Clinimetric properties of visuo-perceptual and visuo-cognitive assessment tools used for children with cerebral visual impairment and cerebral palsy or developmental delay: a systematic review.

PURPOSE: The aim of this systematic review is to evaluate the psychometrics and clinical utility of visuo-perceptual and visuo-cognitive assessment tools in children with cerebral visual impairment (CVI) and cerebral palsy (CP) or neurodevelopmental delay (DD). MATERIALS AND METHODS: Five databases (PubMed, EMBASE, SCOPUS, CINAHL, and Cochrane Database) were comprehensively searched from 1970 till June 2021. The PRISMA checklist was utilised to report on the process of selecting eligible papers. The methodological quality of included studies was evaluated using COnsenus-based Standards for the selection of health Measurement INstrument (COSMIN) checklist. RESULTS: Of the 26 assessment tools identified, only seven tools had psychometric evidence supporting their use. Based on COSMIN guidelines, 60% of included studies were rated as inadequate or doubtful for their methodological quality of measurement properties, with equal number being rated as indeterminate on the overall rating. CONCLUSIONS: Cerebral visual impairment due to its varied clinical presentation is often missed in children with CP and DD. There is a paucity of studies reporting on the validity and reliability of functional vision tools. Further studies are needed to conduct high-quality psychometric reporting using the updated COSMIN guidelines to identify appropriate functional vision tools for children with CP or DD.Implications for rehabilitationThere are paucity of studies evaluating the validity and reliability of existing perceptual and cognitive assessment tools in children with cerebral visual impairment (CVI) and cerebral palsy (CP).Development of age-appropriate assessment tools evaluating all aspects of functional vision will assist in providing more holistic child-centric rehabilitation programs.A combination of detailed perinatal history, direct observation, and clinical assessments of functional vision are important to recognise CVI in children with CP.

Relationship between brain structure and Cerebral Visual Impairment in children with Cerebral Palsy: A systematic review.

BACKGROUND: Cerebral Visual Impairment (CVI) is very common yet often unrecognised visual dysfunction in children with Cerebral Palsy (CP). Magnetic Resonance Imaging (MRI) is the diagnostic tool in the investigation of brain lesions in children with CP and CVI. AIM: The aim of this systematic review is to evaluate the relationship between brain structure and CVI, as determined by MRI in children with CP. METHODS AND PROCEDURES: A comprehensive search of 5 database (PubMed, EMBASE, SCOPUS, CINAHL and Cochrane Database) was undertaken up until June 2019. The PRISMA checklist was then utilised to report on the process of selecting eligible papers. A total of 30 observational studies met the full inclusion criteria. Further, STROBE checklist was employed to report on the observational studies. OUTCOMES AND RESULTS: Periventricular leucomalacia on MRI was found to have a strong association with CVI in all 30 studies. Only 13 (43 %) studies described dorsal and/ ventral stream dysfunction. There was ambiguity in the definition of CVI. CONCLUSIONS AND IMPLICATIONS: The overall level of evidence correlating different patterns of CVI and CP (based on GMFCS, motor type and distribution) and MRI was low. Further studies utilising advances in MRI are needed to understand brain reorganisation and patterns of CVI and suggest rehabilitation therapy inclusive of vision.

Comparison of a single intraoperative posterior sub-Tenon's capsule triamcinolone acetonide injection versus topical steroids for treatment of postcataract surgery inflammation in children.

BACKGROUND: The aim of this study is to compare the clinical efficacy of a single intraoperative posterior sub-Tenon's capsule triamcinolone acetonide injection with postoperative topical steroids in controlling intraocular inflammation in uncomplicated pediatric cataract surgery. MATERIALS AND METHODS: A prospective comparative study of children (<13 years of age) undergoing cataract surgery in a tertiary care eye center in South India. Preoperative evaluation was similar in both groups. The surgical procedure was the same for both groups except at the end of surgery only Group 1 (right eye of bilateral cataracts and all unilateral cataracts) received intraoperative 0.5 ml (40 mg/ml) posterior sub-Tenon's injection of triamcinolone acetonide. Postoperatively, Group 1 was administered only topical antibiotic and Group 2 was put on topical antibiotic, mydriatic, and steroids. Intraocular inflammation and intraocular pressure (IOP) were assessed clinically on day 1, 1st week, 1st month, and 3 months, postoperatively. RESULTS: A total of 30 eyes were included in the study. Age ranged between 1 month and 132 months, with 18 eyes included in Group 1 and 12 eyes in Group 2. The mean postoperative IOP at the 3 months follow-up was not significantly different between groups (P = 0.4). The presence of intraocular lens had no role in postoperative inflammation (P = 1). Group 2 showed more anterior segment inflammation with six eyes (50%) developing posterior synechiae and distortion of the pupil 3 months postoperatively. CONCLUSION: In pediatric cataracts, a single intraoperative sub-Tenon's capsule injection of triamcinolone acetonide appears to be safe and effective in controlling postcataract surgery inflammation.

