UNASSIGNED: Elevated intracranial pressure (ICP) is a serious and potentially life-threatening condition, for which clinically useful non-invasive measures have been elusive, in some cases due to their inadequate sensitivity and specificity. Our aim was to evaluate novel non-invasive ophthalmic imaging of selected pathological features seen in elevated ICP, namely peripapillary hyperreflective ovoid mass-like structures (PHOMS), peripapillary wrinkles (PPW) and retinal folds (RF) as potential biomarkers of elevated ICP.
UNASSIGNED: This single-center pilot study included subjects with untreated or incompletely treated high ICP. The retinas of these subjects were evaluated with averaged en-face optical coherence tomography (OCT), OCT retinal cross-sections (OCT B-scans), adaptive optics scanning light ophthalmoscopy (AOSLO), and fundus photos.
UNASSIGNED: Seven subjects were included in the study. 6 subjects with high ICP (5 idiopathic intracranial hypertension, 1 medication induced, 30.8 ± 8.6 years, 75% female, 5 with papilledema) and 1 control (20-25 years) were included. PHOMS, PPW and RF were present in all subjects with papilledema, but neither in the high ICP subject without papilledema nor in the control subject. Averaged en-face OCT scans and AOSLO were more sensitive for PPW and RF than OCT B-scans and commercial fundus photos.
UNASSIGNED: PPW, RF and PHOMS volume have potential as non-invasive biomarkers of ICP. Novel imaging modalities may improve sensitivity. However, lack of automated image acquisition and processing limits current widespread adoption in clinical settings. Further research is needed to validate these structures as biomarkers for elevated ICP and improve clinical utility.

BACKGROUND: Idiopathic epiretinal membrane (iERM) is a common retinal disease in the elderly population. The exact pathogenesis of iERM is unknown. The present study aimed to evaluate the relationship between aqueous flare and morphology of iERM using swept-source optical coherence tomography (OCT).
METHODS: A consecutive series of 36 eyes of 33 patients with iERM and 109 control eyes of 109 patients were retrospectively examined. Aqueous flare measurements and OCT images were obtained on the same day. The average total retinal, inner retinal, outer retinal, and choroidal thicknesses were calculated using the thickness map mode with an Early Treatment Diabetic Retinopathy Study nine-zone grid that was divided into three zones. The maximum depth of the retinal folds in iERMs was manually measured. The correlation among flare value, maximum depth of folds, and retinal and choroidal thicknesses was evaluated. The morphological changes between the control eyes and the eyes with iERM in different stages were examined.
RESULTS: The result demonstrated a significant positive correlation between the aqueous flare value and total and inner retinal thicknesses in the early stage of iERM. There was a significant positive correlation between the maximum depth of folds and total and inner retinal thicknesses in the early stage of iERM, and the maximum depth of folds significantly increased in the advanced stage. The total and inner retinal thicknesses and proportion of inner retinal thickness significantly increased as the stage of iERM progressed.
CONCLUSIONS: The aqueous flare value was associated with retinal thickness in the early stage of iERM, which supports the idea that inflammation or breakdown of blood-ocular barrier is involved in the process of iERM formation. The maximum retinal folds increased as the stage of iERM progressed and retinal thickness increased, which indicates that the tangential force increases as the iERM stage progresses.

OBJECTIVE: To investigate epiretinal membrane (ERM) formation using en face optical coherence tomography (OCT) after vitrectomy for rhegmatogenous retinal detachment (RRD).
METHODS: We retrospectively reviewed the medical records of 64 consecutive eyes (64 patients) with RRD treated by vitrectomy without ERM and internal limiting membrane peeling. ERMs and retinal folds were detected by B-scan and en face imaging. The maximum depth of retinal folds (MDRF) was quantified using en face imaging. ERM severity was staged using B-scan imaging. Main outcome measures were ERM detection rate with B-scan and en face imaging, MDRF, ERM staging, postoperative best-corrected visual acuity (BCVA; logarithm of the minimum angle of resolution), and risk factors for ERM formation.
RESULTS: The detection rate for ERM formation was significantly higher with en face imaging (70.3%) than with B-scan imaging (46.9%; P = 0.007). There was no significant difference in postoperative BCVA between eyes with ERM formation (0.06 ± 0.26) and those without ERM formation (0.01 ± 0.14; P = 0.298). Forty of 45 (88.9%) eyes with ERM formation were classified as stage 1. Twenty-seven of 45 (60.0%) eyes with ERM formation developed parafoveal retinal folds. The mean MDRF was 27.4 ± 32.2 μm. Multiple retinal breaks and a maximum retinal break size of ≥ 2 disc diameters were significantly associated with ERM formation (P = 0.033 and P = 0.031, respectively).
CONCLUSIONS: Although ERM formation was observed in 70.3% patients after RRD repair, the formed ERM was not severe and had minimal impact on the postoperative visual acuity.

