Outcomes of surgical interventions for the treatment of limbal stem cell deficiency.

Background & objectives: In the current scenario, with availability of different surgical procedures for limbal stem cell deficiency (LSCD), there exists no common consensus as to the standardization of the management protocol for the same. In addition, there also exists diversity in the views about the clinical diagnosis, ancillary investigations and clinical parameters. The objective of the present study was to evaluate the reported outcomes of surgical interventions for the management of LSCD. Methods: A systematic review of published literature on limbal stem cell transplantation (LSCT) was performed using Ovid Medline, Embase and PubMed for a duration of 2009 to 2019. Original studies including prospective, retrospective case series and randomized controlled trials, articles in English language, articles with access to full text and studies with more than or at least 10 patients were included in this review. Data related to clinical and visual outcomes were evaluated, and pool estimates of different surgeries were calculated using random-effects model and individually using Pearson's Chi-square test. Results: A total of 1133 abstracts were evaluated. Finally, 17 studies were included for the analysis. Among these 17 studies, direct limbal lenticule transplantation was performed in five studies, of which autologous tissue from the fellow eye [conjunctival limbal autograft (CLAU)], allograft from a cadaver/live donor [keratolimbal allograft (KLAL)/conjunctival limbal allograft (CLAL)] and combination of CLAU plus KLAL were done in one, three and one studies, respectively. The ex vivo expanded cultivated limbal epithelial transplantation (CLET) was reported in six studies and simple limbal epithelial transplantation (SLET) in four studies. Two were comparative studies comparing CLET and CLAL (living-related CLAL) with cadaveric KLAL, respectively. Outcome analysis of the included studies showed significant heterogeneity. Calculated pool rate for various types of surgeries was calculated. The pool estimate for CLAL was 67.56 per cent [95% confidence interval (CI), 41.75-93.36; I2=83.5%, P=0.002]. For KLAL, this value was 63.65 per cent (95% CI, 31.38-95.91; I2=92.4%, P=0.000). Pool estimate for CLET was 78.90 per cent (95% CI, 70.51-87.28; I2=73.6%, P=0.001). Corresponding values for SLET were 79.08 per cent (95% CI, 74.10-84.07; I2=0.0%, P=0.619). CLAU and combination of CLAU plus KLAL were done in one study each; hence, statistical analysis could not be done. The functional outcome in terms of gain in visual acuity post-operatively was better in KLAL (P<0.005) and SLET group as compared to CLET group. Interpretation & conclusions: The present analysis suggests that though the anatomical success rates were almost identical between SLET, CLET, CLAL, and KLAL procedures, the functional success rates were better following KLAL and SLET procedures as compared to CLET. Decision for LSCT for cases of ocular burns based on either clinical judgement of the surgeon or individual diagnosis remains a suitable option.

Knowledge and attitude towards eye donation among health professionals of northern India.

Background: We aimed to assess the knowledge and attitude of health professionals towards eye donation at an apex tertiary care centre of northern India. Methods: We interviewed 600 health professionals, comprising doctors, nurses, medical as well as nursing students, social workers and allied paramedical staff. A structured questionnaire (12 questions for assessing knowledge and 5 questions for assessing attitude) was used to estimate the awareness of eye donation and willingness to pledge eyes for donation. The responses pertaining to knowledge were graded as 'excellent', 'good' and 'poor' and those pertaining to attitude were grouped into 'positive' and 'negative'. Results: Of the 600 participants, 138 participants (23%) had 'excellent' knowledge and 234 participants (39%) had 'good' knowledge about eye donation. Awareness of eye donation was positively related to the level of literacy (odds ratio [OR] 8.5 [2.30-31.2]; p<0.001). Medical social workers and health supervisors had better knowledge about eye donation (OR 2.01 [1.08-3.72]; p=0.026) than other professional groups. Knowledge of eye donation had no significant association with age, gender, religion, family type and marital status of the respondent. Willingness to pledge eyes for donation was observed in only 6% of the participating health professionals. Pledging of eyes for donation was higher among older participants (OR 7.8 [2.67-22.77]; p<0.001). Conclusion: Our study shows that there is sufficient knowledge about eye donation, but an alarmingly low willingness to pledge eyes for donation among health professionals. Concerted efforts are required to alter their attitude to strengthen the Hospital Cornea Retrieval Programme.

Sutureless Customized Lamellar Corneal Transplant in a Patient with Gelatinous Drop-Like Corneal Dystrophy.