Setting up of a cerebral visual impairment clinic for children: Challenges and future developments.

AIM: The aim of this study is to describe the setting up of a cerebral visual impairment (CVI) clinic in a tertiary care hospital in South India and to describe the spectrum of cases seen. MATERIALS AND METHODS: The CVI clinic, set up in February 2011, receives interdisciplinary input from a core team involving a pediatrician, neurologist, psychiatrist, occupational therapist, pediatric ophthalmologist, and an optometrist. All children, <18 years of age, with cerebral palsy (CP), learning disability, autism, neurodegenerative diseases, and brain trauma are referred to the clinic for functional vision assessment and opinion for further management. RESULTS: One thousand four hundred and seventy-eight patients were seen in the CVI clinic from February 2011 to September 2015. Eighty-five percent of the patients were from different parts of India. In the clinic, 61% had CP, 28% had seizure disorders, autism was seen in 9.5%, and learning disability, neurodegenerative conditions, and brain injury together constituted 1.5%. Most of the children (45%) had moderate CP. Forty percent of CVI was due to birth asphyxia, but about 20% did not have any known cause for CVI. Seventy percent of patients, who came back for follow-up, were carrying out the habilitation strategies suggested. CONCLUSIONS: Average attendance of over 300 new patients a year suggests a definite need for CVI clinics in the country. These children need specialized care to handle their complex needs. Although difficult to coordinate, an interdisciplinary team including the support groups and voluntary organizations is needed to facilitate the successful implementation of such specialized service.

Successful surgical management of bilateral epiretinal membrane in a child with only café-au-lait spots.

A 6-year-old boy diagnosed as anisometropic amblyopia, with only café-au-lait spots and a family history of neurofibromatosis, presented with decrease in vision in the both eyes. Dilated fundus examination showed epiretinal membrane in both eyes over the macula. He underwent successful surgical management of the epiretinal membrane.

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.

A Validation of an Examination Protocol for Cerebral Visual Impairment Among Children in a Clinical Population in India.

INTRODUCTION: Cerebral Visual Impairment (CVI) is a leading cause of vision impairment in developed and developing countries due to and increased survival of preterm and low birth weight infants. There are few data concerning the validity of protocols available to diagnose CVI. AIM: This study aimed to document the face, content and construct validity of an assessment protocol namely, a 15-domain, Structured Clinical Question Inventory (SCQI), which is based on structured history taking and clinical examination, for the diagnosis of CVI in a clinical population of India. MATERIALS AND METHODS: This study was a retro-spective chart analysis of all children below the age of 18years, referred to the CVI clinic of a tertiary care teaching hospital in Southern India from March 2011-Feb 2012. Clinical case-notes including the SCQI findings of all children referred to the clinic were reviewed. The data were extracted after Institutional Review Board approval. STATISTICAL ANALYSIS: Pearson correlation coefficient, Cronbach's alpha and exploratory factor analysis were used to document the content and construct validity of the examination protocol. RESULTS: A total of 342 children (35.7% male, 64.3% female), with a mean age of 3.8 years (range 0-17 years, the median was 3 years) were included in the study and their data were examined. The internal consistency of the SCQI was 0.93 suggesting it as an excellent tool to characterise and profile CVI and a 2-factor model (Dorsal Stream Dysfunction and Ventral Stream Dysfunction) based on a biologically plausible model explained 63% of the variance. CONCLUSION: The results of using the SCQI affirm published data and endorse a theoretical construct similar across cultures. The potential diagnostic accuracy, reliability and utility of this measure for CVI needs to be studied further. The clinical use of a short version of the SCQI may be helpful to contribute to the identification of CVI, especially for middle and low-income countries.

