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Purpose: Retinoschisis is a distinctive condition characterized by intraretinal layer clefts, primarily associated with X‑linked recessive inheritance due to RS1 gene mutations. This study aims to uncover the RS1 mutation spectrum in a cohort of 22 X‑linked retinoschisis cases from South India and emphasizes the genotypic and phenotypic associations within patients harboring only RS1 mutations. Methods: A total of 22 probands were suspected of having X‑linked retinoschisis. All study subjects underwent ophthalmic investigations, including assessments of visual acuity, fundus examination, optical coherence tomography (OCT), and electroretinogram (ERG). RS1 gene screening was conducted using Sanger sequencing, and the pathogenicity of the variants was assessed through Sorting Intolerant from Tolerant (SIFT) and PolyPhen‑2 in silico tools. Results: The study found that the probands had an average visual acuity of 0.79 ± 0.39 log of minimum angle of resolution (logMAR), ranging from 0.17 to 1.77. During fundus examination, the probands exhibited a characteristic spoke wheel‑like pattern in the macular region. Furthermore, OCT analysis revealed distinct alterations in the inner retinal microstructure, and ERG results consistently showed a reduction in b‑wave amplitude. Eventually, Sanger sequencing results showed hemizygous mutations in the RS1 gene in only 12 probands, including a novel missense mutation in the RS1 gene’s signal sequence. Conclusion: This study provides valuable insights into the spectrum of RS1 mutations in X‑linked retinoschisis probands from South India. It reveals distinct genotypic–phenotypic associations and highlights the clinical manifestations associated with the disease pathogenesis. [ABSTRACT FROM AUTHOR]

Purpose: Retinoschisis is a distinctive condition characterized by intraretinal layer clefts, primarily associated with X-linked recessive inheritance due to RS1 gene mutations. This study aims to uncover the RS1 mutation spectrum in a cohort of 22 X-linked retinoschisis cases from South India and emphasizes the genotypic and phenotypic associations within patients harboring only RS1 mutations. Methods: A total of 22 probands were suspected of having X-linked retinoschisis. All study subjects underwent ophthalmic investigations, including assessments of visual acuity, fundus examination, optical coherence tomography (OCT), and electroretinogram (ERG). RS1 gene screening was conducted using Sanger sequencing, and the pathogenicity of the variants was assessed through Sorting Intolerant from Tolerant (SIFT) and PolyPhen-2 in silico tools. Results: The study found that the probands had an average visual acuity of 0.79 ± 0.39 log of minimum angle of resolution (logMAR), ranging from 0.17 to 1.77. During fundus examination, the probands exhibited a characteristic spoke wheel-like pattern in the macular region. Furthermore, OCT analysis revealed distinct alterations in the inner retinal microstructure, and ERG results consistently showed a reduction in b-wave amplitude. Eventually, Sanger sequencing results showed hemizygous mutations in the RS1 gene in only 12 probands, including a novel missense mutation in the RS1 gene’s signal sequence. Conclusion: This study provides valuable insights into the spectrum of RS1 mutations in X-linked retinoschisis probands from South India. It reveals distinct genotypic–phenotypic associations and highlights the clinical manifestations associated with the disease pathogenesis.

Keywords: Refractory macular edema; retinoschisis; subfoveal plaque EN Refractory macular edema retinoschisis subfoveal plaque 2706 2708 3 07/15/22 20220701 NES 220701 X-linked retinoschisis (XLRS) is an inheritable juvenile retinal dystrophy.[[1]] The prevalence ranges from 1:5,000 to 1:20,000, making it one of the most common juvenile-onset retinal degeneration in males involving the macula.[[2]] The pathognomic feature is foveal schisis (98-100% cases) with some displaying typical bicycle-wheel patterns of radial streaks extending from the fovea. The boy was presumed to have XLRS on the basis of fundus features and multi-modal imaging was suggested to investigate the cause of macular edema in RE.{Figure 1} Optical coherence tomography (OCT) revealed retinoschisis involving multiple layers of macula BE, with predominant involvement of the outer plexiform-inner nuclear layer complex. Rao et al.[[4]] reported the largest series yet of the exudative variant with 11 eyes in 18 patients of XLRS that showed signs of subretinal exudation. [Extracted from the article]

Aims: To study the phenotypic characteristics of X-linked retinoschisis (XLRS) and report the clinical, electroretinogram (ERG), and optical coherence tomography (OCT) variables in Indian eyes. Design: A retrospective study. Materials and Methods: Medical records of 21 patients with retinoschisis who were genetically confirmed to have RS1 mutation were reviewed. The phenotype characterization included the age of onset, best-corrected visual acuity, refractive error, fundus findings, OCT, and ERG. Statistical Analysis Used: Data from both the eyes were used for analysis. A P < 0.05 was set as statistical significance. Data were not normally distributed (P < 0.05, Shapiro wilk); hence, nonparametric tests were used for statistical analysis. Results: All were males whose mean age of presentation was 9 years. Visual acuity was moderately impaired (median 0.6 logMAR, interquartile range: 0.47, 1) in these eyes with a hyperopic refractive error of median +1.75 Ds (interquartile range: +0.50 Ds, +4.25 Ds). About 54.7% of the eyes had both foveal and peripheral schisis, isolated foveal schisis was seen in 28.5% of the eyes, and schisis with retinal detachment was seen in 16.6% of the eyes. The inner nuclear layer was found to be commonly involved in the schisis, followed by outer nuclear and plexiform layers as evident on OCT. On ERG, a- and b-wave amplitudes were significantly reduced in eyes with foveal and peripheral schisis when compared to the eyes with only foveal schisis (P < 0.05). Conclusions: XLRS has phenotypic heterogeneity as evident on OCT, ERG, and clinical findings.