Patients with gelatinous drop-like corneal dystrophy need to be effectively managed as the disease is severely debilitating in view of associated pho-tophobia and glare. Here, we report a rare case of gelatinous drop-like corneal dystrophy effectively managed by intraoperative anterior segment optical coherence tomography-guided manual deep anterior lamellar keratoplasty in 1 eye and sutureless fibrin glue-aided, microkeratome-assisted automated lamellar therapeutic keratoplasty in the other eye. The patient, a 22-year old man, presented with gradual diminution of vision associated with foreign body sensation, glare, photophobia, and watering due to corneal lesions, which were consistent with a diagnosis of gelatinous drop-like corneal dystrophy. Visual acuity at pre-sentation was 4/60 and 3/60 in the right and left eye, respectively. The patient received customized component lamellar keratoplasty in both eyes, and host tissue was sent for histopathologic examination. Treatment resulted in a best-corrected distance visual acuity of 6/9 and 6/12 in the right and left eye, respectively. The graft was clear and well apposed, with minimal interface haze bilaterally. The histopathologic report suggested intralamellar amyloid deposition in the form of homogenous, acellular eosinophilic deposits in the epithelium and anterior corneal stroma. This is a first report of the exclusive use of a fibrin-aprotinin tissue adhesive to stabilize a donor corneal lamellar graft as a treatment modality for a patient with gelatinous drop-like corneal dystrophy, suggesting that this treatment could supplant the need for sutures.

Corneal Evaluation in Patients With Parkinsonism on Long-Term Amantadine Therapy.

PURPOSE: To evaluate the progression of corneal endothelial changes in patients with Parkinson disease (PD) on long-term oral amantadine therapy. METHODS: A prospective comparative longitudinal observational study of 90 patients (180 eyes) with PD on more than 6 months of oral amantadine therapy, 30 amantadine naive patients with PD, and 30 healthy controls (age and gender matched). Corneal endothelial cell parameters (endothelial cell density, percentage hexagonality of the cells, and coefficient of variation) and corneal subbasal nerve fiber layer changes were studied over a follow-up period of 1 year. RESULTS: The amantadine patients with PD group had a statistically significant decrease in endothelial cell density (1.51% vs. 0.94% vs. 0.55%) (P = 0.04), decrease of percentage hexagonality of the cells (4.98% vs. 3.56% vs. 2.31%) (P = 0.01), and increase of coefficient of variation (6.12% vs. 4.80% vs. 3.30%) (P = 0.03) compared with amantadine naive patients with PD and controls, respectively. Analysis of changes in the patients with PD based on the daily dosage of amantadine showed greater change in endothelial parameters in patients who were on 400 mg amantadine. CONCLUSIONS: Long-term amantadine therapy seems to effect changes on corneal endothelium.

Primary Bullous Keratopathy in a Patient With Werner Syndrome Treated With Corneal Transplant.

Here, we present, to the best of our knowledge, the first case of Werner syndrome with corneal blindness due to bilateral primary bullous keratopathy. Werner syndrome is a rare autosomal recessive disorder characterized by features of premature aging, insulin-dependent diabetes mellitus, osteoporosis, atherosclerosis, hypergonadotrophic hypogonadism, hypertriglyceridemia, scleroderma-like skin changes, and sarcomas. Among ocular manifestations, cataracts, cystoid macular edema, and retinal detachment have been reported. Because these patients show features of premature aging, they have decreased corneal endothelial function and delayed fibroblast growth. To date, there are few reports of wound dehiscence, bleb formation, and bullous keratopathy following surgical insult that have usually occurred after cataract surgery in patients with Werner syndrome. There have been no reports in the literature regarding Werner syndrome presenting with primary corneal decompensation without any inciting factor. Our patient with Werner syndrome had primary bilateral bullous keratopathy and bilateral corneal blindness for 10 years and was eventually rehabilitated by corneal transplant. Hence, this case highlights the importance of early referral of such patients to the ophthalmologist for prompt diagnosis and early treatment so that blindness could be avoided.

Isolated Primary Corneal Acremonium Eumycetoma: Case Report and Literature Review.