BACKGROUND: Intraoperative optical coherence tomography (iOCT) has recently been introduced for the visualization of retinal structures within the operating microscope.
OBJECTIVE: The aim of this study was to analyze the behavior of the retina and morphological features during surgery for retinal detachment with macular involvement.
METHODS: A retrospective analysis of 41 consecutive eyes with macula-off retinal detachment, which were treated with pars plana vitrectomy (PPV) using iOCT. A qualitative analysis of morphological features of the retina at defined moments during surgery was carried out.
RESULTS: The visualization of macular detachment with iOCT was successful in 63% of cases and in the other cases the height of retinal detachment surpassed the maximum depth of the scan by iOCT. With perfluorodecalin 53.7% of eyes showed subretinal fluid and 22% of eyes showed a wave-like configuration of the outer retinal layers. In 61% of the eyes persisting subretinal fluid could be detected under the final tamponade. The amount of subretinal fluid could not be detected intraoperatively with the naked eye. In one case a macular hole could be newly identified intraoperatively and in three cases macular detachment could not be found at the start of surgery, although a detachment had been expected.
CONCLUSIONS: The use of iOCT rarely leads to changes or extension of the intervention; however, it provides real-time information on intraretinal and subretinal fluid, which is sometimes in contrast to the clinical assessment. The relevance of persistent subretinal fluid and folds of the outer retinal layers after surgery remains unclear. It can be speculated whether this could serve as a prognostic factor for the postoperative outcome.
UNASSIGNED: HINTERGRUND: Seit wenigen Jahren ermöglicht die intraoperative optische Kohärenztomographie (iOCT) die Darstellung retinaler Strukturen im Operationsmikroskop. ZIEL: Das Ziel dieser Studie ist die Analyse des Verhaltens der Netzhaut und die morphologische Befundung während Operationen bei Ablatio retinae mit Makulabeteiligung.
UNASSIGNED: Retrospektive Analyse von 41 konsekutiven Augen mit Makula-off-Ablatio, welche mittels Pars-plana-Vitrektomie (ppV) unter Verwendung von iOCT behandelt wurden, durch qualitative Beurteilung der Netzhautmorphologie zu definierten Zeitpunkten im OP-Ablauf.
UNASSIGNED: In 63 % der Fälle gelang die iOCT-Darstellung der abgehobenen Makula, in den anderen Fällen überstieg die Netzhautabhebung die maximale Scantiefe der iOCT. Unter Perfluorodecalin zeigten 53,7 % der Augen noch subretinale Flüssigkeit, und 22 % der Augen zeigten eine Falte der äußeren Netzhautschichten. In 61 % der Augen konnte persistierende subretinale Flüssigkeit unter der endgültigen Tamponade festgestellt werden. Die subretinale Flüssigkeit konnte intraoperativ in dieser Menge mit bloßem Auge nicht festgestellt werden. In einem Fall konnte intraoperativ ein Makulaformen neu erkannt werden, in 3 Fällen zeigte sich zum Operationszeitpunkt eine wieder anliegende Makula.
UNASSIGNED: Die Informationen der iOCT führen eher selten zu einer Änderung oder Erweiterung des Eingriffs. Sie liefert jedoch in Echtzeit neue Informationen über intra- und subretinale Flüssigkeitsverteilung, die nicht immer der klinischen Einschätzung entsprechen. Die Bedeutung von persistierender subretinaler Flüssigkeit und die Falten der äußeren Netzhautschichten am Ende der Operation sind noch unklar. Sie könnten möglicherweise als prognostischer Faktor für das postoperative Outcome dienen.

