Accelerated hydroxychloroquine toxic retinopathy.

PURPOSE: To report a case series of patients with retinal toxicity due to hydroxychloroquine (HCQ) within a short span of treatment. METHODS: A retrospective review of case records of patients with accelerated HCQ toxicity within 1 year of starting the treatment was done. Systemic co-morbidities, details of HCQ treatment, details of ocular examination, and results of multimodal investigations were noted. RESULTS: Nine patients (1 male, 8 females) with age ranging from 40 to 73 years (mean 54.2 ± 13.4 years) who showed accelerated HCQ toxicity were included. None had systemic conditions or drug history predisposing to early HCQ toxicity. The treatment duration ranged from 2 to 11 months and the cumulative HCQ dose ranged from 18 to 120 g (mean 45.0 ± 33.0 g). The visual acuity was normal in 8 (88.9%) patients and retinal evaluation was normal in 4 (44.4%). Optical coherence tomography was abnormal in 4 (44.4%). Six (66.6%) cases had reduced sensitivity in the parafoveal point on visual field testing. All 9 cases had multifocal electroretinographic changes diagnostic of HCQ toxicity. The HCQ treatment was stopped in 8 and continued with reduced dose in 1 patient. The mean duration of follow-up was 11.2 ± 9.6 months during which 5 patients showed improved mfERG and 1 patient had a stable mfERG. Visual fields improvement was noted in 2 cases. CONCLUSIONS: Patients on HCQ need to be kept on regular monitoring with more frequent follow-ups to detect signs of early onset toxicity and prevent permanent visual impairment. mfERG is an important diagnostic tool for HCQ toxicity.

Outcome of sutured scleral fixated intraocular lens in Marfan syndrome in pediatric eyes.

PURPOSE: To report the clinical outcomes and complications in eyes with Marfan syndrome with subluxated/dislocated lens undergoing sutured scleral fixated intraocular lens (SSFIOL) implantation. METHODS: A retrospective review was done for a consecutive series of 73 eyes of 43 patients with Marfan syndrome, with age less than 18 years who underwent SSFIOL from July 2000 to June 2017. Postoperative best corrected visual acuity, intraocular pressure and refractive error, intraoperative and postoperative complications and time for the development of these complications were analyzed. RESULTS: Visual acuity improved significantly to 0.43 ± 0.30 (p = 0.01), 0.40 ± 0.3 (p = 0.00) and 0.39 ± 0.3 (p = 0.00) logMAR units at 6 weeks, 1 year and at last follow-up, respectively. The refractive error reduced significantly (p = 0.056, 0.039 and 0.031) at 6 weeks, 1 year and final follow-up following surgery. Intraoperative complications included iatrogenic retinal break (n = 3) and surgical trauma to the iris (n = 1). Postoperative complications included increased intraocular pressure in 3 eyes (4.1%), intraocular lens dislocation/subluxation in 5 eyes (6.8%), retinal detachment in 3 eyes (4.1%), choroidal detachment in 1 eye (1.3%), pupillary capture in 15 eyes (20.5%), vitreous hemorrhage in 1 eye (1.3%), pre-retinal hemorrhage in 1 eye (1.3%) and hyphema in 1 eye (1.3%). CONCLUSION: SSFIOL provides good visual outcomes in eyes with ectopia lentis associated with Marfan syndrome. Complications can be seen in some cases, and the parents need to be counseled regarding the same before surgical intervention.

Effect of brilliant Blue-G on cellular stress response in retinal pigment epithelial cells: In vitro.

To assess the cellular stress evoked by exposure of Brilliant Blue-G (BBG), adult retinal pigment epithelial (ARPE-19) cells were treated with various dilutions of BBG in balanced salt solution plus (BSS-PLUS) with and without endoillumination (Alcon Constellation Vision System). The treatments lasted for acute periods of 2 and 5 min. MTT and presto blue assays were performed to assess the changes in cell viability; reactive oxygen species (ROS) production was quantified by DCFDA (dichlorofluorescin diacetate) assay, and the expression of inflammatory stress and endoplasmic reticulum (ER) genes were quantified by qPCR. We observed no reduction in cell viability at 2 min of dye treatment with and without endoillumination while at 5 min exposure, a reduction in cell viability at all concentrations of the dye was observed compared to control. Though there was an increase in ROS with endoillumination, it was insignificant. There was no change in the mRNA expression of TNF-α while that of GRP78, and inflammatory genes viz. IL-8, IL-1ß showed a significant increase at 0.5 mg/ml dye with endoillumination. BBG reduced cell viability with increasing concentration and time. The undiluted concentration of the dye results in inflammatory stress compared to the diluted formulations. Interestingly, increased GRP78 at undiluted concentration indicates a protective response in cells exposed to light. However, further studies are needed to evaluate the effect of cellular stress on the visual outcome. We infer that the commercially available formulation of BBG is safe for the RPE, at the recommended dose for a short duration however its toxicity to other cell types need to be addressed.

