Case series of retinopathy of prematurity blindness in Nigeria: A wakeup call to policy makers, hospitals, ophthalmologists and paediatricians.

BACKGROUND STATEMENT: Inadequate retinopathy of prematurity (ROP) screening coverage portends a high risk for increasing the cases of ROP blindness. This study aims to report the clinical profile of pre-term babies who developed ROP blindness, highlight the usefulness in determining screening criteria and the role of private hospitals in ROP blindness prevention. CASE SERIES REPORT: Online Google form and telephone survey was conducted from May to December 2020 among paediatric ophthalmologists who provided the clinical details of ROP blind children seen between 2016 and 2020. The main outcome measured included type of the hospital of birth, gestational age, birth weight, ROP Screening and treatment, and blinding ROP Stage among children born premature. The SPSS-IBM version 26 was used for the analysis. Eighteen children blind from ROP with an equal male-to-female ratio were reported. Mean gestational age was 28.4 ± 2.2 weeks (range 26 - 34 weeks, median was 28.0 weeks). Mean birth weight was 1173.7 ± 317.9 grams (range 776 - 2100 grams, median 1125 grams). Six (33.3%) babies were born in private hospitals between 28 and 32 weeks. Sixteen (88.9%) children never had ROP screening. Fifteen (83.3%) were blind in both eyes. Six (33.3%) had Stage IVb and 12 (66.7%) Stage V. CONCLUSION: About 90% of the babies who became blind did not undergo ROP screening. It is crucial that all babies born at 34 weeks or earlier and have birth weight of < 1500 grams in public/private hospitals be screened for ROP to prevent this avoidable blindness in Nigeria.

Refractive Error and Visual Impairment Among School Children: Result of a South-Eastern Nigerian Regional Survey.

PURPOSE: To determine the prevalence refractive errors and causes of visual impairment in school children in the south-eastern region of Nigeria. METHODS: School-based cross-sectional samples of children 5 to 15 of age in both urban and rural areas were profiled through cluster sampling. The main outcome measures were presenting, uncorrected, and best-corrected visual acuity using the Refractive Error in School-age Children (RESC) protocol. RESULTS: A total of 5723 children were examined during the study period comprising 2686 (46.9%) males and 3037 (53.1%) females; (M:F ratio 0.9:1) and aged 10.49±2.74SD of mean (range, 5 to 15 years). The age group 12 to <13 accounted for the highest 776 (13.6%) number of the study participants. The uncorrected visual acuity (VA) of <20/40 (6/12) was seen in 188 (3.4%) of the study participants while the presenting and best-corrected visual acuity of <20/40 (6/12) were noted in 182 (3.4%) children and 14 (0.2%) children, respectively. Refractive error was the principal cause of visual impairment. CONCLUSION: Prevalence of refractive error is low. Myopia is the principal cause of refractive error occurring more in females and in urban schools. The main cause of visual impairment is refractive error, and most children that need spectacle correction did not have them. Program to identify children with refractive error in addition to providing free or affordable optical services remains the key to preventing visual impairment from refractive error particularly in resource-poor settings.

Travel burden and clinical presentation of retinoblastoma: analysis of 1024 patients from 43 African countries and 518 patients from 40 European countries.

BACKGROUND: The travel distance from home to a treatment centre, which may impact the stage at diagnosis, has not been investigated for retinoblastoma, the most common childhood eye cancer. We aimed to investigate the travel burden and its impact on clinical presentation in a large sample of patients with retinoblastoma from Africa and Europe. METHODS: A cross-sectional analysis including 518 treatment-naïve patients with retinoblastoma residing in 40 European countries and 1024 treatment-naïve patients with retinoblastoma residing in 43 African countries. RESULTS: Capture rate was 42.2% of expected patients from Africa and 108.8% from Europe. African patients were older (95% CI -12.4 to -5.4, p<0.001), had fewer cases of familial retinoblastoma (95% CI 2.0 to 5.3, p<0.001) and presented with more advanced disease (95% CI 6.0 to 9.8, p<0.001); 43.4% and 15.4% of Africans had extraocular retinoblastoma and distant metastasis at the time of diagnosis, respectively, compared to 2.9% and 1.0% of the Europeans. To reach a retinoblastoma centre, European patients travelled 421.8 km compared to Africans who travelled 185.7 km (p<0.001). On regression analysis, lower-national income level, African residence and older age (p<0.001), but not travel distance (p=0.19), were risk factors for advanced disease. CONCLUSIONS: Fewer than half the expected number of patients with retinoblastoma presented to African referral centres in 2017, suggesting poor awareness or other barriers to access. Despite the relatively shorter distance travelled by African patients, they presented with later-stage disease. Health education about retinoblastoma is needed for carers and health workers in Africa in order to increase capture rate and promote early referral.

Global Retinoblastoma Presentation and Analysis by National Income Level.

