Evidence for persistent UV-induced DNA damage and altered DNA damage response in xeroderma pigmentosa patient corneas.

Xeroderma pigmentosum (XP) is a rare genetic disorder characterized by injury to the ocular surface due to exposure to ultraviolet (UV) radiation. UV-induced damage in the cells leads to the formation of cyclobutane pyrimidine dimers (CPDs) and 6-4 pyrimidine-pyrimidone photoproducts that are repaired by the NER (Nucleotide Excision Repair) pathway. Mutations in the genes coding for NER proteins, as reported in XP patients, would lead to sub-optimal damage repair resulting in clinical signs varying from photo-keratitis to cancerous lesions on the ocular surface. Here, we aimed to provide evidence for the accumulation of DNA damage and activation of DNA repair pathway proteins in the corneal cells of patients with XP. Corneal buttons of patients who underwent penetrating keratoplasty were stained to quantify DNA damage and the presence of activated DNA damage response proteins (DDR) using specific antibodies. Positive staining for pH2A.X and thymidine dimers confirmed the presence of DNA damage in the corneal cells. Positive cells were found in both control corneas and XP samples however, unlike normal tissues, positive cells were found in all cell layers of XP samples indicating that these cells were sensitive to very low levels of UV. pH2A.X-positive cells were significantly more in XP corneas (p < 0.05) indicating the presence of double strand breaks in these tissues. A positive expression of phosphorylated-forms of DDR proteins was noted in XP corneas (unlike controls) such as ataxia telangiectasia mutated/Rad-3 related proteins (ATM/ATR), breast cancer-1 and checkpoint kinases-1 and -2. Nuclear localization of XPA was noted in XP samples which co-localized (calculated using Pearson's correlation) with pATM (0.9 ± 0.007) and pATR (0.6 ± 0.053). The increased presence of these in the nucleus confirms that unresolved DNA damage was accumulating in these cells thereby leading to prolonged activation of the damage response proteins. An increase in pp53 and TUNEL positive cells in the XP corneas indicated cell death likely driven by the p53 pathway. For comparison, cultured normal corneal epithelial cells were exposed to UV-radiation and stained for DDR proteins at 3, 6 and 24 h after irradiation to quantify the time taken by cells with intact DDR pathway to repair damage. These cells, when exposed to UV showed nuclear translocation of DDR proteins at 3 and 6 h which reduced significantly by 24 h confirming that the damaged DNA was being actively repaired leading to cell survival. The persistent presence of the DDR proteins in XP corneas indicates that damage is being actively recognized and DNA replication is stalled, thereby causing accumulation of damaged DNA leading to cell death, which would explain the cancer incidence and cell loss reported in these patients.

Strategies and Application of Compression Sutures With a Modified Technique for Rapid Resolution of Large (Grade III) Hydrops: A Prospective Interventional Study.

PURPOSE: The aim of this study was to describe the strategies and outcomes, with a modified technique, of compression sutures in large acute hydrops. METHODS: This was a prospective interventional study in 29 eyes of 28 patients who underwent compression sutures using a modified technique. The degree of resolution after surgical intervention was quantified by 3 masked observers, using the slit-lamp photographic documentation and optical coherence tomography. The degree of resolution, best-corrected visual acuity, and complications were analyzed. RESULTS: The mean age was 20.89 (7-46) years. Five patients had pellucid marginal degeneration (pellucid marginal corneal degeneration), 23 had keratoconus, and 1 had posttrauma hydrops. The preoperative visual acuity was hand motions in all eyes. The reduction in edema was 76% on day 1, 91.6% on 6 ± 2 days, 98.6% on 18 ± 3 days from surgical intervention. The baseline pachymetry assessed on optical coherence tomography was beyond measurement in 26 eyes. The mean pachymetry at day 1 was 704 µm (range 480-950) which reduced to 607 (range 422-850) microns at 6 ± 2 days and 518 (range 415-718) microns at 18 ± 3 days postintervention ( P < 0.0001). The mean duration between the surgery and complete suture removal was 48 (35-68) days. No suture-related complications were noted. Twenty eyes could attain good visual rehabilitation with glasses/contact lenses (vision could not be assessed in 5 eyes). Penetrating keratoplasty was performed on 1 patient. CONCLUSIONS: Compression sutures using the modified technique was safe and effective in the rapid resolution of acute hydrops in keratoconus and pellucid marginal corneal degeneration. No complications were noted, and visual restoration with spectacles and contact lenses was satisfactory.

