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Purpose: Our study aimed to evaluate the utility of the anterior segment morphometry for objectively assessing anterior segment architectural changes of corneal clouding in the mucopolysaccharidoses (MPS) cohort and to investigate whether these measurements correlate with the slit?lamp findings on the cornea and early diagnosis of glaucoma. Methods: This retrospective study involved 70 eyes of 35 children with cloudy cornea due to MPS variants. Anterior segment architectural alterations were measured using anterior segment imaging and biometry in MPS children and compared with controls. Results: Mean age of the cohort at the time of assessment was 7.9 ± 4.5 years. Males constituted two?thirds of the cohort. Variants of MPS with cloudy cornea were as follows: Type I (62%), Type IV (11%), and Type VI (22%). Morphometric measurements were available in 22 eyes of 11 MPS children and an age?matched healthy control group. There were significant differences between MPS cohort and controls in refraction in Diopters (5.03 ± 0.39 and 0.01 ± 0.04; P < 0.0001), axial length (AXL) in mm (21.39 ± 0.28 and 23.04 ± 0.28; P = 0.0002), average keratometry in Diopters (40.67 ± 0.44 and 42.83 ± 0.44; P < 0.0001), anterior chamber depth (ACD) in mm (2.92 ± 0.07 and 3.65 ± 0.07; P < 0.0001), and intraocular pressure (IOP) in mmHg (25.2 ± 2.0 and 14.1 ± 2.3; P = 0.0003). Secondary glaucoma was observed in 28% of the MPS cohort. Conclusion: The anterior segment morphometry in the cloudy cornea due to MPS provides an objective measurement of anterior segment architectural changes, thus diagnosing early?onset secondary glaucoma. These findings highlight that cloudy cornea due to MPS variants merits close monitoring throughout life
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Purpose: To describe the clinical presentation and demographic distribution of retinitis pigmentosa (RP) in Laurence–Moon–Bardet–Biedl (LMBB) syndrome patients. Methods: This is a cross?sectional observational hospital?based study wherein 244 patients with RP in LMBB syndrome presenting to our hospital network between March 2012 and October 2020 were included. An electronic medical record database was used for data retrieval. Results: There were 244 patients in total, with a hospital?based prevalence rate of 0.010% or 1000/100,000 population. The mean and median age of patients was 15.22 ± 7.56 and 14 (IQR: 10–18.5) years, respectively, with the majority being in the age group of 11–20 years (133/244 patients; 54.50%). Males were more commonly affected (164 patients; 67.21%), and the majority (182 patients; 74.59%) were students. All 244 patients (100%) complained of defective central vision at presentation. More than one?fourth of the patients had severe visual impairment to blindness at presentation. Prominent retinal feature at presentation was diffuse or widespread retinal pigment epithelial degeneration in all patients. Conclusion: Patients with RP in LMBB syndrome present mainly in the first to second decade of life with severe visual acuity impairment to blindness early in life. It is important to rule out LMBB syndrome in early?onset RP with central visual acuity impairment. On the contrary, all patients diagnosed or suspected with LMBB syndrome systemic features at physician clinic should also be referred for ophthalmic evaluation, low vision assessment, rehabilitation, and vice versa
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Purpose: To evaluate patterns of pediatric vitelliform macular dystrophy (PVMD). Methods: This is a retrospective analysis of Indian children with vitelliform macular dystrophy (VMD) presenting within the first decade of life. Records were evaluated for clinical findings, family screening, and investigative findings including optical coherence tomography (OCT), fundus autofluorescence (FAF), full?field electroretinogram (ERG) and electrooculogram (EOG). Electrophysiology was scrutinized and audited for acquisition and interpretation errors. Findings on follow?up were also recorded. Results: 46 eyes of 24 patients were included. Mean age at presentation was 7.17 ± 2.17 years. Mean follow?up duration was 1.55 ± 1.69 years. Best disease was the commonest type of VMD detected (21 patients), while autosomal recessive bestrophinopathy was seen in three cases. Mean logMAR BCVA was 0.364 which decreased to 0.402 on follow?up. Hyperopia was noted in 29 out of 46 eyes (mean being +3.87 D, range ebing +0.75 to +8.75 D). Four eyes of four children had choroidal neovascular membrane at presentation, while another child developed while in follow?up. Solid type subretinal deposit was the commonest OCT finding (n = 29/38) and central hyper FAF was the commonest pattern (n = 18/32). EOG was available for review in 32 eyes, but was unreliable in 11 eyes. Seven eyes demonstrated complete absence of light rise on EOG. Conclusion: PVMD can present in advanced forms. Progression to complications with loss of visual acuity can happen within the first decade of life. EOG shows grossly suppressed waveforms in the light phase in a large number of such children
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Purpose: To determine and validate retinal vascular caliber measurements by using the confocal scanning laser ophthalmoscopy system. Retinal vasculature changes are often regarded as clinical markers for systemic disease. Methods: It was a prospective observational study conducted on 600 eyes of 300 normal subjects with no systemic or ocular illness from January 1, 2016 to June 30, 2017 in a tertiary referral eye center. Non?mydriatic infrared reflectance, blue reflectance, and blue peak blue autofluorescence fundus imaging were done on the confocal scanning laser ophthalmoscopy system. The dimensions of the retinal vessels were measured using inbuilt calipers at 1800 ?m from the center of the optic disc. Internal and external dimensions were measured. Observer variation and its comparison using Image J software were assessed. Results: The median age was 29 years (18–50 years). Mean internal and external diameters for arterioles were 85.1 ± 12.4 ?m and 105.0 ± 12.0 ?m, and for venules were 133.8 ± 16.6 ?m and 145.4 ± 16.1 ?m, respectively. The mean internal and external wall thicknesses were 19.7 ± 8.0 ?m and 11.0 ± 5.6 ?m, and wall thickness?to?lumen ratios were 0.3 ± 0.1 and 0.1 ± 0.1, respectively. Arteriolar?to?venular ratio for lumen and vessel was 0.66 ± 0.1 and 0.74 ± 0.1, respectively. There was no statistically significant difference between age groups. Both inter? and intra?observer reproducibility was >95%. The Bland–Altman plot showed that the difference between measurements using both confocal scanning laser ophthalmoscopy and Image J software lies within the limits of agreement approximately 95% of the time. Conclusion: This is the first effort to develop a normative database by using a simple non?invasive confocal scanning laser ophthalmoscopy system with high observer reproducibility
