A Brief Review on Adult-Onset Coats' Disease.

Adult-onset Coats disease is an uncommon and vision-threatening disease characterized by the development of abnormal blood vessels in the retina. Coats' disease commonly affects children in the first decade of life, but very rarely manifests in adults after the third decade of life, or who characteristically present with unilateral vision loss. Despite being a sight-threatening disease, the etiology remains inconclusive and various genetic and vascular abnormalities are implicated. Diagnosis relies on ophthalmologic examination, fundus photography, fluorescein angiography, and optical coherence tomography. Treatment modalities include laser photocoagulation, intravitreal injections of anti-vascular endothelial growth factor agents, and, in advanced cases, surgical interventions are needed and the treatment is aimed at avoiding complications like retinal detachment and neovascular glaucoma, which were comparatively rare in adult-onset Coats' disease. Despite therapeutic advancements, the prognosis varies, with some patients experiencing significant visual impairment. This review outlines the clinical features, diagnosis, management, and prognosis of adult-onset Coats' disease, underscoring the importance of early detection and intervention in optimizing visual outcomes.

Severe Gliosis Over a Closed Macular Hole Following Anterior Lens Capsular Flap Closure.

Various management strategies, including the use of autologous and allogenic materials, are described for the management of persistent macular holes. An anterior lens capsular flap can be used, especially when cataract surgery is also planned, for a persistent full-thickness macular hole. We report a case of a gentleman in his 60s who underwent anterior lens capsular flap closure for a persistent macular hole. There was an improvement in visual acuity. However, he developed severe gliosis over the closed hole in the postoperative period. This could be due to the proliferation of residual epithelial cells in the lens capsule, micro damage to the retina, or an exaggerated inflammatory response to a foreign tissue placed over the retinal surface.

Exudative vascular anomalous complex-like lesion of the peripapillary area.

PURPOSE: Perifoveal exudative vascular anomalous complex is an uncommon retinal lesion of idiopathic origin characteristically described as an isolated, aneurysmal lesion of the perifoveal region. We report an unusual presentation of an exudative vascular anomalous complex-like lesion affecting the peripapillary area. CASE DESCRIPTION: A 69-year-old diabetic woman presented with blurred vision in her left eye for two months. Fundus examination of the left eye showed a small, reddish-orange lesion just supratemporal to the optic disc with perifoveal hard exudates and retinal thickening. Fundus fluorescein angiography demonstrated a peripapillary hyperfluorescent lesion with minimal leakage. Ocular coherence tomography showed an oval structure extending throughout the outer and inner plexiform and nuclear layers with a hyper-reflective wall accompanied by subfoveal and intraretinal fluid suggestive of an eVAC-like lesion in the peripapillary area. Intravitreal anti-vascular endothelial growth factor injection was given, and the lesion persisted even three weeks after the injection. Later, focal laser photocoagulation of the aneurysmal lesion was done. At 6 weeks follow-up, a complete resolution of the aneurysmal lesion with a marked decrease in retinal edema and an improvement of the visual acuity was observed. CONCLUSION: Exudative intraretinal aneurysmal lesions can occur in areas other than perifoveal area. OCT is an useful investigation for knowing their characteristics and the response to treatment. These aneurysmal lesions irrespective of their location can be called by the name eVAC-like or more aptly retinal capillary macro aneurysms (RCM).

Simplifying biometry in oil-filled eyes: A novel formula for axial length calculation in eyes with 1000 cSt silicone oil.

Purpose: To derive a formula for accurate axial length (AL) assessment using routine ultrasound in silicone oil-filled eyes, where optical biometry is unavailable or not possible. Methods: This was a prospective, consecutive, nonrandomized study of 50 eyes of 50 patients conducted in a tertiary care hospital in North India. AL measurements were taken using both manual A-scan and IOL master, both in silicone oil-filled status and 3 weeks after silicone oil removal. A correction factor of 0.7 was used for AL adjustment in oil-filled eyes. The corrected AL (cAL) was compared with IOL master values in oil-filled eyes. Agreement analysis was carried out using Bland Altman plot. Linear regression analysis was done using uncorrected manual AL to find a new equation. Data was analyzed using Stata 14. A P value <0.05 was taken as significant. Results: The study included 40 males and 10 females, with an age range of 6-83 years (mean 41.9 years). The mean AL of the oil-filled eye as measured by manual A-scan was 31.76 mm ± 3.09 and by IOL master was 24.7 mm ± 1.74. Linear regression analysis was performed in randomly selected 35 eyes of the observed data to obtain a new equation: predicted AL (PAL) = 14 + 0.3 × manual AL. The mean difference between PAL and optically measured AL with silicone oil in situ was 0.98 ± 1.67. Conclusion: We propose a new formula for better prediction of the correct AL in silicone oil-filled eyes using ultrasound-based AL measurement.