Keratoconus in India: Clinical presentation and demographic distribution based on big data analytics.

PURPOSE: This paper aims to describe the clinical presentation and demographic distribution of keratoconus (KCN) in India by analyzing the electronic medical records (EMR) of patients presenting at a multitier ophthalmology hospital network. METHODS: This cross-sectional hospital-based study included the data of 2,384,523 patients presenting between January 2012 and March 2020. Data were collected from an EMR system. Patients with a clinical diagnosis of KCN in at least one eye were included in this study. Univariate analysis was performed to identify the prevalence of KCN. A multiple logistic regression analysis was performed using R software (version 3.5.1), and the odds ratios are reported. RESULTS: Data were obtained for 14,749 (0.62%) patients with 27,703 eyes diagnosed with KCN and used for the analysis. The median age of the patients was 22 (inter-quartile range (IQR): 17-27). In total, 76.64% of adults (odds ratio = 8.77; P = <0.001) were affected the most. The majority of patients were male (61.25%), and bilateral (87.83%) affliction was the most common presentation. A significant proportion of the patients were students (63.98%). Most eyes had mild or no visual impairment (<20/70; 61.42%). Corneal signs included ectasia (41.35%), Fleischer ring (44.52%), prominent corneal nerves (45.75%), corneal scarring (13.60%), Vogts striae (18.97%), and hydrops (0.71%). Only 7.85% showed an association with allergic conjunctivitis. A contact lens clinic assessment was administered to 47.87% of patients. Overall, 10.23% of the eyes affected with KCN underwent a surgical procedure. the most common surgery was collagen cross-linking (8.05%), followed by deep anterior lamellar keratoplasty (1.13%) and penetrating keratoplasty (0.88%). CONCLUSION: KCN is usually bilateral and predominantly affects males. It commonly presents in the second and third decade of life, and only a tenth of the affected eyes require surgical treatment.

Keratoconus in two siblings with congenital distichiasis.

PURPOSE: To describe a previously unreported association of keratoconus in congenital distichiasis. METHODS: Observational case series of ocular findings in 2 siblings with congenital distichiasis. RESULTS: A 17-year-old male presented with tearing and photophobia in both eyes. His parents revealed that he was photophobic since birth. He had a lid surgery previously in both eyes. Clinical examination revealed a central scar with Descemet membrane tear suggestive of healed hydrops in the right eye. The left eye showed topographic features of keratoconus. His younger sibling, a 14-year-old female, also had similar symptoms of photophobia and tearing since birth. She had undergone electrolysis in both eyes. At the present visit, she had an epithelial defect with congestion in the right eye. Electrolysis of the distichiatic eyelashes was performed along with bandage contact lens application, which relieved her symptoms. Her topography revealed subclinical keratoconus in both eyes. The siblings' father also had complaints of photophobia since birth and had lid surgery and electrolysis in the second decade of his life. CONCLUSIONS: Patients with congenital distichiasis may have associated keratoconus. Chronic ocular irritation and subsequent eye rubbing in distichiasis could be the risk factor for keratoconus.

Developmental cataract in congenital ichthyosis.