Although X-linked retinoschisis is a common retinal degeneration condition, the presence of a large retinal cyst obscuring the visual axis in an infant is a rare presentation. Herein, we describe such a case of a child who presented to us with the diagnosis of retinal detachment in both the eyes. However, following multimodal imaging and electrophysiology, the child was found to have bilateral juvenile retinoschisis with a large retinoschisis cyst involving the visual axis seen intraoperatively in the left eye. A limbal approach followed by lensectomy was used to excise the inner retinal layer of the cyst. The intracystic fluid was then drained and the stretched retinal vessels were endocauterized and severed without causing any iatrogenic outer retinal breaks and retinal detachment. The correct diagnosis and meticulous preoperative planning of the surgical procedure helped us manage this challenging case with a favorable anatomical and functional outcome. [ABSTRACT FROM AUTHOR]

We conducted a retrospective review of 11 eyes undergoing eye wall resection between October 1998 and October 2009. The median age of 11 patients was 29 years. Decreased vision (eight) was the most common presenting symptom. Ciliary body medulloepithelioma was the most common clinical diagnosis (six). Medulloepithelioma was the most common histopathological diagnosis (four). The duration of follow-up ranged from 0.5 to 67 months (median 11 months). Three eyes needed to be enucleated in the postoperative period (margin involvement two eyes, recurrence one eye). Postoperative complications among others included retinal detachment (three), vitreous hemorrhage (three), cataract (two), and suprachoroidal hemorrhage (two). To conclude, prognosis of this procedure continues to be guarded needing close postoperative follow-up.

Purpose : To study the optical coherence tomography (OCT) patterns in optic disc pit maculopathy and retinal changes after vitreous surgery. Materials and Methods : Retrospective review of consecutive cases with optic disc pit maculopathy seen at two tertiary eye institutes from January 2005 to June 2009. Results : Twenty-four eyes of 23 patients are included. The presenting visual acuity ranged from 20/400 to 20/20 (median:20/80). The median age at presentation was 24 years (range, 6-57 years). Optical coherence tomography demonstrated a combination of retinoschisis and outer layer detachment (OLD) in 19 (79.17%) eyes, OLD only in 3 (12.5%) eyes and retinoschisis only in 2 (8.33%) eyes. An obvious communication (outer layer hole) between the schisis and OLD was seen in 14 (73.68%) of the 19 eyes with both features. Of the 21 eyes with retinoschisis, schisis was present in multiple layers in 15 (71.43%) and single layer in 6 (28.57%) eyes. Eleven eyes underwent pars plana vitrectomy including creation of posterior vitreous detachment (PVD), fluid-air exchange, low intensity laser photocoagulation at the temporal edge of the optic disc pit and non-expansile perfluoropropane gas (14%) injection. Five (45.45%) of 11 eyes undergoing vitrectomy had complete resolution and 4 (36.36%) eyes had partial resolution of maculopathy. Visual acuity improved in 8 (72.72%) of 11 eyes. Conclusion : Optical coherence tomography demonstrates multiple layer schisis and outer layer detachment as main features of optic disc pit maculopathy. Vitrectomy with PVD induction, laser photocoagulation and gas tamponade results in anatomical and visual improvement in most cases with optic disc pit maculopathy.

Context: Intracameral mydriatic solution can eliminate the disadvantages of repeated eye drop instillation regimen and provide adequate mydriasis for phacoemulsification with added advantages. Aims: Evaluating the role of intracameral irrigating solution (0.5% lignocaine + 0.001% epinephrine) in initiating and maintaining the pupillary mydriasis during phacoemulsification. Secondary aims were to observe the effect of surgical time, nucleus density and ultrasound time on mydriasis during the procedure. Settings and Design: The study is a prospective interventional case series, conducted at tertiary care institution. Materials and Methods: Thirty patients underwent phacoemulsification under topical anesthesia for visually significant cataract. Pupillary dilatation was achieved by intracameral irrigation of mydriatic solution alone. Pupillary diameter was measured serially during surgery and ultrasound time and total surgical time were noted. Statistical Analysis Used: Paired samples student-t test was done to compare means. Spearman correlation coefficient was used to study the effect of various parameters on mydriasis. Results: Thirty eyes completed the study. The pupil size increased from 2.1 mm (Range 2-3.5 mm SD ± 0.32) to 6.9 mm (Range 5-9 mm SD ± 1.02) in 30 seconds time after intracameral mydriatic solution delivery, and was 7.0 mm (Range 3.5 - 9 mm SD ± 0.20) at the end of surgery. Duration of surgery, grade of nucleus and ultrasound time had statistically insignificant effect on mydriasis. Conclusions: Intracameral solution containing 0.5% lignocaine and 0.001% epinephrine provides rapid mydriasis which is adequate for safe phacoemulsification and is unaffected by other parameters.

