The long-term influence of perinatal factors on macular morphology and vessel density in preterm children.

BACKGROUND: Increased survival rate of extremely preterm children is associated with a higher risk of retinopathy of prematurity (ROP) and long-term sequelae, which implicate retinal changes. PURPOSE: To assess an influence of perinatal characteristics on morphology and retinal vascularity of the macula in preterm children. METHODS: A cohort of 123 preterm children at the age of 10.5 years (IQR: 8.12-12.77) was prospectively assessed. Optical coherence tomography angiography (OCTA) was performed using RTVueXR Avanti. Foveal thickness, parafoveal thickness, size of foveal avascular zone (FAZ), superficial and deep vessel density, central choroidal thickness (CCT) were analyzed. The associations between OCTA results and perinatal factors, including the presence of ROP and therapy requirements were assessed in preterm children. RESULTS: Significantly smaller FAZ, higher foveal thickness and vessel density were noted in children with ROP, Respiratory Distress Syndrome, Bronchopulmonary Dysplasia, required erythropoietin, transfusion or steroids. Foveal thickness was increased in children with ROP (p < 0.001) and following laser treatment (p < 0.05). Thinner CCT was noted in children with a history of sepsis (p < 0.05) and ROP required treatment (p < 0.05). Pregnancy bleeding was associated with higher superficial foveal vessel density (p < 0.05) and smaller FAZ (p < 0.05). CONCLUSION: Neonatal factors have a huge impact on retinal development, but the role of prenatal factors should not be neglected in preterm children.

Efficacy of intravitreal injections of melphalan in the treatment of retinoblastoma vitreous seeding.

BACKGROUND: The introduction of intravitreal injections of melphalan (IVIM) has significantly improved the efficacy of retinoblastoma treatment and the prognosis for eye preservation. OBJECTIVES: To evaluate the results of using IVIM to treat retinoblastoma vitreous seeding. MATERIAL AND METHODS: This was a clinical, retrospective, single-center study. Twenty-six children (27 eyes) who met all of the following inclusion criteria qualified for the study: 1) active vitreous seeding at the time of retinoblastoma diagnosis; 2) IVIM performed between 1 January 2017 and 30 September 2020; and 3) a minimum follow-up period of 12 months since the last IVIM. Doses of 20-40 µg melphalan per injection were used. RESULTS: The eye observation period from the last IVIM to the last ophthalmic examination averaged 32.41 months (median 30.00; range 13.00-56.00). Success (no active tumors in the vitreous body) was achieved in 24 eyes (88.9%), and a doubtful result (recurrence in the retina with a difficult-to-determine etiology) in 2 eyes (7.4%). In 1 eye (3.7%), despite treatment, active tumors were still present in the vitreous body. Out of all 27 eyes, 4 eyeballs were removed, but the direct cause of enucleation was not vitreous seeding. There were no complications in the form of intraocular inflammation, extraocular retinoblastoma or distant metastases. There was 1 case of anterior uveitis and 1 case of cataract. CONCLUSIONS: The IVIM is a highly effective and safe form of treatment for retinoblastoma vitreous seeding.

Mapping the chemotactic landscape in NK cells reveals subset-specific synergistic migratory responses to dual chemokine receptor ligation.

BACKGROUND: Natural killer (NK) cells have a unique capability of spontaneous cytotoxicity against malignant cells and hold promise for off-the-shelf cell therapy against cancer. One of the key challenges in the field is to improve NK cell homing to solid tumors. METHODS: To gain a deeper understanding of the cellular mechanisms regulating trafficking of NK cells into the tumor, we used high-dimensional flow cytometry, mass cytometry, and single-cell RNA-sequencing combined with functional assays, creating a comprehensive map of human NK cell migration phenotypes. FINDINGS: We found that the chemokine receptor repertoire of peripheral blood NK cells changes in a coordinated manner becoming progressively more diversified during NK cell differentiation and correlating tightly with the migratory response of the distinct NK cell subsets. Simultaneous ligation of CXCR1/2 and CX3CR1, synergistically potentiated the migratory response of NK cells. Analysis of 9471 solid cancers from publicly available TCGA/TARGET repositories revealed dominant chemokine patterns that varied across tumor types but with no tumor group expressing ligands for more than one chemokine receptor present on mature NK cells. INTERPRETATION: The finding that chemokine stimulation can elicit a synergistic migratory response in NK cells combined with the identified lack of naturally occurring pairs of chemokines-chemokine receptors in human cancers may explain the systematic exclusion of NK cells from the tumor microenvironment and provides a basis for engineering next-generation NK cell therapies against malignancies. FUNDING: The Polish Ministry of Science and Higher Education, the National Science Centre, Poland, The Norwegian Cancer Society, the Norwegian Research Council, the South-Eastern Norway Regional Health Authority, The Swedish Cancer Society, the Swedish Children's Cancer Foundation, The Swedish Research Council, The Center of Excellence: Precision Immunotherapy Alliance, Knut and Alice Wallenberg Foundation and National Cancer Institute.

