Papillary and peripapillary vascular densities and corresponding correlation with peripapillary retinal thicknesses using optical coherence tomography angiography in healthy children and adolescents.

To evaluate the peripapillary retinal thickness (PPRT), vascular density (PPVD), and disc vascular density (PVD) and their correlations in normal healthy children using optical coherence tomography angiography (OCTA). This was a cross-sectional study of 70 eyes from 36 normal healthy children aged 6-18 years who performed optic nerve head scans using OCTA. The PPRT included the peripapillary nerve fiber layer (PP-RNFLT), inner retina (PP-IRT), middle retinal thickness, and outer retinal thicknesses. The PP-RNFLT and PP-IRT were not significantly different between males and females. Superior nasal peripapillary RNFLT and IRT were significantly affected by age (ANOVA, P > 0.05). The PP-IRT and PP-RNFLT were lower in the 7-11 years old group in comparison with the other 3 groups (Post hoc Tukey test, P value < 0.05). Age and sex-matched PVD were not correlated with PPVD (partial correlation, P > 0.05). PPRT was not correlated with PVD, PPVD, superficial and deep retinal vascular densities, and choroidal vascular density. This study demonstrated that PPRT appears to change during growth in childhood. Superior nasal PPRT was affected more in the groups, decreasing from less than 7 years old to 7-11 years old and then back to pre-reduction values after 11 years old.

One-year non-comparative observational study to evaluate corneal tomographic, densitometric, and aberrometric features following accelerated corneal cross-linking in progressive keratoconus.

PURPOSE: To evaluate the 12 months' changes in tomographic, densitometric, and aberrometric parameters in keratoconic eyes after accelerated corneal cross-linking (CCL) and classify a densitometric course in different stages of the keratoconus separately (mild, moderate, and severe). METHODS: In a prospective observational study, 67 keratoconic eyes of 67 patients that underwent accelerated epithelium-off corneal cross-linking (9 mW/cm2 and 10 min) for treatment of progressive keratoconus were included. Corneal tomographic, densitometric, and aberrometric values obtained using the Pentacam HR were recorded at the baseline and 3, 6, and 12 months post-operatively. RESULTS: One year after treatment, corrected distance visual acuity (CDVA) was improved, and maximum keratometry, thinnest pachymetry, higher order, and total root mean square (RMS) were significantly decreased (p < 0.001). Corneal densitometry values showed a significant elevation 3 months post-surgery compared to baseline and then decreased to baseline values at 1 year. Only the Anterior 0-2 mm zone densitometry at the 3rd month was different between the three groups. RMS at 1 year correlated with Anterior 0-2 mm, Anterior 2-6 mm, total corneal 0-2 mm, and total corneal 2-6 mm densitometry values in the 3rd month. Final CDVA at 12th month follow-up correlated with the Anterior 0-2 mm corneal densitometry in the 3rd month. CONCLUSION: Anterior 0-2 mm zone densitometry at the 3rd-month post-accelerated CCL is different in various stages of keratoconus. Due to the correlation between final aberrometric and peak densitometric values in keratoconic eyes, peak densitometric values can be used as a prognostic factor for the final visual outcomes after accelerated CCL.

Quantitative Analysis of Inner, Middle, and Outer Retinal Thickness by Optical Coherence Tomography in Children and Adolescents.

Purpose: To describe the variance of inner, middle, and outer retinal layer thicknesses (IRT, MRT, and ORT) at the macular area in children and adolescents with normal eyes in different age groups. Methods: This cross-sectional study enrolled subjects aged 5-18 years with normal eyes. The macula was scanned by optical coherence tomography (6 mm × 6 mm AngioScan-Optovue). Four age groups were defined (≤7, 7-10, 11-14, and ≥14 years). The influences of age and gender were analyzed. Results: One hundred and thirty-nine eyes of 69 subjects with a mean age of 10.92 ± 3.51 years were registered. The mean whole macular thickness (MT) was 297.32 ± 11.05 in males and 303.197 ± 13.32 in females (P = 0.01, t-test). The MT in each aging group was 301.47 ± 2.5, 295.53 ± 1.71, 300.81 ± 2.12, and 298.6 ± 1.87, subsequently (P = 0.17, analysis of variance test). Significant differences were found between the sexes at the perifoveal area and mainly in IRT. No correlation between eyes was noted. We observed that the RT fluctuates during growth and that gender has some influence on the evolution of RT. IRT and MRT changed reciprocally in all macular areas, whereas ORT expanded in all age groups of children and adolescents. Conclusions: No subsegmental retinal thickness difference between eyes was observed in pediatric groups in this study, while gender had some influence on perifoveal IRT. Despite the fact that this study is not a longitudinal study, we can get some insight into the developmental changes in retinal thickness and its clinical applications in children and adolescents.

