Search

Since the introduction of the first toric intraocular lens (IOLs) in the early 1990s, these lenses have become the preferred choice for surgeons across the globe to correct corneal astigmatism during cataract surgery. These lenses allow patients to enjoy distortion-free distance vision with excellent outcomes. They also have their own set of challenges. Inappropriate keratometry measurement, underestimating the posterior corneal astigmatism, intraoperative IOL misalignment, postoperative rotation of these lenses, and IOL decentration after YAG-laser capsulotomy may result in residual cylindrical errors and poor uncorrected visual acuity resulting in patient dissatisfaction. This review provides a broad overview of a few important considerations, which include appropriate patient selection, precise biometry, understanding the design and science behind these lenses, knowledge of intraoperative surgical technique with emphasis on how to achieve proper alignment manually and with image-recognition devices, and successful management of postoperative complications. [ABSTRACT FROM AUTHOR]

CIPA has two pathognomonic features: the inability to feel pain and temperature, and decreased or absent sweating (anhidrosis). "So many of the males have killed themselves by their late 20s by doing ridiculously dangerous things, not restrained by pain" - Geoff Woods While pain is considered an unpleasant experience, intact normal physiological pain is a fundamental prerequisite for human existence. Congenital corneal anesthesia (CCA) is a rare, complex neurological condition frequently overlooked or under-diagnosed, and often delayed, leading to irreversible damage to the optical quality of the cornea. [Extracted from the article]

Purpose: Congenital corneal anesthesia (CCA) is a rare clinical entity that poses a diagnostic dilemma, particularly in the pediatric age group with very little literature on this. Accurate initial diagnosis, evaluation, early identification of risk factors, aggressive systemic workup, and appropriate therapy are paramount to prevent visual loss due to long-term complications of corneal anesthesia. The purpose of the study was to estimate and compare the corneal neural architecture using real time, in vivo confocal microscopy (IVCM) in patients with CCA as against a control population.Methods: This was a retrospective nonconsecutive, comparative clinical case series in a tertiary hospital in South India from June 2015 to December 2018.Methods: IVCM was accomplished in cooperative children in whom central cornea was relatively clear. The clearest three to five images from each eye were selected, and the nerves were analyzed for length, thickness, density, dichotomous pattern, and beading. Statistical analysis was done using Origin v7.0 (Origin Lab Corporation, Northampton, MA, USA).Results: In total, 15 eyes of 11 cases and 20 eyes of 10 controls were imaged. Measurements on corneal nerve density showed a significant difference (P = 0.0005), cases having a lower mean (3.85 ± 1.38 mm per mm2) compared to the controls (6.74 ± 1.75 mm per mm2). Measurements on corneal nerve length (P = 0.28), thickness (P = 0.45), and presence of beading (P = 0.97) and dichotomous pattern (P = 0.07) did not reveal a significant difference between cases and controls.Conclusion: There is a strong relationship between the functional loss (absent corneal sensation) and anatomical decrease (reduced subbasal nerve density) of corneal nerves in congenital corneal anaesthesia. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the clinico-microbiological profile, donor cornea risk factors, and outcomes of postkeratoplasty endophthalmitis at a tertiary care center.Methods: Retrospective analysis of charts of 28 consecutive patients (28 eyes) of acute endophthalmitis following either an endothelial keratoplasty (EK) or an optical penetrating keratoplasty (PK) surgery, performed between 2006 and 2018 (13-year period). Positive microbiology, identification and classification of predisposing factors, surveillance of utilized paired donors, treatment outcomes, and differences in the rate and severity of the event between optical penetrating and endothelial keratoplasty.Results: The estimated incidence of endophthalmitis was 0.23% in the entire cohort; it was 0.34% and 0.15%, after EK and PK, respectively (P = 0.049). The median time of endophthalmitis was 4.5 days postsurgery. Donor-related endophthalmitis was recognized in 7/28 (25%) eyes. Culture positivity was 68% (n = 19 of 28). Bacteria was isolated in 84% (n = 16 of 19) instances; Gram-negative bacilli were more common (87.5%; 14 of 16), and Pseudomonas species (50%; 7 of 14) was the most common Gram-negative bacterium. Majority (>75%) of the Gram-negative bacteria were resistant to all fluoroquinolones, aminoglycosides, 3rd generation cephalosporins, and meropenam; 1/3rd were resistant to imipenem; and 90% were sensitive to colistin. Treatment included intraocular antibiotic injections (96.4%), vitrectomy (42.9%), and therapeutic keratoplasty (50%). In 85.7% (24 of 28), globe was salvaged. The final vision was 20/200 or better in 39.1% (9 of 23) eyes.Conclusion: EK carried a higher risk of endophthalmitis than PK in this cohort. Bacterial infection was more common in this series, with Gram negative bacilli being the commonest organisms. Multidrug resistance was common (~75%) in Gram negative isolates. [ABSTRACT FROM AUTHOR]

