Visual impairment and blindness due to retinitis pigmentosa in India: 15-year follow-up of the Andhra Pradesh Eye Disease Study cohort

Purpose: To assess the incidence, visual impairment, and blindness due to retinitis pigmentosa (RP) in a rural southern Indian cohort. Methods: This is a population?based longitudinal cohort study of participants with RP from the Andhra Pradesh Eye Disease Study (APEDS) cohorts I and III, respectively. The study included participants with RP of APEDS I who were followed until APEDS III. Their demographic data along with ocular features, fundus photographs, and visual fields (Humphrey) were collected. Descriptive statistics using mean ± standard deviation with interquartile range (IQR) were calculated. The main outcome measures were RP incidence, visual impairment, and blindness as per the World Health Organization (WHO) definitions. Results: At baseline (APEDS I), 7771 participants residing in three rural areas were examined. There were nine participants with RP with a mean age at baseline of 47.33 ± 10.89 years (IQR: 39–55). There was a male preponderance (6:3), and the mean best?corrected visual acuity (BCVA) of 18 eyes from nine participants with RP was 1.2 ± 0.72 logarithm of minimum angle of resolution (logMAR; IQR: 0.7–1.6). Over a mean follow?up duration of 15 years, 5395/7771 (69.4%) were re?examined, which included seven RP participants from APEDS 1. Additionally, two new participants with RP were identified; so, the overall incidence was 370/ million in 15 years (24.7/million per year). The mean BCVA of 14 eyes of seven participants with RP who were re?examined in APEDS III was 2.17 ± 0.56 logMAR (IQR: 1.8–2.6), and five of these seven participants with RP developed incident blindness during the follow?up period. Conclusion: RP is a prevalent disease in southern India that warrants appropriate strategies to prevent this condition.

Clinical presentation and treatment outcomes of an algorithmic approach to uveal effusion syndrome

Purpose: To describe the clinical features and treatment outcomes in spontaneous uveal effusion syndrome (UES). Methods: A 10?year retrospective chart review of UES patients from a tertiary eye center was carried out. Optical coherence tomography (OCT), fundus fluorescein angiography, and ultrasound biomicroscopy (UBM) scans were performed. UES was managed based on presenting best?corrected visual acuity (BCVA), symptoms, and fundus findings. Patients with secondary causes of uveal effusion were excluded. Results: Twenty?five eyes of 16 patients were included. Of the 16 patients, 14 (88%) were male and 9 (56%) had bilateral disease. Fifteen of 25 affected eyes had nanophthalmos (axial length (AL) <20.5 mm) and 6 had hyperopia with AL >20.5 mm. The presenting mean distance BCVA was 0.74 ± 0.64 logMAR (mean Snellen: 20/100). Eleven eyes had exudative retinal detachment, and 4 also had exudative choroidal detachment (CD). Choroidal thickness (CT) was increased in 11 eyes on B?scan ultrasonography, and the mean CT was 1.74 ± 0.38 mm. Sub?retinal fluid (SRF) and retinal folds were the most common OCT findings. UBM findings included shallow angles, peripheral CD, and supra?ciliary effusion. A combination of local and systemic corticosteroids was used to successfully treat 12 eyes, 6 needed surgery, and 7 were observed. Partial sclerectomy with anterior chamber maintainer?assisted SRF drainage was the favored surgery. The median period of follow?up was 6.5 months (0.1–76 months), and the mean distance BCVA at the last follow?up was 0.58 ± 0.42 logMAR (mean Snellen: 20/80). Conclusion: UES can be suitably managed both medically and surgically based on clinical presentation

Effectiveness and future implications of COVID-19-related risk stratification for managing retinopathy of prematurity: The Indian twin cities retinopathy of prematurity study report number 10

