Characterization of Visual Pathway Abnormalities in Infants With Congenital Zika Syndrome Using Computed Tomography and Magnetic Resonance Imaging.

BACKGROUND: Severe visual impairment is present in nearly all infants with congenital Zika syndrome (CZS); however, ocular abnormalities are present only in a subset of these infants. The purpose of this study was to characterize the visual pathway abnormalities seen on computed tomography (CT) and MRI scans in infants with CZS. METHODS: Preliminary neuroimaging information was obtained from a referred sample of 105 infants with clinical and epidemiologic data consistent with CZS in the Pernambuco state of Brazil. Subjects were excluded if Zika virus infection was not confirmed by serologic or cerebrospinal fluid studies or if images were nondiagnostic. Of the 105 subjects initially screened, head CT images adequate for interpretation were available for 54, and brain MRI images adequate for interpretation were available for 20. Four patients had both CT and MRI images. Magnetic resonance imaging and CT scans from infants with CZS were systematically reviewed for globe malformations, optic nerve and chiasmal atrophy, occipital cortical volume loss, white matter abnormalities, ventriculomegaly, and calcifications. Neuroimaging findings were correlated with measures of visual function and with ocular examinations in these infants. RESULTS: Thirty-three males and 37 females were included in the analysis. The mean age of the infants at the time of neuroimaging was 16.0 weeks (range 0 days-15.5 months), and the mean gestational age at the time of birth was 38 weeks. All patients were from the Pernambuco state of Brazil. Overall, 70 of 74 (95%) scans showed occipital volume loss, whereas 9 (12%) showed optic nerve atrophy, 3 (4%) showed chiasmal atrophy, and 1 (1%) showed an ocular calcification. Sixty-two of the infants underwent ophthalmologic examinations. A total of 34 (55%) infants had at least one documented structural ocular abnormality, and 26 (42%) had at least one structural ocular abnormality documented in both eyes. Of those with available visual acuity data, all had visual impairment. Among those with visual impairment and normal eye examinations, 100% had visual pathway abnormalities on neuroimaging, including 100% with occipital cortical volume loss, 8% with optic nerve atrophy, and 8% with chiasmal atrophy. CONCLUSION: Our results suggest that cortical visual impairment related to structural abnormalities of the occipital cortex is likely an important cause of visual impairment in children with CZS with normal eye examinations.

Another piece of the Zika puzzle: assessing the associated factors to microcephaly in a systematic review and meta-analysis.

BACKGROUND: Although it is known that Zika virus (ZIKV) infection during pregnancy may lead to microcephaly in the fetus, the prognostic factors associated with this tragic disorder remain unclear. We conducted a systematic review and meta-analysis to assess the prognostic factors associated with the incidence of microcephaly in congenital ZIKV infection. METHODS: We conducted a comprehensive search in Ovid MEDLINE, Ovid MEDLINE (R) Epub ahead of print, Embase, Embase Classic, Web of Science, CINAHL, Cochrane CENTRAL, LILACS, and various thesis databases to identify human studies reporting microcephaly associated with congenital ZIKV infection. We requested primary data from the authors of the included studies to calculate summary estimates and conduct the meta-analysis of the most prevalent factors. RESULTS: We screened 4106 titles and abstracts, and identified 12 studies for inclusion in the systematic review. The assessment of ZIKV infection and the definition of microcephaly varied among studies. A total of 6154 newborns/fetuses were enrolled; of those, 1120 (18.20%) had a diagnostic of ZIKV infection, of which 509 (45.45%) were diagnosed with microcephaly. Nine studies addressed the link between congenital ZIKV infection and neurological findings in newborns/fetuses. Half of the studies provided primary data. Three out of 11 factors of interest seem to be prognostic factors of microcephaly: infant's sex - males compared to females: Relative Risk (RR) 1.30, 95% Confidence Interval (95% CI) 1.14 to 1.49; the stage of pregnancy when infection occurred - infection in the first trimester of pregnancy compared to infection at other stages of pregnancy: RR 1.41, 95% CI 1.09 to 1.82; and asymptomatic infection compared to symptomatic infection during pregnancy: RR 0.68; 95% CI 0.60 to 0.77. CONCLUSION: Our findings support the female-biased resistance hypothesis and reinforce the risk associated with the stage of pregnancy when ZIKV infection occurs. Continued surveillance of ZIKV infection during pregnancy is needed to identify additional factors that could contribute to developing microcephaly in affected fetuses. PROTOCOL REGISTRATION: This systematic review was registered with the International Prospective Register of Systematic Reviews (PROSPERO), registration no. CRD 42018088075.