Morphology and Vessel Density of the Macula in Preterm Children Using Optical Coherence Tomography Angiography.

Background: Retinal morphology changes may be associated with prematurity and can lead to visual impairment. Optical coherence tomography angiography may contribute to understanding the pathomechanism of structural and vascular retinal impairment in premature children. The aim of this study was to assess an influence of prematurity, neonatal clinical characteristics, and a history of retinopathy of prematurity (ROP) on the morphology and retinal vascularity of macula in children. Methods: A case−control study of 123 preterm children and 86 full-term children was performed. The age of the subjects was 10.45 years (IQR: 8.12−12.77), while the age of the control group was 11.78 years (IQR: 8.81−13.79). Optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA, angio-OCT) were performed using RTVueXR Avanti (Optovue, Fremont, CA, USA). Ganglion cell complex (GCC), foveal thickness (FT), parafoveal thickness (PFT), size of foveal avascular zone (FAZ) in superficial plexus, superficial capillary vessel density (sVD), deep capillary vessel density (dVD), central choroidal thickness (CCT), and presence of macular hypoplasia were analyzed. The association between OCT/angio-OCT results and clinical characteristics including the degree of ROP and therapy requirements was assessed in preterm infants. Results: Foveal morphology was affected in preterm children with high incidence of foveal hypoplasia (24.77%). GCC was thinner in preterm children compared to controls: avgGG 93 µm vs. 100 µm, p < 0.001. No associations between GCC and gestational age (R = −0.085; p = 0.228) and birth weight (R = −0.054; p = 0.446) were found. FAZ in preterm group was smaller than in controls (0.13 ± 0.09 vs. 0.22 ± 0.09; p < 0.001). FAZ area correlated with gestational age (R = 0.456; p < 0.001) and birth weight (R = 0.472; p < 0.001). Deep vessel density in the fovea was higher in preterm children than in control group (p < 0.001). PFT was significantly lower in preterm children compared to control group. However, increased thickness in the fovea was noted in preterm children (p < 0.001). FT was inversely correlated with gestational age (R = −0.562; p < 0.001) and birth weight (R = −0.508, p < 0.001). CCT was lower in preterm children (312 µm vs. 337.5 µm, p < 0.001) Parameters of GCC and FT were higher in patients with ROP required treatment compared to patients without ROP and spontaneously regressed retinopathy. FAZ was smaller in patients with retinopathy than in preterm children without ROP. Conclusion: Prematurity has a significant negative impact on GCC, macular morphology, and vascularization. In premature children, decreased FAZ, increased FT, and vessel density were strongly associated with gestational age, birth weight, Apgar score, ROP stage, and treatment requirement. Optical coherence tomography angiography is a useful tool for detecting retinal changes in premature children.

The Impact of Chronic Heart Failure on Retinal Vessel Density Assessed by Optical Coherence Tomography Angiography in Children with Dilated Cardiomyopathy.