Background: Regular follow up and amblyopia treatment are essential for good outcomes after pediatric cataract surgery. Aim: To study the regularity of follow-up after cataract surgery in children and to gauge the causes of poor compliance to follow up. Subjects: 262 children (393 cataracts) who underwent cataract surgery in 2004-8. Materials and Methods: The children were identified and examined in their homes and a "barriers to follow-up" questionnaire completed. Demographic data collected, visual acuity estimated, and ocular examination performed. Statistical Analysis: SPSS version 19. Results: Of the 262 children, only 53 (20.6%) had been regularly following up with any hospital, 209 (79.4%) had not. A total of 150 (57.3%) were boys and the average age was 13.23 years (Std Dev 5 yrs). Poor follow up was associated with the older age group ( P < 0.001), less education of mother ( P = 0.012), father′s occupation ( P = 0.031), how much money spent on travel ( P = 0.033) and was it paid or free surgery ( P = 0.001). It was not related to gender, numbers of children in family, ordinal status of child, and social strata. Distance and cost were major barriers, as was the inability of the eye care center to communicate the importance of follow up. A prospective follow-up visit showed that 93 children needed Nd: YAG LASER capsulotomy, 5 needed low vision aids, 4 contact lens, and 162 a change of spectacles. The average visual acuity improved in 150 (38.8%) eyes >1 line with regular follow-up. Conclusion: Regular follow-up is important and improves vision; eye care practitioners need to take special efforts to ensure better follow-up.

Orbital complications due to ethmoiditis are rare in neonates. A case of orbital abscess due to acute ethmoiditis in a 28-day-old girl is presented. A Successful outcome was achieved following antimicrobial therapy alone; spontaneous drainage of the abscess occurred from the lower lid without the need for surgery. From this case report, we intend to emphasize on eyelid retraction as a sign of neonatal orbital abscess, and to review all the available literature of similar cases.

Objective: To investigate the correlations between aqueous concentrations of vascular endothelial growth factor (VEGF), monocyte chemoattractant protein-1 (MCP-1), soluble intracellular adhesion molecule-1 (sICAM-1) and diabetic macular edema (DME). Materials and Methods: VEGF, MCP-1 and sICAM-1 concentrations in aqueous humor samples of 22 patients with DME and 23 patients with cataract of a control group were measured with solid-phase chemiluminescence immunoassay. Results: Aqueous VEGF (89.2 ± 58.5 pg/ml versus 48.5 ± 27.8 pg/ml, P = 0.006), MCP-1 (684.2 ± 423.4 pg/ml versus 432.4 ± 230.4 pg/ml, P = 0.019) and sICAM-1 (3213.8 ± 2581.6 pg/ml versus 260.2 ± 212.2 pg/ml, P < 0.001) all vary significantly between DME group and control group. Maximum height of submacular fluid measured by Optical coherence tomography (OCT) was significantly associated with aqueous sICAM-1 (r = -0.45, P = 0.034). The maximum height of macular thickness measured by OCT was not significantly associated with either VEGF (P = 0.300), MCP-1 (P = 0.320) or sICAM-1 (P = 0.285). Conclusions: Our results suggest that sICAM-1 may majorly contribute to the formation of subretinal fluid in DME patients and imply that MCP-1 and sICAM-1 may be the potential therapy targets, besides VEGF.

Rhodotorula mucilaginosa rarely cause keratitis in immunocompromised individuals. A 30 year old male with history of minor trauma presented with cotton wool like stromal infiltration and hypopyon in left eye. Microbiological examination of corneal scraping showed fungal hyphae and yeast cells in direct smear. Molecular identification of the organism was performed which showed 100% homology with Rhodotorula mucilaginosa. Management of these cases is difficult often necessitating surgical procedures. However further reports are necessary to understand the disease and establish a treatment protocol.

Purpose: To determine the effect of a capsular tension ring (CTR) implantation in preventing posterior capsular opacification (PCO) after cataract surgery in patients with high myopia. Materials and Methods: In this prospective single-surgeon standardized-surgical-procedure fellow-eye comparison trial, 34 patients with high myopia had phacoemulsification surgery. Although one eye received an acrylic intraocular lens (IOL) and CTR, other eye received only an IOL as control. PCO, within the capsulorhexis overlap, was documented by standardized digital retroillumination images at least 2 years post-operatively, and the percentage area of PCO was scored (scale 0%-100%) using the POCOman software system. The PCO score and the incidence of neodymium-doped yttrium aluminum garnet (Nd: YAG) capsulotomy of groups, and correlations between PCO score and presence of CTR, age, phaco time, refraction, and axial length (AL) were determined. Results: The mean time interval from surgery to PCO measurement was 43.4 ± 11.2 months for the eyes with a CTR and 43.1 ± 11.6 months for the controls (P = 0.91). The PCO score of the eyes with a CTR was significantly lower than in the controls (5.9 ± 4.3 vs. 22.3 ± 12.2, respectively; P < 0.001). There were statistically insignificant correlations between PCO score and pre-operative refraction (r = 0.02; P = 0.90), AL (r = 0.03; P = 0.80), phaco time (r = 0.11; P = 0.53), and patient′s age (r = 0.23; P = 0.55). No patient with a CTR had a Nd: YAG laser capsulotomy, but it was six in controls (P = 0.025). Conclusions: CTR implantation seems to be effective in reducing the PCO and Nd: YAG laser capsulotomy rates in high myopic eyes.