Assessment of relationship between retinal perfusion and retina thickness in healthy children and adolescents.

PURPOSE: To determine the correlations between inner, mid, and outer retinal thickness (RT) and allied retinal and choroidal vascular densities (VD) at macula in normal healthy children and adolescents. METHODS: This cross-sectional study included a total of 108 eyes of 59 subjects. Optical coherence tomography angiography (OCTA-Optovue) was used to measure the thickness of the inner-retina (IRT), mid-retina (MRT), and outer-retina (ORT) at foveal (central 1mm), parafoveal (1-3 mm), and perifoveal (3-6 mm) areas, as well as the corresponding VD of the superficial capillary plexus (SVD), deep capillary plexus (DVD), and choricapillaris (CVD). RESULTS: The study enrolled 108 normal eyes from 54 participants with a mean age of 10.9 years. Partial correlations showed that the nasal and inferior parafoveal and perifoveal subsegments IRT, MRT and ORT are more affected by all SVD, DVD, and CVD. Nasal parafoveal and perifoveal MRT and all three capillary layers have a constant negative correlation. ORT was not affected by all three layers except for CVD at fovea. The regression analysis revealed that SVD and CVD were significantly associated with foveal and parafoveal and perifoveal IRT. DVD and gender could significantly affect perifoveal IRT. However, only CVD was significantly affected foveal MRT. Based on regression analysis, only CVD was significantly associated with foveal and parafoveal ORT, but not with perifoveal ORT. CONCLUSION: The thickness of different retinal layers correlates with retinal and choroidal VD in different ways according to their zones.

Near-Infrared Confocal Reflectance Scanning Laser Ophthalmoscopy (SLO) and Short-Wavelength Autofluorescence Imaging in Cystic Diabetic Macular Edema.

Objective: To characterize results of short-wavelength autofluorescent (SW-AF) and near-infrared confocal reflectance scanning laser ophthalmoscopy (NIR-cR SLO) imaging in cystic diabetic macular edema (DME). Design: Cross-sectional study. Participants: 104 eyes from 52 naïve treatment patients with DME and mild to moderate nonproliferative diabetic retinopathy (NPDR) Methods: complete ocular examination, best-corrected visual acuity (BCVA), and imaging were performed. Results: In NIR-cR SLO, small/medium and large-sized cysts presented with decreased and increased reflectance, respectively. In SW-AF, increased and decreased autofluorescence, corresponding to medium-/large- and small-sized cysts were noted. Mainly, the lower NIR reflectance was associated with petaloid edema pattern in SW-AF (P=0.011), BCVA (logMAR) (P=0.056), subretinal fluid (P=0.035), and the involved layers of retina by cysts (P < 0.001) in Pearson chi-square test. Fair agreement of 0.31 (P < 0.001) between NIR-cR SLO and late FA leakage was found by the weighted kappa test. In regression analysis, NIR-cR SLO abnormality is highly correlated with outer and inner nuclear layers location of the cystic changes. Conclusions: The size of cysts and involved layers affect presenting features of SW-AF and NIR reflectance.

Quantification of macular perfusion in healthy children using optical coherence tomography angiography.