(1) Introduction: The aim of this study is to assess retinal vessel density (VD) in the superficial capillary plexus layer (SP) and deep capillary plexus layer (DP) in children with chronic heart failure (CHF) in the course of dilated cardiomyopathy (DCM) using optical coherence tomography angiography (OCTA). (2) Methods: Thirty children with CHF due to DCM lasting more than six months, with an enlarged left ventricle and impaired left ventricular systolic function (left ventricular ejection fraction (LVEF) ≤ 55%), were enrolled to have both their eyes assessed for this study. Mean age of the children was 9.9 ± 3.57 years. The control group consisted of an additional 30 children without CHF (mean age 11.27 ± 3.33 years) matched for age and gender against the study group. All participants underwent transthoracic echocardiography to measure LVEF using Simpson method. Blood serum was tested for N-terminal-pro-brain natriuretic peptide (NT-proBNP) marker value. All children underwent OCTA with evaluation of the foveal avascular zone (FAZ), whole superficial vessel density (wsVD), foveal superficial vessel density (fsVD), parafoveal superficial vessel density (psVD), whole deep vessel density (wdVD), foveal deep vessel density (fdVD), parafoveal deep vessel density (pdVD), whole thickness (WT), foveal thickness (FT), and parafoveal thickness (PFT). (3) Results: Retinal VD in SP was significantly lower in children with CHF as compared to the controls. The following SP parameters in the study group were statistically significantly lower than these same measurements for the control group. Details, with study group findings quantified first, include wsVD (46.2% vs. 49.83%, p < 0.05), fsVD (18.07% vs. 24.15%, p < 0.05), and psVD (49.24% vs. 52.51%, p < 0.05). The WT (311.03 micrometers (µm) vs. 323.55 µm, p < 0.05), FT (244.57 µm vs. 256.98 µm, p < 0.05), and PFT (320.63 µm vs. 332.02 µm, p < 0.05). No significant differences in DP retinal VD were found between the two groups. No statistically significant differences in the FAZ were found. The fsVD and FT were correlated with biometry and the age of the study participants. There was a correlation between FAZ and FT (p < 0.001). There were no correlations between retinal VD in both plexuses and refractive error, sex, NT-proBNP, and LVEF. (4) Conclusions: In children with CHF in the course of DCM as compared to the control group, significantly decreased retinal VD in SP was observed. The results of our study indicate that measurements of the OCTA may be a useful diagnostic method in children with chronic heart failure, but it is necessary to conduct further studies in larger groups of participants and long-term observation of these patients.

Choroidal thickness changes in children with chronic heart failure due to dilated cardiomyopathy.

PURPOSE: To evaluate choroidal thickness (CTh) in children with chronic heart failure (CHF) secondary to dilated cardiomyopathy (DCM) using spectral domain optical coherence tomography (SD-OCT) and to compare their values to those of healthy children. METHODS: Sixty eyes of thirty children (mean age 9.9 ± 3.57 years) with chronic heart failure (left ventricular ejection fraction, LVEF ≤ 55%) due to DCM lasting for over 6 months were prospectively enrolled. The control group consisted of 30 age- (mean age 10.16 ± 3.42 years) and sex-matched healthy children. All participants underwent transthoracic echocardiography with LVEF measured using the Simpson method and had the blood serum level of N-terminal-pro-brain natriuretic peptide marker (NT-proBNP) determined. All children underwent SD-OCT and had subfoveal choroidal thickness (SFCTh) and CTh measured at 1500 µm (µm) nasally, temporally, superiorly and inferiorly from the fovea in both eyes by two investigators. RESULTS: CTh at all locations was statistically significantly lower in children with DCM compared to the control group. Mean CTh in the group with CHF compared to the control group were (304.03 vs. 369.72 µm, p < 0.05) at the subfoveal location, (245.87 vs. 284 µm, p < 0.05) 1500 µm nasally from the fovea, (291.5 vs. 355.95 µm, p < 0.05) 1500 µm temporally from the fovea, (303.98 vs. 357.58 µm, p < 0.05) 1500 µm superiorly from the fovea and (290.92 vs. 344.96 µm, p < 0.05) 1500 µm inferiorly from the fovea. The average difference CTh between the study groups ranged from 38.13 to 65.69 µm at individual locations. In both groups, CTh was the thickest at subfoveal location (304.03 vs. 369.72 µm, p < 0.05) and the thinnest was 1500 µm nasally from the fovea (262.37 vs. 336.87 µm, p < 0.05). There was no correlation between CTh and age, gender, biometry and refractive error. No correlation was found between CTh and LVEF and NT-proBNP. CONCLUSION: Patients with CHF due to DCM had a thinner CTh at all measured locations. The results of our research indicate that CHF affects CTh and this parameter may be very helpful in monitoring the clinical course of the disease in children with DCM.