PURPOSE: To report an unusual case of isolated Acremonium eumycetoma presenting as a protuberant mass over the cornea. METHODS: Case report and literature review. RESULTS: A 55-year-old male patient referred to our center with a case of perforated corneal ulcer with uveal tissue prolapse was examined in the casualty department and found to have central melt, approximately 8 mm, along with suspected uveal tissue prolapse. A provisional diagnosis of sloughed corneal ulcer with uveal prolapse was made along with differential diagnoses of fungal ball and infected foreign body granuloma. Tectonic penetrating keratoplasty under general anesthesia was planned. Intraoperatively, the suspected uveal (brown colored) tissue was found to be an epicorneal mass growing over an intact and infiltrated cornea. Histopathological and microbiological analysis of the epicorneal mass and host cornea revealed it to be a fungal ball (mass full of septate hyphae) with growth of Acremonium species on culture. The patient was administered topical and oral antifungal agents postoperatively, in addition to topical antibiotics and cycloplegics. CONCLUSIONS: Isolated corneal Acremonium eumycetoma masquerading as a perforated corneal ulcer with prolapsed uveal tissue is a rare entity. Surgical intervention and appropriate antimicrobial therapy are key to successful outcome.

Isolated severe microblepharon in a neonate: a rare case.

PURPOSE: To report a rare case of isolated severe microblepharon in a neonate. METHODS: A 27 days old male child was brought by parents with redness, photophobia and discharge for two weeks. Thorough ophthalmological and systemic examination was performed. RESULTS: The diagnosis of isolated severe microblepharon with infectious keratitis was made. After the appropriate management of infectious keratitis and achieving complete resolution, the child was subjected to bilateral lid reconstruction was done in the form of upper lid skin grafting and tarsorrhaphy and the patient is being followed up. CONCLUSION: A rare case of bilateral isolated severe microblepharon affecting all four eyelids is being reported. Urgent surgical intervention is recommended in such cases in order to achieve good corneal coverage which results in faster healing of infective keratitis and a good visual outcome.

Retinal nerve fiber layer and ganglion cell layer changes on optical coherence tomography in early multiple sclerosis and optic neuritis cases.

PURPOSE: To study the retinal nerve fiber layer (RNFL) and ganglion cell layer (GCL) changes on optical coherence tomography in early multiple sclerosis (MS) patients. METHODS: A prospective cohort study was conducted at a tertiary care center. Patients of early MS (expanded disability status scale <3) with or without optic neuritis (ON) and idiopathic ON were included. Twenty age-matched individuals were taken as controls. Changes in RNFL and GCL thickness were evaluated along with the correlation with visual function parameters such as visual acuity, contrast sensitivity, and visual evoked response at first visit and again at six months. RESULTS: Forty-four patients of MS with or without ON (24 and 20 patients respectively), 29 patients with idiopathic ON, and 20 healthy controls constituted the cohorts. Mean LogMAR best-corrected visual acuity was found to be significantly reduced in all groups except fellow eyes (FE) of ON group. Mean values of average RNFL thickness and values in superior, temporal, and inferior quadrant were significantly reduced. Similarly, overall mean values of average GCL-inner plexiform layer (IPL) thickness and values in superior, superonasal, superotemporal, inferonasal, and inferotemporal quadrant were significantly reduced in all groups except FE of ON group (P < 0.05). All the visual parameters significantly correlated with GCL + IPL thickness. CONCLUSION: GCL + IPL thickness is a more sensitive clinical structural marker than RNFL in early MS with/without ON and ON patients and correlates with all the visual parameters better than RNFL thickness.

Infective keratitis in Indian patients with corneal dystrophies.

All eight patients seen with various types of corneal dystrophy, a rare hereditary corneal disease, presented with infective keratitis. Unilateral microbial invasion was seen in all patients and the visual acuity of the affected eyes was <6/60 at presentation. We highlight our impression that microbial invasion can occur with all types of epithelial and stromal corneal dystrophy, including macular corneal dystrophy. Regular follow-up to maintain a healthy ocular surface is recommended to avert blindness in such patients.

Awareness Regarding Eye Donation in an Urban Slum Population: A Community-Based Survey.

OBJECTIVES: Our objective was to assess the awareness of eye donation in an urban slum population and willingness to donate eyes after death. MATERIALS AND METHODS: A cross-sectional, population-based study was undertaken in 20 urban slum clusters of the Indian capital, New Delhi. A total of 2004 individuals aged 18 years and older were recruited. After written, informed consent was obtained, knowledge regarding eye donation was assessed through a predesigned close-ended questionnaire. The questionnaire was framed so as to understand the sociodemographic factors influencing the willingness to donate and the awareness of eye donation in this distinct population. RESULTS: The mean age of the recruited individuals was 36.53 ± 13.68 years. Age did not have any significant effect on awareness regarding eye donation. We observed that 34.3% of the study population had no knowledge of eye donation and that 7.78% of the study population had excellent knowledge. Education seemed to be an important determining factor regarding knowledge of eye donation. Multivariable logistic regression demonstrated better awareness among the Hindu population (81.1%) and those belonging to a higher caste (P < .05). The younger age group (those 18-30 years old) showed significant willingness to donate their eyes versus older age groups (P < .001). In our study population, male participants (P = .006), those classified as literate (P < .001), and those classified as Hindu (P < .001) were more willing to pledge their eyes for donation. CONCLUSIONS: Although there is substantial awareness about eye donation, willingness to pledge eyes was very low in the urban slum population. Additional efforts are needed to translate this awareness into actual eye donation in the urban poor population.

