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The crystalline lens is an important structure in the eye that starts to develop as early as the 22nd day of gestation, with further differentiation that continues after the induction. Congenital anomalies of the lens may involve the size, shape, and position of the lens. They may sometimes be associated with anterior segment dysgenesis or persistence of the tunica vasculosa lentis and hyperplastic vitreous and hyaloid system. Manifestations of anomalies of the lens shape are usually seen in early or late childhood however may sometimes be delayed into adulthood based on the level of visual impairment or the presence or absence of any syndromic associations. While lens coloboma has more often been reported in isolation, the more commonly implicated genes include the PAX6 gene, lenticonus in particular anterior is often part of Alport syndrome with extra-ocular manifestations in the kidneys and hearing abnormalities due to mutations in the alpha 5 chain of the Type IV collagen gene. Recognition of these manifestations and obtaining a genetic diagnosis is an important step in the management. The level of visual impairment and amblyopia dictates the outcomes in patients managed either conservatively with optical correction as well as surgically where deemed necessary. This review discusses the various anomalies of the lens shape with its related genetics and the management involved in these conditions. [ABSTRACT FROM AUTHOR]

Peters anomaly (PA) is a rare, often bilateral, congenital corneal opacity, usually with a sporadic inheritance pattern, characterized by corneal opacities and irido-corneal or lenticular-corneal adhesions with a defect in the Descemet's membrane, occurring due to anterior segment dysgenesis during fetal development. Due to other ocular and systemic comorbidities, a team comprising pediatric cornea, glaucoma, and strabismus specialists in addition to a pediatrician and geneticist is necessary for the appropriate management of these children. Since the outcome of pediatric penetrating keratoplasty is variable and has a higher chance of failure when accompanied by additional procedures, such as lensectomy and vitrectomy, minimally invasive alternatives are increasingly being offered to these patients. Of note is the recently reported novel procedure: selective endothelialectomy for PA, which avoids the need for a corneal transplant and results in gradual clearing of the corneal opacity over time. In this overview, we aimed to describe the etiology, classification, pathophysiology, histopathology, clinical features, and management of PA. [ABSTRACT FROM AUTHOR]

Background and objective To describe the clinical features, diagnosis and management of immune stromal keratitis/interstitial keratitis (IK) associated with microsporidial epithelial keratitis. Methods Between October 2020 and January 2021, medical records of IK patients microbiologically proven as microsporidia from samples collected from corneal epithelium on smear examination, and/or molecular analysis were reviewed. Demography, clinical profile and treatment were analysed. Real-time PCR (RT-PCR) for adenovirus (ADV), Epstein-Barr virus (EBV), herpes simplex virus (HSV) and varicella-zoster virus (VZV) was done. Results Twenty of 152 (13%) microbiologically proven cases of microsporidial keratitis were diagnosed as IK during the study period, the mean age and duration of symptoms were 35.7±11.4 years and 46.3±27.7 days, respectively. Half had predisposing risk factors, like trauma; and 30% had prior recurrences. One-fourth of patients were using antivirals on presentation. Characteristic presentations included disciform keratitis(n=12), incomplete/complete ring(n=5), and combination(n=3), along with variable subepithelial infiltrates (n=14). All cases had stromal oedema, with an intact epithelium and fine pigment dusting on endothelium. Corneal epithelial scrapings had scanty microsporidia spores in smears of 17/20 (85%), and pan-microsporidial DNA was identified in 14/20 (70%), with Vittaforma corneae by sequencing in 11/20 (55%). Other viruses detected were ADV (14,70%), VZV (2,10%), EBV (1,5%) and HSV (1,5%). Rapid resolution of inflammation and oedema within 2 weeks of starting steroids was seen in all cases. Conclusion Microsporidia epithelial keratitis induced stromal inflammatory keratitis; is distinguished from microsporidial keratoconjunctivitis and stromal keratitis, by characteristic clinical features, and response to topical steroids. [ABSTRACT FROM AUTHOR]

Background: This report describes a very rare case of progeroid syndrome of De Barsy (Cutis laxa-corneal clouding syndrome). Materials and Methods: A 2 year-old child presented to the pediatric ophthalmology outpatients with bilateral congenital corneal opacification along with dysmorphic facial features, including loose wrinkled skin, progeroid appearance, delayed milestones, short stature, multiple hyper-extensible joints, muscular hypotonia, pectus excavatum and congenital dislocation of the hip joint. The child underwent a detailed ophthalmic work up and systemic evaluation by a clinical geneticist. Results: Ophthalmic management in the form of bilateral sequential penetrating keratoplasties and a left eye trabeculectomy for medically uncontrolled angle-closure glaucoma was performed. Visual rehabilitation with glasses and amblyopia therapy is ongoing. Histopathology of the corneal button revealed loss of the bowman's layer which was replaced by a fibrous pannus while the stroma showed loss of stromal lamellar architecture with anterior and mid stroma showing vascularization. Genetic testing confirmed a mutation in the PYCR1 gene for a homozygous autosomal recessive cutis laxa type IIB. Conclusions: Although rare, De Barsy syndrome is an important cause of corneal opacification at birth with multiple systemic abnormalities that requires intervention. [ABSTRACT FROM AUTHOR]