Coexistence of bilateral macular edema and pale optic disc in the patient with Cohen syndrome.

BACKGROUND: Cohen syndrome (Q87.8;ORPHA:193; OMIM#216550) is an autosomal recessive inherited genetic disorder caused by mutation in the VPS13B/COH1 gene. It is characterized by variable clinical symptoms such as deformity of the head, face, hands and feet, eye abnormalities, abdominal obesity, neutropenia and nonprogressive intellectual disability. The typical lesions in the eyeball in Cohen syndrome include high myopia, retinal dystrophy, strabismus, maculopathy and lens subluxation. The present study describes the coexistence of bilateral macular edema with pale optic disc in a patient with a homozygous deletion in the VPS13B/COH1 gene. MATERIAL AND METHODS: A 6-year-old Caucasian girl with facial dysmorphism, microcephaly, prominent upper incisors, narrow hands with slender fingers, congenital heart defect and ophthalmic symptoms was subjected to genetic testing. The genetic evaluation revealed a homozygous deletion on the long arm of chromosome 8 encompassing 20-25 exons of the VPS13 gene, as confirmed by Cohen syndrome. She underwent a full ophthalmological examination with the assessment of slit lamp examination of anterior segment and fundoscopy, refraction error, biometry, central corneal thickness and additionally electroretinography, optical coherence tomography and fundus photography. RESULTS: In the ophthalmologic examination, the girl had bilateral astigmatism accompanied by myopia and a marked reduction in central corneal thickness. Fundus examination showed pale optic nerve discs and "salt and pepper" retinopathy. Bilateral cystic macular edema was revealed in handheld optical coherence tomography. Electroretinography showed a reduced response amplitude of cones and rods. CONCLUSION: In a patient with high myopia, macular edema, pale optic disc and facial dysmorphism, Cohen syndrome should be considered in the differential diagnosis. The severity of individual clinical features in patients with Cohen syndrome varies. It can be assumed that the type of mutation affects the occurrence and severity of individual symptoms.

The effect of coexisting autoimmune thyroiditis in children with Type 1 diabetes on optical coherence tomography results.

OBJECTIVE: To assess the influence of thyroid hormones status and coexistence of autoimmune thyroiditis on optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA) results in children with Type 1 diabetes (T1D). RESEARCH DESIGN AND METHODS: In the prospective, observational study (n = 175) we analyzed the impact of thyroid hormones on OCT results and the differences between the matched groups of children with T1D (n = 84; age = 13.14 ± 3.6; diabetes duration = 5.99 ± 3.3 years) and the children with T1D and autoimmune thyroiditis (AT) (n = 20; age = 13.94 ± 3.6; diabetes duration = 6.7 ± 4 years). We analyzed the following parameters: fovea avascular zone (FAZ), foveal thickness (FT), parafoveal thickness (PFT), ganglion cell complex (GCC), loss volume (global-GLV, focal-FLV), capillary vessel density: superficial (whole-wsVD, foveal-fsVD, parafoveal-psVD), and deep (whole-wdVD, foveal-fdVD, parafoveal-pdVD. The differences between the groups were tested by the unpaired t-Student test, Mann-Whitney U test as appropriate, whereas p level .05 was recognized as significant. RESULTS: We detected the significant correlations between thyroid-stimulating hormone (TSH) level and PFT (r = -0.14; p < .05), psVD (r = -0.18; p < .005). The level of free triiodothyronine (FT3) was correlated with psVD (r = -0.14; p < .05). We found significant correlation between free thyroxine (FT4) and fsVD (r = -0.17; p < .01). In the studied T1D and AT groups there were statistical differences in FT (p < .005), PFT (p < .03), GCC (p < .01), and GLV (p < .003). We did not observe any significant differences in the FAZ area between the groups. CONCLUSIONS: In our patients the co-occurrence of T1D and AT worsens the status of retinal parameters. Further studies are necessary to observe these relations and their potential influence on the occurrence of diabetic retinopathy (DR).

Influence of Metabolic Parameters and Treatment Method on OCT Angiography Results in Children with Type 1 Diabetes.

