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.

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.

Effects of Myopia and Glaucoma on the Neural Canal and Lamina Cribrosa Using Optical Coherence Tomography.

PRCIS: Glaucoma was associated with axial bowing and rotation of Bruchs membrane opening (BMO) and anterior laminar insertion (ALI), skewed neural canal, and deeper anterior lamina cribrosa surface (ALCS). Longer axial length was associated with wider, longer, and more skewed neural canal and flatter ALCS. PURPOSE: Investigate the effects of myopia and glaucoma in the prelaminar neural canal and anterior lamina cribrosa using 1060-nm swept-source optical coherence tomography. PATIENTS: 19 control (38 eyes) and 38 glaucomatous subjects (63 eyes). MATERIALS AND METHODS: Participants were imaged with swept-source optical coherence tomography, and the images were analyzed for the BMO and ALI dimensions, prelaminar neural canal dimensions, and ALCS depth. RESULTS: Glaucomatous eyes had more bowed and nasally rotated BMO and ALI, more horizontally skewed prelaminar neural canal, and deeper ALCS than the control eyes. Increased axial length was associated with a wider, longer, and more horizontally skewed neural canal and a decrease in the ALCS depth and curvature. CONCLUSION: Our findings suggest that glaucomatous posterior bowing or cupping of lamina cribrosa can be significantly confounded by the myopic expansion of the neural canal. This may be related to higher glaucoma risk associated with myopia from decreased compliance and increased susceptibility to IOP-related damage of LC being pulled taut.

Lowered Decorin With Aberrant Extracellular Matrix Remodeling in Aqueous Humor and Tenon's Tissue From Primary Glaucoma Patients.

Purpose: To evaluate the inflammatory cytokine, growth factors, extracellular matrix (ECM) remodeling genes, profibrotic and antifibrotic molecules in patients undergoing glaucoma filtration surgery (GFS). Additionally, the effect of preoperative antiglaucoma medications (AGMs) and postoperative bleb status were related to these parameters. Methods: Tenon's tissue and aqueous humour (AH) were collected from 207 patients undergoing GFS with primary open-angle glaucoma (POAG) (n = 77), primary angle-closure glaucoma (PACG) (n = 62), and cataract controls (n = 68). Monocyte chemoattractant protein-1 (MCP-1), connective tissue growth factor (CTGF), transforming growth factor ß1/2 (TGF-ß1/2), lysyl oxidase (LOX), lysyl oxidase L2 (LOXL2), elastin (ELN), collagen type 1 α 1 (COL1A1), secreted protein acidic and rich in cysteine (SPARC), α-smooth muscle actin (α-SMA), and decorin (DCN) were determined in tenon's tissue by real-time PCR and in AH using ELISA. Results: A significant increase was observed in the transcripts of MCP-1, TGF-ß2, and SPARC in POAG and PACG (P < 0.05); CTGF, TGF-ß1, LOX, LOXL2, ELN, COL1A1, and α-SMA in PACG (P < 0.05) compared with control. DCN transcript was significantly decreased in POAG and PACG (P < 0.05) compared with control. The protein levels of CTGF, TGF-ß1/ß2, ELN, SPARC, and LOXL2 was significantly elevated in POAG and PACG (P < 0.05); DCN was decreased (P < 0.05) compared with control. These parameters showed significant association with duration of preoperative AGMs and postoperative bleb status. Conclusions: This study demonstrates increased expression of growth factors and ECM molecules, both at protein and transcript levels in GFS patients. A decreased DCN in AH seems striking, and if restored might have a therapeutic role in minimizing postoperative scarring to improve GFS outcome.

Retinal vascular abnormalities in Sturge-Weber syndrome.

Sturge-Weber syndrome (SWS) includes facial, leptomeningeal and choroidal hemangioma. The retinal vasculature is essentially normal. Rare cases of retinal vascular tortuosity and arterio-venous malformations have been reported. We report two cases with rare concomitant retinal vascular abnormalities along with SWS. Both the patients had nevus flammeus, hemifacial hypertrophy, and choroidal hemangioma. In one case, retinal cavernous hemangioma was seen in the affected eye. The other case revealed retinal neovascularization secondary to proliferative diabetic retinopathy in the eye with choroidal hemangioma.

