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Objectives To validate cut-off values of quantitative high-resolution temporal artery compression sonography (TCS) for the diagnosis of cranial GCA (cGCA) in patients with acute arterial ocular occlusions and in an independent control group. Methods Consecutive patients who underwent TCS as part of the diagnostic workup of acute arterial ocular occlusions and controls not suffering from ocular ischaemia/systemic vasculitis were included. The diagnostic accuracy of the established TCS cut-off value of maximum temporal artery wall thickness (≥0.7 mm) and a novel numeric TCS score incorporating the degree of wall thickening in the four temporal artery segments assessed (0–3 points per segment) was tested by receiver operating characteristics analysis. Subgroup analyses were performed for female and male patients and patients older and younger than age of 70 years. Results Of 114 patients with acute ocular arterial occlusions, 30 patients received a final clinical diagnosis of cGCA. The sensitivity and specificity of the ≥0.7 mm TCS cut-off for the diagnosis of cGCA were 100 and 84.5% in the overall cohort. The TCS score did not improve the diagnostic yield (cut-off ≥5; sensitivity 100%, specificity 85.7%). In male patients >70 years of age, the specificity of TCS was limited, secondary to age- and sex-related differences in temporal artery wall thickness, which we confirmed in the independent control group. Conclusion TCS yields high diagnostic accuracy in the diagnosis of cGCA in patients with acute ocular arterial occlusions. Age- and sex-related differences in temporal artery wall thickness influence the diagnostic accuracy of TCS.

Was ist neu? Zentralarterienverschluss (ZAV) und ischämische Optikusneuropathie (ION) Ischämische Erkrankungen der Retina bei ZAV sowie des Sehnervs bei ION sind häufige Ursachen von irreversiblem Sehverlust bei älteren Patienten und bedürfen einer intensiven internistischen Mitbetreuung. Internistische Diagnostik bei ZAV und ION Im ersten Schritt sollte eine Riesenzellarteriitis (RZA) gesichert bzw. ausgeschlossen werden. Die internistische Diagnostik von nichtarteriitischem ZAV und nichtarteriitischer ION (nAION) zielt insbesondere auf die Erfassung des kardiovaskulären Risikoprofils ab. Beim nichtarteriitischen ZAV ist die Emboliequellensuche zentrale diagnostische Aufgabe. Bei Patienten mit nAION sollte ein Schlafapnoe-Screening erfolgen. Transorbitale Sonografie Bei Darstellung eines Spot-Zeichens im Sehnervenkopf kann ein embolischer ZAV gesichert werden. Ein Spot-Zeichen schließt eine arteriitische Genese eines ZAV aus.

ZusammenfassungDrei Wochen nach komplikationsloser kombinierter minimal-invasiver Glaukom- und Kataraktoperation kam es zu einer Sehverschlechterung durch die Eintrübung der Intraokularlinse. Durch die Nichtanwendung der postoperativen antiinflammatorischen Lokaltherapie kam es zu einer homogenen Fibrinbildung auf der Linsenvorderfläche sowie einer beginnenden Vernarbung des Sickerkissens. Wir führten ein Needling mit 5‑Fluorouracil sowie eine Linsenpolitur mit dem Nd:YAG-Laser durch und erreichten so eine suffiziente Druckkontrolle und Sehverbesserung.

ObjectiveTo determine the association of arteriosclerosis, characterised by hyperechogenic intimal lesions (HIL), with wall thickness of the temporal and facial arteries in elderly patients with ocular arterial occlusions.MethodsPatients suffering from non-arteritic ocular perfusion disorders were included. High-resolution compression sonography (18 MHz) images of the temporal arteries (frontal and parietal branch at the upper margin of the auricle) and facial arteries (at the crossing point of the artery over the mandible) were analysed for the presence of HIL (grade 0: absent; grade 1: moderate; grade 2: severe). Characteristics of patients with and without evidence of HIL >grade 1 were compared.ResultsIn total, 330 cranial artery segments of 55 patients were analysed. HIL ≥grade 1 was present in 13.0% of all artery segments and in 38.1% of all patients. Patients with HIL ≥grade 1 in at least one arterial segment displayed significantly increased maximum wall thickness of the temporal arteries (0.62±0.23 mm vs 0.50±0.13 mm; pConclusionThe presence of HIL goes along with a significantly increased wall thickness of the temporal and facial arteries. These findings should be considered when interpreting the results of sonography of the cranial arteries in the diagnostic workup of suspected giant cell arteritis.

