Andrea Zin

Characteristics of infants with severe retinopathy of prematurity in countries with low, moderate, and high levels of development: implications for screening programs.

Objective. Retinopathy of prematurity (ROP) is a potentially avoidable cause of blindness in children. The proportion of blindness as a result of ROP varies greatly among countries depending on their level of development, being influenced by the availability of neonatal care, neonatal outcomes, and whether effective screening and treatment programs are in place. The objective of this study was to compare characteristics of premature infants who developed severe ROP between 1996 and 2002 in highly developed countries with less developed countries. Methods. This was an observational study. A questionnaire was completed by ophthalmologists in countries with low, moderate, and high development rankings (3 highly developed countries and from 10 less well-developed countries) who screen for ROP in which they supplied birth weights and gestational ages (GAs) of infants who were treated for threshold ROP or identified with more advanced stages of the disease. Birth weights and GAs of infants with severe ROP were measured. Results. The mean birth weights of infants from highly developed countries ranged from 737 to 763 g compared with values ranging from 903 to 1527 g in less developed countries. Mean GAs of infants from highly developed countries ranged from 25.3 to 25.6 weeks compared with 26.3 to 33.5 weeks in less developed countries. A total of 13.0% of 1091 infants from poorly developed countries exceeded United Kingdom screening criteria; 3.6% exceeded a criteria of <34 weeks’ GA and/or <1750 g birth weight. Conclusions. These findings suggest that larger, more mature infants are developing severe ROP in countries with low/moderate levels of development compared with highly developed countries. ROP screening programs need to use criteria that are appropriate for their local population.

Retinopathy of Prematurity in 7 Neonatal Units in Rio de Janeiro: Screening Criteria and Workload Implications

OBJECTIVES: The goals were to determine optimal screening criteria for retinopathy of prematurity (ROP) in 7 neonatal units in Rio de Janeiro, Brazil, and to explore the workload implications of applying different criteria. METHODS: Infants with birth weights of ≤2000 g or gestational age of <37 weeks were examined by 3 ophthalmologists in 7 of the largest units in Rio de Janeiro, during a 34-month period. ROP was classified by using the international classification, and laser treatment was given to infants developing type 1 ROP. RESULTS: A total of 3437 (87%) of 3953 eligible infants were examined, of whom 124 (3.6% [range: 2.1%–7.8%]) were treated. Eleven infants were treated for aggressive posterior ROP. Appropriate screening criteria for the 2 NICUs with high survival rates (ie, ≥80% among infants with birth weights of <1500 g) would be ≤1500 g or <32 weeks. For NICUs with low survival rates (ie, <80%), appropriate criteria would be ≤1500 g or ≤35 weeks. UK, US, and previous Brazilian criteria would all miss infants needing treatment. CONCLUSIONS: ROP programs in Brazil should use the wider criteria of ≤1500 g or ≤35 weeks until further evidence-based criteria become available, although this would mean a slight increase in workload across the city, compared with use of the narrower criteria in the better units. Whether survival rates can be used as a proxy to indicate screening criteria requires further investigation.

Proposta de diretrizes brasileiras do exame e tratamento de retinopatia da prematuridade (ROP)

Retinopathy of prematurity is one of the main causes of childhood blindness. Worldwide, there are more than 50,000 children blind due to retinopathy of prematurity. Visual impairment is a consequence of retinal detachment. It can be detected by serial ophthalmologic examination of infants at risk, and those identified with the severe form of the disease can be treated by laser or cryotherapy, which can decrease significantly the blindness due to ROP. The Brazilian Society of Pediatrics, Brazilian Council of Ophthalmology and Brazilian Society of Pediatric Ophthalmology suggest a guideline for the detection and treatment of retinopathy of prematurity in Brazil. This document was based on the results of the I Workshop of Retinopathy of Prematurity and presents the attributes for the implementation of an efficient diagnostic and treatment program.

