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.

Evaluation of a Public Child Eye Health Tertiary Facility for Pediatric Cataract in Southern Nigeria I: Visual Acuity Outcome.

PURPOSE: A retrospective study of the outcome of congenital and developmental cataract surgery was conducted in a public child eye health tertiary facility in children <16 years of age in Southern Nigeria, as part of an evaluation. MATERIALS AND METHOD: Manual Small Incision Cataract Surgery with or without anterior vitrectomy was performed. The outcome measures were visual acuity (VA) and change (gain) in visual acuity. The age of the child at onset, duration of delay in presentation, ocular co-morbidity, non ocular co-morbidity, gender, and pre operative visual acuity were matched with postoperative visual acuity. A total of 66 children were studied for a period of six weeks following surgery. RESULTS: Forty eight (72.7%) children had bilateral congenital cataracts and 18 (27.3%) children had bilateral developmental cataracts. There were 38(57.6%) males and 28 (42.4%) females in the study. Thirty Five (53%) children had good visual outcome (normal vision range 6/6/ -6/18) post-operatively. The number of children with blindness (vision <3/60) decreased from 61 (92.4%) pre-operatively to 4 (6.1%) post-operatively. Post operative complication occurred in 6.8% of cases six week after surgery. Delayed presentation had an inverse relationship with change (gain) in visual acuity (r = - 0.342; p-value = 0.005). Pre-operative visual acuity had a positive relationship with post operative change (gain) in visual acuity (r = 0.618; p-value = 0.000). CONCLUSION: Predictors of change in visual acuity in our study were; delayed presentation and pre-operative VA. Cataract surgery in children showed clinical benefit.

Systemic Comorbidity in Children with Cataracts in Nigeria: Advocacy for Rubella Immunization.

Background. Congenital and developmental cataracts are leading causes of childhood blindness and severe visual impairment. They may be associated with systemic diseases including congenital heart diseases which are among the major causes of morbidity and mortality in childhood. The pattern of systemic comorbidities seen in children diagnosed with cataract in Calabar, Nigeria, was studied. Methods. A retrospective review was conducted on the children who had cataract surgery between 2011 and 2012. Diagnosis of the systemic condition was documented. Results. A total of 66 children were recruited for the study. Cardiac disease was seen in 26 children (39.9%), followed by delayed milestone in 16 (24.2%), intellectual disability in 14 (21.2%), deafness in 11 (16.7%), epilepsy in 4 (6.1%), and physical handicap in 3 (4.5%) of them. Clinically confirmed Congenital Rubella Syndrome was seen in 30 (45%) of the children. The pattern of CHD seen was as follows: patent ductus arteriosus in 16 (24.2%) followed by ventricular-septal defect in 5 (7.6%), atrial-septal defect in 3 (4.5%), and pulmonary stenosis in 2 (3%). Conclusion. Systemic comorbidities, especially cardiac anomalies, are common among children with cataract in Nigeria. Congenital Rubella Syndrome may be a prominent cause of childhood cataract in our environment. Routine immunization of school girls against rubella is advocated as a measure to mitigate this trend.

Economic burden of glaucoma in Rivers State, Nigeria.

BACKGROUND: Primary open angle glaucoma is reported to blind 150,000 people in the Nigerian population and over 7000 in Rivers State, and requires constant follow-up. Compliance is a challenge, given that most inhabitants live below the poverty line. This study was performed to determine how Nigerian patients are affected economically by the disease. METHODS: Consecutive adult patients attending the eye clinic of the University of Port Harcourt Teaching Hospital, Rivers State, Nigeria, with a diagnosis of primary open angle glaucoma and on outpatient antiglaucoma treatment in the first 6 months of 2006, were recruited for the study. The lowest paid government worker was on USD50 (N7500.00) per month and the gross domestic product per capita was USD1150 for the period under review. RESULTS: We enrolled 120 consecutive patients of mean age 52.7 ± 10.4 years, with a male to female ratio of 2:3. The most common occupations were in the civil service (n = 56, 46.7%). All participants were on topical antiglaucoma treatment. The average cost of medical antiglaucoma medication was N6000 (USD40) per month. Computed to include indirect costs, including medical laboratory tests, transportation, and care by patient escorts, an average sum of USD105.4 (N15,810) was spent by each patient per month. Most of the patients (73.3%) were responsible for their own treatment costs. No patient accepted the cheaper option of surgery (USD275.4, N41,310). Eighty of the patients (66.7%) visited our eye clinic monthly. Direct and indirect loss to the economy was USD3,064587 per annum from those already blind. This was in addition to the USD 4.1 million being spent yearly on medical treatment by those who were visually impaired by glaucoma. CONCLUSION: Middle-income earners spent over 50% of their monthly income and low-income earners spend all their monthly earnings on treatment for glaucoma. This situation often resulted in noncompliance with treatment and hospital follow-up visits. To reduce the economic burden of glaucoma, trabeculectomy performed by experienced surgeons should be offered as first-line treatment for glaucoma in this country, rather than medical therapy.

The Rivers State, Nigeria Eye Care Program--the first few steps towards achieving V2020.

AIM: To outline the outcome of the first eye camp of the eye care program in Rivers state which was established only in 2008 to reduce the burden of blindness in the state as revealed by the Nigerian national blindness and visual impairment survey results released in 2008 as much as possible to the lowest before the year 2020. METHODOLOGY: A descriptive analysis of the activities of the Prevention of blindness committee, Rivers established in 2008 by the Ministry of health, Rivers state, Nigeria. An analysis of the first eye camp held at the Rumuigbo health center, Rumuigbo, Port Harcourt is also presented from the records made at the time of the camp. The activities were partly sponsored by the Ministry of Health, Rivers state. Data analysis was with the help of a statistician using SPSS version 11. RESULTS: The recently released Nigerian National Survey on Blindness and Visual Impairment revealed that about 15,000 people are blind from cataract in Rivers state, Nigeria. Over a 3 day period to mark World Sight Day 2010, a total of 455 persons were screened for cataract surgery at the above venue. The mean age was 46.2 years (SD+/- 17.55) with an age range of 1 and half to 99 years. The male female ratio was 1:1. A total of 125 patients (27.5%) had cataract of which 83 were mature. Refractive error also affected 125 people (27.5%). Sixty-five people (14.3%) were suspected to have glaucoma while 13(2.9%) and 45 people (9.9%) presented with corneal opacities and allergic conjunctivitides respectively. Other conditions seen include conjunctival degenerations, inflammatory conditions, staphylomata etc. Cataract surgery was performed on 51 people (40.8% of those with all diagnosed with cataract and 61.4% of those with mature cataract. Other conditions that could not be treated on location were referred to the teaching hospital and the state hospital nearby. The postoperative cases were also referred to the same hospitals for followup. Follow up showed that majority (n=38, 74.5%) attained 6/24 to 6/60 VA range by the end of the first week with improvement to the 6/12 to 6/18 range by the end of the first month in 30 (58.8%) of the patients. CONCLUSION: It is highly desirable for periodic eye camps to take place in Rivers state as it was an eye opener that even within the township, most people who are avoidably blind are within easy reach of secondary and tertiary health facilities that can offer eye care. Satisfactory visual outcome was obtained in the majority of cases operated upon. Government is within their rights to fund subsequent programs adequately as their input is necessary for sustainability. This will help reduce the burden of the cataract blind in the state though it is not likely that V2020 will be achieved at the current rate.