Purpose To compare the safety and efficacy of microshunt implantation augmented with Mitomycin C in patients with pseudoexfoliation glaucoma (PEXG) and primary open-angle glaucoma (POAG). Methods In this retrospective, single centre, interventional study, 46 eyes of 41 patients with PEXG (20 eyes) and POAG (26 eyes) underwent microshunt implantation. Definition of failure was an intraocular pressure (IOP) lower than 5 or higher than 17mmHg on two consecutive visits, an IOP reduction lower than 20% on two consecutive visits, the need of surgical revisions or reoperations or loss of light perception. Outcome was rated as complete success if achieved without medication, otherwise as qualified success. Furthermore, postoperative complications and interventions were compared between the two groups. Results Patient demographics were similar, except for older age in the PEXG group (70.9±8.6 versus 77.6±8; p = 0.02). Mean IOP dropped from 21.5±5.8mmHg (PEXG) and 18.2±4.5mmHg (POAG) at baseline to 12.8±3.0mmHg (ppppp = 0.04) and choroidal detachment (p = 0.03) in the PEXG group. Conclusion Microshunt implantation demonstrated similar efficacy results in PEXG and POAG eyes at a follow-up of 12 months. Higher rates of transient hypotony and choroidal detachment were observed in PEXG eyes.

Pseudoexfoliation (PEX) syndrome is a common, worldwide occurring and age-related disorder characterised by the deposition of extracellular fibrillar material. It is a systemic disease with significant ocular manifestations, including cataract and glaucoma. All structures of the anterior segment are affected leading to multiple specific findings in slit lamp examination. The most prominent findings are white, dandruff like deposits on the anterior surface of the lens or the pupillary border of the iris. Furthermore, PEX syndrome is the most common identifiable cause of open angle glaucoma. PEX glaucoma generally takes a more rapidly progressive course than primary open angle glaucoma. Patients therefore typically need earlier surgical intervention. PEX syndrome is often accompanied by nuclear cataract formation. Cataract extraction tends to be complicated and remains a challenge. Several technique modifications and new devices help to reduce the complication rate. The purpose of this paper is to summarize information regarding ocular manifestations of PEX-syndrome to facilitate an early diagnosis and to present a general view of the treatment of PEX glaucoma.

Although the introduction of BRAF and MEK inhibitors has greatly enhanced treatment possibilities in advanced BRAFV600-mutated melanoma, class-related toxicities are rather frequent and often involve the eye. Ophthalmologic side effects most commonly include central/diffuse serous retinopathy and retinal vein occlusion. Affection of the optic nerve head however has not been described clinically.A 29-year-old man presented in our eye clinic with bilateral blurred vision. Seventeen days earlier, he had been started on trametinib and dabrafenib combination therapy for metastasized melanoma of unknown origin. Visual field testing revealed diffuse bilateral defects, which regressed spontaneously on pause of MEK and BRAF inhibitor treatment.In addition to the widely known class-related retinal toxicity, MEK and BRAF inhibitor-associated adverse events may also involve the optic nerve head, causing visual field defects probably regressing spontaneously after discontinuation of targeted oncologic therapy. In such cases, repeat brain imaging and exclusion of melanoma-associated retinopathy is recommended. Reinitiation of treatment and subsequent dose escalation seem to be feasible, but should be monitored by an ophthalmologist.

