Clinical profile and demographic distribution of Stargardt disease phenotypes: An Electronic medical record-driven big data analytics from a multitier eye care network.

Purpose: To describe the demographics and clinical profile of Stargardt disease in patients presenting a multitier ophthalmology hospital network in India. Methods: This cross-sectional hospital-based study was performed among 2,834,616 new patients presenting between August 2010 and June 2021 in our network. Patients with a clinical diagnosis of Stargardt disease in at least one eye were included as cases. The data were collected using an electronic medical record system. Results: Overall, 1,934 (0.069%) patients were diagnosed with Stargardt disease. Most of the patients were male (63.14%). The most common age group at presentation was during the second decade of life, with 626 (31.87%) patients. The overall prevalence was higher in patients from a higher socioeconomic status (0.077%), in those presenting from the urban geography (0.079%), and in students (0.197%). Systemic history of hypertension was seen in 56 (2.85%) patients, while diabetes mellitus was seen in (2.49%) patients. Of the 3,917 eyes, 1,910 (48.76%) eyes had moderate visual impairment (>20/70-20/200) followed by severe visual impairment (>20/200 to 20/400) in 646 (16.49%) eyes. The most commonly associated retinal signs were retinal flecks in 1,260 (32.17%) eyes, followed by RPE changes in 945 (24.13%) eyes. The most documented investigations were autofluorescence (39.85%), followed by optical coherence tomography (23.90). Cataract surgery was the commonest performed surgical intervention in (0.66%) eyes, followed by intravitreal injection in 4 (0.10%) eyes. The family history of parent consanguinity marriage was reported by 212 (10.79%) patients. Conclusion: Stargardt disease was seen more commonly in males presenting during the second decade of life. It is predominantly a bilateral disease, with the majority of the eyes having moderate visual impairment.

Psychosocial factors among patients undergoing orbital exenteration.

CLINICAL RELEVANCE: Patients undergoing exenteration have significant anxiety and depression, and lower quality of life that worsens after surgery. Therefore, counselling by an eye health-care provider along with the team of psychologist and psychiatrist could be provided pre-operatively to assess baseline psychosocial status and be continued post-operatively to look for any deterioration. BACKGROUND: Orbital exenteration comes with psychological trauma of disfigurement of face. The study aimed to evaluate the quality of life, anxiety and depression in patients undergoing orbital exenteration, before and after the procedure; and also compared the same with patients previously rehabilitated by spectacle-mounted prosthesis after exenteration. METHODS: It was a prospective cohort study. Group I included patients who were planned to undergo orbital exenteration, and group II were those already using spectacle-mounted prosthesis post orbital exenteration for at least 3 months. Level of depression was assessed using Patient Health Questionnaire-9(PHQ-9), anxiety using Generalised Anxiety and Depression scale-7(GAD-7), and quality of life was assessed using Functional Assessment of Cancer Therapy- General (FACT-G) score. RESULTS: A total of 19 patients were recruited in group I and 10 patients in group II. The baseline demographic factors were comparable. The scores for PHQ-9 and GAD-7 were significantly higher at 3-month post-exenteration follow-up, indicating significantly higher levels of anxiety and depression after orbital exenteration in group I. Both these scores were significantly lower in group II compared to group I (3 months follow-up), indicating lower levels of anxiety and depression after rehabilitation with prosthesis. The FACT-G and its subscores were all significantly higher in group II patients compared to post-operative scores of group I patients, indicating higher overall quality of life after rehabilitation with prosthesis. CONCLUSIONS: Patients undergoing exenteration have a significant level of anxiety and depression, and lower quality of life that worsens after orbital exenteration.

Effect of legislation and changing trends of Diwali ocular firecracker injuries in Northern India.

OBJECTIVE: The objective of this study is to study the impact of temporary legislation implemented during Diwali on ocular firecracker injuries in Delhi-National Capital Region (NCR) region. MATERIALS AND METHODS: In this retrospective study, records of the patients presenting with ocular firecracker injury during 10 days of Diwali (prediwali, Diwali and 8 days postdiwali) from 2014 to 2019 were retreived from the medical record section. Clinico-demographic profile, relevant history pertaining to the use of firecracker, and details of eye examination, namely presenting visual acuity (VA), laterality and classification of injury based on Birmingham Eye Trauma Terminology System were noted. RESULTS: Six hundred and sixty-seven eyes of 646 patients were recruited. The median age of patients was 14 years (R = 3-65 years) and male: Female ratio was 4.9:1. Majority of patients (57.7%; n = 373) were from outside Delhi. Most of them were open globe injuries (66.8%). In 55.4% (n = 356) eyes, presenting VA was perception of light to finger counting close to face and 28% (n = 180) eyes could not perceive any light. A significant reduction was noted in the number of ocular firecrcaker injuries after regulation (preregulation [2014-2016] n = 456; postregulation [2017-2019] n = 211; [P = 0.01, t-test]). During preregulation period, presenting VA was worse than 1/60 in significantly higher number of eyes (85.5%; n = 376) than post regulation (78.8%, n = 160) (P = 0.03, Chi-square test). CONCLUSION: Although temporary legislation seems to be effective in reducing ocular firecracker injuries (52.7% reduction) in NCR, uniform implementation of stricter laws nationwide is essential to further minimize the problem.

Peripheral ulcerative keratitis.

Peripheral ulcerative keratitis (PUK) is an inflammatory condition of the peripheral cornea with hallmark features of epithelial defects and stromal destruction as a result of a complex interplay of factors including host autoimmunity and the peculiar anatomic and physiologic features of the peripheral cornea and environmental factors. PUK may be the result of local or systemic causes and infectious or noninfectious causes. Arriving at a specific etiological diagnosis requires a meticulous clinical workup that may include a battery of laboratory and radiological investigations. Management by a team of internists or rheumatologists and ophthalmologists and judicious use of immunosuppressive agents may yield favorable results minimizing adverse effects. We review current clinical knowledge on the diagnosis and management of PUK.