Zika virus and the eye.

PURPOSE OF REVIEW: The aim of this study was to review the ocular findings related to the Zika virus (ZIKV) based on the main studies published to date, describe the patterns of the lesions and risk factors, and identify the public health implications and scientific importance of this emerging disease. RECENT FINDINGS: In most studies, the ZIKV seems related to congenital ocular lesions and most mothers reported mild symptoms during the first pregnancy trimester. Five fundus patterns were seen most often: macular chorioretinal atrophy, chorioretinal atrophy elsewhere, focal pigmentary changes in the macular region, optic nerve abnormalities and combined types. A few studies have suggested that the ZIKV might damage the anterior segment of these babies' eyes. Few reports have described the ocular complications seen in adults during the acute infection, including conjunctivitis, iridocyclitis and chorioretinitis. SUMMARY: Infants with congenital Zika syndrome might have vision-threatening fundus abnormalities. Although the full spectrum of ocular lesions caused by the ZIKV infection is not yet determined, a distinctive new disease has been observed. Recognition of these lesions by ophthalmologists can help ensure appropriate etiologic evaluation and clinical investigation to define the range of anomalies in an affected infant and determine essential follow-up and ongoing care.

Long-term visual and neurodevelopmental outcomes in children with Congenital Zika Syndrome after undergoing strabismus surgery.

Purpose: To assess long-term visual and neurodevelopmental outcomes in children with congenital Zika syndrome (CZS) after strabismus surgery. Methods: A consecutive sample of five children with CZS who underwent strabismus surgery was enrolled. All children underwent a standardized pre- and postoperative protocol including binocular best-corrected visual acuity (BCVA) using the Teller Acuity Cards II (TAC II), ocular alignment, functional vision using the functional vision developmental milestones test (FVDMT), and neurodevelopmental milestone evaluation using the Bayley Scales of Infant Development-Third Edition (BSID-III). Scores of the FVDMT outcomes considering the child's developmental age based on the BSID-III score were compared with scores from postoperative assessment. Results: Five children with CZS (3 girls, 2 boys) were enrolled with a mean age at baseline (preoperative) of 35.0 ± 0.7 months (range, 34-36 months) and at final assessment of 64.4 ± 0.5 months (range, 64-65 months). Preoperative BCVA was 1.2 ± 0.5 logMAR and at final assessment 0.7 ± 0.1 logMAR. Successful strabismus surgery outcome was maintained in 4/5 (80.0%) of children at final assessment. The children's BSID-III scores showed significant neurodevelopment delay at the initial assessment (corresponding developmental mean age was 4.7 months) and at their final assessment (corresponding developmental mean age was 5.1 months). There was improvement or stability in 34/46 items evaluated in the FVDMT (73.9%) when comparing baseline with 2-year follow-up. Conclusions: Strabismus surgery resulted in long-term ocular alignment in the majority of children with CZS. All the children showed improvement or stability in more than 70.0% of the functional vision items assessed. Visual and neurodevelopmental dysfunction may be related to complex condition and associated disorders seen in CZS including ocular, neurological, and skeletal abnormalities.

Effect of the COVID-19 pandemic on cataract surgery by residents who had routine surgical simulator training during residency.