Pigment dispersion syndrome (PDS) is a disorder predominantly affecting young, myopic adults. It is characterised by the liberation of pigment of the iris and deposition of it on various structures of the anterior segment leading to multiple specific findings in slit lamp examination. Typical alterations are a deposition of pigment on the central corneal endothelium, circular iris transillumination defects in the mid periphery, a posterior bowing of the iris and increased pigmentation of the trabecular meshwork. Findings are usually bilateral symmetric. Posterior bowing of the iris causes rubbing of the pigmented iris epithelium against lens structures like zonular fibres with a consecutive liberation of pigment. Trabecular meshwork changes because of pigment deposition reduce aqueous outflow facility with the risk of elevated intraocular pressure and glaucoma. Pigmentary glaucoma (PG) is a secondary open angle glaucoma. Treatment of PG is similar to primary open angle glaucoma, including medical therapy, laser therapy and surgery. Peripheral laser iridotomy was shown to change iris configuration but its efficacy in the prevention of PG has not been confirmed in the literature. The purpose of this paper is to summarize information regarding ocular manifestations of PDS to facilitate an early diagnosis and to present a general view of the treatment of PG.

Background: Risk stratification based on pre-test probability may improve the diagnostic accuracy of temporal artery high-resolution compression sonography (hrTCS) in the diagnostic workup of cranial giant cell arteritis (cGCA). Methods: A logistic regression model with candidate items was derived from a cohort of patients with suspected cGCA (n = 87). The diagnostic accuracy of the model was tested in the derivation cohort and in an independent validation cohort (n = 114) by receiver operator characteristics (ROC) analysis. The clinical items were composed of a clinical prediction rule, integrated into a stepwise diagnostic algorithm together with C-reactive protein (CRP) values and hrTCS values. Results: The model consisted of four clinical variables (age &gt, 70, headache, jaw claudication, and anterior ischemic optic neuropathy). The diagnostic accuracy of the model for discrimination of patients with and without a final clinical diagnosis of cGCA was excellent in both cohorts (area under the curve (AUC) 0.96 and AUC 0.92, respectively). The diagnostic algorithm improved the positive predictive value of hrCTS substantially. Within the algorithm, 32.8% of patients (derivation cohort) and 49.1% (validation cohort) would not have been tested by hrTCS. None of these patients had a final diagnosis of cGCA. Conclusion: A diagnostic algorithm based on a clinical prediction rule improves the diagnostic accuracy of hrTCS.

Purpose To characterize the diagnostic yield of the spot sign in the diagnostic workup of acute arterial occlusions of the eye in elderly patients. Methods Clinical characteristics of consecutive patients aged ≥ 50 years with acute central retinal artery occlusion (CRAO), branch retinal artery occlusion (BRAO) or anterior ischemic optic neuropathy (AION) were recorded. Videos of transocular sonography were assessed for the presence of the spot sign by two blinded readers. Group comparisons were made between CRAO-patients with and without the spot sign. Two experienced cardiovascular physicians allocated CRAO-cases to a presumed aetiology, without and with knowledge on the presence/absence of the spot sign. Results One-hundred-twenty-three patients were included, 46 of whom suffered from CRAO. A spot sign was seen in 32 of 46 of patients with CRAO and in 7 of 23 patients with BRAO. Interobserver agreement was excellent (Cohen`s kappa 0.98). CRAO-patients with the spot sign significantly more frequently had a medical history of cardiovascular disease (62.8 vs. 21.4%, p = 0.03) and left heart valve pathologies (51.9 vs. 10%, p = 0.03). The spot sign was not found in any of the three patients with CRAO secondary to cranial giant cell arteritis. The assumed CRAO aetiology differed in 37% of cases between two cardiovascular physicians, regardless whether transocular sonography findings were known or not. Conclusion The spot sign is a simple sonographic finding with excellent interobserver agreement, which proofs the embolic nature of CRAO, but does not allow exact attribution of the underlying aetiology.

A 78-year-old man suffered sudden visual loss of his right eye. Five years earlier, he had experienced vision loss of his left eye due to central retinal artery occlusion (CRAO); back then, the etiology for the CRAO was not established. Current ocular ultrasound depicted a hyperechoic spot within the optic nerve in both eyes. Echocardiography identified a calcified mass adherent to the mitral valve as the embolic source of the CRAO. This case shows the value of ocular B-mode ultrasound in demonstration and proof of the etiology for CRAO.