Severe visual impairment and blindness in infants: causes and opportunities for control

Childhood blindness has an adverse effect on growth, development, social, and economic opportunities. Severe visual impairment (SVI) and blindness in infants must be detected as early as possible to initiate immediate treatment to prevent deep amblyopia. Although difficult, measurement of visual acuity of an infant is possible. The causes of SVI and blindness may be prenatal, perinatal, and postnatal. Congenital anomalies such as anophthalmos, microphthalmos, coloboma, congenital cataract, infantile glaucoma, and neuro-ophthalmic lesions are causes of impairment present at birth. Ophthalmia neonatorum, retinopathy of prematurity, and cortical visual impairment are acquired during the perinatal period. Leukocoria or white pupillary reflex can be cause by congenital cataract, persistent hyperplastic primary vitreous, or retinoblastoma. While few medical or surgical options are available for congenital anomalies or neuro-ophthalmic disorders, many affected infants can still benefit from low vision aids and rehabilitation. Ideally, surgery for congenital cataracts should occur within the first 4 months of life. Anterior vitrectomy and primary posterior capsulotomy are required, followed by aphakic glasses with secondary intraocular lens implantation at a later date. The treatment of infantile glaucoma is surgery followed by anti-glaucoma medication. Retinopathy of prematurity is a proliferation of the retinal vasculature in response to relative hypoxia in a premature infant. Screening in the first few weeks of life can prevent blindness. Retinoblastoma can be debulked with chemotherapy; however, enucleation may still be required. Neonatologists, pediatricians, traditional birth attendants, nurses, and ophthalmologists should be sensitive to a parent’s complaints of poor vision in an infant and ensure adequate follow-up to determine the cause. If required, evaluation under anesthesia should be performed, which includes funduscopy, refraction, corneal diameter measurement, and measurement of intraocular pressure.

Posterior scleral choristoma in the organoid nevus syndrome (linear nevus sebaceus of Jadassohn)

PURPOSE To highlight the association of posterior osseous and/or cartilaginous ocular choristomas with epibulbar choristomas and the nevus sebaceus of Jadassohn. DESIGN Small case series. PARTICIPANTS Four patients with the organoid nevus syndrome. METHODS Clinical and histopathologic studies in four patients with epibulbar lesions and nevus sebaceus of Jadassohn. MAIN OUTCOME MEASURES Ophthalmoscopic findings of peripapillary lesions. Computed tomographic and ultrasonographic characteristic of posterior scleral lesions. Ocular histopathologic findings in one globe from one of the study subjects. RESULTS Three patients had the triad of posterior osseous/cartilaginous ocular choristomas, anterior epibulbar choristomas, and nevus sebaceus of Jadassohn and one patient had anterior epibulbar choristomas and posterior osseous/cartilaginous ocular choristomas. Ultrasonography and computed tomography were valuable in detecting scleral ossification or epibulbar cartilage or both. The ophthalmoscopic findings were similar to those of a choroidal osteoma. CONCLUSIONS The presence of posterior osseous/cartilaginous ocular choristomas in a patient with epilepsy or epibulbar lesions or both suggests the diagnosis of nevus sebaceus of Jadassohn. Osseous/cartilaginous ocular choristomas should be suspected in patients with nevus sebaceus of Jadassohn and peripapillary hypopigmented fundus lesions.

Prevalence of retinopathy of prematurity in Latin America

The purpose of this work was to review the studies published over the last 10 years concerning the prevalence of retinopathy of prematurity (ROP) in Latin American countries, to determine if there was an improvement in ROP prevalence rates in that period, and to identify the inclusion criteria for patients at risk of developing ROP in the screening programs. A total of 33 studies from ten countries published between 2000 and 2010 were reviewed. Prevalence of any ROP stage in the regions considered ranged from 6.6% to 82%; ROP severe enough to require treatment ranged from 1.2% to 23.8%. There was no routine screening for ROP, and there was a lack of services for treatment of the disease in many countries. Inclusion criteria for patients in the studies ranged between birth weight ≤1500 g and ≤2000 g and gestational age ≤32 and <37 weeks. Use of different inclusion criteria regarding birth weight and gestational age in several Latin American studies hindered comparative analysis of the published data. Highly restrictive selection criteria for ROP screening in relation to birth weight and gestational age should not be used throughout most Latin American countries because of their different social characteristics and variable neonatal care procedures. The studies included in this review failed to provide adequate information to determine if the prevalence of ROP has decreased in Latin America.