BACKGROUND: This study aimed at defining the variance of vessel density (VD) characteristics in the macula of children with normal eyes. METHODS: This was a cross-sectional study in which subjects with normal eyes aged 3-18 years were enrolled. The macula was scanned by optical coherence tomography angiography (OCTA). Four age groups as under 7 years, 7-10 years, 11-14 years and more than 14 years of age were defined. The influences of age, gender, and body mass index on VD were analyzed. RESULTS: A total of 108 normal eyes from 54 participants with a mean age of 10.9 years were enrolled in the study. At the superficial and deep retina capillary plexus (SCP and DCP) and choriocapillaris (CC), respectively, the mean VD of the fovea was 20.10%, 36.99%, and 75.67%; at parafovea, these measurements were 53.12%, 55.81%, and 69.76%; and at perifovea, these measures were 51.38%, 52.46%, and 73.47%. The median foveal avascular zone (FAZ) was 0.30 mm2. No significant differences between superior-hemi VD and inferior-hemi VD were found in the studied plexuses. The VD at parafovea and perifovea CC differed significantly between groups. There was no variation in VD of macular retina and CC between eyes or sex. FAZ areas were different between genders. CONCLUSIONS: No difference between eyes and genders in the retina and CC VD of macular area was noted. FAZ area was larger in male. Even though this is not a longitudinal study, it may provide us with hints about macular vascular development during puberty and clinical implications of OCTA in children.

Vascular density of optic nerve head in diabetic retinopathy using optical coherence tomography angiography.

PURPOSE: To measure optic nerve head (ONH) blood perfusion using optical coherence tomography angiography (OCTA) at various stages of diabetic retinopathy (DR). METHODS: One hundred seventy six eyes of 94 patients included in this retrospective single-centre cross-sectional study. The subjects were studied in normal, no diabetic retinopathy (NDR), non-proliferative diabetic retinopathy (NPDR) and proliferative retinopathy (PDR) groups. The eyes were subjected to AngioDisc ONH imaging using OCTA for papillary (Disc) and peripapillary (RPC) vascular density (VD) evaluation. RESULTS: The mean age of the participants was 56.08 ± 8.87 years and 34 (36.2 percent) were male. With increased DR severity, a statistically significant decrease in peripapillary VD was found. The study showed that only VD of the whole RPC (W-RPC) could be a valid biomarker in the staging assessment. VD of RPC, in all subsections, was considerably different from normal cases in the PDR group. Visual acuity was correlated with whole image ONH VD. The duration of DM, FBS, hyperlipidemia and DME had no effect on the ONH perfusion. CONCLUSIONS: The study showed that only the W-RPC VD could be a reasonable marker in the staging assessment. VDs assessed by OCTA can be useful for assessing and tracking early ONH changes in DR patients.

Lens exchange for management of accommodative intraocular lens tilting.

Accommodative intraocular lens (IOL) tilting, the so-called Z syndrome, is a rare complication of Crystalens (Bausch and Lomb) implantation. We report a significant IOL tilting and subsequent high lenticular astigmatism due to posterior capsular fibrosis 2 months after uncomplicated cataract surgery and Crystalens (AT50AO) implantation. The attempt to correct IOL position with neodymium-yttrium-aluminum garnet laser was unsuccessful, and Crystalens exchange with in-the-sulcus, three-piece monofocal IOL was performed. Accommodative IOL tilting could occur early after the surgery. Laser capsulotomy may be the first intervention to restore IOL position, but patients with a higher amount of lenticular astigmatism may require surgical intervention and IOL exchange.

Effect of venting incisions on graft attachment in Descemet's stripping automated endothelial keratoplasty.