UNASSIGNED: To report a case of posterior microphthalomos (PM) related to PRSS56 gene mutation with long term follow up with multimodal imaging findings.
UNASSIGNED: Single retrospective case report.
UNASSIGNED: A 43-year old male patient presented in 2009 with bilateral reduced vision. Clinical examination and multimodal imaging showed features consistent with posterior microphthalmos with prominent bilateral horizontal papillomacular retinal folds. Posterior pole hyperautofluorescent RPE deposits were present. Gradual worsening of visual acuity and rod and cone photoreceptor function more so on the left was demonstrated during the 8 years of follow up.
UNASSIGNED: Hyperautofluorescent RPE deposits may occur in patients with posterior microphthalmos and such patient\'s may experience only gradual disease progression over long term follow up.

OBJECTIVE: To examine the in vivo microanatomy of retinal folds and geographic lesions in dogs with acquired or inherited retinal dysplasia.
METHODS: Thirteen dogs had retinal microanatomy evaluation under general anesthesia using cSLO/sdOCT; two eyes had noninherited multifocal retinal folds, five had inherited multifocal retinal folds (drd1 or drd2), and 10 geographic retinal dysplasia. Retinas from two drd2 carrier dogs were examined by histology and immunohistochemistry (IHC) after in vivo imaging.
RESULTS: Retinal folds are the common feature of acquired focal/multifocal or geographic retinal dysplasia, are indistinguishable structurally from those associated with syndromic oculoskeletal dysplasia, and represent outer nuclear layer invaginations and rosettes visible by sdOCT. In dogs heterozygous for oculoskeletal dysplasia, the folds form clusters in a perivascular distribution along superior central vessels. IHC confirmed photoreceptor identity in the retinal folds. The geographic dysplasia plaques are not focally detached, but have inner retinal disorganization and intense autofluorescence in cSLO autofluorescence mode that is mainly limited to the geographic lesion, but is not uniform and in some extends beyond the plaques.
CONCLUSIONS: We propose that the autofluorescent characteristic of the geographic lesions is associated with an inner retinal disruption associated with perivascular or infiltrating macrophages and phagocytosis of cellular debris. As well, we suggest restructuring the examination forms to distinguish the folds that are sporadically distributed from those that have a perivascular distribution as the latter likely represent carriers for drd. In this latter group, DNA testing would be a helpful tool to provide specific breeding advice.

Familial exudative vitreoretinopathy (FEVR) is a hereditary retinal vascular disorder. Among the various clinical phenotypes of this disease, retinal folds are the primary and typical feature of FEVR. However, little is known about the clinical characteristics, genetic spectrum, or potential phenotype-genotype correlation of retinal folds. Herein, we describe and analyze the clinical and genetic characteristics of retinal folds in FEVR. Eighty-nine patients with unilateral or bilateral retinal folds were included in this study. Clinical examinations showed that the retinal folds were bilateral in 37 patients (41.6%). Various retinal abnormalities were noted in the fellow eyes in the remaining 52 patients with unilateral folds. Most of the folds were located temporally (98.4%, 124/126), and were complete (97.6%, 123/126). 67.5% (60/89) probands were genetic confirmed FEVR. 25 novel pathogenic mutations (7 in FZD4, 7 in LRP5, 1 in NDP, 4 in TSPAN12, and 6 in KIF11) were identified in 25 families. Overall, 87.5% (14/16) and 73.7%(14/19) patients with LRP5 and FZD4 mutations were with unilateral folds, respectively.Nevertheless, only 25% (2/8), 36.4%(4/11) and 16.7%(1/6) patients with NDP, TSPAN12, and KIF11 mutations were with unilateral folds. Moreover, 85.7%(12/14),100% (6/6) and 100%(8/8) of the patients with mutated TSPAN12, KIF11, and NDP genes presented with symmetry in disease staging, while 55% and 64.7% of patients with FZD4 and LRP5 mutation displayed symmetry in staging. In conclusion, the majority of retinal folds extended completely and radially in the temporal peripheral retina. Patients with causative mutations in NDP, TSPAN12, or KIF11 were more likely to have bilaterally symmetrical severe retinopathy. In contrast, patients with LRP5 and FZD4 mutations displayed a relatively milder but broader spectrum of phenotypes and a higher frequency of asymmetry.