Neovascular glaucoma with combined retinal vascular occlusion in carotid cavernous fistula.

Carotid cavernous fistulas (CCFs) can present with varied ophthalmic manifestations. The most important vision-threatening complications of CCF include glaucoma and retinal vascular occlusions. We report a case of a man in his early 30s who developed a post-traumatic direct CCF. The patient denied undergoing embolisation therapy. This resulted in aggravation of his condition with onset of combined retinal venous and artery occlusion leading to neovascular glaucoma and severe vision loss. He was treated with medical management followed by diode laser photocoagulation to control intraocular pressure. Diagnostic cerebral angiography done 3 months later showed complete closure of the fistula; hence, no further intervention was advocated. Combined vascular occlusion is a rare vision-threatening occurrence in cases of CCF. Timely intervention with closure of the fistula can prevent the development of vision-threatening complications.

Normative data of retinal arteriolar and venular calibre measurements determined using confocal scanning laser ophthalmoscopy system - Importance and implications for study of cardiometabolic disorders.

Purpose: To determine and validate retinal vascular caliber measurements by using the confocal scanning laser ophthalmoscopy system. Retinal vasculature changes are often regarded as clinical markers for systemic disease. Methods: It was a prospective observational study conducted on 600 eyes of 300 normal subjects with no systemic or ocular illness from January 1, 2016 to June 30, 2017 in a tertiary referral eye center. Non-mydriatic infrared reflectance, blue reflectance, and blue peak blue autofluorescence fundus imaging were done on the confocal scanning laser ophthalmoscopy system. The dimensions of the retinal vessels were measured using inbuilt calipers at 1800 µm from the center of the optic disc. Internal and external dimensions were measured. Observer variation and its comparison using Image J software were assessed. Results: The median age was 29 years (18-50 years). Mean internal and external diameters for arterioles were 85.1 ± 12.4 µm and 105.0 ± 12.0 µm, and for venules were 133.8 ± 16.6 µm and 145.4 ± 16.1 µm, respectively. The mean internal and external wall thicknesses were 19.7 ± 8.0 µm and 11.0 ± 5.6 µm, and wall thickness-to-lumen ratios were 0.3 ± 0.1 and 0.1 ± 0.1, respectively. Arteriolar-to-venular ratio for lumen and vessel was 0.66 ± 0.1 and 0.74 ± 0.1, respectively. There was no statistically significant difference between age groups. Both inter- and intra-observer reproducibility was >95%. The Bland-Altman plot showed that the difference between measurements using both confocal scanning laser ophthalmoscopy and Image J software lies within the limits of agreement approximately 95% of the time. Conclusion: This is the first effort to develop a normative database by using a simple non-invasive confocal scanning laser ophthalmoscopy system with high observer reproducibility.

Chronic myeloid leukaemia accelerates proliferative retinopathy in patients with co-existent diabetes: A risk factor not to be ignored.