CHOROIDAL STRUCTURAL CHANGES AND VASCULARITY INDEX IN STARGARDT DISEASE ON SWEPT SOURCE OPTICAL COHERENCE TOMOGRAPHY.

PURPOSE: To evaluate structural changes in the choroid of patients with Stargardt disease using swept source optical coherence tomography scans. METHODS: A retrospective comparison cohort study was conducted on 39 patients with Stargardt disease, and on 25 age and gender matched-healthy controls. Subfoveal choroidal thickness (SFCT) was computed from the swept source optical coherence tomography machine, and the scans were binarized into luminal area and stromal areas, which were then used to derive choroidal vascularity index (CVI). Choroidal vascularity index and SFCT were analyzed independently using linear mixed effects model. RESULTS: There was no significant difference in SFCT between the 2 groups (347.20 ± 13.61 µm in Stargardt disease vs. 333.09 ± 18.96 µm in the control group, P = 0.548). There was a significant decrease in the CVI among eyes with Stargardt disease as compared with the normal eyes (62.51 ± 0.25% vs. 65.45 ± 0.29%, P < 0.001). There was a negative association between visual acuity and CVI (correlation coefficient = -0.75, P < 0.001) and a positive association between visual acuity and SFCT (correlation coefficient = 0.21, P = 0.035). CONCLUSION: Choroidal vascularity index appears to be a more robust tool compared with SFCT for choroidal changes in Stargardt disease. Choroidal vascularity index can possibly be used as a surrogate marker for disease monitoring. A decrease in CVI was associated with a decrease in visual function in eyes with Stargardt disease.

Vitreous opacities after intravitreal triamcinolone injection- a case report.

BACKGROUND: We report occurrence of peculiar tiny white thread like vitreous opacities after intravitreal triamcinolone injection. These persisted without any change for over a year. We ascribe them to aggregation of triamcinolone crystals due to the purification methods. CASE PRESENTATION: Seven patients (8 eyes) with macular edema developed tiny whitish thread like opacities in the vitreous 2-3 months after undergoing an intravitreal injection of triamcinolone acetonide preparation containing benzyl alcohol as preservative. These opacities persisted unchanged for more than a year. The follow up ranged from 91 to 425 days. Vitreous tap was done in one patient which was negative for infection. All patients initially showed improvement but needed re-treatment for recurrence. One patient developed steroid induced rise in intraocular pressure. Microscopic examination of the drug revealed large string like aggregates of triamcinolone crystals. CONCLUSIONS: We hypothesize the possibility of aggregation of triamcinolone crystals into string like structures probably due to the purification methods used during manufacture which led to these thread like opacities in the vitreous.

Surgical management of a case of double optic disc pits with maculopathy.

