Evaluation of adjuvant role of topical cyclosporine 1% in acute Stevens-Johnson syndrome: a randomised control trial.

PURPOSE: To evaluate the role of topical cyclosporine A 1% (CsA) as an adjuvant therapy in patients with acute Stevens-Johnson syndrome (SJS). METHODS: This is a randomised controlled trial in which 44 patients (88 eyes) with acute SJS, presenting within 3 months from the onset of the disease, were enrolled and randomised. Group A (n=44 eyes) patients received treatment with topical CsA 1% along with standard therapy consisting of topical corticosteroids, antibiotics and lubricants. Group B (n=44 eyes) patients received topical saline drops in combination with standard therapy. Various ocular surface parameters were assessed at baseline and the 6-month follow-up. RESULTS: The mean age of patients (years) was 23.9±15.1 in the CsA group and 26.0±18.7 in the control group (p=0.6840). The mean time from disease onset to presentation (days) was 17.0±14.0 and 12.9±11.3 in CsA and control groups, respectively (p=0.1568). At presentation, the mean grades of severity scores of various parameters were comparable. At 6 months, both groups showed a significant improvement in the mean severity grades of conjunctival hyperaemia (A, p=0.001; B, p=0.0001), mucocutaneous junction involvement (A, p=0.001; B, p=0.0001) and meibomian gland involvement (A, p=0.0471; B, p=0.006). Compared with baseline, the grades of corneal keratinisation (baseline, 0.48±0.7; 6 months, 1.02±0.8; p=0.0015) and neovascularisation (baseline, 1.07±1.2; 6 months, 1.57±1.0; p=0.0412) worsened after 6 months of CsA therapy. Intergroup comparison of grades of various parameters however did not reveal any significant difference at 6 months. CONCLUSIONS: Adjuvant treatment with topical CsA is not superior to standard therapy, in cases of acute SJS.

Comparative evaluation of biomechanical changes and aberration profile following accelerated collagen cross-linking using hypo-osmolar and iso-osmolar riboflavin: A prospective study.

PURPOSE: To compare the changes encountered in corneal biomechanics and aberration profile following accelerated corneal collagen cross-linking (CXL) using hypo-osmolar and iso-osmolar riboflavin in corneal thicknesses of <400 and >400 microns, respectively. METHODS: This is a prospective, interventional, comparative study involving 100 eyes of 75 patients with progressive keratoconus. Eyes were divided into two groups based on corneal thickness: group 1 included eyes with a corneal thickness of <400 microns who underwent hypo-osmolar CXL, and group 2 included eyes with a corneal thickness of >400 microns who underwent iso-osmolar CXL. Corneal biomechanical and aberration profiles were evaluated and compared between groups. RESULTS: In group 1, all higher-order aberrations (HOA) except secondary astigmatism significantly decreased from baseline; however, in group 2, only coma and trefoil decreased. The corneal resistance factor and corneal hysteresis significantly improved in both groups, which was significantly greater in group 2 than in group 1. The change in inverse radius, deformation amplitude, and tomographic biomechanical index was significantly improved in group 2 as compared to group 1. CONCLUSION: Improvement in corrected distance visual acuity and decrease in HOA were significantly better in the hypo-osmolar CXL group; however, the improvement in biomechanical strength of the cornea was significantly better in the iso-osmolar group.

Management and prevention of corneal graft rejection.

The management of an episode of corneal graft rejection (CGR) is primarily by corticosteroids. Immunomodulators are useful for long-term immunosuppression and in dealing with cases of high-risk (HR) corneal grafts. The classical signs of CGR following penetrating keratoplasty (PKP) include rejection line, anterior chamber (AC) reaction, and graft edema. However, these signs may be absent or subtle in cases of endothelial keratoplasty (EK). Prevention of an episode of graft rejection is of utmost importance as it can reduce the need for donor cornea significantly. In our previous article (IJO_2866_22), we had discussed about the immunopathogenesis of CGR. In this review article, we aim to discuss the various clinical aspects and management of CGR.

Outcomes of preloaded toric intraocular lens implantation in eyes undergoing phacoemulsification.