Potential for a paradigm change in the detection of retinopathy of prematurity requiring treatment.

Retinopathy of prematurity (ROP) is a major cause of potentially avoidable blindness in children in the middle-income countries of Latin America and Eastern Europe, and is becoming a public health problem in Asia.1 Indeed, the earlier estimate that there were 50 000–60 000 children worldwide who were blind from ROP2 is a marked underestimate, as a recent systematic review suggests that annually 20 000 infants (uncertainty range 15 500–27 200) became blind or severely visually impairment from ROP worldwide in 2010, with a further 12 300 (8300–18 400) being visually impaired.3 Asia has the highest number, reflecting the rapid expansion of services for preterm infants in the region.4 ,5 The rate of severe visual loss from ROP is 1.8–2.6 times higher per million births in East Asia, the Pacific region, Latin America and Eastern Europe than in high-income countries, reflecting both a higher incidence of severe ROP and inadequate detection and treatment. The recognition that prematurity is a major cause of infant and under five mortality rates6 is leading to rapid expansion of neonatal care in many countries such as India,7 China and Russia, which will put an increasing number of infants at risk of ROP. Visual loss from ROP will continue to increase in low-income and middle-income countries with improving preterm survival rates unless there are dramatic improvements in neonatal care coupled with higher coverage of high-quality services for the detection and treatment of ROP. The vast majority of programmes for the detection and treatment of ROP rely on highly skilled ophthalmologists who visit neonatal units on a weekly basis, or more frequently, to examine infants at risk. Many middle-income countries have criteria for examination, often drawn up collaboratively by professional societies of ophthalmologists and neonatologists, and programmes are becoming integrated into health systems. Many use criteria based on …

Screening Criteria for Ophthalmic Manifestations of Congenital Zika Virus Infection

Importance Current guidelines recommend screening eye examinations for infants with microcephaly or laboratory-confirmed Zika virus infection but not for all infants potentially exposed to Zika virus in utero. Objective To evaluate eye findings in a cohort of infants whose mothers had polymerase chain reaction–confirmed Zika virus infection during pregnancy. Design, Setting, and Participants In this descriptive case series performed from January 2 through October 30, 2016, infants were examined from birth to 1 year of age by a multidisciplinary medical team, including a pediatric ophthalmologist, from Fernandes Figueira Institute, a Ministry of Health referral center for high-risk pregnancies and infectious diseases in children in Rio de Janeiro, Brazil. Participants Mother-infant pairs from Rio de Janeiro, Brazil, who presented with suspected Zika virus infection during pregnancy were referred to our institution and had serum, urine, amniotic fluid, or placenta samples tested by real-time polymerase chain reaction for Zika virus. Main Outcomes and Measures Description of eye findings, presence of microcephaly or other central nervous system abnormalities, and timing of infection in infants with confirmed Zika virus during pregnancy. Eye abnormalities were correlated with central nervous system findings, microcephaly, and the timing of maternal infection. Results Of the 112 with polymerase chain reaction–confirmed Zika virus infection in maternal specimens, 24 infants (21.4%) examined had eye abnormalities (median age at first eye examination, 31 days; range, 0-305 days). Ten infants (41.7%) with eye abnormalities did not have microcephaly, and 8 (33.3%) did not have any central nervous system findings. Fourteen infants with eye abnormalities (58.3%) were born to women infected in the first trimester, 8 (33.3%) in the second trimester, and 2 (8.3%) in the third trimester. Optic nerve and retinal abnormalities were the most frequent findings. Eye abnormalities were statistically associated with microcephaly (odds ratio [OR], 19.1; 95% CI, 6.0-61.0), other central nervous system abnormalities (OR, 4.3; 95% CI, 1.6-11.2), arthrogryposis (OR, 29.0; 95% CI, 3.3-255.8), and maternal trimester of infection (first trimester OR, 5.1; 95% CI, 1.9-13.2; second trimester OR, 0.5; 95% CI, 0.2-1.2; and third trimester OR, 0.3; 95% CI, 0.1-1.2). Conclusions and Relevance Eye abnormalities may be the only initial finding in congenital Zika virus infection. All infants with potential maternal Zika virus exposure at any time during pregnancy should undergo screening eye examinations regardless of the presence or absence of central nervous system abnormalities.