A teenage boy who was previously diagnosed to have congenital ichthyosis presented to the eye clinic with complaints of gradually decreasing vision in both eyes since childhood. The best-corrected distance visual acuity was 20/125 in the right eye and 20/40 in the left eye. Clinical examination revealed developmental cataracts in both eyes. He underwent cataract surgery in the right eye and visual acuity improved to 20/25. Hence, we conclude that congenital ichthyosis can be associated with developmental cataracts. Cataract surgery helps in restoring vision in those with visually significant cataracts.

Recent and Evolving Therapies in the Management of Endothelial Diseases.

Corneal endothelium is the innermost layer of the cornea which has both barrier and pump function and very important to maintain cornea clarity. Unlike epithelium, endothelium does not have regenerative potential; hence, endothelial damage or dysfunction could lead to corneal edema and visual impairment. Advanced corneal transplantation which involves selective replacement of dysfunctional endothelium has led to improved and faster visual rehabilitation. But in recent times, alternative therapies in the management of corneal edema and endothelial diseases have been reported. In this review, we aim to give a comprehensive review of various strategies for the management of corneal endothelial dysfunction in order to give treatment which is precisely tailored for each individual patient. A review of all peer-reviewed publications on novel strategies for the management of endothelial dysfunction was performed. The various approaches to the management of endothelial dysfunction are compared and discussed. Shortage of human donor corneas globally is fuelling the search for keratoplasty alternatives. Corneal endothelial dysfunction can be caused following surgery, laser or corneal endothelial dystrophies which could be amenable to treatment with pharmacological, biological intervention and reverse the endothelial dysfunction in the early stages of endothelial failure. Pharmacological and surgical intervention are helpful in cases of good peripheral endothelial cell reserve, and advanced cases of endothelial cell dysfunction can be targeted with cell culture therapies, gene therapy and artificial implant. Treatment strategies which target endothelial dysfunction, especially FECD in its early stages, and gene therapy are rapidly evolving. Therapies which delay endothelial keratoplasty also are evolving like DSO and need more studies of long-term follow-up and patient selection criteria.

Characteristics of Corneal Endothelium in Axenfeld Rieger Spectrum.

PURPOSE: The purpose of this study was to compare the corneal endothelial characteristics in Axenfeld anomaly (AXA), Rieger anomaly (RGA), and Axenfeld-Rieger anomaly/syndrome with age-matched healthy controls. METHODS: This is a retrospective, comparative case-control study of 52 eyes of 30 patients with AXA/RGA and AXA/S and 36 controls. RESULTS: Median age at endothelial imaging was 21.5 years (interquartile range, 13.8-33.3 years). In the study group, the mean endothelial cell density (ECD) was 2112.4 ± 78.5 cells/mm 2 , the mean cell area (MCA) was 526.9 ± 28.5 µm 2 , and the coefficient of variation of cell size was 41.2 ± 1.8%. The ECD was significantly (all, P < 0.0001) lower than controls, while MCA ( P < 0.0001), SD of cell size ( P < 0.0001), and maximum cell area ( P = 0.0007) were significantly higher than controls. Four eyes of 3 patients had guttae on slitlamp evaluation and endothelial imaging. There were no differences in the corneal endothelial characteristics among the clinical subtypes. CONCLUSIONS: Patients with AXA, RGA, and Axenfeld-Rieger anomaly/syndrome have lower ECD and increased MCA compared with normal eyes. The reduced ECD associated with inherent anterior segment alterations can predispose to the risk of postcataract surgery endothelial decompensation in these eyes. The association of guttae in some eyes needs further investigational studies.

Clinical profile and demographic distribution of band shaped keratopathy in India: A study of 8801 patients.