Purpose: To evaluate the effectiveness and future implications of COVID?related risk stratification for managing retinopathy of prematurity (ROP). Methods: A prospective study was conducted at a tertiary eye care center from the beginning of the lockdown in India from 23 March 2020 till the end of the first phase of lockdown on 29 May 2020. We evaluated 200 prematurely born infants (< 34 weeks of gestational age) using the new safety guideline protocols for low?risk babies developed in conjunction with the Indian ROP Society for care during the COVID?19 pandemic. Low risk included babies born at more than 30 weeks of gestational age, post menstrual age 34 weeks or above at presentation, more than 1000 grams of birth weight, and stable systemically with good weight gain. Results: New guidelines were implemented in 106 (53%) infants who were low risk while 94 (47%) infants with high risk were followed up as per the old guidelines. Out of the 106 infants (212 eyes) managed by the new guidelines, good outcome (group 1) was seen in 102 (96.2%) infants. Twenty?seven of the 102 infants had some form of ROP and 5 of these infants needed treatment. None of the low?risk babies with no detachment at presentation managed by new guidelines required surgery later (group 2). Two (1.9%) infants came with retinal detachment at presentation and underwent successful surgery (group 3) and two infants (1.9%) were lost to follow up. Conclusion: New risk stratification during the COVID?19 pandemic was an efficient and safe strategy in managing low?risk ROP babies.

Retinitis pigmentosa in Usher syndrome in India: Electronic medical records driven big data analytics: Report III

Purpose: To describe the clinical presentation and demographic distribution of retinitis pigmentosa (RP) in patients with Usher syndrome (USH). Methods: This is a cross?sectional observational hospital?based study including patients presenting between March 2012 and October 2020. In total, 401 patients with a clinical diagnosis of USH and RP in at least one eye were included as cases. The data were retrieved from the electronic medical record database. For better analysis, all 401 patients were reclassified into three subtypes (type 1, type 2, and type 3) based on the USH criteria. Results: In total, there were 401 patients with USH and RP, with a hospital?based prevalence rate of 0.02% or 2/10,000 population. Further, 353/401 patients were subclassified, with 121 patients in type 1, 146 patients in type 2, and 86 patients in the type 3 USH group. The median age at presentation was 27 years (IQR: 17.5–38) years. There were 246 (61.35%) males and 155 (38.65%) females. Males were more commonly affected in all three subtypes. Defective night vision was the predominant presenting feature in all types of USH (type 1: 43 (35.54%), type 2: 68 (46.58%), and type 3: 40 (46.51%) followed by defective peripheral vision. Patients with type 2 USH had more eyes with severe visual impairment. Conclusion: RP in USH is commonly bilateral and predominantly affects males in all subtypes. Patients with USH and RP will have more affection of peripheral vision than central vision. The key message of our study is early visual and hearing rehabilitation in USH patients with prompt referral to otolaryngologists from ophthalmologists and vice versa.

Retinitis pigmentosa in Laurence-Moon-Bardet-Biedl syndrome in India: Electronic medical records driven big data analytics: Report II

Purpose: To describe the clinical presentation and demographic distribution of retinitis pigmentosa (RP) in Laurence–Moon–Bardet–Biedl (LMBB) syndrome patients. Methods: This is a cross?sectional observational hospital?based study wherein 244 patients with RP in LMBB syndrome presenting to our hospital network between March 2012 and October 2020 were included. An electronic medical record database was used for data retrieval. Results: There were 244 patients in total, with a hospital?based prevalence rate of 0.010% or 1000/100,000 population. The mean and median age of patients was 15.22 ± 7.56 and 14 (IQR: 10–18.5) years, respectively, with the majority being in the age group of 11–20 years (133/244 patients; 54.50%). Males were more commonly affected (164 patients; 67.21%), and the majority (182 patients; 74.59%) were students. All 244 patients (100%) complained of defective central vision at presentation. More than one?fourth of the patients had severe visual impairment to blindness at presentation. Prominent retinal feature at presentation was diffuse or widespread retinal pigment epithelial degeneration in all patients. Conclusion: Patients with RP in LMBB syndrome present mainly in the first to second decade of life with severe visual acuity impairment to blindness early in life. It is important to rule out LMBB syndrome in early?onset RP with central visual acuity impairment. On the contrary, all patients diagnosed or suspected with LMBB syndrome systemic features at physician clinic should also be referred for ophthalmic evaluation, low vision assessment, rehabilitation, and vice versa

