Congenital pterygium with anterior segment dysgenesis: rare ocular manifestation in Rubinstein-Taybi syndrome.

Pterygium is a benign, wing-shaped fibrovascular overgrowth of subconjunctival tissue that can encroach over the cornea. This condition usually occurs in individuals aged 20-40 years but is rarely seen in children. We report a case of an infant with Rubenstein-Taybi syndrome presenting with nebulo-macular corneal opacity and congenital pterygium. On examination under anaesthesia, bilateral infero-nasal nebulo-macular corneal opacity (6 × 5 mm) with a whitish pink tissue originating from nasal bulbar conjunctiva was noticed. The probe test was negative for this tissue. To the best of our knowledge, only two other cases of congenital pterygium have been reported in the literature. The presence of this anomaly supports the hypothesis of genetic factors having a role in the development of pterygium.

Analysis of foveal avascular zone by using spectral-domain optical coherence tomography angiography in healthy Indian eyes.

PURPOSE: To analyze FAZ superficial (s) and deep (d) area and dimensions by using spectral-domain optical coherence tomography angiography (OCTA) in healthy eyes and to assess effect of age, gender, axial length (AL), central foveal thickness (CFT), and central choroidal thickness (CCT) on FAZ. We aimed to study FAZ dimensions with OCTA in healthy Indian eyes with the purpose of creating a normative database. SETTINGS AND DESIGN: Observational cross-sectional study. METHODS: In total, 200 healthy eyes in the age group of 20-60 years having best corrected visual acuity better than 6/12 (Snellen's) with no systemic illness/intraocular surgery were included. FAZ parameters were calculated using OCTA, and the same was evaluated for any correlation with different ocular parameters mentioned above. The data were reported as frequencies/percentages and mean ± SD. The association between quantitative variables was evaluated using Pearson's correlation coefficient. RESULTS: The dFAZ area (0.56 ± 0.12 mm 2 ) was larger than the sFAZ area (0.42 ± 0.13 mm 2 ). Females had larger FAZ than males. AL and CFT had a negative correlation, whereas CCT had a positive correlation with FAZ. Age did not influence FAZ. sFAZ and dFAZ varied significantly in healthy eyes. CONCLUSION: FAZ parameters calculated using OCTA in healthy Indian eyes suggested that the area and dimensions in both SCP and DCP are larger in individuals from the Indian subcontinent when compared to other parts of the world. FAZ area and dimensions, when compared to previous studies, were variable. Thus, there is a need to establish normative data for ethnicity for proper interpretation of FAZ using OCTA.

The Association of Neovascularization With Optical Coherence Tomography Angiography Parameters in Proliferative Diabetic Retinopathy.

AIM: We aim to find an association between neovascularization (NVn) and optical coherence tomography angiography (OCTA) parameters in proliferative diabetic retinopathy (PDR). METHODS: In a prospective study, 41 subjects including 28 (68%) males and 13 (32%) females having PDR were examined for neovascularization disc (NVD) and neovascularization elsewhere (NVE) clinically and with fundus fluorescein angiography (FFA). A total of 79 eyes were found to be involved. We examined OCTA parameters including foveal avascular zone (FAZ) size, perimeter and circularity, and vessel density (VD) in the superficial capillary plexus (SCP), deep capillary plexus (DCP), outer retina (OR), outer retinal chorio-capillaries (ORCC), chorio-capillaries (CC), and choroid (C) in these subjects. RESULTS:  In eyes with NVD, the central foveal thickness (CFT) (p=0.83) and sub-foveal choroidal thickness (SFCT) (p=0.08) were higher, the FAZ area was significantly larger (p=0.005), and the VD was lower in all retino-choroidal layers. However, it was significantly lower in SCP foveal (p=0.005) and ORCC foveal (p=0.05) than in eyes not having NVD. For NVE, the CFT (p=0.03) and SFCT (p=0.01) were more in affected eyes. The eyes without NVE had a better circularity index (p=0.07) and the highest VD in OR slab (p=0.02) than those eyes that had NVE < ½ disc area (DA) and NVE > ½ DA. On comparing eyes without NVE, NVE < ½ DA, and NVE > ½ DA, the latest had the highest VD in SCP (p=0.59) and lowest VD in DCP (p=0.43) and OR (p=0.02). The VD in ORCC, CC, and choroid was highest in the no NVE group, followed by the NVE > ½ DA and NVE < ½ DA groups in that order. The subjects having vitreous hemorrhage (VH) and intra-retinal microvascular abnormalities (IRMA) had higher values for CFT and SFCT than eyes without these. CONCLUSIONS:  An increased CFT and SFCT are associated with the appearance of NVD, NVE, VH, and IRMA. The presence of NVD, VH, and IRMA is associated with a larger FAZ area, while that of IRMA and NVE is associated with reduced FAZ circularity. Eyes with NVD, VH, and IRMA had lesser VD in all the retino-choroidal layers. Eyes with NVE > ½ DA had the highest VD in SCP and lowest in DCP and OR; this pattern of VD foretells severer affection in NVE. IRMA was associated with a larger FAZ area, larger FAZ perimeter, and lesser circularity, indicating the presence of central ischemia.

Evaluating the Quantitative Foveal Avascular Zone and Retino-Choroidal Vessel Density Using Optical Coherence Tomography Angiography in a Healthy Indian Population.