Corneal tomography and biomechanics in primary pterygium.

PURPOSE: To study the Scheimpflug's imaging and corneal biomechanics in primary pterygium. METHODS: A prospective observational study of 55 patients with unilateral primary nasal pterygium was done. The normal fellow eyes of patients with pterygium were taken as controls. Clinical parameters noted included visual acuity, values of corneal curvature by doing Scheimpflug imaging, wavefront aberrations in terms of higher and lower-order aberrations and corneal hysteresis (CH) as well as corneal resistance factor (CRF) values by using ocular response analyzer. RESULTS: Of the total 55 patients, mean age was 43.0 + 11.4 years (range: 20-72 years). Mean LogMar uncorrected visual acuity in pterygium eyes and control eyes was 0.21 + 0.20 and 0.12 + 0.15, respectively (p = 0.016). On Scheimpflug imaging the mean anterior corneal curvature values (Ka1/Ka2 D) were 41.09 + 3.38/44.33 + 2.29 in pterygium eyes, 43.13 + 1.79/43.98 + 2.17 in control eyes (p < 0.0005) and mean posterior corneal curvature (Kp1/Kp2 D) values were 6.14 + 0.39/6.53 + 0.43 in pterygium eyes and 6.13 + 0.28/6.46 + 0.47 in control eyes (p > 0.05). Analysis of corneal aberrations showed significantly higher corneal wavefront aberrations in pterygium eyes. Highest correlation of corneal astigmatism was noted with corneal area encroached by pterygium (ρ = 0.540 for LOA and 0.553 for HOA) and distance from pupillary center (ρ = 0.531 for LOA and 0.564 for HOA). Corneal biomechanical parameters including CH and CRF were found to be lower in the pterygium eyes, though not statistically significant (p value 0.60 and 0.59, respectively). CONCLUSION: Pterygium leads to deterioration of visual performance not only by causing refractive and topographic changes but also by causing a significant increase in corneal wavefront aberrations.

In Vivo Confocal Microscopic Characteristics of Crystalline Keratopathy in Patients with Sclerokeratitis.

PURPOSE: To report in vivo confocal features in a clinical case series of patients with sclerokeratitis presenting as crystalline keratopathy. METHODS: Five cases of crystalline keratopathy following sclerokeratitis are described. Confocal microscopic images of the cornea were captured in all cases to confirm the diagnosis by evaluating the morphology of the crystals. RESULTS: Unilateral and non-progressive peripheral crystalline keratopathy manifested after previous episodes of sclerokeratitis in the involved eye. Confocal microscopy revealed numerous, discrete, hyperreflective, needle-like, shiny crystals in the anterior and posterior stromal layers of the cornea. These deposits were oriented randomly and showed occasional confluence. An extensive ophthalmic and systemic evaluation did not reveal any other contributory factors. CONCLUSION: Crystalline keratopathy, probably resulting from an immune-mediated response, is a possible manifestation of sclerokeratitis. This should be considered during long-term follow-up of such patients and differentiated from infectious crystalline keratopathy.

Eye donation and eye banking in India.

Corneal blindness is a priority condition under the National Programme for Control of Blindness and an important cause of avoidable blindness in India. A multipronged approach is needed to eliminate corneal blindness. Curable or treatable blindness requires a spectrum of care including medication, optical rehabilitation and corneal transplantation. Corneal transplantation is dependent on the availability of safe, donor eyes; however, there is scarcity of donor corneal tissues in India. To improve the eye banking system, the Government of India supports eye banks through recurring grants for operational costs and non-recurring grants for infrastructure costs. Strategic interventions by the government and non-governmental organizations include awareness by health promotion and education, community participation, sustainable source of donor cornea, quality medical standards, accreditation and endeavours to strengthen eye banking systems and procedures through training and research. A model eye banking system in India can be achieved only when it is linked with the targeted infrastructure proposed under 'Vision 2020: Right to Sight- India'. Considering these targets, there is a requirement of at least 20 eye bank training centres, 200 eye banks with corneal transplant facility (collection of nearly 500 corneas per year) and 2000 eye donation centres in the country. This would become a reality if the Hospital Cornea Retrieval Programme is strengthened at all private and government hospitals, uniform medical standards are made mandatory for all eye banks and eye donation centres and the process of registration and eye donation is simplified to enhance community participation.