AIM: To evaluate the influence of metabolic parameters and the treatment method in children with type 1 diabetes (T1D) on the optical coherence tomography angiography (OCTA) results as early markers of diabetic retinopathy (DR). Material and Methods. This prospective study enrolled 175 consecutive children with T1D. OCTA was performed using AngioVue (Avanti, Optovue). Whole superficial capillary vessel density (wsVD), fovea superficial vessel density (fsVD), parafovea superficial vessel density (psVD), whole deep vessel density (wdVD), fovea deep vessel density (fdVD), parafovea deep vessel density (pdVD), foveal thickness (FT), parafoveal thickness (PFT), and foveal avascular zone (FAZ) in superficial plexus were evaluated and analyzed in relation to individual characteristics, i.e., sex, weight, height, body mass index (BMI), and metabolic factors: current and mean value of glycated hemoglobin A1c (HbA1c). Furthermore, the analysis concerned the diabetes duration, age at the T1D onset, and type of treatment-multiple daily insulin injections (MDI) or continuous subcutaneous insulin infusion (CSII). RESULTS: In the study group, we did not identify any patient with DR in fundus ophthalmoscopy. Age at the onset of diabetes correlated negatively with FAZ (r = -0.17, p < 0.05). The higher level of HbA1c corresponded to a decrease of wsVD (r = -0.13, p < 0.05). We found significantly lower fsVD (32.25 ± .1 vs. 33.98 ± .1, p < 0.01), wdVD (57.87 ± .1 vs. 58.64 ± .9, p < 0.01), and pdVD (60.60 ± .2 vs. 61.49 ± .1, p < 0.01) and larger FAZ area (0.25 ± .1 vs. 0.23 ± .1, p < 0.05) in the CSII vs. MDI group. CONCLUSION: The metabolic parameters, age of the onset of diabetes, and treatment method affected the OCTA results in children with T1D. Further studies and observation of these young patients are needed to determine if these findings are important for early detection of DR or predictive of future DR severity.

Investigating Ganglion Cell Complex Thickness in Children with Chronic Heart Failure due to Dilated Cardiomyopathy.

PURPOSE: To assess ganglion cell complex (GCC) thickness in children with chronic heart failure (CHF) due to dilated cardiomyopathy (DCM) using optical coherence tomography (OCT). METHODS: Sixty eyes of 30 patients with chronic heart failure (CHF) due to dilated cardiomyopathy (DCM) and 60 eyes of 30 age- and sex-matched healthy volunteers (control group) were enrolled. The mean age of the patients and controls was 9.9 ± 3.57 (range 5-17) years and 10.08 ± 3.41 (range 4-16) years, respectively. All patients underwent a complete ophthalmic assessment and OCT imaging using RTVue XR Avanti (Optovue). The following OCT-based parameters were analysed: average ganglion cell complex thickness (avgGCC), superior ganglion cell complex thickness (supGCC), inferior ganglion cell complex thickness (infGCC), global loss of volume (GLV) and focal loss of volume (FLV). RESULTS: There were no significant differences in avgGCC (98.13 µm vs. 99.96 µm, p = 0.21), supGCC (97.17 µm vs. 99.29 µm, p = 0.13), infGCC (99.03 µm vs. 100.71 µm, p = 0.25), FVL (0.49% vs. 0.4%, p = 0.25) and GVL (2.1% vs. 1.3%, p = 0.09) between patients with chronic heart failure due to dilated cardiomyopathy and healthy children. There was no correlation between avgGCC, supGCC, infGCC, FLV, GLV and ocular biometry, refractive errors or age. There was no correlation between avgGCC, supGCC, infGCC, FLV, GLV and NT-proBNP or LVEF. There were no significant differences in the studied parameters between the sexes. There were no significant differences in the studied parameters between the left and right eye. CONCLUSION: Our study seems to be the first to analyse ganglion cell complex in paediatric patients with dilated cardiomyopathy. We have demonstrated no changes in the ganglion cell complex thickness parameters in children with chronic heart failure due dilated cardiomyopathy, as compared to their healthy peers.

Optical coherence tomography angiography of superficial retinal vessel density and foveal avascular zone in myopic children.