OBJECTIVE: To determine the impact of metamorphopsia on quality of life after successful retinal detachment (RD) surgery and to determine which retinal changes are related to the most severe distortions.
METHODS: This was a prospective, observational, consecutive study.
METHODS: The study included 58 eyes of 58 consecutive patients who underwent successful RD surgery. At 6 months postoperatively, the incidence and severity of metamorphopsia were assessed by a quality-of-life questionnaire. Microstructure retinal changes were studied with spectral domain optical coherence tomography (OCT). The questionnaire score was compared with pre- and postoperative OCT findings.
RESULTS: Overall, 20 patients (34.5%) had metamorphopsia. Outer retinal folds (ORFs) and the macular status before surgery, together with postoperative ORFs and decrease in ellipsoid and/or interdigitation photoreceptor zone reflectivity, were identified as risk factors of developing metamorphopsia (relative risk, 1.7-4.8). The most severe visual distortions were associated with ORFs.
CONCLUSIONS: Metamorphopsia is a frequent occurrence after RD surgery and its impact on patients\' quality of life appears to be limited. While it may be difficult to prevent photoreceptor loss occurring after surgery, limiting postoperative ORFs may be helpful in reducing their incidence.

OBJECTIVE: To assess the presence of outer and inner retinal folds (RFs) and drop-out of the ellipsoid zone (EZ) occurring after surgical repair of macula-off rhegmatogenous retinal detachment (RRD) with different postoperative posture and preoperative use of adjuvant perfluorocarbon liquid (PFCO).
METHODS: In this prospective study, 56 eyes of 56 consecutive patients affected by RRD were subjected to 23- or 25-gauge pars plana vitrectomy (PPV). The patients were randomized in four groups (14 prone 5 hr without PFCO, 14 supine 5 hr without PFCO, 14 prone 5 hr with PFCO and 14 supine 5 hr with PFCO) and followed up with spectral domain optical coherence tomography (SD-OCT).
RESULTS: Spectral domain optical coherence tomography (SD-OCT) was recorded before surgery, at days 30 and 90 to detect the presence of outer RFs, inner RFs and drop-out of EZ and to follow their variation over time. No statistical significance was found in our groups for outer RFs, inner RFs, drop-out of EZ formation and evolution. The postoperative best-corrected visual acuity (BCVA) improved in all groups (mean preoperative BCVA 1.47 logMar ± 0.19, mean postoperative BCVA 0.27 logMar ± 0.11, p < 0.01), without statistical variations between the four groups in BCVA after surgery.
CONCLUSIONS: The use of adjuvant and variation in postoperative position did not change the risk of presenting outer RFs, inner RFs and drop-out of EZ after RRD.

OBJECTIVE: To describe the outcome of scleral buckling to treat radial retinal folds (RFs) that caused a late-onset and sudden visual decrease with impairment of the fovea.
METHODS: This is an observational case report. Ophthalmic examinations were performed preoperatively and postoperatively and included measurement of the best-corrected visual acuity (BCVA), fundus examination, fluorescein angiography, focal macular electroretinography (FM-ERG), and spectral-domain optical coherence tomography (OCT).
RESULTS: A patient, whose case was reported previously, had a superonasal retinal detachment with a dislocated fovea and good BCVA in her right eye and a sustained BCVA of 40/50 by age 17 years and 3 months. The BCVA decreased suddenly to 20/200 at age 17 years and 11 months. Fundus examinations showed micro-dislocation of the fovea to the RFs and narrowing of the RFs compared with the previous report. FM-ERG showed an almost extinguished response, and OCT images showed abnormalities of the outer nuclear and photoreceptor layers. Encircling and radial scleral buckling was performed. Four months postoperatively, the BCVA improved to 30/50, which corresponded to the recovery of the FM-ERG response and findings on the OCT images. One year and 3 months postoperatively, the BCVA recovered to 35/50 with repositioning of the fovea.
CONCLUSIONS: Simultaneous encircling and radial scleral buckling resulted in the recovery of both retinal function and structure in the current case and is a useful surgical option to treat late-onset progressive RFs due to intensification of traction.