Purpose: To describe the clinical and demographic profile of herpes zoster ophthalmicus (HZO) in patients presenting to a multitier ophthalmology hospital network in India. Methods: Cross-sectional hospital-based study included 3,004,470 new patients between August 2010 and October 2021. Patients with a clinical diagnosis of HZO in at least one eye were included. Data were collected using an electronic medical record system. Results: In total, 1,752 (0.058%) patients were diagnosed with HZO. Nearly two-thirds were male (63.76%) in the seventh decade of life (339;19.35% patients) with unilateral (98.34%) affliction. Higher prevalence was seen in patients from higher socioeconomic status (0.059%) and metropolitan geography (0.062%). Most common ocular signs included eyelid edema (44.19%), conjunctival congestion (65.69%), punctate keratopathy (23.36%), and anterior uveitis (21.22%). Of the 1,781 eyes, mild/ no visual impairment was seen in 952 (53.45%) eyes, moderate in 258 (14.49%) eyes, and severe to blindness in 363 (20.39%) eyes. Oral antivirals were started within 72 h (Group A) in 361 (20.61%) patients and after 72 h in 1391 (79.39%) patients (Group B). Significantly lesser severity of ocular involvement was noted in Group A (P < 0.00001). Surgical intervention was required in 211 (11.85%) eyes. Conclusion: HZO more commonly affects males in the seventh decade of life and is predominantly unilateral. It more commonly affects those from higher socioeconomic strata and metropolitan regions. Half of the eyes have mild or no visual impairment, while others have moderate to severe impairment. Institution of antivirals within 72 h is associated with less severe involvement. Surgical intervention is warranted in a tenth of the eyes. [ABSTRACT FROM AUTHOR]

Genomic testing may provide individuals and families with information on the nature, mode of inheritance, and how test results may help them make informed medical and personal decisions. This knowledge can ultimately inform translational discoveries with the potential for regenerative therapies.[[7]] Therefore, for an ophthalmologist, genetic testing should be as focused and ordered based on the specific phenotype and if testing will answer specific questions. [Extracted from the article]

Ligneous conjunctivitis (LC) is a rare form of pseudomembranous conjunctivitis seen in children, perhaps due to plasminogen deficiency, which manifest as a chronic refractory pseudomembranous conjunctivitis. LC cases are incapable in maintaining their fibrinolytic activity due to plasminogen deficiency; consequently, transudates of plasma assume as a thick, gelatinous, woody membranes over the mucosal surfaces. This is a short case report on a child with a LC, who presented with recurrent pseudomembranous conjunctivitis in conjunction with progressive congenital hydrocephalus due to aqueductal stenosis (Dandy-Walker syndrome). This rare association was clinically confirmed and prompt corrective surgical measures were instituted. [ABSTRACT FROM AUTHOR]

We describe a case of 55-year-old male farmer presented with recurrent corneal abrasions with a spastic entropion in the left eye. Superior cornea showed typical nummular opacities suggestive resolved herpetic eye diseases. On further enquiry, he had similar episodes in the past. Contralateral eye was essentially normal. Following the botulinum toxin injection for the management of spastic entropion, subject developed reactivation of herpetic necrotizing stromal keratitis. Diagnostic corneal scrapings were negative for herpes simplex virus-1 antigen by immunofluorescence assay and for DNA by molecular techniques. The case was successfully managed with topical steroids and antiviral medications. [ABSTRACT FROM AUTHOR]

Compared to adults, keratoconus in children progresses more rapidly and is usually more severe at the time of diagnosis; therefore, early detection and treatment are paramount to prevent serious vision impairment, affecting the child's social and educational development, thus negatively impacting their quality of life. Therefore, as an option, CXL must be offered to every child presenting with progressive keratoconus so that an attempt at early treatment can be made. [Extracted from the article]

Objective: To report the outcomes of ipsilateral rotational autokeratoplasty (RAK) for nonprogressive paracentral corneal opacities in children <16 years of age. Design: Retrospective, consecutive, interventional case series. Participants: Thirty-three eyes of 33 children aged <16 years undergoing RAK for nonprogressive paracentral scars. Methods: Medical records were retrospectively reviewed for the primary etiology of corneal opacity, time of onset, duration of opacity, preoperative visual acuity, formula used for calculation of trephine size, size of the trephine used, and duration of follow-up. Any intraoperative and early and late postoperative complications were noted for all patients. Postoperative visual acuity and astigmatism were noted. Visual acuity was converted to logarithm of the minimum angle of resolution units for analysis. Main Outcome Measures: Primary outcome was postoperative visual acuity. Graft clarity and complications were analyzed as secondary outcomes. Results: The mean age at surgery was 7.2±3.9 months. The mean follow-up duration was 27±37 months. The commonest etiology of corneal opacity was trauma (62.5%), followed by resolved microbial keratitis (21.9%). Postoperative visual acuity (1.25±0.84) was significantly better (P<0.001) than preoperative visual acuity (2.05±0.96). The mean astigmatism at last visit was 4.04±2.21 diopters. Postoperative visual acuity was better in older children (β = −0.01; P = 0.03) and had a shorter delay in presentation (β = 0.02; P = 0.05). At the last follow-up, the graft was clear in 27 cases (81.25%). The cumulative probability of graft survival was 85% at 2 years and 65% at 5 years. Complications included wound leak in 4 eyes, secondary glaucoma in 2 eyes, graft infiltrate and traumatic dehiscence in 1 eye each. Conclusions: Rotational is a autokeratoplasty viable alternative surgical option to allogenic keratoplasty. Graft survival at 2 years seems to be better than allogenic keratoplasty. Younger age and delay in presentation contribute to poorer visual outcomes after surgery. Financial Disclosure(s): The authors have no proprietary or commercial interest in any of the materials discussed in this article. [Copyright &y& Elsevier]