Benign Intracranial Hypertension -Is it Really Benign Always?

BACKGROUND: In children, benign or idiopathic intracranial hypertension (IIH) is uncommon. The presenting symptoms are usually variable, which attribute to delay in diagnosis. Frequently encountered symptoms include; headache, vomiting, visual impairment, neck pain and diplopia. IIH in pre-pubertal children has distinct characteristics from the adult form. Delayed diagnosis in children usually attributes to the inability in picking subtle visual changes, which can subsequently leads to permanent visual damage. OBJECTIVE: We report cases of two adolescent girls having IIH who presented with papilloedema, could be managed with lumboperitoneal shunting which resulted in favorable outcomes in terms of improved visual acuity and relief of symptoms. CASE: We report two cases of IIH, presented with headache and severe visual loss, which showed non responsiveness to medical management and improved with a lumboperitoneal shunt procedure. CONCLUSION: In IIH cases, enhanced awareness, prompt diagnosis and treatment are important, to avoid the risk of permanent visual damage. Urgent surgical intervention is essential in patients who do not respond to medical treatment.

Metastatic renal cell carcinoma presenting as one-and-a-half syndrome.

We report a case of 43-year-old male, presented with sudden onset binocular diplopia on lateral gazes. Ocular examination showed features of ipsilateral one-and-a-half syndrome. Comprehensive systemic work in conjunction with magnetic resonance imaging of the brain illustrated irregular mixed solid and cystic lesions in the brainstem, possibly indicative of brain metastases. Further imaging revealed hidden renal cell carcinoma as a primary neoplasm, which led to secondary manifestations.

Study of death to preservation time and its impact on utilisation of donor corneas.

To evaluate the impact of death-to-preservation time (DPT) on effective utilisation of donor corneas. In a prospective observational study conducted at our tertiary eye centre, donated corneas received over a 15-month period from November 2011 to January 2013 were evaluated. Donor age, donor refrigeration (done or not), DPT, endothelial cell density (ECD), corneal grading, clinical utilisation and surgical outcome after graft transplantation were noted. To analyse the impact of different DPT on donor cornea transplantation, primary outcome measures (corneal grading and endothelial cell density) and secondary outcome measures (primary graft failure and graft infection) were analysed. A total of 990 corneas were assessed. Primary outcomes showed no significant difference for higher DPT ( P > 0.01). ECD, where DPT was >12 h, was better for refrigerated corneas ( P < 0.001). Prolonged DPT had no significant effect on primary graft failure ( P = 0.131) and graft infection ( P = 0.137) in the first month after transplantation. We find that DPT should not be the only criteria to assess the cornea quality; other donor characteristics should be considered equally important. Donor refrigeration should be encouraged in cases where early retrieval is not possible.

Asymptomatic free-floating vitreous cyst masquerading as cysticercosis.

A male patient aged 37 years, referred with the diagnosis of right eye intravitreal cysticercosis, was diagnosed as asymptomatic free-floating vitreous cyst after thorough evaluation. The patient was kept under observation, since baseline visual acuity was unaffected. No change was noted over the period of 6 months.

Superficial Anterior Lamellar Keratoplasty (SALK) for Trauma-induced Post Refractive Surgery Corneal Opacity.

PURPOSE: To report a case of post laser in situ keratomileusis (LASIK), nebulomacular corneal opacity following a trauma induced flap dehiscence and was managed with superficial anterior lamellar keratoplasty (SALK). CASE REPORT: A 32-year-old female underwent LASIK 2.5 years back, with a postoperative unaided visual acuity (VA) of 6/6 in both eyes. She was involved in a road traffic accident and sustained blunt trauma to the right eye 5 months before. At the time of presentation, the VA was 1/60 in the right eye. Slit lamp examination revealed flap dehiscence, stromal scar and descemet folds in that eye. There was a small macular scar in the parafoveal area due to a resolved Berlin's edema. SALK was performed in the affected eye. Unaided VA of 6/36 was noted on post- operative day 1. After 4 weeks of SALK surgery, best corrected VA was 6/24. CONCLUSION: This case highlights that flap adhesions are not very strong even years after LASIK and SALK may be an effective treatment option for post refractive surgery corneal opacities.