Clinical and visual electrophysiological characteristics of vitelliform macular dystrophies in the first decade of life

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

Exudative retinal detachment as an initial presentation of retinopathy of prematurity: Clinical profile and outcomes of a rare presentation

Purpose: To describe the clinical profile and treatment outcomes of infants with retinopathy of prematurity (ROP) presenting with isolated exudative retinal detachment (ERD). Methods: Retrospective interventional case series. Preterm infants diagnosed with ROP with ERD at presentation were included. All demographic details, clinical findings, and treatment given were documented. The anatomical outcome was categorized as good, fair, and poor. Refractive outcome was classified into mild, moderate, and severe according to spherical equivalent at the last visit. Results: Fifteen eyes (8 patients) were included. Mean gestational age was 31.3 weeks, and birth weight was 1462.6 g. All eyes presented with aggressive ROP. Patches of retinal edema in avascular retina were seen in all eyes. A total of 86.6% of eyes had vascular sclerosis while 86.6% of eyes had subretinal exudates. The anatomical outcome was good in all eyes. In addition, 40% of eyes had a mild refractive error. Conclusion: Exudative retinal detachment in ROP is rare. The use of unregulated oxygen can be a contributory factor. Vascular sclerosis is consistent with hyperoxia?induced retinopathy models. Retinal edema and subretinal exudates indicate disrupted inner and outer blood?retinal barrier. Treatment outcomes are good when diagnosed and treated in time.

Evaluation of a revised care plan for babies with retinopathy of prematurity during SARS-CoV-2 pandemic in India

Purpose: To analyze the impact of a revised care plan for retinopathy of prematurity (ROP) during SARS?CoV?2 pandemic in a tertiary eye care facility in eastern India. Methods: In a retrospective study, we analyzed the medical records of babies managed for ROP during the peak of the SARS?CoV?2 pandemic, with particular reference to the challenges, and the revised strategies addressing travel restrictions for five months, from April to August 2020. The strategy included selective referral (babies with higher treatment probability), longer follow?up intervals (babies with non?alarming findings), use of locally available workforce, and teleconsultation whenever feasible. Results: In the given period, 222 babies were examined versus 624 in the preceding year (P = 0.001). The average gestational age, birth weight, and postmenstrual age at presentation were 30.4 weeks, 1.31 kg, and 37.7 weeks, respectively. The first examination was on time in 40.1% of babies but was delayed by a median of 23 days in the remaining babies. In the cohort, 56.7% of babies had any ROP, and 27.9% required treatment (versus 8.8% in the previous year; P < 0.001). The intravitreal anti–vascular growth factor (anti?VEGF) injection was more often used than in the previous year (n = 72 vs 36; P < 0.0001). The treatment outcome was comparable before and after the SARS?CoV?2 lockdown period. There was no report of health issues among the care providers attributable to ROP care. Conclusion: The revised strategy resulted in a smaller pool of babies screened but a larger proportion of babies treated for ROP. This strategy could be used more profitably in future ROP care.

The retinal vascular growth rate in babies with retinopathy of prematurity could indicate treatment need

Purpose: To analyze the weekly rate of retinal vascular growth in treatment?na飗e babies with various stages of retinopathy of prematurity (ROP) and validate if this could be a predictor of treatment need. Methods: Retrospective review of medical charts and retinal images of babies with various stages of ROP. The images were enhanced using red?green image enhancement software. Using the length of the horizontal disc diameter (DD) of each eye, the vessel growth was measured from the disc margin up to the vessel tip in fixed quadrants. The rate of vessel growth was the ratio of vessel length to the number of weeks it took to reach this length. The babies were divided into treatment warranting ROP (group 1), low?risk pre?threshold (type II) ROP (group 2,), and no?ROP (group 3) for analysis. The 搉o?ROP� group acted as normal control. Group 1 was further subdivided into 1A (threshold ROP), IB (aggressive posterior ROP), 1C (hybrid ROP), and ID (high?risk pre?threshold ROP). Results: Out of 436 eyes, groups 1, 2, and 3 had 238, 108, and 90 eyes, respectively. The mean rate of vascular outgrowth along with 95% confidence interval (CI) was 0.490 [0.487,0.520], 0.612 [0.599, 0.638], and 0.719 [0.703, 0.740] DD/week, respectively, for babies with 搕reatment warranting,� 搇ow risk pre?threshold� and 搉o ROP� groups, respectively. In our estimate, more than 80% of eyes with a vessel growth rate of 0.54 DD/week or less required treatment. Conclusion: A rate of retinal vascular growth less than 0.54 DD/week can be used to determine treatment requirements in babies with ROP

