Thinner temporal peripapillary retinal nerve fibre layer in Stargardt disease detected by optical coherence tomography.

PURPOSE: To evaluate peripapillary retinal nerve fibre layer (RNFL) thickness measured by spectral domain optical coherence tomography (OCT) in patients with Stargardt disease (STGD). METHODS: A cross-sectional, monocentric, observational case-control study. Twenty patients (39 eyes) with ABCA4 mutations graded according to the Fishman STGD classification were included. RNFL measurement was performed using Heidelberg Spectralis SD-OCT. RNFL thickness in STGD patients was compared to age-matched data of healthy individuals provided by the device's manufacturer. A manual readjustment of the optic disc-fovea angle was performed when needed. RESULTS: The mean age at first diagnosis of STGD was 22.9 years (range 9 to 50) and 39.1 years (range 18 to 74) at the time of examination. Thirty-nine percent of eyes (15 eyes) needed manual adjustment of the optic disc-fovea angle due to malfixation of the patients during OCT. The temporal quadrant corresponding to the macula showed a RNFL 16% thinner than controls (mean - 12 µm, 95%CI - 9 to -15 µm). However, global RNFL thickness did not differ from controls due to increased RNFL thickness of 12% in the nasal sectors. Duration and stage of STGD were not correlated to thinner RNFL. CONCLUSION: STGD seems to be associated with thinner peripapillary RNFL in the sector of axons projecting to the degenerated macular area. It is yet unclear as to whether this results from anterograde transneuronal degeneration of direct injury to retinal ganglion cells.

Ophthalmic Care in Nursing Homes for the Blind: A Growing Challenge. / Augenärztliche Betreuung in Blindenheimen: eine wachsende Herausforderung.

BACKGROUND: The demographic change in Germany will lead to an increase in irreversible age-related eye diseases. This will increase the need for specialised care facilities for visually impaired people. Due to reduced mobility, residents in such facilities often do not receive adequate ophthalmological care. New concepts must therefore be considered for this group of patients. One approach is to set up an ophthalmological examination unit within the facility combined with regular visits by an ophthalmologist. We now present the experience with such a model in a home for the blind. PATIENTS AND METHODS: The project was initiated in 2009. Since then there have been visits by medical staff of the Eye Center at Medical Center, University of Freiburg, every two weeks. All patient records (2010 - 2017) were reviewed systematically. The following data were extracted in a structured and anonymous way: Age at first presentation, gender, ophthalmological diagnoses and if a therapy was initiated. This data set was finally analysed descriptively. RESULTS: Out of 130 residents aged between 48 and 100 years, half were between 78 and 90 years old. The youngest resident was 48, the oldest 100 years old. The median visual acuity was 0.2. Sixty percent of the residents had at least mild visual impairment according to the WHO (visual acuity < 0.5; category 1 - 6). In one of 6 - 7 residents, visual acuity could not be determined using Snellen charts. The most frequent ophthalmological diagnoses included cataract (44%), age-related macular degeneration (36%) and glaucoma (29%). In 67 residents (52%), the ophthalmological examination lead to treatment, such as application of local therapy or planning an operation. CONCLUSION: In every second resident, the ophthalmologist's visit lead to treatment during the observation period. This underlines the difficulty of providing ophthalmological care even in specialised institutions for the blind and visually impaired, which is possibly due to the residents' mobility problems. The concept presented here has established a low-threshold, sustainable and high-quality ophthalmological service on site. These positive experiences indicate that corresponding measures may also be useful for other locations. However, in order to implement such a project on a larger scale, suitable financing and accounting modalities for the construction measures, the nursing staff and the ophthalmological procedure still need to be developed.

[Use of the Multidisciplinary Specialised Case Conference at the Freiburg Orbital Centre]. / Inanspruchnahme der multidisziplinären spezialisierten Fallkonferenz im Freiburger Orbitazentrum.

BACKGROUND: The treatment of tumors increasingly takes place in specialised interdisciplinary centres. Therapeutic decisions are usually made at case conferences. Ophthalmologists, oromaximillofacial surgeons, ENT physicians, neurosurgeons, as well as pediatricians, radiotherapists and radiologists are all involved in the treatment of orbital diseases. The aim of this article is to present the concept of a multidisciplinary case conference for orbital diseases and to analyse case numbers, indications, and the influence on the patient's therapy. METHODS: We analysed an anonymized data set of patients who presented in the case conference of the University Hospital Freiburg from 2008 to 2018 with regard to clinical diagnosis, histological diagnoses, number of surgical interventions, and number of interdisciplinary therapy decisions. RESULTS: From 2008 to 2018, 545 patients were presented in a weekly conference. Of these, 453 were available for anonymous evaluation. The median age was 56 years (quartiles 41; 69). The most frequent indication was an orbital tumour of unclear malignancy (n = 52; 11%). Further indications included Grave's orbitopathy (n = 39; 9%), orbital pseudotumour (n = 36; 8%), cranial nerve palsy (n = 22; 5%), and orbital lymphoma (n = 22; 5%). The most frequent histological diagnoses were B-cell lymphoma (n = 10; 2%), venous malformation (cavernoma, n = 8; 2%), marginal zone lymphoma (n = 8; 2%), and squamous cell carcinoma (n = 6; 1%). An interdisciplinary therapeutic approach was defined for 174 patients. CONCLUSION: A high demand for the interdisciplinary case conference was demonstrated. The high rate of primary or secondary interdisciplinary decisions indicates the value of such a conference. Hence, the patient is spared multiple examinations in the individual specialist areas and quick and effective therapy decisions can be achieved.

A Pilot Study on the Efficacy and Safety of 0.01% Atropine in German Schoolchildren with Progressive Myopia.

