Evaluation of macular and optic nerve head parameters in amblyopic eyes: An optical coherence tomography study.

PURPOSE: To evaluate morphological differences in retinal nerve fibers layers (RNFL), optic nerve head (ONH), ganglion cell complex (GCC), and macular thickness between amblyopic and normal eyes from spectral domain optical coherence tomography (SD-OCT). METHODS: Of 234 eyes of 117 children, four groups emerged: group A (162 eyes of 81 non-amblyopic subjects); group B (32 fellow eyes of 32 subjects with monolateral amblyopia); group C (32 amblyopic eyes of 32 subjects affected by monolateral amblyopia); group D (8 amblyopic eyes of 4 subjects with bilateral amblyopia). Patients underwent SD-OCT for ONH parameters, RNFL, GCC and macular thickness, retina map, and ONH scan quality index (SQI). Two-sided p values <0.05 were taken as statistically significant. (Analysis: STATA v.13). RESULTS: Parameters with a significant difference between groups (p < 0.005) with their Standard Deviation (SD) are presented: rim area, 2.08 (0.49) mm2 in group A and 1.76 (0.68) mm2 in group C; disk area, 2.43 (0.45) mm2 in group A and 2.02 (0.71) mm2 in group C; central macular thickness, 250.99 (19.74) µm in group A and 267.16 (23.52) µm in group C; nerve fiber ONH SQI, 62.82 (13.15) in group A, 51.26 (15.55) in group C, 48.29 (14.37) in group D; retina map SQI, 63.34 (10.34) in group A and 57.34 (9.84) in group C. For other parameters no significant difference was observed (p > 0.005). CONCLUSIONS: Amblyopia may influence optic nerve morphology, central macular thickness, and OCT scan quality. RNFL and GCC are not affected by monolateral amblyopia.

Optical coherence tomography angiography in healthy children: A comparison of macular structure.

PURPOSE: The purpose of the present study was to investigate the perfusion density (PD) of macular superficial (SCP) and deep capillary plexus (DCP), the size of foveal avascular zone (FAZ) and central macular thickness (CMT) in healthy children using optical coherence tomography angiography (OCT-A). PATIENTS AND METHODS: About 206 eyes of 111 children were analyzed. The correlation of gestational age (GA), birth weight (BW), age, sex, refractive errors, and visual acuity (VA) with OCT-A parameters were investigated. RESULTS: The mean PD of the fovea and the mean FAZ area of SCP were 17.1% (DS: 4.26) and 234.47 (DS: 106.39) µm2. The mean PD of the fovea and the mean FAZ area of DCP were 13.5% (DS: 5.23) and 298.32 (DS: 112.37) µm2. Superficial and deep FAZ areas were not correlated with sex, age, BW, refractive errors, or VA. FAZ area of SCP was correlated with foveal PD (r = -0.76) and with CMT (r = -0.68). FAZ area of DCP was correlated with foveal's PD (r = -0.61). There was no correlation between CMT and refractive errors. CONCLUSION: OCT-A may provide a non-invasive and reliable approach to evaluate macular perfusion in children. As the FAZ area, PD, and CMT change during the growth period, we performed established a reference range for different ages.

Oral vitamin A supplementation for ROP prevention in VLBW preterm infants.

Vitamin A administration may decrease any stage of retinopathy of prematurity (ROP) in preterm infants. To evaluate whether vitamin A oral supplementation could be preventive in ROP incidence and severity in VLBW infants, we compared results from 31 preterm infants, (< 1500 g or < 32 weeks) who, during a previous investigation, prospectively received 3000 UI/kg/die oral retinol palmitate drops, for 28 days, with 31 matching preterm newborns hospitalized in our NICU the same period, as control group. Although ROP incidence was similar, in the supplemented group, we had 9 cases of ROP grade 1, no ROP grade ≥ 2, in the un-supplemented group, 4 cases of ROP grade 1 and 6 ROP grade ≥ 2 (p = 0.018). The percentage of babies requiring treatment for ROP was 0 in treated and 16.6 in the un-treated group (p = 0.020). Moreover, Vitamin A administration showed a protective effect with an 88% risk reduction of developing severe ROP. Since vitamin A parenteral/IM administration presents some awareness, the results of this investigation may be important to plan further trials to confirm the usefulness of oral administration in mitigating the ROP severity of VLBW infants.ClinicalTrials.gov NCT02102711; may 03/06/2014.

Morpho-functional survey in children suspected of inherited retinal dystrophies via video recording, electrophysiology and genetic analysis.

