Acute macular neuroretinopathy secondary to central retinal artery occlusion.

Purpose: Acute Macular Neuroretinopathy (AMN) may be the result of deep retinal capillary plexus (DCP) impairment, but its mechanism remains elusive. A recent study has described simultaneous onset of Paracentral Acute Middle Maculopathy (PAMM) and AMN, suggesting a related pathogenic pathway. In this report, we analyze and describe the imaging characteristics of patients with concomitant Central Retinal Artery Occlusion (CRAO) and AMN and suggest a mechanistic pathway to explain this relationship. Observations: A total of 2 cases of CRAO, arteritic and non arteritic, were included in this report. At initial presentation, outer retinal layers were intact. At the two-week follow-up visit, both cases displayed Henle fiber layer hyperreflectivity and ellipsoid zone disruption consistent with AMN. Conclusions: Secondary development of AMN in CRAO is a new finding. DCP ischemia secondary to CRAO may lead to Henle fiber layer disruption, leading to the characteristic findings of AMN.

Long-Term Outcomes of Bacillary Layer Detachment in Neovascular Age-Related Macular Degeneration.

PURPOSE: To evaluate the clinical characteristics, multimodal imaging features, and long-term treatment outcomes of eyes with neovascular age-related macular degeneration (nAMD) and bacillary layer detachment (BALAD) treated with intravitreal anti-VEGF therapy. DESIGN: Retrospective, longitudinal, case series. PARTICIPANTS: Treatment-naive patients with nAMD (n = 30) showing BALAD on OCT and undergoing anti-VEGF therapy. METHODS: Clinical records and multimodal imaging results of up to 4 years after diagnosis were reviewed. MAIN OUTCOME MEASURES: Best-corrected visual acuity (BCVA) values were compared over time. The cumulative risk of and risk factors for subretinal fibrosis were assessed using Cox regression analyses, and adjusted hazard ratio (aHR) was computed. RESULTS: Thirty eyes of 30 patients were included. Macular neovascularization (MNV) subtypes were distributed as follows: type 1, 63%; type 2, 27%; mixed type 1 and 2, 3%; type 3, 3%; aneurysmal type 1, 3%. The BCVA significantly improved after anti-VEGF loading phase (Snellen equivalent, from 20 of 118 to 20 of 71, P = 0.03), but it returned to the baseline levels at 4 years (Snellen equivalent, 20 of 103, P = 0.6). The cumulative risk of subretinal fibrosis was 77% at 4 years. The risk factors associated with subretinal fibrosis included hemorrhagic BALAD (aHR, 2.02; 95% confidence interval [CI] 1.54-3.22; P < 0.01) and the presence of subretinal hyperreflective material (aHR, 1.83; 95% CI 1.35-3.14; P < 0.01). CONCLUSIONS: BALAD was found in association with all types of MNV in patients with nAMD. Long-term observation revealed poor functional outcomes related to the high risk of subretinal fibrosis.

Meibomian Gland Dysfunction: Intense Pulsed Light Therapy in Combination with Low-Level Light Therapy as Rescue Treatment.

Background and Objectives: Evaporative dry eye disease is frequently associated with meibomian gland dysfunction. Patients are often unhappy because of daily drops, care burden, and suboptimal conventional treatments. In this study, we assessed the efficacy of a novel device, the Eye-light®, a combination of intense pulsed light therapy and low-level light therapy, as a novel treatment for meibomian gland dysfunction and dry eye disease. Materials and Methods: This was a retrospective, single-center study carried out over a 6-week period, in which 22 eyes from 11 patients were included. Each patient received four combined light therapy treatment sessions, once weekly over 4 weeks. Patients underwent a clinical examination and filled out a standardized questionnaire to evaluate symptoms one week prior to treatment, and one week after the fourth session. Results: Combined light therapy improved several ocular surface outcome measures in our patients. This study demonstrates that this adjunctive treatment significantly improves the ocular surface and quality of life of patients with dry eye disease and meibomian gland dysfunction. Conclusions: Combined light therapy may be included in meibomian gland dysfunction treatment protocols as an adjunctive rescue treatment.

BACILLARY LAYER DETACHMENT: MULTIMODAL IMAGING AND HISTOLOGIC EVIDENCE OF A NOVEL OPTICAL COHERENCE TOMOGRAPHY TERMINOLOGY: Literature Review and Proposed Theory.