Purpose: To compare the intra-patient incidence of posterior capsular opacification (PCO) and their post operative course, in children with bilateral cataract, following implantation of acrylic (Group 1) and polymethyl methacrylate (PMMA) (Group 2) intraocular lenses (IOLs). Materials and Methods: This was a prospective, interventional intra-patient case series. Twenty-five children with bilateral cataract, 5 years and older, underwent cataract surgery and implantation of an acrylic (AcrySof MA30BA, Alcon, Fortworth, TX) in one eye and PMMA (Indo Am IAB 203, Ocular Vision, Inc.) IOL in the other eye of each patient. They were followed up for an average of 11.1 ± 9.5 months to assess the incidence of clinically significant PCO and occurrence of postoperative complications. Results: Twenty-three children (46 eyes), were included in this study. Post-operatively, 22(95.6%) patients with acrylic IOLs and 20 (86.9%) patients with PMMA IOLs, either maintained or improved their vision. In the acrylic and PMMA IOL groups respectively, the incidence of clinically significant PCO was 21% (4) and 75% (12) ( P =0.002), with a median onset at 2.9 months and 0.7 months. Other complications included pupillary capture in 2 (8.7%) eyes and uveal prolapse in 1 (4.3%) eye in the acrylic group and increased uveal inflammation in 6 (26.1%) eyes and presumed noninfectious endophthalmitis in 2 (8.7%) eyes in the PMMA group. Conclusion: Incidence of PCO and post operative uveal inflammation is significantly less with acrylic lenses and were safe to use in pediatric eyes.

PURPOSE: To describe the early results of penetrating keratoplasty (PKP) in patients who had earlier received limbal transplantation (LT). METHODS: Prospective, non-comparative interventional case series comprising of four patients with limbal stem cell deficiency (LSCD) due to chemical injury (Cases 1, 2, 4) and xeroderma pigmentosum (Case 3). Cadaveric kerato-limbal allografts or living-related conjunctival-limbal allografts were done in four eyes followed by PKP for visual rehabilitation 3 - 4.5 months later. The following details were noted: demographics, primary aetiology, type of limbal transplant (cadaveric or living-related), immunosuppression, vision and ocular surface stability before and after LT and PKP, surgical complications and outcome of PKP. RESULTS: Three eyes received living-related conjunctival-limbal allotransplantation and one received cadaveric kerato-limbal allograft. Duration of follow up after PKP ranged from 4 to 11 months. Visual acuity improved in the early postoperative period in all patients but reduced in 2 due to endothelial rejection and after trans-scleral cyclophotocoagulation for medically uncontrolled glaucoma. The ocular surface remained stable in all patients. All patients were started on immunosuppression on the first postoperative day. This was continued till the last follow-up visit. Post-PKP complications were punctate epithelial keratopathy, corneal allograft rejection and secondary glaucoma (one patient each). CONCLUSION: Satisfactory visual rehabilitation is possible after PKP following LT without compromising ocular surface stability. However, a prolonged and close follow-up is warranted to avert complications.

The past two decades have witnessed remarkable progress in limbal stem cell transplantation. In addition to harvesting stem cells from a cadaver or a live related donor, it is now possible to cultivate limbal stem cells in vitro and then transplant them onto the recipient bed. A clear understanding of the basic disease pathology and a correct assessment of the extent of stem cell deficiency are essential. A holistic approach towards management of limbal stem cell deficiency is needed. This also includes management of the underlying systemic disease, ocular adnexal pathology and dry eye. Conjunctival limbal autografts from the healthy contralateral eye are performed for unilateral cases. In bilateral cases, tissue may be harvested from a cadaver or a living related donor; prolonged immunosuppression is needed to avoid allograft rejection in such cases. This review describes the surgical techniques, postoperative treatment regimes (including immunosuppression for allografts), the complications and their management. The short and long-term outcomes of the various modalities reported in the literature are also described.

To report the pattern of noninfectious scleritis across a tertiary eye-care network. A three-year retrospective review of patients diagnosed with noninfectious scleritis was performed. Data were retrieved using diagnostic terms assigned to scleritis through the in-house eyeSmart-electronic medical record system. 1103 patients, with a mean age of 44.33 ± 14.38 years and a median follow-up of 199.5 days (range 32–685) were enrolled. Unilateral disease was noted in 85%. Diffuse anterior scleritis (n = 542, 42.51%) and nodular scleritis (n = 482, 38.12%) were the commonest subtypes. Systemic immune disease association was present in 65 (5.89%). Treatment at onset was topical corticosteroids (n = 372, 36.54%) followed by oral non-steroidal anti-inflammatory drugs (n = 351, 34.45%), oral corticosteroids in 184 (19.04%), and immunomodulators in 32 patients (3.54%). This study depicts the pattern of various noninfectious scleritis in a large cohort of patients. The present study helped to further customize the electronic medical records to minimize several data capture limitations. [ABSTRACT FROM AUTHOR]