Catherin Negron

First Travel-Associated Congenital Zika Syndrome in the US: Ocular and Neurological Findings in the Absence of Microcephaly.

A 6-day-old female baby with known diagnosis of congenital Zika infection was referred for ophthalmologic examination. The mother (37 years old) was referred for a pruritic rash, conjunctival hyperemia, and malaise at 12 weeks of gestation while still living in Venezuela. Upon arrival to Miami, Zika virus (ZIKV) exposure was confirmed during prenatal screening. At birth, due to the known exposure, a complete congenital ZIKV workup was performed, including brain ultrasound and MRI, which disclosed calcifications in the frontal lobe. Fundus examination revealed a hypopigmented retinal lesion in the left eye that was documented with retinal imaging. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:952-955.].

Plus Disease in Retinopathy of Prematurity: Improving Diagnosis by Ranking Disease Severity and Using Quantitative Image Analysis.

PURPOSE To determine expert agreement on relative retinopathy of prematurity (ROP) disease severity and whether computer-based image analysis can model relative disease severity, and to propose consideration of a more continuous severity score for ROP. DESIGN We developed 2 databases of clinical images of varying disease severity (100 images and 34 images) as part of the Imaging and Informatics in ROP (i-ROP) cohort study and recruited expert physician, nonexpert physician, and nonphysician graders to classify and perform pairwise comparisons on both databases. PARTICIPANTS Six participating expert ROP clinician-scientists, each with a minimum of 10 years of clinical ROP experience and 5 ROP publications, and 5 image graders (3 physicians and 2 nonphysician graders) who analyzed images that were obtained during routine ROP screening in neonatal intensive care units. METHODS Images in both databases were ranked by average disease classification (classification ranking), by pairwise comparison using the Elo rating method (comparison ranking), and by correlation with the i-ROP computer-based image analysis system. MAIN OUTCOME MEASURES Interexpert agreement (weighted κ statistic) compared with the correlation coefficient (CC) between experts on pairwise comparisons and correlation between expert rankings and computer-based image analysis modeling. RESULTS There was variable interexpert agreement on diagnostic classification of disease (plus, preplus, or normal) among the 6 experts (mean weighted κ, 0.27; range, 0.06-0.63), but good correlation between experts on comparison ranking of disease severity (mean CC, 0.84; range, 0.74-0.93) on the set of 34 images. Comparison ranking provided a severity ranking that was in good agreement with ranking obtained by classification ranking (CC, 0.92). Comparison ranking on the larger dataset by both expert and nonexpert graders demonstrated good correlation (mean CC, 0.97; range, 0.95-0.98). The i-ROP system was able to model this continuous severity with good correlation (CC, 0.86). CONCLUSIONS Experts diagnose plus disease on a continuum, with poor absolute agreement on classification but good relative agreement on disease severity. These results suggest that the use of pairwise rankings and a continuous severity score, such as that provided by the i-ROP system, may improve agreement on disease severity in the future.

Aggressive Posterior Retinopathy of Prematurity and a TUBA1A Mutation inde Morsier Syndrome

The authors report a case of a premature male neonate born at 25.3 weeks gestational age weighing 605 grams with septo-optic dysplasia (SOD) and a heterozygous mutation in TUBA1A c.715A>C, a critical gene for microtubules, who developed asymmetric and aggressive posterior retinopathy of prematurity (ROP). This report presents a novel mutation associated with SOD and proposes that optic nerve hypoplasia may have potentiated a severe ROP phenotype. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:629-632.].

Peripheral Vascular Abnormalities Seen by Ultra-Widefield Fluorescein Angiography in Adults With History of Prematurity: Report of Three Cases

Retinopathy of prematurity (ROP) may lead to late-developing complications due to the abnormal vitreoretinal interface. In this case report, three adult patients (ages 39 years, 62 years, and 64 years) with a history of prematurity underwent imaging with ultra-widefield fluorescein angiography (UWFFA). The UWFFA demonstrated late peripheral vascular hyperfluorescence in all three patients, perivascular leakage in two of the patients, hyperfluorescent foveal avascular zone in two of the patients, peripheral avascular retina in two of the patients, vasoproliferative tumor in one of the patients, peripheral neovascularization in one of the patients, and vascular anastomosis in one of the patients. Descriptive analysis of the images is reported. The authors concluded UWFFA reveals details in adults with history of ROP that may gain importance during the follow-up or in the surgical management of these patients; however, prospective studies are needed. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:278-283.].

Subretinal Cysticercosis Extraction With Bimanual, 3-D, Heads–Up-Assisted Pars Plana Vitrectomy: Clinicopathological Correlation and Surgical Technique

A 16-year-old male presented with blurred vision in the right eye after recent travel to Nicaragua. Funduscopic examination revealed subretinal cysticercosis superior to the optic nerve. The cyst was drained and excised using a bimanual, three-dimensional, heads-up-assisted pars plana vitrectomy without complications. Technical maneuvers for cyst extraction along with clinicopathological correlation are described. Postoperatively, the patient exhibits no signs of recurrence and has excellent vision. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:708-711.].

The Use of Fluorescein Angiography to Evaluate Pediatric Abusive Head Trauma: An Observational Case Series

Background or Purpose: The evaluation of retinal hemorrhages in the setting of suspected abusive head trauma (AHT) has important medical, social, and legal implications. Challenges include late presentation after retinal hemorrhages have resolved and differentiating AHT-related retinal changes from mimicking pathologies. To date, angiographic evidence of peripheral retinal injury in AHT has been reported in small series, but further study is needed to determine the prevalence and long-term sequelae of these findings. The purpose of this study was to describe fluorescein angiography (FA) findings in pediatric patients with a history of definite AHT and demonstrate the potential utility of FA in evaluating cases of suspected abuse with late presentation. Methods: Retrospective case series of 10 eyes in 5 pediatric patients with a history of definite AHT. RetCam fundus photography images and digital FA were performed. Results: Peripheral retinal nonperfusion affected 10 of 10 eyes in patients with a history of definite AHT. These changes persisted after acute retinal hemorrhages had resolved. Conclusions: “Retinal surveys” using FA provide angiographic evidence of peripheral retinal nonperfusion that may be a sign of AHT. This series adds to the growing body of cases reporting similar AHT-related findings and additionally demonstrates the longevity of these pathologic changes after trauma, which has not been previously described. Fluorescein angiography may offer a distinct advantage in evaluating cases of suspected abuse with late presentation to medical care when pathognomonic retinal hemorrhages may no longer be present. Further study is needed to determine the prevalence and long-term sequelae of these findings.