Sodium fluorescein dye as an adjunct in vitrectomy for rhegmatogenous retinal detachment in oculocutaneous albinism.

A 48-year-old male with oculocutaneous albinism (OCA) presented with bilateral diminution of vision. Ocular examination revealed bilateral central corneal thinning, scarring with ectasia, depigmented irides, transillumination defects, and pseudophakia. Examination of the right eye also revealed a hyperoleon, emulsified silicon oil in the vitreous cavity, and an attached retina, while the left eye had a total rhegmatogenous retinal detachment (RRD). This case describes a unique set of challenges (the presence of an ectatic scarred cornea and a hypopigmented fundus) and sodium fluorescein dye as an adjunct in the surgical management of a complex RRD. A review of literature highlighting the association of keratoconus and RRD in OCA is also presented in this report.

Diagnostic Accuracy of Pediatrician-performed Digital Retinal Imaging with 3nethra neo for ROP Screening.

OBJECTIVES: To evaluate the accuracy of pediatrician-performed wide-field digital retinal imaging (WFDRI) for diagnosing Retinopathy of prematurity (ROP), as compared to binocular indirect ophthalmoscopy (BIO) as the reference standard. METHODS: Eligible infants undergoing ROP screening were enrolled consecutively. BIO was performed by trained ophthalmologists, followed by WFDRI (using "3nethra neo" camera) by a pediatrician. An expert pediatric ophthalmologist reviewed de-identified images for quality, presence, and severity of ROP. She was masked to the findings of BIO and the pediatrician. Diagnostic accuracy for detecting any ROP, ROP requiring treatment (Type 1), and ROP requiring referral (Type 1 or 2) were calculated for WFDRI, considering BIO as the reference standard. RESULTS: The analysis included 427 eyes. The sensitivity, specificity, positive likelihood ratio (PLR), negative likelihood ratio (NLR), diagnostic accuracy, and diagnostic odds ratio of WFDRI were 0.88 (95% CI: 0.81, 0.93), 0.89 (0.85, 0.92), 7.8 (5.7, 10.9), 0.14 (0.09, 0.21), 0.89 (0.85, 0.91), and 58.3 (31, 110) respectively for detection of 'any ROP'. For detecting ROP requiring treatment (Type 1), the sensitivity, specificity, NLR, and diagnostic accuracy were 0.90 (0.75, 0.97), 1.00 (0.99, 1.00), 0.11 (0.04, 0.27), and 0.99 (0.98, 1.00) respectively. For ROP requiring referral, the sensitivity, specificity, NLR, and diagnostic accuracy of pediatrician-performed WFDRI were 0.92 (0.80, 0.98), 1.00 (0.99, 1.00), 0.08 (0.03, 0.21), and 0.99 (0.98, 1.00) respectively. No serious adverse events were noted. The pediatrician and ophthalmologist had a near-perfect (k-1.00) and strong (k-0.88) agreement for ROP requiring treatment and any ROP, respectively. CONCLUSIONS: Pediatrician-performed WFDRI is feasible, safe, and has excellent diagnostic accuracy for identifying ROP requiring treatment.

Use of the Aurolab Aqueous Drainage Implant as a buckling element in pediatric retinal detachment with a preexisting glaucoma drainage device.

Purpose: To describe a novel technique for repair of rhegmatogenous retinal detachment in an eye with a previous non-valved glaucoma drainage device, the Aurolab Aqueous Drainage Implant (AADI). Observations: A 5-year-old child with bilateral primary congenital glaucoma presented with an inferior retinal detachment (RD) in the left eye. The left eye had a history of multiple surgical interventions including combined trabeculotomy and trabeculectomy done twice, AADI implantation and subsequently phacoaspiration with IOL implantation, 18 months prior to presentation. The left eye retinal detachment was managed by scleral buckling technique using the plate of the AADI as a buckling element without its explantation. Conclusions: AND IMPORTANCE: Management of retinal detachment in eyes with a pre-existing glaucoma drainage device (GDD) is uniquely challenging. Explantation of the GDD would likely result in intractable glaucoma post-operatively, requiring another surgery. Use of the trimmed plate of the GDD itself as the buckling element helped in settling the RD and preserving intraocular pressure control.