PURPOSE: To clarify the histologic basis of bacillary layer detachment (BALAD) through a review of the current literature and an analysis of retinal imaging. METHODS: The literature for previous reports of BALAD were reviewed. An analysis of retinal images was performed to support anatomical conclusions. RESULTS: A total of 164 unique patients with BALAD on optical coherence tomography (OCT) were identified from the published literature. Twenty-two underlying etiologies, all associated with subretinal exudation, were identified. Forty-one different OCT terminologies were found. The defining OCT feature of BALAD was a split at the level of the photoreceptor inner segment myoid creating a distinctive intraretinal cavity. Resolution of BALAD was followed by a rapid restoration of the ellipsoid zone. Histology of age-related macular degeneration eyes suggests that individual photoreceptors can shed inner segments. Furthermore, detachment of the entire layer of inner segments is a common postmortem artifact. It is proposed that BALAD occurs when outwardly directed forces promoting attachment of photoreceptor outer segments to the retinal pigment epithelium exceed the tensile strength of the photoreceptor inner segment myoid. CONCLUSION: This review serves to strengthen the OCT nomenclature "bacillary layer detachment," based on specific reflectance information obtained by OCT and previously published histologic observations.

UVEITIS TREATED WITH DEXAMETHASONE IMPLANT.

PURPOSE: To evaluate the real-life efficacy and safety of the intravitreal dexamethasone implant in uveitis. METHODS: This retrospective observational multicentric study included 152 eyes treated exclusively by 358 dexamethasone implant injections. The main outcome measures included change in the best-corrected visual acuity, central macular thickness, and vitreous haze score. RESULTS: Patients were treated with dexamethasone implant for macular edema (51.3%), vitritis with macular edema (40.1%), vitritis (5.3%), and other causes (3.3%). The mean duration of follow-up was 19.0 months. The mean gain in best-corrected visual acuity during follow-up was +12.1 letters. An improvement in best-corrected visual acuity ≥5, 10, and 15 letters was found in 64.5, 50.7, and 35.5% of cases, respectively. 59.7% of eyes with macular edema at baseline were found to be anatomical responders. Vitritis resolution (vitreous haze = 0+) was obtained in 81.4% of cases. Ocular hypertension (intraocular pressure ≥25 mmHg and/or gain ≥10 mmHg from baseline) occurred in 28.3% of patients. No filtering surgery/laser therapy was required. A total of 40.2% of phakic subjects underwent cataract surgery on average 11.2 months after the first injection. CONCLUSION: This study confirms the efficacy and safety of the dexamethasone implant in noninfectious uveitis. Cataract and ocular hypertension were not uncommon but easily manageable.

LONG-TERM INCIDENCE AND RISK FACTORS OF OCULAR HYPERTENSION FOLLOWING DEXAMETHASONE-IMPLANT INJECTIONS: THE SAFODEX-2 STUDY.

PURPOSE: To analyze the incidence, risk factors, and time to onset of ocular hypertension (OHT) after intravitreal injections (IVI) of dexamethasone implant and to evaluate the long-term cumulative probability of intraocular pressure elevation. METHODS: Eyes of patients having received at least one dexamethasone implant IVI between October 2010 and February 2015 were included in the present study. Ocular hypertension was defined as intraocular pressure > 25 mmHg and/or an increase of 10 mmHg over the follow-up period compared with baseline intraocular pressure. RESULTS: Four hundred ninety-four eyes were studied in 410 patients. For a total of 1,371 IVI, the incidence of OHT was 32.6% in the study eyes with a mean follow-up period of 30 months (3-62.5) and a median follow-up of 29 months. Pressure-lowering treatment was introduced for 36.9% of eyes. Topical treatment alone was sufficient to manage OHT in 97%. Young age, male sex, uveitis and retinal vein occlusion, and glaucoma treated with a double- or triple-combination topical pressure-lowering medication were found to be risk factors for OHT. The incidence of OHT did not change with an increase in the number of IVI, and there was no cumulative effect, defining by an increase of the incidence of OHT in patients after repeated IVI (P = 0.248). CONCLUSION: This study confirmed that OHT is of moderate incidence, transient, controlled by topical treatment and provides data on the long-term cumulative probability of intraocular pressure elevation in a large cohort of eyes treated with dexamethasone implant IVI. Repeat injections of dexamethasone implant neither increase nor decrease the risk of OHT.

Chorioretinal Toxicity of Perfluorooctane (Ala Octa): Results From 48 Surgical Procedures in Geneva.