Pellucid marginal degeneration (PMD) is an uncommon cause of inferior peripheral corneal thinning disorder, characterized by irregular astigmatism. We analyzed a case of bilateral PMD patient and treated one eye with corneal collagen cross-linking (CXL) therapy. Corneal topography was characteristic for PMD. Visual acuity, slitlamp examinations, tonometry, and corneal thickness were observed. Simulated keratometric and topographic index values were detected with corneal topography. Uncorrected, LogMAR visual acuity has improved from +0.8 to +0.55 during the 6 months and +0.3 during the 8 months follow-up after CXL. Pachymetry values and intraocular pressure showed no changes. Keratometric values and topografic indexes disclosed no progression of the disease. CXL may postpone or eliminate the need of corneal transplantation in cases with PMD.

Aim: To provide the normative data of macular and retinal nerve fiber layer (RNFL) thickness in Indians using spectral domain OCT (Spectralis OCT, Heidelberg Engineering, Germany) and to evaluate the effects of age, gender, and refraction on these parameters. Design: Observational, cross-sectional study. Materials and Methods: The eyes of 105 healthy patients aged between 20-75 years, with no ocular disease and best corrected visual acuity of 20/20, were scanned using standard scanning protocols by a single examiner. Exclusion criteria included glaucoma, retinal diseases, diabetes, history of prior intraocular surgery or laser treatment. The mean macular and RNFL thickness were recorded, and the effects of age, gender, and refraction on these parameters were evaluated. This data was compared with published literature on Caucasians to assess the ethnic variations of these parameters. Results: The normal central foveal thickness in healthy Indian eyes measured using Spectralis OCT was 260.1 ± 18.19 ΅m. The nasal inner quadrant showed maximum retinal thickness (338.88 ± 18.17 ΅m).The mean RNFL thickness was 101.43 ± 8.63 ΅m with maximum thickness in the inferior quadrant. The central foveal thickness showed a gender-based difference (P = 0.005) but did not correlate significantly with age (P = 0.134), whereas the parafoveal, perifoveal thickness, macular volume, and RNFL thickness showed significant negative correlation with age. Conclusions: Our study provides the normative database for Indians on Spectralis OCT. It also suggests that age should be considered while interpreting the macular thickness and RNFL, whereas gender should also be given consideration in central foveal thickness.

Purpose: To investigate the effect of optic neuritis (ON), ischemic optic neuropathy (ION) and compressive optic neuropathy (CON) on multifocal visual evoked potential (mfVEP) amplitudes and latencies, and to compare the parameters among three optic nerve disorders. Materials and Methods: mfVEP was recorded for 71 eyes of controls and 48 eyes of optic nerve disorders with subgroups of optic neuritis (ON, n = 21 eyes), ischemic optic neuropathy (ION, n = 14 eyes), and compressive optic neuropathy (CON, n = 13 eyes). The size of defect in mfVEP amplitude probability plots and relative latency plots were analyzed. The pattern of the defect in amplitude probability plot was classified according to the visual field profile of optic neuritis treatment trail (ONTT). Results: Median of mfVEP amplitude (log SNR) averaged across 60 sectors were reduced in ON (0.17 (0.13-0.33)), ION (0.14 (0.12-0.21)) and CON (0.21 (0.14-0.30)) when compared to controls. The median mfVEP relative latencies compared to controls were significantly prolonged in ON and CON group of 10.53 (2.62-15.50) ms and 5.73 (2.67-14.14) ms respectively compared to ION group (2.06 (-4.09-13.02)). The common mfVEP amplitude defects observed in probability plots were diffuse pattern in ON, inferior altitudinal defect in ION and temporal hemianopia in CON eyes. Conclusions: Optic nerve disorders cause reduction in mfVEP amplitudes. The extent of delayed latency noted in ischemic optic neuropathy was significantly lesser compared to subjects with optic neuritis and compressive optic neuropathy. mfVEP amplitudes can be used to objectively assess the topography of the visual field defect.