Pituitary Dysfunction in Idiopathic Intracranial Hypertension: An Analysis of 80 Patients.

BACKGROUND: Empty sella is a commonly described imaging entity in patients with idiopathic intracranial hypertension (IIH). Though menstrual and hormonal disturbances have been associated with IIH, available literature lacks systematic analysis of pituitary hormonal disturbances in IIH. More so, the contribution of empty sella in causing pituitary hormonal abnormalities in patients of IIH has not been described. We carried out this study to systematically assess the pituitary hormonal abnormalities in patients with IIH and its relation to empty sella. METHODS: Eighty treatment naïve patients of IIH were recruited as per a predefined criterion. Magnetic resonance imaging (MRI) brain with detailed sella imaging and pituitary hormonal profile were done in all patients. RESULTS: Partial empty sella was seen in 55 patients (68.8%). Hormonal abnormalities were detected in 30 patients (37.5%), reduced cortisol levels in 20%, raised prolactin levels in 13.8%, low thyroid-stimulating hormone (TSH) levels in 3.8%, hypogonadism in 1.25%, and elevated levels of gonadotropins were found in 6.25% of participants. Hormonal disturbances were independent and were not associated with the presence of empty sella (p = 0.493). CONCLUSION: Hormonal abnormalities were observed in 37.5% patients with IIH. These abnormalities did not correlate with the presence or absence of empty sella. Pituitary dysfunction appears to be subclinical in IIH and responds to intracranial pressure reduction, not requiring specific hormonal therapies.

Pediatric Lupus Retinopathy: A Rare Manifestation of a Grave Systemic Disorder.

We describe a rare case of pediatric systemic lupus erythematosus (pSLE) and its successful management. A nine-year-old female presented with bilateral diminution of vision, fever, and rash in the malar region, chest, abdomen, back, and arms for three months. Clinical examination and multimodal imaging revealed bilateral extensive retinal vasculitis with macular edema. Laboratory investigations revealed anemia, leucopenia, positive serum antinuclear antibody (ANA), and anti-extractable nuclear antigen (ENA) antibodies. A diagnosis of pediatric lupus retinopathy was made. Ocular and systemic manifestations responded well to intense systemic immunosuppression (pulse intravenous {IV} methylprednisolone, oral prednisolone and hydroxychloroquine {HCQ}, six cycles of IV cyclophosphamide, and oral azathioprine) along with topical steroids and laser photocoagulation, over the next 10 months. Though ocular manifestations are not a part of the diagnostic criteria for SLE, they may be markers of active systemic disease. Ophthalmologists and rheumatologists must treat this complex disease in tandem in order to provide optimum patient care.

Displacement of Submacular Hemorrhage With Vitrectomy Combined With Subretinal Balanced Salt Solution and Air.

PURPOSE: To describe a technique of displacement of submacular hemorrhage (SMH) using subretinal injection of balanced salt solution and filtered air. METHODS: Patients presenting within 2 weeks of massive SMH (>4 disk diameter) were prospectively included. All patients underwent 25-gauge pars plana vitrectomy, posterior vitreous detachment, injection of subretinal balanced salt solution and filtered air followed by partial fluid air exchange, 20% sulfur hexafluoride tamponade, and heads-up positioning postoperatively. Degree of displacement of SMH was assessed at 1 month and change in best-corrected visual acuity was assessed at 3 months. RESULTS: Ten patients with massive SMH who underwent the aforementioned procedure were included. Complete displacement of bleed from the macula was achieved in nine (90%) of 10 eyes at 1 month. There was significant improvement in best-corrected visual acuity from baseline at 1 month ( P = 0.015) and 3 months ( P = 0.043). CONCLUSION: Pars plana vitrectomy with injection of subretinal balanced salt solution and filtered air was well-tolerated and efficacious in displacing large and thick SMH in patients operated within 2 weeks of onset of symptoms.

Retinopathy of Prematurity and Glucose-6-Phosphate Dehydrogenase Activity: A Case-Control Study.