Purpose: To evaluate the clinical outcomes of preloaded toric intraocular lens (IOLs) implantation in eyes undergoing phacoemulsification. Methods: This prospective study included 51 eyes of 51 patients with visually significant cataracts and corneal astigmatism ranging between 0.75 and 5.50 D. All patients underwent phacoemulsification with SupraPhob toric intraocular lens implantation under topical anesthesia. The main outcome measures were uncorrected distance visual acuity (UDVA), residual refractive cylinder, spherical equivalent, and IOL stability at 3 months follow-up. Results: At 3 months, 49% (25/51) of patients had UDVA equal to or better than 20/25 with 100% of eyes achieving better than 20/40. Mean logMAR UDVA improved from 1.02 ± 0.39, preoperatively to 0.11 ± 0.10 at 3 months follow-up (P < 0.001, Wilcoxon signed-rank test). The mean refractive cylinder improved from - 1.56 ± 1.25 D preoperatively to - 0.12 ± 0.31 D at 3 months follow-up (P < 0.001) while the mean spherical equivalent value changed from - 1.93 ± 3.71D preoperatively to - 0.16 ± 0.27D (P = 0.0013). The mean root mean square value for higher order aberrations was 0.30 ± 0.18 µm while the average contrast sensitivity value (Pelli-Robson chart) was 1.56 ± 0.10 log unit, at the final follow-up. The mean IOL rotation at 3 weeks was 1.7 ± 1.61 degrees, which did not change significantly at 3 months (P = 0.988) follow-up. There were no intraoperative or postoperative complications. Conclusion: SupraPhob toric IOL implantation is an effective method for addressing preexisting corneal astigmatism in eyes undergoing phacoemulsification with good rotational stability.

Immunopathogenesis of corneal graft rejection.

The most common cause of corneal graft failure is corneal graft rejection (CGR). Although cornea is one of the immune-privileged sites, it can still get a rejection episode due to a breach in its natural protective mechanism. Both anatomical and structural properties of cornea and anterior chamber contribute toward its immune tolerance. Clinically, every layer of the transplanted cornea can get a rejection episode. A proper understanding of immunopathogenesis will help in understanding the various mechanism of CGR and the development of newer strategies for the prevention and management of such cases.

Cataract surgery outcomes in pediatric patients with systemic comorbidities.

Purpose: The aim of the study was to evaluate the outcomes of cataract surgery in patients of the pediatric age group with systemic comorbidities. Methods: Medical records of 54 eyes (30 patients) of the pediatric age group with systemic comorbidities who had undergone cataract surgery in a tertiary-care center were reviewed. The following parameters were recorded: systemic comorbidity; toxoplasmosis, rubella, cytomegalovirus, herpes simplex, HIV (TORCH) profile, best spectacle-corrected visual acuity (BSCVA), strabismus, nystagmus, and cataract morphology. Results: Thirty patients with a mean age of 55 months (9 months-14 years) were included. On average, every child was seen by three physicians, and the mean duration between the first visit to a physician and presentation to our center was 2.23 ± 0.67 years. The various causes for delay in referral include multiple referrals due to a lack of general anesthesia services in 78% of cases, a long waiting list at the referral hospital in 35% of cases, and a lack of awareness at the primary-care physician level in 50% of cases. The mean BSCVA at presentation was 1.4 logMAR (0.3 to 3 logMAR). The most common cataract morphology was that of zonular cataract (31.48%; 17/54). Strabismus and abnormal eye movements were observed in 27.7% (15/54) and 33.3% (18/54) of eyes, respectively. Various systemic associations were periventricular leukomalacia (12/30), Down's syndrome (6/30), seizure disorder (6/30), cardiac valvular anomalies (6/30), Marfan's syndrome (4/30), hypothyroidism (4/30), rubella (3/20), cytomegalovirus (3/20), cerebral palsy (2/30), nephrotic syndrome (2/30), Type 1 diabetes mellitus (1/30), microcephaly (1/30), cryptogenic West syndrome (1/30), congenital rubella syndrome (1/30), and Tourette syndrome (1/30). The mean postoperative corrected distance visual acuity (CDVA) at 2-year follow-up improved to 1.0 logMAR (0 to 3 logMAR). No postoperative complications were reported at the final follow-up. Around 70% of the parents reported improvement in their child's psychomotor skills. Conclusion: Intellectually impaired pediatric patients with cataract should be operated upon whenever there is a presence of infrastructure, and unnecessary delay in surgery should be avoided by referring the patient to higher centers. Even though objective improvement in visual acuity was suboptimal, there was definitely an improvement in the psychomotor skills of the patients.

Descemet membrane endothelial keratoplasty: Update on preoperative considerations, surgical techniques, and outcomes.

Descemet membrane endothelial keratoplasty (DMEK) is the closest to the physiological replacement of endothelial cells. In the initial years, the technique was surgically challenging. Over the years, with better understanding and modifications in the surgical steps, the technique has evolved as an alternative to more popular procedure Descemet stripping endothelial keratoplasty. The article highlights the various preoperative, intraoperative, and postoperative nuances of DMEK. Additionally, it summarizes the various comparative and noncomparative studies on DMEK outcomes.