Purpose: To describe the clinical profile and demographic distribution of band-shaped keratopathy (BSK) in patients presenting to a multitier ophthalmology hospital network in India. Methods: This cross-sectional hospital-based study included 2,664,906 new patients presenting between January 2011 and January 2021 (10-year period). Patients with a clinical diagnosis of BSK in at least one eye were included as cases. The data were collected using an electronic medical record system. Results: Overall, 8801 (0.33%) patients were diagnosed with BSK. The prevalence rates were 0.47% in children (age: <16 years) and 0.31% in adults. The majority of patients were males (62.87%) with unilateral affliction (85.21%). The mean age of the patients was 40.43 ± 23.14 years. The majority (16.93%) of the patients were in the age bracket of 11-20 years. A larger proportion of the patients were from higher socioeconomic status (60.46%) and the urban region (45.9%). Of the 10,103 eyes affected with BSK, the common ocular comorbidities were status post-vitreoretinal surgery (20.55%) and uveitis (12.7%) in children and corneal scar (41.23%) and spheroidal degeneration (13.7%) in adults. Most of the eyes had mild or no visual impairment (24.74%). Among the eyes that needed surgical intervention, chelation with ethylenediaminetetraacetic acid (EDTA) was the most performed surgical procedure (1.68%) along with phototherapeutic keratectomy (0.32%). Conclusion: BSK commonly affects adult males and is unilateral in nature. The majority of the patients in this cohort belonged to higher socioeconomic strata and urban geography. At initial presentation, visual impairment was mild to moderate in a vast majority of the patients, and the most common surgical intervention performed was chelation with EDTA during the study period.

Clinical and Demographic Profile of Uveal Coloboma: A Hospital-Based Study of 14,371 Eyes of 9557 Indian Patients.

OBJECTIVE: To describe the demographics and clinical profile of uveal coloboma in patients presenting to a multi-tier ophthalmology hospital network in India. DESIGN: Cross-sectional hospital-based study. METHODS: This cross-sectional, hospital-based study included 2,817,766 new patients presenting between August 2010 and May 2021. Patients with a clinical diagnosis of uveal coloboma in at least 1 eye were included as cases. Data were collected using an electronic medical record system. RESULTS: Overall, 9557 patients (0.34%) were diagnosed with uveal coloboma. Just over half of the patients were male (51.82%) and were affected bilaterally (50.37%). The most common age group at presentation was individuals in the second decade of life, comprising 2198 patients (23%). The overall prevalence was higher in patients of lower socio-economic status (0.53%) and from rural areas (0.39%). The most common type was retino-choroidal coloboma in 8049 patients (84.22%), followed by iris coloboma in 2129 patients (22.28%). The most common Ida Mann classification in the eyes with retino-choroidal coloboma was type 4 in 7049 eyes (57.23%), followed by type 2 in 3685 eyes (29.92%). Of the 14,371 eyes, 5696 eyes (39.64%) had a visual impairment of blindness (>20/400), followed by mild or no visual impairment (<20/70) in 2875 eyes (20.01%). In the 2228 eyes (15.5%) that required a surgical/ laser intervention, cataract surgery was performed in 951 eyes (6.62%), vitreo-retinal surgery in 661 eyes (4.6%), followed by laser photocoagulation in 357 eyes (2.48%). CONCLUSION: Uveal coloboma is more common in male individuals and is predominantly bilateral in the population studied. It is more commonly found in patients from lower socio-economic strata and from a rural background. The most common type is retino-choroidal coloboma, and more than one-third of the eyes are affected by blindness.

Clinical Utility of COVID-19 Real Time-Polymerase Chain Reaction Testing of Ocular Tissues of Non-COVID-19 Cornea Donors Deemed Suitable for Corneal Retrieval and Transplantation.