PURPOSE: To assess the effect of the Coronavirus disease 2019 (COVID-19) pandemic on cataract surgery by residents who had mandatory surgical simulator training during residency. METHODS: In this retrospective, observational analytical study, the total number of cataract surgeries and surgical complications by all senior residents of 2019 (2019 class; prepandemic) and 2020 (2020 class; affected by the reduced number of elective surgeries due to the COVID-19 pandemic) were collected and compared. All residents had routine mandatory cataract surgery training on a virtual surgical simulator during residency. The total score obtained by these residents on cataract challenges of the surgical simulator was also evaluated. RESULTS: The 2020 and 2019 classes performed 1275 and 2561 cataract surgeries, respectively. This revealed a reduction of 50.2% in the total number of procedures performed by the 2020 class because of the pandemic. The incidence of surgical complications was not statistically different between the two groups (4.2% in the 2019 class and 4.9% in the 2020 class; p=0.314). Both groups also did not differ in their mean scores on the simulator's cataract challenges (p<0.696). CONCLUSION: Despite the reduction of 50.2% in the total number of cataract surgeries performed by senior residents of 2020 during the COVID-19 pandemic, the incidence of surgical complications did not increase. This suggests that surgical simulator training during residency mitigated the negative effects of the reduced surgical volume during the pandemic.

Central retinal artery occlusion with cilioretinal artery sparing secondary to COVID-19: Additional ocular complication.

We describe a 68-year-old female patient with unilateral central retinal artery occlusion (CRAO) with cilioretinal artery sparing post-coronavirus disease 2019 (COVID-19) infection. The patient presented with acute vision loss in the left eye 11 days after discharge from a severe COVID-19 infection, with altered D-dimer, C-reactive protein, and fibrinogen levels. The best-corrected visual acuity (BCVA) in the left eye was 20/400; fundoscopic examination revealed diffuse pallor retina with a patent arterial branch from the optic disk to the fovea, confirmed by fluorescein angiography. CRAO with a cilioretinal artery sparing post-COVID-19 may be considered an additional ocular manifestation of the post-acute COVID-19 syndrome spectrum.

Analysis of ocular surface and quality of life in patients with corneal and conjunctival tumors.

PURPOSE: To evaluate the impact of corneal and conjunctival tumors on the ocular surface and quality of life of patients before and after surgical treatment. METHODS: This prospective study conducted a preoperative and 30- and 90-day postoperative assessment of patients diagnosed with conjunctival and corneal tumors. Demographic data were collected preoperatively. The 12-Item Short-Form Health Survey (SF-12) and Ocular Surface Disease Index (OSDI) questionnaires were applied to assess patients' quality of life and perception of their vision-related functions. The tear breakup time and Schirmer tests were performed for ocular surface evaluation. The tumor extensions were measured using ImageJ image analysis software. RESULTS: Twenty-three patients were enrolled. The mean age at examination was 52.8 ± 17.3 years (range: 27-9 years). The most common tumor type was squamous cell carcinoma (61.5%). The patients' visual acuity improved significantly at 1 month and 3 months (p=0.018 and p=0.036, respectively). No significant differences were found between tear breakup time and Schirmer tests preoperatively and at 3 months postoperatively (p=0.150 and p=0.490, respectively). The SF-12 scores demonstrated significant differences between the preoperative and 30- and 90-day postoperative mental components (p=0.008 and p=0.026, respectively). Tumor extension was 868.7 ± 344.9 pixels (range, 224.6-1481.6 pixels) and were significantly correlated with the preoperative (p=0.011), 30-day postoperative (p=0.017), and 90-day postoperative (p=0.012) SF-12 mental components, as well as the emotional component at the 30th postoperative day (p=0.016). CONCLUSION: Patients with corneal and conjunctival tumors improved their ocular symptoms, visual acuity, and the emotional component of their quality of life after surgical excision of the tumor.

Astigmatism Profile in a Large Series of Brazilian Patients.