Mucopolysaccharidosis: A broad review.

Mucopolysaccharidosis (MPS) is a group of genetic disorders with seven types and 13 subgroups which are characterized by an inherent deficiency of the enzymes responsible for the degradation of glycosaminoglycans (GAGs). Defective breakdown of GAG products leads to their widespread accumulation within the lysosomes of various organs involving the eye, central nervous system, skeletal, ocular, nervous, respiratory, cardiac, and the gastrointestinal systems. Clinical spectrum varies from mild systemic and ocular abnormalities with a normal life span to severe phenotype, fatal in the first few months of life. Visual disability due to corneal clouding, retinopathy, and optic nerve involvement causes additional impairment of physical and cognitive functions. Treatment modalities such as bone marrow transplantation and enzyme replacement therapies help in increasing the life span as well as the quality of life of the affected patients. For patients with significant corneal clouding, keratoplasty is the answer. The decision to proceed with keratoplasty is governed by various factors such as the motivation of the patient and his family, other systemic affections and anesthesia concerns. A detailed preoperative counseling should be done regarding the expected visual outcomes in the presence of other ocular comorbidities and the postoperative complication such as graft re-opacification, rejection and glaucoma. Future treatment options such as targeted gene therapy and substrate reduction therapy hold promise to reverse corneal clouding, thereby obviating the need for corneal transplantation. These treatment therapies are still in the experimental stages and human trials are needed to validate their outcomes.

Outcomes of surgical intervention in cases of ectopia lentis.

Purpose: To evaluate the outcomes of surgical intervention in cases of ectopia lentis. Methods: This retrospective study included all cases of ectopia lentis that presented between June 2015 and March 2019 in a tertiary care center. They were reviewed retrospectively. The corrected distance visual acuity (CDVA), severity of lens subluxation, type of surgery, intra-operative and post-operative complication, and specular count were recorded. Results: Seventy-eight eyes of 57 cases with a mean age at surgery of 14.73 years were analyzed. Intra-lenticular lens aspiration was the most common (n-62/78; 79.5%) surgical procedure followed by lens aspiration, intra-capsular cataract extraction, phaco-aspiration, and pars-plana lensectomy. Simultaneous intra-ocular lens (IOL) implantation was performed in 46.2% (n-32/78) of the eyes. The mean CDVA improved from 0.85 ± 0.55 logMAR to 0.44 ± 0.29 logMAR at 6 weeks follow-up. The post-operative CDVA was significantly better in the pseudo-phakic group compared to the aphakic group (p-0.02). The patient's age at the time of surgery and the degree of subluxation did not impact the final visual outcome. Intra-operative complication included vitreous hemorrhage (n-1) and lens matter drop (n-1). Post-operative complications were noted in 26.9% of the eyes (n-21/78) with a higher complication rate in the pseudo-phakic group (p-0.00). A second intervention was required in 7.7% of the eyes (n-6/78). Conclusion: Age and degree of subluxation at the time of surgery do not influence the final visual outcome in cases of ectopia lentis undergoing lens extraction surgery. IOL implantation results in better visual outcomes but is associated with a high complication rate.

Ocular manifestations in Kindler syndrome.

We aimed describe the chronic ocular sequelae of Kindler syndrome. All cases of Kindler syndrome with ocular involvement that presented to a tertiary eye care center were included. Three cases of Kindler syndrome with ocular changes were reviewed. Case 1 (10 years, female) had recurrent epithelial breakdown with severe dry eye and corneal opacity secondary to keratitis. Case 2 (28 years, male) had symblepharon , ocular surface keratinization , and severe dry eye. Case 3 (16 years , female ) had partial limbal stem cell deficiency with dry eye. All cases were treated with topical lubricants, short course of low-potency steroids and immuno-modulators. Attention must be paid to the eye in addition to the oro-an-genital mucosa to avoid longterm ocular sequelae.

Early onset unilateral Terrien's marginal degeneration.

A young man in his first decade of life presented with insidious onset, gradually progressive diminution of vision in right eye since 2 months associated with foreign body sensation. Slit-lamp examination revealed superior arcuate band of corneal stromal thinning extending from 9-3 o' clock. There was associated corneal vascularisation and presence of lipid deposition at the leading edge of the furrow formation with intact epithelium without any apparent signs of inflammation. There was no previous history of ocular or systemic disease, trauma, ocular surgery, collagen vascular disease or contact lens wear. Serological tests for rheumatic diseases did not show any abnormalities. Scheimpflug imaging showed high against the rule astigmatism with a 'reverse crab claw' pattern. A provisional diagnosis of right eye Terrien's marginal corneal degeneration was made. On a 1-month follow-up visit, there was significant symptomatic relief with lubricants alone and with spectacles the visual acuity improved to 20/60 in the right eye.