Background: We evaluated the role of hyperhomocysteinemia as a risk factor for retinal vein occlusion (RVO) in Indian patients. Type of Study: Matched case control type of longitudinal study was conducted in 2006-2007. Materials and Methods: Two medical retina specialists examined the eyes having an event of RVO in the last 15 days. A similar number of eyes without RVO were also examined. The serum and urine homocysteine levels of these persons were tested. Matched pair analysis was carried out to determine the risk of RVO among those with hyperhomocysteinemia. Results: We included 20 cases of RVO and 20 age- and sex-matched persons without RVO. The risk of RVO was significantly higher in persons with hyperhomocysteinemia [difference of mean 31.62 μmol/L (95% Confidence Interval 16.60-47 . 86), P = 2.1 × 10−13 ]. The mean urine homocysteine level among cases and controls was not statistically significant. There were 12 persons with hypertension in both cases and control groups. Conclusion: Hyperhomocysteinemia is a risk factor for RVO. Any list of investigations for a case of RVO should include total plasma homocysteine (tHcy) levels.

Purpose: To evaluate the outcome of strabismus surgery for congenital superior oblique palsy (SOP) in relation to correction of head tilt and hypertropia. The cohort of patients mainly involved very young children. This is the first study to use a standardized instrument to objectively measure torticollis before and after surgery. Materials and Methods: A non-comparative interventional case series of 13 cases of congenital superior oblique palsy with head tilt, who underwent simultaneous superior oblique tuck and inferior oblique recession between Jan 2000 and Dec 2008, were studied. Results: The mean duration of SOP until surgery was 36.8 months. Of the 12 unilateral cases, 8 were right-sided. Mean follow-up period was 17 months (range 7-36). The outcome was determined at the last follow-up. Mean pre-and post-operative hypertropia (p.d.) in forced primary position was 19 ± 7 and 2 ± 6, respectively ( P < 0.0001). The head tilt reduced from mean of 17 ± 9 to 2 ± 2 degrees ( P < 0.0001). Success, defined as hypertropia

Aim: To evaluate the relationship between intraocular pressure (IOP) and systemic health parameters such as age, gender, body mass index (BMI), total cholesterol, high density lipoprotein (HDL), and triglyceride (TG) in a Korean population. Materials and Methods: A total of 30,893 healthy subjects underwent automated multiphasic tests, including non-contact tonometry, automated perimetry, fundus photography, and blood samplings for total cholesterol, HDL, and TG. Seven age groups were divided by decades ranging from 20 to 29 years to 80 + years. The association between IOP and BMI, plasma lipid profiles was examined using cross-sectional analysis. Results: The mean age of subjects was 47.7 years. The mean IOP of subjects was 15.4 ± 3.2 mmHg for both eyes. The mean IOP of men was significantly higher than women ( P = 0.000). By multiple linear regression analysis, IOP was positively associated with gender (male), BMI, total cholesterol, and TG and negatively associated with age ( P = 0.000). BMI, total cholesterol, and TG had significantly positive correlations with IOP after adjusting for age, gender, and other variables which can influence the IOP ( P = 0.000). Conclusions: In a Korean population, the mean IOP, total cholesterol, TG, and BMI values of men were higher than women. IOP was found to increase with total cholesterol, TG, BMI, and to decrease with only age regardless of sex.

Optical coherence tomography (OCT) has transformed macular disease practices. This report describes the use of conventional OCT systems for peripheral retinal imaging. Thirty-six eyes with peripheral retinal pathology underwent imaging with conventional OCT systems. In vivo sectional imaging of lattice degeneration, snail-track degeneration, and paving-stone degeneration was performed. Differences were noted between phenotypes of lattice degeneration. Several findings previously unreported in histopathology studies were encountered. Certain anatomic features were seen that could conceivably explain clinical and intraoperative behavior of peripheral lesions. Peripheral OCT imaging helped elucidate clinically ambiguous situations such as retinal breaks, subclinical retinal detachment, retinoschisis, choroidal nevus, and metastasis. Limitations of such scanning included end-gaze nystagmus and far peripheral lesions. This first of its kind study demonstrates the feasibility of peripheral retinal OCT imaging and expands the spectrum of indications for which OCT scanning may be clinically useful.

A 35-year-old female was referred to our hospital with bilateral loss of vision of two days duration. She gave history of consumption of about 150 ml of neem oil five days back.Examination revealed no perception of light in both eyes. Both pupils were dilated and sluggishly reacting to light. Her fundus examination showed bilateral hyperemic, edematous discs and also edema extending along the superior and inferior temporal vascular arcade. Magnetic resonance imaging (MRI) scan showed bilateral putaminal regions with altered signal, hypointensities in T1-weighted images, hyperintensities on T2-weighted, images and hyperintense on Fluid Attenuation Inversion Recovery (FLAIR) images suggestive of cytotoxic edema due to tissue hypoxia. Her vision improved to 20/200 in both eyes with treatment after two months. This is the first case report of such nature in the literature to the best of our knowledge.

While invasive aspergillosis occurs typically in severely immunocompromised patients, cases of surgical site infections have been reported in immunocompetent individuals. The purpose is to report an eye with post-operative Aspergillus endophthalmitis, which achieved a good visual outcome following early and aggressive treatment. A young patient, known case of allergic bronchopulmonary aspergillosis presented to us with post-cataract surgery endophthalmitis. He was treated with pars plana vitrectomy and intravitreal voriconazole and systemic itraconazole. The patient regained a vision of 20/30 with follow up of 2 years.