OBJECTIVE: To determine whether red blood cell glucose-6-phosphate dehydrogenase (G6PD) activity is associated with retinopathy of prematurity (ROP). METHODS: This case-control study was conducted in a Level-3 neonatal unit. Subjects were inborn boys with birth weight <2000 g. "Cases" were consecutive subjects with ROP of any severity. "Controls" were consecutive unrelated subjects without ROP. Recipients of blood or exchange transfusions were excluded. Sixty cases (out of 98 screened) and 60 controls (out of 93 screened) were enrolled. G6PD activity (quantitative assay) as the candidate risk factor was evaluated. RESULTS: Sixty cases with 60 controls [mean (SD) gestation 28.80 (2.2) and 30.60 (2.2) wk respectively] were compared. "Cases" had a higher median (1st, 3rd quartile) G6PD activity compared to "controls" [7.39 (4.7, 11.5) vs. 6.28 (4.2, 8.8) U/g Hb, p = 0.084]. G6PD activity was highest among ROP requiring treatment [8.68 (4.7, 12.3)] followed by ROP not requiring treatment [6.91 (4.4, 11.0)], followed by controls (plinear trend = 0.06). Gestation, birth weight, duration of oxygen, breastmilk feeding, and clinical sepsis were other variables associated with ROP on univariable analysis. On multivariable logistic regression, G6PD activity [Adjusted OR 1.14 (1.03, 1.25), p = 0.01] and gestation [Adjusted OR 0.74 (0.56, 0.97), p = 0.03] independently predicted ROP. C-statistic of the model was 0.76 (95% CI 0.67, 0.85). CONCLUSIONS: Higher G6PD activity was independently associated with ROP after adjusting for confounders. Each 1 U/g Hb increase in G6PD increased the odds of ROP by 14%. Severer forms of ROP were associated with higher levels of G6PD activity.

Endogenous Endophthalmitis in COVID-19 Patients: A Case Series and Literature Review.

PURPOSE: To report outcomes in cases of endogenous endophthalmitis (EE) following COVID-19 infection. MATERIAL AND METHODS: In a retrospective study, patients with EE, who had a recent history of COVID-19 infection requiring hospital admission were recruited. Necessary demographic details, details of ocular examination, and microbiological details were collected. RESULTS: Six patients (10 eyes), with a mean age of 48 + 19.80 years were included. The mean duration of onset of ocular symptoms from the time of diagnosis of COVID was 28.16 + 16.15 days. 8 eyes required surgical intervention, whereas 2 eyes were managed conservatively. Three patients were positive for Candida albicans, two patients were positive for Aspergillus fumigatus, and one patient was a presumed bacterial EE. The majority of the eyes had favorable functional and anatomical outcomes during the post-operative period. CONCLUSION: High-dose corticosteroid therapy in the management of moderate and severe COVID-19 infection may be associated with EE, predominantly fungal.

Papillomacular bundle sparing versus conventional internal limiting membrane peeling for idiopathic macular hole ≤400 µm.

Purpose: To compare the outcomes of papillomacular bundle (PMB) sparing internal limiting membrane (ILM) peeling (group LP) and conventional ILM peeling (group CP) for treatment of idiopathic macular hole (MH) of ≤400 µm. Methods: Fifteen eyes were included in each group. In group CP, conventional 360° peeling was done, while in group LP, ILM was spared over PMB. The changes in peripapillary retinal nerve fiber layer (pRNFL) thickness and ganglion cell-inner plexiform layer (GC-IPL) thickness were analyzed at 3 months. Results: MH was closed in all with comparable visual improvement. Postoperatively, retinal nerve fiber layer (RNFL) was significantly thinner in the temporal quadrant in group CP. GC-IPL was significantly thinner in the temporal quadrants in group LP, whereas it was comparable in group CP. Conclusion: PMB sparing ILM peeling is comparable to conventional ILM peeling in terms of closure rate and visual gain, with the advantage of less retinal damage at 3 months.

Subretinal Hyperreflective Material (SHRM) as biomarker of activity in Exudative and Non- exudative inflammatory choroidal neovascularization.