PURPOSE To implant a small-aperture intraocular lens (IOL) (IC-8) in eyes with severe corneal irregularities to reduce higher-order aberrations and provide better central visual acuity. SETTING University Hospital, LMU Munich, Germany. DESIGN Prospective nonrandomized interventional case series. METHODS Eyes with severe corneal irregularities due to keratoconus, previous penetrating keratoplasty, status postradial keratotomy, or scarring after ocular trauma were enrolled. Exclusion criteria were progressive keratoconus, pseudoexfoliation, glaucoma, maculopathy, reduced endothelial cells (

Purpose To investigate preoperative ocular risk factors and indications for secondary intraocular lens (IOL) implantation and compare postoperative complications, visual and refractive outcomes in a tertiary referral center. Methods Patients older than 14 years that underwent secondary IOL implantation and had a minimum follow-up of 3 months were enrolled in this retrospective case series. Preoperative ocular risk factors, indications for surgery, postoperative complications, and visual and refractive outcomes including prediction error (PE) and absolute error (AE) were evaluated. IOLs were fixated in following positions: anterior chamber (AC), retropupillary iris-claw (IC), sulcus, and capsular bag or sclera. Results One-hundred eighty-two eyes of 174 patients with mean follow-up of 17 ± 13.6 months were evaluated. Leading cause for surgery was IOL dislocation (75%), followed by secondary aphakia (19%) and IOL opacifications (6%). Previous vitrectomy was the major preoperative ocular risk factor (43%). Mean corrected distance visual acuity improved from preoperative 0.68 ± 0.55 to 0.42 ± 0.31LogMAR by the last follow-up (p = 0.001). PE and AE differed highly depending on the indication for surgery (p = 0.041 and p = 0.008, respectively) and the IOL fixation (p = 0.011 and p = 0.028, respectively), with IC-IOLs showing the lowest PE and AE. Postoperative AC-hemorrhage occurred mainly after IC-IOLs (p = 0.003), and postoperative hypotony was significantly higher in eyes with previous uveitis (p = 0.026). Conclusions Previous vitrectomy seems to be a major underreported risk factor in eyes that undergo secondary IOL implantation. Refractive outcomes depend on indication for surgery and fixation type, with retropupillary IC-IOLs providing the best refractive results, though not statistically significant compared to other IOL positions.

The purpose of this study was to determine the quantity and distribution of antigen presenting cells (APC) in various inflammatory and non-inflammatory corneal diseases, comparing in vivo confocal microscopy (IVCM) and immunohistochemistry.Corneae of 41 eyes, composed of group 1 (status post herpes-keratitis), group 2 (keratoconus) and group 3 (graft rejection after keratoplasty) were investigated. IVCM was used preoperatively to assess the distribution and density of dendritic cells in the corneal center versus the paracentral area. Afterwards, all patients underwent penetrating keratoplasty. The host corneas were analyzed by immunohistochemistry for antigen presenting cell distribution, density and characterization by using specific markers for CD207/Langerin, CD209/DC-SIGN and HLA-DR. The IVCM findings were compared with immunohistochemistry results in the corneal epithelium.Cells with branching dendritic morphology were visualized by IVCM mainly in the basal epithelial layer and subepithelial nerve plexus of the central and paracentral cornea. The density of APC in IVCM decreased in all groups towards the central part of the cornea. The highest gradient was observed in group 2, followed by groups 1 and 3. The corneal paracenter showed similiar distribution of APC in group 1 and 2 (76.7 cells/mm(2) and 74.4 cells/mm(2)). The highest density of central APC was observed in group 1 (53.76 cells/mm(2)), followed by group 3 (27.0 cells/mm(2)) and group 2 (24.2 cells/mm(2)). In immunohistochemistry positive stained, APC were distributed similarly to IVCM but with a higher density (p0.05).Distribution, density and stage of maturation of corneal epithelial APCs can be evaluated on morphological basis by IVCM. However, the corneal APCs density was about three-fold lower compared to immunohistochemistry findings.