A 65-year-old lady presented with decreased vision in left eye since seven months. Vision was 6/9 in right eye and 6/36 in left. Examination revealed idiopathic, full-thickness macular hole in left eye; confirmed by optical coherence tomography (OCT). Patient underwent phacoemulsification with intraocular lens (IOL) implantation, vitrectomy, internal limiting membrane (ILM) peeling and 14% C 3 F 8 gas injection. OCT repeated after six weeks revealed type II closure with cuff of subretinal fluid. Four weeks later, patient underwent fluid-gas exchange with 14% C 3 F 8 gas and postoperative positioning. OCT was repeated after two weeks, which showed complete closure of the macular hole. OCT can help in selection of eyes for re-surgery that stand a better chance for hole closure. Macular holes with cuff of subretinal fluid are probably more likely to close on re-surgery than those without. However, larger studies with longer follow-up are required to validate this finding.

Nager syndrome, also called preaxial acrofacial dysostosis, comprises two groups of defects involving the limbs and craniofacial region, respectively. This syndrome is rare and only 70 cases have been reported in the literature. The exact cause of this syndrome is unknown, but there is indication that it is genetically based. Ocular manifestations of this syndrome include widely separated downward slanting eyes, absence of eyelashes, ptosis of upper eyelids and colobomas on the inner aspect of lower eyelids. We report limbal dermoid in a patient with Nager syndrome. We did not find such an association of "Limbal dermoid in Nager acrofacial dysostosis syndrome" on PubMed using Nager acrofacial dysostosis, limbal dermoid and ocular manifestations as the keywords.

A 57-year-old male patient visited our clinic for decreased visual acuity in the right eye for 10 days. He denied any trauma history, but recalled that the symptom developed after straining. He had undergone uncomplicated phacoemulsification and posterior chamber intraocular lens (IOL) implantation in the bag of the right eye 11 years ago. The IOL was a three-piece silicone polyimide-haptics design. On slit-lamp examination, the IOL optic and proximal part of nasal fractured haptic were found in the anterior chamber. The distal part of fractured haptic was observed in the capsular bag. He underwent IOL exchange. The fracture site of the haptic was near the optic-haptic junction. This is the unique case report of a spontaneous fracture of an implanted posterior chamber polyimide IOL haptic, which implies the possibility of IOL haptic fracture in various haptic materials.

X-linked juvenile retinoschisis is a rare hereditary retinal disease characterized by a tangential splitting of the neurosensory retina which may cause early-onset visual impairment. Existence of the retinal neurosensory layer splitting on cross-sectional images of optical coherance tomography (OCT) and the absence of leakage on fluorescein angiography (FA) help confirming the diagnosis. Such diagnostic tests are also helpful in determining the management of the disease. However, most of the retinoschisis cavities remain stable and rarely extend to the posterior pole, many authors suggest laser prophylaxis to avoid the potential risk of retinal detachment due to holes in the outer retinal layer. Herein, we report a case with bilateral foveal retinoschisis accompanying unilateral peripheral retinoschisis who was evaluated with detailed ophthalmologic examination. Visual acuity, fundoscopy, OCT, and FA remained stable in the second year of follow-up after prophylactic argon laser treatment.

Blepharophimosis ptosis epicanthus inversus syndrome (BPES) is a complex eyelid malformation characterized by the classical tetrad of blepharophimosis, telecanthus, ptosis, and epicanthus inversus. It has been reported to be associated with other ocular anomalies such as euryblepharon, strabismus, nystagmus, amblyopia, microphthalmos, lacrimal drainage apparatus abnormality, extra ocular muscle abnormalities, microcornea, trabecular dysgenesis, optic nerve hypoplasias, and colobomas of the optic disk. We describe a case of BPES with Axenfeld-Rieger syndrome, a neurocristopathy characterized by maldevelopment of the anterior segment with predisposition to development of glaucoma. Interestingly, both syndromes are caused by mutations in the same class of genes, namely the winged-helix/forked transcription factors (FOX) involved in a variety of developmental processes.

Cholesterol granuloma is a rare, well-defined lesion of the orbit. In the orbit, diploe of the frontal bone is involved almost exclusively. We report an atypical case of cholesterol granuloma involving superomedial quadrant of orbit. A 42-year-old male presented with progressive, painless, proptosis with infero-temporal displacement of left eye. A large mass was felt beneath the bony orbital margin in the superomedial quadrant of the left orbit. Computerized tomography (CT) scan revealed an extraconal superomedial, heterogeneous enhancing mass which was isodense with brain and pushing the globe inferolaterally and anteriorly. Excision biopsy of the tumor revealed the typical features of a cholesterol granuloma without any epithelial elements. Cholesterol granuloma of the orbit is a rare entity, but it can be diagnosed and differentiated from other lesions of the superior orbit by its characteristic clinical, radiological and histopathological features. An appropriate intervention in time carries a good prognosis with almost no recurrence.