OBJECTIVE: To describe the retinal imaging characteristics, retinopathy management strategies and visual outcomes in cases of diabetes with chronic myeloid leukaemia. DESIGN: Retrospective observational study. PARTICIPANTS: Patients with diabetes and chronic myeloid leukaemia managed at our tertiary eye care centre from January 2015 to December 2017. METHODS: Detailed ophthalmic and systemic evaluation, treatment and follow-up records were reviewed. The main measures studied were visual acuity, intra-ocular pressure, retinopathy severity, and surgical indications and techniques. RESULTS: Of the six patients studied, three had diabetes and chronic myeloid leukaemia at presentation, while in three cases chronic myeloid leukaemia was diagnosed following evaluation for proliferative retinopathy. The visual acuity ranged from 20/20 to perception of light. All eyes had marked proliferative retinopathy out of proportion to the exudation. None of the eyes had significant macular oedema. Pan-retinal photocoagulation (10/12, 83.33%), intravitreal anti-vascular endothelial growth factor injection (8/12, 66.67%), vitrectomy (2/12, 16.67%), cataract surgery (2/12, 16.67%) and trabeculectomy followed by cryoablation (2/12, 16.67%) was performed for management of the ocular disease as indicated. Median follow-up was 16.5 months (range: 6-24 months). Final visual acuity ranged from PL to 20/20 with acuity ⩾ 20/100 in eight eyes. Four eyes had advanced optic neuropathy from neovascular glaucoma. CONCLUSION: Accelerated proliferative retinopathy can be seen in cases of diabetes with chronic myeloid leukaemia at the very initial ophthalmic evaluation. Thus, there is a need to alter screening guidelines for retinopathy in cases of diabetes with chronic myeloid leukaemia. Early detection and aggressive management may help preserve visual acuity in such cases.

In vivo optical biopsy of choroidal osteoma: a swept source optical coherence tomography-based tumor characterization.

OBJECTIVE: To study tumor characteristics of choroidal osteoma by swept source optical coherence tomography. METHODS: A retrospective case series done at tertiary referral center in northern India. All patients diagnosed with choroidal osteoma examined on swept source optical coherence tomography were included. Swept source optical coherence tomography images were analyzed for integrity of retinal layers-intraretinal layers, outer retinal layers (photoreceptor), retinal pigment epithelium, and contour abnormalities. Choroidal changes assessed were tumor attributes such as shape, depth of choroidal involvement, tumor mass reflectivity pattern, tumor vascularity, and evolutionary structural abnormalities such as deossification, focal depressions, or choroidal neovascular membrane. RESULTS: A total of 15 eyes of 11 patients were analyzed. Seven of 11 patients were females. Mean age of presentation was approximately 26 years. Tumor was large in nine cases (>7.5 mm). Deossification was seen in 12 eyes. Inner and outer retinal integrity was maintained in 7 and 2 eyes, respectively. Most common internal tumor reflectivity pattern seen was a lamellar appearance (12/15). Increased signal transmission to choroid and focal area of deep excavation was present in 11 and 4 eyes, respectively. Osteoclastic activity was noted in 12 eyes. Choroidal neovascular membrane was seen in 6 eyes. Small lesions showed lamellar pattern of tumor reflectivity with preservation of retinal pigment epithelium and overlying retina. Larger tumors were deossified with 6 irregular tumor contour, disorganization of the outer retina, increased signal transmission to choroid, and areas of osteoclastic activity. CONCLUSION: Swept source optical coherence tomography was helpful in assessing tumor attributes and predicting the different timelines in tumor evolution.

Posterior segment complications following glaucoma surgeries.

The present review describes the posterior segment complications following surgical management of glaucoma. Although the majority of glaucoma cases are managed medically, still a large number of patients may require surgery. Moreover, with the advent of newer surgical techniques and adjuncts, encountering retinal complications post-surgery is not uncommon. The incidence, signs, management, and prognosis of common complications such as anesthesia-related retinal toxicity, vitreous loss, suprachoroidal hemorrhage, serous choroidal detachment, hypotonic maculopathy, vitreous hemorrhage, retinal detachment and endophthalmitis will be discussed in detail. Awareness of these complications is necessary as their proper and timely management can save vision in an already compromised eye.

COMPARATIVE EVALUATION OF ANTERIOR SEGMENT OPTICAL COHERENCE TOMOGRAPHY, ULTRASOUND BIOMICROSCOPY, AND INTRAOCULAR PRESSURE CHANGES AFTER PANRETINAL PHOTOCOAGULATION BY PASCAL AND CONVENTIONAL LASER.