Purpose: To analyze the outcomes of post-cataract surgery descemetopexy for Descemet membrane detachment using intracameral air injection. Setting: Cornea Department, LV Prasad Eye Institute, Hyderabad, India. Design: Interventional case series. Methods: The clinical data of patients who had descemetopexy from August 2010 to February 2011 for Descemet membrane detachment after cataract surgery using intracameral (100%) air injection were reviewed after institutional review board and ethics committee approval was obtained. Results: Of the 14 patients, 9 had manual small-incision cataract surgery using the Blumenthal and Moisseiev technique, 4 had phacoemulsification, and 1 had combined phacoemulsification with trabeculectomy. The mean duration between cataract surgery and descemetopexy was 19.5 days (range 2 to 49 days). Successful reattachment of Descemet membrane and resolution of corneal edema occurred in all except 1 patient. Two patients had elevated intraocular pressure 1 day postoperatively due to appositional angle closure in 1 and pupillary block in the other. Of the 13 patients with successful Descemet membrane reattachment, the corrected distance visual acuity was 20/40 or better in 11 cases, 20/80 in 1 case, and 20/160 in 1 case. Three of the 13 patients had comorbidity factors affecting vision. Conclusion: Air descemetopexy was a safe option in the management of Descemet detachment after cataract surgery. Financial Disclosure: No author has a financial or proprietary interest in any material or method mentioned. [ABSTRACT FROM AUTHOR]

Aims To compare astigmatism with clear corneal incision versus scleral tunnel incision following paediatric cataract surgery with primary intraocular lens implantation. Methods Retrospective, comparative case series of 218 eyes of 138 children <12 years of age undergoing cataract extraction with intraocular lens implantation. The study cohorts were grouped into two categories based on incision location: group 1 comprising 108 eyes from 65 children with scleral incision; group 2 comprising 110 eyes from 73 children with clear corneal incision. Cycloplegic refraction was performed at 1, 3 and 6 months postoperatively. Results The mean age in group 1 was 61±39 months and in group 2, 51±41 months. The mean postoperative astigmatism in group 1 was 1.28±0.97 D, 1.42±1.00 D and 1.38±0.98 D at 1, 3 and 6 months respectively. The change in astigmatism between 1 and 6 months was non-significant (p=0.26). The mean astigmatism in group 2 was 1.34±1.20 D, 1.13±0.88 D and 1.03±0.89 D at 1, 3 and 6 months respectively. Astigmatism in group 2 decreased significantly with time (p<0.001). The amount of astigmatism was comparable between the two groups at 1 month postsurgery (p=0.90), while it was significantly lower in the corneal incision group at 3 (p=0.03) and 6 months (p=0.01). Conclusions Postoperative astigmatism after paediatric cataract surgery by clear corneal incision was lower compared with scleral incision. However, the difference was small and clinically insignificant. [ABSTRACT FROM AUTHOR]

Objective: To report the investigation for the source of infection and the clinical course and treatment response of 11 cases of acute post-cataract surgery endophthalmitis that developed during an outbreak. Design: Retrospective, consecutive, interventional case series. Participants: Eleven patients who developed acute postoperative endophthalmitis after an uneventful cataract surgery with intraocular lens implantation from September 6 to 29, 2010, at a tertiary eye care center in South India. Methods: Aqueous aspirates, vitreous aspirates, and environmental surveillance specimens were sampled. All specimens were subjected to smear and culture. Positive cultures were subjected to antibiotic susceptibility. Genotypic diversity was determined by polymerase chain reaction (PCR) with enterobacterial repetitive intergenic consensus (ERIC) primers of each strain and was used to establish the clonal relationship between clinical and environmental isolates. The clinical patterns were analyzed. Main Outcome Measures: Positive microbiology, molecular diagnostic similarity among the culture positive endophthalmitis cases, and surveillance specimens. Results: Aqueous and vitreous samples showed gram-negative bacilli in the smears of 8 of 11 eyes, and cultures grew Pseudomonas aeruginosa in 5 of 11 eyes. Among the samples from various surveillance specimens cultured, only the hydrophilic acrylic intraocular lenses and their solution grew P. aeruginosa, with antibiotic susceptibility pattern identical to the clinical isolates. The isolates from the patients and the intraocular lens solution revealed matching patterns similar to an American Type Culture Collection (ATCC) strain of P. aeruginosa on ERIC-PCR. The intraocular lenses of the same make were discontinued at our hospital, and the endophthalmitis did not recur. The final visual acuity improved to ≥20/50 in 8 of 11 patients (72.7%). One patient developed retinal detachment, but was treated successfully, and 2 other patients progressed to phthisis bulbi. Conclusions: Positive microbiology and the ERIC-PCR results proved that contamination of hydrophilic intraocular lenses and the preservative solution was the source of infection in this outbreak. Early detection and a planned approach during the outbreak helped us to achieve good visual and anatomic outcomes, even though the offending organism was identified as P. aeruginosa. Financial Disclosure(s): The authors have no proprietary or commercial interest in any materials discussed in this article. [Copyright &y& Elsevier]