A 65-year-old man developed capsular block syndrome in the early postoperative period, following phacoemulsification surgery. After neodymium-doped yttrium aluminum garnet (Nd:YAG) laser anterior capsulotomy, the intraocular pressure remained elevated for 4 days despite antiglaucomatous medication. On the postoperative fifth day, nonarteritic ischemic optic neuropathy was diagnosed. To the best of our knowledge, this is the first report of a case with nonarteritic ischemic optic neuropathy associated with early postoperative capsular block syndrome after phacoemulsification surgery.

Annular elastolytic giant cell granuloma is a condition characterized histologically by damaged elastic fibers associated with preponderance of giant cells along with absence of necrobiosis, lipid, mucin, and pallisading granuloma. It usually occurs on sun-damaged skin and hence the previous name actinic granuloma. A similar process occurs on the conjunctiva. Over the past three decades only four cases of conjunctival actinic granuloma have been documented. All the previous patients were females with lesions in nasal or temporal bulbar conjunctiva varying 2-3 mm in size. We report a male patient aged 70 years presenting with a 14 mm × 7 mm fleshy mass on right lower bulbar conjunctiva. Clinical differential diagnoses were lymphoma, squamous cell carcinoma in situ and amyloidosis. Surgical excision followed by histopathology confirmed it to be a case of actinic granuloma. This is the first case of isolated conjunctival actinic granuloma of such a large size reported from India.

We report the first case, in Medline-based literature, of conjunctivitis caused by gram negative diplococcus, Neisseria sicca. Although it is not widely accepted as such, isolation from cultures of repeated eye swab samples suggests that N. sicca may be a pathogen in conjunctival infections. Positive culture for this organism should not be readily dismissed. Such conjunctivitis responded favorably to treatment with netilmicin eye drops.

Sphenoid sinus mucocele comprises only 2% of all paranasal sinus mucoceles. In literature, there is a case report on sphenoidal mucocele causing bilateral optic neuropathy, with unilateral partial recovery and cranial nerve palsy, but we did not come across any literature with bilateral optic neuropathy and ophthalmoplegia together caused by spheno-ethmoidal mucocele. We present such a rare case of spheno-ethmoidal mucocele causing bilateral optic neuropathy and unilateral sixth nerve palsy who had postsurgery, unilateral good vision recovery, and complete resolution of sixth nerve palsy.

We are reporting an interesting case of ichthyosis follicularis with alopecia and photophobia syndrome in a daughter and father from the Indian subcontinent associated with Meibomian gland dysfunction. A three-year-old female child presented with pain, redness and severe photophobia in both eyes since one month. Cutaneous examination revealed ichthyosis, absence of hair all over the body including eyebrows, scalp and angular cheilosis. Ocular examination revealed bilateral severe meibomianitis, multiple superficial punctate keratitides in right eye and corneal epithelial defect in the left eye. Other systemic features were prominent high forehead and large ears. Her father had similar symptoms. Her symptoms improved after adequate treatment of meibomian gland dysfunction. She is asymptomatic at present.

Aims: To study the phenotypic characteristics of X-linked retinoschisis (XLRS) and report the clinical, electroretinogram (ERG), and optical coherence tomography (OCT) variables in Indian eyes.Design: A retrospective study.Materials and Methods: Medical records of 21 patients with retinoschisis who were genetically confirmed to have RS1 mutation were reviewed. The phenotype characterization included the age of onset, best-corrected visual acuity, refractive error, fundus findings, OCT, and ERG.Statistical Analysis Used: Data from both the eyes were used for analysis. A P < 0.05 was set as statistical significance. Data were not normally distributed (P < 0.05, Shapiro wilk); hence, nonparametric tests were used for statistical analysis.Results: All were males whose mean age of presentation was 9 years. Visual acuity was moderately impaired (median 0.6 logMAR, interquartile range: 0.47, 1) in these eyes with a hyperopic refractive error of median +1.75 Ds (interquartile range: +0.50 Ds, +4.25 Ds). About 54.7% of the eyes had both foveal and peripheral schisis, isolated foveal schisis was seen in 28.5% of the eyes, and schisis with retinal detachment was seen in 16.6% of the eyes. The inner nuclear layer was found to be commonly involved in the schisis, followed by outer nuclear and plexiform layers as evident on OCT. On ERG, a- and b-wave amplitudes were significantly reduced in eyes with foveal and peripheral schisis when compared to the eyes with only foveal schisis (P < 0.05).Conclusions: XLRS has phenotypic heterogeneity as evident on OCT, ERG, and clinical findings. [ABSTRACT FROM AUTHOR]

X-linked retinoschisis (XLR) is an uncommon bilateral vitreoretinal dystrophy characterized by typical foveoschisis in all patients that may be associated with peripheral retinoschisis. A young male with XLR with retinal detachment in his right eye underwent 23 gauge pars plana vitrectomy with silicone oil tamponade. Postoperatively, best-corrected visual acuity (BCVA) improved to 20/120 with an attached retina. Spectral-domain optical coherence tomography showed macular thinning with the collapse of the schitic cavities with silicone oil in situ. Following silicone oil removal at 6 months follow-up, the retina remained attached with a BCVA of 20/80 however the foveal schitic cavities reappeared. This unusual course has not been described previously.