PURPOSE: To compare intraocular pressure, anterior segment optical coherence tomography, and ultrasound biomicroscopy parameters over 3 months after panretinal photocoagulation (PRP) for proliferative diabetic retinopathy after 1 of 2 sittings by conventional laser (half PRP) and a single sitting of Pattern Scan Laser (PASCAL) PRP. METHODS: This was a prospective, randomized, interventional study. All tests were performed at baseline, and at 1, 6, and 24 hours, and 1, 4, 8, and 12 weeks after PRP. RESULTS: The intraocular pressure at 1 hour and 6 hours after PRP was significantly raised in both groups. Mean intraocular pressure was 21.17 ± 4.01 mmHg after PASCAL and 17.48 ± 3 mmHg after conventional laser at 1 hour, P < 0.001. On anterior segment optical coherence tomography, conventional laser PRP caused a more significant narrowing of angle-opening distance (AOD750) and trabecular-iris space area (TISA 500), P = 0.03 and 0.04, respectively, on Day 1. Ultrasound biomicroscopy showed a significantly narrow angle in both groups on Day 1. A significant increase in ciliary body thickness was observed in both groups, with 57.1% of PASCAL and 100% of conventionally treated eyes showing ciliary effusion on Day 1 that decreased but persisted for the next 3 months. CONCLUSION: Performing PRP in sittings, prescribing previous glaucoma medications in patients at risk, and recording intraocular pressure an hour after the PRP could decrease complications.

Swept-source optical coherence tomography features of regressed macular retinoblastoma.

Purpose: To describe the swept-source optical coherence tomography (SS-OCT) features of regressed macular retinoblastoma (RB). Methods: A cross-sectional observational study was carried out in 13 patients with regressed macular RB with good fixation in at least one eye. Fundus photography and SS-OCT were documented. High-resolution scans with good signal strength were selected. The types of clinical regression and SS-OCT characteristics of the regressed lesions (presence of vitreous detachment, intratumor schisis/cavitation, calcification, foveal dip, and OCT pattern) were noted. Results: Of the 13 eyes, 7 (53%) were group B, 4 (30%) were group C, and 2 (17%) were group D. Lesion involving fovea was seen in seven eyes (53%). On SS-OCT, the lesion was isodense to hyperdense in all cases. Three patterns of regressed RB were noted on OCT. Intralesion calcification was noted in eight cases. Subretinal fluid was not detected in any of the cases. Conclusion: SS-OCT is a useful technology to image and analyze cases of regressed macular RB including large lesions. SS-OCT system helps in successful imaging even in smaller children.

Occurrence of Hemi-Central Retinal Artery Occlusion Following Embolization in a Case of Recurrent Juvenile Nasopharyngeal Angiofibroma.

Preoperative embolization is currently widely followed in the treatment of juvenile nasopharyngeal angiofibroma (JNA) to reduce intraoperative bleeding. However, embolization can result in untoward complications like stroke and blindness. The authors present the case of a 14-year-old boy with recurrent JNA who developed hemi-central retinal artery occlusion after embolization of branches of the external carotid artery. It was managed by intraocular pressure reduction and globe massage to dislodge the possible embolus. At 4 weeks' follow-up, there was near complete recovery in visual acuity; however, residual hemi-field defect persisted. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:385-387.].

Ultra-widefield fundus fluorescein angiography in pediatric retinal vascular diseases.

Purpose: To describe the utility of RetCam ultra-wide-field fundus fluorescein angiography in pediatric retinal vascular diseases. Methods: A retrospective chart review was carried out in 43 eyes of 22 pediatric patients who were diagnosed or suspected to have a retinal vascular disease. Fluorescein angiography was carried out using the 130 degree lens of RetCam 3. Fluorescein angiography guided treatment (laser/cryotherapy) was carried out wherever required. Results: Diseases studied included - coats disease, familial exudative vitreoretinopathy, retinopathy of prematurity, congenital retinal folds, double optic nerve head, persistent fetal vasculature and incontinentia pigmenti. RetCam assisted fluorescein angiography was helpful in establishing a diagnosis in 4 patients (18%), in decision making regarding treatment in 18 patients (82%), in deciding need for retreatment in 5 patients (23%), helped in staging of disease in 5 patients (23%) and in detecting clinically subtle findings in 6 patients (27%). Conclusion: RetCam assisted FFA is extremely useful to document peripheral retinal vascular pathologies in pediatric patients and helps to take crucial therapeutic and retreatment decisions.