Eight-year multimodal follow-up of recurrent idiopathic acute exudative polymorphous vitelliform maculopathy.

PURPOSE: To describe and analyze clinical findings in a patient with recurrent idiopathic acute exudative polymorphous vitelliform maculopathy (AEPVM), followed in detail, and to propose the diagnostic and follow-up algorithm. DESIGN: Retrospective observational analysis. PATIENT: A young adult male patient diagnosed with idiopathic AEPVM who developed two relapses in a 12-month period eight years after the initial onset. METHODS: Review of clinical charts, multimodal imaging, and electrophysiology findings. The patient repeatedly underwent complete ophthalmic examinations, including best-corrected visual acuity testing (BCVA), slit-lamp and fundus examinations; digital fundus photography, time-domain optical coherence tomography (OCT) in 2009 (Stratus OCT, Carl Zeiss Meditec, USA) and spectral-domain OCT in 2017-2018 (Spectralis-OCT, Heidelberg Engineering, Germany), together with fundus autofluorescence (FAF), fluorescein angiography (FA), and indocyanine green angiography (ICGA), all with HRA2 (Heidelberg Engineering, Germany); microperimetry (MP-1 microperimeter, Nidek, Japan). Laser flare photometry (Kowa FM-600, Japan) and electrophysiology testing were also performed. MAIN OUTCOME MEASURES: Clinical features of long-lasting recurrent idiopathic AEPVM, and diagnostic and follow-up algorithm in such rare cases. RESULTS: Case report of a 25-year-old male Caucasian patient with typical features of AEPVM, including serous neuroepithelial detachment with irregular retinal elevations, ophthalmoscopically resembling retinal folds, with subsequent subretinal accumulation of characteristic yellow-white vitelliform deposits. Features in this case rarely described, or even not yet reported, include indocyanine- and fluorescein-negative intraretinal cystic changes, optic disc hyperfluorescence on FA, serous retinal elevations mimicking retinal folds, increased choroidal thickness, lack of rapid visual recovery, and very slow anatomical improvement of the relapses. Bimonthly fundus autofluorescence evaluation together with SD-OCT were the most informative diagnostic methods, demonstrating the evolution of pathological signs. CONCLUSION: AEPVM may be a recurrent or even chronic condition with uncertain long-term visual outcomes. It may have variable clinical presentations depending on the stage of the disease, and both clinical manifestations and imaging features of different stages of the pathologic process may overlap. Patients should be made aware that visual improvement occurs very slowly, if at all. Bimonthly fundus autofluorescence evaluation together with SD-OCT should be recommended in such cases.

Progressive retinal ganglion cell loss in primary open-angle glaucoma is associated with temperature circadian rhythm phase delay and compromised sleep.

Advanced primary open-angle glaucoma (POAG) is characterized by progressive retinal ganglion cell complex (RGCC) damage that may cause subsequent disruption of the circadian rhythms. Therefore, we evaluated circadian body temperature (BT) rhythm and sleep characteristics of 115 individuals (38 men and 77 women) diagnosed with POAG. GLV (global loss volume; %), a measure of RGCC damage, was estimated by high-definition optical coherence tomography, and RGC functional ability was assessed by pattern electroretinogram amplitude (PERGA). Depending on dynamics of POAG progression criteria, two groups were formed that were distinctively different in GLV: Stable POAG group (S-POAG; GLV = 5.95 ± 1.84, n = 65) and Progressive POAG group (P-POAG; GLV = 24.27 ± 5.09, n = 50). S-POAG and P-POAG groups were not different in mean age (67.61 ± 7.56 versus 69.98 ± 8.15) or body mass index (24.66 ± 3.03 versus 24.77 ± 2.90). All subjects performed 21 around-the-clock BT self-measurements during a 72-h period and kept activity/sleep diaries. Results showed pronounced disruption of circadian physiology in POAG and its progression with increasing severity of the disease. The daily mean of BT was unusually low, compared to age-matched controls. Moreover, our results revealed distinctive features of BT circadian rhythm alterations in POAG development and POAG progression. S-POAG is associated with lowered BT circadian rhythm robustness and inter-daily phase stability compared to controls. In the P-POAG group, the mean phase of the circadian BT rhythm was delayed by about 5 h and phases were highly scattered among individual patients, which led to reduced group mean amplitude. Circadian amplitudes of individuals were not different between the groups. Altogether, these results suggest that the body clock still works in POAG patients, but its entrainment to the 24-h environment is compromised. Probably because of the internal desynchronization, bedtime is delayed, and sleep duration is accordingly shortened by about 55 min in P-POAG compared to S-POAG patients. In the entire POAG cohort (both groups), later sleep phase and shorter mean sleep duration correlate with the delayed BT phase (r = 0.215; p = 0.021 and r = 0.322; p = 0.0004, respectively). An RGCC GLV of 15% apparently constitutes a threshold above which a delay of the circadian BT rhythm and a shortening of sleep duration occur.