The ocular features of Goldenhar syndrome (GS) are typically external, like surface dermoids and lid coloboma. Retinal detachment (RD) is rare and has not been reported in absence of other concomitant predisposing congenital ocular disorders. We present a unique case of bilateral rhegmatogenous retinal detachment (RRD) with GS. To the best of our knowledge, this association of GS with RRD is novel and has not been reported earlier in ophthalmic and systemic literature on RRD. [ABSTRACT FROM AUTHOR]

Corneal endothelial dystrophies are a heterogeneous group of diseases with different modes of inheritance and genetic basis for each dystrophy. The genes associated with these diseases encode transcription factors, structural components of the stroma and Descemet membrane, cell transport proteins, and others. Congenital hereditary endothelial dystrophy (CHED) is associated with mutations in two genes, OVOL2 and SLC4A11, for dominant and recessive forms of CHED, respectively. Mutations in three genes are known to cause posterior polymorphous corneal dystrophy (PPCD). They are OVOL2 (PPCD1), ZEB1 (PPCD3), and GRHL1 (PPCD4). The PPCD2 locus involving the collagen gene COL8A2 on chromosome 1 is disputed due to insufficient evidence. Mutations in the COL8A2 gene are associated with early-onset Fuchs' endothelial corneal dystrophy (FECD). Several genes have been associated with the more common, late-onset FECD. Alterations in each of these genes occur in a fraction of patients, and the most prevalent genetic alteration in FECD patients across the world is a triplet repeat expansion in the TCF4 gene. Knowledge of the genetics of corneal endothelial dystrophies has considerably advanced within the last decade and has contributed to better diagnosis of these dystrophies as well as opened up the possibility of novel therapeutic approaches based on the molecular mechanisms involved. The functions of genes identified to date provide insights into the pathogenic mechanisms involved in each disorder. [ABSTRACT FROM AUTHOR]

Purpose To report the surgical outcomes of Descemet's stripping endothelial keratoplasty (DSEK) in cases of congenital hereditary endothelial dystrophy (CHED) cohort and compare it with penetrating keratoplasty (PK) in a paired-eye setting. Methods Ours is a retrospective, comparative, consecutive and interventional clinical case series. All patients less than 14 years of age who underwent PK in one eye and DSEK in the contralateral eye at a single centre from January 2006 and February 2011 were analysed. Main outcome measures were graft clarity, visual outcome and complications. Results The mean age of the patients was 6.6±2.19 years at the time of presentation. The outcomes of two surgeries were compared with 1 year of follow-up at the corresponding follow time. At 1 year, all grafts were clear. There was no significant difference in the spherical component of the refraction; the astigmatism was significantly lower after EK. The refraction stabilised in patients with EK as early as 3 months, while it continued to change up to 1 year after PK. Complications included graft dislocation in two cases of DSEK, which were managed by rebubbling, and a graft dehiscence in one case of PK, which was managed by resuturing. The final visual acuity improved in all the patients. Conclusions Endothelial keratoplasty is a viable option to conventional PK in cases of CHED. It offers an advantage of early visual stabilisation compared with PK. [ABSTRACT FROM AUTHOR]

Management of endothelial dysfunction in phakic patients is sometimes a dilemma for corneal surgeons. Phakic patients with visually significant cataract and endothelial dysfunction are preferably managed by performing combined cataract surgery with endothelial keratoplasty. However, combined surgery may be deferred in eyes with early incipient cataract, younger age and where anterior chamber is poorly visualized. As cataract formation may be accelerated after endothelial keratoplasty, these eyes may need cataract surgery subsequently. Surgical intervention in eyes with endothelial keratoplasty is of concern as this may affect the graft adversely and threaten graft survival. In this report, we describe the intraoperative surgical details and postoperative clinical course of a patient who underwent phacoemulsification with intraocular lens implantation after Descemet stripping automated endothelial keratoplasty (DSAEK). [ABSTRACT FROM AUTHOR]

The article presents a case study of a six-year-old female patient who was presented with redness and eyelid swelling linked with a mucopurulent discharge in the left eye, which started after taking a bath in river water. Information about a microsporidial infestation, such as punctate epithelial keratoconjunctivitis, is presented.