PURPOSE: To assess anterior, posterior, and total corneal astigmatism in a large sample of Brazilian patients. METHODS: In this retrospective cross-sectional study, all patients whose corneas were imaged with the Galilei G6 (Ziemer Ophthalmology) between January 2017 and February 2019 at HOPE Eye Hospital, in Recife, Brazil, were eligible to participate. Anterior, posterior, and total corneal astigmatism values were collected and analyzed. RESULTS: The study included 3,253 eyes of 1,919 patients. The mean magnitude of the anterior, posterior, and total corneal astigmatism was 1.50 ± 1.11, 0.34 ± 0.15, and 1.29 ± 0.98 diopters (D), respectively. Corneal astigmatism was greater than 0.50 D in the anterior cornea of 86.3% of eyes (2,807 eyes) and in the posterior cornea of 13.2% of eyes (429 eyes). Vertical alignment of the steepest corneal meridian was observed in the anterior cornea of 74.5% of eyes (2,423 eyes) and in the posterior cornea of 93.1% of eyes (3,029 eyes). The correlation between the astigmatism magnitude of the anterior and posterior cornea was strong when the steep anterior meridian was aligned vertically (r = 0.720; P < .001), and absent when it was aligned horizontally (r = 0.102; P = .036). CONCLUSIONS: Corneal astigmatism values in the Brazilian population were similar to those found in other ethnicities, suggesting that toric calculators, nomograms, coefficients of adjustment, and formulas that were developed based on astigmatism values of other populations may be used in Brazilian patients with comparable accuracy. [J Refract Surg. 2023;39(1):56-60.].

Telangiectatic Capillaries in Retinal Vascular Occlusions: Multimodal Imaging in a Case Series.

Introduction: To report a cases series of retinal vascular occlusions with telangiectatic capillaries (TelCaps) seen on indocyanine green angiography (ICGA) and multimodal imaging. Methods: In this case series, a new finding (TelCaps) was seen on clinical examination, fundus evaluation, fluorescein angiography, ICGA, and optical coherence tomography (OCT). Results: This series comprised 3 patients with TelCaps findings on ICGA after retinal vascular occlusions. The patients' ages ranged from 52 years to 71 years and the best-corrected visual acuity in the affected eye, from 20/25 to 20/80. Fundus evaluation showed small, hard exudates in the vascular termination close to the macula with a reduction of the foveal reflex. The OCT images showed marginal hyperreflectivity and inner hyporeflectivity that were suggestive of a TelCaps lesion, which was confirmed by hyperfluorescence in the late phase of ICGA. Conclusions: This study highlights the importance of performing multimodal imaging evaluation, including ICGA, in eyes with retinal vein occlusions for early identification and management of the associated lesions.

Characterization of Errors in Retinopathy of Prematurity Diagnosis by Ophthalmologists-in-Training in Middle-Income Countries.

PURPOSE: To characterize common errors in the diagnosis of retinopathy of prematurity (ROP) among ophthalmologistsin-training in middle-income countries. METHODS: In this prospective cohort study, 200 ophthalmologists-in-training from programs in Brazil, Mexico, and the Philippines participated. A secure web-based educational system was developed using a repository of more than 2,500 unique image sets of ROP, and a reference standard diagnosis was established by combining the clinical diagnosis and the image-based diagnosis by multiple experts. Twenty web-based cases of wide-field retinal images were presented, and ophthalmologists-in-training were asked to diagnose plus disease, zone, stage, and category for each eye. Trainees' responses were compared to the consensus reference standard diagnosis. Main outcome measures were frequency and types of diagnostic errors were analyzed. RESULTS: The error rate in the diagnosis of any category of ROP was between 48% and 59% for all countries. The error rate in identifying type 2 or pre-plus disease was 77%, with a tendency for overdiagnosis (27% underdiagnosis vs 50% overdiagnosis; mean difference: 23.4; 95% CI: 12.1 to 34.7; P = .005). Misdiagnosis of treatment-requiring ROP as type 2 ROP was most commonly associated with incorrectly identifying plus disease (plus disease error rate = 18% with correct category diagnosis vs 69% when misdiagnosed; mean difference: 51.0; 95% CI: 49.3 to 52.7; P = .003). CONCLUSIONS: Ophthalmologists-in-training from middle-income countries misdiagnosed ROP more than half of the time. Identification of plus disease was the salient factor leading to incorrect diagnosis. These findings emphasize the need for improved access to ROP education to improve competency in diagnosis among ophthalmologists-in-training in middle-income countries. [J Pediatr Ophthalmol Strabismus. 2023;60(5):344-352.].