PURPOSE: To assess the superficial retinal vessel density (SRVD) and foveal avascular zone (FAZ) in myopic children using optical coherence tomography angiography (OCTA). METHODS: 174 eyes of 89 subjects with myopia and 101 eyes of 54 age-matched, emmetropic volunteers (control group) were enrolled in this study. The mean age of the subjects and controls was 13.9 (SD ± 2.3) and 13.1 (SD ± 2.4), respectively. Myopia was defined as spherical equivalent <- 1.0 diopter. Emmetropic subjects were defined as having spherical equivalent from + 0.5 to - 0.5 diopter. The mean axial length (AL) in myopic patients was 24.58 mm (SD ± 1.22) and 22.88 mm (SD ± 0.65) in the controls. Every patient underwent a complete ophthalmological examination and OCTA, using AngioVue (Optovue). The FAZ area and superficial retinal vessel density, including whole SRVD, fovea SRVD and parafovea SRVD, were analyzed. Foveal thickness (FT) and parafoveal thickness (PFT) were also taken into consideration. RESULTS: Whole SRVD, parafovea SRVD and PFT were significantly higher in controls than in the myopic subjects (p < 0.001, p = 0.007, p < 0.01, respectively). The FAZ area was significantly larger in the myopic group compared to the controls (p = 0.010). Fovea SRVD and FT did not differ significantly between the groups (p = 0.740, p = 0.795 respectively). In overall subjects we found significant correlation between axial length and all the investigative parameters: age, FAZ area, whole SRVD, parafovea SRVD, fovea SRVD, PFT, FT (p < 0.001, p = 0.014, p = 0.008, p < 0.005, p = 0.014, p = 0.010, p = 0.024, respectively). Analyzing only myopic group we confirmed that AL was significantly correlated with age, whole SRVD and parafovea SRVD (p < 0.001, p = 0.014, p = 0.009, respectively). Similarly, in this group the spherical equivalent also correlated with age, whole SRVD and parafovea SRVD (p < 0.001, p = 0.007, p = 0.005, respectively). Such correlations were not confirmed in the non-myopic group. CONCLUSIONS: Our results suggest that superficial retinal vessel density is decreased and FAZ area is enlarged in the entire group of the myopic children compared to emmetropic subjects. Longitudinal observation of these young patients is needed to determine the relevance of the microvascular alterations in future.

Choroidal Thickness and Ganglion Cell Complex in Pubescent Children with Type 1 Diabetes without Diabetic Retinopathy Analyzed by Spectral Domain Optical Coherence Tomography.

AIM: To assess the retinal and choroidal thickness and ganglion cell complex (GCC) in pubescent children with type 1 diabetes (T1D) without diabetic retinopathy (DR), using spectral domain optical coherence tomography (SD-OCT). MATERIALS AND METHOD: Sixty-four right eyes of 64 subjects with T1D and 45 right eyes of 45 age-matched healthy volunteers (control group) were enrolled in this study. The mean age of the subjects and controls was 15.3 (±SD = 2.2) and 14.6 (±SD = 1.5), respectively. SD-OCT was performed using RTVue XR Avanti. Ganglion cell complex (GCC), GCC focal loss volume (FLV), GCC global loss volume (GLV), choroidal thickness (CT), foveal (FT) and parafoveal thickness (PFT), and foveal (FV) and parafoveal volume (PFV) data were analyzed. RESULTS: There was no significant difference between subjects and controls in the CT in the fovea and nasal, temporal, superior, and inferior quadrants of the macula. There were no significant correlations between CT, duration of diabetes, and HbA1C level (p = 0.272 and p = 0.197, resp.). GCC thickness did not differ significantly between the groups (p = 0.448), but there was a significant difference in FLV (p = 0.037). Significant differences between the groups were found in the PFT and PFV (p = 0.004 and p = 0.005, resp.). There was a significant negative correlation between PFT, PFV, and HbA1C level (p = 0.002 and p = 0.001, resp.). CONCLUSIONS: Choroidal thickness remains unchanged in children with T1D. Increased GCC FLV might suggest an early alteration in neuroretinal tissue. Parafoveal retinal thickness is decreased in pubescent T1D children and correlates with HbA1C level. OCT can be considered a part of noninvasive screening in children with T1D and a tool for early detection of retinal and choroidal abnormalities. Further OCT follow-up is needed to determine whether any of the discussed OCT measurements are predictive of future DR severity.