OBJECTIVE: The foveal avascular zone (FAZ) is the round capillary-free zone within the macula and is supplied only by a single-layered parafoveal capillary arcade. This study aimed to evaluate the quantitative FAZ and retino-choroidal vessel density (VD) using optical coherence tomography angiography (OCTA) in a healthy Indian population. METHODS: This was a cross-sectional observational study that was conducted for evaluating the quantitative FAZ and retino-choroidal VD of 200 eyes of 100 healthy Indian subjects, including 62 males and 38 females (age range 17-50 years) having the best-corrected visual acuity (BCVA) of logMAR 0 (20/20; 6/6) and spherical equivalent refractive error of not more than 1 D. The subjects were examined using OCTA automated software on spectral-domain OCT (SD-OCT; Nidek RS 3000 Advance 2; Nidek, Inc., Fremont, CA) on a 3 x 3 mm OCTA macular scan centred on the fovea. The FAZ size, perimeter and circularity index, VD in superficial, deep, and outer retina (OR), outer retinal chorio-capillaries (ORCC), chorio-capillaries (CC) and choroid (C) were analysed in the circular and quadrant-segmented zones. A correlation was found between the FAZ size, perimeter and circularity, and VD in retino-choroidal layers, and between BCVA, age, central foveal thickness (CFT) and sub-foveal choroidal thickness (SFCT), and OCTA parameters. RESULTS: The FAZ and surrounding vascular arcades were intact in all eyes, showing either a vertical or horizontal oval-shaped symmetrical formation without gaps, holes or interruption of the capillary network. The mean value of CFT was 237.5±26.0 microns and SFCT was 269.6±53.0 microns. The mean FAZ area was 0.42±0.23 mm2, FAZ perimeter was 3.3±1.0 mm and FAZ circularity index was 0.46±0.1. The mean VD in superficial capillary plexus (SCP) was 23.87±10.66, in deep capillary plexus (DCP) was 16.03±9.90, in OR was 13.22± 12.27, in ORCC was 39.74±14.32, in CC was 37.02±16.43 and in choroid was 37.43±16.76. The increasing order of VD in different retino-choroidal layers was OR

Iridofundal Coloboma Associated with Vitreous Haemorrhage and an Intraocular Mass: A Case Report.

INTRODUCTION: Iridofundal colobomas, being developmental defects, are known to be accompanied by several ocular anomalies but an association with vitreous hemorrhage and an intraocular mass has not been reported earlier. CASE: We report a case of an 18 years old subject having iridofundal coloboma in both eyes and an association of vitreous hemorrhage and an intraocular mass in the left eye. The diagnosis was confirmed with a detailed ocular examination, fundus fluorescein angiography, swept-source optical coherence tomography and ultrasonography. The laser barrage of coloboma caused a regression in the size of the intraocular mass and prevented recurrence of vitreous hemorrhage over one and half years of follow-up. CONCLUSION: The source of vitreous hemorrhage in this subject is unclear though some anomalous vessels in relation to the supero-nasal mass, optic disc and coloboma of the left eye might have caused it. The laser barrage of coloboma obscured these anomalous vessels resulting in the regression of the intraocular mass.

Predictors of Retinal Nerve Fiber Layer Parameters Following Scleral Buckling Surgery in Primary Rhegmatogenous Retinal Detachment.

OBJECTIVE: This study aimed to examine predictors of retinal nerve fiber layer (RNFL) parameters following scleral buckling (SB) surgery for primary rhegmatogenous retinal detachment (RRD) and to determine the influence of the magnitude of change in qualitative and quantitative parameters on RNFL. METHODS:  In an observational prospective study, 40 subjects who underwent successful retinal reattachment with SB surgery done within one month of RRD were evaluated for the parameters of best-corrected visual acuity (BCVA), refractive error, intraocular pressure (IOP), axial length (AL), anterior chamber depth (ACD), angle opening distance (AOD 500 and AOD 750), trabecular iris surface area (TISA 500 and TISA 750), visual fields, and ganglion cell count (GCC) and RNFL before and three months after SB. We additionally noted qualitative factors like extent, location, and type of buckle; phakic status; and grade of proliferative vitreoretinopathy in the affected eye. The change in value of quantitative parameters was found. The influence of baseline values and magnitude of change of quantitative and qualitative parameters on average RNFL thickness and magnitude of change of RNFL thickness after SB was found. RESULTS:  Post-SB, average RNFL thickness reduced from 108.58±20.38 microns to 103.73±17.98 microns (p =0.042). The baseline temporal upper (TU), temporal lower (TL), and nasal lower (NL) RNFL thickness (p=0.01, p=0.02, p=0.01, respectively) and total deviation (TD) values of visual fields (p=0.01) correlated positively while baseline GCC gross loss of volume (p=0.01) correlated negatively with post-operative RNFL thickness. The TU, TL, and NL RNFL thickness (p=0.04, p=0.01, p=0.01, respectively) and average GCC (p=0.04) correlated negatively with the magnitude of change in RNFL. The magnitude of change in baseline parameters after surgery was correlated with the magnitude of change in average RNFL thickness. It was noticed that change in AL (p<0.01), TISA 500 (p=0.02), TISA 750 (p<0.01), GCC focal loss of volume (p=0.02), and temporal RNFL thickness (p<0.01) correlated positively while the change in refractive error correlated negatively (p=0.04). Except for the grade of proliferative vitreoretinopathy (PVR) (p=0.04), none of the qualitative parameters, including extent, type, and location of the buckle; and phakic status, had a significant association with post-operative average RNFL thickness or magnitude of its change. CONCLUSIONS:  The predictors of average RNFL thickness following SB include AL; myopic shift; TISA; visual fields TD; average, TU, TL, and NL RNFL thickness; average GCC, gross and focal loss of volume; and grade of PVR. So an early surgery to prevent preoperative ganglion cell and RNFL loss and progression of PVR is recommended.