Visual rehabilitation using video game stimulation for Stargardt disease.

BACKGROUND: Stargardt disease, a common form of heredomacular degeneration, leads to severe vision loss. Video game play can act as a positive biofeedback to reinforce visual rehabilitation and fixation training. It can potentially lead to visual improvement. This study was done to explore the possibility of visual improvement using video game stimulation for visual rehabilitation in Stargardt disease. METHODS: We evaluated eight patients with Stargardt disease who had nonatrophic retina surrounding the area of degeneration at the macula. They underwent extensive baseline testing to determine their Snellen visual acuity, pattern visual evoked potentials, retinal sensitivity, and fixation analysis with microperimetry, electroretinography, fundus photography, optical coherence tomography, and autofluorescence. They were given 40 h of training with video game play and re-evaluated on all the tests. RESULTS: They showed both subjective and objective evidence of improvement in visual functions and vision-related tasks. Visual acuity (from 0.77 ± 0.29 to 0.71 ± 0.32 logMAR, p = 0.027), contrast sensitivity (from 1.28 ± 0.25 to 1.46 ± 0.17, p = 0.002), and fixation stability (log of bivariate contour ellipse area from 6.67 ± 0.52 to 5.85 ± 0.84, p = 0.022) improved significantly. The retinal sensitivity improved by 0.47 ± 3.39 dB (p = 0.67). Stereopsis and pattern visual evoked potentials showed improvement. A low vision questionnaire documented subjective improvement. CONCLUSION: Visual stimulation by video game play can result in improvement in visual acuity, fixation pattern, and retinal sensitivity with improvement in vision-related tasks. It can serve as a simple rehabilitatory technique for patients with central vision loss due to Stargardt disease.

Visual rehabilitation using microperimetric acoustic biofeedback training in individuals with central scotoma.

BACKGROUND: Patients with central scotoma have poor fixation stability and poor visual acuity. Acoustic biofeedback training can be an effective way to train such patients to eccentrically fixate. This study analyses the mean retinal sensitivity, saccadic velocity, and fixation stability after acoustic biofeedback training and shows correlation with age and scotoma size. METHODS: Patients with irreversible central scotoma in both the eyes secondary to macular diseases were selected. After undergoing comprehensive low vision assessment, 19 patients who were willing were recruited for the acoustic biofeedback training to the better eye in 10 sessions, using the MP-1 Microperimeter. Mean retinal sensitivity, saccadic velocity, fixation stability before and after the acoustic biofeedback were recorded. RESULTS: There were 17 men and two women. Ages ranged from 19-94 years (mean 54.63 ± 24.66). The scotoma size ranged from four to 20 degrees. Ten patients had age-related macular degeneration, four had Stargardt disease, three had traumatic macular scar, two had scarred myopic choroidal neovascular membrane, and one had myopic macular degeneration. The vision improved from 1.06 ± 0.36 to 0.86 ± 0.33 logMAR (p < 0.0001). The mean retinal sensitivity improved from 2.1 ± 2.9 dB to 2.7 ± 3.1 dB (p = 0.01), with negative correlation with age (p = 0.01) and scotoma size (p = 0.02). Fixation stability improved with reduction in the bivariate contour ellipse area (p = 0.01). It showed negative correlation with age (p = 0.02) and scotoma size (p = 0.10). The saccadic velocity reduced from 0.34°/second to 0.26°/second but was not significant (p > 0.99). The majority (58 per cent) had their preferred retinal locus superior to the fovea. There was good agreement between bivariate contour ellipse area and MP-1 Microperimeter inbuilt fixation parameters. The effect was maintained at six months with slight reduction in fixation stability. CONCLUSION: Acoustic biofeedback can improve fixation behaviour, visual acuity and retinal sensitivity in patients with central scotoma. The results are better with younger age and smaller scotoma size.