Capacity building of nurses providing neonatal care in Rio de Janeiro, Brazil: methods for the POINTS of care project to enhance nursing education and reduce adverse neonatal outcomes

BackgroundIncreased survival of preterm infants in developing countries has often been accompanied by increased morbidity. A previous study found rates of severe retinopathy of prematurity varied widely between different neonatal units in Rio de Janeiro. Nurses have a key role in the care of high-risk infants but often do not have access to ongoing education programmes. We set out to design a quality improvement project that would provide nurses with the training and tools to decrease neonatal mortality and morbidity. The purpose of this report is to describe the methods and make the teaching package (POINTS of care--six modules addressing P ain control; optimal O xygenation; I nfection control; N utrition interventions; T emperature control; S upportive care) available to others.Methods/DesignSix neonatal units, caring for 40% of preterm infants in Rio de Janeiro were invited to participate. In Phase 1 of the study multidisciplinary workshops were held in each neonatal unit to identify the neonatal morbidities of interest and to plan for data collection. In Phase 2 the teaching package was developed and tested. Phase 3 consisted of 12 months data collection utilizing a simple tick-sheet for recording. In Phase 4 (the Intervention) all nurses were asked to complete all six modules of the POINTS of care package, which was supplemented by practical demonstrations. Phase 5 consisted of a further 12 months data collection. In Phase 1 it was agreed to include inborn infants with birthweight ≤ 1500 g or gestational age of ≤ 34 weeks. The primary outcome was death before discharge and secondary outcomes included retinopathy of prematurity and bronchopulmonary dysplasia. Assuming 400-450 infants in both pre- and post-intervention periods the study had 80% power at p = < 0.05 to detect an increase in survival from 68% to 80%; a reduction in need for supplementary oxygen at 36 weeks post menstrual age from 11% to 5.5% and a reduction in retinopathy of prematurity requiring treatment from 7% to 2.5%.DiscussionThe results of the POINTS of Care intervention will be presented in a separate publication.Trial registrationCurrent Controlled Trials: ISRCTN83110114

Persistence of Zika Virus After Birth: Clinical, Virological, Neuroimaging, and Neuropathological Documentation in a 5-Month Infant With Congenital Zika Syndrome

During the Zika epidemic in Brazil, a baby was born at term with microcephaly and arthrogryposis. The mother had Zika symptoms at 10 weeks of gestation. At 17 weeks, ultrasound showed cerebral malformation and ventriculomegaly. At 24 weeks, the amniotic fluid contained ZIKV RNA and at birth, placenta and maternal blood were also positive using RT-qPCR. At birth the baby urine contained ZIKV RNA, whereas CSF at birth and urine at 17 days did not. Seizures started at 6 days. EEG was abnormal and CT scan showed cerebral atrophy, calcifications, lissencephaly, ventriculomegaly, and cerebellar hypoplasia. Bacterial sepsis at 2 months was treated. A sudden increase in head circumference occurred at 4 months necessitating ventricle-peritoneal shunt placement. At 5 months, the infant died with sepsis due to bacterial meningitis. Neuropathological findings were as severe as some of those found in neonates who died soon after birth, including hydrocephalus, destructive lesions/calcification, gliosis, abnormal neuronal migration, dysmaturation of nerve cells, hypomyelination, loss of descending axons, and spinal motor neurons. ZIKV RNA was detected only in frozen brain tissue using RT-qPCR, but infected cells were not detected by in situ hybridization. Progressive gliosis and microgliosis in the midbrain may have contributed to aqueduct compression and subsequent hydrocephalus. The etiology of progressive disease after in utero infection is not clear and requires investigation.