INTRODUCTION: Although the interest is growing in topical low-dose atropine to control myopia in schoolchildren worldwide, its use in children of European ancestry remains controversial and solid evidence is sparse. The Oxford Centre for Evidence Based Medicine (OCEBM) classifies the evidence for this therapy as level I for East Asian populations, but only level IV in non-Asian populations. METHODS: Fifty-six children, aged a median of 11 years (range 6-17), were analysed after 12 months of topical treatment with 0.01% preservative-free atropine in both eyes at bedtime every day. Efficacy was assessed during treatment every 6 months. In a subset of 20 patients, treatment of the second eye was delayed by 1 day to enable a controlled safety assessment of side effects such as pupil dilation, hypoaccommodation, and near vision reduction. RESULTS: Prior to treatment, the mean myopic progression was estimated as 1.05 D/year; after 12 months of treatment with 0.01% atropine, it was 0.40 D/year (p < 0.0001). The only consistently measurable side effect was the induction of 1 mm pupil dilatation, which was only noticeable in comparison to the non-treated eye during the safety investigation. CONCLUSIONS: Topical low-dose atropine appears to be safe and efficacious also in a cohort of European schoolchildren. These data should motivate researchers to conduct more randomised clinical trials.

[Is the Patient Record Sufficient for Prescribing Low-Vision Magnification Aids or Is a Consultation in a Low-Vision Clinic Required?] / Ist die Verordnung von vergrößernden Sehhilfen anhand der Krankenakte möglich oder bedarf es einer Beratung in einer Sehbehindertenambulanz?

BACKGROUND: Many visually impaired and blind patients regain reading ability by using magnification aids. They are tested and prescribed in a time-intensive consultation at our low-vision clinic. Thus, the question arises if it is possible to automate the selection and prescription of low-vision aids only on the basis of the patients' records. METHODS: The following parameters were extracted from the database of medical records from the low-vision clinic between 2001 and 2006: diagnosis, visual acuity, magnification need, prescribed low-vision aid and prescription request. The prescriptions (optic magnifiers, electronic magnifiers and closed-circuit television) were predicted by statistical models and compared with the real prescription. RESULTS: The database research provided the records of 1203 patients. Sixty-four percent were female and the median age was 80 years. The prescriptions consisted of 29% closed-circuit televisions, 11% electronic magnifiers, 34% optic magnifiers and 26% other aids or no aids. Overall, the statistically predicted prescription matched the real prescription of the magnification aids in only 73% of the cases. The magnification need was the most significant statistical factor. CONCLUSION: The reliability of an automated prescription of low-vision magnification aids on the basis of the patient record was not sufficient to implement this in the standard clinical process. Thus, an individual and personal consultation with detailed testing of magnification aids is important. In addition to the prescription of low-vision magnification aids, a psycho-social consultation is also provided in a low-vision clinic.

Neuroprotection and neuroregeneration of retinal ganglion cells after intravitreal carbon monoxide release.

PURPOSE: Retinal ischemia induces apoptosis leading to neurodegeneration and vision impairment. Carbon monoxide (CO) in gaseous form showed cell-protective and anti-inflammatory effects after retinal ischemia-reperfusion-injury (IRI). These effects were also demonstrated for the intravenously administered CO-releasing molecule (CORM) ALF-186. This article summarizes the results of intravitreally released CO to assess its suitability as a neuroprotective and neuroregenerative agent. METHODS: Water-soluble CORM ALF-186 (25 µg), PBS, or inactivated ALF (iALF) (all 5 µl) were intravitreally applied into the left eyes of rats directly after retinal IRI for 1 h. Their right eyes remained unaffected and were used for comparison. Retinal tissue was harvested 24 h after intervention to analyze mRNA or protein expression of Caspase-3, pERK1/2, p38, HSP70/90, NF-kappaB, AIF-1 (allograft inflammatory factor), TNF-α, and GAP-43. Densities of fluorogold-prelabeled retinal ganglion cells (RGC) were examined in flat-mounted retinae seven days after IRI and were expressed as mean/mm2. The ability of RGC to regenerate their axon was evaluated two and seven days after IRI using retinal explants in laminin-1-coated cultures. Immunohistochemistry was used to analyze the different cell types growing out of the retinal explants. RESULTS: Compared to the RGC-density in the contralateral right eyes (2804±214 RGC/mm2; data are mean±SD), IRI+PBS injection resulted in a remarkable loss of RGC (1554±159 RGC/mm2), p<0.001. Intravitreally injected ALF-186 immediately after IRI provided RGC protection and reduced the extent of RGC-damage (IRI+PBS 1554±159 vs. IRI+ALF 2179±286, p<0.001). ALF-186 increased the IRI-mediated phosphorylation of MAP-kinase p38. Anti-apoptotic and anti-inflammatory effects were detectable as Caspase-3, NF-kappaB, TNF-α, and AIF-1 expression were significantly reduced after IRI+ALF in comparison to IRI+PBS or IRI+iALF. Gap-43 expression was significantly increased after IRI+ALF. iALF showed effects similar to PBS. The intrinsic regenerative potential of RGC-axons was induced to nearly identical levels after IRI and ALF or iALF-treatment under growth-permissive conditions, although RGC viability differed significantly in both groups. Intravitreal CO further increased the IRI-induced migration of GFAP-positive cells out of retinal explants and their transdifferentiation, which was detected by re-expression of beta-III tubulin and nestin. CONCLUSION: Intravitreal CORM ALF-186 protected RGC after IRI and stimulated their axons to regenerate in vitro. ALF conveyed anti-apoptotic, anti-inflammatory, and growth-associated signaling after IRI. CO's role in neuroregeneration and its effect on retinal glial cells needs further investigation.