Compassionate and standard operating procedure-based counseling and practice are essential in high-risk infantile anesthesia and managing death in eye surgery

Physicians have to play the role of a team leader and counselor and take written informed consent for high?risk surgeries in many cases. This is the first step toward initiating a mutually trustworthy relationship with the patient and family. The situation is more sensitive when vulnerable patients like small premature babies or the elderly are under consideration. In the event of a death, leadership and processes in place become extremely critical. We share our experience and practice pattern during this process, especially suited to India, but the broad principles would apply to most human situations. Hopefully, some of these can be incorporated into the existing training curriculum for team building and the art of effective physician?patient communication that should be intricately woven into the curriculum for the Science of Ophthalmic care.

Choroidal thickness profile in inherited retinal diseases in Indian subjects.

Purpose: To evaluate changes in choroidal thickness (CT) in inherited retinal diseases and its relationship with age, spherical equivalent, visual acuity, and macular thickness. Methods: Retrospective analysis of 51 eyes with features of retinal dystrophy of 26 subjects, who underwent enhanced depth imaging using spectral domain (SD) optical coherence tomography (OCT), were included. The CT measurements were made at the fovea and at 5 points with an interval of 500 microns in both directions, nasal and temporal from the fovea and were compared with age‑matched healthy subjects. Step‑wise regression was used to find the relationship between age, spherical equivalent, best‑corrected visual acuity (BCVA), central macular thickness (CMT), and subfoveal CT. Results: Disease distribution was as follows: Stargardt’s disease 18 eyes (9 subjects); Best disease 5 eyes (3 subjects); cone‑rod dystrophy 26 eyes (13 subjects); and Bietti’s crystalline dystrophy 2 eyes (1 subject). Mean subfoveal CT was 266.33 ± 76 microns. On regression analysis, no significant correlation was found between subfoveal CT and any other variable such as age (P = 0.9), gender (P = 0.5), CMT (P = 0.1), spherical equivalent (P = 0.3) and BCVA (P = 0.6). While comparing with age‑matched healthy subjects, no significant statistical difference was noted (P < 0.05) among all age groups. Conclusion: Our study reports quantitative changes in CT in various common inherited retinal diseases seen in Indian populations. To validate changes in choroid, a longitudinal study with larger sample size is warranted.

Impact of the day-30 screening strategy on the disease presentation and outcome of retinopathy of prematurity. The Indian twin cities retinopathy of prematurity report number 3.

Context: Outcomes of various screening strategies in retinopathy of prematurity are not well reported. Aim: To assess the impact of a city-wide, ROP screening strategy, on the disease presentation and treatment outcome. Materials and Methods: A retrospective case-control study from a prospectively collected ROP data-base was analyzed. Cases (group 1a) included ROP babies that were screened directly in neonatal intensive care units, and controls (group 1b) were babies referred directly to the institute from other neonatal centers during the same period. Historical controls (group 2) were ROP cases seen in the years preceding establishment of this ROP program and database. Primary outcome measure was the risk of eyes presenting with stage 4 or worse ROP, and main secondary outcome measure was the fi nal anatomic outcome. Results: Of the 643 cases screened, 322 eyes of 161 babies had ROP. The median age of 7.19 months at presentation for the 46 patients (92 eyes) in group 2 was higher than the median age of 1.29 months for the 115 patients (230 eyes) in group 1. Within the group 1, group 1a had lower median age at presentation than group 1b (0.91 months versus 2.30 months). The relative risk of an eye presenting in the stage 4 and 5 in group 2 was 4.7 times higher (95% confi dence interval 3.07 - 7.32) than in group 1. Eyes that could be given treatment in group 2 were signifi cantly less (P < 0.0005) than in group 1. The relative risk of poor outcome in group 2 was 3.83 times higher (95% confi dence interval 2.75 - 5.34) than in group 1. Group 1a eyes had the best outcomes. Conclusion: Early screening before one month of age in neonatal centers detects the disease early where prompt treatment can lead to favorable outcomes. The study provides early results of a model strategy for ROP screening.