Macular corneal dystrophy is autosomal recessive dystrophy characterized by deposits of abnormal glycosaminoglycans in stromal lamellae and within endothelial cells. Deep anterior lamellar keratoplasty is successful in the management of this dystrophy. We herein describe three cases of primary graft failure after uneventful big bubble deep anterior lamellar keratoplasty for macular corneal dystrophy. [ABSTRACT FROM AUTHOR]

Optical coherence tomography (OCT) is analogous to ultrasound biometry in the cross sectional imaging of ocular tissues. Development of current devices with deeper penetration and higher resolution has made it popular tool in clinics for visualization of anterior segment structures. In this review, the authors discussed the application of AS-OCT for diagnosis and management of various corneal and ocular surface disorders. Further, recent developments in the application of the device for pediatric corneal disorders and extending the application of OCT angiography for anterior segment are introduced. [ABSTRACT FROM AUTHOR]

The article discusses the case of a patient who presented with deteriorating vision 23 years after undergoing radial keratotomy to treat her myopia and mentions topics including the complications of radial keratotomy performed in the 1980s and 1990s and new prescription for corrective lenses given to the patient.

Background: Cystinosis, a rare autosomal recessive disease, stems from genetic alterations in the CTNS gene, leading to a malfunction of lysosomal 'cystinosin' protein. This dysfunction causes intracellular cystine accumulation, resulting in nephropathic and ocular abnormalities. Cystinosis is relatively rare in Asian countries, partly due to underreporting and lack of awareness, and cases often lack sufficient genetic evidence to support their diagnosis. This study presents a descriptive case series involving four Indian patients with cystinosis, elucidating clinical and genetic aspects. Methods: All four patients underwent comprehensive ophthalmic evaluations. The corneal cystine crystal (CCC) score was determined using anterior segment optical coherence tomography (AS-OCT) and in vivo confocal microscopy (IVCM). Genetic testing was performed using whole exome sequencing (WES). Results: Corneal crystal deposition, a hallmark of cystinosis, was evident in all cases. Systemic analysis revealed manifestations such as polyuria, bony abnormalities, growth retardation, hypothyroidism, and developmental delay. Genetic testing in two patients identified a homozygous pathogenic variant c.18_21delGACT (p.Thr7PhefsX7) in the CTNS gene, previously reported to cause cystinosis in different ethnic populations. Conclusions: Our case series sheds light on underrepresented cases of cystinosis in the Indian population. The rarity of this condition poses diagnostic challenges, leading to delayed or inaccurate diagnoses. AS-OCT can serve as a viable alternative to IVCM for assessing corneal crystal density status in cystinosis. Timely recognition and management are crucial in preventing complications, and the inclusion of genetic testing can expedite cystinosis diagnosis. [ABSTRACT FROM AUTHOR]

Fanconi's syndrome (FS) is characterized by type-2 renal tubular acidosis, short stature, and renal rickets, along with glycosuria, aminoaciduria, hypophosphaturia, and urinary bicarbonate wasting. The genetic form of FS has been linked to HNF4A variants. Although additional clinical features such as hearing impairment have recently been associated with HNF4A-linked FS, its ocular manifestation has not been described. Presenting a case of a 5-year-old male child with bilateral progressive corneal opacification and the presence of bilateral greyish-white deposits in the interpalpebral region since infancy. A next-generation sequencing (NGS)-based genetic testing was performed for the child followed by parental genetic testing for the identified variant. Furthermore, relevant works of literature were reviewed related to this condition. Detailed corneal findings showed a bilateral band-shaped keratopathy (BSK) in the patient. Physical and systemic findings showed signs consistent with FS. Sequencing analysis revealed a novel heterozygous c.635C>T, (p.Pro212Leu) variant in the HNF4A gene in the proband and mother, while the father had a normal genotype. Our case highlights the occurrence of BSK in an exceptionally rare manifestation of hereditary FS linked to HNF4A gene variant. The variant exists both in proband and asymptomatic mother. Therefore, the variable penetrance which is known to exist in HNF4A is acknowledged in this context. This report suggests the first documented instance establishing a plausible connection between BSK and HNF4A-associated FS, characterized by the variable penetrance attributed to the HNF4A gene. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the outcomes of keratoplasty for xeroderma pigmentosum (XP) performed at a tertiary eye care center.Methods: A retrospective review of medical records of those patients who were clinically diagnosed to have XP (54 eyes of 36 patients) and underwent keratoplasty; either deep anterior lamellar keratoplasty (DALK, four eyes), endothelial keratoplasty (EK, eight eyes), or penetrating keratoplasty (PK, 42 eyes) from 1994 to 2018.Results: The median age at surgery was 20.6 years (interquartile range [IQR], 14.6-27.6 years) and 20 (55.6%) were males. Graft failure occurred in 15 eyes (35.7%) in the PK group and two eyes (50%) in the DALK group; none failed in the EK group. The probability of graft survival in the PK group was 97.2% ± 2.7% at 1 year, 74.0% ± 8.0% at 2 years, and 54.8% ± 11.7% at 5 years. In the PK group, 13 eyes needed antiglaucoma medications, 11 eyes developed graft infiltrate, and 13 eyes needed secondary interventions (cataract surgery, excision biopsy, and tarsorrhaphy). In the EK group, three eyes needed secondary interventions (excision biopsy). Median postoperative endothelial cell density at the last follow-up in the PK group was 1214 cells/mm2 (IQR, 623-2277 cells/mm2).Conclusion: Despite the complexities of the ocular surface and adnexal issues in XP, keratoplasty had reasonably good outcomes. More than half of the PK grafts survived 5 years with no failures in the EK group. Regular follow-up and timely management of suture-related infections raised intraocular pressure, and suspicious ocular surface lesions, in addition to solar protection, are important for the success of keratoplasty in these eyes. [ABSTRACT FROM AUTHOR]

