Ocular manifestations of systemic diseases in children.

Knowledge of ocular diseases and understanding of the complex interplay between eye and systemic health have increased over the years. This knowledge is particularly important when caring for our youngest and most vulnerable paediatric patients when ophthalmic manifestations may provide an insight to underlying systemic diseases and can act as the first indicator of an undiagnosed systemic condition. Further, the visual system can be vulnerable to manifestations of known systemic disease, with vigilant ophthalmic examination generally aiding early identification of ocular complications for collaborative multidisciplinary care to prevent avoidable vision loss. The potential ocular signs and complications of the following developmental, genetic or acquired childhood systemic disorders are presented: premature birth, trisomy 21, albinism, Marfan's syndrome, Stickler's syndrome, septo-optic dysplasia, aniridia, neurofibromatosis 1, Sturge-Weber syndrome, papilloedema, juvenile idiopathic arthritis and vitamin A deficiency. Rather than providing an exhaustive list of diseases, this review offers an overview of the more commonly encountered congenital or acquired childhood systemic conditions that have associated childhood ophthalmic disorders and presents referral and ongoing surveillance recommendations.

Ten-year review of neuroimaging in acute paediatric strabismus.

BACKGROUND: Acute childhood strabismus is often a clinical conundrum faced by ophthalmologists. Currently, there are no clear clinical guidelines on how to investigate a child presenting with acute strabismus. Furthermore, there is no consensus as to whether to initially perform neuroimaging of a child to rule out the small but serious risk of intracranial pathology, or to pursue a careful observational approach. This paper aims to outline a standardised approach to investigating acute strabismus in paediatric patients. METHODS: A retrospective chart review of all paediatric patients that presented over a 10-year period to the ophthalmology departments at two tertiary level hospitals in New Zealand was conducted. Patients under 18 years of age with an acute presentation of strabismus, who underwent neuroimaging, were included. RESULTS: A total of 500 patient records were reviewed. Seventy patients met the study inclusion criteria. Of these patients, 17 (24.3%) had non-isolated strabismus while 53 patients (75.7%) had isolated strabismus. Twelve patients (70.6%) who presented with a non-isolated strabismus had abnormal neuroimaging. Of those who presented with an isolated strabismus, 4 (7.5%) had abnormal neuroimaging. CONCLUSIONS: Acute onset non-isolated strabismus is a significant predictor of underlying neurological abnormality in children and requires urgent neuroimaging. However, a cautious approach in which there is close observation, along with general paediatric and/or paediatric neurology input, may be appropriate for children presenting acutely with an isolated strabismus.

Causes of childhood low vision and blindness in New Zealand.

IMPORTANCE: This is the first national study on childhood visual impairment in a developed nation, New Zealand, describing prevalence, aetiology and preventable causes of low vision and blindness in children. BACKGROUND: Causes of childhood blindness vary between regions. This study aimed to present region-specific data on epidemiology of childhood blindness affecting a developed nation, New Zealand. DESIGN: Retrospective data analysis. PARTICIPANTS: All children enrolled with the Blind and Low Vision Education Network New Zealand (BLENNZ) with best-corrected visual acuity ≤6/18, or binocular visual field <10°. METHODS: 1000 out of 1321 children with visual impairment enrolled with BLENNZ were included. The principal cause of visual loss was determined, and the severity of visual loss categorized as low vision, or blindness according to the World Health Organization criteria. MAIN OUTCOME MEASURES: Main outcome measures were degree of visual impairment, aetiology of visual impairment and treatment modalities for visual rehabilitation. RESULTS: The calculated prevalence of childhood blindness and low vision was 0.05% and 0.06%. Principle causes of blindness were cortical visual impairment (31.5%), optic nerve atrophy (16.5%) and optic nerve hypoplasia (9.0%). The main preventable causes of blindness were neonatal trauma/asphyxia (31.5%), retinopathy of prematurity (18.2%) and non-accidental injury (10.3%). CONCLUSIONS AND RELEVANCE: This is the first national report on prevalence of childhood low vision and blindness in New Zealand. The prevalence and leading causes of low vision and blindness found in this study were comparable to other developed nations; however, preventable causes of low vision and blindness appeared unique to New Zealand.

Visual impairment due to retinopathy of prematurity (ROP) in New Zealand: a 22-year review.