Traumatic endophthalmitis presenting as isolated retinal vasculitis and white-centered hemorrhages: Case report and review of literature.

The article reports a case and review of the literature of endophthalmitis presenting as isolated retinal vasculitis. A 26-year-old male was observed to have white-centered retinal hemorrhages and retinal vasculitis following an occult scleral perforation. At presentation, the visual acuity was 20/60. With clinical suspicion of early endophthalmitis, he underwent wound exploration, scleral tear repair, vitreous biopsy and administration of intravitreal antibiotics. Microbiology evaluation revealed significant presence of methicillin-resistant coagulase-negative Staphylococcus epidermidis. Final visual acuity improved to 20/20 at 6 weeks postoperatively. Literature search revealed eight similar cases, all of them due to Staphylococcus species. Retinal vasculitis and white-centered retinal hemorrhages can be a presenting sign of early endophthalmitis, especially with non-fulminant pathogens like S. epidermidis.

Comparison of immersion ultrasonography, ultrasound biomicroscopy and anterior segment optical coherence tomography in the evaluation of traumatic phacoceles.

Blunt ocular trauma in the elderly can result in anterior dislocation of the crystalline lens into the subconjunctival space (phacocele). Although rare, this presentation can be missed, especially if the patient presents several days after the injury and if the lid is not everted on examination. While a careful clinical examination is adequate in the diagnosis, imaging techniques can be put to use for the accurate location of the associated sclera rupture. We report three cases of post-traumatic phacocele wherein ultrasound biomicroscopy (UBM) was compared to the anterior segment optical coherence tomography (AS-OCT) and B-scan ultrasonography (B-scan), in order to establish the best imaging tool for this condition. We concluded, based on image quality, that UBM could be the imaging modality of choice to aid in the diagnosis of phacocele.

Technical aspects of laser treatment for acute retinopathy of prematurity under topical anesthesia.

Retinopathy of prematurity (ROP) is a significant cause of childhood blindness. The criteria for laser therapy have been revised from threshold ROP to include the earlier stage of high-risk prethreshold ROP. Laser photocoagulation is an established technique for the treatment of ROP. However, the detailed procedure and techniques for laser photocoagulation have not yet been published. Adequate and appropriate laser photocoagulation for ROP is different from the application of lasers in adult retinal vascular diseases, and many ophthalmologists need to be trained in this technique if the outreach of ROP treatment programs is to improve. Laser under topical anesthesia has been practiced in India as a preferred modality especially due to logistics and risks of general anesthesia in these pre-term babies. We discuss the details of the technique as practiced at tertiary care ophthalmic hospitals in India, so that the nuances in treatment parameters and clinical decision-making can be usefully applied to ophthalmic practice. This will ultimately lead to safe and effective treatment delivery in ROP.

Acute postoperative endophthalmitis by Gemella haemolysans.

Endophthalmitis is a rare and serious post-surgical complication. We report a case of acute postoperative endophthalmitis after an uneventful cataract surgery caused by a commensal organism, Gemella haemolysans. The patient was successfully treated with vitrectomy and intravitreal antibiotics like vancomycin, along with topical cefazolin.

Retinal pigment epithelial atrophy following indocyanine green dye-assisted surgery for serous macular detachment.