AIM: To analyze the structural features and therapeutic response in clinical and subclinical inflammatory choroidal neovascularization (i-CNV) detected inside subretinal hyperreflective material (SHRM) using swept-source optical coherence tomography (SS-OCT) and SS-OCT angiography (SS-OCTA). METHODS: In this prospective interventional study, subjects with quiescent posterior uveitis presenting with SHRM on SS-OCT and CNV network on SS-OCTA were included. Subjects with intraretinal fluid/subretinal fluid (IRF/SRF) received intravitreal antivascular endothelial growth factor (anti-VEGF) injections, while those with no IRF/SRF either received treatment or observation for 6 months until they developed IRF/SRF or decrease in best-corrected visual acuity (BCVA)/metamorphopsia. Serial comparisons included SHRM width and height and intrinsic flow signal on OCTA. RESULTS: 28 eyes of 22 subjects (12 males; mean age: 29.52 ± 12.56 years) were evaluated. Subjects with IRF/SRF at baseline (n = 6 eyes; termed as exudative iCNVs) receiving treatment showed significant improvement in BCVA (p = .017), SHRM width/height and flow signal (p < .05). Among eyes with no IRF/SRF (n = 22; termed as non-exudative iCNVs), 7 received treatment and showed significant improvement in SHRM parameters and BCVA (p < .05). 4/15(26.67%) eyes that received no treatment developed IRF/SRF upon 6-month follow-up. CONCLUSION: SHRM may act as a useful biomarker to monitor activity and response to therapy in eyes with iCNV.

Correlation of angiogenic growth factors and inflammatory cytokines with the clinical phenotype of ocular tuberculosis.

PURPOSE: To determine the correlation of angiogenic growth factors and inflammatory cytokines with the clinical phenotype of ocular tuberculosis (OTB). METHODS: Vitreous fluid was analysed for cytokines in patients with OTB and non-OTB uveitis using multiplex fluorescent bead-based flow cytometric assay. The clinical phenotypes were recorded and correlated with vitreous biomarkers. RESULTS: Vitreous humour from OTB patients had elevated levels of interleukin-10 (IL-10), IL-17-A, interferon-gamma (IFN-γ), and tumour necrosis factor-alpha (TNF-α). Angiopoietin (Ang-2) levels were higher in the panuveitis phenotype. OTB posterior uveitis phenotype had relatively higher vascular endothelial growth factor (VEGF) levels and lower fibroblast growth factor (FGF) levels. Additionally, eyes with choroiditis and vasculitis had elevated levels of VEGF and Ang-2 with FGF downregulation. Both IFN-γ and IL-10 were upregulated in the choroiditis phenotype of OTB. CONCLUSION: Angiogenic growth factors and inflammatory cytokines were altered in the vitreous humour of OTB patients. IFN-γ, VEGF, and IL-10 levels are increased in choroiditis and vasculitis phenotypes. Receiver operating characteristic (ROC) curve analysis further emphasized the importance of the IFN-γ assay in the diagnosis of OTB.

Serum Calcitonin Gene-Related Peptide Is Elevated in Patients With Migraine and Ophthalmoplegia.

BACKGROUND: There is ongoing debate about whether the oculomotor (III), trochlear (IV), or abducens (VI) nerve paresis in patients with migraine is directly attributable to migraine (ophthalmoplegic migraine [OM]) or is due to an inflammatory neuropathy (recurrent painful ophthalmoplegic neuropathy [RPON]). As migraine is associated with elevated serum calcitonin gene-related peptide (CGRP) levels, we studied serum CGRP levels among patients with OM/RPON to determine whether they are elevated during and between attacks. This is the first study assessing CGRP levels in the serum of patients with OM/RPON. METHODS: The aim of this case-control study was to assess serum CGRP levels in patients with ophthalmoplegia and a headache consistent with migraine according to ICHD-3 criteria. Serum CGRP levels were measured during the ictal and interictal phases in 15 patients with OM/RPON and compared with age-matched and sex-matched controls without migraine (12 patients). RESULTS: The median serum CGRP levels were significantly elevated ( P = 0.021) during the ictal phase (37.2 [36.4, 43.6] ng/L) compared with controls (32.5 [30.1, 37.3] ng/L). Serum CGRP levels during the attack correlated with the total duration of ophthalmoplegia. A CGRP level of 35.5 ng/L in the ictal phase of the attack had a sensitivity of 86.7% and specificity of 75.0% in diagnosing a patient with OM/RPON. CONCLUSIONS: Elevated serum CGRP levels during the ictal phase of OM/RPON favor migraine as the underlying cause of episodic headache with ophthalmoplegia.