BACKGROUND: Double optic disc pit maculopathy is a rare entity. It can be difficult to manage because of excessive leakage and chronic maculopathy. PURPOSE: To describe surgical management in a case of double optic disc pits with maculopathy. SYNOPSIS: A 42-year-old male presented with double optic disc pits with macular detachment in the left eye. The best-corrected visual acuity (BCVA) was 20/60, N12. Preoperative OCT showed the presence of two disc pits. The macular region had large retinoschisis and subretinal fluid (SRF) with a central foveal thickness of 879 microns and loss of the ellipsoid zone. A shallow communication from the temporal aspect of the disc to the submacular area was also noted. Among the options of observation, laser photocoagulation, and surgery, the patient opted for surgical management. SURGICAL TECHNIQUE: A standard-3 port 23-gauge pars plana vitrectomy was done. After staining the ILM with brilliant blue, ILM peeling was done with the help of forceps and Finesse loop. ILM flaps were inverted over to cover the optic disc pits and sealed with a drop of fibrin glue. Next, 20% SF6 gas was used for tamponade. Pre- and post-surgery parameters such as visual acuity and OCT were evaluated. POSTOPERATIVE EVALUATION: After 6 weeks, left eye BCVA was 20/40 with OCT showing reduced SRF and reduced intraretinal schisis with a foveal thickness of 546 microns. At 3 months of follow-up, the vision in the left eye had improved to 20/30 with further reduction in the retinoschisis and foveal thickness of 482 microns. HIGHLIGHTS: In this interesting case, we demonstrate a unique way of sealing the defect surgically by vitrectomy and inverted ILM flap with fibrin glue over the disc pits. Despite sealing the maculopathy is slow to resolve. VIDEO LINK: https://youtu.be/s9nY5UPe1s4.

Immediate response to intravitreal treatment for macular edema due to diabetes and retinal vein occlusion.

PURPOSE: To objectively assess the immediate response to intravitreal treatment for macular edema and compare it across different agents. METHODS: This retrospective, comparative study included patients with macular edema due to diabetic retinopathy (DME) or vein occlusion who were treated with intravitreal injections of either steroids (triamcinolone acetonide or dexamethasone sustained release implant) or anti-vascular endothelial growth factor antibodies (VEGF). The central retinal thickness (CRT) and the best corrected visual acuity (BCVA) were measured 1 day after the injection and compared with immediate pre-injection values. RESULTS: There were 79 eyes (57 patients) including 51 eyes with DME, 18 with branch retinal vein occlusion edema (BRVO-ME), and 10 eyes with central retinal vein occlusion edema (CRVO-ME). The intravitreal agents were triamcinolone acetonide (TA)(n = 15), dexamethasone sustained release implant (DEX)(n = 22), ranibizumab (n = 19), and bevacizumab (n = 23). Statistically significant improvement in CRT was seen in all injection groups (p < 0.05) while improvement in mean BCVA was significant only in the TA group (p = 0.009). The mean change in CRT was maximum with steroids than with anti-VEGFs; viz. 159.47 µ in TA, 115.45 µ in DEX, 86.10 µ in ranibizumab, and 78.78 µ in bevacizumab group. Least amount of change was noted in the spongy type of macular edema (18.73 µ) while improvement in mean BCVA was statistically significant only in the cystoid group (p = 0.01). CONCLUSIONS: Comparatively, steroid agents showed better immediate response to therapy than anti-VEGFs. Maximum reduction in central retinal thickness was seen following triamcinolone acetonide injection. Cystoid edema showed better immediate response than spongy retinal thickening.

Management of an inadvertent late filtering bleb and hypotony in a scleral fixated intraocular lens implantation.

PURPOSE: To report a case of inadvertent cystic filtering bleb in a patient with scleral fixated intraocular lens (SFIOL) implantation 7 years after initial procedure. CASE PRESENTATION: A 14-year-old boy underwent lensectomy, vitrectomy and a sutured rigid SFIOL for microspherophakia in the right eye. Seven years later, the patient presented with hypotony related ocular changes. Examination revealed an inadvertent cystic filtering bleb at the scleral section. Anterior segment optical coherence tomography (ASOCT) revealed gap in inner lips of the scleral wound, bridged by a thin strip of scleral tissue. A conservative management with bandage contact lens (BCL) failed after an initial improvement. Injection of trypan blue dye in the anterior chamber confirmed the presence and site of wound filtration. Surgical closure of the fistula was done and patient showed both structural and visual improvement. CONCLUSION: Meticulous wound construction and water tight closure at the end of the surgery can avoid such complications. ASOCT can be helpful in diagnosing the area of fistula. A conservative management with BCL can help by tamponading the filtering bleb. However, in unresponsive cases, surgical closure of the fistula is recommended to improve the hypotony-related changes.

Retinal perfusion density can predict cardiovascular disease risk in patients with retinal vein occlusion.