PURPOSE: To investigate the impact of venting incisions on the adherence of graft to the recipient's stroma in Descemet's stripping automated endothelial keratoplasty (DSAEK). METHODS: Fifty-six patients were enrolled in this study. Patients were randomly allocated into two groups. Twenty-eight patients had a DSAEK procedure with venting incisions. The second group was treated by conventional DSAEK with no venting incisions. Slit-lamp examination and anterior segment optical coherence tomography (AS-OCT) were performed in day one and 14 days after surgery to investigate graft attachment. The thickness of cornea and lenticle were also evaluated by AS-OCT. BCVA (logMAR) was measured the day before the surgery and 14 days postoperatively. RESULTS: Subclinical graft detachment in the first day after surgery was significantly lower in patients who had a DSAEK procedure and venting incisions (P = 0.02), but no difference was noted in the rate of clinical graft detachment on day one (P = 0.24) and subclinical and clinical graft detachment on day 14 (P = 0.24, P = 0.50, respectively). The thickness of the cornea and lenticle after the surgery were statistically similar between the two groups (P = 0.903, P = 0.402, respectively). No difference in the improvement of BCVA was observed between the venting and non-venting group (P = 0.143). CONCLUSIONS: Routine use of venting incisions may not be necessary in the standard DSAEK procedures. More studies with larger sample sizes are needed to better confirm the results of this study.

Additional Intrastromal Corneal Ring Segments in Keratoconus.

PURPOSE: To evaluate the outcome of additional KeraRing (Mediphacos, Belo Horizonte, Brazil) implantation in patients with keratoconus with previously implanted INTACS (Addition Technology Inc, Fremont, CA) segments. METHODS: The KeraRing was implanted in 5 eyes of 3 patients with keratoconus without removal of previous intrastromal corneal ring segments. Tunnels were created manually. Snellen uncorrected distance visual acuity (UDVA), corrected distance visual acuity, refractive and keratometric values, and higher-order aberrations were recorded before and after KeraRing implantation. RESULTS: UDVA, corrected distance visual acuity, subjective refraction, and keratometric values were improved in 4/5 of cases and remained stable during a median follow-up of 36 months. UDVA improved from 20/200 and 20/100 to 20/100 and 20/40 in the right and left eyes of patient 1, respectively, and from 20/200 to 20/40 in both eyes of patient 2. The reduction in mean keratometric value was 2.1 and 4.4 D in the right and left eyes of patient 1, and 1.4 and 1.9 D in the right and left eyes of patient 2, respectively. In patient 3, UDVA (20/100) and mean keratometric value (52.4) remained unchanged after second intrastromal corneal ring segment implantation. Total higher-order aberrations were improved in all cases. No intraoperative or postoperative complication was observed. However, one of the cases reported glare under scotopic conditions. CONCLUSIONS: KeraRing implantation in properly selected eyes with previously implanted INTACS could enhance final visual and refractive outcomes.

Descemet's Stripping-automated Endothelial Keratoplasty for symptomatic thioridazine deposits in the cornea.

The aim of this study was to use Descemet's Stripping-automated Endothelial Keratoplasty (DSAEK) as a novel treatment to remove thioridazine corneal deposits. A 53-year-old female presented with a 3-month history of visual loss and glare. She had been taking thioridazine (100 mg/day for 1 year) for a psychiatric disorder. Dense yellowish-brown deposits had developed in the posterior stroma. Thioridazine was discontinued, and she was switched to fluoxetine. One year after discontinuation of thioridazine, her symptoms and signs did not resolve. Standard DSAEK was performed on her left eye. Two weeks after DSAEK, an anterior subcapsular cataract was detected in the same eye. Phacoemulsification and intraocular lens implantation were performed after 3 months. The left cornea became completely clear after DSAEK, and the patient's best-corrected visual acuity improved from 20/40 to 20/20 at 1 month after cataract surgery. All-visual symptoms such as glare and halos improved postoperatively. We suggest that DSAEK can be used as a novel treatment to reduce vision problems caused by thioridazine-induced corneal deposits.

Hyperimmunoglobulin E syndrome: Genetics, immunopathogenesis, clinical findings, and treatment modalities.