PURPOSE: The purpose of this study was to evaluate the prevalence of SARS-CoV-2 in human postmortem ocular tissues of asymptomatic donors and its implications on our eye banking protocols. METHODS: The expression of SARS-CoV-2 RNA was assessed by reverse transcription-polymerase chain reaction in corneal rims and conjunctival tissues from 100 donors who were found suitable for transplantation as per the donor screening guidelines of the Global Alliance of Eye Bank Associations. The donor's clinical history and cause of death were assessed for secondary analysis. RESULTS: Of 200 ocular tissues (100 corneal and 100 conjunctival) from the same 1 eye of 100 surgical-intended donors, between September 2020 and April 2021, the overall positivity rate for SARS-CoV-2 was ∼1% (2/200). Both the ocular samples that tested positive were conjunctival biopsies (2/100, 2%), whereas corneal samples were negative (0/100, 0%) in both donors. The causes of donor death were trauma in 51 donors, suicide in 33, cardiac arrest in 7, electric shock in 5, metabolic cause in 2, malignancy in 1, and snake bite in 1. None of the donors had a medical history suggestive of COVID infection or possible contact. None of the recipients from the donors were reported to have any systemic adverse event after keratoplasty until the follow-up of 6 weeks. CONCLUSIONS: The overall prevalence of SARS-CoV-2 was 1% (2% for conjunctival and 0% for corneal samples, P value = 0.5) in the donors who were found suitable for cornea recovery and transplantation. The findings of exceptionally low positive rates in our samples validate the criticality of history-based donor screening and do not support the necessity of postmortem PCR testing as a criterion for procurement and subsequent use for corneal transplantation.

Early postoperative infection following lamellar keratoplasty: a review.

With the growing popularity of lamellar keratoplasty for selective replacement of diseased corneal tissue, it is important to understand the risk of developing an infection after the procedure. Although lesser than that postpenetrating keratoplasty, the reports on post lamellar keratoplasty infectious keratitis are not negligible. Trends of acute infections arising within 2 months of surgery are a subject of interest. Most of these infections are reported post Descemet's stripping endothelial keratoplasty with a preponderance of Candida species. A donor to host transmission of infection is not uncommon. Among the Candida cases, about 80% seem to occur due to a donor to host transmission. Infections presenting as or progressing to endophthalmitis lead to a poor visual outcome. Strict aseptic measures and protocols during corneal tissue harvesting, tissue processing, tissue storage and surgery are essential to prevent occurrence of these infections. After the infection has occurred, determining the aetiology and drug susceptibility through microbiological testing is vital. This helps to guide treatment protocols and hence determines final outcome of these cases. Most cases require some form of surgical management for resolution of infection, most often a graft removal and therapeutic keratoplasty. Secondary surgical interventions are performed to restore graft clarity and achieve a good final visual outcome.

Outcomes of Descemet-Stripping Endothelial Keratoplasty in 52 Eyes With Iridocorneal Endothelial Syndrome.

PURPOSE: To evaluate the long-term outcomes of Descemet-stripping endothelial keratoplasty (DSEK) in 52 eyes with iridocorneal endothelial (ICE) syndrome. METHODS: Retrospective study of 52 eyes of 52 patients who were diagnosed with ICE syndrome and underwent DSEK between January 2010 and December 2019 with a follow-up of at least 6 months. RESULTS: The mean age was 48.8 ± 10.8 years. Female patients (n = 33) constituted 63.5%. The median duration of follow-up was 2.4 years (range, 0.5-9.1 yrs). The mean best-corrected visual acuity improved significantly after surgery and remained stable (∼20/50) through 5 years. The mean endothelial cell loss was 28%, 37.9%, 43.6%, and 56.9% at 6 months and at 1, 2, and 3 years, respectively. Graft rejection was noted in 5 eyes (9.6%). Postoperatively, increased intraocular pressure (IOP) was seen in 17 eyes (32.7%), and 10 eyes (19.2%) underwent glaucoma surgeries. Fourteen eyes (26.9%) had secondary graft failures. The estimates of graft success were 93.6% ± 3.6% at 1 year, 85.6% ± 5.5% at 2 years, 79.3% ± 6.7% at 3 years, 69.0% ± 8.9% at 4 years, and 59.1% ± 11.9% at 5 years. Postoperative increased IOP was found to be the only significant (P = 0.05) risk factor (hazard ratio 8.92) associated with graft failure. The clinical variant of ICE syndrome did not seem to influence the graft survival (P = 0.68). CONCLUSIONS: In this study, DSEK had a success rate of ∼60% at 5 years. Increased IOP post-DSEK is a significant risk factor for graft failure. Graft survival is not affected by the clinical variant of ICE syndrome.