Factors influencing the choice of low-vision devices for visual rehabilitation in Stargardt disease.

BACKGROUND: Stargardt disease is the most common cause of juvenile macular degeneration leading to early central visual loss. Dispensing of correct low-vision devices based on the residual visual function and specific visual requirements of a patient can result in a positive outcome. It is important to know the factors involved in the selection of these devices. This study was undertaken to assess these factors. METHODS: Patients with Stargardt disease referred to a low-vision clinic underwent evaluation of visual status, disease stage, visual requirements and lifestyle. They were evaluated for suitability for successful use of various low-vision devices. Their education level and occupation were noted. They were counselled regarding the proper use of the devices and lifestyle modifications. For patients with extensive use of computers, modifications related to contrast, font size and audio software were explained. RESULTS: Among the 97 patients in the study (age range 7-66 years, mean 23.7 ± 13.1), there were 49 (50.5 per cent) students, 36 (37.1 per cent) employed, eight (8.2 per cent) unemployed, and four (4.2 per cent) homemakers. Except for seven patients (7.2 per cent), all were literate, with education ranging from primary school to college graduation. The presenting visual acuity for distance was 0.10-1.47 (0.79 ± 0.28), and for near was N4-N40 (N9.95 ± 6.65). This acuity showed positive correlation with age (p < 0.0001, R2 = 0.16) and with magnification required (p < 0.0001, R2 = 0.26). Patients above 40 years preferred higher adds and half-eye glasses, whereas younger patients preferred dome magnifiers. The occupation also influenced the choice. Disease stage was seen to affect the choice with advanced stages requiring higher magnifiers (p = 0.03, R2 = 0.11). Duration of disease and the magnification of low-vision devices showed a positive correlation (p = 0.03, R2 = 0.049). CONCLUSIONS: Age, presenting visual acuity, disease stage, duration, education and occupation influenced the choice of low-vision devices in patients with Stargardt disease. However, they appear to be interdependent and a larger, controlled study is required to provide information on the individual effect.

Non-Self-Sealing (Leaky) Anterior Chamber Paracentesis: A New Technique in Managing Postphacoemulsification Intraocular Pressure Rise in Glaucoma and Normal Eyes.

Phacoemulsification (phaco) for cataract extraction is 1 of the most commonly performed ophthalmic surgeries. With increasing evidence of significant intraocular pressure (IOP) reduction after phaco, the paradigm for glaucoma treatment has been shifting toward more cataract extraction instead of glaucoma surgery; thus, the population of glaucoma patients undergoing phaco is likely to continue to increase in the coming years. Although the safety of surgery has improved over the years with newer technologies and machines, postoperative IOP spike remains an important condition even after an uneventful operation. Glaucoma patients undergoing phacoemulsification are particularly at risk of further glaucomatous optic nerve damage from the transient yet potentially high pressures after phaco. Common treatments include topical, intracameral, oral, and systemic IOP-lowering medications; postoperative anterior chamber paracentesis (ACP); and so on. No single treatment to date can guarantee effective prevention or control IOP rise in the first 24 hours after phaco. Sometimes, the IOP remains high despite all of the above treatments and the risk for further glaucomatous damage may be unavoidable. In this perspective article, we discuss the incidence, causes, and treatments of IOP rise after phaco and introduce a new technique, a non-self-sealing (leaky) ACP that may be of use in regulating postoperative IOP rise, especially for patients with glaucoma.

Swept-source optical coherence tomography study of choroidal morphology in Stargardt disease.