Optical Coherence Tomography-Based Angiography in Retinal Artery Occlusion in Children.

PURPOSE: To evaluate the role of optical coherence tomography (OCT)-based angiography (OCTA) in the diagnosis and monitoring of pediatric patients with retinal artery occlusion (RAO). MATERIAL AND METHODS: This retrospective study was conducted at the Department of Ophthalmology, The Children's Memorial Health Institute, in Warsaw between March 2015 and May 2016. Every patient underwent a complete ophthalmological examination. The diagnosis of the disease was based on fundus examination and fluorescein angiography (FA). OCT and OCTA were performed at baseline and every follow-up visit. RESULTS: Four patients (4 eyes) (2 boys/2 girls, age 8-16 years) with RAO were enrolled in the study. In all cases, initial OCTA images revealed typical ischemic changes in superficial and deep retinal capillary plexuses. Follow-up OCTA revealed increasing areas of ischemia in the RAO region and persistent narrowing of the arteries. The loss of capillary network and the darker, smooth background due to ischemia were visible on OCTA images. CONCLUSIONS: OCTA enables clear visualization of progressive impairment of the retinal vascular perfusion in children with RAO and may be an alternative to the standard FA. Further studies are needed to confirm our results and establish the role of OCTA in pediatric patients.

Correlation between Choroidal Neovascularization Shown by OCT Angiography and Choroidal Thickness in Patients with Chronic Central Serous Chorioretinopathy.

PURPOSE: To assess the occurrence of choroidal neovascularization (CNV) secondary to chronic central serous chorioretinopathy (CSCR) using optical coherence tomography angiography (OCTA) and correlate these findings with choroidal thickness (CT). MATERIALS AND METHODS: This retrospective study included 25 consecutive patients (43 eyes), mean age 48.12 ± 7.8 years, diagnosed with persistent CSCR. All patients underwent a complete ophthalmic examination, fluorescein angiography (FA), indocyanine green angiography (ICGA), optical coherence tomography, and OCTA. RESULTS: CNV was confirmed in 18.6% of eyes using FA and ICGA and in 25.6% of eyes using OCTA. All cases of CNV were associated with irregular retinal pigment epithelial detachment. CT was increased in the affected eyes (mean 491.05 ± 91.98), but there were no statistically significant correlations between CT and CNV and PED occurrence (p = 0.661 and p = 0.614, resp.) and between CT and duration of the disease (p = 0.940). CONCLUSIONS: OCTA detected CNV more frequently than other imaging modalities. CNV coexisted with irregular PED in all cases. CT was increased in eyes with chronic CSCR, but without any correlation with CNV occurrence; therefore, CT cannot be considered as a predictor of CNV occurrence. Further studies with a larger number of patients are needed to confirm these findings.

Optical coherence tomography angiography vessel density in children with type 1 diabetes.

PURPOSE: To assess the optical coherence tomography angiography (OCTA) retinal vessel density and foveal avascular zone (FAZ) in children with type 1 diabetes (T1D) and compare potential pathologic early changes in this population to healthy age-matched controls. METHODS: This study included 130 pubescent children: 94 with T1D (188 eyes) and 36 of their age-matched control group (60 eyes). OCTA was performed using AngioVue (Avanti, Optivue). FAZ area (mm2) in superficial plexus, whole superficial capillary vessel density (wsVD), fovea superficial vessel density (fsVD), parafovea superficial vessel density (psVD), whole deep vessel density (wdVD), fovea deep vessel density (fdVD), parafovea deep vessel density (pdVD), foveal thickness (FT) (µm) and parafoveal thickness (PFT) (µm) were taken into analysis. Among the studied patients with T1D there were assessed codependences regarding the investigated foveal and parafoveal parameters and selected potential predictors, i.e. patient's age (years), diabetes duration time (years), age of onset of the disease (years), mean level of glycated hemoglobin (HbA1C) (%), and concentration of serum creatinine (mg/dL). RESULTS: None of the abovementioned OCT and OCTA parameters was statistically significantly different between the groups. The patient's age statistically significantly did not influent any of the OCT and OCTA parameters. Yet an elevated level of HbA1C tended to reduce the parafovea superficial vessel density (p = 0.039), and parafoveal thickness (p = 0.003) and an increased serum creatinine level correlated with the decreased whole deep vessel density (p < 0.001). The parafovea deep vessel density in the diabetic patients decreased when the serum creatinine level (p = 0.008), age of onset of the disease (p = 0.028), and diabetes duration time (p = 0.014) rose. CONCLUSIONS: Vessel density, both in superficial and deep plexuses, and FAZ area are normal in pubescent children with T1D comparing to healthy subjects. An elevated level of HbA1C correlated with reduced psVD and PFT. Longitudinal observation of these young patients is needed to determine if any of these OCTA measurements are predictive of future DR severity.

