Alemtuzumab-induced alopecia universalis and transient accommodation spasm in a patient with multiple sclerosis.

Herein, we report a case of alopecia universalis and transient accommodation spasm presented after alemtuzumab administration in a patient previously treated with fingolimod. To the best of our knowledge, this is the first report of accommodation spasm as an acute adverse effect of alemtuzumab. Treatment with alemtuzumab in relapsing-remitting multiple sclerosis has been identified as a risk factor for developing secondary autoimmunity within the follow-up period (peak 18-36 months from the first infusion) such as thyroid disorders. This case highlights the need for postmarketing surveillance and the significance of reporting rare side effects related to alemtuzumab; its high efficacy should be weighted with potentially severe adverse events when making a therapeutic decision. Further studies in larger cohorts are needed to elucidate pathomechanisms of alemtuzumab.

Long-term progression of myopic maculopathy in degenerative myopia as imaged by SD-OCT and IR imaging.

CLINICAL RELEVANCE: Clinicians can benefit from developing an understanding of the natural disease progression of myopic maculopathy in degenerative myopia using optical coherence tomography (OCT). BACKGROUND: The prevalence of high myopia is constantly increasing. In this work, infrared imaging and OCT is used to study the natural course of the disease. METHODS: This is a retrospective longitudinal study. Medical records of 72 patients with high myopia (< 6.00 D) and a minimum five-year follow-up period were analysed. Collected data on all enrolled patients included demographic characteristics and medical history, as well as recordings on best-corrected visual acuity, slitlamp examination, OCT, and fluorescein angiography in cases of suspected myopic choroidal neovascularisation. Images were independently marked by two graders. RESULTS: The mean age of patients was 54.6 ± 14.4 years (59.72% female) with baseline logMAR best-corrected visual acuity of 0.22 ± 0.28. At baseline examination, 70.83% of the study group showed signs of maculopathy and 62.5% diffuse or patchy atrophy. During follow-up, 22.2% of patients with any type of atrophy showed enlarged affected areas. Two patients with baseline lacquer cracks developed new lesions. There was a weak correlation between patient age and maculopathy progression (r = 0.233; p = 0.03). While central retinal thickness was not associated with maculopathy progression (p = 0.203), a moderate correlation was found between choroidal thickness and maculopathy progression (r = -0.516; p < 0.001). CONCLUSION: Lesion characteristics in myopic degeneration have been elucidated, taking advantage of the ongoing technological advances in retinal imaging. The understanding of disease patterns and progression is essential for appropriate management of patients, while discovering biomarkers which lead to choroidal neovascularisation development is of urgent importance to establish international diagnostic criteria and treatment protocols.

Hypotony Following Intravitreal Silicone Oil Removal in a Patient With a Complex Retinal Detachment With Giant Retinal Tear.

Postoperative ocular hypotony after silicone oil removal in complex cases of retinal detachment is a complication that can occur in about 20% of cases and can prevent the successful management of retinal detachments. Thus, it is critical to understand the mechanisms of hypotony and the potential interventions that can be done in order to avoid irreversible tissue damage. We present a case of a 35-year-old man who underwent intraocular surgery for removal of silicone oil tamponade following a combined scleral buckling and pars plana vitrectomy (PPV) surgery for a rhegmatogenous retinal detachment associated with a giant retinal tear. On Day 1 after the operation, the patient was found to have hypotony with optic disc edema, chorioretinal folds, and visual acuity of 'hand movement' perception. Two weeks postop, the patient's condition stabilized, with a visual acuity of 0.38 logMAR, an intraocular pressure (IOP) of 12 mmHg, and the absence of macular edema.

Retinal neurodegeneration, macular circulation and morphology of the foveal avascular zone in diabetic patients: quantitative cross-sectional study using OCT-A.

PURPOSE: Using OCT-A to investigate the association between neurodegeneration and vascular morphology in diabetic retinopathy (DR). METHODS: Cross-sectional study. One hundred and sixty-two patients were enrolled and following fundoscopy were assigned to two groups according to DR severity: 54 patients to the group of no clinical signs of DR (noDR) and 54 to the non-proliferative DR (NPDR) group. Fifty-four age-matched patients without known diabetes were recruited as the control group. Patients underwent full ophthalmic examination followed by OCT-A. Central retinal thickness (CRT), vessel density (VD) in the superficial and deep retinal layers and foveal avascular zone (FAZ) area were measured. Additionally, ganglion cell complex (GCC) layer thickness along with global loss volume (GLV) and focal loss volume (FLV) indices was measured. RESULTS: In total, 85 men with mean age of 51.93 ± 9.03 and 77 women with age of 50.14 ± 10.35 were examined. Mean diabetes duration was 4.62 ± 2.16 years in the noDR group and 11.34 ± 2.73 years in the NPDR group (p < 0.001). Superficial VD (sVD) and deep VD (dVD) were significantly different only between noDR and NPDR groups (p < 0.001 for both comparisons), but no statistically significant difference was observed between the controls and the DR groups. Global loss volume was significantly higher in the NPDR (4.38 ± 2.22) compared to the noDR group (3.24 ± 1.76; p < 0.03). Focal loss volume was significantly higher in both noDR (1.22 ± 1.03) and NPDR (2.09 ± 1.72) groups compared to controls (0.95 ± 0.83; p < 0.001 between noDR and NPDR and p = 0.02 between control and noDR groups). Significant associations were found between GLV and deep VD (p < 0.01, r = -0.48), FLV and superficial VD (p < 0.01, r = -0.42) and FLV with deep VD (p < 0.01, r = -0.64). CONCLUSION: In this study, we evaluated the impact of DR in both the vascular layers and neural components of the retina as expressed by FAZ, sVD, dVD and GCC thickness, FLV and GLV using OCT-A. We found that FLV was significantly higher in both noDR and NPDR groups indicating that in progressive DR stages FLV values might be increased, which might serve as an early index of neuronal damage in patients with diabetes even in the absence of overt DR signs.