PURPOSE: To compare outcomes of patient who underwent surgery using perfluorooctane (PFO; C8F18; Ala Octa) with those who underwent surgery with perfluorodecalin (F-Decalin). DESIGN: Retrospective, consecutive, comparative, interventional case series. METHODS: A total of 48 eyes that underwent vitrectomy with PFO were compared to 29 eyes that underwent vitrectomy with perfluorodecalin. Two experienced surgeons performed vitrectomies at the Geneva University Eye Clinic. Visual acuity before, at 8 and 24 weeks after surgery, was documented, and spectral domain optical coherence tomography (SD-OCT) images were analyzed for abnormalities. RESULTS: Two patients experienced severe retinal toxicity, including 1 with severe vision loss. However, no statistical differences in VA were observed between the PFO and perfluorodecalin patients. Analysis of SD-OCT images showed differences in occurrence of several abnormalities, for example, inner segment-outer segment alterations were found in 60.4% of eyes treated with PFO and in 10.3% of perfluorodecalin-treated eyes; retinal atrophic areas were found in 41.7% of PFO and in none of the perfluorodecalin eyes; inner limiting membrane contraction was found in 58.4% of PFO and in none of perfluorodecalin eyes; inner retina cystic alterations were found in 58.3% of PFO eyes and 17.2% of perfluorodecalin eyes; outer retina cystic alterations were found in 39.6% of PFO eyes and 13.8% of perfluorodecalin eyes; retinal holes were found in 14.6% of PFO eyes and in none of the perfluorodecalin eyes; and outer retinal inclusions were found in 20.8% of PFO eyes and in 3.45% of perfluorodecalin eyes. CONCLUSIONS: Perfluorooctane caused significantly more toxic damage than perfluorodecalin. Special consideration should be given to develop a central European Union (EU) control agency for medical devices and to reevaluate safety procedures currently accepted by the EU and International Organization for Standardization for intraocular surgery.

Taurine treatment of retinal degeneration and cardiomyopathy in a consanguineous family with SLC6A6 taurine transporter deficiency.

In a consanguineous Pakistani family with two affected individuals, a homozygous variant Gly399Val in the eighth transmembrane domain of the taurine transporter SLC6A6 was identified resulting in a hypomorph transporting capacity of ~15% compared with normal. Three-dimensional modeling of this variant has indicated that it likely causes displacement of the Tyr138 (TM3) side chain, important for transport of taurine. The affected individuals presented with rapidly progressive childhood retinal degeneration, cardiomyopathy and almost undetectable plasma taurine levels. Oral taurine supplementation of 100 mg/kg/day resulted in maintenance of normal blood taurine levels. Following approval by the ethics committee, a long-term supplementation treatment was introduced. Remarkably, after 24-months, the cardiomyopathy was corrected in both affected siblings, and in the 6-years-old, the retinal degeneration was arrested, and the vision was clinically improved. Similar therapeutic approaches could be employed in Mendelian phenotypes caused by the dysfunction of the hundreds of other molecular transporters.

Evaluation of Efficacy and Safety of Dexamethasone Intravitreal Implants of Vitrectomized and Nonvitrectomized Eyes in a Real-World Study.

PURPOSE: To compare the efficacy and safety of the dexamethasone (DEX) intravitreal implant of vitrectomized and nonvitrectomized eyes in real-world conditions. METHODS: This was a retrospective, multicenter, observational study. All consecutive patients presenting with at least one 0.7-mg intravitreal injection of DEX implant were included in this study. A total of 186 eyes in 170 patients were analyzed. Fifty-nine eyes were vitrectomized at baseline and 127 eyes had no vitrectomy at the last visit. Among the baseline-vitrectomized eyes analyzed, 72.9% were treatment naive eyes, and 44.1% of nonvitrectomized eyes had no prior treatment. RESULTS: There was no statistically significant difference in the variation in best-corrected visual acuity (BCVA) between the 2 groups (P = 0.343). Variations of BCVA and central macular thickness were not significantly different between nonvitrectomized eyes and baseline-vitrectomized eyes. The intraocular pressure profile was the same in both nonvitrectomized eyes and baseline-vitrectomized eyes. The mean interval between injections was 6.9 months (2; 27.7) for nonvitrectomized eyes and 5.2 months (4; 22.1) for baseline-vitrectomized eyes (P = 0.001). The mean number of IVIs was 2 (1; 6) for nonvitrectomized eyes and 2.3 (1; 10) for baseline-vitrectomized eyes (P = 0.188) during the total follow-up period. CONCLUSION: This large cohort shows that vitrectomy does not seem to influence the efficacy and safety profile of dexamethasone intravitreal implant for DME.