Purpose: To report the clinicopathological features of corneal buttons in patients with congenital primary aphakia.Methods: Five corneal specimens of five patients with congenital primary aphakia who underwent penetrating keratoplasty (PKP) were studied by light microscopy, and immunohistochemistry with anti-smooth muscle (SMA) antibody.Results: All patients were born from consanguineous parents. Of the five, two patients were identical twins. All eyes were microphthalmic. In four patients, congenital primary aphakia was bilateral and in one patient (Patient 3), it was unilateral. PKP failed in all eyes due to hypotony. Histologically, Bowman's layer was absent in all specimens. The corneal stroma was thin; however, the stromal collagen showed thick and irregularly arranged fibers with neovascularization in all eyes. Descemet's membrane and the corneal endothelium were absent in all specimens. In three specimens, atrophic iris tissue without dilator muscle was adherent to the posterior corneal surface. In addition, anteriorly displaced hypoplastic ciliary body and/or pigmented and non-pigmented ciliary epithelium were attached to the posterior corneal surface in three of the five specimens. SMA staining demonstrated disorganized ciliary muscle in one case. SMA-positive stromal keratocytes demonstrated their myofibroblast nature.Conclusion: The corneal findings in congenital primary aphakia are similar to that seen in other causes of congenital corneal opacification. The anteriorly displaced hypoplastic ciliary body that was partially excised during keratoplasty explains the ocular hypotony in these eyes. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this study is to evaluate the outcomes of keratoplasty for lattice corneal dystrophy (LCD) performed at a tertiary eye care center.Methods: A retrospective review of medical records of those patients who were clinically diagnosed to have LCD (72 eyes of 57 patients) and underwent either penetrating keratoplasty (PK, 58 eyes of 46 patients) or deep anterior lamellar keratoplasty (DALK, 14 eyes of 13 patients) between the years 1987 and 2014 was performed. The main outcome measures included demographics, clinical features, and outcomes of keratoplasty.Results: The median follow-up after keratoplasty was 3.1 years (interquartile range [IQR], 9 months to 9 years). The median best-corrected visual acuity (BCVA) was 0.18 (IQR, 0.10-0.48) (Snellen equivalent 20/30 [IQR, 20/25-20/60]) at 4 years postoperatively and 0.65 (IQR, 0.18-0.95) (Snellen equivalent 20/89 [IQR, 20/30-20/178]) at 10 years following surgery. DALK eyes had a significantly better BCVA than PK eyes at 2 years following keratoplasty. The median overall survival of grafts was 15.8 years. Late complications included recurrence of LCD (14 eyes), graft infiltrate (23 eyes), graft rejection (15 eyes), graft failure (16 eyes), and glaucoma (14 eyes).Conclusion: The outcomes of graft are similar following PK and deep anterior lamellar keratoplasty; however, the latter appears to provide slightly better visual outcome. Recurrence of dystrophy in the graft and graft infiltrates limit the overall graft survival in both the groups. [ABSTRACT FROM AUTHOR]

A 33-year-old male underwent an optical keratoplasty elsewhere in the right eye following which he developed endophthalmitis and subsequently underwent a pars plana vitrectomy and lensectomy. At presentation, he had a deep stromal crystalline infiltration along the graft-host junction. A large therapeutic keratoplasty was performed, and the excised corneal button was evaluated. Histopathology revealed gram-positive round-to-oval budding structures and microbiology identified the organism as Candida glabrata. He was treated with antifungals in the postoperative period. At 4 months after therapeutic keratoplasty, the patient developed recurrent endophthalmitis, following stoppage of antifungals. The treatment was reinstituted for another year, and the patient did well with a clear graft at 18-month-follow-up period after the recurrence episode. Management of infectious crystalline keratopathy with endophthalmitis is a challenging situation and requires long-term treatment. [ABSTRACT FROM AUTHOR]

