Ocular biometry, anterior chamber morphometry, and their relationship with serum ferritin levels in children with beta thalassemia major.

Background: Ocular biometry and anterior segment evaluations are important to determine ocular development and pathological changes, especially in thalassemia patients in Mediterranean countries such as Turkey. Objectives: The objectives of this study were to compare ocular biometry and anterior segment parameters in children with thalassemia major and healthy controls and to examine the relationship between ferritin levels, anthropometric measurements, and ocular parameters. Design: This is a prospective case-control study. Methods: The height, weight, body mass index, and occipitofrontal circumference values of the participants were recorded. Anterior and vitreous chamber depth, lens thickness, axial length, central corneal thickness, anterior chamber volume, iridocorneal angle, pupil diameter, and mean keratometry were measured. Measurements were compared between patients and healthy children, and between patients with ferritin levels above and below 1000 ng/mL. Results: This study included 40 patients and 45 controls. Height, weight, and body mass index were significantly lower while ferritin level and occipitofrontal circumference were significantly higher in patients compared with the controls (p < 0.001 for all). There were no statistically significant differences in the other ocular parameters (p > 0.05). In comparisons between patients with ferritin levels below (n = 15) and above 1000 ng/mL (n = 25), there were no significant differences in age, height, weight, body mass index, occipitofrontal circumference, or ocular parameters (p > 0.05). Occipitofrontal circumference and mean keratometry value were positively correlated in patients with ferritin levels below 1000 ng/mL (r = 0.573, p = 0.025), while body mass index was negatively correlated with pupil diameter in patients with ferritin levels above 1000 ng/mL (r = -0.469, p = 0.018). Conclusion: Children with thalassemia showed significant growth retardation and large occipitofrontal circumference but did not differ from controls in terms of biometrics and anterior segment morphology. Our results demonstrated a positive correlation between the occipitofrontal circumference and mean keratometry value in children with ferritin levels below 1000 ng/mL and a negative correlation between body mass index and pupil diameter in children with ferritin levels above 1000 ng/mL.

Cold-induced Vascular Changes in the Macula, Optic Disc and Choroid in Children with Raynaud's Phenomena.

AIM: To investigate changes in retinal and choroidal vessels in children with primary and secondary raynaud's phenomena(RP). METHODS: Measurements were performed by optic coherence tomography angiography before as well as during and 10 minutes after the immersion of one hand in ice water. RESULTS: Primary RP patients exhibited enlargement of the foveal avascular region and decrease in vessel density in the parafoveal superficial capillary plexus (SCP) during cold exposure, but values returned to normal when the hands were removed from the cold water. In children with secondary RP, there were decreases in choroidal thickness and vessel density in the SCP and radial peripapillary capillary plexus (RPCP) during cold exposure. Changes in choroid and peripapillary RPCP were still significant 10 minutes after removal from cold water. CONCLUSION: Ocular arteriolar vasospasm seems to be more pronounced in patients with secondary RP, particularly in the choroid and the peripapillary area of the RPCP. CLINICAL TRIAL REGISTRATION NUMBER AND DATE: DECISION NUMBER: 76/1315 - 10.03.2021.

Investigation of ocular microstructural changes according to disease severity in patients with chronic obstructive pulmonary disease.

OBJECTIVE: To investigate retinal nerve fibre layer (RNFL) thickness and vascular density alterations in the retina and optic disc in patients with chronic obstructive pulmonary disease (COPD). METHODS: A total of 66 COPD patients and 54 age- and sex-matched healthy individuals were enrolled in this prospective cross-sectional study. The COPD patients were divided into 3 subgroups (mild, moderate, and severe COPD) based on spirometric parameters as per the Global Initiative for Chronic Obstructive Lung Disease guideline. RNFL thickness, foveal avascular zone area, and vessel density in the superficial capillary plexus, deep capillary plexus, and radial peripapillary capillary plexus were measured by optical coherence angiography and compared among groups. RESULTS: There was no significant difference between COPD patients and control individuals in terms of foveal avascular zone area or RNFL thickness (p = 0.891 and p = 0.896, respectively). Patients with severe COPD showed lower vessel density in the superficial capillary plexus compared with the other groups, but the difference was not significant (p > 0.05). In the deep capillary plexus, vessel density did not differ significantly among groups in the foveal region (p > 0.05) but was significantly lower in all parafoveal quadrants in the severe COPD group. Radial peripapillary capillary plexus vessel density also was lower in the severe COPD group, especially the peripapillary region (p = 0.044). CONCLUSION: Although COPD is primarily a lung disease, the eye seems to be among the tissues affected in its natural course. The effects are more pronounced in patients with severe COPD and in the deep capillary plexus and radial peripapillary capillary plexus.