Purpose: To study the optical coherence tomography (OCT) patterns in optic disc pit maculopathy and retinal changes after vitreous surgery. Materials and Methods: Retrospective review of consecutive cases with optic disc pit maculopathy seen at two tertiary eye institutes from January 2005 to June 2009. Results: Twenty-four eyes of 23 patients are included. The presenting visual acuity ranged from 20/400 to 20/20 (median:20/80). The median age at presentation was 24 years (range, 6-57 years). Optical coherence tomography demonstrated a combination of retinoschisis and outer layer detachment (OLD) in 19 (79.17%) eyes, OLD only in 3 (12.5%) eyes and retinoschisis only in 2 (8.33%) eyes. An obvious communication (outer layer hole) between the schisis and OLD was seen in 14 (73.68%) of the 19 eyes with both features. Of the 21 eyes with retinoschisis, schisis was present in multiple layers in 15 (71.43%) and single layer in 6 (28.57%) eyes. Eleven eyes underwent pars plana vitrectomy including creation of posterior vitreous detachment (PVD), fluid-air exchange, low intensity laser photocoagulation at the temporal edge of the optic disc pit and non-expansile perfluoropropane gas (14%) injection. Five (45.45%) of 11 eyes undergoing vitrectomy had complete resolution and 4 (36.36%) eyes had partial resolution of maculopathy. Visual acuity improved in 8 (72.72%) of 11 eyes. Conclusion: Optical coherence tomography demonstrates multiple layer schisis and outer layer detachment as main features of optic disc pit maculopathy. Vitrectomy with PVD induction, laser photocoagulation and gas tamponade results in anatomical and visual improvement in most cases with optic disc pit maculopathy. [ABSTRACT FROM AUTHOR]

Nodular fasciitis (NF) is a benign proliferation of fibroblasts and myofibroblasts that rarely occurs in the periorbital region. We report what we believe to be the first case of periorbital NF associated with pregnancy. A case of intravascular fasciitis, a NF variant, has been reported during pregnancy, but it was not located in the periorbital region. A weak presence of estrogen receptors has been reported in NF. This may make it more susceptible to the hormone-related changes during pregnancy and contribute to the development of the lesion by stimulating fibroblasts and smooth muscle cell types. Although rare, NF should be considered in the differential diagnosis of periorbital soft-tissue masses arising during pregnancy.

This is a single case report of an elderly patient who had blunt trauma in an eye that had phacoemulsification and two trabeculectomies. She had good vision with a well-functioning bleb before the trauma. She presented during her routine follow-up visit for glaucoma with isolated aniridia and an intact globe. The capsular bag, zonules, and the intraocular lens were intact. The cupping was 0.8, and the rest of the fundus and macula were normal. Pigments were seen over the sclera extending posteriorly upto the fornix. Gonioscopy revealed only faint pigments at the fistula. Following the trauma, the intraocular pressure had increased to 26 mm Hg. The mechanism and the management of the glaucoma are discussed.

Ocular dirofilariasis mostly presents as a subconjunctival or eyelid lesion. [1] Intraocular dirofilarial infestation is rare. [2],[3] We report a case of a young woman who was accidentally detected to have a live motile worm in the anterior segment in one eye and a cystic lesion on the optic disc in the other eye. To our knowledge, bilateral intraocular dirofilariasis has never been reported.

Implantation of iris-claw Artisan intraocular lens (IOL) is a surgical option for correction of aphakia; however, these IOLs have not been used in eyes with uveitis including Fuchs′ heterochromic iridocyclitis (FHI) due to possible risk of severe postoperative intraocular inflammation. In the case reported here, we secondarily implanted an Artisan IOL in a 28-year-old man with FHI who had aphakia with no capsular support due to a previous complicated cataract surgery. Enclavation was easily performed and no intraoperative complication was noted. Postoperative course was uneventful with no significant anterior chamber inflammation during 12 months of follow-up. Although there were few deposits on the IOL surface, the patient achieved a best-corrected visual acuity of 20/20 without developing glaucoma or other complications. Therefore, Artisan IOL may be considered for correction of aphakia in patients with FHI. However, studies on large number of patients are required to evaluate safety of the procedure.

Optical coherence tomography (OCT) has transformed macular disease practices. This report describes the use of conventional OCT systems for peripheral retinal imaging. Thirty-six eyes with peripheral retinal pathology underwent imaging with conventional OCT systems. In vivo sectional imaging of lattice degeneration, snail-track degeneration, and paving-stone degeneration was performed. Differences were noted between phenotypes of lattice degeneration. Several findings previously unreported in histopathology studies were encountered. Certain anatomic features were seen that could conceivably explain clinical and intraoperative behavior of peripheral lesions. Peripheral OCT imaging helped elucidate clinically ambiguous situations such as retinal breaks, subclinical retinal detachment, retinoschisis, choroidal nevus, and metastasis. Limitations of such scanning included end-gaze nystagmus and far peripheral lesions. This first of its kind study demonstrates the feasibility of peripheral retinal OCT imaging and expands the spectrum of indications for which OCT scanning may be clinically useful. [ABSTRACT FROM AUTHOR]