Segmentation errors and motion artifacts in OCT-A associated with epiretinal membranes.

OBJECTIVE: To explore segmentation errors, image quality, and motion-associated artifacts in eyes with idiopathic epiretinal membrane (ERM). METHODS: This is a prospective observational study. We included 39 eyes affected by ERM and 40 eyes from age-matched healthy subjects. Optical coherence tomography-angiography (OCT-A) was performed in both groups. Segmentation was automatically performed by intergraded software. Segmentation was regarded as inaccurate if either border deviated from the correct plane by more than 50 µm. Presence of motion artifacts (blink lines, displacement, stretch artifacts, quilting, vessel doubling) and image quality index were reported. RESULTS: Quality index score was 7.2 ± 0.9 for the ERM patients. Phakic eyes with ERM had quality index score of 7.71 ± 1.06, and pseudo-phakic eyes with ERM had a quality index score of 7.32 ± 0.85 (p = 0.22). Motion artifacts were 1.22 ± 0.7 in the study cohort. Segmentation was accurate in all healthy subjects (n = 40). Segmentation errors occurred in 64.1% of ERM patients. The inner plexiform layer was the segmentation boundary most prone to inaccurate segmentation, followed by the internal limiting membrane. Segmentation of retinal pigment epithelial layer was accurate in 96.7% of all cases. CONCLUSIONS: OCT-A image quality cannot be accurately reproduced in pathological conditions, such as in ERM patients, and is prone to motion artifacts and segmentation errors. Incorrect segmentation results in anatomically incorrect en-face OCT-A images and subsequently in false quantification measures.

Pre-operative intravitreal dexamethasone implant in patients with refractory diabetic macular edema undergoing cataract surgery.

To examine preoperative use of intravitreal dexamethasone implant in patients with refractory diabetic macular edema (DME) undergoing cataract surgery. Participants in this study were 17 patients with DME refractory to previous treatment with anti-vascular endothelial growth factor agents or dexamethasone implant, and co-existent cataract. All participants received intravitreal dexamethasone implant at baseline and underwent phacoemulsification within one month after its insertion. Best-corrected visual acuity (BCVA) and central subfield thickness (CST) changes between baseline, time of cataract surgery and postoperative months 1, 2 and 3 were evaluated. At month 1 after surgery, BCVA improved significantly from 42.3 ± 9.6 to 58.7 ± 11.9 letters compared to baseline (p < 0.001) and the improvement was sustained at month 2 and month 3 postoperatively. One month postoperatively, CST improved significantly compared to baseline (p < 0.001) and the improvement was sustained at month 2 (p < 0.001), while at month 3 CST started to increase, but remained significantly lower than baseline (p = 0.003). At month 3 postoperatively, 35.3% of patients presented recurrence of ME. Patients with refractory DME and cataract can safely undergo phacoemulsification when dexamethasone implant is inserted one month prior to surgery to ensure adequate control of postoperative inflammation and prevent deterioration of ME.

The Role of Disorganization of Retinal Inner Layers as Predictive Factor of Postoperative Outcome in Patients with Epiretinal Membrane.

PURPOSE: The purpose of this retrospective study was to evaluate the disorganization of the retinal inner layers (DRIL) as a potential predictive factor of postoperative visual outcome in patients with idiopathic epiretinal membrane (iERM), treated with pars plana vitrectomy (PPV). METHODS: Participants in the study were 46 consecutive patients diagnosed with iERM, who underwent PPV. Best corrected visual acuity (BCVA) measurement and spectral-domain optical coherence tomography (SD-OCT) were performed at baseline (preoperatively), and at months 3 and 6 postoperatively. DRIL and additional OCT parameters were assessed at 1-mm-wide foveal centered area. RESULTS: DRIL was observed in 47.8% of patients at baseline. There was statistically significant improvement in BCVA and central retinal thickness (CRT) between baseline and months 6 and 12 in all patients. There was a statistically significant difference in BCVA and CRT change between patients with and without DRIL at months 6 and 12 compared to baseline, showing that there was a correlation between change in BCVA or CRT and baseline DRIL. Ellipsoid zone and external limiting membrane were intact in 91.3 and 95.7%, respectively, not affecting our results. CONCLUSIONS: Baseline DRIL has been shown to be predictive of postoperative visual outcome in patients with iERM, treated with PPV.