The hyperimmunoglobulin E syndromes (HIESs) are very rare immunodeficiency syndromes with multisystem involvement, including immune system, skeleton, connective tissue, and dentition. HIES are characterized by the classic triad of high serum levels of immunoglobulin E (IgE), recurrent staphylococcal cold skin abscess, and recurrent pneumonia with pneumatocele formation. Most cases of HIES are sporadic although can be inherited as autosomal dominant and autosomal recessive traits. A fundamental immunologic defect in HIES is not clearly elucidated but abnormal neutrophil chemotaxis due to decreased production or secretion of interferon γ has main role in the immunopathogenesis of syndrome, also distorted Th1/Th2 cytokine profile toward a Th2 bias contributes to the impaired cellular immunity and a specific pattern of infection susceptibility as well as atopic-allergic constitution of syndrome. The ophthalmic manifestations of this disorder include conjunctivitis, keratitis, spontaneous corneal perforation, recurrent giant chalazia, extensive xanthelasma, tumors of the eyelid, strabismus, and bilateral keratoconus. The diagnosis of HIES is inconclusive, dependent on the evolution of a constellation of complex multisystemic symptoms and signs which develop over the years. Until time, no treatment modality is curative for basic defect in HIES, in terms of cytokines/chemokines derangement. Of note, bone marrow transplant and a monoclonal anti-IgE (omalizumab) are hoped to be successful treatment in future.

The Quantitative Measurements of Vascular Density and Flow Areas of Macula Using Optical Coherence Tomography Angiography in Normal Volunteers.

BACKGROUND AND OBJECTIVE: The quantification of the density of macular vascular networks and blood flow areas in the foveal and parafoveal area in healthy subjects using optical coherence tomography angiography (OCTA). PATIENTS AND METHODS: Cross-sectional, prospective study in an institutional setting at the Retina Services of Farabi Eye Hospital. One hundred twelve normal volunteers with no known ocular or systemic disease were included, including patient numbers (one or both eyes), selection procedures, inclusion/exclusion criteria, randomization procedure, and masking. En face angiogram OCTA was performed on a 3 mm × 3 mm region centered on the macula. Automated thresholding and measuring algorithm method for foveal and parafoveal blood flow and vascular density (VD) were used. The density of macular vascular networks and blood flow area in the foveal and parafoveal area were measured. RESULTS: A total of 224 healthy eyes from 112 subjects with a mean age of 36.4 years ± 11.3 years were included. In the foveal region, the VD of the superficial capillary network (sCN) was significantly higher than that of the deep capillary network (dCN) (31.1% ± 5.5% vs. 28.3% ± 7.2%; P < .001), whereas in the parafoveal area, VD was higher in the dCN (62.24% ± 2.8% vs. 56.5% ± 2.5%; P < .001). Flow area in the 1-mm radius circle in the sCN was less than in the dCN. Superficial foveal avascular zone (sFAZ) size was negatively correlated with the VD of the foveal sCN, but in the deep FAZ (dFAZ) was not correlated with VD or blood flow area of the fovea. There was no difference between measured VD and blood flow surface area in both eyes of the subjects. CONCLUSIONS: OCTA could be used as a noninvasive, repeatable, layer-free method in quantitative evaluation of VD and blood flow of macular area. The normal quantities of the vascular plexus density and flow will help in better understanding the pathophysiological basis of the vascular disease of retina. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:478-486.].

Post-photorefractive Keratectomy Pain and Corneal Sub-basal Nerve Density.

PURPOSE: The perceived and reported pain of patients receiving photorefractive keratectomy (PRK) widely varies. We assessed the potential role of the subbasal nerve plexus density as a predictor of postoperative pain level. Consecutive patients scheduled to undergo PRK at the Refractive Surgery Clinic of Farabi Eye Hospital, Tehran, were approached. METHODS: Forty-nine myopic left eyes from 49 patients who consented to undergo scanning slit confocal microscopy assessments preoperatively were included. ImageJ (1.48v) was used to measure the captured subbasal nerve length. Postoperative pain intensity was assessed by the Visual Analog Scale (VAS) (score range: 0 for no pain to 10 for the maximum possible) on the next day of surgery. RESULTS: The mean age of the patients was 27.55 (range: 19-40) years. The median reported pain level was 5. Approximately 32.7% of the subjects reported a pain score of 6 or higher. Mean nerve density was 19.54 (range: 14.34-24.73) mm/mm2. Nerve density was not correlated with the reported intensity of pain (P = 0.172). However, pain was correlated with the reported ocular discomfort, i.e., a pooled index of foreign body sensation, photophobia, burning sensation, and tearing (P < 0.001), and also with the pooled index of ocular inflammatory signs (conjunctival injection and eyelid edema) (P = 0.027). CONCLUSION: Crude density of corneal nerves may not be a good predictor of post-PRK pain while wearing bandage contact lenses. The predominant pain mechanism appears to be of an inflammatory nature (not nociceptive or neuropathic).