Importance: Early diagnosis of retinoblastoma, the most common intraocular cancer, can save both a child's life and vision. However, anecdotal evidence suggests that many children across the world are diagnosed late. To our knowledge, the clinical presentation of retinoblastoma has never been assessed on a global scale. Objectives: To report the retinoblastoma stage at diagnosis in patients across the world during a single year, to investigate associations between clinical variables and national income level, and to investigate risk factors for advanced disease at diagnosis. Design, Setting, and Participants: A total of 278 retinoblastoma treatment centers were recruited from June 2017 through December 2018 to participate in a cross-sectional analysis of treatment-naive patients with retinoblastoma who were diagnosed in 2017. Main Outcomes and Measures: Age at presentation, proportion of familial history of retinoblastoma, and tumor stage and metastasis. Results: The cohort included 4351 new patients from 153 countries; the median age at diagnosis was 30.5 (interquartile range, 18.3-45.9) months, and 1976 patients (45.4%) were female. Most patients (n = 3685 [84.7%]) were from low- and middle-income countries (LMICs). Globally, the most common indication for referral was leukocoria (n = 2638 [62.8%]), followed by strabismus (n = 429 [10.2%]) and proptosis (n = 309 [7.4%]). Patients from high-income countries (HICs) were diagnosed at a median age of 14.1 months, with 656 of 666 (98.5%) patients having intraocular retinoblastoma and 2 (0.3%) having metastasis. Patients from low-income countries were diagnosed at a median age of 30.5 months, with 256 of 521 (49.1%) having extraocular retinoblastoma and 94 of 498 (18.9%) having metastasis. Lower national income level was associated with older presentation age, higher proportion of locally advanced disease and distant metastasis, and smaller proportion of familial history of retinoblastoma. Advanced disease at diagnosis was more common in LMICs even after adjusting for age (odds ratio for low-income countries vs upper-middle-income countries and HICs, 17.92 [95% CI, 12.94-24.80], and for lower-middle-income countries vs upper-middle-income countries and HICs, 5.74 [95% CI, 4.30-7.68]). Conclusions and Relevance: This study is estimated to have included more than half of all new retinoblastoma cases worldwide in 2017. Children from LMICs, where the main global retinoblastoma burden lies, presented at an older age with more advanced disease and demonstrated a smaller proportion of familial history of retinoblastoma, likely because many do not reach a childbearing age. Given that retinoblastoma is curable, these data are concerning and mandate intervention at national and international levels. Further studies are needed to investigate factors, other than age at presentation, that may be associated with advanced disease in LMICs.

Inequitable coverage of vitamin A supplementation in Nigeria and implications for childhood blindness.

BACKGROUND: Vitamin A deficiency (VAD) is of major public health significance; it is a risk factor for childhood deaths from diarrhoea and measles in low and middle-income countries and an important cause of preventable childhood blindness in low income countries. Vitamin A supplementation (VAS) is being implemented in many LMICs and high coverage reduces the prevalence of blinding corneal diseases in children. However, national estimates of coverage may not reveal any inequities in intra country coverage. The aim of this study is to assess factors influencing VAS coverage and also assess the relationship between VAS coverage and childhood corneal blindness in Nigeria. METHODS: Data were collected from the Nigeria Demographic and Health Survey (NDHS) 2013 and the published literature on population-based childhood blindness surveys in Nigeria. The main outcome measure was the proportion of eligible children who received VAS in the last 6 months preceding the survey. Study factors comprised a range of socioeconomic, and individual factors. Data were analysed using STATA V.12.1 (Statcorp, Texas). To explore the effects of the independent variables on VAS coverage, bivariate and multivariate regression was done. Variables with p < 0.05 in the final multivariable model were considered as independent factors. For the population-based childhood blindness surveys, aggregated and disaggregated data were used. Causes of blindness were stratified into corneal blindness and 'others'. Odds ratios were computed to determine the odds of developing corneal blindness in each geopolitical region. Tests of significance were set at the 95% level. RESULTS: The total VAS coverage in 2013 was 41.5%. VAS coverage was inequitable. Children with very educated mothers (OR 3.27 p < 0.001), from the south-south region (OR 2.38 p < 0.001) or in the highest wealth quintile (OR 2.81 p < 0.001) had higher odds of receiving VAS. The northwest zone had the lowest VAS coverage and the highest prevalence of corneal blindness. CONCLUSION: Regional and socioeconomic inequities in VAS exist in Nigeria and these may have grave implications for the causes of childhood blindness. The development and implementation of context specific and effective strategies are needed to reduce these inequities in VAS.

Using Key Informant Method to Determine the Prevalence and Causes of Childhood Blindness in South-Eastern Nigeria.