PURPOSE: To present a detailed study matching functional response and video imaging with genetic analysis in children suspected of inherited retinal dystrophy (IRD). METHODS: Sixteen children underwent fundus examination via video recording (Heine Omega 500 indirect ophthalmoscope with DV1 camera) and electroretinogram (ERG) under general anesthesia to investigate the cause of suspected low vision. The patients [median age 12 (interquartile range 8-57.5) months] had associated genetic analysis performed with next-generation sequencing or array-comparative genomic hybridization. RESULTS: Four children had potential pathogenic variants in genes involved in Leber congenital amaurosis and Joubert syndrome (NMNAT1, CEP290, KCNJ13, IMPDH1); 1 child had a 16p11.2 microdeletion and 1 in 2q22.1. The ERG was altered in 6 patients, fundus imaging showed serious abnormality matching an IRD in 7 children, and less severe fundus alterations were found in 2 subjects. CONCLUSION: Fundus imaging associated with ERG may be significant in IRD diagnosis and visual impairment prognosis, alongside genetic analysis and therapy in selected cases.

Screening and Follow-Up of Acute ROP: Reproducibility of Fluorescein Angiography.

INTRODUCTION: Binocular indirect ophthalmoscopy (BIO) is fundamental for screening of retinopathy of prematurity (ROP). Digital retinal imaging devices with fluorescein angiography (FA) proved to be useful in screening and management of ROP. FA provides valuable additional information that is not detectable through ophthalmoscopy. FA images are relatively easy to interpret even by personnel without specific experience in ROP. The aim of this study is to evaluate reproducibility of FA for the screening and follow-up of ROP. METHODS: A total of 106 pairs of FA images of 30 eyes of 15 premature infants with stage II ROP were evaluated by 5 ophthalmologists: 2 experts, 2 non-experts, and 1 expert in reading FA in adult patients. Each operator gave a score to each of following parameters: leakage, ischemic areas, peripheral plus disease and vascular anomalies. The images were reviewed twice. Intra- and inter-concordance between the readers of the FA findings was evaluated by the means of Cohen's kappa coefficient (κ). RESULTS: The intra-operator concordance was very good (κ > 0.81) for all FA findings. Inter-operator concordance was good (κ > 0.41) for all operators and all FA findings. Global concordance was: substantial (intra-inter readers: κ > 0.61) for leakage, ischemic areas, and plus disease; almost perfect (κ > 0.81) for vascular anomalies; and moderate (κ = 0.41-0.60) for continuity/discontinuity of the ischemic areas. Total FA score was directly correlated to the percentage of treatment: a score ≥ 7 was correlated with 100% treatment and a score ≤ 3 with no treatment. Treatment timing was inversely correlated to FA score: a score ≥ 8 was correlated with a timely treatment (≤ 6 days), and a score ≤ 7 was correlated with a delayed treatment (< 10 days). CONCLUSION: This study showed that FA represents a reproducible imaging technique. It is useful for detecting ROP progression, and to define the treatment timing and type.

Fluorescein angiography-based diagnosis for retinopathy of prematurity: expert-non expert comparison.

PURPOSE: To evaluate accuracy and inter-rater reliability of RetCam fundus images and digital camera fluorangioscopic images in acute retinopathy of prematurity (ROP) by comparing diagnoses given by trainee ophthalmologists with those provided by expert ophthalmologists. 
 METHODS: This is a multicenter retrospective observational study of diagnostic data from 48 eyes of 24 premature infants with classical ROP, stage II, as evaluated by RetCam 3 and fluorescein angiography (FA). Average gestational age was 25.4 weeks, average weight 804.7 g. A staging grid (with ocular fundus divided into 3 concentric zones) and 24 15° sectors centered around the optic papilla were superimposed on 360° retina photomontages (Photoshop) made from RetCam and FA images. Non expert vs expert diagnosis agreement was measured for each sector by means of Cohen kappa (Fleiss, 1981).
 RESULTS: A high degree of concordance was found. Inter-rater agreement between expert and non expert interpretations of retinal photomontages was greater for fluorangiographic images than for RetCam images, with κ = 0.61-1 for 120/152 (78.9%) sectors examined on the RetCam images and κ = 0.61-1 for 168/198 (84.8%) sectors examined on the FA images.
 CONCLUSIONS: The FA images appear to be easier to interpret than RetCam images, both by expert and non expert ophthalmologists. The results confirm that FA is a good examination technique with a high degree of reliability, even where trainee practitioners are involved. This suggests that retinopathy management can be improved by entrusting diagnostic responsibilities to trainee ophthalmologists, in order to extend access to correct diagnosis, recognition of threshold lesions, and prompt treatment.