BACKGROUND: Stargardt disease, a juvenile retinal dystrophy, may show secondary changes in the choroid which may have importance while considering future treatments such as stem cell transplant. OBJECTIVE: To evaluate the choroidal and retinal morphology in patients with Stargardt disease and compare with age-matched normals. SETTING AND DESIGN: This was a case-control study at a tertiary level eye care institute. METHODS: Twenty-six patients (52 eyes) clinically diagnosed with Stargardt disease underwent detailed evaluation with swept-source optical coherence tomography. Retinal and choroidal layers were analyzed and compared with 52 eyes of controls. RESULTS: The median age of patients with Stargardt disease was 23 years. The mean best-corrected visual acuity was 0.82 logMAR (20/125 Snellen). Mean diameter of the lesion was 2810.92 ± 1311.15 µ. The lesion size increased with increasing extent of flecks and was significantly correlated with visual acuity (r = 0.622, P < 0.001). The retinal and choroidal thicknesses (CTs) were significantly reduced in Stargardt group. The mean subfoveal CT was 290.59 ± 60.43 µ(range: 184-395) in Stargardt and 331.31 ± 68.90 µ (range: 199-464) in normal group, P = 0.043. CT in Early Treatment Diabetic Retinopathy Study grid pattern showed significant thinning in Stargardt group. The small choroidal vessel (SCV) layer was more affected than the large choroidal vessel (LCV) layer. There was thinning of SCV and thickening of LCV inside the macular lesion. The CT was not correlated to lesion size, extent of flecks, or visual acuity. CONCLUSIONS: Stargardt disease shows generalized thinning of the choroid affecting mainly the SCVs. In the macular lesion, there is atrophy of SCV with compensatory dilation of LCV. The visual acuity did not correlate with CT but showed worsening with increasing lesion size and wider extent of flecks.

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.

Safety and Efficacy of Intravitreal Dexamethasone Implant for Refractory Uveitic Macular Edema in Adults and Children.

PURPOSE: To assess safety and efficacy of intravitreal dexamethasone (DEX) implant in refractory uveitic macular edema (ME). METHODS: We retrospectively analyzed medical records of patients with nonresponsive ME secondary to chronic, noninfectious intermediate or posterior uveitis, treated with intravitreal DEX implants. RESULTS: A total of 42 eyes of 34 patients (aged 6-67 years) received 56 implants. Mean follow-up was 19.2 ± 2.2 months after DEX implant. The mean visual acuity (0.48 ± 0.06 logMAR to 0.34 ± 0.1 logMAR) and mean central retinal thickness (472.2 ± 35 to 274.7 ± 60.6 µm) improved considerably before and after DEX implant. A total of 11 eyes needed repeat implants after a prolonged time to recurrence (12.6-20.9 months). A total of 10 eyes needed no additional treatment. Oral steroids could be stopped in 40% patients. Intraocular pressure increased in seven and cataract progressed in six eyes. CONCLUSION: Intravitreal DEX implant is safe and effective adjunct therapy. It reduces dependence on systemic steroids, immunosuppressives and provides long-term effects.

Localized retinal degeneration secondary to Waldenström's macroglobulinemia.

A 52-year-old man, treated for Waldenström's macroglobulinemia (WM), continued to experience decreased vision even after 24 months. He was evaluated using multimodal imaging and electroretinography. The retina did not show any hyperviscosity changes but revealed a yellow lesion at macula with atrophic changes causing a pattern on fluorescein angiography similar to a leopard's skin. Optical coherence tomographic imaging revealed uniformly reflective material deposited in the outer retina with degeneration of outer retinal layers. Full-field electroretinography was normal, but multifocal electroretinography revealed reduced foveal responses. This case highlights the degenerative effects of long-standing immunogammopathy maculopathy in WM.

Concurrent retinitis pigmentosa and pigmented paravenous retinochoroidal atrophy phenotypes in the same patient.

We report a unique case of a patient with retinitis pigmentosa (RP) phenotype in one eye and pigmented paravenous retinochoroidal atrophy (PPRCA) phenotype in the other eye. We describe in detail the symptoms, clinical findings, and investigations done for a 32-year-old Indian woman. This patient had phenotypical picture resembling typical RP in the right eye, with characteristic symptoms of night blindness and constricted field of vision and a nonrecordable electroretinogram (ERG). The left eye of the same patient revealed typical PPRCA phenotype, with no night blindness, normal field, and normal ERG. RP and PPRCA phenotypes are part of the same spectrum of genetic disorder. However, it is rare to see them coexist in the same patient.