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Purpose: This study aimed to investigate the efficacy of human?derived umbilical cord mesenchymal stem cells (HDUMSC) and human?derived umbilical cord mesenchymal stem cells expressing erythropoietin (HDUMSC?EPO) to rescue total degenerated retina in a rat model. Methods: The study included four treatment groups, namely negative control using normal saline (HBSS) injection, positive control using sodium iodide 60 mg/kg (SI), SI treated with HDUMSC, and SI treated with HDUMSC?EPO given via subretinal and intravenous routes, to test the efficacy of retinal regeneration following SI?induced retinal degeneration. Retinal function in both phases was tested via electroretinography (ERG) and histological staining examining the outer nuclear layer (ONL). Results: There was a statistically significant result (P < 0.05) in the SI treated with HDUMSC?EPO only when comparing day 11 (mean = 23.6 ?v), day 18 (mean = 25.2 ?v), day 26 (mean = 26.3 ?v), and day 32 (mean = 28.2 ?v) to the b?wave ERG on day 4 rescue injection day (mean = 12.5 ?v). The SI treated with HDUMSC?EPO showed significant improvement in b?wave ERG readings in the Sprague–Dawley (SD) rat but did not restore baseline readings prior to degeneration (day 0). Both treated groups’ ONL thicknesses did not show significant changes compared to the negative control group (HBSS) following rescue therapy. Conclusion: Total retinal degeneration following intravenous SI injection was observed at 60 mg/kg. SI treated with HDUMSC and HDUMSC?EPO showed no regenerative potential compared to baseline in SI?induced total retina degeneration on ERG or histology, whereas SI treated with HDUMSC?EPO group showed a substantial increase in b?wave ERG amplitude over time
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Purpose: To evaluate the outcome of vitrectomy with multilayered inverted internal limiting membrane flap technique (ML?IILM) versus vitrectomy with standard ILM peeling for large macular holes in terms of visual acuity and anatomical closure. Methods: A hospital?based, prospective, randomized, interventional study was conducted during three calendar years with a total 150 eyes (75 in each group) in two groups—vitrectomy with ILM peeling (Group A) and vitrectomy with ML?IILM flap technique (Group B) after informed consent of study participants who met the inclusion criteria. Results: The mean minimum and maximum diameter of macular hole did not differ statistically in both the groups. Macular hole index had no significant difference between both groups Pre?operative visual acuity was not statistically significantly different between the two groups. During follow?up, best corrected visual acuity (BCVA) at 1 month, 3 months, 6 months, and 12 months was significantly better in Group B (0.12 ± 0.07 at 1 month, 0.14 ± 0.10 at 3 months, 0.18 ± 0.11 at 6 months, and 0.19 ± 0.12 at 12 months) compared to Group A (0.20 ± 0.11 at 1 month, 0.22 ± 0.13 at 3 months, 0.30 ± 0.12 at 6 months, and 0.31 ± 0.14 at 12 months) (P = 0.001 for each). Type 1 anatomical closure (flattening of cuff and opposition of edges of hole) was achieved in 78.66% (59/75) cases in Group A and 93.33% (70/75) cases in Group B (P 0.0016). Conclusion: Vitrectomy with multilayered inverted ILM flap technique had significantly higher anatomical closure and better visual outcome than vitrectomy with standard ILM peeling
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Endoilluminator‑assisted scleral buckling combines the advantages of scleral buckling for its external approach and pars plana vitrectomy for its better visual visualization in the management of retinal detachment (RD). It has recently been proven to be safe and efficacious in simple cases. This report discusses successful management of a complex case of RD in a patient with the single functioning eye, where vitrectomy was expected to have a complicated course.
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Traumatic optic neuropathy due to missile injury typically results in unilateral visual loss.[1] We discuss an uncommon case, in which injury from retroocular passage of a single bullet resulted in bilateral orbital perforations, frozen globes, and complete blindness. A 20‑year‑old female presented with bilateral absent light perception detected immediately, following alleged accidental bullet injury. There were no cranial manifestations and sutured skin wounds were visible on the temporal aspects of both the orbits. Bilateral ocular coats were intact with normal intraocular pressure and anterior segments. However, there was bilateral complete afferent pupillary defect and vitreous hemorrhage. Noncontrast computerized tomography (NCCT) imaging revealed multiple orbital and nasal fractures, a bony fragment abutting the right globe [Fig. 1a], and bilateral temporal orbital perforations on reconstructed images [Fig. 1b and c]. The entry wound was left‑sided and slightly lower than the right‑sided exit wound [Fig. 1b and c]. Sonography revealed attached bilateral retinas. The combination of complete afferent and efferent neural dysfunction was attributed to injury of bilateral posterior orbits near the apical region. No ocular intervention was planned due to poor prognoses and absence of any foreign body.