Systemic biomarkers of retinopathy of prematurity in preterm babies.

PURPOSE: Retinopathy of prematurity (ROP) progression is an inter-play of various perinatal and neonatal angiogenic and inflammatory cytokines. A small subset of ROP progresses to ROP requiring treatment. The present study was conducted with the aim to determine whether levels of IL-6, IL-8 and VEGF in serum and urine at the time of first ROP screening visit could be a biomarker for the prediction of development of treatable ROP. METHOD: Prospective single-center observational study of preterm babies screened for ROP. Blood and urine samples were collected as a part of routine sampling at initial ROP screening visit and stored at -80 °C for further processing. The babies were followed up and grouped into 'Group A' comprising of 35 babies who developed treatable ROP and 'Group B' comprising of 36 babies with regressed ROP or no ROP. The evaluation of blood and urine samples was done for IL6, IL8 and VEGF by solid-phase sandwich RayBio® Human ELISA kit. RESULTS: The median serum values for IL-6, IL-8 and VEGF in Group A and Group B were 5.8 pg/ml (IQR 1.5,128.5) and 8.7 pg/ml (IQR 1.5,30.5), 55.9 pg/ml (IQR 28.0, 392.9) and 27.0 pg/ml (IQR 20.5,444.9) and 26.6 pg/ml (IQR 6.3, 39.4) and 30.0 pg/ml (IQR9.2,70.3), respectively. Group A had significantly increased levels of IL-8 (p < 0.05). However, AUROC curve for serum IL-8 demonstrated suboptimal discriminating ability. CONCLUSION: Babies developing ROP requiring treatment had significantly increased levels of IL-8 in the serum at the time of initial screening. However, it could not serve as predictor for treatable ROP.

Morphological characterization of subretinal hyper-reflective material in posterior uveitis using swept-source optical coherence tomography and optical coherence tomography angiography.

Purpose: To analyze the structural features of subretinal hyper-reflective material (SHRM) in posterior uveitis using swept-source optical coherence tomography (SS-OCT) and optical coherence tomography angiography (SS-OCTA). Methods: In this observational study, subjects with quiescent posterior uveitis and the presence of SHRM on SS-OCT were subjected to SS-OCTA to identify the presence of an intrinsic choroidal neovascular (CNV) network. OCT features were compared for SHRM harboring CNV (vascular SHRM) with those without CNV network (avascular SHRM) to identify clinical signs pointing toward the presence of CNVM inside SHRM. Results: Forty-two eyes of 33 subjects (18 males; mean age: 29.52 ± 12.56 years) were evaluated. Two-thirds (28/42) of eyes having SHRM on SS-OCT harbored intrinsic neovascular network (vascular SHRM). Increased reflectivity of SHRM (P < 0.001) and increased transmission of OCT signal underlying SHRM (P = 0.03) were suggestive of the absence of CNVM. The presence of intra/subretinal fluid (P = 0.08) and pitchfork sign (P = 0.017) were important markers of vascular SHRM. Conclusion: SHRM is an important OCT finding in eyes with posterior uveitis. Meticulous assessment of SHRM characteristics on SS-OCT can aid in identifying the underlying intrinsic neovascular network.

MULTICOLOR CONFOCAL SCANNING LASER OPHTHALMOSCOPE IMAGING IN POSTERIOR UVEITIS.

