Abstract: Objective: To describe vascular changes in different stages of Stargardt disease (STGD) via double swept-source optical coherence tomography angiography. Methods and analysis: Prospective, cross-sectional case-control study. Twenty-three patients (45 eyes) with ABCA4 mutations graded according to the Fishman STGD classification and 23 controls (23 eyes) were included. Two independent investigators quantified the foveal avascular zone (FAZ) in the superficial and deep capillary plexus (SCP/DCP) and the areas presenting rarefied flow and complete vascular atrophy in the outer retina to choriocapillaris (ORCC) and choriocapillaris (CC) slab. Results: The mean age at first diagnosis of STGD was 24.0 years (range 9-50) and 37.9 years (range 18-74) at the time of examination. Eleven patients were assigned to the Fishman STGD classification stage (S) 1, three to S2, eight to S3 and one to S4. The FAZ in SCP and DCP was increased in all stages compared with controls (p0.01). Areas with rarefied flow signal and vascular atrophy were detected in the ORCC and the CC layer and grew with increasing stage of disease (p0.01). The duration of disease correlated with the extent of the enlarged FAZ in the SCP/DCP and with the area of reduced flow in the ORCC and CC layer (p

Abstract: Introduction Optic neuritis leads to degeneration of retinal ganglion cells whose axons form the optic nerve. The standard treatment is a methylprednisolone pulse therapy. This treatment slightly shortens the time of recovery but does not prevent neurodegeneration and persistent visual impairment. In a phase II trial performed in preparation of this study, we have shown that erythropoietin protects global retinal nerve fibre layer thickness (RNFLT-G) in acute optic neuritis; however, the preparatory trial was not powered to show effects on visual function. Methods and analysis Treatment of Optic Neuritis with Erythropoietin (TONE) is a national, randomised, double-blind, placebo-controlled, multicentre trial with two parallel arms. The primary objective is to determine the efficacy of erythropoietin compared to placebo given add-on to methylprednisolone as assessed by measurements of RNFLT-G and low-contrast visual acuity in the affected eye 6 months after randomisation. Inclusion criteria are a first episode of optic neuritis with decreased visual acuity to ≤0.5 (decimal system) and an onset of symptoms within 10 days prior to inclusion. The most important exclusion criteria are history of optic neuritis or multiple sclerosis or any ocular disease (affected or non-affected eye), significant hyperopia, myopia or astigmatism, elevated blood pressure, thrombotic events or malignancy. After randomisation, patients either receive 33 000 international units human recombinant erythropoietin intravenously for 3 consecutive days or placebo (0.9% saline) administered intravenously. With an estimated power of 80%, the calculated sample size is 100 patients. The trial started in September 2014 with a planned recruitment period of 30 months. Ethics and dissemination TONE has been approved by the Central Ethics Commission in Freiburg (194/14) and the German Federal Institute for Drugs and Medical Devices (61-3910-4039831). It complies with the Declaration of Helsinki, local laws and ICH-GCP

Abstract: Purpose: Idiopathic intracranial hypertension (IIH) leads to optic nerve head swelling and optic atrophy if left untreated. We wanted to assess an easy to perform volumetric algorithm to detect and quantify papilledema in comparison to retinal nerve fiber layer (RNFL) analysis using optical coherence tomography (OCT). Methods: Participants with and without IIH underwent visual acuity testing at different contrast levels and static perimetry. Spectralis-OCT measurements comprised standardimaging of the peripapillary RNFL and macular ganglion cell layer (GCL). The optic nerve head volume (ONHV) was determined using the standard segmentation software and the 3.45 mm early treatment diabetic retinopathy study (ETDRS) grid, necessitatingmanual correctionwithin Bruch membrane opening. Three neuro-ophthalmologists graded fundus images according to the Frisen scale. A mixed linear model (MLM) was used to determine differences between study groups. Sensitivity and specificity was evaluated using the area under the receiver-operating characteristic (ROC). Results: Twenty-one patients with IIH had an increased ONHV of 6.46 } 2.36 mm3 as compared to 25 controls with 3.20 } 0.25 mm3 (P 0.001). The ONHV cutoff distinguishing IIH from controls was 3.97 mm3 (i.e. no patient with IIH had an ONHV below and no healthy individual above this value). The area under the curve (AUC) for ONHV was 0.99 and for the RNFL at 3.5 mm 0.90. The Frisen scale grading correlated higher with the ONHV (r = 0.90) than with the RNFL thickness (r = 0.68). ONHV measurements were highly reproducible in both groups (coefficient of variation 0.01%).

Abstract: Hintergrund Die endokrine Orbitopathie ist die häufigste extrathyreoidale Manifestation des Morbus Basedow und tritt bei schätzungsweise 25-50 % der betroffenen Patienten auf. Krankheitsbedingt kommt es zu einer entzündlichen Schwellung der Orbitaweichteile. Die Behandlung erfolgt meist konservativ. Bei schweren Fällen mit beeinträchtigendem Exophthalmus oder akuter, steroidrefraktärer Visusbedrohung kann eine chirurgische Orbitadekompression die Beschwerden der Patienten lindern oder das Sehvermögen erhalten. Ein wesentlicher Aspekt der Versorgungsqualität besteht in der Vermeidung postoperativer Doppelbilder. Ziel der Arbeit Erfahrungs- und Ergebnisbericht von 100 chirurgischen Orbitadekompressionen bei 62 Patienten an einem interdisziplinären Orbitazentrum. Patienten mit Kompression der Orbitaspitze wurden mittels pterionaler Dekompression behandelt. Patienten ohne Hinweise auf Orbitaspitzenbeteiligung wurden mittels tiefer lateraler Wandresektion oder pterionaler Dekompression behandelt. Methodik Retrospektive Datenanalyse. Ergebnisse Die mittlere Exophthalmusreduktion betrug 2,9 mm. Augen mit visusbedrohendem Schweregrad gewannen im Mittel 2,2 Zeilen an Sehschärfe, der Visus bei rehabilitativer Indikation blieb stabil. Die Komplikationsrate betrug 4 %. Neue Doppelbilder wurden nach 2 Eingriffen beobachtet. Bei einem Patienten kam es zu einer Visusminderung von 0,8 auf 0,1. In 9 Fällen führte die Operation zu einem vollständigen Rückgang zuvor beklagter Doppelbilder. Diskussion Visusgewinn, Exophthalmusreduktion und Komplikationsrate sind in diesem Kollektiv vergleichbar mit zuvor publizierten Arbeiten. Diese Studie bestätigt die Rolle der Orbitadekompression bei visusbedrohender und schwer beeinträchtigender endokriner Orbitopathie