's syndrome is well described in adults, but not in children. It is caused by bilateral posterior parietal lobe damage and comprises a triad of simultanagnosia (inability to simultaneously see more than a small number of items), optic ataxia (impaired visual guidance of movement of the limbs and body), and apraxia of gaze (inability to volitionally direct gaze despite the requisite motor substrate) often associated with homonymous lower visual field loss. We, here, describe five children (four males, one female; mean age 7.4 years, [range 4-11 years]; birth weight ≤ 2.5 kg; four were born ≤ 36 weeks of gestational age and one at 40 weeks) who presented to the Cerebral Visual Impairment Clinic at a tertiary care center in South India with clinical features remarkably consistent with the above description. In all children neuroimaging showed bilateral parietooccipital gliosis with regional white matter volume loss and focal callosal thinning, consistent with perinatal hypoxic ischemic encephalopathy and possible neonatal hypoglycemia.

Impact of Cerebral Visual Impairments on Motor Skills: Implications for Developmental Coordination Disorders.

Cerebral visual impairment (CVI) has become the primary cause of visual impairment and blindness in children in industrialized countries. Its prevalence has increased sharply, due to increased survival rates of children who sustain severe neurological conditions during the perinatal period. Improved diagnosis has probably contributed to this increase. As in adults, the nature and severity of CVI in children relate to the cause, location and extent of damage to the brain. In the present paper, we define CVI and how this impacts on visual function. We then define developmental coordination disorder (DCD) and discuss the link between CVI and DCD. The neuroanatomical correlates and aetiologies of DCD are also presented in relationship with CVI as well as the consequences of perinatal asphyxia (PA) and preterm birth on the occurrence and nature of DCD and CVI. This paper underlines why there are both clinical and theoretical reasons to disentangle CVI and DCD, and to categorize the features with more precision. In order to offer the most appropriate rehabilitation, we propose a systematic and rapid evaluation of visual function in at-risk children who have survived preterm birth or PA whether or not they have been diagnosed with cerebral palsy or DCD.