PURPOSE: To study the utility of MultiColor confocal scanning laser ophthalmoscope imaging (MCI) in identifying the morphology of uveitic lesions compared with conventional color fundus photography (CFP) in patients with posterior uveitis. METHODS: In this prospective observational study, subjects with posterior uveitis underwent MCI and CFP. The images obtained by the two modalities were analyzed by two independent reviewers for vitreoretinal surface abnormalities, retinal fluid and hemorrhages, and depth/location of lesions. These findings were compared with the clinical findings and other imaging techniques. RESULTS: Sixty-nine eyes of 43 patients (25 men) with mean age of 33.5 ± 13.9 years were studied. MultiColor imaging had better sensitivity and specificity in detecting vitreoretinal interface abnormalities, such as epiretinal membrane and inner retinal striae, compared with CFP. MultiColor imaging failed to detect retinochoroiditis lesions in 5 of 6 eyes (83%) and choroiditis in 9 46 eyes (20%), which were detected on CFP and clinical examination. Also, MCI showed a high false-positive rate of 34% in detecting intraretinal hemorrhages. CONCLUSION: Retinochoroidal lesions in posterior uveitis may be poorly identified on MCI compared with CFP and clinical examination. One must exercise caution in commenting on disease morphology based on MCI alone.

Redrawing vitreoretinal surgical training program in the COVID-19 era: Experiences of a tertiary care institute in North India.

Purpose: The COVID-19 pandemic has brought medical and surgical training to a standstill across the medical sub-specialties. Closure of outpatient services and postponement of elective surgical procedures have dried up opportunities for training vitreoretinal trainees across the country. This "loss" has adversely impacted trainees' morale and mental health, leading to feelings of uncertainty and anxiety. Therefore, there is an urgent need to redraw the surgical training program. We aimed to describe a systematic stepwise approach to vitreoretinal surgical training. Methods: We introduced a three-pronged approach to vitreoretinal surgical training comprising learn from home, wet lab and simulator training, and hands-on transfer of surgical skills in the operating room in our institute. Results: Encouraging results were obtained as evaluated by feedback from the trainees about the usefulness of this three-pronged approach in developing surgical skills and building their confidence. Conclusion: The disruption caused by the COVID-19 global pandemic should be used as an opportunity to evolve and reformulate surgical training programs to produce competent vitreoretinal surgeons of the future.

Clinical Presentation, Investigation and Control of an Outbreak of Adenoviral Conjunctivitis in a Neonatal Unit at a Tertiary Hospital.

BACKGROUND: Nosocomial spread of adenovirus infection has been reported in neonatal, pediatric and adult medical units. This nonenveloped and hardy virus is resistant to numerous disinfectants thus posing a challenge for control and prevention of adenovirus infections in health care settings. METHODS: An epidemiologic outbreak investigation revealed an adenoviral outbreak in the neonatal nursery as well as in the neonatal screening outpatient department for Retinopathy of Prematurity (ROP). All suspected cases (94 neonates) underwent adenoviral conventional polymerase chain reaction (PCR) and representative samples underwent sequencing by Sanger's method. The clinical features and disease course were studied. Infected babies were started on tobramycin eye drops. Topical steroid eye drops were added for those who developed pseudomembranes. RESULTS: We found 58 cases of laboratory-confirmed neonatal adenovirus conjunctivitis (between July 10 and October 24, 2019). Redness (96%) was the most common presentation followed by discharge (68.9%) and lid edema (51.7%). Pseudomembrane were seen in 77.5% of the infected neonates. Prior ROP examination was carried out in 38 (65.5%) neonates. Respiratory symptoms were present in 7 (12.06%) neonates. Sequencing revealed serotype 8 as the cause of the outbreak. Control measures were strictly implemented. Standard Operating Procedures (SOPs) for ROP screening were revisited, revised and reinforced to prevent future outbreaks. CONCLUSIONS: We observed ROP screening as a risk factor for the development of adenoviral conjunctivitis in neonatal care units. Neonates present with different clinical manifestations as compared with adults. Prompt control measures were implemented to control the adenoviral outbreak.

A novel c.1937T>C (p.Leu646Pro) missense mutation in a patient with Leber congenital amaurosis.

Over 21 genes have been associated with the inherited retinal dystrophy, Leber congenital amaurosis (LCA). A comprehensive genotype-phenotype correlation in such heterogenous cases helps guide further genetic studies and therapies. We report 2 children (10-month-old girl and an 8-month-old boy) who presented with low vision in the first year of life. Both patients manifested nystagmus, sluggish pupillary reactions, hyperopia, and normal fundus. Focussed exome sequencing was performed because LCA was suspected. A novel c.1937T>C (p.Leu646Pro) missense mutation was found in exon 9 of the tyrosine kinase domain of the GUCY2D gene in both patients.