Optical Coherence Tomography and Optical Coherence Tomography Angiography in Monitoring Coats' Disease.

Purpose. The aim of this study was to evaluate the usefulness of optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA) in monitoring pediatric patients with Coats' disease. Material and Methods. This retrospective study included 9 Caucasian patients receiving treatment for Coats' disease at the Children's Memorial Health Institute Ophthalmology Department between December 2014 and May 2016. The course of the disease was monitored with OCTA in combination with OCT and fluorescein angiography (FA). Results. OCT B-scans obtained in all patients correlated with FA findings. Reliable OCTA images were obtained in 8 patients. In one patient, numerous artifacts due to poor visual acuity and retinal detachment confounded the interpretation of findings. Conclusions. OCTA and OCT, in combination with FA, are useful in Coats' disease diagnostics and treatment monitoring. As noninvasive methods, OCT and OCTA may be performed more often than FA, which enable precise monitoring of the disease and making decisions as to its further treatment.

Orbital tumor as an initial manifestation of Wegener's granulomatosis in children: a series of four cases.

BACKGROUND: Wegener's granulomatosis (WG) is a rare idiopathic disease in which small and medium-sized arteries are affected by necrotizing granulomatous inflammation. It is associated with a triad of pulmonary (cavitating granulomatous lesions with hemoptysis, cough, and dyspnea), renal (glomerulonephritis with hematuria, proteinuria), and head (otitis media, recurrent sinusitis, eye or orbital involvement) manifestations. CASE REPORT: Four children aged 7-11 years diagnosed with WG between 1995-2008 initially presented with unilateral proptosis and ptosis due to orbital tumor. CT or MRI, orbital lesion biopsy, and laboratory tests (ERS, CRP, ANCA) were part of the diagnostic workup. The diagnoses were based on correlation between clinical presentation and diagnostic findings. All four patients had orbital lesions on contrast-enhanced CT and MRI. Two had lesions of the temporal pyramid. Orbital tumor biopsies showed granulomatous lesions in two patients, necrotizing vasculitis with leukocytoclasia in three, and an orbital pseudotumor in one. ESR and CRP were positive in all. ANCA positivity was variable (c-ANCA did not allow WG diagnosis or there were atypical ANCAs). All had blood and protein in the urine, but only one had advanced renal involvement. All were treated with oral steroid and immunosuppression; remission was successful. CONCLUSIONS: WG is often more difficult to diagnose in children than in adults due to frequent absence of its signature features. The absence of the classic triad and atypical laboratory or biopsy findings do not exclude a diagnosis of WG. Orbital demonstration helps achieve early diagnosis and treatment of this potentially fatal rheumatologic disease.

[Optic nerve atrophy--the main cause of visual impairment in children and young adults]. / Zanik nerwów wzrokowych--glówna okulistyczna przyczyna pogorszenia widzenia u dzieci i mlodziezy.

Blindness and visual disability are a very significant problem all over the world. Inflammation, metabolic disorders, tumours, hereditary optic neuropathies may all lead to visual impairment. The main cause of visual impairment and disability in children and young adults is optic nerve atrophy. THE AIM of the paper is the presentation of clinical features and treatment of nerve atrophy in children. Patients with optic nerve disorders should receive effective refraction and amblyopia treatment.

[Retinopathy of prematurity]. / Retinopatia wczesniaków.

Retinopathy of prematurity is a noteworthy problem in the ophthalmology of children. This paper presents current views on epidemiology, risk factors, pathogenesis and treatment of this disease.