AIM: To evaluate retinopathy of prematurity (ROP)-related visual impairment in New Zealand children. METHODS: 22-year retrospective review of medical records of children with moderate to severe visual impairment registered with the Blind and Low Vision Education Network New Zealand. The cohort was divided into two periods (1991-2004; 2005-2012) for analysis. RESULTS: 232 children with ROP were treated in the study period (109 in period 1, 123 in period 2). 36 children, 63.9% of whom were of male sex, were identified with subsequent significant visual impairment (27 in period 1, 9 in period 2). The incidence of new cases of visual impairment from ROP declined from 271.6 infants/100 000 live very preterm births per annum (period 1) to 146.1 per annum (period 2). Mean gestational age and mean birth weight were comparable between the two study periods. 75% of children with visual impairment from ROP received treatment for their condition (period 1, 74.1%; period 2, 77.8%) and modalities used changed significantly over time. The modal visual outcome overall was Snellen visual acuity <6/18-6/60 (55.6%) (period 1, 51.9%; period 2, 66.7%). The proportion of children with no light perception bilaterally decreased over time (period 1, 3.7%; period 2, 0%). CONCLUSIONS: There has been a reduction in the incidence of infants with significant visual impairment from ROP over time in New Zealand, likely due to progress in clinical management of ROP. Our study suggests the current ROP screening criteria of <31 weeks' gestation or <1250 g are of sufficient breadth.

Cross-sectional study on childhood cerebral visual impairment in New Zealand.

PURPOSE: To determine the prevalence, etiology, and avoidable causes of childhood cerebral visual impairment (CVI) in New Zealand. METHODS: The clinical and educational records of blind and low vision children enrolled in the Blind and Low Vision Education Network, New Zealand (BLENNZ), a national referral center, were retrospectively analyzed. The WHO Program for Prevention of Blindness (WHO/PBL) Eye Examination Record for Children with Blindness and Low Vision was used to record data from children ≤16 years of age diagnosed with CVI and visual acuity ≤6/18 enrolled with BLENNZ. Data analyzed included demographics, etiology, visual acuity, visual fields, educational setting, and rehabilitation plan. RESULTS: A total of 182 children (blind, 143; low vision, 39) were included. The calculated prevalence of childhood CVI in New Zealand was 0.02%. Of these, only 21% required low vision aids. Principle causes of CVI blindness were perinatal hypoxia/asphyxia (25%), nonaccidental injury (7%), and prematurity (7%). Approximately 50% of all cases of CVI blindness were potentially avoidable; of these, 52% were caused by perinatal hypoxia and 14% by nonaccidental injury. CONCLUSIONS: The conservative calculated prevalence of CVI, responsible for 30% of all childhood blindness in New Zealand, was 0.02%. The most common cause of CVI blindness in New Zealand, perinatal asphyxia, is also an avoidable cause.

Cross-sectional study on prevalence, causes and avoidable causes of visual impairment in Maori children.

AIMS: To provide information and comparison pertaining to visual impairment of Maori children with other children in New Zealand in particular: prevalence of blindness, causes of visual impairment, and avoidable causes of visual impairment. METHODS: Retrospective data collection utilising the WHO/PBL eye examination record for children with blindness and low vision at Blind and Low Vision Education Network New Zealand (BLENNZ), Homai. Individuals not of Maori ethnicity or over the age of 16 were excluded from the study. RESULTS: 106 blind and 64 low-vision Maori children were studied. The main cause of blindness in Maori children is cortical visual impairment. Twenty-eight percent of causes of blindness in this population are potentially avoidable with non-accidental injury as the main cause. CONCLUSION: The prevalence of blindness and low vision in children amounts to 0.05% and 0.03%, respectively. The prevalence and causes of childhood blindness are comparable to the other ethnic groups in New Zealand. The main difference lies in avoidable causes of blindness, which appeared to be much higher in the Maori population. The leading cause of avoidable blindness in Maori children is caused by non-accidental injuries.

A novel treatment of central serous chorioretinopathy with topical anti-inflammatory therapy.

A 45-year-old Caucasian female with diagnosis of central serous chorioretinopathy (CSCR) did not improve on conventional observational approach. She was not willing to proceed with photocoagulation or photodynamic therapy. An unconventional approach of topical anti-inflammatory (ketorolac, dexamethasone and hydrocortisone) preparation was prescribed. The course of her CSCR responded well on this unconventional treatment, but relapsed on cessation or tapering of treatment. After 18 weeks of treatment with a gradual taper, her condition resolved. The present case highlights an alternative but unconventional treatment of CSCR with prolonged use of anti-inflammatories.