Clinical profile and microbiological trends of therapeutic keratoplasty at a network of tertiary care ophthalmology centers in India.

OBJECTIVE: To describe the clinical profile and microbiology trends in patients undergoing therapeutic keratoplasty at a multi-tier ophthalmology hospital network in India. METHODS: This cross-sectional hospital-based study included 3147 eyes undergoing therapeutic keratoplasty between Jan 2016 and Dec 2020 (5 years period) as cases. The demographic data, clinical profile and microbiological analysis were collected using an electronic medical record system. RESULTS: Of a total of 13,625 eyes with microbial keratitis(non-viral), 3147 (23.1%) underwent therapeutic keratoplasty during the study duration. Majority of patients were males (68.35%), from a rural geography (49.89%) and in 51-60 years age bracket (23.74%). The mean age of the patients was 50.54 ± 15.83 years. Two-thirds of patients were from lower socio-economic strata (66.63%) with an agrarian background (36.51%). Of the 3,147 eyes, fungus (51.8%) was the most common indication of therapeutic keratoplasty followed by bacteria (16.87%) and parasite (1.27%). No organisms could be identified in about a third (29.33%) of the cases. Between 2016 and 2020, the trend of therapeutic keratoplasties for fungal infections steadily grew (39.9% vs 45.49%) while the bacterial infections showed a steady decline (23.15% vs 11.81%). CONCLUSIONS: Medical cure rate was seen in majority of those with microbial keratitis, and 23.1% eventually required management with therapeutic keratoplasty. Fungal keratitis was the most common indication for therapeutic keratoplasty. Male gender, rural setting, low socio-economic background and agricultural occupation are common risk factors for patients undergoing therapeutic keratoplasty in India.

Clinical, histological and immunohistochemistry characteristics of cornea in the sequelae stage of chronic vernal keratoconjunctivitis.

PURPOSE: To report the clinical outcomes and histopathological and immunohistochemistry (IHC) features in eyes with the sequelae stage of vernal keratoconjunctivitis (VKC). METHODS: Investigative study of corneal samples obtained following surgical intervention for vision restoration in four eyes of three patients with VKC. Patient 1 (an 11-year-old boy) had deep anterior lamellar keratoplasty in both eyes, Patient 2 (a 24-year-old male) underwent superficial keratectomy followed by penetrating keratoplasty, and Patient 3 (a 22-year-old male) underwent penetrating keratoplasty. The corneal samples retrieved after surgical intervention were assessed for histology features and immunohistochemistry (IHC) studies. RESULTS: The grafts were clear till the follow-up of 2-18 months. Histopathology of all four corneal samples showed epithelial hyperplasia, absent Bowman layer, thick hyalinized stromal lamellae, vascularization, and chronic inflammatory cells such as lymphocytes and plasma cells. IHC showed strong expression of CK 3 in both eyes of Patient 1 and no expression in Patients 2 and 3. The marker for limbal stem cells, ABCG2, was absent in all four samples; however, p63α was expressed strongly in Patients 2 and 3, moderately in the right eye of Patient 1, and marginally expressed in the left eye of Patient 1. CONCLUSION: The eyes in the sequelae stage of VKC (having corneal scarring and 360° hypertrophied limbus) can be managed favorably with keratoplasty and amniotic membrane transplantation without allogenic/cadaveric stem cell transplantation. The expression of transient progenitor cells in the scarred corneas of VKC patients in the sequelae stage suggests that the limbal stem cell dysfunction is more likely partial and self-renewal of limbal stem cells is a plausibility in these eyes.

Clinical profile and demographic distribution of Terrien's marginal degeneration in a multitier ophthalmology network in India.