Purpose: To correlate microvascular changes and assess the relationship between microvascular changes and cardiovascular disease (CVD) risk in patients with retinal vein occlusion (RVO). Methods: Patients over 40 years of age with unilateral RVO were included in this prospective study. Those known to have cardiovascular disease were excluded. A detailed medical history was taken and physical exam was done to measure the height, weight, body mass index (BMI), and systolic blood pressure (SBP). A comprehensive eye check-up was followed by optical coherence tomography angiography (OCTA). Microvascular indices such as vessel density (VD) and perfusion density (PD) were noted. A statistical model was developed for prediction of CVD risk and was integrated with the World Health Organization (WHO)'s risk prediction charts. Results: This study included 42 patients with RVO and 22 controls with an age range of 42-82 years. There were 40 males (62.5%) and 24 females (37.5%). Along with age, SBP, and gender, perfusion density was found to have significant impact on CVD risk (P = 0.030). Reduction in PD was associated with increase in CVD risk. PD had a greater influence on CVD in <50 years age than in >70 years group. Using linear regression, a model with accuracy of 72.1% was developed for CVD risk prediction and was converted into color coded charts similar to WHO risk prediction charts. Conclusion: These findings suggest a significant correlation between microvascular parameters and CVD risk in RVO patients. Based on these parameters, an easy-to-use and color-coded risk prediction chart was developed.

Splitting of retinal pigment epithelium and Bruch's membrane due to a focal choroidal excavation.

We report an unusual finding of a split between the Bruch's membrane (BM) and retinal pigment epithelium (RPE) due to a focal choroidal excavation (FCE) in a healthy young girl with sudden onset visual disturbance. She was followed up regularly for over a year with serial optical coherence tomography (OCT) scans which revealed a parafoveal conforming type of FCE. Initial retinal edema subsided in a month. No associated pathology, such as a choroidal neovascular membrane, was seen. A split was seen between the RPE and the BM on one side of the FCE, which remained unchanged. We postulate this novel finding of the split between the RPE and BM to be due to the inelasticity of the BM, causing it to separate from the RPE due to the sudden outward force exerted during the formation of an FCE.

Retinal laser - Protocols and practical tips.

Background: Many a young doctors in training find retinal laser photocoagulation a daunting task. However, if correct protocols are followed and checklists are observed, then it is not difficult to have a successful laser sitting with a happy patient. Most of the complications can be avoided with correct settings and techniques. Purpose: To enumerate the basic protocols of retinal laser photocoagulation and provide practical tips including laser settings and checklists for hassle-free laser experience. Synopsis: Laser settings for a pan-retinal photocoagulation (PRP) for proliferative diabetic retinopathy differ from those for a focal laser for macular edema. A fill in PRP is indicated when an active Proliferative diabetic retinopathy (PDR) is seen after the initial PRP is completed. The settings and protocols for laser photocoagulation for lattice degeneration are different, and various techniques of barrage laser are discussed. Practical tips and checklists are given, which will not be found in any textbooks. Highlights: Animated illustrations and fundus photos are used to explain the correct techniques of performing laser photocoagulation in different indications and scenarios. Detailed instructions and checklists are provided, which can be very useful to avoid complications and medicolegal problems. The practical tips and guidelines in an easy-to-understand manner make this video highly educational for the novice retinal surgeons who want to perfect their technique of retinal laser photocoagulation. Video Link: https://youtu.be/saQ4s49ciXI.

Surgical management for treatment-resistant cases of inflammatory exudative retinal detachment: Mission impossible?

Purpose: To report the outcome of surgical intervention for inflammatory, exudative retinal detachment (ERD). Methods: A retrospective analysis of eyes with ERD that underwent vitrectomy. Results: Twelve eyes (10 patients) with ERD, non-responsive to medical therapy, underwent vitrectomy. The mean age was 35.7 ± 17.7 years. Five eyes (42%) had Vogt-Koyanagi-Harada disease, three (25%) had presumed tuberculosis (TB), two (17%) pars planitis, and one (8%) had sympathetic ophthalmia. The mean time of vitrectomy was 6.76 ± 4.1 months after onset. Six (50%) eyes had a recurrence, two settled with medical treatment, and four underwent re-surgery. The mean follow-up was 2.7 years. At the last visit, 10 (83.3%) eyes had attached retina; the best-corrected visual acuity (BCVA) had reduced to 1.6 ± 0.7 logarithms of the minimum angle of resolution (logMAR) from 1.3 ± 0.7 at baseline. Conclusion: Vitrectomy in ERD can act as an adjuvant to conventional medical therapy and help maintain structural integrity. Early vitrectomy may help preserve visual function.