Amniotic membrane transplantation is a useful in the management of corneal melts and descemetocele. We describe high-resolution anterior segment optical coherence tomography (OCT) (Optovue) in a patient with descemetocele who was managed surgically with amniotic membrane transplantation. A 60-year-old female presented with a corneal melt in the right eye. She was a case of rheumatoid arthritis and was on systemic treatment with immunomodulators. Slit lamp examination revealed a severe thinning paracentrally. High-resolution OCT was performed at the site of descemetocele. She underwent amniotic membrane transplantation using fibrin glue and bandage contact lens application. At 6 weeks postoperative, the bandage contact lens was removed. The area of thinning healed with scarring. OCT at the healed site revealed stable surface and an increase in stromal thickness to 281 μ this case describes the utility of amniotic membrane in the healing of sterile corneal melts by providing tectonic support and its integration in the stroma. The stromal healing and increased thickness at the site of descemetocele could be delineated on high-resolution OCT imaging. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Congenital hereditary endothelial dystrophy (CHED) is a rare form of corneal dystrophy caused by SLC4A11 gene variations. This study aims to find the genetic alterations in SLC4A11, in two Indian familial CHED cases with affected members n = 3 and n = 2 respectively and five sporadic CHED cases using direct sequencing, followed by in silico analysis and characterization of the identified variants.Results: All three affected members of the first CHED family were identified with a novel homozygous c.1514C > G (p.Ser489Trp) variation while second family showed presence of a compound heterozygous variation c.529A > C (p.Arg161Arg) + c.2461insT (p.Val805fs). Among five sporadic cases, two showed novel changes, homozygous c.1487G > T (p.Ser480Ile) and c.620-2A > G, while the other one had previously reported homozygous c.2653C > T (p.Arg869Cys) variation. The remaining two cases did not reveal the presence of SLC4A11-related pathogenic variations. The identified variations were excluded from the Indian control (n = 80). In silico analysis using homology-based protein modeling and pathogenicity prediction tools, which revealed these alterations as pathogenic, changing their protein stability, local flexibility, residue contact clashes, and the hydrogen bond interactions.Conclusions: This study contributed to the CHED mutational spectrum, adding four novel variations and confirming a previously reported one. It demonstrates different type of variations in CHED cases, including coding, non-coding, homozygous, synonymous, and compound heterozygous variations. The identified variations revealed different degrees of pathogenic effects in silico. Moreover, two sporadic cases could not be identified with pathogenic variation emphasizing the involvement of other genes or genetic mechanisms. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

. Purpose: To report the results of Descemet's stripping endothelial keratoplasty (DSEK) for failed therapeutic penetrating keratoplasty (PK). Methods: Twenty-seven eyes of 27 patients undergoing DSEK for failed therapeutic PK were analysed. Results: The mean age of the patients was 36 ± 13.9 years (range: 14-70 years). The median size of the therapeutic graft was 10 mm (inter-quartile range; IQR 9.5-11 mm). Descemet's membrane stripping was performed in all eyes. Graft clarity was achieved in 20/27 eyes. Six eyes had primary graft failure, and one had interface keratitis in the early postoperative period. In all eyes with primary graft failure, there was progressive stromal vascularization, which led to haziness in the graft. Late postoperative complications were rejection in four eyes, infection in two eyes, secondary graft failure in one eye and vortex keratopathy in one eye. Graft size was found to be a significant risk factor for graft rejection in this series. At the last follow-up visit, the best-corrected visual acuity was ≥20/40 in 5/27 eyes (18.5%), 20/60-20/40 in 12/27 eyes (44.4%), 20/100-20/60 in 3/27 eyes (1.5%) and ≤20/200 in 7/27 eyes (25.9%). Conclusions: Considering the limited success of repeat PK in failed large therapeutic keratoplasty, DSEK is a viable option for visual rehabilitation in these eyes, however; visual acuity may be limited due to sub-epithelial/stromal or interface scarring. [ABSTRACT FROM AUTHOR]

We present a comprehensive review of existing literature on surgical corneal neurotization (SCN) as a treatment modality for neurotrophic keratopathy (NK) with an interim report of seven cases where SCN was performed using the indirect approach and followed up till 18 months postoperatively to look for improvement in ocular surface, corneal sensations, and nerve regeneration by using in vivo confocal microscopy (IVCM). A literature search was performed for publications with keywords "corneal nerves," "neurotization," "esthesiometry," "corneal anesthesia," and "neurotrophic keratopathy." All literature available till December 31, 2020 was reviewed and included to describe NK and its management options, particularly SCN. NK is associated with absent or reduced corneal sensations and is managed using a step-ladder algorithm ranging from medical management for symptomatic relief to surgical corneal neurotization. Both direct and indirect approaches of SCN have a favorable outcome with reduced surgical morbidity in the indirect approach using sural nerve graft. Post neurotization, corneal sensation recovery may take up to 3-6 months, while nerve regeneration on confocal microscopy can take as long as 6 months-1 year. [ABSTRACT FROM AUTHOR]