PURPOSE: To describe the clinical profile and demographic distribution of Terrien's marginal degeneration (TMD) in patients presenting to a multitier ophthalmology hospital network in India. METHODS: This cross-sectional hospital-based study included 2,470,793 new patients presenting between September 2012 and September 2020 (~8 years period). Patients with a clinical diagnosis of TMD in at least one eye were included as cases. The data were collected using an electronic medical record system. RESULTS: Overall, 184 (0.007%) new patients were diagnosed. Over half of patients were males (56.52%) with bilateral affliction (54.89%). The mean age of the patients was 38.63 ± 16.75 years. Majority (28.26%) of the patients were between 21 and 30 years of age bracket. The number of patients were from rural and urban districts were comparable (41.85% vs. 48.37%). The most common clinical signs were ectasia/thinning (50.53%), corneal scar (44.21%), and prominent nerves (5.26%). Associated ocular comorbidities included vernal keratoconjunctivitis in 6 (2.11%) eyes, meibomitis in 6 (9.7%) eyes, squamous blepharitis in 2 (3.2%) eyes, dry eye in 2 (3.2%) eyes, cataract in 46 (74.2%) eyes, and glaucoma in 6 (9.7%) eyes. Most of the eyes had mild or no visual impairment (43.86%). Two (0.7%) eyes had spontaneous perforation patients. None of patients had hydrops in the years of follow-up. Keratoplasty was performed in 3.86%. CONCLUSION: TMD is a rare disease affecting patients seeking eye care at the ophthalmology network. It commonly affects adult males and is predominantly bilateral. The disease progression is slow, and risk of spontaneous perforation was low. At initial presentation, visual impairment was mild to moderate in majority and the most common surgical intervention was cataract surgery during the study period.

Clinical profile and demographic distribution of pellucid marginal corneal degeneration in India: A study of 559 patients.

PURPOSE: The aim of this study was to describe the clinical profile and demographic distribution of pellucid marginal corneal degeneration (PMD) in patients presenting to a multitier ophthalmology hospital network in India. METHODS: This cross-sectional hospital-based study included 2,470,793 new patients presenting between September 2012 and September 2020 (~8 years period). Patients with a clinical diagnosis of PMD in at least one eye were included as cases. The data were collected using an electronic medical record system. RESULTS: Overall, 559 (0.02%) new patients were diagnosed with PMD. The prevalence rates were 0.004% in children (age <16 years) and 0.03% in adults. The majority of patients were males (70.13%) with the bilateral affliction (77.1%). The mean age of the patients was 37.91 ± 13.19 years. The majority (30.23%) of the patients were between 31 and 40 years of age. A significant number of patients were from higher socioeconomic status (93.74%) and from the urban region (45.08%). Of the 990 eyes, the most common clinical signs were ectasia/thinning (58.99%), corneal scar (17.47%), and corneal hydrops (1.01%). The majority of the eyes (87.97%) were managed with either spectacles or contact lenses. Among those who had surgical intervention, collagen cross-linking was the most performed procedure (5.25%) followed by cataract surgery (4.14%). CONCLUSION: PMD is a rare disease affecting patients seeking eye care in India. It commonly affects adult males and is bilateral in nature. The disease progression is slow and usually occurs beyond 3 years. Conservative management is more common than surgical intervention.

Intermediate outcomes of therapeutic penetrating keratoplasty for severe microbial keratitis using glycerol-preserved donor corneas during the COVID-19 pandemic.