Firework-related ocular trauma in Pernambuco, Brazil.

PURPOSE: This study aimed to describe the demographic and clinical characteristics of victims of fireworkrelated ocular trauma treated at the ophthalmologic emergency de partments of two reference centers in Pernambuco, Brazil, and to identify risk factors related to poor visual prognosis. METHODS: We retrospectively evaluated the medical records of patients admitted in emergency departments with a report of firework-related trauma between January 2012 and December 2018. Data collected included patient's age, sex, place of origin, month and year of the accident, ocular structures affected, characteristics of the injuries, and type of treatment that patients received. For patients who were followed for >30 days, the final visual acuity and patient's origin were analyzed. RESULTS: Three hundred and seventy eyes from 314 patients were included, of which 248 (79.0%) were male and 160 (51.0%) were from the metropolitan region of Recife. The mean patient age was 25.6 ± 18.8 years. In 56 (17.8%) patients, the ocular trauma was bilateral. A total of 152 (48.4%) cases occurred in June. The most affected sites were the eyelids in 91 (24.6%) eyes and ocular surface in 252 (68.1%). Surgical treatment was required in 87 (23.5%) eyes. After clinical and surgical management, 37 (10.0%) eyes presented final visual acuity of <20/400. Of these, 34 (91.9%) eyes were from patients from the countryside or from another state. Patients from the countryside presented higher risk of developing blindness after a firework trauma than those from the metropolitan area (odds ratio of 5.46). CONCLUSIONS: Victims of firework-related ocular trauma were mostly male, from the metropolitan region of Pernambuco state and mainly pediatric patients or economically active. Those coming from the countryside and other states had higher risk of developing blindness.

Effects of COVID-19 pandemic on stress level of residents and fellows during ophthalmology training.

Purpose: To evaluate the impact of coronavirus disease 2019 (COVID-19) on the mental health of residents and fellows in ophthalmology. Methods: A questionnaire composed of 42 questions was applied to Brazilian residents and fellows in Ophthalmology. The questionnaire addressed the demographics of participants, their working conditions before and during the COVID-19 pandemic, and the Perceived Stress Scale (PSS-10). Results: The study had a total of 271 participants in Ophthalmology training, from which 100 were fellows and 171 were residents. Before the pandemic, Ophthalmology residents and fellows had a higher workload (P < 0.001), and residents worked more hours than fellows (P = 0.001). During the pandemic, the workload of both residents and fellows decreased and equalized (P = 0.195). No correlation was found between the working hours during the pandemic and their stress level were observed (P = 0.760). Higher stress scores were identified in women ophthalmologists (P = 0.001) as well as in residents and fellows that had their surgical training interrupted during the pandemic (P < 0.001). Conclusion: The stress level of residents and fellows during the COVID-19 pandemic was similar, however, those that had their surgical training interrupted during the pandemic presented higher level of stress. Female physicians also presented higher level of stress compared to male physicians.

Isolated abducens nerve palsy associated with coronavirus disease: an 8-month follow-up.