Clinical Profile and Treatment Outcomes of Patients With Acremonium Species Positive Keratitis Managed in a Tertiary Eye Care Center.

PURPOSE: To report the clinical profile and treatment outcomes of patients with culture-positive Acremonium keratitis. METHODS: This is a retrospective observational study. Medical records of all patients treated in a tertiary eye hospital for culture positive infective keratitis from March 2016 to February 2021 were screened, of which those positive for Acremonium species on fungal culture were reviewed. Demographic details, clinical presentation, clinical course, treatment given, total follow-up duration, time taken for ulcer to heal, scar size, and final visual acuity in the last follow-up were recorded. RESULTS: Fifty three cases of fungal keratitis caused by Acremonium species were identified, 22 females and 31 males, with average age of 46.39±18.64 years. The mean duration of symptoms being 54.47±50 days. Only five patients had a history of trauma with vegetative matter. Clinical presentation of patients showed a large number of variations, with 2 patients presenting as peripheral ulcerative keratitis and 1 with epithelial plaque. The mean visual acuity of patients at presentation was 2.43±0.46 logMAR units. Thirty-three of 53 patients presented with perforated corneal ulcer and underwent penetrating keratoplasty; 20 patients were medically managed on topical voriconazole 1%, natamycin 5%, and oral voriconazole. The mean duration of healing of epithelial defect was 95±60.62 days (range 60-165 days). CONCLUSION: Acremonium keratitis has a long and indolent course. A prolonged combination therapy of natamycin and voriconazole seems to be effective in the management. A delay in the diagnosis of Acremonium keratitis often leads to clinical worsening requiring keratoplasty.

Management of descemetocele: Our experience and a simplified treatment algorithm.

Purpose: To formulate a treatment algorithm for the management of descemetocele. Methods: This was a prospective interventional study that was conducted at a tertiary eye-care center. All consecutive cases of descemetocele during the study period (April 1, 2017-March 31, 2018) were evaluated for the following parameters: age, sex, previous medical or surgical therapy, risk factors, preexisting ocular diseases, location, site and size of descemetocele, interventions undertaken, visual acuity, and the fellow eye status. The surgical modalities and fellow eye status were correlated individually with therapeutic and functional outcomes, based on which a treatment algorithm was formulated. Results: The study included 24 eyes of 24 patients (19M, 5F) with a median age of presentation of 45 years. The mean follow-up duration was 6.79 ± 3.97 months (3-12 months). The most common cause of descemetocele was microbial keratitis (66.66%), and most cases were central (50%), small (58.33%), and non-perforated (79.16%). The surgical interventions undertaken were cyanoacrylate glue (CG, 37.5%), penetrating keratoplasty (PKP, 33.33%), patch graft (16.66%), and deep anterior lamellar keratoplasty (DALK, 12.5%). Therapeutic success was noted in 13/24 eyes (54.16%). Final visual acuity > 3/60 was seen in 25% cases. Suboptimal therapeutic (P = 0.07) and visual (P = 0.34) outcomes were noted in subjects with non-functional fellow eye. Conclusion: PKP was preferred for descemetoceles with active microbial keratitis and extensive infiltrates, while CG and DALK were undertaken for healed microbial keratitis, neurotrophic keratitis, and ocular surface disorders with partial limbal stem cell deficiency (LSCD). For total LSCD, amniotic membrane graft was preferred.

Deep blue dot corneal degeneration: a rare corneal degeneration.

A female patient in the age group 55-60 years presented to us with blurring of vision in both eyes. On slit-lamp examination, numerous circular to oval fleck-like discrete blue opacities at the level of deep corneal stroma and Descemet's membrane was observed. These lesions were predominantly seen in the central two-thirds of the cornea. Considering the age of presentation and the clinical features, the probable diagnosis of 'deep blue dot corneal degeneration' was made.

Tuck-in deep anterior lamellar keratoplasty for the management of post-radial keratotomy keratectasia.