Purpose: To report the intermediate outcomes of therapeutic penetrating keratoplasty (TPK) performed for severe microbial keratitis using glycerol-preserved corneas during the Corona virus diseases of 2019 (COVID-19). Methods: Retrospective non-comparative case series from April to August 2020 in a network of tertiary eye care centers. Glycerol-preserved tissues were used for therapeutic keratoplasty (TPK). We reviewed the demographics, microbiology, surgical outcomes such as wound integrity, recurrence, graft melt, epithelialization, and complications. Results: A total of 49 eyes that underwent TPK with glycerol-preserved corneal tissues were analyzed. The primary indication was severe microbial keratitis in 47 eyes. The majority was a fungal infection in 33 eyes (67.3%). The mean age was 53.8 ± 12.2 years, with male predominance (3:1). The corneas were stored for an average of 85.5 ± 53 days prior to transplant. The median donor age was 65 years. The grafts were tectonically stable in 32/36 eyes (88.9%) at 1 month and 20/24 eyes (83.3%) at 3 months. The graft melt was noted in three eyes at 1 and 3 months. The recurrence of the infection was noted in four eyes and all were of fungal etiology. The graft epithelialization was delayed with a mean duration of 48.9 ± 25 days after surgery. Post-TPK, raised intra-ocular pressure (>21 mm Hg) was noted in 51.2% at 1 week, 17.4% at 1 month, and 11.8% at 3 months. Conclusion: Glycerol preservation is a reliable alternative with good therapeutic outcomes in the short and interim postoperative period. Delayed epithelialization and secondary glaucoma were the commonest postoperative complications.

Clinical clues to the early diagnosis and management of ocular surface neoplasia following keratoplasty in xeroderma pigmentosa.

The purpose of this report is to highlight the early clinical signs and management of ocular surface neoplasia following penetrating keratoplasty in xeroderma pigmentosa. A 14-year-old girl reported increased conjunctival hyperemia, tearing, photophobia in the right eye at 6 months after penetrating keratoplasty that worsened rapidly over the subsequent 2 weeks. Slit lamp examination showed increased vascularity, epithelial haze and raised whitish lesions at the graft-host junction. Alcohol keratoepithelectomy was performed for confirming the diagnosis. Histology of the sample revealed severe dysplasia. The lesion resolved after surface plaque brachytherapy. The graft clarity was restored and maintained until the last follow-up of 15 months following brachytherapy. Exacerbated symptoms and clinical signs of increased vascularisation and epithelial lesions should arouse the suspicion of ocular surface neoplasia in grafted eyes. Early diagnosis and management can salvage the graft clarity.

Descemet Stripping Automated Endothelial Keratoplasty in Pediatric Age Group: A Decade of Our Experience.

PURPOSE: This study was to report the outcomes of Descemet stripping automated endothelial keratoplasty (DSAEK) in managing corneal endothelial disorders in children less than 14 years of age. METHODS: Medical records of 180 DSAEK performed, between 2008 and 2020, on 167 eyes of 111 children who had a visually significant endothelial dysfunction were retrospectively reviewed for the primary etiology of corneal decompensation, time of the onset and duration of cloudiness, preoperative visual acuity, the technique of endothelial keratoplasty, surgical modifications, and reintervention. RESULTS: Median age at surgical intervention was 7.9 years (interquartile range, 5.2-11.2 years). At a median postinterventional follow-up of 2.5 years (interquartile range, 0.9-3.4 years), 86.2% (144 of 167 eyes, 95% confidence interval 79.9%-90.1%) maintained a clear graft. Median best-corrected visual acuity (logarithm of the minimum angle of resolution) improved from 1.45 ± 0.70 preoperatively to 0.90 ± 0.06 at the last follow-up visit (P < 0.0001). The cumulative overall long-term graft survival was 92.7%, 86.5%, and 77.7% at 1, 3, and 7 years, respectively. Cox proportional hazards regression analysis showed that the indication for DSAEK (P = 0.007; hazards ratio: 2.17 ± 0.62), age at surgery (P = 0.02; hazards ratio: 0.87 ± 0.05), and any subsequent intervention after DSAEK (P = 0.003; hazards ratio: 0.11 ± 0.08) were significant risk factors for DSAEK failure. The endothelial cell loss was 40.1% at 6 months, 45.4% at 1 year, 55.2% at 5 years, and 61.9% at 7 years. CONCLUSIONS: DSAEK is a safe and effective surgical strategy in managing endothelial disorders among the pediatric age group.