Ophthalmologic manifestations of organoid nevus syndrome: A series of 13 cases.

Background: The organoid nevus syndrome is a rare neurocutaneous syndrome typified by cutaneous sebaceous nevus, seizures and epibulbar choristomas. The condition is associated with multiple ocular abnormalities. Herein, the authors aim to study and report the ophthalmic features of cases diagnosed with organoid nevus syndrome. Methods: The authors retrospectively evaluated the records of patients with the organoid nevus syndrome who had presented to a tertiary care eye hospital in northern India. The ocular features were studied and entered in MS excel and the data were evaluated. Results: Data of 13 patients with the organoid nevus syndrome were found. All 13 patients had cutaneous features in the form of Sebaceous nevus of Jadasson, 8 patients had alopecia of the scalp area, 2 had history seizures and 10 had arachnoid cysts on neuroimaging of the head. All 13 patients had a complex choristoma involving the ocular surface. Conclusions: We conclude that the most common ophthalmologic features associated with organoid nevus are complex choristoma of the bulbar surface, scleral coat calcification and upper eyelid coloboma.

Case report of SARS-CoV-2 with secondary Clostridioides difficile infection.

There has been an overuse of antibiotics in most patients suffering from COVID-19 which predisposes patients to hospital acquired C. difficile Infection (CDI). Also, COVID infection of the gastrointestinal tract also predisposes the patient to CDI. We here present a fatal case of SARS-CoV-2 coinfection with CDI.

Clinical and diagnostic imaging profile of three anterior segment dysgenesis disorders presenting with infantile corneal opacities.

PURPOSE: To describe three anterior segment dysgenesis disorders with infantile corneal opacities, namely, congenital hereditary endothelial dystrophy (CHED), primary congenital glaucoma (PCG), and Peters anomaly (PA) in terms of clinical characteristics, histopathology, genetic association, and diagnostic imaging profiles using imaging modalities such as ultrasound biomicroscopy (UBM) and microscope-integrated intraoperative optical coherence tomography (i-OCT). MATERIALS AND METHODS: Seventy-four eyes with 22 eyes of CHED, 28 eyes of PA, and 24 eyes of PCG were clinically evaluated and underwent imaging using UBM and i-OCT. Corneal buttons of 16 operated patients underwent histopathological analysis, while genetic analysis was done in 23 patients using whole-exome sequencing. RESULTS: Corneal diameters (CD) and UBM parameters like anterior chamber depth (ACD), iris thickness (IT), and ciliary body (CB) thickness revealed a statistically significant difference between the three categories. In PA, 9 eyes had a third rare phenotype with only a posterior corneal defect with no iris adhesions. Genetic mutations were seen in all tested patients with CHED, in 83.3% of patients with PCG, and in 80% of patients with the third type of PA. i-OCT helped in the characterization of corneal opacity, identification of posterior corneal defects, iridocorneal adhesions, and contour of Descemet's membrane. CONCLUSION: Overlapping phenotypes of the above disorders cause a diagnostic dilemma and parameters like CDs, UBM ACD, IT, and CB thickness help differentiate between them. i-OCT can help in classifying the diseases in a high resolution, non-contact manner, and can better delineate corneal characteristics. The rare third type of PA phenotype may have a genetic association.

Ocular Aberrations in Eyes with Primary Congenital Glaucoma.

PURPOSE: To highlight the magnitude of ocular higher order aberrations (HOA) and lower order aberrations (LOA), including component contributions from corneal and internal planes in Primary Congenital Glaucoma (PCG) patients. METHODS: Consecutive treated PCG patients co-operative for ocular examination and aberrometry, were enrolled over two years for this cross-sectional, comparative, single-center, unmasked study. Best-corrected visual acuity, refraction, IOP, wavefront aberrometry and topography (iTrace) were performed and results were compared with unaffected fellow eyes of unilateral glaucoma patients as well as age and sex-matched controls with no ocular anomalies other than treatable refractive error. RESULTS: Both eyes of 32 consecutive PCG patients (17 unilateral, 15 bilateral) and 39 controls were enrolled. The median LogMAR corrected distance visual acuity of PCG eyes was 0.68 (IQR: 0.2-1.8). Total ocular (Root mean square (RMS) 1.7 µm vs 0.3 µm, p = 0.014), corneal (RMS 1.1 µm vs 0.3 µm, p = 0.004) and internal (RMS 1.1 µm vs 0.2 µm, p = 0.013) aberrations, as well as HOAs and LOAs at each plane, were significantly higher in PCG eyes than in controls. Component HOAs from corneal and internal planes were positively correlated with each other (p < 0.001; rs: 0.7). Total aberrations were greater in the affected eyes of PCG compared to the rest. The predominant subtype of HOAs in PCG was coma and trefoil. PCG with corneal opacity/Haab's striae had significantly higher astigmatism than the affected eyes with clear corneae at the corneal plane (p = 0.02). The aberrations were not statistically associated with the corneal diameter or refractive error in PCG eyes. CONCLUSIONS: Significantly greater aberrations (Total, HOAs and LOAs, at corneal as well as an internal plane) were seen among eyes affected with PCG. Though the exact impact of these aberrations on the final visual outcome is difficult to determine, these could play a pertinent role in compromising visual function, thus impacting the management of visual rehabilitation in these patients.