Eccentric Macular Hole after Pars Plana Vitrectomy for Epiretinal Membrane Without Internal Limiting Membrane Peeling: A Case Report.

INTRODUCTION: Postoperative eccentric macular hole formation is an uncommon complication after pars plana vitrectomy (PPV) without internal limiting membrane (ILM) peeling for the treatment of epiretinal membrane (ERM). We present a case of eccentric macular hole formation after PPV for ERM without ILM peeling. CASE REPORT: A 68-year-old male patient presented with ERM and visual acuity of 6/24 in his left eye. He underwent 23-gauge PPV without ILM peeling for treatment of ERM. One week postoperatively the retina was attached and the epiretinal membrane was successfully removed, while visual acuity was 6/9. One month after PPV, a single eccentric retinal hole below the macula was detected using fundoscopy and subsequently confirmed by optical coherence tomography. At this time the visual acuity was 6/9 and the patient reported no symptoms. No further intervention was attempted and at the 9-month follow-up, the visual acuity and the size of the eccentric macular hole remained stable. CONCLUSION: Eccentric macular holes can be developed after PPV even without ILM peeling and are usually managed conservatively by observation.

Full-thickness macular hole formation following anti-VEGF injections for neovascular age-related macular degeneration.

PURPOSE: Macular hole (MH) is part of a group of age-related degenerative diseases characterized by pathology of vitreomacular interface. Similarly, neovascular age-related macular degeneration (nAMD) affects older patients and is a leading cause of irreversible visual loss. The purpose of this case series is to describe the development of full-thickness MH in patients with nAMD, following antivascular endothelial growth factor (anti-VEGF) treatment. METHODS: Participants in this case series were four patients with nAMD, who received anti-VEGF injections with variable therapeutic response to treatment. Patients were examined at baseline (when AMD was diagnosed) and monthly thereafter. The examination included visual acuity measurement, slit-lamp biomicroscopy, and optical coherence tomography. RESULTS: All patients were found to develop full-thickness MH within 1-4 months after the last anti-VEGF injection, even in the absence of pre-existing vitreomacular interface abnormalities in some cases. The median number of injections before the MH formation was 3. CONCLUSION: MH formation may represent an adverse effect of anti-VEGF treatment in patients with nAMD and could be also coexisting pathology with nAMD in older individuals.

Chronic Central Serous Chorioretinopathy-Like Maculopathy as Atypical Presentation of Chorioretinal Folds.

PURPOSE: The purpose of this case report was to present the clinical data and management of a 69-year-old female patient with chorioretinal folds who developed chronic central serous chorioretinopathy-like maculopathy. CASE PRESENTATION: A 69-year-old female patient presented with a recent-onset slight decrease in visual acuity in her left eye. Her past ocular history was clear. Regarding her medical history, she had only hypertension, treated with per os medications. The patient underwent a complete ophthalmological examination. At presentation, the visual acuity was 6/6 in the right eye and 6/7.5 in the left eye. Intraocular pressure was 16 mm Ηg in both eyes. On fundoscopy, chorioretinal folds were noticed in the left eye. Spectral-domain optical coherence tomography, fluorescein angiography, and indocyanine green angiography confirmed the diagnosis of chronic central serous chorioretinopathy-like maculopathy in the left eye, since other causes of chorioretinal folding were excluded. The right eye was normal. No treatment was given to the patient, and at the 6-month follow-up there was improvement in visual acuity, which was 6/6, and decreased subretinal fluid. CONCLUSIONS: Central serous chorioretinopathy-like maculopathy should be taken into account for the differential diagnosis of patients with chorioretinal folds.

A case report of Gordon's syndrome in a 20-year-old male with free medical family history.

Gordon's syndrome is a rare autosomal dominant disease that manifests in childhood. It is characterized by hypertension, hyperkalemic hyperchloremic metabolic acidosis, low renin and usually normal aldosterone levels, and it is sensitive to thiazide diuretics. A 20-year-old male with a history of diagnosed Gordon's syndrome was referred to a nephrology clinic for evaluation. The patient, who was under treatment with hydrochlorothiazide, had been diagnosed with Gordon's syndrome at the age of 11, when he presented hypertension and episodes of hyperkalemic hyperchloremic metabolic acidosis. However, none of his relatives had been diagnosed with this syndrome. Therefore, we assume that our patient might be a case of de novo gene mutation.