To report subretinal migration of indocyanine green dye (ICG) and subsequent retinal pigment epithelial (RPE) atrophy during macular surgery for serous macular detachment. A 65-year-old woman presented with residual epiretinal membrane and serous detachment of the macula following vitreoretinal surgery for epiretinal membrane. She underwent resurgery with ICG-assisted internal limiting membrane peeling and intraocular tamponade. Intraoperatively a large area of subretinal ICG was seen with subsequent RPE mottling and atrophy of the macula in the area involved during follow-up. This case demonstrates that subretinal migration of ICG is possible and can be toxic to RPE.

Severe hemorrhagic retinopathy as initial manifestation of acute retinal necrosis caused by herpes simplex virus.

A case of bilateral acute retinal necrosis due to herpes simplex virus 1, in a child is reported. The case presented as an extensive hemorrhagic retinopathy that was misdiagnosed as non-infective initially. Diagnostic aqueous tap of the blind eye for viral DNA by polymerase chain reaction helped to confirm viral etiology when the other eye was affected. Appropriate antiviral therapy followed by prompt surgeries for subsequent retinal detachment helped to salvage useful vision in the second eye.

Relationship between clinical presentation and visual outcome in postoperative and posttraumatic endophthalmitis in south central India.

PURPOSE: To determine risk factors for poor visual outcome in postoperative and posttraumatic endophthalmitis in a large referral center in south central India. METHODS: In this prospective observational series the authors examined 388 patients of postoperative (n= 206) and posttraumatic (n= 182) endophthalmitis at the L V Prasad Eye Institute in Hyderabad, India between 1991 and 1997.The analysis was confined to 236 patients-128 (62.1%) postoperative and 108 (59.3%) posttraumatic patients who were followed for a minimum period of 3 months. A detailed protocol was followed. Chi-square and logistic regression analysis were used to determine risk factors for visual outcome worse than 6/18 and worse than 6/120. RESULTS: Postoperative endophthalmitis: In univariate analysis the features associated with poor visual acuity (grouped as < 6/18 and < 6/120) included intracapsular cataract surgery, poor presenting visual acuity, presence of vitreous cells, inability to visualise the optic disc on indirect ophthalmoscopy, presence of vitreous membranes on ultrasonography, and a culture-positive vitreous biopsy. In the multivariate analysis, visual acuity of less or equal light perception (LP) at presentation was associated with a 3-month postoperative visual acuity of < 6/18, with an odds ratio of 5.85 [1.25 - 27.42, 95% CI], and vitreous membranes seen on ultrasonography was associated with a final visual acuity of < 6/120, with an odds ratio of 2.47 [1.05 - 5.83, 95% CI]. Posttraumatic endophthalmitis: In univariate analysis the features associated with poor visual acuity (grouped as < 6/18 and < 6/120) included a retained intraocular foreign body (IOFB), trauma by needle (hypodermic or sewing), poor presenting visual acuity, inability to visualise the optic disc on indirect ophthalmoscopy, presence of vitreous membranes on ultrasonography, and a culture-positive vitreous biopsy. In multivariate analysis, IOFB was associated with a 3-month follow-up visual acuity of < 6/18, with an odds ratio of 5.90 [1.85 - 18.78, 95% CI], and trauma by a needle (hypodermic or sewing) and retained IOFB was associated with a final visual acuity of < 6/120, with an odds ratio of 4.47 [1.22 - 16.38, 95%CI] and 3.76 [1.36 - 10.37, 95% CI] respectively. CONCLUSION: This is the largest, single-centre, prospective study on risk factors for poor visual outcome in postoperative and posttraumatic endophthalmitis. The independent risk factor for 3-month follow-up visual acuity of < 6/18 was the presenting visual acuity of < or =LP in postoperative endophthalmitis and a retained IOFB in posttraumatic endophthalmitis. The independent risk factor for 3-month visual acuity of < 6/120 was the presence of vitreous membranes on ultrasonography in postoperative endophthalmitis, and trauma by a needle (hypodermic/ sewing) and retained IOFB in posttraumatic endophthalmitis.