Microscope Integrated Optical Coherence Tomography Guided Descemet Stripping Automated Endothelial Keratoplasty in Congenital Hereditary Endothelial Dystrophy.

PURPOSE: To describe the outcomes of descemet stripping automated endothelial keratoplasty (DSAEK) in congenital hereditary endothelial dystrophy (CHED) and to evaluate the role of microscope integrated optical coherence tomography (Mi-OCT) during the surgery. DESIGN: Retrospective data analysis. METHODS: A retrospective study from the medical records of all those patients who were diagnosed with CHED and underwent DSAEK at our centre from 2015 were evaluated. All patients underwent Mi-OCT-guided standard DSAEK procedure. Intra-operative difficulties, visual outcomes and graft survival were recorded. RESULTS: A total of 48 eyes of 29 patients with a mean age of 9.87 ± 8.2 years and mean follow-up of 17.3 months were evaluated. Thirty-nine eyes underwent primary DSAEK and 9 eyes underwent PKP. Three eyes who underwent PKP had failed graft for which they underwent DSAEK. The mean preoperative Snellen's visual acuity was 1.71 ± 0.66 and the mean preoperative central corneal thickness was 1.10 ± 0.174 mm. Intraoperatively, all the grafts were attached which was confirmed using Mi-OCT. Graft detachment was seen in the immediate postoperative period in 10.4% (4 eyes) of primary DSAEK, out of which DM scoring was not performed in 2 eyes. Following DSAEK, cornea cleared at four-week follow-up in 89.7% eyes and in all the eyes the cornea cleared at six-week follow-up. CONCLUSION: Primary DSAEK could be a preferred option over PKP for CHED with early presentation and in those eyes with failed primary PKP. Mi-OCT is a very useful tool in these eyes for various intraoperative procedures, thereby improving the outcomes of the procedure.

Demography, clinical profile and surgical outcomes of paediatric giant retinal tear related retinal detachments.

OBJECTIVE: To determine the demographic profile, clinical features and surgical outcomes of giant retinal tear (GRT) related retinal detachments (RD) in children. METHODS: In this retrospective study, medical records of children aged 14 years and below, who underwent surgery for RD at our centre in the last 5 years were reviewed. Among these, we selected medical records of children with RDs with GRT, and examined the coloured retinal drawings, ultrawidefield photographs and/or surgical videos. RESULTS: Out of 1536 medical records, 91 eyes of 87 children (5.6% of all RDs) were included. Mean age was 10.21 ± 3.08 years with male:female ratio of 8.6:1. The most common aetiologies for GRTs were high myopia (28 cases-32.18%) and trauma (25 cases-28.73%). Bilateral RD were possibly present in 29/87 (33.3%) cases. Six months follow up data was available for 82/91 eyes. Overall retinal re-attachment was achieved in 52/82 (63.41%) eyes; in 33/82 (40.24%) eyes retinal attachment could be achieved by a single surgery. Absence of proliferative vitreoretinopathy (PVR) (OR: 2.44, p-value:0.03, 95%CI: 1.21-5.08) or PVR-A (OR: 3.62, p-value: 0.03, 95%CI: 1.52-12.26) and presence of preexisting posterior vitreous detachment (OR: 7.14, p-value: 0.02, 95%CI: 1.31-38.73) were associated with successful retinal re-attachment after single surgery. Median time to presentation of cases succeeding after 1 surgery was 10 days. Ambulatory vision (1/60 and better) at final followup could be achieved in 45/82 (54.88%) eyes. CONCLUSION: GRT relatedRDs constitute a significant proportion of paediatric RDs. Anatomical success can currently be achieved in a large number of cases. Early surgery, absence of PVR and presence of PVD are associated with higher surgical success.