PURPOSE: To evaluate the outcomes of tuck-in deep anterior lamellar keratoplasty (DALK) for the management of post-radial keratotomy (RK) keratectasia. SETTING: Institution. DESIGN: Retrospective. METHODS: Medical records of all cases presenting with post-RK keratectasia from January 2012 to December 2019 were included. Data on corrected distance visual acuity (CDVA), refraction, keratometry, ultrasonic pachymetry, corneal topography, endothelial cell count, applanation tonometry, and dilated fundus examination recorded at all follow-up visits were included. Details of surgical steps, including intraoperative and postoperative complications and any secondary procedures performed subsequently, were also recorded. RESULTS: 7 eyes of 5 patients that developed corneal ectasia after a previous RK procedure underwent DALK with peripheral tuck-in. All eyes had CDVA ≤6/24, preoperatively. The surgical procedure was successfully performed in all eyes without any intraoperative complications. CDVA at the final follow-up ranged between 6/9 and 6/60, with 6 eyes achieving CDVA of 6/18 or better. The median keratometric cylinder reduced from a preoperative value of 7.2 ± 9.27 diopters (D) to 2 ± 1.83 D at the final follow-up. The median central corneal thickness at the final follow-up was 598 ± 40.01 µm, and spherical equivalent ranged between -1.75 D and -3.5 D (median -2 ± 0.57). The percentage of endothelial cell loss ranged between 2.1% and 8.4%. All eyes retained graft clarity, and none showed any evidence of significant interface haze or corneal vascularization. CONCLUSIONS: Tuck-in DALK could successfully address corneal ectasia developing after RK, achieving visual gains. Moreover, it was able to restore the peripheral corneal thickness with a minimal risk for dehiscence of RK incisions.

Fungal keratitis: A review of clinical presentations, treatment strategies and outcomes.

Infectious keratitis is a significant cause of corneal blindness worldwide. Although less prevalent in the developed world, cases of fungal keratitis account for almost half of all keratitis cases, occurring in the developing countries. These cases are one of the most refractory types of infectious keratitis and present various challenges to the treating physician such as delayed presentation, long waiting time for culture positivity, limited availability effective antifungal drugs, prolonged duration for response to therapy, a highly variable spectrum of anti-fungal drug sensitivity and a high recurrence rate following keratoplasty. The advent of rapid diagnostic tools, molecular methods, in vitro anti-fungal drug sensitivity testing, alternatives to natamycin, targeted drug delivery and most importantly the results of large randomized controlled trials have significantly improved our understanding and approach towards the diagnosis and management of cases with fungal keratitis. Overall, Aspergillus and Fusarium species are the most common causes ones of fungal keratitis. History of antecedent trauma is a significant predisposing factor. Corneal scrapings for microscopic evaluation and culture preparation, is the standard of care for establishing the diagnosis of fungal keratitis. Molecular identification of cultures offers accurate identification of fungal pathogens, especially the rare species. Natamycin is an approved first-line drug. Voriconazole is the best alternative, especially for non-fusarium cases. Management involves administration of drugs usually by a combination of various routes, the treatment regimen being individualized depending upon the response to therapy. Photodynamic therapy is a newer treatment modality, being tried for non-responsive cases, before resorting to a therapeutic graft.

Evaluation of various preservation media for storage of donor corneas.

PURPOSE: To compare the physical and microbiological characteristics of McCarey-Kaufman (MK), Cornisol, and Optisol-GS media and evaluate the outcomes of keratoplasty performed using corneas stored in these three media. METHODS: The study involved 60 donor corneas which were distributed in 3 groups: MK, Cornisol, and Optisol-GS. Corneas in these groups were further analyzed based on the type of keratoplasty performed (full thickness versus endothelial keratoplasty). At baseline, the endothelial cell density and death to preservation time of donor corneas were recorded. Following keratoplasty, patients were evaluated on day 1, at 1 month, 3 months, and 6 months follow-up. Outcomes were assessed in terms of corrected distance visual acuity (CDVA), endothelial cell density, percentage endothelial cell loss, and corneal thickness. The storage media were also assessed for their physical quality and their microbiological characteristics. RESULTS: Physical characteristics of all three media were found to be within normal limits. Mean CDVA was comparable among the 3 groups at 6-month follow-up. The absolute endothelial cell count values were significantly lower for corneas stored in MK medium (1873.7 ± 261.1 cells/mm2) compared to the Cornisol (2085.0 ± 230.3 cells/mm2) and Optisol-GS media [(2180.3 ± 217.2 cells/mm2) (P = <0.001)]. Corneas stored in Optisol-GS medium were significantly thinner at 1-month follow-up with no significant difference at 6 months (P = 0.66). CONCLUSION: Optisol-GS and Cornisol media were found to preserve endothelial cell density better and stabilize corneal thickness earlier as compared to the MK medium. However, the functional outcomes were comparable among the three groups.