Changes in retinal and choroidal capillary dynamics in patients with multisystem inflammatory syndrome in children.

PURPOSE: To evaluate patients with multisystemic inflammatory syndrome in children (MIS-C) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection using optical coherence tomography angiography (OCTA) during and after resolution of inflammation to investigate the effect of this entity on the retinal and choroidal circulation. METHODS: The study included 38 eyes of 19 patients diagnosed as having MIS-C between March 2021 and June 2021. OCTA measurements of choroidal thickness and vessel density in the radial peripapillary capillary plexus (RPCP), superficial capillary plexus (SCP), and deep capillary plexus (DCP) obtained at time of diagnosis and 60 days later were compared. Correlations between C-reactive protein (CRP) levels at diagnosis and retinochoroidal involvement were investigated. RESULTS: Compared to post-recovery follow-up examinations, patients with active MIS-C showed foveal avascular zone enlargement (p = 0.031), decreased vessel density in the temporal parafoveal SCP (p = 0.047) and all parafoveal areas of the DCP (p < 0.05 for all), and increased choroidal thickness (p = 0.021). Correlation analysis between CRP levels and OCTA changes during MIS-C revealed significant negative correlations with all parafoveal sectors of the SCP and DCP and a significant positive correlation with CT. CONCLUSION: There were especially marked effects on the DCP and choroid in MIS-C patients. Our findings also correlate with CRP levels. The use of optical coherence tomography angiography in patients with multisystemic inflammatory syndrome may have potential future implications for detecting ocular microvascular changes that occur before permanent damage develops. Clinical Trial Registration Number and Date: 77/1340; March 1, 2021.

Evaluation of subclinical ocular involvement in patients with deficiency of adenosine deaminase 2 (DADA2).

PURPOSE: To evaluate changes in the peripapillary, macular, and choroidal microvasculature in the eyes of patients with deficiency of adenosine deaminase 2 (DADA2) and no clinical signs of ocular involvement. METHODS: The study included 12 eyes of 12 patients with DADA2 and 24 eyes of 24 healthy subjects. The foveal avascular zone (FAZ), macular vessel densities (VDs) in the superficial and deep retinal capillary plexuses, peripapillary VDs, and choroidal thickness were evaluated by optical coherence tomography angiography (OCTA). Measurements were compared between DADA2 patients and healthy controls. RESULTS: The median age was 17 (8-25) years in DADA2 patients and 17.5 (7-23) years in control group at the OCTA visit (p = 0.934). FAZ area did not differ between the groups (p = 0.224). In the superficial capillary plexus, whole-image, foveal, and parafoveal VD values were slightly lower in DADA2 patients than in controls (p = 0.054, p = 0.052, p = 0.117). In the deep capillary plexus, whole-image and parafoveal VD values were significantly lower in DADA2 patients than controls (p = 0.010, p = 0.001). VD in the radial peripapillary capillary plexus was also lower in DADA2 patients, with significantly lower peripapillary VD (p = 0.002). Subfoveal choroidal thickness was significantly higher in patients with DADA2 (p < 0.001). CONCLUSIONS: This OCTA study demonstrates that both retinal and choroidal involvement may occur in DADA2 patients before the emergence of evident clinical findings.

Optical coherence tomography angiography in healthy children: normative data and age-related changes in microvascular structure of the optic disk and macula.

PURPOSE: To determine normative data and reference ranges according to age groups by measuring the foveal avascular zone (FAZ), superficial capillary plexus vascular density (SCP-VD), deep capillary plexus vascular density (DVP-VD), radial peripapillary capillary plexus vessel density (RPC-VD), and peripapillary retinal nerve fiber layer (ppRNFL) in healthy children and to determine the age and sex-related changes of these values. METHODS: This prospective study included data from 370 eyes of 370 healthy children (202 girls, 168 boys) aged 7-18 years. Participants were divided into four groups according to their age. Optical coherence tomography angiography (OCTA) measurements were taken using AngioVue (Avanti; Optivue). RESULTS: No statistically significant difference was observed in terms of FAZ, SCP-VD, DCP-VD, RPC-VD, and ppRNFL thickness values according to the age groups (except the RPC-VD superior) (p > 0.05 for all). VDs in all deep parafoveal regions in groups 1 and 2 were higher in girls. While FAZ values were higher in girls in all age groups (statistically significant in groups 1, 3, and 4), ad SPD and DPD values were higher in boys in all age groups (statistically significant in group 1 and 2 for SPD, and group 1 and 3 for DPD). CONCLUSIONS: We report normal reference ranges for macula and disk vessel density and ppRNFL parameters in healthy children aged 7-18 years using OCTA. These normative values could be useful in diagnosing retina and optic disk disease early in childhood.