PURPOSE: To determine the prevalence and causes of childhood blindness in an underserved community in south-eastern Nigeria using the key informant method. METHODS: This was a descriptive cross-sectional study. Key informants (KI) appointed by their respective communities received 1-day training on identification of blind children in their communities. Two weeks later, the research team visited the agreed sites within the community and examined the identified children. The World Health Organization eye examination record for blind children was used for data collection. Data entry and analysis were done with the Statistical Package for Social Sciences (SPSS) version 17.0. RESULTS: Fifteen blind or severely visually impaired children (age range 3 months to 15 years) were identified in this community; nine of these were brought by the KIs. The prevalence of childhood blindness/severe visual impairment (BL/SVI) was 0.12 per 1000 children. By anatomical classification, operable cataract in 6 (40.0%) was the leading cause of BL/SVI in the series; followed by optic nerve lesions (atrophy/hypoplasia) in 3 (20.0%). The etiology of BL/SVI is unknown for the majority of the children (66.7%). It was presumed hereditary in four children (26.7%). Sixty percent of the blindness was judged avoidable. Only three children (20.0%) were enrolled in the Special Education Centre for the Blind. CONCLUSION: The prevalence of childhood BL/SVI in our study population is low but over half of the blindness is avoidable. There may be a significant backlog of operable childhood cataract in south-eastern Nigeria. The KI method is a practical method for case finding of blind children in rural communities.

Dual sensory impairment in special schools in South-Eastern Nigeria.

In a cross-sectional study to determine the magnitude of dual sensory impairment (DSI-combined hearing and vision loss) in children in single-disability special education schools, children in schools for the blind and schools for the deaf in four states in South-East Nigeria were examined by an ophthalmologist and otorhinolaryngologist to determine the level of their disability and to identify other disabilities if any. Participants were all students with childhood blindness or childhood deafness. The magnitude and causes of DSI and the burden of undetected DSI were the main outcome measures. A total of 273 students were examined. About 7% of these students had DSI out of which over 60% (12/19) was previously undetected. There was more DSI in the blind schools than in the deaf schools (p=0.003). There is a large burden of undetected DSI in children in special schools in Nigeria. There is a need to create awareness of this problem and advocate appropriate screening, rehabilitative and educational strategies for children who have it.

Unmet visual needs of children with Down syndrome in an African population: implications for visual and cognitive development.

PURPOSE: To determine the magnitude and types of ocular disorders in children with Down syndrome in an African population and to assess to what extent these visual needs have been met.
 METHODS: This is a cross-sectional study of children with Down syndrome attending a school for the mentally challenged in Southeastern Nigeria. Visual acuity, ocular examination, and cycloplegic refraction were done on all cooperative participants. Information was sought from parents/caregivers to ascertain previous ocular treatment/spectacle usage. 
 RESULTS: A total of 30 children with Down syndrome aged 5-15 years were examined. Uncorrected refractive errors were detected in 76.4% of them. Other lesions included mongoloid slant (53.5%), strabismus (33.3%), ptosis (33.3%), nystagmus (13.3%), and cataract (3.3%). None of the children with refractive errors had ever worn spectacles.
 CONCLUSIONS: Refractive error is a common finding in this population. There is a high unmet visual need in these children. This may have implications for visual and cognitive development. Early screening for ocular disorders in Down syndrome is recommended to detect and treat them.

Challenges in the management of paediatric cataract in a developing country.

AIM: To review the management of cataract in children in a tertiary hospital in a developing country, and to highlight the challenges therein. METHODS: The hospital records of children aged 15 years or less that had cataract surgery at University of Nigeria Teaching Hospital, Enugu from 2005 to 2008 were reviewed retrospectively. Information was obtained on bio-data, pre- and post-operative visual acuity (VA), biometry, and type of surgery, use of intraocular lens (IOL) and presence of co-morbidity. SPSS was used for data entry and analysis. RESULTS: The hospital records of 21 children (26 eyes) were analyzed. There were 12 males (57.1%) and 9 females (42.9%). Pre-operative VA could not be assessed in 11 eyes (42.3%), 14 eyes (53.9%) had VA <3/60 and 1 eye (3.8%) had VA 6/60. Biometry was done in only 5 eyes (19.2%). All eyes had standard extracapsular cataract extraction without primary posterior capsulectomy; 12 eyes (46.2%) had posterior chamber intraocular lens (PC-IOL) implant while 13 eyes (50.0%) had no IOL. After 12 weeks of follow up, vision assessment was available in only 15 eyes. With best correction, VA of 6/18 or better was achieved in only 5 eyes (33.3%). CONCLUSION: Inadequate facilities and inadequate follow up after surgery are some of the challenges in managing paediatric cataract in the developing countries. If these challenges are not addressed, cataract will remain a major cause of childhood blindness and low vision in Africa for many years. There should be collaboration between Paediatric Ophthalmology Centres in industrialized and developing countries to enhance skill transfer. Governmental and International Non-governmental Organizations can go a long way to facilitate this exchange.