The deceptive coloboma.

Background: Scleral fistulas are known to develop following blunt trauma in patients with retinochoroidal coloboma. These cases can be managed by surgical options such as silicone buckles, or with glue and scleral patch graft. Some cases have been shown to close spontaneously. We report the first-ever case managed by vitrectomy, endophotocoagulation, and gas tamponade. Purpose: We present a rare and interesting case of an atypical choroidal coloboma with traumatic scleral fistula due to blunt trauma manifesting with hypotony-related disc edema, maculopathy, and chorioretinal folds, which was managed surgically with vitrectomy, endophotocoagulation, and gas tamponade with a good anatomical and visual outcome. Synopsis: The video contains the case description and surgical management of a traumatic scleral fistula in a patient with atypical superotemporal choroidal coloboma. The patient developed hypotonic maculopathy and disc edema after 3 months following a blunt trauma sustained in a road traffic accident. A scleral fistula was suspected at the temporal edge of the coloboma but could not be accurately localized. In addition, due to the edge effect of the coloboma, the external repair was difficult. Hence, vitrectomy with internal tamponade was attempted. Highlights: The video highlights a different surgical approach to managing a traumatic scleral fistula at the edge of a retinochoroidal coloboma. There was a risk of leakage of intravitreal fluid into the orbit through the fistula; however, the gas bubble gave a better tamponade due to higher surface tension. It sealed the fistula presumably by creating a trap-door-like effect. The endophotocoagulation helped create adhesion between the tissues at the edge of the coloboma effectively sealing it. This was followed by a rapid recovery of the hypotony-related problems with good vision. Traumatic scleral fistula, at a difficult place such as the edge of a coloboma, can be successfully closed from an internal approach with vitrectomy, endolaser, and gas tamponade. Video link: https://youtu.be/6rxgtFyy6cw.

Association of OCT biomarkers and visual impairment in patients with diabetic macular oedema with vitreomacular adhesion.

BACKGROUND: To analyse the distribution of spectral domain optical coherence tomography (SD-OCT) biomarkers in different types of vitreomacular adhesion (VMA) associated visual impairment in diabetic macular oedema. METHODS: A total of 317 eyes of 202 patients were enrolled. Cases were divided into two groups focal VMA and broad VMA and subjects with no VMA were enrolled as controls. A grading platform was used for evaluating the morphology of diabetic macular oedema (DME), using good-quality SD-OCT images. Grading was done for VMA and the biomarkers. Best corrected visual acuity (BCVA), central retinal thickness (CRT) and central subfield thickness (CSFT) was also recorded. RESULTS: The CRT (p = <0.001) and CSFT (p = <0.001) values were statistically significant between the groups. Except for Inner Nuclear Layer Cysts (p = <0.001), absence of Bridging Tissue that is composed of muller cell fibers and bipolar cells (p<0.001), and Hyper Reflective Dots (HRD) in cyst (p = 0.006) there were no significant differences in the distribution of OCT biomarkers among the 3 groups (focal VMA, broad VMA and no VMA). Only Disorganization of Retinal Inner Layers (DRIL) (p = 0.044) showed significant association with vision impairment in all the 3 groups. CONCLUSION: The distribution of OCT biomarkers was similar across all eyes of cases and controls. However, they were more likely to be associated with visual impairment in the presence of VMA than no VMA.

Ocular surface changes following vitreoretinal procedures.

Dry eye disease (DED) can lead to ocular discomfort as well as visual disturbances. Older adults are more likely to develop DED. They are also more likely to develop retinal diseases such as diabetic retinopathy and age-related macular degeneration, which may require vitreoretinal surgeries, lasers, and intravitreal injections. Posterior segment surgery may cause or worsen existing dry eye symptoms, albeit temporarily. Despite good anatomic and functional outcomes, ocular surface dysfunction can have a significant impact on the quality of life, lowering a patient's satisfaction with the retinal treatment. Preexisting DED, intraoperative tissue handling, and postoperative treatment may all contribute to the severity of ocular surface dysfunction. In this article, we have reviewed all relevant studies on the development of ocular surface changes and DED, as well as the impact of vitreoretinal surgeries and procedures on the ocular surface.