Mean intraocular pressure and progression based on corneal thickness in patients with ocular hypertension.

PURPOSE: To determine the incidence of glaucomatous progression at mean intraocular pressure (IOP) levels in patients with ocular hypertension (OHT). METHODS: A retrospective, multicentre, cohort analysis of 230 OHT patients with 5 years of follow-up evaluated for risk factors associated with progressive optic disc and visual field loss to determine the incidence of glaucomatous progression. RESULTS: Forty percent of patients with IOPs > or = 24 mmHg, 18% of patients with IOPs of 21-23 mmHg, 11% of patients with IOPs with 18-20 mmHg, and 3% of patients with IOPs of < or = 17 mmHg progressed to glaucoma. The mean IOP was 19.8+/-2.4 mmHg in the stable group and 21.7+/-2.6 mmHg in the progressed group (P=0.0004). The highest average peak IOP was 23.4+/-4.0 mmHg in the stable group and 25.2+/-3.1 mmHg in the progressed group (P=0.006). Based on the pachymetry values for central corneal thickness, patients with thinner corneas more often progressed to glaucoma (P<0.0001). A multivariant regression analysis to determine risk factors for progression was positive primarily for higher peak IOPs, older age, male gender, argon laser trabeculoplasty, visual acuity > or = 20/50, and no topical medical therapy or beta-blocker therapy prior to the study. CONCLUSIONS: IOP reduction within the normal range over 5 years of follow-up reduces the chance of progression to primary open-angle glaucoma in OHT patients.

Long-term progression at individual mean intraocular pressure levels in primary open-angle and exfoliative glaucoma.

PURPOSE: To describe progression and non-progression rates at individual mean intraocular pressure (IOP) levels for patients with primary open-angle and exfoliative glaucoma. METHODS: A meta-analysis of five previously published retrospective studies describing progression and non-progression rates at individual intraocular pressure levels over 5 or more years of follow-up. All patients had primary open-angle (four studies) or exfoliative glaucoma (one study). RESULTS: This meta-analysis included 822 patients of whom 655 (80%) had primary openangle glaucoma and 167 (20%) had exfoliative glaucoma. In total, 220 patients progressed (27%), while 602 (73%) remained stable over 5 years. The mean IOP was 20.0 for progressed and 17.1 mmHg for stable patients (p=0.0004). The peak IOP was 29.1 for progressed and 23.6 mmHg for stable patients (p=0.0014). At an IOP level >18 mmHg, 49% of patients remained stable; at 18 mmHg, 78%; between 13 and 17 mmHg, 82%; and <13 mmHg, 96%. Additional factors associated with progression were older age (p=0.0004) and exfoliative glaucoma (p=0.0001). However, multivariant regression analysis identified only mean IOP as a risk factor for progression (p=0.039). CONCLUSIONS: This study suggests that maintaining an IOP well within the normal range over 5 years in patients with primary open-angle or exfoliative glaucoma helps to prevent glaucomatousprogression.

Presentation and long-term follow-up of exfoliation glaucoma in Greece, Spain, Russia, and Hungary.

PURPOSE: To evaluate clinical presentation and long-term follow-up of exfoliation glaucoma in separate European population groups. METHODS: A four-center, retrospective, case series analysis in which 200 charts of patients with exfoliation glaucoma or patients with elevated intraocular pressure (IOP) associated with exfoliation syndrome in at least one eye with at least 5 years of follow-up were consecutively reviewed. RESULTS: This study found an average follow-up time of 6.0+/-2.1 years. Patients in Hungary and Spain statistically presented at an older age (79 years) than Greek patients (67 years). Patients with exfoliation glaucoma in Greece and Hungary had more glaucomatous damage, had more severe glaucoma, had a higher untreated IOP (31.8 to 32.1 mmHg), and were more difficult to control, showing a greater number of changes in medicines during the follow-up period, a greater number of medicines at the end of the follow-up period, and more progression. On long-term follow-up, Greek, Russian, and Hungarian patients also had the highest mean IOP (18.8 to 20.8 mmHg) and the greatest incidence of progression (approximately 50%). Spanish patients demonstrated the lowest mean IOP (17.6+/-3.6 mmHg) and the lowest rate of progression (28%) during the follow-up period and the fewest number of medications per patient (0.7) to control the IOP at the end of the follow-up period. CONCLUSIONS: The severity of exfoliation glaucoma presentation and its course may differ within distinct geographic populations in Europe.