Corneal cross-linking is widely used to treat keratoconus. However, to date, only limited data from randomized trials support its efficacy. The efficacy and safety of corneal cross-linking for halting progression of keratoconus were investigated in a prospective, randomized, blinded, placebo controlled, multicentre trial. Twenty-nine keratoconus patients were randomized in three trial centres. The mean age at inclusion was 28 years. Longitudinal changes in corneal refraction were assessed by linear regression. The best corrected visual acuity, surface defects and corneal inflammation were also assessed. These data were analysed with a multifactorial linear regression model. A total of 15 eyes were randomized to the treatment and 14 to the control group. Follow-up averaged 1098 days. Corneal refractive power decreased on average (+/−standard deviation) by 0.35 +/− 0.58 dioptres/year in the treatment group. The controls showed an increase of 0.11 +/− 0.61 dioptres/year. This difference was statistically significant (p = 0.02). Our data suggest that corneal cross-linking is an effective treatment for some patients to halt the progression of keratoconus. However, some of the treated patients still progressed, whereas some untreated controls improved. Therefore, further investigations are necessary to decide which patients require treatment and which do not. NCT00626717 , Date of registration: February 20, 2008.

To analyze the appearance of conjunctival pigmented tumors as seen by in vivo confocal microscopy. Twenty-eight pigmented conjunctival tumors including 6 nevi, 13 acquired melanoses, 7 conjunctival melanomas, and 2 extrascleral growths of uveal melanomas were examined by in vivo confocal microscopy using the Heidelberg Retina Tomograph (HRTII)/Rostock Cornea Modul (RCM). Confocal images were analyzed using predefined criteria by an observer masked to final histological diagnosis and a preliminary diagnosis was established. After excision, histology and immunohistochemistry using antibodies against S-100, Melan-A, HMB-45, Ki-67, CD3, and CD68 were performed in all specimens and compared with in vivo confocal images of the same lesions. Confocal microscopy images confirmed typical histopathological features of conjunctival pigmented tumors. Nest or diffuse collections of medium-sized uniform hyper- or hyperreflective cells in the stroma and stromal cysts lined with a multilayered epithelium were visible in 100% of conjunctival nevi. Small dendritic cells were typically observed in 100% of primary acquired melanoses (PAM) without atypia and in 2 out of 6 nevi. Large networks of hyperreflective dendritic cells were present in 100% of PAM with atypia. Whereas images of PAM without atypia and secondary complexion-associated melanosis showed hyperreflective granules confined to the basal epithelium in 67% of lesions, PAM with atypia presented with hyperreflective granules and patches throughout the epithelium in all cases. Malignant melanomas of the conjunctiva and extrascleral growths of uveal melanomas demonstrated large hyperreflective cells with prominent nuclei and nucleoli. In vivo confocal microscopy showed a sensitivity of 89% and a specificity of 100% to establish the correct diagnosis of conjunctival melanoma compared with histology. High correlations were found between in vivo confocal microscopy using near-infrared laser light and histology in the diagnosis of pigmented conjunctival lesions. In vivo confocal microscopy seems to be a valuable new tool in the differential diagnosis and follow-up of pigmented conjunctival tumors. It does not replace histology, but may assist in performing guided biopsy in tumors suspected clinically and/or with in vivo microscopy. In addition, in vivo confocal microscopy may support the clinical diagnosis of extrascleral involvement in uveal melanoma.

A dense cataract prevents detailed fundus examination which may lead to delayed diagnosis of undiscovered intraocular pathology before cataract surgery. We report two cases where large choroidal melanomas were diagnosed after cataract surgery and/or Nd:Yag laser capsulotomy. In the first case, a dense cataract prevented proper examination of the fundus of an 84-year-old female. A brownish choroidal lesion was seen after cataract surgery and diagnosed as postoperative choroidal detachment. As this lesion persisted over months, the patient was sent to our hospital for evaluation where a diagnosis of choroidal melanoma with extrascleral growth was made and an enucleation with implantation of a dermis-fat graft was performed without complication 10 days later. In the second case, a 58-year-old female patient had Nd:Yag laser treatment 2 years after cataract surgery had been performed. During fundus examination 1 day after treatment, the ophthalmologist noticed a choroidal lesion which he suspected to be a reaction to the laser treatment and referred the patient to our hospital where a diagnosis of a mushroom-shaped choroidal melanoma with serous retinal detachment was made. These cases show that preoperative evaluation of the posterior segment prior to cataract surgery or other therapeutic interventions is essential in order to identify any pathology. In case of a dense cataract, additional imaging such as ultrasound sonography is recommended in order to exclude intraocular lesions and to visualize the optic disc in suspected glaucoma.