Surgical Management of Post-Traumatic Iris Cyst.

PURPOSE: Epithelial cysts of the iris may be primary or secondary. The management of secondary, posttraumatic iris cysts is often challenging. The purpose of this work is to report the successful surgical management of a post-traumatic iris cyst. CASE REPORT: A 38 year-old man presented with an iris cyst associated with epithelial downgrowth and total cataract, six years after sustaining a penetrating injury to his right eye. The iris cyst was excised in toto with the epithelial downgrowth, and the procedure was combined with cataract extraction and intra ocular lens implantation. The patient had good post-operative visual recovery with minimal inflammatory reaction. CONCLUSION: Post-traumatic cysts of the iris can be associated with significant ocular morbidity. Many different modalities of treatment, with varying degrees of success, have been described for the treatment of iris cysts. Excision of the iris cyst in toto is a good surgical management option, which can result in an excellent functional outcome with minimal inflammatory complications.

Spectrum of ocular firework injuries in children: A 5-year retrospective study during a festive season in Southern India.

PURPOSE: Ocular trauma is a major cause of acquired monocular blindness in children. Firework injuries account for 20% of ocular trauma. The purpose of our study was to document the profile of ocular firework injuries in children during the festive season of Diwali and to determine the prevalence of unilateral blindness in them. MATERIALS AND METHODS: A retrospective chart analysis of ocular firework injury in children during the festival of Diwali from 2009 to 2013, conducted in a tertiary care eye center in Tamil Nadu, Southern India. Children below 18 years of age with ocular firework injuries who presented to the emergency department for 3 consecutive days - the day of Diwali, 1 day before, and 1 day after Diwali - were included in this study. RESULTS: Eighty-four children presented with firework-related ocular injuries during the study period. Male to female ratio was 4:1 with mean age 9.48 ± 4 years. Forty-four percentage required hospitalization. The prevalence of unilateral blindness in children due to fireworks was found to be 8% (95% confidence interval - 2-13%). CONCLUSION: Vision 2020 gives high priority to avoidable blindness, especially in children. In our study, for every 12 children who presented with firecracker injury, one resulted in unilateral blindness. This is an avoidable cause of blindness. Awareness needs to be created, and changes in policy regarding sales and handling of firecrackers including mandatory use of protective eyewear should be considered.

Identifying and characterising cerebral visual impairment in children: a review.

Cerebral visual impairment (CVI) comprises visual malfunction due to retro-chiasmal visual and visual association pathway pathology. This can be isolated or accompany anterior visual pathway dysfunction. It is a major cause of low vision in children in the developed and developing world due to increasing survival in paediatric and neonatal care. CVI can present in many combinations and degrees. There are multiple causes and it is common in children with cerebral palsy. CVI can be identified easily, if a structured approach to history-taking is employed. This review describes the features of CVI and describes practical management strategies aimed at helping affected children. A literature review was undertaken using 'Medline' and 'Pubmed'. Search terms included cerebral visual impairment, cortical visual impairment, dorsal stream dysfunction and visual function in cerebral palsy.

Asymptomatic Intracorneal Graphite Deposits following Graphite Pencil Injury.

Reports of graphite pencil lead injuries to the eye are rare. Although graphite is considered to remain inert in the eye, it has been known to cause severe inflammation and damage to ocular structures. We report a case of a 12-year-old girl with intracorneal graphite foreign bodies following a graphite pencil injury.

Uniplanar Nystagmus Associated with Perceptual and Cognitive Visual Dysfunction due to Presumed Focal Ischemic Occipital Cortical Atrophy: A Missed Diagnosis and New Observation.

Uniplanar nystagmus has been described in relation to pathology of the brain stem, retina, optic nerve, sensory visual deprivation, periventricular leucomalacia, and drug toxicity. This paper describes a case of uniplanar nystagmus associated with features of higher visual dysfunction and a presumed focal insult to the occipital lobes following an episode of neonatal apnea.