Serum hypercoagulability states in Coats' disease.

PURPOSE: The purpose of this study was to investigate the serum hypercoagulability state and common viral and protozoan infections in Coats' disease versus a normal control group. MATERIALS AND METHODS: In this comparative case series, 22 consecutive patients with Coats' disease and 19 non-Coats' patients undergoing lensectomy for congenital, traumatic, or senile cataract between January 2011 and June 2014 were included. Laboratory data for hypercoagulability states and common viral and protozoan infections were investigated. RESULTS: The mean age for the Coats' group was 14.5 years (median 8 years, range: 2 months to 59 years), and for the control group it was 30.6 years (median 17 years, range: 2-82 years). In patients aged 10 years or younger, anticytomegalovirus immunoglobulin G (IgG) (P≤0.01), homocysteine (P=0.03), and serum beta globulin (P<0.001) were associated with Coats' disease. In those older than 10 years, higher serum protein S (P=0.04), beta globulin (P=0.05), and gamma globulin (P=0.04) were related to Coats' diagnosis. After adjusting for sex and age as confounding factors, only beta globulin was found to be associated with Coats' disease in logistic regression analysis (odds ratio: 1.8, 95% confidence interval: 1.0-3.1, P=0.02). CONCLUSION: Serum beta globulin levels appear to be elevated in patients with Coats' disease.

Expression of Efflux Pumps and Fatty Acid Activator One Genes in Azole Resistant Candida Glabrata Isolated From Immunocompromised Patients.

Acquired azole resistance in opportunistic fungi causes severe clinical problems in immunosuppressed individuals. This study investigated the molecular mechanisms of azole resistance in clinical isolates of Candida glabrata. Six unmatched strains were obtained from an epidemiological survey of candidiasis in immunocompromised hosts that included azole and amphotericin B susceptible and azole resistant clinical isolates. Candida glabrata CBS 138 was used as reference strain. Antifungal susceptibility testing of clinical isolates was evaluated using Clinical and Laboratory Standards Institute (CLSI) methods. Complementary DNA-amplified fragment length polymorphism (cDNA-AFLP) technology, semi-quantitative RT-PCR, and sequencing were employed for identification of potential genes involved in azole resistance. Candida glabrata Candida drug resistance 1 (CgCDR1) and Candida glabrata Candida drug resistance 2 (CgCDR2) genes, which encode for multidrug transporters, were found to be upregulated in azole-resistant isolates (≥2-fold). Fatty acid activator 1 (FAA1) gene, belonging to Acyl-CoA synthetases, showed expression in resistant isolates ≥2-fold that of the susceptible isolates and the reference strain. This study revealed overexpression of the CgCDR1, CgCDR2, and FAA1 genes affecting biological pathways, small hydrophobic compounds transport, and lipid metabolism in the resistant clinical C.glabrata isolates.

Intrastromal lamellar keratoplasty in patients with pellucid marginal degeneration.

UNLABELLED: We describe a new method (intrastromal lamellar keratoplasty) to treat patients with pellucid marginal degeneration (PMD). After a pocket is created along the marked margins of the thinned inferior cornea with Melles dissectors, a crescent of the same size is marked on the donor cornea. The epithelial and endothelial layers are removed, and the donor tissue is cut in a crescentic shape in the size of the marked corneal pocket. The donor crescentic stroma is then inserted into the stromal pocket. The technique was performed in 10 eyes of 10 patients with PMD. The uncorrected and corrected distance visual acuities had significantly improved after 6 months. The cylinder was significantly reduced after 1 month and this was maintained up to 3 months of follow-up. Intrastromal lamellar keratoplasty appeared to be effective in the treatment of PMD. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.