PURPOSE: To evaluate the outcomes of deep anterior lamellar keratoplasty (DALK) in children. DESIGN: Retrospective interventional case series. METHODS: SETTING: Institutional, L.V. Prasad Eye Institute, a tertiary care center in south India. STUDY POPULATION AND INTERVENTION: All children less than 16 years of age undergoing DALK from January 2003 to January 2011. MAIN OUTCOME MEASURE: Visual outcome and complications. RESULTS: Twenty-six eyes of 26 children (13 male and 13 female) with a mean age of 7.82 ± 4.64 years underwent DALK for keratoconus (8), microbial keratitis (6), corneal scar (6), corneal keloid (3), chemical injury with limbal stem cell deficiency (2), and dermoid (1). Big bubble was achieved in 5 eyes, while manual dissection was done in 21. Follow-up ranged from 1 week to 7.3 years. Seventeen patients with a minimal follow-up of 6 months were evaluated for visual outcomes. Final vision varied from counting fingers to 20/20 (mean sphere 2.32 diopters, mean cylinder -2.5 diopters). Complications encountered were suture-related graft infiltrate (3), graft dehiscence (3), and Descemet membrane detachment (2). CONCLUSIONS: DALK is a feasible option in children with stromal corneal pathology. It offers advantages in the form of lower risk of graft rejection. However, the risk of complications such as suture-related infections and graft dehiscence persists even in these cases. [ABSTRACT FROM AUTHOR]

Purpose To review the clinical profile of graft detachment following Descemet's stripping endothelial keratoplasty (DSEK) and analyse the outcomes of rebubbling for graft detachment. Methods Retrospective review. Results 27 of a total of 309 eyes that underwent DSEK (8.7%) underwent rebubbling with air injection between January 2009 and February 2010. Eighteen (66.6%) of these eyes had complete and 9 (33.3%) had partial detachment of the transplanted lenticule. Successful attachment was observed in 20/27 (74%) eyes (12/18 with total and 8/9 with partial detachments), and clear grafts were achieved in 13 (65%) of these 20 eyes, while 7 (35%) eyes had primary graft failure. Visual acuity was 20/60 or better in 6 of these 20 eyes (35%), between 20/100 and 20/60 in 4 eyes (25%), and <20/200 in 10 eyes. Three of the 10 lenticules that failed to attach with the first rebubbling procedure underwent a second attempt of rebubbling, four underwent a repeat DSEK with good outcomes, and three underwent PK. Univariate analysis showed additional vitrectomy at the time of DSEK to be a statistically significant risk factor for persistent graft detachment following rebubbling (p<0.04). Conclusion Rebubbling following detachment of the donor lenticule was successful in achieving graft adhesion in the majority of patients (74%), but 35% had a primary graft failure. The second attempt at rebubbling (in three patients) led to successful attachment, but the oedema failed to recover. Those who underwent repeat DSEK for unsuccessful rebubbling did well with no complications. [ABSTRACT FROM AUTHOR]

Purpose: This study is aimed to evaluate the role of 14% nonexpansile concentration of perfluoropropane (C3F8) gas in the management of acute hydrops in corneal ectasias. Design: Retrospective, nonrandomized, comparative, interventional case series. Participants and Controls: The study group consisted of 62 eyes of 57 patients and the control group included 90 eyes of 82 patients with acute corneal hydrops who presented within 30 days of onset of symptoms. Intervention: Patients in the control group underwent a single intracameral injection of 0.1 mL of nonexpansile concentration (14%) of C3F8 gas. Patients in the control group were treated conservatively. Patients in both groups were followed regularly for 12.6±7.7 and 13.4±8.3 months in the study and control groups, respectively, and assessed clinically for complete disappearance of epithelial and stromal edema on slit-lamp biomicroscopy. Main Outcome Measures: The primary outcome measure was mean time to resolution of corneal edema, which was calculated both from the date of onset of hydrops and the date of initiation of therapy to the date of resolution in days. Results: The overall time to resolution both from the date of onset of symptoms (90.5±55.8 vs 125±68.9 days; P = 0.0005) and from the date of initiation of therapy (78.7±53.2 vs 117.9±68.2 days; P = 0.0001) was significantly lower in the study group compared with the control group. However, on subgroup analysis a significant difference in the resolution time was found only in eyes with keratoconus (P<0.0001). No difference in the resolution time was seen in eyes with pellucid marginal corneal degeneration (PMCD) or keratoglobus. The main complication of this procedure was reversible pupillary block (16%; P<0.0001). There was no difference in the final visual acuity or endothelial cell counts between the 2 groups. Conclusions: Intracameral C3F8 gas in a nonexpansile concentration is a useful modality for faster resolution of corneal edema in patients with acute corneal hydrops and keratoconus, and its role in the treatment of PMCD and keratoglobus needs further evaluation. Financial Disclosure(s): The authors have no proprietary or commercial interest in any materials discussed in this article. [Copyright &y& Elsevier]

Gold standard is GALT activity measurement in red blood cells.[[2]] In our case, the typical appearance of cataract with history of chronic diarrhea prompted the diagnosis and was confirmed by cataractous lens protein analysis. Galactose peak was observed at 21.16 min in both sample and galactose solution [Figure 2] establishing the diagnosis of galactosemia.{Figure 2} In conclusion, diagnosis of galactosemia can be established by cataractous lens protein analysis where other investigations are not possible. [Extracted from the article]