We report the case of a previously healthy 48-year-old man who developed an isolated abducens nerve palsy 18 days after presenting with coronavirus disease (COVID-19) confirmed by reverse transcriptase polymerase chain reaction. His main complaint at arrival was double vision. Ocular examination revealed a sixth cranial nerve palsy in the left eye. The incomitant esotropia at arrival was 30 prism diopters. Abduction was markedly limited, while adduction was normal in the left eye. The patient underwent complete clinical, neurological, and neuroimaging investigations, including cerebrospinal fluid sample analysis to rule out infectious causes. A conservative approach with orthoptic therapy and Fresnel prism was opted. Eight months after the onset of COVID-19, regression of the strabismus was observed, and the patient reported complete recovery of the diplopia. This case suggests that isolated abducens nerve palsy caused by severe acute respiratory syndrome coronavirus 2 infection may improve with a conservative approach.

Ophthalmological manifestations of the Schuurs-Hoeijmakers syndrome: a case report.

This is a case report of a 2-year-old male patient with cognitive delay, facial abnormalities, and microcornea in the right eye, who was referred for ophthalmological investigation. The initial ophthalmological examination revealed hypertelorism, epicanthus, nystagmus, esotropia, and microcornea in the right eye. The examination under anesthesia revealed microphthalmia in the right eye, and iris, retina, and optic nerve coloboma in both eyes. Whole exome sequencing revealed evidence of a heterozygotic pathogenic variant in PACS1. The PACS1 pathogenic variant in association with the clinical findings confirmed the diagnosis of Schuurs-Hoeijmakers syndrome. To our knowledge, this is the first report to describe microcornea and microphthalmia as additional ocular manifestations of Schuurs-Hoeijmakers syndrome.

Epidemiological and clinical analyses of corneal transplants performed in a reference eye center in Recife, Brazil.

PURPOSE: To evaluate the epidemiological and clinical profiles of corneal transplants performed in a reference eye center in Recife, state of Pernambuco, Northeastern Brazil. METHODS: This cross-sectional study collected epidemiological and clinical data from the medical records of patients who underwent keratoplasty at the Altino Ventura Foundation between January and December 2017. RESULTS: A total of 356 procedures were performed in 327 patients, of whom 165 (50.5%) were female. The mean age at surgery was 50.9 ± 22.6 years (range, 10-89 years). Most patients (n=152 [46.5%]) were from the capital and metropolitan areas. The mean waiting time for keratoplasty was 52.4 ± 58.9 days (range, 0-460 days). The main indications for keratoplasty were infectious keratitis (n=88 [24.7%]), keratoconus (n=80 [22.5%]), and previous transplant failure (n=75 [21.1%]). Penetrating keratoplasty was the most common surgical technique performed (n=213 [59.9%]) and more frequently performed in men (n=132 [76.7%]), whereas posterior lamellar transplant (n=143 [41.1%]) was more frequently performed in women (p<0.001). CONCLUSION: Infectious keratitis was the main indication for keratoplasty, which was similarly performed in economically active adults of both sexes. Penetrating keratoplasty was more frequently performed in men and lamellar transplants in women.

Neurodevelopment in Children Exposed to Zika in utero: Clinical and Molecular Aspects.

Five years after the identification of Zika virus as a human teratogen, we reviewed the early clinical manifestations, collectively called congenital Zika syndrome (CZS). Children with CZS have a very poor prognosis with extremely low performance in motor, cognitive, and language development domains, and practically all feature severe forms of cerebral palsy. However, these manifestations are the tip of the iceberg, with some children presenting milder forms of deficits. Additionally, neurodevelopment can be in the normal range in the majority of the non-microcephalic children born without brain or eye abnormalities. Vertical transmission and the resulting disruption in development of the brain are much less frequent when maternal infection occurs in the second half of the pregnancy. Experimental studies have alerted to the possibility of other behavioral outcomes both in prenatally infected children and in postnatal and adult infections. Cofactors play a vital role in the development of CZS and involve genetic, environmental, nutritional, and social determinants leading to the asymmetric distribution of cases. Some of these social variables also limit access to multidisciplinary professional treatment.